Materiały źródłowe

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

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

  Pulmonary valve disease is a type of heart valve disease. It affects the valve between the heart’s lower right chamber and the artery that delivers blood to the lungs. That artery is called the pulmonary artery. The valve is called the pulmonary valve. […] A diseased or damaged pulmonary valve doesn’t work the way it should. It changes how blood flows from the heart to the lungs. […] When the pulmonary valve doesn’t work as it should, the heart must work harder to pump blood to the lungs. The extra work can cause the right ventricle to become thick.

• #2 Pulmonary Valve Disease: Types & Symptoms

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

  Pulmonary valve disease is any issue that keeps this specific heart valve from working the way it should. Your pulmonary valve is the gatekeeper that lets your blood move from your hearts right ventricle (lower chamber) to your lungs via your pulmonary artery. This allows your blood to get fresh oxygen to distribute to your body. […] A problem with your pulmonary valve makes it harder for your blood to travel to your lungs, which delays getting oxygen to your tissues, cells and organs. […] Causes of pulmonary valve disease vary by condition. […] 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. […] These causes may include pulmonary hypertension, endocarditis, rheumatic fever or certain heart issues present at birth or their treatments. Its very common in people with repaired Tetralogy of Fallot.

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

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

  Changes in the geometry of the left ventricle and decreased left ventricular function can also occur. The changes are proportional to the degree of right ventricular hypertrophy; however, they revert to normal after obstruction of the right ventricular outflow tract is relieved. […] With increasing right ventricular hypertrophy, right ventricular compliance decreases with a resultant increase in end-diastolic pressure and with prominent a waves in the right atrium. As right atrial pressure rises, a right-to-left shunt may occur if the foramen ovale is patent or if an atrial septal defect is present; this change results in systemic arterial desaturation and clinically discernible cyanosis. This shunting may occur even without measurable elevation of right atrial pressure and is attributable to decreased right ventricular compliance. Such a right-to-left shunt can also occur in patients with an underdeveloped (hypoplastic) right ventricle. […] 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.

• #4

  https://www.aurorahealthcare.org/services/heart-vascular/conditions/pulmonary-valve-disease

  The pulmonary valve, located between your heart’s lower right heart chamber and the pulmonary artery, delivers blood to your lungs. The blood picks up oxygen in your lungs to deliver it to the rest of your body. With pulmonary valve disease (sometimes called pulmonic valve disease), the valve doesn’t work as well as it should, disrupting the blood flow from your heart to your lungs. […] Most often pulmonary valve disease is congenital, meaning you were born with it. Doctors arent always sure why the valve doesnt develop properly in the womb. […] Occasionally, certain infections like rheumatic fever, which can happen with an untreated strep throat, can damage the heart valve and lead to pulmonary valve disease.

• #5 Pulmonary Valve Disease: Types & Symptoms

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

  Pulmonary valve disease is any issue that keeps this specific heart valve from working the way it should. Your pulmonary valve is the gatekeeper that lets your blood move from your hearts right ventricle (lower chamber) to your lungs via your pulmonary artery. This allows your blood to get fresh oxygen to distribute to your body. […] A problem with your pulmonary valve makes it harder for your blood to travel to your lungs, which delays getting oxygen to your tissues, cells and organs. […] Causes of pulmonary valve disease vary by condition. […] 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. […] These causes may include pulmonary hypertension, endocarditis, rheumatic fever or certain heart issues present at birth or their treatments. Its very common in people with repaired Tetralogy of Fallot.

• #6 Pulmonary Valve Disease

  https://www.rwjbh.org/treatment-care/heart-and-vascular-care/diseases-conditions/pulmonary-valve-disease/

  In pulmonary valve disease, the pulmonary valve may not be closing properly (regurgitation), may be narrowed (stenosis), or may be missing since birth (atresia). […] Other conditions that contribute to development of pulmonary valve disease include rheumatic fever, autoimmune disease, trauma from injury, and end-stage renal disease. […] Treatment for pulmonary valve disease depends on the severity of your condition, whether you’re experiencing signs and symptoms, and if your condition is getting worse. […] Medical and surgical procedures for pulmonary valve disease may include pulmonary valve repair or replacement.

• #7 Diseases of the Pulmonary Valve | The Texas Heart Institute®

  https://www.texasheart.org/heart-health/heart-information-center/topics/diseases-of-the-pulmonary-valve/

  Diseases of the tricuspid and pulmonary valves are fairly rare. The pulmonary valve regulates the blood flow between the heart’s lower-right chamber (the right ventricle) and the pulmonary artery, controlling the blood flow between the heart and lungs. […] Pulmonary stenosis is most commonly caused by a defect during fetal development. The condition affects approximately 1 out of 8,000 babies. Pulmonary stenosis later in life is associated with rheumatic fever, an infection of the heart’s inner lining (endocarditis), and other conditions that can cause damage to or scarring of the pulmonary valve.

• #8 Pulmonary Valve Disease | Doctor

  https://patient.info/doctor/pulmonary-valve-disease

  Congenital disease represents the majority of all pulmonary valve problems. […] Pulmonary regurgitation occurs only very rarely as a congenital anomaly. […] However, it is a common complication after surgical or percutaneous relief of PS and following repair of Fallot’s tetralogy. […] Pulmonary regurgitation may also occur secondary to a dilated pulmonary valve ring due to pulmonary hypertension or Marfan’s syndrome. […] Significant pulmonary regurgitation may also be caused by primary pulmonary hypertension, secondary pulmonary hypertension, infective endocarditis (rare, but may occur in an intravenous drug user or with an atrial septal defect and a large left-to-right intracardiac shunt), rheumatic heart disease, carcinoid heart disease, Marfan’s syndrome and a few medications (eg, methysergide, pergolide). […] Long-term studies have demonstrated that pulmonary regurgitation may lead to progressive right ventricular dilatation, right ventricular dysfunction, exercise intolerance, ventricular tachycardia, and sudden cardiac death.

• #9 Pulmonary Valve Disease | Structural Heart | Henry Ford Health – Detroit, MI

  https://www.henryford.com/services/structural-heart/conditions/heart-valve-disease/pulmonary-valve-disease

  Pulmonary valve disease occurs when the valve doesnt open or close properly. The two types of this valve disease are: […] Narrowing (stenosis) occurs when the valves flaps become thick and stiff. This change stops the valve from fully opening and restricts blood flow. […] Regurgitation occurs when the valves flaps do not close tightly, allowing blood to leak back into the right side of the heart. […] Disease in the pulmonary valve can be: Congenital pulmonary valve disease: Someone with this condition was born with a pulmonary valve problem. Most forms of pulmonary stenosis are congenital and are most often diagnosed in childhood. […] Acquired pulmonary valve disease: This condition develops later in life from damage to the heart.

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

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

  Pulmonary valve disease narrows the pulmonary valve and affects the critical flow of blood to the lungs. It can cause fatigue, shortness of breath, and potentially heart failure. Many of our patients are born with primary congenital pulmonary valve disease. Other patients develop a form of the disease after birth. […] Pulmonary valve disease can present in several ways: Pulmonary atresia, a congenital heart defect (CHD) that causes the pulmonary valve to not form properly; Pulmonary valve stenosis, a narrowing in the opening of the pulmonary valve; Pulmonary valve regurgitation, when the pulmonary valve doesn’t close properly, allowing blood to reenter the right ventricle; Absent pulmonary valve, a CHD in which the valve has underdeveloped or missing leaflets and causes pulmonary valve stenosis and regurgitation. […] The disease and its presentations can also be associated with: Truncus arteriosus; Aortic valve disease and complications from the Ross procedure; Pulmonary atresia with intact ventricular septum (PA/IVS); Endocarditis involving the pulmonary valve.

• #11 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.

• #12 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.

• #13 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.

• #14 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. Pressure overload of the right ventricle results in increased contractility and dilation leading to increased wall stress and compensatory right ventricular hypertrophy. Increased muscle mass allows for the right ventricle to maintain a normal cardiac output. […] Over time, progressive right ventricular hypertrophy and stiffness can give rise to right ventricular diastolic and systolic dysfunction.

• #15 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. […] Usually, pulmonary valve disease is caused by a heart problem that develops before birth. A heart problem present at birth is called a congenital heart defect. In adults, pulmonary valve stenosis may be a complication of another illness. […] 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.

• #16 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.

• #17 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.

• #18 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. Pressure overload of the right ventricle results in increased contractility and dilation leading to increased wall stress and compensatory right ventricular hypertrophy. Increased muscle mass allows for the right ventricle to maintain a normal cardiac output. […] Over time, progressive right ventricular hypertrophy and stiffness can give rise to right ventricular diastolic and systolic dysfunction.

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

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

  Changes in the geometry of the left ventricle and decreased left ventricular function can also occur. The changes are proportional to the degree of right ventricular hypertrophy; however, they revert to normal after obstruction of the right ventricular outflow tract is relieved. […] With increasing right ventricular hypertrophy, right ventricular compliance decreases with a resultant increase in end-diastolic pressure and with prominent a waves in the right atrium. As right atrial pressure rises, a right-to-left shunt may occur if the foramen ovale is patent or if an atrial septal defect is present; this change results in systemic arterial desaturation and clinically discernible cyanosis. This shunting may occur even without measurable elevation of right atrial pressure and is attributable to decreased right ventricular compliance. Such a right-to-left shunt can also occur in patients with an underdeveloped (hypoplastic) right ventricle. […] 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.

• #20 Pulmonary Regurgitation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557564/

  Pulmonary regurgitation occurs when blood flows from the pulmonary artery back into the right ventricle during diastole. […] Specific pathological conditions may result in excessive and clinically significant regurgitation, negatively impacting proper ventricle function. This regurgitation can manifest as clinical symptoms of right-sided volume overload and heart failure. […] These underlying processes play a significant role in the development of pulmonary regurgitation. […] Understanding the intricate workings of the pulmonary valve and the factors contributing to pulmonary regurgitation is crucial for comprehensive patient care. By elucidating the mechanisms involved in the pathogenesis of pulmonary regurgitation, healthcare professionals can enhance their ability to diagnose, manage, and treat patients with this condition, ultimately improving patient outcomes and quality of life.

• #21 Pulmonary Regurgitation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557564/

  Pulmonary hypertension and congenital heart defects, particularly tetralogy of Fallot, are the leading causes of a dysfunctional pulmonary valve resulting in regurgitation. […] Primary pulmonary hypertension primarily contributes to pulmonary regurgitation in adults, arising from various causative factors. […] Secondary or functional pulmonary regurgitation develops in individuals with a structurally normal pulmonary valve but who exhibit severe pulmonary arterial hypertension and dilation of the pulmonary artery. […] Tetralogy of Fallot, characterized by obstruction of right ventricle outflow, ventricular septal defect, right ventricle hypertrophy, and an overriding aorta, represents the most prevalent form of cyanotic congenital heart disease globally. […] This regurgitation frequently arises as a consequence of surgical valvotomy/valvectomy or balloon pulmonary valvuloplasty performed to address right ventricle outflow tract obstruction as part of the management of conotruncal abnormalities.

• #22 Pulmonary Regurgitation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557564/

  Pulmonary hypertension and congenital heart defects, particularly tetralogy of Fallot, are the leading causes of a dysfunctional pulmonary valve resulting in regurgitation. […] Primary pulmonary hypertension primarily contributes to pulmonary regurgitation in adults, arising from various causative factors. […] Secondary or functional pulmonary regurgitation develops in individuals with a structurally normal pulmonary valve but who exhibit severe pulmonary arterial hypertension and dilation of the pulmonary artery. […] Tetralogy of Fallot, characterized by obstruction of right ventricle outflow, ventricular septal defect, right ventricle hypertrophy, and an overriding aorta, represents the most prevalent form of cyanotic congenital heart disease globally. […] This regurgitation frequently arises as a consequence of surgical valvotomy/valvectomy or balloon pulmonary valvuloplasty performed to address right ventricle outflow tract obstruction as part of the management of conotruncal abnormalities.

• #23 Pulmonary Regurgitation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557564/

  Understanding the pathophysiology of pulmonary regurgitation is essential for determining appropriate management strategies and interventions. […] Pulmonary regurgitation results in a volume overload of the right ventricle, leading to enlargement, impaired function, and functional tricuspid valve regurgitation. […] The enlargement of the chambers also increases the risk of atrial and ventricular arrhythmias, as well as potential morbidity and mortality if left untreated. […] Initially, pulmonary regurgitation is typically well-tolerated, and patients often remain asymptomatic for an extended period. […] However, over time, the right ventricle undergoes dilation in response to the increased volume load while attempting to sustain cardiac output. […] The progressive dilation of the right ventricle eventually leads to impaired right ventricle function.

• #24 Pulmonary Regurgitation – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557564/

  Understanding the pathophysiology of pulmonary regurgitation is essential for determining appropriate management strategies and interventions. […] Pulmonary regurgitation results in a volume overload of the right ventricle, leading to enlargement, impaired function, and functional tricuspid valve regurgitation. […] The enlargement of the chambers also increases the risk of atrial and ventricular arrhythmias, as well as potential morbidity and mortality if left untreated. […] Initially, pulmonary regurgitation is typically well-tolerated, and patients often remain asymptomatic for an extended period. […] However, over time, the right ventricle undergoes dilation in response to the increased volume load while attempting to sustain cardiac output. […] The progressive dilation of the right ventricle eventually leads to impaired right ventricle function.

• #25 Pulmonic regurgitation pathophysiology – wikidoc

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

  Pulmonary regurgitation (PR) may be caused by an acquired alteration in the valvular morphology, idiopathic dilatation of the pulmonary artery (IDPA), pulmonic valve ring dilatation, congenital absence or malformation of the valve, and increasing regurgitation causing right ventricular volume overload. The pathophysiologic mechanism of pulmonic regurgitation includes right ventricular overload resulting in the right ventricular remodeling and progressive decline in function. […] The diastolic pressure difference between main pulmonary artery (MPA) and right ventricle (RV) is usually very small and steers the pulmonic regurgitation. The right ventricular stiffness due to right ventricular hypertrophy (such as in Tetralogy of Fallot and fibrosis) increases the ventricular diastolic pressure. Increased ventricular diastolic pressure decreases the gradient causing regurgitation. A slight increase in the intrathoracic pressure (such as among ventilated patients) can accentuate PR considerably.

• #26 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.

• #27 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.

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

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

  Changes in the geometry of the left ventricle and decreased left ventricular function can also occur. The changes are proportional to the degree of right ventricular hypertrophy; however, they revert to normal after obstruction of the right ventricular outflow tract is relieved. […] With increasing right ventricular hypertrophy, right ventricular compliance decreases with a resultant increase in end-diastolic pressure and with prominent a waves in the right atrium. As right atrial pressure rises, a right-to-left shunt may occur if the foramen ovale is patent or if an atrial septal defect is present; this change results in systemic arterial desaturation and clinically discernible cyanosis. This shunting may occur even without measurable elevation of right atrial pressure and is attributable to decreased right ventricular compliance. Such a right-to-left shunt can also occur in patients with an underdeveloped (hypoplastic) right ventricle. […] 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.

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

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

  Changes in the geometry of the left ventricle and decreased left ventricular function can also occur. The changes are proportional to the degree of right ventricular hypertrophy; however, they revert to normal after obstruction of the right ventricular outflow tract is relieved. […] With increasing right ventricular hypertrophy, right ventricular compliance decreases with a resultant increase in end-diastolic pressure and with prominent a waves in the right atrium. As right atrial pressure rises, a right-to-left shunt may occur if the foramen ovale is patent or if an atrial septal defect is present; this change results in systemic arterial desaturation and clinically discernible cyanosis. This shunting may occur even without measurable elevation of right atrial pressure and is attributable to decreased right ventricular compliance. Such a right-to-left shunt can also occur in patients with an underdeveloped (hypoplastic) right ventricle. […] 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.

• #30 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.

• #31 Pulmonary Stenosis: Symptoms, Diagnosis & Treatment -Children’s Hospital of Orange County

  https://choc.org/heart/congenital-heart-defects/pulmonary-stenosis/

  Pulmonary stenosis is a congenital (present at birth) defect that occurs due to abnormal development of the fetal heart during the first eight weeks of pregnancy. […] Congenital pulmonary stenosis occurs due to improper development of the pulmonary valve in the first eight weeks of fetal growth. It can be caused by a number of factors, though most of the time this heart defect occurs sporadically (by chance), with no clear reason evident for its development. […] Mild pulmonary stenosis may not cause any symptoms. Problems can occur when pulmonary stenosis is moderate to severe. When this is the case, 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. Pressure builds up in the right atrium, and then in the veins bringing blood back to the right side of the heart. Fluid retention and swelling may occur.

• #32 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. […] Congenital pulmonary valve stenosis occurs due to improper development of the pulmonary valve in the first 8 weeks of fetal growth. […] Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families.

• #33 Percutaneous treatment of pulmonary valve and arteries for the management of congenital heart disease

  https://recintervcardiol.org/en/review-articles/percutaneous-treatment-of-pulmonary-valve-and-arteries-for-the-management-of-congenital-heart-disease

  The origin of pulmonary valve stenosis (PVS) is almost exclusively congenital. It amounts to 7% to 10% of all congenital heart diseases (CHD). Although it is often an isolated defect, it can be associated with other congenital malformations. […] Acquired stenosis is extremely rare and is associated with carcinoid syndrome or rheumatic fever. An emergent form is the stenosis of surgical bioprosthesis or valved conduits. […] PVS can coexist with infundibular or supravalvular pulmonary stenosis, the latter often associated with Noonan, Williams or Alagille syndromes as well as with congenital rubella. […] Clinical presentation is varied and goes from critical stenosis or pulmonary valve atresia (PVA) in the newborn baby to mild stenosis that can go untreated. […] Although its presentation in the adult life is often asymptomatic, in cases of severe stenosis, exertional dyspnea, ventricular dysfunction, arrhythmias or sudden death have been reported. In this group, it can have a native presentation after previous surgery or valvuloplasty.

• #34 Percutaneous treatment of pulmonary valve and arteries for the management of congenital heart disease

  https://recintervcardiol.org/en/review-articles/percutaneous-treatment-of-pulmonary-valve-and-arteries-for-the-management-of-congenital-heart-disease

  The origin of pulmonary valve stenosis (PVS) is almost exclusively congenital. It amounts to 7% to 10% of all congenital heart diseases (CHD). Although it is often an isolated defect, it can be associated with other congenital malformations. […] Acquired stenosis is extremely rare and is associated with carcinoid syndrome or rheumatic fever. An emergent form is the stenosis of surgical bioprosthesis or valved conduits. […] PVS can coexist with infundibular or supravalvular pulmonary stenosis, the latter often associated with Noonan, Williams or Alagille syndromes as well as with congenital rubella. […] Clinical presentation is varied and goes from critical stenosis or pulmonary valve atresia (PVA) in the newborn baby to mild stenosis that can go untreated. […] Although its presentation in the adult life is often asymptomatic, in cases of severe stenosis, exertional dyspnea, ventricular dysfunction, arrhythmias or sudden death have been reported. In this group, it can have a native presentation after previous surgery or valvuloplasty.

• #35 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.

• #36 Pulmonary Regurgitation (Pulmonic Regurgitation): Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/157639-overview

  Pulmonary or pulmonic regurgitation (PR) is defined as an abnormal reversal of blood flow from the pulmonary artery into the right ventricle. Most often, PR is not the primary process but a finding secondary to an underlying process, such as pulmonary hypertension or dilated cardiomyopathy. […] Pulmonary or pulmonic regurgitation (PR) or incompetence of the pulmonic valve occurs by one of three basic pathologic processes: dilatation of the pulmonic valve ring, acquired alteration of the pulmonic valve leaflet morphology, or congenital absence or malformation of the valve. PR leads to right ventricular (RV) volume overload, which will subsequently lead to RV enlargement and RV dysfunction. In time, PR will lead to tricuspid regurgitation. […] Disorders that dilate the pulmonic valve ring to create valvular incompetence are the most common cause of PR and include primary or secondary pulmonary hypertension, dilatation of the pulmonary trunk in Marfan syndrome or Takayasu arteritis, and idiopathic causes.

• #37 Pulmonary Valve Disease | Doctor

  https://patient.info/doctor/pulmonary-valve-disease

  Congenital disease represents the majority of all pulmonary valve problems. […] Pulmonary regurgitation occurs only very rarely as a congenital anomaly. […] However, it is a common complication after surgical or percutaneous relief of PS and following repair of Fallot’s tetralogy. […] Pulmonary regurgitation may also occur secondary to a dilated pulmonary valve ring due to pulmonary hypertension or Marfan’s syndrome. […] Significant pulmonary regurgitation may also be caused by primary pulmonary hypertension, secondary pulmonary hypertension, infective endocarditis (rare, but may occur in an intravenous drug user or with an atrial septal defect and a large left-to-right intracardiac shunt), rheumatic heart disease, carcinoid heart disease, Marfan’s syndrome and a few medications (eg, methysergide, pergolide). […] Long-term studies have demonstrated that pulmonary regurgitation may lead to progressive right ventricular dilatation, right ventricular dysfunction, exercise intolerance, ventricular tachycardia, and sudden cardiac death.

• #38 Pulmonary Valve Disease | Doctor

  https://patient.info/doctor/pulmonary-valve-disease

  Congenital disease represents the majority of all pulmonary valve problems. […] Pulmonary regurgitation occurs only very rarely as a congenital anomaly. […] However, it is a common complication after surgical or percutaneous relief of PS and following repair of Fallot’s tetralogy. […] Pulmonary regurgitation may also occur secondary to a dilated pulmonary valve ring due to pulmonary hypertension or Marfan’s syndrome. […] Significant pulmonary regurgitation may also be caused by primary pulmonary hypertension, secondary pulmonary hypertension, infective endocarditis (rare, but may occur in an intravenous drug user or with an atrial septal defect and a large left-to-right intracardiac shunt), rheumatic heart disease, carcinoid heart disease, Marfan’s syndrome and a few medications (eg, methysergide, pergolide). […] Long-term studies have demonstrated that pulmonary regurgitation may lead to progressive right ventricular dilatation, right ventricular dysfunction, exercise intolerance, ventricular tachycardia, and sudden cardiac death.

• #39 Pulmonary Valve Disease: Types & Symptoms

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

  Many cases of atresia have unknown causes, but atresia causes include changes in a fetus genes or exposure to something harmful in the environment or in something ingested during pregnancy. […] Pulmonary valve disease can strain, damage and/or enlarge your hearts right ventricle because it has to use more effort to move blood forward to your lungs. In rare cases, this can lead to heart failure. […] Pulmonary valve disease treatments range from medicine to surgery, depending on the condition you have. […] Pulmonary valve disease treatments include: Balloon valvuloplasty to widen your pulmonary valve. […] You cant lower your risk of having a child with pulmonary valve disease from unknown causes. But if you plan to become pregnant, you should be sure youre up to date with the rubella vaccine.

• #40 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.

• #41 Pulmonary Stenosis: Symptoms, Diagnosis & Treatment -Children’s Hospital of Orange County

  https://choc.org/heart/congenital-heart-defects/pulmonary-stenosis/

  Pulmonary stenosis is a congenital (present at birth) defect that occurs due to abnormal development of the fetal heart during the first eight weeks of pregnancy. […] Congenital pulmonary stenosis occurs due to improper development of the pulmonary valve in the first eight weeks of fetal growth. It can be caused by a number of factors, though most of the time this heart defect occurs sporadically (by chance), with no clear reason evident for its development. […] Mild pulmonary stenosis may not cause any symptoms. Problems can occur when pulmonary stenosis is moderate to severe. When this is the case, 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. Pressure builds up in the right atrium, and then in the veins bringing blood back to the right side of the heart. Fluid retention and swelling may occur.

• #42 Pulmonary Stenosis: Symptoms, Diagnosis & Treatment -Children’s Hospital of Orange County

  https://choc.org/heart/congenital-heart-defects/pulmonary-stenosis/

  Pulmonary stenosis is a congenital (present at birth) defect that occurs due to abnormal development of the fetal heart during the first eight weeks of pregnancy. […] Congenital pulmonary stenosis occurs due to improper development of the pulmonary valve in the first eight weeks of fetal growth. It can be caused by a number of factors, though most of the time this heart defect occurs sporadically (by chance), with no clear reason evident for its development. […] Mild pulmonary stenosis may not cause any symptoms. Problems can occur when pulmonary stenosis is moderate to severe. When this is the case, 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. Pressure builds up in the right atrium, and then in the veins bringing blood back to the right side of the heart. Fluid retention and swelling may occur.

• #43 Pulmonic Valve Disorders | Heart & Vascular | Loyola Medicine

  https://www.loyolamedicine.org/services/heart-and-vascular/heart-vascular-conditions/valvular-disease/pulmonic-valve-disorders

  Pulmonic valve disorders, also known as pulmonary valve disease, is a heart condition characterized by a dysfunctional pulmonary valve. The pulmonary valve regulates blood flow between your right heart ventricle and your pulmonary artery and lungs. […] Pulmonic valve disorders can compromise or reduce blood flow through the heart and to the lungs, which results in a reduced flow of oxygen-rich blood to the rest of the body. […] Pulmonic valve disorders are typically present at birth or develop during pregnancy. However, the heart tissue may be weakened or degenerated by aging, which can cause symptoms to develop. […] Severe health events, such as heart attack, lupus and/or radiation therapy for cancer, can also cause pulmonic valve tissue damage and disorders. Taking some medications, like methysergide, can also cause or lead to pulmonic valve symptoms. […] Several procedures are used to test for and diagnose pulmonic valve disorders. […] There are several ways to treat pulmonic valve disorders, depending on their cause.

• #44 Valvular heart disease | Heart and Stroke Foundation

  https://www.heartandstroke.ca/heart-disease/conditions/valvular-heart-disease

  The pulmonary valve controls blood flow between the right ventricle and the lungs. It opens to let the heart pump blood out of the ventricles into the pulmonary artery toward the lungs so it can pick up oxygen. […] If your pulmonary valve narrows, the flow of oxygen-poor blood from the right ventricle through the pulmonary arteries to the lungs is restricted. This affects your blood’s ability to pick up oxygen and deliver oxygen-rich blood to the rest of your body. With pulmonary valve stenosis, the right ventricle has to work harder to pump blood through the narrowed pulmonary valve and the pressure in the heart is often increased. […] Pulmonary valve regurgitation results when the pulmonary valve doesn’t close properly. The lower right chamber (right ventricle) of the heart pushes blood through the pulmonary artery into the lungs for blood to pick up oxygen. When the pulmonary valve does not close completely, blood can leak back from the lungs into the heart. This backward blood flow mixes oxygen-poor and oxygen-rich blood, and reduces the availability of oxygen-rich blood to fuel the rest of your body.

• #45 Valvular heart disease | Heart and Stroke Foundation

  https://www.heartandstroke.ca/heart-disease/conditions/valvular-heart-disease

  The pulmonary valve controls blood flow between the right ventricle and the lungs. It opens to let the heart pump blood out of the ventricles into the pulmonary artery toward the lungs so it can pick up oxygen. […] If your pulmonary valve narrows, the flow of oxygen-poor blood from the right ventricle through the pulmonary arteries to the lungs is restricted. This affects your blood’s ability to pick up oxygen and deliver oxygen-rich blood to the rest of your body. With pulmonary valve stenosis, the right ventricle has to work harder to pump blood through the narrowed pulmonary valve and the pressure in the heart is often increased. […] Pulmonary valve regurgitation results when the pulmonary valve doesn’t close properly. The lower right chamber (right ventricle) of the heart pushes blood through the pulmonary artery into the lungs for blood to pick up oxygen. When the pulmonary valve does not close completely, blood can leak back from the lungs into the heart. This backward blood flow mixes oxygen-poor and oxygen-rich blood, and reduces the availability of oxygen-rich blood to fuel the rest of your body.

• #46 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.

• #47 Pulmonary Stenosis – ACHA

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

  Pulmonary stenosis (PS) is an obstruction of the blood flow from the right ventricle to the pulmonary artery. It results from a narrowing (stenosis) at several points on or near the pulmonary valve. […] 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. […] If severe obstruction is not treated, the right ventricle can begin to function poorly. This might cause arrhythmias (irregular heartbeats), loss of energy and fluid retention. If the pressure in the right heart is high enough, unoxygenated or blue blood can cross over into the left atrium. It then mixes with red or oxygenated blood. This results in cyanosis or blueness.

• #48 Pulmonary Valve Stenosis: Causes, Symptoms, and Treatment

  https://www.webmd.com/heart-disease/what-to-know-pulmonary-valve-stenosis

  Complications can arise whether you’ve had corrective treatment or not. For example, if you undergo a valve replacement, there are several safeguards you must have in place to prevent inflammation or infection of the valve. […] The heart muscle thickens. Because the heart has to work harder to pump blood, the walls of the heart can get thick. This can cause extra strain on the heart. […] Heart failure. In severe cases, the heart could fail because it’s working extremely hard to pump blood, and not enough blood is going through it.

• #49 Pulmonary Valve Stenosis: Causes, Symptoms, and Treatment

  https://www.webmd.com/heart-disease/what-to-know-pulmonary-valve-stenosis

  Complications can arise whether you’ve had corrective treatment or not. For example, if you undergo a valve replacement, there are several safeguards you must have in place to prevent inflammation or infection of the valve. […] The heart muscle thickens. Because the heart has to work harder to pump blood, the walls of the heart can get thick. This can cause extra strain on the heart. […] Heart failure. In severe cases, the heart could fail because it’s working extremely hard to pump blood, and not enough blood is going through it.

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