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Severe Functional Tricuspid Stenosis Due to Phosphoglyceride Crystal Deposition Disease in Right Atrium.

ABSTRACT: This case report concerns a 72-year-old-female with severe functional tricuspid stenosis due to phosphoglyceride crystal deposition disease and a history of atrial septum closure and tricuspid valvuloplasty. Phosphoglyceride crystal deposition disease is extremely rare, and percutaneous transcatheter biopsy under intracardiac echocardiographic guidance proved to be useful for its diagnosis. (Level of Difficulty: Advanced.).

SUBMITTER: Takeuchi K

PROVIDER: S-EPMC8855130 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature

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Severe Functional Tricuspid Stenosis Due to Phosphoglyceride Crystal Deposition Disease in Right Atrium.

Takeuchi Kimikazu K Tanaka Hidekazu H Kawamori Hiroyuki H Ohta Eri E Suzuki Makiko M Shimoura Hiroyuki H Izawa Yu Y Kodama Yoshinori Y Hirata Ken-Ichi KI

JACC. Case reports 20220216 4

This case report concerns a 72-year-old-female with severe functional tricuspid stenosis due to phosphoglyceride crystal deposition disease and a history of atrial septum closure and tricuspid valvuloplasty. Phosphoglyceride crystal deposition disease is extremely rare, and percutaneous transcatheter biopsy under intracardiac echocardiographic guidance proved to be useful for its diagnosis. (<b>Level of Difficulty: Advanced.</b>). ...[more]

PMID: 35199022

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Project description:ObjectivesThis study sought to evaluate the prognostic value of cardiac magnetic resonance (CMR) feature-tracking-derived right ventricular (RV) free wall longitudinal strain (RVFWLS) in a large multicenter population of patients with severe functional tricuspid regurgitation.BackgroundTricuspid regurgitation imposes a volume overload on the RV that can lead to progressive RV dilation and dysfunction. Overt RV dysfunction is associated with poor prognosis and increased operative risk. Abnormalities of myocardial strain may provide the earliest evidence of ventricular dysfunction. CMR feature-tracking techniques now allow assessment of strain from routine cine images, without specialized pulse sequences. Whether abnormalities of RV strain measured using CMR feature tracking have prognostic value in patients with tricuspid regurgitation is unknown.MethodsConsecutive patients with severe functional tricuspid regurgitation undergoing CMR at 4 U.S. medical centers were included in this study. Feature-tracking RVFWLS was calculated from 4-chamber cine views. The primary endpoint was all-cause death. Cox proportional hazards regression modeling was used to examine the independent association between RVFWLS and death. The incremental prognostic value of RVFWLS was assessed in nested models.ResultsOf the 544 patients in this study, 128 died during a median follow-up of 6 years. By Kaplan-Meier analysis, patients with RVFWLS ≥median (-16%) had significantly reduced event-free survival compared with those with RVFWLS <median (log-rank p < 0.001). By Cox multivariable regression modeling, RVFWLS was associated with increased risk-of-death after adjustment for clinical and imaging risk factors, including RV size and ejection fraction (hazard ratio: 1.14 per %; p < 0.001). Addition of RVFWLS in this model resulted in significant improvement in the global chi-square (31 to 78; p < 0.001).ConclusionsCMR feature-tracking-derived RVFWLS is an independent predictor of mortality in patients with severe functional tricuspid regurgitation, incremental to common clinical and imaging risk factors.

Dataset Information

Severe Functional Tricuspid Stenosis Due to Phosphoglyceride Crystal Deposition Disease in Right Atrium.

Publications

Severe Functional Tricuspid Stenosis Due to Phosphoglyceride Crystal Deposition Disease in Right Atrium.

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