pediatrics EOR - cardio

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leading cause of mitral valve stenosis in the US
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rash associated with acute RFerythema marginatumdescribe morphology of rash associated with acute RFmacular, erythematous, non-pruritic annular rash with rounded, sharply demarcated borders (erythema marginatum; may have central clearing)complications of acute rheumatic feverrheumatic valvular disease → mitral stenosistreatment for acute RFIV PCN G and aspirin 2nd line: cephalosporins or macrolidesWhat must a patient have in order to diagnose acute RFsupporting evidence of recent GABHS infectionMC congenital heart defectventricular septal defectNon-cyanotic congenital heart defectsAtrial septal defect Ventricular septal defect Patent ductus arteriosus Coarctation of aortaatrial septal defect heart murmursystolic ejection murmur with wide, fixed split S2MC type of ASDostium secundumASD measuring less than _______ in diameter can be managed with observationless than 5 mmtrans - thoracic/esophageal echocardiogram with doppler is the initial diagnostic test of choice in suspected ASDthoracicmurmur associated with patent ductus arteriosuscontinuous machine like murmur heard loudest at pulmonic area (2nd ICS at LSB)risk factors associated with PDAprematurity infants < 1000 g fetal hypoxia in utero alcohol exposure congenital rubellafemales/males are at higher risk for PDAfemalespathophysiology of PDAcontinued prostaglandin E2 production and low arterial oxygen content promote patencytreatment for PDANSAIDS → IV indomethacin or ibuprofenMC clinical presentation of PDAmost are asymptomaticmost likely diagnosis A 2-week-old infant with a history of prematurity presents with a pink torso and upper extremity and blue lower extremities. On cardiac auscultation, you notice a rough, continuous "machinery murmur" heard over the left sternal border at the 2nd intercostal spacePDAbest initial test for PDAechoOther than a continuous machine like murmur, what other PEx findings are c/w PDAwide pulse pressure >30 mmHg including bounding peripheral pulses prominent precodial impulse Loud S2Most likely diagnosis An 8-year-old boy who is seen for the first time in your office. His parents report that he tires easily and often complains of weakness in his legs. Physical exam shows a healthy boy with a blood pressure of 141/91 mmHg. You notice that his lower extremities are slightly atrophic with a mottling appearance. Upon further examination, he is found to have very weak and delayed femoral pulses with a blood pressure of 96/60 in the lower extremities. He has a late systolic ejection murmur on cardiac auscultation.coarctation of the aortacoarctation of the aortacongenital narrowing of the aortic lumen at the distal arch or descending aorta, causing HTN in the UE relative to the LEwhat is most commonly associated with coarctation of the aortabicuspid aortic valve (70%) Turner syndrome (5-15% of girls with CoA have Turner)In coarctation of the aorta, the pre/post -ductal type occurs in adults while pre/post -ductal type occurs in infantspostductal; preductalMC presentation of coarctation of the aorta in adultsHTNneonatal clinical presentation of coarctation of the aortafailure to thrive poor feeding 1-2 wks after birthMurmur associated with coarctation of the aortaharsh systolic murmur along the LSB radiating to the back, left infrascapular region, or chest (ejection murmur at aorta)CXR findings c/w coarctation of the aortaposterior rib notching and figure 3 signwhat is the posterior rib notching on CXR in pt who has CoAdue to increased intercostal artery collateral flowECG findings associated with Coarctation of the aortaleft ventricular hypertrophyif pt has coarctation of the aorta and a bicuspid valve, what are they at higher risk for?cerebral berry aneurysmcoarctation of the aorta treatment-Prostaglandin E1 (PGE in newborns (to keep ductus arteriosus open) -Balloon angioplasty and Stenting -Surgical end-to-end anastomosismost likely diagnosis A 2-week-old newborn brought to the ER by his mom who reports a sudden loss of consciousness during feeding and with crying. She also has noticed that the infant's lips have turned blue on three occasions during feeding. blood pressure is 75/45 mmHg, a pulse is 170/min, and respirations are 44/min. A grade 3/6 harsh systolic ejection murmur is heard at the left upper sternal border. A CXR shows a small boot-shaped heart and decreased pulmonary vascular markings.tetralogy of fallot4 features of tetralogy of fallot1. RV outflow obstruction 2. RVH 3. large unrestrictive VSD 4. overriding aortaMC presentation of ToF in infantscyanosis (blue baby syndrome)tet spellssudden, marked increase in cyanosis followed by syncope, and may result in hypoxic brain injury and deathhow does squatting help relieve tet spellsit increases systemic vascular resistance, causing reversal of shunt and increases PaO2murmur associated with ToFCrescendo-decrescendo, holosystolic at LSB radiating to the back - harsh systolic murmur with loud single S2test of choice for ToFechobuzz word boot shaped heartToFdescribe "boot shaped heart"upturned apex and a concave main pulmonary artery segmentwhat is needed prior to surgical repair of ToF?prostaglandin infusion to maintain a patent ductus arteriosustreatment for ToFsurgical repair usually performed in first 12 months of life (ideally at 3-6 mo of age)most likely diagnosis 5-year-old boy presents to the emergency room with 5 days of fevers, ranging from 102-104°F (38.9-40°C). His mother reports that he also has a bad rash that developed on day 3. On physical exam, he has bilateral conjunctivitis, an extensive morbilliform rash on his trunk with desquamation, a bright red tongue, and swollen hands and feet. Labs are remarkable for elevated C-reactive protein, white blood cell count, and erythrocyte sedimentation rate. He is immediately given intravenous immunoglobulin and aspirin and sent for an echocardiogramkawasaki diseasekawasaki diseasemedium and small vessel vasculitis including the coronary arteries, mostly affecting children where the immune system attacks the arteries and damages endothelial cells of blood vesselsrisk factors for Kawasaki dzchildren 3 mo - 5 yrs old boys asian descent (highest risk)diagnosis of Kawasaki dzhigh grade fever at least 5 days PLUS 4 of the following: - conjunctivitis - rash (polymorphorous) - extremity changes - adenopathy -mucositis (strawberry tongue)Complications of Kawasaki diseasecoronary vessel arteritis/aneurysm → death MI, pericarditis, myocarditistreatment for Kawasaki DzIgG and ASA (self limited)describe rash associated with Kawasaki diseaseerythematous morbilliform rash with desquamation on trunk that may spread (can also look different from this too lolllll)what additional study should be ordered after a pt is diagnosed with kawasaki dzechocardiogram → obtained at time of diagnosis and 6-8 wks afterhypertrophic cardiomyopathy inheritenceautosomal dominant