Anomalies Of Systemic Venous Return Research Articles

Systemic Venous Return Abnormality Associated with An Abnormality of Total Pulmonary Venous Return

This is a 9-year-old child hospitalized for an intraoperative partial venous return anomaly and after an exploration we found a complex congenital cardiac pathology such as an anomaly of total venous return associated with an anomaly of systemic venous return which is mainly seen at early ages Mots-clés Anomalie de retour veineux systémique, Anomalie de retour veineux pulmonaire total, Collecteur Confluence pulmonaire, Urgence néonatale

Clinical presentation, conventional/4D spatio-temporal image correlation imaging findings, pregnancy and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects: A retrospective study from two centres in Southern India.

Recent advances in fetal ultrasound imaging, especially four-dimensional (4D) spatio-temporal image correlation techniques permit detailed evaluation of the fetal venous system and its abnormalities. In this report, we present the clinical presentation, conventional/4D spatio-temporal image correlation imaging findings, pregnancy, and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects from two centres in southern India. To report the clinical presentation, conventional/4D spatio-temporal image correlation imaging findings, pregnancy, and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects from two centres in Southern India. Retrospective study (October 2017 to March 2022). All fetuses referred with abnormal cardiac imaging findings who were diagnosed to have anomalies of systemic venous return in the absence of significant intracardiac defects were included. Imaging techniques like 2D with color and 4D spatio-temporal image correlation fetal imaging were used to evaluate systemic venous anomalies. Systemic venous anomalies were grouped into cardinal venous anomalies and umbilico-portosystemic venous anomalies. Thirty-nine fetuses were included; this represented 4.4% of all cardiac anomalies diagnosed during the study period. Cardinal venous anomalies were seen in 29 fetuses (74%); the mean gestation age at diagnosis was 25.5±3.4weeks. Absent dilated coronary sinus was associated with anomalous drainage of superior caval vein through completely unroofed coronary sinus into the left atrium (n=3) which resulted in cyanosis after birth in all infants. Among the three cases, one underwent surgery at 6 months of age due to a progressive increase in cyanosis (Spo2 78%) and others are awaiting surgery. A prenatal genetic evaluation was performed in 17 (58.6%). Cardinal venous anomalies were not associated with genetic abnormalities. Live births occurred in all fetuses with cardinal venous anomalies. Umbilico-portosystemic venous anomalies were seen in 10 (26%). The mean gestation age at diagnosis was 26.5±4.5weeks. Except in a fetus with the extrahepatic portosystemic shunt (umbilical vein to iliac vein), the genetic evaluation was normal in our cohort. Extracardiac anomalies (n=3/10; 30%) were seen in both intra (n=1) and extrahepatic portosystemic shunts (n=2). Anomalies of systemic venous return in the absence of significant intracardiac, extracardiac, and genetic abnormalities carried an overall favorable prognosis in our cohort. Precise characterization of anatomic details using advanced imaging techniques helps in the understanding of this complex three-dimensional anatomy and in the prognostication of these anomalies.

Bilateral superior vena cava with right superior vena cava draining into left atrium

Anomalies of systemic venous return are extremely heterogeneous congenital malformations with variable ranges from completely normal physiology to severe forms of right to left shunting requiring surgical treatment. Anomalous drainage of a right-sided superior vena cava (SVC) to the left atrium (LA) is one of the rarest variants of systemic venous return anomalies, characterized by right-to-left shunt physiology and cyanosis. Here we report a 2years old girl presented with cyanosis which was observed shortly after birth by her parents but not further investigated. She is otherwise active girl and with normal growth and development. Her clinical examination was unremarkable apart from mild clubbing of the fingers and low oxygen saturation of 88–90% in room air. Her ECG and chest X-ray were unremarkable. Echocardiography showed bilateral SVC connected by a small innominate vein. The right SVC drains directly into the LA while the left SVC drains into the right atrium (RA) via a dilated coronary sinus. There is a small superior sinus venosus type atrial septum defect (ASD) with left to right shunt. Also, there is partial anomalous pulmonary venous return with right upper and right middle pulmonary veins draining directly into the right SVC, which is connected to LA. The right lower pulmonary vein and left pulmonary veins drain directly to LA. The rest of her echocardiography demonstrated normal heart structures and function. This patient was referred for surgical correction, including baffling of the right SVC to the RA and closure of the ASD. We describe this case to highlight the importance of recognizing this rare anomalous systemic venous connection as one of the very rare causes of cyanosis in the pediatric age group as well as at older age.

Heart Transplant: Transplantation for Congenital Heart Disease

Cardiac transplantation for congenital heart disease incorporates aspects of both reparative and replacement surgery. Although intracardiac congenital malformations are replaced, and therefore pose few obstacles to the transplant surgeon, extracardiac malformations (congenital, acquired, or iatrogenic) can present considerable challenges to the operative team. Before transplantation, a full comprehension of the operative plan for the management of each individual patient is essential for both the donor team (so that they may harvest donor tissue of appropriate amounts to allow for adequate reconstruction) and the recipient anesthesiasurgical team (so that they may have several contingency plans for the safe establishment of cardiopulmonary bypass and full cardiac support during cardiectomy). Several recent publications have addressed quite extensively various surgical strategies aimed at heart transplantation for complex congenital heart disease; accordingly, the focus of this article is to address those more “common” anatomical challenges, some of which may be encountered in particular by noncongenital heart surgeons, given the increasing incidence of adult patients with congenital disease who have end-stage heart failure. For congenital cardiac transplant candidates, a standard, systematic approach toward surgical planning is recommended. Key considerations include issues of atrial situs, anomalies of systemic venous return, anomalies of the great arteries, and particular problems related to prior catheterbased palliations. Where possible, maximizing the “preparation” (reconstruction) of the recipient anatomy that can be accomplished before implant of the donor heart helps to reduce the overall warm ischemic time (and if well timed, the overall ischemic time). For those recipients who are the beneficiaries of several prior palliative or corrective repairs, often the most expeditious approach—should the reoperative surgical field prove excessively hostile—is (1) performing the cardiectomy under deep hypothermic circulatory arrest simply to obtain a clearer sense of the underlying anatomy, (2) reconstruction to allow for bicaval (or tricaval) cannulation, and (3) reinstitution of bypass with rewarming

Right-sided superior vena cava draining into the left atrium: a rare anomaly of systemic venous return

The most commonly encountered systemic thoracic venous anomaly is a persistent left superior vena cava that drains into the right atrium via the coronary sinus. A much rarer systemic venous anomaly is that of isolated anomalous drainage of a normally positioned right superior vena cava (RSVC) into the left atrium (LA). This has been reported in approximately 20 patients with the diagnosis usually being made by cardiac catheterization. We report the case of a toddler with asymptomatic hypoxemia resulting from anomalous drainage of a normal RSVC into his LA. This was diagnosed non-invasively by contrast-enhanced chest CT.

Anomalies of Systemic Venous Return: Right Superior Vena Cava Draining to the Left Atrium

Anomalies of Systemic Venous Return: Right Superior Vena Cava Draining to the Left Atrium

Anomalous systemic venous return: recognition by two-dimensional echocardiography.

The two-dimensional echocardiographic features of anomalies of systemic and coronary venous return are described as they were seen in 23 patients with this diagnosis proven at angiography. A left-sided superior vena cava draining to the right atrium via an enlarged coronary sinus was correctly identified in all 17 patients with this condition by observing a characteristically moving ovoid structure in the region of the posterior atrioventricular groove. This structure opacified before the right side of the heart following bolus injections of contrast material from a left arm vein. Injection from the right arm in 12 patients or femoral vein in four patients of this group produced contrast echoes within the right heart only. A left-sided superior vena cava draining directly to a left atrium in three patients, or left side of a common atrium in two patients with atrial situs inversus, was identified by the immediate opacification of the left-sided chamber following injection from the left arm. Two patients with hemiazygos inferior vena caval return to the left superior vena cava or left atrium were studied using injections of contrast from the femoral vein which successfully identified the venous abnormality. Two-dimensional echocardiography, when combined with peripheral venous injections of contrast will reliably demonstrate anomalies of systemic venous return, and thus provides important information before more invasive procedures.