Enlarging angioplasty of the aortic arch with an pulmonary artery autograft patch. Interest in a single-stage repair of the syndrome of coarctation and interruption of the aortic arch]

Single stage repair of syndromes of coarctation and interruption of the aortic arch is a routine procedure in many surgical centres with good immediate results. The classical technique of aortic repair is based on the principles of Crafoord's extended resection anastomosis. Recoarctation is not an unusual long-term complication. A technique of enlarging angioplasty of the aorta using a patch of pulmonary artery has been developed and used in 22 neonates with obstruction of the aortic arch associated with ventricular septal defect with an average age and body weight of 15 days and 2.9 Kg respectively. The ventricular septal defect was closed surgically during the same procedure. Total circulatory arrest was not used in these children and all had aortic repairs with selective cerebral perfusion with moderate hypothermia (28-30 degrees C). This technique was used without any procedure-related early morbidity. No early or late deaths were observed in this series. Two patients were reoperated during the first year after the initial procedure: one for residual ventricular septal defect and the other for supraventricular pulmonary stenosis. Two patients, one of whom was reoperated, developed supraventricular pulmonary stenosis with a gradient of over 60 mmHg. These stenoses were observed in the first cases operated and were essentially due to the technique of pulmonary artery reconstruction. Over a median follow-up period of 10 months, no recoarctations were observed: the Doppler ultrasound study showed an isolated mean systolic gradient of 6 +/- 12 mmHg. The authors conclude that angioplasty of the aortic arch with an enlarging patch of pulmonary artery autograft during single stage surgery of syndromes of coarctation and interruption of the aortic arch provides a harmonious and durable repair of the aortic arch.     

Isolated cerebral and myocardial perfusion during aortic arch repair in neonates

OBJECTIVES: To prevent possible neurologic injury after hypothermic circulatory arrest, aortic arch obstruction with cardiac defects is repaired in one stage using isolated cerebral and myocardial perfusion (ICMP). This study investigated serum S-100 protein(S-100) levels in neonates undergoing ICMP. METHODS: Between February 2000 and January 2001, 19 neonate patients underwent repair of critical congenital heart defects. Seven of these patients with aortic coarctation(n = 3) or interrupted aortic arch (n = 4) with ventricular septal defect(ICMP group) underwent primary total repair. An arterial cannula was inserted either into the ascending aorta or into a polytetrafluoroethylene graft which was anastomosed to the innominate artery. During arch repair, a cross-clamp was placed between the innominate and left carotid arteries, and an end-to-end arch anastomosis was performed with cerebral perfusion and heart beating. During ICMP the flow was reduced to maintain a radial artery pressure of 30-45 mmHg. The remaining 12 patients underwent complete transposition of great arteries(n = 9) or total anomalous pulmonary venous connection(n = 3) using a cardiopulmonary bypass(CPB) with flow of 150-180 ml/kg/min(control group). Sequential blood samples for S-100 determinations were taken after induction of anesthesia, 30 min after aortic declamping(post-ACC), 30 min after CPB, and 24 hr after CPB. RESULTS: There were no early and late deaths. Neurologic symptoms were not observed in any patients. Mean ICMP time in ICMP group was 17 +/- 4 min. In all patients, S-100 showed the highest value post-ACC and then declined with time. There were no differences in S-100 between the groups at any other time point. CONCLUSIONS: Selective cerebral perfusion through the innominate artery may be able to maintain brain circulation.     

Repair of Coarctation-Related Aortic Arch Aneurysm and Ventricular Septal Defect in an Adolescent

A saccular aortic arch aneurysm that is secondary to aortic arch coarctation and that is accompanied by a ventricular septal defect is a rare combination in the adolescent patient. Total simultaneous repair of all of these conditions is desirable, because of the higher morbidity and mortality rates of staged procedures—particularly when resection of the saccular aneurysm is delayed.Herein, we discuss the case of a 16-year-old boy who underwent simultaneous surgical correction of these malformations. With the aid of cardiopulmonary bypass on the beating heart, the coarctation and the aneurysmal segment were resected, and a tubular Dacron graft was interposed. The ascending aorta and femoral artery were both then cannulated to ensure whole-body perfusion during cardiopulmonary bypass. The ventricular septal defect was closed with the patient under cardioplegic arrest. After 10 days, he was discharged from the hospital without sequelae. We conclude that single-staged repair of cardiac abnormalities and of an aortic arch aneurysm that is secondary to coarctation of the aortic arch can be performed safely and effectively in adolescent and adult patients by use of our technique.Key words: Aorta/surgery, aortic aneurysm/physiopathology/surgery/ultrasonography, aortic coarctation/complications/physiopathology/surgery/ultrasonography, coronary disease/surgery, magnetic resonance angiography, methods, time factorsUnrepaired coarctation of the aorta can lead to hypertensive cardiovascular sequelae, such as coronary disease, heart failure, stroke, aortic dissection or rupture, and premature death.¹ Although narrowing of the aortic arch is sometimes seen when surgery is performed on congenital cardiac abnormalities, aortic coarctation that persists into adulthood is unusual. Coarctation that originates between the arch vessels is rare, constituting approximately 1% of all coarctation cases.² Aortic coarctation is sometimes accompanied by a fusiform or saccular aneurysm, which is either congenital or secondary to the hemodynamic disturbance caused by the coarctation. Native aortic coarctation alone is usually repaired surgically through a left thoracotomy, but aortic arch coarctation with saccular aneurysms and concomitant cardiac disorders in adolescent patients still present dilemmas for the surgeons.³ To decrease potential complications of anatomic repair, various techniques have been proposed,^3,4 such as extra-anatomic ascending aorta-to-descending aorta bypass, or the use of deep hypothermic circulatory arrest during a simultaneous or staged surgical approach. In our patient, the single-staged repair of a ventricular septal defect (VSD) and a large saccular aortic arch aneurysm that was secondary to an unusually located coarctation of the aortic arch was carried out via median sternotomy.     

Severe obstruction to systemic blood flow in congenitally corrected transposition (discordant atrioventricular and ventriculo-arterial connexions): an analysis of 14 patients

The more common associated cardiac anomalies in any cohort of patients with discordant atrioventricular and ventriculo-arterial connexions (congenitally corrected transposition) include ventricular septal defect, left ventricular (subpulmonary) outlet obstruction, and displacement and/or dysplasia of the morphologically tricuspid valve. There is scant mention in the literature of severe obstruction to systemic blood flow at aortic valve level or beyond in these hearts. The present study reviews those pertinent morphological-clinical and follow-up data on 14 patients with congenitally corrected transposition and aortic valve atresia (one patient); aortic arch interruption or aortic arch atresia (two patients) and coarctation of the aorta (11 patients). All but one patient (the patient with aortic valve atresia) had a perimembranous ventricular septal defect. Mild outflow tract obstruction of the morphologically left ventricle was identified in one patient. Nine patients, however, demonstrated a significant structural and functional disturbance (Ebstein-like with dysplasia) of the systemic morphologically tricuspid valve. No patient in this series was identified with isolated aortic coarctation.     

Transposition of the great arteries and narrowing of the aortic arch. Emphasis on right ventricular characteristics

Of 279 patients with transposition of the great arteries, 14(5%) had narrowing of the aorta, including local coarctation (6), isthmus hypoplasia (6), isthmus atresia (1), and kinking of the aorta (1). There were six deaths in 10 surgically treated patients; in addition four patients died before operation. Two of the four survivors had a subpulmonary malalignment ventricular septal defect with angiocardiographic narrowing of the right ventricular outflow tract, two had evidence of redundant muscle tissue obstructing the right ventricular outflow tract. Necropsy showed the presence of anatomical right ventricular outflow tract or inflow tract obstruction or both in all 10 cases. Outflow tract obstruction was represented by anterior displacement of the infundibular septum and ventriculoinfundibular fold (in hearts with a subpulmonary malalignment ventricular septal defect) or by redundant muscle tissue; inflow tract obstruction was represented by hypoplasia of the tricuspid valve. It is concluded that the combination of transposition of the great arteries and narrowing of the aorta is always accompanied by right ventricular outflow or inflow tract obstruction or both; the right ventricular abnormalities are probably responsible for the presence of the aortic arch anomalies by reducing aortic flow during morphogenesis. Successful surgical treatment of this complex anomaly is feasible in selected cases.     

Transposition of the great arteries and narrowing of the aortic arch. Emphasis on right ventricular characteristics.

Of 279 patients with transposition of the great arteries, 14(5%) had narrowing of the aorta, including local coarctation (6), isthmus hypoplasia (6), isthmus atresia (1), and kinking of the aorta (1). There were six deaths in 10 surgically treated patients; in addition four patients died before operation. Two of the four survivors had a subpulmonary malalignment ventricular septal defect with angiocardiographic narrowing of the right ventricular outflow tract, two had evidence of redundant muscle tissue obstructing the right ventricular outflow tract. Necropsy showed the presence of anatomical right ventricular outflow tract or inflow tract obstruction or both in all 10 cases. Outflow tract obstruction was represented by anterior displacement of the infundibular septum and ventriculoinfundibular fold (in hearts with a subpulmonary malalignment ventricular septal defect) or by redundant muscle tissue; inflow tract obstruction was represented by hypoplasia of the tricuspid valve. It is concluded that the combination of transposition of the great arteries and narrowing of the aorta is always accompanied by right ventricular outflow or inflow tract obstruction or both; the right ventricular abnormalities are probably responsible for the presence of the aortic arch anomalies by reducing aortic flow during morphogenesis. Successful surgical treatment of this complex anomaly is feasible in selected cases.     

Comparison of left ventricular outflow tract obstruction in interruption of the aortic arch and in coarctation of the aorta, with diagnostic, developmental, and surgical implications

A morphometric comparison of the anatomic causes of left ventricular (LV) outflow obstruction in interruption of the aortic arch and in coarctation of the aorta with ventricular septal defect (VSD), based on 30 postmortem cases of each, revealed that posterior malalignment of the conal septum with a conoventricular VSD was significantly more prevalent with interruption (93%) than with coarctation (47%) (p <0.001). The ratio of the aortic valve diameter-to-the pulmonary valve diameter, which provided a quantitative index of the degree of posterior conal septal malalignment and of the consequent LV outflow tract obstruction at and immediately below the level of the aortic valve, was significantly smaller with interruption (相似文献   

Interruption of the aortic arch and malformative cardiac lesions requiring repair under extracorporeal circulation. Apropos of 3 cases

Interruption of the aortic arch is practically always associated with intracardiac malformations of variable complexity, at the least, a ventricular septal defect. Surgery is usually performed in two stages: aortic repair and pulmonary artery banding after intravenous prostaglandin administration. The second stage comprises debanding and repair of the intracardiac lesions under cardiopulmonary bypass. However, in some cases, interruption of the aortic arch is associated with intracardiac lesions which necessitate correction under cardiopulmonary bypass from the onset, this was the situation in two of the three cases described by the authors: aorto-pulmonary window, a lesion which can only be corrected under circulatory arrest and deep hypothermia. One of these two children, operated in the neonatal period, did not survive: the other, operated at 6 weeks, had an excellent result. In the third case, the association of tricuspid atresia and a restrictive ventricular septal defect necessitated enlargement of the septal defect and therefore, open heart surgery under circulatory arrest; the results were favourable.     

Surgical management of the infant with coarctation of the aorta and ventricular septal defect.

Clinical and cardiac catheterization data were collected from 39 infants with coarctation of the aorta and ventricular septal defect, 31 of whom were initially managed only by surgical repair of coarctation. Data were analyzed to determine mortality, morbidity, outcome and factors that might predict survival or the need for septal defect closure. Of the eight patients who did not require surgical treatment before 3 months of age, seven underwent coarctation repair alone at a mean age of 2.3 years. Of the 23 infants managed with coarctation repair alone, before age 3 months, 9 needed no additional surgical treatment and 6 required early and 8 required late repair of the ventricular septal defect. Seven infants underwent coarctation repair and simultaneous pulmonary artery banding and one eventually required debanding after spontaneous closure of the septal defect. The overall mortality rate in this series was 10.3% (mean follow-up time 5.7 years). Of 39 infants, 16 (41%) never required a second operation for ventricular septal defect closure. For patients who had only coarctation or coarctation repair with pulmonary artery banding at less than 3 months of age, ventricular septal defect size was categorized as small (less than 0.5 cm/m2), moderate (less than 1 cm/m2) or large (greater than 1 cm/m2) on the basis of defect size at operative repair or echocardiographic or angiographic assessment. Defect size did not necessarily correlate with the need for operative repair.(ABSTRACT TRUNCATED AT 250 WORDS)     

Morphology of ventricular septal defect associated with coarctation of aorta.

It is now well established that the morphology of the ventricular septal defect associated with interrupted aortic arch is such as to compromise blood flow to the ascending aorta. Though there is some evidence that a similar mechanism may be operative in coarctation with ventricular septal defect, we are unaware of any necropsy study of this hypothesis. We therefore studied 25 hearts from the Heart Museum of Children's Hospital of Pittsburgh with coarctation and ventricular septal defect. Four had a malalignment defect with left ventricular outflow tract obstruction such as is seen in cases with interruption. Twenty of the remaining cases had a particular form of perimembranous defect with aortic overriding. The defects were partially closed by tricuspid valve tissue, the left ventricular outflow tract being further narrowed by various anatomical lesions. In addition, mitral valve malformations were present in 14 cases. The anatomy of the group as a whole suggests a possible reduction of aortic blood flow during fetal development. The clinical implications of these findings remain to be evaluated.     

婴幼儿主动脉缩窄合并心内畸形的Ⅰ期手术纠治

目的:总结主动脉缩窄合并心内畸形的外科治疗经验。方法:回顾分析2007年1月至2011年6月我院收治的婴幼儿主动脉缩窄合并心内畸形Ⅰ期手术治疗的临床病例。共计42例,其中男性26例,女性16例。平均年龄(1.6±0.9)岁(6 d～3岁),平均体质量(7.2±2.5)kg(2.8～12kg)。主动脉缩窄合并心内畸形:37例合并室间隔缺损,5例合并房间隔缺损,7例同时合并主动脉瓣二瓣畸形,手术方法采用双切口I期纠治5例,正中切口I期纠治37例,其中锁骨下动脉翻转法(8例)、人工补片法(6例)、端端吻合法(15例)、端端吻合+补片法(13例)。结果:本组42例中,死亡1例,术后9 d死于肺部感染,病死率2.4%。术后均复查心脏彩超显示降主动脉血流通畅,无明显狭窄,3例提示降主动脉内仍存在20～40 mmHg(1 mmHg=0.133 kPa)压差,心脏彩超示吻合口处狭窄,血流速度增快。结论:婴幼儿主动脉缩窄合并心内畸形采用深低温选择性脑灌注方法经胸骨正中切口I期手术治疗可以达到满意疗效。彻底切除缩窄段及导管组织是手术成功的关键。     

Two-dimensional echocardiographic features of interruption of the aortic arch

The 2-dimensional echocardiographic features of interruption of the aortic arch are presented based on analysis of the echocardiograms and angiograms from 8 infants: 2 with type A and 6 with type B interruption. Each infant had a patent ductus arteriosus, 6 had a conoventricular septal defect with leftward deviation of the conal septum, 1 had truncus arteriosus with truncal valve stenosis, and 1 had a distal aortopulmonary septal defect with an intact ventricular septum. Echocardiographic images obtained from the suprasternal notch or from a high parasternal approach demonstrated the interruption of the aortic arch and continuation of the patent ductus arteriosus into the descending aorta. These findings were compared with those in infants with aortic atresia and a hypoplastic ascending aorta or discrete coarctation of the aorta with tubular hypoplasia of the aortic arch. Types A and B interruption of the aortic arch were easily differentiated and the caliber of the patent ductus arteriosus was assessed. The characteristic conoventricular septal defect was readily visualized from an apex 2-chamber view or from a subcostal sagittal plane view. With this information subsequent angiography can be more expeditiously performed in this group of critically ill infants.     

复杂性动脉导管未闭的外科治疗

目的 :介绍复杂性动脉导管未闭 (PDA)诊断和手术治疗。方法 :采用胸骨正中切口。一期治疗PDA和心内伴发畸形。依据导管粗细 ,肺动脉扩张程度 ,采用结扎法和深低温低流量法经肺动脉缝闭PDA。要确认PDA周围的解剖 ,包括主动脉、左、右肺动脉 ,以免误扎。结果 :本组 72例 ,女性占6 7% ;PDA直径 0 3～ 1 8cm。伴发心内畸形中以室间隔缺损为主占 6 0 % ,法洛四联症 18%。死亡 1例 ,系合并室间隔缺损伴主动脉弓离断 ,误扎生命依赖导管。结论 :一期治疗PDA及伴发的心内畸形 ,避免二次手术的损伤 ,为安全、有效的治疗方法。及时发现恰当处理PDA ,决定治疗的结果 ,需特别引起重视。     

Morphological characterisation of ventricular septal defects associated with coarctation of aorta by cross-sectional echocardiography.

Cross-sectional echocardiograms of 18 neonates and infants with coarctation of the aorta and ventricular septal defect were retrospectively assessed. With a combination of subcostal and precordial cuts the site and anatomical relations of the defects were determined. In one case there was a muscular trabecular ventricular septal defect. Three had a malalignment defect with associated left ventricular outflow tract obstruction. In two there was a doubly committed subarterial defect with associated malalignment of the point of continuity between the aortic and pulmonary valves and the crest of the trabecular septum. Twelve cases had a perimembranous defect, with varying degrees of extension into the inlet, trabecular, or outlet septum. In this group 10 had associated aortic override, with varying degrees of left ventricular outflow tract narrowing. Abnormal insertion of the tricuspid valve was observed in 10 cases, such that it partly obscured the ventricular septal defect. Thus in most cases of coarctation with ventricular septal defect, the morphology of the ventricular septal defect and ventricular outflow tracts is such that left ventricular ejection is directed towards the pulmonary artery rather than the aorta.     

"Acquired" discrete subvalvular aortic stenosis: natural history and hemodynamics

Discrete subvalvular aortic stenosis is a progressive lesion. Most reported cases have had a measurable left ventricular-aortic gradient that progressed in severity. This report describes 35 patients in whom no significant left ventricular-aortic obstruction was noted at initial cardiac catheterization, but who later were shown to have significant subvalvular aortic stenosis. In 24 of the 35 cases, absence of a significant left ventricular-aortic gradient (less than or equal to 10 mm Hg) was documented at initial cardiac catheterization. In 11 patients, a left ventricular-aortic pressure gradient was not obtained or not sought in the absence of clinical evidence of an obstructive lesion. In each case, discrete subaortic stenosis was not noted on angiography. Associated lesions included ventricular septal defect in 7, patent ductus arteriosus in 12, coarctation of the aorta in 8, pulmonary stenosis in 3, atrioventricular canal in 2 and miscellaneous lesions in 3 cases. The 35 patients had documentation of subvalvular aortic stenosis 3 months to 19 years after their initial study based on repeat catheterizations in 26, echocardiography in 6 or discovery at surgery in 3 cases. There were eight children with coarctation and no left ventricular-aortic gradient who developed significant subvalvular stenosis at a median of 2 years 9 months after initial cardiac catheterization. Of 30 patients with associated lesions, 23 had surgical intervention before development of subvalvular aortic stenosis, but only 5 of 17 patients with ventricular septal defect had surgical repair or palliation specifically for the interventricular communication before development of subvalvular aortic stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)     

Ventricular septal defect with congenital mitral valve disease: long-term results of corrective surgery

BACKGROUND: A retrospective analysis of the mortality, morbidity and long-term follow-up of patients undergoing corrective surgery for ventricular septal defect and congenital mitral valve disease is presented. METHODS AND RESULTS: Between January 1991 and December 2000, 69 consecutive patients aged 2 months to 45 years (median 18 months) underwent repair of ventricular septal defect and associated mitral valve disease. In 52 patients (75%), the ventricular septal defects were located in the perimembranous and subarterial area. Forty-six patients had congenital mitral incompetence and 23 had congenital mitral stenosis. The ventricular septal defect was repaired through the right atrium in all. Sixty-five patients underwent reconstruction of the mitral valve and 4 underwent primary mitral valve replacement. Another 4 patients underwent mitral valve replacement after a failed repair. Associated procedures included: patent ductus arteriosus ligation (n=12), aortic valve replacement (n=6), coarctation repair (n=13), interrupted aortic arch repair (n=1), atrial septal defect closure (n=17) and Takeuchi repair (n=1). There were 6 early deaths (8.6%). Three deaths were due to pulmonary arterial hypertensive crisis and one due to residual mitral stenosis. One death was due to intractable congestive heart failure. Another patient died due to persistent low cardiac output. Follow-up ranged from 6 months to 120 months (mean 64.4+/-33.6 months). Reoperation was required in 22 patients, mainly for recurrent/residual mitral valve dysfunction or hemodynamically significant left ventricular outflow tract obstruction. There were 4 late deaths, 2 due to residual mitral stenosis and the other 2 as a result of a thrombosed prosthetic valve. At 10 years, the actuarial survival rate was 850+/-5.0%, and freedom from reoperation was 45%+/-10.0%. CONCLUSIONS: Reconstruction of the mitral valve along with closure of VSD is possible in most cases. However, careful follow-up is recommended to detect changes in the mitral valve status over a course of time.     

Profound hypothermic circulatory arrest in management of aortic aneurysms

A total of 15 patients having aneurysms of aorta were operated from June 1997 to December 1998 using deep hypothermic circulatory arrest as a modality of brain protection. There were 12 males and 3 females. The age ranged from 19 years to 74 years and the mean age was 44.9 years. Nine patients had aneurysms of ascending aorta (group I), one had aneurysm of ascending aorta and arch of aorta (group II), four had aneurysm of the distal aortic arch (group III) and one patient had thoracoabdominal aortic aneurysm (group IV). In group I, six patients underwent Bentall procedure, two underwent Wheat procedure and one patient had repair of pseudoaneurysm of ascending aorta. The only patient in group II had his ascending aorta and arch replaced, with reimplantation of left common carotid and innominate artery. In group III, three patients had interposition Gelseal graft and one had repair of the tear in distal aortic arch. The lone patient in group IV had interposition Gelseal graft of thoracoabdominal aorta. The hypothermic circulatory arrest was used in all of them for brain and/or spinal cord protection. Retrograde cerebral perfusion was used in two patients. There were two (13%) operative deaths. One patient died of cerebrovascular accident on eighth post-operative day and second died of inadequate surgical repair. There was one instance of left hemiparesis secondary to an infarct in right frontoparietal region. To conclude, hypothermic circulatory arrest could provide an adequate brain protection for aortic aneurysm surgery. Retrograde cerebral perfusion could be an adjuvant when the anticipated time of hypothermic circulatory arrest is likely to exceed 45 minutes.     

Long-term results after primary one-stage repair of transposition of the great arteries and aortic arch obstruction.

OBJECTIVES: The study was designed to evaluate perioperative and late results after primary, single-stage arterial switch operation (ASO) associated with aortic arch obstruction repair. Outcome of patients with more than five years of follow-up were analyzed. BACKGROUND: The treatment of patients with transposition of the great arteries, or other forms of ventriculoarterial discordance suitable for an ASO, with coexisting arch obstruction is a difficult task. Single-stage repair has become the treatment of choice at many institutions, but large series with long-term results are seldom reported. METHODS: Between 1990 and 1998, a primary operation including aortic arch repair through a midline sternotomy was performed in 38 patients. The relief of arch obstruction was accomplished during a period of hypothermic circulatory arrest, employing a wide pericardial patch to enlarge the inner curvature of the entire arch in most patients. RESULTS: There were nine (24%) hospital deaths. None could be directly related to aortic arch repair, but additional risk factors for an ASO were common (right ventricular hypoplasia, complex coronary anatomy, uncommon relationship between the great vessels or severe pulmonary hypertension). There were no late deaths. Four patients required cardiac reoperation, whereas three underwent successful treatment of recurrent coarctation with balloon angioplasty. CONCLUSIONS: Infants with ventriculoarterial discordance and aortic arch obstruction represent a high-risk subgroup of candidates for an ASO. Despite a non-negligible operative mortality, single-stage primary repair represents the treatment of choice, and follow-up of operative survivors is favorable. Pericardial patch enlargement is a reliable technique for arch obstruction repair.     

Distal aortopulmonary septal defect,aortic origin of the right pulmonary artery,intact ventricular septum,patent ductus arteriosus and hypoplasia of the aortic isthmus: A newly recognized syndrome

The association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum and Interruption or coarctation of the aortic isthmus has not previously been reported as a syndrome. This combination of anomalies was encountered in five new patients and was previously reported in three. Two patients have undergone surgery with successful results. In contrast to the sagittally oriented conventional proximal aortopulmonary septal defect, the patients in this series had a more distal type of defect, possibly representing a partial persistence of the common arterial trunk. The pulmonary arterial bifurcation may malattach to this undivided truncal segment and, as a result, the right pulmonary artery may be partially or completely shifted into the aorta. This abnormal right pulmonary arterial origin may lead to “steal” from the aortic flow during embryogenesis and to hypoplasia of the aortic arch. This concept is supported by the angiographic observation that the greater the rightward displacement of the right pulmonary artery, the greater the hypoplasia of the arch. The diagnostic angiographic sign is a strikingly high origin of the right pulmonary artery together with aortic arch hypoplasia or atresia. Closure of the aortopulmonary septal defect with implantation of the right pulmonary artery in the pulmonary trunk and repair of the aortic arch anomaly is the recommended surgical treatment.