The coronary sinus interatrial connection with total unroofing coronary sinus discovered late after correction of secundum atrial septal defect.

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 (SatO2 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.

Anatomical anomalies and variations of main thoracic vessels in dogs: a computed tomography study.

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.

Common venous renal trunk in two dogs: Multidetector computed tomographic imaging findings.

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.

Congenital Anomalies of the Superior Vena Cava: Embryological Correlation, Imaging Perspectives, and Clinical Relevance.

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.

Interrupted inferior vena cava in fetuses with omphalocele. Case series of fetuses referred for fetal echocardiography and review of the literature.

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.

Heterotaxy syndrome with malrotation of the gut and interrupted vena cava does not preclude safe procurement of multivisceral graft.

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.

[Anesthetic management of a patient with aortocaval fistula caused by rupture of a huge abdominal aortic aneurysm into the inferior vena cava].

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.

Slit-roundabout signaling regulates the development of the cardiac systemic venous return and pericardium.

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.

Avaliação morfométrica e hemodinâmica comparativa dos vasos envolvidos no shunt portossistêmico congênito em cães / Morphometric and haemodynamic evaluation of the vessels associated with the congenital portosystemic shunting in dogs

The morphometry and haemodynamic aspects of portal vein were studied in 20 normal dogs with less than 120 days of age and in 14 dogs presenting portosystemic shunting with ages between 90 and 360 days. In the control group the hepatic margins were seen 1.50cm to 3.00cm caudal to the costal margin. Collected data indicated that the mean diameter of portal vein (VP), caudal vena cava (VCC) and abdominal aorta (AO) measured respectively, 0.38cm, 0.37cm and 0.41cm. The VP/VCC and VP/AO mean ratios were respectively, 1.10 and 0.94. The average of VP, VCC and AO areas were respectively, 0.12cm², 0.11cm² and 0.14cm². The haemodynamic of portal vein was studied by ultrasound Doppler and the mean velocity of portal blood flow (VMFSP) measured was 17.76cm/s. It was verified that portal blood flow (FSP) average was 83.11ml/min/kg and the congestion index (IC) average was 0.006. In the group of animals presenting portosystemic shunting, the hepatic margins were seen 1.00cm to 2.00cm cranial to the costal margin. The morphometry of VP, VCC and AO presented a mean diameter of 0.40cm, 0.74cm and 0.56cm, respectively. The VP/VCC and VP/AO mean ratios were respectively, 0.54 and 0.69. The average of VP, VCC and AO areas were respectively, 0.14cm², 0.31cm² and 0.25cm². The haemodynamic study demonstrated that the VMFSP measured was 22.29cm/s and the IC average was 0.006.

Foram realizados o estudo morfométrico e o estudo hemodinâmico da veia porta em vinte cães clinicamente normais, de idade igual e inferior a 120 dias e em quatorze cães portadores de shunt portossistêmico, de idades entre 90 e 360 dias. Nos cães do grupo controle, as margens hepáticas apresentaram-se entre 1,50cm e 3,00cm caudalmente à margem costal. Os diâmetros médios da veia porta (VP), veia cava caudal (VCC) e aorta abdominal (AO) obtidas foram respectivamente, 0,38cm, 0,37cm e 0,41cm. As proporções entre os diâmetros médios VP/VCC e VP/AO apresentaram médias de 1,10 e 0,94, respectivamente. As médias das áreas da VP, VCC e AO resultaram respectivamente em 0,12cm² , 0,11cm² e 0,14cm². No estudo hemodinâmico da VP destes animais, utilizando-se o ultrassom Doppler, a velocidade média de fluxo sangüíneo portal (VMFSP) mediu 17,76cm/s. A média de fluxo sangüíneo portal (FSP) resultou em 83,11ml/min/kg. O índice de congestão (IC) apresentou média de 0,006. Para o grupo de cães portadores de shunt portossistêmico, o fígado apresentou redução de seu volume, sendo as margens hepáticas visibilizadas entre 1,00cm e 2,00cm cranialmente à margem costal. No estudo morfométrico, as médias dos diâmetros médios obtidos de VP, VCC e AO resultaram respectivamente em 0,40cm, 0,74cm e 0,56cm. As proporções entre os diâmetros médios VP/VCC e VP/AO resultaram respectivamente em 0,54 e 0,69. As médias das áreas de VP, VCC e AO resultaram respectivamente em 0,14cm², 0,31cm² e 0,25cm². Ao ultrassom Doppler a VMFSP mediu 22,29cm/s e a média do IC da VP obtido foi de 0,006.

[Case report: double-chambered right ventricle (DCRV), ventricular septal defect, and double caudal vena cava in a cat]. / Gecompartimentaliseerde rechter ventrikel, ventrikelseptumdefect en dubbel aangelegde vena cava caudalis bij een kat.

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.

Embryological background of truncular venous malformation in the extracranial venous pathways as the cause of chronic cerebro spinal venous insufficiency.

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.

Imaging diagnosis--left retrocaval ureter and transposition of the caudal vena cava in a dog.

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.

Endovascular treatment of ruptured abdominal aortic aneurysm with aortocaval fistula.

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 use of politetrafluoroethylene stent-graft and vena cava filter in the endovascular treatment of ruptured abdominal aortic aneurysm with aortocaval fistula]. / Zastosowanie politetrafluoroetylenowej protezy wewnatrznaczyniowej i filtra zyly glównej dolnej w leczeniu peknietego tetniaka aorty brzusznej do tyly czczej dolnej.

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.

Non-anatomical intestinal transplantation.

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.

Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting.

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.

Are anomalies of the caval veins more common in complex congenital heart disease?

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.