A case report of heart transplant for ischaemic cardiomyopathy from lupus coronary vasculitis

Abstract

Background: Coronary vasculitis is a rare, life-threatening complication of systemic lupus erythematosus (SLE).

Case summary: A 23-year-old woman with SLE presented with typical angina and worsening dyspnoea on exertion. Coronary angiography revealed severe triple vessel disease with a 'string of beads' appearance classic for coronary vasculitis. Transthoracic echocardiogram revealed ejection fraction of 25-30% with a severely hypokinetic distal septum and distal anterior wall and an akinetic apical wall. Despite vasculitis treatment with cyclophosphamide and pulse-dose steroids, her coronary vasculitis did not improve. She was refractory to anti-anginal and guideline-directed medical therapy for heart failure and successfully underwent orthotopic heart transplant (OHT).

Discussion: This is the first reported case of OHT in the case of SLE coronary vasculitis. Chronic SLE coronary vasculitis is caused by lymphocyic infiltration leading to inflammation and fibrosis of the major epicardial coronary arteries but can be successfully managed with OHT when refractory to medical SLE and heart failure therapies. It can affect patients of all ages with SLE, emphasizing the importance of thorough history taking and clinical evaluation in young patients presenting with cardiac symptoms to establish an appropriate diagnosis and treatment plan.

Keywords: Case report; Coronary vasculitis; Ischaemic cardiomyopathy; Orthotopic heart transplant; Systemic lupus erythematosus.

Figure 1

Initial electrocardiogram on presentation. The electrocardiogram on presentation demonstrates sinus tachycardia with ST-segment elevation in aVR and V1 and diffuse ST-segment depression, most evident in the lateral leads (I, aVL, and V5–V6).

Figure 2

Initial and 2-week post-treatment coronary angiography. Patient's initial coronary angiogram with left anterior oblique cranial (A) of the left coronary system and a left anterior oblique view of the right coronary system (B). The left main coronary artery shows an ostial and proximal 70% stenosis, the left anterior descending demonstrated a proximal left anterior descending 90–95% stenosis, severe diffuse disease and a ‘string of beads’ appearance of the remainder of the vessel. The mid-left circumflex demonstrates a 100% occlusion, and a small right posterior descending artery is totally occluded ostially. The right coronary artery demonstrates diffuse disease and right-to-left collaterals. On her second angiogram, the left anterior descending is 100% stenosed in the mid-segment (C). The first diagonal and obtuse marginal arteries remain severely diseased. The right coronary artery remains diffusely diseased with an ostially occluded R-PDA.

Figure 3

Still images of coronary angiography. These still images of the initial angiography demonstrate a ‘string of beads’ pattern consistent with coronary vasculitis. The coronary anatomy is described in Figure 2. Arrows indicate the ‘string of beads’ or pruned appearance of the coronary arteries consistent with vasculitis.

Figure 4

Cardiac magnetic resonance imaging and coronary computed tomographic angiography images. (A) Cardiac magnetic resonance imaging four-chamber late gadolinium enhancement. About 25–50% mural thickness late gadolinium enhancement in the mid to apical inferoseptal wall and both papillary muscles, and full-thickness late gadolinium enhancement of the apex is seen. (B) Cardiac magnetic resonance imaging showing two-chamber late gadolinium enhancement of left ventricle. About 25–50% mural thickness late gadolinium enhancement of the mid to apical inferior wall and full-thickness late gadolinium enhancement of apex is seen. (C) Cardiac magnetic resonance imaging three-chamber late gadolinium enhancement. About 25–50% mural thickness late gadolinium enhancement of mid to distal anteroseptal and 25–50% of mid to apical inferolateral wall and full-thickness late gadolinium enhancement of apex. (D) Cardiac magnetic resonance imaging short-axis late gadolinium enhancement of left ventricle at the mid-ventricular level. About 25–50% inferolateral, inferoseptal, and anterolateral wall late gadolinium enhancement is seen. (E) Cardiac magnetic resonance imaging short-axis T2-weighted turbo spin-echo imaging of the left ventricle at the mid-ventricular level with fat saturation (matching level as image D) demonstrates faint subendocardial enhancement in the area of late gadolinium enhancement. (F) Coronary computed tomographic angiography does not demonstrate evidence of arterial wall inflammation of the great vessels. Pulmonary artery measured to be 2.6 cm, thought to be mildly dilated due to history of atrial septal defect repair.

Figure 5

Steady-state free procession. Cine imaging of left ventricle four-chamber and short-axis view: left ventricle is mildly dilated without any filling defect or mass. There is thinning and akinesis of the entire apex and moderate to severe hypokinesis of the mid anterior, anteroseptal, inferoseptal, and inferolateral segments. The right ventricle demonstrates mild global hypokinesis. There is mild to moderate aortic regurgitation.

Figure 6

Pathology of explanted heart. (A) Ventral half of the explanted heart, showing a dilated left ventricle (on the left), with small areas of myocardial discolouration near the apex, and a markedly stenotic cross section of the right coronary artery (red arrow), and the left circumflex artery (blue arrow). (B) Image of the right coronary artery, near the area of the arrow in A, showing marked intimal fibrosis that markedly narrows the lumen of the artery, with moderate chronic inflammation deep in the intima. (C) Trichrome stain of discoloured myocardium near the left ventricular apex, showing area of subendocardial ischaemia (pale) with surrounding early fibrosis (blue). Normal myocardium stains red.