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Apical Aneurysms and Mid-Left Ventricular Obstruction in Hypertrophic Cardiomyopathy

Sherrid, Mark V; Bernard, Samuel; Tripathi, Nidhi; Patel, Yash; Modi, Vivek; Axel, Leon; Talebi, Soheila; Ghoshhajra, Brian B; Sanborn, Danita Y; Saric, Muhamed; Adlestein, Elizabeth; Alvarez, Isabel Castro; Xia, Yuhe; Swistel, Daniel G; Massera, Daniele; Fifer, Michael A; Kim, Bette
BACKGROUND:Apical left ventricular (LV) aneurysms in hypertrophic cardiomyopathy (HCM) are associated with adverse outcomes. The reported frequency of mid-LV obstruction has varied from 36% to 90%. OBJECTIVES/OBJECTIVE:The authors sought to ascertain the frequency of mid-LV obstruction in HCM apical aneurysms. METHODS:The authors analyzed echocardiographic and cardiac magnetic resonance examinations of patients with aneurysms from 3 dedicated programs and compared them with 63 normal controls and 47 controls with apical-mid HCM who did not have aneurysms (22 with increased LV systolic velocities). RESULTS:]; P = 0.004). Complete emptying occurs circumferentially around central PMs that contribute to obstruction. Late gadolinium enhancement was always brightest and the most transmural apical of, or at the level of, complete emptying. CONCLUSIONS:The great majority (95%) of patients in the continuum of apical aneurysms have associated mid-LV obstruction. Further research to investigate obstruction as a contributing cause to apical aneurysms is warranted.
PMID: 36681586
ISSN: 1876-7591
CID: 5419392

Sex Differences in Extensive Mitral Annular Calcification With Associated Mitral Valve Dysfunction

Churchill, Timothy W; Yucel, Evin; Bernard, Samuel; Namasivayam, Mayooran; Nagata, Yasufumi; Lau, Emily S; Deferm, Sebastien; He, Wei; Danik, Jacqueline S; Sanborn, Danita Y; Picard, Michael H; Levine, Robert A; Hung, Judy; Bertrand, Philippe B
Mitral annular calcification (MAC)-related mitral valve (MV) dysfunction is an increasingly recognized entity, which confers a high burden of morbidity and mortality. Although more common among women, there is a paucity of data regarding how the phenotype of MAC and the associated adverse clinical implications may differ between women and men. A total of 3,524 patients with extensive MAC and significant MAC-related MV dysfunction (i.e., transmitral gradient ≥3 mm Hg) were retrospectively analyzed from a large institutional database, with the goal of defining gender differences in clinical and echocardiographic characteristics and the prognostic importance of MAC-related MV dysfunction. We stratified patients into low- (3 to 5 mm Hg), moderate- (5 to 10 mm Hg), and high- (≥10 mm Hg) gradient groups and analyzed the gender differences in phenotype and outcome. The primary outcome was all-cause mortality, assessed using adjusted Cox regression models. Women represented the majority (67%) of subjects, were older (79.3 ± 10.4 vs 75.5 ± 10.9 years, p <0.001) and had a lower burden of cardiovascular co-morbidities than men. Women had higher transmitral gradients (5.7 ± 2.7 vs 5.3 ± 2.6 mm Hg, p <0.001), more concentric hypertrophy (49% vs 33%), and more mitral regurgitation. The median survival was 3.4 years (95% confidence interval 3.0 to 3.6) among women and 3.0 years (95% confidence interval 2.6 to 4.5) among men. The adjusted survival was worse among men, and the prognostic impact of the transmitral gradient did not differ overall by gender. In conclusion, we describe important gender differences among patients with MAC-related MV dysfunction and show worse adjusted survival among men; although, the adverse prognostic impact of the transmitral gradient was similar between men and women.
PMID: 36881941
ISSN: 1879-1913
CID: 5432712

Transesophageal Echocardiographic Screening for Structural Heart Interventions

Ro, Richard; Bamira, Daniel; Bernard, Samuel; Vainrib, Alan; Ibrahim, Homam; Staniloae, Cezar; Williams, Mathew R; Saric, Muhamed
PURPOSE OF REVIEW/OBJECTIVE:Percutaneous structural interventions have provided patients with an effective therapeutic option, and its growth has been aided by echocardiography. We describe the vital role that transesophageal echocardiography (TEE) plays in screening patients prior to their procedure. RECENT FINDINGS/RESULTS:A multimodality imaging approach is employed by the valve team, but TEE plays a unique role in diagnosis and planning. Utilization of all TEE views and features such as biplane, 3D imaging, and multiplanar reconstruction ensures accurate assessment of the structural lesion of interest. The role of TEE remains essential in the planning of structural interventions, and these studies should be performed in a systematic and comprehensive manner.
PMID: 36680732
ISSN: 1534-3170
CID: 5405192

Outcomes After Tricuspid Valve Operations in Patients With Drug-Use Infective Endocarditis

Siddiqui, Emaad; Alviar, Carlos L; Ramachandran, Abhinay; Flattery, Erin; Bernard, Samuel; Xia, Yuhe; Nayar, Ambika; Keller, Norma; Bangalore, Sripal
The increase of intravenous drug use has led to an increase in right-sided infective endocarditis and its complications including septic pulmonary embolism. The objective of this study was to compare the outcomes of tricuspid valve (TV) operations in patients with drug-use infective endocarditis (DU-IE) complicated by septic pulmonary emboli (PE). Hospitalizations for DU-IE complicated by septic PE were identified from the National Inpatient Sample from 2002 to 2019. Outcomes of patients who underwent TV operations were compared with medical management. The primary outcome was the incidence of major adverse cardiovascular events (MACEs), defined as in-hospital mortality, myocardial infarction, stroke, cardiogenic shock, or cardiac arrest. An inverse probability of treatment weighted analysis was utilized to adjust for the differences between the cohorts. A total of 9,029 cases of DU-IE with septic PE were identified (mean age 33.6 years), of which 818 patients (9.1%) underwent TV operation. Surgery was associated with a higher rate of MACE (14.5% vs 10.8%, p <0.01), driven by a higher rate of cardiogenic shock (6.1% vs 1.2%, p <0.01) but a lower rate of mortality (2.7% vs 5.7%, p <0.01). Moreover, TV operation was associated with an increased need for permanent pacemakers, blood transfusions, and a higher risk of acute kidney injury. In the inverse probability treatment weighting analysis, TV operation was associated with an increased risk for MACE driven by a higher rate of cardiogenic shock and cardiac arrest, but a lower rate of mortality when compared with medical therapy alone. In conclusion, TV operations in patients with DU-IE complicated by septic PE are associated with an increased risk for MACE but a decreased risk of mortality. Although surgical management may be beneficial in some patients, alternative options such as percutaneous debulking should be considered given the higher risk.
PMID: 36280471
ISSN: 1879-1913
CID: 5365292

Acute valvular emergencies

Bernard, Samuel; Deferm, Sebastien; Bertrand, Philippe B
Acute valvular emergencies represent an important cause of cardiogenic shock. However, their clinical presentation and initial diagnostic testing are often non-specific, resulting in delayed diagnosis. Moreover, metabolic disarray or haemodynamic instability may result in too great a risk for emergent surgery. This review will focus on the aetiology, clinical presentation, diagnostic findings, and treatment options for patients presenting with native acute left-sided valvular emergencies. In addition to surgery, options for medical therapy, mechanical circulatory support, and novel percutaneous interventions are discussed.
PMID: 35912478
ISSN: 2048-8734
CID: 5286322

Impact of Pulmonary Hypertension on Outcomes in Patients With Mitral Annular Calcium and Associated Mitral Valve Dysfunction

Deferm, Sébastien; Bertrand, Philippe B; Yucel, Evin; Bernard, Samuel; Namasivayam, Mayooran; Nagata, Yasufumi; Dal-Bianco, Jacob P; Sanborn, Danita Y; Picard, Michael H; Levine, Robert A; Hung, Judy; Churchill, Timothy W
The prevalence of mitral annular calcium (MAC) is increasing in our aging population. However, data regarding prognostication in MAC-related mitral valve (MV) disease remain limited. This retrospective observational study aims to explore the prognostic impact of systolic pulmonary artery pressure (SPAP) in MAC-related MV dysfunction and define its determinants. We identified 4,384 patients (mean age 78 ± 11 years and 69% female) with MAC-related MV dysfunction (documented transmitral gradient ≥3 mm Hg) from a large institutional echocardiographic database between 2001 and 2019. In Cox regression analysis, higher SPAP strongly associated with all-cause mortality, independent of cardiovascular risk factors and indices of MV dysfunction (adjusted hazard ratio 1.22 per 10 mm Hg SPAP increase, 95% confidence interval 1.17 to 1.27). Patients with SPAP ≥50 mm Hg had significantly higher mortality compared with SPAP <50 mm Hg (log-rank p <0.001), a finding that was consistent across different transmitral gradient subgroups (≤5, 5 to 10, and ≥10 mm Hg). Independent determinants of SPAP included the mean transmitral gradient, mitral regurgitation severity, left ventricular ejection fraction, and ≥moderate aortic stenosis (adjusted p <0.05), and atrial fibrillation and left atrial dimension. The impact of concomitant mitral regurgitation on SPAP decreased at higher transmitral gradients and was no longer significant at gradients ≥10 mm Hg (p = 0.100). In conclusion, SPAP strongly associates with mortality in MAC, independent of cardiovascular risk factors and indices of MAC-related MV dysfunction. These findings suggest an incremental role for SPAP in the risk stratification and prognostication in this increasingly prevalent condition with expanding the scope of possible interventions.
PMID: 34991846
ISSN: 1879-1913
CID: 5107372

Association of Pulmonary Artery Pulsatility Index With Adverse Cardiovascular Events Across a Hospital-Based Sample

Zern, Emily K; Wang, Dongyu; Rambarat, Paula; Bernard, Samuel; Paniagua, Samantha M; Liu, Elizabeth E; McNeill, Jenna; Wang, Jessica K; Andrews, Carl T; Pomerantsev, Eugene V; Picard, Michael H; Ho, Jennifer E
BACKGROUND:The pulmonary artery pulsatility index (PAPi), calculated from the ratio of the pulmonary artery pulse pressure to right atrial pressure, is a predictor of right ventricular failure after inferior myocardial infarction and left ventricular assist device implantation. Whether PAPi is associated with adverse outcomes across a heterogeneous population is unknown. METHODS:We examined consecutive patients undergoing right heart catheterization between 2005 and 2016 in a hospital-based cohort. Multivariable Cox models were utilized to examine the association between PAPi and all-cause mortality, major adverse cardiac events, and heart failure hospitalizations. RESULTS:<0.05 for all). CONCLUSIONS:Compared with the highest PAPi quartile, patients in PAPi quartiles 1 to 3 had a greater risk of all-cause mortality, major adverse cardiac events, and heart failure hospitalizations, with greatest risk observed in the lowest quartile. A low PAPi, even at values higher than previously reported, may serve an important role in identifying high-risk individuals across a broad spectrum of cardiovascular disease.
PMID: 35135302
ISSN: 1941-3297
CID: 5167172

Native mitral valve staphylococcus endocarditis with a very unusual complication: Ruptured posterior mitral valve leaflet aneurysm [Case Report]

Maidman, Samuel D; Kiefer, Nicholas J; Bernard, Samuel; Freedberg, Robin S; Rosenzweig, Barry P; Bamira, Daniel; Vainrib, Alan F; Ro, Richard; Neuburger, Peter J; Basu, Atreyee; Moreira, Andre L; Latson, Larry A; Loulmet, Didier F; Saric, Muhamed
Infective endocarditis (IE) is a life-threatening disease associated with in-hospital mortality of nearly one in five cases. IE can destroy valvular tissue, which may rarely progress to aneurysm formation, most commonly at the anterior leaflet in instances of mitral valve involvement. We present a remarkable case of a patient with IE and a rare complication of a ruptured aneurysm of the posterior leaflet of the mitral valve. Two- and Three-dimensional transesophageal echocardiography, intra-operative videography, and histopathologic analysis revealed disruption at this unusual location-at the junction of the P2 and P3 scallops, surrounded by an annular abscess.
PMID: 34923683
ISSN: 1540-8175
CID: 5108652

Cardiogenic shock complicating multisystem inflammatory syndrome following COVID-19 infection: a case report

Gurin, Michael I; Lin, Yue J; Bernard, Samuel; Goldberg, Randal I; Narula, Navneet; Faillace, Robert T; Alviar, Carlos L; Bangalore, Sripal; Keller, Norma M
BACKGROUND:With the high prevalence of COVID-19 infections worldwide, the multisystem inflammatory syndrome in adults (MIS-A) is becoming an increasingly recognized entity. This syndrome presents in patients several weeks after infection with COVID-19 and is associated with thrombosis, elevated inflammatory markers, hemodynamic compromise and cardiac dysfunction. Treatment is often with steroids and intravenous immunoglobulin (IVIg). The pathologic basis of myocardial injury in MIS-A, however, is not well characterized. In our case report, we obtained endomyocardial biopsy that revealed a pattern of myocardial injury similar to that found in COVID-19 cardiac specimens. CASE PRESENTATION:A 26-year-old male presented with fevers, chills, headache, nausea, vomiting, and diarrhea 5 weeks after his COVID-19 infection. His SARS-CoV-2 PCR was negative and IgG was positive, consistent with prior infection. He was found to be in cardiogenic shock with biventricular failure, requiring inotropes and diuretics. Given concern for acute fulminant myocarditis, an endomyocardial biopsy (EMB) was performed, showing an inflammatory infiltrate consisting predominantly of interstitial macrophages with scant T lymphocytes. The histologic pattern was similar to that of cardiac specimens from COVID-19 patients, helping rule out myocarditis as the prevailing diagnosis. His case was complicated by persistent hypoxemia, and a computed tomography scan revealed pulmonary emboli. He received IVIg, steroids, and anticoagulation with rapid recovery of biventricular function. CONCLUSIONS:MIS-A should be considered as the diagnosis in patients presenting several weeks after COVID-19 infection with severe inflammation and multi-organ involvement. In our case, EMB facilitated identification of MIS-A and guided therapy. The patient's biventricular function recovered with IVIg and steroids.
PMID: 34715788
ISSN: 1471-2261
CID: 5042902

Echocardiographic Features of COVID-19 Illness and Association with Cardiac Biomarkers [Letter]

Churchill, Timothy W; Bertrand, Philippe B; Bernard, Samuel; Namasivayam, Mayooran; Churchill, Jessica; Crousillat, Daniela; Davis, Esther F; Hung, Judy; Picard, Michael H
PMID: 32580898
ISSN: 1097-6795
CID: 4502332