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Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
09:22

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Published on: November 4, 2015

Pathological lesions causing pulmonary hypertension after closure of a ventricular septal defect

Katsuhide Maeda1, Shigeo Yamaki, Mitsunori Nishiyama

  • 1Department of Cardiothoracic Surgery, Faculty of Medicine, University of Tokyo, Tokyo, Japan.

The Japanese Journal of Thoracic and Cardiovascular Surgery : Official Publication of the Japanese Association for Thoracic Surgery = Nihon Kyobu Geka Gakkai Zasshi
|October 8, 2003
PubMed

Insights

Extremely thickened media (ETM) in pulmonary arteries can cause persistent pulmonary hypertension, even after surgical repair. This case highlights how ETM can lead to unresolved pulmonary hypertension post-repair.

Area of Science:

  • Cardiology
  • Pulmonary Medicine
  • Pediatrics

Background:

  • A 15-year-old boy presented with complex congenital heart disease, including ventricular septal defect and Down's syndrome.
  • He had pre-existing pulmonary hypertension and a rare condition of extremely thickened media (ETM) in small pulmonary arteries.

Observation:

  • Microscopic examination of lung tissue from pre-repair and autopsy specimens showed that the ETM in the affected pulmonary arteries remained unchanged.
  • Adjacent arteries connected to those with ETM exhibited severe thickening, indicating disease progression or secondary effects.

Findings:

  • The study demonstrates that a small proportion of pulmonary arteries affected by ETM can be sufficient to drive significant pulmonary hypertension.
  • ETM in pulmonary arteries is a persistent pathological feature that can impede the resolution of pulmonary hypertension post-surgical repair.

Implications:

  • This case highlights a potential mechanism for the failure to resolve pulmonary hypertension after surgical intervention in specific pediatric cardiac conditions.
  • Understanding the role of ETM is crucial for managing complex congenital heart disease and associated pulmonary vascular pathology.

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