Repair Of Anomalous Aortic Origin Research Articles

Histopathology of resected tissue from repair of anomalous aortic origin of a coronary artery: Potential mechanism of coronary artery compression

ObjectiveThis study aimed to describe the histomorphologic characteristics of resected (unroofed) common wall tissue from repair of anomalous aortic origin of a coronary artery and to determine whether the histologic features correlate with clinical and imaging findings. MethodsThe histology of resected tissue was analyzed and reviewed for the presence of fibrointimal hyperplasia, smooth muscle disarray, mucoid extracellular matrix accumulation, mural fibrosis, and elastic fiber disorganization and fragmentation using hematoxylin and eosin and special stains. Clinical, computed tomography imaging, and surgical data were correlated with the histopathologic findings. ResultsTwenty specimens from 20 patients (age range, 7-18 years; 14 males) were analyzed. Anomalous aortic origin of a coronary artery involved the right coronary in 16 (80%), and a slit-like ostium was noted in 18 (90%). By computed tomography imaging, the median proximal coronary artery eccentricity index was 0.4 (range, 0.20-0.90). The median length of intramural course was 8.2 mm (range, 2.6-15.2 mm). The anomalous vessel was determined to be interarterial in 14 patients (93%, 15 had evaluable images). The median distance from a commissure was 2.5 mm above the sinotubular junction (STJ) (range: 2 mm below the STJ–14 mm above the STJ). Prominent histopathologic findings included elastic fiber alterations, mural fibrosis, and smooth muscle disarray. The shared wall of the aorta and intramural coronary artery is more similar to the aorta histologically. Mural fibrosis and elastic fiber abnormalities tended to be more severe in patients >10 years of age at the time of surgery, but this did not reach statistical significance. The extent of vascular changes did not appear to have a clear relationship with the imaging features. ConclusionsThe findings confirm the aortic wall–like quality of the intramural segment of the coronary artery and the presence of pathologic alterations in the wall microstructure.

Surgical repair of the anomalous aortic origin of the coronary arteries: a single-center experience.

Anomalous aortic origin of the coronary artery is a rare congenital cardiac anomaly. The aim of this study was to present our experience with patients who underwent surgery for the anomalous aortic origin of the coronary artery. This was a retrospective review of our experience with patients who had anomalous aortic origin of the coronary artery from 2019 to 2022. Seven patients were managed for anomalous aortic origin of the coronary artery, including five males and two females. The median age of the patients were 16 years (IQR, 14.5-26 years). Five patients had anomalous aortic origin of the right coronary artery and two patients had anomalous aortic origin of the left coronary artery. Five patients were treated surgically, one patient refused surgical treatment despite myocardial ischaemia symptoms, and the other one was not operated because she had no symptoms. Two patients underwent pulmonary root anterior translocation and left main coronary artery unroofing procedure, one patient underwent right coronary artery unroofing procedure, one patient underwent pulmonary artery lateral translocation procedure, and the last patient underwent right coronary artery osteal translocation procedure. The post-operative mortality or myocardial infarction was not observed in any patient. Patients were followed for a median of 10 months (IQR, 6.75-20.5 months) after repair. The data suggest that surgical repair of anomalous aortic origin of the coronary artery can be performed confidently and can be very effective for relieving myocardial ischaemia symptoms. Different surgical techniques can be used in anomalous aortic origin of the coronary artery according to the course and origin of the coronary arteries. To the our knowledge, pulmonary root anterior translocation and coronary artery unroofing procedure were performed for the first time in the literature.

Pattern, behavior, and clinical implications of electrocardiographic changes in patients undergoing repair of anomalous aortic origin of coronary arteries

ObjectivesSurgical repair in anomalous aortic origin of a coronary artery aims at mitigating the risk of sudden cardiac death in a subset of patients. The pattern and behavior of electrocardiogram changes in a large cohort of these patients are lacking. We aim to describe postoperative electrocardiogram changes in this population and its clinical implications on follow-up. MethodsAll patients aged less than 21 years who underwent surgical repair for anomalous aortic origin of a coronary artery between December 2012 and June 2020 at our institution were considered for inclusion. Electrocardiograms were reviewed at 5 defined time intervals, from preoperative to 90-day follow-up, with attention to significant findings of ST-segment changes, abnormal T waves, and pathologic Q waves. The electrocardiogram changes were analyzed for correlation with surgical reintervention and medium-term outcomes. ResultsSixty-two patients met inclusion criteria (median age 13.7 years, 61% male). ST-segment changes in the initial postoperative period were seen in 52 patients (84%), all resolving over time. Abnormal T waves were seen in 19 patients (31%), occurred commonly at the predischarge period, and mostly resolved over time. Pathologic Q waves were observed in only 1 patient and associated with reintervention due to coronary artery stenosis. There was no association between postoperative electrocardiogram changes and inducible myocardial ischemia, ventricular dysfunction, or restriction from exercise at follow-up. ConclusionsST-segment changes and T-wave abnormalities are commonly seen in the postoperative period after anomalous aortic origin of a coronary artery repair, tend to resolve over time, and are not associated with adverse medium-term outcomes. Pathologic Q waves were associated with the need for early coronary reintervention.

Surgical approach for anomalous aortic origin of a coronary artery: A comparison of two techniques.

Un-roofing is the most common technique utilized for repair of anomalous aortic origin of a coronary artery (AAOCA). There are very few publications directly comparing un-roofing to another surgical technique, like reimplantation. The prospectively collected Children's Memorial Hermann Heart Institute Society of Thoracic Surgeon's Database was retrospectively reviewed from 2007 to 2021. Surgical patients were included if they underwent un-roofing or reimplantation of the AAOCA. The primary outcomes of this study were operative characteristics and postoperative outcomes. Secondary outcomes included angiographic outcomes, aortic regurgitation incidence, ventricular function, and symptom relief. From 2007 to 2021, there were 12 patients who underwent either a reimplantation (n = 9, 73%) or un-roofing (n = 3, 27%) for an AAOCA. The hospital length of stay was a median of 1.8 days longer for reimplantation compared to un-roofing. The last follow-up echocardiogram was a median of 52.2 days later in the reimplantation group. There was one patient (11%) in the reimplantation group that had more than or equal to mild aortic regurgitation and mild systolic ventricular dysfunction. Outpatient follow-up was incomplete and there was no postoperative computed tomographic angiography in the un-roofing cohort. Coronary artery reimplantation is a valuable alternative surgical technique to un-roofing for the repair of AAOCA. There are still some concerns with the creation of aortic regurgitation or incomplete symptom relief with any surgical technique. Longer-term follow-up and prospective studies will be needed to show an effective reduction of myocardial ischemia and risk of sudden cardiac death.

Abstract 16051: Pattern and Behavior of Electrocardiographic Changes in Patients Undergoing Repair of Anomalous Aortic Origin of Coronary Arteries

Introduction: Surgical repair in anomalous aortic origin of a coronary artery (AAOCA) aims at mitigating the risk of sudden cardiac death in a subset of patients (pts). The pattern and behavior of electrocardiogram (ECG) changes in a large cohort of these pts is lacking. We aim to describe post-operative (post-op) ECG changes in this population with medium term follow up (f/u). Methods: Observational study evaluating ECGs before and after surgery to describe peri-operative changes through f/u. All patients <21 years (yrs) with AAOCA were prospectively evaluated and managed following a standardized approach from 12/2012-11/2019. A subset underwent repair given high-risk features. ECGs were reviewed at 5 specific time intervals from pre-operative (pre-op) to 90-day post-op f/u. Significant findings included new ST elevation (STE), T wave inversion (TWI) and/or new q waves. Results: A total of 64 pts were identified (median age 13.7 yrs, 64% male). Two patients had evidence of STE and TWI pre-op and were not included. Of 62 pts: 47 (75%) had new STE early post-op and all but 1 had resolution at 90-day f/u; 50 (81%) had no evidence of new TWI or q waves early post-op and 96% of these remained stable at 90-day f/u. Conversely, 12 (19%) had new TWI in the early post-op: 8 had isolated changes (75% resolved at 90 days) and 4 associated with new q waves (50% resolved at 90 days). Including all ECG changes in the total cohort, 92% resolved completely at 90-day f/u. In those pts (8%) with persistent changes, none required late reintervention and all but 1 were cleared for return to exercise. All patients were alive at medium f/u of 4 yrs (IQR 2-6) with normal ventricular function. Conclusions: New STE is a common finding in the early post-op period following repair of AAOCA and is not correlated with persistent ECG changes at 90-day follow up. TWI is infrequently seen but less likely to resolve if associated with new q waves. Despite these changes in the early post-op, clinical outcomes medium-term are excellent.

Repair of Anomalous Aortic Origin of Pulmonary Artery: Technique Matters

The anomalous aortic origin of a pulmonary artery (PA) is uncommon, usually occurring on the right. Less commonly, the left PA is involved, often with a right-sided arch. Although referred to by some as “hemitruncus,” two semilunar valves are present. 1 Jacobs M.L. Congenital heart surgery nomenclature and database project: truncus arteriosus. Ann Thorac Surg. 2000; 69: S50-S55 Abstract Full Text Full Text PDF PubMed Google Scholar Because the right lung is exposed to systemic pressures, and the left receives the entire (and increased) cardiac output, there is high risk for developing pulmonary vascular disease (PVD). Typically, patients present with heart failure in infancy and undergo repair on diagnosis. The crux of the repair is translocation of the anomalous vessel to the main PA using various techniques. 2 Prifti E. Bonacchi M. Vanini V. Bartolozzi F. Double-flap technique for repair of anomalous origin of right pulmonary artery from ascending aorta. Ann Thorac Surg. 2004; 78: 1883-1884 Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar Whereas all report excellent short-term outcomes, varied rates of postoperative PA stenosis have been demonstrated. 3 Nathan M. Rimmer D. Piercey G. et al. Early repair of hemitruncus: excellent early and late outcomes. J Thorac Cardiovasc Surg. 2007; 133: 1329-1335 Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar Outcomes of Surgical Reimplantation for Anomalous Origin of One Pulmonary Artery From the AortaThe Annals of Thoracic SurgeryVol. 111Issue 4PreviewAnomalous origin of one pulmonary artery from the ascending aorta (AOPA) is a rare and potentially deadly anomaly. Little research, aside from case reports on APOA, has been published, especially for patients with late referrals. Full-Text PDF

Surgical repair of anomalous aortic origin of coronary artery in adults.

Anomalous aortic origin of a coronary artery is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. The surgical treatment of this anomaly is highly variable and controversial and is achieved by implementing elaborate techniques of anatomic repair or by simple coronary artery bypass grafting. This review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Two independent reviewers determined whether studies met the inclusion criteria. Eligible papers were published in English, clinical studies describing surgical repair of anomalous aortic origin of a coronary artery including coronary artery bypass in adults, and enrolled >5 patients. In the absence of multicentre trials, 7 single-center retrospective series were included, which demonstrated comparable short- and midterm outcomes of anatomic repair and coronary artery bypass in adults with anomalous aortic origin of a coronary artery. Coronary unroofing was the preferred technique when the anomalous artery has an intramural component, but there is a risk of aortic insufficiency. Anatomic repair is technically demanding and should be carried out in experienced centers. Coronary artery bypass with internal thoracic artery or vein grafts is technically straightforward with low operative risk and comparable midterm outcomes, however, long-term outcomes are unknown. Coronary artery bypass grafting is the technique of choice for older patients, in those with concomitant coronary artery disease, as a bailout procedure for failed anatomic repair, or in centers without experience in anatomic repair for anomalous aortic origin of a coronary artery.

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.

We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries. We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded. We collected 156 surgical patients (median age 39.5 years, interquartile range 15-53) affected by anomalous right (67.9%), anomalous left (22.4%) and other anatomical abnormalities (9.6%). An interarterial course occurred in 86.5%, an intramural course in 62.8% and symptoms in 85.9%. The operations included coronary unroofing (56.4%), reimplantation (19.2%), coronary bypass graft (15.4%) and other (9.0%). Two patients with preoperative cardiac failure died postoperatively (1.3%). All survivors were discharged home in good clinical condition. At a median follow-up of 2 years (interquartile range 1-5, 88.5% complete), there were 3 deaths (2.2%), 9 reinterventions in 8 patients (5 interventional, 3 surgical); 91.2% are in New York Heart Association functional class ≤ II, but symptoms persisted in 14.2%; 48.1% of them returned to sport activity. On Kaplan-Meier analysis, event-free survival at follow-up was 74.6%. Morbidity was not significantly different among age classes, anatomical variants and types of surgical procedures. Furthermore, return to sport activity was significantly higher in younger patients who participated in sports preoperatively. Surgical repair of anomalous aortic origin of coronary arteries is effective and has few complications. Unroofing and coronary reimplantation are safe and are the most common procedures. The occurrence of late adverse events is not negligible, and long-term surveillance is mandatory. Most young athletes can return to an unrestrained lifestyle.

Repair of Intramural Coronary Artery in Anomalous Aortic Origin of a Coronary Artery

It has become increasingly evident that anomalous aortic origin of a coronary artery is considered as an important anatomic anomaly in adolescents and adults. The question is whether or not surgical repair should be performed in asymptomatic patients. Previous reports showed that the numbers of asymptomatic patients who result in a sudden cardiac death are not negligible. Another reason to proceed to surgical intervention is the improvement in understanding this unique anatomy and the refinement of surgical techniques that brought the satisfied outcomes. Physiologic repair, that is, coronary artery bypass grafting was used to be the first choice of surgery; however, anatomic repair, such as coronary implantation and repair of intramural coronary artery is considered favorable in adolescents as "radical" oppose to "palliative" repair. We report our technique to relieve the intramural coronary artery based on the experience in arterial switch operation performed in neonates.

Surgical Unroofing for Anomalous Aortic Origin of Coronary Arteries

Anomalous aortic origin of a coronary artery (AAOCA) from an opposite sinus of Valsalva is an uncommon congenital defect that is typically only discovered on cardiac catheterization or on autopsy following sudden cardiac death. Conjectured mechanisms for the ischemia are generally believed to relate to the unique and easily obstructed geometry of the coronary ostium and the stenotic intramural coronary segment. Given the potentially devastating consequences of not repairing an AAOCA—that is, sudden cardiac death—these patients should undergo repair of the anomaly when it is discovered. Multiple options for surgically correcting the AAOCA have been developed, and the most used approaches include coronary artery bypass grafting, coronary reimplantation, and unroofing of the intramural segment. Here we present the technique for surgical unroofing of the intramural segment of an anomalous coronary artery of aortic origin.

Repair of anomalous aortic origin of coronary arteries with combined unroofing and unflooring technique

Repair of anomalous aortic origin of coronary arteries with combined unroofing and unflooring technique

Repair of anomalous aortic origin of a coronary artery in 113 patients: a Congenital Heart Surgeons' Society report.

Anomalous aortic origin of a coronary artery (AAOCA) encompasses a wide morphologic spectrum, which has impeded consensus regarding indications for the diverse repair strategies. We constructed a profile of current surgical techniques and explore their application to morphologic variants. Patients<30 years old (n=113) with isolated AAOCA who underwent operations at 29 Congenital Heart Surgeons Society (CHSS) institutions from 1998 to 2012 were identified from the CHSS AAOCA Registry. Operative findings were related to surgical techniques at index repairs by cross-tabulation. Anomalous origin of the left main or left anterior descending coronary artery was present in 33 (29%) patients and of the right coronary artery in 78 (69%) patients; 2 arteries originated directly above the commissure between the left and right sinuses. There were 101 (89%) interarterial and intramural (IA/IM) arteries, 10 (9%) were interarterial but not intramural (IA/NIM) and 2 (2%) were neither interarterial nor intramural. Intramural arteries were unroofed in 100 (88%) operations, usually with intimal tacking after incision (n=47) or excision (n=25) of the common wall. Coronary reimplantation (n=11), pulmonary artery relocation (n=7; 5 for IA/NIM), simple ostioplasty (without unroofing; n=3), coronary artery bypass grafting (n=2), and ostial window (n=1) were less common. In 37 (33%) operations, a valvar commissure was taken down; 33 were resuspended. Current surgical repair of AAOCA is individualized to morphology, particularly the presence of intramural and/or interarterial segments. This report is foundational for future planned CHSS studies that will examine interventional and noninterventional outcomes and ultimately guide management of AAOCA.

Surgical repair of anomalous aortic origin of a coronary artery

Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischaemia and sudden death. There is an ongoing controversy over the indications for surgical intervention and the efficacy of that treatment compared with the natural history. The purpose of this study was to evaluate the medium-term results of surgical repair of AAOCA. Seventy-six patients underwent surgical repair of AAOCA at our institution from 1999 to 2013. There were 55 males and 21 females, and the median age at surgery was 15 years. Forty-seven (62%) of the 76 patients had an anomalous right coronary artery, 27 had an anomalous left coronary and 2 had an eccentric single coronary ostia. Forty-one patients had preoperative symptoms of myocardial ischaemia. Surgical repair was accomplished by unroofing of an intramural coronary in 55, reimplantation in 7 and pulmonary artery translocation in 14. There has been no early or late mortality, with a median duration of follow-up of 6 years. One patient presented with severe myocardial ischaemia and subsequently underwent heart transplantation a year following AAOCA surgery. The remaining patients have all remained free of cardiac symptoms. The results of this study demonstrate two major principles. First, surgical repair of AAOCA is quite safe in centres that take care of a significant number of patients with this entity. Secondly, the surgery is highly effective in eliminating symptoms of myocardial ischaemia. The growing amount of data on postoperative patients suggests that surgical repair can prevent the adverse events seen in the untreated 'natural' history. Based on these observations, it is our current recommendation that all teenagers identified with AAOCA should undergo surgical repair.

Chest Pain and Ventricular Tachycardia in a Patient With Surgically Corrected Anomalous Right Coronary Artery From the Left Sinus of Valsalva

We present a case of an adult patient who had anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva that had been treated surgically in the past and who presented years later with chest pain and runs of nonsustained ventricular tachycardia. Coronary angiography showed a patent unroofed RCA with appropriately repositioned origin and no obstructive coronary artery disease. This case presents angiographic documentation of a technically satisfactory repair of anomalous aortic origin of a coronary artery and suggests that potentially lethal arrhythmia can occur despite a technically satisfactory repair.

Exercise performance and quality of life following surgical repair of anomalous aortic origin of a coronary artery in the pediatric population

We sought to evaluate exercise performance and quality of life in children after surgical repair of anomalous aortic origin of a coronary artery with an interarterial course. Patients who had surgery from October 2001 to January 2007 were eligible for inclusion. Exercise performance and quality of life were prospectively assessed by maximal exercise tests and age-appropriate questionnaires, respectively. We used t tests to compare pre- and postoperative exercise data and quality-of-life scores to published normative data. We performed linear regression analyses to assess associations between demographic, anatomic, and exercise variables and quality-of-life score. Of 25/27 patients, 64% were boys, 68% had anomalous right coronary, 32% were asymptomatic. Average age at surgery was 10.8 (+/-4.1) years; median follow-up was 14.5 (2 to 48) months. Postoperative percent-predicted exercise values were: peak heart rate 97 (+/-6), working capacity 91 (+/-15), maximal oxygen consumption 82 (+/-16). In those who had preoperative exercise testing (n = 11), resting and maximal heart rates decreased significantly without significant change in exercise performance. Average child quality of life was 85/100 (+/-13) and parent-proxy 88 (+/-11) compared with normal scores of 83 (+/-15) and 88 (+/-12), respectively. There is mild chronotropic impairment in children and adolescents following anomalous coronary artery repair without a decline in exercise performance. This does not appear to impair their overall quality of life. Because long-term effects on heart rate, exercise performance, and quality of life are unknown, serial exercise tests should be included as routine care of these patients.

Evaluation of Myocardial Ischemia After Surgical Repair of Anomalous Aortic Origin of a Coronary Artery in a Series of Pediatric Patients

Evaluation of Myocardial Ischemia After Surgical Repair of Anomalous Aortic Origin of a Coronary Artery in a Series of Pediatric Patients

Evaluation of Myocardial Ischemia After Surgical Repair of Anomalous Aortic Origin of a Coronary Artery in a Series of Pediatric Patients

We sought to prospectively evaluate evidence of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery with an interarterial course (AAOCA). An AAOCA is a rare anomaly associated with increased myocardial ischemia and sudden death risk in children. Data evaluating ischemia after AAOCA repair are limited. We included children who underwent AAOCA surgery between October 2001 and December 2006. They were prospectively assessed with exercise stress test (EST), stress echocardiogram (SE), and stress myocardial perfusion scan (MPS). Of 24 participating children, 15 (63%) were male, 16 (67%) had anomalous right coronary (ARCA), and 7 (29%) were asymptomatic. Median age was 12 (5 to 18) years; follow-up was 15 (2 to 48) months. All had unobstructed neo-coronary ostia by echocardiogram and were asymptomatic. One anomalous origin of left main coronary artery (ALCA) patient and 8 ARCA patients had post-operative evaluations suggestive of ischemia. The ALCA patient had reversible apical septal and mid-anteroseptal hypokinesis on SE. Of the ARCA patients, 2 had inferior ST-segment depression on EST; subsequently, 1 had normal tests, but the other developed anterolateral Q waves. Two patients had blunted blood pressure response with EST, 1 had fixed apical inferior hypokinesis on SE, 2 had reversible perfusion defects on MPS, and 1 had a fixed perfusion defect on MPS. Subclinical changes suggestive of ischemia might occur despite patent neo-coronary ostia, notably after ARCA repair. The implication of these results on indication for surgery and subsequent sudden death risk is unknown. Serial EST, SE, and MPS are essential in evaluating ongoing ischemia risk after AAOCA repair.

Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery

Background Anomalous aortic origin of a coronary artery (AAOCA) from an incorrect sinus of Valsalva is a relatively rare congenital defect and is associated with sudden death. Several surgical techniques have been described to address this defect, but functional outcome has never been addressed. In this report we evaluate a consecutive series of patients treated with unroofing techniques using transthoracic echocardiography, graded exercise testing, and stress echocardiography to assess functional repair. Methods Nine consecutive patients (range 7 to 65 years) underwent surgical repair of AAOCA from 1995 to 2001. In all patients the intramural segment was at or below the level of the commissure. All patients underwent a modified unroofing procedure to move the coronary artery orifice to the appropriate sinus. In 2 patients, a new orifice was created without significant unroofing and disruption of the commissure. Patients were evaluated prospectively with exercise electrocardiography testing and by resting and stress echocardiography. Results Of the 9 patients, 8 presented with symptoms suggestive of ischemia (chest pain, dyspnea on exertion, or syncope). Six patients had anomalous left main coronary artery arising from the right sinus of Valsalva, and 3 patients had anomalous right coronary artery from the left sinus of Valsalva. Transthoracic echocardiography and graded exercise testing was performed in all 9 patients (mean 29 months, range 4 to 85 months) after repair. Of the 9 patients, 8 also underwent stress echocardiography. In 8 of 9 patients the newly created coronary artery ostium was visualized by either two-dimensional echocardiography or color flow Doppler. All patients were symptom free at the time of follow-up. Exercise stress echocardiography was negative in all patients. Of the 8 patients, 7 had normal left ventricular shortening. No patients had regional wall motion abnormalities suggestive of ischemia. All patients were intervention free except 1 patient who developed severe aortic insufficiency and underwent a subsequent Ross procedure 44 months after his initial procedure. Conclusions Anomalous origin of a coronary artery from an incorrect sinus of Valsalva is known to be associated with increased risk of sudden death. Surgical correction can be carried out with minimal risk and good anatomic and functional results. Manipulation of the commissure can be avoided by creation of a neo-ostia without extensive unroofing of the intramural segment or manipulation of the intercoronary commissure. This may avoid aortic valve malfunction.