[Pulmonary stenosis and atresia with intact ventricular septum]

Abstract

Pulmonary atresia and critical pulmonary stenosis with intact ventricular septum includes a wide spectrum of cardiopathies with great morphological heterogeneity. The pulmonary valve may be completely atretic or may contain a puncture hole if stenosis is present. The obstruction may be membranous and/or muscular. All components of the right ventricle can be affected, even the coronary circulation with ventriculocoronary connections and stenosis or atresia of the pulmonary arteries. Prenatal diagnosis is made when the right ventricle is hypoplastic and hypertrophic. The pulmonary valve is thickened and the pulmonary artery is perfused retrogradely through the ductus arteriosus. Right ventriculocoronary connections may sometimes be seen with fetal echocardiography. Postnatal survival depends on the patency of the ductus arteriosus, requiring prostaglandin E1 infusion. When hypoplastic right ventricle and/or ventricle-dependent coronary circulation exists, biventricular circulation is not possible. In these cases, surgical treatment is palliative. In cases with well-developed right ventricle, transcatheter therapy is usually provided with perforation and balloon dilation of the pulmonary valve. In cases of muscular obstruction of the right ventricle outflow tract, surgery may be considered as first-line therapy. In case of prenatal diagnosis, the medical termination of pregnancy is possible when severe right ventricular hypoplasia exists, precluding biventricular circulation. Postnatally, the prognosis of the patients is highly variable, mainly related to the size of the right cavities and the presence of coronary anomalies.