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Pulmonary veno-occlusive disease associated with hypertrophic cardiomyopathy

R Chetty1, A G Rose1, P J Commerford2

  • 1Department of Anatomical Pathology, University of Cape Town and Groote Schuur Hospital, Cape Town, South Africa.

Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology
|May 21, 2015
PubMed

Insights

Autopsy findings reveal that hypertrophic cardiomyopathy (HCM) frequently co-occurs with pulmonary veno-occlusive disease (PVOD). Further genetic analysis is recommended to explore a potential shared genetic basis for these rare conditions.

Area of Science:

  • Cardiovascular Pathology
  • Pulmonary Vascular Disease

Background:

  • Hypertrophic cardiomyopathy (HCM) is a primary cardiac condition.
  • Pulmonary veno-occlusive disease (PVOD) is a rare and severe vascular disorder affecting the lungs.

Purpose of the Study:

  • To investigate the lung findings in patients with hypertrophic cardiomyopathy.
  • To determine the prevalence and significance of pulmonary veno-occlusive disease in HCM cases.

Main Methods:

  • Autopsy analysis of lung tissue from 14 hypertrophic cardiomyopathy cases.
  • Histological examination focusing on pulmonary vessels.

Main Results:

  • Six out of 14 (43%) hypertrophic cardiomyopathy cases exhibited histological features of pulmonary veno-occlusive disease.
  • The observed coexistence suggests a potential association beyond chance.

Conclusions:

  • There is a notable association between hypertrophic cardiomyopathy and pulmonary veno-occlusive disease.
  • Genetic analysis in PVOD patients is warranted to investigate a possible common genetic etiology with HCM.

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