title: Coconut atrium in long-standing rheumatic valvular heart disease.
abstract: BACKGROUND
  Complete calcification of the left atrium (LA) is called "coconut atrium", which decreases the compliance of LA, leading to the elevation of LA pressure that is transmitted to the right-side of the heart. The pathogenesis of LA calcification in patients with rheumatic heart disease is unknown; however, possible mechanisms include chronic strain force in the atrial wall and inflammation. We report here a patient with long-standing rheumatic valvular heart disease with coconut atrium.
  
  
  METHODS
  A 76-year-old man presented with breathlessness and leg edema due to right-sided heart failure. He was diagnosed with rheumatic fever at 8 years of age. Mitral commissurotomy and the mitral and aortic valve replacement were previously performed to treat mitral and aortic valvular stenosis. The profile view of the chest X-ray indicated a diffuse calcified outline of the LA wall. A transthoracic echocardiogram revealed pulmonary hypertension and dilatation of both atria. Moreover, computed tomography showed nearly circumferential calcification of the LA wall. Despite intense medical treatment, he succumbed to heart failure. An autopsy demonstrated that the LA was markedly dilated, its wall was calcified, and its appearance was similar to the surface of an atherosclerotic aorta. Microscopic examination revealed intensive calcification in the endocardium. Minimal accumulation of inflammatory cells was noted. Although slight fibrosis was observed, the cardiac musculature was preserved.
  
  
  CONCLUSIONS
  To the best of our knowledge, this is the first report that identifies the histological changes of LA calcification associated with long-standing rheumatic valvular heart disease.
authors.affiliation: Department of Cardiovascular Medicine, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Takahisa
authors.initials: T
authors.lastname: Onishi
authors.affiliation: Department of Pathology, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Yuka
authors.initials: Y
authors.lastname: Idei
authors.affiliation: Department of General Medicine, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Kazunori
authors.initials: K
authors.lastname: Otsui
authors.affiliation: Department of Cardiovascular Medicine, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Sachiyo
authors.initials: S
authors.lastname: Iwata
authors.affiliation: Department of Cardiovascular Medicine, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Suzuki
authors.affiliation: Department of Cardiovascular Medicine, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Toru
authors.initials: T
authors.lastname: Ozawa
authors.affiliation: Department of General Medicine, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Domoto
authors.affiliation: Department of Cardiovascular Medicine, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Asumi
authors.initials: A
authors.lastname: Takei
authors.affiliation: Department of General Medicine, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Shinya
authors.initials: S
authors.lastname: Inamoto
authors.affiliation: Department of Cardiovascular Medicine, Kobe Rosai Hospital, Kobe, Hyogo, Japan.
authors.forename: Nobutaka
authors.initials: N
authors.lastname: Inoue
mesh_terms: D000368:Aged; D002114:Calcinosis / Q000175:diagnosis / Q000209:etiology*; D009202:Cardiomyopathies / Q000175:diagnosis / Q000209:etiology*; D017809:Fatal Outcome; D006325:Heart Atria; D006333:Heart Failure / Q000150:complications*; D006349:Heart Valve Diseases / Q000150:complications*; D006801:Humans; D008297:Male; D012214:Rheumatic Heart Disease / Q000150:complications*
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