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.

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.

Lack of tight association between quality of life and exercise capacity in pulmonary arterial hypertension.

Background: In pulmonary arterial hypertension (PAH) health-related quality of life (HRQOL) has been investigated over the short-term (weeks) but little is known about patient's perspective over the medium and long term.

Objective: To analyze how patients on specific PAH therapies do over one year of observation in terms of HRQOL, and to investigate if possible associations between the exercise capacity (EC) and HRQOL persist over the medium term.

Methods: Thirty-four patients on PAH therapies (bosentan and/or sildenafil) were enrolled (age 14 to 58 years, median 35.

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).

Occlusive venopathy phenotype in hereditary pulmonary arterial hypertension.

A 33-year-old male with severe hereditary pulmonary arterial hypertension had a confirmed diagnosis of occlusive venopathy and microvasculopathy. He remained stable for three and a half years on oral sildenafil, 75 mg t.i.

Cone reconstruction in Ebstein's anomaly repair: early and long-term results.

Background: The main Ebstein anomaly (EA) repairs are based on the monocusp reconstruction of the tricuspid valve and are limited by the frequent need for replacement or the high recurrence of valve regurgitation.

Objective: To evaluate the feasibility and effects of anatomical repair of Ebstein's anomaly using the cone reconstruction technique on patients' clinical evaluation, tricuspid valve function and right ventricular morphology.

Methods: We compared the clinical, echocardiographic and radiological data of 52 consecutive patients, with a mean age of 18.

The hypoplastic left heart syndrome is not a risk factor for Fontan operation.

Objective: To show the mortality rate of children undergoing to Fontan operation and determine whether the hypoplastic left heart syndrome (HLHS) is a risk factor for hospital mortality.

Methods: From August 2001 to June 2008, 76 patients underwent Fontan operation and were divided into two groups: group A with 54 patients, 31 (40.7%) patients with tricuspid atresia and variants, six (7.

[Two-year follow-up of pulmonary arterial hypertension patients treated with sildenafil].

Background: The long-term effects of drugs developed for the control of pulmonary arterial hypertension (PAH) are little known, since multicenter studies usually last 12 to 16 weeks.

Objective: To evaluate the two-year outcome of PAH patients receiving monotherapy with sildenafil (a phosphodiesterase-5 inhibitor), with regard to their functional capacity.

Methods: Twenty four patients (ages between 8 and 54 years) with idiopathic PAH (IPAH, n = 9) or congenital heart disease-associated PAH (CHD-PAH, n = 15) were treated with sildenafil for two years, with daily oral doses ranging from 60 to 225 mg (tid).

Hypoplastic left heart syndrome: the report of a surgical strategy and comparative results of Norwood x Norwood-Sano approach.

Objectives: To report a surgical strategy for the Norwood procedure in the hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest time and aortic arch reconstruction with autologous pericardium patch. To compare the results of the modified Blalock-Taussig (mBT) shunt and the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow.

Method: Retrospective study of 78 newborns consecutively operated between March, 1999 and June 2006.

Hypoplastic left heart syndrome: the influence of surgical strategy on outcomes.

Objectives: To report a surgical strategy for the Norwood procedure in the hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest time and aortic arch reconstruction with autologous pericardium patch, and to compare the results of the modified Blalock-Taussig (mBT) shunt with the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow.

Methods: Retrospective study of 71 newborns with HLHS consecutively operated between March, 1999 and February, 2006. One technique for reconstruction of the neoaorta and two different techniques for reestablishment of the pulmonary blood flow were used: the mBT shunt in the first 37 newborns and RV-PA conduit in the last 34.

The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results.

Objectives: We sought to describe a new technique for tricuspid valve repair in Ebstein's anomaly and to report early echocardiographic results, as well as early and midterm clinical outcomes.

Methods: From November 1993 through August 2005, 40 consecutive patients with Ebstein's anomaly (mean age, 16.8 +/- 12.

[Ebstein's anomaly: results of the conic reconstruction of the tricuspid valve].

Objective: To assess the results of a technical modification of tricuspid valvuloplasty in Ebstein's anomaly.

Methods: From November 1993 to August 2002, 21 patients with Ebstein's anomaly of the tricuspid valve, with ages ranging from 20 months to 37 years (mean, 23 years), underwent a new technique of tricuspid valvuloplasty. This technique consisted of total or almost total detachment of the anterior tricuspid megaleaflet from the ventricular wall and valvular ring, transforming it into a cone, whose vertex remained fixed in the right ventricular tip, and the base was sutured to the tricuspid ring, after its plication, adjusting it to the size of the base of the cone (tricuspid ring), including the septal region.