Ventricular Outflow Tract Anomalies Research Articles

Long-Term Postnatal Follow-Up in Monochorionic TTTS Twin Pregnancies Treated with Fetoscopic Laser Surgery and Complicated by Right Ventricular Outflow Tract Anomalies.

Right ventricular outflow tract anomalies (RVOTAs), such as pulmonary stenosis (PS), pulmonary atresia (PA), and pulmonary insufficiency (PI), are typical cardiac anomalies in monochorionic twins, and they are complicated by twin-to-twin transfusion syndrome (TTTS). The aim of this study was to conduct a long-term postnatal cardiological evaluation of prenatal RVOTAs in monochorionic diamniotic twin pregnancies complicated by TTTS and treated with fetoscopic laser surgery (FLS) and to analyze possible prenatal predictors of congenital heart disease (CHD). Prenatal RVOTAs were retrospectively retrieved from all TTTS cases treated with FLS in our unit between 2009 and 2019. Twenty-eight prenatal cases of RVOTAs (16 PI, 10 PS, 2 PA) were observed out of 335 cases of TTTS. Four cases did not reach the postnatal period. CHD was present in 17 of the remaining 24 cases (70.8%), with 10 being severe (58.8%; 10/17); nine cases of PS required balloon valvuloplasty, and one case required biventricular non-compaction cardiomyopathy. The risk of major CHD increased with prenatal evidence of PS and decreased with the gestational age at the time of TTTS and with the prenatal normalization of blood flow across the pulmonary valve. Despite treatment with FLS, the majority of monochorionic diamniotic twin pregnancies complicated by TTTS with prenatal RVOTAs had CHD at long-term follow-up.

“I Dread the Heart Surgery but it Keeps My Child Alive”—Experiences of Parents of Children with Right Ventricular Outflow Tract Anomalies during the Assessment for Cardiac Reoperation

“I Dread the Heart Surgery but it Keeps My Child Alive”—Experiences of Parents of Children with Right Ventricular Outflow Tract Anomalies during the Assessment for Cardiac Reoperation

Right ventricular outflow tract anomalies: Neonatal interventions and outcomes

Right ventricular outflow tract (RVOT) anomalies comprise a wide spectrum of congenital heart disease, typically characterized by obstruction to flow from the right ventricle to pulmonary arteries. This review highlights important considerations surrounding management strategy as well as clinical outcomes for the neonate with RVOT anomaly, including: pulmonary atresia with intact ventricular septum, congenital pulmonary valve stenosis, tetralogy of Fallot, and Ebstein anomaly with anatomic or physiologic RVOT obstruction.

VP18.02: Prenatal right ventricular outflow tract anomalies in monochorionic diamniotic twin pregnancies without TTTS: prenatal course and outcomes

VP18.02: Prenatal right ventricular outflow tract anomalies in monochorionic diamniotic twin pregnancies without TTTS: prenatal course and outcomes

Health-Related Quality of Life in Children With Earlier Surgical Repair for Right Ventricular Outflow Tract Anomalies and the Agreement Between Children and Their Parents.

Background: Children diagnosed with right ventricle outflow tract (RVOT) anomalies require surgical repair early in life, reoperations and lifelong follow-up. The aim is to comprehensively describe their health related quality of life (HRQoL) and to assess the agreement in this regard between children and parents.Methods and Results: Child- and parent-reported HRQoL was assessed in 97 children aged 8–18 years using three different HRQoL questionnaires. The mean age was 12.9 ± 3 years. The mean total score for the child report was lower in the PedsQL Cardiac Module than in the PedsQL 4.0 and DISABKIDS (p ≤ 0.001). The mean score for each domain in PedsQL Cardiac Module ranged between 67 (cognitive function) and 79 (physical appearance), and between 72 (school function) and 82 (physical and social function) in PedsQL 4.0. Nearly half of the children reported problems with shortness of breath during physical activity. In the PedsQL Cardiac Module the child-parent agreement was strong for 13 of 22 items.Conclusion: HRQoL problems as perceived by children with RVOT anomalies are best identified with the PedsQL Cardiac Module and relate mostly to cognitive and physical functioning. The agreement findings suggest the need to take into account both child- and parent reports in the assessment of HRQoL.

Left Ventricular Outflow Tract Anomalies: Echocardiographic Evaluation

The left ventricular outflow is divided into subvalvar area, the aortic valve and supra valvar region. There can occur a number of anomalies in the outflow tract, most commonly of which are obstructive lesions, which can exist in isolation or as a part of association with other defects such as ventricular septal defects or interrupted arch. The following chapter highlights on echocardiography imaging of the left ventricular outflow tract and its anomalies.

Utility of a Single 3-Vessel View in the Evaluation of the Ventricular Outflow Tracts.

Prenatal diagnosis of congenital heart disease can improve neonatal outcomes. The purpose of this study was to evaluate the utility of an isolated 3-vessel view image in evaluating the ventricular outflow tracts. Three-vessel view images were prospectively collected from consecutive patients referred to a regional fetal heart center for fetal echocardiography between gestational ages of 18 weeks and 23 weeks 6 days. Cardiac malformations were categorized as anomalies of the outflow tracts, the 4-chamber view, or combined lesions. A single representative still-frame 3-vessel view image was reviewed by 2 independent and blinded observers who were asked to label each image as "normal" or "abnormal." Test characteristics of the isolated 3-vessel view were calculated. During the study period, 122 consecutive patients (139 fetuses) underwent fetal echocardiography. Eight fetuses with fetal chest anomalies and 12 fetuses with oblique images were excluded. Thirty-four of 119 fetuses (28.6%) had abnormal echocardiograms, including 11 outflow tract anomalies and 16 combined anomalies. Using the 3-vessel view alone, both reviewers achieved 91% sensitivity for the detection of isolated outflow tract anomalies and mean sensitivity of 88% for combined anomalies. All cases of tetralogy of Fallot and transposition of the great arteries were identified in the 3-vessel view. A single 3-vessel view image can serve as a representative view of the outflow tracts and can show ventricular outflow tract anomalies with high sensitivity. Given that the conventional outflow tract views can be difficult to obtain, the 3-vessel view may serve as an effective first-line view when evaluating the ventricular outflow tracts for congenital heart disease.

Use of oversized injectable valves in growing children for total repair of right ventricular outflow tract anomalies (preliminary results).

Right ventricular outflow tract surgery was originally confined to transannular patching, in the belief that pulmonary regurgitation was well tolerated. Because follow-up evaluations revealed the deleterious effects of pulmonary regurgitation, surgery today aims to spare or replace the valve. Available replacement devices have short lifetimes, considering growth mismatch in children. We hypothesize that oversizing the right infundibulum anticipates growth and that a squeezed prosthesis can complete the expansion process. The No-React® Injectable BioPulmonic Valve is designed for right infundibular surgery in adults, and hundreds of implants have shown promising results. We used this device for surgery in babies, with the addition of an innovative oversizing technique. This study evaluates our preliminary results and investigates whether such a technique might reduce growth mismatch. From September 2010 through July 2012, we implanted 11 injectable pulmonic valves. The median age of our patients was 23 months. After opening the right infundibulum, we enlarged it as much as possible with a wide patch. Before completing the patch suture, we injected an oversized valve. No problems occurred during surgery. No major insufficiency or leak was observed. We conclude that prostheses can be quite oversized and perform well even when not completely expanded. Oversized injectable pulmonic valves, shrunken to a smaller diameter, enabled the implantation of a device wider than otherwise possible, without affecting performance. Moreover, the prosthesis tended to return to its original size following growth, thereby reducing growth mismatch. Longer follow-up and larger numbers of patients are needed for verification.

Early Surgical Experience of Right Ventricular Outflow Reconstruction with the RVOT Elan Conduit in Adults and Adolescents with Congenital Heart Disease

Several types of valves and valved conduits have been developed for right ventricular outflow tract reconstruction and yet no one has provided prolonged satisfactory hemodynamic performance. The RVOT Elan is a bioprosthetic heart valve conduit constructed from a vascular graft and a porcine stentless valve indicated specifically for right ventricular outflow tract reconstruction. The present study aimed to evaluate the early clinical and hemodynamic results following implantation of the RVOT Elan in adults and adolescents with congenitally corrected right ventricular outflow tract anomalies requiring reintervention. Nineteen consecutive patients underwent right ventricular outflow reconstruction with the RVOT Elan between June 2012 and May 2013 in a nationally centralized center for surgery of patients with congenital heart disease. Pulmonic transvalvular gradients were evaluated with echocardiograms at discharge. Postoperative data on adverse clinical events were obtained from patient records. Follow-up was performed in October 2013 and was 100% complete (mean 331 ± 102, median 315 days). There were no intraoperative deaths. Survival at 30 days was 100% and there were no late deaths. The mean gradients were 14, 15, 13 mm Hg for the 21, 23, and 25 mm valve size, respectively. There were no cases of endocarditis or valve thrombosis. Freedom from reoperation for any cause was 100%. Freedom from cardiovascular adverse events was 94.4% (data 90.5% complete). The RVOT Elan demonstrates excellent early clinical and favorable hemodynamic results, with low transvalvular gradients, good flow characteristics, and a complete freedom from reoperation. Ease of implantation is evidenced by favorable ischemic times. Long-term results are awaited.

Cost‐analysis of percutaneous pulmonary valve implantation compared to surgical pulmonary valve replacement

To perform cost evaluation and economic modeling of percutaneous pulmonary valve implantation (PPVI) compared to surgical revision. While, PPVI appears to be a viable alternative to surgical conduit revision in select patients with right ventricular outflow tract anomalies, its overall economic burden has yet to be determined. We examined the first 17 patients who underwent PPVI at our institution and compared them with the most-recently placed surgical valves. Economic data were obtained from the actual procedural and in-hospital charges and used as the base estimates for 5- and 10-year future modeling with appropriate sensitivity analysis. Median total hospital and procedural charges incurred by the patient were significantly higher for the surgical valve compared with PPVI ($126,406 ± $38,772 vs. $80,328 ± $17,387, P < 0.001). Median total societal charges were also higher for the surgical valve ($129,519 ± $39,021 vs. $80,939 ± $17,334, P < 0.001) owing to an average wage loss of $3,113 for surgical patients, contrasted to $611 who underwent PPVI, and a shorter length of stay (1.0 ± 0 vs. 5.7 ± 2.2, P <0.001) for PPVI. Sensitivity analysis determined that PPVI would need to fail at a rate of 17% per year (or 93% at 10 years) to lose its cost advantage. PPVI holds a significant cost advantage over the surgical approach, fewer hospital days, and incurs less patient wage loss. Furthermore, it would need to have a very high failure rate at 10 years to lose its cost advantage.

603: Array analysis of left ventricular outflow tract anomalies confirms the genetically heterogeneous nature of this subset of congenital heart defects

An extreme underdevelopment of the left side of the heart and aorta is the main characteristic of hypoplastic left heart syndrome (HLHS). This left ventricular outflow tract (LVOT) anomaly is fatal if untreated directly after birth, therefore antenatal detection is critical. Various studies have revealed potential gene involvement, yet hardly any information exists on copy number variants (CNV), whereas prenatal array is being offered now commonly when antenatally ultrasound abnormalities have been identified.

Right Ventricular Outflow Tract Reconstruction With Contegra Bovine Valved Conduit

A 11-year-old boy affected by truncus arteriosus (type 1) underwent a late complete repair in another institution. The right ventricular outflow tract reconstruction was performed with an 18-mm Contegra conduit (Medtronic Inc, Minneapolis, Minn) despite its contraindication in cases of pulmonary hypertension. An additional apical muscular ventricular septal defect was closed with a percutaneous device. His postoperative course was uneventful. At 18 years of age, he was referred to our institution for right ventricular dilatation and conduit endocarditis. A group D streptococcus was isolated on blood culture. Echocardiography and cardiac catheterization showed right ventricular dilatation, isosystemic right ventricular pressure, and aneurysmal dilatation …