Pathological evaluation of massive left atrial calcification 35 years after mitral-aortic valve replacement

Abstract

IntroductionAlthough calcification of the left atrium is relatively common in the setting of long-lasting rheumatic valve disease, massive calcification of the left atrium, also known as ''porcelain atrium [1],'' ''coconut atrium [2], '' or ''mold-like calcification [3],'' is a rare condition. The left atrial calcification is described as a high-density ring encircling the left atrium on chest X-ray. Noncontrast computed tomographic (CT) scan of the thorax reveals deposition of calcification in the wall of the left atrium. This is, to the best of our knowledge, the first case report for precise histopathological evaluation of rheumatic massive left atrial calcification by autopsy specimen. Case reportAn 84-year-old woman had been treated by our cardiology department with congestive heart failure as an outpatient. Her medical history revealed rheumatic valvulopathy and surgery of mitral-aortic valve replacement to mechanical heart valves, the St. Jude bileaflet valves in 1979 in another institution. Replacements of dysfunctioning prostheses to the St. Jude valves were performed in 1992 and 2000. More recently, she had implanted VVI pacemaker due to atrial fibrillation-related bradycardia in 2008. Warfarin treatment indicated prothrombin time-international normalized ratio 1.2-1.4 during her outpatient therapy. Chronic renal failure was pointed out and her estimated glomerular filtration rate was 45-55 ml/min/1.73 m 2 . She was admitted to our hospital due to worsening of congestive heart failure. Chest roentgenogram showed severe cardiomegaly with rim of calcification outlining the enlarged left atrium (Fig. 1A). Echocardiography revealed enlarged left atrium A B S T R A C T Massive calcification of the left atrium is an uncommon complication of long-standing rheumatic valve disease, and is frequently revealed in patients with a previous operation on the mitral valve. An 84-yearold woman, who had a history of rheumatic valvulopathy and mitral-aortic valve replacement surgery 35 years previously, was admitted to our hospital due to congestive heart failure. Chest X-ray showed severe cardiomegaly with rim of calcification outlining the enlarged left atrium. Computed tomographic scan of the thorax clearly illustrated dense and partially thick plate-like calcification of left atrial free wall and interatrial septum. She died due to congestive heart failure 14 days after hospitalization and an autopsy was performed. Enlargement of left atrium with massive calcification was evident by gross pathology. Thick plate-like calcification with both erosion and mural hemorrhage was clearly shown by a cross-section of atrial wall. Organized and fresh fibrin thrombi were identified at the surface of left atrium. Calcified tissue penetrated into the wall of the left atrium and lack of myocardium underneath the massive calcified lesion was evident. Although rheumatic calcification of left atrium revealed by medical images was previously described, this is the first case report that demonstrates histopathological evaluation of massi...

Measurement of interatrial septal thickness by echocardiography in patients with moderate to severe rheumatic mitral stenosis undergoing percutaneous balloon mitral valvuloplasty

“…Longstanding rheumatic mitral valve disease or previous mitral valve surgery may lead to calcification of the left atrium as an uncommon complication. It has been suggested that the calcification of the left atrium is a response to the chronic strain forces present in the setting of mitral disease [13,14]. A review by Harthorne et al has remarked that the interatrial septum is often spared and free from calcification [1].…”

“…Longstanding severe rheumatic mitral stenosis is associated with changes in the atrial chamber dimensions. It is not specifically known whether there are changes in the IAS dimensions proportionate to the increasing severity of rheumatic mitral stenosis, however, there is some evidence that there may be relative sparing of the interatrial septum [[12], [13], [14]]. Since no previous studies have reported echocardiographic changes in the IAS thickness or thinning in valvular heart disease patients; the aim of this study was to evaluate IAS thickness by transesophageal (TEE) in a consecutive series of patients undergoing percutaneous balloon mitral valvuloplasty for moderate to severe rheumatic mitral stenosis.…”

A Treatise on the Surgical Management of Porcelain Atrium and Coconut Atrium

“…Surgical access to the mitral valve in cases of complete calcification of the left atrium, including the interatrial septum becomes very difficult. Out of 16 reported cases in the published literature only 4 patients could be operated on [51][52][53][54][55][56][57][58][59][60][61][62][63][64].…”

Left atrial calcification on chest CT: atrial ablation replaces rheumatic heart disease as the most identified etiology

“…In fact, despite the significant decrease in the national prevalence of rheumatic fever (RF), left atrial calcification remains a relatively common finding on imaging, both on chest and cardiac computed tomography [2,3]. Importantly, the body of literature gives other possible causes of left atrial calcification, including mitral valve disease and mitral valve replacement, end-stage renal disease, history of radiation therapy, and non-specific endocarditis, but no study has demonstrated modern distribution of pathology [4][5][6][7][8][9][10][11][12]. Indeed, the modern proliferation of endomyocardial ablation techniques and catheter-directed therapies for structural heart disease, raises the question of iatrogenic damage [13,14].…”