Chapter Summary continued

Tetralogy of Fallot is the most common cause of cyanotic heart disease and is characterized by a classic tetrad of pulmonary outflow obstruction/stenosis, right ventricular hypertrophy, ventricular septal defect, and over-riding aorta.

Transposition of the great arteries is an abnormal development of the truncoconal septum that results in inversion of the aorta and pulmonary arteries with respect to the ventricles. Transposition of the great arteries has a poor prognosis without surgery.

Truncus artenosus is a failure to develop a dividing septum between the aorta and the pulmonary artery, resulting in a common trunk. Truncus arteriosus has a poor prognosis without surgery.

Tricuspid atresia is the absence of a communication between the right atrium and ventricle due to developmental failure to form the tricuspid valve. Tricuspid atresia has a poor prognosis without surgery.

Ventricular septal defect is the most common congenital heart defect and consists of a direct communication between the ventricular chambers. The prognosis varies with the size of the defect.

Atrial septal defect is a direct communication between the atrial chambers whose most common type involves the ostium secundum.

Patent ductus arteriosus is a direct communication between the aorta and pulmonary artery due to the continued patency of the ductus arteriosus after birth.

Dilated cardiomyopathy is the most common form of cardiomyopathy and consists of cardiac enlargement with dilatation of all four chambers, resulting in progressive congestive heart failure. The 5-year survival rate is 25%.

Hypertrophic cardiomyopathy is an asymmetric cardiac hypertrophy that is most prominent in the ventricular septum, where it may obstruct the ventricular outflow trad, with resulting increased risk of sudden cardiac death, particularly in young athletes.

Restrictive cardiomyopathy is an uncommon form of cardiomyopathy caused by diseases such as amyloidosis and sarcoidosis that produce restriction of cardiac filling during diastole.

Carcinoid heart disease is a right-sided endocardial and valvular fibrosis secondary to exposure to serotonin in patients with carcinoid tumors that have metastasized to the liver.

Cardiac myxoma is a benign tumor, usually arising within the left atrium near the fossa ovalis. It an cause tumor emboli and ball-valve obstruction of valves.

Cardiac rhabdomyoma is a benign tumor, usually arising within the myocardium. It is associated with tuberous sclerosis.

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