Research priorities in children and adults with congenital heart disease: a James Lind Alliance Priority Setting Partnership.

Objective: To bring together patients, parents, charities and clinicians in a Priority Setting Partnership to establish national clinical priorities for research in children and adults with congenital heart disease.

Methods: The established James Lind Alliance methodology was used to identify and prioritise research on the management of congenital heart disease, focusing on diagnosis, treatment and outcomes. An initial open survey was used to gather potential uncertainties which were filtered, categorised, converted into summary questions and checked against current evidence.

Post-infarction ventricular septal defect: percutaneous or surgical management in the UK national registry.

Aims: Post-infarction ventricular septal defect (PIVSD) is a mechanical complication of acute myocardial infarction (AMI) with a poor prognosis. Surgical repair is the mainstay of treatment, although percutaneous closure is increasingly undertaken.

Methods And Resuts: Patients treated with surgical or percutaneous repair of PIVSD (2010-2021) were identified at 16 UK centres.

Irbesartan in Marfan syndrome (AIMS): a double-blind, placebo-controlled randomised trial.

Background: Irbesartan, a long acting selective angiotensin-1 receptor inhibitor, in Marfan syndrome might reduce aortic dilatation, which is associated with dissection and rupture. We aimed to determine the effects of irbesartan on the rate of aortic dilatation in children and adults with Marfan syndrome.

Methods: We did a placebo-controlled, double-blind randomised trial at 22 centres in the UK.

Permanent pacing post-Fontan is not associated with reduced long-term survival.

Background: The Fontan procedure is the final stage of surgical palliation for a single-ventricle circulation. Significant complications are common including rhythm disturbance necessitating implantation of a permanent pacemaker. This has been widely considered a negative prognostic indicator.

Mid-Term Valve-Related Outcomes After Transcatheter Tricuspid Valve-in-Valve or Valve-in-Ring Replacement.

Background: Transcatheter aortic and pulmonary valves have been used to treat stenosis or regurgitation after prior surgical tricuspid valve (TV) replacement or repair. Little is known about intermediate-term valve-related outcomes after transcatheter tricuspid valve replacement (TTVR), including valve function, thrombus, and endocarditis.

Objectives: The authors sought to evaluate mid-term outcomes in a large cohort of patients who underwent TTVR after surgical TV repair or replacement, with a focus on valve-related outcomes.

Outcomes of Transcatheter Tricuspid Valve-in-Valve Implantation in Patients With Ebstein Anomaly.

We sought to describe the acute results and short- to medium-term durability of transcatheter tricuspid valve-in-valve (TVIV) implantation within surgical bioprostheses among patients with Ebstein anomaly (EA). Cases were identified from a voluntary, multicenter, international registry of 29 institutions that perform TVIV. Demographic, clinical, procedural, and follow-up data were analyzed.

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.

Transcatheter Tricuspid Valve-in-Valve Implantation for the Treatment of Dysfunctional Surgical Bioprosthetic Valves: An International, Multicenter Registry Study.

Background: Off-label use of transcatheter aortic and pulmonary valve prostheses for tricuspid valve-in-valve implantation (TVIV) within dysfunctional surgical tricuspid valve (TV) bioprostheses has been described in small reports.

Methods And Results: An international, multicenter registry was developed to collect data on TVIV cases. Patient-related factors, procedural details and outcomes, and follow-up data were analyzed.

Effect of interventional stent treatment of native and recurrent coarctation of aorta on blood pressure.

The aim of this study was to describe the clinical impact of management of coarctation of the aorta by transcatheter stent placement in the context of longer term management of systemic hypertension. In the long term, poor outlook associated with untreated coarctation of the aorta is likely to relate to uncontrolled systemic hypertension. Transcatheter stent placement to treat native and recurrent coarctation of the aorta is an established therapy in adolescents and adults.

Transcatheter closure of persistent ductus arteriosus in adults.

Objective: To describe the technical aspects and outcome of duct occlusion in adults over a 12-year period.

Methods: A single center review of all transcatheter duct closures performed between 2000 and 2012.

Results: Of 518 transcatheter duct closures performed, 31 patients were over the age of 16 at the time of procedure (6%).

Transcatheter rehabilitation of pulmonary arteries.

Pulmonary arterial stenoses commonly occur in patients with congenital heart disease. Indications for treatment are based around reduction of right ventricular hypertension and equalization of flow to each lung and its constituent segments. There are many treatment approaches for these lesions depending on the anatomy, location and the age of the patient.

Cardiac computed tomography and conventional angiography in the diagnosis of congenital cardiac disease in children: recent trends and radiation doses.

Background: The use of imaging that employs ionising radiation is increasing in the setting of paediatric cardiology. Children's high radiosensitivity and the lack of contemporary radiation data warrant a review of the radiation doses from the latest "state-of-the-art" angiography and computed tomography systems.

Objectives: In children aged less than 16 years with congenital cardiac disease, we aimed to report: recent trends in the use of diagnostic angiography and cardiac dual-source computed tomography; the characteristics, lesions, and imaging histories of patients undergoing these procedures; and the average radiation doses imparted by each modality.

Transcatheter closure of the arterial duct without arterial access.

Objective: To evaluate the safety and efficacy of transcatheter occlusion of the arterial duct without femoral arterial catheterization.

Background: Patent arterial ducts have been closed percutaneously since the 1960s. It remains standard practice to use arterial access for aortography before, during, and after implantation of the device.

Development of a cardiac technician led paediatric echocardiographic service--experience from a district general hospital in the United Kingdom.

Objectives: To report our experience in providing cardiac technician led paediatric echocardiography services in a district general hospital in the United Kingdom.

Methods: We have collected prospectively the numbers of referrals, and the proportion of abnormal echocardiograms, since inception of the service in 2000. In additional, for a period of 12 months, we have audited in detail the patterns of referral to the service, and outcomes, assessing the effect of the service on the outreach clinic run by a visiting paediatric cardiologist.

Pacing activity, patient and lead survival over 20 years of permanent epicardial pacing in children.

Background: We report on pediatric epicardial pacing activity, patient and lead survival for more than two decades in a single center.

Methods: The data cover 96 pacing leads implanted in 59 patients. Median age at implantation was 1.