Premium
Echocardiographic Changes in Cases of Intermittent Acute Porphyria
Author(s) -
PANWAR RAJA BABU,
SETIA VIJAY,
GUPTA BAL KISHAN,
SAKSENA HEM CHAND
Publication year - 1997
Publication title -
echocardiography
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.404
H-Index - 62
eISSN - 1540-8175
pISSN - 0742-2822
DOI - 10.1111/j.1540-8175.1997.tb00714.x
Subject(s) - medicine , acute intermittent porphyria , cardiology , ejection fraction , porphyria , thickening , gastroenterology , excretion , endocrinology , heart failure , chemistry , polymer science
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.