Evaluation of Right Ventricular Function in Patients with Propionic Acidemia-A Cross-Sectional Study.
Alexander KovacevicSven F GarbadeFriederike HörsterGeorg F HoffmannMatthias GorenfloDerliz MerelesStefan KölkerChristian StaufnerPublished in: Children (Basel, Switzerland) (2023)
(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.
Keyphrases
- left ventricular
- mycobacterium tuberculosis
- mitral valve
- aortic stenosis
- ejection fraction
- heart failure
- hypertrophic cardiomyopathy
- blood pressure
- cardiac resynchronization therapy
- end stage renal disease
- left atrial
- cross sectional
- acute myocardial infarction
- newly diagnosed
- pulmonary hypertension
- aortic valve
- chronic kidney disease
- prognostic factors
- systematic review
- peritoneal dialysis
- computed tomography
- oxidative stress
- cardiovascular disease
- transcatheter aortic valve replacement
- patient reported outcomes
- clinical evaluation