Surgical Ventricular Septal Defect Repair.

Ventricular septal defects (VSDs), the most common of congenital heart defects, vary widely in anatomy and size. Surgical treatment of very large VSDs can be challenging because no clear recommendations exist about which defects can undergo biventricular repair with septation and which should instead be palliated. Although Fontan circulation is known to be associated with long-term morbidity, information is limited about how ventricular septation of large VSDs affects cardiac function.

Risk stratification in Eisenmenger syndrome.

Pulmonary arterial hypertension complicating congenital heart disease can progress to the life-threatening irreversible form known as Eisenmenger syndrome. When conservative treatments are inadequate, the risk of death as a result of the disease must be weighed against the risk associated with transplantation. Risk stratification has become a fundamental tool for the prediction of outcomes and the guidance of treatment in pulmonary arterial hypertension.

4D flow cardiac MRI to assess pulmonary blood flow in patients with pulmonary arterial hypertension associated with congenital heart disease.

Purpose: The purpose of this study was to evaluate the accuracy of four-dimensional flow cardiac magnetic resonance imaging (4D flow MRI) compared to right heart catheterization in measuring pulmonary flow (Qp), systemic flow (Qs) and pulmonary-to-systemic flow ratio (Qp/Qs) in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD).

Materials And Methods: The study was registered on Clinical-trial.gov (NCT03928002).

A Modified Technique for Transcatheter Pulmonary Valve Implantation of SAPIEN 3 Valves in Large Right Ventricular Outflow Tract: A Matched Comparison Study.

Introduction: Percutaneous pulmonary valve implantation (PPVI) with a SAPIEN 3 valve is effective for treating treat right ventricle outflow (RVOT) dysfunction. A modified technique was developed without prestenting using a protective valve delivery method. We aimed to compare the procedural results of the modified technique group (MTG) to those of patients in a conventional technique group (CTG).

Thirty years of surgical management of pediatric pulmonary hypertension: Mid-term outcomes following reverse Potts shunt and transplantation.

Background: Reverse Potts shunt (RPS) and lung or heart-lung transplantation are life-extending surgical interventions for pediatric patients with severe pulmonary arterial hypertension (PAH). Robust criteria for identifying patients who will benefit from these procedures remain elusive. Based on 30 years of experience, we sought to refine the surgical indications.

Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.

Background And Aims: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI.

Methods: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries.

Cardiac Drugs in ACHD Cardiovascular Medicine.

Adult congenital heart disease (ACHD) is a growing population that requires life-long care due to advances in pediatric care and surgical or catheter procedures. Despite this, drug therapy in ACHD remains largely empiric due to the lack of clinical data, and formalized guidelines on drug therapy are currently lacking. The aging ACHD population has led to an increase in late cardiovascular complications such as heart failure, arrhythmias, and pulmonary hypertension.

Infective endocarditis after transcatheter pulmonary valve implantation in patients with congenital heart disease: Distinctive features.

The introduction of transcatheter pulmonary valve implantation (TPVI) has greatly benefited the management of right ventricular outflow tract dysfunction. Infective endocarditis (IE) is a feared complication of TPVI that affects valve durability and patient outcomes. Current recommendations provide only limited guidance on the management of IE after TPVI (TPVI-IE).

Multicentre experience with Optimus balloon-expandable cobalt-chromium stents in congenital heart disease interventions.

Objectives: To evaluate bare-metal Optimus and polytetrafluoroethylene (PTFE)-covered Optimus-CVS balloon-expandable, cobalt-chromium, hybrid cell-designed stents in congenital heart disease (CHD) interventions.

Methods: Retrospective multicentre review of patients with CHD receiving Optimus stents. Stent mechanical behaviour, clinical indications and outcomes were assessed.

Long-term outcomes of transcatheter pulmonary valve implantation with melody and SAPIEN valves.

Background: Transcatheter pulmonary valve implantation (TPVI) is effective for treating right ventricle outflow tract (RVOT) dysfunction. Factors associated with long-term valve durability remain to be investigated.

Methods: Consecutive patients successfully treated by TPVI with Melody valves (n = 32) and SAPIEN valves (n = 182) between 2008 and 2020 at a single tertiary centre were included prospectively and monitored.

Multiparametric evaluation of right ventricular function in pulmonary arterial hypertension associated with congenital heart disease.

Introduction And Objectives: Outcome in patients with congenital heart diseases and pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Two-dimensional echocardiographic parameters, such as strain imaging or RV end-systolic remodeling index (RVESRI) have emerged to quantify RV function.

Methods: We prospectively studied 30 patients aged 48±12 years with pretricuspid shunt and PAH and investigated the accuracy of multiple echocardiographic parameters of RV function (tricuspid annular plane systolic excursion, tricuspid annular peak systolic velocity, RV systolic-to-diastolic duration ratio, right atrial area, RV fractional area change, RV global longitudinal strain and RVESRI) to RV ejection fraction measured by cardiac magnetic resonance.

Cardiac tomography-echocardiography imaging fusion: a new approach to congenital heart disease.

Introduction And Objectives: Diagnosis, management, and surgical decision-making in children and adults with congenital heart disease are largely based on echocardiography findings. A recent development in cardiac imaging is fusion of different imaging modalities. Our objective was to evaluate the feasibility of computed tomography (CT) and 3-dimensional (3D) transthoracic echocardiography (TTE) fusion in children and adults with congenital heart disease.

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

Unlabelled: The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present.

Mid-Term Outcomes Following Percutaneous Pulmonary Valve Implantation Using the "Folded Melody Valve" Technique.

Background: The folded valve is a manual shortening of the Melody device, which has been validated as a valuable therapeutic option for the management of dysfunctional right ventricular outflow tracts needing a short valved stent. In this article, we aimed to evaluate, in a multicenter cohort, the mid-term outcomes of patients in whom a percutaneous pulmonary valve implantation was performed using the folded valve technique.

Methods: A 2012 to 2018 retrospective multicenter study was performed in 7 European institutions.

Pulmonary Atresia With Ventriculocoronary Arterial Connections and a Large Conoventricular Septal Defect.

Ventriculocoronary arterial connections are typically found in patients with pulmonary atresia with an intact ventricular septum. This report describes a case of ventriculocoronary arterial connections in a patient with pulmonary atresia with a ventricular septal defect. Our case supports recent data suggesting a primary coronary artery developmental anomaly in pulmonary atresia.