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Published on: November 18, 2018
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.
Background:
The predominance of chronic thromboembolic pulmonary hypertension (CTEPH) in females and association of HLA-B*5201 with CTEPH have been reported in Japan. However, the clinical characteristics of female CTEPH remain uncertain. The purpose of the present study is to clarify the clinical phenotype of female CTEPH in Japan.
Methods And Results:
The 150 consecutive patients (female 103, male 47; age 52.8+/-12.4 years SD) were admitted to Chiba University Hospital, and diagnosis was confirmed using right cardiac catheterization and pulmonary angiography. Among these patients, 78 underwent pulmonary endarterectomy. Clinical characteristics, pulmonary hemodynamics, extent of central disease and surgical outcome in females were compared with those in males. The female patients were elderly and had less deep vein thrombosis, less acute embolic episodes, better cardiac function, lower arterial oxygen tension and more peripheral thrombi, and showed less improvement through surgery than males. When the patients were identified using HLA-B*5201, HLA-B*5201-positive female patients had less embolic episodes and better cardiac function with lower operative mortality. In contrast, HLA-B*5201-negative female patients had less embolic episodes, and more peripheral thrombi, resulting in less improvement by surgery.
Conclusion:
The clinical phenotype of female CTEPH differed from that of male CTEPH. Additionally, gender differences of HLA-B*5201-positive type were dissimilar to those of HLA-B*5201-negative type.
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