Anomalous Aortic Origin of the Coronary Arteries - State of the Art Management and Surgical Techniques.

Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We describe and compare the management and surgical techniques for patients with AAOCA. Patients presenting to the Coronary Artery Anomalies Program are evaluated and managed following a standardized approach. Our approach and data were compared to other single-center and multi-institutional data and results. Patients with AAOCA present as an incidental finding approximately 50% of the time. Advanced axial imaging is essential to define the anatomic characteristics of this lesion. Preoperative and postoperative assessment of myocardial perfusion with provocative testing is feasible and contributes to risk stratification. The surgical techniques for AAOCA repair include coronary unroofing, transection and reimplantation, and neo-ostium creation, among others. In general, surgical repair of AAOCA can mitigate the risk of ischemia with low mortality. The specific morbidities and complications of each different technique should be considered during the surgical planning. Surgical repair of AAOCA can mitigate the risk of ischemia with a low associated mortality but with clinically relevant morbidities. Long-term follow-up is necessary to accurately balance the risks of repaired and unrepaired AAOCA.

Smaller right pulmonary artery is associated with longer survival time without scimitar vein repair.

INTRODUCTION: The optimal management of scimitar syndrome remains incompletely defined. We (1) evaluated the impact of aortopulmonary collateral (APC) occlusion, (2) compared outcomes according to surgical approach for patients who underwent surgery, and (3) identified anatomic factors associated with longer survival time without scimitar vein repair. METHODS: We conducted a single center, retrospective study of 61 patients diagnosed with scimitar syndrome between 1995 and 2019. Right pulmonary artery to total pulmonary artery cross-sectional area (RPA:PA CSA) quantitatively assessed right pulmonary artery size. Anatomical features were analyzed for association with longer survival time without scimitar vein repair. RESULTS: Median follow-up time was 6 years (Q1-Q3, 2-12), with 96% 5-year survival. Twenty-three patients underwent APC occlusion, which significantly decreased symptoms of overcirculation (100%-46%; p = .001) and systolic pulmonary artery pressure (median, 34-29 mmHg; p = .004). Twenty-three patients underwent scimitar vein repair; 5-year freedom from scimitar vein stenosis was 90% among patients who underwent a reimplantation compared with 42% in patients with baffle repair (p = .1). Three patients underwent surgery before the first year of age, with lower 5-year freedom from scimitar vein stenosis (0% vs. 84%; p < .001). On multivariate analysis, a lower RPA:PA CSA was associated with longer survival time without scimitar vein repair (p = .003). CONCLUSIONS: APC occlusion improves the clinical status of young and hemodynamically unstable patients. Repair at an early age is associated with an increased risk of scimitar vein stenosis. Scimitar vein repair might be avoided in patients with a smaller right pulmonary artery.

Mid-Term and Long-Term Impact of Permanent Hypoparathyroidism After Total Thyroidectomy.

BACKGROUND: Permanent hypoparathyroidism (PH) is the most frequent long-term complication after total thyroidectomy. PH is related to many short-term and long-term complications, including clinical manifestations of hypocalcemia, hypercalcemia due to overtreatment, hyperphosphatemia, gastrointestinal, neuropsychiatric symptoms, decrease in renal function and infectious complications. The aim of this study was to identify the most frequent effects of PH and its associated risk factors. METHODS: We performed a retrospective analysis of a single institutional series of patients who developed PH after total thyroidectomy between 2000 and 2016. PH was defined as hypoparathormonemia (≤12 pg/mL) or the need for calcium/vitamin D supplementation to achieve normal calcium levels for more than 12 months. Descriptive and inferential statistics were employed based on the natural scaling of each included variable. RESULTS: Thirty-nine patients fulfilled the criteria for PH. Mean ± SD age was 46.26 ± 13.4 years; 6 (15.4%) were males and 33 (84.6%) females. Mean follow-up was 6.13 ± 3.25 years. Mean calcium carbonate supplementation doses per day were 18.95 ± 17.5 g and 21.4 ± 19.3 g at 1 year and last follow-up, respectively. Hypocalcemic crisis was the most common complication (66.7%), followed by neuropsychiatric (38.5%) and gastrointestinal symptoms (33.3%). Ten patients showed a decrease in renal function (eGFR drop ≥25%) and 4 developed chronic kidney disease. The amount of calcium supplementation was the most relevant related risk factor. CONCLUSIONS: PH is associated with multiple complications, including renal function impairment, gastrointestinal, neuropsychiatric and infectious complications. Lower calcium supplementation doses are related to lower complications rates.

Hypoparathyroidism After Total Thyroidectomy: Importance of the Intraoperative Management of the Parathyroid Glands.

BACKGROUND: Total thyroidectomy is the most common surgical procedure for the treatment of thyroid diseases. Postoperative hypocalcemia/hypoparathyroidism is the most frequent complication after total thyroidectomy. The aim of this study was to evaluate the rate of postoperative hypocalcemia and permanent hypoparathyroidism after total thyroidectomy in order to identify potential risk factors and to evaluate the impact of parathyroid autotransplantation. PATIENTS AND METHODS: We performed a retrospective analysis of 1018 patients who underwent total thyroidectomy at our institution between 2000 and 2016. Medical records were reviewed to analyze patient features, clinical presentation, management and postoperative complications. Descriptive and inferential statistics were employed based on the natural scaling of each included variable. Statistical significance was set at p ≤ 0.05. RESULTS: Mean ± SD age was 46.79 ± 15.9 years; 112 (11.7%) were males and 844 (88.3%) females. A total of 642 (67.2%) patients underwent surgery for malignant disease. The rate of postoperative hypocalcemia, transient, protracted and permanent hypoparathyroidism was 32.8%, 14.43%, 18.4% and 3.9%, respectively. Permanent hypoparathyroidism was significantly associated with the number of parathyroid glands remaining in situ (4 glands: 2.5%, 3 glands: 3.8%, 1-2 glands: 13.3%; p ˂ 0.0001) [OR for 1-2 glands in situ = 5.32, CI 95% 2.61-10.82]. Other risk factors related to permanent hypoparathyroidism were obesity (OR 3.56, CI 95% 1.79-7.07), concomitant level VI lymph node dissection (OR 3.04, CI 95% 1.46-6.37) and incidental parathyroidectomy without autotransplantation (OR 3.6, CI 95% 1.85-7.02). CONCLUSIONS: Identification and in situ preservation of at least three parathyroid glands were associated with a lower rate of postoperative hypocalcemia (30.4%) and permanent postoperative hypoparathyroidism (2.79%).

Correction to: Hypoparathyroidism After Total Thyroidectomy: Importance of the Intraoperative Management of the Parathyroid Glands.

In the original version of the article, the last three column headings in Table 3 were mislabeled. The original article has been corrected. Following is the corrected table.

Myocardial bridges in a pediatric population: Outcomes following a standardized approach.

OBJECTIVE: To describe clinical, functional, surgical, and outcomes data in pediatric patients with a myocardial bridge (MB) evaluated and managed following a standardized approach. METHODS: Prospective observational study included patients evaluated in the Coronary Artery Anomalies Program. Anatomy was determined by computed tomography angiography, myocardial perfusion by stress perfusion imaging, and coronary hemodynamic assessment by cardiac catheterization. RESULTS: In total, 39 of 42 patients with a complete evaluation for MB were included (December 2012 to June 2022) at a median age of 14.1 years (interquartile range, 12.2-16.4). Sudden cardiac arrest occurred in 3 of 39 (8%), exertional symptoms in 14 (36%), and no/nonspecific symptoms in 7 (18%) patients. Exercise stress test was abnormal in 3 of 34 (9%), stress perfusion imaging in 8 of 34 (24%), and resting instantaneous wave-free ratio ≤0.89 or diastolic dobutamine fractional flow reserve ≤0.80 in 11 of 21 (52%) patients. As a result, 15 of 39 (38%) patients were determined to have hemodynamically significant MB, 1 of 15 patients started beta-blocker, and 14 of 15 were referred for surgery. Myotomy (n = 11) and coronary bypass (n = 1) were performed successfully, resulting in improved symptoms and stress testing results. One patient required pericardiocentesis postoperatively, and all were discharged without other complications. At median follow-up time of 2.9 (1.8-5.8) years, all (except 2 pending surgery) were doing well without exercise restriction. CONCLUSIONS: Pediatric patients with MB can present with myocardial ischemia and sudden cardiac arrest. Provocative stress test and intracoronary hemodynamic tests helped risk-stratify symptomatic patients with MB and concern for ischemia. Surgical repair was safe and effective in mitigating exertional symptoms and stress test results, allowing patients to return to exercise without restriction.

Decellularized vs Non-decellularized Allogeneic Pulmonary Artery Patches for Pulmonary Arterioplasty.

We studied pulmonary artery size, reinterventions, and panel reactive antibodies in patients with single-ventricle physiology who underwent a pulmonary arterioplasty with decellularized (DAPAP) and non-decellularized allogeneic pulmonary artery patches (non-DAPAP). Retrospective review identified 59 patients with single-ventricle physiology who underwent pulmonary arterioplasty from 2008 to 2017: 28 patients underwent arterioplasty with DAPAP and 31 patients with non-DAPAP. Demographic and operative variables were similar between groups. Among patients who underwent a Norwood procedure, a right ventricle to pulmonary artery shunt was more commonly used in the DAPAP group (12/20, 60%) and a modified Blalock-Taussig shunt was more commonly used in the non-DAPAP group (17/22, 77%). On multivariable analysis, the use of DAPAP was associated with higher pre-Fontan angiography Z-scores in right (estimate = 0.17, standard error = 0.04, P = 0.0005) and left pulmonary arteries (estimate = 0.12, standard error = 0.05, P = 0.01). No areas of calcification, discrete coarctation, or pulmonary dilation were noted in any of the pulmonary arteries. On multivariable analysis, the use of DAPAP was associated with higher freedom from pulmonary artery reinterventions (Hazard ratio = 0.36, 95% confidence interval = 0.13-0.9, P = 0.04). The median value for Class I panel reactive antibodies was 0% (IQR 0, 4) in the DAPAP and 23% (IQR 14, 36) in the non-DAPAP group. The median value for Class II panel reactive antibodies was 15% (IQR 0, 17) in the DAPAP and 21% (IQR 10, 22) in the non-DAPAP group. Pulmonary arterioplasty with DAPAP was associated with higher pre-Fontan pulmonary artery Z-scores and higher freedom from pulmonary artery reinterventions.

Anomalous Aortic Origin of Coronary Arteries in Children: Postoperative High-risk Anatomic Features.

BACKGROUND: This study aimed to assess postoperative presumed high-risk anatomic features (HRAFs) by using computed tomographic angiography (CTA) in patients with anomalous aortic origin of a coronary artery (AAOCA) after surgical unroofing vs transection and reimplantation (TAR) if unroofing was thought to provide unsatisfactory results. METHODS: The study included 62 children with postoperative CTA performed at a median of 3 months (interquartile range, 3-4 months) after unroofing (n = 45) and TAR (n = 17). HRAFs included slitlike ostium, intramural course, acute angle takeoff (<45o), interarterial course, proximal stenosis >50%, or course through a thickened intercoronary pillar. RESULTS: Median age at surgery was 13.8 years (interquartile range, 10.5-15.8 years). None of the patients had a slitlike ostium or an intramural course on postoperative CTA. Acute takeoff was seen in 100% after unroofing and in 2 of 17 (12%) after TAR (P < .001). After unroofing, the interarterial course improved to 35 of 45 (78%) from 43 of 45 (96%) (P = .003), and a thickened intercoronary pillar improved to 10 of 45 (22%) from 22 of 45 (49%) (P = .0001), compared with none seen after TAR. Preoperative intramural length <5 mm was associated with a postoperative thickened intercoronary pillar in right AAOCA after unroofing (P = .0004). Severe coronary stenosis occurred in 2 of 17 (12%) after TAR, and both patients needed urgent revision procedures. All patients except 2 (97%) returned to exercise activities at a median follow-up of 4.9 years (range, 0.6-9.2 years). CONCLUSIONS: The slitlike ostium and intramural course resolved in all patients. Residual acute angle takeoff, an interarterial course, and mild coronary narrowing related to a thickened intercoronary pillar were common after unroofing. TAR allows resolution of all HRAFs, although severe narrowing requiring surgical revision happened only in TAR. Long-term studies are needed to understand the clinical significance of these residual presumed HRAFs.

Clinical Presentation and Medium-Term Outcomes of Children With Anomalous Aortic Origin of the Left Coronary Artery: High-Risk Features Beyond Interarterial Course.

BACKGROUND: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death in children. Surgery is recommended for interarterial AAOLCA, and other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of 3 AAOLCA subtypes. METHODS: All patients with AAOLCA <21 years old were prospectively enrolled (December 2012-November 2020), including group 1: AAOLCA from the right aortic sinus with interarterial course, group 2: AAOLCA from the right aortic sinus with intraseptal course, and group 3: AAOLCA with a juxtacommissural origin between the left and noncoronary aortic sinus. Anatomic details were assessed using computed tomography angiography. Provocative stress testing (exercise stress testing and stress perfusion imaging) was performed in patients >8 years old or younger if concerning symptoms. Surgery was recommended for group 1 and in select cases in group 2 and group 3. RESULTS: We enrolled 56 patients (64% males) with AAOLCA (group 1, 27; group 2, 20; group 3, 9) at median age of 12 years (interquartile range, 6-15). Intramural course was common in group 1 (93%) compared with group 3 (56%) and group 2 (10%). Seven (13%) presented with aborted sudden cardiac death (group 1, 6/27; group 3, 1/9); 1 (group 3) with cardiogenic shock. Fourteen/42 (33%) had inducible ischemia on provocative testing (group 1, 32%; group 2, 38%; group 3, 29%). Surgery was recommended in 31/56 (56%) patients (group 1, 93%; group 2, 10%; and group 3, 44%). Surgery was performed in 25 patients at a median age 12 (interquartile range, 7-15) years; all have been asymptomatic and free from exercise restrictions at median follow-up of 4 (interquartile range, 1.4-6.3) years. CONCLUSIONS: Inducible ischemia was noted in all 3 AAOLCA subtypes while most aborted sudden cardiac deaths occurred in interarterial AAOLCA (group 1). Aborted sudden cardiac death and cardiogenic shock may occur in AAOLCA with left/nonjuxtacommissural origin and intramural course, thus also deemed high-risk. A systematic approach is essential to adequately risk stratify this population.

Ischemia in Anomalous Aortic Origin of a Right Coronary Artery: Large Pediatric Cohort Medium-Term Outcomes.

BACKGROUND: Anomalous aortic origin of a right coronary artery may cause myocardial ischemia and sudden death in the young. Data on myocardial ischemia or longitudinal outcomes are sparse in pediatric anomalous aortic origin of a right coronary artery population. METHODS: Patients <21 years with anomalous aortic origin of a right coronary artery were prospectively enrolled. Computerized tomography angiography defined morphology. Exercise stress test and stress perfusion imaging (sPI) were performed if >7 years or younger with concern for ischemia. High-risk features included intramural length, slit-like/hypoplastic ostium, exertional symptoms, or evidence of ischemia. RESULTS: A total of 220 patients (60% males) were enrolled December 2012 to April 2020 at a median age 11.4 years (interquartile range, 6.1-14.5), including 168 (76%) with no/nonexertional symptoms (group 1) and 52 (24%) with exertional chest pain/syncope (group 2). Computerized tomography angiography was available in 189/220 (86%), exercise stress test in 164/220 (75%), and sPI in 169/220 (77%). Exercise stress test was positive in 2/164 (1.2%) patients in group 1, both had positive sPI. Inducible ischemia (sPI) was detected in 11/120 (9%) in group 1 and 9/49 (18%) in group 2 (P=0.09). Intramural length was similar in patients with/without ischemia (5 [interquartile range, 4-7] versus 5 [interquartile range, 4-7] mm; P=0.65). Surgery was recommended in 56/220 (26%) patients with high-risk features. In 52 surgical patients (38 unroofing, 14 reimplantation), all subjects were alive and have returned to exercise at last median follow-up of 4.6 (interquartile range, 2.3-6.5) years. CONCLUSIONS: Anomalous aortic origin of a right coronary artery patients can present with inducible ischemia on sPI despite symptoms or intramural length. Exercise stress test is a poor predictor of ischemia and caution should be given to determine low-risk based solely on this assessment. All patients are alive at medium-term follow-up.

Right Ventricle to Pulmonary Artery Conduit Size Is Associated with Conduit and Pulmonary Artery Reinterventions After Truncus Arteriosus Repair.

We studied conduit-related risk factors for mortality, conduit reintervention, conduit replacement, and pulmonary artery (PA) reinterventions after truncus repair. Patients who underwent truncus repair at our institution between 1995 and 2019 were studied. Cox proportional hazards modeling evaluated variables for association with mortality, time to conduit reintervention, time to conduit replacement, and time to PA reintervention. Truncus was repaired in 107 patients at median age of 17 days (IQR 9-45). Median follow-up time was 7 years. Aortic homografts were implanted in 57 (53%) patients, pulmonary homograft in 40 (37%), and bovine jugular conduit in 10 (9%). Median conduit size was 11 mm (IQR 10-12) and median conduit Z-score was 1.71 (IQR 1.08-2.34). At 5 years, there was 87% survival, 21% freedom from conduit reinterventions, 37% freedom from conduit replacements, and 55% freedom from PA reinterventions. Conduit size (HR 0.7, 95%CI 0.4-1.4, p=.41) and type (aortic homograft reference; bovine jugular vein graft HR 0.6, 95% CI 0.08-5.2, p=.69; pulmonary homograft HR 0.7, 95% CI 0.2-2.3, p=.58) were not associated with mortality. On multivariate analysis, the hazard for conduit reintervention, conduit replacement, and PA reintervention decreased with increasing conduit Z-score values of 1 to 2.5 (non-linear relationship, p<.01), with little additional reduction in hazard beyond this range. Implantation of a larger conduit within Z-score values of 1 and 2.5 is associated with a decreased hazard for conduit reintervention, conduit replacement, and PA reintervention after truncus repair. The type and size of the conduits did not impact mortality.

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

OBJECTIVES: Surgical 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. METHODS: All 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. RESULTS: Sixty-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. CONCLUSIONS: ST-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.

Coronary Artery Anomalies Are Associated With Increased Mortality After Truncus Arteriosus Repair.

BACKGROUND: Truncus arteriosus is associated with coronary anomalies. We identified coronary artery lesions in patients undergoing repair of truncus arteriosus, defined the impact of lesions on mortality, and studied the effect of surgical intervention of coronary lesions. METHODS: A retrospective review identified 107 patients with truncus repair (1995-2019). Coronary lesions were categorized as ostial stenosis, intramural, juxtacommissural origin, and single coronary. Survival analysis characterized survival after truncus repair and studied the association of coronary lesions and mortality. RESULTS: Among 107 patients with truncus repair 34 patients had at least 1 coronary lesion. Median follow-up time was 7 years, with 85% 5-year survival. Coronary lesions including ostial stenosis, intramurality, and juxtacommissural origin were associated with increased mortality, whereas single coronaries did not impact survival. Eleven patients had 1 coronary lesion and 6 patients with 2 coronary lesions had similar (80% and 83%, respectively) 5-year survival. Eight patients with 3 coronary lesions had 24% 5-year survival (P = .0003). Among patients with 1 or 2 lesions, surgical intervention on the coronary lesions tended to be associated with longer 5-year survival (100% vs 62%, respectively; P = .06). All patients with 3 lesions underwent coronary artery intervention, with 24% 5-year survival. CONCLUSIONS: Impact of coronary lesions on mortality after truncus repair increases with the number of lesions. Coronary artery intervention may be associated with improved time-related survival among patients with 1 or 2 lesions. Patients with the most complex anomalies (3 lesions) have poor survival and warrant ongoing study of repair techniques.

Outcomes in anomalous aortic origin of a coronary artery after surgical reimplantation.

OBJECTIVE: Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We compared outcomes data of patients who underwent transection and reimplantation (TAR) and patients who underwent an unroofing. METHODS: Patients who presented to the Coronary Artery Anomalies Program were evaluated and managed following a standardized approach. Anatomy was determined using computed tomography angiography, myocardial perfusion using advanced stress imaging, and surgical intervention according to anatomic features. RESULTS: Sixty-one patients underwent surgical repair of AAOCA between 2012 and 2019: 16 (26%) patients underwent TAR of the anomalous coronary without an aortic button and 45 (74%) patients underwent coronary unroofing. Compared with patients who underwent an unroofing, patients who underwent TAR had similar intramural length (5 mm with interquartile range of 4-7.7 vs 6 mm with interquartile range of 5-7; P = .6). One patient with an anomalous right coronary underwent coronary artery bypass grafting after TAR because of persistent postoperative ischemic changes. One patient with unroofing of an anomalous left coronary artery presented with recurrent aborted sudden cardiac death and underwent subsequent TAR, without further events. At last follow-up, 15 of 16 patients (94%) who underwent TAR and 42 of 45 (93%) patients who underwent an unroofing were released to unrestricted exercise activities. CONCLUSIONS: Coronary artery TAR is a useful surgical alternative for AAOCA when there is a course below the commissure, when unroofing does not relocate the ostium to the appropriate sinus, or when unroofing results in compression by the intercoronary pillar.