Pulmonary Atresia with Intact Ventricular Septum

A rare abnormality characterized by a hypoplastic right heart and pulmonary atresia (narrowing or absence of the opening between the right ventricle and pulmonary artery). It accounts for less than 3% of congenital heart defects. OMIM: 265150

Networked: 76 relevant articles (1 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pulmonary Valve Stenosis (Pulmonary Stenosis)
2.	Pathologic Constriction (Stenosis)
3.	Tetralogy of Fallot (Fallot Tetralogy)
4.	Pulmonary Atresia (Pulmonary Valve Atresia)
5.	Myocardial Ischemia (Ischemic Heart Diseases)

Experts

1.	Goldstein, Bryan H: 2 articles (01/2022 - 05/2017)
2.	Chen, Sun: 2 articles (01/2020 - 01/2020)
3.	Wang, Jian: 2 articles (01/2020 - 01/2020)
4.	Zhang, Z W: 2 articles (06/2018 - 06/2018)
5.	Sachdeva, Ritu: 2 articles (05/2017 - 05/2015)
6.	Alejos, Juan C: 1 article (11/2022)
7.	Ameduri, Rebecca K: 1 article (11/2022)
8.	Boyle, Gerard J: 1 article (11/2022)
9.	Cantor, Ryan: 1 article (11/2022)
10.	Gajarski, Robert J: 1 article (11/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pulmonary Atresia with Intact Ventricular Septum:

1.	Biomarkers (Surrogate Marker)IBA 01/01/2020 - "Elabela: A Novel Biomarker for Right Ventricular Pressure Overload in Children With Pulmonary Stenosis or Pulmonary Atresia With Intact Ventricular Septum." 01/01/2020 - "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. "
2.	LigandsIBA 01/01/2020 - "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. "
3.	Apelin ReceptorsIBA 01/01/2020 - "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. "
4.	Anesthetics (Anesthetic Agents)IBA 09/01/2018 - "Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum." 01/01/2006 - "Anesthetic management of bidirectional cavopulmonary shunt in a patient with pulmonary atresia with intact ventricular septum associated with sinusoidal communications."
5.	Ferrosoferric Oxide (Magnetite)FDA Link 09/28/2022 - "We present a case of a two-day-old baby with postnatal diagnosis of pulmonary atresia with intact ventricular septum in whom non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography was used for the assessment of coronary artery anatomy." 09/28/2022 - "Right ventricle-dependent coronary circulation diagnosed by non-invasive ferumoxytol-enhanced 4D cardiac magnetic resonance angiography in pulmonary atresia with intact ventricular septum."
6.	Connexin 43 (Connexin43)IBA 05/01/1997 - "Laterality defects (heterotaxy) have been associated with connexin43 mutations, and mice lacking connexin43 developed pulmonary atresia with intact ventricular septum. "
7.	SolutionsIBA 07/01/2018 - "Transcatheter perforation of atretic pulmonary valve by the stiff end of a coronary wire in neonates with pulmonary atresia with intact ventricular septum: A solution in developing countries."
8.	Proteins (Proteins, Gene)FDA Link 06/01/1991 - "Massive intestinal protein loss was demonstrated and the site of loss determined by abdominal scintigraphy with 99mTc-labeled human serum albumin in a 9-year-old girl following the Fontan operation for pulmonary atresia with intact ventricular septum. "
9.	ProstaglandinsIBA 12/08/1984 - "Despite considerable progress in surgery and intensive care and the advent of prostaglandins, pulmonary atresia with intact ventricular septum remains a severe heart disease. "
10.	Polytetrafluoroethylene (Teflon)FDA Link 11/01/1984 - "The modified Blalock-Taussig shunt, interposing an expanded polytetrafluoroethylene graft between the subclavian and pulmonary arteries, was performed in 30 neonates with a mean age of 8.8 days and a mean weight of 3.14 kg. Underlying lesions included severe tetralogy of Fallot or its variant (N = 10), transposition complex (with pulmonary stenosis or atresia) (N = 6), single ventricle equivalents (with pulmonary atresia or stenosis) (N = 9), and pulmonary atresia with intact ventricular septum (N = 5). "

Therapies and Procedures

1.	Catheters 04/17/2012 - "This study investigated the outcome for all patients undergoing catheter valve perforation for pulmonary atresia with intact ventricular septum (PAIVS) 21 years after the first procedure at their center. " 04/01/2022 - "Increasing Use of Neonatal Catheter Intervention for Pulmonary Atresia With Intact Ventricular Septum: Management Trends From a Single Centre." 01/01/2019 - "Pulmonary atresia with intact ventricular septum (PA/IVS) can be treated by various operative and catheter-based interventions. " 04/17/2012 - "Long-term outcome following catheter valvotomy for pulmonary atresia with intact ventricular septum." 04/01/2009 - "We report a single-institution experience using a collaborative surgical and catheter-based approach to the initial treatment of pulmonary atresia with intact ventricular septum. "
2.	Stents 07/05/2023 - "Hybrid Right Ventricular Outflow Stent Insertion in a Small Neonate with Muscular Pulmonary Atresia with Intact Ventricular Septum: A Case Report." 01/01/2022 - "Differences in patient characteristics exist with more patients with pulmonary atresia with intact ventricular septum and expected biventricular repair undergoing PDA stent." 01/01/2022 - "Pulmonary atresia with intact ventricular septum and expected biventricular repair were more common with PDA stent compared with APS (39.6% versus 21.2%, P<0.001 and 57.9% versus 46.6%, P=0.007, respectively). " 06/01/2018 - "Conclusions: Stage Ⅰ arterial duct stent, stage Ⅱ surgery, and stage Ⅲ atrial septal occlusion to treat pulmonary atresia with intact ventricular septum in children is feasible and effective. " 07/01/2011 - "Pulmonary atresia with intact ventricular septum surgeries were performed in 2 patients at 5 and 7 months after stent implantation. "
3.	Therapeutics 01/01/2022 - "A Novel Modified Hybrid Therapy for Pulmonary Atresia With Intact Ventricular Septum." 05/01/2011 - "Hybrid therapy for pulmonary atresia with intact ventricular septum." 10/29/2010 - "A novel hybrid therapy for pulmonary atresia with intact ventricular septum." 08/01/2007 - "In patients with pulmonary atresia with intact ventricular septum (PAIVS), radiofrequency-assisted perforation of the valve is the most widely used initial therapy when the anatomy is favorable. " 05/01/1989 - "Pulmonary atresia with intact ventricular septum is an uncommon condition and, therefore, its response to various therapies is difficult to evaluate. "
4.	Blalock-Taussig Procedure 01/01/2017 - "In this study, we describe an infant case of pulmonary atresia with intact ventricular septum associated with ventriculo-coronary arterial communication for which a modified Blalock-Taussig shunt operation was performed. " 12/01/2014 - "Postnatally, a pulmonary atresia with intact ventricular septum was supplied by a modified Blalock-Taussig shunt. " 02/01/2000 - "Pulmonary atresia with intact ventricular septum percutaneous radiofrequency-assisted valvotomy and balloon dilation versus surgical valvotomy and Blalock Taussig shunt." 11/08/2023 - "Patent ductus arteriosus ligation without modified Blalock-Taussig shunt placement was performed in three patients with pulmonary atresia with intact ventricular septum and two patients with tetralogy of Fallot, ductus arteriosus was left open in four patients with pulmonary atresia with intact ventricular septum. " 11/01/1984 - "The modified Blalock-Taussig shunt, interposing an expanded polytetrafluoroethylene graft between the subclavian and pulmonary arteries, was performed in 30 neonates with a mean age of 8.8 days and a mean weight of 3.14 kg. Underlying lesions included severe tetralogy of Fallot or its variant (N = 10), transposition complex (with pulmonary stenosis or atresia) (N = 6), single ventricle equivalents (with pulmonary atresia or stenosis) (N = 9), and pulmonary atresia with intact ventricular septum (N = 5). "
5.	Heart Transplantation (Grafting, Heart) 11/01/2022 - "Outcomes of infants with pulmonary atresia with intact ventricular septum listed for heart transplantation: A multi-institutional study." 07/01/2008 - "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. "