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Graphical abstract, Highlights • Quadricuspid aortic valve is a rare finding in patients with aortic valvulopathy. • Patients over age 40 with this finding are more likely to have valve dysfunction. • Transesophageal echocardiogram is preferred to evaluate quadricuspid aortic valves.

Graphical abstract, Highlights • Echo fusion overlays echocardiographic cardiac structures over fluoroscopy. • Echo fusion provides “real-time” feedback during complicated percutaneous procedures. • Echo fusion and 3D TEE imaging can be used to localize PVLs and guide intervention.

Introduction: The response to COVID infection has been associated with intense inflammation, and some patients have cardiac involvement. In a group of patients with COVID infection and shock whose ventricular function was characterized by echocardiography, we examined the relationship between levels of ferritin, an inflammatory marker that is elevated in COVID, and LV ejection fraction (EF). Methods: Of 1275 patients hospitalized with COVID pneumonia, 215 had shock requiring vasopressors. 162 had echocardiography to assess ventricular function and stroke volume. EF was measured using Simpson’s rule and stroke volume (SV) by Doppler. Patients were divided into groups with low or preserved EF ( EF L or EF P , cutoff &lte45%), and low or normal cardiac index, (CI, CI L or CI N , cutoff ≤&lte2.2 L/min/m 2 ). Ferritin within 7 days of the echo was available in 147/162 patients. We examined the association between ferritin levels and ejection fraction. Results: Mean age was 66.7, EF 58.7±13.9; CI 2.41±0.89 L/min/m 2 , ferritin 3036±2318 ng/mL (normal Figure ). High ferritin levels correlated with low EF (r=-0.18, p=0.02), and ferritin levels were greatly increased in patients with a hemodynamic profile of cardiogenic shock. ( EF L CI L , Figure, p=0.017 by ANOVA) Conclusion: COVID-induced shock had a cardiogenic profile (EF L CI L ) in 10% of patients, and another 5% had low EF but normal CI. Ferritin correlated with low EF and was increased in patients with a profile of cardiogenic shock. This suggests that marked inflammation may depress myocardial function in COVID patients with shock - analogous to a similar myocardial depression seen with bacterial-associated septic shock.

Patients with serious COVID infections develop shock frequently. To characterize the hemodynamic profile of this cohort, 156 patients with COVID pneumonia and shock requiring vasopressors had interpretable echocardiography with measurement of ejection fraction (EF) by Simpson's rule and stroke volume (SV) by Doppler. RV systolic pressure (RVSP) was estimated from the tricuspid regurgitation peak velocity. Patients were divided into groups with low or preserved EF (EFL or EFP, cutoff ≤45%), and low or normal cardiac index (CIL or CIN, cutoff ≤2.2 L/min/m2). Mean age was 67 ± 12.0, EF 59.5 ± 12.9, and CI 2.40 ± 0.86. A minority of patients had depressed EF (EFLCIL, n = 15, EFLCIN, n = 8); of those with preserved EF, less than half had low CI (EFPCIL, n = 55, EFPCIN, n = 73). Overall hospital mortality was 73%. Mortality was highest in the EFLCIL group (87%), but the difference between groups was not significant (p = 0.68 by ANOVA). High PEEP correlated with low CI in the EFPCIL group (r = 0.44, p = 0.04). In conclusion, this study reports the prevalence of shock characterized by EF and CI in patients with COVID-19. COVID-induced shock had a cardiogenic profile (EFLCIL) in 9.6% of patients, reflecting the impact of COVID-19 on myocardial function. Low CI despite preservation of EF and the correlation with PEEP suggests underfilling of the LV in this subset; these patients might benefit from additional volume. Hemodynamic assessment of COVID patients with shock with definition of subgroups may allow therapy to be tailored to the underlying causes of the hemodynamic abnormalities.

Calcific aortic stenosis (AS) is one of the most common acquired valvular heart diseases in industrialized nations. It is a slowly progressive disease and with the aging population, the prevalence of AS is expected to increase. Doppler echocardiography is used to classify patients based on severity of stenosis. Research efforts on how to better identify and risk-assess these patients are currently underway using advanced imaging modalities and serum biomarkers. Thus far, medications for AS prevention have been unsuccessful. As technology progresses, the assessment of AS will transition from one heavily weighed on echocardiographic gradients to one of active surveillances with multimodality imaging, serum biomarkers and genetic assessment.

A female patient presented with severe, symptomatic multivessel spontaneous coronary artery dissection (SCAD) with no known medical history or risk factors. The affected vessels were left anterior descending artery (LAD), right coronary artery (RCA), and the ramus. She was treated with conservative medical management. Two months later, repeat coronary angiogram to evaluate for any residual disease was performed which showed near-complete resolution of all involved vessels.

A 72 year old female with a past medical history of bicuspid aortic valve with severe aortic regurgitation status post placement of a 21 mm Magna Ease aortic valve replacement on 6/1/16, chronic atrial fibrillation, diabetes mellitus, and severe mitral regurgitation presented with symptoms of dyspnea upon exertion. Transesophageal echocardiogram (TEE) showed normal left ventricular function with severe mitral regurgitation, severe tricuspid regurgitation and severe pulmonary hypertension. Upon close interrogation of the tricuspid valve, there was evidence of fusion with a raphe present between the septal and anterior leaflets ("a bicuspid" tricuspid valve). The patient was referred for surgical evaluation where she was deemed to be at increased surgical risk and referred for percutaneous treatment for both severe mitral and severe tricuspid regurgitation. After an uncomplicated transseptal puncture, she underwent a placement of a single MitraClip XT clip to the A2/P2 portions of the mitral valve leaflets leaving mild residual mitral regurgitation by color Doppler. Attention was then taken towards the tricuspid valve where using TEE guidance, a MitraClip XT was positioned over the central aspect of the "bicuspid" tricuspid valve with the opened clip arms perpendicular leaflet coaptation. The MitraClip XT was advanced into the right ventricle and slowly retracted to grab both TV leaflets. There was mild, residual tricuspid regurgitation by color Doppler. The mean gradient across the tricuspid valve was 1 mmHg obtained at a heart rate of 52 bpm. This case describes the first known percutaneous treatment of severe tricuspid regurgitation from a "bicuspid" tricuspid valve with a MitraClip device. Abstract 1637 Figure.

Graphical abstract, Highlights • Echocardiography is usually the first imaging modality ordered for evaluation of a cardiac mass. • Although echocardiography cannot define the histopathology of a mass, it can identify the presence, location, and tissue characteristics of the mass, which can thus narrow the differential diagnosis. • Echocardiography is useful in evaluating tumor sequelae such as blood flow obstruction, valvular regurgitation, and myocardial dysfunction.

Prosthesis-patient mismatch (PPM) in TAVR is relatively common Valve oversizing can potentially prevent both paravalvular leak and PPM PPM may be avoidable if the expected Effective Orifice Area/Body Surface Area ratio is considered in decision making pre-TAVR.

Graphical abstract, Highlights • The mechanical complications of acute myocardial infarction (AMI) can be catastrophic. • A Gerbode type defect after AMI is rare. • Surgery is the mainstay of treatment, but percutaneous options are available. • Gerbode defects can be congenital or acquired and can be closed percutaneously. • Echocardiography reliably evaluates the mechanical complications of AMI.

An 86-year-old woman presented with New York Heart Association functional class III symptoms and a large mitral valve prolapse involving the P2 and P3 segments (white arrows, [Figure 1A][1]) with severe mitral regurgitation. A MitraClip (Abbott Vascular, Santa Clara, California) was placed at the

Congenital mitral stenosis can occur at the valvular, supravalvular, or subvalvular levels and is associated with developmental abnormalities in the mitral valve leaflets, mitral valve annulus, and/or the subvalvular apparatus. Congenital mitral stenosis is rare and occurs in 0.4–0.6% of autopsied patients with congenital heart disease [1, 2]. For historical background, see Table 13.1.

Athlete's heart or athletic heart syndrome refers to cardiac adaptations that occur from repetitive strenuous physical exercise. This syndrome encompasses a variety of significant physiological and morphological changes that affect all cardiac chambers and the aorta. Sport-specific cardiac remodeling occurs after repetitive training exercises and leads to differing cardiac adaptations. Understanding the possible normal cardiac adaptations to exercise is necessary to differentiate healthy cardiac adaptations from potentially life-threatening pathology.

Congenital valvular aortic stenosis has multiple morphologic variations including unicuspid aortic valve (AV), bicuspid aortic valve (BAV), or quadricuspid AV (Fig. 15.1). BAV is the most common congenital heart lesion, is estimated to occur in 1–2% of the general population, may be inherited, and has a male predominance.

A 41-year-old woman presented with recurrent episodes of chest pain. She had an elevated troponin level, anterior ST-segment depressions on electrocardiography, and focal left ventricular hypokinesis on echocardiography. A diagnosis was made.

ObjectiveThe prevalence of heart failure (HF) among adult patients with congenital heart disease (ACHD) is rising. Right ventricle (RV) exercise reserve and its relationship to outcomes have not been characterised. We aim to evaluate the prognostic impact of impaired RV reserve in an ACHD population referred for cardiopulmonary exercise testing (CPET).MethodsThis retrospective study evaluates patients with ACHD who underwent CPET (n=147) with first-pass radionuclide ventriculography at a single tertiary care centre. RV reserve was categorised as normal, mild to moderately or severely impaired. The primary composite clinical outcome included clinical right HF, arrhythmia, transplantation or death.ResultsPatients were median age 41±13 years, 50% were female and median follow-up was 1.1 (IQR: 0.7–2.0) years. Exercise RV reserve was impaired in 103 patients (70%), of whom 32% were asymptomatic. Resting RV systolic function poorly predicted RV reserve, with 52% of patients with severe impairment having a qualitatively normal echocardiographic assessment. The severely impaired reserve group had lower peak oxygen consumption (VO2)(17.2 vs 22.5 mL/kg/min, p2, age, sex, RV pathology, QRS duration, New York Heart Association class, resting RV ejection fraction and RV dilation by echocardiography or MRI (HR 3.7, 95% CI 1.1 to 13.0, p=0.039).ConclusionImpaired RV reserve, occurred in asymptomatic patients, was not well predicted by resting systolic function assessment, and strongly predicted adverse cardiovascular outcomes.

Valvular heart disease in women of childbearing age poses an increased risk of adverse maternal and fetal outcomes, and management in pregnancy can be challenging. Ideally, patients with suspected valvular disease should have preconception counseling by a multidisciplinary team including cardiologists with expertise in pregnancy and a maternal-fetal medicine specialist. Preconception planning should include a cardiac assessment of maternal risk, determination of frequency of surveillance, and a cardiovascular management plan during delivery. Women with valvular heart disease should be followed closely by a cardiologist and monitored for signs and symptoms of congestive heart failure and arrhythmias. In general, stenotic lesions may become more symptomatic in pregnancy, whereas regurgitant lesions are generally well tolerated. Left-sided valvular lesions have higher complication rates than right-sided lesions. For patients with asymptomatic valvular stenosis, medical management during pregnancy may include beta blockade and/or diuretics. Exercise stress testing prior to pregnancy in sedentary patients can be helpful to unmask symptoms and determine functional capacity. Patients with symptomatic, severe left-sided valvular obstruction have a high maternal risk of cardiovascular events during pregnancy, and percutaneous balloon valvuloplasty or surgery is recommended prior to pregnancy. The type of prosthetic valve (mechanical vs bioprosthetic) should be selected after a careful discussion with the patient. Invasive procedures are generally reserved for when medical management fails. The second trimester may be the optimal time for intervention as fetal organogenesis is complete and the cardiac positioning has not been affected by the gravid uterus.

The arterial switch operation (ASO) is now the most frequently performed surgical correction in individuals with dextro-transposition of the great arteries (D-TGA). Patients who undergo this procedure as neonates have overall good clinical outcomes yet continued clinical follow-up is important to evaluate for postoperative complications. In this group, the highest mortality is in the immediate postoperative period and is generally associated with reimplantation of the coronary arteries. As these patients live into adulthood, longitudinal follow-up for other ASO complications including neo-pulmonary stenosis, right ventricular outflow tract (RVOT) obstruction, or neo-aortic root dilation and resulting aortic insufficiency should be performed. In adults, extra care should be taken to identify and treat traditional cardiovascular risk factors as individuals with coronary obstruction may not present with typical anginal symptoms. Management of these patients should be performed in collaboration with an adult congenital heart center of excellence. This population offers a unique opportunity to provide timely feedback to adult congenital heart community of providers regarding late outcomes from surgical intervention and in the next decade will hopefully demonstrate a model for clinical feedback cycles in lifelong congenital care.

It is known that sport-specific remodeling of the heart may occur with exercise. Although some overlap exists, exercise activity can be segregated into 2 forms: isotonic exercise (endurance training) and isometric exercise (strength training), with defining hemodynamic differences. Increased cardiac output leads to a volume challenge that occurs with isotonic exercise that affects all 4 heart chambers and may cause an increase in left ventricular (LV) mass, biventricular chamber dilation, biatrial enlargement, and enhanced diastolic function.1 Conversely, isometric exercise (strength training) will increase the peripheral vascular resistance and cause a pressure load on the heart. This results in concentric LV hypertrophy and reduction in LV diastolic function. Although an athlete may have expertise in one particular sport, usually elite athletes train with combined isotonic and isometric components (weight training, plyometrics, speed drills, running, etc.) and may not fit into one specific category of training. See Article by Boraita et al There is limited data on the effect of exercise training on the aorta and whether aortic dilation occurs because of the hemodynamic effects of chronic exercise training. One could hypothesize that with isometric exercise, there is a transient increase in peripheral vascular resistance and systolic hypertension that leads to increased aortic wall tension and chronically may result in aortic dilation. A meta-analysis performed by Iskandar and Thompson2 showed minor enlargement associated with training in elite athletes at the level of the aortic valve annulus and the sinus of Valsalva compared with controls. …

Athletic heart syndrome refers to the physiological and morphological changes that occur in a human heart after repetitive strenuous physical exercise. Examples of exercise-induced changes in the heart include increases in heart cavity dimensions, augmentation of cardiac output, and increases in heart muscle mass. These cardiac adaptations vary based on the type of exercise performed and are often referred to as sport-specific cardiac remodeling. The hemodynamic effects of endurance and strength training exercise lead to these adaptations. Any abnormalities in chamber dilatation and left ventricular function usually normalize with cessation of exercise. Athletic heart syndrome is rare and should be differentiated from pathologic conditions such as hypertrophic cardiomyopathy, left ventricular noncompaction, and arrhythmogenic right ventricular dysplasia when assessing a patient for athletic heart syndrome. This paper describes specific adaptations that occur in athletic heart syndrome and tools to distinguish between healthy alterations versus underlying pathology.

Ebstein’s anomaly is a congenital malformation of the tricuspid valve and the right heart with a spectrum of clinical and morphologic presentations. Minor anomalies of the tricuspid valve may not be recognized until adulthood whereas major anomalies leading to heart failure and cyanosis require surgical intervention earlier in life. Echocardiography is the imaging modality of choice for both diagnosis and management of patients with Ebstein’s anomaly. Surgical correction includes tricuspid valve repair or replacement and associated findings such as interatrial communications and arrhythmias should be addressed at the time of surgery. Pre-pregnancy evaluation should be considered in all Ebstein’s anomaly patients and for those who are cyanotic, surgical correction must be considered due to the maternal and fetal ramifications of cyanosis in pregnancy. Most acyanotic Ebstein’s anomaly patients are able to tolerate pregnancy with manageable or no complications. Those patients with mild anomalies and no right heart dilation can participate in sports whereas those with severe anomalies are discouraged from competitive sports. Physical activity as tolerated is important in all patients with adult congenital heart disease. Adult congenital heart specialists should evaluate patients prior to cardiac or noncardiac surgery. Longitudinal clinical follow-up in all Ebstein’s anomaly patients (both repaired and unrepaired) is warranted to follow for signs and symptoms of heart failure, arrhythmias, cyanosis, and other associated findings. This should be ideally performed in collaboration with an adult congenital heart center of excellence.

Background: In the recent years, there has been a focus on decreasing heart failure readmission rates with many hospital initiatives directed towards implementation of heart failure discharge protocols. We hypothesize that identifying those patients at risk for readmissions may help prevent future readmissions. Methods: All patients with heart failure who presented to a tertiary urban medical center from July 2008 to December 2011 were included. Readmission was defined as being admitted to the hospital within one month of a previous heart failure admission. A multivariate analysis was performed on patients readmitted with goal to identify risk factors. Results: The study period had 3790 admissions for heart failure where 476 (12.7%) of which were readmissions. It was found that for every unit increase in body mass index (BMI) and hemoglobin, there was a significant decrease in odds of readmission. Antiarrhythmic medication, lipid medication, or angiotensin II receptor blockers (ARBs) also have a significant protective effect. Isosorbide, warfarin, cardiac resynchronization therapy (CRT), and for every unit increase in blood urea nitrogen (BUN) there was a significant increase in the patient’s risk of readmission (Table 1). Conclusions: Our single center, retrospective study shows an obesity paradox and a protective effect seen with heart failure medications as well as lipid lowering medications. This information may be used to target at risk patients in order to decrease readmission by establishing close follow-up and maximizing medical therapies.