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[A marked Hegglin syndrome in pheochromocytoma]
Insights
A patient with severe hypertension and cardiac issues was diagnosed with a pheochromocytoma. Surgical removal of the adrenal tumor resolved the patient's Hegglin syndrome and improved left ventricular hypertrophy.
Area of Science:
- Cardiology
- Endocrinology
- Oncology
Background:
- Pheochromocytoma, a rare neuroendocrine tumor of the adrenal medulla, can cause severe hypertension and cardiac complications due to excess catecholamine secretion.
- Patients may present with hypertensive crisis, cardiac hypertrophy, and arrhythmias, mimicking other cardiovascular diseases.
Observation:
- A 46-year-old male presented with paroxysmal headache, chest discomfort, and severe hypertension (up to 300/160 mmHg).
- Cardiac examination revealed aortic regurgitation, S4 gallop, and electrocardiogram/echocardiogram findings consistent with severe left ventricular hypertrophy and hypertrophic cardiomyopathy.
- Phonocardiogram demonstrated marked Hegglin syndrome (prolonged QT and QII intervals).
Findings:
- Abdominal CT and 131I-metaiodobenzylguanidine (MIBG) scintigraphy confirmed a right adrenal pheochromocytoma.
- Post-operative assessment showed complete resolution of Hegglin syndrome (QT: 360 msec, QII: 345 msec) and immediate improvement in electrocardiographic left ventricular hypertrophy patterns.
Implications:
- This case highlights the critical importance of considering pheochromocytoma in patients with severe hypertension and unexplained cardiac abnormalities.
- Surgical resection of pheochromocytoma can lead to rapid and complete reversal of associated cardiovascular manifestations, including arrhythmias and left ventricular hypertrophy.
- Early diagnosis and treatment of pheochromocytoma are crucial for preventing potentially life-threatening cardiovascular complications.
Abstract:
A 46-year-old man was hospitalized with paroxysmal headache and chest discomfort. His blood pressure varied, occasionally being up to 300/160 mmHg. Cardiac examination revealed a decrescendo type of diastolic murmur (aortic regurgitation) and S4 gallop. Both blood and urine catecholamine levels were extremely high. Electrocardiogram and echocardiogram showed severe left ventricular hypertrophy pattern presenting hypertrophic cardiomyopathic changes. The phonocardiogram showed marked Hegglin syndrome (QT; 450 msec and QII; 310 msec). Right adrenal tumor, pheochromocytoma was found on the abdominal CT scanning and 131I-metaiodobenzylguanidine (MIBG). In the post-operative period, Hegglin syndrome was completely abolished (QT; 360 msec and QII; 345 msec,), and also electrocardiographic left ventricular hypertrophy pattern improved immediately after surgical removal of the pheochromocytoma.