Staged Treatment for Critically Ill Neonates With Ebstein Anomaly: Cone Repair After Starnes Procedure.

Following the Starnes procedure, a progressive reduction in the RV cavity occurs, suggesting that the RV was unsuitable for a biventricular repair. For that reason, those patients traditionally followed the single ventricle pathway. In 2019, we performed the initial tricuspid valve (TV) repair cases after Starnes.

The American Association for Thoracic Surgery (AATS) 2025 Expert Consensus Document: Management of Ebstein anomaly in children and adults.

Objectives: Patients with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focusing on preoperative assessment, indications for intervention, and medical management before and after surgical intervention in older children and adults. This is a companion document to a recently issued document regarding management of EA in neonates and infants.

The cone repair allows right ventricle rehabilitation with excellent tricuspid valve function following the Starnes procedure.

Background: We present a case series of right ventricle (RV) rehabilitation after the Starnes procedure in patients with Ebstein anomaly (EA), applying the Cone repair of the tricuspid valve (TV) to achieve 2-ventricle or 1.5-ventricle physiology.

Methods: This is a retrospective database analysis from 2 institutions in North America.

Using DaSilva Cone Operation to Establish 1.5 or 2 Ventricle Circulation After Initial Single Ventricle Palliation with Starnes Procedure.

Ebstein anomaly (EA) is a congenital dysplasia of the tricuspid valve resulting in reduced right ventricular (RV) volume and tricuspid regurgitation. Severe EA in the neonatal period is associated with high mortality. The Starnes procedure (fenestrated RV exclusion) is reserved for EA patients with cardiogenic shock and has previously committed patients to single ventricle (SV) palliation.

Early Outcomes of the Norwood Procedure in a Reference Center in Brazil.

Background: Only two papers have addressed the early outcomes of patients with hypoplastic left heart syndrome (HLHS) undergoing the Norwood operation, in Brazil.

Objectives: We evaluated patients with HLHS undergoing the first-stage Norwood operation in order to identify the predictive factors for early (within the first 30 days after surgery) and intermediate (from early survival up to the Glenn procedure) mortality.

Methods: Patients with HLHS undergoing the stage I Norwood procedure from January 2016 through April 2019, in our service, were enrolled.

Extracorporeal Membrane Oxygenation After Norwood Surgery in Patients With Hypoplastic Left Heart Syndrome: A Retrospective Single-Center Cohort Study From Brazil.

Background: Extracorporeal membrane oxygenation (ECMO) is increasingly being used to support patients after the repair of congenital heart disease.

Objective: We report our experience with patients with a single functional ventricle who were supported by ECMO after the Norwood procedure, reviewing the outcomes and identifying risk factors for mortality in these patients.

Methods: In this single-center retrospective cohort study, we enrolled 33 patients with hypoplastic left heart syndrome (HLHS) who received ECMO support after the Norwood procedure between January 2015 and December 2019.

Critical Ebstein's anomaly with circular shunt: from successful fetal therapy with non-steroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique.

We report an innovative treatment strategy for fetal Ebstein's anomaly with a circular shunt. We used transplacental non-steroidal anti-inflammatory drugs, at the 29th gestational week, to constrict the ductus arteriosus avoiding fetal demise. We addressed the critical neonate with an urgent Starnes procedure.

Cone versus conventional repair for Ebstein's anomaly.

Objectives: We aimed to investigate tricuspid valve function and adverse events after conventional repair and valve replacement for Ebstein's anomaly and compare them with cone repair.

Methods: The medical records of 151 patients (mean age, 25 years; 62% were female) who underwent operation in a single center from 1985 to 2018 were retrospectively analyzed. To determine tricuspid valve regurgitation during follow-up, serial echocardiographic examination was used (n = 2397, tricuspid regurgitation grades were graphed for every patient).

Right Ventricular Systolic Function After the Cone Procedure for Ebstein's Anomaly: Comparison Between Echocardiography and Cardiac Magnetic Resonance.

Although the Cone procedure has improved outcomes for patients with Ebstein´s anomaly (EA), neither RV systolic function recovery in long-term follow-up nor the best echocardiographic parameters to assess RV function are well established. Thus, we evaluated RV performance after the Cone procedure comparing two-dimensional (2DEcho) and three-dimensional (3DEcho) echocardiography to cardiac magnetic resonance (CMR). We assessed 27 EA patients after the Cone procedure (53% female, median age of 20 years at the procedure, median post-operative follow-up duration of 8 years).

Ebstein's anomaly: contemporary management strategies.

Ebstein's anomaly (EA) is a rare congenital cardiac anomaly. It is a disease at a minimum of the tricuspid valve (TV) and the right ventricular myocardium. Presentation varies from a severe symptomatic form during the neonatal period to an incidental detection later in life due to the wide morphological variation of the condition.

Root translocation in congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis, and other lesions.

The pulmonary root translocation (PRT) procedure has been used to correct ventriculoarterial discordance or malposition of great arteries since 1994. It was part of the surgical repair of 62 consecutive patients presenting with congenitally corrected transposition of the great arteries (CCTGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS), or other complex congenital heart disease with malposition of the great arteries, VSD, and PS. PRT was performed as follows: removal of the pulmonary artery (PA) with the pulmonary valve from its abnormal position, closure of the consequent hole with an autologous pericardial patch, resection of some conal septum, creation of an intraventricular tunnel connecting the left ventricle to the aorta, and construction of a new right ventricular outflow tract using the translocated PA.

Therapeutic options to minimize allogeneic blood transfusions and their adverse effects in cardiac surgery: a systematic review.

Introduction: Allogeneic blood is an exhaustible therapeutic resource. New evidence indicates that blood consumption is excessive and that donations have decreased, resulting in reduced blood supplies worldwide. Blood transfusions are associated with increased morbidity and mortality, as well as higher hospital costs.

Da Silva's cone repair for Ebstein's anomaly: effect on right ventricular size and function.

Objectives: Da Silva's cone repair is a novel technique for surgical reconstruction of the tricuspid valve and the right ventricle (RV) in Ebstein's anomaly. The technique consists of extensive leaflet mobilization, longitudinal plication of the atrialized ventricle and cone-shaped reconstruction of the tricuspid valve, allowing for leaflet-to-leaflet coaptation. We evaluated the influence of Da Silva's cone repair on tricuspid competency, right ventricular size and function.

Horizontal right axillary minithoracotomy: aesthetic and effective option for atrial and ventricular septal defect repair in infants and toddlers.

Introduction: Congenital heart defects treatment shows progressive reduction in morbidity and mortality, however, the scar, resulting from ventricular (VSD) and atrial septal defect (ASD) repair, may cause discomfort. Right axillary minithoracotomy approach, by avoiding the breast growth region, is an option for correction of these defects that may provide better aesthetic results at low cost. Since October 2011, we have been using this technique for repairing VSD and ASD defects as well as associated defects.

Role of echocardiography in the ventricular assessment of the transplanted heart versus heart rejection.

Background: Heart transplantation is an alternative for individuals with end-stage heart disease. However, episodes of heart rejection (HR) are frequent and increase morbidity and mortality, requiring the use of an accurate non-invasive exam for their diagnosis, since endomyocardial biopsy (EMB) is not a complication-free procedure.

Objective: To compare the parameters obtained by use of Doppler echocardiography in a group of transplanted patients with HR (TX1) and another group of transplanted patients without rejection (TX0), having as reference a control group (CG) and observing the behavior of the left ventricular systo-diastolic function expressed as the myocardial performance index (MPI) METHODS: Transthoracic echocardiographies were performed from January 2006 to January 2008 to prospectively assess 47 patients divided into three groups: CG (36.

Heart retransplantation in children without the use of blood product.

This article reports a case of a cardiac retransplantation without the use of blood products, in a 6 year old, with severe dilated cardiomyopathy after chronic graft rejection and refractory to clinical treatment. To avoid a blood transfusion in this surgery a multidisciplinary approach was planned, which involved the use of preoperative erythropoietin, acute normovolemic hemodilution and intraoperative cell savage with autologous blood recovery system, as well as a meticulous hemostasis and reduced postoperative phlebotomy.

Ebstein's anomaly of the tricuspid valve: the cone repair.

Ebstein's anomaly is a cardiac malformation affecting the tricuspid valve and right ventricle with a wide range of anatomic and pathophysiologic presentations. The cone repair technique is described in detail, as applied to several types of this complex congenital heart disease. The importance of extensive mobilization of displaced and tethered tricuspid leaflets to permit a good leaflet-to-leaflet coaptation after the cone construction is highlighted.

Pulmonary root translocation in malposition of great arteries repair allows right ventricular outflow tract growth.

Objective: Optimal surgical treatment of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS) remains a matter of debate. This study evaluated the clinical outcome and right ventricle outflow tract performance in the long-term follow-up of patients subjected to pulmonary root translocation (PRT) as part of their surgical repair.

Methods: From April 1994 to December 2010, we operated on 44 consecutive patients (median age, 11 months).