Document Detail

Intracardiac Fontan procedure for heterotaxy syndrome with complex systemic and pulmonary venous anomalies.
MedLine Citation:
PMID:  19695897     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: The extracardiac conduit procedure is widely used for patients with heterotaxy syndrome with complex systemic and pulmonary venous anomalies; however, it lacks conduit-growth potential and requires long-term anticoagulation. We present the intracardiac Fontan procedure, which eliminates the above-mentioned disadvantages.
PATIENTS AND METHODS: Twenty-four patients (mean age, 4.1 years; weight, 13.0 kg) with heterotaxy syndrome underwent intracardiac Fontan operations between March 1995 and March 2008. In each patient, the anomalous systemic venous return with the isolated hepatic vein was redirected to the pulmonary artery using an intra-atrial baffle without obstructing the pulmonary venous pathway; this was accomplished by anterior (n=5), lateral (n=15) or posterior tunnel methods (n=4), depending on the anatomical relationship of the systemic and pulmonary venous pathways.
RESULTS: There was one (4%) in-hospital and two (8%) late deaths in total. Five (21%) patients underwent re-operation for either pulmonary venous obstruction or supraventricular tachycardia. The actuarial 12-year survival was 86% (the Kaplan-Meier survival plot). The freedom from re-operation at 10 years was 77%. Anticoagulation was not required except for two patients (8%) who had prosthetic valves and coagulation disorder. Obstruction of the systemic venous pathway was not observed in any patient; however, five (20%) patients had clinically significant postoperative arrhythmias. At the final follow-up, all survivors were categorised as the New York Heart Association class I.
CONCLUSIONS: Aided by detailed preoperative anatomical and physiological diagnoses, intracardiac Fontan procedures were technically feasible in patients with complex systemic and pulmonary venous anomalies. The specific cardiac anatomy in these patients warranted this procedure; however, taking into consideration the improved outcomes of the modified Fontan procedure, this method should be performed with deliberation.
Yuji Naito; Mitsuru Aoki; Kozo Matsuo; Hiromichi Nakajima; Hiroyuki Aotsuka; Tadashi Fujiwara
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Publication Detail:
Type:  Case Reports; Evaluation Studies; Journal Article     Date:  2009-08-19
Journal Detail:
Title:  European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery     Volume:  37     ISSN:  1873-734X     ISO Abbreviation:  Eur J Cardiothorac Surg     Publication Date:  2010 Jan 
Date Detail:
Created Date:  2010-02-03     Completed Date:  2011-01-11     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8804069     Medline TA:  Eur J Cardiothorac Surg     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  197-203     Citation Subset:  IM    
Copyright Information:
Copyright 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.
Department of Cardiovascular Surgery, Chiba Children's Hospital, Midori-ku, Chiba, Japan.
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MeSH Terms
Abnormalities, Multiple / diagnosis,  surgery
Child, Preschool
Dextrocardia / diagnosis,  surgery
Epidemiologic Methods
Fibrinolytic Agents / therapeutic use
Fontan Procedure / methods*
Genetic Diseases, X-Linked / diagnosis,  surgery
Heart Catheterization
Postoperative Care / methods
Postoperative Complications
Pulmonary Veins / abnormalities,  surgery
Situs Inversus / diagnosis,  surgery
Treatment Outcome
Reg. No./Substance:
0/Fibrinolytic Agents

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