Hemodynamics, anatomy, and outcomes of quadricuspid aortic valves: Multimodality imaging assessment.

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 â€‹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 â€‹± â€‹13.6 years, 61.1 â€‹% male). Isolated significant aortic regurgitation (AR) was present in 75.6 â€‹% of patients. The cohort was dominated by type I (four equal leaflets, 37.8 â€‹%) and type II (3 larger and 1 smaller leaflets, 42.2 â€‹%) QAV. Fused raphe was present in 26.7 â€‹% of patients. ROACT was correlated with AR severity and aortic dilation (41.1 â€‹%, n â€‹= â€‹37). Among patients without AVR at baseline (n â€‹= â€‹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 â€‹mm2, HR â€‹= â€‹4.25, 95%CI 1.49-12.17, p â€‹= â€‹0.007; as a continuous variable (per mm2 increment), HR â€‹= â€‹1.04, 95%CI 1.01-1.07, p â€‹= â€‹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 â€‹= â€‹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.

Imaging characteristics and prognostic factors of Behcet's disease with arterial involvement: A long-term follow-up study.

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.

Development of an Expert-Level Right Ventricular Abnormality Detection Algorithm Based on Deep Learning.

PURPOSE: 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. METHODS: 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%) and 20 normal subjects who suffered from both cardiac MRI. The subjects were separated into training and validation sets in a ratio of 7:3 and were modeled by utilizing a nerve net of deep-learning and six machine-learning algorithms. Eight MRI specialists from multiple centers independently determined whether each subject in the validation group had RV abnormalities. Model performance was evaluated based on the AUC, accuracy, recall, sensitivity and specificity. Furthermore, a preliminary assessment of patient disease risk was performed based on clinical information using a nomogram. RESULTS: The deep-learning neural network outperformed the other six machine-learning algorithms, with an AUC value of 1 (95% confidence interval: 1-1) on both training group and validation group. This algorithm surpassed most human experts (87.5%). In addition, the nomogram model could evaluate a population with a disease risk of 0.2-0.8. CONCLUSIONS: A deep-learning algorithm could effectively identify patients with RV abnormalities. This AI algorithm developed specifically for right ventricular abnormalities will improve the detection of right ventricular abnormalities at all levels of care units and facilitate the timely diagnosis and treatment of related diseases. In addition, this study is the first to validate the algorithm's ability to classify RV abnormalities by comparing it with human experts.

Effectiveness and Safety of Mitral Valve Plasty in Patients with an Anomalous Origin of the Coronary Artery from the Pulmonary Artery.

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 ß 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 ß = 9.22, 95%CI 1.09 to 17.35; LVEDD z-score ß = -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.

Fate of distal aorta after frozen elephant trunk and total arch replacement for type A aortic dissection in Marfan syndrome.

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 + 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.

CT angiography for diagnosis and subcategorization of unroofed coronary sinus syndrome.

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.

Long-term outcomes of frozen elephant trunk for type A aortic dissection in patients with 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 FET and 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.

[Atherosclerotic aortic ulcers monitoring by electron beam CT or multi-slice CT].

OBJECTIVE: To evaluate the evolution of medically treated atherosclerotic aortic ulcers by computed tomography (CT). METHODS: Thirty-five patients (31 men and 4 women, aged from 40 to 79 years, mean 56.2 +/- 10.8 years) with known aortic ulcers were monitored by CT (follow up time 7 - 730 days, mean 135 days), 80 - 100 ml contrast media (Ultravist 300 or 320, or Omnipaque 300 or 320 mg/ml) was injected with a rate of 3.5 - 4.5 ml/s. The scan delayed time was 18 - 30 s. Ulcers dimensions were measured according to maximum depth, maximum length and maximum width. RESULTS: Thirty-one patients with intramural hematomas and 1 patient with atherosclerotic aortic arch aneurysm without intramural hematoma were medically treated and another 3 patients were surgically treated. Intramural hematoma regression was monitored in 31 medically treated patients with intramural hematomas. CT was repeated at 2 weeks, 3 and 6 months. Intramural hematoma resolved gradually during follow up [thickness: (7.69 +/- 4.24) mm at 3 months, (3.06 +/- 1.67) mm at 6 months, P < 0.05 vs. 1st CT: (11.96 +/- 4.16) mm while ulcer maximum depth (11.17 +/- 6.03) mm at 3 months, (11.35 +/- 5.59) mm at 6 months, P < 0.05 vs. 1st CT: (7.36 +/- 6.61) mm, maximum width (14.40 +/- 6.35) mm at 3 months, (18.55 +/- 10.94) mm at 6 months, P < 0.05 vs. 1st CT: (7.15 +/- 6.39) mm, maximum length (17.12 +/- 7.15) mm at 3 months, (18.13 +/- 10.89) mm at 6 months, P < 0.05 vs. 1st CT: (11.64 +/- 10.06) mm increased progressively during follow-up]. CONCLUSION: CT was a useful tool for deflecting atherosclerotic aortic ulcers and monitoring therapeutic effects.