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.     

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.     

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.     

Surgery without catheterization of aortic coarctation in newborn infants and infants under 3 years of age

A retrospective study of 150 children under 3 months of age who underwent repair of coarctation of the aorta in the same center (between 1972 and 1987) was undertaken to assess the problems posed by surgery without cardiac catheterisation or angiography. The patients were divided into two groups: Group A, comprising 104 children operated after invasive investigations, and Group B, comprising 46 children operated on Doppler echocardiographic data alone. The two populations were comparable and "hypoplastic aortic arch" type coarctation was present in over 60% of cases. However, there were more ventricular septal defects in Group B (67.5%) than in Group A (49%). There were no significant diagnostic errors in Group B (one case of interrupted aortic arch diagnosed at surgery). The 1 month survival was the same in the two groups (82%). The indications of pulmonary artery banding were less frequent in Group B although there were more ventricular septal defects in these patients. These results confirm the value of Doppler echocardiography in the context of urgent surgery of congenital heart disease.     

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.     

Coarctation of the aorta and Fallot's tetralogy. Apropos of 2 cases

Two cases of the association of Fallot's tetralogy and coarctation of the aorta are reported. In both cases there was a right aortic arch. In addition, the second case had a cervical aorta and a left subclavian artery isolated from the aorta and vascularised by the left vertebral artery. The two children underwent complete repair: the first patient had a Waterston shunt followed by repair of the Fallot's tetralogy, followed by repair of the coarctation; the second patient first had a Gore-tex tube inserted from the ascending to the descending aorta and then underwent repair of the Fallot's tetralogy. The association of a coarctation with an obstructive lesion of the right heart is very rare; only two cases have previously been reported: one Fallot's tetralogy and one tricuspid atresia with pulmonary stenosis. Both cases also had a right aortic arch. This seems to go against the haemodynamic theory of coarctation: blood flow in the ascending aorta increases while flow in the pulmonary artery decreases. Coarctation could be caused by a partial involution of the terminal segment of the right primitive dorsal aorta as proposed in cases of coarctation with a right sided aortic arch. The second case also presented two uncommon features: a cervical aortic arch and a left subclavian artery isolated from the aorta.     

Determinants of survival following repair of interrupted aortic arch in infancy.

Between January 1971 and March 1987, surgery was performed in 26 infants with interrupted aortic arch. At operation the 14 boys and 12 girls weighted between 1.71 and 4.23 kg (mean +/- SD = 3.1 +/- 0.63 kg) and ranged in age from 2 to 90 days (13 +/- 18 days). The interruption was distal to the left subclavian artery in 4 (15%), between the left carotid and subclavian arteries in 20 (77%) and between the brachiocephalic (innominate) and left carotid arteries in 2 (8%). Associated complex cardiac lesions in 8 patients included complete transposition (2), common arterial trunk (2), aortopulmonary window (2), double inlet left ventricle (1) and tricuspid atresia (1). The remaining patients had an isolated ventricular septal defect. The arch was reconstructed with a prosthetic conduit in 14 patients; by a direct anastomosis in 6; using the subclavian artery in 3; and with the pulmonary trunk and the arterial duct in 2. Twenty patients (77%) underwent palliative surgery as the first stage of management, and banding of the pulmonary trunk was also performed in 16 of these. Five patients (19%) underwent primary complete repair of the interruption and intracardiac anomalies. One patient (4%) died soon after thoracotomy for palliative surgery. Of the 15 (57%, 70% confidence limits CL = 46-69%) early deaths, 7 occurred in patients with complex associated defects and 4 occurred when single stage repair was attempted. Survival following first-stage palliative surgery for arch interruption with isolated ventricular septal defect was 64% (9/14) [70% CL = 47-79%]. All of these patients subsequently underwent complete repair. Chi-squared and t-tests showed the year of operation and the type of operation (two-stage repair) to be associated with improved survival. It is concluded that a two-stage repair of interrupted aortic arch offers a reasonable alternative to primary complete correction and will lead to satisfactory subsequent repair in most cases.     

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.     

Coarctation of the aorta in infancy

Twenty-five infants under 1 year of age (mean weight 3.4 kg) underwent repair of coarctation of the aorta between the years 1965 and 1982. Three patients had coarctation only, three had coarctation with patent ductus arteriosus (PDA), and 19 had associated intracardiac anomalies. Eleven patients underwent resection of the aorta and end-to-end anastomosis. Eight had subclavian flap arterioplasty, five had patch graft arterioplasty, and one had subclavian-to-aortic anastomosis. Additional procedures were performed on seven patients: banding of the pulmonary artery on one, repair of total anomalous pulmonary venous drainage on one, mitral valve replacement on one, aortic valvotomy on one, and aortic valvotomy plus pulmonary artery banding on one. Twenty-one survived the operation. All patients who died had associated intracardiac anomalies. The 21 survivors have been followed from 3 months to 13 years, with three late deaths that were associated with intracardiac anomalies. Five of the survivors underwent additional second operations: one had repair of the re-coarctation, one had replacement of the prosthetic mitral valve, one had aortic valvotomy, and two had pulmonary artery debanding and closure of a ventricular septal defect. Two of the 18 surviving patients have mild hypertension associated with a residual gradient. The others are in good health.     

Interrupted aortic arch: an eight-year experience

INTRODUCTION: Interrupted aortic arch (IAA) is a rare congenital heart defect with a high mortality rate in the neonatal period. Surgical correction of associated intracardiac anomalies can be performed in a one-stage (primary) or two-stage approach. OBJECTIVES: Case review of children with IAA operated in our center and to evaluate the surgical outcomes and the occurrence of complications. METHODS: A retrospective chart review of children operated from June 1998 to October 2006. RESULTS: Twelve children (nine girls and three boys) were operated. Nine patients had ventricular septal defect with septal malalignment, two had univentricular hearts and two had transposition of the great arteries. Primary correction was performed in eleven patients (aged between two and 38 days), including two Norwood procedures. There was no early mortality. The follow-up time ranged from 30 days to six years (median 2.6 years), with late mortality of 33%. Two children required reintervention for aortic arch restenosis, which was successfully treated by catheterization. DISCUSSION: Our experience is that early primary repair of IAA has low early and late mortality and is the method of choice most situations. Late mortality depends the severity of associated anomalies.     

Cardiac malformations in the velocardiofacial syndrome

A similar pattern of congenital anomalies found in 27 children, 9 male and 18 female, aged 2 weeks to 17 years, allows the conclusion that they represent a newly recognized malformation syndrome. All patients had velopharyngeal insufficiency, a submucous or overt cleft of the secondary palate and learning disabilities. A similar facies, characterized by a long vertical face, a large fleshy nose with broad nasal bridge, flattened malar region, narrow palpebral fissures and deep overbite with retruded mandible, was usually present. Other noncardiac anomalies also occurred frequently. Twenty-three (85 percent) had congenital cardiovascular malformations whose occurrence as single or combined lesions far exceeded the expected incidence in children with congenital heart disease. A ventricular septal defect was present in 15, tetralogy of Fallot in 5. Prolapse of the right aortic cusp was present in two patients with a ventricular septal defect, and aortic valve disease with insufficiency in one patient with tetralogy. The aortic arch was right-sided in 12 patients, in conjunction with a ventricular septal defect in 5, in association with tetralogy of Fallot in 4 and in the absence of associated cardiac disease in 3. The right aortic arch descended on the right in 10 patients; in 2 with a ventricular septal defect it crossed retroesophageally to descend on the left, and in one of these it presented as the “third aortic arch syndrome.” Aberrant origin of the left subclavian artery was present in five patients with a right aortic arch. A patent ductus arteriosus had caused congestive heart failure in infancy in one patient with a small ventricular septal defect; three others with a ventricular defect had additional clinically minor anomalies. Two children were brother and sister. Their mother and the mothers of two other children showed evidence of the same syndrome, suggesting the possibility of autosomal dominant or X chromosome-linked dominant transmission.     

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.     

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.     

Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis.

Between December 1985 and April 1990, 50 infants with a variety of congenital cardiac lesions other than the hypoplastic left heart syndrome underwent surgical relief of aortic outflow obstruction by creation of a pulmonary artery to aorta anastomosis. The patients were grouped anatomically by ventriculoarterial alignment. Nineteen had normally aligned great arteries (group I); 25 had transposition of the great arteries, all with a univentricular heart of left ventricular morphology (group II); and 6 had a double-outlet right ventricle (group III). All patients had either aortic stenosis with atresia, subaortic stenosis or a restrictive ventricular septal defect. Sixteen had normal arch anatomy; 34 had arch anomalies consisting of arch hypoplasia (n = 17), coarctation (n = 11), interruption of the arch (n = 4) and complex arch anomalies (n = 2). Surgery was performed at a median age of 10 days (range 2 to 184). Of the 50 infants, 33 survived. No significant difference in early survival (30 days) was noted among the groups of varying ventriculoarterial alignment (68% group I, 72% group II, 83% group III) (p greater than 0.05). Overall actuarial survival was 63% at 18 months. Analysis of actuarial survival by arch anatomy, although not statistically significant, revealed a trend toward better survival at 18 months postoperatively in infants with normal arch anatomy (81%) than in infants with arch anomalies (54%). Of the 33 survivors, 26 have proceeded to the next surgical stage, including the Fontan procedure in 8, superior cavopulmonary anastomosis in 13 and biventricular repair in 5.(ABSTRACT TRUNCATED AT 250 WORDS)     

Supravalvar aortic stenosis: clinical and hemodynamic profile,and surgical outcome

BACKGROUND: Supravalvar aortic stenosis is the rarest of left ventricular outflow obstructions. Data on this rare entity from India are scarce. METHODS AND RESULTS: We retrospectively analyzed the data of 15 patients (13 males, mean age 15.5+/-10.18 years) with a diagnosis of supravalvar aortic stenosis confirmed by cardiac catheterization. Five patients had morphological features of Williams' syndrome. One patient had diffuse while the rest had discrete type of supravalvar aortic stenosis. Five patients did not have any associated lesions. A 9-year-old male had an ascending aortic aneurysm, and 3 patients had associated peripheral pulmonary artery stenosis. One child had a subaortic ventricular septal defect, and another had severe mitral regurgitation. Twelve patients had electrocardiographic evidence of left ventricular hypertrophy. Three patients had mild aortic valvar stenosis while 2 had aortic regurgitation. Six patients had dilated coronary arteries. Two patients with supravalvar aortic gradients of 20 and 40 mmHg were kept on close follow-up. One patient was not willing to undergo surgery while the other is awaiting surgery. Eleven patients underwent surgical correction. Dacron or pericardial patch aortoplasty was done in all the patients. In addition, one patient each underwent pulmonary artery plasty, ventricular septal defect closure, repair of ascending aortic aneurysm, and mitral valve replacement. The patient with diffuse type of supravalvar aortic stenosis underwent augmentation aortoplasty. Two patients died perioperatively. One was lost to follow-up. Two had moderate residual gradients. The rest of the patients were in New York Heart Association functional class I on follow-up of 6.3+/-4.7 years. CONCLUSIONS: Repair of supravalvar aortic stenosis by single sinus aortoplasty is safe and produces good results.     

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.     

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.     

Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, sub-aortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries

We report two rare cases of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta, associated with pulmonary atresia, a ventricular septal defect and a left aortic arch. The cases are unusual because AOLPA is more commonly associated with a right aortic arch and it is more usual for the right pulmonary artery to originate anomalously from the ascending aorta. The pulmonary blood supply to the right lung in both patients was absent and provided instead by major aorto-pulmonary collateral arteries which were stenosed at multiple levels. The AOLPA in both patients originated from the postero-lateral aspect of the ascending aorta just distal to the sino-tubular junction. Only one patient showed the more common association of an unusual aortic arch branching pattern in the form of an anomalous right subclavian artery. Neither patient was in heart failure and the chest X-ray in both revealed differential pulmonary perfusion with prominent vascularity of the left lung. Cardiac catheterisation showed systemic pressures within the anomalous left pulmonary artery. Karyotyping revealed normal chromosomes, and fluorescent in-situ hybridisation done in one patient was negative for chromosome 22q11.2 microdeletion. Both patients have been managed conservatively.     

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。