Cardiovascular Journal of Africa: Vol 33 No 2 (MARCH/APRIL 2022)

CARDIOVASCULAR JOURNAL OF AFRICA • Volume 33, No 2, March/April 2022 AFRICA 97 within five years. Eventually, the patient suffered from recurrent periodic episodes of acute pulmonary oedema. In our case, the patient presented with similar symptoms and arrhythmia during haemodialysis. Pulmonary oedema and bilateral pleural effusion resulted from diastolic congestive heart failure at admission. No effective management of restrictive cardiomyopathy resulting from fibrosis or calcification has been identified. The current medication strategy is conservative treatment, which could reduce the symptoms of pulmonary and general oedema by reducing the filling pressures and enhancing systolic pump function.10 We controlled intravascular fluid status through regular haemodialysis and administered inotropes to preserve cardiac function. Furthermore, strict control of the heart rate and blood pressure was critical in preventing arrhythmia events and sequelae as well as unstable haemodynamics. If optimal medication fails, heart transplantation remains the gold standard for end-stage restrictive cardiomyopathy.11 Ventricular assist devices represent an alternative treatment. Salas de Armas et al. reported a case of implantation of a left ventricular assist device in a heart failure patient with a heavily calcified left ventricle.12 They performed the non-excisional technique to preserve the left ventricular cavity. However, the use of ventricular assist devices for end-stage restrictive cardiomyopathy is rarely reported in the literature, and the benefits are limited because the small left ventricle always results in a suction event. Topilsky et al. analysed 83 end-stage heart failure cases waiting for heart transplantation, including eight restrictive or hypertrophic cardiomyopathies and 75 dilated and ischaemic cardiomyopathies, who received axial-flow left ventricular assist device therapy. The one-year actuarial survival rate did not differ between the two groups.13 These findings support the option of ventricular assist device implantation in patients for whom heart transplantation is not feasible and management with medication is not satisfactory. In addition to the use of a ventricular assist device, minimisation of inflammation, avoidance of calcium, correction of phosphate positive balance, and correction of vitamin D and K deficiencies were reported to be effective in the prevention of cardiovascular calcification.14 Sodium thiosulfate has been used to treat calciphylaxis, but a systematic review and meta-analysis revealed no significant clinical benefit.15 Kuzela et al.16 analysed via autopsy soft tissue calcification in chronic patients who had undergone dialysis, and determined that 78.5% of patients displayed general soft tissue calcification. Notably, our patient had only isolated left-side cardiac calcification, including the mitral annulus, septum and left ventricular myocardium. The calcification surrounding the left ventricle, which restricted the left ventricle in an eggshell, eventually resulted in diastolic dysfunction and pulmonary oedema. Conclusion We report on this rare and interesting case of isolated left ventricular calcification in a patient after 20 years of haemodialysis. Although we preserved his cardiac function with optimal medication, the disease may eventually be exacerbated into end-stage heart failure and pulmonary hypertension. Moreover, the prognosis is expected to be extremely poor in the near future. No accurate survival estimates for diffuse left ventricular calcification are reported in the literature. However, the five-year survival rate for all types of restrictive cardiomyopathy is only approximately 50%. References 1. Budisavljevic MN, Cheek D, Ploth DW. Calciphylaxis in chronic renal failure. J Am Soc Nephrol 1996; 7(7): 978–982. 2. Sayarlioglu H, Acar G, Sahin M, Altunoren O, Yavuz YC, Nacar AB, et al. Prevalence and risk factors of valvular calcification in hemodialysis patients. Iran J Kidney Dis 2013; 7(2): 129–134. 3. Nance JW, Crane GM, Halushka MK, Fishman EK, Zimmerman SL. Myocardial calcifications: pathophysiology, etiologies, differential diagnoses, and imaging findings. J Cardiovasc Comput Tomogr 2015; 9(1): 58–67. 4. Matsui M, Okayama S, Takitsume A, Morimoto K, Samejima K, Uemura S, et al. Heart failure associated with metastatic myocardial calcification in a hemodialysis patient with progressive calcification of the hand. Cardiorenal Med 2012; 2(4): 251–255. 5. El-Bialy A, Shenoda M, Saleh J, Tilkian A. Myocardial calcification as a rare cause of congestive heart failure: a case report. J Cardiovasc Pharmacol Ther 2005; 10(2): 137–143. 6. Sui M, Tang W, Wu C. 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Pediatr Dimens 2018; 3(2): 1–14. 11. Mehra MR, Canter CE, Hannan MM, Semigran MJ, Uber PA, Baran DA, et al. The 2016 International Society for Heart Lung Transplantation listing criteria for heart transplantation: a 10-year update. J Heart Lung Transplant 2016; 35(1): 1–23. 12. De Armas IS, Akay MH, Patel JA, Patel C, Patel MK, Akkanti BH, et al. Implantation of left ventricular assist device in the setting of heavily calcified left ventricular apex using an apex preserving technique. VAD J 2019; 5(2): 1–9. 13. Topilsky Y, Pereira NL, Shah DK, Boilson B, Schirger JA, Kushwaha SS, et al. Left ventricular assist device therapy in patients with restrictive and hypertrophic cardiomyopathy. Circ Heart Fail 2011; 4(3): 266–275. 14. Chen NC, Hsu CY, Chen CL. The strategy to prevent and regress the vascular calcification in dialysis patients. Biomed Res Int 2017; 2017: 9035193. 15. Udomkarnjananun S, Kongnatthasate K, Praditpornsilpa K, Eiam-Ong S, Jaber BL, Susantitaphong P. Treatment of calciphylaxis in CKD: a systematic review and meta-analysis. Kidney Int Rep 2018; 4(2): 231–244. 16. Kuzela DC, Huffer WE, Conger JD, Winter SD, Hammond WS. Soft tissue calcification in chronic dialysis patients. Am J Pathol 1977; 86(2): 403–424.

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