Congenital unilateral absence of a pulmonary artery

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

“…The RVOT gradient ranged between 40 and 80 mmHg in 14 patients. 3,8,[17][18][19][20][21] Pressure values were not determined in two patients. 3,[15][16] The RVOT gradient of the patient on whom we operated was measured as 70 mmHg, whereas the gradient for the other patient was 50 mmHg.…”

“…14 the literature review, though other disease combinations were excluded. 3,8,[15][16][17][18][19][20][21] The true absence of a right pulmonary artery in association with APVS has not yet been encountered in the literature. 3 Patients with ALPA from whom data were obtained were symptomatic and exhibited dyspnea and cyanosis.…”

“…16,17,20,21 In 10 patients, the diagnosis of ALPA was suspected based on chest radiographs demonstrating reduced vascularity and hypoplasia of the left lung. 8,[16][17][18]21 In all patients, cardiac catheterization and angiography demonstrated ALPA and marked dilatation of the right pulmonary artery. The RVOT gradient ranged between 40 and 80 mmHg in 14 patients.…”

“…PA systolic pressure was measured in 11 patients and ranged between 18 and 52 mmHg. 3,[17][18][19][20][21] 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.…”

“…Severe hypoplasia occurs in the pulmonary artery due to closure of the ductus after birth . Only 16 patients with APVS and TOF with true (complete) absence of the left pulmonary artery were included in the literature review, though other disease combinations were excluded . The true absence of a right pulmonary artery in association with APVS has not yet been encountered in the literature…”