Ultrasound Pitfalls in a Complex Fetal Cardiac Malformation—Case Report of a New Arteriovenous Central Communication

Cardiac and cardiovascular malformations are of real interest in terms of definition, epidemiology, and means of early diagnosis by imaging. Although ultrasound examination reaches exceptional performance nowadays, unusual pathologies are still exposed to the risk of either incorrect acquired image or misinterpretation by the specialist in a routine scan. Herein, we present a case of a 20-week-old fetus (from an apparently low-risk pregnancy) with complex cardiac and vascular abnormalities, including an arteriovenous malformation along with ventricular septal defect, ductal coarctation of the aorta, aneurysm of a brachiocephalic vein, and dilation of the entire neck and upper mediastinum venous system, and the limitations that were encountered in the process of diagnosis and management of the case.

Giant Aortic Aneurysm in Child with Cutis Laxa Syndrome: Unusual Presentation, New Surgical Technique

Ascending thoracic aortic aneurysms are rare in childhood and typically are seen in the setting of connective tissue defect syndromes. These aneurysms may lead to rupture, dissection, or valvular insufficiency, so root replacement is recommended. Here, we present a 17-month-old girl who presented with fever, cough, and pericardial effusion. Initially, we suspected this could be a COVID-19 case, so a nasopharyngeal swap was performed. An ascending aorta aneurysm involving the aortic arch was confirmed by echo, and urgent ascending aorta and arch replacement were done by utilizing the descending aorta as a new arch. The final diagnosis came with cutis laxa syndrome. In similar cases, good outcomes can be achieved with accurate diagnosis and appropriate surgical management.

Caution: Air Embolism Related to Heli-Fx EndoAnchor System in Zone 2 Thoracic Aneurysm Repair

Purpose We report a case of significant air embolization to the ascending aorta immediately following deployment of EndoAnchors in the aortic arch during a procedure to correct a type 1A endoleak. Case report The novel Heli-Fx EndoAnchor system (Medtronic Vascular, Santa Rosa, CA, USA) was used to deploy helical anchors in the distal aortic arch during a procedure to correct a type 1A endoleak following Zone 2 thoracic endovascular aortic repair of a saccular proximal descending thoracic aorta aneurysm (DTAA). The patient developed ST-segment elevations principally in the inferior leads and severe hypotension moments after EndoAnchor deployment at the proximal edge of the endograft. Transesophageal echocardiogram revealed severe right ventricular hypokinesis and a large amount of air in the ascending aorta. Subsequent management and clinical and radiological 30-day follow-up is presented in addition to a review of the literature and ex vivo testing with the Heli-Fx system to examine potential causes and solutions. Conclusion Precautions, such as pressurized saline infusion to the side port of guiding sheath, should be used whenever manipulating catheters and sheaths such as the EndoAnchor system in the aortic arch to prevent this potentially lethal complication.

Oxidative Stress Inside the Pathogenesis of Ascending Aorta Aneurysms: A Clearer Vision for Identifying Promising Biomarkers and Therapeutic Targets

Ascending aorta aneurysm (AsAA) is a complex disease, currently defined an inflammatory disease. In the sporadic form, AsAA has, indeed, a complex physiopathology with a strong inflammatory basis, significantly modulated by genetic variants in innate/inflammatory genes, acting as independent risk factors and as largely evidenced in our recent studies performed during the last 10 years. Based on these premises, here, we want to revise the impact of reactive oxygen species (ROS) and oxidative stress on AsAA pathophysiology and consequently on the onset and progression of sporadic AsAA. This might consent to add other important pieces in the intricate puzzle of the pathophysiology of this disease with the translational aim to identify biomarkers and targets to apply in the complex management of AsAA, by facilitating the AsAA diagnosis currently based only on imaging evaluations, and the treatment exclusively founded on surgery approaches.

Chimney Grafts in Acute Endovascular Aortic Repair Due to Ruptured Abdominal Aorta Aneurysm

Parallel grafts, or chimney grafts, are at times a good solutions for EndoVascular Aorta repair (EVAR) in ruptured cases. These photoes shows an acute case with uni-chimney left kidney with succesful outcome.

Custom-made fenestrated stent for mycotic aortic aneurysms: a report of two cases

Background Mycotic aortic aneurysm is a rare and potentially life-threatening lesion, and endovascular repair has become increasingly accepted for intervention. Fenestrated endografts are available options to treat aneurysms involving visceral arteries. Here, we first report two patients with mycotic aortic aneurysm involving paraviscereal aorta who were successfully treated with custom-made fenestrated endograft. Case presentation Two patients were presented with mycotic aortic aneurysm. Due to their comorbidities and the involvement of the renal arteries, company-manufactured fenestrated stents were designed. Meanwhile, antibiotic therapy was administrated for 2 months before endovascular repair. Patients improved well without complications. Conclusions Custom-made fenestrated endovascular stent is an effective and feasible alternative solution to mycotic paravisceral aorta aneurysm.

Coexistence of Rheumatoid Arthritis, Systemic Lupus Erythematosus, Sjogren Syndrome, Antiphospholipid Syndrome, and Ankylosing Spondylitis

A 37-year-old Bangladeshi woman presented with low back and several joints pain and swelling for months together; there was significant morning stiffness for more than two hours. Repeated abortions, dry eye, hair fall, photosensitivity, and oral ulcer were the additional complaints. Clinical examination unveiled asymmetrical peripheral and both sacroiliac joint tenderness, positive modified Schober’s test, and limited chest expansion. Schirmer’s test was positive. The history of rheumatoid arthritis (RA) and ankylosing spondylitis (AS) among 1st-degree relatives was also significant. Biochemical analysis revealed pancytopenia, raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), and mild microscopic proteinuria. The patient was seropositive for rheumatoid factor (RF), antibodies against cyclic citrullinated peptides (anti-CCP), antinuclear antibody (ANA), anti-Sm antibody, anti-Sjögren’s-syndrome-related antigen A and B (anti-SSA/SSB), antiphospholipid (aPL-IgG/IgM), and HLA B27; however, serum complement (C3 and C4) levels were normal. Basal cortisol level measured elevated. Besides, X-ray and MRI of lumbosacral spines demonstrated sacroiliitis. There was radiological cardiomegaly, echocardiography unveiled atrial regurgitation, and ascending aorta aneurysm. Based on the abovementioned information, RA, AS, and systemic lupus erythematosus (SLE) have been diagnosed. Moreover, the patient developed Sjogren’s syndrome (SS), antiphospholipid lipid syndrome (APS), Cushing syndrome, ascending aorta aneurysm, and atrial regurgitation. Her disease activity score for RA (DAS28), DAS for AS (ASDAS), SLE disease activity index (SLEDAI), and Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) scores were 3.46, 2.36, 23, and 5, respectively. The patient received hydroxychloroquine (200 mg daily), pulsed cyclophosphamide, prednisolone (20 mg in the morning), and naproxen 500 mg (twice daily). To our best knowledge, this is the first report documenting RA, AS, and SLE with secondary SS and APS.