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

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.

Pheochromocytoma and paraganglioma in Fontan patients: Common more than expected

“…PHEO or PGL is a catecholamine‐producing tumor that arises from sympathetic lineage–derived cells of the adrenal medulla or the extra‐adrenal paraganglia . Cyanotic congenital heart disease (CHD) was speculated to be associated with PHEO/PGL because of case reports of PHEO/PGL that developed in relatively young patients with cyanotic CHD . Opotowsky et al recently reported that patients with cyanotic CHD had increased odds ratio (6.0; confidence interval, 2.6–13.7; P < 0.0001) for PHEO/PGL development compared with those without cyanotic CHD in a multicenter case series …”

Adrenal mass in a patient with tetralogy of Fallot: beyond expected

“…Secondly, significant pulmonary regurgitation is almost always encountered following a transannular patch repair and although it is usually well tolerated for years it finally leads to symptomatic right ventricular dilation and dysfunction moreover if it coexists with significant tricuspid regurgitation. Both valvular pathologies are suspected, although pulmonary regurgitation is not mentioned, in the patient reported by Tapia-Orihuela et al 1 according to their echocardiographic and auscultatory data. When valvular regurgitations are severe, pulmonary valve replacement and tricuspid annuloplasty should be performed before irreversible right ventricle dysfunction ensues.…”

“…We have read with interest the article published by Tapia-Orihuela et al 1 However, in the wake of that reading, we would like to make a few comments that we believe to be important.…”