Incidence and natural history of neonatal isolated ventricular septal defects: Do we know everything? A 6-year single-center Italian experience follow-up.

BACKGROUND: Despite ventricular septal defects (VSDs) are the most common congenital heart diseases (CHDs) in the neonatal period, their incidence and natural history are still debated and their follow-up and management strategies remain controversial. Our aim was to evaluate the incidence and natural history of isolated VSDs. METHODS: From January 1996 to December 2015 all neonates with a CHD suspicion were referred to the Cardiological Department of Grosseto Misericordia Hospital. Only newborns with confirmed isolated VSD were enrolled in this study and followed for 6 years. RESULTS: Our 343 newborns with an isolated VSD (incidence of 10.45/1000/births) account for 64% of all detected CHDs. VSDs location were as follows: muscular (73.8%), perimembranous (11.3%), inlet (1%), and outlet (0.8%). Of the located VSDs, 90% were small, 7.5% moderate, and 2.5% large, respectively. Spontaneous closure was observed in 96 (29.2%) of the VSD patients at 6-month, 198 (60.2%) at 1-year, 261 (79.3%) at 2-year, and in 302 (91.8%) at 6-year follow-up. Risk factors for defect persistence were a perimembranous location (P = .001; HR: 0.508, CI: 0.342-0.755), detection of multiple defects (P = .043; HR: 0.728, CI: 0.536-0.990), and male gender (P < .048; HR: 0.783, CI: 0.615-0.998), respectively. CONCLUSIONS: We here provide an incidence and natural history of neonatal isolated VSDs in a neonatal Caucasian population. These data may be useful for the development of expert consensus/standard recommendation guidelines for the follow-up and VSD management, data that are currently lacking.

Training-induced right ventricular remodelling in pre-adolescent endurance athletes: The athlete's heart in children.

AIMS: Little is known about the adaptation of the right ventricle (RV) to endurance exercise in children. The aim of this study was to assess the effects of 5months of intensive training on RV morphology and function in preadolescent endurance athletes. METHODS: Ninety-four children were evaluated in this study. Fifty-seven male competitive swimmers (aged 10.8±0.2years) were evaluated before (baseline) and after 5months of the training (peak-training), and compared to 37 age- and sex-matched non-athlete children evaluated at baseline and after 5months of natural growth. All subjects were asymptomatic, with negative family history for cardiomyopathies. RESULTS: At baseline no differences were found between athletes and controls for indexed RV outflow tract (RVOT) (18.5±2.7 vs. 16.8±5.0mm/m2, p=0.18) and RV basal end-diastolic diameter (EDD) (24.9±4.1 vs. 23.6±3.0mm/m2, p=0.15). After 5months, indexed RVOT and RV basal EDD significantly increased in athletes (20.2±2.9mm/m2 and 25.4±3.3mm/m2, p<0.0001 vs. baseline) while no differences were observed in controls (p=0.84 and p=0.25). Despite the increase in RV size, RV function remained normal in athletes, with no changes in RV fractional area change (p=0.97), s' value (p=0.22), and RV longitudinal strain (p=0.28). CONCLUSIONS: Endurance training influences the growing heart of male preadolescent athletes with an addictive increase in RV dimensions, with a preserved RV function. Therefore, in children engaged in endurance sports the increase in RV size associated with normal RV function represents a physiological expression of the athlete's heart and should not be misinterpreted as an expression of incipient RV cardiomyopathy.

Atrial septal defect and training-induced changes in loading conditions: clinical management and implications for competitive athletes.

Atrial septal defect (ASD) is the most common congenital heart disease in adults. When right heart dilation occurs, prompt closure should be considered. In the athletic population, however, the management of ASD can be challenging. Indeed, while the training-induced haemodynamic effects on the right heart of an athlete with open ASD are not well known, possible device-related consequences may occur after percutaneous closure. We report the case of a competitive athlete with secundum ASD in which changes in the training regime significantly affected the right heart. Prompt normalisation of right ventricular size and of pulmonary artery pressures was demonstrated 2 months after percutaneous ASD closure.

RV Longitudinal Deformation Correlates With Myocardial Fibrosis in Patients With End-Stage Heart Failure.

OBJECTIVES: This study was performed to determine the accuracy of right ventricular (RV) longitudinal strain (LS) in predicting myocardial fibrosis in patients with severe heart failure (HF) undergoing heart transplantation. BACKGROUND: RVLS plays a key role in the evaluation of its systolic performance and clinical outcome in patients with refractory HF. METHODS: We studied 27 patients with severe systolic HF (ejection fraction ≤25% and New York Heart Association functional class III to IV, despite full medical therapy and cardiac resynchronization therapy) using echocardiography before heart transplantation. RV free wall LS, right atrial LS, sphericity index (SI), and tricuspid annular plane systolic excursion (TAPSE) were all measured. Upon removal of the heart, from the myocardial histologic analysis, the ratio of the fibrotic to the total sample area determined the extent of fibrosis (%). RESULTS: RV myocardial fibrosis correlated with RV free wall LS (r = 0.80; p < 0.0001), SI (r = 0.42; p = 0.01) and VO2 max (r = -0.41; p = 0.03), with a poor correlation with TAPSE (r = -0.34; p = 0.05) and right atrial LS (r = -0.37; p = 0.03). Stepwise multivariate analysis showed that RV free wall LS (ß = 0.701, p < 0.0001) was independently associated with RV fibrosis (overall model R(2) = 0.64, p < 0.0001). RV free wall LS was the main determinant of myocardial fibrosis. In the subgroup of patients with severe RV fibrosis, RV free wall LS had the highest diagnostic accuracy for detecting severe myocardial fibrosis (area under the curve = 0.87; 95% confidence interval: 0.80 to 0.94). CONCLUSIONS: In late-stage HF patients, the right ventricle is enlarged, with reduced systolic function due to significant myocardial fibrosis. RV free wall myocardial deformation is the most accurate functional measure that correlates with the extent of RV myocardial fibrosis and functional capacity.

Morphological and functional adaptation of left and right atria induced by training in highly trained female athletes.

BACKGROUND: Exercise is able to induce atrial remodeling in top-level athletes. However, evidence is mainly limited to men and based on cross-sectional studies. The aim of this prospective, longitudinal study was to investigate whether exercise is able to influence left and right atrial morphology and function also in female athletes. :ETHODS AND RESULTS- Two-dimensional echocardiography was performed before season and after 16 weeks of intensive training in 24 top-level female athletes. Left and right atrial myocardial deformation was assessed by two-dimensional speckle-tracking echocardiography. Left atrial volume index (24.0±3.6 versus 26.7±6.9 mL/m(2); P<0.001) and right atrial volume index (15.66±3.09 versus 20.47±4.82 mL/m(2); P<0.001) significantly increased after training in female athletes. Left atrial global peak atrial longitudinal strain and peak atrial contraction strain significantly decreased after training in female athletes (43.9±9.5% versus 39.8±6.5%; P<0.05 and 15.5±4.0% versus 13.9±4.0%; P<0.05, respectively). Right atrial peak atrial longitudinal strain and peak atrial contraction strain showed a similar, although non-significant decrease (42.8±10.6% versus 39.3±8.3%; 15.6±5.6% versus 13.1±6.1%, respectively). Neither biventricular E/e' ratio nor biatrial stiffness changed after training, suggesting that biatrial remodeling occurs in a model of volume rather than pressure overload. CONCLUSIONS: Exercise is able to induce biatrial morphological and functional changes in female athletes. Biatrial enlargement, with normal filling pressures and low atrial stiffness, is a typical feature of the heart of female athletes. These findings should be interpreted as physiological adaptations to exercise and should be considered in the differential diagnosis with cardiomyopathies.

Pre-operative left atrial strain predicts post-operative atrial fibrillation in patients undergoing aortic valve replacement for aortic stenosis.

Post-operative atrial fibrillation (AF) is a common and serious complication in patients undergoing aortic valve replacement (AVR). Speckle tracking echocardiography (STE) has recently enabled the quantification of longitudinal myocardial left atrial (LA) deformation dynamics. Our aim was to investigate LA preoperative mechanical function in patients undergoing AVR for aortic stenosis using STE and determine predictors of post-operative AF. 76 patients with aortic stenosis in sinus rhythm, undergoing AVR, were prospectively enrolled. Conventional echocardiographic parameters, and peak atrial longitudinal strain (PALS) were measured in all subjects the day before surgery. PALS values were obtained by averaging all segments in the 4- and 2-chamber views (global PALS). All patients received biological valve prostheses and a standard postoperative care. Postoperative AF occurred in 15 patients (19.7 %). On univariate analysis among all clinical and echocardiographic variables, global PALS showed the highest diagnostic accuracy (HR 6.55 p < 0.0001; AUC of 0.89) with a cut-off value <16.9 %, having sensitivity and specificity of 86 and 91 %, respectively, in predicting postoperative AF. LA volume indexed and E/e' ratio had lower diagnostic accuracy (AUC 0.76 and 0.51, respectively). On multivariate analysis global PALS remains a significant predictor of postoperative AF (p < 0.0001). STE analysis of LA myocardial deformation is considered a promising tool for the evaluation of LA subclinical dysfunction in patients undergoing AVR, giving a potentially better risk stratification for the occurrence of postoperative AF.

Characterization of right atrial function and dimension in top-level athletes: a speckle tracking study.

Although many echocardiographic studies are available about the adaptation of left ventricle to intensive training, right heart function has been poorly investigated and no data are available about the right atrial (RA) function in top-level athletes. The aim of the study was to investigate RA function and dimension by standard echocardiography and 2D speckle tracking echocardiography (STE). One hundred top-levels athletes were recruited from professional sports team and were compared with 78 normal subjects. Athletes during an off-training period or during prolonged forced rest resulting from injuries were excluded. Top-level athletes had higher BSA as compared with controls and, as expected, a lower resting heart rate (p ≤ 0.001). RA area, volume, and volume index were significantly greater in athletes than in controls (p ≤ 0.001). This increase was associated with greater right ventricular and inferior vena cava diameters (p ≤ 0.001). Peak atrial longitudinal strain and peak atrial contraction strain values were significantly lower in athletes in comparison with controls (40.92 ± 9.86% vs. 48.00 ± 12.68%, p ≤ 0.001; 13.05 ± 4.84% vs. 15.99 ± 5.74%, p ≤ 0.001, respectively). Interestingly, while athletes presented a higher E/A ratio (p ≤ 0.001) and a lower peak A velocity (p ≤ 0.001), the E/e' ratio did not differ between the two groups. In top-level athletes the RA presents a physiological adaptation to intensive exercise conditioning which determines not only a morphological but also a functional remodeling. We reported for the first time reference values of RA strain in elite athletes, demonstrating that 2D STE is a useful tool to investigate RA longitudinal myocardial deformation dynamics in athlete's heart.

Reference values of right atrial longitudinal strain imaging by two-dimensional speckle tracking.

BACKGROUND: The role of speckle tracking in the assessment of right atrial (RA) deformation dynamics has not been established yet. The reference ranges of RA longitudinal strain indices were measured by speckle tracking in a population of normal subjects. METHODS: In 84 healthy individuals, peak atrial longitudinal strain (PALS), peak atrial contraction strain (PACS), and time to peak longitudinal strain (TPLS) were measured using a six-segment model for the RA. Strain rate (SR) was also measured starting from the QRS-wave onset, peak positive (x-wave), first peak negative (y-wave), and second negative peak (z-wave). The time from the QRS onset was measured to each wave peak. RESULTS: Adequate tracking quality was achieved in 64% of segments analyzed. Inter- and intraobserver variability coefficients of measurements ranged between 6% and 11%. Global PALS was 49 ± 13%, global TPLS was 363 ± 59 msec, x-wave was 2.12 ± 0.58 sec(-1) , y-wave was -1.91 ± 0.63 sec(-1) , and z-wave was -2.18 ± 0.78 sec(-1) . CONCLUSION: Speckle tracking is a feasible technique for the assessment of longitudinal myocardial RA deformation. Reference ranges of strain indices were reported.

Chronic mitral regurgitation: left atrial deformation analysis by two-dimensional speckle tracking echocardiography.

BACKGROUND: Speckle tracking echocardiography (STE) is a novel method for the angle-independent and objective quantification of myocardial deformation; it has recently evolved, enabling the quantification of longitudinal myocardial left atrial (LA) deformation dynamics. To investigate the effects of chronic mitral regurgitation (MR) on these functional atrial indices, we analyzed LA function by STE in a group of asymptomatic patients with chronic degenerative MR. METHODS: The study population included 36 patients with mild MR, 38 with moderate MR, and 42 with severe MR. 52 age-matched controls were also recruited. Global peak atrial longitudinal strain (global PALS) was measured in all subjects by averaging all atrial segments. RESULTS: Age, gender, and LV ejection fraction in all pathological groups were comparable to those in the controls. Global PALS was higher in the mild MR group (46.7 ± 9.1%) in comparison with the controls (40.5 ± 6.2%; P < 0.001); instead global PALS was lower in the moderate MR group (25.7 ± 7.1%) and further reduced in the severe MR group (13.2 ± 5.2%) in comparison with the controls (40.5 ± 6.2%; overall P < 0.0001 by ANOVA, P < 0.05 for all pairwise comparisons). In multivariate analysis, E/Em ratio emerged as the principal independent determinant of global PALS. CONCLUSIONS: Our study provides new insight for the LA function analysis in response to different degrees of MR, showing that STE measurements of LA longitudinal strain may be considered a promising tool for the early detection of impairment of LA compliance in patients with asymptomatic chronic MR.