SUMMARY.A 19-mm St. Jude Medical valve prosthesis was successfully implanted in a 5-kg, 7-month-old infant with congenitally corrected transposition and severe systemic atrioventricular valve regurgitation. The clinical course and ten-month postoperative catheterization data are reported.
KEY WORDS: Prosthetic atrioventricular valves m InfantsProsthetic heart valves have been used in pediatric patients with increasing frequency over the last decade [5,14]. There is much information on the use of the St. Jude Medical valve prosthesis in adolescents but little on its use in infants. We report our experience with a St. Jude valve inserted in a 5-kg, 7-month-old child with corrected transposition (atrioventricular [AV] and ventriculoarterial discordance) and severe left AV valve regurgitation.
Case ReportThe child was the 3.3-kg product of a full-term pregnancy. At 3 weeks of age, he was brought to a community hospital in congestive heart failure, was treated with digitalis, and was transferred to the University of Maryland Hospital, Baltimore. Respiratory distress was present; respiratory rate was 60 per minute, pulse rate was 200 per minute, and systolic blood pressure was 90 mm Hg; rales were heard throughout the lungs. Cardiac examination revealed increased precordial activity and a gallop rhythm. A grade 3/6 holosystolic murmur was heard at the left lower sternal border and radiated to the apex. The liver was palpable 2 cm below the lower right costal margin. The remainder of the physical examination findings were within normal limits. The admission chest x-ray film revealed cardiomegaly and prominent pulmonary vascular markings (Fig. 1, left). An ECG showed sinus rhythm, an axis of 30 ~ , and evidence of left ventricular hypertrophy. No Q waves were seen in leads V~ or V6. Two-dimensional echocardiography demonstrated a levotransposed aortic valve and an "Ebstein-like malformation" of the left AV valve with displacement of the valve leaflets into a dilated systemic ventricular cavity. Cardiac catheterization confirmed the diagnosis of "corrected transposition" with intact atrial and ventricular septa and marked insufficiency of the systemic AV valve. The patient's respiratory distress responded to digoxin and furosemide therapy, and he was discharged from the hospital.At 7 months of age, he again presented in congestive heart failure. The patient weighed 5 kg (below the third percentile for age) and was in marked distress with respiratory rates of 70 to 100 per minute. Cardiac catheterization (Table) demonstrated low cardiac output, pulmonary hypertension, elevated pulmonary artery wedge pressure, dilated systemic ventricle, dilated left atrium, and severe systemic AV valve regurgitation.Replacement of the affected valve was performed using cardiopulmonary bypass with a single venous cannula in the right atrial appendage and deep hypothermia (20 ~ C) without preliminary surface cooling. The left atrium was incised posterior to the intra-atrial groove. The chamber was lined with glistening, white, fibrotic tiss...
