Cardiac Pathology

6. Tricuspid atresia a. Definition: absence of a communication between the right atrium and ventricle due to developmental failure to form the tricuspid valve b. Associated defects: right ventricular hypoplasia and an ASD

c. Poor prognosis without surgery

7. Ventricular septal defect (VSD)

a. Most common congenital heart defect b. Definition: direct communication between the ventricular chambers c. Small VSD

i. May be asymptomatic and close spontaneously ii. May produce a jet stream that damages the endocardium and increases the risk of infectious endocarditis d. Large VSD may lead to pulmonary hypertension, RVH, reversal of the shunt, and late cyanosis (Eisenmenger complex)

e. Auscultation: systolic murmur f. VSDs are commonly associated with other heart defects g. Treatment: surgical correction of large defects

8. Atrial septal defect (ASD)

a. Definition: direct communication between the atrial chambers b. Most common type: ostium secundum c. Complications i. Eisenmenger syndrome ii. Paradoxical emboli

9. Patent ductus arteriosus (PDA)

a. Definition: direct communication between the aorta and pulmonary artery due to the continued patency of the ductus arteriosis after birth b. Associated with prematurity and congenital rubella infections c. Clinical: machinery murmur, late cyanosis, and CHF

d. Complication: Eisenmenger syndrome E. The Cardiomyopathies

1. Dilated cardiomyopathy a. Most common form of cardiomyopthy b. Definition: cardiac enlargement with dilatation of all four chambers resulting in progressive congestive heart failure c. Etiology i. Idiopathic (majority of cases)

ii. Alcohol iii. Drug related—adriamycin (doxorubicin) and cocaine iv. Viral myocarditis—Coxsackievirus B and enteroviruses v. Parasitic infections—Chagas disease vi. Pregnancy related d. Echocardiogram: decreased ejection fraction e. Presentation: progressive CHF

f. Complications: mural thrombi and cardiac arrhythmias

Bridge to Embryology

In utero the ductus arteriosis is kept open by low arterial oxygen saturation and elevated prostaglandin E2 (PCE2) levels. Functional closure occurs in the first 2 days of life due to increased oxygen saturation and decreased PGE2.

Bridge toJPharmacology

To keep PDA open: prostaglandin E2

To close PDA: indomethacin

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