Course 3: Cardiovascular & Pulmonary (Review)

190 terms by Baze Plus

Ready to study?
Start with Flashcards

Create a new folder

Advertisement Upgrade to remove ads

Pericarditis

Commonly presents with sharp pain, aggravated by inspiration, and relieved by sitting up and leaning forward. ECG shows PR depression and diffuse concave ST elevation. 30% of patients have a pericardial friction rub.

***Acute Pericarditis
Pleuritic chest pain
Friction rub (may be uni-, bi-, or triphasic)
ECG changes

Pneumonia

Pneumonia is associated with cough, sputum, and hemoptysis. Fever is nonspecific.

Lobar
- Streptococcus pneumoniae, or pneumococcus most frequently, Klebsiella (sputum = "red-currant jelly")
- Intra-alveolar exudate --> consolidation; may involve entire lung
- May have bronchial breath sounds over lesion
- Increased tactile fremitus
- Dullness

Bronchopneumonia
- S. aureus, H. flu, Klebsiella, S. pyogens
- Acute inflammatory infiltrates from bronchioles into adjacent alveoli; patchy distribution involving one or more lobes

Interstitial (atypical) pneumonia
- Viruses (RSV, adenovirus), Mycoplasma, Legionella, Chlamydia
- Diffuse patchy inflammation localized to interstitial areas at alveolar walls; distribution involving one or more lobes

Pulmonary Embolus

PE presents with the SUDDEN ONSET of shortness of breath and clear lungs on exam

Clue may be patient is a truck driver or returned from a long plane trip

Pneumothorax

Pneumothorax, when large, will decrease breath sounds on one side. Obstructive lung disease predisposes to pneumothoraces, particularly when there are pleural blebs with COPD.

- Unilateral chest pain with dyspnea, unilateral chest expansion, decreased tactile fremitus, hyperresonance, diminished breath sounds.

Spontaneous Pneumothorax

Accumulation of air in the pleural space. Occurs most frequently in tall, thin, young, males because of rupture of apical blebs. Trachea deviates TOWARD affected lung

Tension Pneumothorax

Usually occurs in setting of trauma or lung infection. Air is capable of entering pleural space but not exiting. Trachea deviates AWAY from affected lung. Decreased breath sounds, hyperresonance, absent tactile fremitus.

Cardiac Syncope

Cardiac syncope, such as an arrhythmia or obstructive cardiac lesions, results in the sudden loss and regaining of consciousness. Ventricular tachycardia or fibrillation result in syncope

Othostatic instability

Leads to syncope in association with a >20-point drop in systolic BP on changing position or a 10-point rise in pulse

Atrial Fibrillation
*Most common sustained arrhythmia

Presents with palpitations and an irregularly irregular pulse.

No discrete P waves (due to rapid atrial activity, 350-600bpm) in between irregularly spaced QRS complexes

Can result in atrial stasis and lead to stroke. Treat with beta-blockers, calcium-channel blockers, or digoxin; prophylaxis against thromboembolism with warfarin (coumadin).

Hyperthyroidism

Results in weight loss, anxiety, tachycardia, diarrhea, and palpitations. ~1/3 of patients have ocular findings such as exophthalmos (eyes are bulged forward)

Carcinoid Syndrome
*Carcinoid tumor secretes serotonin, can cause carcinoid syndrome

Flushing, diarrhea, wheezing, salivation, palpitations, and episodes of hypotension.

Fibrous deposits in right heart valves may lead to TR, PS, and RHF.

ECG showing a SHORT PR INTERVAL or DELTA WAVE

WPW
- can present with atrial arrhythmia alternating with ventricular arrhythmia
- Worsening of the rhythm after giving a calcium channel blocker or digoxin --> block conduction through the normal AV nodal pathway and force conduction through the aberrant tract, resulting in deterioration of the rhythm.

Canon "a" waves

Third-degree or "complete" heart block is associated with canon "a" waves in the neck.

It is often associated with symptomatic hypotension or syncope and that is why pacemaker placement is ALWAYS necessary.

Canon "a" waves result from atrial systole against a closed tricuspid valve.

Complete Heart Block

Leads to bradycardia, hypotension and canon "a" waves

Holosystolic murmur at the apex, radiating to the axilla.

Mitral Regurgitation

Sudden loss of pulse

Sudden loss of pulse can be from asystole, ventricular fibrillation, ventricular tachycardia, or pulseless electrical activity (PEA)

There is no way to distinguish the etiology of pulselessness without an EKG.

Therapy

(1) Asystole: Epinephrine and atropine (muscarinic receptor antagonist)

(2) Ventricular fibrillation and ventricular tachycardia: unsynchronized cardioversion

(3) PEA: Correct the underlying cause, such as tension pneumothorax, pulmonary embolus, hypovolemia, or tamponade

Coarctation of the aorta

Infantile type
- Aortic stenosis proximal to insertion of ductus arteriosus (preductal)
- Associated with Turner syndrome
- Check femoral pulses on physical exam
Infantile: IN close to the heart

Adult type
- Stenosis is distal to ligamentum arteriosum (postductal).
- Associated with notching of the ribs (due to collateral circulation), hypertension in upper extremities, weak pulses in lower extremities.
- Can result in aortic regurgitation
ADult: Distal to Ductus
Most commonly associated with bicuspid aortic valve.

Case: A 28-year old woman is seen on a follow-up visit for severe hypertension. The pressure is repeatedly elevated. Upper extremity BP is greater than lower extremity pressure.

Atrial Myxoma

A benign cardiac tumor that is characterized by a murmur that changes markedly with bodily position. This is also called a tumor plop. Myxoma presents with a murmur that is similar to mitral stenosis because it obstructs diastolic filling. There are also systemic symptoms such as fever, elevated sedimentation rate, and anemia. Transient ischemic attack is likely due to embolizaiton of myxoma, as they can be friable and are prone to embolization. Surgical removal is the only therapy

Peripheral arterial disease

Occurs as pain with any form of exertion of the lower extremities (*Calves) and is relieved by rest. As it worsens, there is loss of skin appendages such as hair follicles and sweat glands.
- The risk factors that favor the development of peripheral artery disease (ie, hyperlipidemia, smoking, hypertension, diabetes) are similar to those that promote the development of coronary atherosclerosis
***Claudication (a reproducible discomfort of a defined group of muscles that is induced by exercise and relieved with rest.)
- Edinburgh Questionnaire
- Improves with walking program that promotes
collateral formation and metabolic
(exercise)adaptation of muscle

The usual relationships between pain location and corresponding anatomic site of arterial occlusive disease can be summarized as follows:

Buttock and hip - aortoiliac disease
Thigh - aortoiliac or common femoral artery
Upper two-thirds of the calf - superficial femoral artery
Lower one-third of the calf - popliteal artery
Foot claudication - tibial or peroneal artery

Spinal Stenosis

Results in bilateral leg pain that is highly dependent on body position. It is much worse with anything that has the patient leaning back, such as walking downhill. It is relieved by leaning forward, such as sitting or bicycling. It is NOT the exertion that leads to the pain; it is the pressure of the spinal cord on the ligamentum flavum in the spinal canal.

Mitral Valve Prolapse (MVP)

- Mid-systolic click followed by a murmur
- Valsalva (Decreases venous return) will worsen only the murmurs of MVP and HOCM.
- The murmur improves with rapid squatting (increases venous return and afterload)
- MVP is confirmed with echocardiography
- Tx: beta-blockers

Case: A 34-year-old woman comes to the office with palpitations and atypical chest pain. The pain has no fixed pattern to exercise. Physical examination reveals a mid-systolic sound followed by a murmur. The murmur worsens with Valsalva and improves with leg raising.

HOCM

- Most often presents with dyspnea
- Proximity of hypertrophied IV septum to mitral leaflet obstructs outflow tract, resulting in systolic murmur and syncopal episodes.
- The murmur has the SAME crescendo/decrescendo pattern as AS but is heard best at the lower left sternal border (AS is heard best at the 2nd right intercostal space and radiates to the carotid arteries). The murmur worsens with Valsalva and improves with squatting.
- 50% of cases are familial AD
- Cause of sudden death in young athletes
- S4
- Tx: Beta-blockers. If syncope occurs, an implantable
cardioverter/defibrillator should be placed

Systolic murmur radiating to the carotid arteries

Aortic Stenosis
- Angina is the MOST COMMON PRESENTATION of aortic stenosis.

Diastolic decrescendo murmur with a wide pulse pressure

Aortic Regurgitation

Mitral Stenosis

- Opening Snap
- Diastolic murmur
- Dysphagia and hoarseness happen from enlargement of the LA pressing on the esophagus and recurrent laryngeal nerve
- Rheumatic disease most common cause
- Worse with AF (no atrial kick), tachycardia (decreased atrial emptying time), and pregnancy (increased preload)
- SOBOE, orthopnea, fatigue, palpitations, peripheral edema, malar flush

Aortic Dissection

Presents with the sudden onset of chest pain radiating to the back, particularly between the shoulder blades.
- Hypertension is by far the most common risk factor.
- Wide pulse pressure
- Difference in BPs and pulses between arms
- CXR --> Widened mediastinum
- Type A: involves ascending aorta +/- aortic arch; requires emergency surgery
- Type B: only involves aorta distal to subclavian artery; emergency surgery only if complications of dissection (requires long-term follow-up to assess aneurysm size).
- Management is aggressive control of systolic BP (100-120 mmHg) and HR (60 bpm) - Sodium nitroprusside and beta-blocker to lower BP and decrease cardiac contractility.

Dilated Cardiomyopathy

- Most common cardiomyopathy (90% of cases)
***Low ejection fraction and systolic dysfunction
- Primary cause = Idiopathic
- Secondary causes = Myocarditis (Parvovirus B19, adenovirus), alcoholism, cocaine, sarcoid, SLE, DM
- Dyspnea, PND, orthopnea, peripheral edema, S3 (rapid ventricular filling), high JVP,
- Tx: Standard HF therapies (ACE inhibitors, beta-blockers, nitrates, diuretics) + corticosteroids, IVIG, antiviral agents.

Hypertrophic Cardiomyopathy

- Most common cause of sudden cardiac death in athletes
- Characterized by asymmetric or symmetric hypertrophy (thickening) of the myocytes ("myofibril disarray")
- Hypertrophied IV septum is "too close" to mitral valve leaflet, leading to outflow tract obstruction.
- Risk = Ventricular arrhythmia
- Longstanding hypertension leads to diastolic dysfunction
- Gradient is worsened by: low preload (e.g. Valsalva), low afterload (standing from squatting), and increased contractility
- Clinical features: syncope, dyspnea, angina
- S4 (often palpable)
- Systolic ejection murmur left lower sternal border (increases with standing from squatting, Valsalva)
- Tx: Decrease contractility (beta-blockers, CCBs), myomectomy (cut out thickened area), septal ablation, treat arrhythmias.

Restrictive cardiomyopathy

- Most common cause is amyloidosis
- Abnormally rigid ventricular walls
- Abnormal filling (diastolic dysfunction)
- Usually systolic dysfunction is preserved until end stage
- Elevated JVP (rapid x and y descent), Kussmaul's sign (Is the rise of JPV with inspiration.), sustained apex, S4, hepatosplenomegaly, edema, LVH.
- Symptom management (diuretics, nitrates)
- Transplant if candidate

Constrictive Pericarditis

- Presents with shortness of breath and edema
- Kussmaul's sign (a rise in JVP on inhalation)
- Most often from TB which causes chronic inflammation of the pericardium
- Friedreich's sign (prominent "y" descent)
- S3 in diastole = pericardial "knock"
- Tx: diuretics, salt restriction, pericardiectomy (only if refractory to medical therapy)
- BP usually normal (and usually NO pulsus paradoxus)
- CT or MRI scan of the chest --> pericardial thickening

***Presenting symptoms may mimic those of restrictive cardiomyopathy (i.e. ascites, hepatosplenomegaly, edema).

***Tamponade
- NO Kussmaul's sign
- ALWAYS pulsus paradoxus
- SEVERE hypotension

Congenital Heart Disease: Cyanotic 5 "T" Lesions

Cyanosis (O2 sat<75%): blue coloration of the mucus membranes, nail beds or skin. Results from the presence of deoxygenated hemoglobin.

**New onset cyanosis = Emergency (ABCs!)

- Survival depends on mixing via shunts (e.g., ASD, VSD*, PDA)

*VSD = Most common lesion (hand grip - increases systemic vascular resistance - increases VSD systolic murmurs)

Investigations: CXR, ECG, Evaluation of Oxygenation (blood gas, saturation), Response to 100% O2 "Hyperoxic Test" (PaO2<100 mmHg --> CARDIAC)

Cyanotic 5 "T" Lesions:
*1) Tetralogy (Right-To-Left Shunt) - "Boot" shaped heart, single S2
*2) Transposition of Great Vessels - "Egg shaped" heart
3) Truncus Arteriosus - "Snowman" heart
4) Tricuspid Atresia - "Boot shaped heart", single S2
5) TAPVR
*Most common

Tetralogy of Fallot - Mnemonic: PROVe
P = Pulmonary stenosis (most important determinant of prognosis)
R = Right ventricular hypertrophy
O = Overriding aorta (overrides the VSD)
V = VSD

Persistent truncus arteriosus - failure of truncus arteriosus to divide into pulmonary trunk and aorta

Tricuspid atresia - characterized by absence of tricuspid valve and hypoplastic right ventricle. Requires both ASD and VSD* for viability

TAPVR - Pulmonary veins drain into right heart circulation (SVC, coronary sinus, etc).

S2 Splitting

Normal Splitting - Aortic valve closes before pulmonic; inspiration increases this difference

- Inspiration --> drop in intrathoracic pressure, which increases capacity of pulmonary circulation. Pulmonic valve closes later to accomodate more blood entering lungs; aortic valve closes earlier because of decreased return to left heart.

Wide splitting associated with pulmonic stenosis or RBBB (conditions that delay RV emptying) - an exaggeration of normal splitting

Fixed splitting associated with ASD - ASD --> Left to right shunt and therefore increased flow through pulmonic valve such that, regardless of breath, pulmonic closure is greatly delayed.

Pardoxical splitting - associated with aortic stenosis or LBBB); conditions that delay LV emptying. On inspiration, P2 moves closer to A2, thereby "paradoxically" eliminating the split.

TOF (Right-to-Left Shunt)

- most common CYANOTIC heart defect diagnosed BEYOND INFANCY

1. Pulmonary stenosis (most important determinant for prognosis)
2. RVH
3. Overriding aorta (overrides the VSD)
4. VSD

Early cyanosis is caused by a right-to-left shunt across the VSD. Right-to-left shunt exists because of the increased pressure caused by stenotic pulmonic valve.

- On x-ray, boot shaped heart due to RVH. Decreased pulmonary vasculature
- Physical exam: single loud S2 due to severe pulmonary stenosis (i.e. RVOTO)

- Patients suffer "cyanotic spells" (hypoxic "tet" spells)
--> primary pathophysiology is hypoxia, leading to increased pulmonary vascular resistance
(PVR) and decreased systemic resistance, occurring in exertional states (e.g. crying, exercise)
--> peak incidence at 2-4 months of age
--> Patient learns to squat to improve symptoms: compression of femoral arteries increases TPR thereby decreasing the right-to-left shunt and directing more blood from the RV to the lungs. Compression --> resistance --> pressure.
(management: O2, knee-chest position, fluid bolus, morphine sulfate, propanolol)

- Treatment: surgical repair (e.g. Blalock-Taussig Shunt) within first two years of life, or earlier if marked cyanosis, "tet" spells,
or severe RV outflow tract obstruction

- TOF is caused by anterosuperior displacement of the infundibular septum

Ebstein's anomaly (Right-to-Left Shunt)
* In Ebstein's Anomaly, the high pressure in the right atrium keeps the PFO open. This connection allows unoxygenated ("blue") blood to flow from the right atrium, bypassing the lungs and going directly to the body. (Tricuspid valve defect --> excessive back-flow into RA --> Right-to-Left shunt)

Congenital defect of the TRICUSPID VALVE in which the septal and posterior leaflets are malformed
and displaced into the RV leading to variable degrees of RV dysfunction, TS, TR or functional
pulmonary atresia if RV unable to open pulmonic valves
RA massively enlarged, interatrial communication patent foramen ovale (PFO) often exists allowing R --> L shunting

TR and accessory conduction pathways (WPW) are often present - often associated with arrhythmia (SVT)

Treatment:
Newborns - closure of tricuspid valve + aortopulmonary shunt, or transplantation

Older children - tricuspid valve repair or valve replacement + ASD closure

Transposition of Great Arteries

***Most common CYANOTIC CHD in NEONATE

- Aorta leaves RV (anterior) and pulmonary trunk leaves LV (posterior) --> separation of systemic and pulmonary circulation. Not compatible with life UNLESS a shunt is present to allow adequate mixing of blood (e.g., VSD***, PDA, or patent foramen ovale).

***No murmur if no VSD; infants without VSD must be repaired within 2 weeks to avoid weak LV muscle. If VSD present, cyanosis is not prominent and infant presents with CHF after a few weeks of life

- Due to failure of the aorticopulmonary septum to spiral. Without surgical correction, most infants die within the first few months of life.

- CXR: egg-shaped heart with narrow mediastinum ("Egg on a String")

Treatment: prostaglandin E1 infusion to keep ductus open until septostomy or surgery (arterial switch procedure).

Hypoplastic Left Heart Syndrome

Most common CAUSE OF DEATH from CONGENITAL heart disease in FIRST MONTH OF LIFE

- Hypoplastic LV
- Narrow mitral/aortic valves
- Coarctation of the aorta (***associated with Turner syndrome; Check FEMORAL pulses on physical exam)
- Contracted aorta

- systemic circulation is dependent on DUCTUS PATENCY; upon CLOSURE of the ductus, infant presents with circulatory shock and metabolic acidosis

Treatment:
• intubate and correct metabolic acidosis
• IV infusion ofPGEI to keep ductus open
• surgical correction (overall survival 50% to late childhood) or heart transplant

Total Anomalous Pulmonary Venous Connection

- characterized by all of the pulmonary veins draining into the right-sided circulation
- no direct oxygenated pulmonary venous return to left atrium
- an ASD MUST BE PRESENT to allow blood to shunt into the LA and systemic circulation

Treatment: surgical repair if severe cyanosis or CHF related to pulmonary venous obstruction

Truncus Arteriosus

A single great vessel arising from the heart which gives rise to the aorta, pulmonary and coronary arteries
- The truncal valve overlies a large VSD

Treatment: surgical repair within first 6 months of life to prevent development of pulmonary vascular disease

Acyanotic Congenital Heart Disease

Left-to-Right Shunts (late cyanosis) - "Blue kids"
1. VSD (most common congenital cardiac anomaly)
2. ASD (loud S1; wide, fixed split S2)
3, PDA (close with indomethacin - PGE2 antagonist (PGE2 maintains ductus arteriosus patency)
only effective in premature infants if necessary; used when other medical treatment for PDA fails after 48 hours.)

- shunt volume dependent upon three factors: size of defect, pressure gradient between chambers
or vessels, peripheral outflow resistance

- untreated shunts can result in pulmonary vascular disease, left ventricular dilatation and
dysfunction, right ventricular hypertension and hypertrophy (RVH), and eventually R to L shunts

Frequency: VSD>ASD>PDA

ASD

1. Ostium primum (common in Down syndrome)
2. Ostium secundum (MOST COMMON type, 50-70%)
3. Sinus venosus (defect located at entry of superior vena cava into right atrium)

- spontaneous closure if ASD diameter < 8mm
- if remains patent, CHF and pulmonary hypertension can develop in adult life

- ASD commonly presents with a PULMONARY FLOW MURMUR (increased flow through pulmonary valve) and a mid-diastolic rumble (increased flow across tricuspid valve - 4th intercostal space, left lower sternal border); blood flow across the ASD does NOT cause a murmur because there is NO PRESSURE gradient. The murmur LATER PROGRESSES to a louder DIASTOLIC MURMUR of PULMONIC REGURGITATION from DILATION of the PULMONARY ARTERY.

- Widely split and fixed S2
- RAD, RVH, RBB
- Increased pulmonary vasculature

Treatment: elective surgical or catheter closure between 2-5 years of age

VSD
***Size of VSD is inversely related to intensity of murmur

Holosystolic, harsh-sounding murmur. Loudest at tricuspid area
- Small (Majority) --> asymptomatic, most close spontaneously

- Moderate to large --> Delayed growth and development. Decreased exercise tolerance, recurrent URTls or "asthma" episodes, CHF by 2 months of age
- Treatment of CHF and surgical closure by 1 year of age

PDA
*functional closure within first 15 hours of life, anatomical closure within first days of life
*Often due to congenital rubella or prematurity

In fetal period, shunt is right to left (normal). In neonatal period, lung resistance decreases and shunt becomes left to right with subsequent RVH and failure (abnormal).

Associated with a continuous, "machine-like" murmur. Patency is maintained by PGE synthesis and low O2 tension.

Uncorrected PDA can eventually result in LATE CYANOSIS in the lower extremities.

History: may be asymptomatic, or have apneic or bradycardic spells, poor feeding, accessory muscle use

***PHYSICAL EXAM:
Heavy "machinery" murmur
High pulse rate
Wide pulse pressure
Hyperactive precordium
Big bounding pulse

Obstructive Lesions - present with pallor, decreased urine output, cool extremities and poor pulses, shock or sudden collapse

Coarctation of the aorta
Aortic Stenosis
Pulmonary Stenosis

Coarctation of the aorta
INfantile: IN close to the heart
ADult: Distal to Ductus
***Most commonly associated with BICUSPID AORTIC VALVE (50%)
***Narrowing of aorta almost always at the level of the ductus arteriosus
***Check femoral pulses on physical exam

Infantile type - Aortic Stenosis proximal to insertion of ductus arteriosus (preductal). Associated with Turner Syndrome (35%)

Physical exam: upper extremity systolic pressures of 140-145 mmHg, DECREASED BLOOD PRESSURE AND WEAK/ABSENT PULSES IN LOWER EXTREMITIES, radial-femoral delay, absent or systolic murmur
with late peak at apex, left axilla. and left back

ECG - RVH early in infancy, LVH later in childhood

Prognosis and Treatment
• if associated with other lesions (e.g. PDA, VSD) can cause CHF
• complications: hypertension
• management: give prostaglandins to keep ductus arteriosus patent for stabilization, balloon
arterioplasty or surgical correction in symptomatic neonate

Adult type - Stenosis is distal to ligamentum arteriosum (postductal). Associated with notching of the ribs (due to collateral circulation), HTN in upper extremities, weak pulses in lower extremities.

Can result in Aortic Regurgitation

Aortic Stenosis

History: often asymptomatic but may be associated with CHF, exertional chest pain, syncope or sudden death

Physical exam: SEM at right upper sternal border (RUSB) with aortic ejection click at the apex

Treatment
• surgical repair if infant with critical aortic stenosis or older child with symptoms or peak gradient >50 mmHg
• Exercise restriction required

Pulmonary Stenosis

Usually part of other congenital heart lesions (e.g. Tetralogy of Fallot)

Critical pulmonic stenosis: inadequate pulmonary blood flow, dependent on ductus for oxygenation, progressive hypoxia and cyanosis

Physical exam: wide split S2 on expiration, SEM at LUSB, pulmonary ejection click

CXR: dilated post-stenotic pulmonary artery

Treatment: surgical repair if critically ill or severe PS, or if presence of symptoms in older
infants/ children

Congenital Cardiac Defect Associations

22q11 Syndromes (e.g. DiGeorge syndrome) - Truncus arteriosus, Tetralogy of Fallot

Down Syndrome - ASD, VSD, AV septal defect (endocardial cushion defect)

Turner Syndrome - Coarctation of aorta

Marfan's syndrome - Mitral prolapse. aortic dissection or insufficiency (late finding), dilated aortic root

CHF
***4 key features: tachycardia, tachypnea, cardiomegaly, hepatomegaly (2 Tachy's and 2 Megaly's)

infant: feeding difficulties, easy fatiguability, exertional dyspnea, diaphoresis when sleeping or
eating, respiratory distress, lethargy, cyanosis, FTT

child: decreased exercise tolerance, fatigue, decreased appetite, failure to thrive, respiratory distress, frequent URTis or "asthma" episodes

CXR - cardiomegaly, pulmonary venous congestion

Management: sitting up, O2, sodium and water restriction, increased caloric intake

***3 D's
Diuretics
Digoxin
Afterload Decreasers (e.g. ACE inhibitors --> Dilate arteries --> Decrease Afterload)

***Left Failure
Low CO (forward) - Fatigue, syncope, systemic hypotension, cool extremities, MR, S3*. peripheral cyanosis, slow capillary refill

*In early diastole during rapid ventricular filling phase. Associated with increased filling pressures (e.g. MR, CHF) and more common in DILATED ventricles, (but normal in children and pregnant women)

Venous Congestion (backward) Dyspnea, orthopnea, PND, cough, crackles

***Right Failure
Right heart failure can mimic most of the symptoms of forward left heart failure if decreased RV output leads to LV underfilling
- TR, S3 (right-sided)

Venous Congestion (backward) - PERIPHERAL EDEMA, ELEVATED JVP with AJR and Kussmaul's sign,
Hepatomegaly, pulsatile liver.

Auscultation of The Heart

Aortic Area - 2nd intercostal space of the RIGHT sternal border - Systolic murmur (AS, Flow murmur)

Left Sternal Border - Diastolic murmur (AR, PR), Systolic murmur (HCM)

Pulmonic Area - 2nd and 3rd intercostal spaces of the left sternal border - Systolic ejection murmur (PS)

Tricuspid Area - 4th intercostal space of the left lower sternal border - Pansystolic murmur (TR, VSD), Diastolic murmur (TS, ASD)

Mitral Area - apex (around the 5th intercostal space in the mid-clavicular line) - Systolic murmur (MR), Diastolic murmur (MS)

MR/TR - Holosystolic, high-pitched "blowing murmur"
MR - loudest at apex and radiates toward axilla; enhanced by squatting*, handgrip and expiration; often due to IHD, MVP, or LV dilation. Symptoms: Dyspnea, PND, orthopnea, palpitations, peripheral edema.

*Increases venous return & afterload

TR - loudest at Tricuspid area and radiates to right sternal border; enhanced by inspiration; due to RV dilation or IE (IV drug use). Symptoms: Peripheral edema, fatigue, palpitations, PULSATILE LIVER

AS - Crescendo-decrescendo systolic ejection murmur following ejection click; Radiates to carotids/apex. Often due to age related calcific aortic stenosis or bicuspid valve. "Pulsus parvus et tardus". Narrow pulse pressure. Symptoms: Exertional angina, syncope, dyspnea, PND, orthopnea, peripheral edema.

VSD - Holosystolic, harsh-sounding murmur. Loudest at tricuspid area

Mitral Prolapse - Late systolic crescendo murmur with midsystolic click. Most frequent valvular lesion. Causes: Myxomatous degeneration of chordae, rheumatic fever, or chordae rupture. Enhanced by maneuvers that decrease venous return (valsalva or standing). Symptoms: Prolonged, stabbing chest pain, dyspnea, anxiety/panic, palpitations, fatigue, presyncope.

AR - Immediate high-pitched "blowing" diastolic murmur (Decrescendo). Wide pulse pressure when chronic; can present with bounding pulses and head bobbing. Often due to aortic root dilation, bicuspid aortic valve, or rheumatic fever. Vasodilators DECREASE intensity of murmur. Symptoms: Dyspnea, orthopnea, PND, syncope, angina.

MS - Follows opening snap (OS; due to abrupt halt in leaflet motion in diastole, after rapid opening due to fusion at leaflet tips). Delayed rumbling late diastolic murmur (Decrescendo-Crescendo). Rheumatic disease most common cause. Enhanced by maneuvers that increase LA return (e.g. expiration). Symptoms: SOBOE, orthopnea, fatigue, peripheral edema, malar flush.

PDA - Continuous machine-like murmurs. Loudest at S2. Often due to congenital rubella or prematurity.

Angiography

- provides an exact number of the degree of stenosis of the coronary artery and the number of vessels involved. The degree of stenosis must be >70% to be considered significant.
- Angiography is the answer when: The cardiac stress test is abnormal, prior to coronary surgery or angioplasty, and in an acute coronary syndrome such as unstable angina.

Ischemia

ST segment depression, T wave inversion (most commonly V1-V6)

Ventricular Fibrillation

A completely erratic rhythm with no identifiable waves. Fatal arrhythmia without immediate CPR and defibrillation

- Patients with Ventricular Fibrillation have NO pulse, respiration or BP and have suffered cardiac arrest.

Force of contraction is proportional to end-diastolic length of cardiac muscle fiber (preload) - Starling's law

Contractile state of myocardium:
(+) Circulating catecholamines (increases activity of calcium pump in sarcoplasmic reticulum), Digitalis (increases intracellular sodium, resulting in increased calcium), Sympathetic stimulation
--> Increase Contractility & SV

(-) Pharmacologic depressants, Loss of myocardium (MI)

Cardiac Output

CO = SV x HR
- If HR is too high, diastolic filling is incomplete and CO decreases (e.g., ventricular tachycardia)

Mean Arterial Pressure = CO x TPR

Pulse Pressure = Systolic Pressure - Diastolic Pressure
Pulse Pressure is proportional to SV

SV = EDV - ESV
SV INCREASES when Preload increases, Afterload decreases, or Contractility increases. SV INCREASES anxiety, exercise and pregnancy.

Preload and Afterload

Preload = ventricular EDV

Afterload = mean arterial pressure (proportional to peripheral resistance)

Venodilators (e.g. nitroglycerin) DECREASE preload

Vasodilators (e.g. hydralazine) DECREASE afterload

Ejection Fraction

EF = SV/EDV = EDV - ESV/EDV

EF is an index of ventricular contractility
EF is normally greater or equal to 55%
EF DECREASES in systolic heart failure

Cardiac Cycle

PHASES - LV
1) Isovolumetric contraction - period between MV closure and AV opening; period of highest O2 consumption

2) Systolic ejection - Period between aortic valve opening and closing

3) Isovolumetric relaxation - period between aortic valve closing and mitral valve opening

4) Rapid filling - period just after mitral valve opening

5) Reduced filling -period just before mitral valve closure

SOUNDS
S1 - Mitral and tricuspid closure. Loudest at MITRAL AREA
* Louder - MS
* Softer - MR
* Occurs on a (and c wave)

S2 - Aortic and pulmonary valve closure. Loudest at LEFT STERNAL BORDER
* Louder - MR
* Softer - AS/PS, HTN, AR (ABSENT S2)
* Occurs on v wave

S3 - in EARLY DIASTOLE during RAPID VENTRICULAR FILLING PHASE. Associated with high filling pressures (e.g. MR, CHF) and more common in dilated ventricles (but NORMAL IN CHILDREN & PREGNANT WOMEN)
* Right-sided increases with inspiration; left-sided increases with expiration

S4 - ("atrial kick") - in LATE DIASTOLE. High atrial pressure. Associated with ventricular hypertrophy. LA must push against stiff LV wall
* Louder - MR, AR

JVP
a wave - atrial contraction
* Prominent a wave = RVH, TS
* Absent a wave = Atrial Fibrillation
* Canon a waves = AV dissociation (3rd degree heart block)

c wave - RV contraction (closed tricuspid valve bulging into atrium)
* CV wave = TR

v wave - High RA pressure due to filling against closed tricuspid valve
* Prominent v wave = TR

x descent - atrial relaxation and downward displacement of closed tricuspid valve during ventricular contraction
* Prominent x descent and a diminutive or absent y descent = Acute Tamponade

y descent - blood flow from RA to RV
* Prominent y descent = Constrictive pericarditis (Most accurate test = CT or MRI scan; Elevated JVP, Positive Kussmaul's sign)
* Slow y descent = TS

Resistance

- Directly proportional to viscosity and indirectly proportional to the radius to the fourth power
- Arterioles account for most of total peripheral resistance --> regulate capillary flow

Torsades de pointes

Ventricular tachycardia, characterized by shifting sinusoidal waveforms on ECG. Can progress to V-fib. Anything that prolongs the QT interval can predispose to Torsades de pointes
(HINT: Look for hypomagnesemia in the question).

Magnesium is effective in Torsades de pointes and digoxin toxicity

PEA

Not a shockable rhythm.

Narrow QRS complex, causes = Hypovolemia, Hypoxemia, H+, Hyperkalemia/Hypokalemia, Hypothermia (5 H's); Thrombosis (ACS), Thrombosis (PE), Tension Pneumothorax, Tamponade (pericardial), Toxins (med OD) (5 T's).

Keys to management = CAB's, Epinephrine (1 mg q3-5 min), Determine the underlying causes, Treat the underlying cause.

The GOLD STANDARD for noninvasive imaging in congenital heart disease is

Echocardiography

AV Block

1st Degree - The PR interval is prolonged (>200 msec). Asymptomatic

2nd Degree, Mobitz type 1 (Wenckebach) - Progressive lengthening of the PR interval until a beat is "dropped" (a P wave not followed by a QRS complex). Usually asymptomatic.

2nd Degree, Mobitz type 2 - Dropped beats that are NOT preceded by a change in the length of the PR interval (as in type 1). These abrupt, nonconducted P waves result in a pathological condition. It is often found as 2:1 block, where there are 2 P waves to 1 QRS response. May progress to 3rd-degree block.

***Type 2 AV block is an indication for permanent pacing

3rd Degree (complete) - The atria and ventricles beat independently of each other. Both P waves and QRS complexes are present, although the P waves bear no relation to the QRS complexes. The atrial rate is FASTER than the ventricular rate. Usually treated with pacemaker. Lyme disease can result in 3rd-degree heart block.

Adenosine

Adenosine is the drug of choice in diagnosing/abolishing supraventricular tachycardia. Very short acting (~15 sec). Toxicity includes flushing, hypotension, chest pain.

Cardiomegaly, pleural effusion, vascular redistribution, Kerley B lines, and bronchiolar-alveolar cuffing (interstitial fluid accumulates around bronchi; causes thickening of bronchial wall; when seen on end, looks like little doughnuts) are x-ray findings in patients with ________

CHF

Cavitation

The following is a useful mnemonic for the differential diagnosis of a cavitating lung lesion:
Cancer (e.g. Squamous Cell Carcinoma)
Autoimmune
Vascular
Infection (e.g. Tuberculosis)
Trauma
Youth (congenital)

BNP

BNP goes UP with CHF and other causes of hypervolemia. BNP is sensitive but NOT specific for CHF. An elevated BNP is confirmed on echocardiogram.

Myocardial O2 demand is increased by:

Increased afterload
Increased contractility
Increased heart size
Increased heart rate

Coronary Artery Anatomy

In the majority of cases, the SA and AV nodes are supplied by the RCA. 80% of the time, the RCA supplies the inferior portion of the left ventricle via the PD/Posterior Interventricular Artery (= right dominant)

Coronary artery occlusion most commonly occurs in the LAD, which supplies the ANTERIOR INTERVENTRICULAR SEPTUM.

Coronary arteries fill during diastole

The most posterior part of the heart is the LA; enlargement can cause dysphagia (due to compression of the esophageal nerve) or hoarseness (due to compression of the recurrent laryngeal nerve, a branch of the vagus)

LAD - supplies APEX and ANTERIOR INTERVENTRICULAR SEPTUM

CFX - supplies POSTERIOR LV

PD - supplies POSTERIOR SEPTUM

Marginal artery - supplies RV

ECG Diagnosis of MI

Infarct Location/Leads with Q waves

Anterior wall - LAD - V1-V4

Anteroseptal - LAD - V1-V2

Anterolateral - LCX - V4-V6

Later Wall - LCX - 1, aVL

Inferior Wall - RCA - 2, 3 aVF

Ischemic Heart Disease
***Major risk factors for IHD:
Smoking, DM, HTN, Family history of MI, Hyperlipidemia

Angina - CAD narrrowing > 75%

1. Stable - mostly secondary to atherosclerosis; ST depression on ECG; Retrosternal chest pain with exertion
- Does NOT require admission
- Need to be on meds. for the secondary prevention of CAD

Tx: ACEi, Beta-Blocker, Statin, ASA, Nitro

2. Prinzmetal's variant - occurs at rest secondary to coronary artery spasm; ST elevation on ECG

3. Unstable - thrombus but no necrosis; ST depression on ECG; Worsening chest pain at rest or with minimal exertion
- Admission
- O2, ASA, NTG, Thrombin inhibitor (heparin, fondaparinux), Beta-Blocker, Clopidogrel (Plavix), +other CAD meds if not on them already
- Exercise stress test* with imaging or angiogram (high risk patient - High HR, Low BP, CHF, Recurrent pain, Renal Dysfunction - more likely to benefit from angiogram)

*Stress testing is the attempt to detect myocardial ischemia in patients without the need for coronary angiography. It is used when the diagnosis of ischemia is not clear in a person who has chest pain.
- Hypotension, lightheadedness, and chest pain are taken as positive results

Myocardial Infarction - Most often acute thrombosis due to coronary artery atherosclerosis; results in MYOCYTE NECROSIS

Sudden cardiac death - death from cardiac causes within 1 hour of onset of symptoms, most commonly due to a lethal arrhythmia (e.g., V-fib)

Chronic ischemic heart disease - Progressive onset of CHF over many years due to chronic ischemic myocardial damage.

Ventricular Action Potential
***Anti-Arrhytmic Drug Classification
Mnemonic: Some Block Potassium Channels
1 - Sodium CB
2 - Beta-Blocker
3 - Potassium CB
4 - Calcium CB

Phase 0 = rapid upstroke - voltage gated Na CHANNELS OPEN

Phase 1 = Initial repolarization - inactivation of voltage-gated Na+ channels. Voltage gated K+ CHANNELS begin to OPEN

Phase 2 = Plateau - Ca2+ INFLUX through voltage-gated Ca2+ channels BALANCE K+ efflux. Ca2+ influx triggers Ca2+ release from SR and myocyte contraction

Phase 3 = Rapid repolarization - massive K+ EFFLUX due to opening of voltage-gated slow K+ channels and closure of voltage-gated Ca2+ channels.

Phase 4 = Resting potential - high K+ permeability through K+ channels.

Bacterial Endocarditis
***Mnemonic Bacteria FROM JANE
Fever (most common symptom)
Roth's spots (round white spots on retina surrounded by hemorrhage)
Osler's nodes (tender raised lesions on finger or toe pads)
Murmur

Janeway lesions (small erythematous lesions on palms or sole)
Anemia
Nail-bed hemorrhage
Emboli

Note: Osler's nodes, Janeway's lesions, splinter hemorrhages are late findings in children

1. Acute - S. aureus (high virulence)
- Large vegetations on previously normal valves
- Rapid onset

2. Subacute - Viridans streptococci (low virulence)
- Smaller vegetations on congenitally abnormal or diseased valves. Sequela of dental procedures. More insidious onset.

***Mitral valve is most frequently involved; Tricuspid endocarditis is associated with IV drug use. Complications: Chordae rupture, glomerulonephritis, pericarditis, emboli.

Endocarditis may also be nonbacterial secondary to malignancy, hypercoagulable state, or lupus. S. bovis is present in colon cancer, S. epidermidis on prosthetic valves.

Antibiotic prophylaxis is necessary for all patients with:
• cyanotic congenital heart disease (including Tetralogy of Fallot, TGA, Ebstein's anomaly,
total anomalous pulmonary venous return)
• rheumatic valve lesions (except if no valve dysfunction)
• prosthetic heart valves
• palliative shunts and conduits
• previous endocarditis
• pacemaker leads

SBE prophylaxis: amoxicillin 50 mg/kg 30 to 60 minutes before procedure (if allergic to penicillin, then use clindamycin 20 mg/kg)

Case: ASD
* 3 year old girl with a murmur noted by her family physician when assessed for a flu-like illness.
*Fixed split S2 (Left-to-Right shunt, increased flow through pulmonic valve --> delayed pulmonic closure)

PE:
Fixed split S2.
A grade III/VI systolic ejection murmur is heard
at the upper left sternal border radiating to the axillae.

CXR: increased pulmonary vascular markings, especially at the hila of the lungs. The main pulmonary artery is also prominent.

ECG: The ECG shows an incomplete right bundle branch block, best seen in the right-sided precordial leads.

Synopsis:
This child has a large secundum atrial septal defect. There is a left-to-right shunt between the atria, and the increased flow enlarges the right side of the heart: right atrium, right ventricle and main pulmonary artery. The murmur is produced by the increased blood flow across the pulmonary valve.

This shunt rarely produces significant symptoms in early childhood. These children usually
come to attention due to the murmur. Adults with large atrial defects often have a higher
incidence of atrial arrhythmias, including atrial flutter, which may not subside when the
defect is closed. Thus these defects are often closed, surgically or by device, before school
age.

Case: VSD
* 9 week old male infant is brought into your office for laboured breathing
*Pansystolic/Holosystolic, harsh-sounding murmur.

Hx:
- He breastfed very well for the first 6 weeks
of life but his mother has noticed that he takes much longer to feed now, and rarely empties her breasts.
- He seems to have difficulty breathing when he feeds and often has to stop for breaks.
- He sleeps more now also, and she often has to wake him to feed.

PE:
- Weight 4.4 kg (~10th %ile)
- His skin is mildly clammy
- He has a mild tracheal tug and intercostal indrawing at rest.
- Tachycardic.
- Crackles
- He has a harsh pansystolic murmur that is loudest at his left lower sternal border

CXR: Marked cardiomegaly and prominent pulmonary vascular markings

ECG: RAD, biatrial and biventricular enlargement

Synopsis: This baby has a large left to right shunt across a ventricular septal defect which produces
enlargement of the left heart: the left atrium and left ventricle.

When the defect is moderate to large in size, the shunt produces symptoms early in life,
often beginning around 6-8 weeks of age. The baby becomes tachypneic and tachycardic
with the increase in pulmonary blood flow and congestive heart failure results.

Surgical closure of the defect with a patch is performed around 4-6 months of age. The baby may
require diuretic therapy and nasogastric feeding supplementation to maintain weight gain
until the baby reaches an optimal size for surgical repair.

Case: AVSD
*3 month old girl with Down syndrome who is having difficulty feeding and
gaining weight.

Hx: She is bottle-fed and has always been quite fussy to feed. Her breathing has become progressively noisier, both at rest and particularly with feeds.

PE:
- Weight 4.2 kg (‹5th %ile) Length 57 cm (~25th %ile).
- Mildly tachypneic and her breathing sounds congested.
- She has mild inercostal indrawing
- There is an RV heave palpable just
below her xiphisternum.
- She has a normal S1, a prominent S2, and a harsh systolic murmur loudest at her left upper sternal border but it radiates widely across her precordium. There is no diastolic component.

CXR: enlarged cardiac silhouette and increased pulmonary vascular markings.

ECG: Right atrial enlargement and biventricular hypertrophy

Synopsis: This child has a large atrioventricular septal defect (AVSD). Forty percent of children with
Down syndrome have congenital heart disease; approximately 40% of these affected
children have AVSD. Children often develop symptoms of heart failure by 2-3 months of
age. They usually have surgical repair, consisting of closure of the septal defects and repair
of the mitral valve, by 6 months of age.

Case: PDA
*A 5 year old boy is seen in your office for a murmur.
*There is a harsh continuous murmur heard at the left upper sternal border that radiates to his back and axillae.

Hx: A murmur was noted when he was seen in the emergency room two months ago for an ear infection which has been treated and resolved.

PE: His peripheral pulses are somewhat bounding.
There is a harsh continuous murmur heard at the left upper sternal border that radiates to his back and axillae.

CXR: His left atrium is enlarged. There are mildly increased pulmonary vascular markings.

Synopsis: This child has a large patent ductus arteriosus (PDA), with a significant shunt causing
enlargement of the left heart. He is asymptomatic, the typical presentation in older children. The PDA is closed, either by surgical ligation, or by occlusion with a device or coil at cardiac catheterization.

Case: Transposition of the great arteries
*You are called to the newborn nursery to assess a baby with cyanosis. The baby is 1 hour old.
*"Egg on a string"

Hx: The baby was noted to be dusky at delivery and was given oxygen by mask but is no pinker with that. He was initially vigorous but seems less active now.

PE: On examination, the child is very dusky and cool to the touch. His tissue perfusion (capillary refill) is 4 seconds.

- His peripheral pulses seem somewhat diminished.
- Single S2

CXR: The pedicle is somewhat narrowed,
with loss of the aortic knob and main pulmonary artery shadow. There are increased pulmonary vascular markings.

Synopsis: This newborn has d-transposition of the great arteries. The baby is cyanotic because the
circulation is in parallel, rather than in series. A shunt is required for mixing of blood. An atrial septostomy may be performed to stabilize the baby prior to definitive repair. The baby usually undergoes arterial switch procedure in the first week of life.

Case: Tetralogy of Fallot.
*A 2 month old girl is brought to your office for assessment of blue lips.
*"Boot" shaped heart

1. Pulmonary Stenosis (most important determinant of prognosis)
2. RVH
3. Overriding aorta (overrides the VSD)
4. VSD

Hx: Her parents have noted that her lips have been blue since birth but they have no other
concerns. She is on vitamin D supplements.

PE: Weight 3.9 kg (‹5th %ile) Length 54 cm (~10th %ile).
- her lips and oral mucosa are dusky.
- peripheral pulses are normal.
- capillary refill is 2-3 seconds
- RV heave and a thrill along the left sternal border
- Soft S2 (PULMONIC STENOSIS)
- There is an ejection click heard best at the left lower sternal border. She has a harsh systolic ejection murmur along her left sternal border.

CXR: "Boot" shaped heart

ECG: Right ventricular hypertrophy

Synopsis: This baby has tetralogy of Fallot. Her pulmonary stenosis was moderate and she had good-sized pulmonary arteries. She had a complete repair with closure of the VSD and a valve sparing
reconstruction of her right ventricular outflow tract at 3 months of age.

A baby with severe pulmonary stenosis may need a right modified BT shunt* done prior to complete
repair, although many centers will perform complete repair even in newborns.

*This procedure diverts blood from an aortic branch to the pulmonary artery, allowing blood to flow to the lungs to receive O2.

If the pulmonary stenosis is extreme, the baby may require prostaglandin infusion after birth to
maintain patency of the PDA to provide adequate pulmonary blood flow.

Upper Respiratory Tract Diseases
- disease above the thoracic inlet characterized by inspiratory stridor, hoarseness, and suprasternal retractions
***Age of onset is an important determinant that can help identify the cause of stridor. Generally, congenital disorders (eg, laryngomalacia, subglottic stenosis and tracheomalacia) present in the FIRST FEW WEEKS OF LIFE

Foreign body aspiration is more commonly seen ABOVE 6 MONTHS OF AGE, when the child becomes able to reach for things and to grab small objects, with incidence peaking around two to three years of age.

Croup most commonly occurs in children 6 to 36 months of age.

DDx for stridor:
• CROUP - Not present since infancy, acute onset, afebrile, BARKING COUGH, COMMON (6 months-4 years, peak incidence fall and early winter), Parainfluenza, RSV, "Steeple Sign" from subglottic narrowing, Tx: Humidified O2, Dexnethasone, Racemic epinephrine, Intubation if unresponsive to treatment

• BACTERIAL TRACHEITIS - Not present since infancy, acute onset, FEBRILE, RARE, S.aureus, H. influenza, similar appearance as croup but MORE RAPID DETERIORATION with high fever, toxic appearance, usually requires intubation & antibiotics

• EPIGLOTTITIS - Not present since infancy, acute onset, FEBRILE, RARE, 2-6 y.o., H. influenza, Beta-hemolytic strep., Severe airway obstruction, Drooling, Dysphagia, Distress, Tripod position, Tx: Intubation, antibiotics, PREVENTED WITH Hib VACCINE

• Foreign body aspiration

• Subglotic stenosis: congenital or iatrogenic - Congenital subglottic stenosis typically improves as the larynx grows; fewer than one-half of the children with this disorder require tracheostomy. Depending upon the severity of the obstruction and the need for supplemental oxygen therapy, congenital subglottic stenosis also may be treated by anterior cricoid split or endoscopic laser therapy

• LARYNGOMALACIA - refers to collapse of the supraglottic structures during inspiration. It is the MOST COMMON CONGENITAL ANOMALY OF THE LARYNX.

- Laryngomalacia manifests with intermittent low-pitched "wet" inspiratory stridor, usually in the neonatal period.
- In most children with laryngomalacia, stridor is loudest at four to eight months of age and resolves by 12 to 18 months.
- Stridor is more intense during upper respiratory tract infections. The stridor is often worse in the supine position and during crying or feeding. It may be present only during sleep or relaxation. Stridor may improve when prone
- Diagnosis is confirmed with flexible fiberoptic laryngoscopy
- In the vast majority of otherwise normal children, laryngomalacia is not dangerous and resolves spontaneously.

Lower Respiratory Tract Diseases
*Obstruction of airways below thoracic inlet. produces more expiratory sounds
Classic symptom: wheezing

DDx of Wheezing:
COMMON
• ASTHMA: recurrent wheezing episodes, identifiable triggers, relief with beta agonist.

Preventative/Controller Therapy
- ICS (FIRST LINE THERAPY)
- Leukotriene receptor antagonists (montelukast/Singulair)
- Combination medications (ICS/LABA)
- 3rd-line therapy in children - different than adults!

Rescue/Reliever Medications
SABA (e.g. salbutamol/Ventolin)

• BRONCHIOLITIS: first episode of wheezing, RSV (Respiratory Syncytial Virus) - responsible for >85% of bronchiolitis cases
-Direct epithelial damage resulting in airway edema, sloughing of necrotic epithelial cells, resulting in airway obstruction and mucus plugging with atelectasis. Long term increased risk of wheezing and cough with viral respiratory illnesses for 2-3 years, may be a marker for underlying asthma.

• RECURRENT ASPIRATION: often neurological impairment

• PNEUMONIA: fever, cough, malaise, incidence is greatest in first year of life, viral cause (CMV, RSV, Influenza virus) is more common in children <5 years old (CXR - diffuse, streaky infiltrates bilaterally; bacterial - fever, CXR - lobar consolidation, possibly pleural effusion, S. aureus, H. influenza, S. pneumoniae)

UNCOMMON
• foreign body: acute wheezing and coughing
• cystic fibrosis: prolonged wheezing, unresponsive to therapy, positive sweat chloride
• bronchopulmonary dysplasia: often develops after prolonged ventilation in the newborn

RARE
• congestive heart failure
• mediastinal mass
• bronchiolitis obliterans
• tracheobronchial anomalies

Bronchiolitis
***affects 50% of children in first 2 years of life
***peak incidence at 6 months, winter or early spring
***occurs in children prone to airway reactivity, increased incidence of asthma in later life

Defined as the 1st episode of WHEEZING associated with URTI and signs of respiratory distress

RSV (Respiratory Syncytial Virus) - responsible for >85% of bronchiolitis cases

- Prodrome of URTI with cough and fever
- Feeding difficulty, irritability
- Wheezing, respiratory distress, tachypnea, tachycardia, retractions, poor air entry lasting for
5-6 days

CXR (only needed in SEVERE disease, poor response to therapy, chronic episode}
- Air trapping, peribronchial thickening, atelectasis, increased linear markings

Supportive therapy
- Maintaining hydration status - with tachypnea, and as obligate nose-breathers, infants can have difficulty maintaining adequate feeding (coordination of suck-swallow-breathe, etc)
- small, frequent feedings; nasogastric feedings if necessary and tolerated
- intravenous fluids if unable to tolerate enteral feeding or in impending respiratory failure
- Supplemental oxygen therapy for respiratory distress +/- hypoxemia

Canadian Paediatric Asthma Consensus Guidelines for assessing adequate control of asthma:

1. daytime symptoms <4 days/wk
2. night time symptoms <1 night/wk
3. normal physical activity
4. no work/school absenteeism
5. need for beta agonist <4 doses/wk

Cystic Fibrosis
***sweat chloride test

- AUTOSOMAL RECESSIVE, CFTR gene found on chromosome 7 (ΔF508 mutation in 70%, over 800
different mutations identified)
- mutation in transmembrane conductance regulator of chloride - causes cells to be impermeable
to Cl which increases the reabsorption of Na. Leads to relative dehydration of airway secretions, resulting in impaired mucociliary transport and airway obstruction

Presentation

NEONATAL - meconium ileus, prolonged jaundice, antenatal bowel perforation

INFANCY - PANCREATIC INSUFFICIENCY with STEATORRHEA and FTT: (despite voracious appetite)

CHILDHOOD - anemia, hypoproteinemia, hyponatremia, wheezing or chronic cough, recurrent chest infections (S. aureus, P. aeruginosa, H. influenzae), hemoptysis, NASAL POLYPS, distal intestinal obstruction syndrome, rectal prolapse, CLUBBING OF FINGERS

OLDER PATIENTS - COPD, Infertility

Tx:
Nutritional Counseling (high calorie diet, pancreatic enzyme replacements, fat soluble vitamin supplements (ADEK))

Management of Chest Disease
- Physiotherapy - percussion and postural drainage ("clapping"), Flutter/PEP (positive end-expiratory pressure devices)
- DNAse/Dornase alpha/Pulmozyme (degrades the DNA "net" released by neutrophils; improves mucus viscosity in order to improve clearance)
- Antibiotics: depends on sputum C&S (e.g. cephalosporin, cloxacillin, ciprofloxacin, inhaled
tobramycin)
- Lung transplantation

Surveillance
1. symptoms
2. cultures (throat swab, sputum, bronchoalveolar lavage)
3. PFTs
4. CXRs

Complications:
- Respiratory failure
- Pneumothorax (poor prognostic sign)
- Cor pulmonale
- Pancreatic fibrosis with DM
- Gallstones
- Infertility
- Cirrhosis with portal hypertension

Bronchiolitis

AGE - Young Infant

CHRONICITY - Acute

PATTERN/SEASONALITY - Winter/RSV Season

ASSOCIATED SYMPTOMS - URTI +/- Fever

PAST MEDICAL HISTORY - Difficulty feeding, healthy

GENERAL APPEARANCE - Well-looking, well-nourished

AUSCULTATION - Wheeze, crackles

CXR - Hyperinflation, atelectasis

TREATMENT: Supportive - hydration, O2; Specific - nebulized epi.

Cystic Fibrosis

AGE - Young age at onset

CHRONICITY - Chronic

ASSOCIATED SYMPTOMS - Signs of malabsorption

PAST MEDICAL HISTORY - Poor growth, jaundice, rectal prolapse

PREVIOUS TX. RESPONSE - Improvement with antibiotics

FAMILY HX - possible CF, Infertility

GENERAL APPEARANCE - Poor growth/FTT

AUSCULTATION - Wheeze, crackles

OTHER FINDINGS - finger clubbing, increased AP diameter, nasal polyps

INVESTIGATIONS - sweat chloride
others:
i mutation analysis
ii fecal elastase
iii fecal fat
iv. CXR

TREATMENT: Nutrition (pancreatic enzymes, high fat/protein diet, fat soluble vitamins), Airway clearance (chest physio., mucolytics), Control infection (systemic antibiotics, inhaled antibiotics)

Asthma

AGE - The younger the age at onset, question diagnosis

CHRONICITY - Acute or chronic

PATTERN/SEASONALITY - URTIs, allergens, exercise, night

ASSOCIATED SYMPTOMS - Allergic rhinitis, eczema

PAST MEDICAL HISTORY - Eczema, allergies

PREVIOUS TX/RESPONSE - Response to bronchodilators

FAMILY HX - Atopy

SOCIAL/ENVIRONMENTAL HX - Assess for allergens

GENERAL APPEARANCE - Well looking

AUSCULTATION - Wheeze on forced expiration

OTHER FINDINGS - Allergic rhinitis, eczema, NO clubbing, Possible nasal polyps ONLY POST-PUBERTAL

INVESTIGATIONS - Allergy testing, spirometry (>age 6)

TX: Education, environmental control, controller therapy (1. ICS 2. LTRA), reliever therapy (SABAs)

STRIDOR - Laryngomalacia
**Respiratory distress
i.Indrawing, tracheal tug
ii.Pallor, cyanosis
iii.Head bobbing (due to activation of sternocleidomastoid muscles)

a.Onset at 2 -3 weeks of age
b.Increased intensity with crying/exertion
c.No effect on sleep, feeding/swallowing, weight gain
d.No pallor/cyanosis
e.Mild/absent in-drawing, tracheal tug

NATURAL HISTORY: Gradual increase in intensity with exertion/crying over the first few weeks followed by stability and gradual improvement such that most cases have completely resolved by 9-12 months of age

STRIDOR - Brachial Tracheitis
***Croup - Child has noisy breathing and barky LOUD cough but LOOKS GENERALLY FAIRLY WELL. Associated with upper respiratory tract symptoms of 1-3 days duration. Onset of stridor often with nap/night sleep and responsive to exposure to COLD air. Fall/winter virus so often resolved by the time the child gets to hospital due to cold air exposure. Temp generally normal or low grade. NO TRACHEAL TENDERNESS

***Epiglottitis - Very QUIET, SICK looking child with HIGH FEVER. Rapid (hours) onset of airway obstruction symptoms. Child often assumes "sniffing position" (ie. head held slightly hyper-extended and forward to maximize airway) and resistant to position changes. Drooling due to airway compromise and pain. Very rare in immunized child.

***Croup - Parainfluenzae virus
Epiglottitis: Hemophilus influenzae type b (non-immunized child)

a.Acute onset of symptoms of upper airway obstruction (stridor)
b. HIGH FEVER
c. Change in voice/cough (MUFFLED)
d. TRACHEAL TENDERNESS
e. LOOKS UNWELL
f. History of immunizations

Staphylococcus aureus, Group A streptococcus, Streptococcus pneumoniae

Emergent stabilization of the airway needed.

No venipuncture, radiographs, or non-emergent procedures PRIOR to airway stabilization. The vast majority of these children will go directly to the operating room (with parent in attendance to help keep them calm) upon presentation to the ER for bronchoscopy (confirmation of the diagnosis, acquisition of specimen for culture) and placement of an endotracheal tube. Intravenous (for fluids, antibiotics) can be started at that time and blood work drawn (CBC and cultures).

Empiric broad-spectrum antibiotics targeted at potential pathogens with modification once culture results available.
- Intravenous antibiotics given until afebrile > 48 hours, voice normal in quality, and pain resolved....then oral agent to complete full 7-10 day course of treatment.

Pain management
- regularly scheduled analgesia necessary
- treatment of pain will also improve airway clearance of secretions as child will not be suppressing cough as much.

Airway clearance
- Suctioning for intubated child
- Physiotherapy helpful for both encouraging coughing and treatment of associated pneumonias (common, roughly 50%)

Follow-up
- Subglottic stenosis can occur as a complication of bacterial tracheitis
- Reinforcement of need to immunize (if not done)

Bacterial pneumonia in children

- ACUTE onset illness with FEVER typically > 38.5ºC, lethargy, pallor.
- Often follows a viral URTI/flu-like illness
- ABDOMINAL PAIN, shoulder pain common...the abdo. pain can be very impressive (ie. Mimics appendicitis)
- May be complaints of trouble breathing, shortness of breath
- On exam, child looks unwell ...with VIRAL URTI they generally do not look that unwell and are still up and about playing, etc.
- May have NASAL FLARING, GRUNTING
- Respiratory rate increased ** highly sensitive and specific
a. RR > 60/min (under 6 months)
b. RR > 50/min (6-11 months)
c. RR > 40/min (12-60 months)
- COUGH almost always present, productive sounding but children under 12 years very unlikely to be able to report or provide sputum sample
- Decreased/absent breath sounds, bronchial breath sounds, crackles (Absent breath sounds suggests associated effusion)

Features distinguishing consolidation from atelectasis
i. Space occupying vs. space reducing
ii. Consolidation not respectful of fissures

- Streptococcus pneumoniae
- Group A streptococcus
- Hemophilus influenzae (type b in non-immunized, nontypable if immunized)
- Mycoplasma pneumoniae/Chlamydia pneumoniae
- Staphylococcus aureus in child under 2 years or compromised child

Tx: Macrolide agents provide coverage for ATYPICALS as well as most of the bacterial pathogens of concern and may be considered as monotherapy in a stable child.

In more significant illness, risk of prescribing an antibiotic in the presence of resistant streptococcus may be too high and dual coverage (ie macrolide and second generation cephalosporin) would be indicated.

Pleural Effusions

Transudate - decreased protein content. Due to CHF, nephrotic syndrome, or hepatic cirrhosis

Exudate - Increased protein content. Due to malignancy, pneumonia, collagen vascular disease, trauma (occurs in states of increased vascular permeability). Must be drained in light of risk of infection.

According to Light's traditional criteria, if at least one of the following three criteria is present, the fluid is defined as an exudate:

1. Pleural fluid protein/serum protein ratio greater than 0.5
2. Pleural fluid LDH/serum LDH ratio greater than 0.6
3. Pleural fluid LDH greater than two-thirds the upper limits of the laboratory's normal serum LDH

Sleep Apnea
*Central Sleep Apnea - No respiratory effort
*Obstructive Sleep Apnea - Respiratory effort against airway obstruction
*Hypoapnea --> 50% reduction in ventilation for 10 or more seconds
*CPAP has been shown to reduce cardiovascular risk and cardiovascular related deaths in patients with obstructive sleep apnea

Person stops breathing for at least 10 seconds repeatedly during sleep.

Central sleep apnea
- Caused by transient episodic decreases in CNS drive to breathe
- No airflow b/c no respiratory effort
- Cheyne-Stokes Respiration (CSR): a form of CSA in which central apneas alternate with hyperapneas to produce a crescendo-decrescendo pattern of tidal volume; seen in severe LV DYSFUNCTION, BRAIN INJURY, and other settings.

Obstructive sleep apnea
- Associated with obesity, loud snoring, systemic/pulmonary HTN, arrhythmias, and possible sudden death.
- Individuals may become chronically tired.
Treatment: weight loss, CPAP, surgery

Investigations: Sleep study (polysomnography)
- evaluates sleep stages, airflow, ribcage movement, ECG, SaO2, limb movements
- indications: excessive daytime sleepiness, unexplained pulmonary HTN or polycythemia, daytime hypercapnia, titration of optimal nasal CPAP

Kussmaul's Sign

Is the RISE of JPV with inspiration
- JPV normally DECREASES with inspiration because of reduced pressure in the expanding thoracic cavity
- Suggests impaired filling of the right ventricle.
- This could be due for example because of fluid in the pericardium or poor compliance of the pericardium or myocardium

Classic quartet of Tamponade

1) Hypotension
2) Increased JVP
3) Tachycardia
4) Pulsus Paradoxus

DDx: Pulsus Paradoxus
= Normally, when we take a breath in, our intrathoracic pressure decreases
which makes it easier for venous blood to flow back into the heart, thus more
blood enters the right side of the heart. The fact that more blood enters the
right side of the heart leads to (pulmonary venous return) flow being reduced
into the left side of the heart. This, in turn, means that there is less blood
available to pump out of the left side of the heart when it contracts. This
manifests itself as a decrease in systolic blood pressure.
* A difference greater than 10 mmHg is abnormal.

1. Cardiac Tamponade
2. Constrictive Pericarditis
3. Severe obstructive pulmonary disease (e.g. asthma)
4. Tension Pneumothorax
5. Pulmonary Embolus
6. Cardiogenic shock - patients usually have signs of left-sided heart failure.
- Low BP, High JVP, Cold Extremities, E.g. Myocardial Ischemia +/- Infarction, arrhythmias

Shock
= Inadequate tissue perfusion potentially resulting in end organ injury
*HIGH JVP - Cardiogenic & Obstructive
*Warm Extremities - Distributive
*Hypovolemic - Look for visible hemorrhage or signs of dehydration

Hypovolemic - Patients have COOL EXTREMITIES due to peripheral vasoconstriction
- High HR, Low BP, Low JVP, Cold Extremities
E.g. Hemorrhagic, dehydration, vomiting, diarrhea

Cardiogenic - Patients usually have signs of left-sided heart failure
- High/Normal/Low HR, Low BP, HIGH JVP, Cold Extremities,
- Bilateral crackles on chest exam
E.g. Myocardial Ischemia +/- Infarction

Obstructive - Varied presentation
- High HR, Low BP, HIGH JVP, Normal/Cold Extremities
E.g. Massive PE, pericardial tamponade, constrictive pericarditis, tension pneumothorax
*Pulsus Paradoxus - Tamponade; Kussmaul's sign - Constrictive Pericarditis; Tracheal Deviation - Tension Pneumothorax;

Distributive - Patients have WARM extremities due to PERIPHERAL VASODILATION
- High HR, Low BP, Low JVP, WARM Extremities
E.g. Sepsis, anaphylactic reaction, neurogenic, toxic
*Look for obvious signs of INFECTION

DDx of Clubbing
*Clubbing is NOT seen in COPD - if present, THINK MALIGNANCY

PULMONARY
1. Cystic Fibrosis
2. Pulmonary Fibrosis
3. Bronchiectasis
4. Lung Abscess
5. Lung Cancer

GI
1. IBD
2. Cirrhosis
3. HCC

CARDIAC
1. Cyanotic congenital heart disease
2. Infective endocarditis

MEDIASTINAL
1. Esophageal Cancer
2. Thymoma

Silhouette Sign
- loss of normally appearing profiles

RUL - loss of upper right heart border, ascending aorta
RML - loss of right heart border
RLL - loss of right hemidiaphragm

LUL - loss of aortic knuckle, left atrium
LLL - loss of left hemidiaphragm
Lingula - loss of left heart border

Lung Cancer
*Bronchial adenomas and hamartomas (Clustered "popcorn" pattern of calcification is pathognomonic for hamartoma) comprise 90% of the benign neoplasms of the lung.
*Squamous cell carcinoma and Small cell carcinoma --> strong relationship with smoking
*Adenocarcinoma = largest NON-SMOKING group

- Leading cause of cancer death

PRESENTATION
- COUGH (75%; beware of CHRONIC cough that CHANGES in character), DYSPNEA (60%), CHEST PAIN (45%), hemoptysis, bronchial obstruciton, wheezing, pneumonic "coin" lesion on x-ray film or non-calcified on CT.

- Metastases TO lung is most common, often from BREAST, COLON, PROSTATE, and BLADDER CANCER

- Sites of Metastases - Adrenals, brain (epilepsy), bone (pathologic fracture), liver (jaundice, hepatomegaly).

*SPHERE of Complications

S = Superior vena cava syndrome
P = Pancoast tumor
H = Horner's syndrome
E = Endocrine (paraneoplastic)
R = Recurrent laryngeal symptoms (hoarseness)
E = Effusions (pleural or pericardial)

Solitary Nodules --> Neoplasm, Granuloma, Hamartoma

Multiple Nodules --> Infectious (Fungal, TB, Septic Emboli), Inflammatory (Rheumatoid, Wegener's), AVMs, Pulmonary Emboli

Lung Nodule present on CXR for >2 years --> Scar, granuloma, AVM (Hereditary Hemorrhagic Telangiectasia - HHT)

2/3 of PRIMARY lung cancer is found in the UPPER LUNG; 2/3 of METASTASES occur in the LOWER lung (hematogenous spread secondary to increased blood flow to the base of the lung)

Potential clinical problems with lung cancer are the following:

1. Symptoms from an endobronchial tumour: cough, hemoptysis

2. Problems of bronchial obstruction: postobstructive pneumonia, dyspnea

3. Pleural involvement: chest pain, pleural effusion, dyspnea

4. Involvement of adjacent structures: heart, esophagus

5. Complications of MEDIASTINAL involvement: phrenic or recurrent laryngeal nerve paralysis, superior vena cava obstruction

Distant metastases: brain, bone or bone marrow, liver, adrenals

Ectopic hormone production: ACTH, ADH, Parathyroid hormone related peptide

Other paraneoplastic syndromes: neurologic, clubbing, hypertrophic osteoarthropathy

Nonspecific systemic effects: anorexia, weight loss

Basis for staging of non-small cell lung cancer includes the following:

1. Size, location and local complications of the primary tumor

2. Hilar and mediastinal lymph node involvement

3. Distant metastasis

Criteria for assessing the likelihood that a solitary pulmonary nodule is malignant are the following:

1. Stability or change in size of the lesion
- Malignant >3cm, irregular, spiculated
- Doubling Time
a. Benign: Doubles in <1 month or >2 years
b. Malignant: Doubles in >1 month or <2 years

2. Presence or absence of calcification; pattern of calcification (Best demonstrated on CT scan)
- Malignant tumors are usually NOT calcified

3. Smooth versus irregular appearance of the border
- Malignant: ill-defined or notched margin

Common features of bronchial carcinoid tumors (BENIGN) are the following
*90% of bronchial adenomas = carcinoids

1. Often found in young adults
2. Hemoptysis
3. Pneumonia distal to an obstructing endobronchial mass
4. Smoking does NOT appear to be a risk factor
5. Secretes serotonin, can cause carcinoid syndrome (flushing, diarrhea, wheezing, salivation). Fibrous deposits in right heart valves may lead to tricuspid insufficiency, pulmonary stenosis, and right heart failure

Adenocarcinoma
*NONSMOKERS & FEMALES
*Location = PERIPHERAL

1. Often manifest as a solitary, peripheral pulmonary nodule
2. May arise in an old parenchymal scar
3. Generally localized when manifest as a peripheral lung nodule
4. Spread to hilar and mediastinal nodes and to distant sites
5. Most common lung cancer in NONSMOKERS and FEMALES
6. Incidence ~40%

Hamartomas (BENIGN LUNG TUMOR)

- Composed of tissue normally present in lung
- Peak incidence at age 60, more common in men, 10% of benign lung lesions (2nd to INFECTIOUS GRANULOMA (80%)).
- Usually peripheral, clinically silent, and benign in behaviour
***clustered "POPCORN" pattern of calcification is PATHOGNOMONIC for hamartoma

Small Cell Carcinoma
*Central
*Hilar and Mediastinal Node Involvement
*Very Aggressive
*Surgery not usually performed since SCLC is generally non-curable
*Concurrent chemotherapy and radiation therapy is the optimal treatment for limited stage small cell lung cancer.
*Prophylactic cranial irradiation (PCI) in patients with excellent response to chemo/RT as PCI reduces incidence of brain mets by 50%; increase survival

1. Location = Central
2. Generally arise in proximal airways
3. Hilar and mediastinal node involvement
4. Early, distant metastatic disease
5. Very aggressive
6. Often associated with ectopic production of ACTH (Cushing's syndrome) or ADH
7. Responsive to chemotherapy
8. Inoperable
9. Incidence ~20%

Median Survival/Untreated/Treated/5 year Survival
Limited/3 months/16-20 months/10%
Extensive/6 weeks/9 months/0%

Squamous Cell Carcinoma
*Smoking
*Central
*Cavitation

1. Generally arise in proximal airways.
2. May cause airway obstruction, leading to distal atelectasis or pneumonia.
3. May cavitate
4. Intrathoracic spread rather than distal metastases
5. Incidence ~30%

Limited Stage - Confined to a single radiation port (one hemithorax and regional lymph nodes)
Tx: Radiation +/- Chemo; Median Survival 1-2 years

Extensive Stage - Extension beyond a radiation port
Tx: Chemotherapy; Median Survival 6 months

Pancoast (Squamous cell carcinoma)
*Lung Apex
*Horner's Syndrome
*Brachial Plexus Palsy (most commonly C8-T1 nerve roots)

Carcinoma occurs in apex of lung and may affect cervical sympathetic plexus, causing Horner's syndrome (ptosis, miosis, anhidrosis).

Ptosis - drooping eyelid
Miosis -decreased pupil size
Anhidrosis - decreased sweating on the affected side of your face

Involvement of the nerves comprising the brachial plexus can result in pain and weakness of the shoulder and arm

Mediastinal Mass

ANTERIOR
1. Thyroid
2. Thymoma
3. Teratoma
4. Lymphoma

MIDDLE
1. Vascular - Aneurysms
2. Cysts - Bronchial, Pericardial, Esophageal
3. Lymphadenopathy - Benign, Malignant

Posterior
1. Neurogenic Tumors
2. Esophageal Lesions
3. Diaphragmatic Hernia

What is the evidence for CXR screening for lung cancer?

- 3 large RCT failed to show mortality benefit from screening CXR
- More early stage cancers were found, but no fewer lung cancer deaths
- Length - time bias?
- Lead - Time bias
- Over diagnosis?

Staging of the mediastinum

Staging of the mediastinum is routinely done with mediastinoscopy prior to resection, as the CT is only ~75% sensitive in detecting mediastinal involvement of lung cancer. If PET scan done and is negative in the mediastinum, mediastinoscopy can be avoided and can proceed directly to surgery.

Pneumoconioses

1) Coal Miner's
- associated with coal miners. Can result in cor pulmonale, Caplan's syndrome (rheumatoid arthritis involvement)
- affects UPPER LOBES

2) Silicosis
- associated with foundries, sandblasting, and mines. Macrophages respond to silica and release fibrogenic factors, leading to fibrosis. It is thought that silica may disrupt phagolysosomes and impair macrophages, INCREASING SUSCEPTIBILITY TO TB and SCLERODERMA
- Affects UPPER lobes
- "Eggshell" calcifications on hilar lymph nodes

3) Asbestos
- associated with shipbuilding, roofing and plumbing. Results in "ivory white" calcified PLEURAL PLAQUES. Associated with an increase of bronchogenic carcinoma and mesothelioma
- Affects LOWER lobes
- Asbestos bodies (aka FERRUGINOUS BODIES) are golden-brown fusiform rods resembling dumbbells located inside macrophages.

Mesothelioma

Malignancy of the pleura associated with asbestosis. Results in hemorrhagic pleural effusions and pleural thickening.

A 65-year old man worked for 20 years in a family owned sandblasting business and used no respiratory precautions during that time. For the past 7 years he has had increasing dyspnea without fever, cough, or chest pain. What is the most likely underlying disease and which inflammatory cell is most crucial to its development?

Silicosis
Macrophages

A granuloma is defined by the present of which cell type(s)?

ONLY macrophages

After a hemicolectomy to remove a colon carcinoma, a 53-year old man develops respiratory distress. He deteriorates and needs ventilation in ICU. A CXR shows increasing opacification in all lung fields. A transbronchial biopsy shows pink staining material lining distended alveolar ducts and sacs. What is the diagnosis and which is the fundamental mechanism most likely underlying it?

ARDS
Leukocyte-mediated injury to alveolar capillary endothelium

A 66 year old man has had progressively worsening dyspnea over the past 10 years. He has lost 5kg in the last 2 years. He has chronic cough with minimal sputum production. On physical examination he is afebrile and normotensive. A CXR shows extensive interstitial disease. Pulmonary function tests show low FVC and a normal FEV1/FVC ratio. Increased exposure to which of the following pollutants is most likely to produce these findings?
A.Silica
B.Tobacco smoke
C.Ozone
D.Wood dust
E.Carbon monoxide

Silica
*Weight Loss is common in ILD patients

Characteristic CT Findings in ILD

"Ground Glass" - early ILD
"Honey Combing" - late ILD, especially IPF

A 70-year old woman is referred to an ophthalmologist because of difficulty with her right eye. She also has pain in her right upper chest. The findings on physical examination include enophthalmos, meiosis, anhidrosis and ptosis. A CXR shows right upper lobe opacification and bony destruction of the right first rib. Which of the following conditions is most likely?
A.Bronchopneumonia
B.Bronchiectasis
C.Bronchogenic carcinoma
D.Sarcoidosis
E.Tuberculosis

Bronchogenic carcinoma

Pancoast tumor - Carcinoma that occurs in the apex of the lung and may affect cervical sympathetic plexus, causing Horner's Syndrome

Horner's Syndrome - Ptosis, Miosis, Anhidrosis

A 78-year old man has incresing dyspnea without cough or increased sputum for the past 4 months. He is afebrile, breath sounds are reduced in all lung fields. A CT scan shows a dense bright right pleural mass. Microscopic examination of a pleural biopsy specimen shows spindle and cuboidal cells that invade adipose tissue. Inhalation of one of the following is the most likely factor related to the pathogenesis of this mass?
A. Ozone
B. Bird dust
C. Silica
D. Cotton fibres
E. Asbestos
F. Coal dust

Asbestos
- Mesothelioma --> invasion of fat

Which disease is NOT associated with restrictive lung disease?
A. Hypersensitivity pneumonitis
B. Non-specific interstitial pneumonitis
C.Berylliosis
D.Chronic bronchitis
E.Pickwickian syndrome
F.Heart Failure

Chronic bronchitis

Restrictive Lung Diseases

INTRINSIC
• interstitial fibrosis
• heart failure, lung edema
• pneumonia
• tuberculosis
• pneumothorax

EXTRINSIC CAUSES
•kyphoscoliosis
•extreme obesity
•pregnancy
•abdominal mass
•pain

NEUROMUSCULAR DISEASE
•paralysis of diaphragm
•muscular dystrophy
•poliomyelitis
•muscle weakness

Obstructive Lung Diseases

Chronic Bronchitis
Emphysema
Asthma
Bronchiectasis
CF

Hypoxemia

1. V/Q Mismatch
2. Hypoventilation*
3. Shunt**
4. Diffusion Barrier
5. Low Inspired O2*
* NORMAL A-a Gradient
**No response to O2

A-a Gradient (CALGARY)

130-31-PaO2
*Normal: 10-15

Increased A-a gradient = LUNG DISEASE
- Give 100% O2 and PaO2 Improves --> V/Q mismatch, airway disease (asthma, COPD), ILD, alveolar disease, pulmonary vascular disease

- Give 100% O2 and PaO2 does NOT improve --> Shunt, atelectasis

Acid Base Simplified

Normal pH: 7.35-7.45
Normal CO2 = 40
Normal HCO3 = 24

Primary RESPIRATORY ACIDOSIS: High CO2, High HCO3- (Compensation)

Primary METABOLIC ALKALOSIS: High HCO3-, High CO2 (Compensation)

Disturbance/PCO2/HCO3-

Respiratory acidosis(a)/+10/+1
Respiratory acidosis(c)/+10/+3

Respiratory alkalosis(a)/-10/-2
Respiratory alkalosis(c)/-10/-4

Metabolic Acidosis/-1/-1
Metabolic Alkalosis/+5-7/+10

Anion Gap = [Na]-({Cl] + [HCO3]); Normal = 10 +/- 2
- If anion gap is increased, is the change in bicarbonate the same as the change in anion gap? If not, consider a mixed metabolic picture

WIDE ANION GAP = ACIDOSIS
Anion Gap Metabolic Acidosis (KARMEL)
K = Ketoacidosis
A = ASA
R = Renal Failure
M = Methanol
E = Ethylene Glycol
L = Lactic Acidosis

[H+] = 80 - [last 2 digits of pH]

Which of the following ABGs is most consistent with
a 40 year old man in the ER with a heroin
overdose?
pH PaCO2 PaO2 HCO3
a. 7.50 30 90 24
b. 7.32 28 85 14
c. 7.25 60 50 26
d. 7.34 60 40 31

c. 7.25 60 50 26
Heroin --> Hypoventilation
Acute Respiratory Acidosis (10:1)

Which of the following ABGs is most consistent with
a 21 year old with diabetic ketoacidosis?
pH PaCO2 PaO2 HCO3
a. 7.50 30 90 24
b. 7.32 28 85 14
c. 7.25 60 50 26
d. 7.34 60 40 31

Diabetic ketoacidosis = Metabolic Acidosis
- LOW HCO3- and LOW CO2 (Compensation)

Answer: b. 7.32 28 85 14

Which of the following ABGs is most consistent with
an anxious, otherwise normal patient who is
terrified of having ABGs?
pH PaCO2 PaO2 HCO3
a. 7.50 30 90 24
b. 7.32 28 85 14
c. 7.25 60 50 26
d. 7.34 60 40 31

Answer = a. 7.50 30 90 24

Which of the following ABGs is most consistent with a stable, long‐term COPD patient?
pH PaCO2 PaO2 HCO3
a. 7.50 30 90 24
b. 7.32 28 85 14
c. 7.25 60 50 26
d. 7.34 60 40 31

Chronic Respiratory Acidosis: CO2 +10/HCO3- +3

Answer = d. 7.34 60 40 31

What would the previous, long‐term COPD patient's ABG look like in an acute exacerbation?
pH PaCO2 PaO2 HCO3
a. 7.34 60 40 31
b. 7.28 70 30 32

Chronic, compensated respiratory acidosis +
ACUTE RESPIRATORY ACIDOSIS during EXACERBATION

Answer = b. 7.28 70 30 32

Impaired Signs and Symptoms in Acute Asthma

RED FLAGS
- Fatigue
- Diminished respiratory effort
- Cyanosis
- Silent chest
- Decreased LOC

RESPIRATORY DISTRESS
- Nasal flaring
- Tracheal tug
- Accessory muscle use
- Intercostal indrawing
- Pulsus Paradoxus

***ABGs
- decreased PaO2 during ATTACK (V/Q mismatch)
- decreased PaCO2 in MILD asthma due to HYPERVENTILATION
- NORMAL or INCREASED PaCO2 is an OMINOUS SIGN as patient is NO LONGER ABLE TO HYPERVENTILATE (worsened airway obstruction or respiratory muscle fatigue)

***Emergency Management
1. Inhaled Beta-agonist first line
2. Add cholinergic
3. Ketamine and succinylcholine for rapid sequence intubation in life threatening cases
4. SC/IV adrenaline, IV salbutamol if unresponsive
5. ALL patients admitted to ER for asthma exacerbations should be considered for corticosteroid therapy at discharge

Which of the following statements is TRUE in
the patient with untreated CHF?
A. Afterload is increased
B. Preload is decreased
C. Contractility is increased
D. None of the above

A. Afterload is increased

CHF (High Preload (elevated JVP), High Afterload, Low Contractility)

***HOCM (Low Preload, Low Afterload, High Contractility)

***Causes of Heart Failure
• pressure overload
• volume overload
• loss of myocytes
• impaired myocyte function
• impaired filling

The causes of isolated right heart failure
include all of the following EXCEPT:
A. Tricuspid regurgitation
B. Right ventricular infarction (isolated)
C. Pericardial constriction
D. Tamponade
E. Aortic regurgitation

E. Aortic regurgitation

Heart Failure - Right Only

• Pulmonary hypertension
• Right sided valve disease
• Isolated RV systolic dysfunction
• Pericardial constriction
• Tamponade

Heart Failure - Acute Management

• Supportive
• Decrease afterload (ace inhibitor)
• Decrease preload (nitroglycerin)
• Diuresis
• Look for cause of decompensation
• Start Beta-blocker only once stable

Beta-blockers → Decrease the rate of ventricular contraction to increase filling time → Increases CO → Increases Forward flow

Digoxin (Inotrope) → Increase the force of ventricular contraction → Increases CO → Increases Forward Flow
- Inhibits Na+/K+ ATP pump
- Slows AV node conduction

NTG → Dilates Veins →Decreases Preload

Diuretics → Decreases intravascular fluid volume → Decrease Preload

ACE inhibitors, hydralazine → Dilate arteries → Decreases afterload

***Mortality Benefit: Beta-blockers, ACE-Inhibitors, Spironaloctone (Aldosterone inhibitor) - NYHA III-IV HF

In a patient with CHF from severe LV systolic
dysfunction secondary to ischemic heart
disease which of the following is TRUE?
A. LVEDP is high
B. Digoxin will decrease mortality
C. Nitro will be of no benefit
D. ACE inhibitors are less effective than
ARBs
E. Elimination of edema is therapeutic goal

A. LVEDP is high

Hoover's Sign (COPD)

paradoxical retraction of the lower interspaces during inspiration (ie, Hoover's sign)

PHYSICAL EXAM:
- With the patient supine, place your hands along the costal margins with your thumbs close to the patient's midline. Ask the patient to breathe out and then breathe in.
- Normally, as the patient breathings in, your thumbs would move apart and return closer together when the patient expires.
- Suspect COPD (chronic obstructive pulmonary disease) if, when the patient breathes in, your thumbs move even closer together.
- The chest is usually overinflated and cannot expand normally with inspiration. As the patient breathes in, the diaphragm pulls the ribs closer together.

Paradoxical Breathing
*Inspiration -Abdomen distends (diaphragm goes down)
* Expiration - Abdomen deflates (diaphragm goes up)

- The abdomen should distend (diaphragm goes
down) when patient takes a breath in, and
deflate (diaphragm goes up) as patient expires.
- Paradoxical breathing is seen when the
diaphragm moves in the opposite direction
than expected, leading the patient to distend
their abdomen as they breathe out and deflate
it as they breathe in.
- Associated with chronic obstructive lung
disease.

Pleural Effusion: Causes by mechanism

INCREASED PERMEABILITY
- Exudative
a. Cancer - metastatic or direct spread
b. Infection (acute = bacterial; chronic = TB)
c. Inflammation (rheumatoid or SLE)

REDUCTION IN ONCOTIC PRESSURE
- Transudate (LOW PROTEIN states - liver, kidney disease)

INCREASE IN MICROVASCULAR PRESSURE
- Transudative (CHF)

Kawasaki Disease
*Age range typically 6 months to 8 years
*< 6 months - slit lamp
examination is helpful in this group
*Slightly more prominent in Asian population
*Peaks in winter and spring
*Associated features - Aseptic meningitis/ meningismus, Sterile pyuria/urethritis
*Cardiac manifestations - Pericarditis, Myocarditis, Coronary artery dilation, Coronary artery aneurysm
*Tx: IVIG

Must have fever >38.5 for at least 5 days

Must also have at least 4/5 of:
- Bilateral non-purulent conjunctiva
- Polymorphous rash (often present in perineum/groin area; never vesicular or bullous)
- Extremity changes (erythema in palms and soles; non-pitting edema)
- Oral mucosal changes (dry, fissured and erythematous lips, strawberry tongue, diffuse erythema of oropharynx)
- Cervical lymphadenopathy > 1.5cm

Case: Examination reveals unwell looking child, temp 39.7 degrees
􀁺 Oropharynx is red
􀁺 Conjunctiva are red
􀁺 Polymorphous rash is present
􀁺 Cervical lymph nodes

Tx: Must occur within 10 days of fever onset
IVIG mainstay of therapy
High dose ASA to treat symptoms acutely
Low dose ASA to to avoid complications
Anticoagulation may be necessary
Exercise restriction for 2 months then only
if residual coronary artery disease

Infective Endocarditis vs. Infective Myocarditis

ACUTE INFECTIVE ENDOCARDITIS
- bacterial (S. aureus, Viridans streptococci)
- fungal (immunosupressed patients)
- vegetation on valves
- underlying risk factors: structural heart disease (congenital or acquired), IV drug use, dental, instrumentation of GI tract (gram-negative bugs). recent heart surgeries (prosthetic valve)
- Cases can be classified as definite, possible, or rejected using the Duke criteria. Two of these criteria are pathological, and three are clinical (definitive diagnosis if: 2 major, or 1 major + 3 minor, or 5 minor) - (+) blood cultures for IE, (+) echo. for IE, New valvular regurgitation; Fever, Peripheral findings (Osler's nodes, Roth's spots)
- Left sided valves
- Splenomegaly
- Cardiomegaly
- Peripheral findings
- Presence of a NEW Murmur (Holosystolic - MR; Diastolic decrescendo - AR)
- ECG: Diffuse ST elevation (Pericarditis), AV Nodal Block
- Tx: Antibiotics
- Persistently positive blood cultures, elevated sedimentation rate

MYOCARDITIS
- viruses ( adenovirus, parvovirus B19, hepatitis C, and herpes virus 6; seasonal)
- inspiratory chest pain
- heart failure
- cardiomegaly, hepatomegaly
- S3 or S4
- Friction rub
- ECG: Diffuse ST elevation (Pericarditis)
- 1/3 get better; 1/3 get worse (--> Non-Ischemic Dilated Cardiomyopathy); 1/3 stay the same

Eisenmenger's Syndrome
*A combination of two conditions (#1 R-to-L; #2 L-to-R) with pulmonary HTN
*The most common defects were ventricular septal defects (33 percent), atrial septal defects (30 percent), and patent ductus arteriosus (14 percent)
*Most patients have diffuse central cyanosis, and clubbing involves all extremities equally

First, it involves having a communication, or hole, between two cardiac chambers that allows oxygenated blood to recirculate back into the right ventricle and to the lungs instead of flowing out of the left ventricle to the rest of the body.

Over time, this extra blood flow to the lungs damages their vessels, causing high pressures or pulmonary hypertension that reverses the flow of blood, so the deoxygenated blood goes out to the rest of the body.

Eisenmenger's syndrome refers to this combination of REVERSED BLOOD FLOW with PULMONARY HTN. It results in cyanosis or low oxygen content in the blood and may eventually result in failure of the right ventricle. However, many patients live well into adulthood with the proper care.

VSD vs. AVSD
*L-to-R shunts
*Present with heart failure
*AVSD more common in Down's Syndrome
*AVSD - Left Superior Axis

AVSD
- 1 common valve (AV valve)
- Anatomically it's a L-to-R shunt
- Acts physiologically like a VSD
- Often problems with valve regurgitation
- Down's Syndrome
- Cardiomegaly
- Pansystolic murmur
- ECG: Middle heart missing, septal defect, AV node pushed posteriorly --> Delayed activation of Left ventricle; ***Left Superior Axis

VSD
- Cardiomegaly
- Pansystolic murmur
- Most common lesion (hand grip - increases systemic vascular resistance - increases VSD systolic murmurs)

Acute Rheumatic Fever
*5 - 15 y.o.
*Group A streptococcal infection
*Major manifestations
1) Migratory arthritis (Large joints)
2) Carditis and valvulitis
3) CNS involvement
4) Erythema marginatum
5) Subcutaneous nodules

Acute rheumatic fever occurs most frequently in children from 5 to 15 years of age; it is rare among children in the first three years of life and among adults.

Acute rheumatic fever is characterized by group A streptococcal infection followed by clinical manifestations outlined below.

The five major manifestations are:
• Migratory arthritis (predominantly involving the large joints)
• Carditis and valvulitis (eg, pancarditis)
• Central nervous system involvement (eg, Sydenham chorea)
• Erythema marginatum
• Subcutaneous nodules

The four minor manifestations are:
• Arthralgia
• Fever
• Elevated acute phase reactants [erythrocyte sedimentation rate (ESR), C-reactive protein (CRP)]
• Prolonged PR interval

TB
*Tuberculin skin test generally for asymptomatic patients
*HIGH risk: AIDS, HIV, Silicosis
*95% of primary infections lead to LATENT TB
*Treatment of ACTIVE TB (Mnemonic: RIPE)
R = Rifampin
I = Isoniazid (INH)
P = Pyrazinamide
E = Ethambutol

- Mycobacterium tuberculosis: slow growing AEROBE (doubling time = 18 h) that is capable of
intracellular survival because it REPLICATES IN MACROPHAGES

PRIMARY INFECTION
Usually ASYMPTOMATIC, although progressive primary disease may occur, especially in children and immunosuppressed patients

SECONDARY INFECTION/REACTIVATION
Usually produces CONSTITUTIONAL symptoms (fatigue, anorexia, night sweats, weight loss) and site-dependent symptoms

i. Pulmonary TB
- Chronic productive COUGH +/- hemoptysis
- CXR consolidation or CAVITATION, lymphadenopathy
- Non-resolving pneumonia despite standard antimicrobial therapy

ii. Miliary TB
- widely disseminated SPREAD especially to lungs, abdominal organs, marrow, CNS
- CXR: multiple small2-4 mm MILLET SEED-LIKE LESIONS

iii. Extrapulmonary TB
- lymphadenitis, pleurisy, pericarditis, hepatitis, peritonitis, meningitis, osteomyelitis
(vertebral= Pott's disease), adrenal infection (causing Addison's disease), renal, ovary

INVESTIGATIONS

PPD/Mantoux skin test: positive result only indicates infection, NOT ACTIVE DISEASE (PPD is not
used to diagnose or exclude active TB)

Morning sputum on 3 consecutive days for ACID FAST BACILLI (AFB), culture ± AMID (TB rRNA
assay)

CXR:
- Nodular or alveolar infiltrates with CAVITATION (middle/lower lobe if PRIMARY, apical if SECONDARY)
- Pleural effusions (usually unilateral and exudative)
- Pulmonary nodules
-Hilar/mediastinal adenopathy (especially in CHILDREN)
- Tuberculoma (semi-calcified well-defined solitary coin lesion 0.5-4 cm that may be mistaken
for lung CA) represents ACTIVE or HEALED lesion
- Miliary TB: discrete NODULES (2-4 mm diameter) scattered throughout lungs

EVIDENCE OF PAST DISEASE:
Calcified hilar and mediastinal nodes, calcified focus, pleural thickening with calcification, apical scarring

In patients with TB, T -cells produce increased amounts of IFNy when re-exposed to TB antigen

PREVENTION
- BCG (Bacille Calmette-Guerin) vaccine
80% effective against pediatric miliary and meningeal TB

TREATMENT OF ACTIVE INFECTION

empiric therapy: INH + rifampin + pyrazinamide + ethambutol + vitamin B6

pulmonary TB: INH + rifampin + pyrazinamide x 2 mos (initiation phase), then INH + rifampin x 4 mos (continuation phase), total 6 mos

extrapulmonary TB: same regimen as pulmonary TB but increase to 12 mos in bone/joint, CNS,
and miliary/disseminated TB + corticosteroids for meningitis, pericarditis

Inspiration

Negative pressure in pleural space
Opens alveoli
Increases airway diameter
Reduces resistance
(Airways on expiration smaller than inspiration; expiratory wheezes)
Abdomen distends (diaphragm goes down)

Beta 2 Agaonist & M3 Antagonist

Tx: Asthma
Beta 2 - Smooth muscle relaxation (vascular + other)
--> Bronchodilation

M3 antagonist (anti-cholinergic)
--> Bronchodilation
--> Reduce gland secretion
--> Atropine, Ipatropium (short acting)

SABA (Salbutamol/Ventolin)
LABA (Salmeterol)

Anti-inflammatories
- ICS
- Systemic corticosteroids (prednisone)
- Leukotriene antagonists

Maintenance COPD, Maintenance Asthma, Acute Exacerbations

General Principles
- acute problems are BEST TREATED with SHORT ACTING DRUGS
- Maintenance is BEST TREATED with LONG ACTING DRUGS
- Prednisone long term has MANY BAD SIDE EFFECTS
- Inhaled steroids have FEW SIDE EFFECTS
- Asthma is very INFLAMMATORY
- COPD is somewhat inflammatory

COPD Maintenance Drugs
1 short acting dilator (beta-2 or anti-M3)
1 long acting dilator (LAAC)*
2 long acting dilators (LAAC + LABA)*
2 long acting dilators + ICS*
2 long acting dilators + ICS + theophylline (improvement in dyspnea )*
*= "plus PRN short acting dilator"

NOTE: ICS used LATER ON

Asthma Maintenance Drugs
Short acting Beta-2 agonists
ICS*
ICS + (LTRA or LABA)*
ICS + LTRA + LABA*
* = "plus PRN short beta-2 dilator"

LTRA = Leukotriene Receptor Antagonist
LAAC = Long-acting Anti-Cholinergics

***ICS + long acting Beta-2 agonists (1st LINE)
(the addition of a LABA to a ICS is more
effective at improving asthma control, lung function and reducing exacerbation frequency than increasing, or even quadrupling, the dose of ICS.)

NEVER PRESCRIBE LABA IN ISOLATION in asthma (down regulates Beta-2 receptors)

ACUTE EXACERBATIONS
Short acting B2
Short acting anti-M3
Systemic steroid (prednisone)
Intravenous B2
Antibiotic (infections are most common trigger)
Ventilator
Inhaled anesthetic (sevoflorane - ASTHMA ONLY)

Anti-arrhythmics

Class 1 = Na channel blocker (wide QRS)
Class 2 - Beta-blocker
Class 3 - Potassium channel blocker (longer QT interval)
Class 4 - Calcium channel blocker

Phase 0 influences CONDUCTION SPEED
(Na channel blocker --> Slows down conduction speed)

Phases 2 and 3 influence the REFRACTORY PERIOD
(K channel blocker --> prolongs refractory period)

Phase 4 determines the RATE OF SPONTANEOUS DEPOLARIZATION (PACEMAKER RATE)

Slow a physiological pacemaker (SA Node) - LENGTHEN phase 4

Increase refractory period - LENGTHEN phase 2/3

Increase the HR - SHORTEN phase 4

SLOW down a RE-ENTRY circuit - LENGTHEN phase 0

See More

Please allow access to your computer’s microphone to use Voice Recording.

Having trouble? Click here for help.

We can’t access your microphone!

Click the icon above to update your browser permissions above and try again

Example:

Reload the page to try again!

Reload

Press Cmd-0 to reset your zoom

Press Ctrl-0 to reset your zoom

It looks like your browser might be zoomed in or out. Your browser needs to be zoomed to a normal size to record audio.

Please upgrade Flash or install Chrome
to use Voice Recording.

For more help, see our troubleshooting page.

Your microphone is muted

For help fixing this issue, see this FAQ.

Star this term

You can study starred terms together

NEW! Voice Recording

Create Set