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Morphologic and functional analysis of myocardial status in pulmonary atresia with intact ventricular septum—an angiographic, histologic and morphometric study

Published online by Cambridge University Press:  19 August 2008

Luciano Daliento ,
Roldano Scognamiglio ,
Gaetano Thiene ,
Anita Hegerty ,
Siew Yen Ho ,
Francesca Caneve  and
Robert H. Anderson
Luciano Daliento*
Affiliation:
From the Department of Cardiology, University of Padua, Padua
Roldano Scognamiglio
Affiliation:
From the Department of Cardiology, University of Padua, Padua
Gaetano Thiene
Affiliation:
Department of Pathology, University of Padua, Padua
Anita Hegerty
Affiliation:
Department of Paediatrics, National Heart and Lung Institute, London
Siew Yen Ho
Affiliation:
Department of Paediatrics, National Heart and Lung Institute, London
Francesca Caneve
Affiliation:
From the Department of Cardiology, University of Padua, Padua
Robert H. Anderson
Affiliation:
Department of Paediatrics, National Heart and Lung Institute, London
*
Dr. L. Daliento, Cattedra di Cardiologia, Policlinico Universitario, via Giustiniani 2, 35128 Padova, Italy.
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Summary

We studied 11 patients with pulmonary atresia and intact ventricular septum who died at ages ranging from one day to three years. All but two neonates died after surgery. Pre-surgical catheterization with left and right cineventriculography was performed in nine patients, and quantitative parameters were calculated. By histologic examination of transverse transmural sections, the incidence and extent of myocardial ischemia or infarction were determined, as well as the percentage area per field occupied by vessels, myocytes orientated longitudinally or transversely, interstitial space, and disarrayed fibers. Comparable sections were obtained from six normal neonates to constitute a control group. Myocardial ischemia or infarction were frequent complications. Morphometric analysis demonstrated a significant difference between the control group and the patients with regard to the percentage of myocytes (longitudinal and transverse), the percentage of area occupied by myocardial disarray, and the presence of fibrosis. The highest values for the proportion of disarray were seen in those patients with small right ventricles. The percentage of myocytes varied, but the highest degree of disarray always correlated with the lowest percentage of myocytes. The highest percentage value of left ventricular disarray was associated with the lowest ratio of left ventricular mass to end diastolic volume (inadequate hypertrophy). The lowest indices of contractility and left ventricular pump function were associated with myocytes less than 55% of normal. A linear correlation was found between the percentage area of fibrosis and the ratio of systolic pressure to end systolic volume in the left ventricle, arid between fibrosis and ejection fraction. Values for fibrosis exceeding 15% were associated with decreased functional indices. Disarray, therefore, seems to express a primary inability of myocardium to respond to anomalous overload with adequate hypertrophy and/or hyperplasia.

Keywords

Morphometric analysisquantitative cineangiographyventricular function
Type
World Forum for Pediatric Cardiology Symposium on Pulmonary Atresia
Information
Cardiology in the Young , Volume 2 , Issue 4 , October 1992 , pp. 361 - 366
Copyright
Copyright © Cambridge University Press 1992

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