Thomas, James D.

Importance Primary mitral regurgitation (MR) is a prevalent valvular lesion. Current American College of Cardiology/American Heart Association guidelines include class I recommendations for intervention for severe primary MR at the onset of symptoms, significant left ventricular (LV) enlargement (end-systolic dimension ≥40 mm), or dysfunction (ejection fraction ≤60%), with a class IIA recommendation for mitral valve repair when performed at an experienced surgical center. Recent data suggest a survival penalty when waiting for class I surgical guideline indications, and novel markers of decompensation are under investigation. Observations Comprehensive assessment of MR severity using echocardiography is critical, and when warranted, cardiac magnetic resonance (CMR) is complementary. Assessment of LV size and function, as well as serial changes in both, is crucial for determining timing of surgery. New-onset atrial fibrillation, left atrial enlargement, pulmonary hypertension, and exercise-induced changes in LV function should also be considered in borderline scenarios. The roles of LV volumes, global longitudinal strain, CMR-derived measures of myocardial dysfunction, and cardiac biomarkers are worthy of further investigation regarding consideration for early surgical intervention. Conclusions and Relevance A more refined approach incorporating assessment of extravalvular cardiac injury, novel imaging markers, and biomarkers is needed to optimize surgical timing in primary MR. Further research is warranted to validate these emerging parameters and refine guidelines to improve patient outcomes.

BACKGROUND: Defining qualitative grades of echocardiographic metrics of right heart chamber size and function is critical for screening, clinical assessment, and measurement of therapeutic response in individuals with pulmonary vascular disease (PVD). In a population enriched for PVD, we sought to establish qualitative grades and prognostic value of right heart chamber size and function. METHODS: We investigated 1053 study participants in PVDOMICS (Redefining Pulmonary Hypertension Through the PVD Phenomics Program) to determine clinical and echocardiographic differences associated with increasing pulmonary vascular resistance severity. Right heart chamber size and function were qualitatively assessed using a percentile-based approach above the median values to create a clinical grading system for right heart adaptation. The relationship between echocardiographic categories and all-cause mortality was examined using survival analyses adjusted for potential confounders. RESULTS: A stepwise increase in adverse right heart remodeling was observed with a concomitant decrease in functional parameters by pulmonary vascular resistance strata ( P <0.001 for all). Mild, moderate, and severe categories of right heart chamber size and dysfunction were defined using a percentile-based approach across the spectrum of PVD. During a median follow-up of 2.07 years (interquartile range, 1.23–3.01 years), 130 participants died (11.4%). Progressive pulmonary vascular resistance increase and 2DE evidence of right heart dysfunction inclusive of fractional area change, and right ventricular global longitudinal strain were independently associated with increased all-cause mortality risk in multivariate analysis adjusted for age, disease duration, and male sex. CONCLUSIONS: In this well-characterized sample of adults with diverse etiologies and varying PVD severity, we define categories of abnormal right heart chamber size and function. Further, we demonstrate a stepwise relationship between these categories of abnormal morphology and function and all-cause mortality. Defining grades of right ventricular dysfunction in individuals with known PVD has important clinical implications for monitoring disease progression and response to therapies.

Abstract Aims There is a wide spectrum of diseases associated with pulmonary hypertension, pulmonary vascular remodelling, and right ventricular dysfunction. The NIH-sponsored PVDOMICS network seeks to perform comprehensive clinical phenotyping and endophenotyping across these disorders to further evaluate and define pulmonary vascular disease. Methods and results Echocardiography represents the primary non-invasive method to phenotype cardiac anatomy, function, and haemodynamics in these complex patients. However, comprehensive right heart evaluation requires the use of multiple echocardiographic parameters and optimized techniques to ensure optimal image acquisition. The PVDOMICS echo protocol outlines the best practice approach to echo phenotypic assessment of the right heart/pulmonary artery unit. Conclusion Novel workflow processes, methods for quality control, data for feasibility of measurements, and reproducibility of right heart parameters derived from this study provide a benchmark frame of reference. Lessons learned from this protocol will serve as a best practice guide for echocardiographic image acquisition and analysis across the spectrum of right heart/pulmonary vascular disease.

The non‐invasive evaluation of prosthetic valve function is challenging. The effects of flow rate, valvular geometry, leaflet motion, and pressure recovery all impact the Doppler assessment of prosthetic performance. Differentiating prosthesis obstruction from pressure recovery in patients who are found to have high Doppler velocities across an aortic valve prosthesis is critical in order to direct appropriate management. In this manuscript, we present two cases of patients with aortic valve prosthesis with high Doppler velocities and review the pathophysiology and evaluation of prosthesis function. © 2014 Wiley Periodicals, Inc.