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Ann Thorac Surg 1991;52:688-695
© 1991 The Society of Thoracic Surgeons

Articles

Hypoplastic left heart syndrome

William I. Norwood, Jr, MD, PhD*

Department of Surgery, University of Pennsylvania School of Medicine, and Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

* Address reprint requests to Dr Norwood, Division of Cardiothoracic Surgery. The Children's Hospital of Philadelphia, 34th St and Civic Blvd, Philadelphia, PA 19104.

The evolution of the present approach to the newborn with hypoplastic left heart is outlined. Preoperatively, maintenance of ductal patency with prostaglandin E1 and balancing of systemic and pulmonary blood flow are essential. Operative details of the first-stage palliation and the definitive second-stage procedure are described. The more recent adoption of an intermediate-stage hemi-Fontan procedure is also described. Since January 1989, 151 patients have been treated using this three-stage approach, with 109 early survivors. Seventy-eight have undergone the hemi-Fontan operation with nine deaths (5 of whom came to this stage early nonelectively because of shunt failure or ventricular dysfunction). Twenty-seven of the 78 patients undergoing hemi-Fontan operation have subsequently undergone definitive Fontan procedures with no deaths.




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