Anomalous Venous Connection Research Articles

Pulmonary vein and atrial wall pathology in human total anomalous pulmonary venous connection

Background Normally, the inside of the left atrial (LA) body and pulmonary veins (PVs) is lined by vessel wall tissue covered by myocardium. In total anomalous pulmonary venous connection (TAPVC), no connection of the PVs with the LA body exists. These veins have an increased incidence of PV stenosis. We describe the consequences of the absent connection for the histopathology of the wall of the LA body and the PVs, and hypothesize on a mechanism predisposing to PV stenosis. Methods and results In 10 human neonates with TAPVC, the wall of the LA body and PVs were studied using histological and immunohistochemical techniques. As controls, 2 normal neonatal and adult hearts and 5 neonatal hearts with partial anomalous venous connection (PAPVC) or situs inversus were studied. In hearts with TAPVC no vessel wall tissue was found in the LA body and its myocardial layer was hypoplastic. No myocardial sleeve was found around the abnormally draining PVs. In hearts with PAPVC, only the non-LA draining PV lacked myocardial covering, whereas in situs inversus PVs connecting to the right-sided LA, were normally myocardialized. Conclusion An open connection of the PVs with the morphological LA is necessary for the presence of vessel wall tissue in the LA and myocardialization of the PVs. Absence of myocardium covering the PVs is hypothesized to enhance susceptibility to PV stenosis and prevent onset of PV originating arrhythmias. The embryonic posterior heart field may be responsible for the abnormal myocardialization and smooth muscle cell formation in TAPVC.

Abnormal direct entry of the umbilical vein into the portal vein: report of a case

Abnormalities of the intra-abdominal umbilical venous connections are rare congenital vascular anomalies. We report a case of an aneurismal dilatation of an umbilical vein directly connected to the portal vein, vital for the delivery of placental blood in the fetus. A term female newborn presented with a dilated umbilical vein detected by prenatal ultrasound. CT angiography revealed an aneurismal dilatation of a vascular structure, from the umbilical ring to the main portal vein. Follow-up CT angiography showed thrombus progression up to the level of SMV inlet. A laparotomy revealed that the normal course of the umbilical vein was absent. The aneurysm was resected and no thrombus was noted in the aneurysm. Post-operative ultrasound revealed an isolated thrombus at the right main portal vein that resolved spontaneously on the follow-up ultrasound. At the 6-month post-operative follow-up, the baby was well and without complications. Although not evaluated for the presence of a ductus venosus, this anomalous umbilical-portal venous connection might have been associated with an absent ductus venosus.

A Rare Form of Supracardiac Total Anomalous Pulmonary Venous Drainage—Evaluation by Computed Tomography and Magnetic Resonance Imaging

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon congenital cardiac anomaly. It constitutes about 2– 5% of all congenital cardiac diseases at autopsy [1,2]. In TAPVD, the pulmonary veins may drain aberrantly into any tributary of the sinus venosus. There are four main patterns of drainage: supracardiac, cardiac, infradiaphragmatic, and mixed. Echocardiography is a useful screening technique for demonstrating the presence and site of anomalous drainage, including assessment of obstruction and associated intracardiac anomalies [3–5]. However, it is limited in its ability to demonstrate the draining vein if this is located away from the chest wall. Angiocardiography still remains the standard investigation [6]. However, this technique is invasive and has a significant risk of complications, especially in cyanosed neonates and infants. The role of computed tomography (CT) and magnetic resonance imaging (MRI) for evaluation of TAPVD has been well described in the literature [6,8–10]. Both these techniques are capable of demonstrating the anomalous venous connection, including the evaluation of obstruction, at any site and in any desired plane. We describe the CT and MRI features in a very rare variant of supracardiac TAPVD, with an intrapulmonary location of the common venous channel. Only 10 such cases have been reported so far, five of whom had normal cardiac anatomy (as in the case presented) [7]. To our knowledge, the CT and MRI findings in this form of drainage have not been described previously.

Prenatal diagnosis of cardiosplenic syndromes by fetal echocardiography: a 10‐year experience

Purpose: To assess the accuracy of fetal echocardiography in the diagnosis of cardiosplenic syndromes.Methods: Retrospective survey of 27 fetuses with the pre‐ or postnatal diagnosis of cardiosplenic syndromes in our database over a period of 10 years.Results: In 24 out of 27 fetuses, the diagnosis of cardiosplenic syndrome was confirmed postpartum. In the remainder, two cases of situs inversus totalis with heart block and multiple cardiac malformations, but no evidence of heterotaxy were diagnosed at autopsy or after birth. In one fetus with situs ambiguus, dextrocardia, bradyarrhythmia and transposition of great arteries (TGA) no autopsy was performed after termination of pregnancy. In 18 fetuses, left atrial isomerism were prenatally diagnosed and confirmed at autopsy or postpartum. Main echocardiographic features were azygos continuation of interrupted inferior caval vein (15), complete atrioventricular septal defect (CAVSD) (11), persistent bradyarrhythmia (11), pulmonary stenosis (PS) (9), ventricular septal defect (VSD) (8), fetal hydrops (8) and dextrocardia (5). Other associated cardiac malformations were l‐TGA (2), double outlet right ventricle (DORV) (3), hypoplastic left ventricle (3), and partial/total anomalous venous connection (1/1). Ten pregnancies were terminated, two fetuses demised in utero, six children survived. In six fetuses, right atrial isomerism was prenatally diagnosed and confirmed postpartum. Echocardiographic features were CAVSD (4), dextrocardia (4), PS (2), l‐TGA (1), azygos continuation of interrupted inferior caval vein (1), fetal hydrops (1), DORV (1) and bradyarrhythmia with cardiomegaly (1). One pregnancy was terminated, two fetuses demised within 10 days postpartum, three children survived.Conclusion: A combination of CAVSD, first and early second trimester persistent bradyarrhythmia and/or azygos continuation of interrupted inferior caval vein, and/or visceral situs ambiguus should suggest specific diagnosis of cardiosplenic syndrome. The correct diagnosis made in utero allows for maternal counseling and transfer to an appropriate facility prior to delivery for corrective surgery.

Retrocardiac Repair of Total Anomalous Pulmonary Venous Connection

Surgical repair of total anomalous pnhnonary venous connection (TAPVC) has involved a number of techniques using various strategies of cardiopulmonary bypass and circulatory arrest, methods of cardioplegia, sites of venous cannulation, and cavitary exposures. 1-a The initial successful report by Cooley in 19574 used a right atrial approach with a transatrial septal exposure for the puhnonary veinto-left atrium anastomosis. In 1964, Williams 5 described a one-stage retrocardiac approach using cardiopuhnonary bypass for type I TAPVC. This concept was expanded by Ebert 6 to include both type I and type III TAPVC using cardiopuhuonary bypass and hypothermia strategies, which avoided deep hypothermia-eireul atory arrest as well as cardioplegia. 7 The clinical outcomes have steadily improved over the years because of such advances as aeeurate eehoeardiographie diagnosis, improved eardiopuhnonary bypass technology, deep hypothermia-eire ulatory arrest strategies, and comprehensive pediatric inten~ sire eare. The operative survival rate has been as high as 95% in some series. Nevertheless, recent clinical investigations have uneovered some longterm negative effects of prolonged deep hypothermiacirculatory strategies and raised questions about their safety even for limited periods, a These reports have triggered new interest in perfornfing these operations with continuous eardiopulmonary bypass using moderate hypotliernfie eonditions and more invasive eannulation techniques. A portion of this volume has been dedicated to the various operative techniques tlmt have been instrumental in improving the clinical outeomes for infants with this eongenital anomaly. This article presents the salient techniques of retroeardiac repair of TAPVC using standard eardiopulmonary bypass and antegrade eardiople~a strategies.

Prenatal Diagnosis of Total Anomalous Pulmnonary Venous Connection

Total anomalous pulmonary venous connection remains a difficult diagnosis to make prenatally. The absence of normal pulmonary veins entering the left atrium may provide an indication of an abnormality. The authors discuss a case of total anomalous pulmonary venous connection in conjunction with hypoplastic left heart syndrome that was diagnosed in utero.

Is Vertical Vein Ligation Necessary in Repair of Total Anomalous Pulmonary Venous Connection?

Background. In the repair of total anomalous venous connection, vertical vein ligation is recommended to eliminate left-to-right shunting. However, the small left heart chambers may not always tolerate the immediate increase in blood flow after combined repair and vein ligation. Methods. A retrospective review of 23 infants and children undergoing correction of total anomalous pulmonary venous connection was undertaken to determine whether vertical vein ligation is a necessary component of successful surgical repair. In 14 patients this vein was ligated, whereas in 9 it was left patent. Six patients who underwent ligation and 5 who did not had pulmonary venous obstruction before operation. Results. The operative mortality rate was 36% (5 of 14 patients) for the ligated group compared with 0% (0 of 9 patients) for the nonligated group ( p = 0.06). All deaths occurred in patients with preoperative obstruction and a low mean left atrial pressure, and four of the deaths were directly attributable to left heart failure. Follow-up echocardiography in patients in whom the vertical vein was not ligated revealed adequate cardiac function and no residual left-to-right flow through the previously patent venous conduit. Conclusion. Vertical vein ligation during the repair of total anomalous pulmonary venous connection is not routinely necessary and actually may be undesirable in patients with preoperative obstruction, in whom the left heart chambers are particularly small.

Letter to the Editor: Intrahepatic Portal Tracts Abnormalities Associated with Anomalous Venous Connections

Letter to the Editor: Intrahepatic Portal Tracts Abnormalities Associated with Anomalous Venous Connections

Bidirectional cavopulmonary shunt in patients with multiple risk factors

Twenty-five patients in whom a modified Fontan operation was deferred because of multiple risk factors underwent a bidirectional cavopulmonary shunt. Two or more of the following risk factors were present in all: age less than 1 year, severe pulmonary artery distortion, impaired left ventricular function, subaortic obstruction, anomalous systemic-pulmonary venous connection, atrioventricular valve incompetence, and increased mean pulmonary artery pressure. Additional procedures included take-down of systemic-pulmonary artery shunt, atrial septectomy, pulmonary artery reconstruction, bulboventricular foramen enlargement, and atrioventricular valve repair. There were three hospital deaths (12%). Mean follow-up is 21 months. There was no late mortality. Mean oxygen saturation increased from 71% to 83%. Results obtained from pulmonary artery reconstruction, enlargement of bulboventricular foramen, and atrioventricular valve repair were satisfactory for the patients in whom these risk factors were present preoperatively. Ventricular function also improved in the survivors in whom it was previously deteriorated, this being related to the suppression of the sources of ventricular volume overload. In this risk group of patients for a modified Fontan operation, a bidirectional cavopulmonary shunt provided adequate palliation at reasonable low risk. Early bidirectional cavopulmonary shunt would minimize complications originating from systemic-pulmonary shunts such as pulmonary artery distortion and the potential harm of chronic ventricular volume overload.

Transesophageal echocardiographic diagnosis of sinus venosus-type atrial septal defect associated with partial anomalous venous connections during cardiac surgery in adults

T RANSESOPHAGEAL echocardiography (TEE) is useful for intraoperative monitoring of cardiac function 1,z and for evaluating valve repair. 3 Recently, TEE has been proposed as a useful intraoperative tool improving diagnostic accuracy and assisting in surgical repair of pediatric congenital heart malformations. 4 Its role in the diagnosis of congemtal cardiac malformations in adults has not been widely discussed. The sinus venosus variant of atrial septal defect (SVASD) occurs in up to 20% of adults with an atrial septal defect (ASD) and is the most difficult to Identify. 5 This defect is frequently associated with anomalous connection of the right pulmonary veins to the junction of the right atrium and the superior vena cava. 6 The authors describe two cases of adult cardmc surgery where intraoperative TEE helped to diagnose previously unsuspected SVASD associated with partial anomalous pulmonary venous connections (PAPVC) and discuss how the echocardiography results led to modification of the planned operation.

Horseshoe lung associated with anomalous pulmonary venous connection without pulmonary hypoplasia

SummaryHorseshoe lung is an exceedingly rare congenital malformation, characterized by unilateral pulmonary hypoplasia together with a midline isthmus producing fusion of the tissues of the lower lobes. It is frequently associated with other cardiac and extracardiac anomalies. We report an infant with a variant of the horseshoe lung with partial anomalous venous connection of intracardiac type, ventricular septal defect, and persistent left superior caval vein. There was no pulmonary hypoplasia.

The modified Fontan operation in hearts associated with atrioventricular valvar atresia or common atrioventricular valve—neoseptation of the atriums using a right atrial flap

SummarySummary Between June 1988 and December 1992, six patients with dominant left and rudimentary right ventricles underwent orthoterminal correction with a modified Fontan operation in which the atriums were neoseptated using a flap constructed from the right atrial wali. Four patients had anomalous systemic venous connections. The operations were performed under direct caval cannulation, standard cardiopulmonary bypass, moderate hypothermia and cardioplegic arrest. After opening the right atrium with a longitudinal incision, the atrial septum was completely resected and the coronary sinus was cut back. The upper wall of the right atrial incision was brought down in such a way that the pulmonary venous atrium was drained into the dominant ventricle via the right-sided or common atrioventricular valve. This was followed by direct connection of the right atrium with its anterior wall reconstructed with pericardium to the pulmonary arteries. In one patient, a left superior caval vein draining to the left atrium was divided and anastomosed to left pulmonary artery. One patient died on the 12th postoperative day with pulmonary infection and sepsis, though he had no hemodynamic problem, and another died on the sixth day due to high pulmonary vascular resistance. The remaining four patients are progressing well at a mean of 23.4 months postoperatively with functional capacity of NYHA I-IT and sinus rhythm. Echocardiographic and angiocardiographic examinations during follow-up showed unobstructed pulmonary and systemic venous pathways in all. This modification of the Fontan operation seems a good alternative technique which creates a contractile left atrium with large enough dimensions and an unobstructed pathway for pulmonary venous flow.

Usefulness of magnetic resonance imaging in the assessment of venoatrial connections, atrial morphology, bronchial situs, and other anomalies in right atrial isomerism

Multiple complex venous and intracardiac anomalies are frequently seen in patients with right atrial isomerism, and complete anatomic information may not always be obtained from combined echocardiography and angiography. Twenty patients with right atrial isomerism were prospectively studied with use of magnetic resonance imaging (MRI); satisfactory imaging was obtained in 18 patients, aged 6 days to 12 years (median 3 months). All patients had undergone echocardiography, cardiac catheterization, and angiocardiography. The purpose of this study was to assess the usefulness of MRI in the evaluation of venoatrial connections, atrial morphology, bronchial situs, and additional anomalies. Bilateral superior venae cavae were documented in 12 patients and anomalous hepatic venous drainage in 11. The pulmonary venous system was imaged in all 18 patients, of whom 12 had total anomalous pulmonary venous connection: 9 supracardiac, 2 infracardiac, and 1 mixed type. Some obstruction to pulmonary venous return was present in 9 of the 12 patients, and the site of obstruction was demonstrated by MRI in 6. Bilateral morphologic right atrial appendages were imaged in 7 patients. Bilateral morphologic right bronchi were identified in 17 patients, asplenia was confirmed in 17 of 18 patients by the absence of splenic tissue on imaging, and hiatal hernia was imaged in 4. Compared with angiography and echocardiography, MRI more clearly defined the pulmonary venous connections in 10 patients with pulmonary venous obstruction. Anomalous hepatic venous connections were better defined by MRI in 2. Therefore, MRI provided valuable supplementary information on the cardiovascular and visceral anomalies in these patients.

Absent left pulmonary vein without anomalous connection: Diagnosis and management in the newborn

Unilateral atresia of the pulmonary vein is an uncommon condition and is often associated with partial anomalous venous connection. Various forms of alternative channels have been described.l-” While the term “atresia” is used to denote the existence of a variable size and length of cordlike pulmonary veins without any lumen, “absence” is used here to denote that no such connection exists between the pulmonary hilum and left atrium, as a result of either agenesis or involution. To our knowledge, only two cases of unilateral absence of the pulmonary vein without anomalous connection have been reported in the English literature.4 We report a case of unilateral pulmonary edema caused by an absent left pulmonary vein without anomalous connection, diagnosed and managed successfully by surgery in a newborn. A full-term male infant (birth weight 3011 gm) was born by emergency cesarean section because of fetal bradycardia to a 23-year-old gravida I, white female. The child was

Magnetic resonance imaging in patients after a modified Fontan operation

SummaryMagnetic resonance imaging was performed in 26 patients who underwent a modified Fontan procedure. Their age ranged from 1.8 to 31 years with a mean of 12.2 years. A valveless anastomosis was performed between the right atrium and the rudimentary right ventricle in 12 patients and between the right atrium and the pulmonary arteries in 10 patients. A cavopulmonary connection was established in the remaining four patients. Spin echo and gradient echo scans of the heart were performed in orthogonal and angulated projections. The cross-sectional area of the atrioventricular, atriopulmonary or cavopulmonary anastomoses was measured using diameters in two orthogonal imaging planes. Recordings were examined for the presence of right atrial thrombosis, the site of drainage of the coronary sinus, compression of the pulmonary veins, as well as for the presence and extent of pericardial effusions. Cine recordings were used for the assessment of the pattern of flow within the right atrium. The cross-sectional area of the anastomoses could be determined in 24 of 26 patients. This was not statistically different between patients with different surgical procedures. Patients with a cavopulmonary connection, however, tended to have a smaller anastomosis (mean 1.4 cm2/m2BSA, S.D. 0.62) than patients with atrioventricular (mean 3.0 cm2/m2, S.D. 2.1) or atriopulmonary (2.4 cm2/m2, S.D. 1.1) connections. When compared to normal values for the size of the tricuspid valve, the size of the anastomosis was within the normal range in only four patients, it was larger in one and smaller in 19 patients. There were signs of right atrial thromboses in the scans in eight of 26 patients, as observed by two independent investigators. The site of drainage of the coronary sinus was imaged in 20 of 26 patients and was in accordance with the description of surgical procedure in eight. Compression of the right pulmonary veins by an enlarged right atrium was present in seven patients. This was severe in two children. Presence and extent of pericardial effusions could be adequately assessed in 11 of26 patients. Systolic regurgitation from the rudimentary right ventricle into the right atrium was shown in eight of 12 patients with an atrioventricular valveless anastomosis. Cine recordings revealed slow forward flow from the right atrium into the pulmonary arteries in seven of 22 patients, and there was a markedly altered pattern of intraatrial flow in two patients with anomalous systemic venous connections. Magnetic resonance imaging allows adequate examination of right atrial anatomy, determination of the size of the anastomosis with the pulmonary arteries, and semiquantitative assessment of pulmonary blood flow in the majority of patients after a modified Fontan procedure.

Assessment of anomalous systemic and pulmonary venous connections by transoesophageal echocardiography in infants and children.

To assess the value of transoesophageal echocardiography in the preoperative definition of systemic and pulmonary venous connections. Transoesophageal echocardiographic studies were performed prospectively under general anaesthesia in 76 consecutive unoperated children. Results were compared with those obtained by earlier transthoracic ultrasound studies (n = 76), cardiac catheterisation (n = 62), and subsequent surgical inspection (n = 58). Two tertiary referral centres. 76 unoperated infants and children (age 0.2-14.8 years, mean age 4.1 years) with congenital heart disease. Identification of anomalous systemic and pulmonary venous connections. Transoesophageal studies showed anomalous venous connections in 14 patients. Two had both anomalous systemic and pulmonary venous connections. Transoesophageal studies showed 12 anomalous systemic venous connections in nine patients. In eight patients these were confirmed at operation or catheterisation: one patient is awaiting operation. Six anomalous systemic venous connections were missed during earlier transthoracic studies. Anomalous pulmonary venous connections (one mixed total, six partial) were shown in seven patients. These were confirmed at operation in six and by cardiac catheterisation in one. Four of these patients were missed during earlier transthoracic ultrasound studies. No patient defined as having normal venous connections by the transoesophageal study was subsequently shown to have anomalous venous connections at operation or angiography. Transoesophageal echocardiography is a highly sensitive tool for the preoperative definition of systemic and pulmonary venous connections. In this series it was better than transthoracic ultrasound and complemented cardiac catheterisation and angiocardiography.

MR Imaging of Partial Anomalous Pulmonary Venous Connections

Magnetic resonance imaging was performed on 11 patients with partial anomalous pulmonary venous connections (PAPVC). Ten of these patients also had echocardiographic examinations, eight of which included color-flow Doppler studies. The diagnosis of PAPVC was confirmed in each of these patients by surgery or angiography. Fourteen anomalous pulmonary venous connections were identified, 10 involving the right upper lobe pulmonary vein and 4 involving the left upper lobe pulmonary vein. This retrospective review demonstrated that all 14 anomalous venous connections were correctly identified by MR imaging, whereas only 8 of 13 (62%) were identified by echocardiography. With MR, 89% of all the pulmonary veins and 93% of the anomalous pulmonary veins were visualized on axial images, while 41% of all pulmonary veins and 71% of anomalous veins were seen on coronal MR images. There were five atrial septal defects (ASDs), four of the sinus venous type and one of the septum secundum type. All five ASDs were correctly identified with MR imaging; three of four ASDs were identified with echocardiography. We conclude that MR imaging provides an accurate noninvasive method of depicting the anatomic abnormalities associated with PAPVC.

Transesophageal echocardiography is superior to transthoracic echocardiography in the diagnosis of sinus venosus atrial septal defect

The purpose of this study was to compare transthoracic and transesophageal echocardiography in the diagnosis of various types of atrial septal defects. Forty-one adult patients with the clinical diagnosis of atrial septal defect were studied by transthoracic and transesophageal echocardiography (30 women, 11 men; 18 to 81 years of age). Transthoracic echocardiography demonstrated the atrial septal defect in 33 patients (secundum type in 28, primum type in 3 and sinus venosus type in 2). Transesophageal echocardiography demonstrated the defect in all 41 patients. Thus, in 8 (20%) of 41 patients the atrial septal defect was demonstrated by transesophageal and not by transthoracic echocardiography. Six of the eight had a sinus venosus type atrial septal defect; the other two patients had a secundum atrial septal defect (one of these two had a technically poor transthoracic echocardiogram and the other had a small atrial septal defect). Transthoracic echocardiography, therefore, failed to demonstrate the sinus venosus defect in six (75%) of eight patients. An anomalous venous connection associated with the sinus venosus defect was visualized by transesophageal echocardiography in seven of the eight patients but was not seen on transthoracic echocardiography in any patient. Sinus venosus type atrial septal defects are frequently not visualized in adults by conventional transthoracic echocardiography. Transesophageal echocardiography is recommended when an atrial septal defect is clinically suspected but cannot be visualized by transthoracic echocardiography.

An enormous shunt between the portal and hepatic veins associated with multiple coronary artery fistulas

A 9-year-old boy with a rare combination of multiple coronary artery fistulas and congenital shunt between the portal and hepatic veins (portal-systemic shunt) is presented. The most likely pathogenesis for the portal-systemic shunt in this case was persistence of the ductus venosus as a bypass tract of the portal vein. This shunt is considered as one cause of cardiomegaly and dilatation of the hepatic vein in this case, and careful follow-up is mandatory because this shunt could induce portal-systemic encephalopathy. There are a few reports of an anomalous portal venous connection to the hepatic vein as a result of abnormal portal embryogenesis. We experienced a unique case of congenital portal-systemic shunt associated with coronary artery fistulas. The present paper reports angiographic findings in this case and the etiological importance of the congenital portal-systemic shunt.

Hepatic venous connection to a persistent inferior caval vein in left isomerism

In 22 cases of left atrial isomerism studied at the Brompton Hospital, four cases were found to have an unusual arrangement of the abdominal vessels. There was persistence of an inferior caval vein, partially anomalous hepatic venous connection and additional continuation of part of the venous return from the lower body through the azygos venous system. This venous pattern had surgical implications in our index case, since redirection of the inferior caval venous return was necessary.