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Pulmonary Stenosis

Author: Joseph Heaton Author: Maria S. Horenstein Editor: Chris Kyriakopoulos Updated: 10/6/2024 3:01:45 PM

Introduction

Pulmonary stenosis is a relatively common cardiac defect that can occur in isolation or, more commonly, in combination with other congenital heart defects, such as tetralogy of Fallot. The obstruction may occur within the right ventricular outflow tract, at the pulmonary valve annulus or pulmonary valve leaflets, or within the main and branch pulmonary arteries. Although pulmonary stenosis occurs in isolation in 7% to 12% of patients, it is more commonly associated with other congenital heart defects, affecting 25% to 30% of patients. Symptomatic patients are generally those with moderate or severe pulmonary stenosis who typically experience dyspnea on exertion or associated fatigue, depending on the severity of the obstruction and cardiac compensatory reserve. In rare cases, patients may experience angina or sudden cardiac arrest. Significantly enlarged pulmonary artery aneurysms may cause angina by compressing the left main coronary artery.[1] 

The diagnosis of pulmonary stenosis is typically made using echocardiography. However, cardiac computed tomography (CCT) and cardiac magnetic resonance (CMR) are also used in cases where intervention is required to relieve right ventricular outflow obstruction.[2][3] In recent years, transcatheter approaches for treating pulmonary stenosis have become increasingly common. However, surgery may be necessary when the anatomy is unsuitable for percutaneous treatment. The severity of flow restriction across the pulmonary valve and the valve anatomy determines the appropriate treatment, guided by the recommendations of the American Heart Association (AHA) and the American College of Cardiology (ACC).[3]

Etiology

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Etiology

Pulmonary stenosis can occur as an isolated valvular lesion or be associated with congenital structural cardiac anomalies, including tetralogy of Fallot, tricuspid atresia, complete and corrected transposition of the great arteries, and double outlet right ventricle. Pulmonary stenosis can also be linked with genetic syndromes such as Noonan syndrome, which is most commonly caused by PTPN11 mutations but can also be caused by KRAS, SOS1, and RAF1 mutations.[4][5][6][7] Peripheral pulmonary stenosis is another form that can be associated with conditions such as Alagille syndrome, caused by a JAG1 mutation on chromosome 12q24, or less frequently, NOTCH2 mutations, and Williams-Beuren syndrome, caused by an ELN mutation in chromosome 7q11.23.[8][9] Lastly, maternal rubella syndrome is also a known cause of congenital valvular pulmonary stenosis, although it is not a genetic defect.[10][11] 

Infrequently, valvular pulmonary stenosis can manifest clinically during pregnancy in previously undiagnosed patients with pulmonary stenosis or patients suffering from an underlying carcinoid syndrome.[12][13] Acquired forms of pulmonary stenosis can also occur in patients with a history of rheumatic heart disease, previous cardiothoracic surgeries, or cardiac tumors such as pericardial sarcoma, teratoma thymoma, or Hodgkin disease.[14]

Epidemiology

Isolated valvar pulmonary stenosis accounts for 7% to 12% of congenital heart diseases.[15] Extracardiac and neurodevelopmental comorbidities affect approximately 56% of patients with pulmonary stenosis. In such cases, a molecular diagnosis is more common. For example, the PTPN11 mutation is identified in 50% of patients with pulmonary stenosis and Noonan syndrome.[7][16] Moreover, a familial form of nonsyndromic pulmonary stenosis has been described and is suspected to be related to GATA4 mutations. Pulmonary stenosis does not seem to have any gender predilection.[17] 

Pathophysiology

Types of Pulmonary Stenosis

Pulmonary stenosis is classified into 3 types based on the location of the obstruction—valvular, subvalvular, and supravalvular. The degree of obstruction can be mild, moderate, severe, or critical.[18][3]

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.[19] 

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. Patients with Noonan syndrome often have a hypoplastic pulmonary annulus and hypoplastic proximal pulmonary arteries. Bicuspid pulmonary valves are more commonly associated with tetralogy of Fallot, and other variations in leaflet quantities, such as quadricuspid valves, have also been reported.[20]

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. In addition, subvalvular pulmonary stenosis may develop in 20% to 30% of patients with Noonan syndrome who have hypertrophic cardiomyopathy.[7] Secondary causes of subvalvular pulmonary stenosis can result from primary valvular stenosis, leading to right ventricular hypertrophy. In such cases, the secondary subvalvular pulmonary stenosis often regresses after a valvotomy or valvuloplasty.[21][22]

Subpravalvular pulmonary stenosis: 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.[23][24][25][26][27][28] Supravalvular pulmonary stenosis can also occur after surgical repair of the transposition of the great arteries.[29]

Degree of Obstruction

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:

  • Mild stenosis: Peak gradient <36 mm Hg
  • Moderate stenosis: Peak gradient between 36 and 64 mm Hg
  • Severe stenosis: Peak gradient >64 mm Hg [3]
  • Critical stenosis: Observed in infants with inadequate anterograde blood flow through a very tight and small pulmonary valve or annulus. These patients require prostaglandin-E1 to keep the ductus arteriosus patent and allow blood flow to the lungs.[18]

History and Physical

Clinical Features

Most patients who have mild pulmonary stenosis are asymptomatic. Symptomatic patients are generally those with moderate or severe pulmonary stenosis who typically experience dyspnea on exertion or associated fatigue, depending on the severity of the obstruction and cardiac compensatory reserve.[1] Rarely patients can experience angina or sudden cardiac arrest. Significantly enlarged pulmonary artery aneurysms can cause angina through compression of the left main coronary artery.[30] In children and adults, the following auscultative findings might help assess the severity of valvular pulmonary stenosis obstruction (see Audio. Pulmonary Stenosis):

  • Mild pulmonary stenosis is characterized by a normal first heart sound (S1) followed by an ejection click. The intensity of the pulmonic component of the second heart sound (P2) ranges from normal to increased.
  • Moderate pulmonary stenosis is marked by S1 being present, but the ejection click becomes closer to S1. S2 is split with a soft P2 component, and the width of the splitting of S2 depends on the severity of the obstruction.
  • Severe pulmonary stenosis is indicated by an absent click or a click that occurs so close to S1 that it cannot be distinguished. A louder murmur is also noted. S2 is widely split, and the P2 component is extremely soft or inaudible due to a decreased flow across the pulmonary valve.[31]

Infants born with critical pulmonary stenosis may experience cyanosis due to right-to-left atrial shunting across a patent foramen ovale or atrial septal defect. They may also show signs of systemic venous congestion caused by right ventricular dysfunction.[18] Progressive right ventricular dilatation can indicate the presence of an atrial septal defect, but decreased right ventricular compliance and increased right atrial pressure can also lead to cyanosis due to right-to-left shunting across the atrial septal defect. In severe cases of pulmonary stenosis, systemic venous congestion can occur.[30] In children or adults with moderate or severe pulmonary stenosis, additional cardiac findings may include a left parasternal heave due to right ventricular hypertrophy. The systolic ejection murmur may radiate to the back.[32] Reduced ventricular compliance can result in the presence of a fourth heart sound in severe cases of pulmonary stenosis.[33]

Evaluation

The evaluation of pulmonary stenosis is typically performed using echocardiography, as it usually provides adequate visualization of the pulmonary valve and surrounding structures.[34][35] In most cases, a transthoracic echocardiogram is sufficient. However, transesophageal echocardiography is used if suboptimal views or endocarditis is suspected.[36][37] Electrocardiographic criteria that support right ventricular hypertrophy correlate with the severity of the pulmonary stenosis. Mild pulmonary stenosis may show right axis deviation, whereas severe cases may show prominent R waves in V1 and aVR, and P-wave enlargement.[38]

Doppler studies using echocardiography provide flow gradients, which are used to grade the severity of the pulmonary stenosis. Guidelines from the European Association of Echocardiography, the American Society of Echocardiography, the AHA, the ACC, and the European Society of Cardiology (ESC) include the following severity categories: [34][35][39]

  • Mild stenosis: The peak Doppler gradient across the pulmonary valve is <36 mm Hg, or the Doppler jet velocity is <3 m/s.
  • Moderate stenosis: The peak Doppler gradient across the valve is between 36 and 64 mm Hg, or the Doppler jet velocity is between 3 and 4 m/sec.
  • Severe stenosis: The peak Doppler gradient across the valve is >64 mm Hg, or the Doppler jet velocity is >4 m/s.

Cardiac catheterization and pulmonary angiography are typically not required for diagnosing pulmonary stenosis. Echocardiography is effective and safe in most cases. However, CMR can be a suitable alternative in case of suboptimal echocardiographic windows or complicated anatomy.[40][41] CMR can also measure right ventricular volume, function, and pulmonary artery blood flow. In addition, fetal CMR can be used with echocardiography to understand cardiac anatomy and branch pulmonary artery anatomy better. CCT may be used if a patient cannot undergo CMR.[3] The sensitivity of plain radiography is insufficient to diagnose pulmonary stenosis. However, radiographic evidence of prominent pulmonary arteries or a prominent right heart border can support a diagnosis of pulmonary stenosis.[30]

Treatment / Management

Pulmonary Stenosis Management

The management of pulmonary stenosis is based on the severity of flow restriction across the pulmonary valve and the valve anatomy. The AHA and ACC have revised and updated guidelines for managing pulmonary stenosis.[36][42](A1)

Surveillance recommendations: The AHA and ACC provide the following monitoring guidelines:

  • Asymptomatic patients with a peak Doppler gradient <30 mm Hg can be followed up every 5 years with an electrocardiogram and Doppler echocardiography.
  • Asymptomatic patients with a peak Doppler gradient >30 mm Hg can be followed up with Doppler echocardiography every 2 to 5 years.

Balloon valvuloplasty: Balloon valvuloplasty is recommended for the following populations:

  • Asymptomatic patients with a domed pulmonary valve with a peak Doppler gradient >60 mm Hg.
  • Symptomatic patients, such as those with unexplained heart failure, cyanosis from an interatrial right to left shunting, or exercise intolerance, with a domed pulmonary valve with a peak Doppler gradient >50 mm Hg or a mean Doppler gradient >30 mm Hg.

Balloon valvuloplasty is not as effective in most dysplastic valves as in domed valves, thus making surgery the preferred option. However, in patients with dysplastic valves, balloon valvuloplasty may be reasonable in the following cases:

  • Asymptomatic patients with a dysplastic pulmonary valve and a peak Doppler gradient >60 mm Hg or a mean Doppler gradient >40 mm Hg
  • Symptomatic patients with a dysplastic pulmonary valve and a peak Doppler gradient >50 mm Hg or a mean Doppler gradient >30 mm Hg

Pulmonary valvuloplasty can be successful; reinterventions due to restenosis might be required. In addition, young age, low weight, small pulmonary annulus diameter, a higher initial systolic gradient across the pulmonary valve, increased right ventricular or systemic pressure ratio, and severe pulmonary stenosis are associated with a higher probability of moderate regurgitation after the valvotomy procedure.[43](B3)

Balloon valvuloplasty is not recommended in the following cases:

  • Asymptomatic patients with normal cardiac output and a Doppler peak instantaneous gradient <50 mm Hg.
  • Symptomatic patients with pulmonary stenosis and severe pulmonary regurgitation.
  • Symptomatic patients with a Doppler peak instantaneous gradient <30 mm Hg.

Surgical intervention: Surgical intervention is indicated in the following patients:

  • Moderate-to-severe valvular stenosis in symptomatic patients who are ineligible for or in whom balloon valvuloplasty was ineffective
  • Severe valvular stenosis with severe pulmonary regurgitation
  • Hypoplastic pulmonary annulus
  • Subvalvular stenosis
  • Supravalvular stenosis
  • Dysplastic pulmonary valves when there is associated severe tricuspid regurgitation and when a Maze procedure is required
  • Patients undergoing concurrent cardiac surgical procedures

Pulmonary artery balloon angioplasty with optional stent placement is an acceptable treatment for supravalvular and subvalvular pulmonary stenosis. 

Antibiotic prophylaxis: Antibiotic prophylaxis before dental procedures and vaginal delivery in patients with pulmonary stenosis is reasonable in the following cases:

  • A prosthetic cardiac valve or prosthetic material used for valve repair
  • Previous infective endocarditis
  • In the presence of other cyanotic lesions, including shunts or conduits

Antibiotic prophylaxis against infective endocarditis is not recommended for non-dental procedures such as esophagogastroduodenoscopy or colonoscopy in the absence of active infection.[30] In general, antibiotic prophylaxis is recommended for individuals with a history of infective endocarditis, those with biological or mechanical prosthetic valves, patients who have received intracardiac prosthetic material within the last 6 months, those with residual intracardiac shunts in the area of or near prosthetic material, and those suffering from cyanotic heart disease.[42](A1)

Critical Pulmonary Stenosis

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. As the patent ductus arteriosus closes, these patients become increasingly cyanotic, and without prostaglandin E1 infusion or ductal stenting, this lesion leads to hypoxemia and death. In the absence of a ventricular septal defect, the right ventricle is hypoplastic, severely hypertrophied and restrictive, and often inadequate to maintain cardiac output. This anatomic arrangement requires the enlargement of a restrictive patent foramen ovale or a small atrial septal defect to allow for the pulmonary and systemic venous return to flow into the subaortic ventricle. This arrangement functions as a single ventricle with mixing in the atria until further surgery or surgeries are performed to stabilize the circulation. An atrial septostomy or Rashkind procedure can be performed in the cardiac catheterization laboratory or at the bedside in the cardiac intensive care unit, using ultrasound guidance with or without fluoroscopy. Static ballooning or atrial stent placement is performed in the cardiac catheterization laboratory.[18][44]

Definitive surgical repair may not be possible if the right ventricle is severely hypoplastic, which might require a multi-staged single ventricle palliation. However, a one-and-a-half ventricle repair palliation can become the initial surgery for those whose right ventricles can hold some cardiac output. This approach is superior to the Fontan procedure as it provides pulsatile flow to the pulmonary circulation, avoiding the development of pulmonary arteriovenous malformations that can develop after the Glenn or the Fontan procedures. Conversion to a biventricular repair can be later attempted.[45][46] Most recently, in-utero pulmonary valvuloplasty in some fetuses with critical pulmonary stenosis or pulmonary valve atresia has allowed right ventricular growth. This intervention has increased the chances of postnatal biventricular circulation in fetuses otherwise predicted to require single-ventricle palliation.[47]

Differential Diagnosis

The differential diagnosis of pulmonary stenosis in infants includes:

  • Congenital heart defects with associated pulmonary stenosis, such as a double-chambered right ventricle, double-outlet right ventricle, absent pulmonary valve, tetralogy of Fallot, atrioventricular septal defect, atrial septal defect, and ventricular septal defect
  • Pulmonary atresia with intact ventricular septum
  • Ventricular septal defect 

In adult patients, differential diagnoses include:

  • Rheumatic valvular heart disease
  • Carcinoid heart disease
  • Pulmonary embolism
  • Right heart failure
  • Cardiac tumor
  • Cardiac sarcoma

Prognosis

The natural history of pulmonary stenosis depends on the degree of pulmonary valve stenosis or the anatomy of the affected vessels. Except for critical stenosis observed during the neonatal period, most patients with mild and even moderate pulmonary stenosis without an associated genetic syndrome or congenital heart defect can lead a typical life, remaining asymptomatic and having an excellent prognosis. However, some patients develop significant pulmonary stenosis and require intervention.[48][49]

Patients with dome-shaped pulmonary valves undergoing balloon valvuloplasty have a better prognosis compared to those with dysplastic valves.[50][51] Complications during balloon valvuloplasty are generally minor, including a vagal response, catheter-induced ventricular ectopy, right bundle branch block, and transient or permanent high-grade atrioventricular nodal block.[52] A rare but serious adverse effect in hypertrophic right ventricles is referred to as the suicidal right ventricle, where dynamic obstruction of the outflow tract causes a sudden drop in the pressure gradient across the pulmonary valve. This condition is a postprocedure life-threatening emergency that can be prevented using beta blockers before the intervention to decrease the risk of hypercontractility of the right ventricular outflow tract following valvuloplasty.[51][53] 

Most patients undergoing surgical revision experience improvements in their maximal exercise capacity, with only 15% to 20% requiring reintervention. Most surgical reinterventions are for significant pulmonary valve regurgitation. In these patients, if supraventricular tachycardia is present, it typically resolves after the reintervention.[54][55]

During pregnancy, patients with mild pulmonary stenosis might present with an asymptomatic systolic murmur; occasionally, they might have exercise intolerance. Pregnancy is well-tolerated unless the pulmonary stenosis is severe. Percutaneous valvuloplasty can be performed during pregnancy, although the need is unusual.[30]

Complications

Infective endocarditis in isolated pulmonary stenosis is rare. In children, most cases occur secondary to an anatomically abnormal pulmonary valve, such as in patients with Noonan syndrome. However, in adults, most cases are secondary to intravenous drug abuse.[56] Complications can occur in individuals who have moderate and severe pulmonary stenosis, including arrhythmias, typically premature atrial contractions, premature ventricular contractions, and ventricular couplets.[57][58]

Consultations

Suspected symptomatic pulmonary stenosis in an adult requires a consultation with an adult congenital cardiologist. For a pregnant patient with pulmonary stenosis, a high-risk maternal-fetal medicine specialist and an adult congenital cardiologist should be consulted, as the hemodynamic changes associated with pregnancy can exacerbate pulmonary stenosis symptoms. Consultation with a pediatric cardiologist is warranted for newborns experiencing symptoms of pulmonary stenosis. Depending on the severity, pediatric critical care may also be required.

Deterrence and Patient Education

Parents should receive education regarding treatments available for pediatric patients with pulmonary stenosis and possible complications. Adult patients, especially those planning to become pregnant, should be educated about the risks, complications, and outcomes since pregnancy can exacerbate their underlying condition. Genetic counseling should be sought to discuss the risks of congenital heart defects in the patient's future children.

Pearls and Other Issues

Athletic Participation in the Setting of Pulmonary Stenosis: American Heart Association and the American College of Cardiology Scientific Statement

The care of athletes with pulmonary stenosis is guided by the following 2015 AHA/ACA recommendations:

  • Athletes with mild pulmonary stenosis, defined as a Doppler-derived peak instantaneous gradient <40 mm Hg and normal right ventricular function, can participate in all competitive sports. An annual reevaluation is recommended.
  • Athletes who received a pulmonary valvotomy or a balloon valvuloplasty and have achieved adequate relief of pulmonary stenosis with a gradient <40 mm Hg can also participate in all competitive sports.
  • Athletes with moderate, defined as Doppler-derived peak instantaneous gradient of 40 to 60 mm Hg or severe pulmonary stenosis with a gradient >60 mm Hg, can consider participation only in low-intensity class IA and IB sports. Class IA refers to low-static and low-dynamic sports such as bowling, golf, and yoga. Class IB includes low-static but moderately dynamic sports such as basketball, softball, table tennis, and volleyball.[59]
  • Athletes with severe pulmonary insufficiency and marked right ventricular enlargement can consider participation in low-intensity class IA and IB sports.[60]

In 2020, the European Association of Preventive Cardiology Group (EAPC) and the ESC's Section of Sports Cardiology and Exercise updated their exercise recommendations for adolescents and adults with congenital heart disease. The revised guidelines are now based on hemodynamic and electrophysiological parameters. Rather than focusing on specific anatomical defects, these parameters merge the patient's hemodynamic status with the common hemodynamic changes and cardiac remodeling that occur from long-term training in different sports. This personalized approach allows for tailored exercise recommendations. The hemodynamic parameters used for the exercise recommendations by the EAPC and the ESC include:

  • Ventricular function
  • Pulmonary artery pressure
  • Aortic dimensions
  • Presence of arrhythmias
  • Arterial O2 saturation at rest and during cardiopulmonary exercise testing

When all parameters are within normal limits, or there is mild ventricular hypertrophy or mild pressure or volume load, athletes can participate in all competitive sports. When one of the parameters is abnormal, the patient is restricted from endurance sports that have high hemodynamic demands and require high-volume training, such as cycling, running, rowing, cross-country skiing, and long-distance skating, or they are restricted to skill sports, such as golf, motor racing, sailing, scuba diving, table tennis, and ski jumping. Patients with marked structural, hemodynamic, or electrophysiologic abnormalities who are symptomatic are restricted to recreational activities.[61]

Enhancing Healthcare Team Outcomes

An interprofessional team approach is crucial for treating patients with pulmonary stenosis, ensuring comprehensive care and improved outcomes. Clinicians, advanced practitioners, nurses, pharmacists, and other healthcare professionals must collaborate, each contributing distinct yet complementary roles. Clinicians and advanced practitioners lead the diagnostic process and develop treatment plans, whereas nurses ensure continuity of care, monitor patient progress, and provide patient education. Pharmacists play a crucial role in managing medications, particularly for patients requiring anticoagulation or managing other comorbidities.

Interprofessional communication enables timely sharing of insights and updates, particularly when care involves complex transitions, such as pregnancy or neonatal care, where cardiologists, obstetricians, and pediatric specialists must collaborate. Clear care coordination ensures that patients receive appropriate interventions, whether in nutrition, mental health, or rehabilitation, whereas timely communication enhances patient safety and fosters better team performance. By focusing on patient-centered strategies and clearly defined roles, the team can optimize care and ensure the best outcomes for those with pulmonary stenosis.

Media


<p>Contributed by Katherine Humphreys</p>

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