Visceral Heterotaxy in the Developing World - 27/07/12
Corresponding author at: Department of Pediatric Cardiology, Institute of Cardio-Vascular Diseases, The Madras Medical Mission, No. 4-A, Dr. J.J. Nagar, Mogappair, Chennai 600037, India. Tel.: +91 44 26561801; fax: +91 44 26565859.Résumé |
Background |
The challenge of visceral heterotaxy (VH) in the developing world has not been analysed in detail.
Method |
Retrospective chart review of 69 consecutive patients over ten years assessed the clinical profile and surgical outcome of VH. Median age: 3 years; median weight: 15kg. Diagnosis was made by echocardiography supplemented with blood smear (Howell Jolly bodies), Multi-Detector Computed Tomography (MDCT) angiogram and/or surgical inspection.
Results |
In right isomerism (RI) group (n=32), 12 patients did not undergo surgery, five had Blalock Taussig shunt, 14 had bidirectional Glenn and one had Fontan completion, with surgical mortality of 5%. In left isomerism (LI) group (n=31), 11 patients underwent two ventricle repair (35%) and 15 (48%) had single ventricle repair, with surgical mortality of 3.8%; five did not have surgery. On follow up (median period 1.5 years), 33% of un-operated patients and 25% of operated patients died, mortality being higher for RI patients. Late mortality was due to sepsis, heart failure or arrhythmia.
Conclusion |
VH can be diagnosed by imaging based criteria. VH tends to present late in the developing world with a significant percentage inoperable. LI had better surgical outcome and higher long term survival.
Le texte complet de cet article est disponible en PDF.Keywords : Visceral heterotaxy, Right isomerism, Left isomerism, Surgical outcome
Plan
Vol 21 - N° 9
P. 598-605 - septembre 2012 Retour au numéro
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