RESUMO
Paediatric neurovascular anomalies associated with the vein of Galen (VG) comprise of a spectrum of rare, complex, and life-threatening conditions. In this group, the "vein of Galen aneurysmal dilatation" (VGAD) is a distinct entity that often presents with progressive neurological symptoms in older children. Acute haemorrhage in VGAD is uncommon. We present an unusual presentation of VGAD in a neonate and discuss the challenges faced in the management.
Assuntos
Hemorragias Intracranianas , Malformações da Veia de Galeno , Humanos , Recém-Nascido , Hemorragias Intracranianas/diagnóstico por imagem , Hemorragias Intracranianas/etiologia , Malformações da Veia de Galeno/complicações , Malformações da Veia de Galeno/diagnóstico por imagem , Masculino , Veias Cerebrais/diagnóstico por imagem , Dilatação Patológica/diagnóstico por imagem , FemininoRESUMO
Vein of Galen malformations (VGAMs) are rare and complex congenital brain vascular anomalies that pose significant diagnostic and treatment challenges. The natural history of this type of vascular anomaly is very poor, with many patients succumbing to complications such as congestive heart failure, hydrocephalus, and brain parenchymal injury. Although the clinical course of most VGAMs was considered unfortunate, with meticulous imaging, a group of lesions with a more placid presentation and course can be identified.
Assuntos
Malformações da Veia de Galeno , Humanos , Veias Cerebrais/anormalidades , Veias Cerebrais/diagnóstico por imagem , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/complicaçõesRESUMO
Significant efforts have been made over the last few decades to improve the diagnosis and management of patients with vein of Galen malformations (VOGMs). The mainstays of treatment remain focused on primary endovascular management by staged transarterial embolizations with adjunctive use of transvenous embolization, medical therapy, and neurosurgical intervention for symptom control in select patients. Innovation in endovascular technology and techniques as well as promising new genomic research elucidating potential therapeutic targets hold significant promise for the future of VOGM treatment.
Assuntos
Embolização Terapêutica , Malformações da Veia de Galeno , Humanos , Embolização Terapêutica/métodos , Procedimentos Endovasculares/métodos , Procedimentos Neurocirúrgicos/métodos , Malformações da Veia de Galeno/terapia , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/cirurgiaRESUMO
PURPOSE: To validate a semiautomated method for segmenting vein of Galen aneurysmal malformations (VGAM) and to assess the relationship between VGAM volume and other angioarchitectural features, cardiological findings, and outcomes. METHODS: In this retrospective study, we selected all subjects with VGAM admitted to the Gaslini Children's Hospital between 2009 and 2022. Clinical data were retrieved from electronic charts. We compared 3D-Slicer segmented VGAM volumes obtained by two independent observers using phase-contrast MR venography to those obtained with manual measurements performed on T2-weighted images. The relationship between VGAM volumes and clinical and neuroimaging features was then explored. RESULTS: Forty-three subjects with VGAM (22 males, mean age 6.56 days) were included in the study. Manual and semiautomated VGAM volumes were well correlated for both readers (r = 0.86 and 0.82, respectively). Regarding reproducibility, the inter-rater interclass correlation coefficients were 0.885 for the manual method and 0.992 for the semiautomated method (p < 0.001). The standard error for repeated measures was lower for the semiautomated method (0.04 versus 0.40 of manual method). Higher VGAM volume was associated with superior sagittal sinus narrowing, jugular bulb stenosis, and aqueductal stenosis (p < 0.05). A weak correlation was found between VGAM volume and straight sinus dilatation (r = 0.331) and superior sagittal sinus index (r = - 0.325). No significant associations were found with cardiac findings, post-embolization complications, and outcome (p > 0.05). CONCLUSIONS: Semiautomated VGAM volumetry is feasible and reliable with improved reproducibility compared to the manual method. VGAM volume is not a prognostic factor for clinical outcome, but it is related to other venous findings with potential hemodynamic effects.
Assuntos
Angiografia por Ressonância Magnética , Malformações da Veia de Galeno , Humanos , Masculino , Feminino , Estudos Retrospectivos , Malformações da Veia de Galeno/diagnóstico por imagem , Angiografia por Ressonância Magnética/métodos , Reprodutibilidade dos Testes , Recém-Nascido , Lactente , Imageamento Tridimensional/métodos , Veias Cerebrais/diagnóstico por imagem , Veias Cerebrais/anormalidadesRESUMO
INTRODUCTION: Vein of Galen aneurysmal malformation (VGAM) is a rare, congenital cerebrovascular malformation with high morbidity and mortality. Parameters to foresee clinical progression and allow individualized parent counseling are lacking. The aim of this study was to evaluate aortic steal measured by Doppler ultrasound as a prognostic parameter in these neonates. METHODS: A retrospective monocentric analysis of cardiac ultrasound exams before embolization in neonates with VGAM was conducted. Percentage of aortic steal measured by time-averaged maximum velocity above and below the zero flow baseline by pulsed Doppler ultrasound at the preductal aortic isthmus was calculated. Association of aortic steal with parameters of acute organ dysfunction (Bicêtre neonatal evaluation score [BNES], neonatal multiple organ dysfunction score [NeoMODS]) and mortality and determination of correlation between aortic steal and cerebral damage on initial and follow-up cerebral magnetic resonance imaging (cMRI) were evaluated. RESULTS: Twelve neonates were included, of which 3 died. Per 10 percentage point increase of aortic steal, BNES decreased by 1.64 (95% confidence interval [CI]: 1.28-2.0) points and the maximum observed NeoMODS increased by 1.25 (CI: 0.94-1.57) points. The odds for mortality increased by 2.3 (CI: 1.14-13.67) per 10 percentage point increase of aortic steal. There was a correlation between aortic steal and cerebral damage at baseline (white matter ρ [rho] = 0.34, gray matter ρ = 0.81) and follow-up (white matter ρ = 0.80, gray matter ρ = 0.72). CONCLUSION: The degree of aortic steal in neonates with VGAM was highly associated with the severity of organ dysfunction, disease progression on cMRI, and mortality.
Assuntos
Malformações da Veia de Galeno , Recém-Nascido , Humanos , Malformações da Veia de Galeno/complicações , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/terapia , Estudos Retrospectivos , Insuficiência de Múltiplos Órgãos/etiologia , UltrassonografiaRESUMO
To elucidate the pathogenesis of vein of Galen malformations (VOGMs), the most common and most severe of congenital brain arteriovenous malformations, we performed an integrated analysis of 310 VOGM proband-family exomes and 336,326 human cerebrovasculature single-cell transcriptomes. We found the Ras suppressor p120 RasGAP (RASA1) harbored a genome-wide significant burden of loss-of-function de novo variants (2042.5-fold, p = 4.79 x 10-7). Rare, damaging transmitted variants were enriched in Ephrin receptor-B4 (EPHB4) (17.5-fold, p = 1.22 x 10-5), which cooperates with p120 RasGAP to regulate vascular development. Additional probands had damaging variants in ACVRL1, NOTCH1, ITGB1, and PTPN11. ACVRL1 variants were also identified in a multi-generational VOGM pedigree. Integrative genomic analysis defined developing endothelial cells as a likely spatio-temporal locus of VOGM pathophysiology. Mice expressing a VOGM-specific EPHB4 kinase-domain missense variant (Phe867Leu) exhibited disrupted developmental angiogenesis and impaired hierarchical development of arterial-capillary-venous networks, but only in the presence of a "second-hit" allele. These results illuminate human arterio-venous development and VOGM pathobiology and have implications for patients and their families.
Assuntos
Doenças Vasculares , Malformações da Veia de Galeno , Humanos , Animais , Camundongos , Malformações da Veia de Galeno/genética , Malformações da Veia de Galeno/patologia , Células Endoteliais/patologia , Mutação , Transdução de Sinais/genética , Mutação de Sentido Incorreto , Proteínas Ativadoras de GTPase/genética , Receptores de Activinas Tipo II/genética , Proteína p120 Ativadora de GTPase/genéticaRESUMO
BACKGROUND AND IMPORTANCE: Vein of Galen malformations (VOGMs) are complex arteriovenous malformations in neonates and young children. Recent advances in endovascular interventions have drastically improved treatment and clinical outcomes in what was previously high-morbidity, high-mortality disease. The high-flow shunt pathophysiology in VOGMs can lead to dynamic changes in the malformation angioarchitecture, and over time patients can develop jugular bulb stenosis. In the setting of inaccessible transvenous access to the malformation for endovascular embolization in cases where transarterial embolization is inadequate, a combined surgical and endovascular technique must be used. We present the first successful modern-day application of direct puncture through transverse sinus for transvenous embolization of a VOGM. CLINICAL PRESENTATION: We present 2 unique cases of complex VOGM malformations in patients who had previously undergone staged endovascular embolization for reduction of flow within the malformation. On follow-up, in both cases, there was development of severe sigmoid sinus and jugular bulb stenosis, increasing intracranial venous congestion and causing marked clinical deterioration. The stenosis prevented traditional transvenous access and treatment. We describe a direct puncture transverse sinus access using a burr hole approach for endovascular transvenous embolization in both cases with successful clinical outcomes. CONCLUSION: Direct access using burr hole craniotomy to the transverse sinus for transvenous endovascular embolization is a safe approach in the setting of severe jugular bulb stenosis for treatment of VOGMs. This technique can be done efficiently to achieve complete flow elimination in the malformation, in cases where that is called for, without significant risks or complications related to the approach.
Assuntos
Embolização Terapêutica , Malformações da Veia de Galeno , Recém-Nascido , Criança , Humanos , Pré-Escolar , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/terapia , Constrição Patológica , Angiografia Cerebral , Embolização Terapêutica/métodos , PunçõesAssuntos
Malformações Arteriovenosas Intracranianas , Malformações da Veia de Galeno , Humanos , Criança , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/cirurgia , Malformações Arteriovenosas Intracranianas/diagnóstico por imagem , Malformações Arteriovenosas Intracranianas/cirurgiaAssuntos
Malformações Arteriovenosas Intracranianas , Malformações da Veia de Galeno , Humanos , Criança , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/cirurgia , Malformações Arteriovenosas Intracranianas/diagnóstico por imagem , Malformações Arteriovenosas Intracranianas/cirurgiaRESUMO
INTRODUCTION: Pediatric cerebrovascular lesions are very rare and include aneurysms, arteriovenous malformations (AVM), and vein of Galen malformations (VOGM). OBJECTIVE: To describe and disseminate a validated, reproducible set of 3D models for optimization of neurosurgical training with respect to pediatric cerebrovascular diseases METHODS: All pediatric cerebrovascular lesions treated at our institution with adequate imaging studies during the study period 2015-2020 were reviewed by the study team. Three major diagnostic groups were identified: aneurysm, AVM, and VOGM. For each group, a case deemed highly illustrative of the core diagnostic and therapeutic principles was selected by the lead and senior investigators for printing (CSG/JM). Files for model reproduction and free distribution were prepared for inclusion as Supplemental Materials. RESULTS: Representative cases included a 7-month-old female with a giant left MCA aneurysm; a 3-day-old male with a large, complex, high-flow, choroidal-type VOGM, supplied from bilateral thalamic, choroidal, and pericallosal perforators, with drainage into a large prosencephalic vein; and a 7-year-old male with a left frontal AVM with one feeding arterial vessel from the anterior cerebral artery and one single draining vein into the superior sagittal sinus CONCLUSION: Pediatric cerebrovascular lesions are representative of rare but important neurosurgical diseases that require creative approaches for training optimization. As these lesions are quite rare, 3D-printed models and open source educational materials may provide a meaningful avenue for impactful clinical teaching with respect to a wide swath of uncommon or unusual neurosurgical diseases.
Assuntos
Malformações Arteriovenosas , Aneurisma Intracraniano , Malformações Arteriovenosas Intracranianas , Malformações da Veia de Galeno , Humanos , Criança , Masculino , Feminino , Lactente , Malformações da Veia de Galeno/cirurgia , Artéria Cerebral Anterior , Aneurisma Intracraniano/diagnóstico por imagem , Aneurisma Intracraniano/cirurgia , Impressão Tridimensional , Malformações Arteriovenosas Intracranianas/diagnóstico por imagem , Malformações Arteriovenosas Intracranianas/cirurgiaAssuntos
Veias Cerebrais , Embolização Terapêutica , Insuficiência Cardíaca , Malformações da Veia de Galeno , Humanos , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/terapia , Veias Cerebrais/diagnóstico por imagem , Insuficiência Cardíaca/terapia , Ultrassonografia , Ultrassonografia de IntervençãoRESUMO
INTRODUCTION: Vein of Galen malformation (VOGM) is an exceptionally uncommon form of congenital intracranial vascular malformations. It is highly unusual for this lesion to spontaneously thrombose. The clinical presentation of a patient may range from being asymptomatic to critically ill. The underlying pathophysiological mechanisms that cause spontaneous thrombosis are still poorly understood. METHODS AND RESULTS: The literature on spontaneous thrombosis of VOGM was systematically reviewed, analyzed, and summarized with a focus on its pathophysiology, types, clinical presentations, diagnosis, management, and outcomes. It was also illustrated with a case presentation. The case presents an unusual presentation and location of a VOGM in a 2-year-old boy who was successfully treated with surgical resection. CONCLUSIONS: A handful of cases of thrombosed VOGM have been reported worldwide where surgery was used to treat the condition. Low-flow fistulas of the mural type are prone to spontaneous thrombosis, have delayed clinical presentations, and are typically diagnosed in young children. Among the many possible manifestations, hydrocephalus is by far the most common. In the absence of blood flow, MRI is the diagnostic test of choice. Depending on the patient's symptoms, surgery to either remove the aneurysm or divert the cerebrospinal fluid usually results in a good prognosis.
Assuntos
Veias Cerebrais , Trombose , Malformações da Veia de Galeno , Criança , Masculino , Humanos , Pré-Escolar , Malformações da Veia de Galeno/complicações , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/cirurgia , Veias Cerebrais/diagnóstico por imagem , Veias Cerebrais/cirurgia , Veias Cerebrais/patologia , Imageamento por Ressonância Magnética , Angiografia CerebralRESUMO
BACKGROUND AND PURPOSE: Vein of Galen malformation is a rare congenital cerebrovascular malformation. In affected patients, increased cerebral venous pressure constitutes an important etiologic factor for the development of brain parenchymal damage. The aim of this study was to investigate the potential of serial cerebral venous Doppler measurements to detect and monitor increased cerebral venous pressure. MATERIALS AND METHODS: This was a retrospective monocentric analysis of ultrasound examinations within the first 9 months of life in patients with vein of Galen malformation admitted at <28 days of life. Categorization of perfusion waveforms in the superficial cerebral sinus and veins into 6 patterns was based on antero- and retrograde flow components. We performed an analysis of flow profiles across time and correlation with disease severity, clinical interventions, and congestion damage on cerebral MR imaging. RESULTS: The study included 44 Doppler ultrasound examinations of the superior sagittal sinus and 36 examinations of the cortical veins from 7 patients. Doppler flow profiles before interventional therapy correlated with disease severity determined by the Bicêtre Neonatal Evaluation Score (Spearman ρ = -0.97, P = < .001). At this time, 4 of 7 patients (57.1%) showed a retrograde flow component in the superior sagittal sinus, whereas after embolization, none of the 6 treated patients presented with a retrograde flow component. Only patients with a high retrograde flow component (equal or more than one-third retrograde flow, n = 2) showed severe venous congestion damage on cerebral MR imaging. CONCLUSIONS: Flow profiles in the superficial cerebral sinus and veins appear to be a useful tool to noninvasively detect and monitor cerebral venous congestion in vein of Galen malformation.
Assuntos
Veias Cerebrais , Hiperemia , Malformações da Veia de Galeno , Recém-Nascido , Humanos , Seio Sagital Superior/diagnóstico por imagem , Malformações da Veia de Galeno/complicações , Malformações da Veia de Galeno/diagnóstico por imagem , Estudos Retrospectivos , Veias Cerebrais/diagnóstico por imagem , Veias Cerebrais/anormalidades , Ultrassonografia DopplerRESUMO
OBJECTIVE: To assess the extent and resolution of pulmonary hypertension (PH), cardiovascular factors, and echocardiographic findings associated with mortality in infants and children with vein of Galen malformation (VOGM). STUDY DESIGN: We performed a retrospective review of 49 consecutive children with VOGM admitted to Boston Children's Hospital from 2007 to 2020. Patient characteristics, echocardiographic data, and hospital course were analyzed for 2 cohorts based on age at presentation to Boston Children's Hospital: group 1 (age ≤60 days) or group 2 (age >60 days). RESULTS: Overall hospital survival was 35 of 49 (71.4%); 13 of 26 (50%) in group 1 and 22 of 23 (96%) in group 2 (P < .001). High-output PH (P = .01), cardiomegaly (P = .011), intubation (P = .019), and dopamine use (P = .01) were significantly more common in group 1 than group 2. Among patients in group 1, congestive heart failure (P = .015), intubation (P < .001), use of inhaled nitric oxide (P = .015) or prostaglandin E1 (P = .030), suprasystemic PH (P = .003), and right-sided dilation were significantly associated with mortality; in contrast, left ventricular volume and function, structural congenital heart disease, and supraventricular tachycardia were not associated. Inhaled nitric oxide achieved no clinical benefit in 9 of 11 treated patients. Resolution of PH was associated with overall survival (P < .001). CONCLUSIONS: VOGM remains associated with substantial mortality among infants presenting at ≤60 days of life owing to factors associated with high output PH. Resolution of PH is an indicator associated with survival and a surrogate end point for benchmarking outcomes.
Assuntos
Hipertensão Pulmonar , Malformações da Veia de Galeno , Humanos , Lactente , Criança , Recém-Nascido , Hipertensão Pulmonar/complicações , Malformações da Veia de Galeno/complicações , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/terapia , Óxido Nítrico , VeiasRESUMO
OBJECTIVE: Vein of Galen Malformations (VoGM) are rare vascular malformations, typically seen in pediatric age groups. Even more rarely, VoGM's may be seen later in adulthood. In this case report and systematic review, we provide a thorough description of the current literature as well as provide a case example exploring the diagnosis, imaging, treatment, and management of VoGM in adults. METHODS: In accordance with PRISMA guidelines, we performed a systematic literature search for all relevant cases and case series of VoGM in adult patients. The reference list of all articles were reviewed for additional relevant cases. Articles were included if they described a VoGM of a patient over the age of 18 years old and published in English. 149 articles were originally identified and 26 described cases met our inclusion criteria. RESULTS: In our literature review we found 26 patients that met our inclusion criteria. We found 14 male patients and 12 female patients. The mean age at presentation was 37.2 years (median=34 years, SD= 13.6 years). The most common presenting symptoms of patient were headache (n = 9), seizure (n = 6), and vomiting (n = 4). Of the 12 cases which clearly reported the subtype of VoGM, the choroidal type was more frequently seen (n = 10) compared to the mural type (n = 2). In 3 patients, the VoGM was thrombosed at time of diagnosis. Of the 26 patients, endovascular treatment was performed most frequently (n = 8) but some received microsurgical treatment (n = 4) or were treated conservatively (n = 6). Other treatment modalities included (ventriculoperitoneal shunt, ventriculostomy) (n = 5). In 3 cases treatment was not specified. In comparison to VoGM seen in pediatric or neonatal populations, VoGM in adults generally resulted in more favorable outcomes with only 2 patients reported to have passed away following treatment. CONCLUSION: VoGM remains a rare finding amongst the adult population. Hence, we described the clinical presentation, treatment modalities, and outcomes of the cases described in the English literature. Perhaps due to the rate of thrombosis and the unique angioarchitecture seen in adult VoGM patients, outcomes were generally more favorable than those described in the literature in pediatric or neonate VoGM patients.
Assuntos
Embolização Terapêutica , Malformações da Veia de Galeno , Adulto , Criança , Feminino , Humanos , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Deficiências do Desenvolvimento , Embolização Terapêutica/métodos , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/terapia , Derivação Ventriculoperitoneal , VentriculostomiaRESUMO
BACKGROUND: Vein of Galen malformation (VOGM) is a rare, life-threatening vascular malformation in neonates and is treated with embolization. However, even at the most experienced centers, patients face high mortality and morbidity. In utero treatment options have been limited by lack of animal models or simulations. OBJECTIVE: To create a novel ultrasound phantom simulator for a preclinical feasibility study of in utero fetal intervention for VOGM. METHODS: Novel phantoms were designed and built in two configurations of spherical and windsock shape from cryogel material to mimic the salient vasculature of the fetal VOGM, based on real-patient fetal MR imaging dimensions. Critical anatomy was realistically mimicked within this model and transtorcular ultrasound-guided coil deployment was simulated. Each phantom model was assessed before and after treatment to evaluate coil mass deposition within the target. RESULTS: The two phantoms underwent pretreatment T2-weighted MR imaging assessment, ultrasound-guided embolization, post-treatment MR and fluoroscopic imaging, and visual inspection of the sliced phantoms for target embolization verification. Postoperative MR scans confirmed realistic compact deposition of the coil masses within the central cavity. Phantom embolization results were submitted as part of the institutional review board and US Food and Drug Administration investigative device exemption approval for a first-in-humans clinical trial of fetal intervention for VOGM. CONCLUSIONS: A phantom simulator for fetal intervention of VOGM produces lifelike results during trial interventions, removing obstacles to feasibility and safety evaluations, typically precluded by non-availability of appropriate animal models. The study provides a proof of concept for potentially wider applications of medical simulation to enable novel procedural advancements in neurointerventions.
Assuntos
Treinamento com Simulação de Alta Fidelidade , Doenças Vasculares , Malformações Vasculares , Malformações da Veia de Galeno , Humanos , Comitês de Ética em Pesquisa , Imageamento por Ressonância Magnética , Estados Unidos , Malformações da Veia de Galeno/terapia , Malformações da Veia de Galeno/cirurgia , Ensaios Clínicos como Assunto , Feminino , GravidezRESUMO
Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM.
Assuntos
Veias Cerebrais , Insuficiência Cardíaca , Hidrocefalia , Hipertensão Pulmonar , Malformações da Veia de Galeno , Recém-Nascido , Humanos , Veia Cava Superior/diagnóstico por imagem , Veia Cava Superior/anormalidades , Hipertensão Pulmonar/complicações , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/anormalidades , Ecocardiografia , Malformações da Veia de Galeno/complicações , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/terapia , Insuficiência Cardíaca/terapia , Insuficiência Cardíaca/complicações , Hidrocefalia/complicaçõesRESUMO
Background: Mural type vein of Galen malformation (mVOGM) is a congenital high flow arteriovenous shunt between choroidal arteries and the prosencephalic vein of Markowski leading to heart failure and hydrovenous disorder in children. Embolizing fistulous connections can be challenging and typically requires adjunctive techniques such as induced hypotension, balloon-assisted flow control, and creation of a coil basket. These maneuvers add time, complexity, and unpredictability. Rapid ventricular pacing (RVP) has been proposed as an alternative strategy with fewer drawbacks, but has not been well studied. The approach involves catheterizing the right ventricle with a pacing catheter connected to a temporary external pacemaker. Prior to embolization, RVP is initiated to lower cardiac output. Following embolization, pacing is discontinued, and the heart returns to sinus rhythm. Methods: We performed RVP in five mVOGM patients from 4/2020 through 7/2021. Accounting for multiple procedures, RVP was utilized in ten cases and twenty-six pedicles. Results: Ventricular capture was achieved in all instances and was well tolerated, without arrhythmia. Casting the arterial pedicle with liquid embolic immediately adjacent to, or traversing, the fistulous point was achieved in 9/10 cases. There were no procedural complications. In 1 case, creation of a coil basket in the venous pouch was required to achieve a stable arterial cast Conclusions: This report describes the largest case series utilizing RVP in mVOGM. The technique appears safe and well tolerated.