Diagnosis and Management of Congenital Coronary Artery Fistulas in Adults.

PURPOSE OF REVIEW: This review describes the presentation, diagnosis, and management of congenital coronary artery fistulas (CAFs) in adults. RECENT FINDINGS: CAFs are classified as coronary-cameral or coronary arteriovenous fistulas. Fistulous connections at the distal coronary bed are more likely to be aneurysmal with higher risk of thrombosis and myocardial infarction (MI). Medium-to-large or symptomatic CAFs can manifest as ischemia, heart failure, and arrhythmias. CAF closure is recommended when there are attributable symptoms or evidence of adverse coronary remodeling. Closure is usually achievable using transcatheter techniques, though large fistulas may require surgical ligation with bypass. Given their anatomic complexity, cardiac CT with multiplanar 3-D reconstruction can enhance procedural planning of CAF closure. Antiplatelet and anticoagulation are essential therapies in CAF management. CAFs are rare cardiac anomalies with variable presentations and complex anatomy. CAF management strategies include indefinite medical therapy, percutaneous or surgical CAF closure, and lifelong patient surveillance.

Primary Aortoenteric Fistula Due to Salmonella typhi-related Mycotic Aneurysm.

Primary aortoenteric fistulas (AEF) are rare. The majority of these are due to atherosclerotic aortic aneurysms. Mycotic aortic aneurysms leading to primary AEF are exceedingly uncommon. Here we report a rare case of primary AEF secondary to Salmonella-related mycotic aneurysm and discuss the diagnostic and therapeutic issues.

A Case of Giant Left Circumflex Coronary Fistula Aneurysm with Unidentified Fistula.

A coronary aneurysm is a rare type of cardiovascular disease. We report a case of a 53-year-old male patient who presented to our hospital with a giant left circumflex coronary fistula aneurysm (LCCA) (75 mm × 70 mm). Since coronary angiography and coronary computed tomography angiography failed to detect the fistula of the coronary aneurysm, interventional occlusion surgery could not be performed. We discovered the fistula in the right atrium by anterograde perfusion with blood-containing myocardial protective fluid after switching to intraoperative exploration during cardiac surgery. The coronary aneurysm's fistula and inlet were then sutured, and the aneurysm was resected. The patient recovered successfully after the operation. This case was instructive in managing LCCA, especially with an unidentified fistula.

Arterio-Ureteral Fistula: Systematic Review of 445 Patients.

PURPOSE: Arterio-ureteral fistula (AUF) is an uncommon diagnosis, but increasingly reported and potentially lethal. This systematic review comprehensively presents risk factors, pathophysiology, location and clinical presentation of AUF aiming to increase clinical awareness of this rare but life-threatening condition, and to put this entity into a contemporary perspective with modern diagnostic tools and treatment strategies. MATERIALS AND METHODS: This review was performed according to the PRISMA (Preferred Reporting Items for a Systematic Review and Meta-Analysis of Individual Participant Data) guidelines. A literature search in PubMed® and EMBASE™ was conducted. In addition, retrieved articles were cross-referenced. Data parameters included oncologic, vascular and urological history, diagnostics, treatment, and followup, and were collected using a standard template by 2 independent reviewers. RESULTS: A total of 245 articles with 445 patients and 470 AUFs were included. Most patients had chronic indwelling ureteral stents (80%) and history of pelvic oncology (70%). Hematuria was observed in 99% of the patients, of whom 76% presented with massive hematuria with or without previous episodes of (micro)hematuria. For diagnosis, angiography had a sensitivity of 62%. The most predominant location of AUF was at the common iliac artery ureteral crossing. AUF-specific mortality before 2000 vs after 2000 is 19% vs 7%, coinciding with increasing use of endovascular stents. CONCLUSIONS: AUF should be considered in patients with a medical history of vascular surgery, pelvic oncologic surgery, irradiation and/or chronic indwelling ureteral stents presenting with intermittent (micro)hematuria. A multidisciplinary consultation is necessary for diagnosis and treatment. The most sensitive test is angiography and the preferred initial treatment is endovascular.

A systematic review of therapies for aortobronchial fistulae.

OBJECTIVE: The aim of the study was to summarize epidemiologic data about aortobronchial fistulae and compare outcomes (mortality, recurrence, reoperation) of open, staged, and endovascular repair of aortobronchial fistula. METHODS: A systematic literature review was conducted to identify eligible studies published between January 1999 and December 2019. The Cochrane Library, PubMed, and Scopus databases were used as search engines. Eligible studies included articles reporting postoperative outcomes (death/follow-up). Literature review revealed only case reports and small case series, and thus, only descriptive data with data heterogeneity were available. The corresponding authors were contacted to provide additional information or outcome updates (recurrence/reoperation/death). RESULTS: Overall, 214 patients (90 studies) underwent 271 procedures (including redo procedures and staged procedures). Most of the patients were treated by endovascular means (72.42%). Open surgical repair was performed in 21.96% and staged procedures in 5.6%. Aortobronchial fistulae were located most often in the descending thoracic aorta (zone 3 or 4) (64.6%) and in zone 2 (23.8%). Fourteen percent of aortobronchial fistulae developed after thoracic endovascular aneurysm repair. Recurrence or infection occurred in 20% (43) patients. Recurrences were, to some extent, associated with the presence of endoleak. Long-term antibiotic administration (>1 month) was instituted in 63 patients (29.4%), whereas 90 patients (42%) did not receive antibiotics beyond hospitalization. From the remaining 61 patients, 3 received lifelong antibiotics and for 58 patients data were not available. Considering outcomes, the mean follow-up was 25.1 months (0-188 months) and not significantly different among treatments. LIMITATIONS: Literature review has revealed only case reports and small case series, and thus, only descriptive data were available. Randomized controlled trials are not available due to the rarity of the disease, which significantly decreases the power of the present study. Also, this study reflects significant data heterogeneity due to the nature of the analyzed manuscripts and would benefit from large patient cohort studies that have not been conducted till today. CONCLUSIONS: Aortobronchial fistula is a complex disease. Endoleaks may be involved in the development and the recurrence process, and they should not be disregarded. Considering major outcomes (length of follow-up), the available treating strategies are equal, and thus, surgeons should feel confident to apply the treatment of their choice, keeping in mind their experience, patient's age, and clinical condition.

Arterio-ureteral fistula: a nationwide cross-sectional questionnaire analysis.

PURPOSE: Arterio-ureteral fistula (AUF) is an uncommon diagnosis, but potentially lethal. Although the number of reports has increased over the past two decades, the true incidence and contemporary urologists' experience and approach in clinical practice remains unknown. This research is conducted to provide insight in the incidence of AUF in The Netherlands, and the applied diagnostic tests and therapeutic approaches in modern practice. METHODS: A nationwide cross-sectional questionnaire analysis was performed by sending a survey to all registered Dutch urologists. Data collection included information on experience with patients with AUF; and their medical history, diagnostics, treatment, and follow-up, and were captured in a standardized template by two independent reviewers. Descriptive statistics were used. RESULTS: Response rate was 62% and 56 AUFs in 53 patients were reported between 2003 and 2018. The estimated incidence of AUF in The Netherlands in this time period is 3.5 AUFs per year. Hematuria was observed in all patients; 9% intermittent microhematuria, and 91% presenting with, or building up to massive hematuria. For the final diagnosis, angiography was the most efficient modality, confirming diagnosis in 58%. Treatment comprised predominantly endovascular intervention. CONCLUSION: The diagnosis AUF should be considered in patients with persistent intermittent or massive hematuria.

Out of sight for the endoscopist? Gastrointestinal bleeding after aortic repair.

BACKGROUND AND STUDY AIM: Secondary Aortoenteric Fistulas (sAEF) are difficult to diagnose and usually result in fatal gastrointestinal (GI) bleeding following aortic repair. Outcomes are largely dependent on a timely diagnosis, but AEFs remain challenging to identify endoscopically and are usually diagnosed on computed tomography (CT) scans. The aim of our study was optimize diagnosis of AEF by identifying patients developing GI bleeding after aortic repair, investigate their clinical course and identify factors specific to different bleeding sources. METHODS: A retrospective, single-center study capturing all patients developing upper or lower GI bleeding after aortic surgery between January 2009 and March 2020 was performed. Electronic health records were screened for diagnostic codes of the relevant procedures. Bleeding was classified into three groups: AEF with demonstrable fistula, ischemic - macroscopic ulceration plus histological confirmation or imaging and "other" due to other recognized conventional cause, such as peptic ulcer disease. RESULTS: 47 GI bleeding episodes in 39 patients were identified. Of these, 10 episodes (21%) were caused by AEF, 16 (34%) by ischemic ulceration and 21 (45%) due to other causes. Patients with AEF exhibited more frequent hemodynamic instability requiring vasopressors and had higher mortality, while ischemic ulcerations were associated with more recent operation or hypotensive episode. CONCLUSIONS: GI bleeding complications are uncommon following aortic surgery. AEF and ischemic ulceration are however frequent bleeding causes in this cohort. In patients presenting with fulminant bleeding, primary CT-scanning should be considered.

In situ bypass and extra-anatomic bypass procedures result in similar survival in patients with secondary aortoenteric fistulas.

OBJECTIVE: The optimal revascularization modality in secondary aortoenteric fistula (SAEF) remains unclear in the literature. The purpose of this investigation was to determine the revascularization approach associated with the lowest morbidity and mortality using real-world data in patients with SAEF. METHODS: A retrospective, multi-institutional study of SAEF from 2002 to 2014 was performed using a standardized database. Baseline demographics, comorbidities, and operative and postoperative variables were recorded. The primary outcome was long-term mortality. Descriptive statistics, Kaplan-Meier survival analysis, and univariate and multivariate analyses were performed. RESULTS: During the study period, 182 patients at 34 institutions from 11 countries presented with SAEF (median age, 72 years; 79% male). The initial aortic procedures that resulted in SAEF were 138 surgical grafts (76%) and 42 endografts (23%), with 2 unknown; 102 of the SAEFs (56%) underwent complete excision of infected aortic graft material, followed by in situ (in-line) bypass (ISB), including antibiotic-soaked prosthetic graft (53), autogenous femoral vein (neoaortoiliac surgery; 17), cryopreserved allograft (28), and untreated prosthetic grafts (4). There were 80 patients (44%) who underwent extra-anatomic bypass (EAB) with infected graft excision. Overall median Kaplan-Meier estimated survival was 319 days (interquartile range, 20-2410 days). Stratified by EAB vs ISB, there was no significant difference in Kaplan-Meier estimated survival (P = .82). In comparing EAB vs ISB, EAB patients were older (74 vs 70 years; P = .01), had less operative hemorrhage (1200 mL vs 2000 mL; P = .04), were more likely to initiate dialysis within 30 days postoperatively (15% vs 5%; P = .02), and were less likely to experience aorta-related hemorrhage within 30 days postoperatively (3% aortic stump dehiscence vs 11% anastomotic rupture; P = .03). There were otherwise no significant differences in presentation, comorbidities, and intraoperative or postoperative variables. Multivariable Cox regression showed that the duration of antibiotic use (hazard ratio, 0.92; 95% confidence interval, 0.86-0.98; P = .01) and rifampin use at time of discharge (hazard ratio, 0.20; 95% confidence interval, 0.05-0.86; P = .03) independently decreased mortality. CONCLUSIONS: These data suggest that ISB does not offer a survival advantage compared with EAB and does not decrease the risk of postoperative aorta-related hemorrhage. After repair, <50% of SAEF patients survive 10 months. Each week of antibiotic use decreases mortality by 8%. Further study with risk modeling is imperative for this population.

Adjuncts to the Management of Graft Aorto-Enteric Erosion and Fistula with in situ Reconstruction.

OBJECTIVE: The objective of this retrospective single centre study was to determine whether different enteric reconstruction methods and adjuncts confer a benefit after in situ reconstructions (ISRs) of graft aorto-enteric erosion (AEnE) and fistula (AEnF). METHODS: Primary endpoints were in hospital mortality and AEnE/F recurrence. Survival was estimated using the Kaplan-Meier method and explanatory factors were searched for using uni- ± multivariable Cox regression analysis. In 2013, a multidisciplinary team meeting was convened and since then the primary operator has always been a senior surgeon. RESULTS: Sixty-six patients were treated for AEnE (n = 38) and AEnF (n = 28, 42%) from 2004 to 2020. All patients with AEnF presented with gastrointestinal bleeding (vs. 0 for AEnE; p < .001). Signs of infection were seen in 50 patients (76% [37 for AEnE vs. 13 for AEnF]; p < .001). Referrals for endograft infection increased over time (n = 15, 23%; one before 2013 vs. 14 after; p = .002). Most patients underwent complete graft excision (n = 52, 79%) with increasing suprarenal cross clamping (n = 21, 32%; four before 2013 vs. 17 after; p = .015). Complex visceral reconstructions decreased over time (n = 31, 47%; 17 before 2013 vs. 14 after; p = .055), while "open abdomens" (OAs) increased (one before 2013 vs. 22 after; p < .001), reducing operating time (p = .012). In hospital mortality reached 42% (n = 28). Estimated survival reached 47.6% (95% confidence interval [CI] 35.0 - 59.1) at one year and 45.6% (95% CI 33.0 - 57.3) at three years and was higher for AEnE than for AEnF (log rank p = .029). AEnE/F recurrence was noted in 12 patients (18%). Older age predicted in hospital mortality in multivariable analysis (p = .034). AEnE/F recurrence decreased with the presence of a primary senior surgeon (vs. junior; p = .003) and OA (1 [4.4%] vs. 11 [26%] for primary fascial closure; p = .045) in univariable analysis. CONCLUSION: Mortality and recurrence rates remain high after ISR of AEnE/F. Older age predicted in hospital mortality. Primary closure of enteric defects ≤ 2 cm in diameter reduced operating time without increasing the recurrence of AEnF.

Coronary Artery-Left Ventricular Fistula with Giant Right Coronary Aneurysm: A Case Report and Literature Review.

Right coronary artery-left ventricular (RCA-LV) fistula with associated giant right coronary artery aneurysm (CAA) is an extremely rare cardiac condition. This case study presents a patient with a large left ventricle (LV) and a giant right CAA with a maximal inner diameter of approximately 56.6 mm and an inner diameter of approximately 22 mm at its communication with the left ventricle. The patient underwent surgical management, involving suturing of the proximal end of the CAA and coronary artery bypass grafting (CABG). RCA-LV fistula with a giant right CAA may involve serious complications, such as thrombosis, rupture, and heart failure. Therefore, it is necessary to establish effective management strategies for this condition. Although this case is not unique, it serves as an illustrative example of the implementation of a classic surgical treatment method.

Giant Atrial Septal Artery Aneurysm with a Fistula to the Right Atrium.

An atrial septal artery aneurysm is a rare disease, especially accompanied with a fistula. During clinical practice, it is very important to clear the anatomical details of the coronary aneurysm before operation. In the current article, we report a giant atrial septal artery aneurysm originating from a branch of the RCA combined with a coronary right atrial fistula. The coronary artery aneurysm should be evaluated using multiple diagnostic and imaging modalities, such as echocardiography, coronary artery angiography, magnetic resonance imaging, and cardiac CT, especially three-dimensional reconstruction, which could help us to distinguish the physiological and anatomical characteristics of the CAA and fistula.

Successful Surgical Treatment of a Giant Left Coronary Artery Aneurysm with Fistula.

Coronary artery aneurysm (CAA) is an aortic catastrophe with low prevalence. Giant CAA is even more uncommon, requiring surgical intervention. Giant CAA usually originates from the proximal segments of the right coronary and the anterior descending arteries. Here we report a rare case of giant left CAA with fistula formation treated with successful surgery.

[Aortoesophageal fistula-A rare differential diagnosis of upper gastrointestinal bleeding]. / Die aortoösophageale Fistel ­ Eine seltene Differenzialdiagnose der oberen Gastrointestinalblutung.

Approximately 1% of all patients are admitted to an emergency room for upper gastrointestinal hemorrhage. Differential diagnostics reveal an aortoesophageal fistula (AEF) as the cause of the bleeding in very few cases. Despite increasing means of diagnostics and treatment, mortality is high in patients with AEF even under maximum medical care. These are often fulminant situations with fatal outcome for the patient. We report a case that supports this observation described from previous cases and give a closer look at this rare emergency situation. A 54-year-old patient was taken to a maximum care hospital with the clinical diagnosis of upper gastrointestinal bleeding after receiving emergency medical treatment. The source of bleeding was quickly identified as an AEF following lobectomy for bronchial cancer. Despite maximum interventional intensive treatment, the patient died a few hours after hospital admission in hemorrhagic shock due to fulminant hemorrhage from the fistula.

Uretero-arterial fistula: Six new cases and systematic review of the literature.

AIM: Secondary uretero-arterial fistulas (SUAF) are uncommon, underrated and threatening for any patient. Gross hematuria is a clinical symptom of this pathology for patients with history of pelvic radiotherapy, complex pelvic surgery or long-term ureteral stenting. The purpose of this work is to assess risk factors, diagnosis and treatment of SUAF. METHODS: Monocentric and retrospective series of 6 new cases illustrated by a literature review through MedLine and Pubmed using the keywords "arterio-ureteral fistula", "arterio iliac fistula" and "ilio-ureteral fistula". We excluded uretero-arterial fistula following vascular surgery. RESULTS: Our series included 4 men and 2 women. All patients had a history of complex pelvic surgery and long-term ureteral stenting. Three patients had history of pelvic radiotherapy. They all had inaugural macroscopic haematuria episode. Two fistula cases were diagnosed on 5 repeated CT-scans. In 2 out of 5 cases, arteriography highlighted the fistula. Fistulas were generally located at the left common iliac artery. An endovascular stent was placed in 5 out of 6 cases. One patient needed open surgery. After treatment, 3 patients remained alive, 3 patients died either by a fistula relapse or by complications late in the treatment. CONCLUSION: SUAF are uncommon, but serious. Today, there is no specific recommendation regarding complex treatment of these fistulas. Endovascular stents seem to be a good therapeutic option. LEVEL OF PROOF: 3.

Pulse Wave Analysis and Pulse Wave Velocity for Fistula Assessment.

BACKGROUND/AIMS: Pulse wave analysis (PWA) and pulse wave velocity (PWV) provide information about arterial stiffness and elasticity, which is mainly used for cardiovascular risk stratification. In the presented prospective observational pilot study, we examined the hypothesis that radiocephalic fistula (RCF)-related changes of haemodynamics and blood vessel morphology including high as well as low flow can be seen in specific changes of pulse wave (PW) morphology. METHODS: Fifty-six patients with RCF underwent local ambilateral peripheral PWA and PWV measurement with the SphygmoCor® device. Given that the output parameters of the SphygmoCor® are not relevant for the study objectives, we defined new suitable parameters for PWA in direct proximity to fistulas and established an appropriate analysing algorithm. Duplex sonography served as reference method. RESULTS: Marked changes of peripheral PW morphology when considering interarm differences of slope and areas between the fistula and non-fistula arms were observed in the Arteria radialis, A. brachialis and arterialized Vena cephalica. The sum of the slope differences was found to correlate with an increased flow, while in patients with fistula failure no changes in PW morphology were seen. Moreover, PWV was significantly reduced in the fistula arm. CONCLUSION: Beside duplex sonography, ambilateral peripheral PWA and PWV measurements are potential new clinical applications to characterize and monitor RCF function, especially in terms of high and low flow.

Wellens' syndrome and finding of multiple coronary cameral fistulae: Is it time to discard this term?

Wellens' syndrome refers to electrocardiographic (ECG) abnormalities in the precordial T-wave segment, which are associated with critical stenosis of the proximal left anterior descending (LAD) coronary artery. According to medical literature, this ECG abnormality is of paramount importance because this syndrome represents a preinfarction stage of coronary artery disease; however, same ECG pattern can also be seen in other conditions. Coronary fistula occurs due to anomalous communications between a coronary artery and a cardiac chamber or other vessel in the vicinity of the heart. We report a case of multiple coronary artery fistulae to the left ventricle in a 74-year-old woman who had a 2-year history of intermittent atypical chest pain and exercise dyspnea with positive criteria mimicking Wellens' syndrome without coronary atherosclerosis.

Severe Dilated Cardiomyopathy Resulted from a Large Single Coronary Artery Fistula Drained Into the Left Ventricle.

BACKGROUND: Coronary artery fistula (CAF) draining into the left ventricle (LV) is a rare condition and dilated cardiomyopathy (DCMP) that results from single coronary artery (SCA) accompanied by CAF also is extremely rare. CASE REPORT: We report the case of a 36-year-old man, who presented with severe DCMP that resulted from SCA with CAF draining into the LV. Transthoracic echocardiogram (TTE) showed severe diffuse hypokinesia of the LV with ejection fraction (EF) of 15-20%. Coronary angiography (CAG) revealed SCA connected between left anterior descending artery (LAD) and posterior descending artery (PDA), course of the PDA was very tortuous from apex to base of the LV, and connected to posterior lateral (PL) branch, which was drained into the LV at distal part of the PL. Coronary artery computed tomography (CACT) showed LAD ran over the apex of the LV and connected to PDA, which was drained into the mid portion of lateral wall of the LV. Cardiac magnetic resonance imaging (CMRI) showed no evidence of irreversible myocardial change in global wall of the LV. The patient underwent surgical ligation of PDA near the base of the posterior wall of the LV as close to the entry of CAF to the LV as possible without any surgery-related complications. Three months after the surgical ligation, follow-up TEE  showed much improved EF of 45-50%. He has been doing well without congestive heart failure (CHF) until now. DISCUSSION: Symptomatic CAF with hemodynamic deterioration may need mechanical correction of CAF, including surgical ligation or percutaneous interventional occlusion. How to treat this condition in terms of methodology is a very difficult issue. The detailed methods related to surgical or interventional correction of CAF have to be determined based on anatomical characteristics of CAF, underlying comorbidities, and relevant complications risk.

Images of a large fistulous communication between the right ventricle and left anterior descending coronary artery.

Coronary artery fistulas are relatively rare congenital or iatrogenic heart defects that can present with or without symptoms. Symptomatic patients manifest as myocardial ischaemia, arrhythmia, or heart failure. We present a asymptomatic child with a large left anterior descending coronary artery to right ventricular fistula.

[Peculiarities of diagnosis and emergency surgical care in aorto-oesophageal fistula]. / Osobennosti diagnostiki i neotlozhnoi khirurgicheskoi pomoshchi pri aorto-pishchevodnom svishche.

An aorto-oesophageal fistula is a rare but life-threatening pathological condition developing on the background of diseases of the aorta and oesophagus, as well as after surgical interventions on the aorta. The article deals with a clinical case report regarding management of a patient presenting with an aorto-oesophageal fistula resulting from a thoracic artery aneurysm. The main clinical manifestations of the diseases included dysphagia (due to oesophageal obstruction caused by thrombotic masses of the aneurysm) and the occurring gastrointestinal haemorrhage. Comprehensive instrumental diagnosis was performed using roentgen examination of the oesophagus, oesophagoscopy, and contrast-enhanced computed tomography of the chest. The obtained findings made it possible to objectively assess the patient's state, to carry out timely treatment in conditions of a surgical hospital, and to avoid severe complications.

Kasabach-Merritt syndrome arising from a vascular fistula.

A 58-year-old woman presented with gum bleeding, hematuria, and cutaneous ecchymoses. Left hip replacement had been performed five years prior. The overall findings of our work-up were consistent with ongoing DIC triggered by the presence of an arterio-venous left femoral fistula. The patient was treated successfully with fresh frozen plasma, the fistula was surgically repaired and a stent was placed. On the second day, bleeding had resolved and laboratory values reverted to normal. This uncommon scenario is reminiscent of the Kasabach-Merritt syndrome and well illustrates that patients with an arterio-venous fistula can sometimes present with atypical features. The Kasabach-Merritt syndrome is reported in pediatric and adult patients with giant hemangiomas and angiosarcomas. Adult cases are described also in association with hematomas and large vascular aneurysms. The underlying pathophysiology is the sequestration and consumption of platelets and clotting factors with uncontrolled formation of microthrombi within the vascular lesion. DIC and a microangiopathic hemolytic anemia can subsequently develop. Mechanistic pathways of the Kasabach-Merritt syndrome in the context of a vascular fistula are shared with the more common causes of the syndrome. We speculate that the endothelial dysfunction and injury caused by the flow shear were the pivotal triggers of the aberrant trapping of platelets, the consumptive coagulopathy, and the formation of microthrombi within the fistula. Mortality rate can be as high as up to 40%. The Kasabach-Merritt syndrome could represent the only clinical feature of an otherwise occult vascular fistula. Emergency physicians should be aware of this condition.