Glue ablation of Rasmussen's aneurysm in a case of epituberculosis.

A woman in her 40s presented with massive haemoptysis and breathlessness for 1 day. She had been diagnosed with pulmonary tuberculosis based on sputum CBNAAT (Cartridge Based Nucleic Acid Amplification Test) and was on antitubercular treatment for previous 2 weeks. Her chest X-ray showed right middle lobe lateral segment dense consolidation with bilateral nodular infiltrates. CT pulmonary angiography (CTPA) revealed a well-defined homogenously enhancing vascular lesion of size 10×11×13 mm in the right hilar region communicating with the descending branch of right pulmonary artery, suggesting a Rasmussen's aneurysm. It was in close proximity to the segmental bronchus that was almost completely occluded, suggesting epituberculosis. Transvenous pulmonary artery glue embolisation successfully achieved complete ablation of the aneurysm with preserved arterial flow. She has later completed 6 months of antitubercular treatment and is cured with no recurrence of haemoptysis. Her lung infiltrates have resolved with some lung scarring.

Subgroups of children with Kawasaki disease: a data-driven cluster analysis.

BACKGROUND: Although Kawasaki disease is commonly regarded as a single disease entity, variability in clinical manifestations and disease outcome has been recognised. We aimed to use a data-driven approach to identify clinical subgroups. METHODS: We analysed clinical data from patients with Kawasaki disease diagnosed at Rady Children's Hospital (San Diego, CA, USA) between Jan 1, 2002, and June 30, 2022. Patients were grouped by hierarchical clustering on principal components with k-means parcellation based on 14 variables, including age at onset, ten laboratory test results, day of illness at the first intravenous immunoglobulin infusion, and normalised echocardiographic measures of coronary artery diameters at diagnosis. We also analysed the seasonality and Kawasaki disease incidence from 2002 to 2019 by subgroup. To explore the biological underpinnings of identified subgroups, we did differential abundance analysis on proteomic data of 6481 proteins from 32 patients with Kawasaki disease and 24 healthy children, using linear regression models that controlled for age and sex. FINDINGS: Among 1016 patients with complete data in the final analysis, four subgroups were identified with distinct clinical features: (1) hepatobiliary involvement with elevated alanine transaminase, gamma-glutamyl transferase, and total bilirubin levels, lowest coronary artery aneurysm but highest intravenous immunoglobulin resistance rates (n=157); (2) highest band neutrophil count and Kawasaki disease shock rate (n=231); (3) cervical lymphadenopathy with high markers of inflammation (erythrocyte sedimentation rate, C-reactive protein, white blood cell, and platelet counts) and lowest age-adjusted haemoglobin Z scores (n=315); and (4) young age at onset with highest coronary artery aneurysm but lowest intravenous immunoglobulin resistance rates (n=313). The subgroups had distinct seasonal and incidence trajectories. In addition, the subgroups shared 211 differential abundance proteins while many proteins were unique to a subgroup. INTERPRETATION: Our data-driven analysis provides insight into the heterogeneity of Kawasaki disease, and supports the existence of distinct subgroups with important implications for clinical management and research design and interpretation. FUNDING: US National Institutes of Health and the Irving and Francine Suknow Foundation.

Multi-centre, randomised, open-label, blinded endpoint assessed, trial of corticosteroids plus intravenous immunoglobulin (IVIG) and aspirin, versus IVIG and aspirin for prevention of coronary artery aneurysms (CAA) in Kawasaki disease (KD): the KD CAA prevention (KD-CAAP) trial protocol.

BACKGROUND: Kawasaki disease (KD) is an acute self-limiting inflammatory vasculitis affecting predominantly medium-sized arteries, particularly the coronary arteries. A number of recent studies conducted in different European countries have demonstrated alarmingly high coronary complications despite treatment with intravenous immunoglobulin (IVIG). These high complication rates now emphasize the need for an urgent reappraisal of IVIG as the sole primary therapeutic agent for KD. The Kawasaki disease CAA prevention (KD-CAAP) trial will test the hypothesis that immediate adjunctive corticosteroid treatment to standard of care IVIG and aspirin will reduce coronary artery aneurysm (CAA) rates in unselected KD patients across Europe. METHODS: KD-CAAP is a multicentre, randomised, controlled, open-label, blinded endpoint assessed trial that will be conducted across Europe supported by the conect4children pan-European clinical trials network. Patients with KD who satisfy the eligibility criteria will be randomised (1:1) to receive either oral prednisolone 2 mg/kg/day plus standard of care therapy IVIG (2 g/kg) and aspirin (40 mg/kg/day); or IVIG and aspirin alone. Further management is dictated by temperature and C-reactive protein (CRP) responses. Co-primary outcomes are as follows: (i) any CAA within the 3 months of trial follow-up; (ii) average estimate of maximum coronary Z-score at weeks 1, 2 and 6 adjusting for rescue treatment. Additional outcomes will be assessed including cost effectiveness, quality of life, corticosteroid toxicity and other safety outcomes. DISCUSSION: Several recent studies have indicated that coronary complications associated with KD across Europe are much higher than early trials of IVIG had initially suggested. KD-CAAP directly addresses this issue by exploring the therapeutic benefit of adjunctive corticosteroids in unselected KD cases. If we find that corticosteroids prevent CAA and are safe, this is a cheap and widely available intervention that could be implemented immediately for the benefit of children. TRIAL REGISTRATION: ISRCTN71987471- March 31, 2020; Eudract 2019-004433-17.

Idiopathic Retinal Vasculitis, Aneurysms and Neuroretinitis: Clinical Improvement with Infliximab.

PURPOSE: Idiopathic retinal vasculitis, aneurysms and neuroretinitis is a rare vision-threatening condition. If untreated vision loss occurs due to complications of progressive retinal ischaemia including retinal neovascularisation, neovascular glaucoma and retinal exudation. Despite the proposed underlying inflammatory aetiology this condition demonstrates poor response to corticosteroid treatment. The aim was to describe two paediatric cases of idiopathic retinal vasculitis, aneurysms and neuroretinitis treated with infliximab. METHODS: Two case reports. RESULTS: Infliximab treatment led to resolution of aneurysmal dilatations and retinal vasculitis, and reversal of some retinal capillary non-perfusion. CONCLUSION: Early infliximab treatment should be considered in cases of idiopathic retinal vasculitis, aneurysms and neuroretinitis.

A Case of Idiopathic Retinitis Vasculitis Aneurysms and Neuroretinitis (IRVAN) Treated with Adalimumab.

PURPOSE: To report a case of IRVAN in a 13-year-old girl responding well to Adalimumab and Azathioprine. RESULTS: A 13-year-old girl presented to us with central scotoma for a duration of 10 months. She was treated earlier with oral steroids with poor response. Fundus examination revealed features of IRVAN. She was treated with intravitreal dexamethasone implant in both eyes with oral Mycophenolate Mofetil (MMF) with transient response to it. So she was switched over to subcutaneous Adalimumab 40 mg once in 2 weeks and oral Azathioprine 50 mg BD. The disease activity was well controlled with the current regime. CONCLUSION: Though various treatment modalities have been described in literature for the treatment of IRVAN. This is the first case of IRVAN to be treated with Adalimumab along with Azathioprine to be reported.

Cateterización percutánea de la arteria glútea superior para el tratamientode un aneurisma de arteria ilíaca interna / Posterior percutaneous superior gluteal artery catheterization for internal iliac artery aneurysm treatment

Introducción: actualmente los aneurismas de la arteria ilíaca interna se tratan en su mayoría por vía endovascular. En pacientes con intervenciones previas puede ser especialmente difícil tratarlos dada la ausencia de accesos arteriales “clásicos”. Caso clínico: varón de 80 años con crecimiento del saco aneurismático de la arteria ilíaca interna derecha en el que era imposible un acceso percutáneo por vía anterógrada ilíaca y por vía retrógrada femoral por intervenciones previas al que se le realizó un tratamiento mediante punción percutánea de la arteria glútea superior mediante embolización transcatéter con coils. Discusión: en ausencia de accesos clásicos para tratamiento endovascular de un aneurisma de ilíaca interna, la punción transglútea es una técnica sencilla y reproducible que ofrece una alternativa en estos escenarios.(AU)

Introduction: currently, internal iliac artery aneurysms are generally treated with endovascular techniques, in patients with previous interventions it can be particularly challenging to treat these lesions due to lack of a patent “classical” access site. Case report: we report a case of an 80-year-old man with a growing right internal iliac aneurysm, in which a percutaneous antegrade access through the iliac artery or a retrograde access to the femoral artery were impossible due to previous interventions, that was managed with trans catheter coil embolization with a percutaneous puncture to the superior gluteal artery. Discussion: when classical endovascular approaches to the intern iliac artery are absent, the transgluteal puncture is a simple and replicable technique, offering an alternative in challenging scenarios.(AU)

A Series of 14 Polish Patients with Thrombotic Events and PC Deficiency-Novel c.401-1G>A PROC Gene Splice Site Mutation in a Patient with Aneurysms

Objectives: Protein C (PC) deficiency is an inherited thrombophilia with a prevalence of 0.5% in the general population and 3% in subjects with a first-time deep vein thrombosis (DVT). Here we report a series of 14 PC-deficient Polish patients with comprehensive clinical and molecular characteristics, including long-term follow-up data and a deep mutational analysis of the PROC gene. Patients and Methods: Fourteen unrelated probands (mean ± SD age 43.8 ± 13.0 years) with suspicion of PC deficiency, who experienced thromboembolic events and a majority of whom received anticoagulants (92.8%), were screened for PROC mutations by sequencing the nine PROC exons and their flanking intron regions. Results: Ten probands (71.4%) had missense mutations, two patients (14.3%) carried nonsense variants, and the other two subjects (14.3%) had splice-site mutations, the latter including the c.401-1G>A variant, reported here for the very first time. The proband carrying the c.401-1A allele had a hepatic artery aneurysm with a highly positive family history of aneurysms and the absence of any mutations known to predispose to this vascular anomaly. Conclusion: A novel detrimental PROC mutation was identified in a family with aneurysms, which might suggest yet unclear links of thrombophilia to vascular anomalies, including aneurysms at atypical locations in women. The present case series also supports data indicating that novel oral anticoagulants (NOACs) are effective in PC deficient patients.

ChEVAR / ChEVAR

La técnica de chimenea para la reparación endovascular de aneurismas (ChEVAR) surgió como una técnica de rescate para revascularizar o preservar ramas críticas cubiertas durante la endoprótesis aórtica. Posteriormente se observó que la ChEVAR ofrecía una opción de tratamiento viable para la reparación aórtica compleja que involucraba una o más ramas, particularmente en situaciones en las que la reparación fenestrada no era una opción debido a restricciones anatómicas u otros problemas logísticos. En este contexto se apreció desde el principio que la técnica ChEVAR ofrecía dos ventajas distintas: disponibilidad de uso y un menor coste económico que permitían la realización de más procedimientos en muchos centros del mundo.La adopción de ChEVAR creció de manera constante a lo largo de los años, pero sufrió una notoria ausencia de evidencia científica sólida para respaldar la técnica. Todo esto cambió en 2015 con la publicación de resultados clínicos en el histórico registro PERICLES, que demostró excelentes resultados en una amplia gama de pacientes con aneurismas de anatomía compleja tratados por expertos en injertos paralelos en centros médicos claves de Europa y Estados Unidos. Los colaboradores del registro PERICLES identificaron dos factores claves en la causa de los canales persistentes (endofugas). Uno era el grado de sobredimensionamiento de la endoprótesis aórtica y el otro estaba relacionado con la longitud de la nueva zona de sellado. Los investigadores sugirieron, en estos casos, el tratamiento con Onyx™ y coils.Pero de importancia crítica, y a menudo pasada por alto, fue la observación de que la mayoría de las endofugas del canal ChEVAR detectadas al completar la angiografía se habían resuelto espontáneamente en el momento en que se realizaba el primer angio TAC posoperatorio.(AU)

The chimney technique for endovascular aneurysm repair (ChEVAR) emerged as a rescue technique to revascularisation or preserving covered critical branches during aortic endografting. ChEVAR adoption grew steadily over the years, but suffered a notorious absence of strong scientific evidence to underpin the technique. This all changed in 2015 with the publication of clinical results in the landmark PERICLES Registry, which demonstrated excellent outcomes in a wide range of complex-anatomy aneurysm patients treated by parallel-graft experts at key medical centres in Europe and the USA.Critically important (and often overlooked) is the observation that the majority of ChEVAR gutter endoleaks detected on completion angiography have resolved spontaneously by the time the first postoperative computed tomography angiography is performed.It is encouraging to see that ChEVAR has been included in the latest 2019 Abdominal Aortic Aneurysm Treatment Guidelines from the European Society for Vascular Surgery, where the technique is recommended in urgent cases and when fenestrated repair is unfeasible or contraindicated.(AU)

Activated Platelet-Homing Nanoplatform for Targeting Magnetic Resonance Imaging of Aneurysm-Related Thrombus in Rabbits.

Thrombosis is closely related to the instability of intracranial aneurysm (IA), whose rupture is associated with high morbidity and mortality. It is difficult to detect an IA-related thrombus because traditional magnetic resonance imaging (MRI) and even contrast-enhanced MRI cannot clearly distinguish a thrombus from the surrounding tissues. Herein, a nanoplatform [(MFe2O4-ZnDPA nanoparticles (NPs)], consisting of Zn0.4Co0.6Fe2O4@Zn0.4Mn0.6Fe2O4 NPs for imaging and Zn(II)-bis(dipicolylamine) (ZnDPA) for thrombus targeting, is constructed to target an experimental aneurysm-related thrombus in rabbits via MRI. In vitro experiments including platelet safety evaluation primarily prove that MFe2O4-ZnDPA NPs with a high MRI transverse relaxation time (T2) have good biocompatibility. MFe2O4-ZnDPA NPs could target a thrombus via the special interaction between ZnDPA and phosphatidylserine of activated platelets in the thrombus through MRI and Fe quantification assays. Moreover, after MFe2O4-ZnDPA NPs are injected into the ear vein of common carotid artery aneurysm model rabbits, MRI shows that MFe2O4-ZnDPA NPs could accumulate in the aneurysm-related thrombus from 0 to 15 min after injection and decrease in the next 45 min. Meanwhile, MFe2O4-ZnDPA NPs could decrease the MRI T2 signal of the aneurysm-related thrombus to enhance the outline of the aneurysm. This study demonstrates that a nanoplatform can enhance the detection of an aneurysm-related thrombus as well as aneurysm itself to assist further treatment of IA.

All trans retinoic acid alleviates coronary stenosis by regulating smooth muscle cell function in a mouse model of Kawasaki disease.

Coronary artery (CA) stenosis is a detrimental and often life-threatening sequela in Kawasaki disease (KD) patients with coronary artery aneurysm (CAA). Therapeutic strategies for these patients have not yet been established. All-trans-retinoic acid (atRA) is a modulator of smooth muscle cell functions. The purpose of this study was to investigate the effect of atRA on CA stenosis in a mouse model of KD. Lactobacillus casei cell wall extract (LCWE) was intraperitoneally injected into 5-week-old male C57BL/6 J mice to induce CA stenosis. Two weeks later, the mice were orally administered atRA (30 mg/kg) 5 days per week for 14 weeks (LCWE + atRA group, n = 7). Mice in the untreated group (LCWE group, n = 6) received corn oil alone. Control mice were injected with phosphate-buffered saline (PBS, n = 5). Treatment with atRA significantly suppressed CA inflammation (19.3 ± 2.8 vs 4.4 ± 2.8, p < 0.0001) and reduced the incidence of CA stenosis (100% vs 18.5%, p < 0.05). In addition, atRA suppressed the migration of human coronary artery smooth muscle cells (HCASMCs) induced by platelet-derived growth factor subunit B homodimer (PDGF-BB). In conclusion, atRA dramatically alleviated CA stenosis by suppressing SMC migration. Therefore, it is expected to have clinical applications preventing CA stenosis in KD patients with CAA.

Superior vena caval obstruction: a rare presentation of Behcet's disease.

A 25-year-old Indian man presented with low-grade fever followed by gradually increasing swelling of neck and face. Physical examination showed bilateral neck swelling, facial swelling and dilated veins in the upper chest. Superior vena cava (SVC) obstruction due to an underlying malignancy was suspected. CT thorax showed large saccular aneurysm with thrombosis of bilateral subclavian arteries of which the right one caused external compression of right innominate vein draining into the SVC. A history of recurrent oral and scrotal ulcers was obtained following which skin pathergy test was done, which was suggestive of a diagnosis of Behcet's disease (BD). He responded to treatment with steroids and azathioprine. This report illustrates that rare nonmalignant cause such as BD could also present with SVC obstruction.

Expression of MMP-2, MMP-9, and NGAL in Tissue and Serum of Patients with Vascular Aneurysms and Their Modulation by Statin Treatment: A Pilot Study.

BACKGROUND: Matrix metalloproteinases (MMPs) are involved in vascular wall degradation, and drugs able to modulate MMP activity can be used to prevent or treat aneurysmal disease. In this study, we evaluated the effects of statins on MMP-2, MMP-9, and neutrophil gelatinase-associated lipocalin (NGAL) in both plasma and tissue in patients with aneurysmal disease. METHODS: We performed a prospective, single-blind, multicenter, control group clinical drug trial on 184 patients of both sexes >18 years old with a diagnosis of arterial aneurysmal disease. Enrolled patients were divided into two groups: Group I under statin treatment and Group II not taking statins. In addition, 122 patients without aneurysmal disease and under statin treatment were enrolled as a control group (Group III). The expression of MMPs and NGAL in plasma was evaluated using ELISA, while their expression in endothelial tissues was evaluated using Western blot. RESULTS: The ELISA test revealed greater plasma levels (p < 0.01) of MMPs and NGAL in Groups I and II vs. Group III. Western blot analysis showed higher expression (p < 0.01) of MMPs and NGAL in Group II vs. Group I, and this increase was significantly higher (p < 0.01) in patients treated with low potency statins compared to high potency ones. CONCLUSIONS: MMPs and NGAL seem to play a major role in the development of aneurysms, and their modulation by statins suggests that these drugs could be used to prevent arterial aneurysmal disease.

Left Ventricular Noncompaction with Multiple Thrombi in Apical Aneurysm.

A 44-year-old man was admitted to our hospital due to heart failure. Transthoracic echocardiography demonstrated global hypokinesis with an ejection fraction of 25%, prominent trabeculation and deep intertrabecular recesses, and apical aneurysm with multiple thrombi (10×13 mm in the inferior wall, 15×8 mm in the anterior wall). Cardiac magnetic resonance imaging showed an increased ratio of noncompacted (NC) to compacted (C) myocardium (NC/C ratio >2.3) and apical aneurysm. Coronary angiography revealed no significant stenosis. He was therefore diagnosed with left ventricular noncompaction complicated by apical aneurysm. Four weeks after starting anticoagulation, the multiple apical thrombi disappeared without clinical signs of embolism.

Proximal Pulmonary Artery Aneurysm Secondary to Suspected Pulmonary Hypertension Treated with Conservative Therapy in Limited Resource Setting.

BACKGROUND Pulmonary artery aneurysm (PAA) is a rare disease in cardiovascular system. This disease is difficult to diagnose and less often considered due to its non-specific clinical manifestations. Until now there are no clear guidelines about its optimal management because of the small number of reported cases. CASE REPORT We report a 56-year-old male with chief complain of atypical bilateral chest pain and shortness of breath. Initial electrocardiogram (ECG) and laboratory evaluation showed no sign of ischemic heart disease. After the patient was stabilized, he was evaluated using chest x-ray, transthoracic echocardiography (TTE), and multi slice computed tomography (MSCT). The patient was then diagnosed with PAA secondary to suspicion of pulmonary hypertension (PH) with chronic obstructive pulmonary disease and heart failure. Conservative treatment was chosen because of the limited resources for surgery and patient's refusal to be referred. The treatment aims to lower the pulmonary artery pressure while monitoring the aneurysm. His 6-month follow-up evaluation showed an improvement in pulmonary artery pressure and persistent of the PAA without any increasement of the diameter. CONCLUSIONS PAA is a rare disease that is difficult to diagnose because of its non-specific nature. Persistent atypical chest pain can be an early symptom of PAA, thus clinicans should be aware in a high-risk patient suffered persistent chest pain, despite normal ECG and laboratory findings. TTE and MSCT evaluation are reliable for diagnosing PH and PAA. With conservative treatment and routine follow-up, patient with PAA secondary to PH could be managed well.