The double shunt technique as a bridge to heart transplantation in a patient with pulmonary atresia with intact septum and right ventricular-dependent coronary circulation.
Ontology highlight
ABSTRACT:
SUBMITTER: Said SM
PROVIDER: S-EPMC8311501 | biostudies-literature |
REPOSITORIES: biostudies-literature
Similar Datasets
Project description: Not available
| S-EPMC8669549 | biostudies-literature
Project description:Background: Assessing right ventricular overload in children is challenging. We conducted this study involving children with pulmonary valvular stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS) to evaluate the potential of a new endogenous ligand of apelin receptor, Elabela (ELA), as a potential biomarker for right heart overload. Methods: In this prospective cohort study, a total of 118 congenital heart diseases patients with right ventricle outflow tract obstruction were recruited from 2018 to 2019. Among them, 44 isolated PS and 7 PA/IVS patients were selected. Their venous blood was collected, and all patients underwent an echocardiographic examination. Among them, post-operative blood was collected from 24 patients with PS after percutaneous balloon pulmonary valvuloplasty. The plasma ELA concentration was measured using enzyme-linked immunosorbent assay. Results: The ELA was significantly associated with the peak transvalvular pulmonary gradient (r = -0.62; p = 0.02), thus reflecting the severity of PS or PA/IVS. The ELA significantly increased at 3 days after intervention, when mechanical obstruction of the right outflow tract was relieved. Based on the receiver-operator characteristic curve results, ELA could be a risk factor for duct dependence in patients with critical PS or PA/IVS who are younger than 6 months (AUC: 0.82). Conclusion: ELA concentration and severity of PS or PA/IVS had a significant negative correlation, indicating that ELA might be a novel biomarker for right ventricular afterload and reflect the immediate pressure changes in the right heart. Furthermore, ELA could predict duct-dependency in PS and PA/IVS patients, as valuable as classical echocardiographic indexes.
| S-EPMC7688667 | biostudies-literature
Project description:OBJECTIVE:Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by various operative and catheter-based interventions. We aim to understand the long-term transplant-free survival of patients with PA/IVS by treatment strategy. METHODS:Cohort study from the Pediatric Cardiac Care Consortium, a multi-institutional registry with prospectively acquired outcome data after linkage with the National Death Index and the Organ Procurement and Transplantation Network. RESULTS:Eligible patients underwent neonatal surgery or catheter-based intervention for PA/IVS between 1982 and 2003 (median follow-up of 16.7 years, IQR: 12.6-22.7). Over the study period, 616 patients with PA/IVS underwent one of three initial interventions: aortopulmonary shunt, right ventricular decompression or both. Risk factors for death at initial intervention included earlier birth era (1982-1992), chromosomal abnormality and atresia of one or both coronary ostia. Among survivors of neonatal hospitalisation (n=491), there were 99 deaths (4 post-transplant) and 10 transplants (median age of death or transplant 0.7 years, IQR: 0.3-1.8 years). Definite repair or last-stage palliation was achieved in the form of completed two-ventricle repair (n=201), one-and-a-half ventricle (n=39) or Fontan (n=96). Overall 20-year survival was 66%, but for patients discharged alive after definitive repair, it reached 97.6% for single-ventricle patients, 90.9% for those with one-and-a-half ventricle and 98.0% for those with complete two-ventricle repair (log-rank p=0.052). CONCLUSIONS:Transplant-free survival in PA/IVS is poor due to significant infantile and interstage mortality. Survival into early adulthood is excellent for patients reaching completion of their intended path independent of type of repair.
| S-EPMC6571047 | biostudies-literature
Project description:We herein report the case of a neonate with pulmonary atresia with intact ventricular septum, right ventricular hypoplasia and severe aortic stenosis. Repetitive aortic valve surgeries in early infancy resulted in early relapse of the aortic stenosis due to the small aortic annulus, bicuspid configuration and duct-dependent pulmonary circulation. The infant underwent a successful mini-Konno procedure using a 10 mm handmade valve and bidirectional Glenn shunt at 6 months of age.
| S-EPMC4051372 | biostudies-literature
Project description:Pulmonary atresia with the intact ventricular septum (PA-IVS) is a rare anomaly that has an absent communication between the right ventricle and pulmonary arterial circulation. PA-IVS has a hypoplastic and hypokinetic and thickened right ventricle with the intact ventricular septum. It can be diagnosed with PA-IVS in routine obstetric ultrasound examination because the four-chamber view of PA-IVS is generally abnormal. The size of the right ventricular cavity is proportional to the Z value of the diameter of the tricuspid valve. The essential associated cardiac finding of PA-IVS is ventricular-coronary arterial communications (VCAC). The hypertensive RV forces blood through the intramyocardial sinusoids with continuous fistulous contact with the epicardial branches of RCA. It is called the VCAC. A color Doppler can detect VCAC due to its location in the pericardium along the coronary artery. If patients have VCAC and tricuspid Z score below -4, all of these conditions are infallible signs of high mortality rates in their fetal or postnatal lives. Our aim in presenting this case is to remind perinatologists if they detect an abnormal four-chamber view of the fetus's heart, they should also carefully examine whether VCAC exists. Herein, we report a case of PA-IVS and VCAC at 21 weeks' gestation with fetal echocardiographic images.
| S-EPMC7378622 | biostudies-literature
Project description:OBJECTIVE:To assess the immediate effects of fetal pulmonary valvuloplasty on right ventricular (RV) size and function as well as in-utero RV growth and postnatal outcome. METHODS:Patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS) who underwent fetal pulmonary valvuloplasty at our center between October 2000 and July 2017 were included. Echocardiographic data obtained before and after the procedure were analyzed retrospectively (median interval after intervention, 1 (range, 1-3) days) for ventricular and valvular dimensions and ratios, RV filling time (duration of tricuspid valve (TV) inflow/cardiac cycle length), TV velocity time integral (TV-VTI)?×?heart rate (HR) and tricuspid regurgitation (TR) velocity. Longitudinal data were collected from only those fetuses followed up in our center. Outcome was assessed using the scoring system as described by Roman et al. for non-biventricular outcome. RESULTS:Thirty-five pulmonary valvuloplasties were performed in our institution on 23 fetuses with PAIVS (n?=?15) or CPS (n?=?8). Median gestational age at intervention was 28?+?4 (range, 23?+?6 to 32?+?1) weeks. No fetal death occurred. Immediately after successful intervention, RV/left ventricular length (RV/LV) ratio (P???0.0001), TV/mitral valve annular diameter (TV/MV) ratio (P???0.001), RV filling time (P???0.00001) and TV-VTI?×?HR (P???0.001) increased significantly and TR velocity (P???0.001) decreased significantly. In fetuses followed longitudinally to delivery (n?=?5), RV/LV and TV/MV ratios improved further or remained constant until birth. Fetuses with unsuccessful intervention (n?=?2) became univentricular, all others had either a biventricular (n?=?15), one-and-a-half ventricular (n?=?3) or still undetermined (n?=?3) outcome. Five of nine fetuses with a predicted non-biventricular outcome, in which the procedure was successful, became biventricular, while two of nine had an undetermined circulation. CONCLUSION:In selected fetuses with PAIVS or CPS, in-utero pulmonary valvuloplasty led immediately to larger RV caused by reduced afterload and increased filling, thus improving the likelihood of biventricular outcome even in fetuses with a predicted non-biventricular circulation. © 2018 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.
| S-EPMC6100104 | biostudies-literature
Project description:Patients with congenital heart disease and duct-dependent pulmonary circulation can undergo stenting of the patent ductus arteriosus (PDA). This case shows that, due to the physiological changes occurring after stent implantation, sometimes it is necessary to close the stented PDA rather than to redilate it.
| S-EPMC5799631 | biostudies-literature
Project description:An unguarded atrioventricular orifice is an extremely rare congenital anomaly characterized by the absence of the atrioventricular valve in varying proportions. While atresia of the mitral or aortic valves are usually described as causes for hypoplastic left heart, our case highlights the role of free atrioventricular valve regurgitation and consequent volume loss of the left heart, giving rise to a small left ventricle. There was an associated double-outlet right ventricle and Type B aortic interruption. While we have attempted to discuss the complex management options in this scenario, the parents decided to withdraw further care.
| S-EPMC6521664 | biostudies-literature
Project description:A bias favoring biventricular (BV) repair exists regarding choice of repair pathway for patients with pulmonary atresia with intact ventricular septum (PAIVS). We sought to determine the implications of moving borderline candidates down a BV route in terms of late functional health status (FHS) and exercise capacity (EC).Between 1987 and 1997, 448 neonates with PAIVS were enrolled in a multi-institutional study. Late EC and FHS were assessed following repair (mean 14 years) using standardized exercise testing and 3 validated FHS instruments. Relationships between FHS, EC, morphology, and 3 end states (ie, BV, univentricular [UV], or 1.5-ventricle repair [1.5V]) were evaluated.One hundred two of 271 end state survivors participated (63 BV, 25 UV, and 14 1.5V). Participants had lower FHS scores in domains of physical functioning (P < .001) compared with age- and sex-matched normal controls, but scored significantly higher in nearly all psychosocial domains. EC was higher in 1.5V-repair patients (P = .02), whereas discrete FHS measures were higher in BV-repair patients. Peak oxygen consumption was low across all groups, and was positively correlated with larger initial tricuspid valve z-score (P < .001), with an enhanced effect within the BV-repair group.Late patient-perceived physical FHS and measured EC are reduced, regardless of PAIVS repair pathway, with an important dichotomy whereby patients with PAIVS believe they are doing well despite important physical impediments. For those with smaller initial tricuspid valve z-score, achievement of survival with BV repair may be at a cost of late deficits in exercise capacity, emphasizing that better outcomes may be achieved for borderline patients with a 1.5V- or UV-repair strategy.
| S-EPMC3838918 | biostudies-literature
Project description:Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital heart defect defined by membranous or muscular atresia of the right ventricular outflow tract where patients display varying degrees of hypoplasia of the right ventricle. This condition results in cyanosis due to an inability of blood to flow from the right ventricle to the pulmonary arteries, thus requiring immediate surgical intervention after birth. An iPSC line was generated from peripheral blood mononuclear cells of a 11-year-old male patient diagnosed with PA-IVS through Sendai virus-mediated reprogramming. This disease-specific iPSC line was characterized by immunocytochemistry, STR analysis, karyotype analysis, and mycoplasma testing.
| S-EPMC9629876 | biostudies-literature