congenital heart disease II Flashcards
What is coarctation of aorta with theories for embryological basis and correlated disease
Narrowing of the aortic lumen. Theories: extension of ductal tissue into aortic arch, disturbance of subclavian migration, decreased blood flow in fetal isthmus resulting in poor development of this area. 15% of patients with Turner’s Syndrome have a coarctation
Narrowing of the aortic lumen. Theories: extension of ductal tissue into aortic arch, disturbance of subclavian migration, decreased blood flow in fetal isthmus resulting in poor development of this area. 15% of patients with Turner’s Syndrome have a coarctation
Anatomy of aortic coarctation
Localized intraluminal projection of a shelf from the lateral, posterior, and sometimes, anterior wall of the aorta in the region of the ductus arteriosus. Isthmus and descending aorta can dilate on either side of the coarctation
Localized intraluminal projection of a shelf from the lateral, posterior, and sometimes, anterior wall of the aorta in the region of the ductus arteriosus. Isthmus and descending aorta can dilate on either side of the coarctation
Describe perfusion in aortic coarctation
Poor descending aorta perfusion occurs due to low pressure.Decreased blood flow to the bowel (necrotizing enterocolitis), Decreased blood flow to leg muscles (claudication), Decreased blood flow to the kidneys (increased RAAS)
Aortic coarctation clinical presentation
May be asymptomatic as a newborn b/c ductus arteriosus allows adequate post-coarctation flow. When ductus closes, Tachypnea, diaphoresis, poor feeding, can present in shock with cardiac failure. Childhood: systemic HTN, lower extremity claudication, headaches. Adult: systemic HTN
May be asymptomatic as a newborn b/c ductus arteriosus allows adequate post-coarctation flow. When ductus closes, Tachypnea, diaphoresis, poor feeding, can present in shock with cardiac failure. Childhood: systemic HTN, lower extremity claudication, headaches. Adult: systemic HTN
Diagnosis of aortic coarctation- physical exam
Tachycardic, Blood pressures different btw upper and lower extremities, pulmonary rales +/- hepatomegaly, loud S2, S3, soft systolic murmur, systolic click if bicuspid aortic valve associated. Absent/weak femoral pulses
Aortic coarctation ECG
Varies with age. Infants: right axis deviation due to increased workload of RV, RVH. Children: modest increase in LV forces. Adults: ST depression, T wave flattening or inversion (strain pattern)
Aortic coarctation CXR
Normal right after birth. Signs of cardiac failure: cardiomegaly, prominent pulmonary arterial markings, pulmonary edema. 3 signs in older children/adults: aortic knob, coarctation, post stenotic dilation
Normal right after birth. Signs of cardiac failure: cardiomegaly, prominent pulmonary arterial markings, pulmonary edema. 3 signs in older children/adults: aortic knob, coarctation, post stenotic dilation
Management of aortic coarctation
Infants: prostaglandins until surgery, then end-to-end anastomosis surgical repair. Young child: Balloon angioplasty vs surgery. Adults: surgery vs stent
Natural history of aortic coarctation and causes of death
Development of collateral arteries to supply tissue/organs below obstruction, Blood pressure differential btw arms and legs (arms are high, legs are low). If untreated, causes of death include heart failure, aortic rupture/dissection, infective endocarditis, cerebral hemorrhage
Aortic stenosis anatomy
Bicuspid valves can become stenotic, underdeveloped aortic valves can result in severe AS in infancy. Stenosis leads to pressure gradient btw LV and aorta
Aortic stenosis physical exam
LV lift is palpable, systolic ejection murmur at right upper sternal border radiating to neck and apex, systolic ejection click over apex
aortic stenosis ECG
normal if AS is mild, LVH with S-T segment depression and T wave inversion in the left precordial leads (“strain”)
normal if AS is mild, LVH with S-T segment depression and T wave inversion in the left precordial leads (“strain”)
Aortic stenosis clinical presentation
Fatigue, exertional dyspnea, chest pain, syncope
Aortic stenosis management
if mild, no intervention. Moderate AS (25-49mmHg gradient): no intervention but restrict from high intensity competitive sports. “Intermediate” group of AS (50-79mmHg): Management controversial and patient and physician dependent. Severe AS (>80mmHg): Intervention with balloon dilation or surgery is recommended. Valve replacement
Hypoplastic left heart syndrome anatomy
underdevelopment of the aorta, aortic valve, left ventricle, mitral valve and left atrium. Has single ventricle physiology where aortic and pulmonary artery saturations are equal.
