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The condition is associated with Turner's syndrome
6%
3/51
The condition is the most common congenital cardiac anomaly
10%
5/51
A boot-shaped heart will be found on chest radiograph
80%
41/51
The condition may be treated with indomethacin
2%
1/51
A chest radiography would reveal notching of the ribs
0%
0/51
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This patient has cyanotic spells, relieved by squatting, consistent with Tetrology of Fallot (TOF). One can expect a boot-shaped deformity on chest radiograph due to right ventricular hypertrophy. In TOF, patients have the classic clinical features of pulmonary stenosis (the most important determinant for prognosis), right ventricular (RV) hypertrophy, and an (overriding) aorta that lies directly over a ventricular septal defect (VSD). Because of pulmonary stenosis (elevated RV pressures) and the overriding aorta, patients experience early cyanosis from right to left intracardiac shunting. Patients may squat to improve symptoms because compressing the femoral arteries increases total peripheral resistance and decreases the right to left shunt, directing more blood from the right ventricle to the lungs and less across the VSD and into the aorta. Saenz discusses caring for infants with congenital heart disease and their families. Congenital heart disease may be classified as acyanotic or cyanotic. The most common acyanotic lesions are ventricular septal defect, atrial septal defect, atrioventricular canal, pulmonary stenosis, patent ductus arteriosus, aortic stenosis, and coarctation of the aorta. The most common cyanotic lesions are tetralogy of Fallot and transposition of the great arteries. In these infants, hypoxia is a bigger problem than congestive heart failure. Frank and Jacobe discuss evaluation and management of heart murmurs in children. The vast majority of murmurs are not pathologic. Elements in the medical history that suggest that the murmur is pathologic include a family history of sudden cardiac death or congenital disease, in utero exposure to toxins such as alcohol, maternal diabetes, rheumatic fever, and Kawasaki disease. Illustration A represents the classic boot shaped deformity found in tetrology of Fallot. Incorrect Answers: Answer 1: Turner's syndrome is associated with coarctation of the aorta. Answer 2: The most common congenital cardiac anomaly is a ventricular septal defect. Answer 4: A patent ductus arteriosus may be treated with indomethacin. Answer 5: Notching of the ribs would be found in a patient with coarctation of the aorta.
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