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

Abstract

The rare combination of tetralogy of Fallot (TOF), absent pulmonary valve syndrome (APVS), and absent left pulmonary artery (ALPA) is reviewed herein. Children with TOF with APVS and ALPA should be closely monitored, even if they are asymptomatic or mildly symptomatic, and should undergo elective surgery at the end of infancy.

Unusual association of isolation of right pulmonary artery in absent pulmonary valve syndrome

“…Even in patients with right aortic arch, arterial duct is often present on the left side, thus making absence of LPA as a common association in APVS. 4,5 Our patient was distinctly different as the isolation and hypoplasia involved the RPA rather than the LPA and this had not been reported earlier. There was marked right lung hypoplasia and ductal continuation of hilar RPA from the undersurface of the right arch.…”

“…1 As the embryonic sixth aortic arch develops into arterial duct distally and mediastinal LPA proximally, they may involute together resulting in APVS with absent LPA. 4 , 5 This neural crest and second heart field anomaly is common in 22q11 deletion mutations. The post hilar LPA may remain hypoplastic and may lead to left lung hypoplasia.…”

“…Even in patients with right aortic arch, arterial duct is often present on the left side, thus making absence of LPA as a common association in APVS. 4 , 5…”

“…3 Absence of left pulmonary artery (LPA) seen in 2% of patients with TOF is more frequent in 14.3% of patients with APVS. 4 , 5 Embryological explanation for this frequent association is the common origin of arterial duct and LPA from embryonic left sixth aortic arch. However, absence of right pulmonary artery (RPA) has not been reported in APVS.…”

Investigating the characteristics of echocardiogram, surgical treatment, chromosome and prognosis for fetal right heart enlargement

“…[17] The absence of pulmonary valve can result in respiratory insufficiency. [18,19] In this study, there were 14 cases with double outlet right ventricle. In the 14 cases with double outlet right ventricle, there were 2 had chromosome abnormalities including one with 18-trisomy syndrome and another with 13-trisomy syndrome.…”