Echocardiographic Changes in Cases of Intermittent Acute Porphyria.

Abstract

Intermittent acute porphyria (IAP) is an inborn metabolic disorder of biosynthesis of haeme, characterized by increased excretion of porphyrin or porphyrin precursor in urine and clinically by gastrointestinal, neuro-psychiatric and cardiovascular manifestations. Significant observations were made on echocardiographic examination of 25 IAP patients in our study. Significant decrease in ejection fraction (48.4 +/- 7.9, control group 63.0 +/- 7.5, P < 0.001) and percentage of fractional shortening (23.5% +/- 6.87%, control group = 36.4 +/- 4.26, P </=.001) was observed in IAP, this shows derangement in left ventricular systolic functions in IAP. The study also shows significant thickening of IVS (1.22 +/- 0.20, control group 0.88 +/- 0.12 cm, P </= 0.001). The most interesting finding was the significantly increased incidence of mitral valve and/or tricuspid valve prolapse in 15 (60%) patients of IAP. Thickening of AML, PML, and calcification of AML were also observed.