Between 1983 and 1995, 546 Blalock-Taussig shunt procedures were performed in 472 patients: 128 (23-0%) were classical shunts, 90 of them on the same side as to the aortic arch, and 418 (77.0%) were modified shunts, 182 on the same side of the arch. At the time of surgery, 78 patients were aged below one week, 270 from one week to 12 months, and 198 patients were over one year of age. The mean pre-operative arterial saturation (71.7%± 16.5%) was significantly increased to 83%±17.9% immediately after the procedure (p=0.017). The overall hospital mortality rate was 2.9% (16/546), with rates of 2.3% (3/128) for the classical, and 3.1% (13/418) for the modified shunts (p= not significant). The rate was significantly higher, however, for classical shunts when the pulmonary arterial diameter was less than 4 mm (15.4% versus zero; p=0.047), though this relationship was reversed for modified shunts (zero versus 3-6%; p=0.338). Early mortality was significantly influenced by the age at surgery, 5/78 (6.4%) in patients aged below 1 week, 3.7% between 1 week and 1 year, and 0.5% over 1 year (p=0.019). Early mortality was also significantly increased in patients weighing 3kg or less, 8/156 (5.1%), versus 3/303 (1.0%), p=O.O37. Overall, 51 shunts failed (9.3%), 10 early and 41 late. Early failure was significantly increased in patients weighing 3kg or less, 8/156 (5.1%) versus 3/303 (1.0%), p=0.0l6. The overall early failure rate was 1.4% (3/215) when heparin was administered intra-operatively and for 48 hours postoperatively, in contrast to an early failure rate of 3.4% (7/203) when heparin was not used (p = 0.294). Overall rates of failure during follow-up were 9-1% (17/188) in heparinized patients versusl3-6% (24/177), (p=0.173) in non-heparinized patients. Failure of classical shunts was 10.2% (13/128), compared with 6.7% (28/418) for modified shunts (p=0.195). Failure was more common overall if the pulmonary arterial diameter was less than 4 mm, 14.7% (9/61), as opposed to 8.7% (26/300) when the diameter was 4 mm or greater, (p=0.l44). Administration of aspirin during follow-up after the modified shunt procedure reduced failure from 11 % (18/163) to 6.7% (10/150), p=0.176. Classical or modified Blalock-Taussig shunts, either on the same side or opposite to the aortic arch, can be performed on patients of any age with minimum postoperative complications and low operative mortality. The use of intra-and post-operative heparin appears to reduce the overall rate of failure, and the administration of aspirin during follow-up appears to reduce failure of modified Blalock-Taussig shunts.

Cor triatriatum is an uncommon cardiac anomaly occurring in 0.1% of children with congenital heart disease. We reviewed our results from 20 patients presenting with cor triatriatum in the last 20 years (from March 1983 to August 2002). Mean age at repair was 20 months (1-132 months). During a mean follow-up period of 31 months (2-156 months) there were no deaths. All patients were in sinus rhythm except one patient who needed pacemaker insertion one week after repair. A total of 14 patients were asymptomatic and 6 were in New York Heart Association Class I. Cor triatriatum is amenable to surgical repair with excellent results when diagnosed early, and when not associated with other severe cardiac anomalies.

Between February 1980 and February 1992, 28 children (17 males and 11 females) were treated for endocarditis on congenital cardiac lesions, most commonly the mitral valve (32%). Their ages ranged between four months and 14 years (mean 8.2 years). The most common infecting organism (in 25% of patients) was Staphylococcus aureus. Patients were divided into two groups. In the first, made up of eight patients, cardiac surgery was undertaken within one week of the start of antibiotic therapy. The second group of 20 patients was further divided into a group of seven patients who underwent surgery during the initial, active stage of endocarditis because of failure of medical management and a group of 13 patients who initially received antibiotic therapy alone. In this last group, three infections with the same organism recurred within two months and surgical intervention was necessary in the active phase of the recurrence. Subsequently, surgery for “healed” endocarditis was undertaken in three more patients. Mortality in the 21 patients undergoing surgery was 19%—one of eight (12.5%) in those undergoing surgery within one week of starting treatment and two of 10 in the remaining patients. Overall, three patients (15%) died from those in whom surgery was not undertaken within one week of the start of treatment. There were also fewer pre- and postoperative complications as well as a lower mortality rate in those undergoing early surgery. We conclude that early surgical intervention, in our hands, is the most appropriate management for endocarditis in congenital cardiac lesions.

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Between January 1985 and April 1994, 42 children aged between 7 and 14 years (mean 11.3 years) underwent repair of severely incompetent rheumatic mitral valves with no accompanying mitral stenosis. In 19 patients the tricuspid valve was severely incompetent, while 8 patients had severe aortic incompetence. Overall, the repair incorporated shortening of elongated tendinous cords and insertion of a Duran or Carpentier prosthetic ring. The repair was the sole procedure in 15 patients, whilst 19 patients also had a De Vega tricuspid valvar annuloplasty and 8 had repair or replacement of the aortic valve. There were no hospital deaths. Postoperative transthoracic echocardiographic studies revealed trivial residual mitral valvar regurgitation in 6 patients, mild regurgitation in 18, moderate in 15, and regurgitation severe enough to warrant replacement of the mitral valve in 3 patients. The mean follow-up period was 37 months (maximum 120 months, minimum 1 month). Of 39 patients followed-up, 28 attended for more than 5 years. Of these, 7 underwent replacement of the mitral valve for severe regurgitation within 4 years of the repair. Severe mitral regurgitation in 3 patients was controlled by medical therapy. One was eventually lost to follow-up, and one patient died of causes unrelated to surgery. The remaining 16 patients had absent to moderate mitral valvar regurgitation. These results contrast with 10 repairs of congenital mitral incompetence, where no patients required re-operation in the immediate 5 years period of follow-up. Every effort should be made in children with rheumatic mitral incompetence to preserve the natural valve by conservative repair, despite the fact that repair of the incompetent rheumatic mitral valve is not so durable as repair of congenitally incompetent valves.