Left pulmonary artery coarctoplasty by using the right pulmonary artery flap near the arterial duct

Coarctation of the left pulmonary artery may coexist with tetralogy of Fallot or pulmonary atresia with ventricular septal defect. Various surgical methods, such as autologous pericardial patching and intraoperative pulmonary artery stenting, have been used for solving this condition. We proposed a simple and effective technique for treating patients with left pulmonary artery coarctation and pulmonary atresia with ventricular septal defect by using the right pulmonary artery flap for left pulmonary artery coarctoplasty, plus central shunt creation by V-Y plasty.     

Anomalous origin of innominate artery from right pulmonary artery in DiGeorge syndrome

We experienced a case of anomalous origin of innominate artery from right pulmonary artery (isolated innominate artery). This patient was a 2-month-old baby girl weighing 3.2 kg with DiGeorge syndrome, who was diagnosed with perimembranous ventricular septal defect, atrial septal defect, and patent ductus arteriosus. This type of anomaly is exceedingly rare.     

Absorbable pulmonary artery banding in tricuspid atresia

A child with tricuspid atresia, concordant ventriculoarterial connections, large ventricular septal defect, and elevated pulmonary artery pressure underwent pulmonary artery banding with a polydioxanone ribbon. This procedure was successful in this patient as the ventricular septal defect became restrictive while the banding was fully absorbed after 5 months. This technique could be included in the panel of surgical strategies for patients with single ventricle physiology and potential but insufficient subpulmonary stenosis in early infancy.     

Pulmonary atresia, ventricular septal defect, and coronary-pulmonary artery fistula

We report 2 patients with pulmonary atresia, ventricular septal defect, and coronary-pulmonary fistula. The fistula originated from the left anterior descending artery in 1 patient, and from the right coronary artery in the other. Both patients survived staged correction in which right ventricular outflow was reconstructed with autologous fistula tissue. One patient with pulmonary hypertension suddenly died 9 months after surgery. Twenty-four patients with pulmonary atresia, ventricular septal defect, and coronary-pulmonary fistula have been reported previously.     

Pulmonary artery banding: analysis of a 25-year experience

A 25-year experience (May 1962 through April 1987) with pulmonary artery banding in 183 patients was reviewed and analyzed. Pulmonary artery banding was performed in a heterogeneous group of patients aged two days to 60 months (median, 10 weeks; mean, 21.8 weeks) and weighing 1.4 to 13.8 kg (mean, 4.2 kg). Diagnosis was ventricular septal defect in 76 (41.5%) and atrioventricular communis in 41 (22.4%). Pulmonary artery banding was also used in patients with d-transposition of the great vessels with ventricular septal defect, double-outlet right ventricle, univentricular heart, tricuspid atresia, and truncus arteriosus. Early death occurred in 39 of 175 patients who underwent pulmonary artery banding at Ochsner Foundation Hospital (22.3%). Definitive operation has been performed in 37 of the patients who underwent pulmonary artery banding since 1979 with excellent outcome in 32 (86.5%). Pulmonary artery banding is a useful palliative procedure for a diverse group of patients with congenital cardiac anomalies and unrestricted pulmonary blood flow. With improved results of primary repair of intracardiac anomalies in small infants, however, pulmonary artery banding should be reserved for severely ill patients with complex lesions not amenable to early definitive correction. Currently, pulmonary artery banding is indicated in patients with excessive pulmonary blood flow and single ventricle or tricuspid atresia. Pulmonary artery banding is also appropriate in certain patients with atrioventricular communis and in patients with muscular or multiple ventricular septal defects. Pulmonary artery banding is an option in patients with ventricular septal defect and coarctation of the aorta.     

Anomalous left anterior descending coronary artery from pulmonary artery with ventricular septal defect

Anomalous origin of the left anterior descending coronary artery with associated congenital defects is very rare. An angiogram of a 47-year-old woman admitted for a ventricular septal defect closure revealed an anomalous left anterior descending coronary artery arising from the left posterior sinus of the pulmonary artery. During the surgical procedure, the origin of the left anterior descending coronary artery was closed with pledgetted polypropylene sutures through the pulmonary artery. The ventricular septal defect was closed with a patch through the right atrium, and the left anterior descending coronary artery was bypassed with the left internal mammary artery.     

Hemodynamic observations during and after pulmonary artery banding

Analysis of cardiac catheterization in 25 patients with nonrestrictive ventricular septal defect (Prv/Plv > 0.6) shows that the pulmonary vascular resistance and systemic vascular resistance function as resistances in parallel. Furthermore, studies in the presence of right ventricular outflow obstruction, by either pulmonary artery band (9 patients) or pulmonary stenosis and ventricular septal defect with left-to-right shunting (12 patients), show that the right ventricular outflow obstruction (band) functions as a resistance in series with the pulmonary vascular resistance. The sum of pulmonary vascular resistance plus band resistance is in parallel with systemic resistance. This concept allows better explanation of the great pressure variability across bands observed at operation. It is impossible to completely evaluate a pulmonary artery band with pressures alone. Flow measurements are necessary for better understanding of pulmonary artery bands. The variability of clinical response after banding may be explained by the variations in pulmonary vascular resistance and systemic vascular resistance during and after pulmonary artery banding.     

Homograft replacement of the pulmonary artery bifurcation

Severe deformity or nonconfluence of the pulmonary arteries was repaired during correction of congenital heart defects by implantation of a complete, fresh, antibiotic-sterilized homograft pulmonary artery bifurcation in 17 patients, aged 15 months to 17 years. Six patients had Fallot's tetralogy; five, pulmonary atresia and ventricular septal defect; three, truncus arteriosus; two, ventricular septal defect; and one, pulmonary atresia with intact ventricular septum. All but one had previously undergone one or more palliative or corrective operations. Thirteen patients survived the operation (76%; 70% confidence interval = 61% to 88%) and were followed up from 2 to 56 months postoperatively (mean = 16 months). All had symmetrical pulmonary perfusion on chest x-ray films and satisfactory homograft function on Doppler echocardiograms. There were no late deaths or reoperations. Four patients (24%; 70% confidence interval = 12% to 39%) died in the hospital of pulmonary or cardiac dysfunction despite a technically satisfactory reconstruction. Comparison of predicted and observed ventricular pressure ratios in nine patients showed that the factor contributed by a right pulmonary artery branch stenois was cancelled in most. The use of a complete, adult-size homograft has thus facilitated reconstruction of the pulmonary artery bifurcation and given encouraging early results.     

Mycotic pulmonary artery aneurysm following pulmonary artery banding

A neonate with situs inversus, transposition of the great arteries, ventricular septal defect, cross-crossventricles and hypoplastic right ventricle underwent pulmonary artery banding at the age of 7 days. The course was complicated by septicaemia and subsequently the development of an aneurysm of the pulmonary artery. Serratia marcessans was grown from the band site. The pulmonary artery aneurysm was resected and the pulmonary artery was repaired. The literature is reviewed with the emphasis on diagnosis, natural history and surgical management.     

Aneurysmal change in an internal mammary artery-pulmonary artery fistula

We treated a rare case of aneurysm of the internal mammary artery-pulmonary artery fistula in a 32-year-old woman with unrepaired pulmonary atresia and ventricular septal defect. This aneurysm communicated with the pulmonary artery system through an aortopulmonary collateral. Aneurysmectomy was successful.     

Thromboembolism originated from the pulmonary artery stump after Fontan operation

Cerebral thromboembolism is a rare but serious complication after Fontan operation. This is the report of a patient who underwent a successful intracardiac thrombectomy for cerebral thromboembolism after Fontan operation. A 2-year-old girl was referred to us with the diagnosis of tricuspid atresia without pulmonary stenosis, normally related great arteries, and a ventricular septal defect. Although she underwent a successful Fontan operation and division of the main pulmonary artery, she developed a cerebrovascular event at 3 weeks after the operation. Echocardiography demonstrated a large thrombus within the residue of the main pulmonary artery, and suggested that the thrombus had migrated into the systemic circulation by way of the ventricular septal defect. At 2 weeks after the cerebrovascular event, she underwent thrombectomy and excision of the pulmonary valve. Although she has developed slight left-sided hemiparesis, she is leading a normal life at 1 year after the operation.     

Thromboembolism originated from the pulmonary artery stump after Fontan operation.

Cerebral thromboembolism is a rare but serious complication after Fontan operation. This is the report of a patient who underwent a successful intracardiac thrombectomy for cerebral thromboembolism after Fontan operation. A 2-year-old girl was referred to us with the diagnosis of tricuspid atresia without pulmonary stenosis, normally related great arteries, and a ventricular septal defect. Although she underwent a successful Fontan operation and division of the main pulmonary artery, she developed a cerebrovascular event at 3 weeks after the operation. Echocardiography demonstrated a large thrombus within the residue of the main pulmonary artery, and suggested that the thrombus had migrated into the systemic circulation by way of the ventricular septal defect. At 2 weeks after the cerebrovascular event, she underwent thrombectomy and excision of the pulmonary valve. Although she has developed slight left-sided hemiparesis, she is leading a normal life at 1 year after the operation.     

Interrupted left aortic arch and isolated right subclavian artery from the right pulmonary artery

We report a 10-day-old newborn, weighing 2.9 kg with an interrupted left aortic arch type B, a large subarterial ventricular septal defect and a right ductus connecting the right pulmonary artery to an isolated right subclavian artery. The patient underwent successful total surgical repair and the isolated right subclavian artery was ligated. He was discharged from hospital without complication and maintains excellent perfusion to the right arm via collaterals.     

Anterior translocation of the right pulmonary artery for airway obstruction caused by pulmonary hypertension

Both the bronchi are often severely squeezed by the dilated pulmonary artery in infants with ventricular septal defect and pulmonary hypertension. To relieve the considerable residual airway obstruction, the right pulmonary artery is translocated anteriorly to the ascending aorta concomitantly with the intracardiac repair. This technique is a useful procedure for infants with intractable respiratory failure caused by a dilated pulmonary artery.     

肺移植治疗儿童肺动脉高压的临床疗效

目的 探讨同种异体肺移植治疗儿童肺动脉高压(PAH)的临床疗效.方法 受者例1、2为特发性肺动脉高压( IPAH)患儿,均经右心导管术确诊为IPAH,术前心功能不全Ⅳ级,肺动脉收缩压、平均压分别为110、70 mm Hg(1 mm Hg =0.133 kPa)和148、72 mm Hg,在全麻体外膜肺氧合(ECMO)支持下行序贯式双侧单肺移植术,术中ECMO支持时间分别为550 min和450 min,出血量分别为3000 ml和1200 ml.受者例3为先天性心脏病室间隔缺损合并艾森曼格综合征心内直视探查术后,超声心动图(UCG)提示先天性心脏病室间隔缺损,双向分流,肺动脉收缩压、平均压为110、60 mm Hg,在全麻低温体外循环(CPB)下行室间隔缺损修补术同期右侧单肺移植术,术中CPB时间244 min.3例受者与供者体型较匹配,ABO血型相同.结果 受者例1、2术后ECMO分别维持16h、13 h后撤离,术后第3、4天均出现不同程度的血流动力学不稳定,诊断为急性左心衰,均于术后第3、6天行气管切开呼吸机辅助正压通气,经强心、利尿、扩血管等治疗,分别于术后第33天、12天脱离呼吸机.受者例3术后3天内移植肺出现中等程度再植反应性肺水肿,术后第7天气管切开,第12天撤离呼吸机;术后第14天出现急性排斥1次,治疗后缓解.3例受者术后UCG提示心脏形态和心功能明显改善,受者例3室间隔缺损修补完整,无残余分流.分别于术后第93天、32天、62天康复出院,心功能均达Ⅰ级,肺动脉收缩压、平均压分别降为54、32 mm Hg,60、36 mm Hg和53、39 mm Hg.术后已随访41、21、82个月,患儿正常工作学习,至今生活质量良好.结论 对终末期经内科保守治疗效果欠佳的PAH患儿行肺移植能很好改善生活质量.     

主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断(附5例报告)

目的 探讨主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断的发病情况,总结诊断和治疗经验.方法 回顾性分析武汉亚洲心脏病医院2005至2009年5例病儿,均经超声心动图、螺旋CT检查,3例10岁以上病儿行心导管检查及心血管造影,确诊为A型主动脉弓离断、Ⅱ型主-肺动脉缺损、右肺动脉起源于升主动脉,均合并粗大动脉导管未闭、无室间隔缺损等心内畸形.年龄1～18岁,其中男4例,女1例.结果 1例18岁病人,应用3个月前列环素(万他维)降肺动脉压力治疗,现心功能Ⅱ级;1例14岁病儿放弃治疗,失访.余3例行一期根治手术,其中1岁病婴并发严重肺部感染,术后第11天死亡;2岁病婴术后仍有重度肺动脉高压,经强心、利尿及降肺动脉压治疗,随访18个月病儿生存,但心功能Ⅲ级;10岁病儿术后恢复好,经强心、利尿及降肺动脉压药物治疗,随访3个月心功能Ⅰ级.结论 这种病例与经典的主动脉弓离断三联征有显著的差异,是一种独特的心血管畸形综合征.超声心动图可作为该疾病的初步诊断方法.确诊及手术指征的判断宜结合心导管检查、心血管造影及螺旋CT检查.肺动脉压力及阻力是影响手术时机及预后的关键因素.手术方式均可采取一期根治术,但病死率较高.如能度过围手术期,则生存的概率很大.

Abstract：

Objective Reviewing the experience in the diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of the aortic arch. Methods Reviewed clinical data from medical records for5 patients (4 boys and 1 girl, age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009. Results All cases had type A interruption of the aortic arch ( according to Celoria and Patron's Classification) , type Ⅱ aorta-pulmonary septal defect( according to Mori Classification) and aortic origin of right pulmonary artery complicated with interruption of the aortic arch. Large patent ductus arteries were observed in all cases,and no cardiac abnormality such as ventricular septal defect was found. All patients received echocardiography and spiral CT examination, and 3 of them over 10 years of age received cardiac catheterization and angiography for the evaluation of the pulmonary artery pressure. Two patients, at the age of 14 and 18 years, had severe pulmonary hypertension and declined to receive the operation. One of the 2 cases had hemoptysis and was given prostacyclin ( Iloprost solution for inhalation, a drug for pulmonary artery hypertention ) for reducing the pulmonary pressure. The patient now has an improved cardiac fu0ction without recurrent hemoptysis. One 14-year-old case gave up the operation because of the financial problem and failed to communicate with us after discharge. Radical surgery was performed in the remaining three cases, one had serious infection in the lung and died 11 days after the operation, in one case ( 2 years old) pulmonary artery hypertension has been persisted even though drug therapy was given for a long time and was found at a poor cardiac function 18 months after the operation. One patient recovered well under routine drug therapy and has been followed-up. Conclusion Aorta-pulmonary septal defect and aortic origin of right pulmonary artery with interruption of the aortic arch ( with intact ventricular septum) are different from the classic triad of inter ruption of the aortic arch, consisted of the interruption of the aortic arch, patent ductts arteriosus and ventricular septal defect.It should be considered as a special kind of cardiovascular malformation syndrome. Echocardiography can be used for the preliminary diagnosis of aorta-pulmonary septal defect and aortic origin of right pulmonary artery with interruption of the aortic arch. The final diagnosis and decision for surgery should be based on the combined information from cardiac catheterization,cardiovascular angiography and spiral CT examination. Pulmonary artery pressure and resistance are the most important factors,because they have great effects on the duration of surgery and the outcome. A radical operation is necessary for all cases, but the mortality rate is high. The prognosis would be great if only the patient can survive successfully during the peri-operative period, a period with a poor cardiac function, potential lung infection and the pulmonary artery hypertension.     

Compression of an anomalous single coronary artery from pulmonary artery by banding

This report describes a case with double outlet right ventricle and doubly committed ventricular septal defect associated with congenital diaphragmatic hernia. The patient underwent pulmonary artery banding and clipping of patent ductus arteriosus after repair of the diaphragmatic hernia. At 6 months, cardiac catheterization revealed anomalous origin of a single coronary artery from the pulmonary artery of the proximal banding. Although ischaemic symptoms had not been observed, the banding had resulted in compression of the coronary ostium. An emergency Rastelli procedure with Damus-Kaye-Stansel anastomosis was successfully performed.     

Multiple pulmonary artery aneurysms due to infective endocarditis

Bilateral pulmonary artery aneurysms developed in the course of staphylococcal endocarditis in a 6-year-old girl with ventricular septal defect. Consecutive computed tomography scans revealed the progressive enlargement of one of the aneurysms. She underwent an urgent left upper lobectomy because of the impending rupture and a possible life-threatening hemorrhage. The second ipsilateral aneurysm was plicated in order to exclude the aneurysm sac. In the next operation the ventricular septal defect was closed and vegetations located on the tricuspid valve were removed. On follow-up spontaneous thrombotic resolution occurred in the right-sided aneurysms.     

Surgical closure of an apical ventricular septal defect through a septal ventriculotomy

Here we report a 3-year and 7-month-old boy with a muscular ventricular septal defect at the apex. At age 7 months, pulmonary artery banding was performed. Surgical treatment consisted of double patch closure via septal ventriculotomy and a main pulmonary artery plasty by an end-to-end anastomosis with cardiopulmonary bypass. There was no residual leak of the ventricular septal defect or ventricular aneurysm and the postoperative course was good. This technique could be a useful surgical option for repair of apical muscular ventricular septal defects.     

Pulmonary artery banding: long-term results in 63 patients.

Sixty-three patients who underwent banding of the pulmonary artery between 1968 and 1975 were studied. Mortality among patients who underwent the banding procedure was 22%, and among those with ventricular septal defect it was 7%. Thirty-eight of the 49 survivors of the banding procedure were investigated for abnormalities of the pulmonary outflow tract caused by the band. Seventy-one percent of these 38 patients had an identifiable abnormality of the pulmonary valve or artery. These complications occurred more frequently in patients banded at a very young age (less than 2 months old) and in patients in whom the band was in place more than two years. Corrective operations have been performed in 24 of the 49 patients who survived banding. Seventy-six percent of the patients with ventricular septal defect survived corrective operation, while only 29% of the patients with more complex lesions survived.