Silent catalyst: Undiagnosed pheochromocytoma triggering acute aortic dissection

A 39-year-old male patient presented with sudden, severe retrosternal chest pain radiating to the back, accompanied by dyspnea, diaphoresis, and dizziness. He had no significant medical or family history. On examination, his blood pressure (BP) was elevated at 190/70 mm Hg, and a diastolic murmur was heard at the left sternal border in the fourth intercostal space. Electrocardiography and 2D echocardiography revealed left ventricular hypertrophy with no acute changes. Given the suspicion of acute aortic dissection, contrast-enhanced computed tomography angiography was performed, which revealed a Stanford type A aortic dissection extending from the aortic root to the abdominal aorta [Figure 1]. Additionally, a 4 × 3 cm left adrenal mass with central necrosis was identified. Laboratory investigations showed significantly elevated plasma-free normetanephrine levels at 12,395.1 pmol/L (reference range: 98.25–611.32 pmol/L), while plasma-free metanephrine and 3-methoxytyramine levels were within normal limits. Other evaluations for secondary hypertension, including renal artery Doppler studies, plasma aldosterone concentration, plasma renin activity, and thyroid function tests, were unremarkable.Figure 1: Computed tomography angiography of the thorax and abdomen showing aortic dissection involving the ascending thoracic aorta, descending thoracic aorta, and abdominal aorta with persistent opacification of the false lumen (red arrow), with left adrenal mass (orange arrow)BP was initially managed with intravenous nitroglycerin and esmolol infusion, aiming for a target BP of <130/80 mm Hg and a pulse rate of 60–80 bpm. This was subsequently followed by the addition of oral prazosin (an alpha-blocker) and metoprolol. The patient underwent a Bentall procedure for type A aortic dissection three days after presentation. The perioperative period was uneventful, with no pheochromocytoma-related crisis. A laparoscopic left adrenalectomy is planned for six weeks later. Genetic testing has been initiated to investigate underlying conditions. Interestingly, a similar case of bilateral pelvic paragangliomas presenting with acute aortic dissection has been previously reported at our institution. This case highlights the importance of considering pheochromocytoma in patients presenting with aortic dissection, as excessive catecholamine secretion can precipitate hypertensive crises leading to vascular complications. Acknowledgment None. Author contributions VJ was involved in manuscript writing and editing. TS helped in proofreading. SKB cared for the patient, and he assisted in the preparation and editing of the final manuscript. Patient consent The patient consent was obtained. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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Journal
IJEM Case Reports
Published
2025-01-01
DOI
https://doi.org/10.4103/ijemcr.ijemcr_2_25
Primary Topic
Adrenal and Paraganglionic Tumors
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article

Silent catalyst: Undiagnosed pheochromocytoma triggering acute aortic dissection

Sanjay Kumar Bhadada, Rama Walia, Tsering Sangdup, Vinitaa Jha
IJEM Case Reports
Adrenal and Paraganglionic Tumors
article

Silent catalyst: Undiagnosed pheochromocytoma triggering acute aortic dissection

Sanjay Kumar Bhadada, Rama Walia, Tsering Sangdup, Vinitaa Jha
article en

Abstract

A 39-year-old male patient presented with sudden, severe retrosternal chest pain radiating to the back, accompanied by dyspnea, diaphoresis, and dizziness. He had no significant medical or family history. On examination, his blood pressure (BP) was elevated at 190/70 mm Hg, and a diastolic murmur was heard at the left sternal border in the fourth intercostal space. Electrocardiography and 2D echocardiography revealed left ventricular hypertrophy with no acute changes. Given the suspicion of acute aortic dissection, contrast-enhanced computed tomography angiography was performed, which revealed a Stanford type A aortic dissection extending from the aortic root to the abdominal aorta [Figure 1]. Additionally, a 4 × 3 cm left adrenal mass with central necrosis was identified. Laboratory investigations showed significantly elevated plasma-free normetanephrine levels at 12,395.1 pmol/L (reference range: 98.25–611.32 pmol/L), while plasma-free metanephrine and 3-methoxytyramine levels were within normal limits. Other evaluations for secondary hypertension, including renal artery Doppler studies, plasma aldosterone concentration, plasma renin activity, and thyroid function tests, were unremarkable.Figure 1: Computed tomography angiography of the thorax and abdomen showing aortic dissection involving the ascending thoracic aorta, descending thoracic aorta, and abdominal aorta with persistent opacification of the false lumen (red arrow), with left adrenal mass (orange arrow)BP was initially managed with intravenous nitroglycerin and esmolol infusion, aiming for a target BP of <130/80 mm Hg and a pulse rate of 60–80 bpm. This was subsequently followed by the addition of oral prazosin (an alpha-blocker) and metoprolol. The patient underwent a Bentall procedure for type A aortic dissection three days after presentation. The perioperative period was uneventful, with no pheochromocytoma-related crisis. A laparoscopic left adrenalectomy is planned for six weeks later. Genetic testing has been initiated to investigate underlying conditions. Interestingly, a similar case of bilateral pelvic paragangliomas presenting with acute aortic dissection has been previously reported at our institution. This case highlights the importance of considering pheochromocytoma in patients presenting with aortic dissection, as excessive catecholamine secretion can precipitate hypertensive crises leading to vascular complications. Acknowledgment None. Author contributions VJ was involved in manuscript writing and editing. TS helped in proofreading. SKB cared for the patient, and he assisted in the preparation and editing of the final manuscript. Patient consent The patient consent was obtained. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

IJEM Case ReportsVol. 3(1)
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