Previous Coarctation Repair Research Articles

Giant Aneurysms Associated with Aortic Coarctation: Management Challenges and Options

The management of giant aneurysms associated with aortic coarctation is challenging. The location, the size of the aneurysm, whether it has ruptured, previous coarctation repair, the presence of stenosis, and the ascending and distal aortic pathology will all influence the treatment approach. With more patients surviving to adulthood after early coarctation repair, a better understanding of the anatomy, pathophysiology, and radiological findings of coarctation-related aneurysms is imperative to providing sound care. We present here our experience with a series of giant aneurysms of the aorta, all with a background of underlying aortic coarctation. We highlight the decision-making process and review current management trends.

Coronary ostial involvement in acute aortic dissection: detection with 64-slice cardiac CT

A 41-year-old man collapsed after lifting weights at a gym. Following admission to the emergency department, a 64-slice cardiac computed tomography (CT) revealed a Stanford Type A aortic dissection arising from a previous coarctation repair. Multiphasic reconstructions demonstrated an unstable, highly mobile aortic dissection flap that extended proximally to involve the right coronary artery ostium. Our case is an example of the application of electrocardiogram-gated cardiac CT in directly visualizing involvement of the coronary ostia in acute aortic dissection, which may influence surgical management.

Role of endovascular repair in the management of late pseudo-aneurysms following open surgery for aortic coarctation

Coarctation of the aorta accounts for almost 5% of all congenital cardiac malformations, and it is usually treated by open surgical procedures. Despite the excellent primary results, many patients may develop anastomotic pseudo-aneurysms, associated with considerable morbidity and mortality rates. We investigated the role of endovascular repair as an alternative to open re-do surgery on the descending aorta. We retrospectively collected data of 11 consecutive patients who presented with pseudo-aneurysms of the descending aorta following previous surgical repair of congenital aortic coarctation. Nine patients developed a pseudo-aneurysm after patch aortoplasty, while an interposition graft repair was performed in two cases. Seven patients underwent an endovascular repair (26.9 years after the previous open surgery). Four patients with a pseudo-aneurysm after coarctation repair associated with arch hypoplasia underwent conventional open re-operation. No complications or death were observed in the conventional surgical group. Technical success was obtained in all patients who received a stent-graft procedure. More than one stent graft was necessary only in one case. A left carotid-left subclavian artery bypass was performed in three patients. Type II endoleak was detected by angiography in one patient who had previously undergone subclavian re-vascularisation. The median follow-up (FU) was 44.5 months and was 100% complete. Neither re-operations nor complications or deaths occurred during FU. Endovascular repair of pseudo-aneurysms following open surgery for aortic coarctation is feasible and safe. However, more patients and longer follow-up are necessary to assess the efficacy of this promising less-invasive alternative to open re-do surgery.

Left Internal Mammary Artery Atherosclerosis: Twenty-Three Years After Repair of Aortic Coarctation

Atherosclerosis of the internal mammary artery is a rare disease. We describe a case in which a 41-year-old man underwent coronary artery bypass surgery 23 years after repair of aortic coarctation. The diseased mammary artery was used to graft the left anterior descending coronary artery after intraoperative confirmation of good patency. Early graft occlusion occurred a few days after the operation. This case demonstrates that atherosclerosis affects mammary arteries after long-term repair of aortic coarctation. Such conduits should not be used, even in the presence of good blood flow.

Initial results of primary device closure of large muscular ventricular septal defects in early infancy using perventricular access

To report our experience with the use of the Amplatzer muscular ventricular septal defect (VSD) occluder, using direct right ventricle free wall puncture for primary closure of muscular VSDs in infants. Young infants with heart failure due to large or multiple muscular VSDs often require intervention at a stage when percutaneous device closure is impractical due to delivery system limitations. There are considerable benefits to avoiding bypass in these infants. Patients with suitable muscular VSDs, considered too small for transvenous closure, underwent periventricular muscular VSD closure under transesophageal echo (TEE) guidance. Eight infants underwent the procedure. The median age was 14 weeks (2-41) with median weight of 4 kg (3-6.6). Four patients had additional VSDs and one patient had previous repair of coarctation. One patient had dextrocardia and severe left ventricular impairment due to an associated cardiomyopathy. The median VSD size was 8 mm (range, 4-10 mm). A single device (6-12 mm) was deployed in each patient, the size chosen being 1-2 mm larger than the TEE measurement. Two patients had subsequent pulmonary artery banding, with absorbable bands, because of moderate residual shunting through additional VSDs. The latest echo study at a median of 7.2 weeks (0.5-66) post device implantation revealed no patients with a hemodynamically significant shunt. Perventricular muscular VSD closure can be safely and effectively undertaken in small infants with suitable muscular defects, and this strategy can avoid the need for bypass.

Anomalous unilateral pulmonary vein in a patient with aortic coarctation

A 34-year-old woman with previous aortic coarctation repair at the age of 5 years was referred for review. She was in good health. On clinical examination, there was no blood pressure difference between the arms, but pedal pulses were weak. A contrast-enhanced 64-slice thoracic CT scan was performed to assess the coarctation repair.

Infrarenal Aortic Infection and Rupture of a Pseudoaneurysm Secondary to Migration of Proximal Aortic Patch: Case Report and Literature Review

Aneurysm formation at the site of prior aortic coarctation patch repair is well documented. Migration of the patch though to the infrarenal aorta, as in this case, with subsequent aortitis and pseudoaneurysm formation has not been described. A 16 y old boy who was operated at the age of 10 for his aortic coarctation, presented with sepsis and abdominal pain. CT scan confirmed the presence of aortitis and an aortic pseudoaneurysm. This was treated with resection of the infrarenal aorta and replacement with superficial femoral vein. The PTFE patch from the previous coarctation repair was found and extracted from the infrarenal aorta.

Aortoesophageal fistula secondary to stent-graft repair of the thoracic aorta after previous surgical coarctation repair

Clinical Summary A 31-year-old woman was admitted to a peripheral hospital with an episode of massive hematemesis. She had undergone a patchtechnique coarctation repair at age 3 years and required balloon dilatation for recoarctation at age 14 years. Annual cardiologic follow-up detected, at age 26 years, a pseudoaneurysm at the coarctation site with dimensions of 2.4 1.9 cm. The pseudoaneurysm size remained stable, but 8 months before the hematemesis, TEVAR was recommended to avert any risk of rupture and a Jomed 48 20-mm covered stent (Abbott Vascular Ltd, Kent, United Kingdom) was deployed at the coarctation site pseudoaneurysm. Post-TEVAR imaging suggested satisfactory exclusion. After the hematemesis, she underwent esophagogastroduodenoscopy, which showed a 1.5-cm bluish polypoid lesion 26 cm from the incisura. Biopsy revealed blood clot only. She was discharged but readmitted 4 weeks later with a further massive hematemesis. A repeat esophagogastroduodenoscopy revealed that the lesion had enlarged 3-fold. This finding, together with the history of coarctation surgery, prompted a computed tomographic (CT) scan (Figure 1), which demonstrated a perigraft hematoma suggestive of AEF. After transfer to our center, aortography confirmed peri–stent-graft extravasation, further supporting a diagnosis of AEF. Another covered stent (38 12 mm, Jostent; Abbott Vascular Ltd) was deployed and the hematemesis abated. However, a postprocedure angiogram demonstrated persistent filling of the pseudoaneurysm. The incomplete pseudoaneurysm exclusion and the perceived risk of further complications prompted surgical referral. Echocardiography demonstrated moderate bicuspid aortic valve stenosis. Surgery was performed via a transsternal thoracotomy in the left fourth intercostal space and comprised mechanical aortic valve replacement, retrocardiac ascending–descending aortic conduit insertion, and an interposition graft between the conduit and the left subclavian artery. The aortic arch was staple-ligated between the left common carotid artery and the left subclavian artery. The descending aorta was stapled below the stent site. The aortic pseudoaneurysm was opened and the previously placed stents were removed. Inspection revealed a section of necrotic and partially absent aortic wall with erosion of the longitudinal esophageal muscle but no mucosal breach. It was assumed that stent deployment had perforated or disintegrated the aortic wall leading to an AEF. The aortic remnant was left open, a feeding enterostomy and decompression gastrostomy performed, and the chest closed with drainage. Recovery was uneventful, with a contrast swallow examination showing no abnormalities. At 12 months, the patient remains free of symptoms, and CT scan (Figure 2) demonstrates resolution of perigraft hematoma and satisfactory anatomic reconfiguration.

Deep vein thrombosis in a patient with congenital heart disease and permanent transfemoral pacing

A 45-year-old woman with previous repair of coarctation of aorta, ventricular septal defect closure, and progressive decline in her exercise capacity was admitted for the treatment of left leg deep venous thrombosis (DVT). She had a history of complete heart block and insertion of a pectoral pacemaker. After numerous problems with pocket infections, multiple box changes and fracture of the atrial lead, the pectoral system was extracted, and a transfemoral permanent pacemaker was implanted. Even though transfemoral pacing is considered a safe alternative to epicardial lead placement, this may not be the case in functionally impaired patients with mobility problems. Physicians caring for such patients should be alert to symptoms of DVT and provide prompt treatment to avoid major complications.

Endovascular management of pseudo-aneurysms after previous surgical repair of congenital aortic coarctation

Whatever the surgical technique used, false aneurysm formation is one of the long-term complications of repair of aortic coarctation. Conservative management is associated with a 100% rate of rupture. The conventional surgical approach is complex and associated with high morbidity and mortality rates. We report our experience of endovascular management of pseudo-aneurysms after previous surgical repair of congenital aortic coarctation. Between October 2005 and 2006, stent-grafting of pseudo-aneurysms after previous surgical repair of congenital aortic coarctation was performed in four patients. Median age was 31.5 years (range: 24-38). Two patients had undergone two previous interventions. The last previous surgery consisted of graft interposition (N=2), subclavian flap aortoplasty (N=1) and aorto-aortic bypass (N=1). Median size of the pseudo-aneurysm was 31.5mm (range: 20-58). Mean time between the last surgery and endovascular treatment was 24 years (range: 3-32). One patient was treated emergently because of hemoptysis in relation with an aorto-bronchial fistula, the three other patients were treated electively. A transfemoral approach was used in all patients. The Zenith TX2 (Cook) thoracic stent-graft was used in all the patients, one patient underwent previous dilatation at the coarctation level. When present, the ostium of the left subclavian artery was always covered (N=3). No major complication occurred during the procedure and no patient died during the follow-up. One patient presented a type II endoleak which spontaneously healed during the first month. Another patient with his left subclavian artery covered presented claudication of the left arm requiring a carotid-subclavian bypass. After a median follow-up of 7.5 months (range: 1-12.9), the patients were asymptomatic and CT scans demonstrated complete exclusion of all treated postcoarctation aneurysms without recoarctation and without any stent-graft-related complication. The endovascular management of pseudo-aneurysms after previous surgical repair of congenital aortic coarctation is feasible. This approach was safe and effective. Long-term clinic and imaging follow-up is mandatory.

Endovascular Approaches for Complex Forms of Recurrent Aortic Coarctation

To review a single-center experience with endovascular treatment of recurrent aortic coarctation in adults. Since 1998, 11 patients (9 men; mean age 48+/-15 years, range 16-63) with recurrent aortic coarctation following previous coarctation repair were referred to our institution for treatment. Clinical presentations included pseudoaneurysm (n=2), restenosis (n=3), pseudoaneurysm accompanied by restenosis (n=4), and rupture of a post-coarctation pseudoaneurysm (n=2). All patients were treated using an endovascular approach as part of a single-center investigational device exemption protocol. Endovascular interventions included Palmaz stent implantation (n=3), implantation of an endoluminal graft (n=2), or a combination of both treatments in 6 patients. Three patients underwent balloon angioplasty before stenting or endografting, and 2 patients had a carotid-subclavian bypass done before the endovascular repair. All repairs were technically and clinically successful. The median length of stay after repair was 2.0+/-2.3 days. In follow-up, 2 patients underwent a carotid-subclavian bypass for left upper extremity claudication. Two patients required reintervention owing to migration of the stent in 1 and an endoleak 2 years after the initial procedure in the other. Endovascular approaches to adult coarctation appear to be safe and effective. With the emergence of endoluminal grafts and the widespread availability of the Palmaz stent, endovascular repair offers an excellent alternative to open surgery for complex cases of recurrent coarctation. Additional studies are indicated to assess the long-term outcomes of these patients.

P.196 Occult persistence of hepatitis C virus

Patients with primary aortic coarctation or complications of a previous coarctation repair may seek treatment as adults. Management options include open, endovascular, hybrid, and extra-anatomic repairs. We evaluated the results of open direct repair with total cardiopulmonary bypass and hypothermic circulatory arrest.Twenty-seven patients with primary coarctation with or without associated aortic abnormalities or with complications of a previous coarctation repair were treated during a 20-year interval. Mean age was 37 years (range, 15-71 years). Thirteen patients had primary coarctation (7 with associated aneurysms), 3 patients had aneurysms of the aorta above or below a noncritical narrowing of the aorta at the isthmus (“pseudocoarctation”), and 11 patients had previous repair of a coarctation. The previous repairs were bypass grafting of the coarctation (n = 5), interposition grafting (n = 3), patch angioplasty (n = 2), and primary repair (n = 1).Mean durations of cardiopulmonary bypass, hypothermic circulatory arrest and hypothermic fibrillation were 104, 36, and 76 minutes, respectively. There were no in-hospital deaths, and no patients sustained stroke, spinal cord ischemic injury, or renal or respiratory failure. Thirteen patients received no blood products. No patient has had evidence of recurrent coarctation or aneurysm formation.Cardiopulmonary bypass with hypothermic circulatory arrest is a safe and suitable technique for treatment of primary and recurrent coarctation. It is associated with low operative risk and absence of major complications.

Endovascular repair of aneurysm after previous surgical coarctation repair

Late aneurysm formation has been reported after every type of surgical coarctation repair, with rupture of such aneurysms being responsible for approximately 7% of all deaths. Secondary surgical repair carries a significant mortality and morbidity. According to the positive experience with endovascular therapy of atherosclerotic thoracic aortic aneurysms, it is worthwhile to evaluate the concept of minimally invasive endovascular stent grafting for secondary repair of postsurgical aneurysms. Data were collected prospectively on consecutive patients who presented with postcoarctation false aneurysms. Since 1999, in a cohort of 46 endovascularly treated patients with thoracic aortic pathologies, 3 patients with postcoarctation false aneurysms underwent endoluminal stent-graft placement. All of these procedures were technically successful without 30-day or 1-year procedure-related mortality. After a mean follow-up of 19 months (range, 7.8-33.5 months), all aneurysm remain excluded without endoleak. According to the current limited experience of small series and short periods of follow-up, the endoluminal repair seems to be a promising alternative to redo operations for postsurgical thoracic aneurysms associated with coarctation repair. Long-term follow-up is required to assess the durability of the stent-graft treatment.

Aortic Coarctation Repair with Patch Graft Aortoplasty and Severe Hemoptysis

This report describes the successful treatment of a confirmed case of hemoptysis from a false aneurysm at the site of a previous coarctation repair. Professionals involved in the clinical care of patients that have undergone previous coarctation repair with patch graft aortoplasty, should be aware of the late risk of false aneurysm occurrence. Periodically screening patients with magnetic resonance imaging to prevent potentially fatal complications is recommended.

Doppler Echocardiographic Profile and Indexes in the Evaluation of Aortic Coarctation in Patients Before and After Stenting

We sought to assess the effect of successful stenting on the Doppler profile of aortic coarctation and to identify echocardiographic indexes that could be used for follow-up of such patients. Doppler echocardiography demonstrates characteristic flow patterns in significant aortic coarctation. We undertook retrospective echocardiographic analyses before and at six to nine months after coarctation stenting in consecutive patients from 2002 to 2003. Peak systolic pressure gradient (SPG), diastolic velocity (DV), end-diastolic tail velocity (EDTV), systolic velocity half-time index (SVHTi) and diastolic velocity half-time index (DVHTi), and systolic pressure half-time index (SPHTi) and diastolic pressure half-time index (DPHTi) were measured. The severity of aortic coarctation was compared with cardiovascular magnetic resonance (CMR) imaging using the coarctation index (CoAi). The patient cohort was divided into two groups: group 1 (13 patients; age 30 +/- 8 years), which consisted of patients with significant aortic coarctation treated with stenting, and group 2 (11 patients; age 39 +/- 16 years), which consisted of patients with previous surgical repair of aortic coarctation without evidence of re-coarctation. After stenting, there was significant reduction in SPG (p = 0.001), DV (p = 0.001), EDTV (p = 0.005), DVHTi (p = 0.001), and DPHTi (p = 0.001) values. In the patient group as a whole, there was a significant correlation between SPG and DV (r = 0.86; p < 0.001), EDTV (r = 0.80; p < 0.001), DVHTi (r = 0.56; p < 0.001), and DPHTi (r = 0.50; p = 0.002). In addition, DV >193 cm/s (100% sensitivity, 100% specificity) and diastolic/systolic velocity ratio >0.53 (100% sensitivity, 96% specificity) had high predictive values for severe aortic coarctation (CoAi <0.25). After stenting, peak SPG, DV, and pressure half-time indexes (i.e., DVHTi and DPHTi) decreased significantly. These findings can confidently be used in the follow-up of coarctation patients after stenting, particularly in those with limited two-dimensional images.

Aortic coarctation repair—lost and found: The role of local long term specialised care

Background Aortic coarctation is associated with significant abnormalities of the underlying vasculature. Surgical repair, although relieving obstruction, is not a cure; patients continue to have a high risk of complications with a significantly reduced life expectancy. It has become increasingly apparent that they require regular specialised follow up lifelong. However provision of such services is limited particularly for patients living some distance from the largest cities where specialised adult congenital heart disease services tend to based. We carried out a notes-based study to look at the adequacy of long term follow up in such patients and to assess the role of a local specialist cardiology service in managing these patients. Methods We carried out a notes-based study of 55 patients with aortic coarctation referred to a new specialist grown up congenital heart disease clinic based in a large district general hospital over 100 miles from the nearest surgical centre specialising in adult congenital heart disease. Results A significant proportion of the patients in this study had already suffered major complications by the time of referral. Despite this, nearly half had, at some stage, been lost to follow up and a third had been referred from the community with new complications. 52% of the women had produced children often with little cardiological support. Few patients had had any specialised imaging. At initial review in the clinic, 41% had significant hypertension, although only a small proportion were on antihypertensives. Following initial review in the specialist GUCH clinic, new medications were initiated in 55%, mostly for hypertension; aortic imaging was performed in 94%; and 22% were referred for further specialist investigation or invasive treatment. Conclusions This study demonstrates that many patients with previous repair of aortic coarctation have not received optimal long term care. Many had been lost to regular cardiology follow up and, even amongst those who had been seen in cardiology clinics, there was a high frequency of poorly treated or unsuspected complications. The provision of local expert care from a cardiologist specialising in congenital heart disease allows earlier and more aggressive treatment of complications and may also improve compliance. Where a localised specialist is not available, general cardiologists need to work to protocol-driven care pathways with easy access to specialist support.

Circulatory arrest for repair of postcoarctation site aneurysm

Background Aneurysm at previous coarctation repair may be seen more frequently as children operated for this condition survive into adulthood. We use deep hypothermic circulatory arrest to repair these aneurysms. Methods A case series was conducted using 12-year, single-institution, retrospective chart review. Results Twenty-one patients underwent left thoracotomy and repair of aneurysm at the site of previous coarctation repair. Three cases presented emergently as aortobronchial fistulas. The age range was 16 to 73 years (median, 26 years). The median circulatory arrest time was 33 minutes (range, 22 to 55 minutes). Repair involved interposition graft replacement. Six patients required additional tube graft replacement of the left subclavian artery. There was 1 operative mortality in a patient having a hypoxic brain injury secondary to an anaphylactic reaction to a plasma expander. There were no embolic strokes or paraplegia. One patient had a recurrent laryngeal nerve paresis. There was 1 case of Horner's syndrome after subclavian artery replacement. Conclusions Circulatory arrest allows for the accurate repair of this difficult pathologic process and avoids the risk of clamp-related injuries. Follow-up out to 16 years demonstrates this technique of repair to be durable, with no late deaths or reoperations for recurrent aneurysm.

Reoperation and coarctation of the aorta: the need for lifelong surveillance

Background. We report a series of reoperations in 23 patients who had undergone previous aortic coarctation repair. Methods. The medical records of these patients were reviewed, and the patients were followed up by telephone interview. Mean age at reoperation was 25 years. There was a mean of 18 years between initial coarctation repair and reoperation. Indications for reoperation included recoarctation (9 patients), aortic aneurysm (8), aortobronchial fistulas with exsanguinating hemorrhage (2), subaortic stenosis (1), ruptured thoracic aneurysm (1), ruptured sinus of Valsalva aneurysm (1), and supramitral stenosing ring (1). Results. There were no specific intraoperative complications. Three patients required reexploration for bleeding. An acutely ischemic lower limb developed in 1 patient secondary to a common femoral artery embolus, which necessitated embolectomy. Conclusions. Reoperation for postcoarctation repair patients can be performed with good results. Sudden life-threatening hemorrhage due to aortobronchial fistulas in patients having undergone Dacron patch aortoplasty, as well as long-term obstructive phenomena seen anywhere along the left ventricular outflow tract, make lifelong surveillance of these patients mandatory.

An unusual case of aortic dissection in Turner's syndrome

Cardiovascular malformations are common in patients with Turner's syndrome. Aortic coarctation and bicuspid aortic valve are the most frequently occurring abnormalities, and are associated with cystic medial necrosis of the aortic wall. Aortic dissection is an uncommon but catastrophic complication of the 'aortopathy' of Turner's syndrome. We report the unusual case of a Turner's syndrome patient (with a bicuspid aortic valve and previous coarctation repair) who died following an intramural haemorrhage of the aortic root that was complicated by dissection and rupture, with no evidence of aortic intimal tear. The role of intramural haemorrhage in the pathogenesis of acute aortic syndromes in Turner's syndrome patients is unclear. The condition may be associated with atypical clinical presentations, it can be difficult to confirm with imaging techniques, and it carries a high risk of progression to classical aortic dissection and death. This case therefore highlights the need for a high index of suspicion when assessing Turner's syndrome patients presenting with chest pain syndromes. Furthermore, the effective management of Turner's syndrome patients with cardiovascular abnormalities requires the development of evidence-based preventive (such as echocardiographic surveillance of aortic dilatation) and interventional strategies.

Long-term results of the lateral tunnel Fontan operation

Objectives: Completion of a total cavopulmonary anastomosis with an intra-atrial lateral tunnel is known to yield good early and midterm results. In this study, we sought to determine the long-term outcome (10 years) after a lateral tunnel Fontan procedure. Methods: Between October 1987 and December 1991, 220 patients (aged 11 months to 32 years) with a wide range of underlying diagnoses underwent a fenestrated or nonfenestrated lateral tunnel Fontan procedure at our institution. Current follow-up information was available for 196 patients (94%, mean follow-up = 10.2 ± 0.6 years). Risk factor analysis included patient-related and procedure-related variables, with death, failure, and bradyarrhythmia or tachyarrhythmia as outcome parameters. Results: There were 12 early deaths (<30 days or hospital death), 7 late deaths, 4 successful takedown operations, and 4 heart transplantations. Kaplan-Meier estimated survival was 93% at 5 years and 91% at 10 years, and freedom from failure was 90% at 5 years and 87% at 10 years. Freedom from new supraventricular tachyarrhythmia was 96% at 5 years and 91% at 10 years; freedom from new bradyarrhythmia was 88% at 5 years and 79% at 10 years. Three patients had evidence of protein-losing enteropathy. Multivariable risk factors for development of supraventricular tachyarrhythmia included heterotaxy syndrome, atrioventricular valve abnormalities, and preoperative bradyarrhythmia. Risk factors for bradyarrhythmia included systemic venous anomalies. The sole risk factor for late failure was a previous coarctation repair. Conclusion: The lateral tunnel Fontan procedure results in excellent long-term outcome even when used in patients with diverse anatomic diagnoses. The incidence of atrial tachyarrhythmia is low and mainly depends on the underlying cardiac morphology and preoperative arrhythmia. The good long-term outcome after an intracardiac lateral tunnel Fontan procedure should serve as a basis for comparison with other surgical alternatives. (J Thorac Cardiovasc Surg 2001;121:28-41)