Congenital Heart Disease Flashcards
What are the acyanotic congenital heart diseases ?
(ASD), (VSD), Atrioventricular Septal Defect (AV Canal), (PDA), aortic coarctation, pulmonary stenosis
What is ASD?
opening in the atrial septum permitting free communication of blood between the atria. Seen in 10% of all CHD
What are symptoms of ASD?
Most are asymptomatic but may have easy fatigability or mild growth failure. Cyanosis does not occur unless pulmonary HTN is present.
What will you find on clinical exam with an ASD?
Hyperactive precordium, RV heave, fixed widely split S2. systolic ejection murmur @ LSB. Mid-diastolic murmur heard over LLSB
What causes the murmurs associated with ASD?
Systolic murmur is caused by increased flow across the pulmonary valve, NOT THE ASD. Diastolic murmur is caused by increased flow across the tricupsid valve
What is the treatment for ASD?
Surgical or catherization laboratory closure is generally recommended for secundum ASD. Closure is performed electively between ages 2 & 5 yrs.
Is endocarditis prophylaxis required for ASD?
No because it’s a low velocity shunt. No lesions or venturi effect
What is VSD?
is an abnormal opening in the ventricular septum, which allows free communication between the Rt & Lt ventricles. Accounts for 25% of CHD.
Why does the left to right shunt occur in VSD?
The left to right shunt occurs secondary to PVR being < SVR, not the higher pressure in the LV. This leads to elevated RV & pulmonary pressures & volume hypertrophy of the LA & LV
What are clinical signs of VSD?
harsh holosystolic murmur heard along the LSB, more prominent with small VSD. Prominent S2, Diastolic murmur. hyperactive precordium. Symptoms develop between 1 – 6 months
What are treatment options for VSD?
Surgery: Ages 6-12 mo w/ large VSD & Pulm. HTN. Age > 24 mo w/ Qp:Qs ratio > 2:1. Supracristal VSD of any size, secondary to risk of developing AV insufficiency
What is an AVSD?
incomplete fusion of the endocardial cushions, which help to form the lower portion of the atrial septum, the membranous portion of the ventricular septum and the septal leaflets of the triscupid and mitral valves.
What genetic disease is commonly associated with AVSD?
Down’s Syndrome (Trisomy 21), Seen in 20-25% of cases
What are symptoms of AVSD?
Congestive heart failure in infancy. Recurrent pulmonary infections. Failure to thrive. Exercise intolerance, easy fatigability. Late cyanosis from pulmonary vascular disease w/ R to L shunt
What clinical signs will you notice on physical exam with AVSD?
Hyperactive precordium. Normal or accentuated 1st hrt sound. Wide, fixed splitting of S2. Pulmonary systolic ejection murmur w/thrill. Holosystolic murmur @ apex w/radiation to axilla. Mid-diastolic rumbling murmur @ LSB. Marked cardiac enlargement on CX-Ray
What is treatment for AVSD?
Surgery is always required. Treat congestive symptoms. Pulmonary banding maybe required in premature infants or infants < 5 kg
What is a PDA?
Persistence of the normal fetal vessel that joins the PA to the Aorta. Accounts for 10% of all CHD
What TORCH infection is PDA associated with?
rubella
Which way does blood flow with a PDA?
As a result of higher aortic pressure, blood shunts L to R through the ductus from Aorta to PA. Large PDA, PA pressures are equal to systemic pressures. Leads to increased pulmonary vascular disease
What are signs and symptoms of PDAs?
Large PDA’s can result in symptoms of CHF, growth restriction, FTT. Bounding arterial pulses. Widened pulse pressure . Enlarged heart, prominent apical impulse. Classic continuous machinary systolic murmur. Mid-diastolic murmur at the apex
What is the treatment of a PDA?
Indomethacin, inhibitor of prostaglandin synthesis can be used in premature infants. PDA requires surgical or catheter closure.
What syndrome is pulmonary stenosis associated with?
Noonan’s Syndrome, secondary to valve dysplasia
What heart changes might you see with pulmonary stenosis?
RV pressure hypertrophy ⇒ RV failure. RV pressures maybe > systemic pressure. Post-stenotic dilation of main PA. W/intact septum & severe stenosis ⇒ R-L shunt through PFO ⇒ cyanosis
What are signs and symptoms of pulmonary stenosis?
Asymptomatic w/ mild PS < 30mmHg. Mod-severe: 30-60mmHg, > 60mmHg. Prominent jugular a-wave, RV lift. Split 2nd hrt sound w/ a delay. Ejection click, followed by systolic murmur. Heart failure & cyanosis seen in severe cases.
What is the treatment for pulmonary stenosis?
Balloon valvuloplasty, treatment of choice. Surgical valvotomy is also a consideration
Which syndrome is supravalvular stenosis found in?
Williams Syndrome
What changes with the heart will you see with aortic stenosis?
Pressure hypertrophy of the LV and LA with obstruction to flow from the LV
What are the symptoms of aortic stenosis?
Mild AS may present with exercise intolerance, easy fatigabiltity, but usually asymptomatic. Moderate AS – Chest pain, dypsnea on exertion, dizziness & syncope. Severe AS – Weak pulses, left sided heart failure, Sudden Death
What are the clinical signs of aortic stenosis?
LV thrust at the Apex. Ejection click, systolic murmur @ RSB/LSB w/ radiation to the carotids
What are the treatment options for the different gradients of aortic stenosis?
Because surgery does not offer a cure it is reserved for patients with symptoms and a resting gradient of 60-80mmHg. For subaortic stenosis it is reserved for gradients of 40-50mmHg because of it’s rapidly progressive nature.
What other heart anomaly is coarctation associated with?
Bicuspid aortic valve, seen in > 70% of cases
What genetic syndrome is coarctation seen in?
Turner’s Syndrome
What heart changes are associated with coarctation of the aorta?
Obstruction of left ventricular outflow ⇒ pressure hypertrophy of the LV
What are the vascular symptoms of aortic coarctation?
diminution or absence of femoral pulse. Higher BP in the upper extremities as compared to the lower extremities. 90% have systolic hypertension of the upper extremities. Pulse discrepancy between rt & lt arms
What are the clinical signs of coarctation of the aorta?
acidosis, HF and shock. systolic ejection murmur @ LSB. Cardiomegaly, rib notching on X-ray
What are the treatment options for coarctation of the aorta?
maintaining the ductus with prostaglandin E is essential. Surgical intervention, to prevent LV dysfunction. Re-coarctation can occur, balloon angioplasty is the procedure of choice
What are the four features of the tetralogy of Fallot?
Malalignment VSD, Overriding Aorta, Pulmonic Stenosis, RVH
What is surgical treatment for tetralogy of Fallot?
creation of systemic-pulmonary shunt or complete repair (takedown of prior shunt, patch VSD, resection of subpulmonic obstruction, transannular patch around pulmonic valve annulus)
What is transpotition D-type?
PA arises from LV, Aorta from RV and anterior/right of PA
What is treatment of transposition D-type?
initially with prostaglandin to keep ductus open and balloon atrial septostomy to improve systemic saturation. repair via “atrial switch” Mustard procedure
What is transposition L-type?
Atrial-ventricular AND ventricular-arterial discordance. Physiologically correct, anatomically incorrect. RV is systemic ventricle, TV is systemic AV valve
What are complications of L-type transposition?
Progressive Heart Failure. Arrhythmias (SCD, AV block, Atrial arrhythmias). Severe AV (tricuspid) regurgitation
What is the clinical presentation of Ebstein’s anomaly?
Pediatric: murmur. Adult (unrepaired with ASD): atrial arrhythmias, murmur, cyanosis, R to L shunt NOT due to PulmHTN but TR jet directed across ASD
What is Eisenmenger’s Syndrome?
Final common pathway for all significant L to R shunting in which unrestricted pulmonary blood flow leads to pulmonary vaso-occlusive disease (PVOD); R to L shunting/cyanosis devleops
What are complications of Eisenmenger?
Coagulopathy/platelet consumption, Brain abcesses, Cerebral microemboli, Airway hemorrhage
What is treatment for Eisenmenger?
Once diagnosis established, avoid aggressive testing as many patients die during cardiovascular procedures. Diuretics prn, oxygen. Definitive: Heart Lung transplant
What are the cyanotic congenital heart defects?
TOF, pulmonary atresia, tricuspid atresia, transposition of great vessels, truncus arteriosus, single ventricle with pulmonic stenosis
