Materiały źródłowe

• #1 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

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

  Pulmonary or pulmonic stenosis is a common congenital heart defect that involves mild, moderate, or severe narrowing of the right ventricular outflow tract, pulmonary valve, or pulmonary arteries, which restricts blood flow from the heart to the lungs. […] Although pulmonary stenosis typically refers to flow obstruction at the pulmonary valve level, it can also occur at the subvalvular or supravalvular level, including the main pulmonary artery and its branches. […] The severity of flow restriction across the pulmonary valve and the valve anatomy determine the course of treatment, including balloon valvuloplasty or surgery. […] 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.

• #2 Pulmonary valve stenosis – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-valve-stenosis/symptoms-causes/syc-20377034

  Pulmonary valve stenosis is a narrowing of the valve between the lower right heart chamber and the lung arteries. In a narrowed heart valve, the valve flaps may become thick or stiff. This reduces blood flow through the valve. […] In pulmonary valve stenosis, one or more of the cusps may be stiff or thick. Sometimes the cusps may be joined together. That means they are fused. So the valve doesn’t open fully. The smaller opening makes it harder for blood to leave the lower right heart chamber. Pressure increases inside the chamber. The increased pressure strains the heart. Eventually the lower right heart chamber wall gets thicker.

• #3 Pulmonary Stenosis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28064

  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. […] Valvular pulmonary stenosis: Isolated pulmonary valve stenosis is a condition in which the pulmonary valve is affected by varying degrees of fibrosis, thickening, and commissural fusion, leading to restricted blood flow. This condition is typically congenital and is diagnosed in pediatric patients. However, in rare cases, it can occur in adults as a result of rheumatic heart disease or carcinoid syndrome. […] Valvular pulmonary stenosis is the most common type. In this condition, the commissures of the valve are partially fused, and the leaflets are thin. This structural anomaly causes a dome-shaped or conical outlet during systole. In some cases, the pulmonary valve may be dysplastic, thickened, and without fusion.

• #4 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

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

  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. […] Pulmonary stenosis is classified into 3 types based on the location of the obstruction: valvular, subvalvular, and supravalvular. […] Valvular pulmonary stenosis is a condition in which the pulmonary valve is affected by varying degrees of fibrosis, thickening, and commissural fusion, leading to restricted blood flow. […] Subvalvular pulmonary stenosis is a cardiac defect that causes obstruction below the pulmonary valve in the infundibular region of the right ventricle. […] Supravalvular pulmonary stenosis, also known as peripheral pulmonary stenosis, is a condition where there is an obstruction above the pulmonary valve.

• #5 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

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

  Pulmonary stenosis is classified into 3 types based on the location of the obstructionvalvular, subvalvular, and supravalvular. The degree of obstruction can be mild, moderate, severe, or critical. […] Isolated pulmonary valve stenosis is a condition in which the pulmonary valve is affected by varying degrees of fibrosis, thickening, and commissural fusion, leading to restricted blood flow. This condition is typically congenital and is diagnosed in pediatric patients. However, in rare cases, it can occur in adults as a result of rheumatic heart disease or carcinoid syndrome. […] Valvular pulmonary stenosis is the most common type. In this condition, the commissures of the valve are partially fused, and the leaflets are thin. This structural anomaly causes a dome-shaped or conical outlet during systole. In some cases, the pulmonary valve may be dysplastic, thickened, and without fusion.

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

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

  Pathologic features of the stenotic pulmonary valve vary. The most common pathology is a dome-shaped pulmonary valve. The fused leaflets of the pulmonary valve protrude from their attachment into the pulmonary artery as a conical, windsock-like structure. The size of the pulmonary valve orifice varies from a pinhole to several millimeters. The orifice is most usually central but can be eccentric. Raphae, presumably fused commissures of the valve, extend from the stenotic orifice to a variable distance down into the base of the dome-shaped valve. The number of the raphe may vary from 0-7. Relatively uncommon variants are unicommissural, bicuspid, and tricuspid valves. The valve annulus is abnormal in most cases, and the fibrous back bone is partially or completely lacking; therefore, a true annulus may not be present.

• #7 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

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

  Subvalvular pulmonary stenosis is a cardiac defect that causes obstruction below the pulmonary valve in the infundibular region of the right ventricle. This condition occurs in patients with a double-chambered right ventricle and those with tetralogy of Fallot. […] Supravalvular pulmonary stenosis, also known as peripheral pulmonary stenosis, is a condition where there is an obstruction above the pulmonary valve. The restriction can occur in the main pulmonary artery, the distal branches of the pulmonary artery, or both places. […] The severity of pulmonary stenosis can be determined using echocardiographic or transcatheter criteria. Stenosis can be classified as mild, moderate, severe, or critical.

• #8 Pulmonary Stenosis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28064

  Subvalvular pulmonary stenosis: Subvalvular pulmonary stenosis is a cardiac defect that causes obstruction below the pulmonary valve in the infundibular region of the right ventricle. This condition occurs in patients with a double-chambered right ventricle and those with tetralogy of Fallot. […] Supravalvular pulmonary stenosis, also known as peripheral pulmonary stenosis, is a condition where there is an obstruction above the pulmonary valve. The restriction can occur in the main pulmonary artery, the distal branches of the pulmonary artery, or both places. Supravalvular pulmonary stenosis can be associated with structural defects such as tetralogy of Fallot, double-outlet right ventricle, transposition of the great arteries, and genetic syndromes, including Noonan, William, and Alagille syndromes.

• #9 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniq | VHRM

  https://www.dovepress.com/pulmonary-valve-stenosis-from-diagnosis-to-current-management-techniqu-peer-reviewed-fulltext-article-VHRM

  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.

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

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

  Hypoplasia of the pulmonary valve ring and dysplastic pulmonary valves may be present in a few of patients. Pulmonary valve dysplasia is characterized by thickened, nodular, and redundant valvular leaflets with minimal or no commissural fusion; hypoplasia of the valve ring; and lack of poststenotic dilatation of the pulmonary artery. The obstruction is mainly related to thickened, myxomatous, immobile pulmonary valve cusps and hypoplasia of the valve ring. […] Changes secondary to pulmonary valve obstruction occur in the right ventricle and pulmonary artery. Hypertrophy of the right ventricular muscle is proportional to the degree (and perhaps the duration) of obstruction. The muscle hypertrophy is particularly prominent in the infundibular region and may become physiologically important; this appears to be related to the degree and duration of obstruction. Mild dilatation of the right ventricular cavity is present. In extremely severe or critical obstruction, the right ventricular cavity may be markedly dilated. In rare cases, the right ventricle may be hypoplastic.

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

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

  The main pulmonary artery is dilated in almost all cases. This dilatation is independent of the severity of the pulmonary valve obstruction and presumably related to a high-velocity jet across the stenotic valve. As noted above, such poststenotic dilatation is remarkably absent in patients with dysplastic pulmonary valves. […] Clinically significant narrowing of a valve or a blood vessel increases pressure proximal to the obstruction. This pressure gradient is necessary to maintain flow across the stenotic site. In pulmonic stenosis, hypertrophy of the right ventricle ensues and maintains this forward flow. The magnitude of right ventricular pressure and the pressure gradient across the pulmonary valve are generally proportional to the degree of obstruction. Under usual circumstances, proportional right ventricular hypertrophy maintains normal pulmonary blood flow. If the normal output is not maintained, right-sided heart failure ensues. This occurs in neonates with critical pulmonary stenosis and in patients with severe obstruction that occurs in childhood or adulthood.

• #12 Pulmonary Valve Stenosis   | American Heart Association

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

  The pulmonary valve opens to let blood flow from the right ventricle to the lungs. Narrowing of the pulmonary valve (valvar pulmonary stenosis) causes the right ventricle to pump harder to get blood past the blockage. […] Normally the right side of the heart pumps blood to the lungs. In a child with PS, the pressure is much higher than normal in the right pumping chamber (right ventricle) and the heart must work harder to pump blood out into the lung arteries. Over time this can cause damage to the overworked heart muscle. […] In most cases, the cause isn’t known. It’s a common type of heart defect. […] Normally the right side of the heart pumps blood to the lungs. In a person with PS, the pressure in the right-heart pumping chamber (right ventricle) is much higher than normal and the heart must work harder to pump blood out into the lung arteries. Over time this can cause damage to the overworked heart muscle. When the valve is regurgitant it can cause the right ventricle to enlarge.

• #13 Pulmonary valve stenosis in the adult patient: pathophysiology, diagnosis and management | Heart

  https://heart.bmj.com/content/105/5/414

  The most common form of right ventricular outflow tract (RVOT) obstruction is pulmonary valve stenosis. […] The primary consequence of PS is pressure overload of the right ventricle, the degree of which is dependent on the severity of the stenosis. […] Over time, progressive right ventricular hypertrophy and stiffness can give rise to right ventricular diastolic and systolic dysfunction.

• #14 Pulmonary valve stenosis in the adult patient: pathophysiology, diagnosis and management | Heart

  https://heart.bmj.com/content/105/5/414.abstract?int_source=trendmd&int_medium=cpc&int_campaign=usage-042019

  The most common form of right ventricular outflow tract (RVOT) obstruction is pulmonary valve stenosis. […] PS can also be an acquired condition such as rheumatic heart disease, carcinoid, infective endocarditis or result from trauma. […] The primary consequence of PS is pressure overload of the right ventricle, the degree of which is dependent on the severity of the stenosis. […] Over time, progressive right ventricular hypertrophy and stiffness can give rise to right ventricular diastolic and systolic dysfunction.

• #15 Pulmonary valve stenosis – Wikipedia

  https://en.wikipedia.org/wiki/Pulmonary_valve_stenosis

  At first, a diastolic dysfunction of the right ventricle occurs, thus right ventricle being able to maintain the blood flow despite the rising afterload. However, advanced changes may lead to also systolic dysfunction and a heart failure development. […] The left ventricle can be changed physically, these changes are a direct result of right ventricular hypertrophy. Once the obstruction is subdued, it (the left ventricle) can return to normal.

• #16 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniq | VHRM

  https://www.dovepress.com/pulmonary-valve-stenosis-from-diagnosis-to-current-management-techniqu-peer-reviewed-fulltext-article-VHRM

  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.

• #17 Pulmonic stenosis | Deranged Physiology

  https://derangedphysiology.com/main/required-reading/cardiovascular-intensive-care/Chapter-36/pulmonic-stenosis

  The pulmonic valve is not one usually prone to isolated stenosis, and when it is found it is usually a congenital thing. […] A gradient of 50mmHg is considered mild, between 50 and 100 mmHg is considered moderate, and over 100mmHg is considered severe pulmonic stenosis. […] As with aortic stenosis, a concentric hypertrophy of the prevalve ventricle occurs. The RV becomes hypertrophic and diastolic RV failure develops. Subendocardial blood supply to the RV becomes compromised and it becomes more prone to ischaemia.

• #18 Pulmonary Valve Stenosis | Causes, Diagnosis, Treatment | Children’s Wisconsin

  https://childrenswi.org/medical-care/herma-heart/conditions/pulmonary-stenosis

  Pulmonary valve stenosis is a congenital (present at birth) defect that occurs due to abnormal development of the fetal heart during the first 8 weeks of pregnancy. […] With pulmonary valve stenosis, problems with the pulmonary valve make it harder for the leaflets to open and permit blood to flow forward from the right ventricle to the lungs. […] Congenital pulmonary valve stenosis occurs due to improper development of the pulmonary valve in the first 8 weeks of fetal growth. […] Mild pulmonary valve stenosis may not cause any symptoms. Problems can occur when pulmonary valve stenosis is moderate to severe, including the following: The right ventricle has to work harder to try to move blood through the tight pulmonary valve. Eventually, the right ventricle is no longer able to handle the extra workload, and it fails to pump forward efficiently.

• #19 Pulmonary stenosis | PPT

  https://www.slideshare.net/slideshow/pulmonary-stenosis-230185677/230185677

  Pulmonary stenosis (also called pulmonic stenosis) is when the pulmonary valve (the valve between the right ventricle and the pulmonary artery) is too small, narrow, or stiff. Symptoms of pulmonary stenosis depend on how small the narrowing of the pulmonary valve is […] Pulmonary Stenosis Rare, usually congenital in origin Common as PDA, flow of blood to the pulmonary artery due to narrowing blood flows back to right ventricle and right atrium right ventricle hypertrophy to compensate for blood volume and force blood to the pulmonary artery […] Pathophysiology The right ventricle – work harder It fails to pump forward efficiently. Pressure builds up in the right atrium Fluid retention and swelling may occur. There is a higher than average chance of developing an infection in the lining of the heart known as bacterial endocarditis.

• #20 Pulmonary Stenosis in Children | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/p/pulmonary-stenosis-in-children.html

  Pulmonary stenosis is a birth defect of the heart (congenital). It can happen when the pulmonary valve doesnt grow as it should in a baby during the first 8 weeks of pregnancy. […] With pulmonary stenosis, it is harder for the flaps to open and the blood to flow as it should. The flaps may be stuck together. Or the flaps may be thick and not able to open all the way. In some cases, the valve may be narrowed. In some cases, the stenosis may not be related to a problem with the valve leaflets but with the area directly below and above the valve. […] Pulmonary stenosis occurs when the pulmonary valve doesnt grow as it should or the area below or above the valve doesn’t grow fully in a baby during the first 8 weeks of pregnancy. Why this happens isn’t known. […] If not treated, moderate to severe pulmonary stenosis can cause complications. The right ventricle has to work harder to try to move blood through the pulmonary valve or narrowed area. In time, the right ventricle becomes enlarged and is no longer able to handle the extra work. It fails to pump well (heart failure). Another complication can be an abnormal heart rhythm (arrhythmia).

• #21 Pulmonary Stenosis – Conditions and Treatments | Children’s National Hospital

  https://www.childrensnational.org/get-care/health-library/pulmonary-stenosis

  Pulmonary stenosis is a birth defect of the heart that makes it hard for the blood to flow from the right ventricle to the lungs. […] Pulmonary stenosis occurs when the pulmonary valve doesnt grow as it should or the area below or above the valve doesn’t grow fully in a baby during the first 8 weeks of pregnancy. Why this happens isn’t known. […] With pulmonary stenosis, it is harder for the flaps to open and the blood to flow as it should. The flaps may be stuck together. Or the flaps may be thick and not able to open all the way. In some cases, the valve may be narrowed. In some cases, the stenosis may not be related to a problem with the valve leaflets but with the area directly below and above the valve. […] If not treated, moderate to severe pulmonary stenosis can cause complications. The right ventricle has to work harder to try to move blood through the pulmonary valve or narrowed area. In time, the right ventricle becomes enlarged and is no longer able to handle the extra work. It fails to pump well (heart failure). Another complication can be an abnormal heart rhythm (arrhythmia).

• #22 Pulmonary Stenosis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28064

  The severity of pulmonary stenosis can be determined using echocardiographic or transcatheter criteria. Stenosis can be classified as mild, moderate, severe, or critical as follows: […] Critical pulmonary stenosis may have near-pulmonary atresia, a ductal-dependent congenital heart defect in which survival depends on maintaining a patent ductus arteriosus to allow adequate pulmonary blood flow.

• #23 Pulmonary Stenosis | Lurie Children’s

  https://www.luriechildrens.org/en/specialties-conditions/pulmonary-stenosis/

  The pulmonary or pulmonic valve is the valve between the right ventricle and the pulmonary artery. It opens to allow blood to flow from the right ventricle to the lungs. A defective pulmonary valve that doesn’t open properly is called stenotic. That means the right ventricle must pump harder than normal and reach a higher than normal pressure to overcome the obstruction. […] If the stenosis is very severe, especially in babies, not enough blood crosses the valve to reach the lungs to become oxygenated. This is called critical pulmonary valve stenosis. Therefore cyanosis (blueness) may occur as the blood finds other pathways to get from the veins returning to the heart back to the body (right to left shunt). […] Sometimes the valve does not open at all, in which case it is called pulmonary atresia. In those cases, the tricuspid valve may be underdeveloped and the right ventricle may also not be well formed and is small or hypoplastic.

• #24 Pulmonary Stenosis | Lurie Children’s

  https://www.luriechildrens.org/en/specialties-conditions/pulmonary-stenosis/

  With very severe pulmonary stenosis or pulmonary atresia, which is noted in newborns, a medicine is sometimes given to stabilize the infant and improve oxygenation. This medication is called prostaglandin and helps to open the ductus arteriosus and provide more blood flow to the lungs. […] Untreated significant pulmonary stenosis can lead to abnormal right ventricular function, rhythm abnormalities and a shortened lifespan.

• #25 Pulsenotes | Pulmonary stenosis

  https://app.pulsenotes.com/medicine/cardiology/notes/pulmonary-stenosis

  This results in reduced blood flow into the pulmonary artery. […] The right ventricle has to work harder to pump blood through the stenotic pulmonary valve. […] This increased end-diastolic volume (and therefore increased right ventricular end-diastolic pressure) resists ventricular filling from the right atrium during diastole. […] The presentation of pulmonary stenosis in the neonatal period is mainly explained by the right-to-left shunt through the foramen ovale. […] The key part of the acute management in severe and critical pulmonary stenosis is starting prostaglandins. […] In severe and critical PS, the ductus arteriosus is the only way for blood to be pumped around the pulmonary circulation. […] The exact type of surgery depends on the morphology of each patient’s pulmonary stenosis. […] The long-term prognosis for patients who undergo both balloon pulmonary valvuloplasty and surgical valvotomy is excellent.

• #26 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%. […] Pulmonary stenosis represents 8-12% of all congenital heart defects in children. In adults, pulmonary stenosis represents approximately 15% of all congenital heart defects. Isolated valvar pulmonary stenosis with an intact ventricular septum is the second most common congenital cardiac defect in children. It may occur in as many as 50% of all patients with congenital heart disease associated with other congenital cardiac lesions.

• #27

  https://continentalhospitals.com/diseases/pulmonary-valve-stenosis/

  Rheumatic fever, a complication of untreated strep throat, can cause damage to the heart valves, including the pulmonary valve, leading to stenosis. […] This rare syndrome, associated with carcinoid tumors that secrete serotonin, can affect heart valves and lead to fibrosis and stenosis. […] A genetic disorder that can cause multiple congenital anomalies, including pulmonary valve stenosis. […] Conditions such as Williams syndrome and Alagille syndrome, which can include a range of heart defects including pulmonary valve stenosis. […] Previous heart surgeries or procedures involving the pulmonary valve can lead to scarring and subsequent stenosis. […] An infection of the inner lining of the heart, including the heart valves, which can cause damage and stenosis over time.

• #28 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniq | VHRM

  https://www.dovepress.com/pulmonary-valve-stenosis-from-diagnosis-to-current-management-techniqu-peer-reviewed-fulltext-article-VHRM

  For the diagnosis of PS and planning of treatment it is of paramount importance the application of multimodality imaging, with the integrated use of echocardiography, cardiac magnetic resonance (CMR) and of cardiac computer tomography (CCT). […] In most cases, echocardiography remains the first-line imaging method for PS, however CMR allows the reconstruction of unlimited image planes and views of PV and RVOT, even when cardiac anatomy or heart position in the chest are challenging. In fact, the optimal visualization of the RVOT with CMR allows identification of site and severity of PS. Compared to the evaluation of the left valves, the pulmonary valve requires specific image views for the RVOT, which can be used to provide a qualitative assessment of severity by observing the valve motion and stenotic jet. Quantitative evaluation of valve dysfunction can be assessed by the combination of high image quality and phase contrast flow mapping sequences. CMR guarantees direct planimetry of the valve orifice from an image acquired through the valve tips at peak opening during systole. It may provide ideal assessment of valve anatomy and quantifies the degree of stenosis.

• #29 Pulmonary Stenosis – StatPearls – NCBI Bookshelf

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

  The severity of pulmonary stenosis can be determined using echocardiographic or transcatheter criteria. […] The management of pulmonary stenosis is based on the severity of flow restriction across the pulmonary valve and the valve anatomy. […] Balloon valvuloplasty is recommended for asymptomatic patients with a domed pulmonary valve with a peak Doppler gradient 60 mm Hg. […] Surgical intervention is indicated in patients with moderate-to-severe valvular stenosis in symptomatic patients who are ineligible for or in whom balloon valvuloplasty was ineffective. […] Newborns with critical pulmonary stenosis may have near-pulmonary atresia, a ductal-dependent congenital heart defect in which survival depends on maintaining a patent ductus arteriosus to allow adequate pulmonary blood flow.

• #30 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniq | VHRM

  https://www.dovepress.com/pulmonary-valve-stenosis-from-diagnosis-to-current-management-techniqu-peer-reviewed-fulltext-article-VHRM

  According to the American Heart Association/American College of Cardiology (AHA/ACC) guidelines, in case of domed pulmonic valve with moderate or severe valvular stenosis and less than moderate pulmonic valve regurgitation, balloon valvotomy is recommended. Otherwise, surgical repair is recommended in case of symptomatic patients with moderate or severe valvular pulmonic stenosis who are ineligible for balloon valvotomy or who have failed it. This includes patients with severe PS and an associated hypoplastic pulmonary annulus, severe pulmonary regurgitation (PR), sub-valvular PS or supravalvular pulmonic stenosis. […] Balloon pulmonary valvuloplasty represents the first line of treatment for PS and should be performed as soon as the diagnosis is made, without waiting for the development of symptoms or the patients to reach a certain size. It has been described for the first time in 1982 by Kan et al, who used the approach of Gruntzing to alleviate PS using the radial forces of balloon inflation of a balloon catheter positioned across the pulmonary valve. BPV is usually indicated when peak-to-peak gradient is more than 50 mmHg with normal cardiac index. It is currently the preferred therapeutic strategy for doming valvular PS.

• #31 Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniq | VHRM

  https://www.dovepress.com/pulmonary-valve-stenosis-from-diagnosis-to-current-management-techniqu-peer-reviewed-fulltext-article-VHRM

  Surgical relief of PS was one of the first congenital heart operation and was described independently by Sellors and Brock in 1948. This first surgical procedure consists in a closed transventricular pulmonary valvotomy via the RV outflow tract. The Brock technique dilates and cuts the stenotic pulmonary valve and likely the pulmonary infundibulum using a valvulotome and dilators through a purse string in the RV below the native valves. […] A wide spectrum of CHDs requires pulmonary valve replacement with or without reconstruction of the right ventricular outflow tract. Pulmonary valve replacement is also the main reintervention in patients with PS who have previously undergone valvotomy with subsequent PR and/or residual PS. Pulmonary valve replacement, if appropriately implemented, effectively alleviates right ventricular dilatation and dysfunction and resolves the related symptoms.

• #32 Pulmonary Valve Stenosis: symptoms, causes and diagnosis

  https://www.childheartspecialist.com/london/pulmonary-valve-stenosis/

  In children with mild pulmonary valve stenosis, it is common occurrence that the stenosis might improve over time. However, children with even mild pulmonary stenosis require lifelong follow-up as the pulmonary valve may become stiffer and therefore work less sometimes later on in adult life. […] Children with severe pulmonary stenosis require treatment of the stenosis as this will ultimately affect the working conditions of the right ventricle, at which point the treatment might become more risky. […] Even though the procedure is very effective at relieving the stenosis it does not make the pulmonary valve normal. The procedure may cause a mild leakage of the pulmonary valve, which is usually very well tolerated.

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

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

  The long-term course of patients with mild PS is indistinguishable from that of the unaffected population. Mild PS tends to be stable in severity as the pulmonic valve orifice size usually increases with body growth. However, untreated severe PS may result in outflow obstruction that progresses over a period of years; 60% of patients with severe PS require intervention within 10 years of diagnosis.

• #34 Congenital pulmonary valve stenosis | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/pulmonary-stenosis/

  Following treatment for pulmonary stenosis, the child will continue to follow-up with their pediatric cardiologist. Reasons for this include reoccurrence of pulmonary stenosis from scar tissue that forms after a valvuloplasty or a valve replacement that doesn’t grow as the child gets bigger. Some children might need several procedures in their lifetime to keep the valve functioning properly.

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