Pulmonary Arteriovenous Malformations Research Articles

Successful treatment of bilateral multiple pulmonary arteriovenous malformations in a patient with brain abscess and severe hypoxemia using a combination of transcatheter embolotherapy and surgical resection: a case report

BackgroundA pulmonary arteriovenous malformation is an abnormal dilated blood vessel that makes direct communication between a pulmonary artery and pulmonary vein and can be associated with hypoxemia or neurological complications, including brain abscess and cerebral infarction. Treatment of pulmonary arteriovenous malformation includes surgical resection and transcatheter embolotherapy, however the adaptation of therapies should be considered when a patient is in bad condition.Case presentationA 51-year-old man was admitted after developing fever, consciousness disorder, and hypoxemia. Magnetic resonance imaging of the brain showed a brain abscess. Bilateral pulmonary arteriovenous malformations were found by contrast computed tomography. Because of a family history of pulmonary arteriovenous malformation, a history of epistaxis, and the existence of oral mucosa telangiectasia, he was diagnosed with hereditary hemorrhagic telangiectasia and brain abscess caused by intrapulmonary right-to-left shunt. The brain abscess improved with antibiotic treatment; however, the administration of oxygen did not ameliorate his hypoxemia. His hypoxemia was exacerbated by positive pressure ventilation. Considering his systemic and respiratory condition, we considered surgery to involve a high degree of risk. After controlling his brain abscess and pneumonia, transcatheter embolotherapy was performed. This improved his systemic condition, enabling surgical treatment.ConclusionsThis middle-aged patient suffering from brain abscess and severe hypoxemia with multiple pulmonary arteriovenous malformations was successfully treated by a combination of transcatheter embolotherapy and surgery. The adaptation and combination of therapies, as well as the sequence of treatments, should be considered depending on the patient status and lesions.

Pilot investigation of circulating angiogenic and inflammatory biomarkers associated with vascular malformations

BackgroundVascular malformations in the central nervous system are difficult to monitor and treat due to their inaccessible location. Angiogenic and inflammatory proteins are secreted into the bloodstream and may serve as useful biomarkers for identifying patients at risk for complications or with certain disease phenotypes.MethodsA validated multiplex protein array consisting of 26 angiogenic and inflammatory biomarkers (Angiome) was assessed in plasma isolated from healthy controls and patients with either sporadic brain arteriovenous malformation (BAVM), familial cerebral cavernous malformation (CCM), or hereditary hemorrhagic telangiectasia (HHT). These samples were obtained from archives of ongoing research studies at the University of California San Francisco and through prospective collection at the Toronto HHT Centre at St. Michael’s Hospital.ResultsWe compared circulating biomarker levels from each patient group to healthy controls and analyzed each pairwise combination of patient groups for differences in biomarker levels. Additionally, we analyzed the HHT samples to determine the association between biomarker levels and the following HHT-specific phenotypes, BAVM, pulmonary arteriovenous malformation (PAVM), liver vascular malformation (LVM), and gastrointestinal (GI) bleeding. Compared to controls, levels of SDF1 were significantly elevated in HHT patients (Proportional Increase [PI] = 1.87, p < 0.001, q = 0.011). Levels of sENG were significantly reduced in HHT patients compared to controls (PI = 0.56, p < 0.001, q < 0.001), reflecting the prevalence of HHT1 patients in this cohort. Levels of IL6 (PI = 3.22, p < 0.001, q < 0.001) and sTGFβR3 (PI = 0.70, p = 0.001, q < 0.029) differed significantly in CCM patients compared to controls. Compared to controls, ten of the biomarkers were significantly different in sporadic BAVM patients (q-values < 0.05). Among the pairwise combinations of patient groups, a significant elevation was observed in TGFβ1 in CCM patients compared to sporadic BAVM patients (PI = 2.30, p < 0.001, q = 0.034). When examining the association of circulating biomarker levels with HHT-specific phenotypes, four markers were significantly lower in HHT patients with BAVM (q-values < 0.05), and four markers were significantly higher in patients with LVM (q-values < 0.05).ConclusionsThis pilot study suggests that the profile of circulating angiogenic and inflammatory biomarkers may be unique to each type of vascular malformation. Furthermore, this study indicates that circulating biomarkers may be useful for assessing phenotypic traits of vascular malformations.

A Rare Case of Pulmonary Arteriovenous Malformation - A Diagnostic Dilemma

Pulmonary arteriovenous malformations (PAVM) are rare anomalies of pulmonary vascular system which may be incidentally detected or can have manifestations resulting from right to left shunt. PAVM can cause various systemic implications including central nervous system complications like stroke, brain abscess due to paradoxical emboli which mostly seen in previously undiagnosed PAVMs indicating importance of early diagnosis and timely intervention in PAVM. Computed Tomography Pulmonary Angiography (CTPA) is the diagnostic method of choice in PAVM. We present here a unique case of complex multiple PAVM presented with refractory hypoxemia in a middle aged patient diagnosed in our centre initially evaluated for malignancy based on chest radiography &amp; High Resolution Computed tomography(HRCT)thorax findings and symptoms, later on after CTPA was diagnosed to have complex PAVM which showed clinicoradiological improvement following pulmonary vascular plugging. This case shows clinical scenario, diagnostic &amp; management methods and differential diagnosis to be discussed in similar clinicoradiological pattern and peculiarity of PAVM despite advanced age.

Unusual Course of Pulmonary and Brain Arteriovenous Malformation: A Case Report

Introduction: Arteriovenous malformations (AVM) are rare chronic congenital diseases whose nature are not yet fully understood. Two different types of these disorders are pulmonary and brain malformations, which are seen sporadically or in combination with other syndromes. Symptoms of these diseases appear at different ages and present differently in patients, but in general, the combination of these two disorders is rarely seen. There are several treatments based on the type of lesion and symptoms and other accompanying conditions, one of which is interventional procedure. Case Presentation: In this article, a rare case of pulmonary and brain malformation has been reported, which has progressed even after treatment and has been closed interventionally. Conclusions: Patients with pulmonary AVMs need careful examination for other congenital disorders. Furthermore, due to the unknown nature of the disease and its progression, the treatment of these lesions is completely personal and varies according to each patient.

Massive haemorrhagic complications of ruptured pulmonary arteriovenous malformations: outcomes from a 12\xa0years\u2019 retrospective study

BackgroundThe life-threatening haemorrhagic complications of pulmonary arteriovenous malformations (PAVMs) are extremely rare, and only described in isolated cases. This study was designed to comprehensively investigate management of ruptured PAVMs.MethodsWe retrospectively assessed clinical and imaging data of ruptured PAVMs to summarize incidence, clinical characteristics, and outcomes following embolisation between January 2008 and January 2021.ResultsEighteen of 406 (4.4%) patients with PAVMs developed haemorrhagic complications. Twelve of 18 patients were clinically diagnosed with hereditary haemorrhagic telangiectasia (HHT). Haemorrhagic complications occurred with no clear trigger in all cases. Eight of 18 patients (44.4%) were initially misdiagnosed or had undergone early ineffective treatment. 28 lesions were detected, with 89.3% of them located in peripheral lung. Computed tomography angiography (CTA) showed indirect signs to indicate ruptured PAVMs in all cases. Lower haemoglobin concentrations were associated with the diameter of afferent arteries in the ruptured lesions. Successful embolotherapy was achieved in all cases. After embolotherapy, arterial oxygen saturation improved and bleeding was controlled (P < 0.05). The mean follow-up time was 3.2 ± 2.5 years (range, 7 months to 10 years).ConclusionsLife threatening haemorrhagic complications of PAVMs are rare, they usually occur without a trigger and can be easily misdiagnosed. HHT and larger size of afferent arteries are major risk factors of these complications. CTA is a useful tool for diagnosis and therapeutic guidance for ruptured PAVMs. Embolotherapy is an effective therapy for this life-threatening complication.

Applicability of Transthoracic Contrast Echocardiography for the Diagnosis and Treatment of Idiopathic Pulmonary Arteriovenous Malformations.

Objective: To explore the preferred test to screen for pulmonary arteriovenous malformations (PAVMs) and to predict the probability of interventional embolization.Methods: We performed a retrospective observational study evaluating patients with idiopathic PAVMs from 2009 to 2019. After clinical evaluation, a total of 105 patients were studied, including 71 patients with positive digital subtraction pulmonary angiography (DSPA) findings and 34 with negative DSPA findings. The following patient data were assessed: blood test, chest radiograph, transthoracic contrast echocardiography (TTCE), and DSPA findings.Results: The majority of patients with idiopathic PAVMs were female (66.2% with positive DSPA findings). We found a good κ-coefficient of 0.77 with strong consistency for inter observer agreement concerning the pulmonary right-to-left shunt (RLS) grade on TTCE, which was superior to conventional chest radiographs. The positive predictive value (PPV) of the radiographic features for PAVMs on DSPA was 0.83 (95% CI 0.64–1.0) and 0.44 for the possibility of embolization (95% CI 0.19–0.70). The PPV of the shunt grade of PAVMs on DSPA was 0.14 (95% CI 0.01–0.29) for grade 1, 0.74 (95% CI 0.60–0.88) for grade 2, and 0.97 (95% CI 0.92–1.0) for grade 3. The PPVs of pulmonary shunt grades 2 and 3 on TTCE for the possibility of embolization for PAVMs were 0.21 (95% CI, 0.05–0.36) and 0.87 (95% CI, 0.79–0.99), respectively.Conclusion: TTCE is the preferred screening test for PAVMs. The pulmonary RLS grade on TTCE not only identifies the likelihood of PAVMs but also predicts the probability for embolization.

Lifesaving Successful Embolization of Aggressive Vertebral Body Hemangioma and a Large Pulmonary Arteriovenous Malformation

Vertebral hemangiomas are incidental finding and most of the time do not necessitate immediate attention unless symptomatic. Vertebral hemangiomas can be very aggressive to the extent that they can produce cord compression and paraplegia/paraparesis. Such cases need evaluation with magnetic resonance imaging to assess the cause, extent, and mass effect on the spinal cord. Transcatheter embolization and decompression surgery are well established modes of treatment as single or combined approach. Pulmonary arteriovenous malformation (AVM) is caused by the direct communication of main pulmonary artery with pulmonary vein, leading to a high-flow right-to-left shunt. This usually presents clinically as hypoxia, cyanosis, and dyspnea. The clinical signs and symptoms vary depending on the size, number, and flow of fistula as well as the associated vascular malformations. Transcatheter embolization is a treatment of choice for pulmonary AVM. However, this method may result in incomplete resolution if the malformation is large in size or supplied by multiple feeding arteries. Surgical resection may be considered in those kinds of cases. As AVM can lead to hemodynamical instability, these lesions need to be addressed first. In this case, pulmonary AVM came to notice post surgical decompression. Here, we report a case of aggressive vertebral body hemangioma and a large pulmonary AVM in the same patient, which were treated by embolization in two different settings.

Traumatic Pulmonary Arteriovenous Malformation Presenting as Spontaneous Hemothorax

Pulmonary arteriovenous malformations (PAVM), also known as pulmonary arteriovenous fistulas, are abnormal connections between the pulmonary arterial and venous systems. The majority occur secondary to the congenital syndrome hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease. Trauma is an extremely rare etiology of PAVM, comprising less than 1% of all reported cases. Trauma can be associated with both immediate and delayed development of PAVM, and present similarly to PAVM associated with HHT. We report a case of a traumatic PAVM that developed in a patient one year following blunt thoracic trauma with a rib fracture. The patient subsequently developed a rupture of the PAVM, resulting in spontaneous hemothorax. She required multi-unit blood transfusion and multiple thoracostomy tube placements. The patient subsequently underwent a failed attempt at angioembolization of the PAVM. She eventually required a thoracotomy for surgical excision of the PAVM. We discuss the traumatic etiologies, clinical presentation, diagnostic assessments, and therapeutic modalities for the management of PAVM.

Pulmonary Arteriovenous Malformation Embolization: So Much Left to Learn

Embolization of pulmonary arteriovenous malformations (PAVMs) has been utilized effectively for more than 40 years. Initially performed using conventional coils and/or detachable balloons in the era of cut-film angiography, currently, there is an armamentarium of embolic devices and methods for effecting therapy. Two studies in this issue of the Journal of Vascular Interventional Radiology compare traditional feeding artery embolization with venous sac (“nidal”) embolization, and in both, sac embolization appeared to improve the occlusion rate of lesions (1,2).

SVEGFR1 Is Enriched in Hepatic Vein Blood-Evidence for a Provisional Hepatic Factor Candidate?

Background: Pulmonary arteriovenous malformations (PAVMs) are common sequelae of palliated univentricular congenital heart disease, yet their pathogenesis remain poorly defined. In this preliminary study, we used paired patient blood samples to identify potential hepatic factor candidates enriched in hepatic vein blood.Methods: Paired venous blood samples were collected from the hepatic vein (HV) and superior vena cava (SVC) from children 0 to 10 years with univentricular and biventricular congenital heart disease (n = 40). We used three independent protein analyses to identify proteomic differences between HV and SVC blood. Subsequently, we investigated the relevance of our quantified protein differences with human lung microvascular endothelial assays.Results: Two independent protein arrays (semi-quantitative immunoblot and quantitative array) identified that soluble vascular endothelial growth factor receptor 1 (sVEGFR1) is significantly elevated in HV serum compared to SVC serum. Using ELISA, we confirmed the previous findings that sVEGFR1 is enriched in HV serum (n = 24, p < 0.0001). Finally, we studied the quantified HV and SVC serum levels of sVEGFR1 in vitro. HV levels of sVEGFR1 decreased tip cell selection (p = 0.0482) and tube formation (fewer tubes [p = 0.0246], shorter tube length [p = 0.0300]) in vitro compared to SVC levels of sVEGFR1.Conclusions: Based on a small heterogenous cohort, sVEGFR1 is elevated in HV serum compared to paired SVC samples, and the mean sVEGFR1 concentrations in these two systemic veins cause pulmonary endothelial phenotypic differences in vitro. Further research is needed to determine whether sVEGFR1 has a direct role in pulmonary microvascular remodeling and PAVMs in patients with palliated univentricular congenital heart disease.

Brain abscess as the initial manifestation in a patient with idiopathic pulmonary arteriovenous malformation and suspected hereditary hemorrhagic telangiectasia: a case report

Brain abscess as the initial manifestation in a patient with idiopathic pulmonary arteriovenous malformation and suspected hereditary hemorrhagic telangiectasia: a case report

A Rare Case of Pulmonary Arteriovenous Malformation Caused by Hereditary Hemorrhagic Telangiectasia in a Hemodialysis Patient

Hereditary hemorrhagic telangiectasia (HHT) is an uncommon autosomal dominant disorder resulting in vascular malformation, such as pulmonary arteriovenous malformation (PAVM). Here, we report a rare case of pulmonary arteriovenous malformation caused by HHT in a hemodialysis (HD) patient. A 34-year-old man receiving maintenance HD via radiocephalic arteriovenous fistula developed progressive dyspnea without definite pulmonary edema. His mother had been diagnosed with HHT. He had experienced multiple episodes of epistaxis and had been intermittently treated with blood transfusions because of severe anemia. Blood gas analysis showed hypoxia. Chest computed tomography revealed multiple dilated vessels of variable sizes, continuous with the pulmonary artery throughout both lung fields, consistent with PAVM. After treating pulmonary artery embolization at the largest PAVM, he recovered from his dyspnea symptoms and hypoxia.

Diagnosis and endovascular management of pulmonary arteriovenous malformations.

Pulmonary arteriovenous malformations (PAVM) are abnormal communication of a branch of the pulmonary artery and pulmonary vein circumventing the intervening pulmonary capillaries. This results in a right-to-left (R-L) shunt and its related manifestations, which include hampered gas exchange leading to hypoxaemia, dyspnoea, paradoxical emboli leading to stroke, cerebral abscess, myocardial infarction and pulmonary haemorrhage due to rupture of the PAVM. Endovascular transcatheter embolization of the feeding vessels with coils or occlusion devices is the current standard care of treatment and preferred treatment modality. The articles aim to provide insights into the current trends in diagnosis, the current recommendations, approach and management options for patients with PAVM.

Abstract No. 218 Diagnostic accuracy of computed tomography angiogram chest in detecting pulmonary arteriovenous malformation: a comparison with invasive catheter pulmonary angiogram

To determine the diagnostic accuracy of computed tomography angiography (CTA) of the chest in the detection of pulmonary arteriovenous malformations (pAVM) compared to invasive pulmonary catheter angiogram of the chest both in HHT and non HHT patients. This retrospective study was approved by the institutional review board. A total of 341 pulmonary angiograms were performed for either embolization (treatment) of pAVM or for diagnosis of pAVM at our institution from January 2003 to June 2020. Two angiograms were excluded as they did not have prior CTA for pAVM. The remaining 339 pulmonary angiograms had an immediate prior CTA of the chest within a mean 7 ± 30 (SD) days prior to pulmonary angiogram. CTA was acquired after intravenous administration of contrast. The kV and mA were selected based on patient weight and ranges between 80 to 140. The mean fluoroscopy time was 33 ± 19 SD with range 2.4 to 120 minutes. Positive CTA of the chest was defined as the presence of pulmonary arteriovenous malformation with at least one well-defined feeding artery and a draining vein. A 2 × 2 contingency table was constructed and sensitivity, specificity, PPV, NPV, and accuracy were calculated. One hundred and eighty-five patients were included with mean age of 46 ± 19 SD, range 3 to 87 years with 68% of female. A total of 337 CTA of the chest were positive and 319 catheter pulmonary angiograms were positive for pulmonary AVM. There were 318 true positive, 19 false positives, 1 true negative, and 1 false negative. CTA angiogram chest demonstrated a sensitivity of 99.7%, a specificity of 5.0%, a positive predictive value of 94.4%, and a negative predictive value of 50.0%. Overall, the diagnostic accuracy of CTA angiogram chest was 94.10%. False positive CTA causes were catheter angiogram did not detect micro shunt (6/339), pulmonary vein to vein shunt (5/339), CTA wrong call for reperfusion (3/339), calcified granuloma (2/339), and tortuous or dilated pulmonary vessel (2/339). In the false negative CTA was not able to detect micro shunt (1/339). The limitation of our study is low specificity as those patients that have negative CTA angiogram chest do not proceed for catheter pulmonary angiogram unless they have other comorbidities and require CTA of the chest. Our result demonstrates that CTA of the chest is highly sensitive in detecting pulmonary arteriovenous malformation. This again highlights the importance of screening of pulmonary AVM in HHT patients.

Abstract No. 180 Global comparative analysis of cardiopulmonary parameters for patients with hereditary hemorrhagic telangiectasia with or without pulmonary arteriovenous malformation

Pulmonary arteriovenous malformations (PAVMs) are low-resistance, high-flow vascular structures that create a shunt in the pulmonary circulation. This shunting affects regional and systemic hemodynamics in Hereditary Hemorrhagic Telangiectasia (HHT). We hypothesized that this could lead to differential cardiac presentations in this setting. Thus, we investigated the cardiac structural and functional differences between HHT patients with PAVMs (HHT-P) compared to other presentations of HHT (HHT-O).

Abstract No. 64 Analysis of complications and unsuccessful embolization of pulmonary arteriovenous malformations

Embolotherapy is the standard treatment for pulmonary arteriovenous malformation (pAVM). The purpose of this study is to explore the reasons for unsuccessful embolization and complications associated with embolotherapy. This retrospective chart review study was approved by the IRB. Data were collected for all the embolization procedures that were performed at our institution for pulmonary AVM treatment between January 2003 and January 2020. All ages of both genders were included in the analysis. The reason for unsuccessful embolization and complications were recorded. One hundred and eighty-seven patients had a total of 341 embolization procedures at our institutions for the treatment of PAVM. There were 240 (70%) women and 101 (30%) men included with a mean age of 49 ± 18 and 38 ± 20 years, respectively. The average fluoroscopic time was 34 minutes (range 3.4 to 120 minutes). Of 341, 47(14%) procedures were unsuccessful. The reason for unsuccessful embolization were (in order of frequency): no PAVM detected on catheter angiogram (n = 17, 36%), pAVM too small to treat (n = 12, 25%), tortuous feeding artery causing technical difficulty assessing the artery (n = 7, 15%), No evidence of reperfusion on follow up pulmonary angiogram (that were done to detect reperfusion) (n = 6, 13%), systemic arterial supply to the pAVM causing vein to vein shunting (n = 3, 6%), patient’s condition (increased respiratory motion and intraprocedural bradycardia) aborting the procedure (n = 2, 4%). There were a total of 9 (2%) intraprocedural complications with 2 major complications aborting the embolotherapy. Embolization of pulmonary arteriovenous malformation is a very successful procedure with minor complications. The main reasons of unsuccessful embolotherapy are no evidence of pAVM during catheter angiogram, very small feeding artery size, and tortuous feeding artery.

Abstract No. 598 Clinical manifestations of patients with novel mutations associated with hereditary hemorrhagic telangiectasia and related vascular anomalies

In this series, 4 patients with HHT were identified with novel GDF2 mutations. They were compared to an individual with capillary malformation-arteriovenous malformation syndrome (CM-AVM) secondary to a RASA1 mutation to highlight the clinical similarity between the two diseases. With IRB exemption, the electronic medical record was searched with the search term HHT, genetic mutation, pulmonary arteriovenous malformation. The eligibility criteria included only those patients with a novel mutation in the GDF2 and RASA1 locus. The initial search resulted in 776 total patients with a suspected diagnosis of HHT. 162 patients had genetic testing done. We derived 5 patients with HHT-like clinical presentations found to have a novel (GDF2, n = 4) or related (RASA1, n = 1) mutation. 5 patients were included with a mean age of 17 years (range, < 1–37) with 60% female. 4 patients had a mutation or deletion in the GDF2 gene, and 1 patient had a RASA1 mutation and was diagnosed with CM-AVM. See Table. All GDF2 patients reported childhood nosebleeds, but only 1/ 4 patients continue to experience epistaxis by adulthood. 2/4 patients reported self-limited nosebleeds that resolved by adulthood. Mucocutaneous telangiectasia was observed only in 1/4 patients and in an atypical location. Unlike classic HHT, AVM and telangiectasia were not observed in the pulmonary or the hepatic vasculatures of GDF2 patients. 1 patient with a missense GDF2 mutation was found to have a hepatic focal nodular hyperplasia. The patient with CM-AVM also did not exhibit pulmonary or hepatic vascular involvement. GDF2 mutations predominately affected the cerebrovasculature. 2/4 GDF2 mutated patients suffered from brain vascular malformation. A third patient presented for evaluation after her daughter was diagnosed with a brain AVM. A frontal AVM and macrocephaly were diagnosed in the CM-AVM patient.3 patients with GDF2 mutations and one with RASA1 mutation were found to have some form of cardiac abnormalities. Clinical manifestations of HHT secondary to GDF2 mutations presented differently and exhibit overlapping features as CM-AVM due to RASA1 mutation.Tabled 1SexAge (years)DiagnosisGeneNucleotide ChangeProtein ChangeMutation TypeSignificanceF3CM-AVMRASA1c.2131C >Tp.Arg711TerNonsensePathogenicF37HHTGDF2c.1063G >Cp.Glu355GlnMissenseUnknownM32HHTGDF2c.1207G >Ap.Val403IleMissenseUnknownF13HHTGDF25.10-Mb deletion of 10q11.22 to 10q11.2336 genes deletedDeletionLikely pathogenicM0HHTGDF25.5-Mb deletion of 10q11.22 to 10q11.23UndeterminedDeletionLikely pathogenic Open table in a new tab

Abstract No. 65 Early experience with a foam-based embolic plug (IMPEDE) for pulmonary arteriovenous malformations

Abstract No. 65 Early experience with a foam-based embolic plug (IMPEDE) for pulmonary arteriovenous malformations

Abstract No. 66 Predictors of reperfusion in treated pulmonary arteriovenous malformation: a retrospective single-center study

Abstract No. 66 Predictors of reperfusion in treated pulmonary arteriovenous malformation: a retrospective single-center study

Impact of bilateral bidirectional Glenn anastomosis on staged Fontan strategy and Fontan circulation.

The aim of this study was to identify the impact of bilateral bidirectional Glenn (BBDG) anastomosis on staged Fontan strategy and late Fontan circulation. Of 267 patients who underwent bidirectional Glenn prior to Fontan completion between 1989 and 2013, 62 patients (23%) who underwent BBDG were enrolled in this study. Age at operation was 0.84 years (25th-75th percentile: 0.58-1.39). Thirty-three patients had heterotaxy syndrome (53%). The mean follow-up period was 12.7 ± 8.1 (max. 30.6) years. The overall survival rate at 15 years was 73%. Although 49 patients (79.0%) went on to Fontan completion, 12 patients (19.4%) died without achieving it. Thrombus formation and poor development in a central pulmonary artery were not observed, but obstruction of the superior vena cava (SVC) occurred in 8 patients (13%), mainly those with right atrial isomerism (P = 0.037). SVC obstruction was not, however, a risk factor for mortality (P = 0.097) or Fontan completion (P = 0.41). The shape of BBDG anastomosis, symmetricity of pulmonary blood flow, impingement of caval blood flow returning from the superior and inferior vena cavae or coexisting interrupted inferior vena cava with azygos or hemi-azygos continuation did not affect late Fontan outcomes, such as overall survival, freedom from protein-losing enteropathy or pulmonary arterio-venous malformation rates. SVC obstruction after BBDG frequently occurred, mainly in patients with right atrial isomerism; however, its direct impact on prognosis or achieving Fontan completion was not identified. Once Fontan circulation was established, the arrangement of the Fontan pathway did not affect late Fontan outcomes.