Balloon atrial septostomy through azygos vein in two cases of D-TGA with left atrial isomerism

Balloon atrial septostomy is a palliative procedure that is performed in D-transposition of great arteries when surgery is not immediately available. Although D-TGA and left isomerism association are rare, it is an important condition as the BAS procedure approach is unique. In this case report, we present two cases of D-TGA with left isomerism where BAS was performed due to restrictive atrial septal defect and lack of immediate availability of the paediatric cardiac surgeon.

Infants with pulmonary atresia intact ventricular septum who require balloon atrial septostomy have significantly higher 18-month mortality

Introduction: Many newborns with pulmonary atresia/intact ventricular septum require intervention to establish pulmonary flow and sufficient cardiac output. The resulting haemodynamic changes are not well characterised and may have unintended consequences. Methods: This is a 30-year (1988–2018) retrospective study of patients with pulmonary atresia intact ventricular septum. Results: Eighty-nine patients were included, and median follow-up was 8 years. Fifty-five per cent had coronary sinusoids and 27% had right ventricular-dependent coronary circulation. Most patients were managed with surgical aortopulmonary or modified Blalock–Taussig shunt (73%), and 12 patients underwent balloon atrial septostomy before surgical intervention. The remaining patients (27%) underwent only transcatheter interventions; 7 required an atrial septostomy and 17 required ductal stentings. All-cause mortality was 10%, most deaths (89%) occurred before 18 months of age. Of these early deaths, 87% required a balloon atrial septostomy and 85% had right ventricular-dependent coronary sinusoids. Eighteen-month mortality was significantly higher for patients who required a balloon atrial septostomy compared to those who did not (36% versus 1.4% p < 0.0001). Discussion: Patients with pulmonary atresia/intact ventricular septum who require balloon atrial septostomy in the newborn period have significantly higher 18-month mortality. Quantifying the mortality difference may help guide prognostication and expectation setting. Infants who had septostomy and a surgical shunt in the newborn period fared better than those who only underwent septostomy (even when accompanied by ductal stenting). For infants with right ventricular-dependent circulation, atrial septostomy should only be performed on an urgent or emergent basis and these patients should be considered for early surgical intervention and neonatal transplant.

Size of atrial septal defect after balloon atrial septostomy does not correlate with immediate improvement in oxygenation

ObjectiveTransposition of great arteries is a common cyanotic heart defect. Balloon atrial septostomy aims to improve circulatory mixing and oxygenation. Previous studies have combined infants with intact ventricular septum and those with ventricular septal defect. Additionally, the septostomy was performed much later after birth. The objectives were to ascertain any correlation between the atrial septal defect size and oxygenation, before and after septostomy, as well the change in parameters pre-post procedure.MethodsWe performed an audit of the last 10 years of clinical and echocardiographic data (2010–2020) for infants with transposition of great arteries with intact ventricular septum. A pediatric cardiologist, masked to clinical data, reviewed the images.ResultsOur study of 25 infants with transposition of great arteries with intact ventricular septum noted that the procedure was performed at a median [interquartile range (IQR)] of 3 (2, 4) hours after birth. Prostaglandin was administered to the majority of infants [20/25 (80%)]. While significant increases in partial pressure of oxygen (24±5 vs 40±6 mmHg, p<0.001) and preductal oxygen saturations (67%±18% vs 81%±11%, p=0.003) were noted, and while the atrial septal defect increased in size from 1.8±0.6 vs 4.8±0.7 mm (p<0.001), no correlation was noted between atrial septal defect size and oxygen saturations.ConclusionsIn our study of infants with transposition of great arteries and intact ventricular septum managed with balloon atrial septostomy, no correlation was noted between the atrial septal defect size and oxygen saturations. Pulmonary vascular resistance and pulmonary blood flow may be important physiological variables determining oxygenation.

Balloon atrial septostomy as initial therapy in pediatric pulmonary hypertension

Balloon atrial septostomy is a palliative procedure currently used to bridge medically refractory pulmonary hypertension patients to lung transplantation. In the current report, we present balloon atrial septostomy as an initial therapy for high-risk pediatric pulmonary hypertension patients at our institution. Nineteen patients with median age of 4.3 years (range 0.1–14.3 years) underwent balloon atrial septostomy during initial admission for pulmonary hypertension. There were no procedural complications or deaths within 24 h of balloon atrial septostomy. Patients were followed for a median of 2.6 years (interquartile range 1.0–4.8 years). Three (16%) patients died, 3 (16%) underwent lung transplantation, and 1 (5%) underwent reverse Potts shunt. Transplant-free survival at 30 days, 1 year, and 3 years was 84%, 76%, and 67% respectively. This single-center experience suggests early-BAS in addition to pharmacotherapy is safe and warrants consideration in high-risk pediatric pulmonary hypertension patients.

Palliative balloon atrial septostomy in two pediatric patients with severe pulmonary arterial hypertension requiring extracorporeal membrane oxygenation support

Pulmonary arterial hypertension is a pernicious disease with a diverse etiology in the pediatric population. Despite the increased availability of drug therapies, pulmonary arterial hypertension continues to cause significant morbidity and mortality. In pediatric patients with severe pulmonary arterial hypertension who have failed medical therapy, a few studies have demonstrated the role of balloon atrial septostomy as a bridge to lung transplantation or a means of improving symptomatology. However, no data exists on the utilization of balloon atrial septostomy as a palliative intervention to wean from extracorporeal membrane oxygenation (ECMO) when all other therapies are exhausted. Here we describe a case series of two pediatric patients with severe pulmonary arterial hypertension, requiring ECMO support, who were successfully weaned from ECMO following balloon atrial septostomy.