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Coronary artery disease in an 18 year old with pseudoxanthoma elasticum: successful surgical therapy
Insights
Pseudoxanthoma elasticum can affect coronary arteries, leading to severe disease in young adults. This case highlights successful surgical intervention for complex coronary artery disease in a patient with this rare genetic disorder.
Area of Science:
- Cardiology
- Genetics
- Vascular Biology
Background:
- Pseudoxanthoma elasticum (PXE) is a rare genetic disorder characterized by the deposition of calcium and elastin in connective tissues, primarily affecting the skin, eyes, and cardiovascular system.
- Cardiovascular manifestations of PXE can include arterial calcification, peripheral arterial disease, and premature coronary artery disease (CAD).
Observation:
- An 18-year-old female with a history of angina pectoris and myocardial infarction presented with three-vessel CAD.
- Coronary angiography revealed significant stenosis in multiple coronary arteries.
- Histological examination of a coronary artery segment showed changes consistent with PXE vascular lesions.
Findings:
- The patient underwent successful triple coronary artery-saphenous vein bypass grafting.
- She remained asymptomatic for one year post-surgery.
- Histopathology confirmed PXE-related vascular changes in the affected coronary artery.
Implications:
- This case underscores the importance of considering PXE in young patients presenting with premature and severe CAD.
- Early diagnosis and aggressive management of cardiovascular risk factors are crucial in patients with PXE.
- Surgical revascularization can be an effective treatment option for managing severe CAD in PXE patients.
Abstract:
An 18 year old girl with pseudoxanthoma elasticum, a 7 year history of angina pectoris and evidence of an old anteroseptal myocardial infarction was found on coronary angiography to have three vessel coronary artery disease. A triple coronary artery-saphenous vein bypass graft was performed, and she has been asymptomatic for 1 year. Histologic examination of a segment of the right coronary artery revealed changes consistent with the vascular lesion of pseudoxanthoma elasticum.