Cardiopulmonary Arrest Caused by Acute Myocardial Infarction with Multivessel Coronary Disease in a Young Adult with Von Recklinghausen's Disease

Abstract

The case involved a 38-year-old man. By 30-year-old, he had undergone surgical resection of schwannoma four times and had been diagnosed with von Recklinghausen’s disease (neurofibromatosis type I; NF1). At 35 years old, invasion of the thoracic vertebral bodies due to NF1 was recognized along with exertional chest and back pain. In November 2014, he collapsed suddenly while walking and was urgently transported to our emergency room after the return of spontaneous circulation. Forrester subset IV heart failure was recognized, and chronic total occlusion was observed in both the right coronary and left circumflex arteries, while the culprit lesion for acute coronary syndrome was in the proximal left anterior descending artery (LAD). During emergent percutaneous coronary intervention (PCI) to the LAD, revascularization was difficult due to slow flow caused by unexpected multiple thrombi that disappeared immediately after argatroban injection. Although we finished PCI with thrombolysis in myocardial infarction grade 3 flow in the LAD, we could not save the patient because of worsening traumatic brain hemorrhage and decreased left ventricular function. Autopsy showed no subacute thrombosis of the drug-eluting stent deployed in the LAD. Autopsy also revealed severe atherosclerosis of multiple vessels, including the coronary vessels, representing a rare finding in typical NF1 patients.