Alpha-blockade during congenital heart surgery admissions: analysis from national database

Introduction: The effects of alpha-blockade on haemodynamics during and following congenital heart surgery are well documented, but data on patient outcomes, mortality, and hospital charges are limited. The purpose of this study was to characterise the use of alpha-blockade during congenital heart surgery admissions and to determine its association with common clinical outcomes. Materials and Methods: A cross-sectional study was conducted using the Pediatric Health Information System database. De-identified data for patients under 18 years of age with a cardiac diagnosis who underwent congenital heart surgery were obtained from 2004 to 2015. Patients were subdivided on the basis of receiving alpha-blockade with either phenoxybenzamine or phentolamine during admission or not. Continuous and categorical variables were analysed using Mann−Whitney U-tests and Fisher exact tests, respectively. Characteristics between subgroups were compared using univariate analysis. Regression analyses were conducted to determine the impact of alpha-blockade on ICU length of stay, hospital length of stay, billed charges, and mortality. Results: Of the 81,313 admissions, 4309 (5.3%) utilised alpha-blockade. Phentolamine was utilised in 4290 admissions. In univariate analysis, ICU length of stay, total length of stay, inpatient mortality, and billed charges were all significantly higher in the alpha-blockade admissions. However, regression analyses demonstrated that other factors were behind these increased. Alpha-blockade was significantly, independently associated with a 1.5 days reduction in ICU length of stay (p < 0.01) and a 3.5 days reduction in total length of stay (p < 0.01). Alpha-blockade was significantly, independently associated with a reduction in mortality (odds ratio 0.8, 95% confidence interval 0.7−0.9). Alpha-blockade was not independently associated with any significant change in billed charges. Conclusions: Alpha-blockade is used in a subset of paediatric cardiac surgeries and is independently associated with significant reductions in ICU length of stay, hospital length of stay, and mortality without significantly altering billed charges.

Perioperative Management of Pheochromocytoma: From a Dogmatic to a Tailored Approach

Background: Perioperative management of pheochromocytoma (PCC) remains under debate. Methods: A bicentric retrospective study was conducted, including all patients who underwent laparoscopic adrenalectomy for PCC from 2000 to 2017. Patients were divided into two groups: Group 1 treated with alpha-blockade, and Group 2, without alfa-blockers. The primary end point was the major complication rate. The secondary end points were: the need for advanced intra-operative hemostasis, the admission to the intensive care unit (ICU), the length of stay (LOS), systolic (SBP), and diastolic blood pressure (DBP). Univariate and multivariate analysis was conducted. A p-value < 0.05 was considered statistically significant. Results: Major postoperative complications were similar (p = 0.49). Advanced hemostatic agents were 44.9% in Group 1 and 100% in Group 2 (p < 0.001). In Group 2, no patients were admitted to the ICU, while only 73.5% of Group 1 (p < 0.001) were admitted. The median length of stay was larger in Group 1 than in Group 2 (p = 0.026). At the induction, SBP was 130 mmHg in Group 1, and 115 mmHg (p < 0.001). The pre-surgery treatment was the only almost statistically significant variable at the multivariate analysis of DBP at the end of surgery. Conclusion: The preoperative use of alfa-blockers should be considered not a dogma in PCC.

Perioperative control of paroxysmal hypertension using esmolol with alpha-blockade in a child with a germline mutated paraganglioma

Summary The use of antihypertensive medications in patients with pheochromocytomas and paragangliomas (PCC/PG) is usually a challenge. We report a case of familial paraganglioma that was successfully treated by esmolol and other antihypertensive medications without associated perioperative complications. Our patient was an 11-year-old girl who presented with classic symptoms and signs of PCC/PG and a CT scan of the abdomen that showed a right-sided paravertebral mass. Her father was diagnosed with paraganglioma a few years ago. Prazosin had been started but she continued to experience uncontrolled paroxysms of blood pressure (BP). She was known to have asthma; hence, she developed serious bronchospasm with atenolol. She was, therefore, switched to esmolol that successfully controlled her BP in addition to prazosin and intermittent doses of hydralazine prior to laparoscopic surgery with no side effects of medications or postoperative complications. Esmolol could be a good alternative to routinely used beta-blockers in children with PCC/PG with labile hypertension and related symptoms in the pre and intra-operative periods. It is titrable, effective, and can be weaned rapidly helping to avoid postoperative complications. Further larger studies on the use of esmolol in children with PCC/PG are needed to confirm our observation. Learning points In addition to alpha-blockers, esmolol could be a good alternative for routinely used beta-blockers to control paroxysmal hypertension and tachycardia in the pre- and intra-operative periods. Esmolol is titrable and an effective beta-blocker. It can be weaned rapidly helping to avoid postoperative complications in children with PCC/PG. Children with PCC/PG and other comorbidity like asthma may particularly benefit from the use of esmolol due to no or less side effects on airway resistance and the advantage of rapid titration of the medication compared to other beta-blockers.

Pheochromocytoma Masquerading as Weight Loss - a Diagnostic and Management Challenge

Introduction: Classic pheochromocytoma symptoms include headache, diaphoresis, palpitations and symptomatic pheochromocytomas are on average around 5cm. They respond to preoperative alpha blockade followed by surgery. We present a case of a giant 15.9cm pheochromocytoma with delayed presentation and diagnosis complicated by atypical symptoms of nausea, weight loss; preoperative preparation included a modified preparation of high-dose phenoxybenzamine but she underwent successful surgery. Case Description: A 62-year-old female with a history of hypertension and diabetes presented with nausea and 80 lbs weight loss over 18 months. CT scan revealed large centrally necrotic mass overlying the left kidney, measuring 15.9 x 12.7 x 14.7cm. Abdominal MRI showed claw sign; she was admitted to the urology service for tube feeding prior to resection of suspected renal cell carcinoma. Given the location of the mass and history of hypertension, plasma metanephrines, included in preoperative workup, were notable for: total metanephrines &gt;40000 pg/ml (reference range &lt;=205), normetanephrines &gt;20000 pg/ml (reference range &lt;=148), metanephrines 57 pg/ml (reference range &lt;=57). Endocrine was consulted and she was started on phenoxybenzamine; she underwent volume replacement with fluids and salt tablets. Due to nausea and vomiting she was unable to reliably tolerate oral medication; BP management was challenging. Oral pill dosing was minimized. Metoprolol was converted to metoprolol SA and a phenoxybenzamine suspension that could be delivered through the feeding tube with good absorption was made. On the day prior to surgery, BP was 117/76 mmHg, supine HR 92 bpm, on a total daily dose of phenoxybenzamine 240mg, metoprolol SA 200mg and amlodipine 10mg. She underwent a radical left nephrectomy and adrenalectomy. Plasma metanephrines six weeks later were normal: total metanephrines 84 pg/ml, normetanephrines 84 pg/ml, metanephrines &lt;25 pg/ml. Pathology showed benign pheochromocytoma. Conclusion: Giant pheochromocytomas present significant challenges for clinicians who must be aware of the potential for nonspecific symptoms which can delay diagnosis. Paroxysmal hypertension happens in only about 50% of pheochromocytoma patients; primary hypertension is often diagnosed, as had been the case with our patient. Additionally, initial imaging related to atypical presenting symptoms may not lead to the specific diagnosis, as in our patient; metanephrine screening revealed the correct diagnosis. Regarding management, preoperative alpha blockade dosage and delivery should be tailored to individual patient needs, with adjustment as needed and alternative preparations considered to optimally control BP and reduce risk of death and other perioperative complications. Our case highlights the diagnostic and management challenge of patients presenting with giant pheochromocytomas.

Glucocorticoids and Pheochromocytoma- a Recipe for Disaster?

Background: Pheochromocytomas are catecholamine-secreting tumors that arise from the chromaffin cells of the adrenal medulla. The catecholamine release manifests as episodic headaches, diaphoresis, and palpitations which can lead to hypertensive emergency known as a pheochromocytoma crisis(PC). This crisis can be precipitated by commonly used medications including corticosteroids, metoclopramide, and anesthetic agents like ketamine and halothane. Case report: We present a 60 year old Caucasian male with a past medical history significant for SVT on metoprolol and diltiazem and long-standing hypertension. He was admitted directly from the clinic to the ED after complaining of severe abdominal pain, multiple episodes of vomiting, and diaphoresis following prednisone intake for a presumed gout flare. He had a blood pressure of 180/100 and tachycardic to 120s on arrival. He was also found to be hypoxemic with a chest x-ray concerning for pulmonary edema along with evidence of a demand related type II NSTEMI. He underwent a CT abdomen pelvis with contrast which showed evidence of high grade distal ileum obstruction with no obstructing mass or inflammatory process. There was also 5-cm solid right adrenal mass which was initially thought to be an incidentaloma. Following stabilization, he underwent an exploratory laparotomy which did not show an obvious mechanical etiology of bowel obstruction. This raised concerns for a pheochromocytoma. Oral verapamil with doxazosin was started to provide appropriate alpha blockade. Subsequently, metoprolol was reintroduced for beta blockade. He was officially diagnosed with glucocorticoid-induced pheochromocytoma after his labs returned with elevated levels of serum metanephrines 43.7(0.00 - 0.49 nmol/L), serum normetanephrine 22.2 (0.00 - 0.89 nmol/L),), 24-hr urine epinephrine 1021 ug/g (0 - 20 ug/g), and 24-hr urine norepinephrine 838 ug/g (normal: 0 - 45 ug/g). Ultimately 3 weeks later he underwent successful R sided adrenalectomy and is doing well. Conclusion: This case demonstrates a rare but life-threatening adverse reaction associated with commonly used glucocorticoids in a patient with an undiagnosed pheochromocytoma. Pheochromocytomas are exceedingly rare and can go undiagnosed in up to 50 percent of patients (1). Failure or delays in diagnosis of PC can lead to increased morbidity and mortality. When managing a pheochromocytoma crisis, it is imperative to avoid selective beta-blockers, as it can worsen hypertension due to unopposed alpha agonism by the secreted catecholamines. This also demonstrates the importance of keeping a broad differential in order to avoid anchoring bias, particularly when there is conflicting clinical information. References: 1. Sutton MG, Sheps SG, Lie JT. Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series. Mayo Clin Proc. 1981 Jun;56(6):354–60. PMID: 6453259.

Pheochromocytoma Rupture Associated With Systemic Anticoagulation: A Case Report

Background: Pheochromocytoma rupture is a rare endocrine emergency. There have been approximately 85 documented cases, and the mortality rate for patients who require emergent surgery is as high as 34% (1). Clinical Case: A 30-year-old man with no past medical history presented to the Emergency Department with abdominal pain and vomiting. He was hypertensive, tachycardic, and hypoxic. CT Chest Angiogram revealed bilateral pulmonary emboli and a 6.5 x 5.5 x 6.4 cm left heterogenous adrenal mass. His plasma free metanephrines were elevated to 31,173 pg/mL (n&lt;62 pg/mL) with normetanephrines &gt;50,000 pg/mL (n&lt;145 pg/mL). His pulmonary emboli was treated with continuous intravenous heparin, and he was also treated with intravenous fluids and phentolamine due to concern for pheochromocytoma. Within hours, he became hypotensive, and his hemoglobin downtrended from 16.4 g/L to 7.3 g/L (n 14–18 g/L). Repeat CT showed that the adrenal mass had ruptured, enlarging to 15.4 x 9.7 x 10.0 cm. He was taken for an emergent laparotomy with piecemeal resection of the mass; surgical pathology confirmed a pheochromocytoma. Fortunately, the patient recovered well after surgery. After 6 months, his plasma free metanephrines normalized to 15 pg/mL with normetanephrines of 80 pg/mL. His DOTATATE scan was negative for malignancy and his genetic testing was negative. Conclusion: The patient’s systemic anticoagulation and critical illness likely increased his risk for pheochromocytoma rupture. Standard preoperative pheochromocytoma management, to include aggressive fluid resuscitation and alpha blockade, is important in reducing risk of morbidity and mortality. Biochemical follow up is required, as there is a 16% risk of recurrence. Reference: (1) Kobayashi T, Iwai A, Takahashi R, Ide Y, Nishizawa K, Mitsumori K. Spontaneous rupture of adrenal pheochromocytoma: Review and analysis of prognostic factors. Journal of Surgical Oncology. 2005;90(1).