Pulmonary Atresia (Pulmonary Valve Atresia)

A congenital heart defect characterized by the narrowing or complete absence of the opening between the RIGHT VENTRICLE and the PULMONARY ARTERY. Lacking a normal PULMONARY VALVE, unoxygenated blood in the right ventricle can not be effectively pumped into the lung for oxygenation. Clinical features include rapid breathing, CYANOSIS, right ventricle atrophy, and abnormal heart sounds (HEART MURMURS).

Also Known As:

Networked: 507 relevant articles (11 outcomes, 19 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Ventricular Heart Septal Defects (Ventricular Septal Defect)
2.	Pathologic Constriction (Stenosis)
3.	Cyanosis
4.	Pulmonary Valve Stenosis (Pulmonary Stenosis)
5.	Aortic Valve Stenosis (Aortic Stenosis)

Experts

1.	Gembruch, U: 5 articles (12/2012 - 08/2006)
2.	McCrindle, Brian W: 4 articles (01/2013 - 08/2003)
3.	Geipel, A: 4 articles (08/2012 - 08/2006)
4.	Berg, C: 4 articles (08/2012 - 08/2006)
5.	Zhang, Hao: 3 articles (08/2014 - 03/2006)
6.	Sano, Shunji: 3 articles (03/2014 - 11/2009)
7.	Kasahara, Shingo: 3 articles (03/2014 - 11/2009)
8.	Chiu, Ing-Sh: 3 articles (12/2012 - 02/2003)
9.	Lee, Meng-Luen: 3 articles (12/2012 - 02/2003)
10.	Mulder, Barbara J M: 3 articles (03/2012 - 07/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pulmonary Atresia:

1.	Coarctation of the aortaIBA 09/01/1983 - "Recently, results of surgery for pulmonary atresia, transposition of the great arteries, total anomalous pulmonary venous drainage, and coarctation of the aorta has been improved significantly. " 04/01/2014 - "Late detection varied by CCHD type from 9 of 120 infants (7.5% [95% CI, 3.5%-13.8%]) with pulmonary atresia to 497 of 801 (62.0% [58.7%-65.4%]) with coarctation of the aorta. " 09/01/2007 - "This is the first report of a child with pulmonary atresia with intact ventricular septum in association with coarctation of the aorta." 09/01/2007 - "Pulmonary atresia with intact ventricular septum coexisting with coarctation of the aorta." 04/01/2000 - "A one dimensional "anatomical" diagnostic hierarchy led to under ascertainment of pulmonary atresia by 27%, coarctation of the aorta by 39%, and interruption of the aorta by 100%. "
2.	Complete atrioventricular septal defectIBA 03/01/2010 - "Thirty-seven consecutive symptomatic nonrandomized neonates with TOF or its variants (including TOF with complete atrioventricular septal defect or absent pulmonary valve but excluding pulmonary atresia) had either a shunt (n = 17) or primary repair (n = 20). " 01/01/1989 - "Eighteen patients with complete atrioventricular septal defect (AVSD) were found to have either associated tetralogy (n = 15) or pulmonary atresia (n = 3). " 03/01/1988 - "Only infants with pulmonary atresia, complete atrioventricular canal, or coronary artery anomalies were shunted initially. " 04/01/2005 - "A case is described in which a two-year-old boy, diagnosed with common atrioventricular canal, pulmonary atresia, major aortopulmonary collateral artery, asplenia, and situs inversus, underwent palliative operations for the following: unifocalization of the right major aortopulmonary collateral artery, right ventricle-to-pulmonary artery shunt, and pulmonary artery plasty. " 10/01/2004 - "Our patient was diagnosed with complete atrioventricular canal and Tetralogy of Fallot with pulmonary atresia at the age of 1 month. "
3.	Alprostadil (Muse)FDA LinkGeneric 09/01/1998 - "We suggest that, in infants with pulmonary atresia and small pulmonary arteries, the optimal pulmonary artery size for BT shunt insertion is achieved after 2 weeks of PGE1 infusion, with no further significant increase in size being observed after this time." 01/01/1994 - "Aneurysmal change of the ductus arteriosus after prostaglandin E1 administration for pulmonary atresia: demonstration with magnetic resonance imaging." 03/01/1980 - "The structural effect of prostaglandin E1 on the pulmonary circulation in pulmonary atresia has been studied by applying quantitative morphometric techniques to the injected and inflated lungs of eight babies who had received prostaglandin E1 for between 30 hours and 12 days. " 03/01/1980 - "Effect of prostaglandin E1 on pulmonary circulation in pulmonary atresia. " 11/01/1978 - "The use of prostaglandin E1 in neonates with either pulmonary atresia or the coarctation syndrome to achieve ductal dilatation with resultant marked clinical improvement preoperatively is a major advance that should become a standard method of therapy over the next few years. "
4.	tebufenozide (Mimic)IBA 01/01/1999 - "We suggest that balloon atrial septostomy combined with construction of a Blalock-Taussig shunt may be effective in view of the hemodynamics, which mimic pulmonary atresia with intact interventricular septum." 06/15/2009 - "The Sonic hedgehog (Shh)-null mouse was initially described as a phenotypic mimic of Tetralogy of Fallot with pulmonary atresia (Washington Smoak, I., Byrd, N.A., Abu-Issa, R., Goddeeris, M.M., Anderson, R., Morris, J., Yamamura, K., Klingensmith, J., and Meyers, E.N. " 09/01/1985 - "Its presentation in the newborn may mimic the ductus dependent and less stable right ventricular hypoplasias such as tricuspid atresia and pulmonary atresia. "
5.	tranilast (N 5')IBA 10/01/2014 - "Coarctation was the most frequently associated heart defect (n = 5) followed by tetralogy of Fallot with or without pulmonary atresia (n = 3). " 03/01/2014 - "Branch pulmonary artery PI was evaluated in age-matched normal control fetuses (n=22) and 20 POTO fetuses (pulmonary stenosis n=15, pulmonary atresia n=5). " 10/01/1997 - "During a 17-month period ending August 1996, 12 patients, aged 1.6 to 39.0 years, underwent occlusion of residual ASD after repair of pulmonary atresia/stenosis with intact ventricular septum (n = 5), modified or fenestrated Fontan for tricuspid or pulmonary atresia (n = 5), and double-inlet left ventricle (n = 2). " 01/01/1995 - "The prior operations performed were placement of a palliative conduit for tetralogy of Fallot with pulmonary atresia (n = 5), repair of truncus arteriosus (n = 2), and repair of absent pulmonary valve syndrome (n = 1). " 03/01/2013 - "All patients had prior RVOT reconstruction; these patients averaged two prior operations (range: 1-4) for the following diagnoses: tetralogy of Fallot (n = 28), pulmonary atresia (n = 6), pulmonary stenosis (n = 6), transposition of the great arteries (n = 3), truncus arteriosus (n = 1), and others (n = 5). "
6.	Right ventricle hypoplasiaIBA 08/01/2012 - "Intrauterine valvuloplasty of a highly stenotic pulmonary valve or a pulmonary atresia with intact ventricular septum in order to prevent hypoplasia of the right ventricle and to enable postnatal biventricular repair is only rarely justified. " 07/01/2012 - "The mortality and morbidity of children with pulmonary atresia with intact ventricular septum (PA/IVS) is closely related with right ventricle hypoplasia and its consequent hemodynamics. " 03/01/2012 - "Management of pulmonary atresia with intact ventricular septum is challenging and depends on the severity of right ventricular hypoplasia. " 02/01/2006 - "Successful management of patients with pulmonary atresia with intact ventricular septum using a three tier grading system for right ventricular hypoplasia." 04/01/2005 - "As just over one-fifth of our survivors required such augmentation, we hypothesize that stenting of the patent arterial duct may be performed as an integral part of primary transcatheter therapy in patients with pulmonary atresia and intact ventricular septum who have moderate right ventricular hypoplasia and a small tricuspid valve."
7.	Pulmonary venous return anomalyIBA 10/01/2013 - "Several distinctive sonographic features of isomerism of the right atrial appendages were present in this fetus: complex cardiac abnormality, ventriculoarterial discordance, juxtaposition of the aorta and the inferior vena cava to the right side, pulmonary atresia, and anomalous pulmonary venous return to the morphological right atrium. " 09/01/1992 - "MRI is of unquestionable value in the evaluation of the thoracic and abdominal aorta and in demonstrating the vascular status in pulmonary atresia and anomalous pulmonary venous return. " 04/01/1989 - "The syndrome of a hypoplastic left heart, double outlow right ventricle, total anomalous pulmonary venous return, arterial truncus and pulmonary atresia have the most adverse prognosis." 09/01/1991 - "Bidirectional cavopulmonary anastomosis using vertical vein for right isomerism, pulmonary atresia, and TAPVR." 11/01/2013 - "We present a patient born with complex congenital heart disease consisting of single ventricle, pulmonary atresia, non confluent pulmonary arteries, and anomalous pulmonary venous return in whom surgical bidirectional Glenn was attempted. "
8.	1,2-didecanoyl-3-phosphatidic acid (PA(10))IBA 01/01/2012 - "Among those having a two-ventricle repair, anatomic pulmonary atresia (APA) was a risk factor for early mortality (46.1%, 6 of 13) compared with those without pulmonary atresia (EA/no-PA; 10%, 1 of 10), P < .05. " 01/01/2013 - "The current study aimed to develop a reliable targeted array comparative genomic hybridization (aCGH) to detect microdeletions and microduplications in congenital conotruncal defects (CTDs), especially on 22q11.2 region, and for some other chromosomal aberrations, such as 5p15-5p, 7q11.23 and 4p16.3. Twenty-seven patients with CTDs, including 12 pulmonary atresia (PA), 10 double-outlet right ventricle (DORV), 3 transposition of great arteries (TGA), 1 tetralogy of Fallot (TOF) and one ventricular septal defect (VSD), were enrolled in this study and screened for pathogenic copy number variations (CNVs), using Agilent 8 x 15K targeted aCGH. "
9.	beta-cyclodextrin tetradecasulfate (CTDS)IBA 01/01/2011 - "CTDs, especially pulmonary atresia with ventricular septal defect and tetralogy of Fallot, are the most common disorders associated with the 22q11.2 deletion syndrome. " 01/01/2013 - "The current study aimed to develop a reliable targeted array comparative genomic hybridization (aCGH) to detect microdeletions and microduplications in congenital conotruncal defects (CTDs), especially on 22q11.2 region, and for some other chromosomal aberrations, such as 5p15-5p, 7q11.23 and 4p16.3. Twenty-seven patients with CTDs, including 12 pulmonary atresia (PA), 10 double-outlet right ventricle (DORV), 3 transposition of great arteries (TGA), 1 tetralogy of Fallot (TOF) and one ventricular septal defect (VSD), were enrolled in this study and screened for pathogenic copy number variations (CNVs), using Agilent 8 x 15K targeted aCGH. "
10.	Polytetrafluoroethylene (Teflon)FDA Link 05/01/1998 - "A 10-day-old boy with pulmonary atresia received a right-sided aortopulmonary polytetrafluoroethylene shunt. " 03/01/1991 - "Between February 1980 and June 1987, 42 shunts were placed in 39 infants with pulmonary atresia: 33 were modified Blalock-Taussig shunts with polytetrafluoroethylene (PTFE) and 9 were classic Blalock-Taussig shunts. " 05/01/1987 - "Our current approach to the management of neonates with pulmonary atresia and intact ventricular septum is to perform a transarterial pulmonary valvotomy through a left anterolateral thoracotomy followed by a polytetrafluoroethylene shunt between the left subclavian artery and the pulmonary trunk at the site of the pulmonary arteriotomy. " 06/01/2011 - "hominis in a male child who had undergone right ventricular outflow tract (RVOT) reconstruction using an expanded polytetrafluoroethylene conduit for tetralogy of Fallot with pulmonary atresia. " 01/01/2004 - "Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. "

Therapies and Procedures

1.	Lasers (Laser) 09/01/1992 - "In patients with pulmonary atresia, either initial opening of the atretic pulmonary valve by laser or by surgery with subsequent balloon dilatation are potentially beneficial in reducing the total number of surgical procedures that these children are likely to require. " 07/01/1992 - "In patients with pulmonary valve atresia, initial opening of the atretic pulmonary valve by either laser or surgery with subsequent balloon dilatation is potentially beneficial in reducing the total number of surgical procedures that these children are likely to require. " 03/01/1997 - "Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy." 08/01/1997 - "Whereas this theory has not proven to be true, as the surface area of these channels is not sufficient for the nutrition of the surrounding myocardial tissue by diffusion or convection, different models have been developed by anatomical, experimental and clinical studies, such as the connection between the laser channels and intramyocardial vessels or capillaries, analogous to ventriculo-coronary connections in human anatomy or pathology as for example those connections described in children with pulmonary atresia and intact ventricular septum or the Thebesian veins. " 11/01/2006 - "Functional pulmonary atresia, secondary to right ventricular systolic dysfunction, resolved in 2 of 2 cases at post-laser echocardiography. "
2.	Stents 12/15/2012 - "Stent implantation for stenotic Blalock-Taussig shunts in a 5.75-year-old boy with pulmonary atresia." 07/01/2011 - "The neonatal pulmonary atresia with intact ventricular septum arterial stent implantation was a feasible and effective procedure and this method could be used as preferred treatment in pulmonary atresia and intact ventricular septum for neonates." 07/01/2011 - "Pulmonary atresia with intact ventricular septum surgeries were performed in 2 patients at 5 and 7 months after stent implantation. " 11/15/2009 - "Despite challenging, also tortuous arterial ducts as in ToF and pulmonary atresia with VSD can be addressed, since flexible open cell coronary stents are available. " 12/01/2007 - "The authors present a 32-year-old lady with a diagnosis of VSD, pulmonary atresia and multiple aortopulmonary collateral arteries (MAPCAs), who was palliated successfully by stent implantation. "
3.	Blalock-Taussig Procedure 04/01/2005 - "The objective of our study was to review the risk factors for stenting of the patent arterial duct, or construction of a Blalock-Taussig shunt, in the patients with pulmonary atresia and an intact ventricular septum who remain duct-dependent following radiofrequency valvotomy and dilation of the imperforate pulmonary valve. " 12/01/2014 - "Postnatally, a pulmonary atresia with intact ventricular septum was supplied by a modified Blalock-Taussig shunt. " 05/01/2014 - "A 12-day-old infant with pulmonary atresia, intact ventricular septum, and pulmonary blood flow through a ductus arteriosus developed complete shunt obstruction within 12 hr of creation of a modified Blalock-Taussig shunt. " 06/01/2012 - "A 1-month-old girl underwent right modified Blalock-Taussig shunt (mBTS) for pulmonary atresia with hypoplastic right ventricle. " 08/01/2007 - "Major cardiac anomalies (e.g., single atrium, single ventricle, common atrioventricular valve, and pulmonary atresia) were also present and had been treated elsewhere by a Blalock-Taussig shunt operation, the Glenn procedure, and pulmonary artery plasty. "
4.	Fontan Procedure (Fontan Operation) 08/01/1991 - "Modifying the Fontan procedure in this fashion was particularly useful in the management of 2 patients with pulmonary atresia and intact ventricular septum who had right ventricular-dependent coronary blood flow. " 05/01/1997 - "The goals of the study were to evaluate the operative and late mortality associated with the Fontan procedure in patients with pulmonary atresia and an intact ventricular septum and to obtain follow-up information on the current clinical status of surviving patients. " 10/01/2014 - "We present a patient with pulmonary atresia and severe right heart hypoplasia who underwent a lateral tunnel Fontan procedure in childhood and presented with an acute ST-segment elevation MI at 19 years of age. " 02/01/2013 - "We report hemodynamic findings in two patients with pulmonary atresia and intact ventricular septum (PAIVS) after "one-and-a-half ventricle repair" and placement of a bidirectional Glenn shunt using four-dimensional (4D) flow magnetic resonance imaging. " 02/01/2011 - "A concomitant one-stage unifocalization and bidirectional Glenn procedure was performed in a patient with a functionally single ventricle, pulmonary atresia, and major aortopulmonary collateral arteries (MAPCAs). "
5.	Lung Transplantation 11/01/2009 - "We retrospectively analyzed our experience with lung transplantation in 32 children with three distinct underlying congenital heart defects: (1) Eisenmenger syndrome (EIS); (2) tetralogy of Fallot with pulmonary atresia (PA); and (3) pulmonary vein stenosis (PVS). " 04/01/1994 - "Indications for lung transplantation in this pediatric population included the following: cystic fibrosis (n = 13), pulmonary hypertension, and associated congenital heart disease (n = 10), pulmonary atresia, ventricular septal defect and nonconfluent pulmonary arteries (n = 3), pulmonary fibrosis (n = 6), and acute respiratory distress syndrome (n = 1). " 04/01/1998 - "Certain defects like congenital pulmonary vein stenosis are eradicated by removal of the diseased lungs, whereas others such as complete atrioventricular canal and pulmonary atresia with ventricular septal defect require cardioplegic arrest of the heart and intracardiac repair in conjunction with the lung transplantation. "