Congenital heart diseasePulmonary vascular disease in Down’s syndrome with complete atrioventricular septal defect☆
Section snippets
Patients
We studied a total of 81 consecutive patients with Down’s syndrome and a complete atrioventricular septal defect, in which the pulmonary artery pressure was at or near systemic level, as evaluated by cardiac catheterization between January 1982 and September 1999 at the Sakakibara Heart Institute. Because inclusion of complicated anomalies would bias the statistical analysis, patients with aortic coarctation, left ventricular hypoplasia, or isomerism of the atrial appendages were excluded from
Results
Of the 81 patients, 43 (53%) had Rastelli type A and the remaining 38 (47%) had type C. No patient had type B morphology. As for the inferior bridging leaflet, 54 patients had an undivided morphology and 15 had a divided leaflet, but in the remaining 12 patients the precise morphology was not determined. There was a reverse correlation between the pulmonary vascular resistance and pulmonary to systemic flow ratio before the operation (r = −0.72, p <0.0001; Figure 1).
Discussion
Recent progress in the surgical techniques and perioperative care for those with complete atrioventricular septal defect has allowed us to perform corrective surgery in early infancy.8, 12, 13, 14 The first 6 months of life is considered to be the best time for definitive repair in view of the progression of PVD1, 2, 3, 4, 5 and atrioventricular valve regurgitation.13, 14 Patients with Down’s syndrome are also considered as having a higher risk for PVD,2, 3, 4, 5, 6 because they often
Acknowledgements
We thank Yoshio Obunai, MD, PhD, Japan Research Promotion Society for Cardiovascular Disease, and Teruaki Kawai and Kazuo Nitta, MD, PhD, the Mokichi Okada Association (MOA) Health Science Foundation, Tokyo, for their help with this study.
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This study was supported by research grants from the Japan Research Promotion Society for Cardiovascular Disease, Tokyo; and the Mokichi Okada Association (MOA) Health Science Foundation, Tokyo, Japan.