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J Thorac Cardiovasc Surg 2006;131:913-914
© 2006 The American Association for Thoracic Surgery
Brief Communication |
a Department of Cardiovascular Surgery, European Hospital Georges Pompidou, Paris, France
b Department of Anaesthesiology and Intensive Care, "Maison Blanche" Hospital, Reims, France.
c Department of Cardiology, "Maison Blanche" Hospital, Reims, France.
Received for publication October 27, 2005; revisions received November 25, 2005; accepted for publication December 1, 2005. * Address for reprints: Jean Michel Grinda, MD, Department of Cardiovascular Surgery, Hôpital Européen Georges Pompidou, 20 rue Leblanc 75015, Paris, France. (Email: jean-michel.grinda{at}hop.egp.ap-hop-paris.fr).
Acute adrenergic cardiomyopathy resulting in intractable pulmonary edema and life-threatening cardiogenic shock is an unusual revelation of a pheochromocytoma.
1-4
Dealing with such a patient, we have performed a bridge-to-bridge (extracorporeal life support [ECLS] secondarily switched for a DeBakey axial pump ventricular assist device) associated to a right surrenalectomy, allowing cardiac function recovery.
Clinical Summary
A 49-year-old man with no previously known pathology or risk factors complained of brutal abdominal pain, vomiting, headache, and malaise. On arrival to his referring hospital, he presented with a massive pulmonary edema, followed by cardiogenic shock and cardiac arrest requiring cardiopulmonary resuscitation. Initial laboratory findings showed troponin I and pro-brain natriuretic peptid levels at 0.23 µg/L and 5667 pg/mL, respectively (normal, <0.06 µg/L and <84 pg/mL, respectively). Arterial blood gases revealed a partial oxygen pressure of 218 mm Hg at a fraction of inspired oxygen of 1. Electrocardiography did not identify signs of ischemia or infarction, and chest radiography showed bilateral infiltration consistent with pulmonary edema. An echocardiogram revealed a severe global hypokinesia (left ventricular ejection fraction, 12%) without ventricular dilatation, no valvulopathy, no pulmonary artery hypertension, and a nonincreased septal thickness. Hemodynamic and respiratory status worsened despite mechanical ventilation, volume expansion, and an intake of dobutamine (8 µg) and norepinephrine (2 µg). The results of coronorography were normal, and during the procedure, an intra-aortic balloon pump was introduced. A thoracoabdominal computed tomographic (CT) scan revealed a 3 x 3-cm mass in the right adrenal gland. The diagnosis was orientated toward pheochromocytoma, and initial dosages of catecholamines were provided.
We were approached as the patient's condition got worse with signs of multiorgan failure. We then decided to implant an ECLS device (Medos Delta stream) by means of peripheral cannulation (common femoral artery and vein) in the referring hospital's emergency department (located 200 km from our department).
Medical transport, although risky, then ensued safely by road to our institution. ECLS provided an adequate flow (4 L/min), allowing norepinephrine and dobutamine disruption on day 2. The dosage of catecholamines was consistent with the presumed diagnosis of pheochromocytoma: both epinephrine and norepinephrine levels were high, at 12.5 nmol/L and 3.5 nmol/L (normal, <4 nmol/L), respectively, and the urinary vanyl mandelic acid value was 81.23 mg/24 hours (normal, <7 mg/24 hours).
At day 4 on ECLS, the patient presented with an intractable hypertension, despite triple therapy (calcium-channel blocker, 
-ß–blocker, and vasodilator), which was soon followed by a massive pulmonary edema. We decided to carry on with the surgical treatment of pheochromocytoma and the placement of a left ventricular assist device (DeBakey axial pump) as a bridge to recovery.
5
ECLS was used as extracorporeal circulation to achieve the surgical procedure, including sternotomy, right subcostal laparotomy, right surrenalectomy, and DeBakey axial pump ventricular assist device (Micromed) implantation. Histologic examination of the adrenal gland confirmed the diagnosis of pheochromocytoma, and cardiac biopsy specimens showed slight nonspecific changes (hypertrophy and fibrosis). Blood and urinary samples for the dosage of catecholamines were obtained on postoperative days 10 and 30. Norepinephrine was at 10.9 and 4.8 nmol/L, and epinephrine was at 0.54 nmol/L and nondetectable, respectively. Urinary metanephrine was 3.8 µmol/24 hours (normal, <3.7 µmol/24 hours) and 1.3 µmol/24 hours, respectively. Progressive cardiac function recovery was confirmed by means of echocardiography and an isotopic scintigraphy, allowing DeBakey ventricular assist device explantation on day 70. The postoperative course was uneventful, and the patient was discharged from the hospital on day 90. At 12 months' follow-up, he has recovered a normal cardiac function (left ventricular ejection fraction, 60%). The thoracoabdominal control CT scan shows no abnormality.
Discussion
Unusual presentations of pheochromocytoma have been reported, including dilated cardiomyopathy, pulmonary edema, cardiogenic shock, sudden death, and even 2 cases of late diagnosis realized after cardiac transplantation for dilated cardiomyopathy.
4
The diagnosis is difficult and frequently made postmortem. To our knowledge, this case is the first requiring mechanical circulatory support ever published. For this patient, the diagnosis was rapidly but fortuitously established on the basis of a systematic CT scan and later confirmed by dosages of catecholamines. The institution of an early aggressive treatment was life saving.
Compared with the scheduled device implantation in patients deteriorating on the waiting list, management of emergencies requiring circulatory support is still a challenging situation. We have developed in our unit a mobile kit of emergency circulatory support to deal with this problem. This ECLS has become a useful adjunct to our cardiac assist device program, allowing patient transfer and secondary implantation of a more long-term sophisticated device.
As expected in this catecholamine-induced cardiomyopathy, the patient recovered after surgical resection of the tumor, but recovery is not so fast as to allow the use of an ECLS device alone, and implantation of a long-term device was mandatory.
Conclusion
Diagnosis and treatment of cardiogenic shock caused by pheochromocytoma is difficult and can end in fatal evolution. This case emphasizes the need for an early aggressive treatment to improve the outcome of this surgically curable pathology.
References
This article has been cited by other articles:
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  J. D. Newton, S. Munir, R. Bhindi, and O. Ormerod What a Headache: Rare Neuroendocrine Indication for Cardiopulmonary Bypass for Severe Left Ventricular Dysfunction and Shock Circ Heart Fail, July 1, 2008; 1(2): 143 - 145. [Full Text] [PDF]
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