Acute myocardial infarction due to giant coronary artery aneurysm and arteriovenous fistula: a challenging case report and review of the literature.

BACKGROUND: A coronary artery aneurysm (CAA) is an abnormal dilation of a coronary artery segment often accompanied by coronary artery fistula (CAF), leading to communication between a coronary artery and a cardiac chamber or a part of the coronary venous system. Both CAAs and CAFs can present with symptoms and signs of myocardial ischemia and infarction. CASE PRESENTATION: We describe the case of a 46-year-old woman with non-ST-elevation myocardial infarction (NSTEMI) caused by a "giant" CAA. Various imaging modalities revealed a thrombus-containing aneurysm located at the right-posterior cardiac border, with established arteriovenous communication with the distal part of left circumflex artery (LCx). After initial treatment with dual antiplatelet therapy, a relapse of pain was reported along with a new increase in troponin levels, electrocardiographic abnormalities, reduced left ventricular ejection fraction (LVEF) and thrombus enlargement. Surgical excision of the aneurysm was favored, revealing its true size of 6 cm in diameter. Τhe aneurysm was excised without complications. The patient remained asymptomatic during follow-up. CONCLUSIONS: Management of rare entities such as "giant" CAAs and CAFs can be challenging. Cases such as this can serve as precedents to facilitate treatment plans and develop consistent recommendations, emphasizing the importance of personalized strategies for future patients.

[Ischemic stroke due to paradoxical embolism through pulmonary arteriovenous malformation in a young patient]. / ACV isquémico debido a embolia paradojal a través de malformación arteriovenosa pulmonar en paciente joven.

Paradoxical embolism due to an isolated pulmonary arteriovenous malformation (PAVM) is a rare cause of ischemic stroke. PAVMs are abnormal high-flow connections between pulmonary arteries and veins, diverting deoxygenated blood into the systemic circulation and they represent a less common source of paradoxical embolisms, especially in young individuals. Endovascular embolization is the preferred treatment for clinically significant PAVMs. We present the case of a 34-year-old woman with a left thalamic ischemic stroke. Severe contrast passage was detected in cerebral arteries through transcranial Doppler. Intracardiac ultrasound did not reveal a patent foramen ovale, prompting further investigation with pulmonary CT angiography, confirming the presence of PAVM. The patient underwent successful endovascular treatment. It is essential to consider PAVM in the etiological diagnosis of ischemic stroke, especially in young patients with signs of abnormal right-to-left communication. Periodic follow-up imaging is recommended to assess potential recurrence or changes in PAVM, emphasizing the importance of appropriate management of these malformations.

La embolia paradojal debido a una malformación arteriovenosa pulmonar (MAVP) aislada es una causa infrecuente de accidente cerebrovascular (ACV) isquémico. Las MAVP son conductos anómalos de alta circulación entre arterias y venas pulmonares, desviando sangre desoxigenada hacia la circulación sistémica y representan una fuente menos común de embolias paradojales, especialmente en personas jóvenes. La embolización endovascular es el tratamiento preferido para MAVP clínicamente significativas. Presentamos el caso de una mujer de 34 años con ACV isquémico talámico izquierdo. Se detectó pasaje de burbujas "en cortina" en arterias cerebrales mediante Doppler transcraneal. En ecografía intracardíaca no se encontró foramen oval permeable, motivo por el cual se avanzó con realización de angiotomografía pulmonar, la cual confirmó la presencia de MAVP. La paciente recibió tratamiento endovascular exitoso. Es esencial considerar la MAVP en el diagnóstico etiológico del ACV isquémico, especialmente en pacientes jóvenes con signos de comunicación anormal de derecha a izquierda. Se recomienda un seguimiento periódico mediante imágenes para evaluar la posible recurrencia o cambios en la MAVP, resaltando la importancia del manejo adecuado de estas malformaciones.

Comparison of pharmacological thrombolysis with mechanical thrombectomy in thrombosed arteriovenous fistulas and grafts: a systemic review and meta-analysis.

AIM: To compare the effectiveness and safety of pharmacological thrombolysis and mechanical thrombectomy. MATERIAL AND METHODS: This review was conducted in accordance with the PRISMA guidelines. Pooled proportions and subgroup analysis were calculated for primary and secondary patency rates, technical success, clinical success, major and minor complications rates. RESULTS: This systematic review identified a total of 6,492 studies of which 17 studies were included for analysis. A total of 1,089 patients comprising 451 (41.4 %) and 638 (58.6 %) patients who underwent thrombolysis and mechanical thrombectomy procedures, respectively, were analysed. No significant differences were observed between thrombolysis and mechanical thrombectomy procedures in terms of technical success, clinical success, major and minor complications rates, primary and secondary patency rates; however, subgroup analysis of overall arteriovenous fistulas (AVFs) and arteriovenous grafts (AVGs) demonstrated a significantly higher rate of major complications within the AVF group (p=0.0248). CONCLUSION: The present meta-analysis suggests that pharmacological thrombolysis and mechanical thrombectomy procedures are similarly effective and safe; however, AVFs are subject to higher major complications compared to AVGs.

Cortical malformation adjacent to a large pial arteriovenous fistula.

The coexistence of an arteriovenous fistula (AVF) and neuronal migration abnormalities is a rare phenomenon. The underlying pathophysiology responsible for these anomalies remains elusive. Neuronal architectural irregularities arise from complex neuronal formation, migration and organisation dysfunctions. Isolated cases of these associations are rarely described in the literature. Here, we present an unusual case involving the coexistence of a pial AVF and a pachygyria-polymicrogyria complex in an early childhood boy. We have provided a detailed description of the neuroimaging characteristics and the therapeutic embolisation in this case, along with follow-up. Additionally, we conduct a comprehensive review of potential hypotheses about the association, referencing prior case reports. The presence of an aberrant blood supply or deviant venous drainage from the developing cortex may contribute to a variety of neuronal migration anomalies.

[Liver failure caused by recurrent biliary bleeding associated with Osler's disease: a case report].

A 34-year-old female patient with epigastric pain was admitted to our hospital. She reported an underlying condition of Rendu-Osler-Weber disease and a history of coil embolization for pulmonary arteriovenous fistula. A blood test revealed high hepatobiliary enzyme levels. An abdominal contrast-enhanced computed tomography revealed numerous arterioportal and arteriovenous shunts in the liver and a high-density area in the bile duct, which was diagnosed as biliary bleeding. She underwent transpapillary biliary drainage by endoscopic retrograde cholangiopancreatography, but recurrent biliary bleeding caused cholangitis, which was complicated by multiple liver abscesses. She was awaiting her turn for liver transplantation from brain-dead donors, but the liver abscesses were difficult to improve. Further, liver failure, septic pulmonary embolism, and disseminated intravascular coagulation were complicated. Thus, recurrent further biliary bleeding resulted in hemorrhagic shock, which required frequent blood transfusions. Furthermore, the continuous abscess to the intrahepatic bile duct in the anterior superior segment penetrated her diaphragm, causing hemothorax and eventually, death. Establishing progressive treatment, including liver transplantation, is considered necessary for this intractable disease.

Open Radial Artery Study.

Radial artery occlusion (RAO) has been the most common postprocedural complication of transradial artery access. The optimal method of prevention of RAO is still lacking. In our study, we aimed to evaluate the effect of patent hemostasis on early (24 hours) and late (2 weeks) RAO prevention. The Open Radial Artery Study was a single-arm, prospective, and multicenter study. The primary end points were early and late RAO at the vascular access site after transradial coronary procedures. Secondary end points were access site hematoma, pseudoaneurysm formation, arteriovenous fistula, and nerve injury. A total of 2,181 patients were analyzed (67% male, mean age 68 years). The mean interventional duration and hemostatic times were 75.6 ± 55.6 and 60 ± 5.6 minutes, respectively. Radial artery spasm occurred in 10% of patients (n = 218). Catheter kinking, radial artery rupture, or dissection were not observed during the procedure. RAO, hematoma, pseudoaneurysm, arteriovenous fistula, or nerve damage was not observed in any of the patients in the early or late period. In patients who undergo coronary diagnostic or interventional procedures through transradial artery access, the patent hemostasis method seems a critical step in the prevention of early and late RAO.

High flow priapism following the insertion of an inflatable penile prosthesis mimicking autoinflation: a case report.

Penile prosthesis implantation is a surgical option for erectile dysfunction when other treatments fail or the patient prefers implantation. Although penile prosthesis is generally considered safe and effective, various complications have been reported in the literature. High-flow priapism, resulting from an arteriovenous fistula between the cavernosal artery and the corpora cavernosa, is a rare complication after penile prosthesis implantation. Managing the condition as autoinflation may lead to unfortunate complications. A 54-year-old male patient underwent a penile prosthesis implantation due to erectile dysfunction lasting for 5 years. Doppler ultrasound revealed arterial insufficiency that was refractory to oral and intracavernosal treatments. A 3-piece inflatable penile prosthesis (Coloplast - Titan) was implanted through a midline penoscrotal incision without any complications. The patient reported uncontrolled tumescence after activating the device, which led us to suspect autoinflation. The final diagnosis was high-flow priapism due to an arteriovenous fistula in the cavernosal artery. The patient was given an antiandrogenic medication and the prosthesis was deflated for 3 months. The fistula closed without any additional intervention. High-flow priapism is a rare but potential complication of penile prosthesis implantation. Careful evaluation and management of patients' symptoms are necessary for diagnosing and treating this condition. This case highlights the importance of considering high-flow priapism as a potential cause of uncontrolled tumescence after penile prosthesis implantation and the possibility of successful non-surgical management.

Two pediatric observations of spinal extradural arteriovenous fistulas presenting with epidural hemorrhages and cord compression.

We report two cases of acute spinal cord compression in children with low-flow spinal epidural arteriovenous fistulas (SEAVFs) and discuss the clinical presentation and management of these vascular anomalies. While most low-flow SEAVFs without radiculomedullary drainage are benign lesions typically diagnosed incidentally, we suggest that asymptomatic lesions may warrant aggressive management in specific circumstances, including lesions diagnosed at an early age or in patients under anticoagulation therapy. Our observations also emphasize that patients with a "spontaneous" epidural hemorrhage should undergo dedicated preoperative or postoperative vascular imaging to identify a possible underlying vascular anomaly.

Elevated intraneural vascularization of the median nerve proximal to the arteriovenous fistula in hemodialysis patients suspected of carpal tunnel syndrome: A case series.

Patients with end-stage kidney disease may require creation of an arteriovenous fistula in order to receive hemodialysis treatment. The creation may result in several complications, including carpal tunnel syndrome. Early diagnosis and treatment are essential to relieve symptoms, prevent permanent nerve damage, and improve quality of life. However, the sensory and motor disturbances resembling carpal tunnel syndrome could be related to other etiologies than external compression of the median nerve underneath the transverse ligament. This case report presents eight patients with a radiocephalic arteriovenous fistula, who all had symptoms of carpal tunnel syndrome. Ultrasonographic examination showed a segmental intraneural hypervascularization of a large vessel inside the median nerve proximal to the wrist and arteriovenous fistula anastomosis with garland-like course as well as multiple flow velocities. The neurophysiological findings showed a significant decreased velocity in the ipsilateral forearm to the arteriovenous fistula.

Outcomes of one-stage and two-stage aneurysm repair in arteriovenous fistulae.

OBJECTIVE: Maintenance of long-term arteriovenous access is important in long-term care for patients with end-stage renal disease. Arteriovenous access is associated in the long term with the development of fistula aneurysms (FAs). This study aims to evaluate the outcomes of staged FA treatment in dialysis access arteriovenous fistulae (AVF). METHODS: A retrospective review of all patients over a 12-year period with primary autogenous AVF was undertaken at a single center. Patients undergoing elective open aneurysm repair were identified and were categorized into three groups: single FA repair (single, control group) and staged and unstaged repair of two FAs (staged and unstaged). A staged repair was a procedure in which the initial intent was to treat both aneurysms in the AVF and in which the most symptomatic aneurysm was treated first. When the incision from the first surgery had healed, the second symptomatic aneurysm in the AVF was treated. An unstaged repair was a procedure in which the initial intent was to repair both symptomatic aneurysms simultaneously. All patients had a fistulogram before the FA repair. Thirty-day outcomes, cannulation failure, line placement, reintervention, and functional dialysis (continuous hemodialysis for 3 consecutive months) were examined. RESULTS: Five hundred twenty-seven patients presented with FA that met requirements for open intervention; 44% underwent single FA repair, whereas the remaining 34% and 22% underwent staged and unstaged repair of two FAs, respectively. The majority of patients were diabetic and Hispanic. Ninety-one percent of the patients required percutaneous interventions of the outflow tract (37%) and the central veins (54%). Thirty-day major adverse cardiovascular events were equivalent across all modalities. Thirty-day morbidity and early thrombosis (<18 days) were significantly higher in the unstaged group (4.3%) compared with the two other groups (1.3% and 2.1%, single and staged, respectively), which led to an increased need for a short-term tunneled catheter (8.9%) compared with the two other groups (3.4% and 4.4%, single and staged, respectively), Unstaged repair resulted in an increased incidence of secondary procedures (5.0%) compared with the two other groups (2.6% and 3.1%, single and staged, respectively). Functional dialysis at 5 years was equivalent in the single and staged groups but was significantly decreased in the unstaged group. CONCLUSIONS: Open interventions are successful therapeutic modalities for FAs, but unstaged rather than staged repair of two concurrent FAs results in a higher early thrombosis, an increased secondary intervention rate, and a need for a short-term tunneled central line. Staged and single FA repairs have equivalent results. In the setting of two symptomatic FAs, staged repair is recommended.

Prevalence and Predictors of Hereditary Hemorrhagic Telangiectasia and Capillary-Malformation Arteriovenous Malformation Syndrome Among Children with Neurovascular Malformations.

OBJECTIVE: To investigate the prevalence and predictors of hereditary hemorrhagic telangiectasia (HHT) and capillary-malformation arteriovenous malformation (CM-AVM) syndrome among children with no prior personal or family history of these diseases who presented with an arteriovenous shunt lesion. STUDY DESIGN: A retrospective chart review was completed on patients aged 0 through 21 years with arteriovenous shunt lesions evaluated at our Cerebrovascular Center. Diagnosis of definite or suspected HHT or CM-AVM was based on clinical features and genetic testing. Associations between final diagnosis and type and number of lesions, epistaxis, telangiectasias, CM, and pulmonary AVMs were assessed. RESULTS: Eighty-nine patients were included. Thirteen (14.6%) had definite HHT, 11 (12.4%) suspected HHT, and 4 (4.5%) definite CM-AVM. Having ≥2 episodes of epistaxis/year and ≥ 2 sites with telangiectasias were each associated with definite HHT (P < .001). Having ≥ 2 CM was associated with definite CM-AVM (P < .001). Pulmonary AVM was associated with increased odds of having definite HHT (OR = 6.3, 95% CI: 1.2-33.4). Multiple lesions (OR = 24.5, 95% CI: 4.5-134.8) and arteriovenous fistulas (OR = 6.2, 95% CI: 1.9-20.3) each increased the likelihood of having definite HHT or CM-AVM. Genetic testing was positive in 31% of patients tested. CONCLUSIONS: We recommend that children with neurovascular shunt lesions be offered genetic testing and undergo further evaluation for HHT and CM-AVM. Awareness and early diagnosis of these conditions is a critical step toward improving long-term outcomes and preventing disease-associated complications.

Characteristics and Outcomes of Patients With Pregnancy-Related End-Stage Kidney Disease.

Importance: The incidence of pregnancy-related acute kidney injury is increasing and is associated with significant maternal morbidity including progression to end-stage kidney disease (ESKD). Little is known about characteristics and long-term outcomes of patients who develop pregnancy-related ESKD. Objectives: To examine the characteristics and clinical outcomes of patients with pregnancy-related ESKD and to investigate associations between pre-ESKD nephrology care and outcomes. Design, Setting, and Participants: This was a cohort study of 183 640 reproductive-aged women with incident ESKD between January 1, 2000, and November 20, 2020, from the US Renal Data System and maternal data from births captured in the US Centers for Disease Control and Prevention publicly available natality data. Data were analyzed from December 2022 to June 2023. Exposure: Pregnancy-related primary cause of ESKD, per International Classification of Diseases, Ninth Revision (ICD-9) and ICD-10 codes reported at ESKD onset by the primary nephrologist on Centers for Medicare and Medicaid Services form 2728. Main Outcomes Measures: Multivariable Cox proportional hazards and competing risk models were constructed to examine time to (1) mortality, (2) access to kidney transplant (joining the waiting list or receiving a live donor transplant), and (3) receipt of transplant after joining the waitlist. Results: A total of 341 patients with a pregnancy-related primary cause of ESKD were identified (mean [SD] age 30.2 [7.3]). Compared with the general US birthing population, Black patients were overrepresented among those with pregnancy-related ESKD (109 patients [31.9%] vs 585 268 patients [16.2%]). In adjusted analyses, patients with pregnancy-related ESKD had similar or lower hazards of mortality compared with those with glomerulonephritis or cystic kidney disease (adjusted hazard ratio [aHR], 0.96; 95% CI, 0.76-1.19), diabetes or hypertension (aHR, 0.49; 95% CI, 0.39-0.61), or other or unknown primary causes of ESKD (aHR, 0.60; 95% CI, 0.48-0.75). Despite this, patients with pregnancy-related ESKD had significantly lower access to kidney transplant compared with those with other causes of ESKD, including (1) glomerulonephritis or cystic kidney disease (adjusted subhazard ratio [aSHR], 0.51; 95% CI, 0.43-0.66), (2) diabetes or hypertension (aSHR, 0.81; 95% CI, 0.67-0.98), and (3) other or unkown cause (aSHR, 0.82; 95% CI, 0.67-0.99). Those with pregnancy-related ESKD were less likely to have nephrology care or have a graft or arteriovenous fistula placed before ESKD onset (nephrology care: adjusted relative risk [aRR], 0.47; 95% CI, 0.40-0.56; graft or arteriovenous fistula placed: aRR, 0.31; 95% CI, 0.17-0.57). Conclusion and Relevance: In this study, those with pregnancy-related ESKD had reduced access to transplant and nephrology care, which could exacerbate existing disparities in a disproportionately Black population. Increased access to care could improve quality of life and health outcomes among these young adults with high potential for long-term survival.

Autoimmune glial fibrillary acidic protein astrocytopathy complicated with low flow perimedullary arteriovenous fistula: a case report.

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy and low-flow perimedullary arteriovenous fistulas (PMAVFs) may cause longitudinal widespread myelopathy. We report a middle-aged male patient with autoimmune GFAP astrocytopathy complicated with low flow PMAVFs disease, presenting with lower extremity weakness and dysuria. Magnetic resonance imaging (MRI) of the spinal cord revealed a significant longitudinal extent of T2 high signal from T11 to L1, with the lesion located proximal to the vascular territory supplied by the anterior spinal artery. Multiple patchy abnormal signals were seen adjacent to the anterior and posterior horns of the lateral ventricles bilaterally and at the centers of the semi-ovals on MRI of the cranial brain, with iso signal in T1Flair, the high signal in T2WI, and no high signal seen in Diffusion Weighted Imaging (DWI). Subsequently, the presence of anti-GFAP antibodies was detected in the cerebrospinal fluid (CSF), and the diagnosis of autoimmune GFAP astrocytopathy in conjunction with low-flow PMAVFs was confirmed through spinal digital subtraction angiography (DSA). This case report aims to increase neurologists' awareness of this disease and avoid missed or misdiagnosed cases that may lead to delayed treatment.

[A Case of Left Renal Cell Carcinoma with Renal Arteriovenous Fistula and Multiple Vascular Malformation Undergoing Nephrectomy].

A 69-year-old woman was referred to our hospital for the treatment of a left renal tumor found by computed tomography (CT) during examination for microscopic hematuria. Contrast-enhanced CT showed a 5 cm tumor in the inferior pole of the left kidney. Left renal cell carcinoma (RCC) (cT1bN0M0) was suspected. In addition, the left renal and gonadal veins were dilated and enhanced in an arterial phase; renal arteriovenous fistula (RAVF) was suspected. Moreover, there were multiple focal arterial dilatations, suggesting the presence of multiple vascular malformation. Hereditary aortic disease, including vascular Ehlers-Danlos syndrome (vEDS), was a concern. In general, surgery is not recommended for patients with vEDS, due to vascular fragility. As such, a panel analysis of genes for hereditary aortic diseases, including vEDS, was performed; no pathogenic variants in candidate genes including COL3A1 were identified. After detailed discussions with the patient, she underwent a left nephrectomy, following transcatheter arterial embolization (TAE) of the left renal artery. We prepared a balloon catheter for aortic occlusion as a preventative measure for massive bleeding; this was not the case, as only a small amount of intraoperative bleeding occurred. Thus, the nephrectomy was performed successfully without using the balloon catheter. The patient recovered uneventfully and was discharged on day 8. Pathological examination showed clear-cell RCC (pT1a) and a RAVF near the tumor. Herein we report this case of left RCC with RAVF and multiple arterial malformation, which was successfully managed by evaluating preoperative risks with a genetic test, followed by TAE of the renal artery and open nephrectomy.

The efficacy of percutaneous transluminal angioplasty and arteriovenous fistula reconstruction for immature arteriovenous fistula.

BACKGROUND: To access the efficacy of percutaneous transluminal angioplasty and arteriovenous fistula reconstruction for immature arteriovenous fistula, compare the long-term patency and post-operative complications between them. MATERIALS AND METHODS: The medical records and Hemodialysis record sheets from 44 patients between May 2020 and January 2022 who underwent percutaneous transluminal angioplasty or arteriovenous fistula reconstruction treatment for immature autogenous arteriovenous fistula (AVF) were retrospectively reviewed. The patients were divided into two groups according to the type of surgery they received, including 25 patients in the PTA group and 19 patients in the AVF reconstruction group. Clinical outcomes were included, such as the primary and secondary patency rates following the procedure, maturation time, peak systolic velocity (PSV) of brachial artery, maximum pump-controlled blood flow at initial dialysis, and post-operative complications rates in the two groups. RESULTS: Technical and clinical success was achieved in 100% of the 44 cases. For patients who underwent percutaneous transluminal angioplasty, the primary patency rate at 3, 6, and 9 months was 84.0%, 68.0%, 60.0%, and the secondary patency rate was 92.0%, 84.0%, 80.0%, respectively. And for patients who underwent arteriovenous fistula reconstruction, the primary patency rate at 3, 6, and 9 months was 89.5%, 73.7%, 68.4%, and the secondary patency rate was 100.0%, 94.7%, 94.7%, respectively. There were no significant differences between the two groups in terms of patency rates (p > .050). In patients whose maturation was successful, the average maturation time of fistula after the PTA procedure was 19.36 ± 13.94 days, and 58.63 ± 18.95 days for the reconstruction procedure (p < .010). The PSV of brachial artery before and after the procedure was 87.64 ± 23.87 cm/s and 153.20 ± 21.69 cm/s in PTA group, for reconstruction group, the number was 86.26 ± 20.59 cm/s and 151.26 ± 29.94 cm/s, respectively. No statistically significant differences (p > .050). The maximum pump-controlled blood flow at initial dialysis was 232.60 ± 16.72 ml/min in PTA group, which was significantly higher than 197.11 ± 10.45 ml/min in reconstruction group (p < .010). Subcutaneous hematoma, restenosis, thrombus formation, and pseudoaneurysm were major complications in PTA group. Restenosis, thrombus formation, and pseudoaneurysm were major complications in reconstruction group, with no statistically significant differences between the two groups (p > .050). CONCLUSION: When immature AVFs require reconstruction surgery, the patency outcomes are comparable to AVFs that undergo successful management by PTA. While, when AVFs are successfully managed by PTA, they have significantly less maturation times and higher maximum pump-controlled blood flow rates at initial dialysis AVF use.

Renal artery stenosis stenting with unmasking and embolisation of a renal arteriovenous fistula.

A man in his 70s was presented to the emergency department with uncontrollable hypertension and worsening renal function on a background of atherosclerosis-related bilateral renal artery stenosis. Following progressive deterioration in renal function and refractory hypertension, the patient was referred to interventional radiology for consideration of renal artery stenting. Following stenting of the right renal artery, a large renal arteriovenous fistula became apparent, which required emergent embolisation. Both procedures were successful, with excellent clinical and radiological responses.