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.

Infected thrombus in a Fontan circulation.

The Fontan operation was introduced in 1968 as a palliative treatment for patients with univentricular heart physiology. Natural history and outcomes are poor. By 10 years after Fontan operation, most patients will develop any complication such as tachyarrhythmias, any spectrum of Fontan-associated liver disease, protein-losing enteropathy, heart failure, thrombosis, and infective endocarditis among others. Echocardiography and magnetic resonance imaging (MRI) are the first-line diagnostic tools for detecting such complications. Clinical and imaging follow-up are a mainstay for the evaluation of this patients.

Echocardiographic diagnosis and follow-up for ALCAPA syndrome treated with the Takeuchi procedure.

We present the case of an 18-year-old man with aborted sudden cardiac death. His initial echocardiogram suggested an anomalous origin of the left coronary artery from the pulmonary artery. Diagnosis was confirmed with coronary angiography. He underwent Takeuchi procedure and fully recovered. A two-year follow-up echocardiogram showed a moderate supravalvular pulmonary stenosis related to the transpulmonary baffle. The presence of extensive collateral circulation should raise suspicion of ALCAPA. Postoperative surveillance in this group of patients needs to be oriented in finding complications such as supravalvular pulmonary stenosis, aortic and pulmonary valve insufficiency, and baffle obstruction and leaks.

Coexisting bicuspid aortic and pulmonary valves diagnosed by 3D transthoracic echocardiography.

Coexisting bicuspid aortic and pulmonary valves is an extremely rare condition, and there have been few published cases. Diagnosis of bicuspid aortic valve is straightforward with 2D echocardiography; however, analysis of the morphology of the pulmonary valve is challenging. In this study, we report on a case of a 32-year-old man with bicuspid aortic and pulmonary valves diagnosed by 2D and 3D transthoracic echocardiography. The enlarged pulmonary artery without any obvious etiology led us to suspect a pulmonary valve anomaly; thus, we comprehensively evaluated it with 2D and 3D echocardiography, which confirmed the diagnosis of bicuspid pulmonary valve.

Impact of Surgery in Ebstein's Anomaly Using Current Surgical Criteria.

BACKGROUND: In Ebstein's anomaly (EA) current surgical criteria may not translate into better long-term survival. The aim of this study was therefore to determine if surgical treatment for EA increases survival, and to analyze factors associated with mortality.Methods and Results:A retrospective study was carried out involving 103 patients with surgical indication using current criteria, comparing operated (n=49; 47.5%) and non-operated patients (n=54; 52.4%); the severity of disease was similar in all cases. Overall follow-up was 12 years (range, 1-49 years). There were no differences in mortality: in the surgical and non-surgical groups, survival at 10 years was 92.8% vs. 90.7%; 20 years, 85.7% vs. 81.0%; and 30 years, 78.5% vs. 72.2%, respectively. On multivariate analysis right ventricular fractional shortening (RVFS) was associated with mortality in both groups. Decreasing RVFS was associated with worse survival according to severity: when RVFS was <20%, survival at 20, 40 and 60 years was 58%, 39%, and 12.5%, respectively (P<0.0013). Left ventricular ejection fraction also correlated with survival (P<0.0013). CONCLUSIONS: Surgery did not translate into benefit in terms of survival, and this was clearly associated with RV function; therefore this should be a key factor in the surgical decision making.

Vasodilator Strain Stress Echocardiography in Suspected Coronary Microvascular Angina.

Background: In patients with Ischemia and non-obstructive coronary artery stenosis (INOCA) wall motion is rarely abnormal during stress echocardiography (SE). Our aim was to determine if patients with INOCA and reduced coronary flow velocity reserve (CVFR) have altered cardiac mechanics using two-dimensional speckle-tracking echocardiography (2DSTE) during SE. Methods: In a prospective, multicenter, international study, we recruited 135 patients with INOCA. Overall, we performed high dose (0.84 mg/kg) dipyridamole SE with combined assessment of CVFR and 2DSTE. The population was divided in patients with normal CVFR (>2, group 1, n = 95) and abnormal CVFR (≤2, group 2, n = 35). Clinical and 2DSTE parameters were compared between groups. Results: Feasibility was high for CFVR (98%) and 2DSTE (97%). A total of 130 patients (mean age 63 ± 12 years, 67 women) had complete flow and strain data. The two groups showed similar 2DSTE values at rest. At peak SE, Group 1 patients showed lower global longitudinal strain (p < 0.007), higher mechanical dispersion (p < 0.0005), lower endocardial (p < 0.001), and epicardial (p < 0.0002) layer specific strain. Conclusions: In patients with INOCA, vasodilator SE with simultaneous assessment of CFVR and strain is highly feasible. Coronary microvascular dysfunction is accompanied by an impairment of global and layer-specific deformation indices during stress.

Clinical Spectrum and Long-term Outcome of 183 Cases of Ebstein's Anomaly, Experience of Mexican Population.

BACKGROUND: Ebstein's anomaly (EA) is a myopathy of the right ventricle that causes a variable spectrum of tricuspid valve delamination failure with diverse clinical and anatomical presentation. We reviewed our data of EA to establish an association between clinical and echocardiographic findings with mortality. METHODS: We divided patients in infants, Children/adolescents (Ch/A), and adults, according to age of presentation. Clinical and echocardiographic parameters were compared among groups. Multivariate analysis was performed for mortality. Survival analysis was plotted using Kaplan Meier curves. RESULTS: Cyanosis, severe forms of AE and heart failure were more frequent among infants, arrhythmias in Ch/A and stroke among adults. Surgery was performed in 71 patients; infants had higher mortality and early complications. We found that the predicted mortality at 40 years of age in the three groups was significantly different (log rank test, p <0.0001): Infants: 38%, Ch/A 16 and 4% in adults. Multivariate model in surgical group showed that progressive drop of right ventricular fractional shortening (RVFS) predicts a higher mortality risk. In the non-surgical group, low RVFS and cyanosis were significantly associated with mortality. CONCLUSION: EA in infants is linked to higher morbidity and mortality, while arrhythmias predominate in Ch/A and stroke in adults. In general, stroke is frequent in patients with EA, some prevention alternative must be implemented. Right ventricular dysfunction is very important in EA and is associated with high mortality. It must be subject of discussion the planning of the type of surgery or even in the decision of to preclude surgical treatment.

Right ventricular free wall strain predicts functional capacity in patients with repaired Tetralogy of Fallot.

To investigate the role of right ventricular free wall strain (RVFWSL) to predict low functional capacity in repaired tetralogy of Fallot (rTOF). We prospectively enrolled 33 patients with rTOF with moderate to severe PR who underwent rest and peak exercise echocardiography on a semisupine cycloergometer. Conventional function and strain imaging parameters of both ventricles were measured. Patients performing < 7 METS were defined to have low functional capacity. Logistic regression was used to identify parameters associated with low functional capacity. Eleven patients (33.3%) had low functional capacity. These patients were shorter (height 155 ± 7 vs 163 ± 9 cm, p = 0.023), more frequently female (27.3 vs 72.7%, p = 0.024) and had history of Blalock-Taussig shunt (45.5 vs 9.1%, p = 0.027). On multivariate analysis RVFWSL was the only predictor of low functional capacity OR 1.39 (CI 95%, 1.06-1.83., p = 0.018) per % change. A RVFWSL < 17% (absolute value) had an AUC of 0.785, sensitivity of 81.8% and specificity of 77.3% to predict low functional capacity. Right ventricular free wall strain is an independent predictor of low functional capacity in repaired tetralogy of Fallot with moderate to severe PR. A value < 17% might be useful in deciding when to perform pulmonary valve replacement, when functional capacity cannot be objectively measured.

Clinical and echocardiographic factors associated with mitral plasticity in patients with chronic inferior myocardial infarction.

Aims: Ischaemic mitral regurgitation (IMR) is consequence of left ventricular (LV) remodelling after myocardial infarction. In some cases, the mitral valve enlarges to compensate for LV remodelling and tenting, improving its coaptation; a process termed 'plasticity'. We sought to identify clinical and echocardiographic factors associated with plasticity in patients with chronic inferior myocardial infarction (CII). Methods and results: This study included 91 revascularized CII patients and 46 controls. Plasticity and IMR severity were evaluated by 2D transthoracic echocardiography. Compared with controls, CII patients were older (59 vs. 25 years) and mostly men (80% vs. 46%), both P < 0.001. Chronic inferior myocardial infarction patients also had significant LV remodelling: larger LV volumes, larger mitral tenting areas, larger coaptation depths, longer mitral leaflets and chords, and worse mitral regurgitation (all P ≤ 0.03). Of 91 CII patients, 60 had mitral plasticity (longer anterior and posterior leaflets and longer posterior chords, all P < 0.001), despite not exhibiting significantly larger LV volumes, tenting area or coaptation depth, when compared with patients with no plasticity. Contralateral (anterior) papillary muscle-to-annulus length tended to be increased in CII plasticity patients (P = 0.05). Also they had less moderate and severe IMR (both P < 0.04) compared with non-plasticity CII patients. Multivariate analysis demonstrated independent associations between plasticity and smoking [odds ratio (OR) 0.03, 0.002-0.57; P = 0.019], duration of type-2 diabetes (OR 1.19, 1.007-1.42; P = 0.04) and haemoglobin (OR 2.17, 1.25-3.76; P = 0.005). Conclusion: Mitral plasticity results in less moderate and severe IMR. Longer time-duration of diabetes mellitus and higher haemoglobin level are independently associated with mitral plasticity, while smoking independently associates with no plasticity. Increased anterior papillary muscle-to-annulus length in CII patients with plasticity suggests complex LV remodelling mechanisms are involved in plasticity.

Echocardiographic and Histologic Correlations in Patients with Severe Aortic Stenosis: Influence of Overweight and Obesity.

BACKGROUND: Severe aortic stenosis (AS), leads to pathological left ventricular remodeling that may worsen with concomitant overweight and obesity (OW/O). METHODS: We aimed to prospectively analyze the impact of OW/O on ventricular remodeling in severe AS, by evaluating the percentage of intraendomyocardial fibrosis (PIEF) and the percentage of infiltrating intraendocardial lipid vacuoles (PIELV) and its relationship to global longitudinal strain (GLS) in patients with OW/O. RESULTS: 44 patients with severe AS were included, 13 non-obese (29%) and 31 OW/O (71%), all of them with left ventricular ejection fraction ≥ 55%. GLS was evaluated with 2D speckle tracking. During valve replacement, an endocardial biopsy was obtained, where PIEF and PIELV were analyzed. Patients with higher PIEF and PIELV had greater body mass index (p < 0.0001) and worse GLS (p < 0.0053). A GLS cut-off point < -14% had a sensitivity of 75%, and a specificity of 92.8% to detect important PIEF (AUC: 0.928, 95% confidence interval: 0.798-1.00). On multivariate analysis, OW/O and PIELV were independently associated to the PIEF, and OW/O and PIEF were independently associated to GLS. A high correlation between the amount of PIELV and PIEF were found. CONCLUSION: Patients with severe AS and OW/O have greater PIEF and PIELV, suggesting more pathological remodeling. GLS is useful to detect subclinical myocardial injury and is potentially useful for endomyocardial fibrosis detection. The presence of higher PIELF may be a trigger factor for the development of intraendomyocardial fibrosis.