Management errors in adults with congenital heart disease: prevalence, sources, and consequences.

Aims: Improved survival has resulted in increasing numbers and complexity of adults with congenital heart disease (ACHD). International guidelines recommend specialized care but many patients are still not managed at dedicated ACHD centres. This study analysed referral sources and appropriateness of management for patients referred to our tertiary ACHD Centre over the past 3 years. Methods and results: We compared differences in care between patients referred from paediatric/ACHD-trained vs. general adult cardiologists, according to Adherence (A) or Non-Adherence (NA) with published guidelines. Non-Adherent cases were graded according to the severity of adverse outcome or risk of adverse outcome. Of 309 consecutively referred patients (28 ± 14 years, 51% male), 134 (43%) were from general cardiologists (19% highly complex CHD) and 115 (37%) were from paediatric cardiology or ACHD specialists (33% highly complex CHD). Sixty referrals (20%) were from other medical teams and of those, 31 had been lost to follow-up. Guideline deviations were more common in referrals from general compared to CHD-trained cardiologists (P < 0.001). Of general cardiology referrals, 49 (37%) were NA; 18 had catastrophic or major complications (n = 2, 16 respectively). In contrast, only 12 (10%) of the paediatric/ACHD referrals were NA, but none of these were catastrophic and only 3 were major. Simple, moderate, and highly complex CHD patients were at increased risk of adverse outcome when not under specialized CHD cardiology care (P = 0.04, 0.009, and 0.002, respectively). Conclusion: Non-adherence with guidelines was common in the ACHD population, and this frequently resulted in important adverse clinical consequences. These problems were more likely in patients who had not been receiving specialized CHD care. Configuring healthcare systems to optimize 'whole of life' care for this growing population is essential.

Surgical and Hybrid Ablation of Atrial Fibrillation.

Atrial fibrillation (AF) is the most common arrhythmia in humans and is known to be associated with an increased risk of stroke, dementia, heart failure and mortality. Non-pharmacological therapy with ablation using either surgical or percutaneous techniques is recommended in drug refractory AF. Early attempts to devise procedures to ablate AF and restore sinus rhythm culminated with the Cox-Maze procedure, the first truly successful procedure. Since then, ablation surgery has been conducted predominately as a concomitant procedure. The Cox Maze procedure is complex and technically demanding and has, therefore, been extensively modified with new techniques for creating the linear ablation lines, new lesion sets, minimally invasive surgical techniques and most recently hybrid surgical-catheter ablation techniques. Surgical ablation techniques result in a marked reduction in atrial fibrillation when compared to conventional therapy with only a small increase in procedural risk. However, further research is required to more accurately quantify those benefits and to determine the optimal lesion sets, specific to the underlying arrhythmia mechanism and the optimal energy sources for ablation.

Fixed left ventricular outflow tract obstruction mimicking hypertrophic obstructive cardiomyopathy: pitfalls in diagnosis.

We present a case series that highlights the diagnostic challenges with left ventricular hypertrophy (LVH) and left ventricular outflow tract obstruction (LVOTO). Fixed structural lesions causing LVOTO with secondary LVH may mimic hypertrophic obstructive cardiomyopathy (HOCM). Management of these two entities is critically different. Misdiagnosis and failure to recognize fixed left ventricular outflow tract (LVOT) lesions may result in morbidity as a result of inappropriate therapy and delay of definitive surgical treatment. It is thus necessary to identify the correct type and level of obstruction in the LVOT by careful correlation of clinical examination, Doppler evaluation, and advanced imaging findings.

Ebstein's anomaly in those surviving to adult life - a single centre experience.

BACKGROUND: Ebstein's anomaly (EA) occurs in about one to five per 200 000 live births. Long-term follow-up data of adults with EA is scarce due to the relatively low frequency of the disease and the variation of its anatomic and haemodynamic severity. METHODS: Since 1995, in our adult congenital heart disease (ACHD) centre, we have practised a uniform approach to management of adults with EA, with surgery reserved for those with refractory arrhythmia (failed medical and/or catheter-based treatment) or worsening symptoms of breathlessness. A retrospective review of medical records of all such patients with EA and normal cardiac connections was performed. RESULTS: Fifty-one EA patients (17 males) were identified. Mean age at diagnosis was 21+/-21 years and mean follow-up time at our centre was 21±14 years. During this time, 18 patients (35%) had documented supraventricular arrhythmia. Sixteen patients (30%) underwent ablation therapy with long-term relief from arrhythmia in nine (56%). Nine patients (18%) underwent tricuspid valve (TV) surgery (four repair and five replacement), with seven patients having undergone a tricuspid valve surgery prior to referral to our unit. Three patients died, one of cardiogenic shock after redo surgery (58 years), one of progressive heart failure (45 years) and one with malignancy. Overall survival was 100% to age 40 years, 95% to age 50 years and 81% to age 60 years. CONCLUSIONS: Ebstein's Anomaly in adulthood often has severe morphological abnormalities but is compatible with good medium-term survival, with a generally symptom driven approach to the indications for interventions.

Right ventricular outflow tract enlargement prior to pulmonary valve replacement is associated with poorer structural and functional outcomes, in adults with repaired Tetralogy of Fallot.

BACKGROUND: Pulmonary valve replacement (PVR) is commonly performed late after Tetralogy of Fallot (TOF) repair. We examined the effects of PVR on cardiac structure, function and exercise capacity in adults with repaired TOF. METHODS: Eighteen adult patients with repaired TOF and severe pulmonary regurgitation (PR) with right ventricular (RV) dilatation requiring PVR for clinical reasons (age; 25±8 years) were recruited to undergo cardiac MRI (1.5T) and cardiopulmonary exercise testing before and 14±3 months after PVR. RESULTS: Reduced indexed RV end-diastolic volume (RVEDVi; 186±32mL/m(2) pre-op vs 114±20mL/m(2) post-op, p<0.001) was observed after PVR. "Normalisation" of RVEDVi (≤108mL/m(2)) was achieved in only seven of 18 patients. Pre-PVR RVEDVi correlated with post-operative change in RVEDVi (change=-72.1±20.4mL/m(2), r=-0.815, p<0.001). Exercise capacity remained high-normal post-PVR (% predicted maximal workload: 93±16% vs 91±12%, p=0.5). Regional RV volumes were assessed; RV outflow tract (RVOT) volumes were compared to the RV muscular corpus. Large pre-PVR RVOT volumes correlated negatively with post-surgical RV ejection fraction, peak VO2 and delta VO2 at anaerobic threshold (p<0.05 for all). CONCLUSIONS: Normalisation of RV volume is unlikely to be achieved above a pre-PVR RVEDVi of 165mL/m(2) or more. In particular, an enlarged RVOT prior to PVR predicts suboptimal structural and functional outcomes.

In patients supported with peripheral veno-arterial extracorporeal membrane oxygenation, what factors are associated with the development of spinal cord ischaemia?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'in patients supported with peripheral veno-arterial extracorporeal membrane oxygenation, what factors are associated with the development of spinal cord ischaemia'? Altogether, more than 22 papers were found using the reported search, of which 10 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Of the 28 patients reported by included studies, the thoracic spinal cord was most commonly affected. Twenty patients (71%) survived to hospital discharge and 7 (25%) were reported to have neurological recovery. Potential confounders included coronary angiography, cardiac arrest requiring chest compressions and concomitant intra-aortic balloon pump. Consequently, all papers highlighted the likely multifactorial aetiology of spinal cord infarction in these patients. We propose that close neurological observation, particularly in patients who have received chest compressions, and management of potential aetiological factors is crucial to aid in timely diagnosis and potential prevention of this rare complication. Limiting sedation and neuromuscular blockade to enable neurologic assessment of the lower limbs may allow more timely diagnosis.

Six-minute walk test distance at time of hospital discharge is strongly and independently associated with all-cause mortality following cardiac surgery.

We investigated the impact of distance covered in the six-minute walk test (6mWT) before being discharged from the hospital after cardiac surgery on the risk of all-cause mortality. Our study included 1127 patients who underwent cardiac surgery and then took part in a standardised physiotherapist-supervised inpatient rehabilitation programme during 2007-2017. The percentage of the predicted 6mWT distance, and the lower limit of normal distance was calculated based on individual patients' age, sex, and body mass index. We used Cox regression with adjustment for confounders to determine multivariable-adjusted hazard ratios (HRs) for mortality. Over a median follow-up period of 6.4 (IQR: 3.5-9.2) years, 15% (n = 169) patients died. We observed a strong and independent inverse association between 6mWT distance and mortality, with every 10 m increase in distance associated to a 4% reduction in mortality (HR: 0.96, 95% CI 0.94-0.98, P < 0.001). Those in the top tertile for predicted 6mWT performance had a 49% reduced risk of mortality (HR: 0.51, 95% CI 0.33-0.79) compared to those in the bottom tertile. Patients who met or exceeded the minimum normal 6mWT distance had 36% lower mortality risk (HR: 0.64, 95% CI 0.45-0.92) compared to those who did not meet this benchmark. Subgroup analysis showed that combined CABG and valve surgery patients walked less in the 6mWT compared to those undergoing isolated CABG or valve surgeries, with a significant association between 6mWT and mortality observed in the isolated procedure groups only. In conclusion, the longer the distance covered in the 6mWT before leaving the hospital, the lower the risk of mortality.

Minimally invasive surgical resection of a large primary pulmonary hydatid cyst: a thoracoscopic approach.

Pulmonary hydatid disease is a rare parasitic disease in Australia with few reported cases. The mainstay of treatment in pulmonary hydatid disease is surgical resection followed by medical treatment with benzimidazoles to reduce the risk of recurrence. We present a case of successful resection of a large primary pulmonary hydatid cyst via minimally invasive video-assisted thoracoscopic surgery approach in a 65-year-old gentleman with incidental hepatopulmonary hydatid disease.

Prevalence and determinants of tricuspid regurgitation after repair of tetralogy of Fallot.

BACKGROUND: The prevalence and determinants of tricuspid regurgitation (TR) in patients with repaired Tetralogy of Fallot (rTOF) remain incompletely understood. OBJECTIVES: To explore the prevalence of and factors associated with TR in patients with rTOF, specifically, the relationship of right ventricular (RV) dilatation with TR severity. METHODS: Patients (≥17 yrs) with rTOF referred to our service (2000-2019) were identified. Those with severe pulmonary stenosis, significant shunt, or previous tricuspid valve surgery were excluded. Using standard cardiac MRI protocols, RV, right atrial (RA) and tricuspid valve (TV) parameters were measured and compared. RESULTS: 68 consecutively eligible patients with rTOF were included in the study (27 ± 9 yrs., 35% female). Despite substantial RV volume overload (mean RVEDVi 153 mL/m2), the majority of the cohort (78%) had no or only mild TR. RA volumes, tenting height/area and annular diameter were normal (4.9 ± 2.0 mm, 1.1 ± 1.0 cm2 and 32.4 ± 6.2 mm, respectively). There was no significant correlation of TR fraction with RVEDVi (r = 0.13; p = 0.30), RVEF (r = 0.09; p = 0.44) or tricuspid annular diameter (r = 0.07; p = 0.62). Only RAVi showed a weak but significant correlation with TR fraction (0.29; p = 0.03). In a pooled cohort analysis, including both rTOF patients and adults with a dilated RV from pre-tricuspid shunt lesions, only rTOF was independently associated with higher TR fraction (p = 0.017). CONCLUSION: Despite substantial RV dilatation in a cohort with rTOF, there was surprisingly little TR. We found poor correlation between RVEDVi, RA volumes, tricuspid annular dilatation and the presence of significant TR. These findings question commonly held notions regarding the pathophysiology of functional TR in these patients.

Perspectives in interventional electrophysiology in children and those with congenital heart disease: electrophysiology in children.

Recent developments in paediatric pacing and ablation of arrhythmia substrate have been characterised by adoption and modification of techniques used in adults. Infants, small children and those of all ages with congenital heart disease are a patient group with a higher risk profile needing a special approach. Current success rates for catheter ablation are high and major complication rates are low. Important issues with respect to long-term outcome include questions about coronary injury, long-term effects of radiation exposure and late recurrence. Non-fluoroscopic electro-anatomical mapping systems (3D systems), cryo-ablation and remote navigation are techniques recently improved such that it is possible to potentially reduce fluoroscopy and complications. Pacing in young children and congenital heart disease often warrants an epicardial approach to avoid embolism, venous occlusion and lead failure related to growth. Defibrillator and resynchronisation therapy are increasingly important tools to reduce mortality, although the indications are not as clear as in adult patients without congenital heart disease.

A Simplified Approach to Predicting Reintervention in the Arterial Switch Operation.

We investigated patients with transposition anatomy suitable for the arterial switch operation (ASO) to evaluate a simplified approach to prediction of reintervention. A retrospective review was performed of 180 consecutive patients who underwent ASO from 2009 to 2018. Patients were classified as Category I (n = 122) d-transposition of great arteries (dTGA) + intact ventricular septum, Category II (n = 28) dTGA + ventricular septal defect (VSD) and Category III (n = 30) dTGA + Aortic arch obstruction (AAO) +/- VSD or Taussig-Bing Anomaly (TBA) +/- AAO. Outcomes included reintervention-free survival (using Kaplan-Meier estimates) and predictors of reintervention. Median follow up was 3.3 (interquartile range 1.7-5.8) years with no difference between categories(P = 0.082). There were 3 mortalities- 2 early (one each in Category I and II) and one late (in Category I). Reintervention-free survival for the whole group at 1, 3, 5 and 8 years was 94%, 91%, 90% and 86% respectively. Conventional criteria predicting reintervention included the presence of TBA(P = 0.0054) and AAO(P = 0.027). Low birth weight did not predict reintervention(P = 0.2). When analyzed by category, multivariable analysis showed that patients in Category III carried a high risk of reintervention [Hazard risk (HR) = 7.43, 95% confidence interval (CI)=(2.39, 23.11), P < 0.001], but so did those in Category II [HR=6.90, 95% CI = (2.19, 21.75, P < 0.001] when compared to Category I. Conventional risk factors for technical difficulty may not be the best predictors of reintervention. A simplified approach highlights Category II patients (dTGA + VSD) as being at substantial risk of re-intervention, and not part of a low risk cohort.

The Ross/Ross-Konno procedure in infancy is a safe and durable solution for aortic stenosis.

OBJECTIVE: The objective of this study was to characterize early and midterm outcomes after the Ross/Ross-Konno procedure performed in infancy for severe aortic valve disease. METHODS: Between January 1995 and December 2018, 35 infants younger than 1 year (13 neonates) underwent a Ross/Ross-Konno procedure. Patients were followed up to a median of 4.1 years (interquartile range [IQR], 2.6-9.5). Primary outcome measures were survival, early morbidity, freedom from reintervention and long-term functional and echocardiographic status. RESULTS: Median age at operation was 49 days (IQR, 17-135) and weight was 4 kg (IQR, 3.4-5.2). Thirty-one (89%) had undergone a previous procedure, including balloon valvuloplasty in 26 (74%). Thirty (86%) required annular enlargement (Konno incision). Five required concomitant aortic arch surgery (2 neonates, 3 infants). There were no early deaths, and 1 late death at 18 months. Freedom from reoperation was 85% (95% confidence interval [CI], 68%-93%) at 1 year, 76% (95% CI, 54%-88%) at 5 years, and 62% (95% CI, 36%-79%) at 10 years. One modified Konno was performed at 5 years after a Ross in infancy. Ten right ventricle to pulmonary artery conduits have required reintervention (2 percutaneous pulmonary valve implantations). One child required a permanent pacemaker for complete heart block. At latest follow-up, 32 (94%) of 34 survivors were asymptomatic. There was no significant change in neoaortic Z-scores between 6 weeks and latest follow-up. CONCLUSIONS: The neonatal and infant Ross/Ross-Konno procedure can be performed with low mortality and achieves a stable left ventricular outflow tract. Significant early morbidity reflects the preoperative condition of the patients but definitive surgery of this type can be considered as a primary approach.

Five-year analysis of operative mortality and neonatal outcomes in congenital heart disease.

BACKGROUND: We sought to compare overall mortality with neonatal outcomes over a five year period to define risk factors for mortality and service development priorities. METHODS: A retrospective cohort study of surgical outcomes following repair or palliation of structural congenital heart defects January 2005-2010. We defined mortality according to contemporary international guidelines and classified surgical procedures using the Risk Adjustment in Congenital Heart Surgery (RACHS-1) score. The effect of age and weight at operation on mortality and annual variation in case-complexity and surgeon case-mix were assessed. Subgroup analysis was performed in patients who were ≤30 days at operation (neonates). RESULTS: Overall mortality within 30 days of operation or prior to hospital discharge was 1.3 and 1.9%, respectively. Mortality was higher in neonates (6.8%) and low birth weight infants (≤2.5kg) (12.1%). Mortality was similar in bypass versus non-bypass procedures (odds ratio 0.74, p=0.425). Annual mortality rates were consistent despite a marked increase in case-complexity. Neonates overall required longer periods of intensive care support and were more likely to suffer serious complications compared to older children. Age, weight and RACHS-1 score were independent risk factors for mortality on multivariate analysis. In neonates undergoing bypass procedures, only RACHS-1 score was a significant risk factor. CONCLUSIONS: This study provides an accurate and contemporary audit of mortality risk associated with congenital heart surgery. Outcomes compare favourably to international benchmarks but highlight the risks of morbidity and mortality associated with neonatal cardiac surgery.

Modified pulmonary artery banding: A novel strategy for balancing pulmonary blood flow with transposed great arteries.

OBJECTIVE: To study the outcomes of a novel modified pulmonary artery banding (mPAB) technique used for staged repair of a subset of patients with complex transposition physiology. METHODS: A total of 13 patients who underwent mPAB during their staged repair (biventricular repair [BVR], n = 6) or palliation (1-1/2 repair, n = 1; univentricular repair [UVR], n = 6) from 2004 to 2020 were studied retrospectively. A restrictive interposition graft was used to reconstruct the main PA between the pulmonary root and the distal pulmonary confluence, functioning as a mPAB. Twelve of the 13 patients (92.3%) underwent a concurrent arterial switch operation (ASO), of which 6 were palliative ASOs for 1-1/2 repair (n = 1) or UVR (n = 5). Patient weight and cardiac anatomy determined the size of interposition graft. RESULTS: The disease spectrum included dextro transposition of the great arteries (d-TGA) with multiple ventricular septal defects (VSDs) (n = 4), Taussig-Bing anomaly (n = 3), d-TGA with VSD and hypoplastic right ventricle (RV) (n = 3), double-inlet left ventricle with l-TGA (n = 2), and congenitally corrected TGA with double-outlet RV (n = 1). The Lecompte procedure was performed in 10 patients. Predischarge echocardiography revealed a band gradient of 61 mm Hg (interquartile range [IQR], 40-90 mm Hg) for BVR/1-1/2 ventricular repair (n = 7) and 49 mm Hg (IQR, 37-61 mm Hg) for UVR (n = 6). Survival was 100% at a median follow-up of 3.7 years (IQR, 2.6-4.0 years). CONCLUSIONS: The mPAB technique is effective and reproducible for staged BVR or UVR for patients with TGA. It effectively regulates pulmonary blood flow, may reduce neopulmonary root distortion, and eliminates complications associated with band migration in standard PAB.

Patient-Specific Approach to Mitral Valve Replacement in Infants Weighing 10 kilograms or less.

BACKGROUND: Mitral valve replacement (MVR) is the only option for infants with severe mitral valve disease that is not reparable; however, previously reported outcomes are not always favorable. Our institution has followed a tailored approach to sizing and positioning of mechanical valve prostheses in infants requiring MVR in order to obtain optimal outcomes. METHODS: Outcomes for 22 infants ≤10 kg who have undergone MVR in Sydney, Australia, from 1998 to 2016, were analyzed. Patients were at a mean age of 6.8 ± 4.1 months (range: 0.8-13.2 months) and a mean weight of 5.4 ± 1.8 kg at the time of MVR. Most patients (81.8%) had undergone at least one previous cardiac surgical procedure prior to MVR, and 36.4% had undergone two previous procedures. Several surgical techniques were used to implant mechanical bileaflet prostheses. RESULTS: All patients received bileaflet mechanical prostheses, with 12 receiving mitral prostheses and 10 receiving inverted aortic prostheses. Surgical technique varied between patients with valves implanted intra-annularly (n = 6), supra-annularly (n = 11), or supra-annularly with a tilt (n = 5). After a mean follow-up period of 6.2 ± 4.4 years, the survival rate was 100%. Six (27.3%) patients underwent redo MVR a mean of 102.2 ± 10.7 months after initial MVR. Four (18.2%) patients required surgical reintervention for development of left ventricular outflow tract obstruction and three (13.6%) patients required permanent pacemaker placement during long-term follow-up. CONCLUSIONS: The tailored surgical strategy utilized for MVR in infants at our institution has resulted in reliable valve function and excellent survival. Although redo is inevitable due to somatic growth, the bileaflet mechanical prostheses used displayed appropriate durability.

Two-dimensional and three-dimensional transthoracic echocardiography in surgical planning for right atrial metastatic melanoma.

Melanoma is the most common form of cardiac metastases. Surgical excision has been shown to be an effective palliative measure. This requires detailed definition of cardiac anatomy in relation to the tumour. Two-dimensional (2D), three-dimensional (3D) transthoracic (TTE) and transoesophageal echocardiography (TEE), spiral computerised tomography (CT) and magnetic resonance imaging (MRI) have all been described in aiding surgical planning for excision of cardiac tumours. In this case report, 3D-TTE provided excellent anatomical definition for surgical planning of a large right atrial melanoma precluding the need for more invasive and expensive investigations.

Congenitally corrected transposition: complex anatomic repair or Fontan pathway?

Background Successful anatomic repair of congenitally corrected transposition of the great arteries achieves excellent outcomes. Several centers report excellent long-term survival with the Fontan pathway as well. We have selectively applied both approaches depending on individual patient morphology, with anatomic repair preferred but utilizing the Fontan pathway when high technical complexity or operative risk is anticipated. Methods Hospital records over an 18-year period (1998-2016) were reviewed to identify patients with congenitally corrected transposition of the great arteries who underwent surgical management. Physiological repairs and hypoplastic ventricles were excluded. Patient- and procedure-related variables were reviewed. Results We identified 19 patients. Group 1 consisted of 12 anatomic repairs, of which 10 (83.3%) required prior interim staging procedures. Mean age at anatomic repair was 2.6 ± 1.3 years, mean follow-up was 8.7 ± 5.3 years. Nine (75%) patients experienced important complications and 4 (33.3%) required reintervention during follow-up. There were no deaths; one patient required heart transplantation. Group 2 (7 patients) underwent Fontan palliation. Mean age at Fontan completion was 7.2 ± 3.8 years, mean follow-up was 6.3 ± 4 years. There was no reintervention, death, or transplant. Conclusion Patients with congenitally corrected transposition of the great arteries and two adequate-sized ventricles do well with both anatomic repair and the Fontan pathway in the medium term. Excellent outcomes with reduced early complication and reintervention rates can be achieved for this cohort of patients when a strategy of avoiding complex anatomic repair in favor of the Fontan pathway is used.

Early outcomes from a new regional programme for the surgical management of hypoplastic left heart syndrome.

BACKGROUND: Early survival and quality of outcome after surgery for hypoplastic left heart syndrome (HLHS) are influenced by patient-specific factors, the quality of surgery and perioperative care. Some skills are common to the care of other complex neonatal presentations but integrating this expertise is a key challenge for new programmes. We began offering surgery for HLHS from 2006 and provided a regional service from January 2009 and report early outcomes. METHODS: Prospectively collected data for neonates with HLHS undergoing surgical palliation from January 2006 until June 2011 were analysed. Standard definitions of high-risk and standard-risk presentations were utilized. RESULTS: Thirty neonates underwent surgical palliation of HLHS with a modified Norwood procedure with an overall survival to stage II palliation of 80%. A total of 46.7% of our patients were categorized as high-risk, mostly on the basis of low birth weight. Survival to stage II palliation was 100% in standard-risk patients and 57.1% in the high-risk group. CONCLUSION: Outcomes for this new programme are comparable to reported outcomes demonstrating the feasibility of integrating a new complex procedure within an existing multidisciplinary unit handling large volumes of other complex neonatal work. Excellent outcomes can be achieved in standard-risk patients. Outcomes in the high-risk group may be improved by alternative approaches and rigorous case selection.

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.