Systemic Venous Return Research Articles

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.

Persistent Right-Sided Umbilical Vein and Azygos Continuity associated with Ophthalmological and Pulmonary Malformations. Prenatal Diagnosis of an Exceptional Case

We report on the case of a fetus in whom a prenatal ultrasound performed at 22 weeks of gestation allowed the diagnosis of a double anomaly of the systemic venous return, with a persistent right umbilical vein and the agenesis of the inferior vena cava with azygos continuity. The fetus showed also a large umbilical hernia and a right sided microphtalmia. Fetal growth was normal. Genetic anomalies were excluded by karyotype and CGH array. Cesarean section was performed at 39 weeks of gestational age for breech presentation. The neonate adapted well. Post-natal examination confirmed pre-natal diagnosis. Thoraco-abdominal CT-scan performed at 8 days of age showed additional right upper lobe bronchial atresia with relative hypoplasia of the right pulmonary artery and right hepatic lobe hypotrophy. It also showed a short segmental infra diaphragmatic aplasia of the vena cava with dilation of the azygos vein running along the homolateral diaphragmatic coupola that drained into superior vena cava and malposition of the mesenterial vessels without any intestinal malrotation. Anomalies of the fetal cardinals and umbilical veins are the result of early abnormal venous system development in the embryo, the etiology of which is unknown. Usually, abnormal systemic venous return only affects one vessel. The presence of a double anomaly as described in this case is exceptional. A persistent right umbilical vein is reported in 0.2 to 0.4% of antenatal screening. The variant with intrahepatic transition is the most frequent and isolated form. The exceptional, form with hepatic bypass, is commonly part of a polymalformative syndrome. Agenesis of the inferior vena cava (IVC) with azygos continuity is a rare anomaly (0,2-3% of antenatal screening). Its significance relates to its frequent association with complex congenital heart diseases. The prognosis of anomalies of cardinals and umbilical veins depends on the presence of associated cardiac and extra cardiac malformations that might impact prenatal counseling and parental decision to continue or interrupt pregnancy. Careful repeated pre and post-natal evaluation is necessary to exclude polymalformative syndrome despite of normal molecular genetic examination.

Pulmonary Root Translocation for Anatomical Repair of Congenitally Corrected Transposition of the Great Arteries

Pulmonary root translocation (PRT) was used to correct ventriculoarterial discordance as part of anatomical repair for congenitally corrected transposition of the great arteries, ventricular septal defect (VSD) and pulmonary stenosis (PS). The Senning procedure was used for completion of the anatomical repair. From March 2004 to June 2014, ten consecutive patients were operated. There was no hospital and one patient died 5 years after the surgical repair. In the postoperative period, control echocardiogram showed no obstruction in the systemic and pulmonary venous return. Also, neither LVOT nor significant RVOT obstruction was found in any patient. Our initial results suggest that pulmonary root translocation is a good option for correcting ventriculoarterial discordance in the anatomical repair of CCTGA with VSD and LVOTO.

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

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.

Imaging for Preintervention Planning

Although all preoperative imaging can be considered surgical planning (SP), it is defined in this article as the act of using preoperative data to simulate the surgical procedure or the result of the procedure. It is the combination, all or in part, of 3-dimensional (3D) medical imaging, applied computer vision, computer-aided design, and computational fluid dynamic (CFD) modeling to mimic and provide visual guidance for surgical procedures. This simulation is generally performed with multiple anatomic and physiological states to determine the robustness of the procedure. There are multiple advantages to this approach such as assessing standard interventions and creation of new surgical strategies without risking the patient’s health; this can potentially have both clinical and economic benefits. Since its first mention ≈30 years ago, SP is now a routine part of interventions in fields such as neurosurgery and orthopedics.1 Translating this paradigm to cardiovascular interventions provides not only enhanced 3D visualization but also the potential to interface with physics-driven computational solvers (eg, CFD) to predict the hemodynamic outcomes. Considering the complexity of fluid-solid interactions and the highly time-varying component of the cardiovascular system, these efforts are largely lagging behind those in the neurological and orthopedic communities, but recent advances appear promising.2 The Fontan operation for single ventricle (SV) patients, in which a conduit (the total cavopulmonary connection [TCPC]) is placed to channel systemic venous return passively into the pulmonary arteries (PAs), is the paradigm for this approach. This category of lesions is the leading cause of morbidity and mortality in congenital heart disease, and although it is generally associated with acceptable short-term outcomes, Fontan failure remains problematic. Progressive ventricular dysfunction, protein-losing enteropathy, poor exercise tolerance, pulmonary arteriovenous malformations (PAVMs), and liver dysfunction are some of the most commonly cited complications. These morbidities are multifactorial, and the underlying causes …

New Transplant Technique With Hemiazygos Continuation and Interrupted Inferior Vena Cava

In the presence of left superior vena cava, cardiac transplantation is performed at the risk of left superior vena cava flow obstruction. In patients with hemiazygos continuation with interrupted inferior vena cava, the patency of left superior vena cava is vital. We introduced a new surgical technique to use a Fontan connection including the native central pulmonary artery as the systemic venous return. The pulmonary artery anastomosis was performed distal to the superior vena cava anastomosis sites. We used this treatment approach successfully to perform cardiac transplantation in two patients who developed severe heart failure after Fontan completion.

Acquired Fontan Physiology Secondary to Acute Mechanical Tricuspid Valve Failure in a Patient With Massive Right Heart Enlargement and Ebstein’s Anomaly

A 25-year-old man with Ebstein’s anomaly and a secundum atrial septal defect had a mechanical tricuspid valve replacement (St. Jude 31-mm valve) and atrial septal defect closure when he was 15 years old. He was under long-term specialist follow-up and had recently been admitted with symptoms and signs of right heart failure secondary to new onset atrial flutter. He was direct-current cardioverted and commenced on ramipril and metoprolol. He then went to have an electrophysiological study and successful atrial flutter ablation. At review 6 months later he reported good exercise tolerance and was now able to play 18 holes of golf, whereas previously he could only manage 9 holes. However, he commented that since the electrophysiological procedure he had been unable to hear his prosthetic valve clicks. On clinical examination he was not cyanosed, the jugular venous pressure was elevated at 4 cm without prominent V waves, there was a soft first heart sound, no audible mechanical valve clicks, and a pansystolic murmur. There were no signs of heart failure or peripheral edema. An electrocardiogram demonstrated sinus rhythm, right axis deviation, first-degree heart block, and right bundle-branch block with QRS duration of 200 ms. Echocardiography and fluoroscopy (Figure 1) showed that the leaflets of the …

Mechanical principle of the respiration-driven variation of cardiac function——Echocardiographic study of the mechanical models

Objective To reveal the exact mechanical principle of the respiration-driven variation of cardiac function by observing the effect of respiratory intrathoracic pressure change (RIPC) on the mechanical models using echocardiography.Methods Model 1 was designed to observe the influence of RIPC on systemic and pulmonary venous return systems (SVR and PVR) respectively.Model 2,as an equivalent mechanical model of septal swing,was used to study the influence of RIPC on the motion of the interventricular septum (IVS).Results Model 1 demonstrated that the simulated RIPC had totally different influences on the simulated SVR and PVR.It increased the volume of the simulated right ventricle when the internal pressure was kept constant (8.16 cm H2O),while it had the opposite effect on PVR.Model 2demonstrated that there was a corresponding relationship between RIPC and the position of the simulated IVS which might be called pressure-position relationship.Conclusions The different anatomical arrangement of the two venous return systems leads to a different effect of RIPC on right and left ventricles,and thus generates a pressure gradient across IVS that tends to shift IVS left-and right-wards with respiration.The swing of the IVS changes the short axis diameters of the ventricles,thus their fillings and then their functions reciprocally. Key words: Echocardiography; Respiration; Heart function tests; Pressure amplifier in the chest

Recurrent Cerebral Abscess Secondary to a Persistent Left Superior Vena Cava

Cerebral abscess is a serious neurological condition that is often of unclear etiology. Management is usually medical therapy with or without direct drainage, and when patients have recurrent episodes a structural abnormality should be considered. Persistent left superior vena cava is an uncommon condition in the absence of other forms of congenital heart disease. This venous connection most often enters the right-sided atrium through the coronary sinus but occasionally can connect directly to the left atrium near the wall between the orifice of the left pulmonary veins and left atrial appendage. This later congenital connection results in systemic venous return entering the left atrium directly. Thus allowing unfiltered, lower saturation blood entering the systemic system. This then places the patient at risk for systemic hypoxemia, paradoxical embolic events, and cerebral abscess. In our case report with recurrent cerebral abscess and a persistent left superior vena cava, we demonstrate when to consider this diagnosis, how to make the diagnosis, and a nonsurgical approach to repair the veno-atrial shunt.

Mechanism Study of Pulsus Paradoxus Using Mechanical Models

Pulsus paradoxus is an exaggeration of the normal inspiratory decrease in systolic blood pressure. Despite a century of attempts to explain this sign consensus is still lacking. To solve the controversy and reveal the exact mechanism, we reexamined the characteristic anatomic arrangement of the circulation system in the chest and designed these mechanical models based on related hydromechanic principles. Model 1 was designed to observe the primary influence of respiratory intrathoracic pressure change (RIPC) on systemic and pulmonary venous return systems (SVR and PVR) respectively. Model 2, as an equivalent mechanical model of septal swing, was to study the secondary influence of RIPC on the motion of the interventriclar septum (IVS), which might be the direct cause for pulsus paradoxus. Model 1 demonstrated that the simulated RIPC had different influence on the simulated SVR and PVR. It increased the volume of the simulated right ventricle (SRV) when the internal pressure was kept constant (8.16 cmH2O), while it had the opposite effect on PVR. Model 2 revealed the three major factors determining the respiratory displacement of IVS in normal and different pathophysiological conditions: the magnitude of RIPC, the pressure difference between the two ventricles and the intrapericardial pressure. Our models demonstrate that the different anatomical arrangement of the two venous return systems leads to a different effect of RIPC on right and left ventricles, and thus a pressure gradient across IVS that tends to shift IVS left- and rightwards. When the leftward displacement of IVS reaches a considerable amplitude in some pathologic condition such as cardiac tamponade, the pulsus paradoxus occurs.

Slit–Roundabout Signaling Regulates the Development of the Cardiac Systemic Venous Return and Pericardium

The Slit-Roundabout (Robo) signaling pathway has pleiotropic functions during Drosophila heart development. However, its role in mammalian heart development is largely unknown. To analyze the role of Slit-Robo signaling in the formation of the pericardium and the systemic venous return in the murine heart. 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. 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.

Persistent left superior vena cava, absence of the innominate vein, and upper sinus venosus defect

Superior vena cava anomalies are rare malformations that are typically seen with other congenital cardiac defects. Although a persistent left superior vena cava is the most common anomaly of the systemic venous return in the thorax, its combination with an upper sinus venosus defect and absence of the innominate vein is extremely rare. Here, we report a patient diagnosed with these anomalies based on a bubble study and confirmed with magnetic resonance imaging.

Effects of norepinephrine on mean systemic pressure and venous return in human septic shock*

Norepinephrine exerts venoconstriction that could increase both the mean systemic pressure and the resistance to venous return, but this has not yet been investigated in human septic shock. We examined the relative importance of both effects and the resulting effect on venous return when decreasing the dose of norepinephrine. Intensive care unit. Sixteen septic shock patients. For estimating the venous return curve, we constructed the regression line between the pairs of cardiac index (pulse contour analysis) and central venous pressure values. These values were measured during 15-sec end-inspiratory and end-expiratory ventilatory occlusions performed at two levels of positive end-expiratory pressure, in view of widening the range of cardiac index:central venous pressure measurements and increasing the accuracy of the regression line. The x-axis intercept of the regression line was used to estimate the mean systemic pressure and the inverse of the slope of the regression line to quantify resistance to venous return. These measurements were obtained before and after decreasing the dose of norepinephrine. Passive leg raising was performed before and after decreasing the dose of norepinephrine. Decreasing the dose of norepinephrine from 0.30 (0.10-1.40) to 0.19 (0.08-1.15) µg/kg/min decreased the mean systemic pressure from 33 ± 12 mm Hg to 26 ± 10 mm Hg (p = .0003). The slope of the multipoint cardiac index:central venous pressure relationship increased (p = .02). The resistance to venous return decreased, i.e., 1/slope decreased. Simultaneously, cardiac index decreased from 3.47 ± 0.86 L/min/m2 to 3.28 ± 0.76 L/min/m (p = .04), indicating a decrease in venous return. Passive leg raising increased cardiac index to a larger extent after (8% ± 4%) than before (1% ± 4%) decreasing norepinephrine (p = .001), suggesting an increase in unstressed blood volume at the lowest dose of norepinephrine. In septic shock patients, decreasing the dose of norepinephrine decreased the mean systemic pressure and, to a lesser extent, the resistance to venous return. As a result, venous return decreased.

Is the Extracardiac Conduit the Preferred Fontan Approach for Patients With Univentricular Hearts?

> “In theory, theory and practice are the same. In practice, they are not.” > > —Albert Einstein There is, perhaps, no disease entity that better exemplifies the complexities, challenges, and creative innovations characteristic of the evolution in congenital heart disease care than the univentricular heart. The univentricular heart encompasses an array of uncommon and severe inborn cardiac malformations that share a similar physiology, with pulmonary and systemic venous return predominantly directed into a functionally single ventricle, precluding biventricular repair.1 A prevalence of ≈1 per 3000 live births has been estimated.2,3 The occasional patient may survive beyond middle age without palliative surgery when certain conditions align, including well-balanced pulmonary and systemic circulations and preserved function of a morphologically left single ventricle.4 For the vast majority, the prognosis without surgical intervention is grim.5 In developed countries, most patients undergo a staged surgical approach in view of an ultimate, albeit imperfect, Fontan procedure. Typically completed at between 18 months and 4 years of age, Fontan surgery essentially separates the systemic from pulmonary circulation by directing systemic venous return to the pulmonary artery, usually without an interposed subpulmonary ventricle. Response by Kogon on p 2525 Since its original description in 1971,6 multiple modifications and adaptations to the Fontan procedure have been widely applied to the spectrum of univentricular hearts. Although it is beyond the scope of this article to detail all proposed variants, the 3 most common categories of Fontan surgery are schematically depicted in Figure 1. The classic Fontan consisted of a valved conduit between the right atrium and pulmonary artery. It was subsequently modified to a direct anastomosis of the right atrium to a divided pulmonary artery (Figure 1A). The intracardiac lateral tunnel was the next major innovation. It consists of an end-to-side anastomosis of the superior …

The Importance of the Muscle and Ventilatory Blood Pumps During Exercise in Patients Without a Subpulmonary Ventricle (Fontan Operation)

The aim of this study was to determine the relative contribution of the muscle and ventilatory pumps to stroke volume in patients without a subpulmonic ventricle. In patients with Fontan circulation, it is unclear how venous return is augmented to increase stroke volume and cardiac output during exercise. Cardiac output (acetylene rebreathing), heart rate (electrocardiography), oxygen uptake (Douglas bag technique), and ventilation were measured in 9 patients age 15.8 ± 6 years at 6.1 ± 1.8 years after Fontan operation and 8 matched controls. Data were obtained at rest, after 3 min of steady-state exercise (Ex) on a cycle ergometer at 50% of individual working capacity, during unloaded cycling at 0 W (muscle pump alone), during unloaded cycling with isocapnic hyperpnea (muscle and ventilatory pump), during Ex plus an inspiratory load of 12.8 ± 1.5 cm water, and during Ex plus an expiratory load of 12.8 ± 1.6 cm water. In Fontan patients, the largest increases in stroke volume and stroke volume index were during zero-resistance cycling. An additional increase with submaximal exercise occurred in controls only. During Ex plus expiratory load, stroke volume indexes were reduced to baseline, non-exercise levels in Fontan patients, without significant changes in controls. With Fontan circulation increases in cardiac output and stroke volume during Ex were due to the muscle pump, with a small additional contribution by the ventilatory pump. An increase in intrathoracic pressure played a deleterious role in Fontan circulation by decreasing systemic venous return and stroke volume.

A Case of Variant Scimitar Syndrome

Scimitar syndrome is one of the large congenital pulmonary venolobar syndromes and is defined as hypogenetic lung associated with partial anomalous pulmonary venous return. We report the case of a 25-year-old man with complex and exceptional variant scimitar syndrome. A chest CT scan with three-dimensional (3-D) reconstruction led us to identify hypoplastic right lung with homolateral hemidiaphragm agenesis and hypogenetic right pulmonary artery. There was a large and sinuous systemic arterial supply and anomalous venous return directed into the left atrium (venous return being usually directed into the right atrium or inferior vena cava in scimitar syndrome). Hyperoxia test showed no shunt. This variant scimitar syndrome has been previously reported and anomalous venous return called "meandering" pulmonary vein. Diagnosis and management of these patients with complex congenital anomalies are difficult because of their exceptional condition. Chest CT scan with 3-D reconstruction offers an accurate noninvasive diagnosis.

Isolated Total Anomalous Systemic Venous Drainage in an Adult: Case Report

Total anomalous systemic venous drainage is a rare form of congenital heart disease. All the systemic venous flow from the body (i.e., from the superior vena cava, inferior vena cava, and coronary sinus) drains abnormally into the left atrium. This condition requires the presence of a left-to-right shunt (atrial septal defect, patent ductus arteriosus, or ventricular septal defect) to allow the systemic venous return to reach the pulmonary circulation. This disorder may be associated with heterotaxy syndromes. This report describes a patient with unique total anomalous systemic venous drainage that was successfully corrected surgically.

Implantable Cardioverter-Defibrillator Insertion in Congenital Heart Disease Without Transvenous Access to the Heart

Sudden cardiac death is the leading cause of mortality in adults with complex congenital heart disease. Challenges to implanting cardioverter-defibrillators (ICDs) may arise as a result of vascular or intracardiac access issues. For example, conduits may divert systemic venous return to extracardiac structures such as pulmonary arteries or tunnels. Creative approaches to ICD implantation have previously involved subcutaneous arrays, often associated with thoracotomy to implant intrathoracic coils. We describe a patient with complex congenital heart disease and no transvenous access via the superior vena cava in whom ICD implantation involved placement of a coil within the azygos vein.

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.