Hypoplastic Arch Research Articles

Surgical correction of aortic arch hypoplasia associated with crestlike protrusion of the superior arch wall and isthmic coarctation.

Aortic arch hypoplasia with varying degrees of severity is frequently seen in infants who have isthmic coarctation. In some cases of severe aortic arch hypoplasia, the superior aortic wall between the origins of the arch vessels becomes invaginated into the lumen like a crest. Extended end-to-end anastomosis can produce only limited relief of the arch hypoplasia. Although optimal surgical management of infants with aortic coarctation and concomitant hypoplastic arch remains controversial, we recognize, on the basis of our experience, that any persistent aortic arch stenosis after surgery can increase the prevalence of chronic systemic arterial hypertension in these patients. For this reason, we strongly believe that aggressive surgical enlargement of the hypoplastic arch segment at the time of infant coarctation repair to restore a homogeneous aortic arch like a “romanesque arch” is mandatory. We describe herein a modification of the technique previously reported by Amato, Rheinlander, and Cleveland,1 which provides an excellent option to the surgeon for the management of arch hypoplasia associated with crestlike protrusion of superior aortic arch wall. Patients. From January 1997 to December 1999, 80 patients underwent surgical repair of aortic coarctation and associated aortic arch hypoplasia. In 36 of these patients, the diameter of the distal aortic arch was less than 30% of that of the descending aorta, and preoperative echocardiographic SURGICAL CORRECTION OF AORTIC ARCH HYPOPLASIA ASSOCIATED WITH CRESTLIKE PROTRUSION OF THE SUPERIOR ARCH WALL AND ISTHMIC COARCTATION

Reverse subclavian flap repair of hypoplastic transverse aorta in infancy

Background. Management of hypoplastic aortic arch associated with coarctation in infancy can be challenging. Reverse subclavian flap aortoplasty plus coarctation resection offers simplicity without needing foreign material or cardiopulmonary bypass.Methods. Since 1988, 46 of 162 infants less than 3 months undergoing coarctation repair had hypoplastic arch enlargement with reverse subclavian flap aortoplasty. Median age was 11 days; mean weight was 3.2 kg. Thirty-seven patients (80%) had associated cardiac defects including single or multiple ventricular septal defects (14 infants), transposition of the great arteries (7), aortic or mitral stenosis (5), and complete atrioventricular septal defect (5 infants). Twenty-eight patients had pulmonary artery banding; 2 had an arterial switch operation through a separate median sternotomy.Results. There were two hospital deaths: one 4 months postoperatively in a patient requiring a Norwood procedure the next day for underestimated left ventricular hypoplasia; the other of sepsis more than 1 month postoperatively. On follow-up from 1 to 129 months (mean, 38 months), there were five recurrent obstructions: three at the coarctation site treated with balloon dilatation and two at the arch site. Twenty-six children had their heart defects corrected with 29 subsequent operations including an arterial switch operation for transposition of the great arteries/ventricular septal defect (3 infants), relief of aortic or mitral stenosis ± ventricular septal defect closure (5), multiple ventricular septal defect closure (3), a bidirectional Glenn (2), complete atrioventricular septal defect (2), and anomalous left coronary with ventricular septal defect repair (1 infant). Four children await debanding and ventricular septal defect closure or Glenn anastomosis. There have been two late deaths (overall survival, 91%).Conclusions. Reverse subclavian flap aortoplasty is excellent for relief of arch hypoplasia and coarctation in infants with low recurrence rates and acceptable operative and intermediate survival.

Surgery for coarctation of the aorta in infants weighing less than 2 kg

Background. Low- and very low-birth weight infants are now candidates for reparative cardiac surgery. Outcomes after coarctation repair have not been characterized in this patient population. Methods. We performed a retrospective review of 18 consecutive neonates less than 2 kg who underwent repair of aortic coarctation between August 1990 and December 1999. Results. Median weight was 1,330 g, and median gestational age was 31 weeks. A ventricular septal defect was present in 5 patients, and Shone’s complex in 4. Sixteen patients had resection and end-to-end anastomosis, and 2 had resection and subclavian flap. Median clamp time was 15.5 minutes. One patient died during hospitalization. Two patients died late postoperatively (5-year estimated survival 80%). Mean follow-up was 28.5 months. Eight patients (44%) had a residual or recurrent coarctation, 5 underwent balloon dilation, and 3 underwent reoperation. Freedom from reintervention for recoarctation was 60% at 5 years. Shone’s complex or a hypoplastic arch was an independent risk factor for decreased survival ( p < 0.001). Very low birth weight was a multivariate predictor for increased risk of recoarctation ( p = 0.01). Conclusions. Coarctation repair in less than 2-kg premature non-Shone’s infants can be performed with a low mortality. The rate of recoarctation is higher in the very low-birth weight infants, but can be managed with low risk.

Extra-anatomic aortic bypass via sternotomy for complex aortic arch stenosis in children.

Recurrent aortic narrowing after repair of aortic coarctation or interrupted aortic arch, as well as diffuse, long-segment aortic hypoplasia, can be difficult to manage. Extra-anatomic ascending aorta-descending aorta bypass grafting through a sternotomy is an alternative approach for this problem. Since 1985, 19 patients aged 2 months to 18 years (mean 10.7 years) underwent extra-anatomic bypass with 10- to 30-mm Dacron grafts. The initial diagnosis was coarctation with hypoplastic arch in 15, interrupted aortic arch in 3, and diffuse long-segment aortic hypoplasia in 1. Seventeen of the children had a total of 22 previous operations: transthoracic interposition or bypass graft (n = 7), end-to-end anastomosis (n = 7), subclavian arterioplasty (n = 6), and synthetic patch (n = 2). The mean time from initial repair was 8.0 years (range 0.6-18 years). Three children had previous sternotomies. Cardiopulmonary bypass was avoided in all but 6 patients (5 with simultaneous intracardiac repairs). No hospital or late deaths occurred. On follow-up from 4 months to 14.7 years (mean 7.9 years), no reoperations for recurrent stenosis were performed. Two patients have arm-to-leg pressure gradients: 20 mm Hg at rest in 1 patient and a 60-mm Hg systolic exercise gradient with no resting gradient in the other. One patient required exclusion of an aortic aneurysm at the old coarctation repair site 13 years after extra-anatomic bypass. Three children had subsequent successful cardiac operations. Extra-anatomic bypass is an effective and relatively easy approach for selected cases of complex or reoperative aortic arch obstruction. It should be considered as an alternative operative technique for complex aortic arch reconstruction.

Subclavian flap angioplasty: Does the arch look after itself?

Objectives: We sought to assess the early and long-term results of subclavian flap angioplasty in neonates and infants, with particular attention to growth of the hypoplastic arch. Methods: A retrospective analysis of 185 consecutive patients who underwent subclavian flap angioplasty between 1974 and 1998 was carried out. The patients included 125 neonates and 60 infants, with a median age of 18 days. Sixty-six (36%) patients had an additional ventricular septal defect, 41 (22%) patients had aortic arch hypoplasia diagnosed preoperatively, 141 (76%) had an associated patent ductus arteriosus, and 41 (22%) had additional complex heart disease. Follow-up was with transthoracic Doppler echocardiography in all patients. Results: The early mortality was 3%. Recoarctation, defined as a Doppler gradient of 25 mm Hg or more, occurred in 11 (6%) patients at a median follow-up of 6.2 years (6.2 ± 4.6 years). This included 4 of the 41 patients in whom arch hypoplasia was diagnosed preoperatively. There were no complications with the left arm. By multivariate analysis, risk factors for death were determined to be residual arch hypoplasia and low birth weight. The only risk factor for recoarctation was persistent arch hypoplasia after surgical treatment. However, angiographic imaging of the aorta showed that recoarctation was not due to a hypoplastic transverse arch, and it was probably at the site of ductal tissue. Survival at 5 and 10 years was 98% and 96%, respectively. Freedom from reoperation for recoarctation at 2 years was 95%, and at 5, 10, and 15 years, it was 92%. Conclusions: Subclavian flap repair remains an effective technique for repair of aortic coarctation with excellent results and low mortality. In the majority of patients, arch hypoplasia regresses after this procedure. (J Thorac Cardiovasc Surg 2000;120:224-9)

Down syndrome: imaging of multiorgan involvement.

Down syndrome (trisomy 21) has many manifestations that affect multiple organ systems, and we describe the wide array of imaging findings. Common cardiovascular and gastrointestinal entities are congenital heart disease (atrioventricular canal), bowel atresias (duodenal and anal), and Hirschsprungs disease. Children with Down syndrome have an 18-20 fold increased incidence of leukemia. Pulmonary hypoplasia, lung cysts, and pig bronchus (origin of the right upper lobe bronchus from the trachea) have been described. Neurologic findings include mineralizing vasculopathy of the basal ganglia, Moyamoya disease, and cerebellar/vermian hypoplasia. Musculoskeletal manifestations are numerous and include eleven ribs, hypersegmented sternum, abnormal pelvis, joint laxity/dislocations, and DDH (developmental dysplasia of the hip). Of special importance is the "triple jeopardy" of the upper cervical spine (atlanoaxial subluxation, hypoplastic posterior arch of C1, and atlantooccipital instability) and the resulting controversial cervical spine radiographic screening of children with Down syndrome. Knowledge of the many anomalies associated with Down syndrome can aid the clinician, not only in diagnosing abnormalities in these patients, but also in counseling families for potential problems that can occur in these children.

Early and Late Results After Repair of Aortopulmonary Septal Defect and Associated Anomalies in Infants < 6 Months of Age

The Richardson classification system for aortopulmonary septal defect (APSD) includes simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III). These are rare lesions that must be repaired in early infancy to avoid development of pulmonary vascular disease. Few reports have focused on patients with complex, associated lesions who underwent repair in early infancy. Between 1972 and 1995, 24 patients with Richardson type I (n = 11), II (n = 7), or III (n = 6) defects underwent repair at ages ranging from 2 to 172 days (median 34). Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). The most recent 7 patients were diagnosed by echocardiography without cardiac catheterization. There were no early or late deaths among the 12 patients with simple APSD. Four patients with complex, associated lesions died in the early postoperative period and another died 4 months after surgery. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. Altogether, 6 early survivors have required reintervention, and all survivors are in New York Heart Association class I at follow-up ranging from 2 to 25 years. Thus, long-term survival after repair of APSD in early infancy is excellent. Late sequelae are likely to be related either to associated lesions or to obstruction at the APSD repair site. Almost all cases of APSD in young infants can be diagnosed and evaluated by echocardiography without catheterization.

Hypoplastic aortic arch morphology pertinent to growth after surgical correction of aortic coarctation

Whether a hypoplastic transverse arch will grow after successful coarctectomy remains controversial. We studied 15 coarctation specimens with hypoplastic transverse arch. Eight patients were less than 1 month old and 7 were between 1 and 3 months. The diameter and length of the various segments of the aortic arch were measured. The number of elastin lamellae was determined histologically. Collagen density was quantified with a microdensitophotometer. Using immunohistochemistry, we determined alpha-actin-positive smooth muscle cells in the media of the ascending aorta and the hypoplastic transverse arch. Despite a hypoplastic transverse arch, the ascending and descending aorta grew. The absolute number of elastin lamellae in the hypoplastic transverse arch was low, but when expressed as a ratio versus its diameter, this number was high (p < 0.05). Collagen density showed high absolute values in the descending aorta. In the older group, 4 of 7 showed no staining for alpha-actin in the hypoplastic transverse arch, whereas under 1 month of age, only 2 of 8 cases were negative. The hypoplastic transverse arch is characterized by a relatively high number of elastin lamellae. Fewer alpha-actin-positive cells in the hypoplastic transverse arch occur in older specimens, which could indicate a diminished potential growth.

Lateral meningocele syndrome: Three new patients and review of the literature

One female and two male patients with multiple lateral meningoceles are presented. They do not have neurofibromatosis or Marfan syndrome and share findings with the two previously described patients with multiple lateral meningoceles. The original report by Lehman et al. [1977: J Pediatr 90:49-54] was titled "familial osteosclerosis," because osteosclerosis was present in the proposita and her mother; the patient described by Philip et al. [1995: Clin Dysmorphol 4:347-351] also had increased bone density of the skull base and the sutures. Thickened calvaria were present in one of our patients; two had a prominent metopic suture. Other shared findings include multiple lateral meningoceles, Wormian bones, malar hypoplasia, downslanted palpebral fissures, a high narrow palate, and cryptorchidism in males. In addition, our patients showed ligamentous laxity, keloid formation, hypotonia, and developmental delay. A short umbilical cord was noted in two patients. One had a hypoplastic posterior arch of the atlas and an enlarged sella, as reported by Lehman et al. [1977]. Our patients appear to have the same syndrome as previously reported. We suggest it be called "lateral meningocele syndrome," because of this unique finding.

Management of Arch Hypoplasia After Successful Coarctation Repair

Background. Pronounced arch obstruction can be seen after a well-repaired coarctation, and this probably results from the failure of a somewhat hypoplastic arch to grow or from clamp injury at the time of the initial repair, or from both causes. Because of mediastinal adhesions and minimal collateral circulation, use of extraanatomic bypass grafts appears to be the preferred approach.Methods. Six children or young adults presented with arch obstruction over a 3-year period. Their mean age was 13.5 ± 4 years, and the mean interval from the time of the initial repair was 10 ± 4 years. The mean age of the patients at the time of the initial repair was 3.2 ± 5 years. Symptoms included exertional headache and chest pain. The mean systolic gradients, as shown by echocardiography and cardiac catheterization, were 34 ± 7 mm Hg and 33 ± 6 mm Hg, respectively. Repair was accomplished through a midsternotomy using a polytetrafluoroethylene patch placed in the concavity of the arch, which extended from the ascending to the descending aorta. Dissection was kept close to the aorta and arch to minimize injury to the phrenic and recurrent laryngeal nerves. Cardiopulmonary bypass and moderate hypothermia (25° to 27°C bladder temperature) without circulatory arrest were used.Results. All patients were discharged home 4 to 20 days postoperatively (mean, 7 ± 6 days). All patients were found to be normotensive at a mean follow-up of 1.3 ± 1 years. Postoperative echocardiograms, which were obtained in all patients, revealed no residual gradients. Exercise blood pressure was evaluated in 2 patients and found to be normal.Conclusions. Transsternal arch enlargement using cardiopulmonary bypass and moderate hypothermia without circulatory arrest is an attractive and safe approach for the treatment of arch obstruction after coarctation repair. Unlike the use of extraanatomic bypass grafts, it allows complete relief of the obstruction, unhampered aortic growth, the minimal use of foreign material, and a repair that is protected deep within the mediastinal space.(Ann Thorac Surg 1997;63:975–80)

Transverse arch hypoplasia predisposes to aneurysm formation at the repair site after patch angioplasty for coarctation of the aorta

Objectives. This study used magnetic resonance imaging (MRI) to evaluate the morphology and pathophysiology of aneurysm formation after patch angioplasty for coarctation of the aorta.Background. Late aneurysm formation at the repair site is a well known and frequent complication after patch angioplasty. However, because the underlying mechanisms remain unresolved, postoperative outcome is unpredictable and adequate follow-up difficult.Methods. Seventy-three of 85 patients with patch angioplasty for coarctation of the aorta were screened for aneurysm formation. Magnetic resonance imaging was performed in all 33 patients with an aneurysm, and results were compared with those for 13 control patients and 10 normal subjects. Mean (±SD) time between operation and MRI was 12.0 ± 2.0 years. Aneurysm was defined as the ratio of the diameter of the aorta at the repair site to the diaphragmatic aorta ≥1.5. Hypoplasia of the transverse arch and recoarctation at the repair site were defined as a ratio <0.9. Transverse arch ratios on MRI were compared with those on preoperative cineangiography and the pressure gradient between the patient's right and left arm.Results. All 33 patients with an aneurysm had a hypoplastic transverse arch. The 13 patients with a normal ratio at the repair site had a normal transverse arch ratio (chi square, p < 0.0001). Logarithmic regression showed a significant negative correlation (r = 0.62) between the repair site and transverse arch ratios. A significant pressure difference between the patient's right and left arm was found in patients with versus those without aneurysm (p = 0.0009). No significant difference was found between transverse arch ratios on preoperative cineangiography and postoperative MRI (mean 0.014 ± 0.1, p = 0.4).Conclusions. Aneurysm formation at the repair site is highly related to hypoplasia of the transverse arch. Sufficient catch-up growth of a hypoplastic transverse arch is rare after late patch angioplasty. Dynamic phenomena, such as flow acceleration and turbulence, originating in a narrow transverse arch, may contribute to aneurysm formation at the repair site after patch angioplasty.

Reconstruction of the Hypoplastic Posterior Arch of the Atlas with Calvarial Bone Grafts for Posterior Atlantoaxial Fusion

Reconstruction of the Hypoplastic Posterior Arch of the Atlas with Calvarial Bone Grafts for Posterior Atlantoaxial Fusion

Aortic coarctation with hypoplastic arch in neonates: A spectrum of anatomic lesions requiring different surgical options

Hypoplasia of the transverse aortic arch is frequently associated with isthmic coarctation in many patients referred for operation in early infancy, and the surgical technique should be adjusted to suit each type of anatomic lesion. Referring to the anatomic description of hypoplastic aortic arch reported by Moulaert and associates, between January 1988 and July 1991 we operated on 32 consecutive infants (≤3 months old) using a surgical approach based on the echocardiographic and angiographic findings; 20 patients (62%) were younger than 2 weeks of age and 20 patients (62%) had associated intracardiac lesions. According to the location, extension, and size of the hypoplasia of the aortic arch, we had three groups of patients: in group 1 (21 patients) we performed resection and extended end-to-end anastomosis, as previously described in 1985; in group 2 (5 patients) we performed resection, posterior end-to-end anastomosis, and anterior subclavian flap enlargement; and in group 3 (6 patients) we performed direct side-to-end anastomosis between ascending and descending aorta through a median sternotomy. One patient died during the postoperative course in group 3. With a mean follow-up time of 26 months we had 4 cases (13%) of “residual” or “recurrent” coarctation in group 1, successfully repaired at 2 months of age by an anterior approach in 2 patients and by percutaneous angioplasty in the others. In conclusion, hypoplastic aortic arch in neonates represents a common difficulty, and optimal reconstruction of the entire aortic arch is mandatory to reduce operative mortality and incidence of recoarctation, especially when there are complex associated intracardiac lesions or left ventricular dysfunction.

Rare case of double aortic arch with hypoplastic right dorsal segment and associated tetralogy of Fallot: MR findings

We report a rare case of double aortic arch with a hypoplastic right dorsal arch segment, which was also associated with tetralogy of Fallot. Accurate characterization of the hypoplastic right arch segment by MRI preoperatively determined the optimal surgical approach to releasing the symptomatic vascular ring. MRI was also useful in assessing and monitoring associated tracheomalacia.

Repair of hypoplastic or interrupted aortic arch via sternotomy

Herein we describe our experience with repair of interrupted aortic arch and coarctation plus hypoplastic aortic arch in 55 consecutive infants (1984 to 1990). Median age at operation was 6 days and median weight 3.1 kg. Associated severe intracardiac anomalies were the rule. All patients had significant congestive cardiac failure, and the majority required prostaglandin E1 resuscitation and inotropic support (with or without ventilation) before the operation. All operations were performed via sternotomy with core cooling and circulatory arrest. Isolated myocardial perfusion was used in 13 patients during arch repair. A complete intracardiac (biventricular) repair was performed except in patients expected to require a Fontan operation as definitive treatment. The operative mortality overall was 14.5% (confidence limits 10% to 22%). For arch repair plus biventricular intracardiac repair, the operative mortality was 9% (confidence limits 5% to 15%), and for arch repair plus palliative intracardiac repair, 40% (confidence limits 22% to 60%). The mortality in the isolated myocardial perfusion group was 0% (confidence limits 0% to 14%), which may be related to reduced myocardial ischemic time (p less than 0.05). Actuarial survival was 75% (confidence limits 65% to 83%) at 12 months, with no subsequent deaths over 1294 patient-months (mean 28 months) of follow-up. Actuarial freedom from recurrent arch obstruction was 69% (confidence limits 48% to 85%) at 46 months' follow-up. Primary repair of interrupted aortic arch and coarctation plus hypoplastic arch compares favorably with a staged approach and is recommended even when complex intracardiac anatomy is present.

Growth of the hypoplastic aortic arch after simple coarctation resection and end-to-end anastomosis

Surgical treatment of a hypoplastic aortic arch associated with an aortic coarctation is controversial. The controversy concerns the claimed need to surgically enlarge the diameter of the hypoplastic arch, in addition to resection and end-to-end anastomosis. The purpose of this prospective study is to determine the fate of the hypoplastic aortic arch after resection of the aortic coarctation and end-to-end anastomosis. Between July 1, 1988, and January 1, 1990, 15 consecutive infants less than 3 months of age with an aortic coarctation were evaluated echocardiographically. A Z-value was calculated, being the number of standard deviations the aortic arch differs from the expected value, derived from a control group. Eight of these 15 infants had a hypoplastic aortic arch with a mean Z-value of -7.14 +/- 1.39. The other seven infants had a "normal" aortic arch with a mean Z-value of -1.85 +/- 1.08. All 15 infants underwent simple coarctation resection and end-to-end anastomosis. Six months after operation the mean Z-value increased significantly in those with a hypoplastic arch to -1.08 +/- 0.69 (p less than 0.0001) and in those with a "normal" aortic arch to 0.106 +/- 0.99 (p = 0.004). No infant died in our series (0%; CL 0% to 12%) and a recoarctation developed once (12.5%; CL 2% to 36%). Therefore we believe that simple resection and end-to-end anastomosis is the operation of choice for aortic coarctation associated with a hypoplastic aortic arch despite the presence of a ventricular septal defect and that enlargement of the hypoplastic aortic arch is not necessary.

Hypoplastic posterior arch of C-1 in children with Down syndrome: a double jeopardy.

Radiographs of 38 children with Down syndrome (aged 2-3 years) were retrospectively evaluated for hypoplasia of the posterior arch of the C-1 vertebra. The anteroposterior dimensions of the posterior arch of C-1 were measured and compared with those of an age- and sex-matched control group. The posterior arches of C-2 to C-5 were similarly evaluated. Radiographs demonstrated hypoplasia of the posterior arch of C-1 in 26%. This anomaly of C-1 was an isolated event; sagittal diameters of C-2 to C-5 were within normal limits compared with those of the control group and published standards. Because of the known, potentially devastating atlantoaxial dislocation in Down syndrome, the increased prevalence of a second C-1 anomaly causing preexistent narrowing of the vertebral canal is of clinical concern. A hypoplastic posterior arch of C-1 may amplify the risk of spinal cord damage following atlantoaxial subluxation, as the subsequently smaller vertebral canal has less room for dorsal movement of the odontoid process. This potential "double jeopardy" of C-1 vertebral abnormalities should be recognized and assessed on cervical spine screening radiographs in children with Down syndrome.

Operations for subaortic stenosis in univentricular hearts

Optimal prevention and treatment of subaortic stenosis (SAS) in the univentricular heart with subaortic outlet chamber and high pulmonary blood flow remains controversial, especially when complicated by aortic arch obstruction. Herein we analyze our surgical results. Group 1 consisted of 11 infants (mean age, 10 days) with univentricular heart and SAS. Ten required repair of interrupted aortic arch (n = 7) or coarctation with hypoplastic arch (n = 7). Four patients had relief of SAS by either Damus-Kaye-Stansel connection (n = 2) or aortopulmonary window (n = 2), with three operative deaths and one late death. Six had one-stage arterial switch and atrial septectomy with arch repair ( 5 / 6 ) with one operative death and one late death. Two survivors have progressed to bidirectional cavopulmonary shunt, a third has had a Fontan operation, and a fourth awaits Fontan. In group 2, 11 children required operation for acquired SAS after pulmonary artery banding. Nine have progressed to Fontan operation with either staged (n = 3) or concurrent (n = 6) relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Fontan mortality was 11% (70% confidence limits, 2% to 32%). Group 3 consisted of 3 patients without pulmonary artery banding who had SAS diagnosed at Fontan evaluation. All 3 survived Fontan operation and relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Group 4 consisted of 1 patient with previous pulmonary artery banding (no SAS) who underwent Fontan operation but required Damus-Kaye-Stansel connection 30 months later for SAS. We conclude that arterial switch, arch repair, and atrial septectomy effectively palliate newborns with univentricular heart and SAS, although ultimate Fontan suitability is yet to be established. Both Damus-Kaye-Stansel connection and subaortic resection provide good long-term relief of SAS in select patients, and previous or concurrent treatment of SAS does not preclude successful Fontan operation in otherwise suitable patients.

Hypoplastic transverse arch and coarctation in neonates: Surgical reconstruction of the aortic arch: A study of sixty-six patients

From Jan. 1, 1983, to Jan. 1, 1988, 66 consecutive neonates with coarctation and severe hypoplasia of the transverse arch underwent coarctation repair by resection of the coarctation and reconstruction of the aortic arch. Mean age at operation was 14 +/- 8 days, ranging from 2 to 30 days; 63% of the newborn infants were less than 2 weeks of age. The coarctation was isolated in 23%, associated with a ventricular septal defect in 39%, and associated with complex anomalies in 38%, including 16 cases of transposition of the great arteries or doublet-outlet right ventricle plus ventricular septal defect, two cases of simple transposition, two of corrected transposition plus ventricular septal defect, and five cases of "hypoplastic" left ventricle. The surgical technique comprises a wide resection of the coarctation extended to the contiguous ductal tissue followed by the reconstruction of the aortic arch in bringing the descending aorta into the concavity of the aortic arch. This technique is able to relieve the obstruction of the aortic arch provided that (1) the descending aorta is widely dissected to allow mobilization and (2) the incision of the transverse arch is extended proximal to the ostium of the left carotid artery. The operation was performed through a left thoracotomy in 62 patients and through a sternotomy in four additional neonates with transposition and ventricular septal defect who underwent a one-stage repair with aortic reconstruction, closure of the defect, and arterial switch. The overall early mortality rate (less than 30 days) was 14% (9/66; 95% confidence limits = 5% to 22%), including four deaths occurring within the first month, at a concomitant or subsequent repair of the associated anomaly. There were six late deaths, all related to the associated lesions. The overall mortality rate was 23% (15/66; 95% confidence limits = 13% to 33%). The mean follow-up was 21 +/- 10 months, ranging from 6 to 66 months. Actuarial survival rates at 5 years are 72% +/- 10% for the overall group; 87% +/- 17% for simple coarctation; 88% +/- 12% for coarctation and ventricular septal defect; and 52% +/- 18% for complex coarctation. The rate of recurrent coarctation was 12.5% (95% confidence limits = 2% to 23%), leading to five reoperations with no deaths. Freedom from reoperation was 89.5% +/- 9% at 5 years. This technique of coarctation repair offers several advantages: low operative mortality, complete relief of the left ventricular obstruction, wide resection of the ductus tissue, absence of prosthetic material, and preservation of the left subclavian artery.

Growth of the Aorta after Prosthetic Patch Aortoplasty for Coarctation in Infants

We have shown that patch aortoplasty relieves the aortic pressure gradient substantially better than resection with end-to-end anastomosis in infants with coarctation of the aorta. Growth of the intact posterior wall of the unresected coarctation after aortoplasty, however, has not yet been demonstrated to occur. We studied 21 infants less than 2 years of age who underwent prosthetic patch repair of coarctation after 1975. Associated lesions were present in 16 patients, and 12 had a concomitant procedure. Two patients died in the hospital, and 4 died later, all of causes unrelated to the coarctation repair. Systolic blood pressure in the right arm declined from 140 ± 41 mm Hg (mean ± standard deviation) preoperatively to 101 ± 19 mm Hg postoperatively in the whole group and to 95 ± 12 in 10 patients followed for more than 3 years. The right arm–left leg systolic pressure gradient declined from 66 ± 22 mm Hg to 5 ± 14 in all patients and to 4 ± 13 in the group followed more than 3 years. Hypoplastic transverse arch was seen in all but 2 patients. Its relative diameter increased in 6 of 9 patients who underwent catheterization postoperatively and increased more than 50% in 3 patients. Two patients have evidence of narrowing of the aorta at the patch, but both have normal blood pressure in the right arm. In the patient with longest follow-up, an 8-month-old infant seen 8 years after operation, angiograms show the diameter of the aorta at the coarctation to be greater than that at the transverse arch and at the descending aorta. Hemodynamic and angiographic results up to 8 years after patch repair remain good, and there is evidence that both the hypoplastic and intact posterior wall of the aorta can grow after patch repair of coarctation in infancy.