Congenital Heart and Rheumatic Heart Disease

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Congenital Heart and Rheumatic Heart Disease

Congenital Heart Disease prevalence:

8/1000 live births, most prevalent with chromosomal abnromalities (down syndrome), 70-85% live to adulthood.

Congenital Heart disease complications:

Respiratory infections, CHF, hypoxemia, growth trstriction, developmental delays, pulmonary vascular disease/pulmonary hypertension, failure to thrive, death, heart failure

Assessment of Cardiac Function: Obstetrical hx

Genetic predisposition (siblings or parents), maternal disease (rubella, IDDM), maternal age >40, maternal alcoholism, environmental triggers, down syndrome

Assessment of cardiac function: Current Hx:

Feeding problems, FTT, respiratory distress, frequent respiratory infection, exercise tolerance

Assessment of cardiac function: Physical Exam:

Cyanosis, pulse ox, clubbing of fingers and toes, edema, weak, absent or irregular pulses (compare upper and lower pulses), murmur (Apetoman)


CXR, ECG, Echocardiogram, cardiac catheterization, exercise stress test, MRI

Circulation of Blood through the heart:

SVC, Deoxy blood to RA, Triscuspid valve, RV, pulmonary arteries, lung, PV, LA, bicuspid or mitral valve, LV, aorta valve, aorta, to rest of body

Acyanotic defects: Left to right shunt:

Atrial Septal Defect (ASD) and Ventricular Septal Defect (VSD): Opening in wall, fusion defect. Patent ductus arteriosus (PDA)

Left to right shunt: Oxygen --> left ventricles to lungs:

Through ASD or VSD, increases work load of right side. Increased pulmonary blood flow, more pressure on left side (Tachypnea, dyspnea, pulmonary edema, pulmonary hypertension), heart failure, right ventricular hypertrophy, reverse right to left shunt.

Left to right shunt S/s:

Cardiac (Tachycardia, Right ventricular hypertrophy)
Pulmonary (Tachypnea, dyspnea, pulmonary edema, pulmonary artery hypertension)

ASD (Atrial Septal Defect):

Abnormal opening b/t right and left atria. Altered hemodynamics: L atrial pressure > R atrial pressure so oxygenated blood flows into right atrium -->RV to lungs, Extra volume is well tolerated by R ventricle

ASD clinical manifestations:

Systolic ejection murmur at 2nd-3rd interspace along LSB. Will hear in pulmonic area near pulmonic valve.

Management of ASD:

If defect is small, it may close spontaneously, if moderate to large, surgical closure by 2-4 years of age is indicated

Ventricular Septal Defect (VSD):

Abnormal opening b/t left and right ventricles. Blood flows from L ventricle to R ventricle.

VSD Clinical manifestations:

Loud, harsh, pansystolic murmur, FTT, can hear it everywhere, child older than 2 years may have: Frequent respiratory infection, fatigue, right sided hypertrophy and CHF (Digoxin slows HR and strengthens beat)

VSD management:

If defect is small and child is asymptomatic (or symptoms can be controlled), wait and hope for spontaneous closure, If defect is large or if symptoms cannot be controlled by digoxin and diuretics - open heart surgery by age of 1 year.

Patent Ductus Arteriosus (PDA)

Common in preterm babies, ductus arteriosus doesn't close after birth, PA has lower pressure than aorta, oxygenated blood shunts from aorta into PA, oxygenated blood recirculated through lungs. Increased blood flow to lungs

PDA clinical manifestations:

Murmur at mid upper LSB, enlargement of L atrium, L ventricle, and R ventricle. Leads to CHF, frequent respiratory infections and FTT

PDA management:

May close spontaneously, surgical ligation of patent ductus, with critically ill NB, may attempt to close ductus with indomethacin

Acyanotic Obstruction defects:

Traffic jam, bottlenecks and backs up.

Coarctation of aorta:

Increased blood flow to head and extremities from back up. Narrowing of aorta after aorta splits off to brain, location and degree of stricture vary, Altered hemodynamics: Increased pressure proximal to the defect, decreased pressure distal to the defect

Coarctation of aorta clinical manifestations:

Marked difference in BP of upper and lower extremities, weak or absent femoral pulses, infants usually show some signs of FTT and CHF, hypertension, dizziness, fainting, headache, epistaxis, murmur may or may not be present

Coarctation of Aorta management:

Surgical repair recommended within first 2 years. Resection with end to end anastomosis or enlargement using graft.

Aortic stenosis:

Narrowing or stricture of aortic outflow tract. Several types: Valvular, subvalvular, supravalvular. Altered hemodynamics: Resistant to ejection of blood from LV, LV hypertrophy, increased LA pressure, increased pressure in pulmonary veins, pulmonary congestion/edema, backs up LA --LV and to lungs

Aortic stenosis clinical manifestations:

Fainting, epigastric or anginal pain, exercise intolerance (dyspnea, fatigue), dizziness after prolonged standing, murmur heard at 2nd intercostal space, URSB

Aortic stenosis management:

Commisurotomy, incise fibrous ring in subvalvular type, valve replacement

Pulmonic stenosis:

Narrowing at entrance to pulmonary artery, cuspus of valve fused or malformed. Altered hemodynamics: Blood cannot glow readily from RV to PA

Pulmonic stenosis clinical manifestations:

Pulmonic edema/systemic edema, systolic ejection murmur heard at 2nd intercostal space of LSB, dyspnea on exertion, fatigue, majority of children with PS are asymptomatic, G &D normal.

Pulmonic stenosis management:

If mild stenosis -watch, balloon angioplasty, pulmonary valvulotomy

Cyanotic defects:

Right to left shunt, tetralogy of fallot

Right to left shunt:

Deoxygenated blood shunted to left side. Hypoxemia from deoxygenated blood being pumped to body. Results: Polycythemia, increased H&H, risk for thrombus (CVA), acidosis

Tetralogy of Fallot (Tetra means 4)

Most common cyanotic defect, actually consists of 4 defects.
1. Pulmonary stenosis
2. VSD (usually large and high in septum)
3 Overriding aorta
4. RV hypertrophy

Tetralogy of Fallot: Altered hemodynamics:

Degree of pulmonary stenosis detemines blood flow, will have R to L shunting of blood through VSD if pulmonary stenosis is significant, unoxygenated blood is forced through VSD and out overriding aorta to systemic circulation. Mixing of deoxy and oxy blood.

Tetralogy of fallot clinical manifestations:

1. Newborns may not be cyanotic initially
2. When PDA closes, pulmonic stenosis becomes more severe over time.
3. Infants may have "tet" spells (desat, gets blue, squatting, knee chest position with babies)
4. Older child characteristically assumes a squatting position but is rarely seen today due to early repair
5. Clubbing of fingers caused by chronic tissue hypoxia
6. Developmental delays
7. Pansystolic murmur at LLSB
8. Polycythemia may lead to CVA

Tetralogy of Fallot management:

Repair early, prior to surgery, supplemental iron to prevent iron deficiency anemia, keep well hydrated, avoid respiratory infection. Lifelong: Abx prophylaxis and good dental hygiene to prevent bacterial endocarditis

Cyanotic: Mixed defects:

Transportation of great vessels, truncus arteriosis, hypoplastic left heart syndrome, congenital heart disease

Hypoplastic left heart:

LV is under developed. Severely under developed left side of heart, stenosis or closure of mitral and aortic valves, may be asymptomatic until PDA closes, can cause sudden cyanosis and death

Transportation of the great vessels:

Pulmonary artery comes off the LV, aorta exits from RV. Altered hemodynamics: Not compatible with life unless there is also a VSD, ASD, or PDA to allow mixing of blood. Can give prostaglandin to keep PDA open.

Transportation of the great vessels clinical manifestations:

Cyanosis always present, CHF within first weeks of life, hyperpnea, murmur related to accompanying defect

Transportation of the Great vessels management:

Palliative measures until surgery can be done. Prostaglandin to NB to keep PDA open, enlarge septal defect at time of cardiac catheterization with balloon allows more mixing of blood. Corrective surgery (Jantene procedure)

Truncus Arteriousus:

Single vessel arises from both ventricles with overriding a VSD. Single valve is often malformed, stenosed or incompetent. Mixed blood is not oxygenated.

Truncus Arteriousus: Altered hemodynamics:

Blood from L and R ventricles mixes in truncus, blood flow to lungs is greater

Truncus Arteriousus clinical manifestations:

Cyanosis, CHF, systolic ejection murmur at LSB, bounding pulses and widened pulse pressure due to increased pulmonary blood flow

Truncus Arteriosus management:

First month- aggressive treatment of CHF, surgical correction involves closing of VSD


Complication of congenital heart defects, Inability of the heart to pump an adequate amount of blood to the lungs (systemic circulation) to meet the body's metabolic demands.

CHF Due to one of the following:

1. Volume overload (L to R shunts)
2. Pressure overload (obstructive lesions)
3. Decreased contractility (cardiomyopathy)
4. High output demands (sepsis, anemia)

CHF: left sided failure: Backs up to lungs:

LV end diastolic pressure rises --> increases pressure in pulmonary veins -->lung congestion --> pulmonary edema --> pulmonary HTN.

CHF: Right sided failure: Backs up to vena cava, systemic edema:

RV end diastolic pressure rises -->increases central venous pressure--> systemic venous engorgement--> systemic venous hypertension-->hepatomegaly and edema

CHF Compensation:

Initially heart tries to compensate in two ways.
1. Hypertrophy of cardiac muscle
2. Tachycardia

CHF S/S pulmonary congestion: Left sided failure:

1. Tachypnea
2. Dyspnea
3. Orthopnea
4. Pleural effusion
5. Crackles, cough
6. Tachycardia
7. Diaphoresis
8. Weakness, fatigue, poor exercise tolerance
9. Irritable, restless
10. Decreased urine output
11. Pale, cool extremities
12. Weak peripheral pulses
13. Decreased blood pressure
14. Cardiomegaly
15. Gallop rhythm (S3 and S4)
16. Anorexia

CHF s/s systemic congestion: right sided failure:

1. Sudden weight gain.
2. Edema
3. Ascites, pleural effusions
4. Neck vein distention or bulging fontanels
5. Hepatomegaly
6. Developmental delays

CHF management:

Reduce workload of heart and improve cardiac function.


Slows and strengthens pulse, monitor for signs of toxicity, low pulse, vomiting, halos, teach parents home care.

Angiotensin converting enzyme ACE inhibitors:

Inhibits reabsorption of water and salt.


Monitor for s/s of hypokalemia, fluid restriction, sodium restriction, decrease cardiac demands


Caloric needs increase, breast v/s bottle, feedings take longer.

Relieve respiratory distress:

Oxygen, position, knee chest

Prevent infection:

Protect from respiratory infections

Prevent skin breakdown

Support parents and child, promote bonding

Rheumatic heart disease manifestations:

Strep infection 2-3 weeks prior, carditis, arthritis, chorea, chronic progressive damage to heart and valve

Rheumatic Heart disease assessment:

Hx sore throat, skin: maculopapular rash, neuro chorea: jerky movements, CV- murmur, musculoskeletal - painful joints

RHD therapeutic management:

Eradicating the bacteria: 10 days of PCN or erythromycin, Treat inflammation and fever (steroids and nsaids), prevent permanent heart damage( monthly injections of pVK, dental prophylaxis)

RHD nursing mgmt:

Teaching to assure compliance with abx, allow verbalizations of frustration of chorea, pain mgmt, steroids, nsaids

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