A rare case of stiff left atrial syndrome caused by both coconut left atrium and vertebral compression: a case report

Abstract

Background Calcification of the left atrium (LA) is a rare condition and can be the result of long-standing rheumatic mitral stenosis or an unusual complication after mitral valve replacement. Cases of massive LA calcification have sometimes been referred to as those with ‘coconut LA’ or ‘porcelain LA’. Case summary A 75-year-old woman was referred to our hospital because of chest discomfort and exertional dyspnoea. Doppler echocardiography revealed the presence of elevated filling pressure with significant LA dysfunction. A cardiac catheter examination revealed a quite impressive pulmonary capillary wedge pressure waveform with a steep up-slope and prominent v wave of 43 mmHg (mean: 15 mmHg). Multidetector row computed tomography revealed that LA was sandwiched by dense calcifications along the roof and bottom of the LA, and the posterior wall was compressed by a vertebral body. Integration of these functional and anatomical findings ultimately led to the diagnosis of ‘stiff LA syndrome’. Discussion She had a history of tuberculosis but no history of rheumatic fever or cardiac surgery. Thus, it appeared that the tuberculous pericarditis might have led to the calcified LA by long lasting inflammation. In this case, the LA was encased by a dense calcification and compressed by vertebral body from the posterior direction. Therefore, we speculated that the LA pressure could easily elevate even with a slight haemodynamic load in this special case, and thus eventually resulting in decompensated heart failure.

Coconut Atrium Causing Restrictive Physiology in the Right Ventricle

“…Stiff LA syndrome is defined as the combination of LA reservoir dysfunction and heart failure symptoms. Atrial fibrillation, catheter ablation, and left ventricular diastolic dysfunction reportedly contribute to its pathogenesis (6)(7)(8). As shown in Fig.…”

Spatiotemporal incremental change of radiofrequency catheter ablation‐associated pulmonary vein calcifications

“…Here, we present a detailed time course of RFCA‐associated PV calcification process for the first time. It is known that progressive calcification has the potential to reduce the function of the LA as a reservoir or booster pump 5 . Because RFCA‐associated PV calcification may progress over time even with a single ablation, long‐term caution should be paid in cases of especially extensive ablation and/or patients with stiff LA syndrome.…”

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].…”