Pulmonic Valve Disease

Bernardo D. Morantte Jr. M.D. Dept. of Medicine College of Medicine Pamantasan Ng Lungsod Ng Maynila

Pulmonic Valve Disease

Pulmonic regurgitation is the back flow of blood from the pulmonary artery into the right ventricle during diastole. It is more often seen in the adult population Pulmonic stenosis is the obstruction in the systolic flow of blood from the right ventricle to the pulmonary artery. .

Pathophysiology of Pulmonic Regurgitation

Pulmonic regurgitation causes right ventricular and eventually also right atrial dilatation.

Secondary dilatation of the tricuspid annulus results in tricuspid regurgitation

Pathophysiology of Pulmonic Regurgitation

Right Heart
RA
Red Arrow = regurgitant jet

PA

Pulmonic valve

Tricuspid valve RV

Etiology of Pulmonic Regurgitation

Pulmonary hypertension of whatever etiology is the most frequent cause of pulmonic regurgitation such as: Chronic obstructive lung disease Large Pulmonary emboli Left sided valvular diseases such as: MS. MR, AS, AR Idiopathic pulmonary hypertension Bacterial endocarditis Marfans syndrome After balloon valvulotomy for pulmonic stenosis and repair of Tetralogy of Fallot

Symptoms
Symptoms related to the primary disease such as dyspnea, orthopnea / PND, cough, wheezing, chest pain Right upper abdominal quadrant pain due to hepatomegaly Peripheral edema Fever in endocarditis

Physical Examination
Key finding: the presence of low to high pitched early diastolic murmur at the 2nd left intercostal space ( Graham Steele murmur) which increases on inspiration. Signs of Pulmonary hypertension P2, sternal lifting, subxyphoid pulsation due to RV dilatation, right sided S3 Other findings: Neck vein distention Hepatomegaly Ascites and Peripheral edema

Diagnostics
EKG _ RV and RA hypertrophy

Chest x-ray *Dilated PA *Prominent right heart border *Obliteration of the retrostrernal space in the left lateral view due to dilated RV *Evidence of pulmonary disease V/Q lung scan when pulmonary emboli is suspected.

Echocardiography
Key Finding: On doppler, a regurgitant jet is present below the pulmonic valve. Elevated pulmonary pressures RV and PA dilatation Other findings depending on etiology such as: bacterial vegetations on the pulmonic valve, congenital anomalies

Medical Therapy
Treatment of the underlying cause or pulmonary disease may alleviate the pulmonary hypertension and reduce the degree of pulmonic regurgitation Treatment for CHF Diuretics Digoxin Vasodilators SBE prophylaxis

Surgical Therapy
Pulmonic valve replacement Pulmonic valve replacement is rarely indicated unless medical therapy fails to improve the symptoms of right heart failure.

Etiology of Pulmonic Stenosis

Congenital_ 95% of cases a. Isolated _ valvular, infundibular, supravalvular b. With other anomalies such as: Tetralogy of Fallot, Noonans syndrome, double outlet right ventricle Carcinoid syndrome

In born errors of metabolism such as mucopolysaccharoidosis, Homocystinuria

Rheumatic heart disease Connective tissue diseases

Pathophysiology of Pulmonic Stenosis

Right Heart
RA

PA Pulmonic valve

Tricuspid valve RV
Red arrow = regurgitant jet

Diffferential Diagnosis
Benign pulmonic flow murmur the murmur is usually short and gr I-II / VI in intensity Atrial septal defect Fixed wide splitting of S2 Prominent pulmonary arterial vasculature in the chest x-ray suggesting the presence of a left to right shunt. VSD blowing holosystolic murmur is usually heard along the left lower sternal border Aortic stenosis the murmur is in the aortic area (2nd RICS): paradoxical splitting of S2

Symptoms
Isolated pulmonic stenosis maybe asymptomatic if pressure gradient across the pulmonic valve is < 40 mm Hg Easifatigability RUQ pain due to hepatomegaly Edema palpitations

Physical examination
Key finding: Gr III-IV / VI harsh crescendo-decrescendo systolic murmur at the 2nd LICS which increases on inspiration S1 with systolic ejection click P2, Right sided S4 present Jugular venous distention with prominent A Hepatomegaly Peripheral edema

Diagnostics
EKG_ RVH with strain pattern, RAH Chest x-ray : Dilated or prominent PA Obliterated retrosternal space in the left lateral view Prominent right heart border

Echocardiogram
Key finding: On doppler an increase in velocity across the pulmonic valve which indicates the presence of a pressure gradient RVH and dilatation RA dilatation Pulmonic valve may appear deformed and rigid

Cardiac Catheterization
Not indicated for diagnosis; maybe performed if other congenital anomalies are suspected

Medical Therapy
Asymptomatic patient only SBE prophylaxis is required Treatment for right sided CHF Diuretics Digoxin Balloon valvulotomy for symptomatic patient with pressure gradient of > 40 mm

* Repeat valvulotomy in 11% of patients in 10 years

Surgery
Pulmonic valve replacement for dysplastic valve with severe stenosis, in Tetralogy of Fallot, and after repair of the Tetralogy

END