The Perioperative Management of a Patient with Complex Single Ventricle Physiology and Pheochromocytoma

Abstract

Pheochromocytoma is associated with intense physiologic effects of alpha- and beta-adrenergic stimulation from catecholamine secretion. Perioperative management for these patients includes alpha-adrenergic receptor blockade, intravascular volume replacement, and, if necessary, beta-adrenergic receptor blockade. Significant perioperative changes in preload and afterload, fluid status, heart rate and rhythm, and inotropy can occur and may be contrary to anesthetic management goals for patients with certain conditions of congenital heart disease. We report the perioperative management with doxazosin of a patient with single ventricle physiology and cavo-pulmonary and aorto-pulmonary lung perfusion who presented for resection of a pheochromocytoma.

Anesthesia for pheochromocytoma resection in a child with Fontan circulation

“…Although catecholamine release can have dramatic consequences in patients with Fontan physiology, the anesthetic considerations during pheochromocytoma resection are the same as in other patients. [2][3][4][5][6][7][8][9][10] The general management in our patient followed the principles discussed in previous reports, but a few aspects warrant special attention.…”

“…[5][6][7][8] Tjeuw et al reported a case of an 11-yr-old girl with a single ventricle whose symptoms had been palliated with a Blalock-Taussig shunt. 5 Subsequent reports included a case of a 27-yr-old woman with a right-sided Glenn anastomosis and a central aortopulmonary shunt, 6 a 24-yr-old man with a modified Fontan repair, 7 and a 13-yr-old boy whose symptoms were palliated with a Glenn anastomosis.…”

“…13 Doxazosin was used in the two adult patients who were previously reported with single ventricle physiology and pheochromocytomas. 6,7 It might be argued that the postoperative course could have been problematic even if doxazosin had been used; one of the adult patients treated with doxazosin needed prolonged treatment with vasoconstrictors after surgery and was intubated for a week because of arrhythmias and fluid accumulation; 7 however, a comparison between phenoxybenzamine and doxazosin does favour the latter. Prys-Roberts and Farndon noted no difference between the drugs in terms of the efficacy of intraoperative control of BP and HR, but to maintain BP postoperatively, patients treated with doxazosin needed less fluid resuscitation and lower doses of phenylephrine compared to those treated with phenoxybenzamine.…”

Perioperative Management of Severe Hypertension during Laparoscopic Surgery for Pheochromocytoma

“…Anestezi indüksiyonu, orofaringeal entübasyon ve perioperatif dönemde katekolamin salgılanmasıyla oluşabilecek hipertansiyon yönetimi için; Nikardipine (kalsiyum kanal blokeri), esmolol (β bloker), fentolamine, sodyum nitroprussid, fenoldopam, remifentanil, magnezyum sülfat ve nadiren de adenozin veya prostoglandin E1 gibi kısa etkili ajanlar tercih edilmektedir (4,7,8). Remifentanil adrenokortikal tümör rezeksiyonu yapılan hastada entübasyona ve cerrahi insizyona stres yanıtı önleyebilmesine ve hemodinamik stabiliteyi sağlayabilmesine rağmen, anestezi indüksiyonu sonrasında belirgin kardiyovasküler depresyonla ilgilidir ve feokromasitomalı hastalarda tümör manipülasyonu sırasında katekolamin seviyelerinde artış ile ilişkili hemodinamik değişiklikleri önlemede yetersizdir (8).…”

A Case of Malignant Pheochromocytoma with Holt-Oram Syndrome

“…The associations between TBX5, SALL4, and SDH mutations have not been described. However, pheochromocytoma has been reported in patients with various other cyanotic heart diseases such as Fallot complex, single ventricle physiology, and tricuspid atresia [17,18]. Furthermore, a significant correlation between chronic hypoxia and peripheral neuroblastic tumors, including pheochromocytoma, has also been reported [19][20][21][22].…”