(See also Overview of Cardiac Valvular Disorders Overview of Cardiac Valvular Disorders Any heart valve can become stenotic or insufficient (also termed regurgitant or incompetent), causing hemodynamic changes long before symptoms. Most often, valvular stenosis or insufficiency... read more .)
Etiology of Pulmonic Stenosis
Pulmonic stenosis is most often congenital and affects predominantly children; stenosis may be valvular or just below the valve in the outflow tract (infundibular). It commonly is a component of tetralogy of Fallot Tetralogy of Fallot Tetralogy of Fallot consists of 4 features: a large ventricular septal defect, right ventricular outflow tract obstruction and pulmonic valve stenosis, right ventricular hypertrophy, and over-riding... read more .
Less common causes are Noonan syndrome Primary hypogonadism Male hypogonadism is decreased production of testosterone, sperm, or both or, rarely, decreased response to testosterone, resulting in delayed puberty, reproductive insufficiency, or both. Diagnosis... read more (a familial syndrome similar to Turner syndrome but with no chromosomal defect) and carcinoid syndrome Carcinoid Syndrome Carcinoid syndrome develops in some people with carcinoid tumors and is characterized by cutaneous flushing, abdominal cramps, and diarrhea. Right-sided valvular heart disease may develop after... read more in adults.
Symptoms and Signs of Pulmonic Stenosis
Many children with pulmonic stenosis remain asymptomatic for years and do not present to a physician until adulthood. Even then many patients remain asymptomatic. When symptoms of pulmonic stenosis develop, they resemble those of aortic stenosis Aortic Stenosis Aortic stenosis (AS) is narrowing of the aortic valve, obstructing blood flow from the left ventricle to the ascending aorta during systole. Causes include a congenital bicuspid valve, idiopathic... read more (syncope, angina, dyspnea).
Visible and palpable signs reflect the effects of right ventricular (RV) hypertrophy and include a prominent jugular venous a wave (due to forceful atrial contraction against a hypertrophied RV), an RV precordial lift or heave, and a left parasternal systolic thrill at the 2nd intercostal space.
Widened 2nd heart sound (S2) and delayed pulmonic component of S2 (P2)
Harsh crescendo-decrescendo ejection murmur
On auscultation, the 1st heart sound (S1) is normal and the normal splitting of the S2 is widened because of prolonged pulmonic ejection (P2 is delayed). In RV failure and hypertrophy, the 3rd and 4th heart sounds (S3 and S4) are rarely audible at the left parasternal 4th intercostal space. A click in congenital PS is thought to result from abnormal ventricular wall tension. The click occurs early in systole (very near S2) and is not affected by hemodynamic changes. A harsh crescendo-decrescendo ejection murmur is audible and is heard best at the left parasternal 2nd (valvular stenosis) or 4th (infundibular stenosis) intercostal space with the diaphragm of the stethoscope when the patient leans forward.
Unlike the aortic stenosis murmur, a pulmonic stenosis murmur does not radiate, and the crescendo component lengthens as stenosis progresses. The murmur grows louder immediately with Valsalva release and with inspiration; the patient may need to be standing for this effect to be heard.
Diagnosis of Pulmonic Stenosis
Diagnosis of pulmonic stenosis is confirmed by Doppler echocardiography, which can characterize the severity as
Mild: Peak gradient < 36 mm Hg (peak velocity < 3 m/second)
Moderate: Peak gradient 36 to 64 mm Hg (peak velocity 3 to 4 m/second)
Severe: Peak gradient > 64 mm Hg (peak velocity > 4 m/second)
ECG Electrocardiography The standard electrocardiogram (ECG) provides 12 different vector views of the heart’s electrical activity as reflected by electrical potential differences between positive and negative electrodes... read more may be normal or show RV hypertrophy or right bundle branch block.
Right heart catheterization Cardiac Catheterization Cardiac catheterization is the passage of a catheter through peripheral arteries or veins into cardiac chambers, the pulmonary artery, and coronary arteries and veins. Cardiac catheterization... read more is indicated only when 2 levels of obstruction are suspected (valvular and infundibular), when clinical and echocardiographic findings differ, or before intervention is done.
Treatment of Pulmonic Stenosis
Sometimes balloon valvuloplasty
Prognosis of pulmonic stenosis without treatment is generally good and improves with appropriate intervention.
Treatment of pulmonic stenosis is balloon valvuloplasty, which is indicated for symptomatic patients with moderate or severe valvular stenosis and for asymptomatic patients with severe stenosis.
Percutaneous valve replacement may be offered at highly selected congenital heart centers, especially for younger patients or those with multiple previous procedures, in order to reduce the number of open heart procedures. When surgical replacement is necessary, bioprosthetic valves are preferred due to the high rates of thrombosis of right-sided mechanical heart valves; anticoagulation is temporarily required (see Anticoagulation for patients with a prosthetic cardiac valve Anticoagulation for patients with a prosthetic cardiac valve Any heart valve can become stenotic or insufficient (also termed regurgitant or incompetent), causing hemodynamic changes long before symptoms. Most often, valvular stenosis or insufficiency... read more ).
Pulmonic stenosis is usually congenital, but symptoms (eg, syncope, angina, dyspnea) usually do not appear until adulthood.
Heart sounds include increased splitting of S2 and a harsh crescendo-decrescendo ejection murmur heard best at the left parasternal 2nd or 4th intercostal space when the patient leans forward; the murmur grows louder immediately with Valsalva release and with inspiration.
Balloon valvuloplasty is done for symptomatic patients and asymptomatic patients with normal systolic function and a peak gradient > 40 to 50 mm Hg.
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