Surgical versus transcatheter palliation for insufficient pulmonary blood supply in infants with cyanotic CHD.

Infants with complex cyanotic CHD can become symptomatic from insufficient pulmonary blood supply following either ductal closure or due to outflow tract obstruction. Blalock-Taussig shunt mortality remains significant and recent studies have highlighted the advantages of using transcatheter alternatives. We present here our experience in changing our primary choice of palliation from the Blalock-Taussig shunt to transcatheter palliation with either a ductal stent or, if antegrade flow is present, a right ventricular outflow tract stent.This is a retrospective, single-unit cohort study. Eighty-seven infants underwent palliation for insufficient pulmonary blood flow at under 3 months of age between 2012 and 2019. On an intention-to-treat basis, 29 underwent insertion of a Blalock-Taussig shunt, 36 duct stents, and 22 right ventricular outflow tract stents at median ages of 15, 9, and 32 days, respectively, and median weights of 3.3, 3.1, and 3.1 kg, respectively. No primary Blalock-Taussig shunts have been performed in our institution since 2017.At 30-days there had been one death in each group (univariable p = 0.93) and deaths prior to repair totalled three in the shunt group, four in the ductal stent group, and two in the right ventricular outflow tract stent group (univariable p = 0.93). Reintervention on the pulmonary circuit prior to next stage of surgery was more frequent in those undergoing transcatheter intervention, reaching statistical significance by logrank (p = 0.012).In conclusion, within this work we provide further evidence of the safety and efficacy of transition from a primary surgical to primary transcatheter palliation pathway in infants with insufficient pulmonary blood supply.

Deformation and stenosis of the sinus-SuperFlex-DS stent after ductal stenting for the hybrid stage 1 procedure.

We present five cases of sinus-SuperFlex-DS stent stenosis during early follow up that resulted in inadequate ductal patency and required urgent re-stenting with a balloon-expandable stent. This causes concern that these stents lack sufficient radial force against ductal constriction and if used need to be kept under close scrutiny.

Transcatheter recanalization of chronic total occlusion in congenital heart disease: case reports and lessons from percutaneous coronary interventions.

Background: Chronic complete vascular occlusion causes significant morbidity and mortality in patients with congenital heart disease (CHD). The diversity of lesions, lack of dedicated equipment, and small number of procedures performed by individual operator continue to pose a challenge to congenital interventional cardiologists. Case summary: We report two cases of percutaneous recanalization in CHD using percutaneous coronary intervention (PCI) equipment for chronic total occlusion (CTO). Discussion: Careful pre-procedural planning, access site choice, use of catheter guiding systems for support, and selection of penetrating guidewires and techniques used in CTO-PCIs are key steps to a successful outcome.

Post-myocardial infarction ventricular septal defect closure with Gore Cardioform atrial septal defect occluder to improve tissue interactions: case report.

Background: Gore Cardioform Atrial Septal Defect Occluder (GCA) is composed of a platinum-filled nitinol wire frame covered with expanded polytetrafluoroethylene (ePTFE). This makes the device highly occlusive and resistant to residual shunts through the device, as well as conforming well to the surrounding anatomy. In defects with poor rims to hold a device for closure or where one of the 'rims' is the free wall of the left ventricle, such as in a post-infarct apical ventricular septal defect (VSD), successful closure with standard nitinol mesh devices can be unachievable. Case summary: We present the occlusion of a post-myocardial infarction VSD with a GCA device in a critically ill patient at risk for closure failure and intravascular haemolysis with conventional nitinol mesh devices. The device conformed well to the anatomy even in the absence of an apical interventricular septum. Discussion: GCA can be used for intracardiac high-velocity shunts in selected cases where conventional devices are unsuitable due to haemolysis or poor tissue and poor rims. With growing experience using GCA for the closure of atrial septal defects, interventionists should consider the potential advantages of ePTFE material and apply them to other lesions where these could be beneficial for patients.

Successful treatment of refractory chylothorax with MEK inhibitor trametinib in a child with Noonan syndrome: case report.

Background: Refractory chylous effusions due to lymphatic dysplasia related to Noonan syndrome cause significant morbidity and mortality due to protein and immunoglobulin losses. Very few cases have been published reporting successful treatment of patients with trametinib where all conventional treatments had failed. Case summary: We present a girl with Noonan syndrome and hypertrophic cardiomyopathy who presented with life-threatening refractory chylothorax where all conventional treatment options failed. She was successfully treated with mitogen-activated extracellular signal-regulated kinase inhibitor trametinib. Discussion: MEK inhibition with trametinib is emerging as a possible salvage treatment option for a subset of patients with Noonan syndrome and severe pulmonary lymphangiectasia. More experience is required to establish optimal treatment regimen and long-term outcomes.

A novel device for atrial septal defect occlusion (GORE CARDIOFORM).

The GORE CARDIOFORM atrial septal defect (ASD) Occluder (GCA) is composed of a platinum-filled nitinol wire frame covered with expanded polytetrafluoroethylene, making it softer and more conformable compared with nitinol mesh devices. After the ASSURED clinical study confirmed the efficacy and safety of the device, it received U.S. Food and Drug Administration approval and a European conformity mark. Our aim was to understand the learning curve implicated in using the GCA for ASD closure in paediatric and adult patients as well as to study the early outcomes. To this end, a review of ASD device closures with GCA in 4 UK centres was conducted between January 2020 and January 2023. Implantation success was the primary outcome; the secondary outcomes were serious adverse events, including new onset arrhythmia. In all, 135 patients were included, and 128 (95%) had successful ASD device closure with GCA. The median patient age was 49 years, the median defect size was 18 mm, and the median device size was 37 mm. The median follow-up time was 6 months (interquartile range 1-14). One device embolisation occurred, and 15 patients (12% of GCA implantations) developed new onset arrhythmia - this was not related to patient age, defect diameter or device oversizing but was positively associated with device size. With growing experience using GCA, the device can be applied to a wide variety of ASD sizes and morphologies. Given the number of successful implantations with an absence of aortic erosion, as well as the ability to perforate through the device should procedures be required in the left atrium, the GCA device is an important addition for interventionists who close atrial septal defects.

Alternative Hybrid Approach to Promote Native Pulmonary Artery Growth in Pulmonary Atresia VSD MAPCAs.

We present a case of right ventricle to pulmonary artery hybrid perforation and stenting in a patient with pulmonary atresia with ventricular septal defect major aortopulmonary collaterals and diminutive native pulmonary arteries, then discuss how it compares with established approaches. (Level of Difficulty: Advanced.).

Comparative analysis of surgical and percutaneous pulmonary valve implants over a 20-year period.

OBJECTIVES: Since percutaneous pulmonary valve implantation (PPVI) was introduced to prolong the lifetime of surgically placed right ventricular to pulmonary artery conduits, valve technology has evolved and the indications for PPVI expanded to native and larger right ventricular outflow tracts. We explore how indications, patient populations and outcomes compare to surgical pulmonary valve replacement (PVR). METHODS: This is a retrospective cohort study of PPVI and PVR procedures between 1998 and 2020 at a single UK centre. One hundred and twenty-eight patients underwent PPVI and 365 patients PVR. Primary outcome measures were survival, infective endocarditis and reintervention. RESULTS: The most common indication for PVR was replacement of the native pulmonary valve for pulmonary regurgitation whereas PPVI was more commonly used to treat pulmonary stenosis in a previously placed bioprosthetic conduit or valve. Treatment indications for PPVI expanded over the study to include the native right ventricular outflow tract. Survival was similar for PPVI and PVR (92% PPVI and 96.8% PVR at 5 years; 85.8% PPVI and 95.1% PVR at 10 years). Preprocedural New York Heart Association class 3 and 4 was the most important predictor of poor outcome. Annualized infective endocarditis rate was significantly higher for the Melody PPVI (0.024 vs 0.0024/person/year for PVR, P < 0.05). Both groups showed significant symptomatic improvement postprocedure with remodelling of ventricular volumes and improvement in cardiac output. Long-term follow-up for PVR showed half of patients will need replacement at 10-15 years post-index procedure. CONCLUSIONS: An increasing number of patients requiring PVR can now be treated percutaneously. A lifetime strategy for re-valving should be considered at the first valve implant.

A case series of three patients with unilateral disconnected pulmonary artery supplied by an ipsilateral patent ductus arteriosus: neonatal ductal stenting as palliation to preserve pulmonary arterial patency.

BACKGROUND: Disconnected branch pulmonary arteries with a systemic arterial origin of the disconnected vessel is a rare, but well-described entity. Most will have ductal tissue connecting the pulmonary artery to the aorta. CASE SUMMARY: We describe in this paper the haemodynamic result in three neonates presenting with ductal origin of a single branch pulmonary artery in the context of trans-catheter stenting procedures to maintain or re-recruit vessel patency. All were faced with potential or actual ductal closure and proceeded to trans-catheter stenting to re-cannalate the duct-dependent pulmonary artery. Two patients with otherwise normal anatomy struggled post-procedure with pulmonary hypertension and right ventricular dilatation. Both required surgical re-anastomosis of the disconnected pulmonary artery during the same admission-one 26 days post-stenting following failure to wean from high-flow respiratory support and the second 8 days post-stenting following failed extubation. In contrast, a patient with tetralogy of Fallot born at 2.5 kg underwent sequential stenting of the right ventricular outflow tract and then the left-sided ductus. He had a good post-procedural course and thrived for several months before complete repair. DISCUSSION: We describe the clinical courses and discuss the resultant haemodynamics, highlighting the importance of flow to each lung, the resulting haemodynamic implications and the compounding effects of additional lesions.