Abnormal Vascular Communication Research Articles

A Rare Case of Successful Device Closure of a Large Coronary CameralFistula to Right Atrium

Coronary artery anomalies are a rare group of congenital cardiac disorders with an incidence of less than 1% in general population. Coronary cameral fistula (CCF) are abnormal vascular communications between coronary artery and a cardiac chamber. These fistulae result from congenital abnormalities or are acquired as a result of trauma or cardiac surgery. We present the transcatheter closure of a large CCF from right coronary artery (RCA) to right atrium in an eight-year-old girl.

Overview of peripheral arteriovenous malformations: From diagnosis to treatment methods

Based on the latest classification by the International Society for the Study of Vascular Anomalies in 2018, vascular malformations (VMs) can be categorized into simple, combined VMs of major named vessels, and VMs associated with other anomalies. Simple VMs include lymphatic, venous, capillary, and arteriovenous malformations (AVMs). AVMs represent disorders of direct arteriovenous shunts caused by the absence of a capillary bed between the involved arteries and veins. This abnormal vascular communication causes arterial blood to accumulate in the venous vessels, thus resulting in venous hypertension and characteristic clinical manifestations, such as pulsation, tremors, and elevated temperature. AVMs can occur sporadically or as manifestations of syndromic lesions and are considered among the most complex and challenging VMs. The diagnosis and treatment of AVMs can vary depending on the lesion location and associated clinical symptoms, thus complicating their management. Herein, we discuss peripheral AVMs in terms of their clinical manifestations, imaging examinations, and staging systems to provide a comprehensive reference for the treatment, evaluation methods, and follow-up procedures for this vascular anomaly.

Coronary cameral fistula connecting right coronary artery to left ventricle – A case report

A Coronary Cameral Fistula (CCF) is a congenital or acquired abnormal vascular communication of coronary arteries with any of the cardiac chambers. The prevalence of CCF in adult patients was low (0.09 – 0.5%) and only 3-5% of all CCF drains into the left ventricle.

Congenital Portosystemic Shunts in Dogs and Cats: Classification, Pathophysiology, Clinical Presentation and Diagnosis.

Congenital portosystemic shunts (CPSS) are abnormal vascular communications between the portal and the systemic circulation, bypassing the hepatic parenchyma and resulting in liver hypoplasia and hepatic insufficiency. Such connections develop in utero and persist postnatally. CPSS are among the two most common congenital vascular anomalies of the liver in small animals, along with primary hypoplasia of the portal vein without portal hypertension (PHPV without PH). CPSS can be extrahepatic (ECPSS), most commonly diagnosed in small and toy breed dogs and cats, or intrahepatic (ICPSS), most commonly seen in large breed dogs. Single ECPSS is the most common type encountered in both dogs and cats. Clinical signs of CPSS are non-specific and may wax and wane, while laboratory findings can raise clinical suspicion for CPSS, but they are also not specific. Definitive diagnosis will be established by evaluation of liver function tests, such as determination of fasting plasma ammonia (FA) levels, and pre- and postprandial serum bile acids concentrations, and diagnostic imaging. The purpose of this article is to review the definition, classification, pathogenesis, clinical presentation, and diagnosis of CPSS in dogs and cats, highlighted by the authors' clinical experience.

Case series of carotid cavernous fistula: Clinical and management outcomes

ABSTRACT A carotid-cavernous sinus fistula (CCF) is an abnormal vascular communication between internal carotid artery or external carotid artery and veins within cavernous sinus (CS). CCF commonly present with ophthalmic manifestations due to venous drainage of the orbit from the CS. Here, we present case series of four CCFs; two direct and two indirect and discuss their ophthalmological and neuroradiological features and management outcomes with digital subtraction angiography and endovascular coiling. CCF is a rare clinical presentation and its early diagnosis and treatment prevents life and vision-threatening complications.

Multiple systemic artery to pulmonary vessel fistulas (SAPVFs) completely resected by video-assisted thoracoscopic surgery: a case report

BackgroundSystemic artery to pulmonary vessel fistula (SAPVF) is an uncommon abnormal vascular communication between systemic arteries and the lung parenchyma. It has been reported that the appropriate treatment for SAPVF is embolization or surgical resection. However, in patients such as ours, who have many aberrant vessels or multiple lesions, surgery should be considered as the first-choice treatment.Case presentationThis case report describes multiple SAPVFs and huge bullae at the apex of the left lung in a 43-year-old man that were resected completely with the video-assisted thoracoscopic surgery (VATS). The patient had an uneventful postoperative recovery without any complications and was discharged 9 days postoperatively. He had heavy smoking history, and the giant bullae and the diffuse emphysematous change were found in the lung. Therefore, the chronic inflammation may have been present in the thoracic cavity, which caused multiple SAPVFs.ConclusionsWe describe the clinical course and management of the patient with multiple SAPVFs who had no obvious history of surgery, trauma, or various inflammatory or infection diseases. VATS should be the first-choice treatment in patients with many abnormal vessels or multiple lesions.

246 A Case Report on Bilateral Spontaneous Carotico-Cavernous Fistula with Literature Review

Abstract Background CCF is an abnormal vascular communication that allows blood to either directly or indirectly flow from the carotid arteries to the cavernous sinus. We present a rare case of spontaneous bilateral direct CCF. Coil embolization is a very effective treatment for CCF. Case report 86-year-old lady presented in ED with two weeks history of progressive painful, swollen right eye & drooping of eyelid with double vison in left eye. Patient also had a history of intermittent headache and had pulsatile tinnitus in right ear. There was no history of head or eye trauma. On examination, the lady had proptosis, chemosis and restricted movement in right eye, 3rd nerve palsy in left eye. Corrected vision +0.3 in right eye, +0.1 in left eye. Pupils were bilaterally sluggish, there was no relative afferent pupillary defect. CTA showed bilateral direct CCF along with dilated superior ophthalmic veins on both sides (L>R). No other associated or underlying pathologies were seen. She was started on oral Acetazolamide, Travoprost and Brimonidine topically. Patient was urgently referred to neurosurgery department and Coil embolization of her CCF was done successfully the following day. Eye casualty review was done day after her coil embolization. IOP pressure was decreased to normal limits -12 RE and 8 LE. Although her VA was slightly down compared to her preoperative visit, her other symptoms were improving. Six months later her vision has returned to normal. Conclusions Bilateral spontaneous CCF is a rare entity. Early diagnosis and treatment lead to successful outcomes. Neuro-radiological intervention is an effective treatment in acute setting.

Case Report of a Post-Traumatic Carotido-Cavern Fistula

Carotid-cavernous fistulas (CCF) are defined as abnormal vascular communication between the internal and external carotid arteries or their meningeal branches and the cavernous cavity. The objective of our work is to show the radiological signs allowing the diagnosis of a carotid-cavernous fistula. We report the case of a 40-year-old patient with a left carotid-cavernous fistula following a head trauma, who consulted the emergency room 3 days after his trauma because of persistent pulsatile headaches and the appearance of a left exophthalmos. A CT scan, a Doppler ultrasound followed by a cerebral MRI after injection of contrast medium were performed. An angio-MRI confirmed the diagnosis and showed the presence of a left carotid-cavernous fistula. The mode of revelation of post-traumatic CFC is often stereotyped and the association of intracranial murmur and pulsatile exophthalmos immediately evokes the diagnosis. Cross-sectional brain imaging allows the diagnosis to be made. Arteriography remains irreplaceable to confirm the existence of the breach and to analyze all the angiographic elements that will be important at the time of the final therapeutic decision. The diagnosis of carotid-cavernous fistula suspected clinically is confirmed on imaging in the presence of evocative signs.

Congenital coronary artery-to-pulmonary fistula with giant aneurysmal dilatation and thrombus formation: a case report and review of literature

BackgroundCoronary artery-to-pulmonary artery fistula is a rare disorder characterized by abnormal vascular communication between the coronary artery and pulmonary artery. While most patients remain asymptomatic, some might exhibit symptoms of myocardial ischemia, congestive heart failure, or even sudden cardiac death if coronary aneurysm, thrombosis, infective carditis, or other congenital cardiac defects coexist.Case presentationWe present a 66-year-old male complaining of angina pectoris with a history of hypertension and active smoking. He was diagnosed with a coronary aneurysm based on coronary computed tomography angiography. We subsequently identified a coronary artery-to-pulmonary artery fistula with giant aneurysmal dilation on coronary angiography. Ultimately we conducted surgery ligation and aneurysmorrhaphy. During surgery, we discovered newly formed thrombus within the aneurysmal cavity. Histological analysis of the aneurysmal wall supported the diagnosis of the congenital disorder. Our patient was successfully discharged and remained asymptomatic at two months of follow-up.ConclusionWe presented a rare and complex combination of congenital coronary artery-to pulmonary artery fistula, giant coronary aneurysmal dilatation, and thrombosis through multi-modality evaluations.

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update.

Pulmonary arteriovenous malformations (PAVMs) are abnormal direct vascular communications between pulmonary arteries and veins which create high-flow right-to-left shunts. They are most frequently congenital, usually in the setting of hereditary hemorrhagic telangiectasia (HHT). PAVMs may be asymptomatic or present with a wide variety of clinical manifestations such as dyspnea, hypoxemia, or chest pain. Even when asymptomatic, presence of PAVMs increases patients’ risk of serious, potentially preventable complications including stroke or brain abscess. Transcatheter embolotherapy is considered the gold standard for treatment of PAVMs. Though previous guidelines have been published regarding the management of PAVMs, several aspects of PAVM screening and management remain debated among the experts, suggesting the need for thorough reexamination of the current literature. The authors of this review present an updated approach to the diagnostic workup and management of PAVMs, with an emphasis on areas of controversy, based on the latest literature and our institutional experience.

Balloon-assisted Onyx Embolization in the Management of Complicated Dural Arterioevenous Fistula.

Dural arteriovenous fistula (dAVF) is an abnormal vascular communication between the meningeal artery and vein at the dura mater, with frequent recruitment of collateral arterial pedicles. In recent years, Onyx embolization has become the mainstay intervention for dAVF at various locations, although an unfavorable vascular anatomy often limits effective obliteration of the lesion. Balloon assistance may facilitate curable occlusion of the abnormal shunt with Onyx while preserving the patency of the affected sinus, even in complicated dAVFs. We retrospectively reviewed the clinical and angiographic findings of patients with dAVF treated endovascularly in our institution between September 2018 and August 2019. Based on the detailed analysis of individual angioarchitecture, we defined complicated dAVF as lesions for which a complete cure is considered difficult to achieve with simple transarterial Onyx embolization alone, primarily due to a high flow and/or diffuse shunt with or without drainage to the functioning but compromised sinus. We evaluated the preoperative symptoms, anatomical classification, endovascular procedure, radiologic results, and clinical outcomes of these patients. Five patients met our criteria, all of whom were treated with balloon-assisted Onyx embolization (two superior sagittal sinus dAVF, two transverse-sigmoid sinus dAVF, and one torcular dAVF). In four cases, Onyx was injected from a tiny branch of the middle meningeal artery under balloon occlusion of the collateral arteries or balloon devascularization of the competitive inflow. In three cases, balloon sinus protection was performed to prevent the inadvertent occlusion of the vital venous outflow with Onyx. In one case, for the complementary occlusion of the remnant shunt through the previously coiled but recanalized occipital artery, Onyx was injected from the wire lumen of a dual-lumen balloon catheter to avoid undesirable reflux. The angiographic results were an anatomical cure in four cases and near-complete occlusion in one case. No procedural complications were observed in any cases. The clinical outcome was a complete cure in four cases and improvement in one case. Our cases suggest that balloon devascularization can improve the unfavorable flow environment and simplify the vascular anatomy. Balloon sinus protection can support not only securing the patency of the normal venous outflow but also encourage aggressive intervention. Onyx injection via a dual-lumen balloon catheter can augment the controllability of embolization while preventing reflux. Each of these techniques can facilitate safe and effective Onyx embolization in the treatment of complicated dAVFs, and their combination may further expand the therapeutic horizon.

Hemobilia: an unusual and life threatening complication of laparoscopic cholecystectomy

Hemobilia though rare, occurs due to abnormal vascular and biliary communication established as a result of trauma, gallstones, neoplasms, inflammatory process and vascular disorders. It can rarely complicate a laparoscopic cholecystectomy as a result of hepatic artery injury, clip migration, clip erosion, diathermy and subsequent arteriobiliary fistula, or pseudoaneurysm eroding into the extra hepatic bile ducts. A 34 years female underwent laparoscopic cholecystectomy for cholelithiasis, suffered bile leak that settled on by 13th postoperative day, met with sudden right upper abdominal pain and hypovolemic shock on 15th postoperative day due to massive hemobilia arising out of rupture of pseudoaneurysm of the right hepatic artery into the biliary system. Urgent CT Angiography showed a large pseudoaneurysm of right hepatic artery branch with adjacent small collection, IHBR and CBD dilatation suggestive of hemobilia. Emergency CT guided trans-arterial hepatic artery embolization done to control hemobilia and save the patient. An immediate CT angiography for early diagnosis, in all postoperative laparoscopic cholecystectomy cases with suspicion of hemobilia due to an underlying hepatic artery pseudoaneurysm, followed by CT guided trans-arterial hepatic artery embolization forms the gold standard management in such patients.

Endovascular Management of a Penetrating Zone III Retroperitoneal Gunshot Wound Injury; A Case Report

Traumatic iliac vessels injuries secondary to gunshot wound can often be fatal at the scene. One of the intriguing complications of vascular injuries is arteriovenous fistula. If the patient survives, these lesions may often not be diagnosed on first evaluation and patients may present with clinical signs and symptoms years later. Open surgical repair can have prohibitive morbidity and mortality and endovascular techniques, an effective treatment alternative, can interrupt the abnormal vascular communication and preserve artery vein patency. We describe a unique case of iliac arteriovenous fistula (AVF), secondary to a bullet injury, identified by imaging studies and subsequently treated with endovascular surgery. In conclusion, traumatic AVF are rare. Traditional teaching mandates that zone III pelvic retroperitoneal hematomas secondary to penetrating trauma be explored.

Case report. Multiple Mediastinal Arteriovenous Fistulas in a Patient with Myocardial Ischemia Symptoms and Tachy-Brady Arrhythmias

Abstract Arteriovenous fistulas are abnormal vascular communications between the arterial and venous system and may lead to the development of arteriovenous shunts. These malformations are extremely rare, and their diagnosis can be difficult due to the lack of symptoms or the absence of hemodynamic consequences. In many cases, arteriovenous fistulas are discovered by chance. In this brief report, we present the case of a 31-year-old male with symptoms suggestive of angina pectoris, tachycardia-bradycardia syndrome and multiple episodes of syncope. Multi-slice computed tomography revealed the presence of multiple mediastinal fistulas connecting the aorta, superior vena cava, pulmonary veins and azygos system.

Immediate and Intermediate Results of Patent Ductus Artriosus Transcatheter Closure in Kurdistan (Multicenter Experience)

Background: Patent Ductus Artriosus (PDA) is abnormal vascular communication between aorta and pulmonary artery and it is a common congenital heart anomaly seen in pediatric practice forming 5% - 10% of all congenital heart defects. A retrospective study aims to review our experience and determine the efficacy and safety of transcatheter PDA closure in different age groups and has the procedure replaced the conventional surgical closure of the PDA in the majority of the cases. To assess the results and outcomes of early experience, transcatheter closure of patent ductus Arteriosus in all three cardiac centers in Kurdistan, using the Amplatzer Duct Occluder type I and II (ADO), and Cook coil for the first time in Kurdistan are analyzed. Patients & Methods: A total of three hundred and nine patients which included 228 females & 81 males were diagnosed to have PDA, between March 2008 to February 2011 in all three cardiac centers in Kurdistan, namely Hawler, Sulaimani and Duhok cardiac centers, where they underwent transcatheter closure of the PDA with ADO type I and II. The common age on closure was 1 - 5 years old (range 5.5 months old to 32 years old), and weight ranged from 6 kg to 58 kg. We retrospectively analyzed medical records, echocardiography, angiographic and hemodynamic data and follow-up results of the patients. Patients included in the study were according to standard recommendation for transcatheter closure of PDA, and in any age they should be above 6 kilograms with exclusion of cases with pulmonary hypertension and interrupted IVC. Results: Among 309 cases did catheterization procedures, the ratio of female to male ratio was 2.6:1 and the age of doing PDA closure at time of procedure was between 5.5 months old to 33 years old, the mean age was 6.3 ± 6.5 years old and the weight ranges between 6 kg and 58 kg, mean of 11.5 ± 7.5 kg. The most commonly used device was Amplatzer I PDA device with high closure success rate of 97.1% after 6 and 12 months followup and the first case of Amplatzer II PDA Device closure done in Iraq and Kurdistan. There was immediate & complete closure in 271 on angiography. Complication was noted as embolized PDA device to Right Pulmonary Artery (RPA) in two patients, one of them retrieved by cath and other one by surgery. Conclusion: Transcatheter Amplatzer duct occluder is safe and effective in closing PDA in most patients with PDA even large as much as 10 mm and the advantage of new Amplatzer PDA II device allows small delivery sheath in which it can be done for children with less than 6 kg with fewer complications. Disadvantage is that it cannot close more than 6 mm PDA.

Sinus pericranii: A case report

A healthy 6-week-old white male presented with an intermittently enlarging reddish-blue plaque on the midline forehead and frontal scalp. Neuroimaging revealed an abnormal vascular communication between the extracranial and intracranial venous systems. This rare condition is termed sinus pericranii. The authors provide a review of sinus pericranii and describe its imaging findings, embryologic derivation, differential diagnosis, and clinical management.

Successful Treatment of a Large Pulmonary Arteriovenous Malformation by Repeated Coil Embolization.

Pulmonary arteriovenous malformations (AVMs) are caused by abnormal vascular communications between the pulmonary arteries and pulmonary veins, which lead to the blood bypassing the normal pulmonary capillary beds. Pulmonary AVMs result in right-to-left shunts, resulting in hypoxemia, cyanosis, and dyspnea. Clinical signs and symptoms vary depending on the size, number, and flow of the AVMs. Transcatheter embolization is the treatment of choice for pulmonary AVMs. However, this method can fail if the AVM is large or has multiple complex feeding arteries. Surgical resection is necessary in those kind of cases. Here, we report the case of a patient with a 6-cm pulmonary AVM with multiple feeding arteries that was successfully treated by repeated coil embolization without surgery.

Sensorineural Hearing Loss: What Lies Beneath? Neurovascular Conflict Secondary to a Dural Arteriovenous Malformation

This middle-aged gentleman with no previous medical history presented to the local ENT outpatient clinic complaining of right-sided hearing loss. No history of trauma or previous head and neck surgery was elicited.
 Following clinical and auditory assessment a right sensorineural hearing loss was confirmed. A right-sided facial palsy was additionally identified on examination.
 A MRI of the internal auditory meati was performed (Figure 1a & 1b). Following radiologist review, MRI and MRA of the brain was undertaken.
 
 DISCUSSION
 
 Auditory impairment is a condition with a legion of potential causes. One of the routine aspects of the assessment process for those with sensorineural hearing loss is MR imaging (MRI) of the internal auditory meati (IAMS).
 
 The vast majority of MRI studies are normal, however one of the more commonly identified pathologies are cerebrovascular abnormalities. The most well recognised is neurovascular conflict of the vestibulocochlear nerve by a vascular loop at the root entry zone (REZ), however a broader range of potential responsible structural abnormalities are known. A wide range of processes for auditory dysfunction have been outlined.1 These include; cerebral ischaemia events, subarachnoid haemorrhage, cerebrovascular malformations and rarely dural arteriovenous fistulas (dAVFs).
 
 Dural AVF's are abnormal vascular communications between the dural venous sinuses and an arter(ies) - most frequently branches of the external carotid artery. Sensorineural hearing impairment is one of the rarer presenting symptoms. The mechanism for hearing impairment is believed to result from either direct vascular compression on the vestibulocochlear nerve from an enlarged aberrant draining vein or from a vascular steal phenomenon (Figures 2a & 2b). An engorged draining vein from the dAVF causing mechanical compression on the nerve is the most well recognized.2 A single prior case has been reported of compression from an intraossesous dAVF of the skull base.3
 
 The arteriovenous fistula may be directed identified (Figure 3) along with the associated signs of enlarged cerebral cortical veins and white matter change of venous hypertension (Figure 4).

Spinal dural arteriovenous fistula associated with L-4 isthmic spondylolisthesis

The authors describe a case of a 79-year-old man with a lumbar spinal dural arteriovenous fistula (DAVF) and isthmic spondylolisthesis at the same level. The patient's thoracic spine MRI study demonstrated swelling and increased T2 signal in the spinal cord and regional dilated perimedullary vessels. Lumbar spine MRI showed L-4 isthmic spondylolisthesis with severe bilateral L4-5 foraminal stenoses. Spinal angiography revealed a fistulous connection at the left L-4 nerve root sleeve between perimedullary veins and a dural branch of the L-4 radicular artery. Based on previous reports about secondary spinal DAVFs, the abnormal vascular communication likely developed secondary to the microtrauma and inflammation on the left L-4 nerve root sleeve, which was attributable to the isthmic spondylolisthesis. The authors performed disconnection of the arteriovenous shunt as well as an L4-5 decompression and posterior instrumented fusion with pedicle screws. The patient's postoperative course was uneventful, and he improved neurologically. It is important to bear in mind that a spinal DAVF may develop as a consequence of any sort of trauma or inflammation involving nerve roots. One should consider the concomitant treatment of both the spinal DAVF and the underlying pathology that may have given rise to the spinal DAVF.

PP-239 A Rare Coronary Anomaly in a Patient with Pulmonary Hypertension Secondary to Scleroderma: Multiple Coronary Cameral Fistulae

A 63-year-old female with a history of scleroderma was referred to our institution for the investigation of dyspnea and atypical chest pain. On physical examination, her blood pressure was 132/88 mmHg and pulse rate was 116 beats/min. She was cyanotic and had an arterial oxygen saturation level of 85%. On cardiac examination, a left parasternal heave was noted; there were soft S1 and hard S2 with a grade III/VI holosystolic murmur heard over the left sternal border, which increased on inspiration. A 12-lead electrocardiogram demonstrated sinus tachycardia and right bundle branch block. Echocardiography showed a normally functioning left ventricle and enlarged right atrium, right ventricle and D-shaped septum (Figure 1). There was severe tricuspid regurgitation, and the estimated pulmonary artery systolic pressure was 106 mmHg. The patient was hospitalised for medical stabilisation. Furthermore, cardiac catheterisation revealed no atherosclerotic coronary artery disease; however, it incidentally revealed diffuse coronary cameral fi stulae (CCF) involving the circumfl ex-obtuse marginal system and diagonal branches emptying into the left ventricle (Figure 2), delineating the endocardial border very well (Figures 3, 4). Because of the fi stulae were diffuse and many smalls, an intervention was not considered. CCF are rare congenital or acquired anomalous with abnormal vascular communications between the coronary arteries and cardiac chamber. A vast majority (approximately 90%) of CCF communicates with the right-sided chambers of the heart, and in the remaining cases, they drain to the left side of the heart or to both sides (1) . The pathophysiology of CCF is unclear. Rudraiah et al. suggested that angiogenesis secondary to hypoxia may play a role in the development of CCF in the absence of acquired causes (e.g. exogenous trauma, previous invasive cardiac procedures or atherosclerosis) (2) . In this respect, hypoxia occurring in the setting of scleroderma may contribute to the development of CCF in the present case.