Absent pulmonary valve syndrome in infancy: Surgery reconsidered

“Absent” pulmonary valve with atrial septal defect and patent ductus arteriosus

“…Through advances in the techniques of myocardial support and cardiopulmonary bypass, it is now feasible, and we believe preferable, to attempt total correction in symptomatic infants with APV, thus obviating the need for palliative procedures [4] . Because of reported sudden deaths even in "asymptomatic" infants [4], aggressive management of respiratory compromise, pulmonary hypertension, and RV failure is the preferred approach .…”

“…Because of reported sudden deaths even in "asymptomatic" infants [4], aggressive management of respiratory compromise, pulmonary hypertension, and RV failure is the preferred approach . Several areas of debate exist as to the necessity for either pulmonary valve insertion or pulmonary artery plication (aneurysmorrhaphy) .…”

“…[11] and Dunnigan et al . [4] published the data from three infants in whom pulmonary artery plications were performed intraoperatively . This procedure was thought to be helpful in the reduction of respiratory embarrassment from vascular compression of the airways .…”

Tetralogy of Fallot with Absent Pulmonary Valve and Bronchial Compression: Treatment with Endobronchial Stents

“…The relief of airway obstruction in APVS by extensive pulmonary artery aneurysmorrhaphy [5,12] has been reported. Success after aneurysmorrhaphy is variable [5,12] and does not always alleviate the airway obstruction.…”

“…Success after aneurysmorrhaphy is variable [5,12] and does not always alleviate the airway obstruction. Endoscopic placement of bronchial stents to maintain airway patency has been used in older children and adults for etiologies as varied as endobronchial tuberculosis, pulmonary malignancies, and inoperable mediastinal tumors [3,10,11].…”