Surgery for coarctation of the aorta in infants younger than 3 months: end-to-end repair versus subclavian flap angioplasty: is either operation better?

OBJECTIVE: Recurrent coarctation is a complication which is seen at a consistent rate following all types of repair for coarctation of the aorta. Particularly disappointing late results are reported in younger infants, under 3 months of age. This retrospective analysis was undertaken to compare the outcomes on late follow-up between subclavian flap angioplasty and resection and end-to-end repair, in this age group. METHODS: Over a 12-year period, between 1982 and 1994, 86 infants under 3 months of age underwent surgical repair of coarctation (39 resections and end-to-end repair, and 47 subclavian flap angioplasty procedures). Operative mortality was not significantly different (P = 0.6) between resection and end-to-end repair (5.1%) and subclavian flap angioplasty (8.5%). All operative deaths (six patients) were in infants with associated ventricular septal defects. The mean follow-up for all patients was 7.95 years +/- 4.10 (range 0-14.5 years). The 5-year survival for resection and end-to-end repair was 87 +/- 5%, compared to 75 +/- 7% for subclavian flap angioplasty (P = 0.2). RESULTS: Recurrent coarctation occurred in nine patients who needed reoperation. The reoperation-free rates at both 5 and 10 years for resection and end-to-end anastomosis, and subclavian flap repair were 86 +/- 6% and 90 +/- 5%, respectively. The recurrence in the resection and end-to-end anastomosis group were due to constrictive scarring at the anastomosis, whereas periductal tissue and growth of posterior aortic ridge caused recurrence in the subclavian flap angioplasty group. There were no deaths during reoperation for recurrence. CONCLUSIONS: Both procedures are extremely effective for coarctation repair in young infants and run a similar risk of recurrence, which are due to completely different mechanisms. The surgeon's expertise is the major determinant of outcome.     

Aortic coarctation and complex heart disease. Surgical treatment during the first year of life

During the five years 1990 through 1994, 70 neonates and infants had surgery for coarctation of the aorta. 30 patients with complex coarctation of the aorta underwent a first stage correction with supplementary ductus ligation and pulmonary artery banding as needed. The mode of surgical repair of coarctation was end-to-end anastomosis in ten patients and patch graft in 20 patient. Mean follow-up was 766 (range 3-1812) days. Mortality after primary procedure was 16.6% (5/30) and after secondary procedure 11.1% (2/18). Three surviving patient developed recoarctation, but only one patients needed re-operation. In spite of improvement in neonatal cardiac surgery, we conclude that a staged approach is still the current treatment in most cases of complex coarctation of the aorta.     

National survey of radionuclide gastric emptying studies

The optimal surgical approach for complex aortic coarctation or an interrupted aortic arch with associated intracardiac defects is not universally agreed upon. We reviewed our experience with 18 consecutive patients (10 with coarctation, 8 with interrupted aortic arch) undergoing a 1-stage repair through median sternotomy between September of 1995 and February of 1997. Age at operation ranged from 3 days to 3 months (mean 23 days) and weight ranged from 1,700 g to 5,100 g (mean 3,350 g). Under hypothermic circulatory arrest, the aortic arch was reconstructed using native tissue-tissue anastomoses, and coexisting intracardiac anomalies were repaired by standard techniques. All patients survived the procedure and were ultimately discharged from the hospital. There were 2 late deaths in the interrupted aortic arch group, 1 during reoperation for subaortic stenosis and the other from noncardiac causes 5 months after discharge. Another interrupted aortic arch patient required a Ross-Konno procedure 8 months later. There has been no recoarctation among the 16 survivors. Thus a 1-stage repair for complex aortic arch obstruction in neonates can be accomplished with low operative risk, although long-term outcome is strongly influenced by the presence of subaortic obstruction.     

Cost-effectiveness of consensus double reading of screening mammograms

Subclavian flap aortoplasty is one of the best procedures for coarctation repair, but recoarctation still remains a problem in neonates and infants. We employed subclavian flap aortoplasty with resection of the whole layer of aortic ductal tissue in 9 patients less than 3 months of age with coarctation of the aorta and obtained satisfactory results.     

Changes in characteristics of chronic pancreatitis during the course of disease

OBJECTIVES: This study was undertaken to investigate the incidence of posttransplant recoarctation of the aorta, delineate the mode of presentation, identify risk factors that predict recoarctation and examine the results of intervention for posttransplant recoarctation. BACKGROUND: Patients with aortic arch hypoplasia require extended arch reconstruction at transplant, with an inherent possibility of subsequent recoarctation of the aorta. METHODS: This was a retrospective review of all children (age <18 years) who underwent cardiac transplantation over a 10-year period. Collected data included pretransplant diagnosis, details of the transplant procedure and posttransplant data including development of recoarctation of the aorta, interventions for recoarctation and the most recent follow-up assessment of the aortic arch. RESULTS: Two hundred eighty-eight transplants were performed on 279 children (follow-up = 1,075 patient-years; range 0 to 133 months, median 43.7). Thirty-two of 152 patients (21%) who underwent extended aortic arch reconstruction subsequently developed recoarctation. All but one patient developed recoarctation within 2 years after transplant; 87% were hypertensive at presentation. Of 30 patients who underwent intervention for recoarctation (balloon angioplasty [n = 26] and surgical repair of recoarctation [n = 4]), 26 (87%) have remained recurrence-free (follow-up = 133 patient-years; range 8 to 106 months, median 47). CONCLUSIONS: The high frequency of recoarctation after cardiac transplantation with extended aortic arch reconstruction mandates serial echocardiographic evaluation of the aortic arch. Patients typically present with systemic hypertension within the first two years after transplantation. Balloon angioplasty is a safe, effective and durable method of treatment.     

Revised technique of cardiopulmonary bypass in one-stage repair of interrupted aortic arch complex

Twenty infants with interrupted aortic arch associated with various intracardiac anomalies underwent primary complete repair using pulsatile high-flow cardiopulmonary bypass with a short period of circulatory arrest. Age at repair ranged from 11 to 126 days (mean age, 43 days). Weight ranged from 2.2 to 5.5 kg (mean weight, 3.4 kg). Associated cardiac lesions included ventricular septal defect (14 patients), truncus arteriosus (3), transposition of the great arteries (2), and aortopulmonary window (1 patient). Left ventricular outflow tract obstruction was relieved in 2 patients. The aortic arch was reconstructed with a polytetrafluoroethylene graft in 7 patients and by direct anastomosis in the most recent 11 patients. An arterial cannulation method has been devised to facilitate direct anastomosis between the ascending aorta and the descending aorta, to lessen circulatory arrest time, and to prevent dangerous laceration and postoperative narrowing of the thin, small ascending aorta at the cannulation site. Two patients died, a surgical mortality rate of 10%. There has been one late death, which was due to severe truncal valve insufficiency. The other patients are doing well with a mean follow-up of 3 years 6 months. Restenosis of the direct anastomosis has not been noted in any patient. However, subaortic stenosis with pressure gradients of 30 to 40 mm Hg developed in 3 patients. In conclusion, one-stage repair including direct anastomosis for the aortic arch reconstruction and repair of all coexisting intracardiac defects is thought to be the treatment of choice.     

Repair of coarctation of the aorta during infancy minimizes the risk of late hypertension

BACKGROUND: Recent surgical reports on coarctation of the aorta have primarily focused on the relative merits of various operative techniques. However, appropriate timing for elective repair remains unclear. METHODS: In a retrospective analysis we examined the surgical outcomes in 176 consecutive patients undergoing repair of coarctation of the aorta in our institution over a 25-year period. Ninety-nine percent of the patients had follow-up for a median of 7.5 years. RESULTS: A total of 13 patients have died (7.4% overall mortality). Nine of these patients had associated complex intracardiac anomalies. There was no mortality in the 113 patients with isolated coarctation. Residual or recurrent coarctation occurred in 27 patients (15.3%). The age at operation and the type of surgical repair did not have an effect on the incidence of recurrence. Persistent or late hypertension was identified in 18 of the 107 patients who have been followed up for more than 5 years (16.8%). A total of 48 patients operated on during infancy have been followed up for more than 5 years. Only 2 have developed late hypertension (4.2%). Both of these patients had recurrence. In contrast, 16 of the 59 patients operated on after a year of age had late hypertension (27.1%). CONCLUSIONS: To minimize the risk of persistent hypertension, elective repair of coarctation should be performed within the first year of life.     

The management of severe subaortic stenosis, ventricular septal defect, and aortic arch obstruction in the neonate

Neonates with ventricular septal defect and aortic arch obstruction frequently have subaortic stenosis resulting from posterior deviation of the infundibular septum. Because the aortic anulus is often hypoplastic, making direct resection of the infundibular septum through the standard transaortic approach difficult, the optimal method of repair is uncertain. From September 1989 through November 1991, seven patients with ventricular septal defect, coarctation (n = 4), or interrupted aortic arch (n = 3) and severe subaortic stenosis underwent repair with use of a technique that included transatrial resection of the infundibular septum. Their ages ranged from 5 to 63 days (median 15 days) and weights from 1.3 to 5.4 kg (mean 3.1 kg). Only one patient was older than 1 month. The systolic and diastolic ratios of the diameter of the left ventricular outflow tract to that of the descending aorta were 0.53 +/- 0.09 mm (standard deviation) and 0.73 +/- 0.11, respectively. At operation, the posteriorly displaced infundibular septum was partially removed through a right atrial approach by resecting the superior margin of the ventricular septal defect up to the aortic anulus. The resulting enlarged ventricular septal defect was then closed with a patch to widen the subaortic area. In each patient the aortic arch was repaired by direct anastomosis. All patients survived operation; there was one late death from noncardiac causes 3 months after repair. The survivors remain well from 3 to 14 months after repair (mean 8 months). All are in sinus rhythm and none has a residual ventricular septal defect. One patient underwent successful balloon dilation of a residual aortic arch gradient late after repair. No patient has significant residual subaortic stenosis, although one has valvular aortic stenosis. This series suggests that in neonates with ventricular septal defect and severe subaortic stenosis resulting from posterior deviation of the infundibular septum, direct relief can be satisfactorily accomplished from a right atrial approach. This method provides effective widening of the left ventricular outflow tract and is superior to palliative techniques or conduit procedures.     

Repair of ventricular septal defect in the first two years of life using profound hypothermia-circulatory arrest techniques

Ventricular septal defect repair had been performed in 57 infants ages 21 days to 21 months and under 10 kg in weight using profound hypothermia-circulatory arrest technics. Severe congestive heart failure was the indication for operation in all but two infants under 6 months of age, and in those under 3 months there was usually an associated moderate or large sized atrial septal defect or patent ductus arteriosus or a coarctation. In infants over 6 months controlled heart failure was accompanied by failure to thrive and often recurrent respiratory infections. The main indication for surgery in three infants was repeated severe respiratory infections and in 7 infants, ages 10-15 months, an elevation of pulmonary vascular resistance of 6 units M2 or more. There were two hospital deaths among the 49 infants without coarctation (ages 6 and 20 months) and two among the 8 with coarctation. Postoperative respiratory and other complications were uncommon. On late review there was no significant residual VSD amongst the 11 recatheterized patients. Psychometric studies in 19 children who had reached the age of three to four years gave no evidence of cerebral damage due to the circulatory arrest period. In view of these results palliative pulmonary artery banding is no longer performed for VSD in infancy unless there is a Swiss cheese septum or an associated severe coarctation.     

Unilateral absence of pulmonary artery and ventricular septal defect in an infant

A 2-month-old boy was diagnosed as having a rare combination of absence of left pulmonary artery and ventricular septal defect. He underwent intracardiac repair at the age of 8 months. Reconstruction of the left pulmonary artery was impossible because it could not be visualized through a median sternotomy. Although his early postoperative course was uneventful, he needed ipsilateral pneumonectomy 3 months later because of unremitting hemoptysis. Hilar left pulmonary artery, which used to exist, was not found even by histologic examination.     

Coarctation of the aorta in children. Late results after surgery

From 1960 to 1970, 110 patients underwent operation for coarctation of the aorta. The overall mortality was 23%. However, in infants operated on at less than 6 months of age, the mortality was 63%, whereas there was only a 4% mortality in those operated on after 6 months of age. Late follow-up was available in 87 patients from five to 15 years postoperatively. There was a 14% frequency of recoarctation in the group of long-term survivors. Systemic hypertension, defined as an upper extremity blood pressure greater than 140/90 mm Hg, was found in 23% of the survivors. This study has demonstrated a significant frequency of postcoarctectomy hypertension and residual hemodynamic and angiographic abnormalities in patients without clinical evidence of recoarctation. This indicates the need for continued long-term follow-up of these patients.     

One-stage midline unifocalization and complete repair in infancy versus multiple-stage unifocalization followed by repair for complex heart disease with major aortopulmonary collaterals

BACKGROUND: Patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have traditionally required multiple unifocalization staging operations before undergoing complete repair. Recently, the feasibility of a single-stage unifocalization and repair was demonstrated by Hanley. In this report, we describe our experience with each approach. METHODS AND RESULTS: Since 1989, 11 of 12 patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone complete surgical correction. The first seven patients were subjected to staged bilateral unifocalizations, with repair being achieved in six (group I). The last five patients have undergone a single-stage midline unifocalization and repair via a sternotomy (group II). Four of these were infants (2 weeks to 9 months) and one was 13 years old. All patients in group I had tetralogy of Fallot, whereas in group II three patients had tetralogy of Fallot, one patient had double-outlet right ventricle, and one patient had complete atrioventricular canal and transposition. In group I, the median age at the first operation was 43 weeks. Complete repair was performed at a median age of 3.5 years, with a mean number of 3.3 operations required. In group II, only one operation was required to achieve complete repair at a median age of 28 weeks. The postoperative right ventricular/left ventricular pressure ratio was 0.49 in group I and 0.45 in group II. One intraoperative death and one late death occurred in group I and no early or late deaths in group II. Currently, four patients in group I and all five patients in group II are alive and well. CONCLUSIONS: Early intervention with both surgical approaches can lead to complete biventricular repair in most patients. Because the single-stage midline unifocalization and repair can achieve a completely repaired heart in infancy with one operation, it is currently our approach of choice.     

A case report of one-stage repair for transposition of the great arteries with intact ventricular septum and coarctation of the aorta in neonate

We report a neonatal case of one-stage repair for transposition of the great arteries with intact ventricular septum (TGA with IVS) and coarctation of the aorta (CoA). The surgery was performed at 27 days of age when the patient, a male, weighed 3408 g. Preoperative cardiac catheterization and angiography confirmed the diagnosis of TGA with IVS, CoA, atrial septal defect, patent ductus arteriosus, persistent left superior vena cava, left sided juxta-positioning of appendages and dextrocardia. The procedure was simultaneous subclavian flap aortoplasty through a left thoracotomy and an arterial switch operation through a median sternotomy. The patient's postoperative course was uneventful and he has grown well. Postoperative cardiac catheterization revealed mild pulmonary stenosis (pressure gradient of 19 mmHg between the right ventricle and the pulmonary artery) and mild aortic arch kinking from the arterial switch maneuver. One-stage repair, the combination of subclavian flap aortoplasty and arterial switch operation, provides a good clinical result in this complex malformation.     

Atrioventricular valve function after single patch repair of complete atrioventricular septal defect in infancy: how early should repair be attempted?

BACKGROUND: Though repair of complete atrioventricular septal defect in infancy has become routine at most centers, it is not unusual for very young infants to be managed medically because of concerns about the fragility of the atrioventricular valve tissue. METHODS: Since July 1992, seventy-two infants have undergone primary repair of complete atrioventricular septal defects at a median age of 3.9 months (40% < 3 months). A single-patch technique was used in all patients. The cleft was closed completely in 61 patients and partially (n = 10) or not at all (n = 1) in select patients at risk for valve stenosis. Left atrioventricular valve annuloplasty was performed in 18 patients. On the basis of transesophageal echocardiographic findings, 10 patients were returned to bypass for revision of the valve repair. RESULTS: There was one early death in a patient with single left papillary muscle, no early reoperations, and no new permanent arrhythmias. Only three patients had moderate left atrioventricular valve regurgitation at discharge. During a median follow-up of 24 months, there was one late death and five reoperations for left atrioventricular valve regurgitation (n = 2) and/or systemic outflow obstruction (n = 4). Follow-up left atrioventricular valve regurgitation was moderate in three patients, mild in 14, and none/trace in 54. Age had no relation to postoperative atrioventricular valve regurgitation, death, or reoperation. CONCLUSIONS: Despite concerns about fragility of valve tissue in very young patients, excellent results can be achieved with meticulous techniques. From neonates to older infants, age at repair does not influence outcome or valve function.     

Transxiphoid approach without median sternotomy for the repair of atrial septal defects

BACKGROUND: Interest in minimally invasive procedures has recently increased because it results in less surgical trauma, decreased patient discomfort, short hospital stay, reduced costs, and better cosmetic appearance. Based on these facts, we have been using the transxiphoid process approach without sternotomy for the correction of atrial septal defects. METHODS: From July 1996 to January 1997, the xiphoid process window approach was performed in 10 patients with ostium secundum atrial septal defect. Ages ranged from 6 months to 14 years (mean, 5.3 years). In all patients, extracorporeal circulation was carried out by means of cannulation of the femoral artery and both caval veins and of aortic cross-clamping. Videothoracoscopy was used to improve visualization of the aorta. RESULTS: There were no intraoperative or postoperative complications, and in all but 1 patient, extubation was possible while in the operating room. CONCLUSIONS: The xiphoid process window, with no median sternotomy, permitted closure of the atrial septal defects with good results and could be used as a less invasive technique for their correction.     

Blood grouping problems

From 1965 to 1974, 53 children with coarctation of the aorta (COA) and an associated ventricular septal defect (VSD) underwent cardiac catheterization. Thirty-one patients presented with congestive heart failure. Twenty-five of 27 patients (92%) who underwent cardiac catheterization under age 3 months had either systemic hypertension, a systolic gradient across the coarctation greater than 20 mm Hg or both. Pulmonary hypertension was present in all 25 patients. COA repair was performed in 39 patients and there were seven deaths. Of the 32 survivors, 23 have no residual gradient; six are normotensive but have a mild residual gradient; three are hypertensive or have a gradient greater than 20 mm Hg. Repair of the VSD or pulmonary artery banding has been performed in 11 of 44 patients who survived infancy. Spontaneous closure of the VSD has occurred in three cases and 25 patients have a small VSD that does not warrant surgical repair. Surgical repair of COA during infancy may be unavoidable but conservative medical management of the associated VSD is often successful.     

Arch growth after staged repair of interrupted aortic arch using carotid artery interposition

BACKGROUND: Between 1980 and 1990, our practice was to perform carotid artery interposition as part of a staged repair of interrupted aortic arch with various associated cardiac defects. METHODS: This procedure was used in 16 patients with IAA type B. The median age at operation was 4.5 days and the median weight, 3.2 kg. Ten of the patients had an associated ventricular septal defect. Six more had complex anatomy. There were two death at carotid interposition, two interim deaths, and two deaths after intracardiac repair. Preoperative echocardiographic and angiographic studies were compared with postoperative studies in 11 survivors of arch repair to assess sequential growth of the interposed carotid artery. Measurements of the carotid artery were normalized to the descending aorta. RESULTS: Preoperatively, the left carotid artery had a median diameter of 3.7 mm and was 42.9% of the descending aortic diameter. Postoperative studies performed at a median age of 5.7 months disclosed that the interposed carotid artery had grown to a median diameter of 7.0 mm and was 69.6% of the diameter of the descending aorta (normal > or = 81%). On follow-up at a median time of 4 years, 6 of 9 patients have no gradient by blood pressure measurements or echocardiographic Doppler studies, and 3 have modest gradients. No patient has required revision of the arch repair. CONCLUSIONS: Survival is good after carotid artery interposition for interrupted aortic arch and growth of the carotid artery approaches that of a normal arch. Carotid artery interposition is a viable alternative for repair of this lesion should primary definitive repair not be feasible.     

Vascular and neuroendocrine components in altered blood pressure regulation after surgical repair of coarctation of the aorta

STUDY OBJECTIVE: To investigate potential vascular and neuroendocrine determinants of altered blood pressure (BP) regulation in patients previously operated on for aortic coarctation. DESIGN, SETTING AND PATIENTS: We prospectively re-evaluated 45 patients operated on for aortic coarctation at Strasbourg University Hospital over a 13-year period. Four of these patients were less than 2 years old at the time of the operation and four were older than 20 years. Patient age and time since the operation were on average 21+/-13 years and 8+/-3 years, respectively. Surgery consisted of a resection with end-to-end anastomosis for 18 patients, angioplasty (8), prosthesis (4) or sub-clavian flap (15). RESULTS: Despite repair of the coarctation, about 40% of the patients showed an abnormal BP status at rest. The majority of these patients had uncomplicated borderline hypertension. The orthostasis test as well as the BP circadian rhythm were frequently abnormal. While the ankle/arm systolic pressure index measured at rest was generally within the normal range, diminished carotid-femoral pulse wave velocity was observed. Plasma adrenaline and aldosterone levels were elevated in about 50% of the patients examined. CONCLUSIONS: These new findings suggest that there are 'cause and effect' relationships between aortic structural and functional vascular abnormalities, and augmented plasma adrenaline and aldosterone in some patients after coarctation repair. These phenomena are likely to be involved in altered BP regulation and might result in recurrent hypertension.     

The superior approach for correction of the supracardiac type of total anomalous pulmonary venous return

An alternative approach for correction of supracardiac (type I) total anomalous pulmonary venous return is described. A median sternotomy is used. The posterior wall of the left atrium and the common pulmonary venous trunk are exposed through the transverse sinus. A direct anastomosis between these structures, ligation of the systemic-venous connection (vertical vein), and closure of the interatrial septal defect results in a one-stage repair. In our experience with the supracardiac anomaly in 20 patients, we have found that this approach consistently affords better exposure than other techniques currently in use for surgical correction of this anomaly.     

High incidence of aneurysm formation following patch plasty repair of coarctation

Repair of aortic coarctation was performed in 152 adolescent and adult patients (mean age 28.5 years, range 14-67 years). Ninety patients were treated with patch plasty, 33 with end-to-end anastomosis, 18 with interposition of a tubular graft, 6 with prosthetic bypass and 5 with direct plasty. There were two (1.3%) early and ten (6.6%) late deaths after 2.9 to 11.8 (mean 6.6) years. Of the remaining 140 patients, 129 (92.1%) were reexamined with computed tomography and angiography after 1.5 to 17.2 (mean 9.1) years postoperatively. In 27 patients (35.1%) of the patch plasty group significant dilatation at the operative site was observed and reoperation for aneurysm formation was required in 15 patients (19.5%). Resection of the intimal crest did not increase the probability of aneurysm formation, whereas Dacron as patch material and late hypertension had a significant influence. Six of the ten late deaths occurred in the patch plasty group. Rupture of an aneurysm at the operative site was proved in two of these patients, and three patients died suddenly for unknown reasons. In the other groups significant dilatation was observed in 13 patients and 3 local aneurysms required reoperation (2 after end-to-end anastomosis and 1 after direct plasty). We conclude that patch plasty repair of coarctation should be abandoned in adults. End-to-end anastomosis is advisable only if possible without excessive tension. Reoperation with interposition of a tubular graft on left heart bypass proved to be a safe method.