Outcomes of the arterial switch for transposition during infancy using a standardized approach over 30 years.

Objectives: The aim of this study was to describe the early and late outcomes of the arterial switch for transposition.

Methods: A single-centre retrospective cohort study was conducted to assess the early and late outcomes of arterial switch performed during infancy using a standardized institutional approach between 1988 and 2018, compared by morphological groups.

Results: A total of 749 consecutive patients undergoing arterial switch during infancy were included, 464 (61.

Supravalvular pulmonary stenosis: A risk factor for reintervention in Noonan syndrome with pulmonary valve stenosis.

Objectives: To assess the short- and long-term outcomes of balloon pulmonary valvuloplasty (BPV) in children with Noonan syndrome (NS).

Background: Pulmonary stenosis (PS) is the most common congenital heart lesion in NS. BPV is the accepted first line treatment in PS.

Trends in surgical and catheter interventions for isolated congenital shunt lesions in the UK and Ireland.

Objective: To evaluate time trends in the use of catheter and surgical procedures, and associated survival in isolated congenital shunt lesions.

Methods: Nationwide, retrospective observational study of the UK National Congenital Heart Disease Audit database from 2000 to 2016. Patients undergoing surgical or catheter procedures for atrial septal defect (including sinus venosus defect), patent foramen ovale, ventricular septal defect and patent arterial duct were included.

Stenting and overdilating small Gore-Tex vascular grafts in complex congenital heart disease.

Background: Gore-Tex grafts are integral in the management of congenital heart disease. Issues of graft stenosis or somatic outgrowth may precipitate high-risk early surgery, and catheter intervention is a relatively under-reported management option.

Objectives: To assess efficacy, safety, and outcomes of stenting and overdilating small Gore-Tex vascular grafts with the aim of optimizing surgical timing.

Duct Stenting Versus Modified Blalock-Taussig Shunt in Neonates With Duct-Dependent Pulmonary Blood Flow: Associations With Clinical Outcomes in a Multicenter National Study.

Background: Infants born with cardiac abnormalities causing dependence on the arterial duct for pulmonary blood flow are often palliated with a shunt usually between the subclavian artery and either pulmonary artery. A so-called modified Blalock-Taussig shunt allows progress through early life to an age and weight at which repair or further more stable palliation can be safely achieved. Modified Blalock-Taussig shunts continue to present concern for postprocedural instability and early mortality such that other alternatives continue to be explored.

Outcome after transcatheter occlusion of patent ductus arteriosus in infants less than 6 kg: A national study from United Kingdom and Ireland.

Objectives: This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing <6 kg.

Background: The technique of transcatheter PDA closure has evolved in the past two decades and is increasingly used in smaller patients but data on safety and efficacy are limited.

Methods: Patients weighing < 6 kg in whom transcatheter PDA occlusion was attempted in 13 tertiary paediatric cardiology units in the United Kingdom and Ireland were retrospectively analyzed to review the outcome and complications.

Transcatheter Retrieval of Cardiovascular Foreign Bodies in Children: A 15-Year Single Centre Experience.

There has been a rapid increase in the practice of interventional catheter treatment of congenital heart disease. Catheter retrieval of embolized cardiac devices and other foreign bodies is essential, yet no large studies have been reported in the paediatric population. Retrospective 15-year review of all children who underwent transcatheter foreign body retrieval in a tertiary cardiac centre from January 1997 to September 2012.

Loss-of-function mutations in the X-linked biglycan gene cause a severe syndromic form of thoracic aortic aneurysms and dissections.

Purpose: Thoracic aortic aneurysm and dissection (TAAD) is typically inherited in an autosomal dominant manner, but rare X-linked families have been described. So far, the only known X-linked gene is FLNA, which is associated with the periventricular nodular heterotopia type of Ehlers-Danlos syndrome. However, mutations in this gene explain only a small number of X-linked TAAD families.

Stenting of the left pulmonary artery after palliation of hypoplastic left heart syndrome.

Background: Left pulmonary artery stenosis and hypoplasia is a well-recognized complication following surgical palliation of hypoplastic left heart syndrome. These lesions produce increased after load in a circulation in series so need to be effectively treated.

Methods: Between 2000 and 2011, 86 patients after surgical palliation for hypoplastic left heart syndrome had left pulmonary artery stents implanted.

Initial experience with the cook formula balloon expandable stent in congenital heart disease.

Introduction: Balloon expandable stents are an integral part in the catheter treatment of congenital heart disease. In the growing child, stents require dilatation to greater diameters over time. The Cook Formula stent is a recent 316 stainless steel open-cell design licensed for peripheral vascular work.

Twenty-year outcome of anomalous origin of left coronary artery from pulmonary artery: management of mitral regurgitation.

Background: This study is a single-center experience with surgical repair of anomalous origin of left coronary artery from pulmonary artery (ALCAPA) with focus on the management of associated mitral regurgitation (MR).

Methods: We performed a retrospective analysis of cases presenting to a quaternary referral center between November 1990 and October 2011.

Results: In all, 25 patients (18 female) presented with a diagnosis of ALCAPA at a median age of 5 months (range, 1.

Stenting of the right ventricular outflow tract.

Objective: To assess the indication, technical aspects, and outcome of stenting of the right ventricular outflow tract (RVOT) in the management of symptomatic patients with severely limited pulmonary blood flow.

Methods: Retrospective case note and procedure review of patients undergoing stenting of the RVOT over an 8 year period.

Patients: Between 2005 and 2012, 52 selected patients underwent percutaneous stent implantation into a very narrow RVOT to improve pulmonary blood flow.

Catheter interventions in the staged management of hypoplastic left heart syndrome.

Aim: To analyse the current practice and contribution of catheter interventions in the staged management of patients with hypoplastic left heart syndrome.

Methods: This study is a retrospective case note review of 527 patients undergoing staged Norwood/Fontan palliation at a single centre between 1993 and 2010. Indications and type of catheter interventions were reviewed over a median follow-up period of 7.

The management of neonatal pulmonary hypertension.

Most neonates with clinically significant pulmonary hypertension (PH) will have either persistent PH of the newborn (PPHN) or bronchopulmonary dysplasia. Cyanotic congenital heart disease must be actively ruled out as part of the differential diagnosis of PPHN. The maintenance of ductal patency with prostaglandins E1 or E2 in cases of doubt is safe and potentially beneficial given their pulmonary vasorelaxant properties.

Transcatheter interventions in the early postoperative period after the Fontan procedure.

Objective: To analyze the safety and clinical impact of interventional cardiac catheter procedures in the management of early postoperative problems after completion of an extracardiac Fontan procedure.

Background: The mortality after Fontan procedure has consistently decreased over the last decade. The role of interventional catheterization to address early postoperative problems in this setting has not been studied systematically.

Acute interventions for stenosed right ventricle-pulmonary artery conduit following the right-sided modification of Norwood-Sano procedure.

Introduction: The Norwood stage 1 procedure was modified by Sano with right ventricle-pulmonary artery (RV-PA) conduit replacing BT shunt. In our institution, this has been further modified by placing the conduit from the RV outflow tract to the right side of the neo-aorta.

Patients And Methods: Between April 2002 and October 2008, 227 modified Norwood procedures were performed.

Surgical management of hypoplastic left heart syndrome at the Birmingham Children's Hospital.

Currently, a three-stage surgical palliation remains the treatment of choice at Birmingham Children's Hospital. After initial introduction of the classical Norwood with pulmonary blood flow provided by a modified Blalock-Taussig shunt, a right ventricular to right pulmonary artery conduit at stage 1 Norwood palliation is now used in most cases, a bi-directional 'Glenn' shunt at second stage and an extra-cardiac Fontan completion at third stage. Mortality and morbidity has improved after modification of the technique.

The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction.

Objective: The aim of this study was to compare the outcome of the double-switch procedure for congenitally corrected transposition of the great arteries for patients completing morphologic left ventricle training by means of pulmonary artery banding with the outcome of patients whose morphologic left ventricle did not require training.

Methods: A retrospective study of all patients undergoing the double-switch procedure from 1991 through 2004 was performed. Patients were divided into 2 groups: those not requiring morphologic left ventricle training (n = 33) and those completing morphologic left ventricle training by means of pulmonary artery banding (n = 11).

Transcatheter closure of ventriculopulmonary artery communications in staged Fontan procedures.

Background: Ventricle-pulmonary artery connections in patients after the Fontan procedure lead to ineffective volume loading and can cause long term problems. In patients with a cavopulmonary shunt anterograde pulmonary blood flow is frequently maintained, but can cause significant volume loading of the heart or complicate the subsequent Fontan procedure.

Objective: To evaluate the use of transcatheter closure of a ventricle-pulmonary artery communication in the setting of a cavopulmonary shunt or after the Fontan procedure.

Experience with intraoperative ultrasound in paediatric cardiac surgery.

Objective: Intraoperative ultrasound was introduced to evaluate the adequacy of repair after surgical repair of congenital cardiac malformations. Our purpose was to review the evolution of this technique at our centre.

Methods: We evaluated all intraoperative ultrasound studies undertaken between 1997 and 2002, reviewing the data from 1997 through 2001 retrospectively, but undertaking a prospective audit of studies undertaken from 2002 onwards.

The influence of pulmonary artery morphology on the results of operations for major aortopulmonary collateral arteries and complex congenital heart defects.

Objective: Congenital heart defects with major aortopulmonary collateral arteries show marked variability in the size and distribution of native pulmonary arteries. We sought to classify the size and distribution of native pulmonary arteries and to determine their influence on surgical outcome.

Methods: Between 1989 and 2002, 164 patients underwent surgical intervention for congenital heart defects with major aortopulmonary collateral arteries (median age, 10 months).

Surgical aortic valvotomy in infancy: impact of leaflet morphology on long-term outcomes.

Background: Surgical valvotomy for critical aortic stenosis in children enables relatively accurate commissurotomies to be fashioned, resulting in the formation of two or three leaflets. We hypothesized that outcomes after surgery may be best in patients in whom three leaflets are produced.

Methods: A retrospective review of infants undergoing primary surgical valvotomy at our institution during a 12-year period was carried out.

Midterm results after restoration of the morphologically left ventricle to the systemic circulation in patients with congenitally corrected transposition of the great arteries.

Objective: This study was undertaken to determine the outcomes of patients with congenitally corrected transposition of the great arteries after restoration of the morphologically left ventricle to the systemic circulation.

Methods: Between November 1991 and June 2001, a total of 54 patients (median age 3.2 years, range 7 weeks-40 years) with either congenitally corrected transposition of the great arteries (n = 51) or atrioventricular discordance with double-outlet right ventricle (n = 3) underwent anatomic repair.