Hostname: page-component-848d4c4894-4hhp2 Total loading time: 0 Render date: 2024-06-09T09:42:09.471Z Has data issue: false hasContentIssue false
  • English
  • Français

Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum

Published online by Cambridge University Press:  19 August 2008

Robert H. Anderson ,
Christine Anderson  and
James R. Zuberbuhler
Robert H. Anderson*
Affiliation:
From the National Heart & Lung Institute, London and the Children's Hospital of Pittsburgh, Pittsburgh
Christine Anderson
Affiliation:
From the National Heart & Lung Institute, London and the Children's Hospital of Pittsburgh, Pittsburgh
James R. Zuberbuhler
Affiliation:
From the National Heart & Lung Institute, London and the Children's Hospital of Pittsburgh, Pittsburgh
*
Dr. Robert H. Anderson, National Heart & Lung Institute, Dovehouse Street, London SW3 6LY, United Kingdom
Get access Rights & Permissions [Opens in a new window]

Summary

Atresia of the outflow tract of the right ventricle in the presence of an intact ventricular septum poses major problems for surgical management. In the light of known problems, 43 autopsied hearts with this congenital abnormality were evaluated to note the arrangement of the myocardium and the cavity of the right ventricle, the state of the tricuspid valve, the precise substrate for pulmonary atresia, and the presence offistulous communications between the ventricular cavity and the coronary arteries. The specimens could be divided into two groups. The first group (38 hearts) had hypoplasia of the cavity of the right ventricle with mural hypertrophy while the second group (5 hearts) had a dilated right ventricular cavity with thinning of the wall. The larger group could be divided into two subgroups on the basis of the substrate for pulmonary atresia. Twenty hearts had muscular atresia of the right ventricular outflow tract and 18 had a potentially patent outflow tract blocked by an imperforate pulmonary valve. A comparison of these two subgroups revealed that mural hypertrophy and cavitary hypoplasia were more severe in hearts with muscular atresia. The tricuspid valve was hypoplastic and its leaflets were not dysplastic in this group but the leaflets were dysplastic in hearts with valvar pulmonary atresia. Fistulous communications between the ventricular cavity and the coronary arteries were only seen in the cases with muscular atresia. In this series, recognition of muscular pulmonary atresia would have identified those patients with the worst surgical prognosis.

Type
Original Articles
Information
Cardiology in the Young , Volume 1 , Issue 2 , April 1991 , pp. 105 - 113
Copyright
Copyright © Cambridge University Press 1991

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, Dische, MR, Rowe, RD. The tricuspid valve in pulmonary atresia with intact ventricular septum. A morpho logical study of 6O cases. Arch Pathol Lab Med 1978; 102: 28–31.Google Scholar
2.Zuberbuhler, JR, Allwork, SP, Anderson, RH. The spectrum of Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1979; 77: 202–211.CrossRefGoogle ScholarPubMed
3.O'Connor, WN, Cottrill, CM, Johnson, GL, Noonan, JA, Todd, EP. Pulmonary atresia with intact ventricular septum and ventriculocoronary communications: surgical significance. Circulation 1982; 65: 805–809.Google Scholar
4.Freedom, RM. The morphologic variations of pulmonary atresia and intact ventricular septum: guidelines for surgical intervention. Pediatr Cardiol 1983; 4: 183–188.Google Scholar
5.Calder, AL, Co, EE, Sage, MD. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol 1987; 59: 436–442.CrossRefGoogle ScholarPubMed
6.Gittenberger de Groot, AC, Sauer, U, Bindl, L, Babic, R, Essed, CE, Buhlmeyer, K. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol 1988; 18: 243–258.CrossRefGoogle ScholarPubMed
7.Bull, C, de Leval, MR, Mercanti, C, Macartney, FJ, Anderson, RH. Pulmonary atresia with intact ventricular septum: a revised classification. Circulation 1982; 66: 266–271.CrossRefGoogle ScholarPubMed
8.de Leval, M, Bull, C, Stark, J, Anderson, RH, Taylor, JFN, Macartney, FJ. Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification. Circulation 1982; 66: 272–280.Google Scholar
9.de Leval, 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 52–60.Google Scholar
10.Freedom, RM, Benson, LN, Trusler, GA. Pulmonary atresia and intact ventricular septum: a consideration of the coronary circulation and ventriculo-coronary artery connections. In: Yacoub, M (ed). Annual of Cardiac Surgery. Current Science, London, 1989, pp 38–44.Google Scholar
11.Zuberbuhler, JR, Fricker, FJ, Park, SC, Anderson, RH, Lenox, CC, Neches, WH, Mathews, RA. Pulmonary atresia with intact ventricular septum: morbid anatomy. In: Godman, MJ, Marquis, RM (eds). Paediatric Cardiology. Volume 2. Heart Disease in the Newborn. Churchill Livingstone, Edinburgh, 1979, pp 285–296.Google Scholar
12.Fricker, FJ, Zuberbuhler, JR. Pulmonary atresia with intact ventricular septum. In: Anderson, RH, Macartney, FJ, Shinebourne, EA, Tynan, M (eds). Paediatric Cardiology. Volume 2. Churchill Livingstone, Edinburgh, 1987, pp 711–720.Google Scholar
13.Davignon, AL, Greenwold, WE, Dushane, JW, Edwards, JE. Congenital pulmonary atresia with intact ventricular septum: clinicopathologic correlation of two anatomic types. Am Heart J 1961; 62: 591–602.CrossRefGoogle ScholarPubMed
14.Allan, LD, Crawford, DC, Tynan, MJ. Pulmonary atresia in prenatal life. J Am Coil Cardiol 1986; 8: 1131–1136.CrossRefGoogle ScholarPubMed
15.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.CrossRefGoogle ScholarPubMed
16.Qureshi, SA, Rosenthal, E, Tynan, M, Anjos, R, Baker, EJ. Transcatheter laser-assisted balloon pulmonary valve dilation in pulmonic valve atresia. Am J Cardiol, in press.Google Scholar
17.Todras, T, Presbitero, P, Gaglioti, P, Demarie, D. Pulmonary stenosis with intact ventricular septum: documentation of development of the lesion echocardiographically during fetal life. Int J Cardiol 1988; 19: 355–360.Google Scholar
18.Allan, LD. Development of congenital lesions in mid or late gestation. Editorial note. Int J Cardiol 1988; 19: 361–362.CrossRefGoogle Scholar
19.Milanesi, O, Daliento, L, Thiene, G. Solitary aorta with bilateral ductal origin of non-confluent pulmonary arteries in pulmo nary atresia with intact septum. Int J Cardiol 1990; 29: 90–91.CrossRefGoogle Scholar
20.Leung, MP, Mok, CK, Hui, PW. Echocardiographicassessmenr of neonates with pulmonary atresia and intact ventricular septum. J Am Coil Cardiol 1988; 12: 719–725.CrossRefGoogle ScholarPubMed