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.     

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.     

Prenatal diagnosis of left-sided aortic arch,right descending aorta and right-sided ductus arteriosus associated with anomalous origin of left pulmonary artery from ascending aorta

Left-sided aortic arch (LAA), right descending aorta (rDAo), and right-sided ductus arteriosus (RDA) constitute a rare aortic arch anomaly. Moreover, anomalous origin of the pulmonary artery from the ascending aorta, especially that of the left pulmonary artery, is also a rare anomaly of the pulmonary artery branches. Because of the presence of the ductus arteriosus, prenatal ultrasound is an optimal diagnostic tool for the LAA with rDAo. Four-dimensional color Doppler can clearly demonstrate the spatial relationship between the LAA, rDAo, and RDA and the anomalous origin of the left pulmonary artery from the ascending aorta.     

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.     

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: 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 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 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.     

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.     

Absent pulmonary valve and origin of left pulmonary artery from ascending aorta

A rare combination of tetralogy of Fallot, absent pulmonary valve and absent left pulmonary artery is described. The clinical and angiographic profile and the impact on management are discussed. The embryology is outlined.     

12例一侧肺动脉异常起源于升主动脉的外科治疗总结

目的 分析一侧肺动脉异常起源于升主动脉的外科治疗疗效。方法 回顾性分析我院2000年 至 2020 年因一侧肺动脉异常起源于升主动脉行外科治疗的 12 例患者临床资料,其中男性 8 例,女性 4 （33.3%）例。年龄1月~42岁,平均（6.77±11.94）岁。体重3.5~58.0 kg,平均（15.45±16.06）kg。左肺动脉起源 异常（AOLPA）2例,右肺动脉起源异常（AORPA）10例,其中合并房间隔缺损（ASD）5例,室间隔缺损（VSD） 1例,卵圆孔未闭（PFO）2例,动脉导管未闭（PDA）9例,法洛四联症（TOF）2例,右心室双出口（DORV）1例, 二尖瓣关闭不全（MR）1 例,三尖瓣关闭不全（TR）5 例。结果 12 例患者均行外科手术治疗,合并其他畸形 术中同期处理,其中3例合并其他复杂先天性心脏畸形的患者术后因心力衰竭术后而死亡。体外循环时间 79~310 min,平均（137.83±69.92）min;主动脉阻断时间34~130 min,平均（77.08±29.21）min;ICU停留时间38~ 427 h,平均（113.17±119.58）h;术前超声估计肺动脉压力,平均（81.00±34.34）mmHg（1 mm Hg=0.133 kPa）,术 后及随访期间肺动脉压力均大致正常。术后随访时间 9~97 个月,平均（26.17±35.71）月,随访期间未发现 肺动脉狭窄现象。结论 肺动脉起源异常于升主动脉应尽早行外科手术治疗,可改善患者远期预后及生 活质量。     

Repair of anomalous origin of right coronary artery from the pulmonary artery

Anomalous origin of the right coronary artery from the pulmonary artery is a rare cardiac malformation. Between July 2002 and July 2005, we operated on 4 patients with this defect. There were 2 males and 2 females, aged from 18 months to 42 years. Three patients underwent direct re-implantation of the right coronary artery into the aorta, and one had an intrapulmonary tunnel repair (intrapulmonary artery baffle with an autologous pericardial patch for tunneling to the anomalous right coronary ostium). Cardiopulmonary bypass was used in 2 patients, and an off-pump technique in the other 2. One patient had an atrial septal defect that was closed with an Amplatzer septal occluder through the right atrium under transesophageal echocardiography, without cardiopulmonary bypass. All patients survived and recovered uneventfully. Follow-up ranged from 3 to 39 months (mean, 17 months). All patients were doing well and free from symptoms, with normal exercise tolerance. Surgical correction of anomalous right coronary artery shows good early and midterm results.     

Identification of anomalous origin of one pulmonary artery from ascending aorta by two-dimensional and colour Doppler echocardiography

The anomalous origin of the right pulmonary artery from the ascending aorta in a neonate was diagnosed by two-dimensional and colour directed Doppler echocardiography. An anomalous arterial vessel coursing towards the right lung originated from the posterolateral aspect of the ascending aorta. It could be visualized in parasternal and suprasternal views. The normal bifurcation of the pulmonary trunk was absent. Colour Doppler examination aided in clearer delineation and consequent correct identification of the anomalous vessel as the right pulmonary artery.