A Case of High-Output Heart Failure.

CASE PRESENTATION: A 55-year-old woman with a medical history of hereditary hemorrhagic telangiectasia (HHT) complicated by recurrent nosebleeds, severe blood loss anemia, hepatic arterial-venous malformation (AVM), pulmonary hypertension, and severe tricuspid regurgitation presented to the HHT specialty clinic with acute hypoxic respiratory failure (new 3-L O2 requirement), weight gain, and volume overload. She was directly admitted to the pulmonary hypertension unit of our hospital. She had two recent admissions for similar symptoms thought to be due to worsening pulmonary arterial hypertension. In prior admissions, she had undergone right heart catheterization demonstrating mild pulmonary hypertension (pulmonary arterial pressure, 29 mm Hg, cardiac output by Fick 5.76, and cardiac index 3.22, mildly elevated pulmonary vascular resistance to 5.5 woods units). She would undergo diuresis with symptomatic improvement; however, after discharge she would rapidly develop recurrent heart failure symptoms. She reported compliance with guideline-directed medications, diuretics, and dietary restrictions and was still suffering severe symptoms. Notably she had previously elevated liver enzymes concerning for cirrhosis and had begun a workup to evaluate for causes of cirrhosis; she had a history of mild alcohol use, negative hepatitis viral serology, and no known history of liver disease.

Endovascular Repair of Diffuse Pulmonary Arteriovenous Malformations with Systemic Arterial Shunts.

Diffuse pulmonary arteriovenous malformations (PAVMs) are defined as arteriovenous malformations (AVMs) involving subsegmental, segmental, or both types of arteries in at least 1 lung lobe and are more extensive than multiple PAVMs. Diffuse PAVMs involving systemic arterial shunts are very rare. We describe a rare case, in which diffuse PAVMs involving systemic arterial shunts were successfully treated with coil embolization.

Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations.

Hereditary haemorrhagic telangiectasia (HHT) also known as Osler-Weber-Rendu syndrome is an autosomal dominant disorder affecting 1 in 8000 individuals. The eponym recognises the 19th-century physicians William Osler, Henri Jules Louis Marie Rendu and Frederick Parkes Weber who each independently described the disease. It is characterised by epistaxis, telangiectasia and visceral arteriovenous malformations. Individuals with HHT have been found to have abnormal plasma concentrations of transforming growth factor beta and vascular endothelial growth factor secondary to mutations in ENG, ACVRL1 and MADH4. Pulmonary artery malformations (PAVMs) are abnormal communications between pulmonary arteries and veins and are found in up to 50% of individuals with HHT. The clinical features suggestive of PAVMs are stigmata of right to left shunting such as dyspnoea, hypoxaemia, cyanosis, cerebral embolism and unexplained haemoptysis or haemothorax. The authors present the case of a 33-year-old woman presenting with progressive dyspnoea during the COVID-19 pandemic. She had a typical presentation of HHT with recurrent epistaxis, telangiectasia and pulmonary arteriovenous malformations. Although rare, PAVM should be considered in individuals presenting to the emergency department with dyspnoea and hypoxaemia. Delayed diagnosis can result in fatal embolic and haemorrhagic complications.

Recurrent Hemoptysis: A Bronchial Dieulafoy's Lesion in a Pediatric Patient.

OBJECTIVE: This paper presents a case of a bronchial Dieulafoy's lesion in a pediatric patient with recurrent hemoptysis. CASE REPORT: A 11-year old female presented multiple times with dry cough and hemoptysis to an outside hospital, each time leading to a diagnosis of epistaxis and subsequent discharge. When she arrived to our tertiary center with heavy hemoptysis and no evidence of epistaxis, the patient was urgently taken to the operating room by both the otolaryngology and pediatric pulmonology services. Active bleeding from a Dieulafoy's lesion on the right lower bronchus was found and selective embolization of two tortuous arteries was subsequently performed. The patient was discharged in stable condition without recurrence of hemoptysis over the last two months. CONCLUSION: While rare, especially in pediatric patients, bronchial Dieulafoy's lesions may cause severe hemoptysis and should be considered in the differential diagnosis when the etiology for hemoptysis is unclear.

Capillary-venule malformation is a microfistulous variant of arteriovenous malformation.

OBJECTIVE: To describe typical clinical presentation of patients with microfistular, capillary-venule (CV) malformation as a variant form of arteriovenous malformations (AVM). METHODS: A retrospective clinical analysis of 15 patients with CV-AVM confirmed by a computational flow model enrolled in a prospective database of patients with congenital vascular malformation between January 2008 and May 2018. RESULTS: The mean age of the patients at first time of presentation was 30 years with balanced sex ratio. Presentation was dominated by soft tissue hypertrophy (n = 12 [80.0%]) and atypical varicose veins (n = 11 [73.3%]). The anatomic location of enlarged varicose veins gave no uniform pattern and did not correspond with the typical picture of primary varicose vein disease. Most often, symptomatic CV-AVM was found at the lower extremities in this series of unselected patients. The most frequent compartment affected was the subcutis (n = 14 [93.3%]), involvement of muscle was recorded in one-third and cutis in one-fourth of patients. CONCLUSIONS: A high grade of clinical suspicion is needed to recognize CV-AVM and to prevent inadequate therapy owing to missed diagnosis.

A Rare Case of Dysplastic Axillary Artery Aneurysm Associated with Arteriovenous Malformation.

BACKGROUND: Axillary artery aneurysms are rare conditions, and their causes are various. They can determine severe complications, so the treatment is extremely important. METHODS: We report the case of a young man affected by a saccular axillary artery aneurysm associated with intramuscular arteriovenous malformation, without symptoms except for the presence of a pulsatile mass. Duplex scan and computed tomography scan have been essential for a correct diagnosis and planning of the treatment. At first, the patient was submitted to coil embolization of an efferent vessel, and then he was treated surgically through ligation and detachment of the aneurysm and replacement of part of the axillary artery with a Dacron graft (Vascutek, Inchinnan, Renfrewshire, Scotland, UK). RESULTS: Follow-up at 1 and 6 months revealed normal patency of the axillary arterty and the prosthetic graft with complete exclusion and thrombosis of the aneurysm sac.No sensitive nor motor deficit were observed. CONCLUSIONS: Aneurysms of the axillary artery associated with intramuscular arteriovenous malformations are very rare, but have to be suspected. The treatment is challenging and can be surgical, endovascular, or hybrid, based on the patient's conditions and aneurysm's anatomical features.

RhoA activation-mediated vascular permeability in capillary malformation-arteriovenous malformation syndrome: a hypothesis.

Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is a class of capillary anomalies that are associated with arteriovenous malformations and arteriovenous fistulas, which carry a risk of hemorrhages. There are no broadly effective pharmacological therapies currently available. Most CM-AVMs are associated with a loss of RASA1, resulting in constitutive activation of RAS signaling. However, protein interaction analysis revealed that RASA1 forms a complex with Rho GTPase-activating protein (RhoGAP), a negative regulator of RhoA signaling. Herein, we propose that loss of RASA1 function results in constitutive activation of RhoA signaling in endothelial cells, resulting in enhanced vascular permeability. Therefore, strategies aimed at curtailing RhoA activity should be tested as an adjunctive therapeutic approach in cell culture studies and animal models of RASA1 deficiency.

Superselective Arterial Embolization of Arteriovenous Malformation of Internal Pudendal Artery, a Rare Cause of Hematuria: A Case Report.

Arteriovenous malformation (AVM) is a common form of vascular malformation, an abnormal direct communication between an artery and a vein without passing through the capillaries. AVM may just present as hematuria without plain or other symptoms. The article presents a case of a 52-year-old male with gross hematuria diagnosed as AVM of internal pudendal artery, which was successfully managed with superselective arterial embolization using temporary embolization materials.

A Case of an Aggressive Intraosseous Arteriovenous Malformation in the Lower Extremity: Special Considerations for Diagnosis and Management.

BACKGROUND: Arteriovenous malformations (AVMs) are rare, congenital vascular anomalies. Intraosseous AVMS most frequently arise in the head and neck, with only a small fraction occurring in the extremities. Herein, we report the findings of a combined soft tissue and intraosseous AVM involving the lower extremity of a 13-year-old child. This case highlights the necessity of an interdisciplinary approach for the effective treatment and management of these rare vascular anomalies. CASE DESCRIPTION: A 13-year-old female presented with a 4-year history of intermittent pain and swelling over her right lateral malleolus. The patient was evaluated with radiologic imaging revealing an AVM involving the right distal leg, ankle, and hindfoot with intraosseous involvement of the distal tibia and talus. She was then referred to Vascular and Plastic Surgery and an angiogram was performed demonstrating shunting from the anterior tibial, peroneal, and posterior tibial arteries to the AVM. Venous drainage was to the anterior tibial and greater saphenous veins. Three embolizations were performed over the course of 6 months. Following the third embolization, the patient was taken to the operating room where Plastic and Orthopedic Surgery performed total resection of the nidus and involved bone which was then grafted with injectable synthetic bone graft. RESULTS: Successful resection of the nidus was achieved, and the patient had an uncomplicated recovery. Within 6 months postoperatively, the patient demonstrated full range of lower extremity motion and was able to participate in age appropriate gross motor activities. Radiologic evaluation 7 months postoperatively showed no evidence of nidus recurrence. CONCLUSION: Intraosseous involvement of AVMS is rare and presents a therapeutic challenge due to its invasive potential and high incidence of recurrence. Wide local excision with bone grafting and interdisciplinary management are paramount for complete resection.

Pulmonary Arteriovenous Malformations in Non-hereditary Hemorrhagic Telangiectasia Patients: An 18-Year Retrospective Study.

PURPOSE: Pulmonary arteriovenous malformations (PAVMs) are most commonly associated with hereditary hemorrhagic telangiectasia (HHT). Patients with PAVMs can present with serious complications including stroke, transient ischemic attack (TIA), and brain abscess. PAVMs are rare in non-HHT patients and little is known about this patient population. The aim of this retrospective study is to better understand clinical presentation and outcomes of PAVMs occurring exclusively in non-HHT patients. METHODS: Non-HHT patients with PAVMs at the Mayo Clinic-Rochester between 01/01/2000 and 12/31/2018 were reviewed. Patients with Curacao score > 1 were excluded. Demographics, imaging characteristics, neurological complications, and follow-up imaging were analyzed. RESULTS: Seventy-seven patients with PAVMs were identified. The mean age at diagnosis was 48.2 ± 18.3 years with female preponderance (59.7%). The majority of PAVMs had lower lobe predominance (66.7%) and were simple and single in 75.3% and 89.6% of cases, respectively. Most patients were asymptomatic (46.8%) with dyspnea being the most common symptom (28.6%). Neurologic complications occurred in 19.5% of patients. The majority of PAVMs were idiopathic (61%). Thirty patients (39%) had one or more possible risk factors including previous thoracic surgery (23.4%), congenital heart disease (19.5%), and chest trauma (10.4%). Embolization was performed in 37 (48.1%) patients and only 4 (5.2%) underwent surgical resection. CONCLUSIONS: Non-HHT PAVMs occur more commonly in females, are most commonly simple and single, and have lower lobe predominance and a high rate of neurologic complications. Potential predisposing risk factors were identified in about 40% of the cases. Clinicians should be aware of the risk of PAVM development in patients with history of chest trauma, congenital heart disease, lung infection/abscess, and thoracic surgery.

Uterine arterio-venous malformation, an uncommon life-threatening condition: a case report.

Uterine Arteriovenous Malformation is a rare gynaecological disorder which commonly presents with profuse vaginal bleeding. This case report presents a patient referred to the Military Hospital, Rawalpindi from Pakistan Aeronautical Complex Hospital Kamra, a peripheral secondary care hospital. Patient was diagnosed as a case of Uterine Arteriovenous Malformation at the Military Hospital and was successfully treated with uterine artery embolization..

Ischiofemoral Impingement Syndrome Secondary to Arteriovenous Malformation of the Quadratus Femoris: A Case Report.

CASE: Ischiofemoral impingement syndrome (IFIS), an unusual presentation of hip pain, is frequently missed in clinical practice. We report a case of a 27-year-old man with complaints of gradual onset nonprogressive right gluteal pain with hip movement restriction for the past 5 years that was not relieved with conservative measures. Right hip magnetic resonance imaging revealed a vascular abnormality of the right quadratus femoris muscle suggestive of IFIS. This abnormal muscle was surgically excised, and its histologic evaluation was remarkable for an arteriovenous malformation. CONCLUSION: The patient's longstanding symptomatic IFIS was ultimately attributed to a rare arteriovenous malformation of the quadratus femoris that resolved with surgical excision.

Angiopoietin-2 predicts morbidity in adults with Fontan physiology.

Morbidity in patients with single-ventricle Fontan circulation is common and includes arrhythmias, edema, and pulmonary arteriovenous malformations (PAVM) among others. We sought to identify biomarkers that may predict such complications. Twenty-five patients with Fontan physiology and 12 control patients with atrial septal defects (ASD) that underwent cardiac catheterization were included. Plasma was collected from the hepatic vein and superior vena cava and underwent protein profiling for a panel of 20 analytes involved in angiogenesis and endothelial dysfunction. Ten (40%) of Fontan patients had evidence of PAVM, eighteen (72%) had a history of arrhythmia, and five (20%) were actively in arrhythmia or had a recent arrhythmia. Angiopoietin-2 (Ang-2) was higher in Fontan patients (8,875.4 ± 3,336.9 pg/mL) versus the ASD group (1,663.6 ± 587.3 pg/mL, p < 0.0001). Ang-2 was higher in Fontan patients with active or recent arrhythmia (11,396.0 ± 3,457.7 vs 8,118.2 ± 2,795.1 pg/mL, p < 0.05). A threshold of 8,500 pg/mL gives Ang-2 a negative predictive value of 100% and positive predictive value of 42% in diagnosing recent arrhythmia. Ang-2 is elevated among adults with Fontan physiology. Ang-2 level is associated with active or recent arrhythmia, but was not found to be associated with PAVM.

Interventional Management of Arteriovenous Malformations.

Arteriovenous malformations (AVMs) are fast flow malformations characterized by the presence of arteriovenous shunting. These congenital lesions can be evolutive, leading to serious complications such as bleeding, skin ulceration, and cardiac failure. The interventional radiologist plays an important role in the management of these patients. He should be involved in the clinical evaluation to make the proper diagnosis, evaluate the symptoms and potential indication for endovascular treatment. This evaluation should be done in a multidisciplinary clinic with access to plastic surgeons, internal medicine and dermatologist, as well as specific specialists that might need to be implicated (ENT surgeon in the face and neck area, for example). The Schobinger clinical classification is important to assess patient evolution and indicate intervention. We recommend to treat symptomatic or evolutive AVMs. Doppler ultrasound is the first imaging examination that should be performed. Then, MR angiography or computed tomography angiography (CTA) can be proposed depending on the anatomic area involved. Embolization is currently the first line of treatment for these patients. There is currently promising research in the identification of genetic markers and molecular target(s) but there is no recognized pharmacologic treatment for AVM available yet. Digital substraction angiography (DSA) is usually performed for guidance during the embolization session but is also essential to properly classify a specific lesion, according to its anatomy. The anatomic classifications proposed by Cho and Yakes are both useful to choose the best therapeutic approach: Endovascular, direct puncture, retrograde venous approach or a combination of these techniques. Ethanol is the most efficient agent but is at higher risk of skin necrosis and nerve injury and should therefore be used with caution in dangerous territories. Glue and Onyx are liquid agents that are also well suited to occlude the nidus; they can be used in association with ethanol. On the venous side, mechanical occlusion with coils or Amplatzer plugs is mostly used. Again, they can be used in association with a liquid agent (Ethanol, glue or Onyx) to reflux in the nidus. Surgery can be indicated to resect residual AVM following embolization if residual symptoms are present and the planned surgery is feasible, with relative safety.

The Oxygen Saturation in Vascular Abnormalities Depends on the Extent of Arteriovenous Shunting in Diabetic Retinopathy.

Purpose: Diabetic retinopathy is characterized by disturbances in retinal blood flow mediated by capillary occlusion, intraretinal microvascular abnormalities (IRMAs), neovascularizations, and omega loops and reduplications. It is likely that the study of oxygen saturation in these abnormalities can provide knowledge about their role in the development of diabetic retinopathy. Methods: The oxygen saturation in IRMA vessels and venous loops and reduplications were studied in 40 diabetic patients with severe nonproliferative or proliferative diabetic retinopathy. The saturation values in the studied vascular abnormalities were compared to those of the larger retinal arterioles and venules. Results: There was a similar oxygen saturation (mean ± SD) in IRMAs observed to connect arterioles with venules (78.6% ± 11.8%, n = 22) and IRMAs connecting venules with venules (79.2% ± 9.0%, n = 12; P > 0.999). The saturation in IRMAs was significantly lower (P < 0.0002) than in arterioles (97.4% ± 5.2%, n = 40) and significantly higher (P < 0.0001) than the saturation in omega loops and reduplications (54.2% ± 19.3%, n = 6), which in turn showed no significant difference from the saturation in the venules (61.8% ± 6.8%, n = 40, P = 0.4). Conclusions: The findings suggest that the oxygen saturation in vascular abnormalities in diabetic retinopathy depends on the extent of arteriovenous (A-V) shunting, with venous saturation due to no A-V shunting in venous loops and reduplications, and intermediate oxygen saturation due to moderate shunting in IRMAs. This may precede the development of neovascularizations with arterial oxygen saturation due to high A-V shunting.

Endovascular Management of Massive Hemoptysis Secondary to Systemic Collaterals in Previously Treated Pulmonary Arteriovenous Malformation.

Pulmonary arteriovenous malformations (PAVMs) are abnormal connections between pulmonary artery and vein through a thin-walled aneurysmal sac, leading to a right-to-left shunt. These are commonly associated with hereditary hemorrhagic telangiectasia, and treatment guidelines have been set on how to approach their management. Incorrect proximal placement of an embolization device can lead to delayed recruitment of systemic collaterals leading to presentation with hemoptysis. We are presenting a case of a 17-year-old male with treated PAVMs and with fresh hemoptysis. In this case report, we attempt to explain the management of such a complication and follow the principles of arteriovenous malformation embolization on the left side.

Embolisation of pulmonary arteriovenous malformations using high-frequency jet ventilation: benefits of minimising respiratory motion.

BACKGROUND: To evaluate patient radiation dose and procedural duration recorded during pulmonary arteriovenous malformation (PAVM) embolisation performed using high-frequency jet ventilation (HFJV) as compared with conventional intermittent positive pressure ventilation (IPPV) METHODS: Patients undergoing PAVM embolisation with HFJV assistance after April 2017 were retrospectively identified as group A, and those treated with IPPV before April 2017 as group B. Primary outcomes were patient radiation dose and procedural duration between groups A and B. Secondary outcomes were difference in diaphragmatic excursion between groups A and B, in group A with/without HFJ assistance, technical/clinical success, and complications. RESULTS: Twelve PAVMs were embolised in 5 patients from group A, and 15 PAVMs in 10 patients from group B. Mean patient radiation was significantly lower in group A than in group B (54,307 ± 33,823 mGy cm2 [mean ± standard deviation] versus 100,704 ± 43,930 mGy cm2; p = 0.022). Procedural duration was 33.4 ± 16.1 min in group A versus 57.4 ± 14.9 min in group B (p = 0.062). Diaphragmatic excursion was significantly lower in group A (1.3 ± 0.4 mm) than in group B (19.7 ± 5.2 mm; p < 0.001) and lower with near statistical significance in group A with HFJV than without HFJV (1.3 ± 0.4 mm versus 10.9 ± 3.1 mm; p = 0.062). Technical and clinical success was 100% in both groups, without relevant complications. CONCLUSION: HFJV-assisted PAVM embolisation is a safe, feasible technique resulting in reduced patient radiation doses and procedural time.