Absence Of Right Superior Vena Cava Research Articles

Right-Sided Permanent Pacemaker Implantation in Patients with Persistent Left Superior Vena Cava with Absent Right Superior Vena Cava: 3-Case Series, Technique and Discussion

Persistent left superior vena cava (PLSVC) is the most common variant of abnormal venous return to the heart. While usually asymptomatic, it is known to complicate transvenous cardiac procedures, such as implantation of cardiac electronic devices and ablations. PLSVC can present with or without the concomitant absence of right superior vena cava (RSVC). Depending on the operator's preference, implantation of permanent cardiac pacemakers (PPMs) may be performed from the left or right side. As most often the PLSVC is only identified at the time of intervention, it follows that the variant with the absence of RSVC can be diagnosed in practice only when implanting from the right side. For this reason, the true prevalence of this variant is largely unknown because most published cases of cardiac device implantations in patients with PLSVC have been performed from the left side. We present a short 3-case series of PPM implantations in a tertiary center from the right side in patients with PLSVC and absent RSVC. We found that the use of a standard curve for ventricular lead septal placement and a wide C-curve for right atrial lead placement in these patients was a feasible technique with good outcomes.

Prenatal Diagnosis of Absent Right Superior Vena Cava in Referrals for Fetal Echocardiography

AbstractA persistent LSVC with an absent right superior vena cava (RSVC) is unusual, encountered in about 10% of patients with persistent LSVC. We present 14 patients with a prenatal diagnosis of single LSVC and situs solitus, including prenatal findings and information on medium-term follow-up. We identified patients with situs solitus and a persistent LSVC born between March 2004 and March 2020, which had been diagnosed prenatally between December 2003 and November 2019. From this cohort, we identified those with absent RSVC. In the population of women undergoing fetal echocardiography, the prevalence of persistent LSVC in situs solitus was 0.43% (84/19,712). For the 84 identified patients and for the entire population respectively, 14/84 (17%) and 14/19,712 (0.07%) had a single LSVC (absent RSVC). Of 14 patients with a single LSVC, 8 (57%) were male. For the 84 identified patients and for the entire population respectively, 70/84 (83%) and 70/19,712 (0.36%) had bilateral SVCs. For a single LSVC (absent RSVC) and situs solitus, the majority had no associated cardiac, extracardiac, or syndromic abnormalities.

Persistent left superior vena cava with absent right superior vena cava in adults: CT and clinical findings.

Persistent left superior vena cava (PLSVC) with absent right superior vena cava (ARSVC) is rare. We evaluated CT and clinical findings of adults with ARSVC. Our study included 11 adults with ARSVC (mean age, 63 ± 17years). We evaluated CT findings of the azygos vein system and coronary sinus (CS) using thin slice data of > 64 MDCT. Arrhythmia and congenial heart disease were assessed. We compared the CS diameters between the ARSVC group and the control group of 120 adults with normal vena cava (mean age, 60 ± 4years). Adults with ARSVC had no azygos vein arch and the hemiazygos vein continued to PLSVCs via the superior intercostal vein. Eight adults had arrhythmia including conduction disturbance and one arrhythmia was associated with atrial septal defect. All adults with ARSVC had dilated CS larger than 11mm. The mean diameter of the CS in the ARSVC group was 18.4mm, and that of the control group was 6.8mm. CS diameter in the ARSVC group was larger than that in the control group (p < 0.001). Adults with ARSVC had dilated CS and no azygos vein arch. Four adults had conduction disturbance.

Persistent left superior vena cava with absent right superior vena cava detected during emergent coronary artery bypass grafting surgery.

Although persistent left superior vena cava (PLSVC) itself is a common venous anomaly in congenital heart disease, PLSVC with absent right superior vena cava (RSVC) is a rare venous congenital malformation. Due to the lack of symptoms, this malformation is often detected fortuitously when patients undergo central venous catheter placement, pacemaker implantation, or open cardiac surgery. This particular venous malformation is rare, but clinicians in many fields should be well aware of its variations and management techniques to avoid complications. Anesthesiologists should know that patients with PLSVC rarely have absent RSVC. TEE was helpful in the diagnosis of PLSVC with absent RSVC during emergent surgery.

Absent right superior vena cava with persistent left superior vena cava and normal atrial arrangement

Background: This study aims to investigate the role of echocardiography in the detection of venous anomalies of large thoracic veins in patients with large coronary sinus. Methods: Between January 2010 and March 2012, six patients (3 boys, 3 girls; mean age 4.1; range 3 days - 9 years) were diagnosed with an absent right superior vena cava (RSVC) accompanied by persistent left superior vena cava (PLSVC) in our clinic. The diagnosis of the absent RSVC was established by echocardiography and angiography. Results: Diagnosis of the absent RSVC was confirmed by echocardiography in four patients. The diagnosis was based on cardiac catheterization and angiography in two patients. Additional cardiac anomalies included peripheral pulmonary stenosis, patent ductus arteriosus, tetralogy of Fallot, atrial septal defect, ventricular septal defect and interrupted aortic arch type B. One patient had Trisomy 9p syndrome, while another one was the baby of a diabetic mother. Three patients were followed clinically, while other three patients were operated. Conclusion: Absent RSVC should be thoroughly investigated in cases with a large coronary sinus along with increased coronary sinus flow and PLSVC, as evidenced by echocardiography.

Heterotaxy syndrome associated with left ventricular non-compaction

A 20-year-old asymptomatic man was referred to our clinic for the evaluation of a 2/6 grade systolic cardiac murmur. The ECG was normal. On his transthoracic echocardiography (TTE), subaortic membrane causing peak 25 mm Hg gradient on left ventricular outflow tract as the cause of the murmur (Fig. 1A) and an enlarged coronary sinus (CS) (Fig. 1B) were detected. We also suspected left ventricular non-compaction (Fig. 1C, D). We performed agitated saline injection via left antecubital vein for diagnosis of persistent left superior vena cava (PLSVC) and bubbles reached to CS before right atrium, indicating PLSVC. The injection was repeated via right antecubital vein to evaluate the right superior vena cava (RSVC) because of that the absence of RSVC may be associated with PLSVC. After the injection, the coronary sinus was opacified before right atrium, indicating the absence of RSVC. Left ventricular non-compaction was confirmed with cardiac magnetic resonance imaging (Fig. 2A, B). Thoracal computed tomographic (CT) venography was performed, and it confirmed the PLSVC and also revealed the absence of RSVC (Fig. 3A). Surprisingly, it also demonstrated absent inferior vena cava (IVC). Therefore, it forced us to perform abdominal CT angiography. It revealed the venous return of lower limbs and abdomen maintaining with dilated hemiazygous vein, indicating absent IVC. His abdominal CT faced us to another interesting findings, polysplenia (Fig. 3B). Subsequently, all findings in the entire story reached us to diagnose heterotaxy syndrome (HS). Coexistence of HS, subaortic membrane and left ventricular non-compaction has not been reported in the literature.

Persistent left superior vena cava with an absent right superior vena cava in a 72-year-old male with multivessel coronary artery disease

Congenital anomalies of systemic veins are usually asymptomatic and found incidentally during ultrasonography, computed tomography (CT) or magnetic resonance examinations performed for other clinical indications. Persistent left superior vena cava (PLSVC) with absent right superior vena cava (RSVC) is the congenital aberration in the thoracic venous system which occurs in only 0.09%to 0.13% of patients who have congenital heart defects. In this paper, we present the extremely rare case of a 72-year-old male with PLSVC associated with an absence of RSVC, referred for coronary CT angiography. Multidetector CT angiography is a powerful tool for the detection of venous anomalies, which is essential before invasive procedures such as the implantation of pacemakers.

Persistent left superior vena cava and absent right superior vena cava: Not only an anatomic variant

Introduction:A 71 year old asymptomatic woman came for an echocardiogram because of a left bundle branch block. A much dilated coronary sinus (CS) with an entering large vessel was found along with a mild left ventricular systolic dysfunction. Cardiac Magnetic Resonance (CMR) showed a persistent left superior vena cava (PLSVC), and an absent right superior vena cava (ARSVC). PLSVC drained into the dilated CS. No other cardiac abnormalities were found. Any late Gadolinium enhancement was also not seen. PLSVC and ARSVC are associated with sinus node and conduction tissue maldevelopment and atrial arrhythmias, and thus clinical follow up is indicated.Conclusion:CMR is a useful addition to echocardiogram to search for further cardiac abnormalities, and outline the anatomy with precision in doubtful cases.

Prenatal diagnosis of persistent left and absent right superior vena cava.

We report a case of persistent left superior vena cava (LSVC) with absent right superior vena cava (RSVC) diagnosed prenatally. At 27weeks' gestation, routine fetal ultrasonography showed an abnormal four-chamber view. Fetal echocardiography revealed a markedly enlarged coronary sinus in the four-chamber view. An absent RSVC and a persistent LSVC were demonstrated in the three-vessel view. No additional cardiac malformations were seen before birth. Postnatal imaging confirmed the prenatal diagnosis. The combination of persistent LSVC and absent RSVC without any other cardiac malformations is an extremely rare cardiac anomaly and less commonly detected in utero. Identification of a dilated coronary sinus in the four-chamber view, with atypical features in the three-vessel view, may lead to the antenatal diagnosis of this condition.

Clinical consideration of cardiac pacemaker implantation through a persistent left superior vena cava with an absent right superior vena cava

Persistent left superior vena cava (PLSVC) with an absent right superior vena cava (RSVC) is a rare anatomic variation (0.07-0.13%) (1) that is typically asymptomatic and discovered incidentally during device implantation. Serious complications, including PLSVC-like shock, cardiac arrest or angina, can occur during catheterisation, which is likely due to the manipulation of the catheter in the coronary sinus or the perforation of the brachiocephalic vein, which can occur in the absence of the RSVC (2). This report presents a patient with PLSVC and an absent RSVC with no other cardiac abnormalities, who was diagnosed incidentally during pacemaker implantation (PMI). A 65-year-old male patient with hypertension was referred to our clinic for pacemaker implantation. The patient had suffered from vertigo with several presyncopal episodes. A 24-hour Holter revealed a 2:1 second-degree atrioventricular block with an intermittent third-degree atrioventricular block. These results prompted pacemaker implantation. The guidewire did not advance to the RSVC after routine puncture of the left axillary vein. Instead, an acute downward angula- tion was performed on the left side, parallel to the right-sided vena cava. A venogram was performed, and the diagnosis of PLSVC with an RSVC that descended into the coronary sinus (CS) was confirmed (Fig. 1A-D). Two 60-cm leads (Setrox S 60-B active fixation, Biotronik GmbH & Co., Berlin, Germany) were advanced separately in the right

Persistent left superior vena cava draining to the left atrium: A case report and review of the literature

Persistent left superior vena cava is a rare but important congenital vascular anomaly. However, PLSVC with absent RSVC (isolated PLSVC) is a very rare venous malformation We report on a rare case of persistent left superior vena cava (PLSVC) with absent right superior vena cava (RSVC). This venous malformation was identified incidentally in a 69-year-old woman during chest multi-detector computed tomography (MDCT). On chest MDCT, the SVC was noted on the left side. A bridging vein drained the right jugular and right subclavian veins and joined the left brachiocephalic vein in order to form the PLSVC, which descended on the left side of the mediastinum and drained into the left atrium (LA). The patient had no additional cardiac anomaly. Isolated PLSVC is usually asymptomatic but it can pose difficulties for establishing central venous access, pacemaker implantation and cardiothoracic surgery. This condition is also associated with an increased incidence of congenital heart disease, arrhythmias and conduction disturbances. A wide spectrum of clinicians should be aware of this anomaly, its variations and possible complications.

Isolated persistent left superior vena cava with absent right superior vena cava in two cases

Persistent left superior vena cava (LSVC) with absent right superior vena cava (RSVC) is a very rare congenital anomaly. Its isolated existence is even rarer. Persistent LSVC is usually asymptomatic and discovered incidentally. We present persistent LSVC with absent RSVC in two asymptomatic patients, namely, a 52-year-old woman and 65-year-old man. The diagnosis was confirmed by cardiac computed tomography in both cases.

Mitral Valve Replacement in Patient with Absent Right Superior Vena Cava, Pectus Excavatum, Marfan Syndrome and Severe Mitral Regurgitation

Combination of persistent left superior vena cava (PLSVC) draining into right atrium via the coronary sinus with a normal right superior vena cava (RSVC) is the most common systemic venous anomaly in visceroatrial situs solitus. On the other hand, a PLSVC in absence of RSVC is an extremely rare anomaly. Cardiovascular abnormalities are frequently encountered in patients with Marfan's syndrome but absence of RSVC with PLSVC is not reported in these patients. We report on a 52-year-old man with Marfan syndrome, an absence of right superior vena cava, severe pectus excavatum and severe mitral regurgitation who underwent successful mitral valve replacement. CT scan chest revealed marked cardiac deviation to the left associated with severe pectus excavatum, absent right superior vena cava and persistent left superior vena cava. During surgery replacement of mitral valve was performed through midsternotomy successfully.

Absent Right Superior Vena Cava and Persistent Left Superior Vena Cava: The Perioperative Implications

A 22-yr-old female patient was admitted for repair of tetralogy of Fallot. Preoperative transthoracic echocardiography revealed large subaortic ventricular septal defect, 50% aortic over-ride, severe valvular and subvalvular pulmonary stenosis, persistent left superior vena cava (PLSVC), and a large coronary sinus (CS). After anesthetic induction, central venous cannulation of the right internal jugular vein was attempted with a 20-guage cannula using Seldinger’s technique. Despite free flow through the cannula, the guidewire could not be advanced beyond 8 cm; therefore, a pediatric (8 cm, 5.5F) triple-lumen catheter was inserted. Intraoperative transesophageal echocardiography (TEE) imaging of the midesophageal (ME) bicaval view with the transducer steered through 90–120° suggested absence of the right superior vena cava (RTSVC) (Figs. 1a and b) (please see video loops available at www.anesthesia-analgesia.com). However, a large PLSVC and CS (Fig. 2) (see video loop) were seen midway between the ME four-chamber and a deep four-chamber view; the probe was slightly rotated counterclockwise. The absent RTSVC was evident on opening the pericardium. After aortic and inferior vena cava cannulation and institution of cardiopulmonary bypass a separate cannula was inserted extrapericardially in the PLSVC. The surgical repair and postoperative course were uneventful. An absent RTSVC is always associated with a PLSVC, with a reported incidence of 0.1% (1). It should be suspected when the chest radiograph does not show its characteristic shadow, particularly with an extra shadow (PLSVC) above the aortic knob (1). On TEE examination, the RTSVC is usually well visualized in the ME bicaval view. Agitated saline administered centrally appears as contrast in the RTSVC and right atrium (RA). With an absent RTSVC, contrast appears in the RA via the CS and can be viewed just below the ME four-chamber view and in bicaval view; however, if the CS is unroofed [absence of common wall between CS and left atrium (LA)], the contrast appears in both the LA and RA. A PLSVC descends along the left paramediastinal structures, anterior to the aortic arch and left pulmonary artery. It then passes inferiorly; in its course receives its tributary the hemiazygos vein, and then descends medially between the left pulmonary veins and left atrial appendage joining the CS in the atrioventricular groove (Fig. 3) causing an enlarged CS. Enlargement of the CS ( 1 cm) may indicate right atrial hypertension, an anomalous systemic venous channel draining through it (e.g., connection of PLSVC, hepatic veins, inferior vena cava), fistulous communication with coronary arteries and total or partial anomalous pulmonary venous connection. A PLSVC can be seen in ME views in the vicinity of the left atrial appendage and left upper pulmonary vein with the probe rotated counterclockwise, and can be traced to its entry into the CS. Occasionally, a PLSVC can be misdiagnosed as descending thoracic aorta, however, it can be differentiated from the descending thoracic aorta by pulse-wave Doppler assessment that shows continuous flow in the PLSVC and pulsatile flow in the descending thoracic aorta. The diagnosis of PLSVC is classically made by injecting contrast into the left-sided brachiocephalic veins and demonstrating contrast appearance in the PLSVC, CS, and RA (in that sequence). In 10% of patients, a PLSVC may drain into the LA either directly or via an unroofed CS; in such a situation, contrast first appears in the LA. Because of the PLSVC draining into the LA (right to left shunt) the arterial blood gas analysis or pulse oximetry will show desaturation. Although, this finding will not help evaluation of cyanotic patients, it can create a diagnostic and management dilemma in patients with acyanotic lesions. This article has supplementary material on the Web site: www.anesthesia-analgesia.org.