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[A false diagnosis of hypertrophic myocardiopathy in pheochromocytoma]
H F Lopes1, H B Silva, C de C Frimm
1Instituto do Coração do Hospital das Clínicas - FMUSP.
Insights
Pheochromocytoma, a rare adrenal tumor, caused severe hypertension and left ventricular hypertrophy in a young man. Surgical removal normalized blood pressure and reversed cardiac changes, indicating the hypertrophy was secondary to the tumor.
Area of Science:
- Cardiology
- Endocrinology
- Oncology
Background:
- Pheochromocytoma is a rare neuroendocrine tumor causing severe hypertension.
- Hypertension can lead to left ventricular hypertrophy (LVH).
- Differentiating secondary LVH from hypertrophic cardiomyopathy is crucial.
Observation:
- A 24-year-old man presented with hypertensive crises and suspected neurofibromatosis/pheochromocytoma.
- Electrocardiogram revealed left ventricle hypertrophy; echocardiogram showed thickened interventricular septum (16mm) and posterior wall (11mm).
- Elevated urinary vanillylmandelic acid and metanephrines confirmed pheochromocytoma.
Findings:
- Pheochromocytoma was localized via 131I MIBG scan and CT scan.
- Successful right adrenalectomy normalized blood pressure.
- One-year follow-up showed a healthy patient with normal cardiac structure and function.
Implications:
- This case highlights pheochromocytoma as a reversible cause of secondary hypertension and LVH.
- Prompt diagnosis and treatment of pheochromocytoma can prevent long-term cardiovascular complications.
- It underscores the importance of considering secondary causes of hypertension in young adults with cardiac abnormalities.
Abstract:
A 24 year-old man was admitted with hypertensive crises and diagnosis hypothesis of neurofibromathosis and pheochromocytoma with blood pressure of 150 x 110mmHg and in use of anti-hypertensive drugs. The electrocardiogram (EKG) showed left ventricle hypertrophy. An echocardiogram showed interventricular septum (IVS) thickness of 16mm, posterior wall (PW) thickness of 11mm (ratio IVS/PW was 1.4). Diastolic ventricular diameter was 39mm with gradient of 52mmHg and mild mitral-valve murmur by pulsate Doppler. Increased vanillylmandelic acid and metanephrines in a 24-hour sample of urine has confirmed diagnosis of pheochromocytoma within was localized by 131I metaiodobenzyl-guanidine scan and computerized axial tomography. The patient was submitted to right adrenalectomy. Blood pressure was normalized. Evaluation an year later revealed a healthy man with normal laboratory exams, EKG and echocardiogram. It seems that the hypertrophy was consequence of the hypertension and pheochromocytoma, was not hypertrophic cardiomyopathy.