scholarly journals A Retrospective Cohort Study of Intravenous Immunoglobulin Therapy in the Acute Phase of Kawasaki Disease: The Earlier, the Better?

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Wei Li ◽  
Xiufang He ◽  
Li Zhang ◽  
Zhouping Wang ◽  
Yanfei Wang ◽  
...  

Background. Although intravenous immunoglobulin (IVIG) is expected to prevent coronary artery abnormalities of Kawasaki disease (KD) in the acute phase, the timing and effectiveness of IVIG remain to be determined. The association of timing of IVIG administration in KD patients with coronary artery abnormalities is evaluated in this cohort study. Methods. We systematically studied KD patients from two participating institutions between 2015 and 2017. To reveal the effectiveness of IVIG treatment, these patients were classified into four groups regarding the time of IVIG treatment. Primary outcome was coronary artery abnormalities by echo at diagnosis and 12 months follow-up; secondary outcomes included inflammatory markers. Results. A total of 1281 patients were included in this study. The best time of IVIG treatment cut-off values in 12 months follow-up for predicting coronary artery abnormalities was days 7.5 of illness onset. According to the best time of IVIG treatment cut-off values, all patients were classified into 4 groups. Group 1 was defined as earlier IVIG treatment administration on days ≤4 of the illness ( n = 77 ). Group 2 was defined with days 5-7 ( n = 817 ), group 3 with days 8-10 ( n = 249 ), group 4 with days >10 ( n = 138 ). A greater proportion of IVIG-resistant KD patients were group 4 than the other three groups, and there were significant differences ( p < 0.05 ). The incidence of coronary artery lesions (CALs) and coronary artery aneurysms (CAAs) in group 3 and group 4 was higher than that in group 1 ( p < 0.05 ) and group 2 ( p < 0.05 ) during a 12-month follow-up. Additionally, the incidence of CALs in group 1 was higher than that in group 2 but without statistical significance ( p > 0.05 ). The OR was significantly higher for those who started IVIG administration more than 7 days from the onset was positively associated with the occurrence of CALs (OR, 5.3; 95% CI, 2.0-13.9) and CAAs (OR, 13.5; 95% CI, 2.9-14.1) 12 months after initial onset. Multivariate regression revealed that the timing of IVIG treatment and IVIG-resistance was independent risk factors of CALs. Conclusions. IVIG treatment less than 7 days after illness onset are found to be sufficient for preventing developing coronary artery abnormalities in KD patients. Earlier IVIG treatment administration within 4 days may not increase the higher incidence of coronary artery abnormalities and IVIG resistance (Chinese Clinical Trial Registry:ChiCTR1800015800).

2020 ◽  
Author(s):  
Zhiyan Zhang ◽  
Runhui Tang ◽  
Heyang Sun ◽  
Haiyang Dai ◽  
Kangyin Chen ◽  
...  

Abstract We examined characteristics of chest CT during different time periods for patients with coronavirus disease 2019 (COVID-19) pneumonia in Huizhou, China. This study included 56 COVID-19 patients having abnormal CT acquired between January 22 and March 3, 2020. Scans of 56 patients were classified into 4 groups (Group 1-Group 4) based on the date on which scan was obtained at the 1st, 2nd, 3rd week and longer than 3 weeks after illness onset. Forty-five patients with follow-up scans were categorized into 4 groups according to extents that lesions reduced. GGO was prevalent in Groups 1-4 (58.1-82.6%). Consolidation was the more common in Group 2 (26.2%) and then declined in Group 3 and 4 (20.0%; 9.7%). The highest frequency of fibrous stripes occurred in Group 3 (46.7%) and then decreased to 35.5% in Group 4. CT scores were higher for Group 2 than others. Among 45 follow-up patients, 11 (24.4%) of them recovered with lesions reducing ≥75% and had shorter hospital durations compared with others. There were temporal patterns of lung abnormalities in COVID-19 patients, with the highest extent of lesion involvement occurring in the 2nd week. Isolation and review are required for COVID-19 patients who have been discharged from hospital.


2018 ◽  
Vol 100-B (10) ◽  
pp. 1372-1376 ◽  
Author(s):  
H. Bao ◽  
Z. Liu ◽  
M. Bao ◽  
Z. Zhu ◽  
P. Yan ◽  
...  

Aims The aim of this study was to investigate the impact of maturity status at the time of surgery on final spinal height in patients with an adolescent idiopathic scoliosis (AIS) using the spine-pelvic index (SPI). The SPI is a self-control ratio that is independent of age and maturity status. Patients and Methods The study recruited 152 female patients with a Lenke 1 AIS. The additional inclusion criteria were a thoracic Cobb angle between 45° and 70°, Risser 0 to 1 or 3 to 4 at the time of surgery, and follow-up until 18 years of age or Risser stage 5. The patients were stratified into four groups: Risser 0 to 1 and selective fusion surgery (Group 1), Risser 0 to 1 and non-selective fusion (Group 2), Risser 3 to 4 and selective fusion surgery (Group 3), and Risser 3 to 4 and non-selective fusion (Group 4). The height of spine at follow-up (HOSf) and height of pelvis at follow-up (HOPf) were measured and the predicted HOS (pHOS) was calculated as 2.22 (SPI) × HOPf. One-way analysis of variance (ANOVA) was performed for statistical analysis. Results Of the 152 patients, there were 32 patients in Group 1, 27 patients in Group 2, 48 patients in Group 3, and 45 patients in Group 4. Significantly greater HOSf was observed in Group 3 compared with Group 1 (p = 0.03) and in Group 4 compared with Group 2 (p = 0.02), with similar HOPf (p = 0.75 and p = 0.83, respectively), suggesting that patients who undergo surgery at Risser grade of 0 to 1 have a shorter spinal height at follow-up than those who have surgery at Risser 4 to 5. HOSf was similar to pHOS in both Group 1 and Group 2 (p = 0.62 and p = 0.45, respectively), indicating that undergoing surgery at Risser 0 to 1 does not necessarily affect final spinal height. Conclusion This study shows that fusion surgery at Risser 0 may result in growth restriction unlike fusion surgery at Risser 3 to 4. Despite such growth restriction, AIS patients could reach their predicted or ‘normal’ spinal height after surgery regardless of baseline maturity status due to the longer baseline spinal length in AIS patients and the remaining growth potential at the non-fusion levels. Cite this article: Bone Joint J 2018;100-B:1372–6.


2021 ◽  
pp. 036354652110469
Author(s):  
Benjamin R. Saks ◽  
Vivian W. Ouyang ◽  
Elijah S. Domb ◽  
Andrew E. Jimenez ◽  
David R. Maldonado ◽  
...  

Background: Access to quality health care and treatment outcomes can be affected by patients’ socioeconomic status (SES). Purpose: To evaluate the effect of patient SES on patient-reported outcome measures (PROMs) after arthroscopic hip surgery. Study Design: Cohort study; Level of evidence, 3. Methods: Demographic, radiographic, and intraoperative data were prospectively collected and retrospectively reviewed on all patients who underwent hip arthroscopy for femoroacetabular impingement syndrome (FAIS) and labral tear between February 2008 and September 2017 at one institution. Patients were divided into 4 cohorts based on the Social Deprivation Index (SDI) of their zip code. SDI is a composite measure that quantifies the level of disadvantage in certain geographical areas. Patients had a minimum 2-year follow-up for the modified Harris Hip Score (mHHS), Nonarthritic Hip Score (NAHS), International Hip Outcome Tool—12, and visual analog scale (VAS) for both pain and satisfaction. Rates of achieving the minimal clinically important difference (MCID) and patient acceptable symptom state (PASS) were calculated for the mHHS, NAHS, and VAS pain score. Rates of secondary surgery were also recorded. Results: A total of 680 hips (616 patients) were included. The mean follow-up time for the entire cohort was 30.25 months. Division of the cohort into quartiles based on the SDI national averages yielded 254 hips (37.4%) in group 1, 184 (27.1%) in group 2, 148 (21.8%) in group 3, and 94 (13.8%) in group 4. Group 1 contained the most affluent patients. There were significantly more men in group 4 than in group 2, and the mean body mass index was greater in group 4 than in groups 1 and 2. There were no differences in preoperative radiographic measurements, intraoperative findings, or rates of concomitant procedures performed. All preoperative and postoperative PROMs were similar between the groups, as well as in the rates of achieving the MCID or PASS. No differences in the rate of secondary surgeries were reported. Conclusion: Regardless of SES, patients were able to achieve significant improvements in several PROMs after hip arthroscopy for FAIS and labral tear at the minimum 2-year follow-up. Additionally, patients from all SES groups achieved clinically meaningful improvement at similar rates.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
Y Furukawa ◽  
T Yamada ◽  
T Morita ◽  
S Tamaki ◽  
M Kawasaki ◽  
...  

Abstract Background Catheter ablation (CA) for atrial fibrillation (AF) is a curable treatment option. However, AF recurrence after CA remains an important problem. Although the success rate has been improved after catheter ablation (CA) in patients with paroxysmal AF (PAF), outcome data after CA for persistent AF (PeAF) are highly variable. Previous studies showed the PeAF is one of independent predictors for AF recurrence in comparison to PAF. However, there are little information available on the prognostic significance of AF duration after CA for AF. The aim of this study is to evaluate the impact of AF duration on long-term outcomes of AF ablation in patients with PeAF compared with PAF. Methods We enrolled 778 consecutive patients, who were referred our institution between August 2015 and December 2017 for undergoing the first time CA for AF. We divided 5 groups (Group 1; PAF (n=442), Group 2; PeAF duration ≤6 months (n=198), Group 3; PeAF duration of 6 months to 2 years (n=87), Group 4; PeAF duration of 2–5 years (n=30) and Group 5; PeAF duration ≥5 years (n=21)). All patients followed up for at least 1 year. Outcome data on recurrence of AF after ablation were collected. Results There were no significant differences in baseline clinical characteristics before CA among 5 groups, except for the prevalence of congestive heart failure, left atrial diameter and left ventricular ejection fraction. During a mean follow-up period of 511±298 days, 217 patients had AF recurrence. Kaplan-Meier analysis revealed that AF recurrence was significantly higher in group 2 compared to group 1 (31% vs 20%, p=0.002) and in group 4 compared to group 3 (83% vs 30%, p<0.0001). However, AF recurrence was no significantly differences between groups 2 and 3 (31% vs 30%, p=0.76) and between groups 4 and 5 (83% vs 81%, p=0.45). Of 217 patients with AF recurrence, 154 patients had undergone multiple procedures. After last procedures, during a mean follow-up period of 546±279 days, 61 patients had AF recurrence. Kaplan-Meier analysis revealed that AF recurrence was significantly higher in group 2 compared to group 1 (10% vs 3%, P=0.0005) and in group 4 compared with group 3 (35% vs 10%, p=0.0001). However, AF recurrence was no significantly difference between groups 2 and 3 (10% vs 10%, p=0.91) and between groups 4 and 5 (47% vs 35%, p=0.47). AF Free Survival Curve Conclusion Although patients with PeAF within 2 years had significantly higher AF recurrence compared to PAF, AF ablation might still be a good contributor as the first line approach to improve outcomes in patient with PeAF within 2 years.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3285-3285
Author(s):  
Franck E. Nicolini ◽  
Nathalie Grardel ◽  
Sandrine Hayette ◽  
Jean-Michel Cayuela ◽  
Agnès Buzyn ◽  
...  

Abstract Chronic Myelogenous Leukemia (CML) originates in the chromosome (Ph1), a reciprocal translocation, corresponding to the BCR-ABL fusion oncogene. A small proportion (1–2%) of CML patients show breakpoints falling outside of the M-BCR gene on chromosome 22, leading to the synthesis of a variety of atypical BCR-ABL transcripts [either shortened: e1a2 (m-BCR), e6a2, e8a2, b2a3 (e13a3), b3a3 (e14a3), or elongated transcripts: e19a2 (m-BCR)] and to the synthesis of different BCR-ABL proteins. In this study, we retrospectively analysed the clinical characteristics and outcomes of cohorts of CML patients harbouring atypical transcripts in and treated with imatinib (IM). Twenty-two patients were analysed: 9 e1a2 [Group 1 (G1)], 4 e6a2 [Group 2 (G2)], 5 e19a2 [Group 3 (G3)], and 4 e8a2 [Group 4 (G4)] BCR-ABL transcripts. Two patients were in myeloid blastic phase at onset (1 in G1 and 1 in G2) and others in chronic phase. Age at diagnosis was significantly younger for e19a2 patients (39.5 years versus 64 for G1, 58.5 for G2, 72 for G3, p=0.005). Female patients were predominant for G1 (5F/3M), but not for other groups. All patients presented a classical Ph1 at karyotyping analysis at diagnosis, but 1 had a -7 (G1), 1 an additional t(11;16) with the Ph1 (G2), 1 a +8 (G3) and 1 a -Y (G3). The majority of patients presented typical CML features at diagnosis, however number of differences could be found: WBC counts were higher for e1a2 and e8a2 patients (74.2 109/l and 62.7 respectively vs 20.9 for G2, and 37.8 for G3, p&lt;0.03). A significant relative monocytosis was present for e1a2 patients (10% vs 4 (G2), 2.5 (G3), 5.5 (G4), p&lt;0.05), and a marked basophilia was present for e6a2 patients vs others (p&lt;0.0008). There was a trend for higher platelet counts in G3 vs others. Hasford and Sokal scores were somewhat comparable in all groups. Median follow-up since diagnosis was 24 months for G1, 10 for G2, 17 for G3 and 31 for G4. Only one patient received interferon for 7 months before IM (G1), all other patients did not receive any other treatment than hydroxyurea before IM. All patients were treated with IM alone initially at 400–600 mg/day. Median duration of IM was 18 months for G1, 9 for G2, 12 for G3, and 30 for G4. At time of analysis 1 pt in G2 and 1 patient in G3 died of progression (blastic phase), and the overall survival (OS) since IM start was better for e19a2 and e8a2 patients, but patients remain very few. However, these OS do not seem different from what has been observed for M-BCR transcripts (IRIS study). In conclusion, atypical BCR-ABL transcripts CMLs show particular diagnosis features, but their poor prognosis reputation seems abrogated by IM.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4885-4885
Author(s):  
Sobia Yaqub ◽  
Todd W. Gress ◽  
Oscar Ballester

Abstract Abstract 4885 Introduction: Fludarabine has been reported to increase the incidence of relapse and histological transformation in chronic lymphocytic leukemia (Thornton PD, Leukemia research, 2005) and Waldenstrom macroglobulinemia (Leleu X, J Clincal Oncology, 2009). The purpose of our study was to investigate the role of Fludarabine and the risk of transformation and relapse in follicular lymphoma (FL). Patients and Methods: This is a retrospective single institution study. We included 50 patients consecutively diagnosed with FL Grade I and II based on WHO classification of lymphoid malignancies. Grade III patients were excluded from the study. Median follow up is 2.86 years. Patients were grouped according to the initial therapy chosen by their treating physicians: Group 1(n=14) included patients on observation and radiation therapy, Group 2 (n=6) included patients on Fludarabine based regimens, Group 3(n=13) included CVP-R and other rituximab regimens and Group 4(n=17) included R-CHOP. Data collected included time to the onset of biopsy proven transformation, time to relapse, mortality and overall survival. Level of significance was set at <0.05. Results: Median age of the patients was 56.5 and it was not significantly different for the various groups. High risk FLIPI score was seen in 66% of patients treated with Fludarabine regimens as compared to 61% of R-CHOP treated patients. Overall, relapse occurred in 38% patients and transformation occurred in 16% patients during the follow up period. Fludarabine treated patients had the highest relapse rate: 50% (p=0.03). R-CHOP group has lowest relapse rate: 11%. Transformation rate was highest in the Fludarabine group: 33%, as compared to 13% to 17% in other groups (p=0.10). Mortality rate was 7% in group 1, 16% in group 2, 23% for group 3 and 5% in group 4 (p=0.44). Time to relapse/progression in group 1 was 2.9 years; in group 2 was 2.1 years; in group 3 was 2.7 years and in group 4 was 5.8 years. Conclusions: In our study, Fludarabine treated patients appear to be at higher risk for relapse and transformation compared to patients treated with R-CHOP. The differences can not be explained on the basis of known prognostic factors such as age or FLIPI score. The retrospective nature of the study and the small numbers of patients preclude more definitive conclusions. Further research is needed with large number of patients. Disclosures: No relevant conflicts of interest to declare.


2012 ◽  
Vol 38 (3) ◽  
pp. 267-271 ◽  
Author(s):  
K. Iba ◽  
T. Wada ◽  
K. Kanaya ◽  
G. Oki ◽  
T. Yamashita

We carried out arthrography in 19 thumbs of 18 patients in whom duplication was observed at the interphalangeal (Wassel type II) or metacarpophalangeal (Wassel type IV) joints on radiographs. The average age at surgery was 12.3 months and average duration of post-surgical follow-up was 21.3 months. Based on the arthrographic findings, the types of cartilaginous connections were subdivided into five groups. In group 1, there was a cartilaginous connection at the base of duplicated phalanges. In group 2, there was a cartilaginous connection of the radial digit between the distal and proximal phalanges, or between the proximal phalanx and metacarpal. In group 3, the phalanges separated at a common joint without any cartilaginous connection. In group 4, the radial digit demonstrated fibrous attachment to the capsule without any joint formation. In group 5, each joint was completely separated without any cartilaginous connection. These arthrographic findings could not be detected on radiographs. Different surgical procedures were carried out according to the form of cartilaginous connection.


2020 ◽  
Vol 27 (3) ◽  
pp. 36-48
Author(s):  
V. O. Shumakov ◽  
I. E. Malynovska ◽  
N. M. Tereshchenko ◽  
L. M. Babii ◽  
O. V. Voloshina

The aim – to study the clinical and functional characteristics of patients after myocardial infarction (MI) who referred stage II of cardiac rehabilitation (CR) with physical training (PT) during 3 years follow-up. Materials and methods. The study included 91 patients with primary Q-MI in the absence of contraindications to the CR. Criteria for inclusion were an early postinfarct angina, large aneurysm of the left ventricle, intracavitary formation of thrombus, the reduction of the EF to 35 % and below, complex cardiac arrhythmias and disturbances of conduction, the atrial fibrillation at the time of inclusion in the study, multivessel lession of coronary arteries, left bundle branch block, disorders of the musculoskeletal system, which prevented holding bicycle ergometry test, acute violation of cerebral circulation in the anamnesis, cancer and decompensation of comorbidities. Treatment was carried out according to modern recommendations; at admission coronary angiography with stenting of the infarct-occluded coronary artery was performed. Depending on the volume of rehabilitation measures, the patients were divided into two groups: group 1 consisted 47 patients who in the early post-hospital phase accomplished the program of PT on the bicycle ergometer; group 2 consisted of 44 patients in whom CR was carried out only in the form of distance walking and complexes of therapeutic exercises. Dosed physical load test on a bicycle ergometer, echocardiography, lipid metabolism indexes were evaluated in all patients at discharge from hospital. All exams were performed in dynamics in 4 months (the period corresponding to the end of the program 30 PT), after 1, 2 and 3 years. Results and discussion. At baseline the patients of both groups did not differ in any of the clinical-functional and anamnestic data. The clinical course was evaluated by the following indices: recurrent MI, coronary artery bypass grafting and stenting. Events increased after 2 (7 patients in 1 and 9 patients in 2 group) and 3 (6 and 15 patients, respectively) years. During the first year, all patients took 100 % of P2Y12 receptor blockers, rosuvastatin and beta-blockers; aspirin was used in 95 % of patients in each group; the number of patients who have received ACE inhibitors increased to 81 % in group 1 and 91 % in group 2. A decrease in the doses of statins at the outpatient stage as they move away from acute MI has led to an increase in LDL cholesterol over the years. This index in 4 months after MI in 1st group was 1.82 (1.39–2.20) and 2nd group was 1.83 (1.49–2.21) mmol/l, after 1 year – 1.79 (1.48–2.04) and 2.80 (2.33–3.21) mmol/l, after 2 years – 2.48 (2.12–2.98) and 2.34 (1,93–3.01) mmol/l, after 3 years – 2.29 (2.15–2.49) and 2.40 (2.26–2.61) mmol/l, respectively. The tolerance to physical load with the best hemodynamic efficiency of the work has increased significantly to (140.0; 125.0–150.0) W after 1 year compared with the 2nd group (p<0,01). For 3 years, it remained high in the 1st group, and it decreased to baseline levels in the 2nd group. Postinfarction remodeling processes were manifested by a decrease in EDV and an increase in EF, especially in the 1st group (p<0,01) after six months without significant dynamics for 3 years.Conclusions. CR with PT (30 sessions) contributed to an increase in exercise tolerance maximally after the end of training and lasted for 3 years. The aspects of psychological rehabilitation and health education for patients and their relatives were important (38 % of patients of the 1st group continued PT on their own at home). The training start time (on average, on the 15th or 40th day of MI) did not affect the results of the load test. It is important that a large percentage of patients continued to take the drugs recommended at discharge, but during 3 years follow up, the doses of the drugs were significantly reduced with insufficient control of hemodynamic and biochemical parameters, which led to the growth of one of the main factors in the progression of atherosclerosis – the level of low density lipoproteins.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L Di Serafino ◽  
H Gamra ◽  
P Cirillo ◽  
M Zimarino ◽  
I J Amat-Santos ◽  
...  

Abstract Background Duration of Dual Antiplatelet Therapy (DAPT) following Acute Coronary Syndromes (ACS) or Stable Coronary Artery Disease (SCAD) treated with coronary stenting is still debated. Although current guidelines consider several “clinical” criteria to decide for short DAPT (<6 months), standard DAPT (12 months) and prolonged DAPT (>12 months), the relationship between DAPT duration, treatment of bifurcations and its impact on clinical outcome has been poorly investigated in real world registries. Purpose We evaluated the impact of DAPT duration on clinical outcomes in consecutive all-comers patients treated with stenting of coronary artery bifurcation lesions included in the Euro Bifurcation Club -P2BiTO - registry. Methods Data on 5036 consecutive patients who underwent PCI on coronary bifurcation at 17 major coronary intervention centres between January 2012 and December 2014 were collected. The primary endpoint of the study was the cumulative occurrence of Major Adverse Cardiac Events (MACCE), defined as a composite of overall-death death, non-fatal myocardial infarction (MI), target vessel revascularization (TVR) and stroke during the follow-up; the secondary endpoints were the single occurrence of any of the above mentioned events. Results Data on DAPT duration was available for 3992 patients (79%). Patients were divided into 3 groups: Group 1) DAPT <6-months (n=720); Group 2) DAPT >6-months but <12-month (n=1602); Group 3) DAPT >12-months (n=1670). Follow up was completed in 3935 (98%) patients with a median of 20 months (C.I.=12–28). At 24 months after the index procedure, MACCE occurred more frequently in the DAPT <6-month group (Group 1) as compared with both Group 2 and 3 (respectively, 102 (14%) versus 154 (10%) and 164 (10%), HR: 0.72 (0.64–0.82), p<0.001). This difference remains after adjustment for clinical and angiographic characteristics (HR: 0.66 (0.58–0.77), p<0.001). On the contrary, no significant difference was found between Group 2 and Group 3 patients. At the Kaplan-Meier analysis (Figure 1), freedom from MACCE survival was significantly lower in patients receiving DAPT for less than 6 months (Log-Rank: 29.5, p<0.001). Figure 1. Kaplan-Meier curves Conclusions In the P2BiTO registry, short DAPT duration of less than 6 months was associated with a significantly higher risk of MACCE compared to longer DAPT in a real-world registry of patients treated for coronary artery bifurcation stenosis.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Tam T Doan ◽  
Carlos Bonilla-Ramirez ◽  
Shagun Sachdeva ◽  
J. Kevin Wilkes ◽  
Dana L Reaves-O’neal ◽  
...  

Introduction: Anomalous aortic origin of a right coronary artery (AAORCA) may lead to myocardial ischemia (ISCH) and sudden death in the young. Current guidelines deem asymptomatic patients (pts) low-risk if normal exercise stress test (EST). Hypothesis: The frequency of ISCH in AAORCA pts is seen more commonly on stress perfusion imaging (sPI) compared to EST. Method: Pts <21 yrs with AAORCA were prospectively enrolled and managed following a standardized approach (12/2012-04/2020). Coronary CTA was used to define details of AAORCA. EST and sPI were performed in pts >7 yrs and in younger pts if concern for ISCH. sPI included nuclear imaging initially and dobutamine stress cardiac MRI since 06/2014. High-risk features included intramural length (IML), abnormal ostium, symptoms or evidence of myocardial ISCH. Shared decision-making directed medical/surgical management. Results: Of 220 pts (male 135, 61%) enrolled at median age of 12 [IQR 6-15] yrs, 168 (76%) had no/non-exertional symptoms (Group-1) and 52 (24%) had exertional chest pain/syncope (Group-2). Coronary CTA was available in 180 and EST in 164 pts. EST was positive in 2/164 (1%), both had positive sPI. Of 162 negative EST, inducible ISCH was seen in 10/96 (10%) in Group-1 and 8/42 (19%) in Group-2. No difference in IML was noted between pts with/without ISCH (5.7±2.4 vs 6.0±2.1 mm, p=0.6). Surgery was indicated in 67 (30%) pts with high-risk features, not indicated in 143 (65%), and 10 (5%) are undergoing evaluation. In 51 operated pts (38 unroofing; 13 translocation), 3 await post-operative assessment, 48 on normal physical activities; median follow-up of 4.6 [IQR 2.3-6.3] yrs. All patients were alive at last follow-up; median of 2.9 [IQR 1.2-5.1] yrs. Conclusions: AAORCA pts can present with reversible ISCH on sPI regardless of symptoms or IML. EST may be a poor predictor of ISCH and caution should be given to determine low-risk. Most high-risk patients return to exercise and remain well at medium-term follow-up.


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