Magnetic resonance lymphangiography abnormalities as extracardiac biomarkers of pulmonary hypertension and functional phenotype in Fontan-Kreutzer univentricular circulation.

Introduction: Lymphatic abnormalities have been documented on magnetic resonance lymphangiography in patients with functionally univentricular circulation.

Objective: The aim of this study is to evaluate associations between findings on magnetic resonance lymphangiography, cardiac catheterisation and functional phenotype in patients with Fontan-Kreutzer circulation.

Materials And Methods: Between January 2017 and October 2019, seven patients with Fontan-Kreutzer circulation (16.

The World Society for Pediatric and Congenital Heart Surgery: 2024 Update of the World Database for Pediatric and Congenital Heart Surgery.

Background: The World Database for Pediatric and Congenital Heart Surgery (WDPCHS), sponsored by the World Society for Pediatric and Congenital Heart Surgery (WSPCHS), provides complex congenital heart surgery outcomes analyses for member programs. This report represents the seven-year descriptive analysis of outcomes from active members of the WDPCHS.

Methods: Individual institutions submit data based on the specific procedure via a password protected platform.

Fifth "Jatene Lecture on Surgical Innovation": Innovation in Congenital Heart Surgery: Contributions From South America.

We present the fifth "Jatene Lecture on Surgical Innovation" on Innovation in Congenital Heart Surgery, given at the Eighth Scientific Meeting of the World Society for Pediatric and Congenital Heart Surgery and Eighth World Congress of Pediatric Cardiology and Cardiac Surgery in Washington DC in 2023. We highlight what surgical innovation is and how innovation was accomplished in cardiac surgery and particularly in congenital heart surgery. A brief history of the development of congenital heart surgery across the world is summarized and we finally illustrate the South American contributions to congenital heart surgery, acknowledging the great innovations of Adib Jatene and Guillermo Kreutzer to our field.

Tetralogy of Fallot Repair After Neonatal Right Ventricular Outflow Tract Stenting: Initial Multicenter Experience in Argentina.

Initial management of patients with tetralogy of Fallot, unfavorable anatomy, and reduced pulmonary blood flow is controversial and continues to be a clinical challenge. Pulmonary to systemic shunt anastomosis or primary correction in neonates and small infants is associated with higher morbimortality and increased number of reoperations. Initial right ventricle outflow tract stenting palliation has emerged as an attractive alternative.

Percutaneous Closure of Ruptured Sinus of Valsalva Aneurysm in a Child Using a Patent Ductus Arteriosus Device.

Ruptured sinus of Valsalva aneurysm is rare, although if left untreated, potentially fatal disease. Surgical approach has been the main treatment in most series; nevertheless, percutaneous closure has been described in selected cases. We report a 5-year-old boy presenting with rapid clinical deterioration who underwent percutaneous closure using a patent ductus arteriosus device, with the resolution of symptoms.

Technical Aspects and Common Pitfalls of Norwood With Modified Blalock Taussig Shunt.

The Stage 1 Norwood procedure is the first of 3 stages in the surgical palliation of hypoplastic left heart syndrome and certain other single ventricle lesions with systemic outflow obstruction. In this article, we address some technical aspects and common pitfalls of the Norwood procedure with systemic to pulmonary shunt for HLHS palliation. We report our results with the Norwood with Blalock Taussig shunt in a cohort of 44 patients over a 7-year period in 2 institutions in Argentina.

Giant Right Ventricular Fibroma: Prenatal Diagnosis and Partial Resection in Early Infancy.

Congenital cardiac fibromas are very rare and prenatal diagnosis has been reported in just a few cases. We describe a four-month-old infant presenting a symptomatic giant right ventricular fibroma discovered during prenatal scanning at 33 weeks of gestation, which was confirmed after delivery on echocardiogram and cardiac magnetic resonance imaging. Due to progressive hemodynamic deterioration, partial surgical resection was performed and the patient recovered uneventfully.

Bailout procedure in obstructed supracardiac total anomalous pulmonary vein drainage.

Total anomalous pulmonary venous drainage is a rare and diverse anomaly, accounting for 1% to 3% of patients with congenital heart disease. Newborns with diagnosis of an obstructed total anomalous pulmonary venous dainage are extremely ill soon after birth and often present with severe cyanosis, pulmonary hypertension and low cardiac output requiring urgent surgical intervention. Transcatheter palliative stenting of the obstructive vertical vein can be an acceptable alternative as a bailout intervention before complete surgical correction is undertaken.

Magnetic resonance lymphangiography in group 1 paediatric pulmonary arterial hypertension.

Pulmonary hypertension could have thoracic lymphatic abnormalities caused by right ventricular failure. Since there is no description of such abnormalities, the purpose of this study was to investigate them with magnetic resonance. Prospective review magnetic resonance T2-weighted lymphangiography was performed between January 2017 and October 2019 through quantitative thoracic duct diameter, diameter index and qualitative lymphatic abnormalities types: 1 - little or none abnormalities, 2 - abnormalities in supraclavicular region, 3 - abnormalities extending into the mediastinum and 4 - abnormalities extending into the lung.

CAT25 defines microsatellite instability in colorectal cancer by high-resolution melting PCR.

Background: CAT25 (Tmononucleotide repeat of the Caspase 2 gene), is a promising DNA marker for detecting microsatellite instability (MSI) in colorectal cancer. CAT25 has the potential to be incorporated into the Bethesda panel, a commonly used panel of DNA microsatellites, or replace it in its entirety. We aimed to develop and validate a high-resolution melting-PCR (HRM-PCR) method for CAT25 instability detection in clinical samples.

Percutaneous Transhepatic Fontan-Kreutzer Completion of Hepatic Vein Inclusion.

We report the case of an 11-year-old girl with heterotaxy syndrome, dextrocardia, and azygos continuation of an interrupted inferior vena cava who had developed pulmonary arteriovenous fistulas after a Kawashima procedure consisting of bilateral superior cavopulmonary anastomoses. She presented with profound cyanosis, fatigue, and failure to thrive. An operative procedure to direct hepatic vein effluent to the pulmonary circulation was performed with placement of an extracardiac conduit between the hepatic veins and the left pulmonary artery.

A Rare Cause of Pediatric Stroke.

We describe a case of sudden-onset left-sided hemiparesis and dysarthria in a five-year-old boy. Acute vascular malformation bleeding or ischemic stroke was suspected. Neurological examination three weeks after the initial event revealed mild residual facial paresis.

Repair of the Left Pulmonary Artery After Bilateral Banding in Hybrid Procedures: Results Using "Sutureless" Repair Technique.

We describe the use of the so-called "sutureless" technique for repair of stenosis of the left pulmonary artery after bilateral banding in the management of two patients with hypoplastic left heart syndrome who had undergone initial palliation with a hybrid procedure. After bilateral pulmonary artery debanding, arterioplasty with autologous pericardial patch and sutureless technique was carried out to decrease the risk of restenosis. Both patients survived the operation, and the exit pulmonary angiography revealed adequate flow across the arterioplasty sites.

Absent pulmonary valve, tricuspid atresia, and congenital heart block.

We describe management of a patient with a prenatal diagnosis of absent pulmonary valve, tricuspid atresia, ventricular septal defect, and congenital heart block. Initial treatment consisted of temporary pacemaker implantation, and subsequent palliation included a central shunt during the neonatal period and placement of a permanent pacemaker. At seven months of age, a bidirectional Glenn anastomosis was performed.

Left main coronary artery atresia and associated cardiac defects: report on concomitant surgical treatment.

A 9-year-old boy with congenital atresia of the left main coronary artery underwent myocardial revascularization. Coarctation of the aorta and ventricular septal defect were diagnosed at the age of 1 year. At age 7 years, the child presented with syncope while exercising.

Surgical treatment of pulmonary venous tunnel stenosis [corrected] after modified Senning procedure.

A 13-year-old male was admitted to undergoing correction of a pulmonary venous baffle stenosis (PVBS) after a modified Senning procedure was performed by the age of five months. Recurrent Pulmonary congestion and pneumonia episodes were followed by echocardiography and cardiac catheterization that confirmed PVBS. Previous catheter balloon angioplasty was attempted, and a surgical revision was done under cardiopulmonary bypass.

[Granulomatous peritonitis due to the starch used in surgical gloves].

Granulomatous peritonitis due to surgical glove starch is an entity of difficult diagnosis and low incidence. Patients present with abdominal pain, fever and prolonged functional ileus three to four weeks postoperatory of a laparotomy where corn starch as glove's lubricant had been used. In surgical re-exploration both the peritoneum and omentum are widely affected with multiple small white nodules together with peritoneal fluid.