RIGHT VENTRICULAR DYSFUNCTION IN THIAMINE-RESPONSIVE MEGALOBLASTIC ANEMIA SYNDROME: A CASE REPORT

Authors

SHAHEED RAJAEI CARDIOVASCULAR, MEDICAL AND RESEARCH CENTER, TEHRAN UNIVERSITY OF MEDICAL SCIENCES, VALI-ASRE AVE., ADJACENT TO MELLAT PARK,TEHRAN,IRAN

Abstract

Thiamine or Vitamin B1 is an essential cofactor for many metabolic processes in numerous tissues.
Thiamine-Responsive Megaloblastic Anemia Syndrome is a genetic disorder affecting thiamine transportation with consequent bone marrow, pancreatic, neurological, and cardiac functional and developmental anomalies. There are limited reports of the cardiac manifestations of this syndrome.
Here, we present a case of this syndrome in a male patient with right ventricular dysfunction and atrial dysrhythmias.

Keywords