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We report a case of a malignant fibrous histiocytoma (MFH) of the aortic bifurcation, which manifested as a pseudoaneurysm with the formation of an ilio-caval fistula, a complication about which, to our knowledge, nothing has been published previously. Spiral CT, catheter arteriography and venography were complementary in the diagnostic procedure. [ABSTRACT FROM AUTHOR]

A coronary sinus (CS) interatrial connection is a rare congenital anomaly which can be in various types from atrial septal defect type unroofing CS to the total absence of the CS. The pathology usually accompanies the left superior caval vein (LSCV) draining to CS and in case of its absence directly to the left atrium (LA). We present a 53-year-old woman after surgical correction of a secundum atrial septal defect in 1974, with a CS interatrial connection and paroxysmal atrial fibrillation (PAF). She gave a history of PAF and was admitted to our clinic due to progressive exertional intolerance, peripheral edema, and mild hypoxia (SatO 2 92%) with subsequent cyanosis. Transthoracic echocardiography showed a left-to-right shunt in the posteroinferior part of the atrial septum. Computed tomography revealed a persistent LSCV draining directly into the LA, the absence of the CS, and cardiac veins draining into the LA. The right atrium (RA) and the LA were connected via a tunnel with a visible contrast passage from the left to the right side-the persisting mouth of the coronary sinus. The patient was qualified for surgical correction. A glutaraldehyde-treated autologous pericardial patch was used to construct the tunnel connecting the LSVC and the RA. The second part of the patch was used to close the atrial communication at the inferior vena cava level. The patient had an uncomplicated postoperative course and is now classified in New York Heart Association Class II., (© 2020 Wiley Periodicals LLC.)

Introduction: There is scarce information about the prevalence of anomalies and anatomical variations of the main great thoracic vessels in dogs, particularly in dogs without congenital heart disease., Animals: The study included 878 privately owned dogs., Material and Methods: Computerized tomography (CT) thoracic studies carried out between 2011 and 2014 for a variety of reasons were reviewed. The prevalence of anomalies and anatomical variations of the aorta and vena cava, the arterial branches of the aortic arch and the main branches of the intrathoracic veins in dogs with no evidence of congenital heart disease was evaluated. Poor-quality CTs, CTs with thoracic pathology that impaired visualization or those of young dogs with clinical evidence or suspicion of congenital cardiac disease were excluded., Results: Eight hundred two CT studies were analysed. Eight dogs (1%) showed an anatomic anomaly. The most common anomaly was an aberrant retroesophageal right subclavian artery (n = 7, 0.8%). One dog showed a dilated azygos vein secondary to an interrupted vena cava. Three types of branching of the common carotid arteries were observed: both arteries arising at the same point (type I: n = 506/742; 68.2%), separated (type II: n = 212/742; 28.6%) or from a common trunk (type III: n = 24/742; 3.2%)., Conclusions: Major anatomical variations or anomalies of the main great thoracic vessels in dogs without congenital cardiac disease were rare. An aberrant retroesophageal right subclavian artery was the most common anomaly found. Three slight variations of common carotid artery branching were identified. These findings might be of relevance for surgical or catheterization procedures., (Copyright © 2018 Elsevier B.V. All rights reserved.)

In dogs, variation in the branching pattern of renal veins is rare with only few patterns reported. This report describes two unusual anomalies of the renal vein branching patterns in two dogs. In dog 1, a common renal trunk drained both kidneys, in a T-shape pattern, in the caudal vena cava after a long right perirenal course. In dog 2, a common venous trunk branched cranially from the pre-renal segment of an azygos-caudal vena cava venous trunk and divided into the renal veins in a Y-shape pattern. Proper knowledge of the possible anatomical variations in renal venous drainage may be helpful during imaging assessment and surgical planning of several canine diseases involving the abdominal vasculature and retroperitoneal space.

There is a wide spectrum of congenital anomalies of the superior vena cava, which are more increasingly recognized in cross-sectional imaging. Although some of these anomalies are asymptomatic, others have important clinical and interventional implications. Imaging modalities such as computed tomography and magnetic resonance imaging play an important role in the accurate characterization of these anomalies, which is essential for mapping prior to surgeries or interventions. In this article, we review a wide range of anomalies of the superior vena cava, including the embryological basis, cross-sectional imaging findings, and clinical implications, particularly from an interventional radiology perspective. We also discuss the treatments and complications of these anomalies., (Copyright © 2016 Canadian Association of Radiologists. Published by Elsevier Inc. All rights reserved.)

Objectives: Congenital heart disease is reported in 15-45% of omphalocele cases. Associated abnormalities of systemic veins have occasionally been reported in children and rarely documented in the fetus. We report a case series of interrupted inferior vena cava (Int-IVC) in association with omphalocele and review the literature., Methods: From our fetal database we identified all cases of omphalocele referred for fetal echocardiography (FE) between 1997 and 2012. We reviewed pre and postnatal medical records and performed a literature search from 1975 to present to identify previous relevant publications., Results: Of 9627 fetuses referred for FE, 34 had an omphalocele. Gestational age at FE was 17(+6) to 26(+4)weeks. Seven of the 34 fetuses were shown to have an Int-IVC with azygos continuation to a right-sided superior vena cava (SVC). The heart was structurally normal in all but one case. The abdominal wall defect was large and contained liver in all. There were three fetal demises and one neonatal death. Three cases were operated successfully. Since 1975, we identified 12 publications reporting omphalocele with systemic venous abnormalities. Abnormal IVC angulation may lead to surgical complications. Failure of IVC formation is likely to be a developmental rather than a situs abnormality. Int-IVC with a dilated azygos influences venous access and may predispose to venous thrombosis., Conclusion: We have documented an association between large omphalocele and Int-IVC with azygos continuation to the SVC. In this small series, this did not have surgical implications. It will however, influence route of any future cardiac catheterisation and may have long-term implications., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

We report the first successful procurement and transplantation of a multivisceral graft from a pediatric donor with polysplenic heterotaxy syndrome, including intestinal malrotation, midline liver with left-sided gallbladder and an interrupted inferior vena cava with azygous continuation. Procurement of the graft presented a surgical challenge in the presence of above anomalies. Modified approach to standard organ procurement and minor technical adaptation enabled successful transplantation. In an era of severe organ shortage of pediatric multivisceral grafts, a valuable organ offer should not lightly be declined for reasons of anatomic imperfections that might be overcome., (© Copyright 2014 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Aortocaval fistula is a rare complication of ruptured abdominal aortic aneurysm. We report anesthetic management of a patient with aortocaval fistula caused by rupture of a huge abdominal aortic aneurysm into the inferior vena cava. A 51-year-old man who had complained of low back pain and general fatigue was referred to our hospital because of his liver damage. Aortocaval fistula due to rupture of a huge abdominal aortic aneurysm was diagnosed from physical examination, enhanced computed tomography and color Doppler ultrasonography. Anesthesia was induced with propofol and rocuronium, and was maintained with sevoflurane and remifentanil. After induction of anesthesia, the central venous pressure and cardiac index showed remarkably high values because of arteriovenous shunt. When the aneurysm was incised after the clamping of the abdominal aorta, massive venous bleeding occurred from the fistula and caused severe hypotension. Blood pressure recovered by digital compression of the bleeding point and the use of an autotransfusion device. After the repair of the aortocaval fistula, the hemodynamics became stable. The patient had a high output but a good cardiac function in preoperative examination. Therefore anesthesia was managed successfully without worsening high-output heart failure.

Rationale: The Slit-Roundabout (Robo) signaling pathway has pleiotropic functions during Drosophila heart development. However, its role in mammalian heart development is largely unknown., Objective: To analyze the role of Slit-Robo signaling in the formation of the pericardium and the systemic venous return in the murine heart., Methods and Results: Expression of genes encoding Robo1 and Robo2 receptors and their ligands Slit2 and Slit3 was found in or around the systemic venous return and pericardium during development. Analysis of embryos lacking Robo1 revealed partial absence of the pericardium, whereas Robo1/2 double mutants additionally showed severely reduced sinus horn myocardium, hypoplastic caval veins, and a persistent left inferior caval vein. Mice lacking Slit3 recapitulated the defects in the myocardialization, alignment, and morphology of the caval veins. Ligand binding assays confirmed Slit3 as the preferred ligand for the Robo1 receptor, whereas Slit2 showed preference for Robo2. Sinus node development was mostly unaffected in all mutants. In addition, we show absence of cross-regulation with previously identified regulators Tbx18 and Wt1. We provide evidence that pericardial defects are created by abnormal localization of the caval veins combined with ectopic pericardial cavity formation. Local increase in neural crest cell death and impaired neural crest adhesive and migratory properties underlie the ectopic pericardium formation., Conclusions: A novel Slit-Robo signaling pathway is involved in the development of the pericardium, the sinus horn myocardium, and the alignment of the caval veins. Reduced Slit3 binding in the absence of Robo1, causing impaired cardiac neural crest survival, adhesion, and migration, underlies the pericardial defects.

Aortocaval fistula (ACF) is a rare complication of abdominal aortic aneurysm (AAA). We report the endovascular repair of an AAA rupture into the inferior vena cava. A 78-year-old woman was admitted to our hospital for acute hypotension. She presented with a pulsatile abdominal mass and became rapidly anuric. Abdominal computed tomography (CT) showed an AAA rupture into the inferior vena cava. The features of the AAA made it suitable for endovascular repair. To prevent pulmonary embolism caused by the presence of sac thrombosis near the vena cava lumen, a temporary vena cava filter was deployed before the procedure. A bifurcated stent-graft was placed with the patient under local anaesthesia, and the AAA was successfully treated. A transient type II endoleak was detected on CT 3 days after endograft placement. At routine follow-up 6 and 12 months after the procedure, the patient was in good clinical condition, and the type II endoleak had sealed completely. Endovascular treatment offers an attractive therapeutic alternative to open repair in case of ACF; however, only small numbers of patients have been treated, and long-term follow-up interval is lacking.

The truncular venous malformation (VM) represents an embryologically defective vein where developmental arrest has occurred during the vascular trunk formation period in the 'later stage' of the embryonic development. A relatively simple truncular VM lesion such as a venous web at the hepatic venous outlet causes portal hypertension giving a profound damage/impact to the liver. A similar condition involving the head and neck venous system may cause chronic cerebro-spinal venous insufficiency (CCSVI) and may be involved in the development or exacerbation of multiple sclerosis.

The clinical signs and symptoms, radiographic and echocardiographic findings, and the results of cardiac post-mortem and histopathological examination of a 1-year-old female European shorthair cat with a double-chambered right ventricle (DCRV), small ventricular septal defect, and double caudal vena cava are described. A review of the literature is given with respect to the symptoms, diagnostic techniques, and therapy of DCRV in the cat. DCRV is a rare congenital defect in which stenosis inside the right ventricle causes symptoms similar to those seen in pulmonary stenosis. A diagnosis can be made by echocardiography. Little is known about its natural history and prognosis. Medical treatment, balloon dilatation, and surgery have been used to treat this defect with variable outcome. A double caudal vena cava is not clinically relevant.

Retrocaval ureter and transposition of the caudal vena cava are each, rare developmental anomalies. We describe the usefulness of static fluid magnetic resonance urography and dynamic contrast-enhanced magnetic resonance urography in the diagnosis of these anomalies. Basic techniques, benefits, and drawbacks of magnetic resonance urography are presented.

Congenital portosystemic shunts are the anomalies in which the mesenteric venous drainage bypasses the liver and drains directly into the systemic circulation. This is a report of a rare case of LDLT in a four-yr old male child suffering with biliary atresia (post-failed Kasai procedure) associated with (i) a large congenital CEPSh from the spleno-mesentric confluence to the LHV, (ii) intrapulmonary shunts, (iii) perimembranous VSD. The left lobe graft was procured from the mother of the child. Recipient IVC and the shunt vessel were preserved during the hepatectomy, and the caval and shunt clamping were remarkably short while performing the HV and portal anastomosis. Post-operative course was uneventful; intrapulmonary shunts regressed within three months after transplantation and currently after 18 months following transplant child is doing well with normal liver functions. CEPSh has been extensively discussed and all the published cases of liver transplantation for CEPSh were reviewed.

Introduction: Intestinal transplantation is the only long-range treatment option for patients with intestinal failure. We report an exceptional case of isolated intestinal transplantation with the implant in a non-anatomical position., Clinical Case: The patient was a thirty-eight-year-old man (60 kg weight, 180 cm height, 18.3 body mass index) with intestinal failure and home parenteral nutrition due to a short-bowel syndrome for which intestinal transplantation was indicated. The patient had a vascular malformation with the cava vein located left to the aorta, and the intestine was implanted with a 180 masculine rotation around the mesenteric axis, so that the implant s superior mesenteric artery and vein matched the recipient s cava and aorta. Postoperative follow-up was excellent and the patient was discharged after six weeks with a 10-kg gain in body weight., Discussion: This non-anatomical intestinal implantation of the small bowel, previously unreported, offers technical advantages over other options. Adequate intestinal function represents a unique model to prove the viability of intestinal implants in a non-anatomical position.

We report on a the endovascular treatment of ruptured abdominal aortic aneurysm with aortocaval fistula. The stent-graft was placed with the patient under general anaesthesia, and the abdominal aorta aneurysm was successfully treated. To prevent pulmonary embolism vena cava filter was deployed before the implantation of the sten-graft. The aneurysm was excluded and no endoleak or communication between the aorta and inferior vena cava was seen on computed tomographic imaging at the 3-month evaluation. Endovascular treatment offers an attractive therapeutic alternative to open repair in case of aortocaval fistula.

Background and Purpose: Anomalies of the caval veins are considered to be common cardiac malformations. The knowledge of the varieties of the caval venous system is essential for cardiovascular surgery, pacemaker implantation, intensive care medicine or cardiac catheterization. The authors wanted to know, if anomalies of the caval veins are related to the type and complexity of congenital heart disease., Material and Methods: The records of 1,631 patients who were catheterized between 1991 and 2003 were reviewed., Results: 92 of these had anomalies of either the superior or inferior caval vein. 23 had simple congenital heart disease, while in 69 this was considered to be complex (p < 0.001). Embryologic considerations are discussed., Conclusion: It could be proven that anomalies of the caval veins are more often related to complex congenital heart disease. Embryologic considerations show at least a coincidence of the development of the caval veins and of congenital heart disease. For clinical work with these patients the knowledge of these anomalies is important.

In recent years substantial progress occurred in the techniques of cardiopulmonary bypass, but the factor potentially limiting the flexibility of cardiopulmonary bypass remains the drainage of the systemic venous return. In the daily clinical practice of cardiac surgery, the amount of systemic venous return on cardiopulmonary bypass is directly correlated with the amount of the pump flow. As a consequence, the pump flow is limited by the amount of venous return that the pump is receiving. On cardiopulmonary bypass the amount of venous drainage depends upon the central venous pressure, the height differential between patient and inlet of the venous line into the venous reservoir, and the resistance in the venous cannula(s) and circuit. The factors determining the venous return to be taken into consideration in cardiac surgery are the following: (a) characteristics of the individual patient; (b) type of planned surgical procedure; (c) type of venous cannula(s); (d) type of circuit for cardiopulmonary bypass; (e) strategy of cardiopulmonary bypass; (f) use of accessory mechanical systems to increased the systemic venous return. The careful pre-operative evaluation of all the elements affecting the systemic venous drainage, including the characteristics of the individual patient and the type of required surgical procedure, the choice of the best strategy of cardiopulmonary bypass, and the use of the most advanced materials and tools, can provide a systemic venous drainage substantially better than what it would be allowed by the simple "Law of universal gravitation" by Isaac Newton.

Malformations of the abdominal venous system are rare vascular disorders. These entities are associated with other malformations and with chromosomal anomalies such as trisomy 21. Abdominal venous malformations are probably the most frequent congenital vascular malformations in Down syndrome. Prenatal diagnosis allows the early follow-up and treatment of complications. We present a case of Down syndrome associated with an abdominal venous malformation diagnosed at the first trimester of pregnancy.

Coronary artery fistulae are abnormal communications between a coronary artery and a cardiac chamber or major vessel (vena cava, pulmonary veins, pulmonary artery). They are usually diagnosed by coronary arteriography. Clinical presentations are variable depending on the type of fistula, shunt volume, site of the shunt, and presence of other cardiac conditions. In this article, we review the literature regarding etiology, incidence, clinical manifestation, image studies, and management.

Background: The presence of major aortopulmonary collateral arteries (MAPCA) in patients with right isomerism may alter the prognosis, which is still unclear., Methods: From 1994 to 2003, 138 out of 155 patients (89%) with right isomerism had pulmonary stenosis or pulmonary atresia and constituted the study population., Results: Two groups of patients were identified, one with MAPCA and the other without. The MAPCA originating from descending thoracic aorta or aortic arch was noted in 9 patients (6.5%), with a median number of MAPCA being 3 (range, 2 to 4). Patients with MAPCA commonly had congestive heart failure (8/9, 89%) and frequent lower airway infection (6/9, 67%). In comparison with patients with MAPCA and without, the intrapericardial pulmonary artery was more frequently absent (2 of 9 vs 1 of 129, p < 0.001), and the pulmonary arterial size was smaller (McGoon ratio <1.5, 8 of 9 vs 57 of 129, p = 0.009) in the MAPCA group. The mean pulmonary arterial pressure measured was often higher than the suggested limit for Fontan circulation. Major aortopulmonary collateral arteries were addressed at a median of 6 months old (2 to 19 months old) in 5 patients. Although surgical mortality was not different for first-stage palliation and Glenn shunt in both groups, total cavopulmonary connection could only be performed in one patient with MAPCA with poor results. However, the 5-year survival of patients with and without MAPCA was not statistically different (74.1% vs 55.2%, p = 0.19)., Conclusions: The presence of MAPCA was uncommon but still noted in patients with right isomerism. It may cause heart failure and a less favorable pulmonary vasculature for a complete Fontan-type operation.

We report a unique case of portosystemic shunt with polysplenia and hypoplastic left heart syndrome. The abnormal vessel connected the right renal vein and the splenic vein. The shunt flow was from the systemic vein to the portal vein before the Fontan-type operation, but it reversed and ran from portal vein to systemic vein after the Fontan-type operation.

Situs ambiguous is a rare anomaly, which includes various abnormalities of position and development of trunk organs and results in diagnostic and therapeutic problems during major abdominal intervention. We report the case of a woman with peripheral papillary cholangiocarcinoma and hilar extension, developed on situs ambiguous associated with the following abnormalities: agenesis of the retrohepatic vena cava, preduodenal portal vein, a variant of the hepatic arteries, truncated pancreas, polysplenia, and mesenteric malrotation. After complete anatomical assessment, resection of segments 4 to 8 extended to the common bile duct with lymphadenectomy and reconstruction by hepaticojejunostomy was performed with no surgical complications. The patient was alive with no signs of recurrence at 18 month follow-up. The specificities of situs ambiguous must be identified by anatomical assessment but do not prevent complex abdominal surgery.

Radio-isotopic techniques may be useful in diagnosis and staging of chronic diffuse liver diseases. Liver angioscintigraphy (LAS) and per-rectal portal scintigraphy (PRPS) are at well discriminating portal hypertension (PHT), very early cirrhosis hemodynamic failure and compensatory arterialisation of liver perfusion. Supplied information is related to PHT, liver morphology and mesenchimal activity in liver, spleen and bone marrow. Correlation of LAS and PRPS may diagnose installing of PHT earlier than any actual morphologic imagistic method. Our experience (after more than 300 PRPS and 500 LAS) suggests that PHT and portal-cave shunts (PCS) may be classified in five functional stages. These five patterns (types) are characteristic for portal dynamics, supporting disease staging and follow-up of evolution to cirrhosis. All five dynamics may be assessed by PRPS and LAS. Scintigraphic techniques also explore portal thromboses, perfusion differences between the lobes of cirrhotic liver, betablockers effect in PHT, earliest stages of PHT, malignant tumours occurring on cirrhosis, the different characteristics of alcoholic liver comparing to viral etiology.

A 15-month-old neutered male cat was presented for progressive paraparesis of 3 months' duration and suspected cardiomegaly. Neuroanatomical localization was a T3-L3 myelopathy. On abdominal ultrasound, an anomalous vessel with turbulent blood flow was identified arising from the caudal vena cava. Myelography showed a bilateral ventrolateral extradural spinal cord compression from T12 to L4. Nonselective angiography and contrast-enhanced computed tomography clearly indicated a vascular complex and vena caval aneurysm with an engorged internal vertebral venous plexus. Surgical occlusion of the anomalous vessels was unsuccessful.

The value of ultrasonography was evaluated in 85 dogs and 17 cats presented with a clinically suspected portosystemic shunt (PSS). A PSS was confirmed in 50 dogs and nine cats (single congenital extrahepatic in 42, single congenital intrahepatic in 11, and multiple acquired in six). Six dogs and one cat had hepatic microvascular dysplasia, and 29 dogs and seven cats had a normal portal system. Ultrasonography was 92% sensitive, 98% specific, and had positive and negative predictive values of 98% and 89%, respectively, in identifying PSS, with an overall accuracy of 95%. When a PSS was identified with ultrasonography, extrahepatic, intrahepatic, and multiple acquired PSS could be correctly differentiated in 53/54 patients (98%). The combination of a small liver, large kidneys, and uroliths had positive and negative predictive values of 100% and 51% for the presence of a congenital PSS in dogs. The portal vein/aorta (PV/Ao) and portal vein/caudal vena cava (PV/ CVC) ratios were smaller in animals with extrahepatic PSSs compared with animals with microvascular dysplasia, intrahepatic PSSs and those without portal venous anomalies (P<0.001). All dogs and cats with a PV/Ao ratio of < or = 0.65 had an extrahepatic PSS or idiopathic noncirrhotic portal hypertension. Dogs and cats with PV/Ao and PV/CVC ratios of > or = 0.8 and > or = 0.75, respectively, did not have an extrahepatic PSS. Reduced or reversed portal flow was seen in four of four patients with multiple acquired PSSs secondary to portal hypertension. The presence of turbulence in the caudal vena cava of dogs had positive and negative predictive values of 91% and 84%, respectively, for the presence of any PSS terminating into that vein.

There is a description of a case of sudden intrauterine unexplained death (SIUD) of healthy twins at term. The course of the pregnancy was regular. There were no significant sinister symptoms of fetal impend. No malformations or other abnormalities were observed after delivery by cesarean section. The probable cause of SIUD seems to be aortic-vain syndrome.

Although previously described in patients undergoing staged palliation for univentricular heart disease, the mechanism by which hepatic venous flow prevents development of pulmonary arteriovenous malformations is still not completely understood. We present a case in which successful H-type Fontan revision with rerouting of hepatic venous flow through a hemiazygous vein successfully reversed the progression of severe left pulmonary arteriovenous malformations.

In a young adult patient having situs solitus with dextrocardia the attempted pulmonary artery catheter placement for emergency mitral valve replacement required an unduly long length (50cm) of catheter insertion to get into right ventricle and then into pulmonary artery. Although catheter coiling was suspected initially, chest x-ray taken after successfully placement revealed an uncommon congenital anomalous venous connection i.e. right internal jugular opening into left sided superior vena cava then into inferior vena cava after running all along the left border of the heart. With the result, it required to pass 50cm of PA catheter to get into right ventricle in our patient. This emphasizes the need to look for abnormal venous connections during echocardiography and x-ray screening in congenital heart disease. Fluoroscopy is recommended when an unusual length of pulmonary artery catheter insertion is required to enter the pulmonary artery.

Congenital anomalies of the thoracic veins, although uncommon, can be of the utmost clinical and surgical significance. These venous anomalies may be subdivided into systemic and pulmonary. Most systemic venous abnormalities are incidental findings, whereas pulmonary vein malformations coexist with coronary heart disease, especially atrial septal defect. Although cardiac ultrasonography remains the initial noninvasive imaging modality, magnetic resonance imaging with ultrafast sequences is of great diagnostic value because multiplanar 3-dimensional reconstructions may be made.

Objectives: To systematically describe the imaging features and clinical correlates of a partial anomalous pulmonary venous connection diagnosed on computed tomography (CT) in adults., Methods: Twenty-nine adults with a partial anomalous pulmonary venous connection on CT were retrospectively identified. There were 19 women and 10 men, with a mean age of 53 (range: 19-83) years. Four cases were identified by review of 1825 consecutive chest CT reports from July 2000-July 2001, and 25 cases were culled from chest radiology teaching files at 3 institutions. Inclusion criteria were availability of CT images and medical charts. Chest radiographs (25 of 29 cases) were reviewed for mediastinal contour abnormalities, heart size, and pulmonary vascular pattern. Chest CT scans were reviewed for location, size, and drainage site of the anomalous vein; presence or absence of a pulmonary vein in the normal location; cardiac size and configuration; and pulmonary vasculature. Charts were reviewed for evidence of pulmonary and cardiovascular disease, history of congenital heart disease, and results of other cardiac imaging., Results: The prevalence of a partial anomalous pulmonary venous connection was 0.2% (4 of 1825 chest CT reports). Seventy-nine percent (23 of 29 patients) had an anomalous left upper lobe vein connecting to a persistent left vertical vein, only 5% (1 of 23 patients) of whom had a left upper lobe vein in the normal location. Seventeen percent (5 of 29 patients) had an anomalous right upper lobe vein draining into the superior vena cava, 60% (3 of 5 patients) of whom also had a right upper lobe pulmonary vein in the normal location. One patient (3%) had an anomalous right lower lobe vein draining into the suprahepatic inferior vena cava. Chest radiographic findings were abnormal left mediastinal contour in 64% (15 of 25 patients), abnormal right mediastinal contour in 8% (2 of 25 patients), and cardiomegaly in 24% (6 of 25 patients). Computed tomography findings were cardiomegaly in 48% (14 of 29 patients), right atrial enlargement in 31% (9 of 29 patients), right ventricular enlargement in 31% (9 of 29 patients), and pulmonary artery enlargement in 14% (4 of 29 patients). Pulmonary or cardiovascular symptoms were present in 69% (20 of 29 patients), 55% (11 of 20 patients) of whom had specific alternative diagnoses (excluding congestive heart failure and pulmonary hypertension) to explain the symptoms. Only 1 patient (3%) was diagnosed with a secundum atrial septal defect., Conclusions: A partial anomalous pulmonary venous connection was seen in 0.2% of adults on CT. In contrast to previous series focusing on children, the anomalous vein in adults was most commonly from the left upper lobe, in women, and infrequently associated with atrial septal defects.

The objectives of this study were to investigate the temporal response of left ventricular (LV) matrix metalloproteinase (MMP) activity and collagen volume fraction (CVF) induced by an aortocaval fistula and the role of cardiac mast cells in regulating MMP activity. LV tissue was analyzed for MMP activity, CVF, and mast cell number in rats euthanized at 0.5, 1, 2, 3, 5, 14, 21, 35, and 56 days. Additional rats treated with the mast cell membrane-stabilizing drug cromolyn sodium were euthanized 1, 2, and 3 days postfistula. Marked increases in MMP activity occurred rapidly and remained significantly elevated for 5 days before returning toward normal. A significant decrease in CVF occurred by day 5, but thereafter CVF rebounded to normal or above normal values. The number of myocardial mast cells also significantly increased postfistula, and there was a close association between mast cell density and MMP activity. Cromolyn treatment prevented the increase in mast cell number and MMP activity. Thus it is concluded that cardiac mast cells play a major role in the regulation of MMP activity.

Due to the complexity of their developmental stages, the venae cavae may undergo a very large number of congenital anomalies. All the possible abnormalities which, to our knowledge, have been observed in the literature are reported, differentiating those of the superior vena cava and the azygos system, those of the inferior vena cava and the complex anomalies that concern the venous system as a whole. Moreover, we present three new variants: a right double inferior vena cava with azygos continuation of the posterior-medial vein; an agenesis of the superior vena cava with drainage through the azygos and hemiazygos veins to the inferior vena cava; and a double inferior vena cava with hemiazygos and azygos continuation of the left one.

Non-syndromic cardiac septation defects are common, yet the causative factors remain largely uncharacterised. Septation defects are an integral part of many syndromes, some of which are associated with chromosomal alterations. For the majority, the physiopathogenesis is believed to be multi-factorial, hindering the identification of causative factors. Ten mutations in the gene encoding the transcription factor CSX/NKX2-5 have been described in individuals with ASD and/or atrioventricular conduction defects. In addition, several other cardiac abnormalities were observed, yet the mildest forms are reminiscent of non-syndromic septation defects. The CSX/NKX2-5 gene is thus a good candidate for various cardiopathies. We have collected two families with inherited predisposition to cardiac abnormalities. Some members of the families presented ASD and AV block. In both families a novel CSX/NKX2-5 mutation was identified in the homeodomain. Variable expressivity in the phenotype was observed in both families. Importantly, mutation carriers did not present any symptoms at young age. In addition, anomalous venous return, a phenotype not previously associated to CSX/NKX2-5 mutations, was observed in one of the families. We also screened the CSX/NKX2-5 gene in sporadic and familial cases of other cardiopathies. As additional mutations were not found, substitutions in CSX/NKX2-5 gene seem to be a rare cause of cardiopathies without conduction defect., (Copyright 2002 Wiley-Liss, Inc.)

This article presents as a diagnostic problem a rare mediastinal venous anomaly detected in a patient with a primary intrathoracic tumor. Its appearance on computed tomography (CT) is discussed and compared with that of other developmental mediastinal venous anomalies. The individual CT characteristics of these anomalies and their clinical significance with respect to the management of patients with cancer are also reviewed., (Copyright 2001 Wiley-Liss, Inc.)

Objectives: Anomalies of the fetal venous system are poorly documented and their pathogenesis is not well understood. The present study was undertaken to review the spectrum of fetal central veins and umbilico-portal system anomalies, and to propose a classification system., Methods: A 7-year restrospective survey was conducted., Results: Nineteen fetuses showed abnormal connection between central veins and the fetal heart. Three fetuses showed abnormal connections of the cardinal veins, two of which had interruption of the inferior vena cava, and one had isolated persistent left superior vena cava. Anomalies of pulmonary veins were seen in four fetuses: in two with asplenia syndrome, a vertical confluent pulmonary artery was observed. In a further two cases total anomalous pulmonary venous connections were found. Abnormalities of the umbilical vein (UV) were seen in 10 cases; seven had persistent right UV, and three had a spectrum of anomalies: One had abnormal connections of the UV to the left iliac vein associated with agenesis of the ductus venosus (DV) and hydrops fetalis. One case showed in utero occlusion of the DV by echogenic foci that resulted in a persistent left proximal UV and porto-systemic shunt. One case had obliteration of the DV secondary to in utero fetal hepatic fibrosis. Abnormalities of the vitelline veins or portal system were demonstrated in two cases. One had a left porto-systemic shunt which resolved spontaneously at 3 months of age, and one had secondary partial occlusion of the left portal system with liver echogenicities and direct communication of the UV with the right atrium. None of the 19 cases had an abnormal karyotype or evidence of in utero infection., Conclusions: Detection of various fetal vein anomalies in utero is feasible. The anomalies vary according to embryologic precursors or etiology. Two major mechanisms seem to be involved in the genesis of fetal vein anomalies: in most cases primary maldevelopment of the venous system occurs, while in the minority secondary anomalies from possible thromboembolic events or systemic disease may play a role.

The biplanar umbilical vein portovenograms of 49 newborn puppies and the biplanar mesenteric vein portovenograms, obtained during surgery, of 42 adult dogs with left divisional intrahepatic portosystemic shunts consistent with a patent ductus venosus (PDV) were reviewed. On the basis of the combined surgical, postmortem and imaging data, the left divisional intrahepatic portosystemic shunts were consistent, each having a straight vessel which drained into a venous ampulla before draining into the caudal vena cava at the level of the diaphragm. The left phrenic vein and the left hepatic vein both entered the ampulla independently of the shunting vessel. The morphology of the ductus venosus in the pups was similar and consistent with the morphology of the left divisional intrahepatic PDV shunt of the adult dogs. It is concluded that this form of left divisional shunt is correctly named a PDV and is the result of the persistence of the fetal ductus venosus. From the surgical records it is concluded that all the shunts described as a PDV were attenuated by the direct manipulation of the ductus venosus before its entry into the ampulla.

Anomalies of thoracic veins are a diverse but important group of abnormalities. MR imaging is useful in delineating the venous anomalies, provides physiologic information, and is a valuable complementary technique to echocardiography, angiography, and CT scan.

The preoperative ultrasound images and biplanar operative mesenteric portograms of 28 dogs and six cats with congenital intrahepatic portacaval shunts were reviewed retrospectively. On the basis of the combined surgical, postmortem and imaging data, the shunts were classified according to their location within the liver and their shape. Thirteen dogs and four cats had a left-divisional shunt with a relatively consistent bent tubular shape that drained into the left hepatic vein. Another 13 dogs had a central-divisional shunt that took the form of a foramen between dilated portions of the intrahepatic portal vein and caudal vena cava. One cat with a central-divisional shunt had a tortuous vessel. The remaining two dogs and one cat had right-divisional shunts that were large, tortuous vessels. The morphology of a left-divisional shunt is compatible with patent ductus venosus, but the pathogenesis of central and right-divisional shunts is unknown. It is concluded that intrahepatic portacaval shunts in dogs and cats may be classified as left, central or right divisional. Ultrasound enables a preoperative morphological assessment that correlates well with the results of portography and may aid surgical planning.