Computed Tomography-Derived Normative Values of Right Ventricular Outflow Tract Structures in the Pediatric Population.

Recent advances in available percutaneous device technology require accurate measurements and quantification of relationships between right ventricular outflow tract (RVOT) structures in children with and without congenital heart disease to determine device suitability. To date, no population study has described normal reference ranges of these measurements by computed tomography (CT). We aimed to establish normative values for four CT-derived measurements between RVOT structures from a heterogeneous population without heart disease and develop z scores useful for clinical practice. Patients without heart disease who underwent cardiac CT between April 2014 and February 2021 at Children's Hospital Colorado were included. Distance between the right ventricular (RV) apex to pulmonary valve (PV), PV to pulmonary trunk bifurcation, and bifurcation to the right and left pulmonary artery was measured. Previously validated models were used to normalize the measurements and calculate Z scores. Each measurement was plotted against BSA and Z scores distributions were used as reference lines. Three-hundred and sixty-four healthy patients with a mean age of 8.8 years (range 1-21), 58% male, and BSA of 1 m2 (range 0.4-2.1) were analyzed. The Haycock formula was used to present data as predicted values for a given BSA and within equations relating each measurement to BSA. Predicted values and Z-score boundaries for all measurements are presented.We report CT-derived normative data for four measurements between RVOT structures from a heterogeneous cohort of healthy children. Knowledge of this normative data will be useful in both determining device fit and customizing future devices to accommodate the diverse pediatric size range.

Transcatheter closure of a large coronary cameral fistula presenting as a calcified cystic right atrial mass.

A 51-year-old patient with progressive right heart dysfunction was found to have a large calcified right atrial mass on echocardiography. As part of the work up for an intracardiac mass he had a cardiac computed tomogram which detailed a large coronary cameral fistula from the circumflex coronary artery to the right atrium associated with a spherical calcific pseudo-aneurysmal sac. Transcatheter occlusion of the exit point into the atrium with a vascular plug was performed directly from a right atrial approach without the need for an arteriovenous wire loop. This case details a unique presentation of a coronary cameral fistula to an unusual position within the right atrium which facilitated the rare ability to occlude the fistula from a venous approach without creating an arteriovenous wire rail.

Percutaneous Stenting of a Stenotic Berlin Heart Outflow Cannula Graft in a 2 Year Old Child.

Left ventricular assist device (LVAD) outflow obstruction is a rare complication of long-term LVAD support. We present the first case of successful percutaneous stent implantation in a pediatric patient with LVAD outflow obstruction.

Radiofrequency Perforation of an Atretic Pulmonary Valve with a Modified Coronary Wire and Electrocautery Pencil.

Radiofrequency (RF) perforation of an atretic pulmonary valve is commonly performed in patients with pulmonary atresia with intact ventricular septum with specifically designed RF wires. In difficult anatomy or low-resource centers, this may instead be successfully performed with a modified coronary guide wire and an electrocautery surgical pencil.

Establishing Carotid Seldinger as Routine Access in Infants; Planning, Performance, and Follow-Up Protocols.

Percutaneous carotid access (PCA) in infants has been reported in small multicenter cohorts, case reports and wider studies over the last 20 years. Compare outcomes after implementation of a systematic approach to PCA in a single center including an imaging follow-up protocol. Retrospective case-control study of PCA at Children's Hospital Colorado was performed from January 2013 to December 2022. Seventy-four patients underwent 82 PCAs for cardiac catheterization. The median age (range) was 14 days (1-359), and weight was 3.25-kg (1.9-7.9). Median sheath size was 4-Fr (3.3-6). Seventy-seven interventions performed included PDA stenting, aortic valvoplasty, BTT shunt stenting, and coarctation stenting. Vascular access was performed using a modified 21 g butterfly needle. A protocolized approach was implemented in 2020 reversing the patient head-to-toe orientation on the catheterization table, maintaining intubation and sedation for 4-h during recovery and routine use of a specific vascular ultrasound protocol. Following these changes, time to access significantly improved with no major complications. Before 2020, two access related complications occurred. One requiring surgical vascular repair and one occlusive thrombus. A significant increase in sheath time in post-era was associated with increased case complexity. Longer sheath times were not associated with increased risk of vessel injury or thrombus. No neurological insults were reported. Our experience confirms that PCA is safe and achievable with preserved vessel patency regardless of patient weight or sheath size. A protocolized planning, recovery, and follow-up regimen is recommended to establish safe practice and identify and treat complications as necessary.

Staged Acclimatization in a Failing Fontan by AFR in AFR: Ever-Decreasing Circles.

We describe serial implantation of atrial flow regulator (AFR) devices in the Fontan fenestration of a 4-year-old patient. Initially, the fenestration size was decreased using a 6/5 AFR, resulting in improved saturations and hemodynamics. One year later, further improvement was achieved by placing a 4/10 AFR inside the original device. (Level of Difficulty: Advanced.).

Factors influencing the choice-of-care pathway and survival in the fetus with hypoplastic left heart syndrome in New Zealand: a population-based cohort study.

OBJECTIVES: To better understand the relative influence of fetal and maternal factors in determining the choice-of-care pathway (CCP) and outcome in the fetus with hypoplastic left heart syndrome (HLHS). DESIGN: A retrospective, population-based study of fetuses with HLHS from a national dataset with near-complete case ascertainment from 20 weeks' gestation. Fetal cardiac and non-cardiac factors were recorded from the patient record and maternal factors from the national maternity dataset. The primary endpoint was a prenatal decision for active treatment after birth (intention-to-treat). Factors associated with a delayed diagnosis (≥24 weeks' gestation) were also reviewed. Secondary endpoints included proceeding to surgical treatment, and 30-day postoperative mortality in liveborns with an intention-to-treat. SETTING: New Zealand population-wide. PARTICIPANTS: Fetuses with a prenatal diagnosis of HLHS between 2006 and 2015. RESULTS: Of 105 fetuses, the CCP was intention-to-treat in 43 (41%), and pregnancy termination or comfort care in 62 (59%). Factors associated with intention-to-treat by multivariable analysis included a delay in diagnosis (OR: 7.8, 95% CI: 3.0 to 20.6, p<0.001) and domicile in the maternal fetal medicine (MFM) region with the most widely dispersed population (OR: 5.3, 95% CI: 1.4 to 20.3, p=0.02). Delay in diagnosis was associated with Maori maternal ethnicity compared with European (OR: 12.9, 95% CI: 3.1 to 54, p<0.001) and greater distance from the MFM centre (OR: 3.1, 95% CI: 1.2 to 8.2, p=0.02). In those with a prenatal intention-to-treat, a decision not to proceed to surgery was associated with maternal ethnicity other than European (p=0.005) and the presence of major non-cardiac anomalies (p=0.01). Thirty-day postoperative mortality occurred in 5/32 (16%) and was more frequent when there were major non-cardiac anomalies (p=0.02). CONCLUSIONS: Factors associated with the prenatal CCP relate to healthcare access. Anatomic characteristics impact treatment decisions after birth and early postoperative mortality. The association of ethnicity with delayed prenatal diagnosis and postnatal decision-making suggests systemic inequity and requires further investigation.

The novel use of an advanced thrombectomy system to manage a complex pericardial effusion associated with kaposiform lymphangiomatosis.

A 4-year-old boy presented to our institution with pancytopenia, consumptive coagulopathy, hepatosplenomegaly and recurrent complex pericardial effusion secondary to kaposiform lymphagiomatosis. Due to extensive loculation, conventional drainage was minimally effective. As an adjunct to medical therapy, the Indigo™ aspiration system was used to remove thrombus within the pericardial space. Our patient had good medium-term results with complete resolution of his pericardial effusion at 4 months.

Case Report: Catheter-based mechanical thrombectomy using the Indigo aspiration system in a case of systemic-to-pulmonary shunt thrombosis.

A 53-day-old girl with absent right atrioventricular (AV) connection, malposed great vessels, and pulmonary atresia underwent placement of a central shunt on the sixth day of her life. Her postoperative course was complicated by progressive desaturation, and computed tomographic angiography (CTA) demonstrated near-complete occlusion of her left pulmonary artery (LPA). Angiography demonstrated a nonocclusive thrombus in the distal central shunt and a thrombus with complete occlusion of the LPA. The Indigo aspiration system (Penumbra) was used to remove the thrombus from the central shunt and LPA, allowing placement of a stent in the narrowed LPA. Subsequent angiography showed a wide patient central shunt and LPA. The Indigo aspiration system (Penumbra) provides a viable option for removing thrombus in a patients refractory to other methods.

Outcomes of the arterial switch operation in patients with Taussig-Bing anomaly.

BACKGROUND: The arterial switch operation (ASO) is associated with poorer outcomes in patients with Taussig-Bing anomaly (TBA) compared with transposition of the great arteries (TGA). We describe the outcomes after ASO in patients with TBA at a single institution. METHODS: Between 1983 and 2009, 57 patients with TBA underwent the ASO at the Royal Children's Hospital in Melbourne. RESULTS: Hospital mortality was 5.3% (3 of 57). Larger weight at operation (p=0.015), pulmonary artery banding prior to ASO (p=0.049) and concurrent pulmonary artery banding (p=0.049) were risk factors of early death. Actuarial survival was 94% at 15 years. Follow-up was 84% complete with a mean follow-up of 9.8±6.7 years (range, 6 days to 19.1 years). There was no late mortality. Reintervention was required in 24.4% (11 of 45). Longer cross-clamp time (p=0.027) was a risk factor for reintervention. Freedom from reintervention was 75.3% at 15 years. After ASO, 2.2% (1 of 45) presented with sub-neopulmonary obstruction and 13.3% (6 of 45) had moderate or more neoaortic insufficiency (neo-AI). Surgery prior to ASO was a risk factor for sub-neopulmonary obstruction (p=0.049) and moderate or more neo-AI (p=0.016). Freedom from moderate or more neo-AI was 91.1% at 10 years. CONCLUSIONS: Early mortality has improved over time with no mortality occurring in the last decade. Although patients are doing well on late follow-up, many patients require reintervention and show progression of neo-AI. Close long-term follow-up is warranted as patients are likely to require further reintervention in the second decade after TBA repair.