A Rare Case of Adult Aortopulmonary Window Combined with Anomalous Origin of the Right Pulmonary Artery from the Aorta Leading to Eisenmenger Syndrome.

Aortopulmonary window is a rare congenital heart disease that can increase pulmonary vascular resistance, exacerbate left-to-right shunt and lead to heart failure and respiratory tract infections. Most patients die during childhood. We report a 53-year-old male patient with a large aortopulmonary window combined with anomalous origin of the right pulmonary artery from the aorta, with Eisenmenger syndrome and without surgery.

Late diagnosis of anomalous right pulmonary artery originated from ascending aorta: Associated with small pulmonary artery aneurysm.

Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) is an extremely rare congenital cardiac malformation, commonly observed early in life. Patients with AORPA usually present with nonspecific clinical symptoms, commonly associated with other congenital cardiac anomalies and some complications as pulmonary hypertension and heart failure. A high index of clinical suspicion and noninvasive diagnostic imaging studies play an important role in diagnosis and preventing high mortality rates. We report a case of anomalous origin of the right pulmonary artery from the ascending aorta in a 15-year-old girl that was diagnosed with severe pulmonary hypertension, pulmonary hemorrhage, and small pulmonary artery aneurysm. Clinical, radiographic imaging and other investigations' findings are described. To the best of our knowledge, this is the first reported case of anomalous origin of the right pulmonary artery from the ascending aorta with large patent ductus arteriosus associated with a small pulmonary artery aneurysm.

Type A interrupted aortic arch and type III aortopulmonary window with anomalous origin of the right pulmonary artery from the aorta.

Interruption of the aortic arch, aortopulmonary window, and anomalous origin of the right pulmonary artery from the ascending aorta are very rare congenital anomalies. It is even rarer to have all three anomalies in the same setting. We present a case of a newborn who was diagnosed with these lesions and describe the primary repair of these anomalies.

Staged Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta in a Low Birth Weight Infant : A Surgical Option for the Relief of the Postoperative Right Pulmonary Artery Stenosis / 日本心臓血管外科学会雑誌

A premature boy was born after 35 weeks gestation (1,561 g in weight) with a diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) and perimembranous ventricular septal defect (VSD). The fourteenth day after birth, banding of the right pulmonary artery was performed as a palliative operation. At the age of 5 months (3.9 kg in weight), the right pulmonary artery branched from the ascending aorta, and was anastomosed to a flap made by the lateral wall of the main pulmonary artery with pericardial patch augmentation. VSD patch closure was performed concomitantly. Severe stenosis of the right pulmonary artery compressed by the ascending aorta and left pulmonary hypertension were revealed 3 weeks after the repair. At the age of 11 months, a surgical relief of the right pulmonary artery stenosis was performed. Transection of the ascending aorta provided an excellent exposure of the right posterior pulmonary artery. After patch plasty of the stenotic pulmonary artery, the divided ascending aorta was restored using a strip form patch on 4/5 circle of its posterior wall to extend the aorta and widen the space for the right pulmonary artery. This technique preserves growth potential of the ascending aorta. There are few reports of surgical repair of AORPA with VSD in low birth weight infants. We presented here a case with surgical relief of post-operative right pulmonary artery stenosis. Long term observation of repaired right pulmonary artery, and requiring residual slight hypertension of the left pulmonary artery.

Double ductus arteriosus and anomalous origin of the right pulmonary artery from the right-sided duct.

Double ductus arteriosus with anomalous origin of the right pulmonary artery is a rare congenital heart disease. Echocardiography is the first-choice method to diagnose pulmonary arteries and presence of the arterial ductus. Computed tomography (CT) angiography confirms the final diagnosis of the anomalous origin of the pulmonary artery. We report a case diagnosed using 40-slice CT angiography where this case demonstrated presence of both ducts and an anomalous origin of right pulmonary artery from the right-sided ductus arteriosus. .

Two-Stage Repair of Absence of Right Pulmonary Artery / 日本心臓血管外科学会雑誌

Isolated unilateral absence of the pulmonary artery without any intracardiac anomaly is a rare congenital cardiovascular disorder. We performed a successful anatomical repair after systemic-to-pulmonary shunt. The patient was a 1-day-old boy who was transferred to our institution because of continuous murmur. Cardiac echography revealed anomalous origin of the right pulmonary artery from the ascending aorta (AORPA). This aorto-right pulmonary arterial blood flow, however, disappeared in the following 2 days. We altered the diagnosis to right unilateral absence of pulmonary artery (UAPA) from AORPA. A right systemic-to-pulmonary shunt using a 3 mm polytetrafluoroethylene (PTFE) graft was placed, aiming for growth of the right pulmonary artery at the age of 8 days. Forty days after the initial surgery, he underwent a definitive procedure. The right pulmonary artery was anatomically reconstructed with an 8-mm PTFE graft. He was discharged in excellent condition on postoperative day 41. Cardiac catheterization, 1 year later, showed the surgically created right pulmonary artery was patent. A pulmonary perfusion scintigraphy showed satisfactory blood distribution in the right lung.

One-stage total repair of anomalous origin of right pulmonary artery from aorta by the double-flap technique, followed by coarctation repair using extended end-to-end arch reconstruction.

The anomalous origin of the right pulmonary artery from the ascending aorta combined with coarctation of aorta is a rare congenital malformation. The method chosen for performing a prompt surgery to correct the multiple disease lesions is important. Here we report one-stage surgical strategy which involved a double-flap technique alongside an extended end-to-end arch reconstruction in a newborn baby.