Case presentation

A 21-year-old man with a history of Diamond–Blackfan anaemia, myelodysplastic syndrome, bone marrow transplantation, and graft versus host disease presented to an outside emergency room with acute chest pain, electrocardiogram changes, and elevated cardiac enzymes. He had a history of a bone marrow transplant at 17 years of age secondary to myelodysplastic syndrome (chromosome 1:7 translocation). His pre-transplant cardiac work-up was normal. He underwent conditioning with total body irradiation and melphalan, followed by bone marrow transplantation with grafts from an unrelated donor (10/10 human leukocyte antigen-matched and ABO mismatched). He demonstrated engraftment on day +29, but required an additional CD34 stem cell transfusion on day +77 secondary to poor graft function. On day +67, he developed grade IV graft versus host disease of the skin with painful scleroderma requiring initial treatment with methylprednisolone, cyclosporine, and daclizumab infusions. He continued chronic treatment with azathioprine and photochemotherapy for three years.

On presentation to the emergency room, a 12-lead electrocardiogram demonstrated new-onset right-bundle branch block with ST-segment depression in leads V1 to V3. Cardiac enzymes were elevated as follows: troponin – I 2.19 mg/mL, normal <0.04; creatine kinase-MB – 20.5 ng/mL, normal 0–4.5; creatine phophokinase – 278 IU/L, normal 55–170. A limited transthoracic echocardiogram demonstrated severely depressed left ventricular systolic function with a left ventricular ejection fraction of 25%. Aspirin and metoprolol were given. Cardiac enzymes continued to rise, prompting transfer to a tertiary care institution.

On the evening after transfer, he developed haemodynamically stable complete heart block prompting discontinuance of metoprolol. Cardiac catheterisation with coronary angiography was deferred given the significant graft versus host disease in the inguinal area. Cardiac MRI was performed with a 1.5 T Siemens Avanto magnet. Cine cardiac MRI images revealed a wall motion abnormality in the inferior left ventricular wall and a left ventricular ejection fraction of 55% (Supplementary Video 1). Wall motion abnormalities were also observed in the diaphragmatic and basal free wall of the right ventricle. Late gadolinium delayed enhancement imaging showed a transmural infarct in the inferior left ventricular wall extending from the base to the apex and in the right ventricular basal and free wall consistent with infarction involving the right coronary artery distribution (Fig 1, Supplementary Video 2). Subsequent CT angiogram of the chest with coronary angiography showed complete occlusion of the proximal right (Fig 2) and 50% occlusion of the left anterior descending coronary arteries. Luminal irregularity was present to a less extent throughout the remainder of the coronary arteries, consistent with coronary vasculitis. Given the transmurality of the infarction and lack of viable myocardium, the patient was not a candidate for reperfusion interventions.

Figure 1 A late delayed gadolinium enhancement image obtained using gradient echo imaging. This short-axis image at the mid-ventricular level illustrates hyperenhancement in the right ventricular inferior and free walls right ventricular inferior and free wall. There is also hyperenhancement of the left ventricular inferoseptal, inferior and inferolateral walls. There is an area of no re-flow in the centre of the infarction.

Figure 2 An image of a CT angiogram of the chest with coronary angiography that illustrates complete occlusion of the proximal right coronary artery.

Medical treatment with captopril, simvastatin, furosemide, and metoprolol – following spontaneous conversion to sinus rhythm – was initiated. At three weeks after admission he developed a large posterior pericardial effusion with no echocardiographic evidence of tamponade. Because of the posterior location, percutaneous drainage was not feasible and medical management was initiated. Despite escalation of medical therapy, the next morning the patient had an acute cardiac arrest. Resuscitation efforts were initiated but discontinued at the family’s request.