Gender differences in chronic thromboembolic pulmonary hypertension in Japan

Ayako Shigeta1, Nobuhiro Tanabe, Hidefumi Shimizu

  • 1Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan. ayakosh@nifty.com

Insights

Female patients with chronic thromboembolic pulmonary hypertension (CTEPH) exhibit distinct clinical features compared to males. These differences are further influenced by HLA-B*5201 status, impacting embolic episodes and surgical outcomes in CTEPH.

Area of Science:

  • Cardiology
  • Pulmonology
  • Genetics

Background:

  • Chronic thromboembolic pulmonary hypertension (CTEPH) predominantly affects females in Japan.
  • The association of HLA-B*5201 with CTEPH has been noted in Japanese populations.
  • Clinical characteristics of female CTEPH patients require further elucidation.

Purpose of the Study:

  • To clarify the clinical phenotype of female CTEPH patients in Japan.
  • To compare the clinical characteristics, hemodynamics, disease extent, and surgical outcomes of female CTEPH patients with those of male CTEPH patients.
  • To investigate the influence of HLA-B*5201 status on the clinical phenotype and outcomes in female CTEPH.

Main Methods:

  • Retrospective analysis of 150 consecutive CTEPH patients diagnosed via right cardiac catheterization and pulmonary angiography.
  • Comparison of clinical data, pulmonary hemodynamics, disease extent, and surgical outcomes between female and male CTEPH patients.
  • Stratification of female patients based on HLA-B*5201 status to analyze genotype-specific clinical features and outcomes.

Main Results:

  • Female CTEPH patients were older, had less deep vein thrombosis and acute embolic episodes, better cardiac function, lower arterial oxygen tension, and more peripheral thrombi compared to males.
  • Females showed less improvement after pulmonary endarterectomy surgery than males.
  • HLA-B*5201-positive females presented with fewer embolic episodes and better cardiac function, alongside lower operative mortality.
  • HLA-B*5201-negative females exhibited fewer embolic episodes and more peripheral thrombi, leading to diminished surgical improvement.

Conclusions:

  • The clinical phenotype of female CTEPH patients differs significantly from that of male CTEPH patients.
  • Gender-specific differences in CTEPH are influenced by HLA-B*5201 status.
  • The interplay between gender, HLA-B*5201, and clinical presentation impacts CTEPH management and surgical outcomes.
Abstract

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