Controversy About a High-Risk and Innovative Fetal Cardiac Intervention.

2018

2018 Aug 10

1098-4275

<p>A 20-week-old fetus was diagnosed with critical pulmonary valve stenosis. Given the ultrasound findings, the outcome was difficult to predict. The fetal cardiologists discussed the possibility of a pulmonary valvuloplasty (an experimental procedure) with the parents, wherein the fetal right ventricle would be punctured with a long 18G needle, and through it, a wire advanced across the pulmonary valve, allowing for balloon dilation of the valve. The experimental procedure had been performed at a handful of centers. There were some reports of success. The parents sought an opinion at one of the referral centers that had tried the procedure. The doctors there recommended against it. The doctors at the original center were unsure whether they should try the procedure. The parents wanted it. In this ethics rounds, doctors and the parents discuss the arguments for and against a high-risk, innovative in utero procedure.</p>

10.1542/peds.2017-3595

Pediatrics

30097527

Outcomes After Decompression of the Right Ventricle in Infants With Pulmonary Atresia With Intact Ventricular Septum Are Associated With Degree of Tricuspid Regurgitation: Results From the Congenital Catheterization Research Collaborative.

2017

2017 May

1941-7632

<p><strong>BACKGROUND: </strong>Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies.</p>

<p><strong>METHODS AND RESULTS: </strong>Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers. Primary end point was reintervention post-RV decompression; secondary end points included circulation type at latest follow-up. Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th-75th, 2-5) days of age. Seventy-one patients (72%) underwent at least 1 reintervention after decompression. Median duration of follow-up was 3 years (range, 1-10). Freedom from reintervention was 51% at 1 month and 23% at 3 years. In multivariable analysis, reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interval [CI], 0.28-091; P=0.027), smaller RV length (HR, 0.94; 95% CI, 0.89-0.99; P=0.027), and ≤mild tricuspid regurgitation (TR; HR, 3.58; 95% CI, 2.04-6.30; P&lt;0.001). Patients undergoing surgical shunt or ductal stent were less likely to have virtual atresia (HR, 0.36; 95% CI, 0.15-0.85; P=0.02) and more likely to have higher RV end-diastolic pressure (HR, 1.07; 95% CI, 1.00-1.15; P=0.057) and ≤mild TR (HR, 3.50; 95% CI, 1.75-7.0; P&lt;0.001). Number of reinterventions was associated with ≤mild TR (rate ratio, 1.87; 95% CI, 1.23-2.87; P=0.0037). Multivariable analysis indicated that &lt;2-ventricle circulation status was associated with ≤mild TR (odds ratio, 18.6; 95% CI, 5.3-65.2; P&lt;0.001) and lower RV area (odds ratio, 0.81; 95% CI, 0.72-0.91; P&lt;0.001).</p>

<p><strong>CONCLUSIONS: </strong>Patients with pulmonary atresia with intact ventricular septum deemed suitable for RV decompression have a high reintervention burden although most achieve 2-ventricle circulation. TR ≤mild at baseline is strongly associated with reintervention and &lt;2-ventricle circulation at medium-term follow-up. Degree of baseline TR may be an important marker of long-term outcomes in this population.</p>

10.1161/CIRCINTERVENTIONS.116.004428

Circ Cardiovasc Interv

28500137

Radiation Safety in Children with Congenital and Acquired Heart Disease: A Scientific Position Statement on Multimodality Dose Optimization from the Image Gently Alliance.

2017

2017 May 17

1876-7591

<p>There is a need for consensus recommendations for ionizing radiation dose optimization during multi-modality medical imaging in children with congenital and acquired heart disease (CAHD). These children often have complex diseases and may be exposed to a relatively high cumulative burden of ionizing radiation from medical imaging procedures including cardiac computed tomography, nuclear cardiology studies and fluoroscopically guided diagnostic and interventional catheterization and electrophysiology procedures. Although these imaging procedures are all essential to the care of children with CAHD and have contributed to meaningfully improved outcomes in these patients, exposure to ionizing radiation is associated with potential risks, including an increased lifetime attributable risk of cancer. The goal of these recommendations is to encourage informed imaging to achieve appropriate study quality at the lowest achievable dose. Other strategies to improve care include a patient-centered approach to imaging, emphasizing education and informed decision making and programmatic approaches to ensure appropriate dose monitoring. Looking ahead, there is a need for standardization of dose metrics across imaging modalities, so as to encourage comparative effectiveness studies across the spectrum of CAHD in children.</p>

10.1016/j.jcmg.2017.04.003

JACC Cardiovasc Imaging

28514670