Extensive triple vessel coronary artery disease in a young male with juvenile idiopathic arthritis
We present a case of a 27-year-old male with a 1-month history of neck and lower back pain associated with worsening dyspnea on exertion. He had no prior history of angina, syncope, palpitations, flu-like illness, vomiting or diarrhoea. In addition, he had no family history of premature CVD or traditional ASCVD risk factors. Specifically, he reported no history of cigarette smoking or any form of substance abuse.\nOn clinical examination, he had conjunctival pallor, grade three pedal oedema and abdominal ascites. Examination of the cardiovascular system revealed an elevated jugular venous pressure, a myopathic displaced apex beat with a right parasternal heave and an S3 gallop. He was in New York Heart Association functional class III. His respiratory examination was normal.\nThe musculoskeletal examination revealed loss of cervical lordosis, tender and swollen joint count of eight, fixed flexion deformity of the elbows, ankylosis of the wrists and the proximal interphalangeal joints of both hands. The clinical disease activity index score was 34, signifying a high rheumatologic disease activity.\nAll inflammatory markers were elevated (), and the rheumatologic serological studies were unremarkable (). The Human immunodeficiency virus ELISA screen was negative. A resting electrocardiogram (ECG) showed a narrow QRS complex sinus tachycardia (). Echocardiography demonstrated four-chamber enlargement with global hypokinesis of the left ventricle and a left ventricular ejection fraction of 20–25% (). The diagnostic coronary angiogram revealed extensive triple vessel disease (), with a syntax score I of 36.5. Furthermore, computed tomography angiography of the neck and brain revealed large vessel vasculitis of the common carotid and middle cerebral arteries. The vasculitis flare was subsequently treated with two cycles of cyclophosphamide at 13 mg/kg, administered 2 weeks apart.\nThe patients’ rheumatic symptoms were treated with pulsed solumedrol 1 g for 3 days, followed by prednisone at 0.5 mg/kg. As part of the heart failure therapy, the patient was acutely initiated on furosemide, low dose carvedilol, an angiotensin-converting enzyme inhibitor and spironolactone, to which he responded well. The patient was also treated with simvastatin, baclofen, chloroquine, methotrexate, folic acid and carbamazepine. The ECG did not show any features suggestive of chloroquine-induced QT prolongation (QTc > 450 ms) as both the QT and corrected QT interval were 308 and 429 ms, respectively.\nThe differential diagnoses were ischaemic cardiomyopathy secondary to accelerated atherosclerosis and a vasculopathy of the coronary arteries as a sequela of the juvenile idiopathic arthritis (JIA). A differential diagnosis of cyclophosphamide-induced cardiomyopathy was also entertained.\nThe institutional heart team decided to refer the patient for elective coronary artery bypass graft surgery once the inflammatory markers had settled. Unfortunately, 3 weeks later, the patient experienced a sudden cardiac arrest while at home. The likely cause of death was a lethal arrhythmia such as ventricular tachycardia. An intracardiac defibrillator would have been an ideal therapy to prevent his sudden cardiac death. However, in our local clinical setting, such devices are not readily available due to their high cost.