Hostname: page-component-78c5997874-lj6df Total loading time: 0 Render date: 2024-11-10T08:14:44.054Z Has data issue: false hasContentIssue false
  • English
  • Français

Determinants for outcome of hypoplastic right ventricle with duct-dependent pulmonary blood flow presenting in the neonatal period

Published online by Cambridge University Press:  19 August 2008

Michael L. Rigby ,
Micelia Salgado  and
Celia Silva
Michael L. Rigby*
Affiliation:
From the Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospitals, London
Micelia Salgado
Affiliation:
From the Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospitals, London
Celia Silva
Affiliation:
From the Department of Paediatric Cardiology, The Royal Brompton National Heart & Lung Hospitals, London
*
Dr Michael L. Rigby, Consultant Paediatric Cardiologist, Royal Brompton National Heart & Lung Hospital, Sydney Street, London SW3 6NP, United Kingdom.
Get access Rights & Permissions [Opens in a new window]

Summary

A retrospective study was undertaken of patients with hypoplastic right ventricles, either with pulmonary atresia and intact ventricular septum or critical pulmonary valvar stenosis, and duct-dependent pulmonary blood flow who were investigated at the Royal Brompton Hospital between January 1976 and December 1990. The diagnosis was made on the basis of at least one diagnostic method (cross-sectional echocardiography, cardiac catheterization and angiography, or autopsy). Of the patients, 56 (82%) were found to have an imperforate pulmonary valve or infundibulum (pulmonary atresia), while 12(18%) had critical pulmonary valvar stenosis. The ratio of the diameters of the tricuspid and mitral valvar orifices was measured angiographically during diastole, and the right ventricle was analyzed according to the presence or overgrowth of the inlet, apical trabecular and outlet components. A correlation was made between the severity of the disease and the outcome. The overall mortality was 53% when those not undergoing any surgery were excluded, and significant differences were found between the group dying and those who survived. The incremental risk factors for death were a ratio between the diameter of the tricuspid and mitral valves of less than 0.6; the presence of fistulous communications with the coronary arteries; and obliteration of the apical trabecular component of the right ventricle. There was no significant difference between the group with pulmonary valvar atresia and the group with critical stenosis of the pulmonary valve.

Keywords

Pulmonary atresia with intact septumcritical pulmonary stenosisanalysis of results
Type
World Forum for Pediatric Cardiology Symposium on Pulmonary Atresia
Information
Cardiology in the Young , Volume 2 , Issue 4 , October 1992 , pp. 377 - 381
Copyright
Copyright © Cambridge University Press 1992

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Freedom, RM, Keith, JD. Pulmonary atresia with normal aortic root. In: Keith, JD, Rowe, RD, Vlad, P (eds). Heart Disease in Infancy and Childhood. Macmillan, New York, 1979, pp 406–517.Google Scholar
2.Bull, C, de Leval, MR, Mercanti, C, Macartney, FJ, Anderson, RH. Pulmonary atresia with intact ventricular septum: a revised classification. Circulation 1982; 62: 266–271.CrossRefGoogle Scholar
3.Freedom, RM, Wilson, G, Trusler, GA, Williams, WG, Rowe, RD. Pulmonary atresia and intact ventricular septum: A review of the anatomy, myocardium, and factors influencing right ventricular growth and guidelines for surgical intervention. Scand J Thorac Cardiovasc Surg 1983; 17: 1–28.Google Scholar
4.Calder, AL, Co, EE, Sage, MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol 1987; 59: 436–442.Google Scholar
5.Gersony, WM,Bernhard, WF,Nadas, AS,Gross, RE. Diagnosis and surgical treatment of infants with critical pulmonary outflow tract obstruction. Study of thirty-four infants with pulmonary stenosis or atresia and intact ventricular septum. Circulation 1967; 35: 765–776.Google Scholar
6.Rowlatt, UF, Rimoldi, HJA, Lev, M. The quantitative anatomy of the normal child's heart. PediatrClin North Am 1963; 10: 499–588.CrossRefGoogle Scholar
7.Leung, MP, Mok, CK, Dui, PW. Echocardiographic assessment of neonates with pulmonary atresia and intact ventricular septum. J Am Coll Cardiol 1988; 12: 719–725.CrossRefGoogle ScholarPubMed
8.de Leval, M, Bull, C, Stark, J, Anderson, RH, Taylor, JFN, Taylor, JFN, Macartney, FJ. Pulmonary atresia with intact ventricular septum: surgical management based on a revised classification. Circulation 1982; 66: 272–280.CrossRefGoogle Scholar
9.de Level, M, Bull, C, Hopkins, R, Rees, P, Deanfield, J, Taylor, JFN, Gersony, W, Stark, J, Macartney, FJ. Decision making in the definitive repair of the heart with a small right ventricle. Circulation 1985; 72 (Suppl II): II 52–II 60.Google Scholar
10.Freedom, RM. The morphologic variations of pulmonary atresia and intact ventricular septum: guidelines for surgical intervention. Pediatr Cardiol 1983; 4: 183–188.Google Scholar
11.Anderson, RH, Anderson, C, Zuberbuhler, JR. Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum. Cardiol Young 1991; 1: 105–113.Google Scholar