Diseases of the valves of the heart. Rheumatic disease. Cardiomiopathy

Ventricular Septal Defect (VSD)

A hole in the wall that separates the heart's lower chambers. This occurs when the wall between the ventricles (lower chambers) does not develop fully before the baby is born.

• Ventricular Septal Defect (VSD)

♦ Most common cardiac defect seen in children

♦ Classified according to size of defect, e.g., large, small(<5 mm)

♦ Most occur in membranous portion of interventricular septum

♦ May predispose to infective endocarditis

Small VSD

♦ Infrequent, minor symptoms usually

♦ Systolic murmur with mild shunt

♦ Pansystolic murmur

♦ May close spontaneously as patient

gets older

Large VSD
♦ More serious than small VSDs
♦ Large left-to-right shunts producing:
– Volume overload
– Biventricular hypertrophy
– Pulmonary hypertension
– Eventual reversal of shunt with cyanosis (Eisenmenger’s syndrome)

Cardiomyopathy

Cardiomyopathy is a heart muscle disease that affects how well the heart pumps blood to the body.

Types of Cardiomyopathy

There are different types of cardiomyopathy, such as:
-dilated
-hypertrophic
-restrictive
-arrhythmogenic
each with its own causes and symptoms.

Hypertrophic Cardiomyopathy

• Clinical

♦ 50% familial (autosomal dominant), myosin heavy chain defect

♦ HLA linkage to chromosome 6

♦ Clinical evaluation needed to make diagnosis

♦ Symptoms of left ventricular outflow obstruction

♦ Cause of sudden death during exercise

♦ Treated by beta adrenergic blockage, septal myotomy or myomectomy

• Macroscopic

♦ Asymmetric septal hypertrophy

♦ Plaque in the left ventricular

♦ Mitral valve thickening and enlarged left atrium

• Microscopic

♦ Nonspecific myofiber disarray, hypertrophy, interstitial fibrosis

• Differential Diagnosis