Repair of Congenital Right Atrial Aneurysm Associated With Wolff-Parkinson-White Syndrome in a 5-Year-Old Girl.

This report describes our experience with a 5-year-old girl who had an extremely rare presentation of a right atrial aneurysm and associated Wolff-Parkinson-White syndrome. Before being referred to our department, she underwent an ineffective radiofrequency ablation for repeated episodes of paroxysmal supraventricular tachycardia that were causing dizziness, palpitations, and chest discomfort. We resected the aneurysm with good results; she was doing well and was in normal sinus rhythm at the time of her 1-year follow-up visit.

Presence of reduced regional left ventricular function even in the absence of left ventricular wall scar tissue in the long term after repair of an anomalous left coronary artery from the pulmonary artery.

Background: We sought to assess left ventricular regional function in patients with and without left ventricular wall scar tissue in the long term after repair of an anomalous origin of the left coronary artery from the pulmonary artery.

Methods: A total of 20 patients aged 12.8±7.

Myocardial perfusion, scarring, and function in anomalous left coronary artery from the pulmonary artery syndrome: a long-term analysis using magnetic resonance imaging.

Background: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. We aimed to examine the role of cardiac magnetic resonance imaging (MRI) in the long-term surveillance of repaired ALCAPA with regard to myocardial scarring, wall motion abnormalities, perfusion deficits, and myocardial function.

Methods: Twenty-one patients after direct reimplantation of ALCAPA (median age at operation, 2.

Rare and private variations in neural crest, apoptosis and sarcomere genes define the polygenic background of isolated Tetralogy of Fallot.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Its genetic basis is demonstrated by an increased recurrence risk in siblings and familial cases. However, the majority of TOF are sporadic, isolated cases of undefined origin and it had been postulated that rare and private autosomal variations in concert define its genetic basis.

Outcome of surgical correction of congenital supravalvular aortic stenosis with two- and three-sinus reconstruction techniques.

Background: Several surgical techniques for the treatment of congenital supravalvular aortic stenosis have been developed, yet there is no consensus about the optimal approach. We reviewed our institutional experience with 2- and 3-sinus reconstruction techniques.

Methods: Thirty-eight patients operated on for supravalvular aortic stenosis between 1987 and 2012 in our institution were analyzed retrospectively.

Altered right ventricular function in the long-term follow-up evaluation of patients after delayed aortic reimplantation of the anomalous left coronary artery from the pulmonary artery.

This study aimed to evaluate regional and global ventricular functions in the long term after aortic reimplantation of the anomalous left coronary artery from the pulmonary artery (ALCAPA) and to assess whether the time of surgical repair influences ventricular performance.The study examined 20 patients with a median age of 15 years (range 3-37 years) who had a corrected ALCAPA and 20 age-matched control subjects using echocardiography and tissue Doppler imaging (TDI). The median follow-up period after corrective surgery was 6 years (range 2.

Surgery impacts right atrial function in tetralogy of Fallot.

Objective: To analyze the impact of surgery and pericardial integrity on right atrial function and total heart volume variation in the setting of pulmonary valve insufficiency.

Methods: Right atrial function and total heart volume variation were analyzed in 2 subgroups of patients with pulmonary valve insufficiency compared with healthy controls: group 1 with surgically repaired tetralogy of Fallot (n = 20 patients) and group 2 after balloon angioplasty of pulmonary valve stenosis in patients with isolated valve disease without surgery (n = 7 patients). Volumetric analysis of magnetic resonance imaging data revealed parameters of atrial function (reservoir, conduit, and pump functions and cyclic volume change) and of total heart volume (end-diastolic and end-systolic total heart volume and the variation).

Analysis of the risk factors for early failure after extracardiac Fontan operation.

Background: We analyzed risks for severe morbidity in the early period after extracardiac Fontan operation.

Methods: Between November 1995 and May 2011, 140 patients (median age, 3.8 years) underwent extracardiac Fontan operation.

Improved early postoperative outcome for extracardiac Fontan operation without cardiopulmonary bypass: a single-centre experience.

Objectives: The use of modified extracardiac Fontan operation (ECFO) for total cavo-pulmonary connection allows cardiopulmonary bypass (CPB) to be avoided and seems to improve early postoperative results. We evaluated our experience with the off-pump technique for ECFO.

Methods: Since 2009, the last 17 consecutive patients of 137 (median age 3.

Risk factors for distal Contegra stenosis: results of a prospective European multicentre study.

Objectives The EUCon study was designed to identify risk factors for distal anastomotic stenosis after bovine jugular vein (Contegra) implantation in children. Methods Between March 2006 and August 2008, 104 devices were implanted in nine European centers. Preoperative, intraoperative, and follow-up data (at discharge, 6, 12, 24 months) including standardized echocardiography were prospectively registered, source data verified and collected in a central database.

Suturing of penetrating wounds to the heart in the nineteenth century: the beginnings of heart surgery.

The beginnings of cardiac surgery go back to the 19th century. This article describes the history of the first attempts to operate on the heart. In 1882, Dr Block from Danzig, and in 1895, Simplicio Del Vecchio, published reports of animal experiments showing that the suturing of heart wounds is possible.

Adaptive growth and remodeling of transplanted hearts in children.

Objective: We aimed to evaluate the adaptive growth and remodeling behavior of the transplanted heart in pediatric heart-transplant recipients by comparing donor body surface area (BSA) and cardiac dimensions during transplantation with the corresponding parameters of the recipient over a period of time.

Methods: A retrospective review of medical and echocardiographic records of 167 children (8.65 ± 5.

Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants and children.

Objective: Although mortality after direct aortic reimplantation for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has significantly decreased, many questions remain unanswered.

Methods: Between 1986 and June 2010, we operated on 27 consecutive pediatric patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). All patients underwent reestablishment of a dual coronary system with direct aortic reimplantation of the left coronary artery into the aorta.

Rescue extracorporeal membrane oxygenation in children with refractory cardiac arrest.

We describe our experience with extracorporeal cardiopulmonary resuscitation (CPR) using extracorporeal membrane oxygenation (ECMO) in children with refractory cardiac arrest, and determine predictors for mortality. ECMO support was instituted on 42 children, median age 0.7 years (1 day-17.

Pediatric heart transplantation: 23-year single-center experience.

Objective: Early and late mortality have significantly improved during recent decades in pediatric patients after heart transplantation (HTx). Nevertheless early and late morbidity and mortality are influenced by acute rejection, cardiac allograft vasculopathy (CAV), malignancy, renal failure, and graft failure.

Methods: We evaluated our results after HTx in children under the age of 18 years with 23 years of follow-up.

Single-center experience with treatment of cardiogenic shock in children by pediatric ventricular assist devices.

Background: Pediatric ventricular assist devices may be superior to extracorporeal membrane oxygenation in some respects, especially for medium- and long-term cardiac support. We present our nearly 20-year experience with pediatric ventricular assist devices.

Methods: Between 1990 and April 2009, Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) was implanted in 94 children.

Predictors for the use of left ventricular assist devices in infants with anomalous left coronary artery from the pulmonary artery.

Background: Left ventricular (LV) mechanical circulatory support (MCS) may be necessary after repair of anomalous left coronary artery from the pulmonary artery. We evaluated LV function parameters for their ability to predict postoperative need for MCS.

Methods: Fourteen infants (median age, 3.

The first closure of the persistent ductus arteriosus.

Congenital heart disease began to be a treatable condition when, in 1938, Robert Edward Gross first successfully ligated a persistent ductus arteriosus. This overview traces the historical development from Munro's first idea of how to close a patent ductus, presented in 1907, to the clinical ligation or division of the ductus. Surgical treatment of the infected ductus began with an unsuccessful attempt by Strieder, but it was not until Tubbs' and Touroff's successful operations that it was actually accomplished.

Extracorporeal membrane oxygenation for intraoperative cardiac support in children with congenital heart disease.

Objectives: Extracorporeal membrane oxygenation (ECMO) is commonly used in children to allow recovery from ischemic injury or cardiac surgery, to support the circulation in case of end-stage cardiomyopathy, as bridge-to-bridge therapy and as bridge to transplantation as well. It has achieved success in providing cardiac support for these kind of patients with expected mortality due to severe myocardial dysfunction. In this modern era, ECMO support should be considered an important option for children with cardiopulmonary failure refractory to medical therapy or resuscitation.

The practical clinical value of three-dimensional models of complex congenitally malformed hearts.

Objective: Detailed 3-dimensional anatomic information is essential when planning strategies of surgical treatment for patients with complex congenitally malformed hearts. Current imaging techniques, however, do not always provide all the necessary anatomic information in a user-friendly fashion. We sought to assess the practical clinical value of realistic 3-dimensional models of complex congenitally malformed hearts.

Long-term cardiopulmonary exercise capacity after modified Fontan operation.

Objective: Early circuit separation enhances the long-term success of Fontan haemodynamics. To test this hypothesis, we analysed the postoperative cardiopulmonary capacity in children and adults.

Patients: Spiroergometry was performed at least twice in 43 patients with a median age of 14 (range: 7-43) years, with a median time interval of 4.

Exercise capacity reflects ventricular function in patients having the Fontan circulation.

Background: In this study we sought to determine, first, whether maximal exercise capacity reflects ventricular function, and second, whether the age of the patient, and the age of completion of the Fontan circulation, influence ventricular function and exercise performance.

Methods And Results: Cardiac magnetic resonance imaging and cardiopulmonary exercise testing were performed in 29 patients at a median time of 6.9 years after completion of the Fontan circulation.

Absence of pulmonary artery growth after fontan operation and its possible impact on late outcome.

Background: The purpose of this study was to evaluate the development of the pulmonary arteries (PAs) after Fontan operation in children at long term.

Methods: Thirty-five patients in whom Fontan operation was performed at median age of 4.2 years (range, 1.

Partial anomalous pulmonary venous drainage in young pediatric patients: the role of magnetic resonance imaging.

Studies of larger patient groups for systematic assessment of the anatomical accuracy of magnetic resonance imaging (MRI) for partial anomalous pulmonary venous drainage (PAPVD) have been performed so far only in adults. This study was undertaken to evaluate whether MRI can precisely depict pulmonary venous anatomy in infants and young children. Data on 26 children under 10 years old that underwent MRI over the past 2 years for suspected PAPVD were assessed.

Analysis of arrhythmias after correction of partial anomalous pulmonary venous connection.

Background: Several methods for surgical repair of partial anomalous pulmonary venous connection have been described. Sinus node dysfunction is known as a cause of morbidity after surgical repair. In this retrospective study, we attempted to determine the incidence of arrhythmias after use of two different techniques for repair of partial anomalous pulmonary venous connection.