Pulmonary Atresia With Ventricular Septal Defect

An isolated case of a father and son with these two abnormalities suggests autosomal dominant inheritance. Multiple genes and environmental factors are likely involved in its etiology. OMIM: 178370

Networked: 46 relevant articles (1 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Complete atrioventricular septal defect
2.	Pulmonary Vein Stenosis
3.	Tetralogy of Fallot (Fallot Tetralogy)
4.	Pulmonary Atresia (Pulmonary Valve Atresia)
5.	Ventricular Heart Septal Defects (Ventricular Septal Defect)

Experts

1.	Sun, Kun: 2 articles (01/2021 - 12/2011)
2.	Wang, Jian: 2 articles (01/2021 - 12/2011)
3.	Nagata, Hazumu: 1 article (10/2021)
4.	Nagatomo, Yusaku: 1 article (10/2021)
5.	Ohga, Shouichi: 1 article (10/2021)
6.	Yamamura, Kenichiro: 1 article (10/2021)
7.	Chen, Alex F: 1 article (01/2021)
8.	Chen, Sun: 1 article (01/2021)
9.	Chittithavorn, Voravit: 1 article (01/2021)
10.	Duangpakdee, Pongsanae: 1 article (01/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pulmonary Atresia With Ventricular Septal Defect:

1.	1-O-hexadecyl-2-N-methylcarbamol -sn-glycerol-3-phosphocholineIBA 11/01/2016 - "Pediatric CPAF cases were usually associated with pulmonary atresia with ventricular septal defect. " 10/01/2017 - "In cases of pulmonary atresia with ventricular septal defect (PA-VSD), coronary-pulmonary arterial fistula (CPAF) as the main source of pulmonary blood supply is extremely rare. "
2.	ProstaglandinsIBA 01/01/1992 - "The previously described and now confirmed anatomic differences of ductus arteriosus or different levels of endogenous prostaglandins may explain persistent patency of the ductus in pulmonary atresia with ventricular septal defect. "
3.	Polytetrafluoroethylene (Teflon)FDA Link 02/01/2015 - "We evaluated our clinical experiences on rehabilitation of native pulmonary arteries (PAs) with a central shunt using an expanded polytetrafluoroethylene (ePTFE) tube graft in management of pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs) with diminutive PAs. "
4.	Polyethylene Terephthalates (Polyethylene Terephthalate)IBA 07/01/1992 - "The diagnosis was transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction in 51, truncus arteriosus in 36, pulmonary atresia with ventricular septal defect in 25, tetralogy of Fallot in 19, double-outlet right ventricle in 10, pulmonary atresia with intact ventricular septum in 6 and atrioventricular canal with pulmonary atresia in 1. A Dacron porcine-valved conduit was used in 37, a homograft conduit in 106 and a nonvalved conduit in 5. There were 13 early deaths overall (8.8%); 8 (22%) of the early deaths occurred in the 37 patients who received a Dacron graft, 4 (3.8%) occurred in the 106 patients who received a homograft and 1 occurred in a patient with a nonvalved Gore-Tex conduit. "
5.	Phenobarbital (Luminal)FDA Link 01/01/2021 - "Pulmonary atresia with ventricular septal defect (PA/VSD) is a rare congenital heart disease (CHD) characterized by a lack of luminal continuity and blood flow from either the right ventricle or the pulmonary artery, together with VSDs. "
6.	Midazolam (Versed)FDA LinkGeneric 04/01/1999 - "A 6-month-old infant had pulmonary atresia with ventricular septal defect (PA + VSD) accompanied by excess pulmonary artery flow via major aortopulmonary collateral artery, underwent aortopulmonary shunt with artificial graft under midazolam-fentanyl anesthesia. "
7.	Heparin (Liquaemin)FDA LinkGeneric 08/01/1998 - "Univariate analysis revealed age (p = 0.02), a diagnosis of pulmonary atresia with ventricular septal defect and systemic-pulmonary collaterals (p = 0.001), reimplantation of collaterals during the procedure (p < 0.001), and intravenous heparin administered after operation (p < 0.0001) as risk factors for symptomatic perigraft seroma. "
8.	Fentanyl (Sublimaze)FDA LinkGeneric 04/01/1999 - "A 6-month-old infant had pulmonary atresia with ventricular septal defect (PA + VSD) accompanied by excess pulmonary artery flow via major aortopulmonary collateral artery, underwent aortopulmonary shunt with artificial graft under midazolam-fentanyl anesthesia. "
9.	Dihydrotachysterol (AT 10)IBA 02/25/1998 - "In the cohorts with shorter follow-up, at 20 years after surgery the cumulative incidence was 4.0% for dextrotransposition of the great arteries; at 10 years, the cumulative incidence was 1.1% for complete atrioventricular septal defect, 5.3% for pulmonary atresia with an intact ventricular septum, and 6.4% for pulmonary atresia with ventricular septal defect. "
10.	Coenzyme A (CoA)IBA 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. "

Therapies and Procedures

1.	Lung Transplantation 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. "
2.	Operative Surgical Procedures 01/01/2019 - "The study enrolled 32 patients with pulmonary atresia with ventricular septal defect and MAPCAs who were undergoing their first surgical procedure. " 02/01/1996 - "Six months after operation 2 patients are in New York Heart Association functional class I and 2 are in class II. Transverse thoracosternotomy gives excellent access to the anatomical structures necessary to correct complex cases of pulmonary atresia with ventricular septal defect and may reduce the number of surgical procedures."
3.	Aftercare (After-Treatment) 05/01/2016 - "Long-term outcome after treatment of pulmonary atresia with ventricular septal defect: nationwide study of 109 patients born in 1970-2007."
4.	Stents 10/04/2021 - "We report a 6-month-old infant with pulmonary atresia with ventricular septal defect who was successfully treated with a novel technique of percutaneous major aortopulmonary collateral artery (MAPCA) banding with a covered stent. " 08/01/2015 - "We report our experience with implantation of an Absorb bioresorbable stent in the stenotic major aortopulmonary collateral artery (MAPCA) of a 1-year-old girl born with pulmonary atresia with ventricular septal defect." 04/01/2007 - "Pulmonary atresia with ventricular septal defect and hypoplastic pulmonary arteries represents one such cohort where the placement of fixed nondegradable stents has been difficult. " 01/01/2002 - "Use of small stents for rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect." 04/01/1997 - "Use of Palmaz stents to palliate pulmonary atresia with ventricular septal defect and stenotic aortopulmonary collaterals."
5.	Blalock-Taussig Procedure 02/01/2000 - "A newborn with pulmonary atresia with ventricular septal defect and transposition of the great arteries was palliated with a modified Blalock-Taussig shunt at 5 days of age. " 04/01/1995 - "A 5-yr-old male had undergone a right-modified Blalock-Taussig shunt operation at the age of 1 mo for stenotic patient ductus arteriosus and pulmonary atresia with ventricular septal defect. " 01/01/1994 - "The patient was 11-year-old female, who had undergone Ross-Rastelli operation with a 16 mm Carpentier-Edwards valved conduit for pulmonary atresia with ventricular septal defect at six year-old following Blalock-Taussig shunt at four year-old. " 05/01/2014 - "A 3-year-old boy with pulmonary atresia with ventricular septal defect, who had undergone placement of a modified Blalock-Taussig shunt, presented with a 1-week history of high fever. "