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Pulmonary arterial size was measured during cineangiography in 23 patients, 1.9 months before, and 14 months after bidirectional cavopulmonary connection (BCPC). Measurements were standardized for body surface area using the method of Nakata and co-workers (pulmonary artery index). There was a significant reduction in pulmonary artery index after BCPC. These data suggest that pulmonary arterial growth is impaired after the creation of a BCPC. This may be related to an absolute reduction in pulmonary arterial flow, and/or the loss of systolic expansion of the pulmonary artery.

Between August 1983 and January 1991, seven patients with Marfan syndrome underwent surgery for severe cardiovascular complications. The mean age at presentation was 5.7 months (range 4 to 9 months) in the infant group (n = 3), and 13.3 years (range 10 to 16 years) in a group of older children (n = 4). The primary indications for surgery in the infant group (performed at a mean of 3 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in one patient, and severe mitral valve prolapse with regurgitation in two. In the older group, surgical indications (performed at a mean of 2.8 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in three patients and acute aortic dissection in one. For aortic surgery, a composite valved conduit was used in four patients, and an aortic homograft in one. For mitral valve surgery, mechanical prostheses were used. All patients survived the primary operation. Over a mean follow-up of 17.5 patient-years (range 1 to 9 years), two patients in the infant Marfan group went on to further successful surgery (prosthetic mitral valve replacement and aortic root repair with aortic homograft) at a mean interval of 4.3 years after the initial surgery. Our results suggest that the major cardiovascular risk factors of Marfan syndrome in the young, even in those diagnosed during infancy, have been favorably changed by surgery with an encouraging medium-term outlook. The correct timing of surgery is aided by echocardiography.

Twenty patients had a repair of an atrioventricular septal defect with tetralogy of Fallot (n = 13) or double outlet right ventricle (n = 7). Mean age was 3.5 years. Surgical technique included transatrial-transpulmonary resection of right ventricular outflow tract obstruction and transatrial two patch repair of the atrioventricular septal defect. Ten patients required a transannular patch and one patient had a right ventricle-pulmonary artery conduit placed. There was no hospital mortality, and mean hospital stay was 15 days. One patient had late sudden death of unknown cause. Six patients have required reoperation because of residual ventricular septal defect (VSD), mitral incompetence, residual right ventricular outflow tract obstruction, and/or conduit stenosis. No patient was reoperated on because of left ventricular outflow tract obstruction. Fifteen patients are asymptomatic, one has exertional dyspnea, and two have intermittent occasional bronchospasm. The transatrial-transpulmonary two patch repair and extensive relief of right ventricular outflow tract obstruction have given good immediate results. Reoperation rate has been high mainly due to residual VSD and mitral incompetence.

The optimal management of infants with pulmonary atresia with intact ventricular septum (PA.IVS) remains a controversy. Attempts have been made to base the surgical approach on various geometrical or morphological characteristics of the right ventricle (RV). However, the overall results remain poor when compared to other complex congenital heart defects. Forty-eight neonates with PA.IVS were admitted to our unit between 1980 and 1992. The management plan has evolved to be based entirely on the echocardiographic assessment of the state of development of the infundibulum of the RV. In neonates with a well-formed infundibulum (n = 31), the initial palliation consisted mainly of pulmonary valvotomy (without cardiopulmonary bypass) and PTFE shunt from the left subclavian artery to the main pulmonary artery. There was one death from initial palliation in this subgroup. If necessary, the RV cavity was later enlarged by excision of the hypertrophic muscle of both the trabecular and infundibular portions, before finally attempting biventricular repair. The actuarial probability of achieving a biventricular repair at 40 months of age was 60% (95% CL = 39.5% to 71.3%). Thirteen patients have undergone biventricular repairs with one late death over a total follow-up of 1,720 patient months. In one patient, the RV failed to grow satisfactorily, necessitating a Fontan procedure. Seventeen patients without a well-formed infundibulum were approached with a Fontan procedure in mind. The initial palliation in these patients consisted of a modified Blalock-Taussig shunt only. Ten have undergone a Fontan procedure so far and five are awaiting such repairs. In this group there were four operative deaths: two after initial palliation, and two after Fontan procedures. In patients with a well-developed infundibulum, the actuarial survival probability was 93% (95% CL = 74% to 98%) at 8 months with no further late deaths over 120 months follow-up, whereas in patients without a well-formed infundibulum it was 75% at 40 months (95% CL = 46% to 89%). The overall survival probability at 104 months was 77% (95% CL = 51% to 90%).

A baby weighing 6.2 kg with anomalous origin of the left coronary artery from the pulmonary artery developed profound left ventricular failure during surgical repair. He was supported for 41 hours with centrifugal pump left heart assist. Recovery following cessation of ventricular assist was uneventful and ventricular function was improved compared to preoperative status.

Pectus excavatum deformity may present problems when median sternotomy is required for repair of intracardiac defects. We have used a partial median sternotomy extending from the manubrium to the level of the second intercostal space with extension into that space. The perichondral sheaths are disconnected from the sternum on the right side after removing the deformed costal cartilages. Exposure is thus provided for the cardiac procedure. Following this, the upper sternal fragments are sutured together and the repair is completed in a manner similar to the Ravitch technique.