Virchows Archiv

Publication date: 2002-01
Volume: 441 Pages: 264 - 270
ISSN: 0945-6317, 1432-2307 PMID: 12242523
DOI: 10.1007/s00428-002-0647-4
Publisher: Springer-Verlag


Gorenflo, Matthias
Ullmann, M ; Sebening, C ; Brockmeier, K ; Hagl, S ; Ulmer, HE ; Otto, HF ; Schnabel, PA


Science & Technology, Life Sciences & Biomedicine, Pathology, congenital heart disease, children, pulmonary hypertension, lung, pathology, VENTRICULAR SEPTAL-DEFECT, LUNG-BIOPSY, HYPERTENSION, INFANTS, ARTERIES, Child, Child, Preschool, Heart Defects, Congenital, Hemodynamics, Humans, Hypertension, Pulmonary, Infant, Lung, Postoperative Complications, Retrospective Studies, 1103 Clinical Sciences


OBJECTIVE: We asked whether a scoring system [index of pulmonary vascular disease (IPVD)] that quantifies the individual pulmonary vascular pathology would relate to postoperative survival in patients with congenital heart disease and pulmonary hypertension (PH). METHODS: Lung biopsy specimens from 28 patients at a median age of 6 months (1 month to 21 years) were analysed qualitatively and morphometrically. The IPVD and other morphometric parameters were related to haemodynamic findings and survival. RESULTS: Mean pulmonary artery pressure (PAP) was 44 mmHg (15-72 mmHg), and the resistance to pulmonary perfusion was 5 U x m(2) (0.9-14 U x m(2)). There were three early (in-hospital) and three late deaths during the follow-up period of 2.5 years (6 months to 7 years). Incipient plexiform lesions were observed in one infant with trisomy 21 and complete atrioventricular septal defect (cAVSD). An IPVD score above the upper critical limit (>2.2) was not observed during the first year of life. On discriminant analysis, morphometric parameters could not predict mortality ( P=0.08). CONCLUSIONS: The IPVD is not helpful to predict surgical mortality during the first year of life. Patients with trisomy 21 and cAVSD may show advanced pulmonary vascular disease in infancy.