The anomalous origin of the branch pulmonary artery from the ascending aorta

Abstract

The anomalous origin of one pulmonary artery branch from the aorta (AOPA) is rare. We report our single-institution surgical experience with this condition. Between January 1994 and February 2011, 17 patients (age: 1 month-25 years) with AOPA underwent surgery at our institute. Thirteen patients had an anomalous origin of the right pulmonary artery (RPA) while four had an anomalous origin of the left pulmonary artery (LPA) from the aorta. In patients with anomalous RPA, 11 patients had the proximal type and two patients had the distal type of AOPA. Four patients had associated Tetralogy of Fallot (TOF). In 14 patients, direct implantation into the main pulmonary artery was performed, while three patients required interpositon of a graft. There was one operative death due to persistent hypoxia in a 7-month old child with TOF and an anomalous LPA from the aorta. At a median follow-up of 36.5 months (range: 2-192 months), all 16 survivors were asymptomatic. On echocardiography, two patients showed a gradient of 25 and 30 mmHg across the anastomosis and are being followed up. In our experience, early repair of AOPA results in acceptable haemodynamic and anatomic results. Long-term survival can be expected with a low incidence of re-operation or re-intervention.

Figure 1:

Preoperative chest X-ray of Patient No. 12 shows cardiomegaly with hypervascularity of the right lung with features of pulmonary arterial hypertension. The left lung is hypovascular.

Figure 2:

Preoperative and postoperative chest X-rays of Patient No. 6. (A) The preoperative chest X-ray shows an increased vascularity of the right lung compared with the left lung with cardiomegaly. (B) The postoperative chest X-ray shows a decrease in heart size with normal vascularity of both the lungs.

Figure 3:

Cine-angiograms of Patient No. 9 show (A) the right pulmonary artery arising from the ascending aorta and (B) the left pulmonary artery arising normally from the right ventricle.

Figure 4:

Cine-angiograms of Patient No. 15 with hemitruncus and Tetralogy of Fallot showing (A) left pulmonary artey arising from the aorta and (B) a hypoplastic right pulmonary artery arising from the right ventricle.

Figure 5:

A cine-angiogram of Patient No. 14 with hemitruncus and Tetralogy of Fallot showing the left pulmonary artery arising from the right ventricle. The right pulmonary artery could not be entered and was visualized only on CT angiogram.

Figure 6:

CT angiograms of Patient No. 14. (A) Volume rendered (VR) reconstruction of the CT angiogram (CTA) in a left anterior oblique and cranial angulation, showing the main (*) and the left pulmonary artery (arrow). (B) VR reconstruction of CTA from a posterior vantage, showing the stump (arrow) of the occluded right pulmonary artery (RPA) arising from the ascending aorta (AA). # indicates the reformed distal RPA.