Anomalous origin of the right pulmonary artery from the ascending aorta: successful surgical repair

A case of isolated anomalous origin of the right pulmonary artery from the ascending aorta with severe pulmonary hypertension is reported. Surgical repair was achieved by anastomosis of the anomalous vessel to the pulmonary artery trunk using a continuous absorbable suture. Special emphasis is laid on the favorable postoperative evolution of the pulmonary hypertensive disease following the successful repair. The poor natural history of this uncommon congenital defect if untreated makes early corrective surgery imperative.     

Anomalous origin of the right pulmonary artery from the ascending aorta. Its surgical correction in early infancy]

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation associated with a high mortality during the first year of life. Up to 1990, 75 cases have been reported in the literature. We are reporting 2 cases diagnosed and operated before 3 months of age. Repair was facilitated by the use of deep hypothermic circulatory arrest. The duration of follow up is 5 and 6 years, respectively. Pre and postoperative cardiac catheterization pressure data and angiograms are shown. We review the literature, presenting the physiopathological, embryological and surgical features of this rare congenital anomaly.     

Anomalous origin of the left pulmonary artery from the ascending aorta. Successful surgical correction in an infant with Fallot's tetralogy

We describe the case of a 40-day-old female patient with a history of breathlessness since birth who was referred to our hospital for surgical correction of common arterial trunk. The invasive investigation disclosed a Fallot cent s tetralogy anatomy associated with an anomalous origin of the left pulmonary artery from the ascending aorta. Immediately after diagnosis, the patient underwent a successful total surgical correction of the defect, including simultaneous anastomosis of the left pulmonary artery to the pulmonary trunk.     

Prenatal diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta

We report a case of anomalous origin of the right pulmonary artery from the ascending aorta that was diagnosed by fetal ultrasound at 21 weeks of gestation. The clue to the diagnosis was present in the three-vessel view, this being one of the views that we use for fetal cardiac screening. The anomaly was corrected surgically at 11 days of age. We discuss the importance of prenatal diagnosis in the management of this rare anomaly.     

Single origin of right and left pulmonary arteries from ascending aorta, with main pulmonary artery from right ventricle.

A previously undescribed anomaly is presented in which right and left pulmonary arteries arise via a single vessel from the ascending aorta while the main pulmonary artery arises normally from the right ventricle. This main pulmonary artery has no branches supplying the lungs and connects via a ductus arteriosus to the descending aorta. Additional anomalies were an atrial septal defect and tubular hypoplasia of the aortic isthmus.     

Radionuclide diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta (so-called hemitruncus)

A 5-week-old infant presented with signs of severe congestive heart failure and pulmonary hypertension. Injection of technetium-99m pertechnetate demonstrated anomalous perfusion of the right lung. Subsequently, anomalous origin of the right pulmonary artery from the ascending aorta was proven at cardiac catheterization and repaired. Repeat injection of radionuclide 1 week postoperatively demonstrated normal flow to the right lung.     

Abnormal origin of the right pulmonary artery from the ascending aorta. Echocardiographic diagnosis apropos of 2 cases

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation. Formerly identified at angiography and during surgery, it can now be diagnosed by two-dimensional echocardiography, as was done in the two cases reported here. The first case was a neonate in heart failure in whom echocardiography revealed the anomalous origin of the right pulmonary artery; this diagnosis was confirmed at post-mortem examination. The second case was a 4-month old infant in heart failure; echocardiography displayed the malformation which was confirmed at angiography and surgery. In both cases echocardiography showed discontinuity between the pulmonary trunk and the right pulmonary artery and continuity between the pulmonary trunk and the left pulmonary artery. There also was continuity between the right pulmonary artery and the ascending aorta. Doppler ultrasound showed a systolic and a diastolic flow in the right pulmonary artery and only a systolic flow in the left pulmonary artery. A colour-coded doppler examination in one case confirmed the anomalous origin of the right pulmonary artery and the absence of any other abnormality. Two-dimensional echocardiography combined with colour-coded doppler ultrasound therefore is the method of choice to diagnose anomalous origin of the right pulmonary artery from the ascending aorta.     

Anomalous origin of the right pulmonary artery from the ascending aorta: repair without cardiopulmonary bypass

Three cases of anomalous aortic origin of the right pulmonary artery are reported. All patients presented with severe cardiac failure, two of them in the neonatal period. Clear visualization of the anomalous origin of the right pulmonary artery was obtained by cross-sectional echocardiography and the diagnosis was confirmed by cardiac catheterization. All patients underwent correction without the aid of cardiopulmonary bypass. The continuity between main and right pulmonary arteries was established by a polytetrafluoroethylene graft. One early death was due to bleeding and, probably, a pulmonary vasoconstriction crisis. In all patients the right ventricular pressure was systemic or suprasystemic before surgery and dropped to near normal in the operating room after correction. Two to five years after surgery the survivors are well. This rare, potentially lethal anomaly is amenable to correction; however, diagnosis should be followed by immediate surgical treatment.     

Surgical repair of the anomalous origin of the right pulmonary artery from the ascending aorta

The anomalous origin of the right pulmonary artery (AORPA) from the ascending aorta is a rare congenital malformation. We describe an infant who underwent a surgical correction with direct anastomosis between the right pulmonary artery and the pulmonary trunk. Eighteen months later, the patient remains asymptomatic, and no significant residual stenosis was detected on angioresonance.     

Anomalous origin of one pulmonary artery from the ascending aorta

We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions. The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. PATIENTS AND METHODS: Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. RESULTS: At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prolonged intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessful balloon dilation, underwent surgical reoperation. CONCLUSIONS: Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.     

Anomalous origin of the right coronary artery from the ascending aorta

A 47 yr-old man had an anomalous origin of the right coronary artery from the left anterior wall of the ascending aorta. This was demonstrated by aortography and by selective right coronary angiography.     

Anomalous origin of the left pulmonary artery from the ascending aorta in a patient with tetralogy of Fallot and "absent pulmonary valve".

The rare association of tetralogy of Fallot, rudimentary formation of the leaflets of the pulmonary valve, and anomalous origin of the left pulmonary artery from the ascending aorta is described in a two-month-old infant. The diagnosis was made by cardiac catheterisation and angiography.     

Echocardiography findings in isolated anomalous origin of the right pulmonary artery from the ascending aorta]

Origin of the right pulmonary artery from the ascending aorta is extremely rare. We describe the echocardiographic findings of this lesion in a single case. The absence of an image of the right pulmonary artery in the echocardiographic short axis view at the level of the great arteries, is highly suggestive of this lesion. Differentiation from truncus arteriosus, aorto-pulmonary window and transposition of the great arteries must, and can be made by a comprehensive echocardiographic study.     

Double anomalous coronary origin from the pulmonary artery: successful surgical correction in an infant

We report an extremely rare entity in which all coronary arteries originate from the pulmonary artery. Only a few cases have been reported and corrected, and fewer still have had a favorable outcome. The survival range reported for these patients is from 9 hours to 1 year. This case was diagnosed and surgically corrected in a patient at the age of 2 months, and the patient is doing well 27 months after the procedure.     

Anomalous origin of the right coronary artery from the pulmonary artery associated with tetralogy of Fallot: description of the pre-surgical diagnosis and surgical repair

Anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital defect. We describe the case of an infant with anomalous origin of the right coronary artery from the pulmonary artery in association with tetralogy of Fallot. This patient had a pre-operative echocardiographic diagnosis, which was confirmed by angiography, and later underwent a successful surgical repair.