In Utero Valvuloplasty for Pulmonary Atresia With Hypoplastic Right Ventricle: Techniques and Outcomes (original) (raw)
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Fetal Diagnosis and Therapy
Introduction: Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (PS) has been performed with small single-institution series reporting technical and physiological success. We present the first multicenter experience. Objectives: Describe fetal and maternal characteristics of those being evaluated for FCI, including pregnancy/neonatal outcome data using the International Fetal Cardiac Intervention Registry (IFCIR). Methods: We queried the IFCIR for PAIVS/PS cases evaluated from January 2001 to April 2018 and reviewed maternal/fetal characteristics, procedural details, pregnancy and neonatal outcomes. Data were analyzed using standard descriptive statistics. Results: Of the 84 maternal/fetal dyads in the registry, 58 underwent pulmonary valvuloplasty at a median gestational age of 26.1 (21.9–31.0) weeks. Characteristics of fetuses undergoing FCI varied in terms of tricuspid valve (TV) size, TV regurgi...
Journal of the American Society of Echocardiography, 2006
Objective: We sought to identify in utero predictors of postnatal outcomes in fetal patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis. Background: Although PAIVS or critical pulmonary stenosis can be diagnosed in utero by echocardiography, our ability to predict outcomes is limited. Methods: Fetal echocardiograms from 28 patients with PAIVS/critical pulmonary stenosis were retrospectively reviewed. Tricuspid valve (TV) annulus, right and left ventricular internal dimensions, and degree of tricuspid regurgitation were recorded. To establish normal fetal values, echocardiograms from healthy patients were analyzed in an identical fashion. Results: Both a fetal TV z score of ؊4 or less beyond 23 weeks of gestation and a fetal TV annulus of 5 mm or less beyond 30 weeks of gestation were predictive of poor postnatal outcomes. In addition, right:left ventricular length or width less than 0.5 and/or the absence of tricuspid regurgitation were predictive of poor outcome. Conclusions: TV annulus size, right:left ventricular ratios, and presence of tricuspid regurgitation on fetal echocardiograms may aid in guiding prenatal counseling regarding postnatal outcome in PAIVS.
Pulmonary stenosis and atresia with intact ventricular septum during prenatal life
Ultrasound in Obstetrics and Gynecology, 2003
K E Y W O R D S: congenital heart disease; fetus; prenatal diagnosis; pulmonary atresia; pulmonary stenosis ABSTRACT Aim To identify fetal echocardiographic characteristics predictive of perinatal outcome in cases with a prenatal diagnosis of pulmonary stenosis or pulmonary atresia.
European Journal of Pediatrics, 1998
In neonates with pulmonary atresia and intact ventricular septum the aims of therapy are maintenance of pulmonary blood¯ow and right ventricular decompression in order to achieve right ventricular support of the pulmonary circulation. Recent developments in interventional heart catheterization with pulmonary radiofrequency-assisted balloon valvuloplasty and ductal stent implantation oer an alternative to the classical surgical approach. We report on a neonate with membranous pulmonary atresia and intact ventricular septum, in whom a large interatrial right-to-left shunt via the foramen ovale persisted after radiofrequency-assisted pulmonary balloon valvuloplasty on the 2nd day of life. The interatrial shunt prevented adequate right ventricular ®lling and antegrade pulmonary perfusion leading to severe cyanosis (transcutaneous oxygen saturation 40%). In order to increase pulmonary blood¯ow and raise left atrial pressure, the arterial duct was stented. After ductal stenting, prostaglandin was discontinued and the transcutaneous oxygen saturation remained stable around 89%. At follow up after 7 weeks the foramen ovale had decreased in size and only a small left-to-right shunt was present, documenting the eectiveness of this approach. Conclusion Based on the present case we propose a stepwise interventional approach for the neonate with pulmonary atresia and intact ventricular septum. If cyanosis persists after isolated pulmonary valvuloplasty despite adequate right ventricular function, ductal stent implantation can reduce interatrial shunting and thus improve oxygen saturation.
Progress in Pediatric Cardiology, 2006
Numerous studies have shown progression of heart disease in utero. Fetal cardiac interventions were first performed in left heart disease, but right heart defects also deserve attention. Pulmonary atresia with intact ventricular septum is a lesion with high mortality and morbidity. It can evolve from critical pulmonary stenosis and result in failure of ventricular growth. Postnatally biventricular repair can be achieved only in 32% to 60%. Therefore, if critical stenosis or membranous atresia of the pulmonary valve can be opened early enough before birth, one could speculate that outcome may improve. Fetal intervention may reverse pulmonary atresia, thus improving growth of the tricuspid valve and the right ventricle. Reduction in right ventricular pressure may prevent continuing damage to the myocardium, resulting in improved mortality and morbidity and a better chance for biventricular repair with less potential for late complications. Successful intracardiac interventions have already been performed in 5 fetuses with pulmonary atresia with intact septum. These cases have demonstrated that interventions in this lesion are feasible, can cause improvements in fetal hemodynamics and may affect outcome. Other right heart lesions that might become targets for future interventional procedures are pulmonary atresia with ventricular septal defect and aorto-pulmonary collateral arteries and Ebstein's anomaly with severe tricuspid regurgitation.
Circulation, 2004
Background-Hypoplastic left heart syndrome (HLHS) with intact or very restrictive atrial septum is a highly lethal combination. We review our 13-year institutional experience treating this high-risk subgroup of patients with emergent catheter therapy. Methods and Results-Infants with HLHS requiring catheter septostomy within the first 2 days of life were compared with a matched control group with adequate interatrial communication. Preoperative, early postoperative, and medium-term survival were evaluated. Earlier experience was compared with recent results to assess the effect of changes in catheterization and surgical and intensive care unit management strategies over the study period. From 1990 to 2002, 33 newborns with HLHS (11% of newborns with HLHS managed during this period) underwent urgent/semiurgent catheterization to create or enlarge an interatrial communication before surgical palliation. Preoperative and early postoperative mortality were high (48%) compared with control HLHS patients, regardless of prenatal diagnosis and despite successful catheter-based atrial septostomy with clinical stabilization. Mortality trended down during the later part of the study period. Those who survived the neonatal period had late survival, pulmonary artery pressure, and resistance similar to those of control subjects. Conclusions-Neonatal mortality in the subgroup of HLHS patients with intact or highly restrictive atrial septum remains high despite successful urgent septostomy. Persistently poor outcomes for these patients have prompted efforts at our center to develop techniques for fetal intervention for this condition, in the hope that prenatal relief of left atrial and pulmonary venous hypertension may promote normal pulmonary vascular and parenchymal development and improve both short-and long-term outcomes.
Ultrasound in Obstetrics and Gynecology, 2008
K E Y W O R D S: congenital heart disease; fetal echocardiography; pulmonary atresia with intact ventricular septum (PAIVS); tricuspid regurgitation; ventriculocoronary connections (VCC) ABSTRACT Objective Pulmonary atresia with intact ventricular septum (PAIVS) is a rare cardiac malformation with a broad spectrum of anatomical manifestations, varying from types suitable for biventricular repair to those with diminutive right ventricle and primitive ventriculocoronary connections (VCC), more suitable for single-ventricle palliation or heart transplantation. We sought to test whether tricuspid regurgitation is an easily detectable prenatal criterion with which to identify PAIVS patients at lower risk of needing postnatal single-ventricle palliation. Method We identified retrospectively patients with both fetal diagnosis and postnatal confirmation of PAIVS who were seen at Bambino Gesù Hospital between January 2000 and December 2006. Tricuspid valve/mitral valve (TV/MV) ratio, presence and severity of tricuspid regurgitation and direct visualization of VCC were evaluated by echocardiography both pre-and postnatally. Results We identified 22 patients with a prenatal diagnosis of PAIVS. Four pregnancies were terminated and one fetus was lost to follow-up, leaving 17 patients for the analysis. Based on postnatal cardiac catheterization and/or echocardiography we divided our population in two groups: Group 1 included 10 patients with VCC; Group 2 included seven patients without VCC. At fetal echocardiography, tricuspid regurgitation was absent in all ten Group 1 patients and present in all seven Group 2 patients. VCC were seen directly in 6/10 Group 1 patients and in no Group 2 patients. A cut-off value of 0.56 for the TV/MV ratio was highly predictive of VCC during fetal life, with a sensitivity of 100% and a specificity of 90%. Conclusions The absence of tricuspid regurgitation in fetuses affected by PAIVS is a strong prenatal echocardiographic predictor of VCC, as is a TV/MV ratio < 0.56. Fetuses presenting with tricuspid regurgitation and relatively large right ventricle are at lower risk of needing single-ventricle palliation postnatally. This information could be helpful for appropriate prenatal counseling and postnatal decision-making.