Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly with limited reports. This study characterizes QPV morphology and function by multimodal imaging, evaluates predictors of moderate-to-severe pulmonary regurgitation (PR), and assesses mid-term prognosis. Retrospective search of the imaging database at a tertiary medical center identified patients diagnosed with QPV between October 2004 and September 2024. Multimodal imaging was utilized to assess the characteristics of QPV and associated abnormalities. Among 1,367,280 cardiac CT and MR exams, 16 patients were diagnosed with QPV (38

An effective prognostic nomogram to predict the prognosis for supravalvular aortic stenosis (SVAS) patients is lacking. A multi-center retrospective study of consecutive SVAS patients with surgery between 2002 and 2020 was conducted. Patients underwent McGoon repairs, Doty repairs, and other repairs. The primary outcome was the re-operation or restenosis at follow-up. The nomogram based on Cox regression and Kaplan–Meier method was used to show the risk factors of the primary outcome. The predictive accuracy was determined by the concordance index (C-index) and calibration curve. The results were validated using the bootstrap resampling method. Of the 291 SVAS patients, 143 (49.1 http://www.chictr.org.cn , ChiCTR2300067851, 2023.01.29 (retrospectively registered).

To investigate the short-term/long-term impact of pulmonary artery intramural hematoma (PA-IMH) in patients with acute Stanford type A aortic dissection (ATAAD) following surgical repair. Consecutive patients with ATAAD who received surgical repair at Beijing and Yunnan Fuwai Hospital in 2010–2021 were retrospectively reviewed. Patients with hemorrhage extending along the PA were identified as the PA-IMH group. Multivariable logistics regression was used to obtain the odds ratio (OR), and the Kaplan-Meier method was used to estimate the survival rate. Of the 2046 ATAAD patients, 324 (15.8

BACKGROUND:Quadricuspid aortic valve (QAV) is a rare congenital heart disease with a limited body of literature. This retrospective cohort study investigates QAV morphology, function, and clinical outcomes. METHODS:Echocardiography was used to assess valvular function. Morphological characteristics such as phenotypes, raphe, regurgitant orifice area (ROA), and aortic dilation (diameter >40 ​mm) were assessed by cardiac CT. Patients were followed up for the combined event of all-cause death and aortic valve replacement (AVR). RESULTS:Ninety QAV patients (screened from 322385 CT scans) were included (mean age 55.2 ​± ​13.6 years, 61.1 ​% male). Isolated significant aortic regurgitation (AR) was present in 75.6 ​% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 ​%) and type II (3 larger and 1 smaller leaflets, 42.2 ​%) QAV. Fused raphe was present in 26.7 ​% of patients. ROACT was correlated with AR severity and aortic dilation (41.1 ​%, n ​= ​37). Among patients without AVR at baseline (n ​= ​60), one died and 17 underwent AVR during a median follow-up of 35.0 months (IQR:17.3-62.8). ROACT was associated with an increasing risk of combined event (as a categorical variable with a cut-off of 21.4 ​mm2, HR ​= ​4.25, 95%CI 1.49-12.17, p ​= ​0.007; as a continuous variable (per mm2 increment), HR ​= ​1.04, 95%CI 1.01-1.07, p ​= ​0.003). Additionally, ROACT had incremental prognostic value when added to the AR severity model (area under the receiver-operating characteristic curve increased from 86.8 to 88.4, p ​= ​0.004). CONCLUSION:QAV is characterized by variable anatomy, progressive AR, concomitant cusp fusion and aortic enlargement. ROACT may be a potential ancillary prognostic marker in patients with QAV.

Background: The appropriate age for surgical repair of asymptomatic congenital supravalvular aortic stenosis (SVAS) is still unknown. The purpose of this research was to assess the safety and effectiveness of various operation ages when managing SVAS. Methods: Consecutive asymptomatic SVAS pediatric patients in the Beijing Fuwai and Yunnan Fuwai hospitals over a period of 18 years were retrospectively analyzed. Patients were classified as follows: age <2.0 years (y) (n = 84), 2.0-5.0 y (n = 72), and >5.0 y (n = 92). The primary safety endpoint was in-hospital death or extracorporeal membrane oxygenation (ECMO) needed. The primary effectiveness outcome was re-operation or restenosis during follow-up. To calculate the hazard ratios (HR), Cox regression with inverse probability of treatment weighted was utilized. Results: At the time of surgery, the median age of the 248 patients that were included was 4 y (interquartile range (IQR): 1.8-6.5). For the primary safety outcome, 7 (8.3%) patients in the age <2.0 y group had in-hospital death or ECMO needed, while no patients in the age 2.0-5.0 y and age>5.0 y groups (p = 0.001). The median follow-up was 25.5 months (IQR: 7.0-59.0). Compared with the age 2.0-5.0 y group, the age <2.0 y group and age >5.0 y group had a higher risk of re-operation or restenosis (age <2.0 y, HR = 3.27, 95% CI 1.25-8.60; age >5.0 y, HR = 2.87, 95% CI 1.19-6.91). Conclusions: Asymptomatic children with SVAS without other cardiovascular abnormalities should be considered for delayed surgical intervention until 2 years of age, and then surgery should be conducted as soon as possible. Children with severe symptoms should undergo surgery immediately, regardless of age. Clinical Trial Registration: ChiCTR2300067851, https://www.chictr.org.cn/showproj.html?proj=177491.

Problem: Chest radiography is a crucial tool for diagnosing thoracic disorders, but interpretation errors and a lack of qualified practitioners can cause delays in treatment. Aim: This study aimed to develop a reliable multi-classification artificial intelligence (AI) tool to improve the accuracy and efficiency of chest radiograph diagnosis. Methods: We developed a convolutional neural network (CNN) capable of distinguishing among 26 thoracic diagnoses. The model was trained and externally validated using 795,055 chest radiographs from 13 datasets across 4 countries. Results: The CNN model achieved an average area under the curve (AUC) of 0.961 across all 26 diagnoses in the testing set. COVID-19 detection achieved perfect accuracy (AUC 1.000, [95% confidence interval {CI}, 1.000 to 1.000]), while effusion or pleural effusion detection showed the lowest accuracy (AUC 0.8453, [95% CI, 0.8417 to 0.8489]). In external validation, the model demonstrated strong reproducibility and generalizability within the local dataset, achieving an AUC of 0.9634 for lung opacity detection (95% CI, 0.9423 to 0.9702). The CNN outperformed both radiologists and nonradiological physicians, particularly in trans-device image recognition. Even for diseases not specifically trained on, such as aortic dissection, the AI model showed considerable scalability and enhanced diagnostic accuracy for physicians of varying experience levels (all P < 0.05). Additionally, our model exhibited no gender bias (P > 0.05). Conclusion: The developed AI algorithm, now available as professional web-based software, substantively improves chest radiograph interpretation. This research advances medical imaging and offers substantial diagnostic support in clinical settings.

Background An effective prognostic nomogram to predict the prognosis of patients with supravalvular aortic stenosis (SVAS) is lacking. This study sought to report the outcomes of different surgical techniques, identify the risk factors for postoperative re-operation or restenosis, and establish a prognostic nomogram. Methods This multi-centred retrospective cohort study included consecutive patients with SVAS who underwent surgery between 2002 and 2020. Patients underwent McGoon, Doty, and other repairs. The primary outcome was re-operation or restenosis at follow-up. The nomogram based on Cox regression and the Kaplan–Meier method was used to identify the risk factors for the primary outcome. The predictive accuracy was determined using the concordance index (C-index) and calibration curve. The results were validated using the bootstrap resampling method. Results Of the 291 patients with SVAS, 143 (49.1%) used McGoon repairs, 118 (40.5%) used Doty repairs, and 30 (10.3%) used other repairs. The median age at operation was 4.9 years (inter-quartile range (IQR) 2.3–9.9). After a median follow-up of 24 months (IQR 6.0–54.0), no difference in re-operation or restenosis was found between McGoon and Doty repairs. Age, gender, SVAS type, pulmonary artery stenosis, aortic valve stenosis, sinotubular junction z-score, and gradient were considered independent risk factors by least absolute shrinkage and selection operator regression and were included in the nomogram. The C-index of the nomogram was 0.71 (95% CI, 0.61 to 0.81). The calibration curve for the probability of re-operation or restenosis showed good agreement between prediction by nomogram and actual observation. Conclusions McGoon and Doty repairs showed no difference in re-operation and restenosis risk. The nomogram accurately predicted re-operation or restenosis in patients with SVAS after surgery. Trial Registration:

Background: To determine the effectiveness and safety of different patch materials in the treatment of pediatric patients with congenital supravalvular aortic stenosis (SVAS). Methods: 218 consecutive SVAS patients (age <14 years) who underwent surgery from Beijing Fuwai and Yunnan Fuwai hospital between 2002 and 2020 were included. Patients were divided into the pericardium patch group (133 (61.0%)), modified patch group (43 (19.7%)) and artificial patch group (42 (19.3%)). The primary safety endpoint was patch-related adverse complications (post-operation patch hemorrhage or aortic sinus aneurysm at 2-year follow-up). The primary effectiveness outcome was the re-operation or restenosis at 2-year follow-up. Multivariable cox regression was used to obtain the hazard ratio (HR). Results: The median age at operation was 43.5 months (IQR 24.0–73.0). Only three patients had patch-related adverse complications, and no difference existed among the three groups (p = 0.763). After a median follow-up of 24.0 months (IQR 6.0–48.0), patients with a pericardium patch had a lower re-operation or restenosis rate compared with the other two groups (pericardium patch vs modified patch, HR = 0.30, 95% CI 0.12–0.77; pericardium patch vs artificial patch, HR = 0.33, 95% CI 0.13–0.82), even in the main subgroup and sensitivity analysis. Conclusions: In pediatric patients, the safety of autologous pericardium patch is acceptable, along with lower rates of middle-term re-operation or restenosis. Clinical Trial Registration: http://www.chictr.org.cn, number: ChiCTR2300067851.

PURPOSE:To investigate the imaging characteristics and prognostic factors for the long-term survival of Behcet's disease (BD) with arterial involvement. METHODS:In this retrospective study, BD patients with arterial involvement were identified from January 2003 to January 2020. Arterial lesions were detected by ultrasonography, traditional arteriography, and/or computed tomography angiography (CTA). Cox proportional hazards regression analyses were performed to identify the prognostic factors. RESULTS:Totally, 84 BD patients with arterial involvement were identified (73.8 % males). The mean age at BD diagnosis was 39.1 ± 13.1 years. Arterial involvement was the initial manifestation in 33.3 % of the patients, and the median time from BD diagnosis to arterial involvement was 6 (IQR 1-15.5) years for the rest of patients. Systemic artery involvement and pulmonary artery involvement (PAI) were found in 64 and 27 patients, respectively. Approximately 94.0 % (79/84) of the patients had more than one artery involved concurrently or successively during the course of BD. Aneurysm/dilation was the most prevalent lesion in the aorta (76.0 %), while stenosis/occlusion was the main lesion of the coronary artery (90.9 %) and other aortic branches (74.5 %). Pulmonary hypertension was found in 70.4 % (19/27) of patients with PAI. The 5- and 10-year survival rates of BD patients with arterial involvement were 87.4 % and 84.1 %, respectively. Cardiac involvement (HR: 4.34) and pulmonary artery aneurysm/dilation (HR: 4.89) were independently associated with mortality. CONCLUSIONS:Arterial lesions associated with BD usually involve multiple arteries and manifest differently in different types of arteries. Cardiac involvement and pulmonary artery aneurysm/dilation are independent prognostic factors of BD patients with arterial involvement.

The study aimed to determine the effectiveness and safety of anomalous coronary artery from pulmonary artery (ACAPA) patients with moderate or severe mitral valve regurgitation (MVR) receiving mitral valve plasty (MVP) concurrently. Consecutive ACAPA patients undergoing surgery between 2015 and 2021 were retrospectively included. Patients were divided into three groups: moderate MVR without MVP (non-MVP (moderate) N = 14), moderate MVR with MVP (MVP (moderate) N = 13), and severe MVR with MVP (MVP (severe) N = 13). The primary safety endpoint was in-hospital surgery-related complications. The primary effectiveness outcome was left ventricular ejection function (LVEF) and left ventricular end-diastolic diameter (LVEDD) z-score at 2- and 24-month follow-ups. Multivariable linear regression models were used to obtain the β coefficient. The median age of the included patients was 7.5 years (IQR 1.4–26.5). The in-hospital surgery-related complication rates were 7.1%, 15.4%, and 7.7% in non-MVP (moderate), MVP (moderate), and MVP (severe) groups, separately. At the 2-month follow-up, the non-MVP (moderate) group had a better LVEF and LVEDD z-score compared with the MVP (moderate) group (LVEF β = 9.22, 95%CI 1.09 to 17.35; LVEDD z-score β = −2.49, 95%CI −4.53 to −0.45). At the 24-month follow-up, the LVEF of all patients and the LVEDD z-score of 90% of patients in the three groups returned to normal. For ACAPA patients with moderate MVR, MVP was not necessary, especially for pediatric patients (age < 3 years) and patients with secondary MVR. Further studies for ACAPA patients with severe MVR are still needed.

Studies relating to the right ventricle (RV) are inadequate, and specific diagnostic algorithms still need to be improved. This essay is designed to make exploration and verification on an algorithm of deep learning based on imaging and clinical data to detect RV abnormalities. The Automated Cardiac Diagnosis Challenge dataset includes 20 subjects with RV abnormalities (an RV cavity volume which is higher than 110 mL/m2 or RV ejection fraction which is lower than 40

Objective To optimize gadolinium contrast agent injection protocol according to CMRI scanning time and image quality.Methods A total of 40 patients who underwent enhanced CMRI scanning in our hospital were collected.1.5T Somanton Avanto Tim 2D gradient echo （GRE） FLASH sequence was used for collecting myocardial short axis delayed image.Through comparing CMRI scanning time,contrast agent dosage and image quality,the gadolinium contrast agent injection protocol was optimized.Results The statistical results showed that there was statistical differences in the interval between the end of the first perfusion scan and the start of the delayed scant[t=6.49,P＜0.05,SATD 95%CI 205.85 （141.62-270.08）].There was statistical differences in contrast agent dosage between the observation group and the control group[t=2.86,P=0.007 SATD 95%CI 3.53 （1.03-6.03）].There was no statistical significance in the differences of short-axis delayed images between the observation group and the control group （P＞0.05）.Conclusions During CMRI examination,the dual-tube single-phase dual-flow injection protocol not only shortened the scanning time,but also reduced the dosage of gadolinium contrast agent under the premise of ensuring the same image quality.

目的:探讨永存第五对主动脉弓(PFAA)的解剖学特征,评价几种常用影像学方法的诊断价值.方法:回顾分析2010年10月至2019年9月中国医学科学院阜外医院诊断的8例PFAA患者,收集其临床、影像及手术资料,总结其解剖及影像学特征、类型和伴发畸形情况,对比X线胸片、超声心动图、CT血管造影(CTA)检查结果.结果:本组8例患者中,7例的PFAA与主动脉弓位于同侧、不形成血管环结构,4例的PFAA伴发狭窄及闭锁,8例PFAA均起自升主动脉远端无名动脉开口旁或对侧,6例止于降主动脉近端,2例止于一侧肺动脉干;8例患者均合并心血管畸形,包括室间隔缺损、主动脉弓离断、法乐四联症、动脉导管未闭、共同动脉干、右心室双出口、肺动脉闭锁,以室间隔缺损和主动脉弓离断最常见;CTA对8例PFAA及伴发的其他心血管畸形患者均做出准确诊断,其准确度优于超声心动图及造影结果.结论:PFAA的解剖特征为:起自升主动脉远端无名动脉开口旁或对侧、止于降主动脉近端或一侧肺动脉干,呈弓状血管结构,部分伴发狭窄及闭锁,常合并其他心血管畸形.CTA为PFAA首选的无创检查方法,可部分替代或补充血管造影结果.

目的 探讨冠状动脉旁路移植术前应用多层螺旋CT血管造影术(MSCTA)评估胸廓内动脉解剖特性的临床价值.方法 应用MSCTA技术对199例CABG术前患者的胸廓内动脉进行分析,测量左胸廓内动脉长度,观察其起源、走行、分支、变异情况.结果 199例胸廓内动脉主干及其终支均能清晰显现,其中45例发生起源变异,变异率为22.6％,不同性别之间比较无统计学意义(P>0.05).变异类型以甲状颈干共干多见,占42例(21.1％),另有1例(0.5％)起自肩胛上动脉,2例(1.0％)起自腋动脉.左胸廓内动脉平均长度(193.80±28.74)mm,性别差异无统计学意义(P>0.05).结论 MSCTA可以清晰显示胸廓内动脉的起源、主要分支及变异等解剖结构,对冠状动脉旁路移植术前预评估有重要意义.

Poster: ECR 2019 / C-2883 / CT diagnosis and prognosis in patients with aortic dissection or intramural haematoma type A involving pulmonary artery by: A. Zhi , R. Dai, P. Zhang, S. Jiang, B. Lv; Beijing/CN

Objective: The use of the frozen elephant trunk technique for type A aortic dissection in Marfan syndrome is limited by the lack of imaging evidence for long-term aortic remodeling. We seek to evaluate the changes of the distal aorta and late outcomes after frozen elephant trunk and total arch replacement for type A aortic dissection in patients with Marfan syndrome. Methods: Between 2003 and 2015, we performed frozen elephant trunk thorn total arch replacement for 172 patients with Marfan syndrome suffering from type A aortic dissection (94 acute; 78 chronic). Mean age was 34.6 +/- 9.3 years, and 121 were male (70.3%). Early mortality was 8.1% (14/172), and follow-up was complete in 98.7% (156/158) at a mean of 6.2 +/- 3.3 years. Aortic dilatation was defined as a maximal diameter of greater than 50 mm or an average growth rate of greater than 5 mm/year at any segment detected by computed tomographic angiography. Temporal changes in the false and true lumens and maximal aortic size were analyzed with linear mixed modeling. Results: After surgery, false lumen obliteration occurred in 86%, 39%, 26%, and 21% at the frozen elephant trunk, unstented descending aorta, diaphragm, and renal artery, respectively. The true lumen expanded significantly over time at all segments (P < .001), whereas the false lumen shrank at the frozen elephant trunk (P < .001) and was stable at distal levels (P > .05). Maximal aortic size was stable at the frozen elephant trunk and renal artery (P > .05), but grew at the descending aorta (P = .001) and diaphragm (P < .001). Respective maximal aortic sizes before discharge were 40.2 mm, 32.1 mm, 31.6 mm, and 26.9 mm, and growth rate was 0.4 mm/year, 2.8 mm/year, 3.6 mm/year, and 2.6 mm/year. By the latest follow-up, distal maximal aortic size was stable in 63.5% (99/156), and complete remodeling down to the mid-descending aorta occurred in 28.8% (45/156). There were 22 late deaths and 23 distal reoperations. Eight-year incidence of death was 15%, reoperation rate was 20%, and event-free survival was 65%. Preoperative distal maximal aortic size (mm) predicted dilatation (hazard ratio, 1.11; P < .001) and reoperation (hazard ratio, 1.07; P < .001). A patent false lumen in the descending aorta predicted dilatation (hazard ratio, 3.88; P < .001), reoperation (hazard ratio, 3.36; P = .014), and late death (hazard ratio, 3.31; P = .045). Conclusions: The frozen elephant trunk technique can expand the true lumen across the aorta, decrease or stabilize the false lumen, and stabilize the distal aorta in patients with Marfan syndrome with type A aortic dissection, thereby inducing favorable remodeling in the distal aorta. This study adds long-term clinical and radiologic evidence supporting the use of the frozen elephant trunk technique for type A dissection in Marfan syndrome.

Objective: The use of the frozen elephant trunk (FET) technique for repair of type A aortic dissection (TAAD) in Marfan syndrome (MFS) is controversial. We seek to evaluate the efficacy of FETand total arch replacement (TAR) for TAAD in patients with MFS.Methods: The early and long-term outcomes were analyzed for 106 patients with MFS (mean age, 34.5 +/- 9.7 years) undergoing FET + TAR for TAAD.Results: Operative mortality was 6.6% (7 of 106). Spinal cord injury and stroke occurred in 1 patient each (0.9%), and reexploration for bleeding occurred in 6 patients (5.7%). Extra-anatomic bypass was the sole risk factor for operative mortality and morbidity (odds ratio [OR], 7.120; 95% confidence interval [CI], 1.018-49.790; P = .048). Follow-up was complete in 97.0%(96 of 99), averaging 6.3 +/- 2.8 years. Late death occurred in 17 patients. Patients with acute TAAD were less prone to late death than those with chronic TAAD (OR, 0.112; 95% CI, 0.021-0.587; P = .048). Twelve patients required late reoperation, including thoracoabdominal aortic repair in 8, thoracic endovascular aortic repair for distal new entry in 3, and coronary anastomotic repair in 1. At 5 years, survival was 86.6% (95% CI, 77.9%-92.0%) and freedom from reoperation was 88.8% (95% CI, 80.1%-93.4%), and at 8 years, survival was 74.1% (95% CI, 61.9%-83.0%) and freedom from reoperation was 84.2% (95% CI, 72.4%-91.2%). In competing risks analysis, mortality was 4% at 5 years, 18% at 8 years, and 25% at 10 years; the respective rates of reoperation were 10%, 15%, and 15%; and the respective rates of survival without reoperation were 86%, 67%, and 60%. Survival was significantly higher in patients who underwent root procedures during FET + TAR (P = .047). Risk factors for reoperation were days from diagnosis to surgery (OR, 1.160; 95% CI, 1.043-1.289; P = .006) and Bentall procedure (OR, 12.012; 95% CI, 1.041-138.606; P = .046).Conclusions: The frozen elephant trunk and total arch replacement procedure can be safely performed for TAAD in MFS with low operative mortality, favorable long-term survival and freedom from reoperation. A concomitant Bentall procedure was predictive of better long-term survival and increased risk for late reoperation. These results argue favorably for the use of the FET + TAR technique in the management of TAAD in patients with MFS.

BACKGROUND:To evaluate the role of CT angiography (CTA) in the diagnosis and subcategorization of unroofed coronary sinus syndrome (URCS).METHODS:We retrospectively analyzed 46 URCS patients diagnosed by CTA. Based on the defect location and size of coronary sinus (CS), URCS was divided into four types: complete defect as type I, partial defect of proximal CS as type II, partial defect of distal CS as type III, partial defect in which a communication occurs between CS and left atrial as type IV. According to presence of left superior vena cava (LSVC), all types were divided into 2 subtypes as a and b. All 46 patients underwent echocardiography.RESULTS:According to subcategorization of URCS by CTA, type I was observed in 23 cases (Ia 7, Ib 16), type II in 10 cases (IIa 3, IIb 7), type III in 12 cases (IIIa 3, IIIb 9), and type IV in 1 case classified as IVb subtype. In these 46 cases, 21 were detected by echocardiography as URCS (46%). The sensitivity of echocardiography in detecting URCS was significantly lower compared with cardiac CTA (P<0.05). In type I patients, the mean CS diameter indexed to body surface area (CS index) was larger than other types (P<0.05). Thirty patients were successfully treated by surgery and the diagnosis of URCS was confirmed by operative findings. Among them, data were available in 22 cases for analysis; and patients with types I, II and IIIa differ significantly from those with types IIIb and IV (P<0.05) with respect to surgical repair.CONCLUSIONS:CTA and imaging reconstruction can provide excellent anatomical delineation of the heart, great vessels, and CS, and allows for precise diagnosis of URCS. This CTA classification scheme of URCS is simple and easy to use, and has important clinical implications for diagnosis and treatment.

Objective: To explore the imaging and clinical features of Behcet's disease with coronary artery involvement in order to improve the diagnosis and treatment in clinical practice. Methods: A total of 6 Behcet's disease patients with coronary involvement diagnosed and treated in our hospital from 1999-08 to 2016-11 were analyzed. Coronary angiography (CAG) and coronary CTA were performed for diagnosis. There were 5 cases received CAG and 5 cases received CTA examinations. Results: All 6 patients had the first clinical visit by sudden pericardial tapenade, myocardial infarction or cardiac shock. For diagnosis: the imaging presentation included coronary stenosis, occlusion and pseudo aneurysm formation. 3 patients had anterior descending artery involvement, 1 had the far-end of left circumflex involvement and 2 had the middle segment of right coronary involvement; all patients were combining large amount of pericardial effusion. For treatment: there were 3 patients with aneurysm received conservative treatment (1 case had open thoracic exploration), 1/3 lost contact and 2 having long-term normal life; 3 patients received coronary stent implantation without optimal effect. Conclusion: The patients of Behcet's disease with coronary involvement may easily form pseudo aneurysm; CAG/CTA has been helpful for diagnosis. Clear pre-operative diagnosis of etiology is important for treatment.

Objective: To evaluate the clinical characteristics in patients with coronary sinus oriifce atresia (CSOA). Methods: We retrospectively studied 22 CSOA patients for their clinical characteristics of cardiovascular involvement. They were treated in our hospital from 2005-02 to 2014-05 including 15 male and 7 female at the age of 1.5-76 (50.88 ± 14.97) years. All patients receive echocardiography and coronary CTA examination. Results: The diagnosis of CSOA was confirmed by contrast-enhanced cardiac MDCT in all patients. According to coronary vein blood flow direction, the patients were classified into 3 types: LA (left atria) type,n= 9 patients with blood flow back into LA including 3 cases from left superior vena cava (LSVC) to LA and 2 combining with complicated congenital cardiac abnormalities. RA (right atria) type,n=5 patients with blood flow back to RA including 2 cases directly to RA and 3 cases indirectly from LSVC to RA. Mixed type,n=8 patients with small branch of blood flow back to both LA and RA including 4 cases combining LSVC involvement. There were 9/22 patients with LSVC involvement including 3 with blood flow directly to LA and 6 with blood flow via in-nominate vein to RSCV then to RA. Echocardiography could not diagnose CSOA. ECG showed 5 patients with arrhythmia and 3 of them had successful RFCA, 1 failed RFCA and 1 received permanent pace maker implantation. There were 6/22 patients combining congenital cardiac abnormalities including 1 of atrial septal defect (ASD), 5 of complicated congenital heart disease (CHD), and 3 of them with confirmed CSOA diagnosis by contrast-enhanced MDCT before operation, 2 having post operative symptom and the diagnosis was confirmed by CT. All 5 CHD patients received operation and no one had operation for CSOA alone. Conclusion: Clear diagnosis of CSOA and its associated arrhythmia, LSVC involvement and cardiac abnormalities are important for preventing cardiovascular accident. Contrast-enhanced MDCT has the important value for diagnosing the patients with CSOA.