It is the second common congenital heart disease after congenital aortic valve stenosis. Many are asymptomatic until adult age.
The defect results in slight to moderate left to right shunting of blood. The shunt is more marked if the septal defect is associated with mitral valve prolapse. Pulmonary hypertension is a rare sequela. A systolic murmur is heard on the left side of the sternum. It is tolerated during pregnancy. However, the defect is better being surgically corrected before pregnancy. Peripartum prophylaxis against SBE is required for all cases with atrial septal defect.
1. Development of cardiac failure in the last month of pregnancy or within 5 months after delivery
2. Absence of an identifiable cause for the cardiac failure
3. Absence of recognizable heart disease prior to the last month of pregnancy, and
4. Left ventricular systolic dysfunction demonstrated by classic echocardiographic criteria such as depressed shortening fraction or ejection fraction.
In 1997, the National Heart, Lung, and Blood Institute and the Office of Rare Diseases convened a workshop that established the following diagnostic criteria (Pearson and associates, 2000): Persistence > 6 months postpartum carries a bad prognosis
signs and symptoms: CHF Dyspnea ,orthopnea, cough, palpitations, and chest pain (Sheffield and Cunningham, 1999).
The hallmark finding usually is impressive cardiomegaly ( Fig. 44-3).
Echocardiographic : EF < 45 percent or a fractional shortening < 30 percent, or both, and an end-diastolic dimension greater than 2.7 cm/m2 (Hibbardand colleagues, 1999)
FIGURE 44-3 Cardiomyopathy with mild pulmonary edema. Anterior-posterior projection chest radiograph of a woman with an abnormally enlarged heart ( black arrows) and mild perihilar opacification consistent with dilated cardiomyopathy.
Management. Therapy consists of treatment for heart failure
Diuretics are given to reduce preload, and afterload reduction is accomplished with hydralazine or another vasodilator.
Because of marked fetal effects angiotensinconverting enzyme inhibitors are withheld until the woman is delivered
Digoxin is given for its inotropic effects unless complex arrhythmias are identified.
Long-Term Prognosis There seems to be no doubt that women with peripartum cardiomyopathy who regain ventricular function within 6 months have a good prognosis (Lampert and associates, 1997).
This autosomal dominant disease of connective tissue can result in significant complications including dissection of the aorta and aortic rupture.
The defect is in fibrillin which is a constituent of elastin and is a general defect. However, aortic dilatation and dissecting aneurysm are the most serious complications and are more common during pregnancy.
Marfan ‘s Syndrome: A condition, due to a defect in fibrillin (an essential component of elastic fibers), in which myxomatous degeneration is common.