Repair of the truncal valve and associated interrupted arch in neonates with truncus arteriosus

OBJECTIVE: Truncal valve regurgitation and interrupted aortic arch have frequently been identified as risk factors in the repair of truncus arteriosus. We wished to examine these factors in the current era including the impact of truncal valve repair. METHODS: Between January 1992 and August 1998, 50 patients underwent surgical repair of truncus arteriosus. Their ages ranged from 2 days to 6 months (median, 2 weeks). Nine patients had associated interrupted aortic arch. Of the 14 patients (28%) in whom truncal valve regurgitation was diagnosed preoperatively, 5 had mild regurgitation, 5 had moderate regurgitation, and 4 had severe regurgitation. Five underwent truncal valve repair and 1 underwent homograft replacement of the truncal valve with coronary reimplantation. RESULTS: The actuarial survival was 96% at 30 days, 1 year, and 3 years. There were no deaths in patients with associated interrupted aortic arch. The 2 deaths in the series occurred in patients with truncal valve regurgitation, neither of whom underwent repair. Postoperative transthoracic echocardiography in patients who underwent valve repair showed minimal residual valvular regurgitation. None of the patients has required reoperation because of truncal valve problems or aortic arch stenosis at a median follow-up of 23 months (range, 1-60 months). Conduit replacement has been done in 17 patients (34%) after a mean duration of 2 years. The freedom from reoperation for those who had an aortic homograft was 4 years and for those who had a pulmonary homograft was 3 years. CONCLUSION: Despite the magnitude of the operation, excellent results can be achieved in complex forms of truncus arteriosus. In the current era interrupted aortic arch is no longer a risk factor for repair of truncus. Aggressive application of truncal valvuloplasty methods should neutralize the traditional risk factor of truncal valve regurgitation.     

完全性大血管错位和右心室双出口肺动脉瓣下室间隔缺损伴主动脉弓病变的一期矫治手术

目的评估一期纠治完全性大血管错位(TGA)和右心室双出口肺动脉瓣下室间隔缺损(Taussig-B ing)伴主动脉弓病变的手术疗效。方法2001年1月—2004年6月对8例伴主动脉弓病变的TGA(3例)和Taussig-B ing(5例)行一期手术治疗。3例TGA中,室间隔完整型1例,伴室间隔缺损2例;主动脉弓病变为7例主动脉缩窄、1例主动脉弓中断。手术年龄1例为8个月,7例为5 d~3个月,平均40 d,体重3.5~6.3 kg,平均(4.3±0.5)kg。均采用胸骨正中切口。手术先在深低温、停循环下矫治主动脉弓病变,然后在深低温、低流量下行大动脉转换术(Sw itch术)。体外循环转流时间107~159 m in,平均(126±23)m in,主动脉阻断时间63~118 m in,平均(92±16)m in,停循环14~45 m in,平均(30±12)m in。结果手术死亡1例,为8个月Taussig-B ing伴主动脉弓发育不良、冠状动脉畸形患儿,术后因低心排血综合征、Ⅲ度房室传导阻滞、肺高压危象死亡;1例3月龄患儿术后5 d喂奶时窒息死亡。6例随访5个月~2年,生长发育良好,1例Taussig-B ing主动脉弓中断出现吻合口狭窄,压差60 mm Hg;2例出现主动脉瓣轻微返流,1例肺动脉瓣轻度返流。结论一期纠治TGA和Taussig-B ing伴主动脉弓病变能取得较好手术效果,手术死亡原因为肺动脉高压和冠状动脉畸形。     

Improving early and intermediate results of truncus arteriosus repair: a new technique of truncal valve repair.

BACKGROUND: Despite improved surgical results for truncus arteriosus, overall mortality rates, remain higher than those reported for other complex congenital heart diseases, especially with truncal valve regurgitation or an interrupted aortic arch. METHODS: Seventeen patients had complete repair of the truncus arteriosus at the Cleveland Clinic Foundation between August 1993 and June 1997. The age at operation ranged from 2 days to 4.5 years. Associated abnormalities included interrupted aortic arch in 3 patients and abnormal coronary artery anatomy in 3. Four patients had more than moderate truncal valve insufficiency requiring concomitant truncal valve repair. RESULTS: There were no early deaths and only one late death at a mean follow-up of 24 months. The death occurred 3 months postoperatively and resulted from refractory pulmonary vascular obstructive disease in a patient who was referred at 1 year of age. Reoperation was required in 4 patients. CONCLUSIONS: Even in the presence of associated anomalies complete repair was performed with a low mortality rate. Truncal valve repair can be performed safely in the neonate with good results.     

Repair of persistent truncus arteriosus with interrupted aortic arch.

OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.     

Neonatal repair of truncus arteriosus: continuing improvement in outcomes

BACKGROUND: Repair of truncus arteriosus in the neonatal and early infant periods has become standard practice at many centers. We reviewed our recent experience with repair of truncus arteriosus in neonates, with a focus on early and intermediate outcomes. METHODS: From July 1992 to December 1999, 65 patients 1 month of age or less underwent primary complete repair of truncus arteriosus. Median age was 10 days, and median weight was 3.2 kg. Major associated anomalies included moderate or severe truncal valve regurgitation in 15 patients (23%), interrupted aortic arch in 8 (12%), coronary artery abnormalities in 12 (18%), and nonconfluence of the pulmonary arteries in 3 (5%). Median durations of cardiopulmonary bypass and cardioplegic arrest were 172 minutes and 90 minutes, respectively. Circulatory arrest was employed only in 7 patients undergoing concomitant repair of interrupted arch. Reconstruction of the right ventricular outflow tract was achieved with an aortic (n = 39) or pulmonary (n = 26) allograft valved conduit (median diameter, 12 mm). Replacement (n = 6) or repair (n = 5) of a regurgitant truncal valve was performed in 11 patients, and interrupted arch was repaired in 8. RESULTS: There were three early deaths (5%). Early reoperations included reexploration for bleeding in 3 patients, emergent replacement of a pulmonary outflow conduit that failed acutely in 1 patient, and placement of a permanent pacemaker in 1. Mechanical circulatory support was required in 1 patient. During the median follow-up of 32 months, there were two deaths. The Kaplan-Meier estimate of survival was 92% at 1 year and beyond. The only demographic, diagnostic, or operative factors significantly associated with poorer survival over time were operative weight of 2.5 kg or less (p = 0.01) and truncal valve replacement (p = 0.009). Actuarial freedom from conduit replacement among early survivors was 57% at 3 years. CONCLUSIONS: Repair of truncus arteriosus in the neonatal period can be performed routinely with excellent survival, even in patients with major associated abnormalities.     

Biventricular repair for aortic atresia or hypoplasia and ventricular septal defect.

OBJECTIVE: Aortic valve atresia or hypoplasia can present with a ventricular septal defect and a normal mitral valve and left ventricle. These patients may be suitable for biventricular repair, although the optimal initial management strategy remains unknown. METHODS: From January 1991 through March 1999, 20 patients with aortic atresia or hypoplasia and ventricular septal defect underwent operation with the intent to achieve biventricular repair. Aortic atresia was present in 7 patients, and aortic valve hypoplasia was present in 13 patients. Among those patients with aortic hypoplasia, Z-scores of the aortic valve anulus ranged from -8.8 to -2.7. Associated anomalies included interrupted aortic arch (n = 12 patients), coarctation (n = 6 patients), aortopulmonary window (n = 1 patient), and heterotaxia (n = 1 patient). Nine patients were staged with an initial Norwood procedure followed by biventricular repair in 8 patients. One patient awaits biventricular repair after a Norwood procedure. The conditions of 11 patients were corrected with a single procedure. RESULTS: Among the 9 patients who underwent staged repair, there were no deaths after the Norwood procedure and 1 death after biventricular repair. For the 11 patients who underwent a primary biventricular repair, there was 1 early death and 2 late deaths from noncardiac causes. Follow-up ranged from 1 to 85 months (mean, 28 months). Actuarial survival for the entire group was 78% +/- 10% at 5 years and was not significantly different between staged repair (89%) and primary biventricular repair (73%). CONCLUSIONS: Both primary and staged biventricular repair for patients with aortic atresia or hypoplasia and ventricular septal defect may be performed with good late survival. Refinements in technique of conduit insertion and arch reconstruction have resulted in primary biventricular repair becoming our preferred approach.     

Aortic valve replacement for Takayasu's arteritis.

We report 12 cases of aortic valve replacement performed for Takayasu's arteritis and discuss the genesis of aortic regurgitation and the clinical outcome after aortic valve replacement. This group of twelve patients who underwent aortic valve replacement between April 1982 and March 1990 included four male and eight female patients, aged 24 to 67 years (mean age 48 years). Preoperative angiography showed systemic multiple stenoocclusive or aneurysmal dilated vascular lesions in addition to aortic regurgitation. The multiple lesions included a lesion in the aortic arch branch in nine (75%), in the pulmonary artery in seven (58%), an aneurysmal dilation in the ascending aorta of more than 6 cm in four (33%), a coronary lesion in four (33%), a thoracic aortic lesion in six (50%), and a lesion in the abdominal aorta and its visceral branch in six (50%). Simple aortic valve replacement alone was performed in two patients and in combination with another operation in ten patients, with aortic root reconstruction in two, ascending aortic plication in three, coronary artery bypass grafting in two, aortic arch branch bypass grafting in one, aortic arch branch bypass grafting and coronary ostium endarterectomy in one, and mitral valve replacement and ascending aortic plication in one. There was no operative death, and only one patient died later, 18 months after the operation, because of secondary amyloidosis. The postoperative recovery of the clinical status and cardiac function was good. Intraoperative observations suggested that aortic valve regurgitation may be caused by an extension of aortitis, although histopathologic examinations of the valve showed nonspecific findings. One of the characteristic problems in Takayasu's arteritis is the necessity for prednisolone administration in some patients preoperatively or postoperatively, or both. We conclude that aortic valve replacement for patients with Takayasu's arteritis is an effective and safe treatment. Our data related to the genesis of aortic regurgitation in Takayasu's arteritis remain insufficient to draw conclusions, and further analysis is planned.     

Reverse subclavian flap and aorto-pulmonary window technique for repair of interrupted aortic arch and truncus arteriosus

We report a surgical strategy for repairing an interrupted aortic arch (IAA) with truncus arteriosus (TA) by using a reverse subclavian flap and an aorto-pulmonary (A-P) window technique for preserving the pulmonary artery architecture. A 10-day-old neonate with type B IAA and type I TA with echocardiographic evidence of a significant distance between the ascending and descending aorta underwent surgical repair at the Bristol Royal Hospital for Children. The superior part of the arch was reconstructed using a reverse subclavian flap and the undersurface with a pulmonary homograft patch. The ascending aorta was separated from the pulmonary arteries using a Gore-Tex patch (A-P window type of repair) without disconnecting the branch pulmonary arteries, in order to preserve their architecture. The continuity between the right ventricle and the pulmonary artery bifurcation was established using a 12 mm Contegra conduit. The postoperative course was uneventful, and the neonate was discharged after 12 days. At follow-up, the patient remains well, gaining weight, with no echocardiographic evidences of obstruction. Reverse subclavian flap with homograft patch combined with and 'A-P window' technique for preservation of the pulmonary artery architecture is a useful and effective surgical strategy for neonates presenting with IAA associated with TA.     

Development of neo-coarctation in patients with transposed great arteries and hypoplastic aortic arch after Lecompte modification of anatomical correction

We report on three patients with kinking in the proximal aortic arch that developed after Lecompte modification of the arterial switch operation. Two patients had a previous subclavian patch repair of coarctation of the aorta and had an associated hypoplasia of the transverse aortic arch, and one patient had hemodynamically mild coarctation at the anatomical repair. A severe pressure gradient across the kinked area ("neo-coarctation") necessitating reoperation developed in one patient. The acute arch angulation appears to be due to an excessive posterior displacement of the ascending aorta by the anterior relocation of either the right or left main pulmonary artery branch from underneath the aortic arch. A foreshortened and frequently hypoplastic transverse aortic arch, a common association with coarctation of the aorta, appears to be especially vulnerable to the development of "neo-coarctation" after the Lecompte modification of the anatomical repair of transposed great arteries.     

Cardiopulmonary bypass using argatroban as an anticoagulant for a 6.0-kg pediatric patient

A patient was born with transposition of the great arteries, double-outlet right ventricle, interrupted aortic arch, and a ventricular septal defect and underwent a Damus-Kaye-Stansel procedure with a modified Blalock-Taussig shunt at 14 days old. Three months later, this patient presented with hypoxia and bradycardia was found to have a thrombus present in the main pulmonary artery extending to right pulmonary artery. After initiation of thrombolytic therapy, the patient became severely hypoxic and required the institution of extracorporeal membrane oxygenation. As the result of unknown heparin resistance independent of adequate antithrombin III levels, argatroban therapy was used to achieve desired anticoagulation. The patient was taken to the operating room and converted to conventional cardiopulmonary bypass once adequate activated clotting times were achieved using argatroban. This case report summarizes the use of argatroban as an anticoagulant for a 6.0-kg pediatric patient undergoing cardiopulmonary bypass.     

Successful staged repair of corrected transposition of great arteries and mitral atresia associated with double aoric arch

We report a rare case of corrected transponsition of great arteries and mitral atresia associated with double aoric arch. The patient showed polypnea and cyanosis soon after birth. She underwent balloon atrioseptostomy at the age of 18 days and pulmonary artery banding (PAB) at 24 days. But she showed severe persistent stridor after PAB. Esophagography and left counter-current aortography revealed double aortic arch. At the age of 49 days, division of the left lesser arch and ligation of patent ductus arteriosus were performed through left lateral thoracotomy. Stridor disappeared completely following operation. She underwent total cavopulmonary connection using a 18 mm Gore-Tex graft as intraatrial conduit concomitant with pacemaker implantation at the age of 8 years. Postoperative course was uneventful. To our knowledge, this is the first successful case report of surgical correction of corrected transposition of great arteries and mitral atresia associated with double aoric arch.     

半旋转动脉干调转术治疗完全性大动脉转位合并室间隔缺损和肺动脉狭窄

目的 报道半旋转动脉干调转术治疗完全大动脉转位(TGA)合并室间隔缺损(VSD)和肺动脉狭窄(PS)的近期疗效.方法 2例病儿男、女各1例,分别为16岁和19个月.均为TGA/VSD/PS.采用半旋转动脉干调转术治疗.结果 2例病儿均生存.女病儿术后发生低心排出量综合征和毛细血管渗漏综合征,治疗后痊愈.男病儿术后恢复良好.术后分别随访10、9个月,心功能Ⅰ级.结论 半旋转动脉干调转术可有效治疗TGA合并VSD和PS.长期效果仍需观察.     

Damus-Kaye-Stansel biventricular repair for transposition of the great arteries with pulmonary hypertension

An 18-year-old female with transposition of great arteries and severe pulmonary hypertension was successfully treated with Damus-Kaye-Stansel biventricular repair. Results of the 12-year follow-up showed satisfactory hemodynamics with the aortic valve staying closed throughout the cardiac cycle and without the pulmonary valve regurgitation.     

Neonatal aortic arch reconstruction avoiding circulatory arrest and direct arch vessel cannulation.

BACKGROUND: Aortic arch reconstruction in neonates routinely requires deep hypothermic circulatory arrest. We reviewed our experience with techniques of continuous low-flow cerebral perfusion (LFCP) avoiding direct arch vessel cannulation. METHODS: Eighteen patients, with a median age of 11 days (range 1 to 85 days) and a mean weight of 3.2 +/- 0.8 kg, underwent aortic arch reconstruction with LFCP. Seven had biventricular repairs with arch reconstruction, 9 underwent the Norwood operation and 2 had isolated arch repairs. In 1 Norwood and 7 biventricular repair patients, LFCP was maintained by advancing the cannula from the distal ascending aorta into the innominate artery. In 8 of 9 Norwood patients, LFCP was maintained by directing the arterial cannula into the pulmonary artery confluence and perfusing the innominate artery through the right modified Blalock-Taussig shunt fully constructed before cannulation for cardiopulmonary bypass. In 2 patients requiring isolated arch reconstruction, the ascending aorta was cannulated and the cross-clamp was applied just distal to the innominate artery. RESULTS: LFCP was maintained at 0.6 +/- 0.2 L x min(-1) x m(-2) for 41.0 +/- 13.9 minutes at 18.5 degrees C +/- 1.1 degrees C. In 10 of the 18 patients, blood pressure during LFCP was 15 +/- 8 mm Hg remote from the innominate artery (left radial, umbilical or femoral arteries). In 8 of the 18 patients, right radial pressure during LFCP was 24 +/- 10 mm Hg. The mean mixed-venous saturation was 79.8% +/- 10% during LFCP. Two patients had preoperative seizures, whereas none had seizures postoperatively. One patient died. CONCLUSIONS: Neonatal aortic arch reconstruction is possible without circulatory arrest or direct arch vessel cannulation. These techniques maintained adequate mixed-venous oxygen saturations with no associated adverse neurologic outcomes.     

Intermediate-term survival and functional results after arterial repair for transposition of the great arteries.

An assessment of late morbidity and mortality is essential before arterial repair can be considered truly corrective for patients with transposition of the great arteries. We describe the early and intermediate-term results in 126 patients who underwent arterial repair. Operation was performed at a median age of 6 days, with 76 patients operated on within the first 7 days of life. Coronary artery anatomy differed from the usual arrangement in 37 patients. Simultaneous procedures included ventricular septal defect closure (35) and repair of interrupted aortic arch (2) or coarctation (5). Hospital mortality was seven of 126 (5.5%), with three deaths among the most recent 100 patients (3%). There were one late, noncardiac death and one late death after reoperation. Reoperation for pulmonary artery stenosis was required in 10 of the first 63 patients (16%), all of whom underwent pulmonary artery reconstruction with separate patches for closure of the coronary excision sites. Of the last 63 patients, all of whom underwent pulmonary artery reconstruction with a single pantaloon-shaped pericardial patch, one (2%) required reoperation for pulmonary artery stenosis. Doppler flow studies and echocardiography performed in 115 of 119 surviving patients at a mean of 12 months after repair demonstrated normal left ventricular function, minimal left ventricular outflow gradients, and no more than trivial aortic regurgitation. Peak gradient across the right ventricular outflow tract was 19 +/- 3 mm Hg in patients with separate pulmonary artery patches and 5 +/- 2 mm Hg in those with a single pantaloon patch (p = 0.0001). Follow-up is 96% complete from 1 month to 8 years after operation (mean 2.5 years). The actuarial survival rate at 5 years, including operative mortality, was 92%. All patients are in sinus rhythm, and none requires antiarrhythmic medications. These data suggest that pulmonary artery reconstruction with a single pantaloon patch may be associated with a decreased requirement for reoperation. Intermediate-term survival and functional results are excellent after arterial repair for transposition of the great arteries.     

Single-Stage Arterial Switch With Aortic Arch Enlargement for Transposition Complexes With Aortic Arch Obstruction

Background. Patients with transposition complexes and aortic arch obstruction are a surgical challenge with significant mortality. We have adopted an aggressive approach of concurrent aortic arch repair and arterial switch operation with excellent results.

Methods. Since 1989, 12 of 13 patients with aortic arch obstruction and transposition of the great arteries or double-outlet right ventricle with subpulmonary ventricular septal defect have undergone complete single-stage repair. One patient underwent a two-stage repair because of hemodynamic instability. The median age of repair was 27 days and the median weight was 3.5 kg. Surgical technique involved the arterial switch operation and ventricular septal defect closure when present in 12 patients. One patient with severe subaortic stenosis underwent a modified Damus-Kaye-Stansel operation with concomitant aortic arch enlargement. The aortic arch was enlarged in 12 of 13 patients with a pulmonary homograft patch.

Results. There have been no early deaths and only one late death at 39 months postoperatively from hepatoblastoma. The mean follow-up is 42 months. There have been no reoperations for recurrent aortic arch obstruction. All survivors are currently well from a cardiac point of view.

Conclusions. Concomitant single-stage repair for transposition complexes with aortic arch obstruction achieves excellent survival and should be the surgical procedure of choice.     

Hybrid antegrade repair of the arch and descending thoracic aorta with a new integrated stent-Dacron graft in acute type A aortic dissection: a look into the future with new devices

A young male patient underwent supracoronary replacement of the ascending aorta for acute type A dissection under hypothermic circulatory arrest. After discharge, he was readmitted two weeks later due to severe aortic regurgitation and acute arch redissection. Under a second period of hypothermic circulatory arrest three weeks after the initial operation, radical treatment with aortic valve replacement, replacement of the ascending aorta and arch, together with antegrade deployment of a stent-graft in the true lumen for frozen elephant-trunk technique, were successfully performed. Computed tomography at four weeks showed complete proximal repair and thrombosis of the false lumen. Transesophageal echocardiography at eight weeks confirmed repair. The patient is currently leading an active life. A hybrid approach for complex cases of acute type A dissection with arch involvement can be considered for the future.     

Truncus arteriosus type A3: complex repair with cryopreserved pulmonary homograft

A 6-year-old girl with truncus arteriosus type A3, one of the rarest anatomic types, underwent corrective surgery using a cryopreserved valved pulmonary homograft. The special anatomic features in this case were individual origin of the pulmonary arteries. The right pulmonary artery came directly off the truncal vessel, whereas the left pulmonary artery was connected to the aortic arch via a stenotic ductus or ductus-like vessel. The preoperative pulmonary blood flow distribution was 94% on the right versus 6% on the left side. The underperfusion of the left lung may have been related to pulmonary hypoplasia due to long lasting stenosis at the left pulmonary artery take off. The postoperative pulmonary blood flow distribution was 67% on the right versus 33% on the left side. The operative and postoperative course has been uneventful; presently, 6 months after the operation, the patient is in improved clinical condition.     

Truncus arteriosus repair: outcomes, risk factors, reoperation and management.

OBJECTIVES: Truncus arteriosus (TA) continues to be associated with significant morbidity and mortality, but there have been clinically significant improvements with early repair. METHODS: Sixty patients underwent physiological correction of TA between November 1978 and January 2000. The average age was 76 days (range, 3 days--20 months). Associated cardiac anomalies were frequently encountered, the most common being severe truncal valve regurgitation (n=7), interrupted aortic arch (n=6), coronary artery anomalies (n=6), non-confluent pulmonary arteries (n=4), and total anomalous pulmonary venous return (n=1). Truncal valve replacement was performed initially or subsequently in seven patients with severe regurgitation (mechanical prostheses in six patients and a cryopreserved aortic homograft in one patient). Right ventricle--pulmonary artery continuity was established with an aortic (n=16) or pulmonary homograft (n=32) in 48 patients, a Dacron polyester porcine valved conduit in five, a non-valved polytetrafluoroethylene (PTFE) tube in three, direct anastomosis to the right ventricle with anterior patch arterioplasty in three, and a bovine jugular venous valve conduit in one patient. RESULTS: There were ten hospital deaths (17%; 70% confidence limit, 7--25%). Multivariate and univariate analyses demonstrated a relationship between hospital mortality and associated cardiac anomalies. In the 43 patients without these associated cardiac anomalies, the early survival was 91% (group I). In the 17 patients with one or more of these risk factors, the survival was 71% (group II, P=0.002). There was one late death. Twenty-three patients (46%) required reoperation for right ventricular outflow tract (RVOT) obstruction at a mean follow-up time of 59.1 months. In 23 patients, the RVOT reconstruction was performed with a PTFE monocusp, and six patients had of a variety of replacement conduits inserted. Postoperatively, there were 34 (68%) patients in New York Heart Association functional class I and 16 (32%) in class II. Twenty-eight surviving patients are reported as doing well without any medication. The freedom of reoperation in the 39 hospital survivors (group I) without risk factors was 64% at 7 years; and 36% at 10 years in the 11 patients (group II) surviving with risk factors. CONCLUSIONS: Associated cardiac anomalies were risk factors for death after the repair of TA. In the absence of these associated lesions, TA can be repaired with an excellent surgical outcome in the neonatal and early infancy period.     

Report of a case of an atypical huge patent ductus arteriosus

An atypical huge patent ductus arteriosus (PDA) associated with extreme pulmonary hypertension in a 6-month old female infant was reported. The left 3rd intercostal thoracotomy revealed huge PDA which has an appearance of pulmonary-ductus-descending aorta-trunk (PDDT) on the left side. The right side of the ductus continued to the aortic arch without demarcation between the ductus and the aorta. The median sternotomy clarified the ascending aorta, normal aortic arch, and main pulmonary artery. However, the top of the main pulmonary artery seemed to be fused with the aortic arch. The diagnosis of distal type of aortopulmonary window with wide communication was made and the total circulatory arrest with deep hypothermia and the intracavitary patching for separation of systemic and pulmonary blood flow seemed to be required for the complete repair. Total repair was decided to be postponed one week later and the chest was closed. The patient died of multi-organ failure originating from acute respiratory failure 23 days after exploratory thoracotomy without being repaired. Postmortem examination revealed a huge PDA freely communicating with the ascending aorta and the aortic arch. This is a report of an atypical huge PDA rarely seen and the conventional division or ligation is unfeasible. The circulatory arrest with deep hypothermia and intracavitary patching are obligatory for the surgical repair.