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From 1975 to 1982, 31 infants were operated upon in the first year of life for aortic coarctation and congestive heart failure. Operations performed were resection and end-to-end anastomosis (RETE) in 14, subclavian flap aortoplasty (SFA) in six, patch aortoplasty (PA) in five, and other procedures in six. Thirty of the thirty-one (97%) survived the operation. To assess the effect of operation, 26 infants were studied noninvasively with Doppler arm-to-leg pressure measurements at rest and with stress. Preoperatively, the median arm-to-leg gradient at rest was 77 mm Hg. Serial postoperative Doppler studies demonstrated progressive changes in arm-to-leg pressure gradients: 69% had residual arm-to-leg gradients that spontaneously resolved, 13% had residual gradients that persisted, 13% had progressive increase in gradient, and one child had neither early nor late gradient. Stress testing often unmasked gradients not present in the resting state. No differences were noted among the three surgical groups: RETE, SFA, and PA. From our experience, we have made four conclusions with regard to repair of coarctation of the aorta in infants. First, surgical survival is expected. Second, the effect of the operation is dynamic, with four patterns defined: (1) complete relief of coarctation, (2) transient residual coarctation, (3) persistent residual coarctation, and (4) recurrent coarctation. Third, optimal surgical therapy seems to be an eclectic approach. Fourth, physiological evaluation of coarctation in infants can be obtained by Doppler techniques in conjunction with stress testing.

The acute and chronic consequences of subclavian artery transection were analyzed in the noninvasive vascular laboratory. Twenty-eight patients (aged 1 day to 42/12 years, median 2 months) underwent subclavian artery transection (23 Blalock-Taussig, four subclavian aortoplasty for coarctation or interrupted arch, and one division of aberrant left subclavian). Bilateral systolic brachial artery pressure (BAP) was measured by Doppler instrumentation to obtain a “BAP index”: BAP1= (operated side BAP/control side BAP). Velocity waveform tracings and bilateral forearm skin temperatures were also obtained during studies before and sequentially after operation (4 hours to 12 years). Five patients underwent exercise testing of the upper extremity. Nine patients were studied for manual preference and limb development. Before operation, mean BAP, was 0.99. Immediately (4 to 48 hours) after operation, mean BAP, was 0.39. Three weeks postoperatively, BAP, was 0.62, and thereafter it remained at 0.70. All differences between preoperative, immediate postoperative and late postoperative BAP, are significant (p < 0.001). Exercise resulted in a significant (p < 0.01) increase in BAP bilaterally. Forearm skin temperature was initially lower (p < 0.01) on the operated side but approximated the control side by 1 week. Limb girth was less on the operated side (p < 0.01), without evidence of altered manual preference. In conclusion, subclavian artery transection causes permanent reduction in BAP,. The affected limb appears to respond to increased metabolic demand by increasing limb blood flow.

From September, 1978, to January, 1983, 44 cyanotic infants and children underwent a systemic-pulmonary artery shunt for the treatment of reduced pulmonary blood flow. Age ranged from 18 hours to 4 years (mean age = 0.49 years). Weight ranged from 1.7 kg to 13.2 kg (mean weight = 4.9 kg). There were 12 classic Blalock-Taussig shunts, five central polytetrafluoroethylene shunts, six interposition modified Blalock-Taussig shunts, and 21 Great Ormond Street type of modified Blalock-Taussig shunts. No direct aorta-pulmonary artery anastomoses were performed. There was one postoperative death (1/44 = 2.3%) in a 1,700 gm neonate born with pulmonary atresia and intact ventricular septum (shunt patent). Four shunts required early revision: one thrombosed central shunt, a kinked patent interposition Blalock-Taussig shunt, a small but patent Blalock-Taussig shunt, and one excessive Great Ormond Street type of Blalock-Taussig shunt. Two late deaths were probably shunt-related: one Blalock-Taussig and one central. All four types of shunts provided good palliation, but the Great Ormond Street type of Blalock-Taussig shunt is our preferred shunt because of (1) low operative risk, (2) predictable patency (100% in our series), (3) lack of distortion of pulmonary arterial anatomy, and (4) technical ease of insertion as well as takedown.

Two neonates with asplenia syndrome, subdiaphragmatic total anomalous pulmonary venous connection, and pulmonary stenosis underwent a palliative operation without cardiopulmonary bypass. With the use of a side-biting clamp, an anastomosis was created between the pulmonary venous confluence and the right atrium. (Since children with asplenia syndrome have common mixing lesions, pulmonary venous drainage to the right atrium is not physiologically detrimental.) The descending vertical vein was ligated. A systemic-pulmonary shunt was performed. Both infants were discharged from the hospital less than 1 week after the operation. Both infants subsequently died, at 4 months and at 16 months of age. At autopsy, pulmonary venous drainage was unobstructed, with a surgical orifice 86% to 90 % of the aortic anulus. We conclude that, in infants with asplenia syndrome and obstructed total anomalous pulmonary venous drainage, relief of pulmonary venous obstruction can be accomplished without cardiopulmonary bypass.

A pair of type B thoracopagus twins with complex cyanotic heart disease had shared coronary arteries and coronary venous drainage. Surgical separation was not attempted and the twins died at 10.5 months of age. Antemortem angiography demonstrated that Twin A's right coronary artery supplied Twin B's diaphragmatic and anterior ventricular myocardial free wall. A midline communication existed between each twin's right atrium at a common coronary sinus. The crossing coronary artery coursed alongside this connection and was visualized echocardiographically. At postmortem examination, the great cardiac vein of Twin A drained into the orifice of the common coronary sinus on Twin B's side of the midline.

Pulmonary vasoreactivity at sea level was studied in 22 children before and in 15 children after corrective cardiac surgery for congenital heart disease and pulmonary artery hypertension; 8 children were studied both before and after cardiac surgery. During cardiac catheterization in 28 children, pulmonary and systemic hemodynamics were determined in room air and during breathing of 15% oxygen, which corresponds to a maximal hypoxic level commonly encountered during airplane travel.