Surgical correction for one pulmonary artery arising from ascending aorta--report of five cases

Over a 10-year period we encountered 5 infants with a pulmonary artery branch arising from ascending aorta. Surgical re-implantation of this vessel was carried out at ages 2.5, 5, 8, 9, and 19.5 months. Pre-operative cardiac catheterization demonstrated severely raised pulmonary artery pressures in all, equal to systemic in 3, and suprasystemic in 2. Four patients had origin of the right pulmonary artery from ascending aorta with a left aortic arch, and the remaining patient had an anomalous left pulmonary artery associated with a right-sided aortic arch. All patients had substantial reduction in pulmonary artery pressures immediately following surgery. One patient died 18 days post-operatively from extensive lung disease. In all 4 of the survivors, post-operative cardiac catheterization (11 to 85 months after surgery) has shown a drop in pulmonary artery pressures. One patient has been left with mildly elevated systolic values but normal diastolic levels. In 2 of the children, mild stenosis has been found at the site of reimplantation of the pulmonary artery. This anomaly should always be considered as a cause in the setting of a large left to right shunt with tricuspid incompetence and severe right ventricular hypertrophy. Prompt surgical repair, after confirmation of the diagnosis, should prevent death from heart failure or the development of irreversible pulmonary vascular disease.     

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.     

New surgical repair for an anomalous origin of the left coronary artery adjacent to the pulmonary cusps with numerous large side branches

A new surgical procedure for anatomical correction of an anomalous origin of the left main coronary artery (LCA) from the pulmonary artery is described for those patients in whom the origin of the LCA is located adjacent to a pulmonary cusp and several side branches are arising immediately behind the ostium of the LCA. Hence the LCA can not be disconnected from the pulmonary artery for either coronary arterial transfer or bypass grafting. A tunnel-type repair may interfere with the movement of the pulmonary leaflets. In those cases our proposed surgical correction consists of an arteriotomy of the pulmonary trunk, end-to-end anastomosis of a vein graft to the ostium of the LCA and its implantation into the ascending aorta. The resulting defect in the pulmonary artery is closed with a pericardial patch avoiding kinking or stenosis of the pulmonary trunk.     

完全性肺静脉异位引流的外科治疗

目的总结完全性肺静脉异位引流（total anomalous pulmonary venous drainage，TAPVD）的外科治疗经验。方法TAPVD27例（心上型15例，心内型12例）均在体外循环下手术。心上型6例采用传统双心房联合切口，9例采用心上法，后者经上腔静脉与升主动脉之间显露左心房与共同静脉干行-一侧吻合：心内型切开房间隔缺损与冠状静脉开口之间的残余房间隔壁，自体心包将肺静脉开口与冠状静脉窦隔入左心房。结果早期死亡2例，其他病人痊愈出院，术后随访3个月-4年，恢复良好，无吻合口狭窄发生，出院后无因心、肺衰竭死亡病人。结论TAPVD早期诊断极为重要，手术治疗的关键在于左心房与肺总静脉吻合口应足够大，避免狭窄的发生。     

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.     

A Different Therapeutic Strategy for Severe Tetralogy of Fallot with Origin of the Left Pulmonary Artery from the Ascending Aorta: Stenting of the Right Ventricular Outflow Tract before Complete Repair

The origin of pulmonary artery branches (particularly the left pulmonary artery) from the ascending aorta is a rare condition. We detected prominent hypoplasia of the main and right pulmonary arteries in a 3.5‐month‐old 3.7 kg female infant who had tetralogy of Fallot with origin of the left pulmonary artery in the ascending aorta. In order to ensure the development of the right pulmonary artery, a stent was put in that extended from the right ventricular outflow tract to the right pulmonary artery. During follow‐up, after the patient's right pulmonary artery had developed sufficiently, a complete repair surgery was done. It is common practice for patients with abnormal origin of the left pulmonary artery to perform the complete repair using the direct reimplantation technique. However, we think that another possibility is to implant the stent in patients with hypoplastic pulmonary artery and branches in the early stages, wait for a short period of time and perform the complete repair surgery before permanent pulmonary hypertension develops.     

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.     

“象鼻”支架术治疗Stanford B型主动脉夹层胸主动脉腔内修复术后Ⅰ型内漏

目的:回顾应用支架"象鼻"术治疗Stanford B型主动脉夹层胸主动脉腔内修复术(TEVAR)术后Ⅰ型内漏的临床效果。方法:2009年3月至2013年1月,首都医科大学附属北京安贞医院,收治的Stanford B型主动脉夹层TEVAR术后Ⅰ型内漏12例患者进行回顾性分析。12例患者均为男性,平均年龄(50.2±6.2)岁,平均身高(171.92±4.98)cm,平均体质量(77.50±8.43)kg,所有患者均无相关家族遗传病史,均有高血压病史,与第一次TEVAR术间隔时间平均34.4个月。支架"象鼻"术均在全麻体外循环下进行。如漏口暴露清楚,可缝闭漏口;如置入支架裸区妨碍象鼻支架缝合,可剪除部分裸露部分金属支架或将置入支架取出;如漏口累及左锁骨下动脉开口,可将左锁骨下动脉近端缝闭,截断左锁骨下动脉,其远端与左颈总动脉行端侧吻合,建立左颈总动脉至左锁骨下动脉转流或8mm人工血管行升主动脉至左腋动脉或左锁骨下动脉转流。于之前置入的支架内置入26~30mm Microport术中支架血管,覆盖内漏破口。术后随访6~48个月,平均6.53个月。结果:12例支架"象鼻"术均为择期手术,其中7例患者漏口位置累及左锁骨下动脉,4例剪除置入支架金属裸区;1例将置入支架取出;3例术中支架血管近端吻合于左锁骨下动脉以远,8例吻合于左颈总动脉与左锁骨下动脉之间;4例同期行左颈总动脉至左锁骨下动脉转流术;2例行升主动脉-左锁骨下动脉转流术;2例行升主动脉-左腋动脉转流术;1例患者因合并二尖瓣关闭不全同期行二尖瓣置换术;1例合并迷走右锁骨下动脉的患者同期行升主动脉-右腋动脉转流术。12例患者手术时间为平均(5.92±1.14)小时,平均住院时间为(21.92±9.14)天,2例患者术后出现围术期并发症,其中1例胸骨哆开,行再次胸骨固定痊愈;1例术前肾功能不全,术后出现急性肾衰竭,经透析治疗后肾功能恢复。本组患者均顺利出院。所有患者术后内漏均消失,术后随访6~48个月,平均6.53个月,均无复发。在术后随访期间,3例患者支架远端病变需再次处理,1例患者术后7个月出现支架感染、咯血、死亡。结论:应用支架"象鼻"术治疗Stanford B型主动脉夹层TEVAR术后Ⅰ型内漏,有较好的临床疗效,但远期效果仍需进一步观察。     

Results of the surgical treatment of tetralogy of Fallot before 6 months of age. A consecutive series of 62 cases with 49 complete repairs

From January 1980 to July 1988, 62 infants aged under 6 months with an uncomplicated Tetralogy of Fallot (single ventricular septal defect, normal coronary arteries, no localised pulmonary artery branch stenosis) underwent 64 surgical procedures. The indications for surgery were increasing cyanosis and/or anoxic spells. Fourteen systemic-pulmonary shunts (21.5%), 49 complete repairs (75.4%) and one enlargement of the right ventricular outflow tract and of the main pulmonary artery without closure of the ventricular septal defect, were performed. The results of palliative shunts are preoccupying: cumulative mortality of 36 per cent; high rate of early reoperation for complete repair: 14 per cent. Complete repair was associated with an operative mortality of 14 per cent. Only one child had to be reoperated. There was no late death after complete repair compared with 2 late deaths after shunt. Ultimate results of complete repairs are good. Some risk factors were statistically significantly associated with complete repair: age (2.5 months or less), weight (4,500 g or less), measurements of the pulmonary arteries estimated by the diameter of the right pulmonary artery (5 mm or less). Conversely there was no death in the subgroup of 31 infants aged more than 2.5 months without major pulmonary hypoplasia (diameter of the right pulmonary artery over 3.5 mm). One-stage complete repair give the best short and medium-term surgical results in treatment of uncomplicated Tetralogy of Fallot in infants, irrespective of age and weight providing they have no diminutive pulmonary arteries.(ABSTRACT TRUNCATED AT 250 WORDS)     

Right Pulmonary Artery Obstruction Is a Long‐term Complication of Aortopulmonary Window Repair

The early outcomes of transaortic patch repair closure for aortopulmonary window are satisfactory, but the lifelong fate of the aorta and pulmonary artery remains unknown. We describe a 40‐year‐old patient with right pulmonary artery occlusion accompanied by aneurysmal dilation of the ascending aorta 38 years after transaortic repair of an aortopulmonary window. Operative findings revealed patch shrinkage and thrombotic occlusion of the right pulmonary artery. The dilated ascending aorta firmly adhered to the right pulmonary artery. After pulmonary artery thrombectomy, the right pulmonary artery was reconstructed and the dilated ascending aorta was replaced.     

Subaortic obstruction in complex congenital heart disease: management by proximal pulmonary artery to ascending aorta end to side anastomosis

Six patients with univentricular heart and one patient with d-transposition of the great arteries had transection of the main pulmonary artery with an end to side anastomosis of the main pulmonary artery to the ascending aorta to relieve subaortic obstruction. Two operations were performed as a palliative procedure within the first 6 months of life and five were performed as part of a definitive repair (four modified Fontan procedures and one repair of transposition of the great arteries with ventricular septal defect). There was one surgical death (14%) occurring 1 day postoperatively from low cardiac output. The remaining six patients are doing well 1 to 19 months postoperatively (mean 11.4 months). The proximal pulmonary artery to ascending aorta end to side anastomosis is an effective means of bypassing subaortic obstruction associated with complex congenital heart disease.     

Anomalous origin of the right coronary artery from the main pulmonary artery. Report of the case (author's transl)]

A case of anomalous origin of the right coronary artery from the main pulmonary artery in a 22 y.o. female is reported. The patient underwent successfully surgical correction by reimplantation of the anomalous vessel to the ascending aorta. The literature has been reviewed; the clinical features, the surgical approach and technique are described.     

Anomalous origin of the right coronary artery from the pulmonary artery. Two case reports

Anomalous origin of the right coronary artery originating from the pulmonary trunk (ARCAPA) is a rare but potentially fatal anomaly. We are presenting two cases of ARCAPA and reviewing the main previous published data on this lesion. The first patient presented at the age of 5 months with respiratory distress and severe chest infection. He was found to have heart murmur and cardiomegaly on chest X ray. Echocardiographic and angiographic data confirmed an ARCAPA associated to a large malalignment ventricular septal defect and distal pulmonary artery aneurysms. He underwent surgical closure of the ventricular septal defect and reimplantation of the ARCAPA on the aorta with good result.The second case is an 11 year old male patient, complaining of dizziness and chest pain on exertion. Echocardiographic and angiographic data confirmed ARCAPA associated to a valvular pulmonary stenosis. He was operated on successfully. He got also direct reimplantation of the anomalous coronary artery on the aorta and a pulmonary valve commissurotomy. An anomalous origin of the right coronary artery is a rare condition but may lead to myocardial ischemia and sudden death. Diagnosis is mainly made by echocardiography and confirmed by conventional coronary arteriography. Operative correction is the appropriate treatment for an anomalous coronary artery arising from the pulmonary trunk.     

Subaortic obstruction after the use of an intracardiac baffle to tunnel the left ventricle to the aorta.

Postoperative hemodynamic studies in five patients document subaortic obstruction after surgical repair utilizing an intracardiac baffle to establish continuity between the left ventricle and the aorta. Four of the patients had a Rastelli procedure for D-transposition of the great arteries with a ventricular septal defect and pulmonary stenosis; one patient had repair of double outlet right ventricle with a ventricular septal defect and pulmonary stenosis. The left ventricular outflow was shown to be a long narrow tunnel by angiography in four of five patients and by echocardiography in one patient. Resting aortic peak systolic pressure gradient ranged from 10 to 42 mm Hg (mean 24). The obstruction was localized to the proximal end of the left ventricule to aorta tunnel (i.e., at the site of ventricular septal defect) in five patients. One patient with a gradient of 42 mm Hg has angina and decreased exercise tolerance. Subaortic obstruction is a newly described sequelae after the Rastelli procedure for transposition or repair of double outlet right ventricle. The obstruction may be hemodynamically significant and should be searched for at postoperative cardiac catheterization.     

Left-sided aortic arch, right-sided descending aorta, and right-sided arterial duct associated with interruption of the aortic arch and presence of Abbott's artery

We describe a male infant, seen at the age of 10 days, with a very rare form of vascular ring. The aortic arch was left-sided, but the aorta descended on the right, with a right-sided persistently patent arterial duct associated with interruption of the aortic arch and presence of Abbott's artery. We performed end-to-side anastomosis of the descending aorta to the ascending aorta, divided the Abbott's artery and the right-sided arterial duct, and banded the pulmonary trunk. Despite our best efforts, the patient died on the eighth day after the surgical intervention.     

Natural history of supravalvular aortic stenosis and pulmonary artery stenosis

Data from 35 patients with supravalvular aortic stenosis or pulmonary artery stenosis, or both, undergoing cardiac catheterization between 1973 and 1989 were analyzed retrospectively. Twenty-seven patients had supravalvular aortic stenosis: 11 required surgery after the first investigation and 8 (80%) of 10 others undergoing serial investigation showed an increase in the left ventricle to aorta pressure gradient. Angiographic measurements showed that the increase in the aortic pressure gradient was related to failure of normal growth of the ascending aorta lumen. Nineteen patients had pulmonary artery stenosis, with a right ventricular pressure greater than 33 mm Hg. At restudy, right ventricular pressure had decreased in 9 (82%) of 11 patients. This decrease in right ventricular pressure was associated with an increase in the systolic distensibility of the proximal pulmonary arteries, although there was no increase in the diastolic diameters. One patient had a rapid early increase in right ventricular pressure and no pulmonary artery growth. In two patients, multiple peripheral pulmonary artery stenoses became evident with time and produced persistent right ventricular hypertension. Supravalvular aortic stenosis is usually a progressive lesion, with an increase in left ventricular outflow tract pressure gradient related to poor growth of the ascending aorta. Pulmonary artery stenosis usually improves and only rarely limits prognosis.     

New technique for single-staged repair of aortic coarctation and coexisting cardiac disorder

The management of adults with aortic coarctation and a coexisting cardiac disorder is still a surgical challenge. Single-staged procedures have lower postoperative morbidity and mortality rates than do 2-staged procedures. We present our experience with arch-to-descending aorta bypass grafting in combination with intracardiac or ascending aortic aneurysm repair.From October 2004 through April 2010, 5 patients (4 men, 1 woman; mean age, 45.8 ± 9.4 yr) underwent anatomic bypass grafting of the arch to the descending aorta through a median sternotomy and concomitant repair of an intracardiac disorder or an ascending aortic aneurysm. Operative indications included coarctation of the aorta in all cases, together with severe mitral insufficiency arising from damaged chordae tendineae in 2 patients, ascending aortic aneurysm with aortic regurgitation in 2 patients, and coronary artery disease in 1 patient. Data from early and midterm follow-up were reviewed.There was no early or late death. Follow-up was complete for all patients, and the mean follow-up period was 34.8 ± 18 months (range, 18 mo-5 yr). All grafts were patent. No late graft-related sequelae or reoperations were observed.For single-staged repair of aortic coarctation with a coexistent cardiac disorder, we propose arch-to-descending aorta bypass through a median sternotomy as an alternative for selected patients.     

Double outlet right ventricle with anomalous left pulmonary artery

We describe a rare case of anomalous origin of the left pulmonary artery from the ascending aorta with concomitant double-outlet right ventricle in a 2-year-old boy. He underwent successful 2-stage surgical treatment with transluminal balloon pulmonary valvuloplasty, followed by complete repair. A follow-up examination at 4 years after the operation showed good results.     

Double outlet right ventricle: Opinions regarding management

Optional statement In the current era of superb surgical results for congenital heart disease, several management options are available for patients born with double outlet right ventricle (DORV). The surgical repair of DORV is tailored to address the variety of abnormalities associated with this lesion. The treatment strategies are dependent upon the anatomy and relationship of the ventricular septal defect (VSD) and the great arteries. For patients with subaortic or doubly committed VSDs, without right ventricular outflow tract obstruction, the usual repair is an intraventricular tunnel from the VSD to the aorta. If right ventricular outflow tract obstruction exists, then augmentation of the right ventricular outflow tract or conduit placement from the right ventricle to the pulmonary artery is necessary. For the “Taussig-Bing anomaly” (subpulmonary VSD) an arterial switch operation is indicated with tunneling of the VSD to the neoaorta. For patients with a remote VSD, complex atrioventricular valve abnormalities, or unbalanced ventricles, a staged palliative approach is warranted. This approach will result in the patient having “single ventricle” physiology, and “Fontan anatomy” after the staging surgeries. Outcomes are determined by the surgery performed. Patients who require conduit placement from right ventricle to pulmonary artery will need subsequent surgery for replacement of the conduit. Complex intraventricular tunnels are at risk for developing subaortic obstruction. Patients who had arterial switch operations have thus far had excellent results. However, these patients may be at risk for development of neoaortic valve regurgitation requiring subsequent reoperation. Complex biventricular intracardiac repairs have been associated with a higher risk of reoperation. In the past 10 to 15 years, patients undergoing Fontan operations have had improved early and late survival. However, long-term problems after the Fontan operation include arrhythmia and development of protein-losing enteropathy.     

Anomalous origin of one pulmonary artery from the ascending aorta: a review of echocardiographic, catheter, and morphological features.

Six patients with anomalous origin of one pulmonary artery from the ascending aorta were reviewed. Four had anomalous origin of the right pulmonary artery and two had anomalous origin of the left pulmonary artery from the ascending aorta. Two of these six patients had tetralogy of Fallot. Two patients died in the first month of life. No changes in the pulmonary vasculature were seen at necropsy. Corrective surgery was attempted in two patients with associated tetralogy of Fallot when they were two years old but both died. At necropsy there was severe pulmonary vascular disease in the lung supplied by the anomalous pulmonary artery but no pulmonary vascular hypertensive changes in the lung supplied by the pulmonary artery from the right ventricle. Two recent patients underwent successful anastomosis of the anomalous pulmonary artery to the main pulmonary artery at three months and one month and three weeks of age respectively. Intraoperative lung biopsy in the latter patient showed early changes in both lungs. Both echocardiography and cardiac catheterisation were used in the diagnoses. Systemic or suprasystemic pressures were found in the pulmonary artery arising from the right ventricle as well as the anomalous pulmonary artery in the three patients without tetralogy of Fallot. Anomalous origin of a pulmonary artery from the ascending aorta is a distinct entity and differs from other aorto-pulmonary arterial connections. Early surgical intervention is recommended in all patients (including those patients with associated tetralogy of Fallot) because of the risk of rapid development of irreversible pulmonary vascular disease.