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

♦ Approximately 7% of congenital heart abnormalities

♦ Caused usually by valve cusp fusion

♦ In case of pulmonary atresia, right ventricle will be

hypoplastic

Tetralogy of Fallot

Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are:
1.pulmonary stenosis, which is narrowing of the exit from the right ventricle;
2.a ventricular septal defect, which is a hole allowing blood to flow between the two ventricles;
right ventricular hypertrophy, which is 3.thickening of the right ventricular muscle; and
4.an overriding aorta, which is where the aorta expands to allow blood from both ventricles to enter.

• Tetralogy of Fallot

♦ Most common cyanotic congenital anomaly

♦ Presents at birth with severe cyanosis

♦ Poor prognosis if not corrected surgically

♦ Four classic anatomical findings:

– Ventricular septal defect (VSD)

– Dextroposed aorta which overrides right ventricular outflow tract, and leads to:

– Pulmonary stenosis, which leads to:

– Right ventricular hypertrophy

Transposition of Great Arteries

Transposition of the great arteries (TGA) is a congenital heart defect in which the two main arteries leaving the heart are reversed. This means that the aorta is attached to the right ventricle, and the pulmonary artery is attached to the left ventricle, instead of the normal arrangement.

• Transposition of Great Arteries

♦ Approximately 4% of congenital heart defects

♦ Cyanosis at birth

♦ Child doesn’t survive without some interatrial communication (PDA, VSD)

♦ Can be corrected surgically

♦ Grossly, aorta arises from morphologic right ventricle and is anterior and to right of pulmonary artery (normal lie posterior and to right of pulmonary artery)