Abstract
Endomyocardial fibrosis (EMF) is a progressive type of obliterative/restrictive cardiomyopathy characterized by fibrosis of the apical endomyocardium of the ventricles. Although the prognosis of EMF patients is poor in tropical regions, the exact clinical course and pathogenesis of patients with EMF are not known. Here, we report the rare case with EMF in the seventh decade, who showed the disappearance of papillary muscles, chordae tendineae, and part of the tricuspid valves due to massive right ventricular thrombus. Because of those unusual findings of the isolated RV involvement, we observed continuous forward pulmonary artery flow, as occurring in a Fontan circulation.
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References
Mocumbi AO, Ferreira MB, Sidi D, Yacoub MH (2008) A population study of endomyocardial fibrosis in a rural area of Mozambique. N Engl J Med 359:43–49
Seth S, Thatai D, Sharma S, Chopra P, Talwar KK (2004) Clinico-pathological evaluation of restrictive cardiomyopathy (endomyocardial fibrosis and idiopathic restrictive cardiomyopathy) in India. Eur J Heart Fail 6:723–729
Mocumbi AO, Yacoub S, Yacoub MH (2008) Neglected tropical cardiomyopathies: II. Endomyocardial fibrosis: myocardial disease. Heart 94:384–390
Mocumbi AO, Falase AO (2013) Recent advances in the epidemiology, diagnosis and treatment of endomyocardial fibrosis in Africa. Heart 99:1481–1487
Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248
Shimizu M, Miyamoto K, Nishimura Y, Izumi G, Sakai S, Inai K, Nishikawa T, Nakanishi T (2015) Risk factors and serological markers of liver cirrhosis after Fontan procedure. Heart Vessels. doi:10.1007/s00380-015-0743-4
Bjork VO, Olin CL, Bjarke BB, Thoren CA (1979) Right atrial-right ventricular anastomosis for correction of tricuspid atresia. J Thorac Cardiovasc Surg 77:452–458
Perazzolo Marra M, Thiene G, Rizzo S, De Lazzari M, Carturan E, Tona F, Caforio AL, Cacciavillani L, Marcolongo R, Tarantini G, Corbetti F, Iliceto S, Basso C (2014) Cardiac magnetic resonance features of biopsy-proven endomyocardial diseases. JACC Cardiovasc Imaging 7:309–312
Salemi VM, Rochitte CE, Shiozaki AA, Andrade JM, Parga JR, de Avila LF, Benvenuti LA, Cestari IN, Picard MH, Kim RJ, Mady C (2011) Late gadolinium enhancement magnetic resonance imaging in the diagnosis and prognosis of endomyocardial fibrosis patients. Circ Cardiovasc Imaging 4:304–311
Patel AK, Ziegler JL, D’Arbela PG, Somers K (1971) Familial cases of endomyocardial fibrosis in Uganda. Br Med J 4:331–334
Kishimoto C, Shioji K, Hashimoto T, Nonogi H, Lee JD, Kato S, Hiramatsu S, Morimoto SI (2014) Therapy with immunoglobulin in patients with acute myocarditis and cardiomyopathy: analysis of leukocyte balance. Heart Vessels 29:336–342
Frustaci A, Russo MA, Chimenti C (2009) Randomized study on the efficacy of immunosuppressive therapy in patients with virus-negative inflammatory cardiomyopathy: the TIMIC study. Eur Heart J 30:1995–2002
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Shiraishi, Y., Kohno, T., Fujii-Nishimura, Y. et al. Endomyocardial fibrosis: missing tricuspid valve and Fontan-like circulation. Heart Vessels 31, 1579–1582 (2016). https://doi.org/10.1007/s00380-015-0792-8
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DOI: https://doi.org/10.1007/s00380-015-0792-8