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Ann Thorac Surg 2006;81:1802-1807
© 2006 The Society of Thoracic Surgeons
Pediatric Heart Center, UCSF Children's Hospital, San Francisco, California
Accepted for publication December 1, 2005.
* Address correspondence to Dr Karl, 513 Parnassus Ave, S-549, San Francisco, CA 94143-0117 (Email: karlt{at}surgery.ucsf.edu).
Presented at the Poster Session of the Forty-first Annual Meeting of The Society of Thoracic Surgeons, Tampa, FL, Jan 24–26, 2005.
BACKGROUND: Congenital absence of the pulmonary valve syndrome (APVS) is a rare cardiac defect characterized by aneurysmal pulmonary arteries, hypoplastic pulmonary valve cusps, and tracheobronchial abnormalities. Absence of the pulmonary valve syndrome usually occurs in conjunction with ventricular septal defect (VSD) and right ventricular outflow tract obstruction (RVOTO). Surgical mortality rates as high as 16% to 56% have been reported. Here, we describe the surgical results using the Lecompte maneuver, reduction pulmonary arterioplasty, and a valved right ventricle to pulmonary artery (RV-PA) conduit.
METHODS: Medical records and operative and echocardiography reports for all surgical APVS cases were retrospectively examined for pertinent clinical variables. A patient with left bronchial compression due to enlarged pulmonary arteries associated with totally anomalous pulmonary venous drainage (TAPVD) is included to illustrate the value of the Lecompte maneuver.
RESULTS: From January 2002 to December 2004, 4 children with APVS had surgery at a median age of 5 months (range, 3 months to 3.5 years). Three had malalignment VSD and RVOTO. Four had respiratory signs (cough, wheeze, tachypnea, oxygen dependence, ventilator dependence), and all 5 had evidence of tracheobronchial compression by computed tomography or magnetic resonance imaging. Repair included a Lecompte maneuver, a valved conduit with reduction pulmonary arterioplasty, and VSD closure as necessary. The TAPVD patient had repair of the anomalous veins and a Lecompte maneuver. There have been no deaths, and all patients were discharged in good condition. Follow-up is complete at a median of 24 months (range, 9 to 35). Echocardiography has shown no progressive enlargement of the pulmonary arteries.
CONCLUSIONS: Use of the Lecompte maneuver, reduction arterioplasty, and a valved conduit for repair of APVS provides favorable early and midterm results.
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L. Nolke, N. Alphonso, and T. R. Karl Reply Ann. Thorac. Surg., February 1, 2007; 83(2): 727 - 728. [Full Text] [PDF]
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