Complete repair of the interrupted aortic arch by an anterior approach. Apropos of 9 cases

Interrupted aortic arch is a duct-dependent heart disease, and this is why the use of prostaglandins E, which keep the ductus arteriosus open to feed the descending aorta, has transformed its formerly sombre prognosis. The authors report the cases of 9 neonates and infants presenting with interrupted aortic arch associated with ventricular septal defect who underwent total repair of the malformations under extracorporeal circulation. Five children were less than 10 years' old. The aortic lesions were of type A in 2 cases (interruption distal to the left subclavian artery) and of type B in 7 cases (interruption between the left carotid and subclavian arteries). In the second group the lesions were of type B1 in 5 cases (without retro-oesophageal right subclavian artery) and of type B2 in 2 cases (right retro-oesophageal artery distal to the interruption). There was one death (11 p. 100). So far, no child has been reoperated upon for residual aortic obstruction or reopened ventricular septal defect. This technique of total repair in one stage, with closure of the ventricular septal defect, seems to be preferable to the two-stage technique in which stage one includes, in addition to aortic arch repair, cerclage of the pulmonary artery, a procedure with numerous disadvantages and a higher overall mortality rate.     

Echocardiographic assessment of interrupted aortic arch

BACKGROUND: In patients with interrupted aortic arch echocardiography provides detailed information about the anatomy of the aortic arch and the associated cardiac anomalies. Only a few reports have evaluated the reliability of this non-invasive diagnostic procedure by correlation with angiographic and surgical findings. METHODS: From 1988 through 1993, 45 infants with interrupted arch underwent surgical repair (mean age 13.02 days). Of the patients, 33 had interruption of the arch between the left common carotid and subclavian arteries; 25 patients had a ventricular septal defect, and the remaining 20 had coexisting complex congenital heart defects. Preoperative diagnosis was made exclusively by echocardiography in 25 of the patients. Accuracy of echocardiographic diagnosis was evaluated retrospectively by comparing preoperative studies with angiography and surgical reports. We then investigated whether the morphologic features of the interrupted arch might influence surgical procedure or outcome. RESULTS: Intracardiac anatomy was accurately diagnosed by echocardiography in all cases; in 2 patients angiography provided additional information concerning the morphology of the aortic arch. Operative notes described differences in morphology of the arch in 7 patients, but these did not influence the surgical procedure. Direct anastomosis of the interrupted segments was possible in 38 patients, and 36 patients underwent primary intracardiac repair. Echocardiographic measurements revealed that the diameter of the ascending aorta was related to the number of vessels originating from the proximal aortic arch. The distance between the interrupted segments was significantly different according to the site of interruption, but not between cases with an isolated ventricular septal defect versus those with complex heart disease. It did not influence the method of arch repair, nor was it related to recurrent or residual obstruction. CONCLUSION: Preoperative echocardiography offers accurate and complete diagnosis in the critically ill neonate with interrupted aortic arch and associated intracardiac abnormalities.     

Interrupted aortic arch. A series of 15 patients

Between 1983 and 1989, 15 children underwent surgical repair of interrupted aortic arch at 1 to 20 days of age. The anatomical form was a Celoria and Patton type B in all patients with an associated perimembranous ventricular septal defect in all but one who had multiple ventricular septal defects, and patent ductus arteriosus. Six children had a retro-esophageal right subclavian artery, two had subaortic stenosis and two had a right-sided descending thoracic aorta. In two children with severe hypoplasia of the ascending aorta the repair was performed in one stage with two deaths due to left ventricular failure. In the other B cases, a two-stage repair was carried out. The reconstruction of the aortic arch varied according to the individual case. All children had pulmonary artery banding. Seven children survived longer than 30 days. Six of them later underwent a complete repair. The only survivors were those patients in whom the neo-aortic arch grew harmoniously. The authors conclude that: a two-stage repair gave disappointing results in this series of consecutive patients, mainly because of the poor quality of the reconstruction of the aortic arch by thoracotomy.     

Is banding of the pulmonary trunk obsolete for infants with tricuspid atresia and double inlet ventricle with a discordant ventriculoarterial connection? Role of aortic arch obstruction and subaortic stenosis.

Banding the pulmonary trunk may exacerbate or promote the development of subaortic stenosis in patients with double inlet ventricle or tricuspid atresia with a dominant left ventricle and discordant ventriculoarterial connection and, therefore, may be an inappropriate palliative procedure for such patients. To examine this possibility, 102 consecutive infants were studied who presented with this anatomy between 1972 and 1987. Obstruction of the aortic arch was present in 52 patients. In 28 patients (17 with aortic arch obstruction), subaortic stenosis was already apparent at presentation. Of the remaining 74 patients, 19 received no palliative surgery and 55 underwent banding of the pulmonary trunk either with (n = 22) or without (n = 33) aortic arch repair. Outcome was significantly worse in patients with associated aortic arch obstruction. All such patients either died or developed subaortic stenosis by 3 years of age (survival free of subaortic stenosis 0 of 22 versus 22 of 33 for patients with isolated banding of the pulmonary trunk, p less than 0.001). After isolated banding, there was a lower ratio of the ventricular septal defect to ascending aorta diameters at presentation in the patients who developed subaortic stenosis than in the patients who did not (0.60 +/- 0.08 versus 1.03 +/- 0.15, p less than 0.001). Of the latter, 18 (95%) of 19 patients fulfilled criteria for a Fontan procedure at recatheterization. Thus, the presence of aortic arch obstruction is associated with rapid development of subaortic stenosis after banding of the pulmonary trunk. Alternative initial surgery, even though high risk, may be indicated. In the absence of such obstruction, banding the pulmonary trunk can be performed at reasonable risk and, provided that the ventricular septal defect is of adequate size, satisfactorily prepares most patients for a later Fontan procedure.     

Interrupted aortic arch in an adult single-stage extra-anatomic repair

Interrupted aortic arch is a rare congenital malformation of the aortic arch that occurs in 3 per million live births. Defined as a loss of luminal continuity between the ascending and descending portions of the aorta, this anomaly entails a very poor prognosis without surgical treatment. To our knowledge, the world medical literature contains only 12 reports of isolated interrupted aortic arch diagnosed in adults. Nine of these patients underwent successful surgical repair, but 1 died during the early postoperative period. We describe a 10th successful surgical repair, which involved a 42-year-old woman who had an asymptomatic type B interrupted aortic arch (characterized by interruption between the left subclavian and left carotid arteries). We performed a single-stage extra-anatomic repair by placing a 16-mm extra-anatomic Dacron graft between the ascending and descending portions of the thoracic aorta and by interposing a 7-mm extra-anatomic Dacron graft between the 16-mm graft and the left subclavian artery. The patient recovered uneventfully and continued to do well 6 months later.     

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.     

Rapid formation of collateral arteries in a neonate with interruption of the aortic arch

In a neonate born prior to term with a weight of 1825 grams, and diagnosed prenatally as having atrioventricular septal defect and Down's syndrome, we found the aortic arch to be interrupted between the left carotid artery and the left subclavian artery, with the arterial duct being the only route of distal perfusion. Three days later, however, echocardiographic interrogation revealed marked collateral connections between the aortic arch and the descending aorta, the picture then mimicking coarctation rather than interruption of the aortic arch. The rapid development of the collateral arteries was confirmed by magnetic resonance imaging and during cardiac surgery.     

Interruption of the aortic arch associated with deletion of chromosome 22q11 is associated with a subarterial and doubly committed ventricular septal defect in Japanese patients.

OBJECTIVES: The purpose of this study was to clarify the clinical characteristics of interruption of the aortic arch associated with chromosome 22q11deletion. BACKGROUND: About half of patients with interruption of the aortic arch between the left common carotid and the left subclavian artery have deletion of chromosome 22q11. METHODS: In total, 20 patients with interruption of the aortic arch were studied with fluorescence in situ hybridization using peripheral lymphocytes and a DiGeorge syndrome chromosomal probe (Oncor N25). Cardiovascular anomalies in these patients were diagnosed by cross-sectional echocardiography and angiocardiography, and were confirmed at intracardiac repair. RESULTS: Of 13 patients with interruption between the left common carotid artery and the left subclavian artery, seven had the deletion. All 7 also showed thymic hypoplasia and hypocalcemia, together with a nasal voice and peculiar facies. Six of the seven patients had complete deficiency of the muscular outlet septum, with the defect extending to the perimembranous area. Such complete absence of the muscular outlet septum was not present in any of the patients without the deletion. CONCLUSIONS: Interruption of the aortic arch between the left common carotid and the left subclavian artery, absence of the thymus, and complete absence of the muscular outlet septum, were characteristic in Japanese patients with interruption of the aortic arch associated with deletion of chromosome 22q11.     

Balloon dilation angioplasty of postoperative aortic obstructions

Balloon dilation angioplasty (BDA) was attempted 29 times in 27 patients, aged 3 months to 22 years, with postoperative aortic obstructions. Previous operations included end-to-end anastomosis (n = 10), subclavian flap angioplasty (n = 7) and patch angioplasty (n = 3) for aortic coarctation, end-to-end anastomosis for interrupted aortic arch type B (n = 4) and aortic arch reconstruction for hypoplastic left heart syndrome (n = 3). Two of the patients with interrupted arch had multiple areas of obstruction. Balloon sizes were between 2 and 6 times the diameter of the lesion and up to twice the diameter of the proximal transverse arch (mean 1.1). BDA was at least partially successful (more than 50% decrease in gradient and more than 30% increase in diameter) in 26 of the 29 procedures (90%). BDA failed in the 2 lesions with an initial diameter of more than 8 mm. No differences were apparent in the success rate among any of the clinical groups. Peak systolic gradient decreased from 42 +/- 14 to 14 +/- 15 mm Hg (p less than 0.01) and mean diameter increased from 4.1 +/- 2.6 to 6.8 +/- 3.2 mm (p less than 0.01). There was no mortality or significant acute morbidity associated with the procedure. After 1 to 24 months of follow-up, restenosis has occurred in only 1 patient. Aneurysm formation was found in 2 of 5 patients who had undergone repeat catheterization; both aneurysms occurred in patients with repaired interruption of the aortic arch.(ABSTRACT TRUNCATED AT 250 WORDS)     

Interrupted right aortic arch in DiGeorge syndrome.

The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.     

Interrupted right aortic arch in DiGeorge syndrome

The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.     

Atresia of the aortic arch, with a collateral artery from the right subclavian artery supplying the descending aorta

An infant of 2 months presented with absence of the femoral pulses, albeit with no signs of cardiac failure. The mother was known to have ingested Valproate during pregnancy. Echocardiography showed the aortic arch to be interrupted between the left common carotid and left subclavian arteries, so-called type B interruption, in the setting of an intact ventricular septum. Angiography, and multislice computed tomography, revealed the descending aorta to be supplied by a collateral artery originating from the right subclavian artery. Corrective surgery was successfully performed, but revealed an atretic segment of the arch at the site of interruption of flow.     

Aberrant patent ductus arteriosus associated with double brachiocephalic trunk and interrupted left subclavian artery

Although patent ductus arteriosus (PDA) is the most common type of extracardiac shunt, aberrant PDA associated with other cardiac malformations as double brachiocephalic trunk and interrupted left subclavian artery is extremely rare. To the best of our knowledge, the literature contains no other report of a patient who has PDA originated from left subclavian artery associated with ventricular septal defect (VSD), double brachiocephalic trunk and interrupted left subclavian artery.     

Interrupted aortic arch: brief review and summary of an eighteen-year experience

Interrupted aortic arch (IAA) is an uncommon congenital anomaly representing approximately 1% of congenital heart disease. More than 97% of the cases also have associated cardiac anomalies complicating their treatment. Because the median age at death in untreated cases is 10 days, this condition usually occurs as a complicated neonatal surgical emergency. There are three types of IAA: Type A is interrupted distal to the left subclavian artery. Type B, which is the most common form, is interrupted between the left common carotid and the left subclavian arteries. Type C, which is the most uncommon type, is interrupted between the innominate and left common carotid arteries. Since the first patient with this congenital anomaly was treated at our institution in 1965, 39 patients have undergone surgical treatment. Two of these (4.6%) had no associated intracardiac lesions, and both survived bypass grafting without complications. There were 12 Type A (30%), 26 Type B (67%) and one Type C (3%). A variety of individualized techniques were used to repair the defects. The three basic techniques were (1) direct aortic to aortic anastomosis bridging the gap, (2) "turndown" or "turnup" of one of the arch vessels to the aorta across the gap, or (3) bypass of the interruption with graft material. Because aortic arch and associated cardiac anomalies represent a neonatal surgical emergency, our therapeutic plan consists of treatment for biventricular failure and PGE(1) infusion to maintain ductal patency, and surgical correction as soon as feasible.     

Interrupted aortic arch in an adult female

Interrupted aortic arch is a rare and usually lethal malformation, representing approximately 1% of congenital heart disease. This presents as a missing segment of the aortic arch and is divided into three types: A-called extreme form of coarctation, and is characterized by disruption of aorta's continuity distal to the left subclavian artery (30-40%), B-disruption between the left subclavian and the left carotid arteries (55-60%), and C-the most uncommon type, interruption proximal to the left common carotid artery. The suspicion of coarctation of the aorta can be made from a combination of physical findings including systolic ejection murmur, the murmurs of collateral blood vessels, diminished or absent femoral pulse, and difference in blood pressure between arms and legs. Interrupted aortic arch is an extremely rare anomaly in adult patients. To our knowledge, the world medical literature contains only about 13 reports of interrupted aortic arch diagnosed in adults.     

Interruption of the aortic arch at the isthmus with DiGeorge syndrome and 22q11.2 deletion.

A 6-day-old male with interruption of the aortic arch at the isthmus (type A) had the typical phenotype of DiGeorge syndrome. There was also a doubly committed juxta-arterial ventricular septal defect and an unobstructed left ventricular outflow tract. Hypoplasia of the thymus was confirmed during a modified Blalock-Park operation. He had persistent hypocalcemia, and was susceptible to infection. He was subsequently revealed by the use of fluorescence in situ hybridization analysis to have 22q11.2 deletion. Interruption of the aortic arch at the isthmus is presumed to reflect abnormal fetal hemodynamics, and is considered a distinct pathogenetic entity from interruption between the left common carotid and subclavian arteries, the latter being the variant more frequently associated with DiGeorge syndrome. In our case, the 22q11.2 deletion likely played a major role in the etiology of the interrupted aortic arch.     

Type B Interrupted Left Aortic Arch with Isolated Right Subclavian Artery

Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left‐sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).     

头臂血管成形术治疗成人主动脉弓及头臂血管发育不良

目的:总结并探索主动脉弓及头臂血管发育不良的手术治疗方式。方法:2009年2月至2011年12月,北京安贞医院大血管中心对4例主动脉弓及头臂血管发育不良的患者行主动脉弓成形术治疗,手术均采用右侧腋动脉和股动脉及右心房插管建立体外循环,在深低温停循环选择性脑灌注下进行,沿主动脉弓长轴靠近头臂血管根部横行切开主动脉弓,切口两端达正常段,再纵形剖开3枝头臂血管开口及近段(狭窄段)前壁,分别将无名动脉和左颈总动脉、左颈总动脉和左锁骨下动脉相邻血管壁连续缝合在一起,将3枝血管融合成一个共同腔的后壁,再用人工血管片作为前壁补片加宽主动脉弓及头臂血管共同腔,以完成对发育不良的主动脉弓和头臂血管的手术矫治。结果:患者术后均恢复顺利,复查结果显示主动脉弓及头臂血管形态良好,上下肢压差<20 mmHg(1 mmHg=0.133 kPa)。结论:将发育不良的头臂血管近段剖开融合成一个共同腔,再用人工血管片加宽可理想地矫正主动脉弓及头臂血管发育不良。     

One-stage repair of cardiac and arch anomalies without circulatory arrest

Between 1999 and 2002, 23 patients underwent single-stage complete repair of cardiac anomalies and aortic arch obstruction, without circulatory arrest. Median age was 1.2 years. Intracardiac defects included ventricular septal defect in 9, double-outlet right ventricle in 6, d-transposition of the great arteries and ventricular septal defect in 2, subaortic obstruction in 3, and atrial septal defect in 3. Fourteen patients had coarctation of the aorta, 6 had coarctation with hypoplastic aortic arch, and 3 had interrupted aortic arch. Simple techniques were employed such as cannulation of the ascending aorta near the innominate artery and maintaining cerebral and myocardial perfusion. After correction of arch obstruction, intracardiac repair was undertaken. The mean cardiopulmonary bypass time was 169 min, aortic crossclamp time was 51 min, and arch repair took 16 min. There was no operative mortality or neurological deficit. In follow-up of 1-43 months, no patient had residual coarctation. This simplified technique avoids additional procedures, reduces ischemic time, and prevents problems related to circulatory arrest.     

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.