A double nellix and chimney covered stents: challenging treatment of pararenal aortic aneurysm

A 77-year-old male patient presented with a symptomatic, 66-mm pararenal aortic aneurysm. The patient was classified as unsuitable for open surgery due to significant comorbidities. Fenestrated or branched endografts were contraindicated due to the poor iliac access (6 mm diameter). A double Nellix with chimney endovascular aneurysm sealing (ChEVAS) technique was selected to exclude the pararenal aortic aneurysm and to preserve renal arteries and the superior mesenteric artery. Technical preplanning considered the ideal proximal landing zone to be close to the origin of the almost occluded celiac trunk and the distal common iliac arteries as the ideal distal landing zone. The total length of the aorta to cover was estimated as >180 mm, requiring 2 aortic EVAS systems, bilaterally overlapped. Technical success was achieved, and the patient was discharged on postoperative day 8 in good general condition. Successful aneurysm exclusion and target vessel patency without endoleak or stent-graft kinking or migration were confirmed at angio-computed tomography at 6 months

Rupture of Splenic Artery Aneurysm in Patient with ACTN2 Mutation

Here, we report a case of splenic artery aneurysm rupture in a patient with known heterozygosity mutation of the ACTN2 gene (variant c.971G > A p.Arg324Gln). The patient came to our emergency department with epigastric pain radiating to the lumbar area, with an absence of peritonism signs. An abdominal computed tomography angiography showed a ruptured huge (5 cm) splenic artery aneurysm. Therefore, the patient underwent emergency endovascular coil embolization with complete aneurysm exclusion. The postoperative course was uneventful, until postoperative day five when the patient developed a symptomatic supraventricular tachycardia in the absence of echocardiographic alterations. The signs and symptoms disappeared after three days of medical management. The patient was discharged on the 14th postoperative day in good clinical condition under verapamil and anti-platelet therapy. Although ACTN2 mutation was associated with cardiac and peripheral vascular disease occurrence, to the best of our knowledge, the present case is the first report of a visceral (splenic) aneurysm directly linked with this rare mutation

DOPPLER ULTRASONOGRAPHY AND EXERCISE TESTING IN DIAGNOSING A POPLITEAL ARTERY ADVENTIRIAL CYST

We describe popliteal arterial adventitial cystic disease which causes intermittent claudication in a young athletic man, with atypical manifestation, without loss of foot pulses on knee flexion nor murmur in the popliteal fossa. The findings obtained from Magnetic Resonance Imaging were non-diagnostic. The diagnosis resulted from Echo-Doppler ultrasonography along with peak exercise testing. Ultrasonography also provided useful physiopathological informations suggesting that a popliteal artery adventitial cyst can become symptomatic if muscle exertion increases fluid pressure within the cyst, enough to cause hemodynamically significant endoluminal stenosis. Rapid diagnosis is essential to prevent progressive claudication threatening limb viability. To guarantee this professional sportsman a reliable and durable outcome, instead of less aggressive management, we resected the involved arterial segment and interposed an autologous saphenous-vein graft

Spectrum of mutations in Italian patients with familial hypercholesterolemia: New results from the LIPIGEN study

Background Familial hypercholesterolemia (FH) is an autosomal dominant disease characterized by elevated plasma levels of LDL-cholesterol that confers an increased risk of premature atherosclerotic cardiovascular disease. Early identification and treatment of FH patients can improve prognosis and reduce the burden of cardiovascular mortality. Aim of this study was to perform the mutational analysis of FH patients identified through a collaboration of 20 Lipid Clinics in Italy (LIPIGEN Study). Methods We recruited 1592 individuals with a clinical diagnosis of definite or probable FH according to the Dutch Lipid Clinic Network criteria. We performed a parallel sequencing of the major candidate genes for monogenic hypercholesterolemia (LDLR, APOB, PCSK9, APOE, LDLRAP1, STAP1). Results A total of 213 variants were detected in 1076 subjects. About 90% of them had a pathogenic or likely pathogenic variants. More than 94% of patients carried pathogenic variants in LDLR gene, 27 of which were novel. Pathogenic variants in APOB and PCSK9 were exceedingly rare. We found 4 true homozygotes and 5 putative compound heterozygotes for pathogenic variants in LDLR gene, as well as 5 double heterozygotes for LDLR/APOB pathogenic variants. Two patients were homozygous for pathogenic variants in LDLRAP1 gene resulting in autosomal recessive hypercholesterolemia. One patient was found to be heterozygous for the ApoE variant p.(Leu167del), known to confer an FH phenotype. Conclusions This study shows the molecular characteristics of the FH patients identified in Italy over the last two years. Full phenotypic characterization of these patients and cascade screening of family members is now in progress

Insight from an Italian Delphi Consensus on EVAR feasibility outside the instruction for use: the SAFE EVAR Study

BACKGROUND: The SAfety and FEasibility of standard EVAR outside the instruction for use (SAFE-EVAR) Study was designed to define the attitude of Italian vascular surgeons towards the use of standard endovascular repair (EVAR) for infrarenal abdominal aortic aneurysm (AAA) outside the instruction for use (IFU) through a Delphi consensus endorsed by the Italian Society of Vascular and Endovascular Surgery (Societa Italiana di Chirurgia Vascolare ed Endovascolare - SICVE). METHODS: A questionnaire consisting of 26 statements was developed, validated by an 18 -member Advisory Board, and then sent to 600 Italian vascular surgeons. The Delphi process was structured in three subsequent rounds which took place between April and June 2023. In the first two rounds, respondents could indicate one of the following five degrees of agreement: 1) strongly agree; 2) partially agree; 3) neither agree nor disagree; 4) partially disagree; 5) strongly disagree; while in the third round only three different choices were proposed: 1) agree; 2) neither agree nor disagree; 3) disagree. We considered the consensus reached when >70% of respondents agreed on one of the options. After the conclusion of each round, a report describing the percentage distribution of the answers was sent to all the participants. RESULTS: Two -hundred -forty-four (40.6%) Italian Vascular Surgeons agreed to participate the first round of the Delphi Consensus; the second and the third rounds of the Delphi collected 230 responders (94.3% of the first -round responders). Four statements (15.4%) reached a consensus in the first rounds. Among the 22 remaining statements, one more consensus (3.8%) was achieved in the second round. Finally, seven more statements (26.9%) reached a consensus in the simplified last round. Globally, a consensus was reached for almost half of the proposed statements (46.1%). CONCLUSIONS: The relatively low consensus rate obtained in this Delphi seems to confirm the discrepancy between Guideline recommendations and daily clinical practice. The data collected could represent the source for a possible guidelines' revision and the proposal of specific Good Practice Points in all those aspects with only little evidence available

Familial hypercholesterolemia: The Italian Atherosclerosis Society Network (LIPIGEN)

Background and aims Primary dyslipidemias are a heterogeneous group of disorders characterized by abnormal levels of circulating lipoproteins. Among them, familial hypercholesterolemia is the most common lipid disorder that predisposes for premature cardiovascular disease. We set up an Italian nationwide network aimed at facilitating the clinical and genetic diagnosis of genetic dyslipidemias named LIPIGEN (LIpid TransPort Disorders Italian GEnetic Network). Methods Observational, multicenter, retrospective and prospective study involving about 40 Italian clinical centers. Genetic testing of the appropriate candidate genes at one of six molecular diagnostic laboratories serving as nationwide DNA diagnostic centers. Results and conclusions From 2012 to October 2016, available biochemical and clinical information of 3480 subjects with familial hypercholesterolemia identified according to the Dutch Lipid Clinic Network (DLCN) score were included in the database and genetic analysis was performed in 97.8% of subjects, with a mutation detection rate of 92.0% in patients with DLCN score \ue2\u89\ua56. The establishment of the LIPIGEN network will have important effects on clinical management and it will improve the overall identification and treatment of primary dyslipidemias in Italy

Familial hypercholesterolemia: The Italian Atherosclerosis Society Network (LIPIGEN)

BACKGROUND AND AIMS: Primary dyslipidemias are a heterogeneous group of disorders characterized by abnormal levels of circulating lipoproteins. Among them, familial hypercholesterolemia is the most common lipid disorder that predisposes for premature cardiovascular disease. We set up an Italian nationwide network aimed at facilitating the clinical and genetic diagnosis of genetic dyslipidemias named LIPIGEN (LIpid TransPort Disorders Italian GEnetic Network). METHODS: Observational, multicenter, retrospective and prospective study involving about 40 Italian clinical centers. Genetic testing of the appropriate candidate genes at one of six molecular diagnostic laboratories serving as nationwide DNA diagnostic centers. RESULTS AND CONCLUSIONS: From 2012 to October 2016, available biochemical and clinical information of 3480 subjects with familial hypercholesterolemia identified according to the Dutch Lipid Clinic Network (DLCN) score were included in the database and genetic analysis was performed in 97.8% of subjects, with a mutation detection rate of 92.0% in patients with DLCN score 656. The establishment of the LIPIGEN network will have important effects on clinical management and it will improve the overall identification and treatment of primary dyslipidemias in Italy

Hybrid treatment of anastomotic pseudoaneurysm of the isthmus portion of the thoracic aorta

[No abstract available

Stent-assisted coil embolization of a complex wide-neck splenic artery aneurysm

We describe the endovascular treatment of a wide-neck splenic artery aneurysm (SAA) using a stent-assisted coil embolization technique. A 55-year-old woman who was admitted for intermittent epigastric pain was diagnosed with a wide-neck aneurysm of the intermediate splenic artery. The SAA had a maximum diameter of 2.2 cm and originated from a tortuous vessel. After percutaneous access through the left brachial artery, a self-expandable stent was initially deployed across the origin of the aneurysm using a 4-French platform. The aneurysm sac was subsequently filled with coils through a microcatheter. The procedure was successful with no postoperative clinical complications. Patency of splenic artery and complete exclusion of the aneurysm were confirmed by follow-up computed tomographic angiography 1 year after treatment. In challenging anatomic situations, stent-assisted coil embolization may represent a first-choice endovascular treatment option for the exclusion of SAAs