Fistula from Congenital Subclavian Artery to Azygos Vein with Congestive Heart Failure in an Adult.

Congenital arteriovenous communications arising from subclavian artery are very rare. We report the first case of multiple congenital arteriovenous fistulas between the right subclavian artery and azygos vein presenting with congestive heart failure.

Portal hypertension in patients with cirrhosis: indirect assessment of hepatic venous pressure gradient by measuring azygos flow with 2D-cine phase-contrast magnetic resonance imaging.

OBJECTIVES: To measure azygos, portal and aortic flow by two-dimensional cine phase-contrast magnetic resonance imaging (2D-cine PC MRI), and to compare the MRI values to hepatic venous pressure gradient (HVPG) measurements, in patients with cirrhosis. METHODS: Sixty-nine patients with cirrhosis were prospectively included. All patients underwent HVPG measurements, upper gastrointestinal endoscopy and 2D-cine PC MRI measurements of azygos, portal and aortic blood flow. Univariate and multivariate regression analyses were used to evaluate the correlation between the blood flow and HVPG. The performance of 2D-cine PC MRI to diagnose severe portal hypertension (HVPG ≥ 16 mmHg) was determined by receiver operating characteristic curve (ROC) analysis, and area under the curves (AUC) were compared. RESULTS: Azygos and aortic flow values were associated with HVPG in univariate linear regression model. Azygos flow (p < 10(-3)), aortic flow (p = 0.001), age (p = 0.001) and presence of varices (p < 10(-3)) were independently associated with HVPG. Azygos flow (AUC = 0.96 (95 % CI [0.91-1.00]) had significantly higher AUC than aortic (AUC = 0.64 (95 % CI [0.51-0.77]) or portal blood flow (AUC = 0.40 (95 % CI [0.25-0.54]). CONCLUSIONS: 2D-cine PC MRI is a promising technique to evaluate significant portal hypertension in patients with cirrhosis. KEY POINTS: • Noninvasive HVPG assessment can be performed with MRI azygos flow. • Azygos MRI flow is an easy-to-measure marker to detect significant portal hypertension. • MRI flow is more specific that varice grade to detect portal hypertension.

Leaving antegrade pulmonary blood flow results in reversal of flow in the azygous vein and decompression of the cavopulmonary circulation: mechanism of cyanosis defined with cardiac magnetic resonance imaging.

We report the case of a 2.5-year-old patient with single-ventricle physiology who underwent cardiac magnetic resonance study for cyanosis after Kawashima operation. Magnetic resonance imaging study showed a veno-venous collateral redirecting systemic venous flow, responsible for cyanosis.

Percutaneous embolization of a giant collateral vessel originating from the azygos vein via the inferior vena cava.

We present the case of a 45-year-old man with univentricular heart, small outlet chamber, and L-transposition of the great vessels. As an infant, the patient underwent multiple palliative surgical interventions. He presented with worsening dyspnea and fatigue and was found to have systemic oxygen saturation of 85% on 2 L of oxygen by nasal cannula, whereas he had chronically remained between 90 and 95% throughout most of adulthood. There was no evidence of significant valvular regurgitation or stenosis, nor was there an overt intracardiac shunt by echocardiography. Cardiac CT and cardiac MRI revealed a large serpiginous systemic to pulmonary venovenous collateral located behind the left atrium. The collateral drained into the lower right pulmonary vein as it entered the left atrium. The tributary veins to the "giant" collateral were determined by these images modalities. The patient underwent a percutaneous embolization of this giant venovenous collateral via a remnant supracardinal vein originating from the infrarenal inferior vena cava using two Amplatzer Vascular Plug II. Immediately after the procedure the patient's oxygen saturation increased to 90% on room air at rest. At 2 months follow-up the patient had a marked clinical improvement with oxygen saturation as high as 95% on room air while walking. Our case illustrates a successful embolization of a giant collateral via an embryological venous remnant connecting the IVC to the azygos system.

Transhepatic approach to create stent fenestration in the extracardiac Fontan conduit in a child with dextrocardia and interrupted inferior vena cava with azygos continuation.

Plastic bronchitis is a rare life-threatening complication of Fontan operation. When medical treatment is ineffective in the setting of high systemic venous pressures, Fontan fenestration may be considered to decompress venous pressures and improve cardiac output by creation of the right-to-left shunting. However, transcatheter approach can be difficult in patients with complex venous anatomy. We report a 4-year-old girl born with hypoplastic left ventricle and heterotaxy syndrome, who developed plastic bronchitis following extracardiac Fontan procedure. Her venous anatomy was complex with dextrocardia and interrupted inferior vena cava with azygos continuation. Stent fenestration was successfully performed via transhepatic approach, which was selected based on the anatomical relationship (between extracardiac conduit, left atrium, and hepatic veins) delineated by pre-catheterization cardiac MRI. Simultaneous transesophageal echocardiography guided the intervention. Her plastic bronchitis improved significantly in 3 months but slowly progressed after the stent fenestration. At her 8-month follow-up, stent fenestration remains open and she is currently under heart transplantation evaluation due to persistent plastic bronchitis. Treatment of plastic bronchitis can be undertaken with Fontan fenestration, with pre-procedural MRI playing an essential role in patients with complex venous anatomy.

Venous angioplasty in multiple sclerosis: neurological outcome at two years in a cohort of relapsing-remitting patients.

An open study was conducted with the aim of reporting long-term clinical outcome of endovascular treatment for chronic cerebrospinal venous insufficiency (CCSVI) in patients with multiple sclerosis (MS). Twenty-nine patients with clinically definite relapsing-remitting MS underwent percutaneous transluminal angioplasty for CCSVI, outside a clinical relapse. All the patients were regularly observed over at least two years before the first endovascular treatment and for at least two years after it (mean post-procedure follow up 30.6±6.1 months). The following clinical outcome measures were used: annual relapse rate and Expanded Disability Status Scale (EDSS) score. All the patients were observed intensively (mean 6 hours) on the day of the endovascular treatment to monitor for possible complications (bleeding, shock, heart attack, death). We compared the annual relapse rate before and after treatment (in the two years before and the two years after the first endovascular treatment) and the EDSS score recorded two years before versus two years after the treatment. Overall, 44 endovascular procedures were performed in the 29 patients, without complications. Thirteen of the 29 patients (45%) underwent more than one treatment session because of venous re-stenosis: 11 and two patients underwent two and three endovascular treatments respectively. The annual relapse rate of MS was significantly lower post-procedure (0.45±0.62 vs 0.76±0.99; p=0.021), although it increased in four patients. The EDSS score two years after treatment was significantly lower compared to the EDSS score recorded at the examination two years before treatment (1.98±0.92 vs 2.27±0.93; p=0.037), although it was higher in four patients. Endovascular treatment of concurrent CCSVI seems to be safe and repeatable and may reduce annual relapse rates and cumulative disability in patients with relapsing-remitting MS. Randomized controlled studies are needed to further assess the clinical effects of endovascular treatment of CCSVI in MS.

Chronic cerebrospinal venous insufficiency and multiple sclerosis.

A chronic state of impaired venous drainage from the central nervous system, termed chronic cerebrospinal venous insufficiency (CCSVI), is claimed to be a pathologic phenomenon exclusively seen in multiple sclerosis (MS). This has invigorated the causal debate of MS and generated immense interest in the patient and scientific communities. A potential shift in the treatment paradigm of MS involving endovascular balloon angioplasty or venous stent placement has been proposed as well as conducted in small patient series. In some cases, it may have resulted in serious injury. In this Point of View, we discuss the recent investigations that led to the description of CCSVI as well as the conceptual and technical shortcomings that challenge the potential relationship of this phenomenon to MS. The need for conducting carefully designed and rigorously controlled studies to investigate CCVSI has been recognized by the scientific bodies engaged in MS research. Several scientific endeavors examining the presence of CCSVI in MS are being undertaken. At present, invasive and potentially dangerous endovascular procedures as therapy for patients with MS should be discouraged until such studies have been completed, analyzed, and debated in the scientific arena.

Superficial and deep vein thrombosis associated with congenital absence of the infrahepatic inferior vena cava in a young male patient.

BACKGROUND: Congenital absence of the inferior vena cava (AIVC) is a rare vascular anomaly that may be associated with deep vein thrombosis (DVT). It is underreported and may be present in up to 5% of young patients with DVT. We report a unique case of simultaneous thrombosis of both superficial and deep veins in a patient with AIVC. METHODS AND RESULTS: A 20-year-old man presented with a 2-week history of a swollen, painful, left lower limb. On examination, the left leg and thigh were found to be swollen and varicosities were present along the lower abdominal wall. Ultrasound showed extensive superficial and deep venous thrombosis of the entire left lower limb. Computed tomography venogram revealed an infrahepatic AIVC with lower limb drainage through enlarged intrathoracic continuations of the azygous and hemiazygous veins. The patient was put on oral anticoagulant therapy and was well at 6-month follow-up. CONCLUSION: The hypothesis for DVT in patients with AIVC is that venous drainage of the lower limbs is inadequate, leading to venous stasis and thrombosis. All young patients presenting with idiopathic DVT should be investigated for inferior vena cava anomalies with computed tomography if ultrasound does not visualize the inferior vena cava.

Mediastinal hemangioma arising in setting of partial anomalous pulmonary venous drainage to azygous vein in a 57-year-old woman.

Partial anomalous pulmonary venous drainage (PAPVD) to the azygous vein and benign posterior mediastinal hemangioma in adults are both rare entities in isolation. The coexistence of these two lesions in the same patient has not been reported. We describe a unique case of PAPVD to the azygous vein in an adult woman, where the anomalous left inferior pulmonary vein transited first through a large hemangioma, and then eventuated in the azygous vein.

Evaluation of thoracic pedicle screw placement in adolescent idiopathic scoliosis.

Pedicle screw fixation is a challenging procedure in thoracic spine, as inadvertently misplaced screws have high risk of complications. The accuracy of pedicle screws is typically defined as the screws axis being fully contained within the cortices of the pedicle. One hundred and eighty-five thoracic pedicle screws in 19 patients that were drawn from a total of 1.797 screws in 148 scoliosis patients being suspicious of medial and lateral malpositioning were investigated, retrospectively. Screw containment and the rate of misplacement were determined by postoperative axial CT sections. Medial screw malposition was measured between medial pedicle wall and medial margin of the pedicle screw. The distance between lateral margin of the pedicle screw and lateral vertebral corpus was measured in lateral malpositions. A screw that violated medially greater than 2 mm, while lateral violation greater than 6 mm was rated as an "unacceptable screw". The malpositions were medial in 20 (10.8%) and lateral in 34 (18.3%) screws. Medially, nine screws were rated as acceptable. Of the 29 acceptable lateral misplacement, 13 showed significant risk; five to aorta, six to pleura, one to azygos vein and one to trachea. The acceptability of medial pedicle breach may change in each level with different canal width and a different amount of cord shift. In lateral acceptable malpositions, the aorta is always at a risk by concave-sided screws. This CT-based study demonstrated that T4-T9 concave segments have a smaller safe zone with respect to both cord-aorta injury in medial and lateral malpositions. In these segments, screws should be accurate and screw malposition is to be unacceptable.

Venous stent patency may be affected by collateral vein lumen size.

PURPOSE: Deep venous thrombosis causes blood flow deviation. It is hypothesized that with stent placement, developed collateral veins become redundant. This article evaluates the relation between the surface area of the collaterals and stent patency. METHODS: The azygos and hemiazygos veins were identified and the largest surface area was measured at thoracic level. Patency rates of stented tracts were evaluated and related to collateral vein lumen size. RESULTS: The vena cava occlusion and the azygos and hemiazygos vein surface area measurements were positive and statistically significant related (OR 1.01, 95% CI 1.003-1.019, p = 0.004) respectively (and OR 1.007, 95% CI 1.001-1.013, p = 0.004). An azygos surface area measurement of 23 (p<0.001) and hemiazygos surface area measurement of 40 (p = 0.008) was shown as cut-off point related to higher occlusion rates. CONCLUSIONS: The surface area of major venous collateral pathways seems to be related to stent occlusion in deep venous interventions.

Unusual Drainage of the Bifurcated Left Renal Vein Into a Dilated Lumbar Azygos Vein and Inferior Vena Cava.

We report a unique case of unusual drainage of the bifurcated retroaortic left renal vein, with the cranial wider branch draining into a dilated lumbar azygos vein and caudal thinner branch connecting with the inferior vena cava. The right renal vein was duplicated. The anomaly was discovered on multimodal 18F-labeled fluorodeoxyglucose positron emission tomography/computed tomography performed for oncological purposes. The basis enabling occurrence of such variation was probably persistent developmental extra left-right venous connections, intercardinal, or intersupracardinal, depending on the theory. The embryology of the chest and abdominal veins is a complicated process and there is no unanimity concerning its concepts. The old models are currently being questioned and reevaluated. Knowledge of possible variants of renal and azygos veins course is important from clinical, imaging, and surgical points of view. The retroaortic left renal veins course may sometimes cause pain, hematuria, proteinuria, and pelvic congestion syndromes. Dilated parts of uncommonly located veins, because of assuming a nodular shape on transverse images, may be mistaken for abnormal lymph nodes, other tumors or aneurysms on imaging. During a variety of surgical procedures, including venous sampling, renal transplantation, or any retroperitoneal surgery, knowledge of an aberrant venous course may be important for the success of the procedure and may be crucial even earlier during the qualification process.

Magnetic resonance imaging findings of persistent primitive hepatic venous plexus with complex congenital cardiac disease.

Persistence of the primitive hepatic venous plexus (PPHVP) is an uncommon anomaly that can present associated with complex congenital heart disease and inferior vena cava (IVC) development anomalies and complicate endovascular and surgical procedures. We report a case of PPHVP in a patient with complex congenital heart disease with unexplained persistent arterial desaturation after Kawashima operation and describe magnetic resonance angiography (Angio-MRI) findings leading to the diagnosis. We recommend that IVC-Angio-MRI be performed in preoperative evaluation of patients with complex congenital heart disease and left isomerism when redirection of systemic venous blood to the pulmonary arterial circulation is considered.

Surgical Challenge in Pediatric Kidney Transplant Vascular Anastomosis.

During pediatric kidney transplant, surgical challenges occasionally occur. In particular, vascular anastomosis should be considered for children with small body weight < 12 kg, multiple renal arteries, vascular anomaly, and inferior vena cava occlusion. In pediatric patients, a living-donor renal graft is usually donated from a parent. Therefore, the renal artery and vein are too large to be anastomosed with the recipient's internal iliac artery and external iliac vein. In children who are > 12 kg, the renal artery and vein could be anastomosed with the external iliac artery and the external iliac vein. In children who are < 10 kg, the renal artery and vein should be anastomosed directly with the aorta and inferior vena cava. A pediatric transplant surgeon should consider arterial and venous anastomosis sites before transplant surgery. In small children with partial or total inferior vena cava occlusion, the venous anastomosis site should be evaluated. If the graft is placed on the left side, a venous graft must be used as a bridge between the renal vein and inferior vena cava. In 13 kidney transplants in children with inferior vena cava occlusion, 7 were on the left and 6 were on the right side. A patent segment of the inferior vena cava, the left original renal vein, an ascending lumbar vein, an azygos vein, the first graft renal vein, and a portal vein were used for venous anastomosis in 6, 2, 2, 1, 1 and 1 recipient, respectively. One child had graft loss due to renal vein thrombosis and one died of hemorrhage immediately posttransplant. Three had grafts with relatively long-term function, but these were lost due to chronic allograft nephropathy 100, 122, and 137 months posttransplant. However, the other 8 recipients have so far maintained graft function from 6 to 138 months since transplant.

Double Inferior Vena Cava (IVC) With Intrahepatic Interruption, Hemiazygos Vein Continuation, and Intrahepatic Venous Shunt.

The duplication of the inferior vena cava (IVC) is a rare congenital anomaly, which also has some variations regarding the complex embryological development of the IVC. In the typical form, infrarenal IVC segments are duplicated and the left IVC joins the left renal vein, which crosses anterior to the aorta in the normal fashion to join the right IVC. In variant forms, the interruption of the intrahepatic segment of the IVC, azygos or hemiazygos continuation, or retroaortic course of the renal vein may be seen. An intrahepatic venous shunt accompanying a double IVC variant is an extremely rare anomaly. We report a case of 40-year-old female patient with double IVC, hemiazygos continuation, intrahepatic IVC interruption, and a transhepatic venous shunt.

Evaluation of the effects of combined endoscopic variceal ligation and splenectomy with pericardial devascularization on esophageal varices.

AIM: To detect the hemodynamic alterations in collateral circulation before and after combined endoscopic variceal ligation (EVL) and splenectomy with pericardial devascularization by ultrasonography, and to evaluate their effect using hemodynamic parameters. METHODS: Forty-three patients with esophageal varices received combined EVL and splenectomy with pericardial devascularization for variceal eradication. The esophageal vein structures and azygos blood flow (AZBF) were detected by endoscopic ultrasonography and color Doppler ultrasound. The recurrence and rebleeding of esophageal varices were followed up. RESULTS: Patients with moderate or severe varices in the esophageal wall and those with severe peri-esophageal collateral vein varices had improvements after treatment, while the percentage of patients with severe para-esophageal collateral vein varices decreased from 54.49% to 2.33%, and the percentage of patients with detectable perforating veins decreased from 79.07% to 4.65% (P < 0.01). Color Doppler flowmetry showed a significant decrease both in AZBF (43.00%, P < 0.05) and in diameter of the azygos vein (28.85%, P < 0.05), while the blood flow rate was unchanged. The recurrence rate of esophageal varices was 2.5% (1/40, mild), while no re-bleeding cases were recorded. CONCLUSION: EVL in combination with splenectomy with pericardial devascularization can block the collateral veins both inside and outside of the esophageal wall, and is more advantagious over splenectomy in combination with pericardial devascularization or EVL in preventing recurrence and re-bleeding of varices.

[Portal congestion and thrombosis after esophagogastric devascularization and splenectomy]. / Indice de congestão portal e a ocorrência de trombose portal pós-dape.

BACKGROUND: The study compared the preoperative portal vein congestion index estimated by Doppler ultrasound and the postoperative portal vein thrombosis of patients submitted to esophagogastric devascularization and splenectomy (EDS). METHODS: 65 patients with portal hypertension due to schistosomiasis and previous gastrointestinal bleeding submitted to EDS were divided into two groups: GROUP A (28 patients without postoperative portal vein thrombosis) and GROUP B (37 patients with postoperative portal vein thrombosis). The following parameters of preoperative Doppler ultrasound of the portal vein were analyzed: diameter, area, mean blood flow velocity and blood flow, whereupon the congestion index was calculated. RESULTS: The diameter, area and blood flow of the portal vein were greater in group B (mean of 1.52 cm; 1.77 cm(2) and 2533.12 ml / min) than in group A (mean of 1.33 cm; 1.44 cm(2) and 1609.03 ml / min) with p = 0.03; 0.03 and 0.04 respectively. Difference of the congestion index was not statistically significant between the two groups (p = 0.07). CONCLUSIONS: The portal vein congestion index at the preoperative of EDS estimated by Doppler ultrasound was not predictive of portal vein thrombosis in the postoperative of patients with portal hypertension due to schistosomiasis.