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Ann Thorac Surg 1990;50:98-102
© 1990 The Society of Thoracic Surgeons

Articles

Morphology of pulmonary atresia with ventricular septum in patients dying after operation

Henry J.C.M. van de Wal, MD*, Audrey Smith, PhD, Anton E. Becker, MD, James L. Wilkinson, FRCP, David I. Hamilton, FRCS

The Royal Liverpool Children's Hospital, Liverpool, England

Accepted for publication February 16, 1990.

* Address reprint requests to Dr van de Wal, Department of Cardiothoracic Surgery, Sint-Radboud University Hospital, Geert Grooteplein zuid 10, 6500 HB Nijmegen, the Netherlands.

In 1976, we adopted staged surgical management of pulmonary atresia with intact ventricular septum: stage 1 = establishment of a systemic to pulmonary artery shunt; stage 2 = open reconstruction of the right ventricular outflow tract and pulmonary valve; and stage 3 = closure of the shunt and interatrial communication. The morphological features of nine specimens obtained from 10 patients who died were reviewed. Special attention was given to features that might have influenced the poor surgical outcome in these patients. Survival after stage 1 depends on adequate systemic to pulmonary artery blood flow, initially as a combination of ductus arteriosus and shunt flow, with subsequent modification if the ductus closes. After stage 2, survival is influenced by left ventricular function and mitral valve function. The success of final correction (stage 3) depends largely on the morphology of both ventricles and their atrioventricular valves. It appears that the behavior of the ductus arteriosus and the size of the shunt are of vital importance for the survival of the infant. In 3 of the specimens, no right ventricular outflow tract was present, and in 2 others, short chordal attachments of the mitral valve were observed. Staged surgical correction appears to be a satisfactory approach if these considerations are taken into account.




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T. Akiba and A. E. Becker
Disease of the left ventricle in pulmonary atresia with intact ventricular septumThe limiting factor for long-lasting successful surgical intervention?
J. Thorac. Cardiovasc. Surg., July 1, 1994; 108(1): 1 - 8.
[Abstract] [Full Text]


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