[Skip to content](https://www.ekohealth.com/blogs/clinical-education/pulmonary-valve-disease#MainContent) [$75 off the Rose Gold CORE 500™](https://www.ekohealth.com/pages/rose-gold) [New EFAST AI model with structural murmur detection](https://www.ekohealth.com/pages/efast-cardiac-ai-model) [Use your HSA/FSA funds on Eko](https://www.ekohealth.com/pages/hsa-fsa-stethoscopes) By **Rosalie McDonough, MD, MSc** Posted in [**Clinical Education**](https://www.ekohealth.com/blogs/clinical-education) March 19, 2026 * * * Pulmonary valve disease encompasses both pulmonic stenosis and pulmonary regurgitation, representing important clinical entities in both congenital and acquired heart disease. While pulmonic stenosis accounts for 7-12% of all congenital heart disease, pulmonary regurgitation is most commonly an iatrogenic consequence of interventions for right ventricular outflow tract obstruction.1-3  With the growing population of adults living with congenital heart disease, understanding the diagnosis and management of pulmonary valve disease has become increasingly important for clinicians. ##### ## **Pulmonic stenosis** ##### **Etiology and epidemiology** Virtually all cases of pulmonary valve stenosis are congenital in origin, as the pulmonary valve is the least likely valve to be affected by acquired heart disease.2  Pulmonic stenosis has a prevalence of approximately 1 in 1,800 live births and represents 80-90% of all right ventricular outflow tract obstructions.2-4 ##### **Pathophysiology** Pulmonic stenosis creates obstruction to right ventricular outflow, resulting in elevated right ventricular systolic pressure and compensatory right ventricular hypertrophy. The degree of obstruction determines the severity of hemodynamic consequences.2 ##### **Clinical presentation** **Symptoms:** Symptoms are unusual in children or adolescents with pulmonary valve stenosis, even when severe.2 Depending on the severity, adults may experience: | Category | Mild pulmonic stenosis | Severe pulmonic stenosis | | --- | --- | --- | | Symptoms | Usually asymptomatic | Dyspnea, fatigue, exertional syncope; cyanosis if PFO present with R→L shunt | | Jugular venous pulse | Normal | Elevated with prominent "A" wave | | Precordium | No RV lift | RV lift present | | Auscultation | Ejection click decreasing with inspiration; soft murmur ending mid-systole | Loud, long murmur; loss of ejection click; wide split S2 with soft/absent P2; right-sided S4 | | Other findings | None | Signs of right heart failure (late) | **Physical examination:** The severity of pulmonic stenosis can often be estimated based on physical examination findings. **Classic findings include2,5:** - Systolic ejection murmur at the left upper sternal border - Systolic ejection click that varies with respiration (decreases with inspiration) - Wide splitting of the second heart sound with soft or absent pulmonic component - Right ventricular heave (in severe cases) - Signs of right heart failure (in advanced disease) ##### **** ##### **Diagnostic evaluation** - **Echocardiography** is the primary diagnostic modality.1,6 - **Cardiac catheterization** may be performed when echocardiographic findings are inconclusive or when intervention is planned.2 ##### **Management** - **Medical management:** There is no specific medical therapy for pulmonic stenosis. Management focuses on surveillance and timely intervention.2 - **Balloon valvuloplasty:** Balloon pulmonary valvuloplasty has largely supplanted surgical pulmonary valvotomy as the first-line therapy for isolated valvular pulmonic stenosis.2,7,8 - **Surgical intervention** is recommended for certain patients, e.g., those with more severe disease, dysplastic pulmonary valves, or anatomy not suitable for percutaneous treatment. 1,6 ##### **Natural history and prognosis** Patients with mild native pulmonary valve stenosis have a reassuring natural history, and intervention is not usually necessary. Mild pulmonic stenosis rarely progresses.8 Patients with severe pulmonic stenosis typically require intervention in childhood and have a good prognosis into adulthood. Patients with moderate stenosis have a more variable course, with some requiring intervention and others managed conservatively with good long-term outcomes.6 ## **Pulmonary regurgitation** ##### **Etiology and epidemiology** Pulmonary regurgitation as an isolated congenital lesion is uncommon, occasionally seen with idiopathic dilation of the pulmonary artery or connective tissue disorders. However, pulmonary regurgitation is an almost unavoidable result of either surgical or balloon valvuloplasty for valvular pulmonic stenosis or surgical repair of tetralogy of Fallot.3,5 Among patients with pulmonic stenosis who underwent surgical valvotomy, 87% developed pulmonary regurgitation on follow-up echocardiography. Pulmonary regurgitation also commonly occurs after successful repair of tetralogy of Fallot, where it represents the most common indication for reoperation late after repair.3 **Primary causes:** - Congenital abnormalities - Rheumatic disease - Carcinoid syndrome - Myxomatous degeneration - Infective endocarditis - Trauma **Secondary (functional) causes3,5:** - Post-intervention for pulmonic stenosis or tetralogy of Fallot - Pulmonary hypertension with annular dilation - Idiopathic dilated pulmonary trunk - Marfan syndrome ##### **Pathophysiology** Chronic pulmonary regurgitation causes right ventricular volume overload. The right ventricle initially remodels to accommodate the regurgitant flow, and right ventricular stroke volume increases to maintain effective forward blood flow. Over time, this leads to progressive right ventricular dilation and eventual dysfunction. Hemodynamic changes include widened pulmonary artery pulse pressure and low pulmonary artery diastolic pressures. As regurgitation increases, right ventricular end-diastolic pressure becomes elevated, and systemic cardiac output is reduced, especially with exercise.3,5 ##### **Clinical presentation** **Symptoms:** Pulmonary regurgitation is tolerated well for many years, even decades. However, the chronic effects of long-term volume overload eventually impair right ventricular function. Patients often do not notice symptoms until right ventricular dysfunction is severe.9 Symptoms may include: | Category | Findings | | --- | --- | | Symptoms | Exercise intolerance, dyspnea, fatigue, palpitations (atrial/ventricular arrhythmias), signs of right heart failure | | Auscultation | Low-frequency early diastolic murmur at LUSB (often subtle); systolic ejection murmur (residual stenosis or increased stroke volume); absent P2 if prior valvotomy/transannular patch | | Precordium | RV heave | | Other findings | Elevated JVP, hepatomegaly, peripheral edema, ascites (with RV failure) | **Physical examination:** Pulmonary regurgitation is frequently overlooked on physical examination. For example, because the diastolic murmur is soft and short.5,9 **However, findings may include5:** - Soft, low-pitched diastolic murmur at the left upper sternal border - Right ventricular heave - Increased cardiothoracic ratio on chest radiograph - Signs of right heart failure ##### **Diagnostic evaluation** **Echocardiography** provides a qualitative and semi-quantitative assessment of pulmonary regurgitation severity. However, quantitative assessment can be challenging, particularly in the adult congenital heart disease population.3 **Cardiac Magnetic Resonance (CMR)** is the gold standard for evaluating right ventricular volumes, function, and severity of pulmonary regurgitation.3 ##### **Management** **Medical management:** There is no specific medical therapy for pulmonary regurgitation. Management focuses on: - Diuretics for symptomatic relief of congestion - Treatment of arrhythmias - Surveillance for progression **Pulmonary valve replacement:** Pulmonary valve replacement is the definitive treatment for severe pulmonary regurgitation with right ventricular dysfunction.6,10 **Surgical pulmonary valve replacement:** Surgical pulmonary valve replacement typically involves removal of the existing transannular patch or conduit and resection of any aneurysmal dilation of the right ventricular outflow tract, followed by replacement with either a bioprosthetic valve or conduit. The procedure can be performed with low surgical risk (1-2%) in experienced centers.9,11 **Transcatheter Pulmonary Valve Replacement (TPVR):** TPVR has become a common alternative to surgical replacement in patients with a stenotic or regurgitant conduit or bioprosthetic valve.12 TPVR is generally recommended for patients with symptoms and right ventricular outflow tract dysfunction. Indications are currently the same as for surgical pulmonary valve replacement.6 ##### **Natural history and prognosis** The natural history of pulmonary regurgitation depends on its severity and underlying etiology. Mild pulmonary regurgitation may be well-tolerated indefinitely. However, chronic severe pulmonary regurgitation leads to progressive right ventricular dilation and dysfunction over decades. Studies of patients after repair of pulmonic stenosis show that the rate of symptom development increases markedly after age 40.3 Pulmonary valve replacement can lead to improvement in functional class, substantial decrease or normalization of right ventricular volumes, stabilization of QRS duration, and decreased incidence of atrial and ventricular tachyarrhythmias. However, right ventricular ejection fraction may show little change, particularly if intervention is delayed until severe dysfunction has occurred.3,11 ##### **Conclusion** Pulmonary valve disease encompasses a spectrum from congenital pulmonic stenosis to iatrogenic pulmonary regurgitation following interventions for right ventricular outflow tract obstruction. Early recognition of disease progression and appropriate timing of intervention are essential for optimal long-term outcomes in this growing population of adults with congenital heart disease.10 ##### **References** 1\. Marchini F, Meossi S, Passarini G, Campo G, Pavasini R. Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects. _Vascular Health and Risk Management_. 2023. 2\. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease. _Journal of the American College of Cardiology_. 2008. 3\. Bonow RO, Carabello BA, Chatterjee K, et al. 2008 Focused Update Incorporated Into the ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease. _Journal of the American College of Cardiology_. 2008. 4\. Bessière F, Waldmann V, Combes N, et al. Ventricular Arrhythmias in Adults With Congenital Heart Disease, Part I: JACC State-of-the-Art Review. _Journal of the American College of Cardiology_. 2023. 5\. Geva T, Wald RM, Bucholz E, et al. Long-Term Management of Right Ventricular Outflow Tract Dysfunction in Repaired Tetralogy of Fallot: A Scientific Statement From the American Heart Association. _Circulation_. 2024. 6\. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease. _Journal of the American College of Cardiology_. 2019. 7\. Morray BH, McElhinney DB. Semilunar Valve Interventions for Congenital Heart Disease: JACC State-of-the-Art Review. _Journal of the American College of Cardiology_. 2021. 8\. Feltes TF, Bacha E, Beekman RH, et al. Indications for Cardiac Catheterization and Intervention in Pediatric Cardiac Disease: A Scientific Statement From the American Heart Association. _Circulation_. 2011. 9\. Warnes CA. The Adult With Congenital Heart Disease: Born to Be Bad? _Journal of the American College of Cardiology_. 2005. 10\. Bokma JP, Geva T, Sleeper LA, et al. Improved Outcomes After Pulmonary Valve Replacement in Repaired Tetralogy of Fallot. _Journal of the American College of Cardiology_. 2023. 11\. Lee C, Kim YM, Lee CH, et al. Outcomes of Pulmonary Valve Replacement in 170 Patients With Chronic Pulmonary Regurgitation After Relief of Right Ventricular Outflow Tract Obstruction: Implications for Optimal Timing of Pulmonary Valve Replacement. _Journal of the American College of Cardiology_. 2012. 12\. Armstrong AK, Balzer DT, Cabalka AK, et al. One-Year Follow-Up of the Melody Transcatheter Pulmonary Valve Multicenter Post-Approval Study. _JACC: Cardiovascular Interventions_. 2014. ##### **Medical advice disclaimer** DISCLAIMER: THE CONTENT SET FORTH HEREIN DOES NOT PROVIDE MEDICAL ADVICE NOR IS AN ATTEMPT TO PRACTICE MEDICINE The information, including but not limited to, text, graphics, images, and other material contained on this website are for informational purposes only. No material on this website or document are intended to be a substitute for professional medical education, advice, diagnosis, or treatment [Heart Facts](https://www.ekohealth.com/blogs/clinical-education/tagged/heart-facts) #### About the Author Rosalie McDonough, MD, MSc \- Clinician Scientist Rosalie McDonough, MD, MSc, is a clinician scientist with a diverse background in medical research and clinical practice. She earned her Master of Science degree from McGill University in Montreal, Canada, later completing her medical degree at the University Medical Center Hamburg-Eppendorf in Hamburg, Germany. Dr. McDonough’s career has spanned various fields within medicine, focusing on clinical research and health technology. Her interests have encompassed cardiopulmonary disease, lung cancer, molecular cardiology, occupational medicine, and acute ischemic stroke imaging. She is currently the Director of Medical Affairs at Eko Health. - Choosing a selection results in a full page refresh. - Opens in a new window. shop.app # shop.app is blocked This page has been blocked by an extension - Try disabling your extensions. ERR\_BLOCKED\_BY\_CLIENT Reload This page has been blocked by an extension www.ekohealth.com # www.ekohealth.com is blocked This page has been blocked by an extension - Try disabling your extensions. 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