Long-term fate of isolated congenital absent pulmonary valve

Absence of Left Pulmonary Artery in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

“…Pulmonary and systemic flow rates were also calculated and ranged between 1.4 and 3.3. Some patients may develop right ventricular dysfunction due to the combination of pulmonary regurgitation and a single pulmonary artery with APVS, which may ultimately result in pulmonary hypertension . This pulmonary hypertension may cause worsening pulmonary regurgitation and right ventricular dysfunction.…”

Absent Pulmonary Valve with Intact Ventricular Septum Presenting as Cardiorespiratory Failure at Birth

“…Although some newborns with absent pulmonary valve syndrome have been successfully treated with porcine valve insertion [1] or an aortic homograft conduit [6], little is known about the long-term outcome following these procedures. Chronic volume overload of the right ventricle in our case might induce right-sided heart failure over the long term [13]. Therefore sequential ultrasonographic evaluation of right ventricular performance is important.…”

“…Although absent pulmonary valve associated with tetralogy of Fallot is known to seriously compromise the airway during early infancy, that with intact ventricular septum has been described mostly in adults [2,10,13]. We are aware of only nine other patients who reportedly became symptomatic during the neonatal period [1, 3, 5-7, 9, 12, 14], all of whom had patent ductus arteriosus.…”

Operative Timing for Absent Pulmonary Valve with Obstructive Sleep Apnea

“…Most adults are asymptomatic and present with exercise intolerance later in life. [1][2][3][4] Right ventricular (RV) dilatation, which refl ects the severity and duration of ventricular insuffi ciency, is usually observed in patients with isolated pulmonary regurgitation. However, severe heart failure due to RV overload is a rare symptom in those without coexisting cardiac or pulmonary factors.…”

“…1,2 Isolated pulmonary regurgitation appears to be well tolerated over a long period, despite substantial regurgitant blood fl ow. [2][3][4] In neonates, this anomaly usually causes severe respiratory distress due to tracheobronchial compression by the dilated pulmonary arteries. Most adults are asymptomatic and present with exercise intolerance later in life.…”