Heterotaxy Syndrome Combining Univentricular Heart, Polysplenia and Truncated Pancreas: A Case Report.

BACKGROUND AND

OBJECTIVES

Univentricular heart is a complex congenital heart disease with rare survival into adulthood. It could be part of a heterotaxy syndrome when, apart from the cardiac malformations, there are associated abnormal arrangements of extra-cardiac organs like the lungs, spleen and pancreas. The syndrome is said to be rarer than the univentricular heart and to have a worse clinical outcome.

METHODS

After clinical assessment, the patient was investigated with Chest X-ray, Electrocardiography, Echocardiography, and contrast-enhanced CT scan of the chest and abdomen to make the diagnosis.

RESULTS

We hereby report the case of a 19-year-old female who survived into adulthood with a functional single ventricle, double outlet right ventricle, atrial septal defect and dextrocardia without surgical intervention. She also had other extra-cardiac congenital anomalies like a truncated pancreas and polysplenia.

CONCLUSION

Though survival to adulthood is rare without surgical intervention, the left isomerism subtype recorded here is milder and has better survival prospects.