Therapeutic Hypothermia for Cardiac Arrest in a Patient With Scleroderma and Raynaud's Phenomenon

Abstract

INTRODUCTION: Therapeutic hypothermia in patients with cardiopulmonary arrest has been shown to favorably impact neurologic outcomes. Its safety profile and the outcomes in patients with systemic sclerosis and RaynaudaTMs phenomenon (RP) have not been described. CASE PRESENTATION: A 52 year-old male with scleroderma and RP, coronary artery disease, and recent active cocaine use was found in cardiopulmonary arrest due to ventricular fibrillation. Cardiopulmonary resuscitation was administered with return of spontaneous circulation within 20 minutes. Post-arrest he was moving all extremities, but was responsive only to noxious stimuli. Chronic superficial digital ulcers were present on all fingertips. Labs revealed a WBC of 18 and troponin of 2.29. ECG and transthoracic echocardiogram were without evidence of acute ischemia. Therapeutic hypothermia was induced via surface cooling with a temperature of approximately 33 degrees celsius followed by gradual rewarming. During the cooling phase, non-blanching erythema developed on the dorsum of the feet, ankles, wrists and tibial surfaces. Dusky necrosis developed on the left fourth digit seventy-two hours later. Nifedipine was uptitrated; a warming blanket was applied. He regained full neurologic function. Cardiac catheterization revealed non-obstructive coronary artery disease and patent stents. An AICD was implanted. Skin findings, except for the fingertip necrosis, had substantially improved by hospital discharge to his home. His neurologic function at discharge was normal. DISCUSSION: Patients with systemic sclerosis and RP seem to be at high risk for ischemic complications related to therapeutic hypothermia. In scleroderma, digital ulcers with tissue necrosis occur from underlying vasculopathy exacerbated by RP. Sympathetic nervous system hyperactivity increases the vasoconstrictor response to cold in RP. As a potentially more serious complication, cold-induced myocardial ischemia (myocardial RP) has also been described in patients with systemic sclerosis. On the other hand, therapeutic hypothermia decreases myocardial injury following cardiac arrest if provided early. Overall, therapeutic hypothermia did not induce unacceptable complications in our patient with systemic sclerosis and RP. A reversible purpuric rash and digital ischemia with necrosis of the tip of one finger occurred, but no adverse cardiac events related to hypothermia were observed. CONCLUSIONS: To our knowledge, this is the first reported case of a patient with scleroderma and RP who underwent therapeutic hypothermia for witnessed cardiac arrest with successful neurologic recovery. The benefits of therapeutic hypothermia outweighed the risks of cardiac and acral ischemia.