Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 10-04: Pulmonary Valve Stenosis + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ High-pitched systolic ejection murmur maximal in the second left interspace with radiation to the left shoulder P2 delayed and soft or absent Pulmonary ejection click often present and decreases with inspiration—the only right heart sound that decreases with inspiration; all other right heart sounds increase Echocardiography/Doppler is diagnostic Patients with peak pulmonic valve gradient > 64 mm Hg or a mean of 35 mm Hg by echocardiography/Doppler should undergo intervention regardless of symptoms; otherwise, percutaneous or surgical intervention may be indicated for symptoms or evidence of right ventricular (RV) dysfunction +++ General Considerations ++ Often congenital and associated with other cardiac lesions In valvular pulmonic stenosis, pulmonary blood flow preferentially goes to the left lung Most patients with valvular pulmonic stenosis have a domed valve, although some patients have a dysplastic valve Peripheral pulmonic stenosis Can accompany valvular pulmonic stenosis May be part of a variety of clinical syndromes, including the congenital rubella syndrome Noncongenital postoperative pulmonic valvular or main pulmonary artery (PA) stenosis May be seen in patients who have had the Ross procedure for aortic valve disease Occurs due to an immune response in the homograft RV outflow obstructions can also occur when there is A conduit from the RV to the pulmonary artery that becomes stenotic from degenerative changes over time Degeneration of a bioprosthetic replacement pulmonary valve + Clinical Findings Download Section PDF Listen +++ ++ Mild cases of pulmonic stenosis are asymptomatic Moderate to severe pulmonic stenosis may cause Dyspnea on exertion Syncope Chest pain RV failure In mild to moderate pulmonic stenosis A loud ejection click can be heard to precede the murmur This sound decreases with inspiration as the increased RV filling from inspiration prematurely opens the valve during atrial systole when inspiratory increased blood flow to the right heart occurs In severe pulmonic stenosis The second sound is obscured by the murmur Pulmonary component of S2 may be diminished, delayed, or absent A right-sided S4 and a prominent a wave in the venous pulse are present when there is RV diastolic dysfunction or a c-v wave may be observed in the jugular venous pressure if tricuspid regurgitation is present Stenosis of the pulmonary valve or RV infundibulum increases the resistance to RV outflow, raises the RV pressure, and limits pulmonary blood flow + Diagnosis Download Section PDF Listen +++ +++ Imaging ++ Radiography Heart size may be normal A prominent RV and RA or gross cardiac enlargement may be present, depending on the severity Echocardiography/Doppler Diagnostic Can provide evidence for a doming valve versus a dysplastic valve Can estimate the gradient across the valve Peak gradients Mild pulmonic stenosis: < 36 mm Hg Moderate pulmonic stenosis: 36–64 mm Hg Severe pulmonic stenosis: > 64 mm Hg (or mean gradient > 35 mm Hg) MRI and CT do not add additional information unless there is concern regarding associated cardiac lesions or peripheral pulmonary arterial lesions +++ Diagnostic Studies ++ ECG Right axis deviation or RVH is noted Peaked P waves provide evidence of right atrial (RA) overload Catheterization Usually unnecessary for the diagnosis Should be used only if the data are unclear or in preparation for either percutaneous intervention or surgery + Treatment Download Section PDF Listen +++ ++ Class I (definitive) indications for intervention All symptomatic patients All patients with a resting peak-to-peak gradient > 64 mm Hg or a mean gradient > 35 mm Hg, regardless of symptoms Percutaneous balloon valvuloplasty is treatment of choice for domed pulmonary valve stenosis Surgical commissurotomy can be done when pulmonary valve regurgitation is too severe or the valve is dysplastic Percutaneous pulmonary valve replacement Both the Medtronic Melody valve and the Edwards Sapien XT valve are FDA approved Relieves pulmonary outflow tract obstruction due to RV to PA conduit stenosis or following the Ross procedure or due to homograft pulmonary valve stenosis Have also been performed off-label for patients with native pulmonary valve disease, including those who have had tetralogy of Fallot repair (assuming the PA root size is small enough to seat a percutaneous valve) + Outcome Download Section PDF Listen +++ +++ Follow-Up ++ The degree of stenosis worsens with time in a few patients, so serial follow-up is important +++ Prognosis ++ Mild pulmonic stenosis: Patients have a normal life span with no intervention Moderate stenosis May be asymptomatic in childhood and adolescence Symptoms often appear as patients grow older Severe stenosis Rarely associated with sudden death Can cause right heart failure in patients as early as in their 20s and 30s Pregnancy and exercise tend to be well tolerated except in severe stenosis +++ When to Refer ++ All symptomatic patients (regardless of gradient) All asymptomatic patients whose peak pulmonary valve gradient is > 64 mm Hg or whose mean gradient is > 35 mm Hg should be referred to a cardiologist with expertise in adult congenital heart disease + References Download Section PDF Listen +++ + +Boudjemline Y. Percutaneous pulmonary valve implantation: what have we learned over the years? EuroIntervention. 2017 Sep 24;13(AA):AA60–7. [PubMed: 28942387] + +Fathallah M et al. Pulmonic valve disease: review of pathology and current treatment options. Curr Cardiol Rep. 2017 Sep 16;19(11):108. [PubMed: 28916901] + +Hansen RL et al. Long-term outcomes up to 25 years following balloon pulmonary valvuloplasty: a multicenter study. Congenit Heart Dis. 2019 Nov;14(6):1037–45. [PubMed: 31250555] + +Hascoet S et al. Infective endocarditis risk after percutaneous pulmonary valve replacement with the Melody and Sapien valves. JACC Cardiovasc Interv. 2017 Mar 13;10(5):510–7. [PubMed: 28279319]