A 63-Year-Old Man With Palpitations, Dizziness, and Severe LV Dysfunction

A 63-year-old male was transferred to our institution with the chief complaint of palpitations and dizziness for several weeks. His extensive medical history included insulin-dependent diabetes mellitus, hypertension, severe peripheral arterial disease status post right below-knee amputation, and prior ischemic cerebral vascular accident with residual hemiparesis. Preceding the below-knee amputation, which was 2 months prior to current hospitalization, he underwent ischemic evaluation via a pharmacological nuclear (thallium) single-photon emission computed tomography (SPECT) with rest and stress imaging interpreted as transmural apical myocardial infarction (MI) with a small component of inducible ischemia involving the septum. The ejection fraction (EF) was calculated at 32%. A cardiac catheterization was not performed. The patient was assumed to have an ischemic cardiomyopathy by the referring physicians, and guideline-directed medical therapy was initiated.

Upon admission at our institution, he denied angina or recent heart failure symptoms. He underwent transthoracic echocardiography that revealed an EF of 25-30% with severe global left ventricular (LV) hypokinesis and, most significantly, anteroseptal and apical akinesis. An electrocardiogram (ECG) showed sinus rhythm with evidence of previous anterior MI (Figure 1). Troponin I was 0.038 (99th percentile upper limit of normal 0.045) with a creatine of 1.06 mg/dl and a glomerular filtration rate >60 mL/min/1.73m2. Given his severe LV dysfunction, electrophysiology consultation was obtained for consideration of an implantable cardioverter defibrillator (ICD). Cardiac catheterization was requested to solidify an etiology of ischemic cardiomyopathy, and the patient underwent diagnostic coronary angiography (Figure 2 and Video 1).

Figure 1. Baseline 12-lead ECG

Figure 1

Figure 2. Serial angiographic images illustrating (left) the typical "nest" of tortuous vasculature associated with coronary to pulmonary artery fistulae (white arrow), (middle) the course of the left anterior descending (LAD) artery (white outline) with the diagonal marked (white arrow), and (right) right anterior oblique (RAO) caudal view providing additional fistula visualization.

Figure 2

Video 1. RAO cranial angiography revealing delayed angiographic filling of the distal LAD, likely a result of both multiple fistulous vessels and some degree of concomitant coronary artery disease. Note the absence of the typical dilated feeder vessels or distal connection to the pulmonary artery as a result of spontaneous closure.

Given this patient's presenting symptoms and angiographic findings, was would the most likely diagnosis and next step in clinical management include?

Show Answer