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.

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.

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.

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.

[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.

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.

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.

[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.

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.

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.

Systemic venous drainage: can we help Newton?

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.

[Abdominal vascular malformations and Down syndrome]. / Malformaciones vasculares abdominales y síndrome de Down.

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.

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.

Major hepatectomy for peripheral papillary cholangiocarcinoma with hilar extension in a patient with situs ambiguous.

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.

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.

Progressive AV-block and anomalous venous return among cardiac anomalies associated with two novel missense mutations in the CSX/NKX2-5 gene.

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.

Identification and analysis of left atrial isomerism.

The veno-atrial connections, atrial morphology, atrioventricular (AV) junction, ventricular mass, ventriculoarterial (VA) connection and great arteries in 22 autopsied hearts, diagnosed as having bilateral left-sidedness because of the morphology of the atrial appendages, were studied. The findings were correlated with the arrangement of the thoracic-abdominal organs. A solitary spleen was found in 3 and double spleens in 2 hearts (the remaining 17 hearts had multiple spleens) but left bronchial isomerism existed in all hearts in which bronchial arrangement could be determined. The heart was in the left chest in 14 cases, in the right chest in 5 and midline in 3. The apex pointed to the left in 18 hearts while in 4 hearts it pointed to the right. Fifteen hearts had a biventricular and ambiguous AV connection, 3 hearts had an absent left AV connection and 4 had double-inlet connection via a common valve (to the left ventricle in 3 and the right ventricle in 1). The VA connection was concordant in 14 hearts, discordant in 1, double outlet from the right ventricle in 4, double outlet from a solitary indeterminate ventricle in 1 and single outlet from the right ventricle through a pulmonary trunk with aortic atresia in 2 hearts. Superior caval veins were present bilaterally in 13 hearts. There was interruption of the infrahepatic inferior caval vein with azygos or hemiazygos continuation in 19 hearts while in 3 hearts the inferior caval vein continued upwards to drain into the right-sided morphologically left atrium.(ABSTRACT TRUNCATED AT 250 WORDS)

Left isomerism and complete atrioventricular block: a report of six cases.

Six children, aged 12 days to 13 years, with left isomerism and complete atrioventricular (A-V) block are presented. In all six patients the diagnosis of left isomerism was suggested by an interrupted inferior vena cava found during cardiac catheterization and angiocardiography; four patients had complex heart disease consisting of endocardial cushion defect, five had a common atrium, three had pulmonary stenosis, three had patient ductus arteriosus and two had dextrocardia. Further anatomic abnormalities included situs inversus of the viscera (four patients) as well as partial malrotation of the bowel. Of the six patients, four had congenital complete A-V block, whereas the remaining two had A-V conduction disturbances documented during early infancy that progressed to complete A-V block later in life. All six patients required pacemaker implantation and five of the six patients died. This report discusses the clinical presentation of complete AV block and left isomerism and reviews the literature.