Visceral heterotaxy in the developing world.
BACKGROUND
The challenge of visceral heterotaxy (VH) in the developing world has not been analysed in detail.


METHODS
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.


CONCLUSIONS
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.
Department of Pediatric Cardiology, Institute of Cardio-Vascular Diseases, The Madras Medical Mission, Mogappair, Chennai, India.
Deepak Kishinchand
DK
Changlani
Monika
M
Kotecha
Trupti Dongre
TD
Changlani
Roy
R
Varghese
Raghavannair Suresh
RS
Kumar
D000293:Adolescent; D000328:Adult; D002648:Child; D003914:Dextrocardia / Q000097:blood / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D018572:Disease-Free Survival; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000097:blood / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D059446:Heterotaxy Syndrome / Q000097:blood / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007194:India / Q000453:epidemiology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate