Document Detail

The Lecompte maneuver for relief of airway compression in absent pulmonary valve syndrome.
MedLine Citation:
PMID:  16631676     Owner:  NLM     Status:  MEDLINE    
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.
Lars Nölke; Anthony Azakie; Petrous V Anagnostopoulos; Nelson Alphonso; Tom R Karl
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Annals of thoracic surgery     Volume:  81     ISSN:  1552-6259     ISO Abbreviation:  Ann. Thorac. Surg.     Publication Date:  2006 May 
Date Detail:
Created Date:  2006-04-24     Completed Date:  2006-05-25     Revised Date:  2007-03-06    
Medline Journal Info:
Nlm Unique ID:  15030100R     Medline TA:  Ann Thorac Surg     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  1802-7     Citation Subset:  AIM; IM    
Pediatric Heart Center, UCSF Children's Hospital, San Francisco, California, USA.
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MeSH Terms
Anastomosis, Surgical
Aorta / surgery
Blood Vessel Prosthesis Implantation
Bronchial Diseases / etiology,  surgery*
Cardiac Surgical Procedures*
Child, Preschool
Dilatation, Pathologic
Heart Defects, Congenital / surgery*
Heart Septal Defects, Ventricular / epidemiology,  surgery
Pulmonary Artery / pathology,  surgery
Pulmonary Valve / abnormalities*,  surgery
Retrospective Studies
Ventricular Outflow Obstruction / epidemiology
Comment In:
Ann Thorac Surg. 2007 Feb;83(2):727; author reply 727-8   [PMID:  17258038 ]

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