Pulmonic Valve Disease

Bernardo D. Morantte Jr. M.D.Dept. of Medicine

College of MedicinePamantasan 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.

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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 HeartRA

RV

PA

Tricuspid valve

Pulmonic valve

Red Arrow = regurgitant jet

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 Marfan’s 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 dilatationOther 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, Noonan’s syndrome, double outlet right ventricle

Carcinoid syndrome

In born errors of metabolism such as mucopolysaccharoidosis, Homocystinuria

Rheumatic heart diseaseConnective tissue diseases

Pathophysiology of Pulmonic StenosisRight Heart

RA

RV

PA

Tricuspid valve

Pulmonic valve

Red arrow = regurgitant jet

Diffferential DiagnosisBenign pulmonic flow murmur

the murmur is usually short and gr I-II / VI in intensityAtrial 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 requiredTreatment 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