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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.

SUBMITTER: Nandkeolyar S

PROVIDER: S-EPMC7042145 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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

Nandkeolyar Shuktika S Kim Hyungjin B HB Doctorian Tanya T Stoletniy Liset N LN Sandhu Vaneet K VK Yu Micah M Zuppan Craig W CW Razzouk Anees A Hilliard Anthony A Parwani Purvi P

European heart journal. Case reports 20191031 4

<h4>Background</h4>Coronary vasculitis is a rare, life-threatening complication of systemic lupus erythematosus (SLE).<h4>Case summary</h4>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 a ...[more]

PMID: 32123802

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Project description:BackgroundAntineutrophil cytoplasmic antibody (ANCA)-associated pulmonary renal vasculitis is an uncommon disease entity. Its presentation as acute heart failure for the first time in a patient with established coronary artery disease (CAD) is even rarer. We present here a case of such an association and an approach to managing this clinical situation.Case summaryA 60-year-old male patient presented to the emergency room with recent-onset dyspnoea New York Heart Association Class IV. He was having hypertension, uncontrolled diabetes mellitus, chronic kidney disease (CKD), and CAD. He also underwent a percutaneous coronary intervention to left anterior descending in the past for acute coronary syndrome and had moderate left ventricular dysfunction. He was being managed as a case of acute decompensated heart failure (ADHF) and was mechanically ventilated. Suddenly his ventilator requirement increased and endotracheal aspirate contained blood. The chest radiograph showed bilateral hilar infiltrates. Simultaneously he also had recurrent episodes of ventricular tachycardia (VT) requiring direct current (DC) cardioversion. Blood investigations showed deranged renal function and severe hyperkalaemia, but no evidence of coagulopathy. High-resolution computed tomography chest showed features of diffuse alveolar haemorrhage. Further investigations revealed high titres of c-ANCA and raised inflammatory biomarkers. A diagnosis of ANCA-associated vasculitis presenting as acute on CKD with dyselectrolytaemia (hyperkalaemia) leading to VT was made. Apart from standard management for associated illness, he was treated with plasma exchange, steroids, and cyclophosphamide to which he responded and was later on discharged.DiscussionAntineutrophil cytoplasmic antibody-related pulmonary renal vasculitis can lead to rapidly progressing renal failure and may present as ADHF in a patient with existent CAD. The associated VT storm in our patient can be attributed to hyperkalaemia secondary to acute renal failure. A multidisciplinary approach is required for the successful management of such a complex clinical scenario.

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

Publications

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

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