Pulmonic stenosis is commonly considered a benign condition for the majority of patients. Although generally not life-threatening beyond the neonatal stage, complications and the emergence of new symptoms may necessitate intervention in adults.
This condition involves a stiffening of the pulmonic valve, leading to obstructed blood flow. While often congenital and benign, it is typically diagnosed in pediatric patients, and potentially curative treatments are available. In adults, pulmonic stenosis is frequently associated with severe structural cardiac diseases.
Epidemiology
Pulmonic stenosis is most frequently diagnosed as a congenital heart defect, often identified in infancy or childhood. The incidence and prevalence of pulmonic stenosis can vary, but it is generally considered a relatively uncommon congenital heart anomaly. The condition is often present at birth, and its prevalence may differ among various populations and ethnic groups.
Females and males can be affected equally. The prevalence may also be influenced by factors such as genetics and familial predisposition, with a higher likelihood of occurrence in individuals with a family history of congenital heart defects.
While pulmonic stenosis is typically benign and manageable, especially in pediatric cases, its prevalence in adults is often associated with underlying structural cardiac diseases. In adults, it may occur as a consequence of other cardiac conditions, such as valve abnormalities or congenital heart defects that become apparent later in life.
Anatomy
Pathophysiology
In most cases, pulmonic stenosis is congenital. The valve itself may be abnormal, with thickening, fusion of the valve leaflets, or the presence of additional tissue restricting its normal movement. The primary consequence of the narrowed pulmonary valve or artery is an obstruction to the flow of blood from the right ventricle to the lungs. This obstruction causes increased pressure in the right ventricle as it works harder to pump blood through the narrowed pathway.
The increased workload on the right ventricle leads to hypertrophy. Over time, this can result in the right ventricle becoming more muscular and less compliant. The increased pressure in the right ventricle is transmitted to the pulmonary artery, leading to changes in its structure. The pulmonary artery may also become stenotic, especially in cases where the obstruction is not solely at the level of the valve.
The severity of pulmonic stenosis determines the clinical manifestations. Mild cases may be asymptomatic, while moderate to severe cases can result in symptoms such as fatigue, shortness of breath, chest pain, and syncope. These symptoms are often related to the compromised ability of the right ventricle to pump blood effectively.
Pulmonic stenosis can be an isolated condition, but it is also commonly associated with other congenital heart defects. For example, it may coexist with ventricular septal defects or atrial septal defects. Mild cases of pulmonic stenosis may have a relatively benign course. However, moderate to severe cases can lead to complications such as bacterial endocarditis, arrhythmias, and, in extreme cases, right-sided heart failure.
Etiology
Pulmonic stenosis is frequently linked with congenital structural cardiac syndromes, such as Noonan syndrome and tetralogy of Fallot. Another notable contributor to congenital pulmonic stenosis is maternal rubella syndrome, although it is important to note that this condition is not rooted in genetic defects.
The emergence of symptomatic pulmonic stenosis in adults may occur during pregnancy in individuals with undiagnosed pulmonic stenosis or in those grappling with an underlying carcinoid syndrome. Moreover, individuals with a history of rheumatic heart disease, prior cardiothoracic surgeries, or the presence of a cardiac tumor may develop acquired pulmonic stenosis.
Genetics
Prognostic Factors
The prognosis for the majority of patients is highly favorable. With the exception of critical stenosis in the neonatal period, most individuals can expect to lead a normal life.
Clinical History
Most individuals with pulmonic stenosis do not exhibit symptoms, potentially resulting in a delayed diagnosis until an event prompts investigation. When symptoms arise, patients commonly report exertional dyspnea or fatigue, the intensity of which correlates with the degree of obstruction and the compensatory capacity of the heart.
Infrequently, individuals may encounter angina or sudden cardiac arrest. Patients may display signs of cyanosis in cases with an associated septal defect.
Physical Examination
During a physical examination, various signs of pulmonic stenosis may be apparent, contingent upon the severity of the condition and the proficiency of the practitioner. A cardiac examination might disclose a left parasternal heave attributable to right ventricular hypertrophy.
Auscultation at the left upper sternal border may uncover a systolic ejection murmur extending to the back. Additionally, there might be an accompanying ejection click and a split S2 arising from delayed pulmonary closure. Lastly, pulmonic auscultation may be linked with a fourth heart sound and tricuspid regurgitation.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Cardiac Sarcoma
Cardiac Tumor
Right Heart Failure
Sinus of Valsalva aneurysm
Tetralogy of Fallot
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Infective Endocarditis Prophylaxis
The choice of pharmacological treatment is individualized based on the patient’s specific symptoms, overall health, and any other cardiac conditions that may be present. Patients with pulmonic stenosis, particularly those with surgical intervention or associated congenital heart defects, may be prescribed antibiotics before certain dental or medical procedures to prevent infective endocarditis.
Symptomatic Management
Beta-blockers can help manage symptoms such as palpitations and chest pain. In cases where there are associated arrhythmias, antiarrhythmic medications may be prescribed to stabilize the heart’s rhythm. Diuretics may be used to reduce excess fluid and relieve symptoms for patients with fluid retention or signs of heart failure.
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
Surgical intervention for pulmonic stenosis is considered when the severity of the condition warrants more aggressive treatment. The specific approach to surgical intervention can vary depending on the individual patient’s anatomy, the degree of stenosis, and any associated cardiac issues.
Valvotomy or Valvuloplasty: This procedure involves incision of the narrowed pulmonic valve to widen it and improve blood flow. It can be done as open-heart surgery or through minimally invasive techniques. Balloon valvuloplasty is a less invasive variation of this procedure, where a catheter with a balloon on its tip is inserted into the narrowed valve and inflated to widen it.
Valve Replacement: In cases where the pulmonic valve is severely damaged or cannot be effectively repaired, valve replacement may be necessary. This can involve replacing the valve with a prosthetic valve, mechanical or bioprosthetic, or using a homograft.
Patch Enlargement: If the pulmonary artery is also narrow, in addition to a stenotic valve, a surgical patch may be used to enlarge the artery and improve blood flow.
Ross Procedure: In some cases, especially in younger patients, the Ross procedure may be considered. This involves using the patient’s pulmonary valve to replace the diseased aortic valve and then using a pulmonary homograft to replace the pulmonary valve.
Subvalvular or Supravalvular Surgery: Surgery may involve removing or repairing the obstructing tissue or area when the stenosis is located below or above the valve itself (subvalvular or supravalvular stenosis).
Pulmonary Artery Surgery: In cases of severe supravalvular stenosis, where the narrowing occurs above the valve, surgery may involve repairing or reconstructing the pulmonary artery.
Pulmonic stenosis is commonly considered a benign condition for the majority of patients. Although generally not life-threatening beyond the neonatal stage, complications and the emergence of new symptoms may necessitate intervention in adults.
This condition involves a stiffening of the pulmonic valve, leading to obstructed blood flow. While often congenital and benign, it is typically diagnosed in pediatric patients, and potentially curative treatments are available. In adults, pulmonic stenosis is frequently associated with severe structural cardiac diseases.
Pulmonic stenosis is most frequently diagnosed as a congenital heart defect, often identified in infancy or childhood. The incidence and prevalence of pulmonic stenosis can vary, but it is generally considered a relatively uncommon congenital heart anomaly. The condition is often present at birth, and its prevalence may differ among various populations and ethnic groups.
Females and males can be affected equally. The prevalence may also be influenced by factors such as genetics and familial predisposition, with a higher likelihood of occurrence in individuals with a family history of congenital heart defects.
While pulmonic stenosis is typically benign and manageable, especially in pediatric cases, its prevalence in adults is often associated with underlying structural cardiac diseases. In adults, it may occur as a consequence of other cardiac conditions, such as valve abnormalities or congenital heart defects that become apparent later in life.
In most cases, pulmonic stenosis is congenital. The valve itself may be abnormal, with thickening, fusion of the valve leaflets, or the presence of additional tissue restricting its normal movement. The primary consequence of the narrowed pulmonary valve or artery is an obstruction to the flow of blood from the right ventricle to the lungs. This obstruction causes increased pressure in the right ventricle as it works harder to pump blood through the narrowed pathway.
The increased workload on the right ventricle leads to hypertrophy. Over time, this can result in the right ventricle becoming more muscular and less compliant. The increased pressure in the right ventricle is transmitted to the pulmonary artery, leading to changes in its structure. The pulmonary artery may also become stenotic, especially in cases where the obstruction is not solely at the level of the valve.
The severity of pulmonic stenosis determines the clinical manifestations. Mild cases may be asymptomatic, while moderate to severe cases can result in symptoms such as fatigue, shortness of breath, chest pain, and syncope. These symptoms are often related to the compromised ability of the right ventricle to pump blood effectively.
Pulmonic stenosis can be an isolated condition, but it is also commonly associated with other congenital heart defects. For example, it may coexist with ventricular septal defects or atrial septal defects. Mild cases of pulmonic stenosis may have a relatively benign course. However, moderate to severe cases can lead to complications such as bacterial endocarditis, arrhythmias, and, in extreme cases, right-sided heart failure.
Pulmonic stenosis is frequently linked with congenital structural cardiac syndromes, such as Noonan syndrome and tetralogy of Fallot. Another notable contributor to congenital pulmonic stenosis is maternal rubella syndrome, although it is important to note that this condition is not rooted in genetic defects.
The emergence of symptomatic pulmonic stenosis in adults may occur during pregnancy in individuals with undiagnosed pulmonic stenosis or in those grappling with an underlying carcinoid syndrome. Moreover, individuals with a history of rheumatic heart disease, prior cardiothoracic surgeries, or the presence of a cardiac tumor may develop acquired pulmonic stenosis.
The prognosis for the majority of patients is highly favorable. With the exception of critical stenosis in the neonatal period, most individuals can expect to lead a normal life.
Most individuals with pulmonic stenosis do not exhibit symptoms, potentially resulting in a delayed diagnosis until an event prompts investigation. When symptoms arise, patients commonly report exertional dyspnea or fatigue, the intensity of which correlates with the degree of obstruction and the compensatory capacity of the heart.
Infrequently, individuals may encounter angina or sudden cardiac arrest. Patients may display signs of cyanosis in cases with an associated septal defect.
During a physical examination, various signs of pulmonic stenosis may be apparent, contingent upon the severity of the condition and the proficiency of the practitioner. A cardiac examination might disclose a left parasternal heave attributable to right ventricular hypertrophy.
Auscultation at the left upper sternal border may uncover a systolic ejection murmur extending to the back. Additionally, there might be an accompanying ejection click and a split S2 arising from delayed pulmonary closure. Lastly, pulmonic auscultation may be linked with a fourth heart sound and tricuspid regurgitation.
Cardiac Sarcoma
Cardiac Tumor
Right Heart Failure
Sinus of Valsalva aneurysm
Tetralogy of Fallot
Infective Endocarditis Prophylaxis
The choice of pharmacological treatment is individualized based on the patient’s specific symptoms, overall health, and any other cardiac conditions that may be present. Patients with pulmonic stenosis, particularly those with surgical intervention or associated congenital heart defects, may be prescribed antibiotics before certain dental or medical procedures to prevent infective endocarditis.
Symptomatic Management
Beta-blockers can help manage symptoms such as palpitations and chest pain. In cases where there are associated arrhythmias, antiarrhythmic medications may be prescribed to stabilize the heart’s rhythm. Diuretics may be used to reduce excess fluid and relieve symptoms for patients with fluid retention or signs of heart failure.
Surgical intervention for pulmonic stenosis is considered when the severity of the condition warrants more aggressive treatment. The specific approach to surgical intervention can vary depending on the individual patient’s anatomy, the degree of stenosis, and any associated cardiac issues.
Valvotomy or Valvuloplasty: This procedure involves incision of the narrowed pulmonic valve to widen it and improve blood flow. It can be done as open-heart surgery or through minimally invasive techniques. Balloon valvuloplasty is a less invasive variation of this procedure, where a catheter with a balloon on its tip is inserted into the narrowed valve and inflated to widen it.
Valve Replacement: In cases where the pulmonic valve is severely damaged or cannot be effectively repaired, valve replacement may be necessary. This can involve replacing the valve with a prosthetic valve, mechanical or bioprosthetic, or using a homograft.
Patch Enlargement: If the pulmonary artery is also narrow, in addition to a stenotic valve, a surgical patch may be used to enlarge the artery and improve blood flow.
Ross Procedure: In some cases, especially in younger patients, the Ross procedure may be considered. This involves using the patient’s pulmonary valve to replace the diseased aortic valve and then using a pulmonary homograft to replace the pulmonary valve.
Subvalvular or Supravalvular Surgery: Surgery may involve removing or repairing the obstructing tissue or area when the stenosis is located below or above the valve itself (subvalvular or supravalvular stenosis).
Pulmonary Artery Surgery: In cases of severe supravalvular stenosis, where the narrowing occurs above the valve, surgery may involve repairing or reconstructing the pulmonary artery.
medtigo
Pulmonary stenosis
Updated :
May 22, 2024
Pulmonic stenosis is commonly considered a benign condition for the majority of patients. Although generally not life-threatening beyond the neonatal stage, complications and the emergence of new symptoms may necessitate intervention in adults.
This condition involves a stiffening of the pulmonic valve, leading to obstructed blood flow. While often congenital and benign, it is typically diagnosed in pediatric patients, and potentially curative treatments are available. In adults, pulmonic stenosis is frequently associated with severe structural cardiac diseases.
Pulmonic stenosis is most frequently diagnosed as a congenital heart defect, often identified in infancy or childhood. The incidence and prevalence of pulmonic stenosis can vary, but it is generally considered a relatively uncommon congenital heart anomaly. The condition is often present at birth, and its prevalence may differ among various populations and ethnic groups.
Females and males can be affected equally. The prevalence may also be influenced by factors such as genetics and familial predisposition, with a higher likelihood of occurrence in individuals with a family history of congenital heart defects.
While pulmonic stenosis is typically benign and manageable, especially in pediatric cases, its prevalence in adults is often associated with underlying structural cardiac diseases. In adults, it may occur as a consequence of other cardiac conditions, such as valve abnormalities or congenital heart defects that become apparent later in life.
In most cases, pulmonic stenosis is congenital. The valve itself may be abnormal, with thickening, fusion of the valve leaflets, or the presence of additional tissue restricting its normal movement. The primary consequence of the narrowed pulmonary valve or artery is an obstruction to the flow of blood from the right ventricle to the lungs. This obstruction causes increased pressure in the right ventricle as it works harder to pump blood through the narrowed pathway.
The increased workload on the right ventricle leads to hypertrophy. Over time, this can result in the right ventricle becoming more muscular and less compliant. The increased pressure in the right ventricle is transmitted to the pulmonary artery, leading to changes in its structure. The pulmonary artery may also become stenotic, especially in cases where the obstruction is not solely at the level of the valve.
The severity of pulmonic stenosis determines the clinical manifestations. Mild cases may be asymptomatic, while moderate to severe cases can result in symptoms such as fatigue, shortness of breath, chest pain, and syncope. These symptoms are often related to the compromised ability of the right ventricle to pump blood effectively.
Pulmonic stenosis can be an isolated condition, but it is also commonly associated with other congenital heart defects. For example, it may coexist with ventricular septal defects or atrial septal defects. Mild cases of pulmonic stenosis may have a relatively benign course. However, moderate to severe cases can lead to complications such as bacterial endocarditis, arrhythmias, and, in extreme cases, right-sided heart failure.
Pulmonic stenosis is frequently linked with congenital structural cardiac syndromes, such as Noonan syndrome and tetralogy of Fallot. Another notable contributor to congenital pulmonic stenosis is maternal rubella syndrome, although it is important to note that this condition is not rooted in genetic defects.
The emergence of symptomatic pulmonic stenosis in adults may occur during pregnancy in individuals with undiagnosed pulmonic stenosis or in those grappling with an underlying carcinoid syndrome. Moreover, individuals with a history of rheumatic heart disease, prior cardiothoracic surgeries, or the presence of a cardiac tumor may develop acquired pulmonic stenosis.
The prognosis for the majority of patients is highly favorable. With the exception of critical stenosis in the neonatal period, most individuals can expect to lead a normal life.
Most individuals with pulmonic stenosis do not exhibit symptoms, potentially resulting in a delayed diagnosis until an event prompts investigation. When symptoms arise, patients commonly report exertional dyspnea or fatigue, the intensity of which correlates with the degree of obstruction and the compensatory capacity of the heart.
Infrequently, individuals may encounter angina or sudden cardiac arrest. Patients may display signs of cyanosis in cases with an associated septal defect.
During a physical examination, various signs of pulmonic stenosis may be apparent, contingent upon the severity of the condition and the proficiency of the practitioner. A cardiac examination might disclose a left parasternal heave attributable to right ventricular hypertrophy.
Auscultation at the left upper sternal border may uncover a systolic ejection murmur extending to the back. Additionally, there might be an accompanying ejection click and a split S2 arising from delayed pulmonary closure. Lastly, pulmonic auscultation may be linked with a fourth heart sound and tricuspid regurgitation.
Cardiac Sarcoma
Cardiac Tumor
Right Heart Failure
Sinus of Valsalva aneurysm
Tetralogy of Fallot
Infective Endocarditis Prophylaxis
The choice of pharmacological treatment is individualized based on the patient’s specific symptoms, overall health, and any other cardiac conditions that may be present. Patients with pulmonic stenosis, particularly those with surgical intervention or associated congenital heart defects, may be prescribed antibiotics before certain dental or medical procedures to prevent infective endocarditis.
Symptomatic Management
Beta-blockers can help manage symptoms such as palpitations and chest pain. In cases where there are associated arrhythmias, antiarrhythmic medications may be prescribed to stabilize the heart’s rhythm. Diuretics may be used to reduce excess fluid and relieve symptoms for patients with fluid retention or signs of heart failure.
Surgical intervention for pulmonic stenosis is considered when the severity of the condition warrants more aggressive treatment. The specific approach to surgical intervention can vary depending on the individual patient’s anatomy, the degree of stenosis, and any associated cardiac issues.
Valvotomy or Valvuloplasty: This procedure involves incision of the narrowed pulmonic valve to widen it and improve blood flow. It can be done as open-heart surgery or through minimally invasive techniques. Balloon valvuloplasty is a less invasive variation of this procedure, where a catheter with a balloon on its tip is inserted into the narrowed valve and inflated to widen it.
Valve Replacement: In cases where the pulmonic valve is severely damaged or cannot be effectively repaired, valve replacement may be necessary. This can involve replacing the valve with a prosthetic valve, mechanical or bioprosthetic, or using a homograft.
Patch Enlargement: If the pulmonary artery is also narrow, in addition to a stenotic valve, a surgical patch may be used to enlarge the artery and improve blood flow.
Ross Procedure: In some cases, especially in younger patients, the Ross procedure may be considered. This involves using the patient’s pulmonary valve to replace the diseased aortic valve and then using a pulmonary homograft to replace the pulmonary valve.
Subvalvular or Supravalvular Surgery: Surgery may involve removing or repairing the obstructing tissue or area when the stenosis is located below or above the valve itself (subvalvular or supravalvular stenosis).
Pulmonary Artery Surgery: In cases of severe supravalvular stenosis, where the narrowing occurs above the valve, surgery may involve repairing or reconstructing the pulmonary artery.
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