Sex-Specific Prognosis of Left Ventricular Size and Function Following Repair of Degenerative Mitral Regurgitation.

BACKGROUND: Prior studies have demonstrated worse long-term outcomes for women after surgery for severe mitral regurgitation (MR). The current Class I indications for surgery for severe degenerative MR use cutoffs of left ventricular end-systolic dimension (LVESD) and left ventricular ejection fraction (EF) that do not account for known sex-related differences. OBJECTIVES: The primary objective of this study was to assess long-term mortality following mitral valve repair in women compared with men on the basis of preoperative left ventricular systolic dimensions and EF. METHODS: Consecutive patients who underwent isolated mitral valve repair for degenerative MR at a single institution between 1994 and 2016 were screened. Adjusted HRs for all-cause mortality were compared according to baseline LVESD, LVESD indexed to body surface area (LVESDi), and EF for men and women. RESULTS: Among 4,589 patients, 1,825 were women (40%), and after a median follow-up period of 7.2 years, 344 patients (7.5%) had died. The risk for mortality for women increased from the baseline hazard at an LVESD of 3.6 cm, whereas an inflection point for increased risk with LVESD was not evident in men. Regarding LVESDi, the risk for women increased at 1.8 cm/m2 compared with 2.1 cm/m2 in men. For EF, women and men had a similar inflection point (58%); however, mortality was higher for women as EF decreased. CONCLUSIONS: After mitral valve repair, women have a higher risk for all-cause mortality at lower LVESD and LVESDi and higher EF. These results support consideration of sex-specific thresholds for LVESDi in surgical decision making for patients with severe MR.

Refractory right ventricular myocarditis induced by immune checkpoint inhibitor despite therapy cessation and immune suppression.

BACKGROUND: Immune checkpoint inhibitors (ICIs) are currently widely used for treatment of various types of cancers. ICI-induced myocarditis, though uncommon, accounts for high risk of major adverse cardiac events and mortality, which makes appropriate diagnosis important. We here present a unique, challenging case of ICI-induced, refractory and isolated right ventricular (RV) myocarditis. CASE PRESENTATION: A 32-year-old female with breast cancer presented with newly onset chest pain and dyspnea shortly after initiation of Pembrolizumab. Coronary angiography showed normal coronary arteries and a cardiac magnetic resonance (CMR) revealed myocarditis involving the right ventricle with chamber dilation and severe dysfunction. ICI therapy was stopped, and high dose steroid therapy was initiated and symptoms resolved. However, three months after initial presentation, the patient was hospitalized for DKA and decompensated right heart failure, and a repeat cardiac MRI at that time showed recurrent, isolated right ventricular myocardial inflammation/edema without LV involvement. High dose steroid therapy was started again and at 6-month follow up, surveillance CMR continued to show persistent right-sided myocarditis, patient was eventually treated with Abatacept with resolution of HF symptoms, RV dysfunction and biomarkers at 10-month follow up. CONCLUSIONS: We describe a unique case of isolated ICI-induced right ventricular myocarditis leading to right ventricular failure, that was refractory despite ICI therapy cessation and immune suppression by repeated high dose steroids. Co-stimulatory pathway modulation with Abatacept eventually lead to the normalization of RV function and dilation ten months after initial myocarditis onset.

Patient-Specific Quantitative In-Vivo Assessment of Human Mitral Valve Leaflet Strain Before and After MitraClip Repair.

PURPOSE: Mitral regurgitation (MR) is a highly prevalent and deadly cardiac disease characterized by improper mitral valve (MV) leaflet coaptation. Among the plethora of available treatment strategies, the MitraClip is an especially safe option, but optimizing its long-term efficacy remains an urgent challenge. METHODS: We applied our noninvasive image-based strain computation pipeline [1] to intraoperative transesophageal echocardiography datasets taken from ten patients undergoing MitraClip repair, spanning a range of MR etiologies and MitraClip configurations. We then analyzed MV leaflet strains before and after MitraClip implementation to develop a better understanding of (1) the pre-operative state of human regurgitant MV, and (2) the MitraClip's impact on the MV leaflet deformations. RESULTS: The MV pre-operative strain fields were highly variable, underscoring both the heterogeneity of the MR in the patient population and the need for patient-specific treatment approaches. Similarly, there were no consistent overall post-operative strain patterns, although the average A2 segment radial strain difference between pre- and post-operative states was consistently positive. In contrast, the post-operative strain fields were better correlated to their respective pre-operative strain fields than to the inter-patient post-operative strain fields. This quantitative result implies that the patient specific pre-operative state of the MV guides its post-operative deformation, which suggests that the post-operative state can be predicted using pre-operative data-derived modelling alone. CONCLUSIONS: The pre-operative MV leaflet strain patterns varied considerably across the range of MR disease states and after MitraClip repair. Despite large inter-patient heterogeneity, the post-operative deformation appears principally dictated by the pre-operative deformation state. This novel finding suggests that though the variation in MR functional state and MitraClip-induced deformation were substantial, the post-operative state can be predicted from the pre-operative data alone. This study suggests that, with use of larger patient cohort and corresponding long-term outcomes, quantitative predictive factors of MitraClip durability can be identified.

Mitral and tricuspid stenosis caused by light chain cardiac amyloid deposition.

Cardiac amyloidosis results in an infiltrative restrictive cardiomyopathy, with a number of characteristic features: biventricular hypertrophy, abnormal myocardial global longitudinal strain with relative apical sparing, biatrial dilation, and small pericardial effusion along with conduction abnormalities. Amyloid deposits leading to hemodynamically significant valvular heart disease are very rare. We describe a rare case of concomitant moderately severe tricuspid and mitral valve stenosis because of ongoing amyloid deposition in a patient with progressive multiple myeloma and fat pad biopsy-proven light chain amyloidosis. Worsening infiltrative cardiomyopathy and valvulopathy despite evidence-based chemotherapy and heart failure pharmacotherapy led to end-stage disease and death. Valvular involvement in cardiac amyloidosis requires early recognition of the underlying disease condition to guide directed medical therapy and prevent its progression. In this instance, valvuloplasty or valve replacement is not a viable option.

Atrial Fibrillation in Transthyretin Cardiac Amyloidosis: Predictors, Prevalence, and Efficacy of Rhythm Control Strategies.

OBJECTIVES: This study sought to determine the incidence and prevalence of atrial fibrillation (AF) in transthyretin cardiac amyloidosis (ATTR-CA); to study the factors associated with the development of AF in this population; to study the prognostic implications of AF and maintenance of normal sinus rhythm (NSR) in patients with ATTR-CA; and to determine the impact of ATTR-CA stage on AF prevalence, outcomes, and efficacy of rhythm control strategies. BACKGROUND: AF is common in patients with ATTR-CA. The aim of this study was to determine the predictors, prevalence, and outcomes of AF in patients with ATTR-CA in addition to the efficacy of rhythm control strategies. METHODS: This was a retrospective cohort study of 382 patients with ATTR-CA diagnosed at our institution between January 2004 and January 2018. Means testing, and univariable and multivariable models were used. RESULTS: AF occurred in 265 (69%) patients. Factors associated with the development of AF included older age, advanced ATTR-CA stage, and higher left atrial volume index. Antiarrhythmic therapy (AAT) was used in 35% of patients with AF; cardioversion was performed in 45%, and 5% underwent AF ablation. Rhythm control strategies were substantially more effective when performed earlier in the disease course. During a mean follow-up of 35 months, no difference in mortality between patients with AF and those without AF was observed (65% vs. 49%; p = 0.76). On Cox proportional hazards analyses, maintenance of normal sinus rhythm and tafamidis use were associated with improved survival, whereas advanced ATTR-CA stage and higher New York Heart Association functional class were associated with increased mortality. CONCLUSIONS: With advancing ATTR-CA stage, AF became more prevalent, occurring in 69% of our entire study cohort. Rhythm control strategies including AAT, direct-current cardioversion, and AF ablation were substantially more effective when performed earlier during the disease course.

Pericardial Effusions: Causes, Diagnosis, and Management.

The presentation of a patient with a pericardial effusion can range from an incidental finding to a life-threatening emergency. Accordingly, the causes of pericardial effusions are numerous and can generally be divided into inflammatory and non-inflammatory etiologies. For all patients with a suspected pericardial effusion, echocardiography is essential to define the location and size of an effusion. In pericardial tamponade, the hemodynamics relate to decreased pericardial compliance, ventricular interdependence, and an inspiratory decrease in the pressure gradient for left ventricular filling. Echocardiography provides insight into the pathophysiologic alterations, primarily through an assessment of chamber collapse, inferior vena cava plethora, and marked respiratory variation in mitral and tricuspid inflow. Once diagnosed, pericardiocentesis is performed in patients with tamponade, preferably with echocardiographic guidance. With a large effusion but no tamponade, pericardiocentesis is rarely needed for diagnostic purposes, though is performed if there is concern for a bacterial infection. In patients with malignancy, pericardial window is preferred given the risk for recurrence. Finally, large effusions can progress to tamponade, but can generally be followed closely until the extent of the effusion facilitates safe pericardiocentesis.