Biatrial arrhythmogenic substrate in patients with hypertrophic obstructive cardiomyopathy.

Background: Atrial fibrillation (AF) in patients with hypertrophic obstructive cardiomyopathy (HOCM) may be caused by a primary atrial myopathy. Whether HOCM-related atrial myopathy affects mainly electrophysiological properties of the left atrium (LA) or also the right atrium (RA) has never been investigated.

Objective: The purpose of this study was to characterize atrial conduction and explore differences in the prevalence of conduction disorders, potential fractionation, and low-voltage areas (LVAs) between the RA and LA during sinus rhythm (SR) as indicators of potential arrhythmogenic areas.

Biomechanical response of ultrathin slices of hypertrophic cardiomyopathy tissue to myosin modulator mavacamten.

Hypertrophic cardiomyopathy (HCM) is the most common inherited myocardial disorder of the heart, but effective treatment options remain limited. Mavacamten, a direct myosin modulator, has been presented as novel pharmacological therapy for HCM. The aim of this study was to analyze the biomechanical response of HCM tissue to Mavacamten using living myocardial slices (LMS).

Sino-Atrial Node Mapping in Children and Adults With Congenital Heart Disease.

Background: Sinus node dysfunction (SND) and atrial tachyarrhythmias frequently co-exist in the aging patient with congenital heart disease (CHD), even after surgical correction early in life. We examined differences in electrophysiological properties of the sino-atrial node (SAN) area between pediatric and adult patients with CHD.

Methods: Epicardial mapping of the SAN was performed during sinus rhythm in 12 pediatric (0.

Early and late post-operative arrhythmias after surgical myectomy: 45 years of follow-up.

Aims: The aims of this study are to investigate the incidence and determinants of post-operative atrial arrhythmias, conduction disorders and mortality in hypertrophic obstructive cardiomyopathy (HOCM) patients undergoing transaortic myectomy.

Methods And Results: This retrospective single-center study was conducted in 249 patients (median age 54 years [40-64], 42% female) undergoing transaortic myectomy. Post-operative atrial fibrillation (AF) was reported in 84 patients (33.

Impact of sex on timing and clinical outcome of septal myectomy for obstructive hypertrophic cardiomyopathy.

Background: Sex disparities are common in hypertrophic cardiomyopathy (HCM). Previous research has shown that at time of myectomy, women are older, have greater impairment of diastolic function and more advanced cardiac remodeling. The clinical impact of these differences is unknown.

Protein Quality Control Activation and Microtubule Remodeling in Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder. It is mainly caused by mutations in genes encoding sarcomere proteins. Mutant forms of these highly abundant proteins likely stress the protein quality control (PQC) system of cardiomyocytes.

Tetralogy of Fallot in the Current Era.

Only few studies have reported long-term outcome of the transatrial-transpulmonary approach in the current era of management of tetralogy of Fallot (ToF). We investigated 15-year outcome of correction via a transatrial-transpulmonary approach in a large cohort of successive patients operated in the 21st century. All infant ToF patients undergoing transatrial-transpulmonary ToF correction between 2000 and 2015 were included (N = 177, 106 male, median follow-up 7.

Surgical correction of a coronary fistula between the left main coronary artery and the coronary sinus in a neonate.

We present a rare case of a 6-day old female neonate with a particularly exceptional coronary anatomy encompassing a fistula between the left main coronary artery and the coronary sinus with steal phenomenon, mandating surgical correction. The condition is extremely rare with an estimated incidence of about 0.1-0.

Outcome after aortic valve replacement in children: A systematic review and meta-analysis.

Objective: Despite an increasing interest in pediatric aortic valve repair, aortic valve replacement in children may be unavoidable. The evidence on outcome after pediatric aortic valve replacement is limited and usually reported in small case series. This systematic review and meta-analysis aims to provide an overview of reported outcome of pediatric patients after aortic valve replacement.

Long-term benefit of myectomy and anterior mitral leaflet extension in obstructive hypertrophic cardiomyopathy.

Severely symptomatic patients with obstructive hypertrophic cardiomyopathy (HC) may benefit from surgical myectomy. In patients with enlarged mitral leaflets and mitral regurgitation, myectomy can be combined with anterior mitral leaflet extension (AMLE) to stiffen the midsegment of the leaflet. The aim of this study was to evaluate the long-term results of myectomy combined with AMLE in patients with obstructive HC.

Carcinoid heart disease: outcomes after surgical valve replacement.

Objectives: To describe the early and late outcomes of carcinoid patients undergoing surgical heart valve replacement.

Methods: In a retrospective study, records of patients with symptomatic carcinoid heart disease referred for valve surgery between 1993 and 2010 at two academic centres were reviewed. The perioperative and postoperative outcomes were analysed.

Right ventricular outflow tract reconstruction with an allograft conduit in patients after tetralogy of Fallot correction: long-term follow-up.

Background: In tetralogy of Fallot (TOF) pulmonary regurgitation is a frequent complication after initial repair. The objective of the present study was to describe the long-term experience with the use of allograft conduits for right ventricular outflow tract (RVOT) reconstruction after correction of TOF in our institution.

Methods: Between 1987 and 2009, 133 allografts were implanted in 126 patients (mean age, 27.

Clinical outcome and health-related quality of life after right-ventricular-outflow-tract reconstruction with an allograft conduit.

Objective: Allograft conduits are used for reconstruction of the right ventricular outflow tract in congenital heart malformations (biventricular repair) and autograft procedures. A retrospective evaluation of allograft reconstruction of the right-ventricular-outflow-tract reconstruction was conducted and a cross-sectional quality of life study was performed.

Methods: Between August 1986 and March 2009, 509 allografts (435 pulmonary and 74 aortic) were implanted in 463 pediatric and adult patients (308 right-sided congenital heart malformations and 155 autograft procedures).

Relevance of colloid oncotic pressure regulation during neonatal and infant cardiopulmonary bypass: a prospective randomized study.

Objective: In neonatal and infant cardiac surgery with cardiopulmonary bypass (CPB), hemodilution with reduction of plasma albumin concentration and low colloid oncotic pressure (COP) are the main factors associated with tissue edema and postoperative weight gain. The aim of our study was to evaluate the influence of two different COP regulatory strategies on post-bypass body weight gain, fluid balance, and clinical outcomes.

Methods: Seventy elective patients with body weight<10 kg underwent first-time cardiac surgery with CPB and were randomized into two groups.

Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation.

Aim Of The Study: To investigate the long-term outcome of surgical treatment for congenitally corrected transposition of the great arteries (CCTGA), in patients with biventricular repair with the right ventricle as systemic ventricle.

Methods: A total of 32 patients with CCTGA were operated between January 1972 and October 2008. These operations comprised 18 patients with a repair with a normal left ventricular outflow tract, 11 patients with a Rastelli repair of the left ventricle to the pulmonary artery and 3 patients with a cardiac transplantation.

Long-term outcome of alcohol septal ablation in patients with obstructive hypertrophic cardiomyopathy: a word of caution.

Background: The impact of alcohol septal ablation (ASA)-induced scar is not known. This study sought to examine the long-term outcome of ASA among patients with obstructive hypertrophic cardiomyopathy.

Methods And Results: Ninety-one consecutive patients (aged 54+/-15 years) with obstructive hypertrophic cardiomyopathy underwent ASA.

Alveolar recruitment strategy and PEEP improve oxygenation, dynamic compliance of respiratory system and end-expiratory lung volume in pediatric patients undergoing cardiac surgery for congenital heart disease.

Objective: Optimizing alveolar recruitment by alveolar recruitment strategy (ARS) and maintaining lung volume with adequate positive end-expiratory pressure (PEEP) allow preventing ventilator-induced lung injury (VILI). Knowing that PEEP has its most beneficial effects when dynamic compliance of respiratory system (Crs) is maximized, we hypothesize that the use of 8 cm H(2)O PEEP with ARS results in an increase in Crs and end-expiratory lung volume (EELV) compared to 8 cm H(2)O PEEP without ARS and to zero PEEP in pediatric patients undergoing cardiac surgery for congenital heart disease.

Methods: Twenty consecutive children were studied.

Rapid method for intraoperative assessment of aortic coarctation using three-dimensional echocardiography.

Aims: The availability of three-dimensional (3D) echography with its multiplanar review analysis software on board now allows detailed examination in assessing morphological details. We evaluated the feasibility of 3D echography in assessing intraoperative morphological details of aortic coarctation (CoA) and its repair.

Methods And Results: Nine consecutive children scheduled for surgery of CoA were intraoperatively evaluated.

Outcome after reoperation for atrioventricular septal defect repair.

Results of surgical repair of atrioventricular septal defect (AVSD), both partial (PAVSD) and complete (CAVSD), have improved. However, reoperation is not uncommon. This report describes our experience in 59 patients who underwent reoperation after AVSD repair, between 1977 and 2008.

Usefulness of intraoperative real-time 3D transesophageal echocardiography in cardiac surgery.

Background: Recent advances in three-dimensional (3D) echocardiography allow to obtain real-time 3D transesophageal (RT3DTEE) images intraoperatively.

Methods: Preoperative transthoracic echocardiography (TTE) revealed: hypertrophic ventricular septum (TTE:19.3 mm), systolic anterior motion (SAM) not causing obstruction and malcoaptation of the anterior mitral valve leaflet (AMVL), and posterior mitral valve leaflet (PMVL) with severe mitral regurgitation.

Intraoperative cell salvage in infants undergoing elective cardiac surgery: a prospective trial.

Background: For a long time intraoperative cell salvage was considered not to be applicable in paediatric patients due to technical limitations. Recently, new autotransfusion devices with small volume centrifugal bowls and dedicated paediatric systems allow efficient blood salvage in small children. The purpose of this prospective non-randomised study was to determine the impact of intraoperative cell salvage on postoperative allogeneic blood products transfusion in infant patients undergoing cardiac surgery with cardiopulmonary bypass.