Hypoplasia of the small pulmonary arteries in hypoplastic left heart syndrome with restrictive atrial septal defect

Article

关键词: CONGENITAL DIAPHRAGMATIC-HERNIA;    VASCULAR-DISEASE;    CARDIAC TRANSPLANTATION;    QUANTITATIVE-ANALYSIS;    STRUCTURAL-CHANGES;    VENOUS CONNECTION;    GREAT-ARTERIES;    AORTIC ATRESIA;    LUNG GROWTH;    TRANSPOSITION;   

DOI  :  10.1161/01.CIR.0000138223.74524.4e

来源: SCIE

【 摘 要 】

Background-Restrictive atrial septal defect (ASD) (including intact atrial septum [IAS]) has been reported to be a risk factor that negatively impacts survival in hypoplastic left heart syndrome (HLHS). Although lymphangiectasia and arterialization of the veins of the lung in HLHS with restrictive ASD have been reported, they cannot fully explain the high mortality. We have introduced a new method of evaluating the development of the pulmonary vasculature in histological sections and used it to assess patients' lungs. We tested the hypothesis that the small pulmonary arteries (SPA), which are pulmonary arteries in a histological section whose radii are approximate to25 mum to 250 mum, in HLHS with restrictive ASD are hypoplastic, but that the alveoli are not, to elucidate the mechanism underlying the -poor outcome of these patients. Methods and Results-Fourteen HLHS patients between 1 day and 40 days of age were studied. In 8 cases, the ASD was restrictive [R(+) group], and in the other 6 cases it was not [R(-) group]. Specimens from 12 autopsies of cases with no congenital heart or pulmonary disease were examined as a control group (C group). As a novel histological parameter, we assessed the size of SPA in relation to the size of accompanying bronchioles to identify SPA underdevelopment. To evaluate the development of alveoli and interstitial tissue, radial alveolar counts (RAC), which reflect alveolar maturity and complexity, were also performed. Statistical comparisons between groups were made by analysis of covariance with age as a covariant factor. When the radius of the accompanying bronchiole was 100 mum, the radius of the SPA was 34.0 +/- 10.8 mum in the R(+) group, and significantly lower than the 46.6 +/- 8.5 mum in R(-) group (P=0.0022) and 70.5 +/- 8.4 Am in the C group (P<0.0001). The RAC was in 3.5 +/- 0.9 in the R(+) group, 3.4 +/- 0.6 in the R(-) group, and 3.7 +/- 0.9 in the C group (no significant differences between groups). Conclusion-The SPA in HLHS with restrictive ASD were underdeveloped compared with the SPA in HLHS with nonrestrictive ASD and the controls, but their alveoli were not hypoplastic. Based on these results, it is speculated that SPA hypoplasia may be responsible for the poor outcome of HLHS with restrictive ASD.

【 授权许可】

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