Key result
Propionic acidemia is linked to RV diastolic dysfunction in ~33% of patients.
Why the study?
Cardiomyopathy contributes to mortality in propionic acidemia, but there were no published data on right ventricular function in these patients.
What is the prevalence of systolic and diastolic right ventricular dysfunction assessed by echocardiography in patients with propionic acidemia, and how does it correlate with left ventricular function?
Cross-Sectional (n=18)
No
What is the prevalence of systolic and diastolic right ventricular dysfunction assessed by echocardiography in patients with propionic acidemia, and how does it correlate with left ventricular function?
This study provides the first characterization of right ventricular function in propionic acidemia, demonstrating that diastolic RV dysfunction occurs in up to one-third and systolic RV dysfunction in 16.7% of patients, and was present in all patients who subsequently died.
Answers are grounded in this paper and cite their sources.
RV dysfunction may merit monitoring in propionic acidemia; leaves open its prognostic value and need for intervention.
(1) Background: In propionic acidemia (PA), myocardial involvement often leads to progressive cardiac dysfunction of the left ventricle (LV). Cardiomyopathy (CM) is an important contributor to mortality. Although known to be of prognostic value in CM, there are no published data on right ventricular (RV) function in PA patients. (2) Methods: In this cross-sectional single-center study, systolic and diastolic RV function of PA patients was assessed by echocardiography, including frequency, onset, and combinations of echocardiographic parameters, as well as correlations to LV size and function. (3) Results: N = 18 patients were enrolled. Tricuspid annulus S’ was abnormal in 16.7%, RV-longitudinal strain in 11.1%, tricuspid annular plane systolic excursion (TAPSE) in 11.1%, Tricuspid valve (TV) E/e’ in 33.3%, and TV E/A in 16.7%. The most prevalent combinations of pathological parameters were TV E/A + TV E/e’ and TAPSE + TV S’. With age, the probability of developing abnormal RV function increases according to age-dependent normative data. There is a significant correlation between TAPSE and mitral annular plane systolic excursion (MAPSE), and RV/LV-longitudinal strain (p ≤ 0.05). N = 5 individuals died 1.94 years (mean) after cardiac evaluation for this study, and all had abnormal RV functional parameters. (4) Conclusions: Signs of diastolic RV dysfunction can be found in up to one third of individuals, and systolic RV dysfunction in 16.7% of individuals in our cohort. RV function is impaired in PA patients with a poor outcome. RV functional parameters should be used to complement clinical and left ventricular echocardiographic findings.
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Kovacevic et al. (2023) conducted a cross-sectional in Propionic acidemia (n=18). Propionic acidemia (evaluated by transthoracic echocardiography for RV function) vs. Published age-dependent normative echocardiographic data was evaluated on Prevalence of abnormal right ventricular diastolic function (Tricuspid valve E/e') and systolic function (Tricuspid annulus S'). Propionic acidemia patients exhibited diastolic right ventricular dysfunction (abnormal TV E/e') in 33.3% and systolic RV dysfunction (abnormal TV S') in 16.7% of cases.
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