https://onlinelibrary.wiley.com/doi/pdfdirect/10.1002/joa3.12815

Efficacy and safety of zero‐fluoroscopy ablation of ventricular arrhythmias originating from the right ventricular outflow tract: Comparison with fluoroscopy‐guided ablation without a three‐dimensional electroanatomic mapping system

Background Midaortic syndrome (MAS) is a rare medical condition characterized by thoracoabdominal aortic stenosis or occlusion, resulting in decreased blood supply to the organs and tissues in the lower half of the body. We report a very rare presentation of MAS in a 14-year-old female that was found to have severe stenosis extending from the descending aorta to the level above the bifurcation. Case Presentation A 14-year-old female with Williams syndrome presented with complaints of headache and chest discomfort on mild exertion. Examination revealed dysmorphic features of William syndrome and different blood pressure between the upper and lower limbs with weak femoral pulses. An ECG showed sinus rhythm with left ventricular hypertrophy. 2-D color flow Doppler echocardiogram revealed mild central mitral regurgitation, minimal aortic regurgitation, and concentric left ventricular hypertrophy. CT angiogram revealed a hypoplastic descending thoracic aorta, an aberrant right subclavian artery, and an arterio-venous fistula communicating between the bronchial artery and the left brachiocephalic vein. The patient was started on two antihypertensive medications, and vascular surgery was consulted for possible future intervention. Conclusion Management of midaortic syndrome necessitates a holistic approach customized to the specific patient's requirements and risk assessment. Early detection and intervention are critical in averting severe complications and enhancing long-term prognoses. Continuous investigation and advancement of novel therapies may present supplementary possibilities for addressing this complex and rare ailment.

Long Segment Midaortic Stenosis in Williams Syndrome: Report of a Very Rare Presentation

Background: Radio Frequency Catheter Ablation (RFCA) for Right Ventricular Outflow Tract Ventricular Arrhythmias (RVOT VAs) without fluoroscopy is increasingly performed but usually uses steerable catheter and intracardiac echocardiography, all of which may be too expensive or unavailable in many places. We introduced a novel approach which uses a non-steerable decapolar catheter to map the RVOT first without the assistance of echocardiography. Methods and findings: A single center, prospective study in patients undergoing catheter ablation for RVOT VAs from May 2020 to November 2021. The approach includes first using a non-steerable decapolar catheter to fluorelessly map the RVOT via the superior vena cava, then the ablation catheter is advanced to the RVOT via the femoral vein followed the 3D-road map built by quadripolar catheter. A total of 43 consecutive patients with electrocardiographic features of RVOT VAs were enrolled. Mean age was 53.5 (23-83 years old), female/male rate was 3/1. Most of the patients presented with only PVC (79%), while 09 patients (20.9%) with non-sustained VTs, and only 01 patient with sustained VT. In 38 patients (88.4%) with total-zero fluoroscopy, acute success rate was 100% without any significant complication. In the rest 5 cases who required minimal fluoroscopy, acute success rate was 80%, the procedure failed in 1 patient due to the epicardial site of the VA foci. After a mean follow-up time of 4.8 ± 5.1 months, the long-term success rates of fluoroless ablation and combined ablation were 89.5% and 75%, respectively. Overall, the acute success rate reached 97.7%, the long-term success rate was 86.0%, and the recurrence rate was 11.6%. Conclusion: Performing fluoroless RVOT approach using further non-steerable decapolar diagnostic catheter via SVC without echocardiography is feasible and safe. The non-steerable decapolar catheter could be used for activation mapping of RVOT VAs as an initial strategy of procedure.

Zero-fluoroscopy catheter ablation for right ventricular outflow tract ventricular arrhythmias with a novel initial approach

Abstract Background Mid-aortic syndrome (MAS) is characterized by the congenital coarctation of the abdominal aorta, abdominal and limb claudication, and hypertension. The etiology of this disorder is very diverse and often manifests in conjunction with Takayasu's arteritis, Williams-Beurens syndrome, and neurofibromatosis. The isolated mid-aortic syndrome is very rare with only a few cases reported in the literature. Case presentation A 45 years old man was admitted to the Emergency Department with sudden muscle weakness and facial paralysis on the left side. Imaging studies reveal right middle cerebral artery infarction at the M1 section. Incidental findings include multiple moderate to severe stenoses in the right internal carotid artery, and total abdominal aorta occlusion. A variant at the ELN gene ( Elastin , OMIM*130,160): c.1768G &gt; A/wt (p.Ala590Thr) was identified. Conclusion This is the first reported case of ELN related mid-aortic syndrome in Vietnam which was diagnosed through careful clinical and genetic workup. The finding of mid-aortic syndrome, in this case, was incidental and the decision to reverse the occlusion was postponed as there was no immediate risk of renal failure or reduced blood flow to the lower limb. 

/pdf/eln-missense-variant-in-patient-with-mid-aortic-syndrome-23d79wt9.pdf

ELN missense variant in patient with mid-aortic syndrome case report

Results of hybrid surgery for treatment of thoracic aortic aneurysm

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Zero-fluoroscopy catheter ablation for right ventricular outflow tract ventricular arrhythmias with a novel initial approach

ELN missense variant in patient with mid-aortic syndrome case report

Results of hybrid surgery for treatment of thoracic aortic aneurysm