title: 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.
authors.affiliation: Cardiology Division, Department of Medicine, S.P. Medical College, Bikaner (Rajasthan&rpar, India.
authors.forename: Raja Babu
authors.initials: RB
authors.lastname: Panwar
authors.forename: Vijay
authors.lastname: Setia
authors.forename: Bal Kishan
authors.initials: BK
authors.lastname: Gupta
authors.forename: Hem Chand
authors.initials: HC
authors.lastname: Saksena