6.10: Pulmonary Valve Stenosis
- Page ID
- 42760
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\(\newcommand{\avec}{\mathbf a}\) \(\newcommand{\bvec}{\mathbf b}\) \(\newcommand{\cvec}{\mathbf c}\) \(\newcommand{\dvec}{\mathbf d}\) \(\newcommand{\dtil}{\widetilde{\mathbf d}}\) \(\newcommand{\evec}{\mathbf e}\) \(\newcommand{\fvec}{\mathbf f}\) \(\newcommand{\nvec}{\mathbf n}\) \(\newcommand{\pvec}{\mathbf p}\) \(\newcommand{\qvec}{\mathbf q}\) \(\newcommand{\svec}{\mathbf s}\) \(\newcommand{\tvec}{\mathbf t}\) \(\newcommand{\uvec}{\mathbf u}\) \(\newcommand{\vvec}{\mathbf v}\) \(\newcommand{\wvec}{\mathbf w}\) \(\newcommand{\xvec}{\mathbf x}\) \(\newcommand{\yvec}{\mathbf y}\) \(\newcommand{\zvec}{\mathbf z}\) \(\newcommand{\rvec}{\mathbf r}\) \(\newcommand{\mvec}{\mathbf m}\) \(\newcommand{\zerovec}{\mathbf 0}\) \(\newcommand{\onevec}{\mathbf 1}\) \(\newcommand{\real}{\mathbb R}\) \(\newcommand{\twovec}[2]{\left[\begin{array}{r}#1 \\ #2 \end{array}\right]}\) \(\newcommand{\ctwovec}[2]{\left[\begin{array}{c}#1 \\ #2 \end{array}\right]}\) \(\newcommand{\threevec}[3]{\left[\begin{array}{r}#1 \\ #2 \\ #3 \end{array}\right]}\) \(\newcommand{\cthreevec}[3]{\left[\begin{array}{c}#1 \\ #2 \\ #3 \end{array}\right]}\) \(\newcommand{\fourvec}[4]{\left[\begin{array}{r}#1 \\ #2 \\ #3 \\ #4 \end{array}\right]}\) \(\newcommand{\cfourvec}[4]{\left[\begin{array}{c}#1 \\ #2 \\ #3 \\ #4 \end{array}\right]}\) \(\newcommand{\fivevec}[5]{\left[\begin{array}{r}#1 \\ #2 \\ #3 \\ #4 \\ #5 \\ \end{array}\right]}\) \(\newcommand{\cfivevec}[5]{\left[\begin{array}{c}#1 \\ #2 \\ #3 \\ #4 \\ #5 \\ \end{array}\right]}\) \(\newcommand{\mattwo}[4]{\left[\begin{array}{rr}#1 \amp #2 \\ #3 \amp #4 \\ \end{array}\right]}\) \(\newcommand{\laspan}[1]{\text{Span}\{#1\}}\) \(\newcommand{\bcal}{\cal B}\) \(\newcommand{\ccal}{\cal C}\) \(\newcommand{\scal}{\cal S}\) \(\newcommand{\wcal}{\cal W}\) \(\newcommand{\ecal}{\cal E}\) \(\newcommand{\coords}[2]{\left\{#1\right\}_{#2}}\) \(\newcommand{\gray}[1]{\color{gray}{#1}}\) \(\newcommand{\lgray}[1]{\color{lightgray}{#1}}\) \(\newcommand{\rank}{\operatorname{rank}}\) \(\newcommand{\row}{\text{Row}}\) \(\newcommand{\col}{\text{Col}}\) \(\renewcommand{\row}{\text{Row}}\) \(\newcommand{\nul}{\text{Nul}}\) \(\newcommand{\var}{\text{Var}}\) \(\newcommand{\corr}{\text{corr}}\) \(\newcommand{\len}[1]{\left|#1\right|}\) \(\newcommand{\bbar}{\overline{\bvec}}\) \(\newcommand{\bhat}{\widehat{\bvec}}\) \(\newcommand{\bperp}{\bvec^\perp}\) \(\newcommand{\xhat}{\widehat{\xvec}}\) \(\newcommand{\vhat}{\widehat{\vvec}}\) \(\newcommand{\uhat}{\widehat{\uvec}}\) \(\newcommand{\what}{\widehat{\wvec}}\) \(\newcommand{\Sighat}{\widehat{\Sigma}}\) \(\newcommand{\lt}{<}\) \(\newcommand{\gt}{>}\) \(\newcommand{\amp}{&}\) \(\definecolor{fillinmathshade}{gray}{0.9}\)Etiology and Pathology
Pulmonary valve stenosis can be caused congenital, carcinoid and rheumatic disorders or extrinsic compression. The typical dome-shaped pulmonary valve stenosis is the most common form of right ventricular outflow tract obstruction. Stenosis is caused by fusion of the pulmonary valve leaflets and a narrowed central orifice. The valve is usually mobile and associated with medial abnormalities and dilation of the pulmonary trunk. Pulmonary valve stenosis may be associated with Noonan, Williams, Alagille, Keutel or rubella syndromes.[56]
Clinical Presentation
Most patients with mild to moderate pulmonary valve stenosis are asymptomatic. Severe pulmonary valve stenosis may cause exertional dyspnea and fatigue, chest pain, palpitations and syncope. On physical examination, thrill along the left sternal edge, and a long systolic ejection murmur with late peak may be appreciated. S2 may be widely split with reduced or absent P2.
Diagnostic Options
Chest Radiography
Chest radiography may show dilated pulmonary arteries, occasionally with calcification of the pulmonic valve. In case of severe pulmonary valve stenoisis, oligemic lung fields can be seen.
Electrocardiography
Right ventricular hypertrophy can be seen on electrocardiography as an axis deviation to the right. Right bundle branch block may also be present. Patients with Noonan syndrome invariably have a left bundle branch block.
Echocardiography
Transthoracic echocardiography confirms the diagnosis. The level of obstruction, valvular, supravalvular or right ventricular outflow tract, can be identified. Valvular stenosis is characterized by mildly thickened leaflets with restricted systolic excursion. The pressure gradient can be measured. Associated cardiac conditions can be demonstrated with echocardiography.
Cardiac catheterization
Cardiac catheterization is not necessary to diagnose pulmonic valve stenosis. Hemodynamic consequences and severity of pulmonary valve stenosis can be assessed with cardiac catheterization.
Treatment
Invasive intervention is recommended in case of symptomatic disease, or when the gradient across the valve is >40 mmHg.
Medical
Supportive and symptomatic treatment of right ventricular failure is recommended.
Surgical
The treatment of choice for stenosis at the valvular level is balloon valvuloplasty. Long-term results are satisfactory and the procedure relatively safe. Surgical valvotomy is very effective with minimal recurrence, however significant pulmonary regurgitation may occur. Pulmonary valve replacement is indicated if the patient is not suitable for balloon valvuloplasty or surgical valvotomy.