Diagnosis and Management of Congenital Coronary Artery Fistulas in Infants and Children.

PURPOSE OF REVIEW: Coronary artery fistulas (CAFs) are rare coronary anomalies that most often occur as congenital malformations in children. Although most children with CAFs are asymptomatic at the time of diagnosis, some present with symptoms of congestive heart failure in the setting of large left-to-right shunts. Others may develop additional complications including coronary artery ectasia and coronary thrombosis. Surgical and transcatheter closure techniques have been previously described. This review presents the classifications of CAFs in children and the short and long-term outcomes of CAF closure in children in the reported literature. We also summarize previously-reported angiographic findings and post-treatment remodeling characteristics in pediatric patients. RECENT FINDINGS: With advancements in cross-sectional imaging technologies, anatomic delineation of CAFs via these modalities has become crucial in procedural planning. Recent reports of surgical and transcatheter closure of CAFs in children have reported good procedural success and low rates of short-term morbidity and mortality. Distal-type CAFs have elevated risk for long-term sequelae post-closure compared to proximal-type CAFs. A recent report of a multi-institutional cohort also describes post-closure remodeling classifications which may predict long-term outcomes in these patients as well as guide individualized anticoagulation management. Invasive closure of significant CAFs via surgical or transcatheter techniques is feasible and safe in most children with good short and intermediate-term outcomes. However, close clinical and imaging follow-up is required to monitor for late complications even after successful closure. Antiplatelet and anticoagulation regimens remain important aspects of post-closure management, but the necessary intensity and duration of such therapy remains unknown.

MRI for Guided Right and Left Heart Cardiac Catheterization: A Prospective Study in Congenital Heart Disease.

BACKGROUND: Improvements in outcomes for patients with congenital heart disease (CHD) have increased the need for diagnostic and interventional procedures. Cumulative radiation risk is a growing concern. MRI-guided interventions are a promising ionizing radiation-free, alternative approach. PURPOSE: To assess the feasibility of MRI-guided catheterization in young patients with CHD using advanced visualization passive tracking techniques. STUDY TYPE: Prospective. POPULATION: A total of 30 patients with CHD referred for MRI-guided catheterization and pulmonary vascular resistance analysis (median age/weight: 4 years / 15 kg). FIELD STRENGTH/SEQUENCE: 1.5T; partially saturated (pSAT) real-time single-shot balanced steady-state free-precession (bSSFP) sequence. ASSESSMENT: Images were visualized by a single viewer on the scanner console (interactive mode) or using a commercially available advanced visualization platform (iSuite, Philips). Image quality for anatomy and catheter visualization was evaluated by three cardiologists with >5 years' experience in MRI-catheterization using a 1-5 scale (1, poor, 5, excellent). Catheter balloon signal-to-noise ratio (SNR), blood and myocardium SNR, catheter balloon/blood contrast-to-noise ratio (CNR), balloon/myocardium CNR, and blood/myocardium CNR were measured. Procedure findings, feasibility, and adverse events were recorded. A fraction of time in which the catheter was visible was compared between iSuite and the interactive mode. STATISTICAL TESTS: T-test for numerical variables. Wilcoxon signed rank test for categorical variables. RESULTS: Nine patients had right heart catheterization, 11 had both left and right heart catheterization, and 10 had single ventricle circulation. Nine patients underwent solely MRI-guided catheterization. The mean score for anatomical visualization and contrast between balloon tip and soft tissue was 3.9 ± 0.9 and 4.5 ± 0.7, respectively. iSuite provided a significant improvement in the time during which the balloon was visible in relation to interactive imaging mode (66 ± 17% vs. 46 ± 14%, P < 0.05). DATA CONCLUSION: MRI-guided catheterizations were carried out safely and is feasible in children and adults with CHD. The pSAT sequence offered robust and simultaneous high contrast visualization of the catheter and cardiac anatomy. LEVEL OF EVIDENCE: 2 TECHNICAL EFFICACY STAGE: 1.

Pre-procedural CT imaging aids neonatal PDA stenting for ductal-dependent pulmonary blood flow with reduction in overall procedural morbidity.

Patent ductus arteriosus stenting for ductal-dependent pulmonary blood flow is a technically challenging neonatal procedure to maintain a stable pulmonary circulation. Pre-procedural computed tomography imaging aids in outlining ductal origin, insertion, size, course and curvature. Computed tomography imaging may add value to procedural outcomes and reduce overall procedural morbidity in neonatal patent ductus arteriosus stenting. We conducted a single centre retrospective chart review of neonates with ductal-dependent pulmonary blood flow who underwent patent ductus arteriosus stenting between January 1, 2014 and June 31, 2020. We compared patients variables between patients who underwent pre-procedural computed tomography imaging to those who did not. A total of 64 patients were referred for patent ductus arteriosus stenting with 33 (52%) obtaining pre-procedural computed tomography imaging. Average age [19 days; range 1-242 days (p = 0.85)] and weight [3.3 kg (range 2.2-6.0 kg; p = 0.19)] was not significantly different between the groups. A diagnosis of pulmonary atresia was made in 42 out of 64 (66%) patients prior to patent ductus arteriosus stenting. The cohort with pre-intervention computed tomography imaging had a significant reduction in the total number of access sites (1.2 versus 1.5; p = 0.03), contrast needed (5.9 versus 8.2 ml/kg; p = 0.008), fluoroscopy (20.7 versus 38.8 minutes; p = 0.02) and procedural time (83.4-128.4 minutes; p = 0.002) for the intervention. There was no significant difference in radiation burden between the groups (p = 0.35). Pre-procedural computed tomography imaging adds value by aiding interventional planning for neonatal patent ductus arteriosus stenting. A statistically significant reduction in the number of access sites, contrast exposure, as well as fluoroscopic and procedural time was noted without significantly increasing the cumulative radiation burden.

3D advanced imaging overlay with rapid registration in CHD to reduce radiation and assist cardiac catheterisation interventions.

Novel commercially available software has enabled registration of both CT and MRI images to rapidly fuse with X-ray fluoroscopic imaging. We describe our initial experience performing cardiac catheterisations with the guidance of 3D imaging overlay using the VesselNavigator system (Philips Healthcare, Best, NL). A total of 33 patients with CHD were included in our study. Demographic, advanced imaging, and catheterisation data were collected between 1 December, 2016 and 31 January, 2019. We report successful use of this technology in both diagnostic and interventional cases such as placing stents and percutaneous valves, performing angioplasties, occlusion of collaterals, and guidance for lymphatic interventions. In addition, radiation exposure was markedly decreased when comparing our 10-15-year-old coarctation of the aorta stent angioplasty cohort to cases without the use of overlay technology and the most recently published national radiation dose benchmarks. No complications were encountered due to the application of overlay technology. 3D CT or MRI overlay for CHD intervention with rapid registration is feasible and aids decisions regarding access and planned angiographic angles. Operators found intraprocedural overlay fusion registration using placed vessel guidewires to be more accurate than attempts using bony structures.

Interventional Cardiovascular Magnetic Resonance Imaging (iCMR) in an Adolescent with Pulmonary Hypertension.

The interventional cardiac magnetic resonance imaging (iCMR) catheterization procedure is feasible and safe for children and adults with pulmonary hypertension and congenital heart defects (CHD). With iCMR, the calculation of pulmonary vascular resistance (PVR) in children with complex CHD with multilevel shunt lesions is accurate. In this paper, we describe the role of the MRI-guided right-sided cardiac catheterization procedure to accurately estimate PVR in the setting of multiple shunt lesions (ventricular septal defect and patent ductus arteriosus) and to address the clinical question of operability in an adolescent with trisomy 21 and severe pulmonary hypertension.

Use of Amplatzer Vascular Plugs for the treatment of combined extralobar and intralobar pulmonary sequestration in a 5-year-old child.

Pulmonary sequestration is a rare congenital anomaly that can be asymptomatic or present with recurrent infections, respiratory symptoms, or rarely heart failure. Sequestration is classified as intralobar or extralobar on the basis of whether there is separation from normal lung tissue by its own visceral pleura. Classically, patients are treated with surgical resection. We present a case of multivessel, combined intralobar and extralobar pulmonary sequestration treated with transcatheter embolisation.

Usefulness of PREDIC3T Case Type Risk Category in the CRISP Registry.

Procedural risk in Congenital Cardiac Catheterization (PREDIC3T) was recently reported as the contemporary procedure-type risk metric by the Congenital Cardiac Catheterization Project on Outcomes (C3PO) registry. The usefulness of this metric has not been evaluated elsewhere. The CRISP registry of Congenital Cardiovascular Interventional Study Consortium (CCISC) data set was analyzed. The study period was 14 years (2009 to 2022). The primary outcome was significant adverse event (SAE). Cases were assigned to the 6 PREDIC3T risk categories. Univariate and multivariable logistic regression models were used to evaluate the association between PREDIC3T and the primary outcome. The model discriminative performance was evaluated by the c-statistic. In a total of 64,419 enrolled cases, PREDIC3T case types were assigned in 59,822 cases (93%). The frequency for PREDIC3T category was 0 = 7,494 (12.5%), 1 = 16,932 (28.3%), 2 = 17,023 (28.5%), 3 = 9,885 (16.5%), 4 = 4,403 (7.4%), and 5 = 4,085 (6.8%). SAE was observed in 2,474 cases (4.1%). The SAE rates for category were 0 = 1.0%, 1 = 2.3%, 2 = 4.0%, 3 = 6.2%, 4 = 8.2%, and 5 = 9.0%. In a multivariable model, PREDIC3T case type risk category (odds ratios for category: 0 = 0.49, 1 = 1.00, 2 = 1.40, 3 = 2.06, 4 = 2.79, and 5 = 3.15; p <0.001) were significantly associated with SAE (c-statistic of 0.707) after adjusting for age, preprocedural inotropic support and systemic illness, low systemic saturation, high pulmonary vascular resistance, and the use of general anesthesia. The PREDIC3T case type risk category was associated with the risk of SAE in the CRISP registry data set and appeared to be a useful procedural risk classification tool.

Role of Cross-Sectional Imaging in Pediatric Interventional Cardiac Catheterization.

Management of congenital heart disease (CHD) has recently increased utilization of cross-sectional imaging to plan percutaneous interventions. Cardiac computed tomography (CT) and cardiac magnetic resonance (CMR) imaging have become indispensable tools for pre-procedural planning prior to intervention in the pediatric cardiac catheterization lab. In this article, we review several common indications for referral and the impact of cross-sectional imaging on procedural planning, success, and patient surveillance.

Bioresorbable Stent to Manage Congenital Heart Defects in Children.

Intravascular stents for pediatric patients that degrade without inhibiting vessel growth remain a clinical challenge. Here, poly(L-lactide) fibers (DH-BDS) at two thicknesses, 250 µm and 300 µm, were assembled into large, pediatric-sized stents (Ø10 - Ø20 mm). Fibers were characterized mechanically and thermally, then stent mechanical properties were compared to metal controls, while mass loss and degradation kinetics modeling estimated total stent degradation time. Thicker fibers displayed lower stiffness (1969 ± 44 vs 2126 ± 37 MPa) and yield stress (117 ± 12 vs 137 ± 5 MPa) than thinner counterparts, but exhibited similar fail strength (478 ± 28 vs 476 ± 16 MPa) at higher strains (47 ± 2 vs 44 ± 2%). Stents all exhibited crystallinity between 51.3 - 54.4% and fiber glass transition temperatures of 88.6 ± 0.5 °C and 84.6 ± 0.5 °C were well above physiological ranges. Radial strength (0.31 ± 0.01 - 0.34 ± 0.02 N/mm) in thinner stents was similar to metal stents (0.24 - 0.41 N/mm) up to Ø14 mm with no foreshortening and thicker coils granted comparable radial strength (0.32 ± 0.02 - 0.34 ± 0.02 N/mm) in stents larger than Ø14 mm. Both 10 mm (1.17 ± 0.02 % and 0.86 ± 0.1 %) and 12 mm (1.1 ± 0.03% and 0.89 ± 0.1%) stents exhibited minimal weight loss over one year. Degradation kinetics models predicted full stent degradation within 2.8 - 4.5 years depending on thickness. DH-BDS exhibiting hoop strength similar to metal stents and demonstrating minimal degradation and strength loss over the first year before completely disappearing within 3 to 4.5 years show promise as a pediatric interventional alternative to current strategies.