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J Thorac Cardiovasc Surg 2003;126:295-297
© 2003 The American Association for Thoracic Surgery

Brief communication

Bilateral lung transplantation for pulmonary hypoplasia caused by congenital diaphragmatic hernia

^a From the Division of Cardiothoracic Surgery and the Department of Pediatrics, Washington University, St Louis, Mo, USA

St Louis, Mo Received for publication September 7, 2002; accepted for publication December 2, 2002.

^* Address for reprints: Richard Lee, MD, Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic Foundation, 9500 Euclid Ave, Desk F-24, Cleveland, OH 44195, USA
leer2@ccf.org

The first 300 words of the full text of this article appear below.

Congenital diaphragmatic hernia (CDH) remains a life-threatening neonatal anomaly. Patients who have CDH without pulmonary hypoplasia have an expected mortality that ranges from 10% to 25%.¹ However, when pulmonary hypoplasia is associated with CDH, the prognosis is even worse. Although the introduction and use of extracorporeal membrane oxygenation (ECMO) has improved survival in patients with unilateral pulmonary hypoplasia, mortality still ranges from 20% to 40% in this group.¹ CDH with bilateral pulmonary hypoplasia is a condition that has previously been incompatible with life.

Several innovative therapies have been offered in an attempt to improve the outcome in these high-risk patients. One of these therapies is lung transplantation. This option is often discussed as a potential solution and has some experimental support.^2,3 However, the clinical experience in the literature is limited to a case report of a unilateral lung transplantation,⁴ followed by a transplant pneumonectomy nearly 5 years after transplantation.⁵ Here we present the outcomes of 3 patients in whom bilateral lung transplantation was performed for pulmonary hypoplasia associated with CDH at our institution.

Clinical Summaries

PATIENT 1. A diagnosis of CDH was established at 18 weeks’ gestation in a female fetus. An ultrasound demonstrated the stomach, bowel, and part of the left lobe of the liver in the left chest and the heart in the right chest. Displacement of the heart made evaluation difficult. Shortly after birth, the child was blue and without respiratory effort. Initial physical examination was notable for absent breath sounds on the left, as well as heart sounds on the right. She was immediately intubated. Chest radiography demonstrated viscera in the left chest. Echocardiography demonstrated double-inlet left ventricle, transposition of the great arteries, subaortic stenosis, coarctation of the aorta, a patent ductus arteriosus, and a patent foramen ovale with left-to-right flow.

The patient’s condition was extremely difficult to . . . [Full Text of this Article]