HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

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:
Pulmonary Valve Atresia; Atresia, Pulmonary Valve; Atresias, Pulmonary; Atresias, Pulmonary Valve; Pulmonary Atresias; Pulmonary Valve Atresias; Valve Atresia, Pulmonary; Valve Atresias, Pulmonary; Atresia, Pulmonary
Networked: 417 relevant articles (9 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

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

Experts

1. Hanley, Frank L: 6 articles (01/2019 - 01/2011)
2. Sakamoto, Kisaburo: 4 articles (06/2022 - 09/2014)
3. Kasahara, Shingo: 4 articles (01/2022 - 11/2009)
4. Kotani, Yasuhiro: 4 articles (01/2022 - 11/2009)
5. Glatz, Andrew C: 4 articles (10/2019 - 08/2018)
6. Goldstein, Bryan H: 4 articles (10/2019 - 08/2018)
7. Petit, Christopher J: 4 articles (10/2019 - 08/2018)
8. Qureshi, Athar M: 4 articles (10/2019 - 08/2018)
9. Asija, Ritu: 4 articles (01/2019 - 01/2018)
10. McElhinney, Doff B: 4 articles (01/2019 - 10/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pulmonary Atresia:
1. Alprostadil (Muse)FDA LinkGeneric
2. beta-cyclodextrin tetradecasulfate (CTDS)IBA
3. Contrast MediaIBA
4. 1,2-didecanoyl-3-phosphatidic acid (PA(10))IBA
5. Radioisotopes (Radionuclides)IBA
6. Polytetrafluoroethylene (Teflon)FDA Link
7. ProstaglandinsIBA
8. Anesthetics (Anesthetic Agents)IBA
9. Coenzyme A (CoA)IBA
04/02/2017 - "The diagnoses most likely to be unrecognized at discharge included critical coarctation of the aorta (COA) (75.00%), total anomalous pulmonary venous connection (61.54%), pulmonary atresia (PA) with ventricle septal defect (VSD) (61.45%), single ventricle (SV) (60.10%) and critical aortic stenosis (52.94%). "
06/01/2003 - "Primary diagnoses included variant of single ventricle (8), ventricular septal defect (VSD) (4), tetralogy of Fallot (TOF) (2), pulmonary atresia (PA) with VSD (3), simple transposition of the great arteries (TGA) (1), TGA with VSD (TGA/VSD) (2), atrioventricular canal (AVC) (4), double outlet right ventricle (DORV) (3), aortic coarctation (CoA) (2), and interrupted aortic arch with VSD (IAA/VSD) (3). "
11/15/2023 - "The congenital cardiac abnormalities noted included tetralogy of Fallot (ToF) with pulmonary atresia and collaterals (n = 1), ToF with absent pulmonary valve (n = 1), ToF (n = 2), discontinuous right pulmonary artery (RPA) (n = 1), tricuspid atresia (TA) with normally related great arteries and pulmonary valve stenosis or atresia (n = 2) and coarctation of aorta (CoA) with bicuspid aortic valve (BAV) and borderline left-sided structures (n = 1). "
04/01/2009 - "There were 675 cases with ventricular septal defect with pulmonary hypertension (VSD/PH), 138 with complete transposition of the great arteries (TGA), 155 with tetralogy of Fallot (TOF), 111 with totally abnormal pulmonary venous connection (TAPVC), 54 with coarctation of aorta or interrupted aortic arch with ventricular septal defect [CoA(IAA)/VSD], 46 with double outlet right ventricle (DORV), 25 with pulmonary atresia with ventricular septal defect (PA/VSD), 24 with pulmonary atresia with intact interventricular septum (PA/IVS) and so on. "
01/01/1989 - "The most frequent congenital heart defects (CHD) were ventricular septal defect (VSD) (31.41%), atrial septal defect (ASD) (11.37%), aortic stenosis (AS) (7.64%), pulmonary stenosis (PS) (7.13%), coarctation of the aorta (CoA) (5.77%), and transposition of the great arteries (TGA) (5.43%), followed by persistent ductus arteriosus (PDA) (4.75%), atrioventricular septal defect (AVSD) and hypoplastic left heart syndrome (HLHS) (4.07% each), tetralogy of Fallot (TF) (3.56%), and pulmonary atresia (PA) (2.38%). "
10. Dinoprostone (PGE2)FDA Link

Therapies and Procedures

1. Lasers (Laser)
2. Stents
3. Operative Surgical Procedures
4. Blalock-Taussig Procedure
5. 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. "
01/01/2019 - "In patients with pulmonary atresia with an intact ventricular septum (PA/IVS), there are no reports about the impact of sinusoidal communication (SC) on left ventricular (LV) performance after a Fontan operation; therefore, the purpose of this study was to fill this gap in the literature. "
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. "
01/01/2022 - "This review article describes the utilization of CMR-derived flow data in surgical decision-making in three distinct subgroups: (1) patients with borderline left ventricle (LV) with emphasis on the ascending aortic flow and other physiologic parameters, (2) single ventricle patients who undergo bidirectional cavopulmonary shunt with emphasis on the impact of superior vena cava blood flow on postoperative physiology, and (3) patients with pulmonary atresia and major aortopulmonary collateral arteries with emphasis on the impact of total pulmonary blood flow and systemic-to-pulmonary flow ratio on clinical outcomes."
01/01/2022 - "Patients with pulmonary atresia and intact ventricular septum (PA-IVS) require intervention early in life, and most survive to a definitive procedure of either Fontan circulation or right ventricle to pulmonary artery (RV-PA) repair. "