Anomalous Systemic Venous Return Research Articles

Anomalous systemic venous return: An extremely rare cause of hypoxemia in a neonate.

Anomalous systemic venous return: An extremely rare cause of hypoxemia in a neonate.

105 A CASE OF RARE ATYPICAL ATRIAL STRUCTURE

Abstract A 27-year-old woman, without previous cardiological history, was admitted at our emergency department in the clinical context of an acute pericarditis. Echocardiography documented mild pericardial effusion (8 mm) without any sign of hemodynamic impact. The patient was then admitted at our Cardiology Unit. During the hospitalization a complete transthoracic echocardiogram showed a normal biventricular morphology and function, normal valvular function and found an anechoic structure on the postero-lateral left atrial wall (1.75×1.5 cm). To better characterize this accidental finding, a transesophageal echocardiography was performed unmasking a dilated coronary sinus with anomalous course. A subsequent cardiac computed tomography was performed showing anomalous systemic venous return with the persistency of the left superior vena cava draining into the coronary sinus. No other congenital anomalies were found. PLSVC is present in approximatively 0.5% of individuals in the general population and is the most frequent congenital malformation of the thoracic venous return. In about 90% coexists with the right superior vena cava and co-occur in almost 40% of patients with other congenital heart abnormalities such as atrial septal defect, aortic coarctation, bicuspid aortic valve, tetralogy of Fallot, transposition of the great vessels, anomalous connections of the pulmonary veins, dextroversion and cor triatatum. Patients usually are asymptomatic. Nevertheless, this congenital malformation may cause chest discomfort, cardiac arrhythmias, thromboembolic events, decreased exercise tolerance and syncope. Moreover, the correct identification of this condition has important clinical implications. In fact, it is a relative contraindication to the administration of retrograde cardioplegia during cardiac surgery. Furthermore, the placement of central venous-access line, pulmonary artery catheterization or the implantation of pacemaker/resynchronization leads could be difficult or can result in incorrect positioning. In these cases, it is recommended to access to the right heart through the right subclavian vein instead the left one. Legend: *: Anomalous course of coronary sinus; LA: Left Atrium; LV: Left Ventricle; Ao: Aorta; RA: Right Atrium; R-SVC: Right superior vena cava; L-SVC: Left superior vena cava; LSPV: Left superior pulmonary vein.

One-Stage Correction with Intra- and Extraatrial Rerouting of Anomalous Systemic and Pulmonary Venous Return and Intraventricular Repair of Double Outlet Right Ventricle in a Patient with Heterotaxy Syndrome

We report a very rare case of successful intracardiac correction in a patient with heterotaxy syndrome. The cardiac malformations included dextrocardia, double outlet right ventricle, pulmonary stenosis, interrupted inferior vena cava, hemiazygos continuation and total anomalous pulmonary venous return. One-stage correction was performed. The atrial procedure consisted of intra- and extraatrial rerouting of the anomalous systemic and pulmonary venous return. The hepatic veins were detached and diverted to the left atrium via an extracardiac conduit. The correction of the double outlet right ventricle was accomplished by intraventricular redirection of the blood flow from the left ventricle to the aorta. The right ventricular outflow was ultimately remodeled using a valved conduit. For better perception of the complex morphology, a three-dimensional model was designed, using CT scan images. This proved to be very useful for surgical planning, especially with regard to the intraatrial reconstruction of the systemic and pulmonary venous rerouting.

Echo Rounds: Partial Anomalous Systemic and Pulmonary Venous Return.

Anomalous drainage of a right-sided superior vena cava to the left atrium is a rare variant of systemic venous return anomalies. During the course of investigation for a heart murmur, an asymptomatic child was found to have a superior sinus venosus atrial septal defect with partial anomalous systemic and pulmonary venous drainage. Despite the right superior vena cava overriding the upper end of the atrial septum and draining into the left atrium, the child did not exhibit hypoxemia. Saline contrast transesophageal echocardiography suggested an explanation for the absence of hypoxemia.

A Rare Anomalous Systemic Venous Return With Bilateral Absence of Superior Vena Cava

The Video can be viewed in the online version of this article [10.1016/j.athoracsur.2021.02.056] on http://www.annalsthoracicsurgery.org. The Video can be viewed in the online version of this article [10.1016/j.athoracsur.2021.02.056] on http://www.annalsthoracicsurgery.org. A 24-month-old boy, who had history of abnormal echocardiograms showing possible anomalous systemic venous return, underwent cardiac magnetic resonance imaging (MRI) for further evaluation. Cardiac MRI confirmed bilateral absence of the superior vena cava (SVC). The right subclavian vein (RSV) and the right jugular vein (RJV) drain into the azygos vein (AV) (4.8-mm diameter), which courses inferiorly. The left subclavian vein (LSV) and the left jugular vein (LJV) drain into the hemiazygos vein (HAV) (7.7 × 6.6 mm diameter), which also courses inferiorly and is dominant. The hemiazygos and azygos veins join together at approximately T9 (asterisks), the level of the diaphragm into a single dominant vein to the left of the midline. This anomalous vein courses inferiorly from T9 to approximately the L1 level (12.2 × 6.6 mm diameter at T12) and then loops superiorly and to the right to join the prominent inferior vena cava (IVC) above the level of the renal veins (Figure 1; Video). Figure 1 is the posteroanterior (PA) view. The aorta (Ao) looks small in the images because of the timing of the image acquisition. Congenital anomalies of the SVC are rare and often coexist with other cardiac abnormalities.1Oliveira J.D. Martins I. Congenital systemic venous return anomalies to the right atrium review.Insights Imaging. 2019; 10: 115Crossref PubMed Scopus (19) Google Scholar Total absence of the SVC is a very rare anomaly, and we present 3-dimensional MRI images of this anomaly. https://www.annalsthoracicsurgery.org/cms/asset/51dac1ee-4da9-46a3-a866-6767ee5e74eb/mmc1.mp4Loading ... Download .mp4 (0.89 MB) Help with .mp4 files Video

Anesthesia for noncardiac intervention in a patient with total anomalous systemic venous return

Anesthesia for noncardiac intervention in a patient with total anomalous systemic venous return

Defects in Laterality with Emphasis on Heterotaxy Syndromes with Asplenia and Polysplenia: An Autopsy Case Series at a Single Institution

Heterotaxy is a rare disease with high morbidity and mortality. Controversy exists over how to classify these syndromes with most cases stratified into asplenia/polysplenia syndromes or right/left isomerism. In an effort to review comprehensively specific pheonotypes associated with heterotaxy syndromes, we reviewed published cases series, adopted a classification scheme based on spleen status, and evaluated autopsy cases retrospectively with abnormal laterality at our institution. We categorized 116 cases as situs inversus totalis, polysplenia, asplenia, and single right-sided spleen. Cardiovascular abnormalities occurred in 87.1% of polysplenia, 90.5% of asplenia, and all cases of single right-sided spleen. For polysplenia, 48.9% had bilateral bilobed lungs, 87% had right-sided stomach, 58.1% had midline symmetric liver, and 60.4% had malrotated intestines. For asplenia, 51.9% had bilateral trilobed lungs, 86.7% had right-sided stomach, 45.8% had symmetric liver, and 65.5% had malrotated intestines. Atrioventricular septal defects occurred in 91.2% of asplenia compared to 56.8% of polysplenia cases. Eight percent had pulmonary/aortic stenosis or atresia. Double outlet right ventricle was more common in polysplenia (32.6%) compared to asplenia (21.4%). Total anomalous systemic venous return was described in 55.6% of polysplenia and total anomalous pulmonary venous connections in 81% of asplenia cases. Greater than half of the cases had no heterotaxy diagnosis. Although, we found similar heterotaxy-associated characteristics, the frequencies differed from previous studies. We found great variation in how heterotaxy-associated defects were described, diagnosed, and reported. Although there are known associated characteristics with the polysplenia/asplenia syndromes, correct identification requires a standardized approach for diagnosis and reporting.

The Anomalies of Systemic Venous Connections in Children with Congenital Heart Disease

Anomalous systemic venous return (AVSR) may sometimes present as pathologic entities itself, or associated with other congenital heart defects. The presence of AVSR may require significant changes in surgical technique during the repair of congenital heart defects. The aim of our study was to assess the prevalence of anomalous systemic venous return and to determine the congenital heart anomalies accompanying AVSR in children. This study is based on a retrospective review of the medical records of 175 children who consecutively underwent cardiac catheterisation because that congenital heart disease. The most extensively diagnosed cardiac malformation was VSD (26.8%). ASVR was prevalent in 4.5% of patients. The most commonly diagnosed ASVR was persistent left superior vena cava (3.4%) and a second ASVR type was associated interruption of the inferior vena cava (IVC) with azygos vein continuation (1.1%). A systematic study of systemic venous connections should be performed in all those undergoing cardiac catheterisation as a prelude to open heart repair because it may require significant changes in surgical technique during the repair.

Total anomalous systemic venous drainage with heterotaxia syndrome: a rare case.

Total anomalous systemic venous return is a very rare anomaly, where vena cava inferior, vena cava superior, and coronary sinus drain into left atrium. Two-day-old male baby was admitted with cyanosis and tachypnea after the birth. Left atrial isomerism with anomalous systemic venous drainage was found on echocardiographic examination. We present an unusual case of total anomalous systemic venous drainage in to the left atrium.

Unroofed coronary sinus and persistent left superior vena cava: A case report

The unroofed coronary sinus is a spectrum of cardiac anomalies in which part or all of the common wall between the coronary sinus and the left atrium is absent. Most cases are associated with anomalous systemic venous return, such as persistent left superior vena cava.The diagnosis of this lesion is important to the prognosis of the patient, but it is often difficult because of nonspecific clinical features.Here we report a case of a complete unroofed coronary sinus and persistent left superior vena cava discovered during a computed tomographic investigation for pulmonary hypertension.

Impact of Genetic Diagnosis on Clinical Management of Patients With Congenital Heart Disease

The first Blalock-Taussig shunt surgery in 1945 changed major congenital heart disease (CHD) from a universally lethal condition into a survivable one.1 In the 50 years that followed, rapid progress in anatomic diagnosis via echocardiography and dramatic improvement in surgical and catheterization techniques resulted in survival to adulthood and greatly improved quality of life for many individuals with CHD. Despite this, continuing progress in caring for individuals with the most complex forms of CHD has become incremental. One of the particularly frustrating aspects of managing patients with very complex CHD is that the outcomes are highly variable, even when accounting for surgical skill and anatomic complexity. In this issue of Circulation , Nakhleh et al2 show that a subset of patients with CHD and heterotaxy have airway ciliary dysfunction that may account for increased morbidity and mortality independently of the complexity of the CHD. This finding highlights a critical point that will surely affect patient care in the years to come; improving our understanding of the genetic origin of CHD is likely to affect response to therapy. The hope is that individualized therapy for CHD based on the underlying genetics will contribute to the next major improvement in outcome. Article see p 2232 Heterotaxy is defined as any arrangement of organs across the body's left-right axis that differs from complete situs solitus and complete situs inversus. CHD is one of the hallmarks of heterotaxy and is characterized by a wide range of highly complex cardiac anatomy,3,4 including but not limited to anomalous systemic and pulmonary venous return, complex atrioventricular canal, functionally single ventricle, and a range of abnormalities of the outflow tract (Table). Thus, the cardiac lesions associated with heterotaxy represent a significant surgical challenge; however, the surgical outcome in heterotaxy patients remains significantly …

Index of Suspicion * Case 1: Irritability and Signs of Peritonitis in a Neonate * Case 2: Red Eye, Excessive Tearing, Photophobia, Irritability, and Splenomegaly in an Infant * Case 3: 2-year-old Girl Who Has Cough, Right Pulmonary Abnormalities

* ALL: : acute lymphoblastic leukemia CT: : computed tomography IVC: : inferior vena cava NA: : neonatal appendicitis NEC: : necrotizing enterocolitis WBC: : white blood cell A 7-day-old boy born at term is brought to the emergency department by his foster mother for refusal to feed for ∼20 hours and increased crying, although he is intermittently consolable. When given his bottle, the infant starts to suck but then cries as though in pain. He has not vomited, and his last bowel movement was yesterday. The stool has been moderately well formed without blood or mucus, and wet diapers have continued. There is no history of respiratory symptoms, lethargy, rashes, or jaundice. The biological mother is a 27-year-old woman with a known history of previous drug abuse; she had received some prenatal care. This infant was born by scheduled cesarean delivery. Initial physical examination reveals a crying but consolable neonate. His temperature is 37.7°C, heart rate is 170 beats/minute, and respiratory rate is 56 breaths/minute; the remainder of his findings are normal. His complete blood count (white blood cell [WBC] 11.7 × 109/L, 78.5% neutrophils, 11.4% monocytes, 9.8% lymphocytes), serum concentrations of electrolytes and glucose, urinalysis, and cerebrospinal fluid studies are normal. Cultures are obtained. An abdominal radiograph is normal and abdominal ultrasonography reveals trace pelvic fluid. He is given a bolus of normal saline and is started on antibiotics to treat possible sepsis. A few hours after admission, repeat examination is concerning for inconsolability, poor suck reflex, and lethargy. His abdomen is distended, firm, and tender to palpation. Bowel sounds are normal. Further studies are performed that reveal the diagnosis. A previously healthy 10-month-old boy is seen for a 2-week history of an erythematous left eye with tearing. Earlier, he was diagnosed as having conjunctivitis at an urgent care clinic and was treated with a 10-day course of polymyxin B/trimethoprim eye drops without improvement. He has felt warm intermittently but has not had a …

Prenatal diagnosis of abnormal cardinal systemic venous return without other heart defects: a case series

To describe fetal spectrum and echocardiographic characteristics of anomalous systemic venous return (ASVR, cardinal veins) without other structural heart defects (isolated ASVR), evaluate associated extracardiac and genetic anomalies and review neonatal outcome. From 2003 to 2009, 369 consecutive fetuses were diagnosed with cardiac malformation, including 27 cases with an isolated ASVR. The following variables were collected: type of ASVR, extracardiac anomalies, karyotype and short-term outcome. The most common forms of ASVR were persistent left superior vena cava (SVC) (n = 15) and interrupted inferior vena cava (IVC) (n = 6). The other ASVR types were a persistent left SVC without a right SVC (n = 4), bilateral agenesis of the SVC with cephalic venous drainage toward the IVC through the azygos system (n = 1) and total ASVR to the coronary sinus (n = 1). Genetic disorders were confirmed in five cases, all with a persistent left SVC, with extracardiac anomalies present in four of them. Ten other patients had extracardiac anomalies detected prenatally or postnatally. When available, ASVR diagnosis was confirmed postnatally in all cases. Isolated ASVRs can be accurately diagnosed prenatally and are frequently associated with extracardiac and genetic anomalies. A detailed morphological ultrasound and fetal karyotype should be suggested. Neonatal outcome was mainly related to the associated anomalies.

Mouse Models of Congenital Heart Defects

One of the puzzling things about genetic disruptions in mice that affect heart development is the overwhelming number of conotruncal anomalies and the infrequency with which certain other malformations are reported. For instance, venous malformations such as total anomalous pulmonary venous return (TAPVR) or partial anomalous pulmonary venous return (PAPVR) have not been reported in a mutant mouse. Coronary artery anomalies are reported infrequently, and when they are reported it is because they are obvious. Conotruncal anomalies such as common trunk, double-outlet right ventricle, and pulmonary stenosis with overriding aorta are frequently associated with mouse germ line and conditional gene deletions. Although conotruncal malformations represent the highest percentage of congenital heart defects in the human population, TAPVR and PAPVR occur in a significant number of babies. Further, coronary anomalies affecting the stems of the coronary arteries frequently are seen in conotruncal malformations in babies but are rarely reported in mouse models. Article see p 314 Isolated TAPVR is a rare lesion and occurs in only about 1 in 17 000 live births.1 PAPVR is much more common and has been reported in 1 in 160 individuals (0.6%) and 0.4% to 0.7% of postmortem examinations, with 90% of these associated with an atrial septal defect.2 The majority of patients with TAPVR have symptoms of cyanosis and congestion within the first year of life. If left untreated, about 80% would die. In contrast, PAPVR frequently is not discovered until adulthood and is not usually a cause of death. Although the overall incidence of PAPVR in autopsy cases has been estimated to be 0.7%,3 the true prevalence may be higher,4 as several reports have appeared with PAPVR as an incidental finding that in most of these cases is asymptomatic. PAPVR can present as an isolated structural abnormality, but it …

Intraatrial Baffle Repair of Anomalous Systemic Venous Return Without Hepatic Venous Drainage in Heterotaxy Syndrome

A 7-month-old boy with heterotaxy syndrome had partial atrioventricular septal defect and interrupted inferior vena cava with hemiazygos continuation to a left superior vena cava. The left side of the common atrium receiving all the venous drainage was in connection with the left ventricle and the aorta. The small atrium and the proximity of the pulmonary and hepatic vein orifices precluded complete baffling. This report describes an intraatrial baffle repair of anomalous systemic venous return without hepatic venous drainage. This resulted in good oxygenation postoperatively, with oxygen saturation ranging from 93% to 98%.

Prenatal diagnosis of isolated total anomalous systemic venous return to the coronary sinus

Prenatal diagnosis of isolated total anomalous systemic venous return to the coronary sinus

Intracardiac Fontan procedure for heterotaxy syndrome with complex systemic and pulmonary venous anomalies☆

The extracardiac conduit procedure is widely used for patients with heterotaxy syndrome with complex systemic and pulmonary venous anomalies; however, it lacks conduit-growth potential and requires long-term anticoagulation. We present the intracardiac Fontan procedure, which eliminates the above-mentioned disadvantages. Twenty-four patients (mean age, 4.1 years; weight, 13.0 kg) with heterotaxy syndrome underwent intracardiac Fontan operations between March 1995 and March 2008. In each patient, the anomalous systemic venous return with the isolated hepatic vein was redirected to the pulmonary artery using an intra-atrial baffle without obstructing the pulmonary venous pathway; this was accomplished by anterior (n=5), lateral (n=15) or posterior tunnel methods (n=4), depending on the anatomical relationship of the systemic and pulmonary venous pathways. There was one (4%) in-hospital and two (8%) late deaths in total. Five (21%) patients underwent re-operation for either pulmonary venous obstruction or supraventricular tachycardia. The actuarial 12-year survival was 86% (the Kaplan-Meier survival plot). The freedom from re-operation at 10 years was 77%. Anticoagulation was not required except for two patients (8%) who had prosthetic valves and coagulation disorder. Obstruction of the systemic venous pathway was not observed in any patient; however, five (20%) patients had clinically significant postoperative arrhythmias. At the final follow-up, all survivors were categorised as the New York Heart Association class I. Aided by detailed preoperative anatomical and physiological diagnoses, intracardiac Fontan procedures were technically feasible in patients with complex systemic and pulmonary venous anomalies. The specific cardiac anatomy in these patients warranted this procedure; however, taking into consideration the improved outcomes of the modified Fontan procedure, this method should be performed with deliberation.

Evaluation of Anomalous Systemic and Pulmonary Venous return in a Young Child with Heterotaxy: A Case Study—Implications for Military Dependent Care

The case of a young male patient with a complex admixture lesion who required a comprehensive anatomic evaluation before palliative cardiac surgery is presented. We describe a safe anesthesia protocol for obtaining the late-acquisition, gadolinium-enhanced, magnetic resonance angiographic images necessary to define the complex pulmonary and systemic venous anatomic features of his cardiac admixture lesion. Subspecialty physician staffing implications for the care of military dependants with congenital heart disease who might benefit from evaluation using this safe simple protocol and readily available magnetic resonance imaging technology are addressed.

Operative Correction of Cor Triatriatum Sinister With Systemic Venous Return Anomaly (Inferior Vena Cava?Left Atrium)

Cor triatriatum sinister is a rare cardiac anomaly. It is characterized by the presence of a fibromuscular membrane in the left atrium, thus forming a proximal chamber receiving the pulmonary veins and a distal chamber communicating with the mitral valve and the left atrial appendage. This rare pathology tends to coexist with a persistent left superior vena cava, whereas the presence of an anomalous systemic venous return is unusual. In this article, we report successful treatment of a patient with cor triatriatum sinister who has an additional systemic venous return anomaly.

A complex anomaly of systemic and pulmonary venous return associated with sinus venosus atrial septal defect

This report describes a rare case of anomalous systemic and pulmonary venous return that was surgically corrected. It consisted of left inferior vena cava with hemiazygous continuation into the persistent left superior vena cava, partial anomalous pulmonary venous return from the right lung and sinus venosus atrial septal defect.