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Pulmonary Valve Stenosis (Pulmonary Stenosis)

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The pathologic narrowing of the orifice of the PULMONARY VALVE. This lesion restricts blood outflow from the RIGHT VENTRICLE to the PULMONARY ARTERY. When the trileaflet valve is fused into an imperforate membrane, the blockage is complete.

Also Known As:

Pulmonary Stenosis; Stenosis, Pulmonary; Pulmonary Stenoses; Pulmonary Valve Stenoses; Pulmonic Stenosis; Stenoses, Pulmonary; Stenoses, Pulmonary Valve; Stenosis, Pulmonary Valve; Valvular Pulmonic Stenosis; Pulmonary Stenose; Pulmonic Stenoses; Pulmonic Stenoses, Valvular; Pulmonic Stenosis, Valvular; Stenose, Pulmonary; Stenoses, Pulmonic; Stenosis, Pulmonic; Valvular Pulmonic Stenoses

Networked: 869 relevant articles (43 outcomes, 39 trials/studies)

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Disease Context: Research Results

Cardiovascular Diseases: 37259
- Heart Diseases: 18762
  - Heart Valve Diseases: 960
    - Pulmonary Valve Stenosis: 869
  - Ventricular Outflow Obstruction: 94
    - Pulmonary Valve Stenosis: 869

Related Diseases

1.	Pathologic Constriction (Stenosis)
2.	Double Outlet Right Ventricle
3.	Takayasu Arteritis (Arteritis, Takayasu's)
4.	Tetralogy of Fallot (Fallot Tetralogy)
5.	Pulmonary Atresia (Pulmonary Valve Atresia)

Experts

1.	National Birth Defects Prevention Study: 8 articles (10/2015 - 07/2009)
2.	Correa, Adolfo: 5 articles (10/2015 - 07/2009)
3.	Shaw, Gary M: 4 articles (11/2014 - 02/2010)
4.	Lin, Angela E: 4 articles (10/2014 - 07/2008)
5.	Bussadori, C: 4 articles (09/2013 - 12/2000)
6.	Park, Chun Soo: 3 articles (08/2015 - 06/2010)
7.	Marshall, Audrey C: 3 articles (08/2015 - 07/2002)
8.	Fujii, Yoko: 3 articles (01/2015 - 05/2007)
9.	Anderka, Marlene: 3 articles (10/2014 - 03/2012)
10.	Botto, Lorenzo D: 3 articles (10/2014 - 01/2010)

Drugs and Biologics

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

1.	Coarctation of the aortaIBA 09/01/1986 - "Percutaneous balloon dilatation for pulmonary stenosis and coarctation of the aorta is a safe and effective alternative to operation. " 09/01/2013 - "Lesions causing obstruction to blood flow from the heart include aortic and pulmonary valve stenosis and coarctation of the aorta. " 01/01/2012 - "Parental smoking in early pregnancy can be considered a risk factor for valvar pulmonary stenosis and coarctation of the aorta. " 09/01/2002 - "Other features seen in this kindred included unilateral dysplastic kidney and cardiovascular malformation (i.e., bicuspid aortic valve, bicuspid aortic valve with coarctation of the aorta, or valvular pulmonary stenosis), which have not been previously associated. " 01/01/1987 - "Investigations demonstrated severe pulmonary valve stenosis and duct-dependent coarctation of the aorta. "
2.	Alprostadil (Muse)FDA LinkGeneric 01/01/1995 - "The patient had a critical pulmonary stenosis and was treated with an infusion of prostaglandin E1, which resulted in excessive pulmonary blood flow through the ductus arteriosus. " 11/01/1984 - "Experience with 36 consecutive neonates less than 1 month of age with critical pulmonary stenosis (PS) with intact ventricular septum was analyzed to define the role of pulmonary valvotomy and of a concomitant systemic-pulmonary shunt as well as the impact of prostaglandin E1 (PGE1) therapy in the management of this disorder. " 06/01/1981 - "An infant with Ebstein's malformation of the tricuspid valve and severe pulmonic stenosis underwent a 39-day course of prostaglandin E1 infusion, and a histologic study of the ductus arteriosus was undertaken after autopsy. " 09/01/1991 - "Oral prostaglandin E1 derivative (OP-1206) in an infant with double outlet right ventricle and pulmonary stenosis. " 06/01/1985 - "In 12 patients with pulmonary atresia or severe pulmonary stenosis, PGE1 infusion was followed by an improvement in hypoxemia and acidemia (group I). "
3.	Right ventricle hypoplasiaIBA 01/01/2006 - "Outcome of fetuses with critical pulmonary stenosis (critical PS) or atresia of the pulmonary valve (PA) with intact ventricular septum (IVS) is closely related with right ventricle hypoplasia and its consequent hemodynamics. " 10/01/1980 - "Extreme right ventricular hypoplasia after relief of severe pulmonary stenosis. " 02/01/1990 - "The early postoperative gradient was 20 +/- 2 mm Hg; the post-balloon valvotomy gradient was 18 +/- 3 mm Hg. We conclude that balloon pulmonary valvotomy yields good results in patients with critical pulmonary stenosis with essentially normal-sized right ventricle, whereas surgical pulmonary valvotomy is required for patients with right ventricular hypoplasia." 03/01/2012 - "We describe a case followed from the foetal age for a relative right ventricular hypoplasia and suspicious pulmonary stenosis and diagnosed after birth to have cor triatriatum dexter with an obstructing membrane in the right atrium, needing surgical correction, done successfully." 03/01/2007 - "A 2-month-old patient with transposition of the great arteries, a ventricular septal defect, pulmonary stenosis, and severe hypoplasia of the right ventricle successfully underwent a biventricular repair utilizing the aortic translocation technique. "
4.	ReninIBA 03/01/1988 - "The fall of blood pressure (22 +/- 10 mmHg; P less than 0.001) in the rats with pulmonic stenosis was significantly greater (P less than 0.01) and was strongly related to plasma renin concentration. " 09/01/1980 - "In patients with tetralogy of Fallot and pulmonary stenosis, this meant that plasma renin activity became normal when the preoperatively small left ventricle reached its normal dimension. " 09/01/1980 - "Patients with tetralogy of Fallot and pulmonary stenosis had higher plasma renin activity values than the others. " 03/01/1988 - "Our objectives were to examine the role of vasopressin and renin in the regulation of peripheral vascular tone in an animal model of right ventricular failure due to chronic (10 wk) pulmonic stenosis. " 03/01/1988 - "The rats with pulmonic stenosis showed significant hemodynamic changes, a hypertrophy of the right ventricle, increased levels of plasma renin concentration, and inappropriately elevated plasma levels of vasopressin in comparison to control animals. "
5.	Staphylococcal Protein A (A, Protein)IBA 07/01/2003 - "We report a 17-year-old boy with double-outlet right ventricle, atrioventricular septal defect, pulmonary stenosis, and persistent left superior vena cava who developed a protein-losing enteropathy, which was cured by coil embolization of the left azygos vein, thereby interrupting the vein-to-vein shunt."
6.	esmolol (Brevibloc)FDA LinkGeneric 03/01/1989 - "Infusion of esmolol partially relieved the dynamic sub-pulmonary stenosis. " 03/01/1989 - "Esmolol (Brevibloc), a new, ultra-short acting, cardioselective beta-adrenergic blocking agent with half-life of 9.2 min following i.v. administration was given to a 4-year-old child with known dynamic and fixed sub-pulmonary stenosis post Senning repair for complete transposition of the great vessels. "
7.	Complete atrioventricular septal defectIBA 04/01/2015 - "We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. " 04/01/2015 - "Complete atrioventricular septal defect and pulmonary stenosis diagnosed in a 49-year-old woman after 10 uneventful births." 11/01/2003 - "In multivariate analysis, complete atrioventricular septal defect-typed lesion, pulmonary venous obstruction, total anomalous pulmonary venous connection, and interruption of aortic arch were risk factors for long-term survival, while pulmonary stenosis was demonstrated as a favorable prognostic factor. " 04/01/2000 - "The general categories of TOF are: classic TOF with varying degrees of pulmonary stenosis, TOF with common atrioventricular canal defect, and TOF with absent pulmonary valve. " 08/01/1997 - "Two infants and 1 young child with isomeric right atrial appendages, complete atrioventricular canal, univentricular atrioventricular connection with a double-inlet right ventricle through a common atrioventricular valve, pulmonary atresia (n = 2) or pulmonary stenosis (n = 1), and bilateral superior venae cavae presented with marked dilatation of the annulus of the common atrioventricular valve and severe regurgitation between the bridging leaflets. "
8.	Idiopathic pulmonary hypertensionIBA 10/01/2011 - "The study was designed to compare RV morphological and functional parameters derived from conventional and myocardial deformation echocardiography in two instances of right heart pressure overload: pulmonary arterial hypertension (PAH) and pulmonary stenosis (PS). " 04/15/2008 - "Displacement and strain imaging echocardiographic studies were performed in 20 patients with RVPO from pulmonary arterial hypertension or pulmonic stenosis (mean age 53 +/- 16 years, New York Heart Association class 2.6 +/- 0.7, and peak RV systolic pressure 73 +/- 28 mm Hg) and 20 age-matched normal subjects (mean age 47 +/- 16 years). " 01/01/2009 - "Pulmonary stenosis development and reduction of pulmonary arterial hypertension in atrioventricular septal defect: a case report." 09/01/1998 - "All patients had severe pulmonary arterial hypertension except one who had associated pulmonic stenosis. " 12/01/1983 - "The prevalence of mitral valve prolapse (MVP) was examined in 16 patients with right ventricular pressure overload with a peak systolic pressure ranged from 60 to 204 mmHg (Group 1; 9 cases of primary pulmonary hypertension and 7 cases of pulmonary valve stenosis), and the clinical and echocardiographic data were compared to those of 12 patients with idiopathic MVP (Group 2) and 10 healthy persons (Group 3). "
9.	SASIBA 07/01/2010 - "Boxer are predisposed to subaortic (SAS) and pulmonic stenosis (PS). " 01/01/2009 - "Boxers are predisposed to subaortic and pulmonic stenosis (SAS, PS). " 01/01/1990 - "Our experience indicates that in the surgical treatment of the patient with SAS and associated severe multiple peripheral pulmonary stenoses, a staged approach, that is initial staged repair of the bilateral pulmonary stenoses by arterioplasty through thoracotomy followed by definitive intracardiac repair of SAS, is the procedure of choice." 01/01/1990 - "Successful surgical correction of supravalvular aortic stenosis (SAS) syndrome associated with severe peripheral pulmonary stenosis by a staged approach in a 6-year-old boy is reported. " 12/01/2000 - "Subaortic stenosis (SAS) and pulmonic stenosis (PS) are, with PDA, the most common canine congenital heart defects in almost all epidemiological studies or frequency tabulations of the various European countries and the United States. "
10.	tranilast (N 5')IBA 11/01/2007 - "Nine infant pigs (3-5 kg) underwent surgery involving an RVOT patch and creation of pulmonary insufficiency (PI, n = 4) or pulmonary stenosis (PS, n = 5). " 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). " 01/01/1994 - "Associated cardiac defects were ventricular septal defect (n = 7), pulmonary stenosis (n = 5), straddling tricuspid valve (n = 2), and straddling mitral valve (n = 1). " 11/01/1990 - "These abnormalities included isolated pulmonary valve stenosis (n = 9), septal (dynamic) subpulmonary stenosis (n = 5), anatomic (fixed) subpulmonary stenosis (n = 7), abnormal mitral chordae attachments (n = 2) or a combination of abnormalities (n = 7). " 07/15/2014 - "Miscellaneous group: 21 CCP stents in 17 patients: closure of Fontan-circuit fenestration (n=5), restoration of superior caval vein (n=2) or pulmonary artery (n=3) patency, relief of supra-pulmonary stenosis (n=2), exclusion of aberrant pulmonary arteries (n=1), cavopulmonary conduit expansion (n=2), Blalock-Taussig shunt flow reduction (n=1), and defibrillator lead protection from sharp stents (n=1). "

Therapies and Procedures

1.	Balloon Dilation 03/01/2004 - "Percutaneous balloon valvuloplasty was effective and safe for the treatment of pulmonary valve stenosis with excellent short- and long-term results." 07/01/2000 - "In conclusion, right ventricular diastolic filling indices in patients with pulmonary stenosis did not improve after pulmonary balloon valvuloplasty in the first day but when re-examined by the sixth month there was a significant improvement. " 01/01/1991 - "Percutaneous balloon valvuloplasty gives good results in nearly all patients with pulmonary valve stenosis." 01/01/1988 - "In this report, rapid improvement in both right and left ventricular function following successful percutaneous balloon valvuloplasty is described in a patient with severe isolated valvular pulmonic stenosis and biventricular dysfunction. " 09/01/2013 - "Dogs with severe pulmonic stenosis undergoing pulmonary balloon valvuloplasty (HR 0 · 47, P = 0 · 047) and asymptomatic dogs with moderate pulmonic stenosis (HR 0 · 10, P = 0 · 042) had a better outcome. "
2.	Angioplasty (Angioplasty, Transluminal) 03/01/1993 - "To assess the efficacy of arterial duct angioplasty in maintaining adequate ductal patency in neonates with critical pulmonary valve stenosis. " 12/01/2014 - "We have used an anterior wall of the MPA as a flap for LPA angioplasty in 42 patients with TOF and pulmonary stenosis (26 males and 16 females) since February 2007. " 04/01/2008 - "Although percutaneous trans-pulmonary angioplasty was necessary in 1, 13, and 3 patients, there was 1, 1, and 0 reoperation for pulmonary stenosis in the 3 groups, respectively. " 04/01/2000 - "Twenty-two patients (10 boys, 12 girls) with pulmonary valve stenosis whose mean age was 6.7 +/- 4.1 years (range 1 to 14 years) at time of the procedure underwent balloon dilatation angioplasty. " 03/01/1998 - "At 19 month of age, he was removed pulmonary stenosis using patch angioplasty with mono cusp. "
3.	Catheters 09/01/1987 - "The often remarkably good results of operations performed under less than ideal operating conditions for lesions such as isolated pulmonary stenosis encouraged the interventional cardiologist and radiologist, working together, to adapt the cardiac catheter, used previously for exploration of the vascular system and diagnosing intravascular lesions, to therapeutic purposes. " 11/01/1979 - "In a neonate suffering from this syndrome, the pulmonary valve stenosis was relieved by rupturing the fused valve with a balloon catheter introduced transvenously. " 01/01/2014 - "Entrapped Catheter across the Fossa Ovalis in an Adult with Pulmonary Stenosis - A Case Report of Surgical Relief." 07/01/2009 - "This anomaly needs to be differentiated from valvar pulmonary stenosis as both anomalies require different modality of treatment (surgical/catheter)." 01/01/2009 - "Surgical repair of peripheral pulmonary stenosis is indicated when preoperative intervention is unsuccessful and can include a combination of patch arterioplasty as well as intraoperative application of catheter-based techniques. "
4.	Stents 07/01/2013 - "Clinical and hemodynamic improvement after stent implantation for pulmonary stenosis in failing Fontan circulation." 12/01/2012 - "We describe a palliative application of the self-expandable stent in a patient with pulmonary stenosis after total correction of TOF who lacked a conduit between the right ventricle and pulmonary artery." 01/01/2012 - "However we have limited data of pulmonary arterial stent in paediatric population as the treatment of branch pulmonary stenosis. " 02/15/2011 - "Pulmonary artery (PA) stents are utilized to treat branch pulmonary stenosis (BPS). " 01/01/2007 - "Another subject of interest has been theuse of the cutting balloon in peripheral pulmonary stenosis and the implantation of stents in obstruction of conduits between the right ventricle and pulmonary artery. "
5.	Balloon Angioplasty 09/01/1993 - "This study was designed to assess the success rate and determinants of success or failure of balloon angioplasty for postoperative pulmonary stenosis in patients with transposition of the great arteries. " 01/01/2011 - "Balloon angioplasty for supravalvular pulmonary stenosis could be the initial treatment of choice owing to the high success rate. " 06/01/2002 - "Interventional catheterization management of perioperative peripheral pulmonary stenosis: balloon angioplasty or endovascular stenting." 09/01/2000 - "Four patients in XP-group required balloon angioplasty for pulmonary stenosis and 5 patients in XP-group underwent reoperation, but no patients in AP-group required balloon angioplasty or reoperation for pulmonary stenosis. " 05/20/1997 - "Transballoon intravascular ultrasound imaging during balloon angioplasty in animal models with coarctation and branch pulmonary stenosis."