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Updated: Jun 16, 2026
09:22
Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Right ventricular dysplasia with massive thromboemboli in tuberous sclerosis complex
Yong-Hyun Kim1, Gi Young Jang, Chang Sung Son
1Division of Cardiology, Korea University College of Medicine, 126-1 Anamdong 5-ga, Seongbuk-gu, Seoul 136-705, Republic of Korea.
Pediatric Cardiology
|February 10, 2010
Summary
A boy with tuberous sclerosis and right ventricular dysplasia developed severe dyspnea due to large blood clots in his heart and lungs. This case highlights a rare but serious complication of tuberous sclerosis.
Area of Science:
- Cardiology
- Pulmonology
- Genetics
Background:
- Tuberous sclerosis is a genetic disorder that can affect multiple organs.
- Right ventricular dysplasia is a rare congenital heart condition.
- Pulmonary thromboembolism is a serious condition involving blood clots in the lungs.
Observation:
- A 12-year-old boy with a history of tuberous sclerosis and right ventricular dysplasia presented with severe dyspnea.
- Imaging revealed large thrombi within the right ventricle and bilateral pulmonary arteries.
Findings:
- The patient had massive bilateral pulmonary thromboemboli originating from the right ventricle.
- No cardiac tumor was identified as the source of the emboli.
- The findings suggest a link between right ventricular dysplasia in tuberous sclerosis and thromboembolism.
Implications:
- This case underscores the importance of considering thromboembolic events in patients with tuberous sclerosis and cardiac abnormalities.
- Early diagnosis and management of pulmonary thromboembolism are crucial for improving outcomes.
- Further research may elucidate the specific mechanisms linking tuberous sclerosis, right ventricular dysplasia, and thrombogenesis.
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