Venoarterial Extracorporeal Membrane Oxygenation After Autologous Stem Cell Transplantation With Pancytopenia: JACC Patient Care Pathways.

Venoarterial extracorporeal membrane oxygenation (VA-ECMO) provides temporary mechanical circulatory support and simultaneous extracorporeal gas exchange for acute cardiorespiratory failure. By providing circulatory support, VA-ECMO gives treatments time to reach optimal efficacy or may be used as a bridge to a more durable mechanical solution for patients with acute cardiopulmonary failure. It is commonly used when a readily reversible etiology of decompensation is identified with very strict inclusion criteria for extracorporeal cardiopulmonary resuscitation use. We present a unique case in which VA-ECMO/extracorporeal cardiopulmonary resuscitation was used after cardiac arrest with pulseless electrical activity in a patient with recurrent lymphoma of the left thigh with recent autologous stem cell transplant.

Bicuspid aortic valve: long-term morbidity and mortality.

BACKGROUND AND AIMS: Bicuspid aortic valve (BAV) is the most common congenital heart anomaly. Lifetime morbidity and whether long-term survival varies according to BAV patient-sub-groups are unknown. This study aimed to assess lifetime morbidity and long-term survival in BAV patients in the community. METHODS: The authors retrospectively identified all Olmsted County (Minnesota) residents with an echocardiographic diagnosis of BAV from 1 January 1980 to 31 December 2009, including patients with typical valvulo-aortopathy (BAV without accelerated valvulo-aortopathy or associated disorders), and those with complex valvulo-aortopathy (BAV with accelerated valvulo-aortopathy or associated disorders). RESULTS: 652 consecutive diagnosed BAV patients [median (IQR) age 37 (22-53) years; 525 (81%) adult and 127 (19%) paediatric] were followed for a median (IQR) of 19.1 (12.9-25.8) years. The total cumulative lifetime morbidity burden (from birth to age 90) was 86% (95% CI 82.5-89.7); cumulative lifetime progression to ≥ moderate aortic stenosis or regurgitation, aortic valve surgery, aortic aneurysm ≥45 mm or z-score ≥3, aorta surgery, infective endocarditis and aortic dissection was 80.3%, 68.5%, 75.4%, 27%, 6% and 1.6%, respectively. Survival of patients with typical valvulo-aortopathy [562 (86%), age 40 (28-55) years, 86% adults] was similar to age-sex-matched Minnesota population (P = .12). Conversely, survival of patients with complex valvulo-aortopathy [90 (14%), age 14 (3-26) years, 57% paediatric] was lower than expected, with a relative excess mortality risk of 2.25 (95% CI 1.21-4.19) (P = .01). CONCLUSION: The BAV condition exhibits a high lifetime morbidity burden where valvulo-aortopathy is close to unavoidable by age 90. The lifetime incidence of infective endocarditis is higher than that of aortic dissection. The most common BAV clinical presentation is the typical valvulo-aortopathy with preserved expected long-term survival, while the complex valvulo-aortopathy presentation incurs higher mortality.

Venoarterial Extracorporeal Membrane Oxygenation After Autologous Stem Cell Transplantation With Pancytopenia: JACC Patient Care Pathways.

Venoarterial extracorporeal membrane oxygenation (VA-ECMO) provides temporary mechanical circulatory support and simultaneous extracorporeal gas exchange for acute cardiorespiratory failure. By providing circulatory support, VA-ECMO gives treatments time to reach optimal efficacy or may be used as a bridge to a more durable mechanical solution for patients with acute cardiopulmonary failure. It is commonly used when a readily reversible etiology of decompensation is identified with very strict inclusion criteria for extracorporeal cardiopulmonary resuscitation use. We present a unique case in which VA-ECMO/extracorporeal cardiopulmonary resuscitation was used after cardiac arrest with pulseless electrical activity in a patient with recurrent lymphoma of the left thigh with recent autologous stem cell transplant.

Mitral Annular Disjunction of Degenerative Mitral Regurgitation: Three-Dimensional Evaluation and Implications for Mitral Repair.

BACKGROUND: The dynamic consequences of mitral annular disjunction (MAD) on the mitral apparatus and the left ventricle remain unclear and are crucial in the context of mitral surgery. Thus, the aim of this study was to assess mitral valvular, annular, and ventricular dynamics in mitral valve prolapse (MVP) stratified by presence of MAD. METHODS: In 61 patients (mean age, 62 ± 11 years; 25% women) with MVP and severe mitral regurgitation undergoing mitral surgery between 2009 and 2016, valvular and annular dimensions and dynamics by two-dimensional transthoracic and three-dimensional transesophageal echocardiography and left ventricular dimensions and dynamics were analyzed stratified by presence of MAD before and after surgery. RESULTS: MAD (mean, 8 ± 3 mm) was diagnosed in 27 patients (44%; with a mean effective regurgitant orifice area of 0.55 ± 0.20 cm2 and similar to patients without MAD), more frequently in bileaflet prolapse (52% vs 18% in patients without MAD, P = .004), consistently involving P2 (P = .005). Patients with MAD displayed larger diastolic annular areas (mean, 1,646 ± 410 vs 1,380 ± 348 mm2), circumferences (mean, 150 ± 19 vs 137 ± 16 mm), and intercommissural diameters (mean, 48 ± 7 vs 43 ± 6 mm) compared with those without MAD (P ≤ .008 for all). Dynamically, mid- and late systolic excess intercommissural diameter, annular area, and circumference enlargement were associated with MAD (P ≤ .01 for all). MAD was also associated with dynamically annular slippage, larger prolapse volume and height (P ≤ .007), and larger leaflet area (mean, 2,053 ± 620 vs 1,692 ± 488 mm2, P = .01). Although patients with MAD compared with those without MAD showed similar ejection fractions (mean, 65 ± 5% vs 62 ± 8%, respectively, P = .10), systolic basal posterior thickness was increased in patients with MAD (mean, 19 ± 2 vs 15 ± 2 mm, P < .001), with higher systolic thickening of the basal posterior wall (mean, 74 ± 27% vs 50 ± 28%) and higher ratio of basal wall thickness to diameter (P ≤ .01 for both). However, after mitral repair, MAD disappeared, and LV diameter, wall thickness, and wall thickening showed no difference between patients with MAD and those without MAD (P ≥ .10 for all). CONCLUSIONS: MAD in patients with MVP involves a predominant phenotype of bileaflet MVP and causes profound annular dynamic alterations with considerable expansion and excess annular enlargement in systole, potentially affecting leaflet coaptation. MAD myocardial and annular slippage simulates vigorous left ventricular function without true benefit after surgical annular suture. Thus, although MAD does not hinder the feasibility and quality of valve repair, it requires careful suture of ring to ventricular myocardium, lest it persist postoperatively.

Reduction in Right Atrial Pressures Is Associated With Hemodynamic Improvements After Transcatheter Edge-to-Edge Repair of the Tricuspid Valve.

BACKGROUND: Investigational transcatheter edge-to-edge repair (TEER) for severe tricuspid regurgitation (TR) has shown promise as an alternative to surgery, but factors influencing outcomes, optimal patient selection, and procedural timing remain incompletely defined. Given the limitations of determining TR severity by conventional echocardiography, our objectives were to determine whether invasive right atrial (RA) pressures performed during the procedure are related to patient outcomes. METHODS: This study was a retrospective review of patients who underwent off-label tricuspid TEER using MitraClip (Abbott Vascular, Menlo Park, CA) for significant TR at a single institution. Intraprocedural mean RA pressure, RA peak V-wave, RA pressure nadir, and systolic increase in RA pressure (XV height) were recorded. RESULTS: Thirty-eight patients underwent tricuspid TEER; 33 underwent concomitant mitral TEER for mitral regurgitation. The study cohort was 39% female with a mean age of 78.6±14.3 years. Median follow-up was 339 days (interquartile range, 100-601). Any reduction in mean RA pressure, RA peak V-wave, RA nadir, and XV height occurred in 74%, 82%, 45%, and 87% of patients, respectively. At 1 year, event-free survival was 47%. Postprocedure XV height correlated with TR severity as determined by echocardiography (P<0.0001). The highest quartile of postprocedure XV height (>8 mm Hg) had worse event-free survival compared with those who had concluding XV height ≤8 mm Hg (P=0.02). Attainment of a concluding XV height less than or equal to median value was associated with a lower creatinine the next day (1.27±0.47 versus 1.64±0.47 mg/dL, P=0.04). CONCLUSIONS: Intraprocedural XV height correlates with TR severity after tricuspid TEER, and lower concluding pressures are associated with improved outcomes. Analysis of RA pressures may serve as a complementary tool for the evaluation of disease severity and procedural guidance.

Noninvasive Hemodynamic Assessment of Shock Severity and Mortality Risk Prediction in the Cardiac Intensive Care Unit.

OBJECTIVES: This study sought to define the 2-dimensional and Doppler echocardiographic hemodynamics associated with each Society for Cardiovascular Angiography and Interventions (SCAI) stage, and to determine their association with mortality. BACKGROUND: The SCAI shock stages classification stratifies mortality risk in cardiac intensive care unit (CICU) patients, but the echocardiographic and hemodynamic parameters that define these SCAI shock stages are unknown. METHODS: Unique CICU patients admitted from 2007 to 2015 who had a transthoracic echocardiogram within 1 day of CICU admission were included. Echocardiographic variables were evaluated as a function of SCAI shock stage. Multivariable logistic regression determined the association between echocardiographic parameters with adjusted hospital mortality. RESULTS: We included 5,453 patients with a median age of 69.3 years (interquartile range: 58.2 to 79.0 years) (37% women), and a median left ventricular ejection fraction (LVEF) of 50% (interquartile range: 35% to 61%). Higher SCAI shock stages were associated with lower LVEF and worse systemic hemodynamics. Hospital mortality was higher in patients with LVEF <40%, cardiac index <1.8 l/min/m2, stroke volume index <35 ml/m2, cardiac power output <0.6 W, or medial early mitral valve inflow velocity to early diastolic annular velocity (E/e') ratio >15 (particularly in SCAI shock Stages A to C). After multivariable adjustment, only stroke volume index <35 ml/m2 (adjusted odds ratio: 2.0; 95% confidence interval: 1.4 to 3.0; p < 0.001) and E/e' ratio >15 (adjusted odds ratio: 1.52; 95% confidence interval: 1.04 to 2.23; p = 0.03) remained associated with higher hospital mortality. CONCLUSIONS: Noninvasive 2-dimensional and Doppler echocardiographic parameters correlate with the SCAI shock stages and improve risk stratification for hospital mortality in CICU patients. Low stroke volume index and high E/e' ratio demonstrated the strongest association with hospital mortality.

What Is New in Low Gradient Aortic Stenosis: Surgery, TAVR, or Medical Therapy?

BACKGROUND: A significant proportion of patients with aortic stenosis (AS) have discordance in severity by mean gradient/peak velocity and aortic valve area. Low gradient aortic stenosis (LG-AS) is defined when the aortic valve area is < 1 cm2 consistent with severe AS and mean aortic gradient is < 40 mmHg consistent with non-severe AS. LG-AS represents a diagnostic and therapeutic challenge. PURPOSE OF REVIEW: To summarize the different categories, diagnosis, management, and prognosis of LG-AS. LG-AS is classified as classical (ejection fraction (EF) < 50%, indexed stroke volume (SVi) < 35 ml/m2), paradoxical (EF > 50%, SVi < 35 ml/m2), pseudo-severe (moderate AS with reduced EF), or normal flow low gradient AS. RECENT FINDINGS: Recent findings emphasize the importance of low-dose dobutamine stress echocardiography and CT calcium score in the assessment of aortic valve. In addition, flow reserve (increase in SV > 50%) can be evaluated during dobutamine stress echocardiography and helps predict perioperative prognosis. Patients with LG-AS have better survival with aortic valve replacement (AVR) compared to medical therapy, irrespective of presence or absence of flow reserve. Some recent studies suggest that transcatheter aortic valve replacement (TAVR) may be superior to surgical AVR for patients with a lack of contractile flow reserve or those with paradoxical LG-AS, but further investigation is needed to clarify optimal treatment. The role of TAVR in patients with moderate AS and reduced EF is also under investigation.

Guidelines for the Use of Transesophageal Echocardiography to Assist with Surgical Decision-Making in the Operating Room: A Surgery-Based Approach: From the American Society of Echocardiography in Collaboration with the Society of Cardiovascular Anesthesiologists and the Society of Thoracic Surgeons.

Intraoperative transesophageal echocardiography is a standard diagnostic and monitoring tool employed in the management of patients undergoing an entire spectrum of cardiac surgical procedures, ranging from "routine" surgical coronary revascularization to complex valve repair, combined procedures, and organ transplantation. Utilizing a protocol as a starting point for imaging in all procedures and all patients enables standardization of image acquisition, reduction in variability in quality of imaging and reporting, and ultimately better patient care. Clear communication of the echocardiographic findings to the surgical team, as well as understanding the impact of new findings on the surgical plan, are paramount. Equally important is the need for complete understanding of the technical steps of the surgical procedures being performed and the complications that may occur, in order to direct the postprocedure evaluation toward aspects directly related to the surgical procedure and to provide pertinent echocardiographic information. The rationale for this document is to outline a systematic approach describing how to apply the existing guidelines to questions on cardiac structure and function specific to the intraoperative environment in open, minimally invasive, or hybrid cardiac surgery procedures.

Institutional learning experience for combined edge-to-edge tricuspid and mitral valve repair.

BACKGROUND: Transcatheter edge-to-edge repair with MitraClip is only approved for treatment of mitral regurgitation but is increasingly used to treat concomitant tricuspid regurgitation (TR) due to its common coexistence and association with poor outcomes. This study aimed to describe the learning curve associated with the challenge of off-label treatment of concomitant TR. METHODS: This is a retrospective review of initial and consecutive patients who underwent combined edge-to-edge repair of mitral and tricuspid valves (TVs) at our institution from August 2017 to October 2019. RESULTS: Repair of both valves with MitraClip was performed in 22 patients (median age 81.5 years, 32% female). Mean procedure time was 176 ± 47 min; mean fluoroscopy time was 65 ± 24 min. Procedure duration in the first tertile was significantly longer (223 ± 13 min) than in the third tertile (143 ± 23 min, p = .0003). Median number of total clips placed per case was 3; in 15 patients (68%), the anterior and septal leaflets of the TV were clipped. The average changes in mean right atrial (RA) and left atrial (LA) pressures were -1.7 ± 2.5 mmHg (p = .0080) and -3.2 ± 4.6 mmHg (p = .0045), respectively. The average changes in RA and LA V-wave heights were -3.3 ± 4.0 mmHg (p = .0009) and -8.1 ± 9.9 mmHg (p = .038), respectively. There was a significant trend toward decreasing residual TR over the course of the series (p = .046). At 30 days, survival was 100% and mean NYHA class decreased from 2.8 to 1.8 (p < .0001). CONCLUSIONS: Combined edge-to-edge tricuspid and mitral valve repair is safe and feasible. With experience, procedure duration and residual TR decreased.

Diastolic Blood Pressure and Heart Rate Are Independently Associated With Mortality in Chronic Aortic Regurgitation.

BACKGROUND: The prognostic significance of diastolic blood pressure (DBP) and resting heart rate (RHR) in patients with hemodynamically significant aortic regurgitation (AR) is unknown. OBJECTIVES: This study sought to investigate the association of DBP and RHR with all-cause mortality in patients with AR. METHODS: Consecutive patients with ≥ moderate to severe AR were retrospectively identified from 2006 to 2017. The association between all-cause mortality and routinely measured DBP and RHR was examined. RESULTS: Of 820 patients (age 59 ± 17 years; 82% men) followed for 5.5 ± 3.5 years, 104 died under medical management, and 400 underwent aortic valve surgery (AVS). Age, symptoms, left ventricular ejection fraction (LVEF), LV end-systolic diameter-index (LVESDi), DBP, and RHR were univariable predictors of all-cause mortality (all p ≤ 0.002). When adjusted for demographics, comorbidities, and surgical triggers (symptoms, LVEF, and LVESDi), baseline DBP (adjusted-hazard ratio [HR]: 0.79 [95% confidence interval: 0.66 to 0.94] per 10 mm Hg increase, p = 0.009) and baseline RHR (adjusted HR: 1.23 [95% confidence interval: 1.03 to 1.45] per 10 beat per min [bpm] increase, p = 0.01) were independently associated with all-cause mortality. These associations persisted after adjustment for presence of hypertension, medications, time-dependent AVS, and using average DBP and RHR (all p ≤ 0.02). Compared with the general population, patients with AR exhibited excess mortality (relative risk of death >1), which rose steeply in inverse proportion (p nonlinearity = 0.002) to DBP starting at 70 mm Hg and peaking at 55 mm Hg and in direct proportion to RHR starting at 60 bpm. CONCLUSIONS: In patients with chronic hemodynamically significant AR, routinely measured DBP and RHR demonstrate a robust association with all-cause death, independent of demographics, comorbidities, guideline-based surgical triggers, presence of hypertension, and use of medications. Therefore, DBP and RHR should be integrated into comprehensive clinical decision-making for these patients.

Quantitative Three-Dimensional Echocardiographic Correlates of Optimal Mitral Regurgitation Reduction during Transcatheter Mitral Valve Repair.

BACKGROUND: Patient selection for transcatheter edge-to-edge mitral valve repair (TMVR) remains challenging because of heterogenous mitral valve pathology and highly variable anatomy. The aim of this study was to investigate whether quantitative three-dimensional (3D) transesophageal echocardiographic modeling parameters are associated with optimal mitral regurgitation (MR) reduction in patients undergoing TMVR. METHODS: Fifty-nine patients underwent 3D transesophageal echocardiography during TMVR. Volumetric data sets were retrospectively analyzed using mitral valve quantitative 3D modeling software (Mitral Valve Navigator). Optimal MR reduction was defined as less than moderate residual MR. Logistic regression was used to correlate 3D transesophageal echocardiographic quantitative data to procedural success. RESULTS: Thirty-five patients had primary MR, 24 had mixed or secondary MR, and all patients had grade ≥ 3/4 MR before the procedure. Optimal MR reduction was achieved in 40 of 59 patients (68%). Univariate correlates of optimal MR reduction in patients with primary MR were lower mitral leaflet tenting volume (P = .049) and lower tenting height (P = .025); tenting height < 3 mm and tenting volume < 0.7 mL were associated with increased likelihood of optimal MR reduction (92% vs 48% [P = .01] and 81% vs 47% [P = .03], respectively). In mixed or secondary MR, annular height ≥ 5.5 mm was associated with increased likelihood of optimal MR reduction (94% vs 38%; P = .03). During follow-up, redo TMVR or surgical mitral valve replacement occurred exclusively in patients with suboptimal anatomy defined by 3D transesophageal echocardiography (10% vs 0%, P = .045). CONCLUSIONS: Quantitative 3D echocardiographic data are associated with favorable response to TMVR and could help optimize patient selection.

Prognostic Implications of Left Atrial Enlargement in Degenerative Mitral Regurgitation.

BACKGROUND: Left atrial enlargement is frequent in degenerative mitral regurgitation (DMR), but its link to outcomes remains unproven in routine clinical practice. OBJECTIVES: The purpose of this study was to assess whether left atrial volume index (LAVI) measured in routine clinical practice of multiple sonographers/cardiologists is associated independently with DMR survival. METHODS: A cohort of 5,769 (63 ± 16 years, 47% women) consecutive patients with degenerative mitral valve disease, in whom LAVI was prospectively measured, was enrolled and the long-term survival was analyzed. RESULTS: LAVI (43 ± 24 ml/m2) was widely distributed (<40 ml/m2 in 3,154 patients, 40 to 59 ml/m2 in 1,606, and ≥60 ml/m2 in 1,009). Overall survival throughout follow-up (10-year 66 ± 1%) was strongly associated with LAVI (79 ± 1% vs. 65 ± 2% and 54 ± 2% for LAVI <40, 40 to 59, and ≥60 ml/m2, respectively; p < 0.0001) even after comprehensive adjustment, including for DMR severity (adjusted hazard ratio [HR]: 1.05 [95% confidence interval (CI): 1.03 to 1.08] per 10 ml/m2; p < 0.0001). Mortality under medical management was profoundly affected by LAVI (adjusted HR: 1.07 [95% CI: 1.04 to 1.10] per 10 ml/mm2 and 1.55 [95% CI: 1.31 to 1.84] for LAVI ≥60 ml/m2 vs. <40 ml/m2; both p < 0.0001) incrementally to adjusting variables (p < 0.0001) and in all subgroups, particularly sinus rhythm (adjusted HR: 1.25 [95% CI: 1.21 to 1.28]) or atrial fibrillation (adjusted HR: 1.10 [95% CI: 1.06 to 1.13] per 10 ml/m2; both p < 0.0001). Thresholds of excess mortality in spline curve analysis were approximated at 40 ml/m2 in all subgroups. Survival markedly improved after mitral surgery (time-dependent adjusted HR: 0.43 [95% CI: 0.36 to 0.53]; p < 0.0001) but remained modestly linked to LAVI (10-year survival 85 ± 3% vs. 86 ± 2% and 75 ± 3% for LAVI <40, 40 to 59, and ≥60 ml/m2, respectively; p < 0.0001). CONCLUSIONS: The frequent left atrial enlargement of DMR as measured by LAVI in routine practice displays, overall and in all subsets, a powerful, incremental, and independent link to excess mortality, which is partially alleviated by mitral surgery. Hence, LAVI measurement should be part of routine DMR evaluation and the clinical decision-making process.

Coexistent bicuspid aortic valve and mitral valve prolapse: epidemiology, phenotypic spectrum, and clinical implications.

AIMS: Bicuspid aortic valve (BAV) and mitral valve prolapse (MVP) are common but the prevalence and significance of coexistent conditions are unknown. This study investigated the prevalence, phenotypic expression, and clinical significance of coexistent MVP-BAV. METHODS AND RESULTS: Retrospective comparison of MVP-BAV and MVP-tricuspid aortic valve (TAV) prevalence including de novo echocardiographic analysis of all MVP-BAV patients between 2005 and 2015 was performed. The community prevalence of MVP-BAV was 2.7% vs. 3.4% for MVP-TAV (P = 0.45). Posterior mitral leaflet (PML)-MVP was the most common phenotype in both BAV and TAV (P = 0.38), but anterior mitral leaflet (AML)-MVP was twice more prevalent in BAV (31% vs. 15%, P < 0.0001). Among 130 subjects with coexistent MVP-BAV (81% men, 51 ± 16 years old), 31 (24%) exhibited AML:PML length ratio ≥3:1, termed large-AML prolapse (LAP-BAV), who had predominant BAV regurgitation when compared with those with non-LAP-BAV (P ≤ 0.001). An extreme phenotype of LAP-BAV with giant-AML prolapse and diminutive PML (GAP-BAV) was identified in 18/130 (14%) subjects. Compared with posterior-MVP-BAV, GAP-BAV patients were younger (42 ± 15 vs. 64 ± 12 years, P < 0.0001), had larger aortic annulus (28 ± 3 vs. 26 ± 2 mm, P = 0.01), and 61% had ≥ moderate BAV regurgitation (vs. 16%, P = 0.0007). Mitral repair occurred in 37/130 (28%) subjects. After median follow-up 5.5 months (4-83), 4/5 (80%) GAP-BAV patients required redo surgery for recurrent mitral regurgitation vs. 2/31 (6%) for non-LAP-BAV (P = 0.001). CONCLUSION: The community prevalence of coexistent MVP-BAV is comparable to MVP-TAV and their most common phenotype is posterior-MVP. However, anterior-MVP is twice as prevalent in MVP-BAV. A large-AML phenotype (LAP-BAV) with predominant BAV regurgitation affects 24% of MVP-BAV patients. An extreme phenotype of anterior-MVP (GAP-BAV) affects 14% of BAV patients; characterized by exceptionally large AML, diminutive PML, high mitral and aortic regurgitation prevalence, and high mitral repair failure rate.

Echocardiography in Sarcoidosis.

PURPOSE OF REVIEW: Cardiac sarcoidosis (CS) is associated with significant morbidity and mortality. The diagnosis of CS is challenging and typically one that is only entertained after many other conditions have been ruled out. A high index of suspicion is necessary in order to correctly determine appropriate testing for the disease. Transthoracic echocardiography is the most readily available imaging modality available to help establish a diagnosis in a potential patient. However, no one echocardiographic feature is pathognomonic. RECENT FINDINGS: On echocardiography, unusual wall motion abnormalities, which do not fit a classic coronary distribution, along with diastolic dysfunction may alert one to the presence of cardiac sarcoid, particularly in the right clinical context. Myocardial strain imaging on echocardiography may increase the sensitivity of identifying cardiac sarcoidosis. Alternative imaging with cardiac magnetic resonance imaging or positron emission tomography have become more frequently utilized to establish a diagnosis of CS. Cardiac sarcoidosis remains a difficult condition to diagnose. However early diagnosis is critical to decrease the associated high mortality. Endomyocardial biopsy is highly specific but lacks sensitivity due to the patchy nature of the granulomatous deposition. Thus, imaging plays a role in diagnosis as well as for follow-up. Echocardiography remains an hallmark during the workup for CS. Decreased sensitivity of echocardiography has facilitated the use of other techniques to establish the presence of CS.

Three-Dimensional Echocardiographic Assessment of Mitral Annular Physiology in Patients With Degenerative Mitral Valve Regurgitation Undergoing Surgical Repair: Comparison between Early- and Late-Stage Severe Mitral Regurgitation.

BACKGROUND: Ventricular-annular decoupling is thought to exist in all degenerative myxomatous mitral valve (MV) diseases. However, the annular physiology of degenerative MV disease may differ when severe mitral regurgitation (MR) presents at different stages. The aim of this study was to assess differences in mitral annular physiology and surgical effects between early- and late-stage severe MR. METHODS: Three-dimensional (3D) transesophageal echocardiography was performed before and after MV surgery in 74 patients with degenerative MV disease, including 57 with early-stage severe MR (without left ventricular remodeling) and 17 with late-stage MR (with left ventricular remodeling). A control group comprised 46 patients without MV disease. Novel 3D MV software was used to evaluate mitral annular dynamics. The degree of annular saddle shape was calculated as the ratio of annular height (AH) to lateromedial diameter (LM). Ventricular-annular decoupling was defined as insufficient systolic AH/LM compared with the control group. RESULTS: Prebypass 3D measurements demonstrated that systolic AH/LM in the early-stage group (0.19 ± 0.04) was similar to that in the control group (0.21 ± 0.05; P = .101), while systolic AH/LM in the late-stage group (0.17 ± 0.04) was lower than that in the control group (P = .011). Postbypass comparison showed saddle shape accentuation in the early-stage group (0.20 ± 0.04), similar to that in the control group (P = .3127); the mitral annulus remained flat in the late-stage group (0.17 ± 0.03; P = .004). CONCLUSIONS: Ventricular-annular decoupling, present in the late-stage group, was absent in the early-stage group. MV repair surgery did not disrupt mitral annular saddle shape in the early-stage group; however, it failed to correct annular dysfunction in the late-stage group. Sequential 3D transesophageal echocardiographic analysis provides comprehensive mitral annular evaluation beyond conventional two-dimensional parameters for determining stages of severe MR.

Hypereosinophilic syndrome: cardiac diagnosis and management.

Hypereosinophilic syndrome (HES) is a heterogeneous group of conditions that is defined at its core by hypereosinophilia (HE) (blood eosinophil count of >1.5×10(9)/L) and organ damage directly attributable to the HE. Cardiac dysfunction occurs frequently in all forms of HES and is a major cause of morbidity and mortality. Once a significantly elevated eosinophil count is identified, it must be confirmed on repeat testing and the aetiology for the HE must be rigorously sought out with a focus on identifying whether organ dysfunction is occurring. Echocardiography is routinely performed to assess for cardiac involvement, looking for evidence of left ventricular and/or right ventricular apical obliteration or thrombi or a restrictive cardiomyopathy. Cardiac magnetic resonance imaging and CT are often useful adjuncts to establish the diagnosis but endomyocardial biopsy remains the gold standard. To decrease the degree of eosinophilia, treatment can include corticosteroids and/or imatinib based on the aetiology. Anticoagulation, standard heart failure therapy for a restrictive cardiomyopathy and finally cardiac transplantation may be indicated in the treatment algorithm.

Dynamic phenotypes of degenerative myxomatous mitral valve disease: quantitative 3-dimensional echocardiographic study.

BACKGROUND: Fibro-elastic deficiency (FED) and diffuse myxomatous degeneration (DMD) are phenotypes of degenerative mitral valve disease defined morphologically. Whether physiological differences in annular and valvular dynamics exist between these phenotypes remains unknown. METHODS AND RESULTS: We performed triple quantitation of cardiac remodeling and of mitral regurgitation severity and of annular and valvular dimensions by real-time 3-dimensional-transesophageal-echocardiography. Forty-nine patients with degenerative mitral valve disease classified as FED (n=31) and DMD (n=18) by surgical observation showed no difference in age (65±10 versus 59±13; P=0.5), body surface area (2.0±0.2 versus 2.0±0.2 m(2); P=0.5), left ventricular and atrial dimensions (all P>0.55), and mitral regurgitation regurgitant orifice (P=0.62). On average, annular dimensions were larger in DMD versus FED, but height was similar resulting in lower saddle shape. Dynamically, annular DMD versus FED display poorer contraction and saddle-shape accentuation in early systole and abnormal enlargement, particularly intercommissural, in late-systole (all P<0.05). Valvular dynamics showed stable valvular area in systole in FED versus considerable systolic increased area in DMD (P<0.001). Prolapse height and volume increased little throughout systole in FED versus marked increase in DMD (P<0.001). CONCLUSIONS: Our novel observations show that FED and DMD, although both labeled myxomatous, display considerable physiological phenotypic differences. In DMD, the annular increased size and profoundly abnormal dynamics demonstrate DMD-specific annular degeneration compared with the enlarged but relatively normal FED annulus. DMD does not incur more severe mitral regurgitation, despite larger prolapse and valve redundancy, underscoring potential compensatory role of tissue redundancy of DMD (or aggravating role of tissue paucity of FED) on mitral regurgitation severity.

Cleft-like indentations in myxomatous mitral valves by three-dimensional echocardiographic imaging.

OBJECTIVES: Cleft-like indentations (CLI) are deep separations between scallops of the mitral posterior leaflet observed in myxomatous mitral valve disease (MMVD), but their diagnosis, mechanisms and implications are unknown. Using 3D transoesophageal echocardiography (3DTOC), we aimed at assessing diagnostic accuracy and defining mechanisms of CLI in patients undergoing surgery for MMVD. METHODS: 3DTOC of mitral valve was acquired in 49 patients with MMVD and severe regurgitation prior to valve repair. Qualitative review compared 3DTOC diagnosis of CLI with surgical inspection. Mitral, annular and leaflet dimensions were quantified with dedicated software and compared between those with and without CLI. RESULTS: Diagnosis of CLI was made by 3DTOC in 17 (35%) while none was identified by 2D and was confirmed in 15 (88%) by surgical inspection. Mechanistically, LV diameters and mitral regurgitant volume (RVol) were similar with and without CLI (p>0.49). Conversely, mitral annulus was smaller with CLI (anteroposterior diameter 42.2±7.1 vs 47.0±7.5 mm, p=0.04; circumference 133±16 vs 148±19 mm, p=0.009; area 1289±326 vs 1619±427 mm(2), p=0.008). Prolapse volume tended to be smaller with CLI (1.9±1.2 vs 4.0±4.3 mL, p=0.06) involving single posterior scallop at surgery (82% vs 44%, p=0.007) with smaller 3DTOC leaflet area (1574±409 vs 2019±652 mm(2), p=0.01). During valve repair, surgical closure of all surgically diagnosed CLI was required. CONCLUSIONS: Posterior leaflet CLI are frequent in MMVD, are identified by 3DTOC with high accuracy and require closure during valve repair. CLI are mechanistically not related to excess annular enlargement or excess prolapse. Conversely, CLI occur in the context of single scallop prolapse with tissue paucity causing excess separation of scallops. These 3DTOC data enhance diagnostic and mechanistic comprehension of the diversity of MMVD phenotypical presentation.

Interventional echocardiography.

Echocardiography guidance for interventions in the catheterization laboratory allows for reduction in radiation exposure from fluoroscopy as well as superior anatomic definition and visualization. The additional information provided over fluoroscopy has translated into an increasing use during interventional procedures. Procedures such as transeptal puncture, percutaneous valvular interventions, myocardial biopsy, echo-guided pericardiocentesis and other interventions have evolved to a complexity level that requires combined echocardiographic and fluoroscopic guidance. Different imaging modalities are utilized in the catheterization laboratory including intracardiac echocardiography, two-dimensional (2D) or three-dimensional (3D) transthoracic echocardiography, and 2D or 3D transesophageal echocardiography. This review is intended to provide an overall summary of the impact echocardiography has had in the catheterization laboratory. We will describe how echocardiography is utilized to guide a diverse array of interventional procedures, emphasizing specific practical issues with respect to echocardiographic guidance of interventional procedures and also pointing out the limitations of echocardiography.