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Funny Lumps, Flaming Pheo, and a Broken Heart: A Rare Case of Pheochromocytoma

Pheochromocytoma is the underlying etiology in 0.1% of hypertensive cases. However, it may be present in up to 5.7% of patients with neurofibromatosis I (NF1). The burst of catecholamines inherent in pheochromocytoma has significant effects on the mechanical and electrical activity of the myocardium...

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Autores principales: Butt, Khurram, Ali, Saeed, Sattar, Zeeshan, Ur Rahman, Asad, Burt, Jeremy R
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351109/
https://www.ncbi.nlm.nih.gov/pubmed/30723645
http://dx.doi.org/10.7759/cureus.3646
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author Butt, Khurram
Ali, Saeed
Sattar, Zeeshan
Ur Rahman, Asad
Burt, Jeremy R
author_facet Butt, Khurram
Ali, Saeed
Sattar, Zeeshan
Ur Rahman, Asad
Burt, Jeremy R
author_sort Butt, Khurram
collection PubMed
description Pheochromocytoma is the underlying etiology in 0.1% of hypertensive cases. However, it may be present in up to 5.7% of patients with neurofibromatosis I (NF1). The burst of catecholamines inherent in pheochromocytoma has significant effects on the mechanical and electrical activity of the myocardium. Different theories have been postulated for myocardial stunning in patients with pheochromocytoma that include microvascular spasm, impaired fatty acid metabolism, increased production of oxygen-derived free radicals and dynamic left ventricular mid-cavity obstruction. QT interval prolongation is seen in 16% to 35% of patients with pheochromocytoma. Takotsubo cardiomyopathy (TS) is now being increasingly identified and it may be responsible for up to 40% of cases of acute catecholamine cardiomyopathy. These manifestations may sometimes precede or cloud the typical triad of a headache, sweating, and tachycardia. We herein present a case of a 42-year-old female with a unique combination of QT prolongation, torsades de pointes, and TS caused by pheochromocytoma in the background of NF1. All these complications are potentially reversible with the removal of the underlying adrenal tumor, underscoring the importance of a high suspicion for pheochromocytoma in patients with NF1.
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spelling pubmed-63511092019-02-05 Funny Lumps, Flaming Pheo, and a Broken Heart: A Rare Case of Pheochromocytoma Butt, Khurram Ali, Saeed Sattar, Zeeshan Ur Rahman, Asad Burt, Jeremy R Cureus Cardiology Pheochromocytoma is the underlying etiology in 0.1% of hypertensive cases. However, it may be present in up to 5.7% of patients with neurofibromatosis I (NF1). The burst of catecholamines inherent in pheochromocytoma has significant effects on the mechanical and electrical activity of the myocardium. Different theories have been postulated for myocardial stunning in patients with pheochromocytoma that include microvascular spasm, impaired fatty acid metabolism, increased production of oxygen-derived free radicals and dynamic left ventricular mid-cavity obstruction. QT interval prolongation is seen in 16% to 35% of patients with pheochromocytoma. Takotsubo cardiomyopathy (TS) is now being increasingly identified and it may be responsible for up to 40% of cases of acute catecholamine cardiomyopathy. These manifestations may sometimes precede or cloud the typical triad of a headache, sweating, and tachycardia. We herein present a case of a 42-year-old female with a unique combination of QT prolongation, torsades de pointes, and TS caused by pheochromocytoma in the background of NF1. All these complications are potentially reversible with the removal of the underlying adrenal tumor, underscoring the importance of a high suspicion for pheochromocytoma in patients with NF1. Cureus 2018-11-28 /pmc/articles/PMC6351109/ /pubmed/30723645 http://dx.doi.org/10.7759/cureus.3646 Text en Copyright © 2018, Butt et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Cardiology
Butt, Khurram
Ali, Saeed
Sattar, Zeeshan
Ur Rahman, Asad
Burt, Jeremy R
Funny Lumps, Flaming Pheo, and a Broken Heart: A Rare Case of Pheochromocytoma
title Funny Lumps, Flaming Pheo, and a Broken Heart: A Rare Case of Pheochromocytoma
title_full Funny Lumps, Flaming Pheo, and a Broken Heart: A Rare Case of Pheochromocytoma
title_fullStr Funny Lumps, Flaming Pheo, and a Broken Heart: A Rare Case of Pheochromocytoma
title_full_unstemmed Funny Lumps, Flaming Pheo, and a Broken Heart: A Rare Case of Pheochromocytoma
title_short Funny Lumps, Flaming Pheo, and a Broken Heart: A Rare Case of Pheochromocytoma
title_sort funny lumps, flaming pheo, and a broken heart: a rare case of pheochromocytoma
topic Cardiology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351109/
https://www.ncbi.nlm.nih.gov/pubmed/30723645
http://dx.doi.org/10.7759/cureus.3646
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