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Valvar, subvalvar, and supravalvar pulmonic stenosis (PS) and peripheral pulmonic stenosis (PPS) in children: Clinical manifestations and diagnosis

Authors
Lynn F Peng, MD
Stanton Perry, MD
Section Editor
David R Fulton, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Pulmonic stenosis (PS) is a common congenital heart defect, characterized by obstruction to flow from the right ventricle to the pulmonary arteries. PS can occur in isolation or be associated with other types of cardiac defects.

The pathophysiology, clinical features, and diagnosis of valvar, subvalvar, supravalvar PS and peripheral PS (PPS) in children will be presented here. The management and prognosis of PS in children are discussed separately. (See "Valvar, subvalvar, and supravalvar pulmonic stenosis (PS) and peripheral pulmonic stenosis (PPS) in children: Management and outcome".)

PS in adults is also discussed separately. (See "Clinical manifestations and diagnosis of pulmonic stenosis in adults" and "Natural history and treatment of pulmonic stenosis in adults" and "Echocardiographic evaluation of the pulmonic valve and pulmonary artery".)

ANATOMY AND EMBRYOLOGY

Anatomy — PS is defined as obstruction to right ventricle (RV) outflow at the level of the pulmonary valve. The obstruction can occur at several different locations, as follows:

Valvar PS – Valvar stenosis is the most common type of PS, and is typically characterized by fused or absent commissures with thickened leaflets of the pulmonary valve. In most patients, the valve is a dome-shaped structure with a small orifice (movie 1) [1].

                        
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Literature review current through: Nov 2017. | This topic last updated: Aug 29, 2017.
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