Transcatheter Hepatic Conduit-Azygous Vein Connection Reduces Pulmonary Arteriovenous Malformations in a Cyanotic Fontan Patient.

The authors report a closed-chest, transcatheter large-vessel connection (hepatic conduit to azygous vein) to reverse pulmonary arteriovenous malformations in a 10-year-old patient after Fontan for heterotaxy/interrupted inferior vena cava, with an increase in oxygen saturation from 78% to 96%. Computational fluid dynamics estimated a 14-fold increase in hepatic blood flow to the left pulmonary artery (from 1.3% to 14%).

Selective Use of Pulmonary Vasodilators in Patients with Fontan Physiology.

Background: Fontan-associated liver disease is a well-known sequela following the Fontan procedure for patients living with single-ventricle heart disease. Pulmonary vasodilators, such as phosphodiesterase type 5 inhibitors, have emerged as a potential therapeutic option for lowering central venous pressures by reducing pulmonary vascular resistance.

Method: We performed a single-center retrospective review of Fontan patients who were placed on pulmonary vasodilator therapy with prehemodynamic and posthemodynamic, MR elastography, and histologic assessments.

Pulmonary Atresia With Intact Ventricular Septum: From Radiofrequency Perforation to Transcatheter Pulmonary Valve.

Objectives: We intend to describe an entirely transcatheter management pathway for patients with pulmonary atresia with intact ventricular septum (PA/IVS).

Background: PA/IVS is a rare cyanotic congenital heart lesion traditionally palliated with multiple procedures and surgeries. Entirely non-surgical transcatheter management from infancy to adulthood may be possible; however, the pathway from neonatal radiofrequency (RF) pulmonary valve perforation to later transcatheter pulmonary valve replacement (TPV) is not well described.

Institutional Trend in Device Selection for Transcatheter PDA Closure in Premature Infants.

We report our experience with transcatheter patent ductus arteriosus (PDA) closure in premature infants and compare patients grouped by the device used for closure: the Microvascular Plug, "MVP" (Medtronic, Minneapolis, MN); Micro Plug Set, "Micro Plug" (KA Medical, Minneapolis, MN); and Amplatzer Piccolo Occluder, "Piccolo" (Abbot, Santa Clara, CA). We also report trends in device selection over time. Studies examining outcomes according to device selection for PDA closure in premature infants are lacking.

Selective Valve Removal for Melody Valve Endocarditis: Practice Variations in a Multicenter Experience.

Guidelines for management of Melody transcatheter pulmonary valve (TPV) infective endocarditis (IE) are lacking. We aimed to identify factors associated with surgical valve removal versus antimicrobial therapy in Melody TPV IE. Multicenter retrospective analysis of all patients receiving Melody TPV from 10/2010 to 3/2019 was performed to identify cases of IE.

Intravascular Ultrasound (IVUS) Provides the Filling for the Angiogram's Crust: Benefits of IVUS in Pediatric Interventional Cardiology.

Background: Intravascular ultrasound (IVUS) is a catheter-based imaging modality that generates cross-sectional views of vessel walls and lumens. This technique is used in adult interventional and vascular surgeries to guide the management of coronary artery and peripheral arterial disease. IVUS has been described as superior to angiography in providing data about lesions of interest, including degree of vessel stenosis and stent apposition following intervention.

Expanded Use of the One-Step Technique for Simultaneous Landing Zone Stenting and Placement of the Melody Transcatheter Pulmonary Valve.

Objectives: We report a multicenter experience with simultaneous right ventricular outflow tract (RVOT) stenting and transcatheter pulmonary valve implantation using the Melody valve (Medtronic).

Background: Prestenting the RVOT before Melody valve implantation is now the standard of care. Prestenting is usually performed as a separate step.

4D Flow MRI Quantification of Congenital Shunts: Comparison to Invasive Catheterization.

Purpose: To compare invasive right heart catheterization with four-dimensional (4D) flow MRI for estimating shunt fraction in patients with intracardiac and extracardiac shunts.

Materials And Methods: In this retrospective study, patients who underwent 4D flow MRI and invasive right heart catheterization with a shunt run between August 2015 and November 2018 were included. The primary objective was comparison of estimated shunt fraction (ratio of pulmonary-to-systemic flow, Q/Q) at 4D flow and catheterization.

Bronchial stenting in infants with severe bronchomalacia: Technique and outcomes.

Objectives: To report the first use of a balloon expandable bare metal stent for treating infant bronchomalacia.

Background: Infant bronchomalacia often requires prolonged mechanical ventilation and can be life-threatening. Effective treatment for severe infant bronchomalacia continues to be elusive.

Patent Ductus Arteriosus Stenting for All Ductal-Dependent Cyanotic Infants: Waning Use of Blalock-Taussig Shunts.

[Figure: see text].

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.

Purpose-Built Transcatheter Cavopulmonary Anastomosis Device Requirements: Multi-Modality Imaging Evaluation.

Background/purpose: Patients with a functional single ventricle undergo multiple, palliative open-heart surgeries. This includes a superior cavopulmonary anastomosis or bidirectional Glenn shunt. A less-invasive transcatheter approach may reduce morbidity.

An Elusive Prize: Transcutaneous Near InfraRed Spectroscopy (NIRS) Monitoring of the Liver.

We postulate a relationship between a transcutaneous hepatic NIRS measurement and a directly obtained hepatic vein saturation. If true, hepatic NIRS monitoring (in conjunction with the current dual-site cerebral-renal NIRS paradigm) might increase the sensitivity for detecting shock since regional oxygen delivery changes in the splanchnic circulation before the kidney or brain. We explored a reliable technique for hepatic NIRS monitoring as a prelude to rigorously testing this hypothesis.

Transjugular liver biopsy for Fontan associated liver disease surveillance: Technique, outcomes and hemodynamic correlation.

Background: Fontan associated liver disease (FALD) is attributed to the limitations of the Fontan circulation, resulting in congestive hepatopathy. The technique and outcomes of transjugular liver biopsies (TJLB) in Fontan patients warrant definition as part of a rigorous FALD surveillance program.

Method: Four year review of patients with Fontan physiology who underwent a TJLB during surveillance catheterizations.

Serial Dilation of Low-Profile Stents Delivered in the Aorta and Pulmonary Arteries in Pediatric Patients Leads to Spontaneous Fractures but Not to Adverse Events.

Objectives: Low-profile stents placed in pediatric patients with congenital heart disease must be expanded by balloon angioplasty to accommodate patient growth. During the process of serial dilation, some stents may spontaneously fracture. The incidence and safety profile of spontaneous fracture is unclear.

Abnormal pulmonary perfusion heterogeneity in patients with Fontan circulation and pulmonary arterial hypertension.

Key Points: The distribution of pulmonary perfusion is affected by gravity, vascular branching structure and active regulatory mechanisms, which may be disrupted by cardiopulmonary disease, but this is not well studied, particularly in rare conditions. We evaluated pulmonary perfusion in patients who had undergone Fontan procedure, patients with pulmonary arterial hypertension (PAH) and two groups of controls using a proton magnetic resonance imaging technique, arterial spin labelling to measure perfusion. Heterogeneity was assessed by the relative dispersion (SD/mean) and gravitational gradients.

Alternative Access in Congenital Heart Disease.

Limited arterial vascular access precluded necessary transcatheter intervention in a 22-year-old woman with repaired interrupted aortic arch type B. Alternative transcaval vascular access enabled percutaneous therapy. This practice evolution is likely to benefit the growing numbers of adults with congenital heart disease.

Early experience with the Micro Plug Set for preterm patent ductus arteriosus closure.

Objectives: We intend to describe early experience using a new, commercially available Micro Plug Set for preterm neonate and infant transcatheter patent ductus arteriosus (PDA) occlusion.

Background: Transcatheter PDA occlusion in premature neonates and small infants is safe and effective. The procedure is early in its evolution.

Radiation Risk Categories in Cardiac Catheterization for Congenital Heart Disease: A Tool to Aid in the Evaluation of Radiation Outcomes.

To stratify diverse procedure types into categories with similar radiation exposure in cardiac catheterization for congenital heart disease. Radiation exposures for a comprehensive list of specific procedure types and stratification of outcomes based on radiation risk are not currently available. Data between January 2014 and December 2015 were collected on all cases performed at sites participating in C3PO-QI (Congenital Cardiac Catheterization Outcomes Project-Quality Improvement Initiative) and 9 centers were included.

Endograft rescue of compromised interposition aortic graft in an adult patient with congenital heart disease.

In a 19-year-old male with interrupted aortic arch and complex congenital heart disease, we report percutaneous repair of a compromised aortic conduit. The patient had aortic arch repair in childhood utilizing a 12 mm Hemashield Dacron conduit. CT angiography showed multiple segments of this conduit were dilated to 16 mm suggesting conduit degeneration and failure with pseudoaneurysm formation.

Perforation and right ventricular outflow tract stenting: Alternative palliation for infants with pulmonary atresia/ventricular septal defect.

Background: Right ventricular outflow tract (RVOT) stents have been used as palliation in patients with severe tetralogy of Fallot (TOF). Radiofrequency perforation of the RVOT has also been described in patients with pulmonary atresia (PA)/ventricular septal defect (VSD). However, RVOT stenting in conjunction with radiofrequency perforation as a means for establishing reliable pulmonary blood flow in patients with PA/VSD has not previously been reported.

First-in-Human Closed-Chest Transcatheter Superior Cavopulmonary Anastomosis.

Background: In the care of patients with congenital heart disease, percutaneous interventional treatments have supplanted many surgical approaches for simple lesions, such as atrial septal defect. By contrast, complex congenital heart defects continue to require open-heart surgery. In single-ventricle patients, a staged approach is employed, which requires multiple open-heart surgeries and significant attendant morbidity and mortality.

Percutaneous obliteration of left ventricular cavity to eliminate aortic regurgitation and presumed coronary steal in an infant with hypoplastic left heart syndrome.

The phenomenon of coronary steal is well known in the setting of HLHS (Hypoplastic Left Heart Syndrome) early after the Classical Norwood Operation. We report a rare case of an infant with HLHS [Severe Aortic Stenosis (AS), Mitral Stenosis (MS) and small Left Ventricle (LV)], who developed aortic regurgitation and presumed coronary steal late after the Sano Modification of the Norwood Procedure. Coronary steal developed secondary to progressive aortic and mitral regurgitation and resulted in poor right ventricular function and severe tricuspid regurgitation.