Hypoplastic Transverse Arch Research Articles

Mid-Term Outcomes of a Modification of Extended Aortic Arch Anastomosis with Pulmonary Artery Banding in Single Ventricle Neonates with Hypoplastic Transverse Arch.

There is less certainty regarding the best strategy for treating neonates with functional single ventricle (SV) and hypoplastic aortic arch. We have applied a modified extended aortic arch anastomosis (EAAA) and main pulmonary artery banding (PAB) as an initial palliation in neonates with transverse arch hypoplasia and assessed the mid-term outcomes. In total, 10 neonates with functional SV and extensive hypoplasia or interruption of the arch underwent a modified EAAA (extended arch anastomosis with a subclavian flap) concomitant with main PAB through a thoracotomy without cardiopulmonary bypass. Patient age and weight ranged from 4 to 14 days and 2.3 to 3.8 kg, respectively. There were no hospital deaths although there were two late deaths. Gradients across the arch were 0 to 7 mmHg at postoperative day 1 and no arch reoperations were required. Two patients required balloon aortoplasty. Nine underwent bidirectional cavopulmonary shunt and two of them needed concomitant Damus-Kaye-Stansel (DKS) anastomosis. Six have completed Fontan. Our modification of EAAA with main PAB for SV neonates may benefit a certain population with transverse arch hypoplasia as an option to be considered. Patients with the potential for developing outflow obstruction may be best managed with an initial DKS-type palliation.

Endovascular stenting in transverse aortic arch hypoplasia

Describe outcomes from stenting transverse aortic arch hypoplasia. Hypoplasia of the transverse arch may result in residual systemic hypertension and may be amenable to stenting. Outcomes for transverse aortic arch hypoplasia stenting were collated from four centers between 2000 and 2010. Primary endpoints were reduction in peak systolic catheter gradient, dimensions of the stented segment, and systolic right arm blood pressure. Changes in antihypertensive medication and early and late complications were recorded. Data were collated for 21 patients (16 male, 5 female), median age of 16.5 years (range, 0.25-25.9 years) and median weight of 55 kg (range, 4.5-103 kg). 19/21 patients were hypertensive at baseline, excluding the two neonates after repair of interrupted aortic arch. Median transverse arch diameter increased from 7 to 14 mm after stenting (P < 0.001). Median ratio of the transverse arch to descending aorta at the diaphragm level improved from 0.43 to 0.9 (P < 0.001). Mean gradient across the hypoplastic transverse arch was 38 mm Hg (range, 14-76) at baseline and 5 mm Hg (range, 0-13) after stenting (P < 0.001). There were no deaths and 6 early complications occurred in 5 patients. Follow-up (median 24 months) data were available for 19 patients. 17/19 hypertensive patients had follow-up data. Two neonates developed intimal hyperplasia within the stent with a stent fracture in one. Median systolic blood pressure was 153 mm Hg (range, 117-180) prestent and 130 mm Hg (range, 105-150) poststent (P = 0.0002). In 13/17 patients, the antihypertensive medication could be reduced. Stenting of transverse aortic arch hypoplasia, although technically challenging, produced good angiographic and haemodynamic results with an early improvement in blood pressure control. These results appear to be sustained in the medium term.

Stenting for infantile adult aortic coarctation with successful conception of zygomatic twins at 4 years’ post-intervention

INTRODUCTIONAortic coarctation is a congenital defect which rarely presents in adulthood but results in significant morbidity and mortality. Endovascular techniques present novel therapeutic options for managing this anomaly with comparable results to traditional open surgical repair. PRESENTATION OF CASEWe convey a case of postductal aortic coarctation in a symptomatic 31-year-old woman, undetected, despite 27 years history of congenital bicuspid valve with aortic incompetence. We staged a successful endovascular repair following presentation with hypertension, abdominal pain, and worsening lower limb claudication. This lady recovered well, eventually giving birth to twins after a successful caesarean section delivery. The anatomical, clinical and technical features of the case are appraised. DISCUSSIONAortic coarctation is classified anatomically as preductal (infantile) or postductal (adult) and the morphological spectrum of abnormality ranges from a discrete stenosis distal to the left subclavian to a hypoplastic transverse arch and aortic isthmus presenting in infancy. Its incidence is 0.2–0.6 per 100 live births. Traditional open surgical repair of aortic coarctation is associated with high morbidity and mortality. Advances in endovascular techniques with their associated shorter hospital time and lower costs, reduced short term morbidity and mortality mean that they are likely to become mainstays of treatment. CONCLUSIONThe presentation of aortic coarctation for the first time in an adult is extremely unusual. Error and delayed diagnosis can never be fully exempted in the practice of medicine even with the most advanced diagnostic tools. Follow-up is mandatory by trans-oesophageal echo in all endovascular patients.

Left cervical aortic arch associated with multiple vascular anomalies

Cervical aortic arch is a rare anomaly occasionally associated with other cardiovascular abnormalities. We present a case of tortuous left cervical aortic arch associated with hypoplastic transverse arch, coarctation of the aorta, and right brachiocephalic arteries arising below the coarctation and stenotic origin of the left subclavian artery. These multiple anatomic anomalies, which are associated in our case, have not been described in a single patient previously.

A simple technique to circumvent the aorto-pulmonary size mismatch in arterial switch operation with arch repair: a report of five cases

The discrepancy in the sizes of the aorta and the pulmonary artery is a major technical concern in arterial switch operations for transposition of great arteries with ventricular septal defect, arch hypoplasia/interruption and Taussig Bing anomaly. The size mismatch increases the difficulty in neoaortic reconstruction and securing hemostasis. It alters the Sino tubular anatomy and contributes to development of Neoaortic Regurgitation (NAR). We present five cases of successful repairs for Transposition of Great Arteries (TGA) with hypoplastic transverse arch and Coarctation where the extended homologous gluteraldehyde treated pericardial patch was used to augment the isthmus, transverse arch and the ascending aorta upto the line of transaction to avoid the aorto-pulmonary size mismatch.

Hypoplastic Circumflex Retroesophageal Right-Sided Cervical Aortic Arch With Unusual Vascular Arrangement and Severe Coarctation

We report the case of a 12-year-old boy with a hypoplastic retroesophageal circumflex right-sided cervical aortic arch and coarctation. After the incidental finding of a heart murmur when the boy was 9 years old, cardiac magnetic resonance showed a right-sided cervical aortic arch, hypoplastic transverse arch, and separate origin of the left common carotid, right common carotid, right vertebral, and right subclavian arteries. The left subclavian artery arose from the proximal descending aorta next to the coarctation. An extra-anatomical ascending to descending aorta tube graft was inserted through a right lateral thoracotomy with good results.

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)

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.

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.

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.