Background: Bicuspid aortic valve (BAV) is the most common congenital heart disease, affecting 0.5%–2% of the general population. It is associated not only with notable valvular risk (aortic stenosis and/or regurgitation, endocarditis) but also with aortopathy with a wide spectrum of unpredictable clinical presentations, including aneurysmal dilation of the aortic root and/or ascending thoracic aorta, isthmic coarctation, aortic dissection, or wall rupture. Methods: The REgistro della Valvola Aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging is a retrospective (from January 1, 2010)/prospective, multicenter, observational registry, expected to enroll 3000 patients with definitive diagnosis of BAV made by transthoracic and/or transesophageal echocardiography, computed tomography, cardiovascular magnetic resonance, or at surgery. Inclusion criteria were definitive diagnosis of BAV. Patients will be enrolled regardless of the presence and severity of aortic valve dysfunction or aortic vessel disease and the coexistence of other congenital cardiovascular malformations. Exclusion criteria were uncertain BAV diagnosis, impossibility of obtaining informed consent, inability to carry out the follow-up. Anamnestic, demographic, clinical, and instrumental data collected both at first evaluation and during follow-up will be integrated into dedicated software. The aim is to derive a data set of unselected BAV patients with the main purpose of assessing the current clinical presentation, management, and outcomes of BAV. Conclusions: A multicenter registry covering a large population of BAV patients could have a profound impact on the understanding of the natural history of this disease and could influence its management.

Aim: We employed an echocardiographic (ECHO) system as the backbone for the collection of electrocardiogram (ECG) and heart rate variability (HRV) data. The system was tested using an exercise model in which C57 male mice were exposed to sham or forced wheel running. Methods: Peak/peak (RR) interval was recorded over a 3 min period using the ECG platform of the ECHO system. Isoflurane-anesthetized male mice were divided into two groups (n = 8/group): sedentary (S) and forced wheel trained (T). HRV was analyzed in time and frequency domains (Fast Fourier Transform). Exercise training (T) was performed on a motorized wheel at low intensity 1 h/day, 5 days/week, 8 weeks duration. Cardiac morphometry and function were analyzed using ECHO while ECG was the basis to measure HRV. The sampling rate was 8000 Hz. Results show that the trained mice presented a reduction in heart rate as compared to the sedentary group. This was associated with lower cardiac sympathetic and higher parasympathetic modulation leading to an improved sympathetic/parasympathetic ratio (low-frequency band/high-frequency band). The trained group showed a reduction in isovolumetric relaxation time, reduced myocardial performance index, increased relative wall thickness, and left ventricle mass when compared to the sedentary group. Conclusion: Results document the utility of combining the ECHO and the ECG platform, allowing for the dual measurement of autonomic and cardiac function in mice.

Introduction: Doppler echocardiography with early diastolic transmitral velocity (E)/early mitral annular diastolic velocity (E') ratio has been proposed as the best predictor for evaluating left ventricle (LV) filling pressure. A dimensionless index E/(E' × S') ratio (S' = systolic mitral annulus velocity) resulted in readily, reproducible, and reliable predictor of LV filling pressure. We assessed the prognostic impact of E/(E' × S') in patients with asymptomatic heart failure (HF). Materials and Methods: We calculated E/(E' × S') in 337 patients (179 male, 53%; age 54.7 ± 13.7 years) using the average of septal and lateral mitral annular velocities. We considered a composite endpoint as follows: all-cause death, acute myocardial infarction, stroke, and HF exacerbation. Results: Baseline ejection fraction resulted 60.2 ± 11.8%; E/(E' × S') was 1.45 ± 0.8, with S' 7.4 ± 2.4 cm/s and E/E' 9.5 ± 5.4. After a 22-month median follow-up, there were 42 events: 5 deaths (12%), 3 acute myocardial infarctions (7%), 1 stroke (2%), and 33 HF hospitalizations (79%). In patients reaching the composite endpoint, E/(E' × S') resulted 2.07 ± 1.1 versus 1.3 ± 0.7 in event-free population (P < 0.001). In a Cox-regression analysis, adjusted for confounding clinical factors and conventional echo parameters, E/(E' × S') (P < 0.001), age (P < 0.001), and male gender (P = 0.03) resulted independent predictors of the composite endpoint. Conclusions: E/(E' × S') was an independent predictor for the future cardiac events in asymptomatic HF.

Background: Transthoracic Doppler echocardiographic examination is commonly performed to define the diastolic ventricular function since it is widely available, noninvasive, and inexpensive with respect to other diagnostic imaging modalities. However, data regarding age- and gender-matched reference values are scanty and sometimes conflicting. This study aims to explore the physiologic variations of left ventricular (LV) E/e' ratio as assessed in a large cohort of healthy adults and to investigate clinical and echocardiographic correlates. Methods: From June 2007 to February 2014, 1168 healthy Caucasian adults (mean age 45.1 ± 15.6 years) performed standard echocardiographic examination (transthoracic echocardiogram). Results: E/e' constantly increases across all the age classes (P < 0.0001, analyses of variance both for males and females) with a strong statistically significant linear positive correlation with age. Stepwise multiple linear regression analysis identified age (P < 0.0001), LV mass (P < 0.001), LV end-diastolic volume (P < 0.01), and left atrial volume (P < 0.001) as the only independent determinants of E/e' ratio (model R² = 0.54, P < 0.0001). Conclusions: In healthy subjects, transmitral E velocity to e' ratio changes in relation to the age: it increased with a statistically significant correlation in individuals older than 60 years. Hence, differences related to demographic and anthropometric measurements may potentially develop a misclassification of otherwise normal individuals when established on dichotomically suggested normal reference values. Our study can demonstrate that it is indispensable to apply specific cutoff related to the age and gender to properly assess LV diastolic function.

Background: Elevated mean pulmonary artery pressure (mPAP) and right heart failure increase mortality in patients with chronic kidney disease (CKD). Objectives: The objective of this study is to determine the prevalence of elevated mPAP in children with CKD compared with matched controls and to ascertain the relationship between elevated mPAP with right ventricular dysfunction and history of hemodialysis. Materials and Methods: A cross-sectional comparative study of mPAP and tricuspid annular plane systolic excursion of 21 children with CKD and age- and sex-matched controls asymptomatic for cardiac disease was conducted. Results: Median mPAP was 27.69 (18.3–36.1) mmHg in CKD patients compared with 14.55 (13.5–17.1) mmHg in controls (P = 0.002). Elevated mPAP was present in 42.9% of CKD group and 0% in controls (P < 0.001). The prevalence of right ventricle (RV) dysfunction in CKD was 9.5% and 0% in controls (P = 0.49). Right ventricular dysfunction was significantly more common in patients with elevated mPAP compared with those with normal mPAP (P < 0.001). Children with CKD who had a history of having been dialyzed were less likely to have elevated mPAP (P < 0.001). Conclusion: Elevated mPAP is significantly more common in children with CKD compared with controls. CKD population with mPAP elevation is more likely to have impaired RV function. The occurrence of elevated mPAP was more common in those who were never dialyzed.

Aims: The aim of this study is to measure the effect of positive-pressure ventilation on heart chamber dimensions, left ventricular (LV) systolic function, LV diastolic function, right ventricular (RV) systolic function, and RV pressure using transthoracic echocardiography. Settings and Design: This is a prospective study in a single secondary health-care center. Materials and Methods: A total of 107 patients with obstructive sleep apnea on continuous positive airway pressure (CPAP) therapy were recruited as participants between April and September 2016. Transthoracic echocardiography was performed twice on each participant, before and 15 min after, they used their own CPAP machines, and the echocardiography parameters of both scans were compared. Statistical Analysis Used: The parametric paired t-test was used to compare heart chamber dimensions, left heart diastolic function, left heart systolic function, right heart systolic function, and right heart pressure effect, without and with CPAP. These data were further examined among several subgroups defined by CPAP when the cutoff point was set at 8 cmH₂O and 10 cmH₂O. The level of significance was set at 0.05. Statistical analyses were performed using IBM SPSS version 22 (IBM, Armonk, NY, USA). Results: There were statistically significant reductions, after the application of CPAP, in the heart dimensions, and LV and RV systolic function. There were no significant changes in diastolic function. Concerning right heart pressure, with CPAP, there was a significant increase in the inferior vena cava (IVC) diameter and there was also a significant decrease in IVC variability from 44.56% ± 14.86% to 36.12% ± 11.42%. The maximum velocity of tricuspid regurgitation (TR) decreased significantly from 180.66 ± 6.95 cm/s to 142.30 ± 52.73 cm/s. Such changes were observed in both low and high CPAP subgroups. Conclusions: When placed on positive pressure, the clinically significant change in IVC diameter and variability and change in trans-TR velocity mean that it would be inaccurate to predict right heart chamber pressure through echocardiogram. Alternative methods for predicting right heart pressure are recommended.

Hypertrophic cardiomyopathy (HCM) is the most common known inherited heart disorder, with a prevalence of 1:500 of the adult population. Etiology of HCM can be heterogeneous, with sarcomeric gene disease as the leading cause in up to 60% of the patients, and with a number of possible different diseases (phenocopies) in about 10%–15% of the patients. Early diagnosis of storage and infiltrative disorders, particularly those with specific treatments (i.e., Fabry disease and/or amyloidosis), means early management and treatment, with a significant impact on patients prognosis. Here, we report on four different cases of HCM, highlighting difficulties to make differential diagnosis of different forms of cardiomyopathies, and their potential impact on the management.

In recent years, with an increasing number of central venous access procedures and cardiac implantable electronic device implantation, the incidence of infective endocarditis (IE) has become more prevalent. Two-dimensional transthoracic echocardiography (2D-TTE) and transesophageal echocardiography (TEE) are a key part of the evaluation of IE, but advances in three-dimensional echocardiography have enabled a better spatial resolution and visualization of cardiac structures, allowing the identification of any valvular vegetations, abscesses, or nodules. Herein, we report the usefulness of 3D-TEE in a difficult diagnosis of hemodialysis catheter endocarditis hidden in intracaval stent.

We report a case of a woman who presented with worsening shortness of breath due to a migrated ventriculoatrial shunt catheter into the pulmonary artery causing severe pulmonary insufficiency. She underwent surgical catheter removal. The majority of the catheter was easily retrieved; however, there were areas where the catheter was embedded into the myocardium, which would have posed a challenge with an endovascular approach.

Sinus of valsalva aneurysm (SVA) is a rare cardiac disease. The most common complication of SVA is rupture into the right atrium or right ventricle. Rupture into the left chambers is very rare. Patients with ruptured SVA are likely to die of heart failure or endocarditis. We present a 29-year-old man who was hospitalized for hepatic transplantation with rupture of SVA. Transthoracic echocardiography and transesophageal echocardiography showed rupture of a noncoronary SVA into the left atrium. Mitral valve infective endocarditis developed and surgery was planned for the patient, but the patient died due to multiple organ dysfunction syndrome.

Severe chronic mitral regurgitation (MR) is known to cause left ventricular volume overload and subsequently left ventricular dilatation. Here, we present a case of a 68-year-old man with severe chronic MR which happened to coexist with atrial septal defect of secundum type leading to the right ventricular dilatation.

Myocardial non-compaction (NC) is a rare genetic cardiomyopathy commonly believed to develop an intrauterine arrest of endomyocardial morphogenesis. NC is characterized by markedly hypertrabeculations in left ventricle or both ventricles with deep intertrabecular recesses. NC is usually seen isolated, but sometimes other congenital heart abnormalities may accompany to the myocardial NC. In this article we have presented an adult patient with biventricular myocardial NC cardiomyopathy accompanied by severe valvular pulmonary stenosis and patent foramen ovale.

We report a late clinical presentation of an aorto-right ventricular fistula (RVF) extending from the right sinus of Valsalva into the RV outflow tract with significant left-to-right shunt and severe pulmonary hypertension. A three-dimensional transesophageal echocardiogram and a chest-computed tomography scans were performed to better characterize the abnormality. Aorto- RVF can be either congenital or acquired, secondary to endocarditis, Valsalva aneurysm rupture, chest trauma, or aortic dissection.

Cor-triatriatum is uncommon and cor-triatriatum dexter is rarer, and patients may remain asymptomatic in isolated cases especially if the partitioning is mild and nonobstructing. There may be other cardiac defects associated with it which are mostly right-sided cardiac defects such as atrial septal defect, Ebstein anomaly, and pulmonary stenosis. However, left-sided heart anomaly such as aortic regurgitation has been associated with it, but its association with transposition of the great vessels has not been documented before now. Therefore, the case of a 3-month-old girl with cor-triatriatum dexter with transposition of the great vessels, and atrial septal defect is reported.

Free-floating right heart thrombus (RHT) is an extreme medical emergency in the context of acute massive pulmonary embolism (PE). Despite the advances in early diagnosis, the management is still very debatable due to lack of consensus. We reported the case of a 66-year-old male, with a history of moderate renal dysfunction and dilated cardiomyopathy, who presented to the emergency department for acute dyspnea. His angiographic magnetic resonance imaging revealed bilateral extensive PE. Transthoracic echocardiography showed RHT with moderate right ventricular dysfunction and pulmonary hypertension. Venous Doppler of the lower extremities noted the presence of a floating clot in the right common femoral vein. The patient was managed successfully by thrombolytic therapy with tenecteplase. To the best of our knowledge, this is the first case report of RHT and PE from Lebanon. Published cases from Middle Eastern countries are scarse.