Materiały źródłowe

• #1 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10320808/

  Only a small percentage of PS is acquired and caused by rheumatic disease, carcinoid disease and neoplastic lesions or may occur after surgical reconstruction for other complex congenital cardiac disorders: reconstruction often entails the placement of a pulmonary valve prosthesis, right ventricle-to-pulmonary artery homograft, or valved conduit, which degenerates over time, manifesting as stenosis, regurgitation, or both. […] The natural history of PS leads to secondary changes in other cardiac structures. In particular, the right ventricular systolic pressures needed to override the outlet stenosis can be even higher than systemic left ventricular pressures. Pressure overload increases wall stress and, in order to maintain a normal cardiac output, there is an increase in contractility and a compensatory right ventricular hypertrophy, an increase of end-systolic volume and end-diastolic volume and high right ventricular end-diastolic pressures. At first, these compensatory adaptations enable the right ventricle (RV) to maintain stroke volume in presence of increased afterload. Over time, progressive right ventricular hypertrophy and stiffness can determine right ventricular diastolic and systolic dysfunction and this leads to fibrosis of the endocardium and of the tricuspid valve apparatus, right ventricular ischemia, hypertension and hypertrophy of the right atrium and even arrhythmias. Therefore, elevated levels of NT-proBNP in neonates might be used as a biomarker for diagnosis of severe PS.

• #2 What Is Pulmonary Stenosis?

  https://www.icliniq.com/articles/respiratory-health/pulmonary-stenosis

  Pulmonary valve stenosis occurs at birth or due to systemic manifestation in adults. […] In congenital heart disease, the development of the pulmonary valve is not complete. It occurs during pregnancy and is termed a birth defect. The pulmonary valve comprises three cusps (a flap-like tissue). These cusps open and close, corresponding to each heartbeat. During the opening and closing of the valve, the blood flows in the right direction. The thickness of the pulmonary valve increases and becomes stiff due to pulmonary valve stenosis. […] In severe cases of stenosis, one or more cusps fuses and block the blood flow. The blood flows through the narrowest valve. It results in improper blood flow to the right ventricle (lower right heart chamber). This eventually increases the pressure in the right ventricle. This pressure is created as the heart tries to pump the blood through the small opening in the valve. It causes a strain in the muscular wall of the right ventricle. The thickness of the muscular wall increases due to the pressure.

• #3 Pulmonary Valve Disease: Types & Symptoms

  https://my.clevelandclinic.org/health/diseases/pulmonary-valve-disease

  Pulmonary valve stenosis happens in 7% of people with congenital (present at birth) heart disease. […] Many cases of pulmonary stenosis have an unknown cause, but some may be due to several genetic disorders or a mother having rubella during pregnancy.

• #4 Clinical manifestations and diagnosis of pulmonic stenosis in adults – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-pulmonic-stenosis-in-adults

  Stenosis of the pulmonary valve is a relatively common congenital defect, occurring in approximately 7 percent of children with congenital heart disease. There is a slight female predominance, and familial occurrence has been reported in 2 percent of cases in the absence of a genetic disorder. […] Pulmonic stenosis can occur at three locations: Valvular, Subvalvular.

• #5 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10320808/

  In the classical form of PS the valve is dome shaped, characterized by a narrow central opening with preserved valve motion. There are generally two to four rudimentary raphes without a real separation into valve leaflets. Valve calcification is rare but it is seen in some elderly patients. Less commonly the valve may be uni, bi or tricuspid with various degrees of commissural fusion and thickened cusps. Approximately 10-20% of cases involve pulmonic valve dysplasia. Dysplastic valves are trileaflet with thickened cusps composed of myxomatous tissue and little or no fusions, with relative valve immobility. This type of valve defect is a common component of Noonan syndrome. PS may also occur as part of complex congenital lesions (i.e., tetralogy of Fallot, complete atrioventricular canal, double outlet RV, univentricular heart). Most patients develop dilation of the pulmonary trunk (post stenotic dilation), with a degree of dilation not always proportional to the severity of obstruction. One exception to this finding is peripheral pulmonary artery stenosis usually found in Noonans and Williams syndrome.

• #6 Pulmonary Stenosis – ACHA

  https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/pulmonary-stenosis/

  Pulmonary stenosis occurs when the pulmonary valve does not form correctly. This happens in the first 8 weeks of fetal development. We dont know exactly why. Most of the time it occurs by chance. […] Pulmonary stenosis is classified according to location and severity of the defect. There are 3 types: Valvar pulmonary stenosis is the most common. In this, the pulmonary valve is dome-shaped and the opening is narrow. The leaflets are fused together. If the leaflets are thick, it is called a dysplastic valve. Pulmonary valvar stenosis can occur alone or with other congenital heart defects. These include tetralogy of Fallot or corrected transposition of the great arteries. It may also be part of a genetic syndrome, such as Noonans syndrome. […] When the muscle under the pulmonary valve is thickened, it is called subvalvar pulmonary stenosis. This is caused by additional muscle bundles in the right ventricle. […] Supravalvar stenosis occurs when there is narrowing in the pulmonary artery above the pulmonary valve. There can be one or more than one areas of narrowing. This may be seen with genetic syndromes such as Williams or Alagille syndrome.

• #7 Pulmonary Valve Stenosis

  https://medmovie.com/library_id/3172/topic/cvml_0059a/

  The pulmonary valve is located between the right ventricle and the large blood vessel to the lungs which is called the pulmonary artery. Pulmonary Valve Stenosis is a condition in which the pulmonary valve leaflets are thickened and fused, making it harder for blood to flow from the right ventricle to the lungs. […] Pressure rises in the right ventricle in order to pump blood through the narrowed valve. […] When the pressure build-up is significant, the muscle of the right ventricle becomes thickened, known as hypertrophy, and this can eventually lead to muscle damage. […] A significantly narrowed valve can be opened by balloon dilation. […] This procedure is called a transcatheter balloon valvuloplasty. […] Because the balloon valvuloplasty often tears the pulmonary valve leaflets, they may not close tightly. […] This causes leakage of blood backward into the ventricle, also known as insufficiency or regurgitation. […] Later in life patients may require a valve replacement if the insufficiency or regurgitation becomes severe.

• #8 Pulmonary Stenosis – ACHA

  https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/pulmonary-stenosis/

  Pulmonary stenosis occurs when the pulmonary valve does not form correctly. This happens in the first 8 weeks of fetal development. We dont know exactly why. Most of the time it occurs by chance. […] Pulmonary stenosis is classified according to location and severity of the defect. There are 3 types: Valvar pulmonary stenosis is the most common. In this, the pulmonary valve is dome-shaped and the opening is narrow. The leaflets are fused together. If the leaflets are thick, it is called a dysplastic valve. Pulmonary valvar stenosis can occur alone or with other congenital heart defects. These include tetralogy of Fallot or corrected transposition of the great arteries. It may also be part of a genetic syndrome, such as Noonans syndrome. […] When the muscle under the pulmonary valve is thickened, it is called subvalvar pulmonary stenosis. This is caused by additional muscle bundles in the right ventricle. […] Supravalvar stenosis occurs when there is narrowing in the pulmonary artery above the pulmonary valve. There can be one or more than one areas of narrowing. This may be seen with genetic syndromes such as Williams or Alagille syndrome.

• #9 Pulmonary Stenosis – ACHA

  https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/pulmonary-stenosis/

  Pulmonary stenosis occurs when the pulmonary valve does not form correctly. This happens in the first 8 weeks of fetal development. We dont know exactly why. Most of the time it occurs by chance. […] Pulmonary stenosis is classified according to location and severity of the defect. There are 3 types: Valvar pulmonary stenosis is the most common. In this, the pulmonary valve is dome-shaped and the opening is narrow. The leaflets are fused together. If the leaflets are thick, it is called a dysplastic valve. Pulmonary valvar stenosis can occur alone or with other congenital heart defects. These include tetralogy of Fallot or corrected transposition of the great arteries. It may also be part of a genetic syndrome, such as Noonans syndrome. […] When the muscle under the pulmonary valve is thickened, it is called subvalvar pulmonary stenosis. This is caused by additional muscle bundles in the right ventricle. […] Supravalvar stenosis occurs when there is narrowing in the pulmonary artery above the pulmonary valve. There can be one or more than one areas of narrowing. This may be seen with genetic syndromes such as Williams or Alagille syndrome.

• #10 Pulmonary Valve Stenosis | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/pulmonary-valve-stenosis

  Pulmonary valve stenosis occurs due to improper development of the pulmonary valve in the first eight weeks of fetal growth. Most of the time, this heart defect occurs by chance, with no clear reason for its development.

• #11 Pulmonary Stenosis: Causes and Symptoms

  https://www.massgeneral.org/children/pulmonary-stenosis

  Pulmonary stenosis is usually caused by abnormal development of the heart during the first few months of pregnancy. This heart defect usually occurs at random and the exact cause is unknown. Pulmonary stenosis is not anyones fault.

• #12 Pulmonic valve stenosis: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001096.htm

  Pulmonic stenosis is a heart valve disorder that involves the pulmonary valve. […] Narrowing of the pulmonary valve is most often present at birth (congenital). It is caused by a problem that occurs as the baby develops in the womb before birth. The cause is unknown, but genes may play a role. […] The defect may occur alone or with other heart defects that are present at birth. The condition can be mild or severe. […] Pulmonic stenosis is a rare disorder. In some cases, the problem runs in families.

• #13 Valvar Pulmonary Stenosis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/891729-overview

  Pulmonary valve stenosis is primarily due to maldevelopment of the pulmonary valve tissue and the distal portion of the bulbus cordis, which is characterized by fusion of leaflet commissures, resulting in a thickened and domed appearance of the valve. […] Although familial forms of pulmonary stenosis are described, it is generally considered to be multifactorial in origin. […] Rates of recurrence in siblings are on the order of 2-3%. […] The prevalence of pulmonary stenosis in the offspring of a parent with pulmonary stenosis is 3.6%. […] Aberrant flow patterns in utero may also be partly associated with maldevelopment of the pulmonary valve.

• #14 Clinical manifestations and diagnosis of pulmonic stenosis in adults – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-pulmonic-stenosis-in-adults

  Stenosis of the pulmonary valve is a relatively common congenital defect, occurring in approximately 7 percent of children with congenital heart disease. There is a slight female predominance, and familial occurrence has been reported in 2 percent of cases in the absence of a genetic disorder. […] Pulmonic stenosis can occur at three locations: Valvular, Subvalvular.

• #15 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560750/

  Pulmonary stenosis can occur as an isolated valvular lesion or be associated with congenital structural cardiac anomalies, including tetralogy of Fallot, tricuspid atresia, complete and corrected transposition of the great arteries, and double outlet right ventricle. […] Pulmonary stenosis can also be linked with genetic syndromes such as Noonan syndrome, which is most commonly caused by PTPN11 mutations but can also be caused by KRAS, SOS1, and RAF1 mutations. […] Peripheral pulmonary stenosis is another form that can be associated with conditions such as Alagille syndrome, caused by a JAG1 mutation on chromosome 12q24, or less frequently, NOTCH2 mutations, and Williams-Beuren syndrome, caused by an ELN mutation in chromosome 7q11.23. […] Lastly, maternal rubella syndrome is also a known cause of congenital valvular pulmonary stenosis, although it is not a genetic defect.

• #16 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10320808/

  In the classical form of PS the valve is dome shaped, characterized by a narrow central opening with preserved valve motion. There are generally two to four rudimentary raphes without a real separation into valve leaflets. Valve calcification is rare but it is seen in some elderly patients. Less commonly the valve may be uni, bi or tricuspid with various degrees of commissural fusion and thickened cusps. Approximately 10-20% of cases involve pulmonic valve dysplasia. Dysplastic valves are trileaflet with thickened cusps composed of myxomatous tissue and little or no fusions, with relative valve immobility. This type of valve defect is a common component of Noonan syndrome. PS may also occur as part of complex congenital lesions (i.e., tetralogy of Fallot, complete atrioventricular canal, double outlet RV, univentricular heart). Most patients develop dilation of the pulmonary trunk (post stenotic dilation), with a degree of dilation not always proportional to the severity of obstruction. One exception to this finding is peripheral pulmonary artery stenosis usually found in Noonans and Williams syndrome.

• #17 Pulmonary stenosis – The Cardiovascular

  https://ecgwaves.com/topic/pulmonic-valve-stenosis/

  Pulmonary stenosis is virtually always a consequence of congenital heart disease. The stenosis can be fixed or dynamic, depending on the underlying etiology. […] Valvular pulmonic stenosis can be caused by dysplastic, bicuspid or unicuspid valves. […] 60% of all individuals with Noonan syndrome have pulmonic stenosis. The stenosis is subvalvular, causing a narrowing of the RVOT (right ventricular outflow tract). […] Carcinoid syndrome is a paraneoplastic syndrome that occurs due to carcinomas secreting kallikrein and serotonin. In the heart, this can lead to thickening of the pulmonary valve and, subsequently, narrowing of the valvular orifice. […] Rheumatic heart disease […] Sinus of Valsalva aneurysm: The aneurysm may compress the pulmonary outflow. […] Myxomas may compress the pulmonary outflow. […] Aortic aneurysms may compresses the RVOT.

• #18 Pulmonary valve disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-valve-disease/symptoms-causes/syc-20350654

  Pulmonary valve stenosis. The valve is narrowed. This reduces the blood flow from the heart to the pulmonary artery and lungs. […] Usually, pulmonary valve disease is caused by a heart condition that develops before birth, called a congenital heart defect. But pulmonary valve disease may happen later in life as a complication of another illness. […] A heart condition that you’re born with, also called a congenital heart defect. Some congenital heart defects affect the shape of the pulmonary valve and how it works. […] German measles, also called rubella. Having German measles during pregnancy increases the risk of pulmonary valve stenosis in the baby. […] Noonan syndrome. This condition is caused by changes in genes. Pulmonary valve stenosis is a common heart complication seen in people with Noonan syndrome.

• #19 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560750/

  Pulmonary stenosis can occur as an isolated valvular lesion or be associated with congenital structural cardiac anomalies, including tetralogy of Fallot, tricuspid atresia, complete and corrected transposition of the great arteries, and double outlet right ventricle. […] Pulmonary stenosis can also be linked with genetic syndromes such as Noonan syndrome, which is most commonly caused by PTPN11 mutations but can also be caused by KRAS, SOS1, and RAF1 mutations. […] Peripheral pulmonary stenosis is another form that can be associated with conditions such as Alagille syndrome, caused by a JAG1 mutation on chromosome 12q24, or less frequently, NOTCH2 mutations, and Williams-Beuren syndrome, caused by an ELN mutation in chromosome 7q11.23. […] Lastly, maternal rubella syndrome is also a known cause of congenital valvular pulmonary stenosis, although it is not a genetic defect.

• #20 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560750/

  Pulmonary stenosis can occur as an isolated valvular lesion or be associated with congenital structural cardiac anomalies, including tetralogy of Fallot, tricuspid atresia, complete and corrected transposition of the great arteries, and double outlet right ventricle. […] Pulmonary stenosis can also be linked with genetic syndromes such as Noonan syndrome, which is most commonly caused by PTPN11 mutations but can also be caused by KRAS, SOS1, and RAF1 mutations. […] Peripheral pulmonary stenosis is another form that can be associated with conditions such as Alagille syndrome, caused by a JAG1 mutation on chromosome 12q24, or less frequently, NOTCH2 mutations, and Williams-Beuren syndrome, caused by an ELN mutation in chromosome 7q11.23. […] Lastly, maternal rubella syndrome is also a known cause of congenital valvular pulmonary stenosis, although it is not a genetic defect.

• #21 Pulmonary valve stenosis causes – wikidoc

  https://www.wikidoc.org/index.php/Pulmonary_valve_stenosis_causes

  Causes in Alphabetical Order include Alagille syndrome, Amyloidosis, Bicuspid pulmonic valves, Blalock-Taussig shunt, Carcinoid syndrome, Cardiac myxoma, Cardiofaciocutaneous syndrome, Carpenter syndrome, Congenital heart disease, Congenital rubella syndrome, Congenitally corrected transposition of great arteries, Costello syndrome, Double-chambered right ventricle, Ebstein’s anomaly, Eisenmenger syndrome, Endocarditis, Endomyocardial fibrosis, Infective endocarditis, Ivemark syndrome, Keutel syndrome, LEOPARD syndrome, Neonatal lupus, Noonan syndrome, Rheumatic fever, Rheumatic heart disease, Right ventricular tumors, Tetralogy of Fallot, Weill-Marchesani syndrome, Williams syndrome, Williams-Beuren syndrome, Wolf-Hirschhorn syndrome.

• #22 Pulmonary valve stenosis causes – wikidoc

  https://www.wikidoc.org/index.php/Pulmonary_valve_stenosis_causes

  Causes in Alphabetical Order include Alagille syndrome, Amyloidosis, Bicuspid pulmonic valves, Blalock-Taussig shunt, Carcinoid syndrome, Cardiac myxoma, Cardiofaciocutaneous syndrome, Carpenter syndrome, Congenital heart disease, Congenital rubella syndrome, Congenitally corrected transposition of great arteries, Costello syndrome, Double-chambered right ventricle, Ebstein’s anomaly, Eisenmenger syndrome, Endocarditis, Endomyocardial fibrosis, Infective endocarditis, Ivemark syndrome, Keutel syndrome, LEOPARD syndrome, Neonatal lupus, Noonan syndrome, Rheumatic fever, Rheumatic heart disease, Right ventricular tumors, Tetralogy of Fallot, Weill-Marchesani syndrome, Williams syndrome, Williams-Beuren syndrome, Wolf-Hirschhorn syndrome.

• #23 Pulmonary valve stenosis causes – wikidoc

  https://www.wikidoc.org/index.php/Pulmonary_valve_stenosis_causes

  Causes in Alphabetical Order include Alagille syndrome, Amyloidosis, Bicuspid pulmonic valves, Blalock-Taussig shunt, Carcinoid syndrome, Cardiac myxoma, Cardiofaciocutaneous syndrome, Carpenter syndrome, Congenital heart disease, Congenital rubella syndrome, Congenitally corrected transposition of great arteries, Costello syndrome, Double-chambered right ventricle, Ebstein’s anomaly, Eisenmenger syndrome, Endocarditis, Endomyocardial fibrosis, Infective endocarditis, Ivemark syndrome, Keutel syndrome, LEOPARD syndrome, Neonatal lupus, Noonan syndrome, Rheumatic fever, Rheumatic heart disease, Right ventricular tumors, Tetralogy of Fallot, Weill-Marchesani syndrome, Williams syndrome, Williams-Beuren syndrome, Wolf-Hirschhorn syndrome.

• #24 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560750/

  Pulmonary stenosis can occur as an isolated valvular lesion or be associated with congenital structural cardiac anomalies, including tetralogy of Fallot, tricuspid atresia, complete and corrected transposition of the great arteries, and double outlet right ventricle. […] Pulmonary stenosis can also be linked with genetic syndromes such as Noonan syndrome, which is most commonly caused by PTPN11 mutations but can also be caused by KRAS, SOS1, and RAF1 mutations. […] Peripheral pulmonary stenosis is another form that can be associated with conditions such as Alagille syndrome, caused by a JAG1 mutation on chromosome 12q24, or less frequently, NOTCH2 mutations, and Williams-Beuren syndrome, caused by an ELN mutation in chromosome 7q11.23. […] Lastly, maternal rubella syndrome is also a known cause of congenital valvular pulmonary stenosis, although it is not a genetic defect.

• #25 Pulmonary Stenosis – ACHA

  https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/pulmonary-stenosis/

  Pulmonary stenosis occurs when the pulmonary valve does not form correctly. This happens in the first 8 weeks of fetal development. We dont know exactly why. Most of the time it occurs by chance. […] Pulmonary stenosis is classified according to location and severity of the defect. There are 3 types: Valvar pulmonary stenosis is the most common. In this, the pulmonary valve is dome-shaped and the opening is narrow. The leaflets are fused together. If the leaflets are thick, it is called a dysplastic valve. Pulmonary valvar stenosis can occur alone or with other congenital heart defects. These include tetralogy of Fallot or corrected transposition of the great arteries. It may also be part of a genetic syndrome, such as Noonans syndrome. […] When the muscle under the pulmonary valve is thickened, it is called subvalvar pulmonary stenosis. This is caused by additional muscle bundles in the right ventricle. […] Supravalvar stenosis occurs when there is narrowing in the pulmonary artery above the pulmonary valve. There can be one or more than one areas of narrowing. This may be seen with genetic syndromes such as Williams or Alagille syndrome.

• #26 Pulmonary valve stenosis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/pulmonary-valve-stenosis?embed_domain=external.radpair.com&lang=us

  Pulmonary valve stenosis, or pulmonic valve stenosis, is a valvulopathy that describes the narrowing of the opening of the pulmonary valve between the pulmonary trunk and the right ventricle. […] Isolated pulmonary stenosis is almost always congenital. Causes of congenital pulmonary stenosis are protean (e.g. Noonan syndrome, Williams syndrome, tetralogy of Fallot, etc.), and can be classified as being either supravalvular (most common), valvular, or subvalvular. […] In addition to congenital pulmonary stenosis, there are other rare (5%) acquired valvular causes which can present in adulthood: carcinoid heart disease (most common acquired cause, always present with pulmonary regurgitation), rheumatic heart disease, nonbacterial thrombotic endocarditis, infective endocarditis, cardiac tumors (generally not considered 'true’ pulmonary stenosis).

• #27 Pulmonic Stenosis (Pulmonary Stenosis): Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/157737-overview

  Causes of acquired pulmonic stenosis (pulmonary stenosis) (PS) include the following: […] PS is a rare manifestation of rheumatic heart disease, and it follows involvement of the mitral and aortic valves. […] Carcinoid may result in development of myxomatous plaques in the RV outflow tract, with distortion and constriction of the pulmonic ring, as well as fusion or destruction of pulmonary valve leaflets, resulting in both stenosis and regurgitation. […] Rarely, cardiac tumors can grow on or into the RV outflow tract and cause flow obstruction. […] Sinus of Valsalva aneurysms and aortic graft aneurysms are extracardiac entities that can cause PS by external compression.

• #28 Pulmonary valve stenosis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/pulmonary-valve-stenosis?embed_domain=external.radpair.com&lang=us

  Pulmonary valve stenosis, or pulmonic valve stenosis, is a valvulopathy that describes the narrowing of the opening of the pulmonary valve between the pulmonary trunk and the right ventricle. […] Isolated pulmonary stenosis is almost always congenital. Causes of congenital pulmonary stenosis are protean (e.g. Noonan syndrome, Williams syndrome, tetralogy of Fallot, etc.), and can be classified as being either supravalvular (most common), valvular, or subvalvular. […] In addition to congenital pulmonary stenosis, there are other rare (5%) acquired valvular causes which can present in adulthood: carcinoid heart disease (most common acquired cause, always present with pulmonary regurgitation), rheumatic heart disease, nonbacterial thrombotic endocarditis, infective endocarditis, cardiac tumors (generally not considered 'true’ pulmonary stenosis).

• #29 Pulmonary stenosis – The Cardiovascular

  https://ecgwaves.com/topic/pulmonic-valve-stenosis/

  Pulmonary stenosis is virtually always a consequence of congenital heart disease. The stenosis can be fixed or dynamic, depending on the underlying etiology. […] Valvular pulmonic stenosis can be caused by dysplastic, bicuspid or unicuspid valves. […] 60% of all individuals with Noonan syndrome have pulmonic stenosis. The stenosis is subvalvular, causing a narrowing of the RVOT (right ventricular outflow tract). […] Carcinoid syndrome is a paraneoplastic syndrome that occurs due to carcinomas secreting kallikrein and serotonin. In the heart, this can lead to thickening of the pulmonary valve and, subsequently, narrowing of the valvular orifice. […] Rheumatic heart disease […] Sinus of Valsalva aneurysm: The aneurysm may compress the pulmonary outflow. […] Myxomas may compress the pulmonary outflow. […] Aortic aneurysms may compresses the RVOT.

• #30 Pulmonic Stenosis (Pulmonary Stenosis): Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/157737-overview

  Causes of acquired pulmonic stenosis (pulmonary stenosis) (PS) include the following: […] PS is a rare manifestation of rheumatic heart disease, and it follows involvement of the mitral and aortic valves. […] Carcinoid may result in development of myxomatous plaques in the RV outflow tract, with distortion and constriction of the pulmonic ring, as well as fusion or destruction of pulmonary valve leaflets, resulting in both stenosis and regurgitation. […] Rarely, cardiac tumors can grow on or into the RV outflow tract and cause flow obstruction. […] Sinus of Valsalva aneurysms and aortic graft aneurysms are extracardiac entities that can cause PS by external compression.

• #31 Pulmonary Valve Stenosis: Causes, Symptoms, and Diagnosis

  https://www.healthline.com/health/pulmonary-valve-stenosis

  Pulmonary valve stenosis is when the pulmonary valve does not open properly or wide enough. […] Doctors dont know the exact cause of pulmonary valve stenosis. The pulmonary valve in a fetus may fail to develop properly during pregnancy. The disease may also have a genetic component. […] Adults can also experience the condition as a complication of an illness that affects the heart. Conditions that can have pulmonary valve stenosis as a complication include rheumatic fever and carcinoid tumors in the digestive system.

• #32 Pulmonary valve stenosis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/pulmonary-valve-stenosis?embed_domain=external.radpair.com&lang=us

  Pulmonary valve stenosis, or pulmonic valve stenosis, is a valvulopathy that describes the narrowing of the opening of the pulmonary valve between the pulmonary trunk and the right ventricle. […] Isolated pulmonary stenosis is almost always congenital. Causes of congenital pulmonary stenosis are protean (e.g. Noonan syndrome, Williams syndrome, tetralogy of Fallot, etc.), and can be classified as being either supravalvular (most common), valvular, or subvalvular. […] In addition to congenital pulmonary stenosis, there are other rare (5%) acquired valvular causes which can present in adulthood: carcinoid heart disease (most common acquired cause, always present with pulmonary regurgitation), rheumatic heart disease, nonbacterial thrombotic endocarditis, infective endocarditis, cardiac tumors (generally not considered 'true’ pulmonary stenosis).

• #33 Pulmonary valve stenosis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/pulmonary-valve-stenosis?embed_domain=external.radpair.com&lang=us

  Pulmonary valve stenosis, or pulmonic valve stenosis, is a valvulopathy that describes the narrowing of the opening of the pulmonary valve between the pulmonary trunk and the right ventricle. […] Isolated pulmonary stenosis is almost always congenital. Causes of congenital pulmonary stenosis are protean (e.g. Noonan syndrome, Williams syndrome, tetralogy of Fallot, etc.), and can be classified as being either supravalvular (most common), valvular, or subvalvular. […] In addition to congenital pulmonary stenosis, there are other rare (5%) acquired valvular causes which can present in adulthood: carcinoid heart disease (most common acquired cause, always present with pulmonary regurgitation), rheumatic heart disease, nonbacterial thrombotic endocarditis, infective endocarditis, cardiac tumors (generally not considered 'true’ pulmonary stenosis).

• #34 Pulmonic Stenosis (Pulmonary Stenosis): Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/157737-overview

  Causes of acquired pulmonic stenosis (pulmonary stenosis) (PS) include the following: […] PS is a rare manifestation of rheumatic heart disease, and it follows involvement of the mitral and aortic valves. […] Carcinoid may result in development of myxomatous plaques in the RV outflow tract, with distortion and constriction of the pulmonic ring, as well as fusion or destruction of pulmonary valve leaflets, resulting in both stenosis and regurgitation. […] Rarely, cardiac tumors can grow on or into the RV outflow tract and cause flow obstruction. […] Sinus of Valsalva aneurysms and aortic graft aneurysms are extracardiac entities that can cause PS by external compression.

• #35 Pulmonic Stenosis (Pulmonary Stenosis): Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/157737-overview

  Causes of acquired pulmonic stenosis (pulmonary stenosis) (PS) include the following: […] PS is a rare manifestation of rheumatic heart disease, and it follows involvement of the mitral and aortic valves. […] Carcinoid may result in development of myxomatous plaques in the RV outflow tract, with distortion and constriction of the pulmonic ring, as well as fusion or destruction of pulmonary valve leaflets, resulting in both stenosis and regurgitation. […] Rarely, cardiac tumors can grow on or into the RV outflow tract and cause flow obstruction. […] Sinus of Valsalva aneurysms and aortic graft aneurysms are extracardiac entities that can cause PS by external compression.

• #36 Pulmonary stenosis – The Cardiovascular

  https://ecgwaves.com/topic/pulmonic-valve-stenosis/

  Pulmonary stenosis is virtually always a consequence of congenital heart disease. The stenosis can be fixed or dynamic, depending on the underlying etiology. […] Valvular pulmonic stenosis can be caused by dysplastic, bicuspid or unicuspid valves. […] 60% of all individuals with Noonan syndrome have pulmonic stenosis. The stenosis is subvalvular, causing a narrowing of the RVOT (right ventricular outflow tract). […] Carcinoid syndrome is a paraneoplastic syndrome that occurs due to carcinomas secreting kallikrein and serotonin. In the heart, this can lead to thickening of the pulmonary valve and, subsequently, narrowing of the valvular orifice. […] Rheumatic heart disease […] Sinus of Valsalva aneurysm: The aneurysm may compress the pulmonary outflow. […] Myxomas may compress the pulmonary outflow. […] Aortic aneurysms may compresses the RVOT.

• #37 Pulmonic Stenosis (Pulmonary Stenosis): Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/157737-overview

  Causes of acquired pulmonic stenosis (pulmonary stenosis) (PS) include the following: […] PS is a rare manifestation of rheumatic heart disease, and it follows involvement of the mitral and aortic valves. […] Carcinoid may result in development of myxomatous plaques in the RV outflow tract, with distortion and constriction of the pulmonic ring, as well as fusion or destruction of pulmonary valve leaflets, resulting in both stenosis and regurgitation. […] Rarely, cardiac tumors can grow on or into the RV outflow tract and cause flow obstruction. […] Sinus of Valsalva aneurysms and aortic graft aneurysms are extracardiac entities that can cause PS by external compression.

• #38 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10320808/

  Only a small percentage of PS is acquired and caused by rheumatic disease, carcinoid disease and neoplastic lesions or may occur after surgical reconstruction for other complex congenital cardiac disorders: reconstruction often entails the placement of a pulmonary valve prosthesis, right ventricle-to-pulmonary artery homograft, or valved conduit, which degenerates over time, manifesting as stenosis, regurgitation, or both. […] The natural history of PS leads to secondary changes in other cardiac structures. In particular, the right ventricular systolic pressures needed to override the outlet stenosis can be even higher than systemic left ventricular pressures. Pressure overload increases wall stress and, in order to maintain a normal cardiac output, there is an increase in contractility and a compensatory right ventricular hypertrophy, an increase of end-systolic volume and end-diastolic volume and high right ventricular end-diastolic pressures. At first, these compensatory adaptations enable the right ventricle (RV) to maintain stroke volume in presence of increased afterload. Over time, progressive right ventricular hypertrophy and stiffness can determine right ventricular diastolic and systolic dysfunction and this leads to fibrosis of the endocardium and of the tricuspid valve apparatus, right ventricular ischemia, hypertension and hypertrophy of the right atrium and even arrhythmias. Therefore, elevated levels of NT-proBNP in neonates might be used as a biomarker for diagnosis of severe PS.

• #39 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10320808/

  Only a small percentage of PS is acquired and caused by rheumatic disease, carcinoid disease and neoplastic lesions or may occur after surgical reconstruction for other complex congenital cardiac disorders: reconstruction often entails the placement of a pulmonary valve prosthesis, right ventricle-to-pulmonary artery homograft, or valved conduit, which degenerates over time, manifesting as stenosis, regurgitation, or both. […] The natural history of PS leads to secondary changes in other cardiac structures. In particular, the right ventricular systolic pressures needed to override the outlet stenosis can be even higher than systemic left ventricular pressures. Pressure overload increases wall stress and, in order to maintain a normal cardiac output, there is an increase in contractility and a compensatory right ventricular hypertrophy, an increase of end-systolic volume and end-diastolic volume and high right ventricular end-diastolic pressures. At first, these compensatory adaptations enable the right ventricle (RV) to maintain stroke volume in presence of increased afterload. Over time, progressive right ventricular hypertrophy and stiffness can determine right ventricular diastolic and systolic dysfunction and this leads to fibrosis of the endocardium and of the tricuspid valve apparatus, right ventricular ischemia, hypertension and hypertrophy of the right atrium and even arrhythmias. Therefore, elevated levels of NT-proBNP in neonates might be used as a biomarker for diagnosis of severe PS.

• #40 Pulmonary Valve Stenosis   | American Heart Association

  https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/pulmonary-valve-stenosis

  Estenosis pulmonar […] In most children, the cause isn’t known. It’s a common type of heart defect. […] In most cases, the cause isn’t known. It’s a common type of heart defect. Babies born to mothers who had rubella (German measles) during pregnancy were more likely to develop pulmonary stenosis along with deafness and patent ductus arteriosus. Some patients can have other heart defects along with PS.

• #41 Pulmonary valve disease – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-valve-disease/symptoms-causes/syc-20350654

  Pulmonary valve stenosis. The valve is narrowed. This reduces the blood flow from the heart to the pulmonary artery and lungs. […] Usually, pulmonary valve disease is caused by a heart condition that develops before birth, called a congenital heart defect. But pulmonary valve disease may happen later in life as a complication of another illness. […] A heart condition that you’re born with, also called a congenital heart defect. Some congenital heart defects affect the shape of the pulmonary valve and how it works. […] German measles, also called rubella. Having German measles during pregnancy increases the risk of pulmonary valve stenosis in the baby. […] Noonan syndrome. This condition is caused by changes in genes. Pulmonary valve stenosis is a common heart complication seen in people with Noonan syndrome.

• #42 Pulmonary Stenosis | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/pulmonary-stenosis

  When the pulmonary valve is too narrow, the heart must work harder to pump enough blood through the valve and to the body. […] Pulmonary stenosis can run in families, so be sure to tell your cardiologist if any other close family members have a heart murmur. […] In rare cases, surgery is needed to treat pulmonary stenosis. Surgeons will cut open or cut out the valve. […] Subpulmonic and supravalvular pulmonic stenosis do not get better with cardiac catheterization and may require surgery. Surgery for subpulmonic stenosis involves cutting out the extra muscles below the valve. Surgery for supravalvular pulmonic stenosis involves using a patch to make the pulmonary artery bigger.

• #43 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560750/

  Pulmonary stenosis can occur as an isolated valvular lesion or be associated with congenital structural cardiac anomalies, including tetralogy of Fallot, tricuspid atresia, complete and corrected transposition of the great arteries, and double outlet right ventricle. […] Pulmonary stenosis can also be linked with genetic syndromes such as Noonan syndrome, which is most commonly caused by PTPN11 mutations but can also be caused by KRAS, SOS1, and RAF1 mutations. […] Peripheral pulmonary stenosis is another form that can be associated with conditions such as Alagille syndrome, caused by a JAG1 mutation on chromosome 12q24, or less frequently, NOTCH2 mutations, and Williams-Beuren syndrome, caused by an ELN mutation in chromosome 7q11.23. […] Lastly, maternal rubella syndrome is also a known cause of congenital valvular pulmonary stenosis, although it is not a genetic defect.

• #44 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10320808/

  Only a small percentage of PS is acquired and caused by rheumatic disease, carcinoid disease and neoplastic lesions or may occur after surgical reconstruction for other complex congenital cardiac disorders: reconstruction often entails the placement of a pulmonary valve prosthesis, right ventricle-to-pulmonary artery homograft, or valved conduit, which degenerates over time, manifesting as stenosis, regurgitation, or both. […] The natural history of PS leads to secondary changes in other cardiac structures. In particular, the right ventricular systolic pressures needed to override the outlet stenosis can be even higher than systemic left ventricular pressures. Pressure overload increases wall stress and, in order to maintain a normal cardiac output, there is an increase in contractility and a compensatory right ventricular hypertrophy, an increase of end-systolic volume and end-diastolic volume and high right ventricular end-diastolic pressures. At first, these compensatory adaptations enable the right ventricle (RV) to maintain stroke volume in presence of increased afterload. Over time, progressive right ventricular hypertrophy and stiffness can determine right ventricular diastolic and systolic dysfunction and this leads to fibrosis of the endocardium and of the tricuspid valve apparatus, right ventricular ischemia, hypertension and hypertrophy of the right atrium and even arrhythmias. Therefore, elevated levels of NT-proBNP in neonates might be used as a biomarker for diagnosis of severe PS.

• #45 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10320808/

  Only a small percentage of PS is acquired and caused by rheumatic disease, carcinoid disease and neoplastic lesions or may occur after surgical reconstruction for other complex congenital cardiac disorders: reconstruction often entails the placement of a pulmonary valve prosthesis, right ventricle-to-pulmonary artery homograft, or valved conduit, which degenerates over time, manifesting as stenosis, regurgitation, or both. […] The natural history of PS leads to secondary changes in other cardiac structures. In particular, the right ventricular systolic pressures needed to override the outlet stenosis can be even higher than systemic left ventricular pressures. Pressure overload increases wall stress and, in order to maintain a normal cardiac output, there is an increase in contractility and a compensatory right ventricular hypertrophy, an increase of end-systolic volume and end-diastolic volume and high right ventricular end-diastolic pressures. At first, these compensatory adaptations enable the right ventricle (RV) to maintain stroke volume in presence of increased afterload. Over time, progressive right ventricular hypertrophy and stiffness can determine right ventricular diastolic and systolic dysfunction and this leads to fibrosis of the endocardium and of the tricuspid valve apparatus, right ventricular ischemia, hypertension and hypertrophy of the right atrium and even arrhythmias. Therefore, elevated levels of NT-proBNP in neonates might be used as a biomarker for diagnosis of severe PS.

• #46 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10320808/

  In the classical form of PS the valve is dome shaped, characterized by a narrow central opening with preserved valve motion. There are generally two to four rudimentary raphes without a real separation into valve leaflets. Valve calcification is rare but it is seen in some elderly patients. Less commonly the valve may be uni, bi or tricuspid with various degrees of commissural fusion and thickened cusps. Approximately 10-20% of cases involve pulmonic valve dysplasia. Dysplastic valves are trileaflet with thickened cusps composed of myxomatous tissue and little or no fusions, with relative valve immobility. This type of valve defect is a common component of Noonan syndrome. PS may also occur as part of complex congenital lesions (i.e., tetralogy of Fallot, complete atrioventricular canal, double outlet RV, univentricular heart). Most patients develop dilation of the pulmonary trunk (post stenotic dilation), with a degree of dilation not always proportional to the severity of obstruction. One exception to this finding is peripheral pulmonary artery stenosis usually found in Noonans and Williams syndrome.

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