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Pachydermoperiostosis accompanied by heart failure
Kwen-Chul Shin1, Wook-Jin Chung, Ki Young Lee
1Division of Cardiology, Department of Internal Medicine, Gachon University Gil Hospital, Incheon, Korea.
Journal of Cardiovascular Ultrasound
|July 28, 2010
Summary
Pachydermoperiostosis, a rare condition, can present with heart failure. This case report details a patient with pachydermoperiostosis and severe left ventricular systolic dysfunction, successfully treated with medication.
Area of Science:
- Cardiology
- Rheumatology
- Genetics
Background:
- Pachydermoperiostosis (primary hypertrophic osteoarthropathy) is a rare genetic disorder.
- It is characterized by a distinct phenotype including digital clubbing, pachydermia, and periostosis.
- Cardiac involvement in pachydermoperiostosis is not well-documented.
Observation:
- A patient presented with the complete form of pachydermoperiostosis.
- Clinical features included digital clubbing, hand enlargement, facial skin thickening, and lower leg periosteal new bone formation.
- Echocardiography revealed severely decreased left ventricular systolic function, indicative of heart failure.
Findings:
- This is the first reported case of pachydermoperiostosis associated with heart failure.
- Medical treatment led to improvement in cardiac function and patient symptoms.
- The findings suggest a potential link between pachydermoperiostosis and cardiac complications.
Implications:
- This case highlights the importance of considering cardiac evaluation in patients with pachydermoperiostosis.
- Further research is needed to understand the pathophysiology of cardiac involvement in this condition.
- Early diagnosis and management of heart failure may improve outcomes for affected individuals.
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