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Submitted: 06 May 2016
Revision: 04 Sep 2016
Accepted: 28 Sep 2016
ePublished: 30 Sep 2016
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J Cardiovasc Thorac Res. 2016;8(3): 132-136.
doi: 10.15171/jcvtr.2016.27
PMID: 27777699
PMCID: PMC5075362
  Abstract View: 1852
  PDF Download: 938

Case Report

Tetralogy of Fallot and pheochromocytoma in a situs inversus totalis: An unusual association

Rubén Kevin Arnold Tapia Orihuela 1,2*, Jorge Huaringa Marcelo 1,3, David Loja Oropeza 3,4

1 Universidad Nacional Mayor de San Marcos, Facultad de Medicina de San Fernando, Lima, Perú
2 Sociedad Científica de San Fernando, Lima, Perú
3 Hospital Nacional Arzobispo Loayza, Lima, Perú
4 Universidad Nacional Federico Villarreal, Facultad de Medicina, Lima, Perú
*Corresponding Author: Email: rubentsv@hotmail.com

Abstract

Introduction: Situs inversus totalis is an uncommon anomaly which exist a complete transposition of organs and it’s occasionally associated with congenital heart diseases, such as tetralogy of fallot. Pheochromocytoma is a rare neuroendocrine tumor with an annual incidence of 2-8 cases per million people and for years has been studied its relationship with the hypoxic pathway.
Case Report: A 29 year old male with a history of tetralogy of fallot corrected at 10 years and situs inversus totalis. He was admitted to hospital with a progressive story of four months of constipation, palpitations, headache, dyspnea and sweating. Physical examination revealed a thinned man with peripheral cyanosis, clubbing and signs of decompensated congestive heart failure as hepatomegaly, legs edema, multifocal systodiastolic murmurs, abdominal distension and jugular venous distention. The echocardiogram shows severe right ventricular dysfunction and severe pulmonary hypertension. Furthermore, abdominal computed tomography shows right adrenal mass. Elevated metanephrines and catecholamines confirmed the diagnosis of pheochromocytoma. Surgical removal is decided and preoperative management begins with alpha-adrenergic blockade, however the patient had a hemodynamic decompensation with an unfavorable evolution.
Discussion: In conclusion, there are few reports of cyanotic congenital heart disease with pheochromocytoma. Several studies show a significant association between both of them due to chronic hypoxia leads sustained hyperresponsiveness in adrenal medulla and it would cause the tumor. Special preoperative management of pheochromocytoma is recommended when there underlying heart disease and congestive heart failure. We present the first international report of tetralogy of fallot and pheochromocytoma in a patient with situs inversus totalis.
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