A moribund athlete.

In December, 2011, a 39-year-old woman presented to the local emergency department complaining of a 1-day history of vomiting and abdominal pain. She had had three or four episodes, with no infective prodrome. She was a physiotherapist and a competent long-distance runner; 2 days earlier she had run a 5 km race, and that morning had done her daily exercise workout. On examination there were no physical signs other than sinus tachycardia. Within 2 h, she collapsed and was moribund in acute cardiogenic shock. She was trans ferred to intensive care for mechanical ventilation and escalating inotropic support. Chest radiography showed pulmonary congestion. Trans thoracic echocardiography excluded valvular and con genital abnormalities and showed a slightly dilated, poorly functioning heart with no pericardial eff usion. Cardiac catheterisation was done to exclude coronary artery disease, and an intra-aortic balloon pump was placed for circulatory support. She had no arrhythmias. She was not pregnant and took no prescribed or recreational drugs; all infection screens were negative. She continued to deterior ate and needed two periods of chest compressions for loss of cardiac output. Acute myocarditis was suspected and she was transferred to our cardiothoracic centre for consideration of escalation of mechanical circu latory support. A consultant cardiologist and an intensivist accompanied her for the 70-mile journey, during which boluses of epinephrine were needed to maintain her circulation. She arrived 8 h after fi rst presentation, was found to be in extremis, with fi xed mottling of her trunk and limbs and hypotension despite epinephrine infusing at >1 μg/kg per min. She was anuric, profoundly acidaemic, and hypoxic on 100% oxygen (PaO2 6·1 kPa, pH 6·9, lactate 16 mmol/L, base excess –13). She was taken directly to theatre for median sternotomy. The heart was tense and dilated and a myocardial biopsy sample was taken from the left ventricle. Central veno-arterial extracorporeal membrane oxygenation (ECMO) was established; a cannula was inserted via the abdominal wall into the right atrium to drain systemic venous blood into the ECMO pump, which returned oxygenated blood via a separate cannula into the ascending aorta (fi gure). This procedure restored systemic cardiac output, and the sternum was closed. Her condition rapidly improved and the inotropes were stopped. During the next 24 h, signifi cant hypertension developed. The biopsy sample showed no evidence of myocarditis but did suggest catecholamine-induced myocyte damage. Continuing resistant hypertension raised the possibility of a catecholamine-secreting tumour. CT of the abdomen showed a large left adrenal mass (5×6 cm). Left adrenal ectomy was done (day 3) with concurrent ECMO support. Subsequent echo cardiography showed recovering biven tricular function (day 5) and ECMO was discontinued. She recovered fully, and was discharged in February, 2012. Histology confi rmed a benign phaeochromocytoma. Phaeochromocytomas are rare catecholamine-secreting tumours of the chromaffi n cells of the adrenal gland. They have diverse presentations including vomiting and abdominal pain, hypertension, as well as sweating, headache, and palpitations, which are typically episodic. Most cases are insidious, and although catecholamine-induced cardiomyopathy is well recognised, such a fulminant presen tation is rare. In our patient’s case, timely referral, and escalation of mechanical circulatory support were essential for resuscitation and diagnosis. Without special ised anaesthetic work-up before the adren alectomy, ECMO enabled control of the circulation during this operation. There were several support options for our patient but central ECMO could be established exped itiously and allowed examination and biopsy of the heart. Physicians should consider central ECMO in such cases as the mech an ical circulatory support of choice for “bridge to diagnosis”.