The role of lung biopsy in children with perinatally acquired AIDS.

There is a need for a rapid and efficacious method of diagnosis of pulmonary infiltrates in perinatal HIV infection. However, controversy still exists about which method--open lung biopsy (OLB) versus bronchoscopic techniques--is the best for this population. We present our results with OLB in 24 children with HIV-related lung disease. Over a 6-year period, 27 OLBs were performed on 24 children with diagnosis of HIV infection. The procedures were performed under general anesthesia using a limited anterolateral thoracotomy. Suspicious areas were removed with the autostapler. The specimens were studied for the presence of non-infectious as well as bacterial, viral, fungal, and mycobacterial diseases. There were no operative deaths related to the procedure. Morbidity was limited to prolonged but self-resolving air leaks in two patients (8.3%). Five hospital deaths occurred between 3 and 12 weeks postoperatively and 11 late deaths between 3 months and 6.5 years. All deaths were related to AIDS. Eight patients (33.3%) are still alive 2 to 8 years postoperatively. A total of 43 pathologies were found in 27 specimens. A positive pathologic finding was obtained in all patients, with two patients having nonspecific minimal changes. This resulted in a change of therapy in all but one case. The technique of OLB in children with AIDS is safe and simple. It should be performed early in the course of the disease and, a careful selection of candidates can minimize the incidence of complications.

Replacement of obstructed extracardiac conduits with autogenous tissue reconstructions.

Tissue-valved prosthetic extracardiac conduits fail in 6% to 30% of patients within 5 years of implantation. Failure is caused both by valve degeneration and by conduit peel formation. This report describes a technique, performed in 16 children, in which an obstructed right ventricle-to-pulmonary artery valved conduit was removed and a new conduit constructed using the conduit bed as the posterior wall and a patch of xenograft pericardium (n = 10), homograft dura mater (n = 5), or Dacron (n = 1) as the roof of the conduit. One child with pulmonary hypertension required a Björk-Shiley pulmonary valve; in the others no valve was inserted. This technique simplifies conduit replacement, allows for a generous-sized outflow tract that may grow with patient growth, and uses material unlikely to become obstructed.

Late results after Starr-Edwards valve replacement in children.

Selection of types of prosthetic heart valves for children remains controversial. The case histories of 50 children surviving valve replacement with Starr-Edwards prostheses between 1963 and 1978 were reviewed to evaluate the long-term performance of mechanical valves. The 31 boys and 19 girls ranged from 6 months to 18 years in age (mean 10.4 years); 19 patients had had aortic valve replacement, 24 patients had had mitral valve replacement, and one patient had had both. Among the six patients who had had tricuspid valve replacement, four had corrected transposition, so that the tricuspid valve was the systemic atrioventricular valve. Mean (+/- standard deviation) follow-up interval was 7.9 +/- 4.9 years (maximum 17 years). For all patients, the 5 year survival rate was 86% +/- 6%. At 10 years postoperatively, the survival rate (+/- standard error) was 90% +/- 7% after aortic valve replacement and 76% +/- 8% after systemic atrioventricular valve replacement. At follow-up, 39 patients were alive, and 38 were in New York Heart Association Class I or II. Of the 11 deaths, four were valve-related. Seven patients had major (requiring hospitalization) thromboembolic events, and five patients had minor transient neurological symptoms suggesting thromboembolism; 50% of these patients were not taking warfarin (Coumadin) at the time of the thromboembolic event. The incidence of late (greater than 30 days) thromboembolism was 5.3 per 100 patient-years after aortic and 2.0 per 100 patient-years after systemic atrioventricular valve replacement. At 10 years postoperatively, 66% +/- 15% of patients who had had aortic valve replacement and 91% +/- 6% of those who had had systemic atrioventricular valve replacement were free of thromboembolism. The excellent long-term survival, absence of mechanical failure, and relatively low rate of thromboembolism with this prosthesis contrast with our experience with biological valves, in which 41% of children required reoperation in 5 years. Currently, mechanical valves, such as the Starr-Edwards prostheses, are our preferred valves for pediatric patients.

Reoperation for obstructed pulmonary ventricle-pulmonary artery conduits. Early and late results.

Obstruction of pulmonary ventricle-pulmonary artery conduits can result from neointimal peel formation or valvular degeneration and calcification. To determine the risks and outcome of reoperation, we reviewed the records of 100 consecutive patients who had replacement of severely stenotic pulmonary ventricle-pulmonary artery conduits. At reoperation, the 70 male and 30 female patients had a mean age of 13.3 +/- 4.8 years. During operation, 37 homografts, 62 Dacron grafts with integral xenograft valves, and one nonvalved conduit were replaced with valved (80) or nonvalved (17) prostheses. The mean transconduit pressure gradient decreased from 81 +/- 26 mm Hg preoperatively to 7 +/- 8 mm Hg postoperatively (p less than 0.01). Concomitant cardiac valve replacement was performed in seven patients, and residual ventricular septal defect closure was accomplished in 28. Operative mortality (less than 30 days) was 7%, but there were no deaths among the 47 patients who had no associated defects. At 3 and 5 years postoperatively, probability of survival among patients dismissed from the hospital was 94% +/- 3% and 86% +/- 6%, respectively. In our experience, risk of reoperation for conduit obstruction alone is low, so that the effect of graft failure on overall survival is minimized.

Left ventricle-aortic conduits in pediatric patients.

From August, 1974, to January, 1982, left ventricle-aortic porcine valved conduits were inserted in three patients less than 2 years old (Group 1) and in 10 patients between 2 and 14 years of age (Group 2) for relief of severe left ventricular outflow tract obstruction. The distal anastomosis was made to the ascending aorta in seven patients and to the supraceliac abdominal aorta in six patients. In six patients, the conduit was sutured directly to the left ventricle, and in seven a stented right-angle connector was employed. The left ventricle-aortic gradients were relieved in all cases (mean residual gradient = 4.3 mm Hg). All three patients in Group 1 had associated endocardial fibroelastosis and all died. There was one early death in Group 2 (10% mortality). Reoperation was required in seven of nine survivors (78%) 2.7 to 5.2 years postoperatively for conduit valve failure (five patients), progression of mild native aortic valve insufficiency (one patient), or both (one patient). One of the seven required another reoperation for re-replacement of the conduit valve. There was one late death associated with reoperation. At follow-up 3.4 to 7.5 years postoperatively, four patients are in Functional Class I, two are in Class II, and two are convalescing from reoperation. Left ventricle-aortic conduits provide excellent relief of left ventricular outflow tract obstruction. However, the high incidence of late complications suggests better results might be anticipated with aortoventriculoplasty (Konno).