Congenital Heart Disease Flashcards
A 32 yo Down Syndrome pt presents with a holosystolic murmur, atrial arrhythmias, and clubbing. You see a prominent RV impulse and hear a 2nd heart sound. What test should you order? What are you looking for?
Ostium Primum ASD
ECG, CXR, Echo, 2D Echo, Doppler, or echo-contrast
Look for a defect adjacent to the AV vales and deformed/regurgitant valves
A pt with early signs of Eisenmenger’s syndrome presents with differential cyanosis (toes only). On exam you hear a thrill and a continuous “machinery” murmur w/ a late systolic accent. What do you suspect? What is the pathological cause?
Patent Ductus Arteriosus
The vessel leading from the bifurcation of the pulm artery to the aorta didn’t close off at birth
Blood flows from the pulm a to the aorta
A pt presents with a short systolic murmur, normal S2 and signs of severe pulmonary HTN. Echo shows a small single mid-muscular septal defect and aortic valve regurgitation. What complications are you worried about? What treatment do you recommend?
Complications: RV outflow tract obstruction and Hemodynamic pattern (like Tet)
Tx:
Small VSDs spontaneously close
Surgery or a trans-catheter closure can be done
A 38 yo pt presents with cyanosis and clubbing. On exam you see a palpable pulmonary artery and hear a split 2nd heart sound. You order an Echo which shows abnormal ventricular septal motion and a midseptal defect. What is the diagnosis? What treatment do you recommend assuming it is an uncomplicated case and pulm/systemic flow is > 1.5?
Ostium Secundum ASD
Surgery (patch, percutaneous transcath device closure)
A pt presents with DOE, chest pain, syncope, hemoptysis, cyanosis, and clubbing. Doppler shows a R-to-L shunt. What is the likely diagnosis? What is the treatment?
Eisenmenger’s Syndrome
Surgery has a good prognosis if Tx’d early
A male pt presents with a harsh systolic murmur. An echo shows a vegetation on the biscuspid valve, a dilated ascending aorta, and LVH. What is the most likely diagnosis? What is the most likely pathological cause?
Congenital Aortic Stenosis
Bicuspid aortic stenosis is MC cause
d/t obstructed LV flow
What is the most common cardiac birth defect?
Ventricular Septal Defect
You find your pt has pulmonary arterial HTN. During your work-up you perform an echo which shows pulmonary artery dilation and a defect high in the atrial septum. What do you suspect? If the pt had no significant symptoms, what medical management would you recommend?
Sinus Venous ASD
Anti-arrhythmic medications
Treat pulm HTN
Treat any respiratory tract infections
You perform an echo on your pt and see a membranous diaphragm/fibromuscular ring circling the LV outflow tract below the aortic valve. You suspect the jet impact is causing progessive fibrosis. What treatment do you recommend for this pt?
Subaortic stenosis
Excision of the membrane/fibromuscular ring
Your pt presents with a holosystolic murmur and a normal S2. After running tests you diagnose restrictive VSD. What contraindications or other complications are you worried about?
Pregnancy and BCP - CI
Erythrocytosis - Tx w/ phelbolomy
Iron-Deficiency - Tx w. Fe repletion
Hyperviscosity - Tx by removing blood & replacing w/ saline
A pt presents with an elfin face and mental retardation. He is cheerful and loves music. An echo shows dilated, tortuous, sclerotic arteries. What disease do you suspect?
Supravalvular Aortic Stenosis
Describing Williams-Beuren syndrome
A pt presents with a harsh systolic murmur, thickening & calcification of the aortic valve, LVH, and a dilated ascending aorta. How do you treat this pt? What if he had a critical obstruction? What do you do if surgery is CI’d in this pt?
Congenital Aortic Stenosis
Digoxin, diuretics, B-blockers, or decrease Na
Surgery (aortic valve replacement) if critical obstruction
Aortic balloon valvuloplasty when surgery is CI’d
A pt presents with a thrill, continuous “machinery” murmur, and a late systolic accent. He has blue toes (fingers are fine) and a Doppler shows a R –> L shunt. What do you suspect? What treatment do you recommend if the pt doesn’t have severe pulmonary Dz? What if the pt tells you he had a heart infection 2 months ago?
Patent Ductus Arteriosus
Closure by surgical ligation
Wait several months post-IE
What are the 3 types of VSD & what characterizes them?
Restrictive VSD - holosystolic murmur, norm S2
Muscular VSD - short systolic murmur
Loud Short Systolic VSD - murmur, single S2
A pt with Williams-Beuren syndrome presents. What is the underlying pathophysiology of his disease? What associated congenital heart disease does he have?
Supravalvular Aortic Stenosis
Localized, diffuse narrowing of the ascending aorta
A pt presents with a prominent jugular venous pulse but is otherwise asymptomatic. On exam you find hear S4 and a harsh systolic crescendo-decrescendo murmur and thrill. What disease do you suspect? What is the pathological cause?
Pulmonary Stenosis
Supravalvular, valvular, or subvalvular RV outflow obstruction
What are the 4 components of Tetralogy of Fallot?
- Malaligned VSD
- RV outflow obstruction
- Overriding aortic
- RV hypertrophy
A 48 yo male presents with HA, epistaxis, and cold extremities. He mentions that he gets leg pain during exercise. You hear a heart murmur, note that his BP is 155/95, and find delayed femoral pulses. ECG shows LVH and a CXR shows a dilated L subclavian and ascending aorta. What is the diagnosis? How do you treat?
Coarctation of the Aorta
Surgical resection
Percutanous catheter balloon dilation w/ stent
A pt is brought in with severe cyanosis. ECG shows RVH and CXR reveals a boot-shaped heart, prominent RV, and diminished pulm markings. What is your diagnosis?
Tetralogy of Fallot
What is the most common form of RV obstruction?
Valvular pulmonic stenosis
Which congenital heart disease occurs when an ASD is present allowing oxygenated blood to reach the RV and there is a total anomalous pulmononary venous connection through which oxygenated blood is rerouted to the RA
Total Anomalous Venous Return
Your 59 yo male pt presents for his annual physical. Although he is asymptomatic you order an echo and a CXR. What findings would you expect to see if your pt had coarctation of the aorta?
ECG - LVH
CXR - dilated L subclavian artery and ascending aorta
Your pt presents with complete heart block. In the past he has had progressive RV dysfunction and Ebstein-type anomalies. What disease does he have? What is the underlying pathology?
Complete Transposition of the Great Arteries
Dexxtro-transposition
RV connects to aorta (oxy blood –> LA –> RV –> aorta)
LV connects to pulmonary arteries (deoxy blood –> RA –> LV –> pulm trunk)
A pt brings in her child who has severe cyanosis, especially during crying and feeding. CXR shows a boot shaped heart, prominent RV, concave pulmonary conus, and diminished pulmonary markings. What disease are you worried about? What treatment do you recommend?
Tetralogy of Fallot
Balloon or blade catheter
Surgically create intraatrial communication
Severe obstruction: create systemic pulmonary artery anastomoses
