Anomalous origin of the right pulmonary artery from the ascending aorta.

Five cases of anomalous origin of the right pulmonary artery from the ascending aorta were seen at our hospital. Patients 1 and 2 had an intact right pulmonary artery originating from a right posterior aspect (proximal form), and primary anastomosis of the right pulmonary artery and main pulmonary trunk was performed. Patients 3 and 4 showed a narrowing right pulmonary artery originating from near the base of the innominate artery (distal form). The stenotic right pulmonary artery was reconstructed with an 8-mm graft in patient 3, whereas patient 4 became inoperable because complete obstruction had developed in the right pulmonary artery during the 3 months that the patient was waiting to undergo operation. In patient 5, primary anastomosis was undertaken, but morphologically the anomalous origin was of the distal form, so the occurrence of stenosis in the reconstructed right pulmonary artery was a matter of concern. The morphological type was found to be related to the surgical options in this anomaly. Therefore, primary anastomosis was considered the best choice for a correction of the proximal form, whereas a graft interposition with a resection of the stenotic portion on the right pulmonary artery was deemed preferable in the distal form.     

Neonatal repair of anomalous origin of the right pulmonary artery from the ascending aorta. A case report and review in the literature

A seven-day-old neonate presented with respiratory distress and an early diagnosis was achieved through the echocardiographic studies which successfully visualized an anomalous origin of the right pulmonary artery arising from the ascending aorta. Subsequently a surgical correction with direct anastomosis of the right pulmonary artery to the main pulmonary trunk was accomplished in the neonatal period. The English literature in the last two decades is reviewed to discuss the characteristics, the diagnosis and treatment of neonatal cases with anomalous origin of the right pulmonary artery arising from the ascending aorta.     

New technique in the transfer of an anomalously originated left coronary artery to the aorta.

A 21-month-old girl with an anomalous origin of the left coronary artery underwent a transfer of the left coronary artery to the aorta using a new technique of coronary prolongation. Because the anomalous left coronary artery arising from the left anterior aspect of the pulmonary trunk was too short to reach the aorta, a simple transfer of the left coronary artery to the aorta was deemed impossible. Therefore, a transfer was performed with the help of a coronary prolongation technique using the cuff of the pulmonary trunk and an aortic flap. The new route of the left coronary artery was established anterior to the pulmonary trunk. Postoperative angiography showed a patent left coronary artery without any narrowing or kinking, as well as an improved contractility of the left ventricle. Postoperative cardiac scintigraphy showed a decreased ischemic area. This technique is thus considered applicable when the orifice of the anomalous left coronary artery is too distant from the aorta for a direct anastomosis.     

Anomalous origin of the right pulmonary artery from the ascending aorta with fibrous continuity to the pulmonary trunk

A 1-month-old patient was brought to our institution with clinical signs of pulmonary hypertension. Cross-sectional echocardiography suggested a diagnosis of aortopulmonary window. At the time of surgery, we found that the right pulmonary artery was arising anomalously from the left side of the ascending aorta, but was also connected to the pulmonary trunk by a fibrous cord. We reimplanted the right pulmonary artery into the pulmonary trunk, closing the resultant opening in the ascending aorta by direct suture. The postoperative course was uneventful. On follow-up, the patient is asymptomatic without medication.     

Aberrant right pulmonary artery and double outlet ventricle: one-stage repair

We report a 13-month-old male child with anomalous origin of the right pulmonary artery from the ascending aorta and a double outlet right ventricle. Aortic wall was used for elongation of the pulmonary artery and side-to-end connection to the pulmonary trunk. Special emphasis is made on this particular operative technique for strictly laterally originating right pulmonary artery that requires no prosthetic material, avoids extreme stretching, and may enable normal growth potential.     

Repair of aortic atresia and hypoplastic left heart syndrome without using graft material.

We present a modification of the Norwood stage 1 operation, where the neo-aorta was reconstructed without using graft material. After extensive mobilization of the descending aorta the ductus arteriosus was excised and an end-to-end anastomosis was created between the main pulmonary artery and the distal ductal-aortic junction. The proximal ascending aorta was anastomosed side-to-side to the neo-aorta. Finally either a direct side-to-side anastomosis was created or a 3.5-mm Gore-Tex graft was implanted between the innominate artery and the right pulmonary artery. The first three patients had a favourable outcome: echocardiography showed good ventricular function and acceptable saturation (85%) at the follow up.     

False aneurysm of the ascending aorta from an expanded polytetrafluoroethylene (Gore-Tex) aortopulmonary shunt

A false aneurysm of the ascending aorta developed in a 25-month-old male infant with tetralogy of Fallot because of an expanded polytetrafluoroethylene (PTFE) shunt that had been inserted between the ascending aorta and right pulmonary artery when the patient was 3 months of age. Surgical repair of tetralogy of Fallot with ligation of the PTFE graft was performed at 19 months of age. The false aneurysm at the site of the systemic anastomosis to the shunt was discovered 6 months later and was successfully repaired. Because of the potential for graft dehiscence secondary to growth, we recommend that PTFE shunts be removed completely or, at least, ligated and divided at the time of corrective surgical intervention.     

Anomalous origin of the left coronary artery from the pulmonary artery A case using the autologous pulmonary arterial wall graft.

A 10-year-old girl with anomalous origin of the left coronary artery from the pulmonary artery underwent a two coronary system reconstruction with a new technique. Her left coronary artery was stenotic next to its origin and had a severe adhesion with the aorta. We made a bypass grafting between the aorta and the left coronary artery with a roll shaped transected main pulmonary arterial wall graft. The postoperative angiogram showed a good patency of a reconstructed left coronary artery. This technique is considered useful surgical option for bypass grafting to the left coronary artery when the left coronary artery has an obstructive lesion or a difficulty to dissect and mobilize.     

Anomalous origin of the right pulmonary artery from the ascending aorta associated with aortopulmonary window.

A rare case of anomalous origin of the right pulmonary artery from the ascending aorta associated with an aortopulmonary window and severe pulmonary hypertension in a 13-year-old girl is reported. The window was closed using a Dacron patch and the continuity between the anomalous vessel and the main pulmonary artery was restored with a tubular Dacron graft. After operation the pulmonary pressure dropped to about one-half of the systemic pressure, and equal perfusion and ventilation of both lungs was demonstrated. Although early recognition and surgical treatment of this condition are mandatory to prevent the onset of irreversible pulmonary vascular disease, the presence of severe pulmonary hypertension did not contraindicate surgical correction in our patient.     

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

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.     

Primary repair of interrupted aortic arch and severe aortic stenosis in neonates

Two infants, aged 36 days old (Case 1) and 18 days old (Case 2) with interrupted aortic arch types B and A, respectively, and with severe aortic stenosis, were successfully operated on by use of pulsatile cardiopulmonary bypass. The great arteries were normally related in Case 1 and were transposed in Case 2. Repair involved the following procedure: ligation of the patent ductus arteriosus, restoration of aortic continuity with an 8 mm polytetrafluoroethylene graft, placement of an internal patch to tunnel all left ventricular blood from the left ventricle through the ventricular septal defect into the pulmonary artery in Case 1 and patch closure of the ventricular septal defect in Case 2, transection of the main pulmonary artery, anastomosis between the proximal pulmonary artery and the ascending aorta, and interposition of a valved conduit between the right ventricle and the distal pulmonary artery. The operative field could be approached easily through a median sternotomy. Postoperative cardiac catheterization revealed satisfactory anatomical and hemodynamic results in both cases.     

A case report of anomalous origin of right pulmonary artery from innominate artery associated with left sided unilateral pulmonary hypertension

A 7-year-old girl, who was diagnosed as anomalous origin of right pulmonary artery from innominate artery associated with left sided unilateral pulmonary hypertension due to left patent ductus arteriosus, was reported. The right pulmonary artery had a stenotic segment of it's origin containing ductal tissue. Operation was performed without the aid of cardiopulmonary bypass. The continuity between main and right pulmonary arteries was established by a pericardial roll graft. The postoperative course was uneventful and she is very well three years after correction. To our knowledge, this is a very rare case with such combination of congenital anomalies as anomalous origin of right pulmonary artery and left sided unilateral pulmonary hypertension to be reported in the literature in Japan.     

Endarterectomy and graft replacement of severely calcified (porcelain) ascending aorta with coronary ostial involvement in a patient requiring aortic valve replacement.

We report a successful aortic valve replacement within an extensively calcified (porcelain) aorta, involving the left coronary artery ostium. Clamping such an aorta can result in embolization, dissection, and mural laceration. A 72-year-old female presented with a severely calcified and stenotic aortic valve with a peak pressure gradient of 101 mmHg. Computed tomography demonstrated extensive calcification of the ascending aorta. Coronary angiogram showed a 50% ostial left coronary artery stenosis. Under deep hypothermic circulatory arrest, the aorta was transected at the proximal arch and distal graft anastomosis was performed. This was followed by endarterectomy of the porcelain ascending aorta and the left coronary ostium. Aortic valve replacement, proximal aortic graft anastomosis, and a coronary artery bypass grafting (CABG) with the left internal thoracic artery (LITA) anastomosed to the left anterior descending artery (LAD) were then performed in a sequential manner.     

Surgical correction of tetralogy of Fallot with anomalous origin of left pulmonary artery from the ascending aorta

A surgically corrected case of tetralogy of Fallot with anomalous origin of left pulmonary artery from the ascending aorta is presented. The patient was under-developed 3-year-old girl. She weighed only 1,000 grams at birth and had a systolic heart murmur and slight cyanosis. Echocardiography demonstrated the features of tetralogy of Fallot shortly after birth. Chest roentgenogram showed right ventricular enlargement, but vascular marking of lung was almost normal. Catheterization and cineangiography demonstrated the findings of tetralogy of Fallot. Final diagnosis of anomalous origin of left pulmonary artery from ascending aorta was confirmed by aortography. Surgical correction was accomplished using cardiopulmonary bypass with surface cooling. The left pulmonary artery was detached from the aorta and anastomosed to the main pulmonary artery. Pulmonary valve stenosis and severe infundibular hypertrophy were corrected, and ventricular septal defect was closed with a patch. Postoperative recovery was uneventful. On follow up after surgery, she appears to be in very good condition.     

Right-sided intrathoracic bypass graft for complex or recurrent coarctation of the aorta

A simple technique is described for the interposition of a tube graft between the ascending and descending aorta in the right chest. Directing the graft posteriorly preserves the integrity of the pericardium and avoids potential damage to the prosthesis at a subsequent sternotomy for open heart surgery. This procedure was used successfully and without complication in an 18-month-old boy who had undergone two previous operations for coarctation of the aorta, and in a 20-year-old girl with William's syndrome and diffuse hypoplasia of the distal aortic arch.     

Technique for removal of percutaneously placed intraaortic balloons

A unique case of anomalous origin of the right pulmonary artery from the ascending aorta associated with subtotal left cor triatriatum and severe pulmonary hypertension in a 4-month-old infant was successfully repaired using cardiopulmonary bypass and circulatory arrest.     

Extraanatomic aortic bypass for repair of aortic coarctation

We describe two adult patients who underwent extraanatomic ascending aorta-to-descending thoracic aorta bypass grafting for repair of aortic coarctation through a median sternotomy and posterior pericardial approach. Of the two patients, one presented with coarctation and concurrent cardiovascular disorders, and the other, with residual coarctation. Cardiopulmonary bypass was established with double arterial cannulation in the aorta or axillary artery and the femoral artery ensure adequate perfusion proximal and distal to coarctation and bicaval cannulation. The heart was retracted cephalic and superiorly, and the descending thoracic aorta was exposed through the posterior pericardium. After achieving distal anastomosis, the graft was directed anterior to the inferior vena cava and lateral to the right atrium, and anastomosed to the right lateral aspect of the ascending aorta. A 14-mm graft was used. In one patient receiving concomitant procedures, mitral valve repair and replacement of the ascending aorta was performed after the distal anastomosis.     

Hypothermic circulatory arrest through a left thoracotomy in a 12-year-old child with aortic coarctation

Surgical correction of adult complex aortic coarctation using hypothermic circulatory arrest often requires central cannulation to secure cerebral perfusion. It is not easy to place the cannula in the ascending aorta, however, especially in children undergoing surgery through a left thoracotomy. In a 12-year-old male with hypoplastic distal aortic arch, we placed an arterial cannula in the ascending aorta using the Seldinger puncture technique through the stenotic segment of the distal aortic arch. Replacement of the stenotic segment with a 20 mm-size Dacron graft was then routine. The ascending aorta was exposed only for the proximal anastomosis. The left subclavian artery was also reconstructed. This central cannulation technique is simple and is useful in repairing complex aortic coarctation.     

Reconstruction of stenotic or nonconfluent pulmonary arteries simultaneously with a Blalock-Taussig shunt

Stenosis or discontinuity of the pulmonary arteries associated with congenital cardiac defects was repaired in 12 patients, in conjunction with the Blalock-Taussig shunt. This approach was based on the following concepts: (1) The shunted blood is more uniformly distributed to both lungs, which avoids predominance of flow to the ipsilateral lung and long-term pulmonary vascular damage; (2) because adequate blood flow is maintained, the contralateral lung growth will be near normal; (3) the possibility of future surgical correction is enhanced. The ages of the patients ranged from 2 to 24 months. Five had a primary diagnosis of tetralogy of Fallot, three had double outlet of the right ventricle, three had single ventricle, and one had a truncus type anomaly. All operations were performed without cardiopulmonary bypass. Dilation of the stenotic segment was performed in two patients, enlargement of the stenotic segment in three, and resection of the segment and end-to-end anastomosis in four. Nonconfluent pulmonary arteries were corrected in three patients. In one, the stenotic segment was resected and an anastomosis was made between the left pulmonary artery and pulmonary trunk. In another patient, a segment of the right subclavian artery was interposed between the pulmonary trunk and intrahilar left pulmonary artery. In the third patient, the right pulmonary artery was disconnected from the aorta and a tube was interposed between the right and left pulmonary arteries. There were no intraoperative or late deaths, and postoperative angiographic evaluations were satisfactory. We believe that in infants needing a Blalock-Taussig shunt the pulmonary artery anomalies, if present, should be corrected simultaneously.     

The spectrum of anomalies of aortopulmonary septation.

Embryologically related defects resulting from abnormal septation of the aortopulmonary trunk in 13 patients were reviewed and grouped according to a new classification system. Seven patients (54 percent) had typical aortopulmonary septal defects or windows (Type I), three (23 percent) had a more cephalad defect between the ascending aorta and the origin of the right pulmonary artery (Type II), and three (23 percent) had anomalous origin of the right pulmonary artery from the ascending aorta (Type III). Type I defects are caused by incomplete septation of the aortopulmonary trunk; Type II and III defects result from unequal partitioning of the aortopulmonary trunk by the conotruncal ridges. Three of six patients survived correction of Type I defects; both patients having correlation of Type II defects and all three patients with Type III defects survived and remain well 2 to 216 months (mean 61 months) postoperatively. The recommended technique for Type I and II defects is transaortic closure of the defect by prosthetic patch with the aid of cardiopulmonary bypass, supplemented by profound hypothermia (20 degrees C.), and circulatory arrest for improved exposure in selected cases. For Type III defects, division of the anomalous connection plus direct implantation of the right pulmonary artery into the main pulmonary artery is the preferred approach.