Subclavian steal syndrome (SSS) refers to the phenomenon of retrograde flow in an ipsilateral branch of the subclavian artery due to hemodynamically significant stenosis or occlusion of the ipsilateral proximal subclavian artery. While SSS is usually asymptomatic, it can manifest as vertebrobasilar insufficiency (VBI), ischemia of the affected extremity, or cardiac angina when an internal mammary artery (IMA) is used as a bypass graft. The underlying etiology is most often atherosclerosis but can include Takayasu arteritis, thoracic outlet syndrome, cervical rib, and stenosis secondary to surgical repair of aortic coarctation or tetralogy of Fallot. There are several case reports describing unique presentations of SSS as well as limited reports of double SSS, where the brachiocephalic steno-occlusive disease causes flow reversal in both the ipsilateral vertebral and carotid arteries. We report herein the first documented case, to our knowledge, of a patient with SSS previously treated with left subclavian artery stenting and left common carotid-subclavian bypass who developed recurrent SSS in conjunction with orthostatic cerebral hypoperfusion syndrome (OCHOS) secondary to severe vasculopathy. She presented with recurrent, paroxysmal vertigo and near-syncope associated with left upper extremity paresthesias that would only abate with sitting in the context of left subclavian artery stent restenosis and occlusion of her left common carotid-subclavian bypass graft. Interestingly, her initial presentation entailed retrograde flow from the left vertebral artery to the left subclavian artery, classic for SSS, but recurrence of her SSS involved retrograde flow from the left common carotid artery to the left subclavian artery, a phenomenon which has also not been described in the literature to our knowledge. As her symptoms of VBI appeared to be triggered by standing and not left arm movement, they were considered to be primarily secondary to OCHOS. Consequently, her primary treatment was to increase salt and fluid intake and thus increase intravascular volume for improved cerebral perfusion as she was not deemed to be a suitable candidate for regrafting of the left subclavian artery.

The present case report focuses on a rare presentation of aortic coarctation. A 38-year-old man with well-controlled arterial hypertension, minimal change glomerulonephritis and colitis ulcerosa was suffering from recurrent acute renal failure episodes during viral gastroenteritis. No other symptoms at rest or during physical activity were present. The workup included renal duplex sonography, which unmasked tardus parvus profile in both kidneys without any acceleration of blood flow in the renal arteries. Further examination included CT angiography, which confirmed the diagnosis of aortic coarctation. The observed narrowing of the aorta measured 4 mm and was treated with percutaneous transluminal angioplasty and stent implantation (final diameter 12 mm). After the procedure, the patient had normal blood pressure values without the need of any medication; duplex sonography showed improved renal perfusion. The present case confirms the importance of evaluation for secondary hypertension and thorough workup of acute renal failure in young patients.

Chronic heart failure (CHF), a conventional, complex, and severe syndrome, is generally defined by myocardial output inadequate to satisfy the metabolic requirements of body tissues. Recently, miR-568 was identified to be down-regulated in CHF patients' sera and negatively correlated with left ventricular mass index in symptomatic CHF patients with systolic dysfunction. Nevertheless, the role of miR-568 during CHF development remains obscure. The current study is aimed to investigate the role of miR-568 in CHF. The MTT assay, flow cytometry analysis, RT-qPCR analysis, western blot analysis and luciferase reporter assays were conducted to figure out the function and potential mechanism of miR-568 in vitro. Rats were operated with aortic coarctation to establish CHF animal model. The effects of miR-568 and SMURF2 on CHF rats were evaluated by hematoxylin-eosin staining, Masson's staining, serum index testing, cardiac ultrasound detection, and TUNEL staining assays. We discovered that miR-568 level was downregulated by H2O2 treatment in cardiomyocytes. In mechanism, miR-568 directly targeted and negatively regulated SMURF2. In function, SMURF2 overexpression reversed the effects of miR-568 on cardiac function and histological changes in vivo. Additionally, SMURF2 overexpression reversed the effects of miR-568 on the content of LDH, AST, CK and CK-MB in vivo. Moreover, SMURF2 overexpression reversed the effects of miR-568 on oxidative stress response in vivo. MiR-568 mitigated cardiomyocytes apoptosis, oxidative stress response and cardiac dysfunction via targeting SMURF2 in CHF rats. This discovery may serve as a potential biomarker for CHF treatment.

We present a case of an adult with concurrent severe aortic coarctation, bilateral carotid artery stenosis, and anomalous right subclavian artery, posing the interventional dilemma of accepting potential cerebral hyperperfusion syndrome vs hypoperfusion ischemic injury. Transcatheter stenting of the aortic coarctation was successfully performed without any neurological deficits. (Level of Difficulty: Beginner.).

Survival of patients after repair of coarctation of Aorta (CoA) has improved significantly over the decades, but patients have decreased life expectancy as compared to the general population. This has been attributed to increased hypertension, cerebrovascular disease, and coronary artery disease. There has also been an increasing concern of overweight and obesity in patients with adult congenital heart disease. While there have been studies looking at the impact of long-term hypertension on myocardial performance and outcomes in this population, this study aims to assess the impact of obesity in these patients on their myocardial performance. Ventriculo-arterial coupling is used as a measure of myocardial performance which reflects the interaction between cardiac contractility and arterial elastance. Patients after CoA repair are known to have hypertension affecting the arterial elastance. Obesity affects cardiac contractility as well. This study demonstrated that in a group of young patients after CoA repair, body mass index (BMI) has a relationship with left ventricular (LV) contractility and myocardial performance. This relationship was independent of blood pressure. BMI itself was not seen to affect the determinants of diastolic function in this study, suggesting that LV contractility may be affected before one can notice a change in the diastolic function secondary to BMI.

This is the first report of total arch replacement to repair re-coarctation. A 14-year-old boy with hypoplastic left heart syndrome developed re-coarctation, severe stenosis of neck vessels, and right ventricle dysfunction after a Norwood procedure. We performed total arch replacement; the postoperative course was unremarkable. He was followed up until 18 years of age and did not need re-intervention. Using artificial blood vessels in total arch replacement is rarely indicated but can be safely achieved when required. Mismatch between patient and graft size may be an issue in the future.

Evidence on the effectiveness and comparative effectiveness of stent implantation and balloon angioplasty for native coarctation of the aorta (CoA) and recurrent CoA separately is lacking. The present meta-analysis was performed to assess the efficacy and safety of stent implantation and balloon angioplasty in native (NaCo) and recurrent (ReCo) CoA.A systematic computerized literature search was conducted to retrieve all relevant studies of stent implantation and balloon angioplasty for CoA. Both single-arm and comparative studies were included. Data on NaCo and ReCo were pooled separately.A post-procedure gradient of ≤ 20 mmHg was achieved in 97% and 92% of patients undergoing stent implantation and balloon angioplasty for NaCo, and in 98% and 90% for ReCo, respectively. A post-procedure gradient of ≤ 10 mmHg was achieved in 97% and 83% of patients undergoing stent implantation and balloon angioplasty for NaCo, and in 86% and 78% for ReCo, respectively. Comparative results confirmed that stent implantation provided a significantly higher success rate compared with balloon angioplasty (odds ratio [OR] = 2.09; 95% confidence interval [CI] = 1.13-3.86; P = 0.019) in treating NaCo. Incidences of all-cause complications, mortality, reintervention, and aneurysm formation were similar between the groups. Patients undergoing stent implantation had a significantly lower incidence of dissection (OR = 0.15; 95% CI = 0.02-0.92; P = 0.040).Current evidence indicates that stent implantation might have superior efficacy compared with balloon angioplasty for the treatment of NaCo with higher success rates and similar complication rates. However, whether this superior effect is also present in ReCo patients needs further evaluation.

Moyamoya disease is a cerebrovascular disorder which causes a decrease in the cerebral blood flow rate. In this study, a lumped parameter model describing the pressures and flow rates in the heart chambers, circulatory system, and cerebral circulation with the main arteries in the circle of Willis, pial circulation, cerebral capillaries, and veins was used to simulate Moyamoya disease with and without coarctation of the aorta in adults and children. Cerebral blood flow rates were 724 mL/min and 1072 mL/min in the healthy adult and child cardiovascular system models. The cerebral blood flow rates in the adult and child cardiovascular system models simulating Moyamoya disease were 676 mL/min and 1007 mL/min in stage 1, 627 mL/min and 892 mL/min in stage 2, 571 mL/min and 831 in stage 3, and 444 and 537 mL/min in stage 4. The cerebral blood flow rates were 926 mL/min and 1421 mL/min in the adult and child cardiovascular system models simulating coarctation of the aorta. Furthermore, the cerebral blood flow rates in the adult and child cardiovascular system model simulating Moyamoya disease with coarctation of the aorta were 867 mL/min and 1341 mL/min in stage 1, 806 mL/min and 1197 mL/min in stage 2, 735 mL/min and 1121 in stage 3, and 576 and 741 mL/min in stage 4. The numerical model utilised in this study can simulate the advancing stages of Moyamoya disease and evaluate the associated risks with Moyamoya disease.

Coarctation of the aorta is a rare congenital abnormality, with an incidence of 6-8% of all congenital heart problems. It is usually diagnosed in childhood during routine clinical examination and adults mostly present with hypertension. Various investigations like transthoracic echocardiography, computed tomography, and magnetic resonance angiography can help with diagnosis. Prognosis depends on age at presentation and the severity of coarctation. Treatment options available are open and endovascular repair. Extra-anatomical bypass is the preferred option in cases with unfavorable anatomy. Long term follow up is required post repair due to risk of restenosis and aneurysm formation. Here is a case in which a young female presented with hypertension, was diagnosed with coarctation of the aorta, and was treated a left subclavian artery to descending thoracic aorta bypass. Her postoperative course was uneventful and she had improvement in hypertension.

Objective: To explore the current situation of fetal heart defects in Yunnan Province and surrounding high altitude areas and the social factors affecting pregnancy outcome. Methods: This is a retrospective study. Pregnant woman who underwent fetal echocardiography and diagnosed as fetal cardiac defects in Yunnan Fuwai Cardiovascular Hospital from June 2017 to January 2021 were included. According to the clinical prognostic risk scoring system and grading criteria of fetal cardiac birth defects, the cases were divided into grade Ⅰ to Ⅳ. The disease distribution and proportion of each prognostic grade, pregnancy outcomes were analyzed and compared. The cases were divided into continued pregnancy group and terminated pregnancy group according to pregnancy outcome. The social factors that may affect the selection of pregnancy outcomes were analyzed by multivariate logistic regression analysis. Results: A total of 4 929 fetal echocardiography examination data were collected, and 4 464 cases (90.57%) were from Yunnan Province and surrounding high altitude areas. 2 166 cases of heart defects were finally analyzed, including 998 cases of congenital heart disease (CHD), 93 cases of cardiac tumors, cardiomyopathy and arrhythmia, 1 075 cases of foramen ovale, ductus arteriosus abnormalities and normal variations. The pregnant women were (29.2±5.0) years old with (25.6±3.8) gestational weeks. The number of cases with prognostic grade from Ⅰ to Ⅳ was 1 037 (47.88%), 620 (28.62%), 314 (14.50%), and 44 (2.03%), respectively. And 151 cases (6.97%) were not classified. The cases of normal variation and thin aortic arch development accounted for 42.66% (924/2 166), 5.22% (113/2 166), respectively. The top 3 diseases of grade Ⅱ were ventricular septal defect, coarctation of aorta and mild-moderate pulmonary stenosis, respectively, and their distribution was 11.63% (252/2 166), 3.92% (85/2 166) and 2.35% (51/2 166) respectively in all cases of heart defects, and 25.25% (252/998), 8.52% (85/998) and 5.11% (51/998) respectively in cases of CHD. Among the cases rated as grade Ⅲ and Ⅳ, most of them were complicated congenital heart disease, and the disease types are scattered. The more common cases in grade Ⅲ were complete transposition of great arteries (accounting for 2.40% (52/2 166) of all cases with heart defects, 5.21% (52/998) of all cases with CHD) and pulmonary artery occlusion (type Ⅰ to Ⅲ) with ventricular septal defect (accounting for 2.17% (47/2 166) of all cases with heart defects, and 4.71% (47/998) of all cases with CHD). In grade Ⅳ, single ventricle (0.74% (16/2 166) of all cases with heart defects, 1.60% (16/998) of all cases with CHD) and left ventricular dysplasia syndrome (0.65% (14/2 166) of all cases with heart defects, 1.40% (14/998) of all cases with CHD) are more common. A total of 1 084 cases were successfully followed up, and 675 cases were born, 392 cases were terminated, spontaneous abortion occurred in 17 cases. The proportion of terminated pregnancy cases was significantly increased from grade Ⅰ to Ⅳ, accounting for 5.24% (21/401), 27.78% (70/252), 89.54% (214/239) and 95.56% (43/45), respectively. Among the terminated pregnancy cases, those with grade Ⅲ accounted for the highest proportion (54.59% (214/392)). The distribution of terminated pregnancy cases was mainly complex congenital malformations or diseases with very poor prognosis (pregnancy outcome grade Ⅲ and Ⅳ), and proportion of terminated pregnancy with pregnancy outcome grade Ⅰ and Ⅱ cases (normal variation or good prognosis) accounted for 5.36% (21/392) and 17.86% (70/392), respectively. The results of multivariate logistic regression analysis showed that pregnant women with low education (high school and below: OR=2.73, 95%CI 1.26-5.93, P<0.001; illiteracy: OR=3.27, 95%CI 1.29-7.10, P<0.001) and low family income (Annual income<100 000 yuan: OR=2.47, 95%CI 1.69-5.12, P<0.001) were more likely to choose termination of pregnancy in case of fetal heart defect. Conclusion: In Yunnan province and the surrounding high altitude areas, the disease distribution of fetal heart defect is mainly simple or low-risk disease, but the complex malformation, especially the disease with poor pregnancy outcome, accounts for a relative high proportion. Pregnancy termination also occurs in some cases with good pregnancy outcome. The education level and family income of pregnant women may affect their choice of pregnancy outcome in case of fetal heart defect.

Background Abnormal aortic elastic properties are major notable vasculopathy involved in coarctation of the aorta (CoA). However, there are no reports on aortic wall elastic characteristics in fetuses with CoA. Methods and Results Fifty-six fetuses with CoA and 56 normal controls were included in this prospective case-control study. The dimensions of the cardiac chamber, the size of the aorta, left ventricular myocardial performance indexes, and aortic elastic properties, including the global circumferential strain, fractional area change and mean longitudinal strain, were measured serially in utero. The global circumferential strain, fractional area change, and mean longitudinal strain in fetuses with CoA were smaller than those in the normal group at both the first and last examinations (18.50% versus 37.73% for global circumferential strain, 38.90% versus 57.55% for fractional area change, 6.61% versus 11.81% for mean longitudinal strain at first scan, 16.62% versus 42.05% for global circumferential strain, 36.54% versus 59.7% for fractional area change, 6.2% versus 11.46% for mean longitudinal strain at last scan, all P<0.001). There were negative correlations between aortic elastic properties and left ventricular myocardial performance indexes in fetuses with CoA (P<0.01). Aortic elastic properties were correlated positively with aortic isthmus size in fetuses with CoA (P<0.01). Conclusions Aortic strain and the fractional area change were decreased in fetuses with CoA. Impairments of these aortic elastic properties were associated with diminished heart function and aortic isthmus size in utero. Further large-scale longitudinal studies are required to confirm the potential predictive value of cardiovascular morbidity (ie, hypertension) in fetuses with CoA.

Congenital abdominal aortic aneurysm (AAA) with coarctation has been considered an extremely rare condition. In this study, we present a 3-year-old boy, who was diagnosed by chance with congenital AAA at first operation. We replaced the AAA+coarctation with a 6-mm polytetrafluoroethylene (PTFE) graft. Histological examination of the aortic wall revealed no particular abnormalities. Collateral vessels were noted to develop over 14 years of followup. Good blood flow to both lower limbs and no intermittent claudication were observed. After growth, at the age 17, he underwent extra-anatomical bypass using a 12-mm PTFE graft. This is the first report of successful treatment of congenital AAA+coarctation with longterm followup.

Coarctation of the aorta (CoA) is a congenital heart defect that is associated with a bicuspid aortic valve (BAV), ascending aorta dilatation, intracerebral aneurysms, and premature atherosclerotic disease. The first presentation during late adulthood is rare and is frequently driven by late sequelae. Hypertrophic collateral arteries can develop aneurysms which are at risk for spontaneous rupture, however, treatment recommendations for these aneurysms are scarce. Here, we describe the clinical course and percutaneous treatment strategy of a patient with a late diagnosis of a pin-point CoA, a BAV with moderate regurgitation, and an exceptionally large aneurysm of a collateral artery. A 59-year-old woman was diagnosed with Streptococcus bovis endocarditis of a BAV with moderate aortic valve regurgitation and small vegetation (<5 mm) on the non-coronary cusp. Work-up revealed hypertension and adenocarcinoma in situ of the ascending colon, considered the bacteremia porte d'entrée, for which a curative hemicolectomy was performed. Echocardiography showed a narrowing of the aorta distal from the origin of the left subclavian artery with the antegrade diastolic flow with a pathognomonic "sawtooth" pattern and an estimated pressure gradient of >70 mmHg. Computed tomography angiography (CTA) showed a network of well-developed collateral arteries and a levoatriocardinal vein. One of the collateral arteries arising from the left subclavian artery revealed an exceptionally large aneurysmatic dilation (29 × 24 × 24 mm). The invasive assessment confirmed a hemodynamically significant CoA. Treatment involved balloon dilatation and placement of a covered stent at the site of the pin-point CoA and a percutaneous coronary intervention (PCI) of the stenosis in the left anterior descending artery. No residual gradient over the CoA was observed. Antihypertensive drugs could be discontinued, and CTA performed 4 months later showed regression and thrombosis of the numerous collaterals and, importantly, thrombosis of the large aneurysm. This case illustrates the late diagnosis of CoA with associated congenital heart defects and late sequelae including hypertension, BAV endocarditis, coronary artery disease, and aneurysm formation of the extensive collateral network. The patient underwent pharmacological and percutaneous treatment, ultimately resulting in the alleviation of the CoA, normalization of the blood pressure, reduction of collateral flow, and thrombosis of the large aneurysm of the collateral artery.

Contrast pooling (CP) reconstruction is widely used in computed tomography (CT) studies of congenital heart diseases. However, endovascular devices are usually obscured in CP. To improve visualization of the vascular lumen, we developed jellyfish angiography (JFA), a semitransparent blood pool inversion technique. Ten CT studies of patent ductus arteriosus (PDA) or coarctation of the aorta (CoA) were selected retrospectively for reconstruction using both CP and JFA. Four of the studies were conducted before the endovascular intervention, and six were conducted after the intervention. Radiology residents and pediatric cardiologists completed questionnaires regarding the reconstruction models. For radiology residents, JFA was superior to CP in postintervention PDA diagnosis, device evaluation, and overall satisfaction. For pediatric cardiologists, JFA outperformed CP in both PDA and CoA postintervention cases. Our findings show that JFA overcomes the disadvantages of CP and can improve the visualization of intraluminal devices which is essential for endovascular treatment evaluation.

The carotid artery is a valuable vascular access that can be used in patients who have undergone repetitive interventional and surgical procedures and premature babies. In the past, cut-down was used but nowadays, mostly the procedure is performed under ultrasonographic guidance. Complications such as bleeding, haematoma, and pseudoaneurysm may occur when the carotid artery is used as a vascular access for the procedures such as aortic balloon valvuloplasty, coarctation balloon angioplasty, or after interventional or surgical treatments to the carotid artery. Although pseudoaneurysm is very rare, prompt diagnosis and accurate treatment planning are life-saving. In this article, the diagnosis and treatment of pseudoaneurysm in the left common carotid after transcatheter coarctation balloon angioplasty in a 6-month-old infant will be presented.

Although balloon-expandable stent implantation for native aortic coarctation is a preferred method in the adolescent age group, there are insufficient data about indications for and the efficacy of the procedure in a younger age group. The aim of this study was to compare and evaluate the data of young pediatric and adolescent patients who underwent balloon-expandable stent implantation because of native aortic coarctation. The retrospective analysis included the demographic characteristics and data related to the procedure and follow-up of patients who underwent stent implantation for native aortic coarctation between August 2010 and November 2017. Patients with re-coarctation were excluded from the study. The patients were separated into 2 groups as the adolescent group (Group I: 10-18 years) and the pediatric group (Group II: ≤ 9.9 years). Group-I comprised of 18 patients and Group-II, 32 patients. Covered stent was implanted to 32 (73%) patients and uncovered stent to 12 (27%) patients. The procedural success rate was 100%. Following stent implantation, peak systolic gradient decreased significantly in both groups (P < .0001) (Group-I: from 35.9 ± 16.6 mm Hg-2.2 ± 3.4 mm Hg, Group II: from 34 ± 13.3 mm Hg-3 ± 4.09 mm Hg). Complications developed in 3 patients, and all in Group I. Femoral hematoma developed in 1 patient, balloon rupture occurred during the procedure in 1 patient, and there was temporary loss of pulse in 1 patient. All the complications were treated successfully. All the patients were taking anti-hypertensive drugs before intervention and during the mean 23-month follow-up period (range, 2-84 months), hypertension recovered in 35 (80%) patients and drugs were terminated. Stent implantation for aortic coarctation in the pediatric age group may provide pleasing results, reducing the coarctation gradient, providing effective dilatation in the lesion area and eliminating hypertension.

Left ventricular diverticulum is a rare cardiac abnormality characterised by focal protrusion of the ventricular wall containing all the three layers with synchronous contractility. They have been described more commonly in paediatric patients and may be seen associated with congenital midline thoracoabdominal abnormalities. There are few reports of left ventricular diverticulum in cases of patent ductus arteriosus and coarctation with subaortic diverticula in the bicuspid aortic valve. Here, we report a case of left ventricular diverticulum in association with bicuspid aortic valve and pseudocoarctation in a 53-year-old female patient with a pertinent review of the literature.