Name
PULMONARY VALVE STENOSIS
DESCRIPTION
DETAIL
CAUSES : β’ Congenital β’ Rubella embryopathy -------------------------------------------------------------------------- DIFFERENTIAL DIAGNOSIS β’ Dysplastic pulmonic valve stenosis β’ Discrete infundibular stenosis β’ Subinfundibular obstruction β’ Isolated pulmonary artery stenosis β’ Supravalvar pulmonary stenosis β’ Tetralogy of Fallot (Pink)* ECG - generally sinus rhythm, occasional supraventricular arrhythmias, tall peaked P waves, rightward axis, severity correlates with R/S ratio in leads V1 and V6, right ventricular hypertrophy IMAGING: . X-ray - post stenotic dilatation of the pulmonary trunk, prominence of right atrium and ventricle . Echocardiogram - mobile dome, thickened pulmonic valve, post stenotic dilatation of the pulmonary trunk, small valve annulus; continuous wave Doppler provides an estimate of the transvalvular gradient . Color-flow Doppler - delineates area of obstruction, defects pulmonary regurgitation DIAGNOSTIC PROCEDURES : . Cardiac catheterization . Not indicated in mild pulmonic stenosis . Essential in severe pulmonic stenosis . Used to assess morphology of the right ventricle, pulmonary outflow tract and the pulmonary arteries . Also used to rule out associated lesions e.g., atrial septal defect (ASD), though echocardiography may suffice.
TYPENOTES
RISK FACTORS : Family historyGENERAL MEASURES: . Though infective endocarditis is rare, SBE prophylaxis is advisable . Diagnostic treatment for critical pulmonary stenosis in newborns . Intervention . None required for mild pulmonic stenosis . Intervention of asymptomatic patients with moderate PS is controversial. At minimum, regular assessment is advisable. SURGICAL MEASURES : Percutaneous balloon valvotomy (preferred) or surgical pulmonic valvotomy required for patient with severe obstruction DRUG(S) OF CHOICE : β’ No specific regimen in the absence of congestive heart failure β’ Endocarditis prophylaxis PATIENT MONITORING : β’ Postoperative (or post-balloon valvotomy) Doppler ultrasound suggested at approximately 1 year after procedure β’ Post valvotomy SBE prophylaxis still required β’ Regular followup assessment for patients not undergoing surgical correction POSSIBLE COMPLICATIONS: β’ Up to 10% late mortality following valvotomy in critical pulmonary stenosis in neonates β’ Slower recovery in those with chronic severe right ventricular hypertrophy β’ Post valvotomy pulmonic regurgitation reported in up to 50% (variable severity) β’ Residual ASD or patent foramen ovale β’ Persistent repolarization abnormalities on ECG associated with severe postoperative pulmonic regurgitation β’ Late atrial arrhythmias EXPECTED COURSE/PROGNOSIS : Outcome following either balloon or surgical valvotomy is excellent in general
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
X-RAY CHEST P.A. VIEW( NORMAL ), COMPLETE BLOOD COUNT, ECHOCARDIOGRAPHY, ECG, CARDIAC DOPPLER