Anesthetic Management of Pheochromocytoma Resection in Adults with Single Ventricle Physiology

Suffredini, Giancarlo; Diaz-Rodriguez, Natialia; Chakravarthy, Krishnan; Mathur, Aarti; Hayanga, Heather K.; Frank, Steve; Ringel, Richard; Freiberg, Stephen; Barodka, Viachaslau; Steppan, Jochen

doi:10.7759/cureus.1928

Cited by 3 publications

(7 citation statements)

References 10 publications

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“…In such cases, PVR and intravascular volume must be controlled to maintain pulmonary circulation because Fontan physiology is preload dependent and requires low PVR [8,13]. In particular, CVP and PVR may be the most important parameters in the perioperative management of Fontan circulation [8,[13][14][15]. We managed intraoperative CVP at preoperative levels under fluid infusion as previous case reports [13,15] and utilizing NO to decrease PVR in all cases.…”

Section: Discussionmentioning

confidence: 95%

“…In a Fontan circulation, the pulmonary blood flow is passively determined by a combination of CVP, left atrial pressure, systemic ventricular end-diastolic pressure, and PVR because of the lack of a responsible ventricle for determining pulmonary circulation. In such cases, PVR and intravascular volume must be controlled to maintain pulmonary circulation because Fontan physiology is preload dependent and requires low PVR [8,13]. In particular, CVP and PVR may be the most important parameters in the perioperative management of Fontan circulation [8,[13][14][15].…”

Section: Discussionmentioning

confidence: 99%

“…Chronic hypoxia exposure followed by aberrant activation of common hypoxia cellular pathways and hypoxia-inducible factors in chromaffin cells appear to mechanism for the pathogenesis of P-PGL [1]. Although several case reports of P-PGL with Fontan circulation have been reported, the information on its perioperative anesthetic management is sparse (online only Supplement) [3][4][5][6][7][8][9][10][11][12][13].…”

Section: Introductionmentioning

confidence: 99%

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Anesthetic management of pheochromocytoma and paraganglioma for patients with Fontan circulation: a case series

et al. 2023

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Background Anesthetic management of pheochromocytoma and paraganglioma with Fontan circulation is challenging for physicians, with attention to cardiovascular physiology. Case presentation We performed anesthetic management for pheochromocytoma and paraganglioma in three patients with Fontan circulation. We maintained intraoperative central venous pressure at preoperative level under fluid infusion and administrating nitric oxide to decrease pulmonary arterial resistance. We administered noradrenaline or vasopressin if low blood pressure was present despite adequate central venous pressure. Although noradrenaline is prevalent for the case of noradrenaline-secreting tumor especially after resection, we could maintain blood pressure to administrate vasopressin without increasing central venous pressure. Retroperitoneal laparoscopic approach which could avoid intra-abdominal adhesions might be selectable as case 3. Conclusions Sophisticated management is required for pheochromocytoma and paraganglioma with Fontan circulation.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Anesthetic management of pheochromocytoma and paraganglioma for patients with Fontan circulation: a case series

et al. 2023

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“…Initial treatment is with an alpha-blocker together with management by a cardiologist specializing in CHD. There are also specific anaesthetic management techniques for resection of pheochromocytomas in adulthood, which is usually carried out at a specialized centre [6, 7] .…”

Section: Discussionmentioning

confidence: 99%

Not So Incidental: Pheochromocytoma in an Adult with Unrepaired Cyanotic Heart Disease

Adunuri

Mrkobrada

2018

European Journal of Case Reports in Internal Medicine

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Pheochromocytoma, a rare neuroendocrine tumour, is often encountered in the general internal medicine clinic as an adrenal incidentaloma. Even rarer is its presence in adult cyanotic heart disease, although there are a few documented reports of this association in various paediatric populations, with chronic hypoxia being the likely driving force. Here we present the case of a 38-year-old adult with unrepaired complex cyanotic congenital heart disease with biochemically proven pheochromocytoma presenting as an adrenal incidentaloma to a general internal medicine clinic.LEARNING POINTSThere is an association between pheochromocytoma and congenital cyanotic heart disease.It is important to maintain clinical vigilance for laboratory testing for pheochromocytoma in patients with cyanotic heart disease who are seen at general internal medicine clinics.

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“…на 100 000 новорожденных, 2 на 100 000 взрослых [7]. Еще более редко его сочетание с феохромоцитомой: в литературе описано всего 8 таких случаев [8][9][10][11][12][13][14].…”

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Sustained ventricular tachycardia in a patient with a single ventricle of the heart and a pheochromocytoma

Melkozerov

Кузнецов

Kalashnikov

et al. 2019

Probl Endokrinol (Mosk)

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A pheochromocytoma is a rare tumor that develops from adrenomedullary chromaffin cells and produce ones or more catecholamines, including adrenaline, norepinephrine, and dopamine. On rare occasions a pheochromocytoma is hormonally inactive. Cyanotic heart disease is also a relatively rare pathology. One of its least frequently occurring variants is the single ventricle of the heart. Presumably, in patients with cyanotic heart defects, the occurrence of pheochromocytes and paragangliomas will be higher due to the presence of certain germinative and somatic mutations. In cyanotic heart defects, the development of malignant arrythmias is one of the frequent causes of death. A combination of a pheochromocytoma with a single ventricle of the heart is extremely rare: only eight such cases have been described in the literature. This article describes a young patient with a unique case of a single ventricle of the heart, pheochromocytoma and sustained ventricular tachycardia. The cause of the ventricular tachycardia, in all likelihood, was inappropriate medical care in this case, a prescription for verapamil. The surgical excision of the pheochromocytoma and the referral of the patient for cardiac surgery became possible only after correcting the antihypertensive and antiarrhythmic therapy. Verapamil was replaced with a combination of doxazosin and amiodarone, resulting in relatively satisfactory blood pressure readings and sinus rhythm.

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Anesthetic Management of Pheochromocytoma Resection in Adults with Single Ventricle Physiology

Cited by 3 publications

References 10 publications

Anesthetic management of pheochromocytoma and paraganglioma for patients with Fontan circulation: a case series

Anesthetic management of pheochromocytoma and paraganglioma for patients with Fontan circulation: a case series

Not So Incidental: Pheochromocytoma in an Adult with Unrepaired Cyanotic Heart Disease

Sustained ventricular tachycardia in a patient with a single ventricle of the heart and a pheochromocytoma

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