CARDIOVASCULAR JOURNAL OF AFRICA • Volume 33, No 2, March/April 2022 AFRICA 95 Restrictive cardiomyopathy caused by diffuse calcification of the left ventricle after 20 years of haemodialysis Cheng-Chieh Yang, Chien-Sung Tsai, Yi-Ting Tsai, Chih-Yuan Lin, Jia-Lin Chen, Po-Shun Hsu Abstract Valvular and vascular calcifications are common among patients with end-stage renal disease, but diffuse calcification of the left ventricle is rarely reported. We report on a rare case of restrictive cardiomyopathy resulting from severe myocardial calcification and review the literature. A 77-yearold man was diagnosed with end-stage renal disease after having received regular haemodialysis for 20 years. He was referred to our emergency room due to exertional dyspnoea and exacerbated shortness of breath. A chest X-ray revealed severe pulmonary oedema and bilateral massive pleural effusion. Transthoracic echocardiography revealed impaired diastolic function of the left ventricle but preserved systolic function with a 50% ejection fraction. Repeat chest computed tomography demonstrated exacerbation of the calcification from the mitral annulus to the whole circular left ventricle. A coronary angiogram revealed non-significant stenosis, and right heart catheterisation demonstrated elevated pulmonary capillary wedge pressure. He was discharged after two weeks of conservative medication. Keywords: cardiac calcification, restrictive cardiomyopathy, heart failure Submitted 8/7/20, accepted 24/6/21 Published online 5/7/21 Cardiovasc J Afr 2022; 33: 95–97 www.cvja.co.za DOI: 10.5830/CVJA-2021-031 Diffuse left ventricular calcification is rare, and only a few cases have been reported in the literature. Generally, the pathogenesis of calcification is dystrophic or metastatic. Dystrophic calcification results from calcium deposition in necrotic tissue, which can subsequently cause injury of the myocardium, most commonly resulting from ischaemia. Metastatic calcification develops in normal tissue due to elevated serum calcium levels, which are typically associated with renal failure or calcium-related metabolic diseases. Calcific uraemic arteriolopathy is observed in approximately 1% of patients with chronic renal failure and 4.1% of patients undergoing regular haemodialysis.1 The prevalence of valvular calcification, aortic valve calcification and mitral valve calcification is 33, 23.3 and 21.7%, respectively, in patients undergoing dialysis.2 However, the prevalence of myocardial calcification in patients undergoing haemodialysis is uncertain because only a few cases have been reported in the literature. Case report Our patient was a 77-year-old man with a history of end-stage renal disease who had received regular haemodialysis for 20 years. He was diagnosed with an old cerebrovascular disease with right hemiplegia, diabetes mellitus and coronary artery disease after percutaneous coronary intervention. He was also diagnosed with severe mitral annulus calcification without stenosis or regurgitation two years earlier. At this visit, he was sent to our emergency room due to symptoms of exertional dyspnoea, exacerbated shortness of breath, palpitation and hypotension during haemodialysis. His heart rate was 164 beats per minute and his blood pressure was 101/63 mmHg. A 12-lead electrocardiography revealed atrial flutter. The chest X-ray in the emergency room revealed pulmonary oedema and bilateral pleural effusion (Fig. 1). Transthoracic echocardiography revealed impaired diastolic function of the left ventricle but preserved systolic function with a 50% ejection fraction. Chest computed tomography revealed diffuse circular calcification of the left ventricle and massive bilateral pleural effusion (Fig. 2). Thyroid-stimulating hormone level was 1.50 µU/ml and free T4 level was 1.51 ng/dl, which suggested normal thyroid function. The free calcium level was also in the normal range of 4.93 mg/dl. The coronary angiogram revealed no significant stenosis requiring percutaneous coronary intervention. However, Division of Cardiovascular Surgery, Department of Surgery, Tri-Service General Hospital, National Defense Medical Cenrtre, Taipei, Taiwan Cheng-Chieh Yang, MD Chien-Sung Tsai, MD Yi-Ting Tsai, MD Chih-Yuan Lin, MD Po-Shun Hsu, MD, waterdrunken@gmail.com Department of Anaesthesia, Tri-Service General Hospital, National Defense Medical Centre, Taipei, Taiwan Jia-Lin Chen, MD Case Reports
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