Vasospastic angina in patients with systemic triglyceride storage disease with Jordans' anomaly and cardiomyopathy

Shin Ichi Ando, Makoto Usui, Takahiro Matsumoto, Kensuke Egashira, Akira Takeshita, Fumio Terasaki, Hirofumi Deguchi, Keishiro Kawamura

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4 Citations (Scopus)

Abstract

Vasospastic angina was demonstrated clinically and angiographically in a 54-year-old patient with systemic triglyceride storage disease and cardiomyopathy. He and his younger sister had been diagnosed in 1985 as having systemic triglyceride storage with Jordans' anomaly. In 1993, he began complaining of rest and effort chest pain in the morning, which was accompanied by ST depression by ECG. Sublingual nitroglycerine was cffective for treating this pain. Intracoronary injection of acetylcholine induced severe coronary vasoconstriction in the left anterior descending artery. Left ventricular contraction was diffusely impaired. Deposits of numerous triglyceride droplets and a decrease in the density of myofibrils in cardiocytes were found in the specimens obtained by endomyocardial biopsy. The impaired left ventricular contraction may have been due to the changes in myocardial cells. His sister complained of a similar chest pain that was completely controlled by calcium channel antagonist. Thus, our cases demonstrated vasospastic angina associated with cardiomyopathy secondary to systemic triglyceride storage disease with Jordans' anomaly, though the causal relationship between these conditions remains unclear.

Original languageEnglish
Pages (from-to)124-129
Number of pages6
JournalJAPANESE CIRCULATION JOURNAL
Volume60
Issue number2
DOIs
Publication statusPublished - Apr 11 1996

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All Science Journal Classification (ASJC) codes

  • Physiology
  • Cardiology and Cardiovascular Medicine

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