Elsevier

The Annals of Thoracic Surgery

Volume 64, Issue 6, December 1997, Pages 1795-1802
The Annals of Thoracic Surgery

Original Articles
Causes of Death After the Modified Norwood Procedure: A Study of 122 Postmortem Cases

https://doi.org/10.1016/S0003-4975(97)01041-2Get rights and content

Abstract

Background. Although the results of the modified Norwood procedure as palliation for the hypoplastic left heart syndrome have improved considerably, in-hospital mortality remains high (28% to 46%).

Methods. To establish the causes of death and consider their therapeutic applications, we reviewed our pathology experience from 1980 to 1995, inclusive, regarding 122 patients who died after undergoing the Norwood procedure.

Results. The most important causes of death were found to be impairment of coronary perfusion (33 patients, 27%), excessive pulmonary blood flow (23 patients, 19%), obstruction of pulmonary arterial blood flow (21 patients, 17%), neoaortic obstruction (17 patients, 14%), right ventricular failure (16 patients, 13%), bleeding (9 patients, 7%), infection (6 patients, 5%), tricuspid or common atrioventricular valve dysfunction (6 patients, 5%), sudden death from presumed arrhythmias (6 patients, 5%), and necrotizing enterocolitis (3 patients, 3%). In 26 patients (21%), more than one factor appeared responsible for death.

Conclusions. The leading causes of death after the Norwood procedure were found to be largely correctable surgical technical problems associated with perfusion of the lungs (36%), of the myocardium (27%), and of the systemic organs (14%).

Section snippets

Material and Methods

Between January 1980 and December 1995, autopsies were performed on 122 patients who died after undergoing the Norwood procedure (stage I palliation) for the hypoplastic left heart syndrome at the Children’s Hospital in Boston, Massachusetts.

We reexamined the heart specimens of these 122 patients with special emphasis on the pathologic anatomy, all details of the surgical palliation, the myocardial histology, and the pathologic findings. These data were correlated with the clinical, cardiac

Impairment of Coronary Arterial Perfusion

The most frequent cause of death was impairment of coronary artery perfusion (33 patients, 27%, Table 2, Table 3). Precoronary stenosis was present in 26 of these patients (21% of the series). At autopsy, the opening into the native aortic root, from which the coronary arteries almost always arose, was nonpatulous, ie, not widely patent. On initial inspection from within the reconstructed neoascending aorta, the coronary ostia could not be seen. Although the opening into the native aortic root

Comment

Before the pioneering work of Norwood and his colleagues that was published in 1980 [1], the hypoplastic left heart syndrome was essentially 100% fatal. Now, because of the steady, incremental improvements in understanding, surgical technique, and perioperative management, the modified Norwood procedure has become almost routine at major pediatric cardiovascular centers. Although the survival of such patients has improved considerably 3, 4, 5, 6, 7, there remains room for improvement.

Acknowledgements

This study was supported in part by a grant from the Deutsche Forschungsgemeinschaft.

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