BACKGROUND: In patients with absent pulmonary valve syndrome, the relief of the pulmonary regurgitation at the time of primary repair improves both the early and late results. Though homograft and heterograft valves and conduits have been used for this purpose, both are not easily available and are known for late failure. Monocusp and bicuspid semilunar valves made out of pericardium have their own problems. Hence, a technique of reconstructing an autologous competent 3-cusp valve from the native tissues was developed. METHODS: Two posterolateral semilunar cusps were fashioned from the anterior wall of the main pulmonary artery. The anterior cusp was made from autologous pericardium stitched to the autologous pericardial patch used to widen the right ventricular outflow tract. RESULTS: This method of reconstruction was used in two patients aged 9 and 22 years, respectively. Visual assessment and passive testing after reconstruction revealed well functioning neopulmonary valves in both patients. The second patient, who had an unevenful hospital course, showed only mild pulmonary regurgitation at 5 years postreconstruction. CONCLUSIONS: As 2 of the 3 cusps are fashioned from the pulmonary arterial wall as a pedicled graft, we believe that they will retain their viability and grow with the pulmonary artery. Simultaneous reduction in the size of the pulmonary arteries will relieve bronchial compression when present. The anterior pericardial cusp, even if it eventually shrivels up, is unlikely to produce serious hemodynamic derangements.

Adult↗

PubMedDate not supplied

Surgical experience with congenital heart disease in Down's syndrome.

Children with Down's syndrome and congenital heart defects have multiple problems. The role of cardiac surgery in the management of these patients was investigated by reviewing the clinical data, hospital course and follow-up of 21 patients (9 males and 12 females, age range 1 month to 14 years) with Down's syndrome and congenital heart defects operated in our institute. Twelve (57%) of these were infants and nine (43%), older children. Five were in congestive cardiac failure, four were hypothyroid. The heart lesions ranked in incidence as follows: atrioventricular septal defect 7 (33.3%), tetralogy of Fallot 3 (14.3%), tetralogy of Fallot & atrioventricular septal defect both 2 (9.5%), double outlet right ventricle with pulmonary stenosis 1 (4.8%), patent ductus arteriosus 2 (9.5%), patent ductus arteriosus plus coarctation 1 (4.8%), ventricular septal defect 2 (9.5%), atrial septal defect plus ventricular septal defect 1 (4.8%), atrial septal defect plus patent ductus arteriosus plus right pulmonary artery stenosis 1 (4.8%) and transposition of great arteries with multiple ventricular septal defect 1 (4.8%). Four (19%) patients had palliative procedures while the rest (81%) underwent primary repair. All survived the operation. The post-operative period was complicated in 6 (28.5%), with respiratory infections in 3, pulmonary hypertensive crisis in 2 and complete heart block in 1. The early mortality was 0, while there were 2 (9.5%) late deaths. The number of hospitalisations was markedly reduced according to the parents. Follow-up showed near normal pulmonary artery pressure in 50 percent children with large shunts and a good developmental spurt was seen in 60 percent. From a purely surgical viewpoint, the prognosis for children with Down's syndrome and congenital heart disease is good.

Adolescent↗