Garden-Hose Mitral Regurgitation: A Variant That Can Result in Underestimation of Severity: A Multimodality Imaging Case Study.

The quantitative assessment of mitral regurgitation (MR) by echocardiography has limitations. Cardiac magnetic resonance (CMR) imaging has an emerging role in the quantitation of MR, and preliminary studies indicate that CMR assessment may more accurately quantify MR and better correlate with postsurgical left ventricular reverse remodeling. The authors here report a case of MR in which multimodality imaging with CMR and transesophageal echocardiography was crucial in accurately diagnosing the severity of MR when transthoracic and provocative supine bike echocardiography underestimated the degree of MR in a unique variant known as "garden-hose" MR.

A Case of Transient Mitral Regurgitation: Not Everything Is Always What It Seems.

Mitral regurgitation (MR) is a common form of valvular heart disease that is associated with significant morbidity and mortality. MR can be broadly classified into 2 different categories: primary and secondary MR. Primary MR usually is caused by leaflet abnormalities, whereas secondary MR is a chronic disease secondary to geometric distortion of both the annulus and subvalvular apparatus because of left ventricular remodeling. Without acute changes in loading conditions, myocardial blood flow, or rhythm disturbances, functional MR typically is not transient. In this E-Challenge, the authors show a transient and completely reversible acute and severe form of functional MR with the use of multimodal echocardiography.

Tissue Doppler Imaging (E/e') and Pulmonary Capillary Wedge Pressure in Patients With Severe Aortic Stenosis.

OBJECTIVE: Although American and European consensus statements advocate using the ratio of the transmitral E velocity and tissue Doppler early diastolic mitral annular velocity (E/e') in the assessment of left-sided heart filling pressures, recent reports have questioned the reliability of this ratio to predict left atrial pressures in a variety of disease states. The authors hypothesized that there is a clinically significant correlation between E/e' and pulmonary capillary wedge pressure (PCWP) in patients with severe aortic stenosis. DESIGN: Retrospective cohort study. PARTICIPANTS: The study comprised 733 consecutive patients with severe aortic stenosis who underwent transcatheter aortic valve replacement for severe aortic stenosis. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: PCWP and E/e'ave (average of the lateral and medial annulus tissue Doppler velocities) were measured with a pulmonary artery catheter and transthoracic echocardiography during preprocedural evaluation. Patients were grouped by left ventricular ejection fraction (LVEF) ≥50% and LVEF <50%. Spearman rank correlation, analysis of variance, and t and chi-square tests were used to analyze the data. Seventy-nine patients met the inclusion criteria. There was no significant correlation between E/e'ave and PCWP (n = 79, Spearman r = 0.096; p = 0.3994). This correlation did not improve when ventricular function was considered (LVEF <50%: n = 11, Spearman r = -0.097; p = 0.776 and LVEF ≥50%: n = 68, Spearman r = 0.116; p = 0.345). There was no statistically significant difference in mean PCWP between each range of E/e'ave. CONCLUSION: A clinically relevant relationship between E/e' and PCWP was not observed in patients with severe aortic stenosis.

Structural abnormalities in hypertrophic cardiomyopathy beyond left ventricular hypertrophy by multimodality imaging evaluation.

BACKGROUND: Echocardiography- and cardiovascular magnetic resonance (CMR)-based studies have revealed a wide range of phenotypic manifestations in hypertrophic cardiomyopathy (HCM) apart from hypertrophy. This study sought to comprehensively describe a number of structural abnormalities in HCM beyond hypertrophy, by multimodality imaging. METHODS: A total of 100 HCM patients were prospectively enrolled, undergoing standard and contrast echocardiography, and CMR. Morphological characteristics involving mitral valve leaflets (MVL), subvalvular apparatus, and left ventricular cavity and wall were investigated. Seventy healthy volunteers served as control population. RESULTS: As assessed by echo, MVLs were longer in HCM patients than in controls (anterior method 1: 24[22,28] vs 19[18,20] mm, P < 0.01; anterior method 2: 27[24, 29] vs 21[19, 23] mm, P < 0.01; posterior: 15[12,19] vs 14[13,15] mm, P < 0.01). Abnormal chordal attachment to anterior MVL, anterior papillary muscle displacement, and accessory apical-basal muscle bundle were present in 42 (42%), 61 (61%), and 35 (35%) patients, respectively (P values vs controls <0.01); direct papillary muscle insertion into MVL and hypertrabeculation were found in two and five patients, respectively. Contrast echocardiography (n = 94) detected myocardial crypts in 15 patients (16%). Overall, 83% of HCM subjects had at least one of these phenotypic manifestations. Echocardiography and CMR agreement for MVL length was poor, while for structural characteristics was moderate to substantial (Cohen's Kappa: 0.53-1.00). Except for posterior MVL length and hypertrabeculation, the phenotypic characteristics studied had acceptable reproducibility by echocardiography and CMR. CONCLUSIONS: Structural abnormalities in HCM beyond hypertrophy are significantly common. Multimodality imaging approach to these HCM facets by echocardiography and CMR is feasible and desirable.

Late Clinical Presentation of Prosthesis-Patient Mismatch Following Transcatheter Aortic Valve Replacement.

Prosthesis-patient mismatch (PPM) is relatively common after aortic valve replacement (AVR) and generally is associated with reduced regression of left ventricular (LV) mass. PPM after valve-in-valve transcatheter aortic valve replacement (TAVR) was reported to be 38%. PPM generally is manifested clinically by dyspnea and echocardiographically by high transvalvular gradients. In this E-Challenge, the authors will review a case of a late clinical presentation of PPM 1-year following a valve-in-valve TAVR.

Underappreciated occurrence of discrete subaortic membranes producing left ventricular outflow obstruction in hypertrophic cardiomyopathy.

Subaortic obstruction due to systolic anterior motion (SAM) of the mitral valve with ventricular septal contact is a major cause of progressive heart failure symptoms in patients with hypertrophic cardiomyopathy (HCM). However, we have recently observed a unique, but not uncommon subgroup of HCM patients with outflow tract obstruction due only to discrete subaortic membrane or who have a membrane in addition to SAM-septal contact. HCM patients with subaortic membranes may be at increased risk for developing progressive heart failure symptoms. Identification requires a high index of suspicion and raises important management considerations, including need for surgical myectomy for definitive relief of obstruction with associated excellent outcomes, as the alternative invasive treatment option with percutaneous alcohol septal ablation would be ineffective.

Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association.

BACKGROUND: Acute rheumatic fever remains a serious healthcare concern for the majority of the world's population despite its decline in incidence in Europe and North America. The goal of this statement was to review the historic Jones criteria used to diagnose acute rheumatic fever in the context of the current epidemiology of the disease and to update those criteria to also take into account recent evidence supporting the use of Doppler echocardiography in the diagnosis of carditis as a major manifestation of acute rheumatic fever. METHODS AND RESULTS: To achieve this goal, the American Heart Association's Council on Cardiovascular Disease in the Young and its Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee organized a writing group to comprehensively review and evaluate the impact of population-specific differences in acute rheumatic fever presentation and changes in presentation that can result from the now worldwide availability of nonsteroidal anti-inflammatory drugs. In addition, a methodological assessment of the numerous published studies that support the use of Doppler echocardiography as a means to diagnose cardiac involvement in acute rheumatic fever, even when overt clinical findings are not apparent, was undertaken to determine the evidence basis for defining subclinical carditis and including it as a major criterion of the Jones criteria. This effort has resulted in the first substantial revision to the Jones criteria by the American Heart Association since 1992 and the first application of the Classification of Recommendations and Levels of Evidence categories developed by the American College of Cardiology/American Heart Association to the Jones criteria. CONCLUSIONS: This revision of the Jones criteria now brings them into closer alignment with other international guidelines for the diagnosis of acute rheumatic fever by defining high-risk populations, recognizing variability in clinical presentation in these high-risk populations, and including Doppler echocardiography as a tool to diagnose cardiac involvement.

Preoperative three-dimensional echocardiography to assess risk of right ventricular failure after left ventricular assist device surgery.

BACKGROUND: Right ventricular failure (RVF) is associated with significant morbidity after left ventricular assist device (LVAD) surgery. Hemodynamic, clinical, and 2-dimensional echocardiographic variables poorly discriminate patients at risk of RVF. We examined the utility of 3-dimensional echocardiography (3DE) right ventricular (RV) volumetric assessment to identify patients at risk for RVF. METHODS AND RESULTS: RVF was defined as the need for inotropic infusion for >14 days after LVAD surgery or the need for biventricular assist device support. Preoperative RV volumes and ejection fraction (EF) were measured, blinded to clinical data, from transthoracic 3DE full volume data sets in 26 patients. Baseline variables and 3DE RV indices were compared between patients with and without RVF. Twenty-four patients received continuous-flow LVADs, and 2 required biventricular support devices. Ten patients required prolonged inotropes after LVAD placement. Baseline characteristics associated with RVF included higher right atrial pressure, higher right atrial pressure to pulmonary capillary wedge pressure ratio, and lower cardiac index and RV stroke work index (RVSWI). Echocardiographic indices associated with RVF included 3DE indexed RV end-diastolic and end-systolic volumes (RVEDVI and RVESVI) and RV ejection fraction (RVEF). The relationship between 3DE quantification of RV volumes and the development of RVF was independent from RVSWI: RVEDVI: odds ratio (OR) 1.16, 95% confidence interval (CI) 1.00-1.33 (P = .04); RVESVI: OR 1.14, 95% CI 1.01-1.28 (P = .03). CONCLUSIONS: Quantitative 3DE is a promising method for pre-LVAD RV assessment. RV volumes assessed by 3DE are predictive of RVF in LVAD recipients independently from hemodynamic correlates of RV function.

Simultaneous Right and Left Atrial Appendage Thrombus in a Patient with Atrial Fibrillation: A Lesson to Remember.

We report a case of simultaneous right and left atrial appendage thrombi in a 76-year-old-man with atrial fibrillation. Although transesophageal echocardiography is considered a routine diagnostic procedure, complete and comprehensive visualization of both atrial appendages is of paramount importance, particularly with the introduction of new therapeutic options like left atrial appendage exclusion devices. We expect to raise awareness among clinicians of the prevalence and implications of bilateral atrial appendage thrombi in common clinical practice.

Ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia.

Ectopia cordis, defined as partial or complete displacement of the heart outside of the thoracic cavity, is a rare congenital malformation. If not surgically corrected during the early years of life, ectopia cordis can prove to be a fatal abnormality. However, due to the presence of multiple intracardiac and extracardiac malformations, a corrective surgery might not always be successful. The pathology of ectopia cordis with a double outlet right ventricle, large ventricular septal defect, malposed great arteries and left ventricular hypoplasia is discussed, highlighting the complexities involved in such a rare disorder.

Mechanically unloading the left ventricle before coronary reperfusion reduces left ventricular wall stress and myocardial infarct size.

BACKGROUND: Ischemia/reperfusion injury worsens infarct size, a major determinant of morbidity and mortality after acute myocardial infarction (MI). We tested the hypothesis that reducing left ventricular wall stress with a percutaneous left atrial-to-femoral artery centrifugal bypass system while delaying coronary reperfusion limits myocardial injury in a model of acute MI. METHODS AND RESULTS: MI was induced by balloon occlusion of the left anterior descending artery in adult male swine. In the MI group (n=4), 120 minutes of left anterior descending artery occlusion was followed by 120 minutes of reperfusion without mechanical support. In the mechanically supported group (MI+unload; n=4), percutaneous left atrial-to-femoral artery bypass was initiated after 120 minutes of ischemia, and left anterior descending artery occlusion was prolonged for an additional 30 minutes, followed by 120 minutes of reperfusion with device support. All animals were euthanized after reperfusion, and infarct size was quantified by triphenyltetrazolium chloride staining. Compared with baseline, mean left ventricular wall stress and stroke work were not changed at any point in the MI group but were decreased after reperfusion in the MI+unload group (mean left ventricular wall stress, 44 658 versus 22 963 dynes/cm(2); stroke work, 2823 versus 655 mm Hg·mL, MI versus MI+unload). Phosphorylation of reperfusion injury salvage kinase pathway proteins from noninfarcted left ventricular tissue was unchanged in the MI group but was increased in the MI+unload group. Compared with the MI group, total infarct size was reduced in the MI+unload group (49% versus 28%, MI versus MI+unload). CONCLUSIONS: These data support that first unloading the left ventricle despite delaying coronary reperfusion during an acute MI reduces myocardial injury.

Hypertrophic cardiomyopathy with right ventricular outflow tract and left ventricular intracavitary obstruction.

Obstruction of the right ventricular outflow tract (RVOT) is a rare finding in hypertrophic cardiomyopathy (HCM) patients unlike left ventricular outflow tract (LVOT) obstruction. Although there are guidelines that aid in clinical decision making in patients with LVOT obstruction, there are none addressing RVOT obstruction. As RVOT obstruction may pose serious clinical implications similar to LVOT obstruction, appropriate medical and surgical management is very important. A unique phenotype of HCM with RVOT obstruction in conjunction with left ventricle (LV) intracavitary obstruction is discussed.

Doppler markers of elevated left atrial and ventricular filling pressure in a critically ill patient.

Accurate assessment of intracardiac flows by Doppler echocardiography may add important hemodynamic information in the critically ill patient. Detailed analysis of flow gradients through different sites and their correlation with timing of cardiac events may help us in better understanding the pathophysiology of the underlying disease. We report the clinical case of a 78-year-old patient with septic shock, in whom an A-dip of aortic regurgitation, shortening of diastolic filling, and diastolic mitral regurgitation were detected by Doppler analysis. Pathophysiologic explanation of these phenomena and their evolution after inotropic drug infusion are reported.

The syndrome of degenerative calcific aortic stenosis: prevalence of multiple pathophysiologic disorders in association with valvular stenosis and their implications.

BACKGROUND: We hypothesized that degenerative calcific aortic stenosis (DCAS) is a syndrome influenced by factors beyond aortic valve stenosis (AS). The aim of this study was to assess how frequently DCAS is complicated by increased vascular load, systolic and/or diastolic left ventricular (LV) dysfunction, and comorbid disorders. METHODS: In 215 consecutive patients > 60 years of age with severe and moderate AS, we analyzed systemic arterial compliance, global hemodynamic load, LV ejection fraction (EF), the presence of diastolic dysfunction, and other valvular or systemic disorders. RESULTS: A total of 164 patients had severe AS and 51 had moderate AS. In patients with severe AS, the prevalence of increased vascular load was 42%; LV systolic and diastolic dysfunction was present in 27% and 42%; other valve diseases in 23%; and comorbid disorders in 82%. In the moderate AS group, abnormal vascular load was found in 52%; LV systolic and diastolic dysfunction was prevalent in 26% and 31%; other valve diseases in 17%; and comorbid disorders in 78% patients. More than half the patients in both groups had symptoms. In both severe and moderate AS groups, the prevalence of increased vascular load and systolic dysfunction was higher in the symptomatic group. CONCLUSION: Considerable number of patients with DCAS have abnormal vascular load, abnormal LV function, and significant coexisting disorders. These could influence the total pathophysiologic burden on the heart and symptom expression. Thus, DCAS should not be considered just as valvular stenosis, but a syndrome of DCAS because of the diagnostic, prognostic, and therapeutic implications of various factors associated with it.

Left ventricular stroke volume index following transcatheter aortic valve replacement is an early predictor of 1-year survival.

BACKGROUND: Adverse cardiac events are common following transcatheter aortic valve replacement (TAVR). Our aim was to investigate the low left ventricular stroke volume index (LVSVI) 30 days after TAVR as an early echocardiographic marker of survival. HYPOTHESIS: Steady-state (30-day) LVSVI after TAVR is associated with 1-year mortality. METHODS: A single-center retrospective analysis of all patients undergoing TAVR from 2017 to 2019. Baseline and 30-day post-TAVR echocardiographic LVSVI were calculated. Patients were stratified by pre-TAVR transaortic gradient, surgical risk, and change in transvalvular flow following TAVR. RESULTS: This analysis focuses on 238 patients treated with TAVR. The 1-year mortality rate was 9% and 124 (52%) patients had normal flow post-TAVR. Of those with pre-TAVR low flow, 67% of patients did not normalize LVSVI at 30 days. The 30-day normal flow was associated with lower 1-year mortality when compared to low flow (4% vs. 14%, p = .007). This association remained significant after adjusting for known predictors of risk (adjusted odds ratio [OR] of 3.45, 95% confidence interval: 1.02-11.63 [per 1 ml/m2 decrease], p = .046). Normalized transvalvular flow following TAVR was associated with reduced mortality (8%) when compared to those with persistent (15%) or new-onset low flow (12%) (p = .01). CONCLUSIONS: LVSVI at 30 days following TAVR is an early echocardiographic predictor of 1-year mortality and identifies patients with worse intermediate outcomes. More work is needed to understand if this short-term imaging marker might represent a novel therapeutic target.

Recommendations for the Use of Echocardiography in the Evaluation of Rheumatic Heart Disease: A Report from the American Society of Echocardiography.

Acute rheumatic fever and its chronic sequela, rheumatic heart disease (RHD), pose major health problems globally, and remain the most common cardiovascular disease in children and young people worldwide. Echocardiography is the most important diagnostic tool in recognizing this preventable and treatable disease and plays an invaluable role in detecting the presence of subclinical disease needing prompt therapy or follow-up assessment. This document provides recommendations for the comprehensive use of echocardiography in the diagnosis and therapeutic intervention of RHD. Echocardiographic diagnosis of RHD is made when typical findings of valvular and subvalvular abnormalities are seen, including commissural fusion, leaflet thickening, and restricted leaflet mobility, with varying degrees of calcification. The mitral valve is predominantly affected, most often leading to mitral stenosis. Mixed valve disease and associated cardiopulmonary pathology are common. The severity of valvular lesions and hemodynamic effects on the cardiac chambers and pulmonary artery pressures should be rigorously examined. It is essential to take advantage of all available modalities of echocardiography to obtain accurate anatomic and hemodynamic details of the affected valve lesion(s) for diagnostic and strategic pre-treatment planning. Intraprocedural echocardiographic guidance is critical during catheter-based or surgical treatment of RHD, as is echocardiographic surveillance for post-intervention complications or disease progression. The role of echocardiography is indispensable in the entire spectrum of RHD management.

Three-dimensional echocardiographic analysis of right atrial volume in normal and abnormal hearts: comparison of biplane and multiplane methods.

BACKGROUND: Although right atrial size has clinical and prognostic importance, few data exist regarding the optimal three-dimensional echocardiography (3DE) method for assessing right atrial volume (RAV). While measuring RAV in multiple planes may improve the accuracy, it also increases analysis time. The purpose of this study was to determine the minimum number of planes required for optimal assessment of RAV, using eight-plane 3DE measurements as a reference standard. METHODS: 3DE was performed in 70 subjects (35 normal and 35 abnormal). 3DE RAV was obtained from three methods by tracing right atrial endocardial borders at ventricular end-systole in two orthogonal planes (two-plane), four equiangular planes (four-plane), and eight equiangular planes (eight-plane) in each subject. The time required for analysis was recorded for each method. RESULTS: Based on eight-plane RAV, mean RAV was 41 ± 13 mL in the normal group, and 82 ± 35 mL in the abnormal group. The average time for two-plane, four-plane, and eight-plane analysis was 69 ± 29, 118 ± 29, and 203 ± 37 s, respectively (P < 0.001 for comparisons between all groups). Four-plane approach had close agreement with eight-plane (2.6% underestimation for total group), while two-plane measurement underestimated RAV by an average of 19% for the total group, compared to eight-plane. CONCLUSION: Four-plane measurement of RAV shows good agreement with eight-plane measurement, while reducing the time required for analysis. Compared to eight-plane analysis, biplane measurement of RAV can result in underestimation of RAV, particularly in abnormal subjects with right atrial enlargement and remodeling.

A case of aortic stenosis and hypertrophic cardiomyopathy.

A patient with severe aortic valvular stenosis and coexisting obstructive hypertrophic cardiomyopathy, in whom diagnostic difficulties and management dilemmas arose, is presented. The complex issues involved in such a combination are discussed.

Eroded valves, recurrent pleural effusions, and ultimately heart-liver transplant.

A patient with eroded tricuspid and pulmonic valves, who eventually developed elevated right atrial and systemic venous pressure that led to hepatic cirrhosis and recurrent pleural effusion, is presented. The complex issues involved over a long period in this patient, who ultimately required combined heart and liver transplant, are discussed.