Left Superior Caval Vein Research Articles

The Atrioventricular Conduction Axis in Man and Mouse

Those using the mouse for the purposes of electrophysiological research presume that the morphology of the conduction axis is comparable with the human arrangement. As yet, however, to the best of our knowledge, no direct comparison has been made between the species. By comparing our extensive histological findings in the human heart with comparable serially-sectioned datasets prepared from adult murine hearts, we aimed to provide this information. When comparing the gross anatomy, we used three-dimensional datasets of neonatal mice hearts prepared using episcopic microscopy. The overall cardiac architecture is comparable, although the mouse has a persistent left superior caval vein draining via the coronary sinus. An inferior pyramidal space and an infero-septal recess are both present in the murine heart, although they are not as well developed as in the human heart. The overall arrangement of the conduction axis is similarly comparable, albeit with subtle differences reflecting the incomplete wedging of the subaortic outflow tract in the murine heart. Most significantly, the findings in both species reveal the presence of extensive superior septal pathways, which perhaps explain the finding of base-to-apex activation of the ventricular mass known to occur in the murine heart.

Prenatal Coffin-Siris Syndrome: Expanding the Phenotypic and Genotypic Spectrum of the Disease.

Coffin-Siris syndrome is an autosomal dominant disorder with neurological, cardiovascular, and gastrointestinal symptoms. Patients with Coffin-Siris syndrome typically have variable degree of developmental delay or intellectual disability, muscular hypotonia, dysmorphic facial features, sparse scalp hair, but otherwise hirsutism and fifth digit nail or distal phalanx hypoplasia or aplasia. Coffin-Siris syndrome is caused by pathogenic variants in 12 different genes including SMARCB1 and ARID1A. Pathogenic SMARCB1 gene variants cause Coffin-Siris syndrome 3 whereas pathogenic ARID1A gene variants cause Coffin-Siris syndrome 2. Here, we present two prenatal Coffin-Siris syndrome cases with autosomal dominant pathogenic variants: SMARCB1 gene c.1066_1067del, p.(Leu356AspfsTer4) variant, and a novel ARID1A gene c.1920+3_1920+6del variant. The prenatal phenotype in Coffin-Siris syndrome has been rarely described. This article widens the phenotypic spectrum of prenatal Coffin-Siris syndrome with severely hypoplastic right ventricle with VSD and truncus arteriosus type III, persisting left superior and inferior caval vein, bilateral olfactory nerve aplasia, and hypoplastic thymus. A detailed clinical description of the patients with ultrasound, MRI, and post mortem pictures of the affected fetuses showing the wide phenotypic spectrum of the disease is presented.

Coronary anomalies associated with ventricular septal defect.

Coronary anomalies encompass different conditions in terms of anomalous origin, coursing, and branching, which are occasionally associated with congenital heart disease. This study aimed to explore coronary anomalies associated with ventricular septal defect that was a stereotypical congenital heart disease. We retrospectively reviewed angiographic findings in patients with ventricular septal defect who required corrective surgery, and identified coronary abnormalities, including anomalous origin, coursing, and branching of coronary arteries based on angiography. We studied the prevalence and types of coronary anomalies among them. A total of 998 patients with ventricular septal defect was studied. Age and weight were 2.0 (2.1-15.2) months and 5.7 (4.1-8.7) kg, respectively. There were 115 patients (12%) with syndrome or genetic disorder, and 34 patients (3%) with extracardiac major organ anomalies. The overall prevalence of coronary anomalies was 6.2%. All coronary anomalies were anomalous origin and coursing, among 3 patients accompanied anomalous intrinsic coronary anatomy. Originating within aortic root above the Valsalva sinus was the most common coronary anomaly. Coronary anomalies were independently correlated with bicuspid aortic valve (odds ratio [OR]: 8.02, 95% confidence interval [CI]: 2.34-23.4) and persistent left superior caval vein (OR: 5.02, 95% CI: 1.93-11.7). We showed the possibility that minor cardiac variants, such as bicuspid aortic valve and persistent left superior caval vein, contribute to higher prevalence of coronary anomalies in patients with ventricular septal defect.

Imaging characteristics and associations of left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers on multidetector CT.

To study the imaging characteristics and the associations of left atrioventricular valve atresia with patent aortic root and lateralized atrial chambers on multidetector computed tomography (CT) angiography. We retrospectively evaluated all the CT angiography studies performed for the evaluation of various congenital heart diseases at our center from January 2014 to December 2021. Cases with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers were identified. The types of cardiovascular involvement as identifiable on CT angiography were studied and compiled. A total of 38 patients with left atrioventricular valve atresia and patent aortic root were included (median age: 5 months, males: 31). A total of 89.5% had usual atrial arrangement and 10.5% had mirror imagery of atria. Normal drainage of right and left superior caval veins and inferior caval vein into systemic venous atrium was seen in 77.8%, 61.5%, and 86.6%, respectively. Anomalous pulmonary venous drainage was seen in 3 (7.9%) patients. Atrial and ventricular septal defects were the most common associations. Imperforate left atrioventricular membrane was seen in 9 (23.7%) patients and absent left atrioventricular connection in 27 (71.1%). Discordant right atrioventricular connection was seen in 5 (13.1%) patients. While a double outlet right ventricle (76.3%) was the most common ventriculoarterial connection, discordant ventriculo-arterial connection (transposition) in 4 (10.5%). Most patients had good sized pulmonary arteries. Patent arterial duct and right aortic arch were seen in 29% and 21%, respectively. Aberrant right subclavian artery was the most common arch vessel anomaly. Coronary anomalies were seen in 7 patients (19%); single coronary artery being the most common. Majority of the patients with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers have usual atrial arrangement, normal right atrioventricular connections and a double outlet configuration of the right ventricle. Absent left atrioventricular connection is a more common than imperforate left atrioventricular valve membrane. Atrial and ventricular septal defects are the most common associations. Comprehensive CT based evaluation of vascular and nonvascular thoracic structures is imperative before surgery or interventions in patients with mitral atresia.

Dextroversion and D-Transposition of the Great Arteries, Left-Handed Ventricular Topology Related to Multiple Ventricular Septal Defects, and Left Juxtaposition of Atrial Appendage in a 1-Year-Old Child

We present a 1-year-old female infant case in natural history, affected by D-transposition of the great arteries with multiple ventricular septal defects in dextroversion, left juxtaposition of atrial appendages, and persistent left superior caval vein draining into coronary sinus. This is an uncommon case, and its particularity is due to clinical and anatomical findings diagnosed late in a setting of a complex cardiac malformation. After the diagnosis, the patient underwent palliative arterial switch operation without complications.

BPC 157 Therapy and the Permanent Occlusion of the Superior Sagittal Sinus in Rat: Vascular Recruitment.

We show the complex syndrome of the occluded superior sagittal sinus, brain swelling and lesions and multiple peripheral organs lesions in rat. Recovery goes centrally and peripherally, with the stable gastric pentadecapeptide BPC 157, which alleviated peripheral vascular occlusion disturbances, rapidly activating alternative bypassing pathways. Assessments were gross recording, venography, ECG, pressure, microscopy, biochemistry. The increased pressure in the superior sagittal sinus, portal and caval hypertension, aortal hypotension, arterial and venous thrombosis, severe brain swelling and lesions (cortex (cerebral, cerebellar), hypothalamus/thalamus, hippocampus), particular veins (azygos, superior mesenteric, inferior caval) dysfunction, heart dysfunction, lung congestion as acute respiratory distress syndrome, kidney disturbances, liver failure, and hemorrhagic lesions in gastrointestinal tract were all assessed. Rats received BPC 157 medication (10 µg/kg, 10 ng/kg) intraperitoneally, intragastrically, or topically to the swollen brain at 1 min ligation-time, or at 15 min, 24 h and 48 h ligation-time. BPC 157 therapy rapidly attenuates the brain swelling, rapidly eliminates the increased pressure in the ligated superior sagittal sinus and the severe portal and caval hypertension and aortal hypotension, and rapidly recruits collateral vessels, centrally ((para)sagittal venous collateral circulation) and peripherally (left superior caval vein azygos vein-inferior caval vein). In conclusion, as shown by all assessments, BPC 157 acts against the permanent occlusion of the superior sagittal sinus and syndrome (i.e., brain, heart, lung, liver, kidney, gastrointestinal lesions, thrombosis), given at 1 min, 15 min, 24 h or 48 h ligation-time. BPC 157 therapy rapidly overwhelms the permanent occlusion of the superior sagittal sinus in rat.

Persistent left superior caval vein draining into right atrium, but not through the coronary sinus

Persistence of the left superior caval vein is the most commonly reported thoracic venous anomaly. The vein usually drains into the right atrium through the coronary sinus, reflecting its developmental origin. We describe an unusual variant, in which the vein drained directly into the right atrium.

Pulmonary vein isolation in a case of common drainage of a persistent left superior caval vein and the left-sided pulmonary veins into the left atrium

Preprocedural imaging before catheter ablation of atrial fibrillation is often limited. We present a case of a 67-year-old female with unsuspected anatomy relevant to procedure planning. Routine computed tomography revealed an undetected persistent left-sided superior caval vein (LpSVC). All left-sided pulmonary veins drained with the LpSVC by way of a common vessel at the roof of the left atrium (LA). This common vein exhibited low-amplitude signals connected to the LA beyond 25 mm. Both right-sided caval veins and the coronary sinus drained to the right atrium. We struggled to keep the ablation lesions distant from the common vein to avoid an increase of the left-to-right shunt by unintended narrowing of this vessel. The encircling ablation lines around the right-sided pulmonary veins and the common vein were close together so an additional roof line was done. In summary, imaging definitively helped to prepare the ablation procedure and to avoid complications.

Abernethy malformation draining above the diaphragm to the coronary sinus and its embryological explanations.

Abernethy malformation connecting to a supradiaphragmatic vein may be missed due to their rarity or lack of awareness. Embryological reasons explain the involvement of the left superior caval vein in these connections.

Incidence of systemic venous anomalies in tetralogy of Fallot on multidetector CT angiography: A retrospective single‐center study of 973 patients

The present study sought to estimate the incidence and evaluate the patterns and associations of systemic venous anomalies in patients with tetralogy of Fallot on multidetector computed tomography (CT) angiography. We retrospectively reviewed CT angiographies of 973 pediatric age group patients diagnosed with tetralogy of Fallot, performed at our institution between 1st January 2015 and 31st December 2019, for systemic venous drainage patterns. We also proposed a structured classification and reporting system on CT angiography which would simplify the characterization, classification, and communication of these anomalies. Variations in systemic venous drainage patterns were observed in 146 (15.01%) patients. The most commonly observed anomaly was persistence of left superior caval vein, which was seen in 112 (11.51%) patients, with the most prevalent pattern being the presence of bilateral superior caval veins with no intercommunicating vein (n = 85). The second most common anomaly observed was presence of anomalous brachiocephalic vein (39/973 [4.01%]). Patients with an anomalous course of brachiocephalic vein had significantly higher prevalence of a right aortic arch than those without (23/39 [58.97%] vs 188/934 [20.13%]; P < .0001). Similarly, a higher prevalence of pulmonary atresia was noted in patients with an anomalous course of brachiocephalic vein compared to those without (8/39 [20.51%] vs 80/934 [8.57%]; P = .0109). There is a high incidence of anomalies of superior caval veins and brachiocephalic vein in the presence of tetralogy of Fallot. With advancements in corrective cardiac surgery and use of intraoperative cardiopulmonary bypass, the presence of systemic venous anomalies assumes greater significance making preoperative identification of these anomalies imperative.

Early and late outcomes of surgical repair of double-chambered right ventricle: a single-centre experience.

Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery. We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated. Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1). The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.

Is analysis of physiological late gestation ventricular-arterial disproportion futile?

Ventricular-arterial disproportion is a frequent observation in late gestation and mostly considered physiological. It is also an indirect prenatal sign for postnatal coarctation. To investigate the remnant risk for coarctation and its possible risk factors in a fetal population with physiological late gestation asymmetry. This was a monocentric, retrospective study conducted from 2014 till 2017 in a Fetal Cardiology Unit. All foetuses referred for third trimester asymmetry with pulmonary artery/aorta ratio > 1.2 were included. Annular sizes, arterial Z-scores, valve ratios, isthmus size if available and minor associated cardiac defects were analyzed. One hundred and one foetuses were referred at a mean gestational age of 33.5 SA. After foetal echocardiography, 2% (3/151) were considered at high risk for coarctation because of a hypoplastic arch and/or a small left outflow tract or isthmus. They were scheduled to deliver in a tertiary referral center. Two of these three newborns developed coarctation. In 98% of foetuses, asymmetry was considered physiological with a normally sized aortic arch without the “shelf sign”. All infants had an early ambulatory echocardiography that revealed coarctation in 7/148 cases (4.7%). There were no significant differences in mean arterial annular sizes, aortic and pulmonary Z-scores, pulmonary artery/aorta ratio, atrioventricular annular sizes, tricuspid/mitral valve ratio, presence of ventricular septal defect or left persisting superior caval vein between the two groups. Remnant risk for coarctation in a foetal population with physiological late gestation asymmetry is < 5%. Prenatal counselling can be reassuring since the majority of infants will have a normal heart. However early cardiac evaluation of these newborns remains recommended to exclude the rare event of postnatal coarctation in absence of prenatal predictive signs.

Is analysis of physiological late gestation ventricular-arterial disproportion futile?

Ventricular-arterial disproportion is a frequent observation in the third trimester and mostly considered physiological. It is also an indirect prenatal sign for postnatal coarctation. We sought to investigate the remnant risk for coarctation and its possible risk factors in a fetal population with physiological late gestation asymmetry. This was a monocentric, retrospective study conducted from 2014 till 2017 in a Fetal Cardiology Unit. All fetuses referred for third trimester asymmetry with pulmonary artery/aorta ratio > 1.2 were included. Annular sizes, arterial Z-scores, valve ratios, isthmus size if available and minor associated cardiac defects were analyzed. 151 fetuses were referred at a mean gestational age of 33.5 SA. After fetal echocardiography, 2% (3/151) were considered at high risk for coarctation because of a hypoplastic arch and/or a small left outflow tract or isthmus. They were scheduled to deliver in a tertiary referral center. Two of these 3 newborns developed coarctation. In 98% of fetuses asymmetry was considered physiological with a normally sized aortic arch without the “shelf sign”. All infants had an early ambulatory echocardiography that revealed coarctation in 7/148 cases (4.7%). There were no significant differences in mean arterial annular sizes, aortic and pulmonary Z-scores, pulmonary artery/aorta ratio, atrioventricular annular sizes, tricuspid/mitral valve ratio, presence of ventricular septal defect or left persisting superior caval vein between the two groups. Remnant risk for coarctation in a fetal population with physiological late gestation asymmetry is < 5%. Prenatal counselling can be reassuring since the majority of infants will have a normal heart. However early cardiac evaluation of these newborns remains recommended to exclude the rare event of postnatal coarctation in absence of prenatal predictive signs.

Second-Generation Cryoballoon AtrialFibrillation Ablation in Patients With Persistent Left Superior Caval Vein.

This study sought to assess the acute success rate, periprocedural complications, and long-term outcomes in patients with atrial fibrillation (AF) and persistent left superior vena cava (PLSVC) treated with second-generation 28-mm cryoballoon (CB2). PLSVC is a cardiac anomaly associated with AF. Between July 2012 and October 2018, 8 patients from 4 German high-volume centers referred for pulmonary vein isolation (PVI) demonstrated a PLSVC. PVI and ablation within the PLSVC was performed using the CB2. A total of 2,876 patients were treated with CB2-based PVI. Eight patients (0.28%; mean 65 ± 7 years of age, 2 paroxysmal, 6 with persistent AF, mean left atrial size of 44 ± 4 mm) presenting with PLSVC were evaluated. All patients underwent PVI, and 3 of 8 patients with documented triggered activity from PLSVC underwent PLSVC ablation with CB2. Electrical isolation of PLSVC was achieved in 2 of 3 patients. Mean procedure and fluoroscopy times were 120 ± 22 min and 32 ± 18 min, respectively. In 2 of 8 patients, major complications (right phrenic nerve palsy) occurred during right PV ablation. After 3 months, 1 of 2 patients recovered from right phrenic nerve palsy. Two patients underwent a redo procedure after AF recurrence, demonstrating PV reconnection but no triggers from PLSVC. Freedom from AF after 332days of follow-up was 63%. CB2 ablation for AF in patients with PLSVC is feasible, with an increased risk for right phrenic nerve palsy. Electrical isolation of PLSVC can be achieved with the CB2 in most patients.

Unilateral unidirectional superior cavopulmonary anastomosis in a patient with bilateral superior caval veins and atretic left pulmonary artery.

A unidirectional superior cavopulmonary anastomosis was performed on the right side in a patient with a functionally univentricular heart, atresia of main and left pulmonary artery, bilateral superior caval veins, and a patent arterial duct in the right pulmonary artery. Anastomosis of the left superior caval vein to the right superior caval vein created a neo-innominate vein without using prosthetic material.

Atrioventricular septal defect with anomalous systemic venous and pulmonary venous connection

An anomalous pulmonary venous and systemic connection associated with a partial atrioventricular septal defect (AVSD) is rare. A 23-year-old patient, who presented with dyspnea and cyanosis on exertion, was found to have an anomalous pulmonary and systemic venous drainage with separate drainage of the left superior caval vein and hepatic veins into the left atrium and a partial AVSD. A patch diversion was successfully performed using two patches of glutaraldehyde-treated pericardium to create a nonobstructive pulmonary and systemic venous drainage.

Imaging topography and morphometry of persistent left superior caval vein and its variations, detected on cardiac implantable electronic device implantation.

Persistent left superior caval vein (PLSCV) is a rare, anatomically diverse developmental anomaly of systemic veins. Clinically asymptomatic PLSCVs are detected incidentally during medical procedures that utilise systemic veins, such as cardiac implantable electronic device (CIED) placement, and whose successful completion depends on favourable morphometric parameters of these vessels. The aim of this paper was to present topography and morphometry of PLSCV variations encountered during CIED implantation procedures. We analysed a group of 5,010 patients for detection of PLSCV during de-novo CIED implantation procedures with transvenous lead placement in the years 2003-2015. PLSCVs were detected intraprocedurally based on venographic images illustrating the venous anomaly and its morphometric parameters, and were subsequently confirmed via postoperative diagnostics. PLSCVs were detected in 10 patients (mean age 66.0 ± 14.0 years; 5 females and 5 males), who constituted 0.2% of the analysed group. There were 6 cases of double superior vena cava (DSVC), 3 of which had a brachiocephalic vein (BCV) connection and did not have BCV bridging. Four patients with a PLSCV had right superior vena cava agenesis; this very rare variation is known as 'single PLSCV'. All of the detected PLSCV variations drained into the right atrium via the coronary sinus. Our data from a period of 13 years illustrate how rare the PLSCV-type venous anomaly is. The three distinct anatomical PLSCV types showed inter-individual morphometric variations. Due to asymptomatic nature of this anomaly, all cases were detected incidentally, during CIED implantation procedures.

0458: Outcomes of pediatric hypertrophic cardiomyopathy associated with Rasopathy

The outcomes of pediatric hypertrophic cardiomyopathy (HCM) associated to RASopathies are scarcely known. To describe outcomes of pediatric HCM associated with RASopathy. 80 cases of children with a clinical phenotype of RASopathy and HCM were included. Age at diagnosis of HCM was 11.7 months (range 0 to 168). The HCM was diagnosed prenatally in 9 cases, and before one year of age in 58 cases. 67 had Noonan syndrome, 9 had Cardiofacio-cutaneous syndrome, 3 had Costello syndrome, and 1 had Noonan syndrome with multiples lentigines. The HCM was obstructive in 37 cases. Associated cardiac defects were right ventricular outflow tract obstruction in 40 children, polyvalvular dysplasia in 18, atrial septal defect (ASD) in 13, ventricular septal defect in 3, congenital mitral regurgitation in 2, persistent arterial duct in 2, left superior caval vein in 2, aortic arch anomaly in 2, coronary anomaly in 1, and systemic arteriovenous fistula in 1. Nineteen children underwent a Konno operation for obstructive HCM. Additional surgical procedures were 9 right ventricular outflow tract surgical reliefs, 9 percutaneous dilatations of pulmonary valve stenosis, 4 percutaneous closures of ASD, 1 tricuspid valve repair, 1 Brom procedure with aortic valve repair, 1 aortic valve replacement, and 1 coronary artery bypass. Overall mortality was 21%. Fourteen (82%) deaths occurred before 1 year of life. Causes of death were cardiac in 10 cases including two sudden deaths, related to respiratory diseases causes in 5, and in utero fœtal death in 1. Factors predicting mortality were diagnosis before 2 months of life (HR=4,33, p<0,001), fœtal diagnosis of HCM (HR=3,64, p<0,001), and surgery before one year of life (HR=3,43, p=0,001). Mortality of children with HCM due to RASopathy is high and occurs early in life. Main risk factors are early diagnosis of HCM including prenatal diagnosis, and need for surgery before one year of life.

Slow pathway cryoablation in a child with absent right superior caval vein and persistent left superior caval vein draining into the coronary sinus

A 12year old boy was referred to our institution for recurrent episodes of paroxysmal supraventricular tachycardia. The baseline ECG showed non-sinus atrial rhythm with heart rate of 75beats/min (Fig. 1-A). The echocardiogram revealed a normal cardiac structure with persistence of a left superior caval vein (PLSCV) draining into a dilated coronary sinus (CS). After a written informed consent, a transcatheter ablation procedure was planned because of agonist sport practice and parent choice. A fast anatomic map of the inferior caval vein, right atrium, CS and PLSCV was performed using an electroanatomic 3D mapping system (CARTO 3).

Recanalisation of the left superior caval vein after Fontan procedure: not so rare complication: possibilities of percutaneous closure using various devices

Evaluation of possibilities of percutaneous closure of recanalised left superior caval vein after total cavopulmonary connection. We analysed 19 patients after total cavopulmonary connection catheterised because of a sudden increase of desaturation. In four of them, the recanalisation of the left superior caval vein was identified. For this reason, the balloon occlusion tests of the veins were made temporarily. In all cases, the haemodynamic status of patients did not change, and arterial oxygen saturation increased significantly. Thus, using different types of implants, these veins were closed effectively in all patients. During the short-term follow-up, the effectiveness of treatments and constantly maintaining a high level of saturation were confirmed. Meticulous investigation of unclear causes of desaturation in cyanotic patients after Fontan completion is necessary. Almost all causes of desaturation, including recanalised additional left superior caval vein, can be effectively treated percutaneously.