Pathogenesis of Persistent Left Superior Vena Cava with a Coronary Sinus Connection

The basis for persistence of the left superior vena cava (LSVC), usually associated with cardiac malformations, is poorly understood. We examined 351 staged, serially sectioned human embryos in the Carnegie Embryological Collection and 1208 specimens with congenital cardiovascular malformations in the Pathology Collection of the Johns Hopkins Hospital. A standardized questionnaire was answered for each embryo and autopsy case and a computer program was employed to tabulate concurrent anatomic features. In the normal embryos a symmetric venous system appeared with the heart tube at Carnegie stage 9; the sinoatrial junction translocated to the right and the relationship of the coronary sinus to the LSVC was established by stage 12. The LSVC was patent through stage 20 and subsequently underwent luminal obliteration by compression between the left atrium and the hilum of the left lung. Among the 1208 hearts with a congenital abnormality, 104 (9%) had a persistent LSVC with a coronary sinus connection. Statistically, significantly more frequent associations were found between persistent LSVC and atrioventricular canal defects, cor triatriatum, and mitral atresia and a significantly less frequent association was observed between persistent LSVC and atrial septal defect or patent foramen ovale as a primary defect. The normally late embryonic obliteration of the LSVC suggests that its persistence would be secondary to reduce cardiac compression or to blood flow redistribution at an early stage, and the malformations associated with persistent LSVC support that view. Identification of a persistent left superior vena cava with coronary sinus connection should suggest an associated malformation, especially atrioventricular canal, cor trialriatum, or mitral atresia.     

Persistent Left Superior Vena Cava Draining Into an Unroofed Coronary Sinus in Tricuspid Atresia: An Uncommon Anomaly

This report describes the preoperative diagnosis of a 6-month-old child with tricuspid atresia related to an unroofed coronary sinus. The anomaly usually is diagnosed after the Fontan operation due to persistent arterial desaturation. Preoperative recognition of this association is important. All cases of tricuspid atresia should be screened for associated persistent left superior vena cava draining into an unroofed coronary sinus.     

Absent Left Superior Vena Cava with Persistent Right Superior Vena Cava in Visceroatrial Situs Inversus

We report an unusual anomaly of the upper body venous drainage in a patient with visceroatrial situs inversus. The patient had a persistent right superior vena cava draining into the left-sided right atrium by way of the coronary sinus, with the absence of the left superior vena cava associated with atrioventricular discordant levocardia, pulmonary atresia, and ventricular septal defect.     

Amplatzer Septal Occluder Closure of Secundum Atrial Septal Defects in the Presence of Persistent Left Superior Vena Cava to Coronary Sinus

Secundum atrial septal defects (ASDs) are routinely closed using transcatheter devices. In patients with left superior vena cava (LSVC) draining to the coronary sinus (CS), the device must not obstruct CS drainage. We report five cases of successful ASD device closure without obstructing flow from the LSVC or dilated CS.     

Coronary Sinus Ostial Atresia Associated With Functionally Single Ventricle: Persistent Left Superior Vena Cava Should Be Ligated Upon Coronary Sinus Unroofing and Bidirectional Glenn Shunt

An 11-month-old boy with a functionally single ventricle, coronary sinus ostial atresia, and bilateral superior vena cava (SVC) underwent coronary sinus unroofing upon a right bidirectional cavopulmonary shunt. A persistent left SVC left open to guarantee dual coronary venous drainage became dilated with cephalocaudal blood flow reversal and desaturation. The left SVC was surgically ligated on postoperative day 35.     

Lutembacher Syndrome With Unroofed Left Superior Vena Cava: A Diagnostic Dilemma

Lutembacher syndrome involving the association of congenital atrial septal defect (ASD), usually of the ostium secundum variety, and mitral valve disease is a well-known entity. Its association with a coronary sinus, ASD, and a persistent left superior vena cava (LSVC) draining into the left atrium (LA) (Raghib syndrome) is rarely described in the literature. This association in a 15-year-old boy erroneously deemed to be inoperable before referral to the authors’ hospital due to cyanosis in the presence of atrial septal defect (ASD) and mitral stenosis is described in this report. Evaluation by echocardiography followed by cine angiography confirmed the cause of cyanosis to be drainage of the LSVC into the LA together with an ASD and rheumatic mitral stenosis, a combination of Raghib and Lutembacher syndromes. The boy underwent successful surgical correction. The authors believe this is the second such case to be reported in the English literature and the first of its kind to be managed by surgical intervention.     

Persistent Left Superior Vena Cava Resulting in Left-to-Right Shunt

We present an infant with unusual anatomy and physiology of a persistent left superior vena cava (LSVC). The adverse physiologic consequences of the LSVC were corrected with percutaneous placement of vascular plugs.     

Persistent Left Superior Vena Cava in Cardiac Congenital Surgery

Persistent left superior vena cava (LSVC) is a relatively frequent finding in congenital cardiac malformation. The scope of the study was to analyze the timing of diagnosis of persistent LSVC, the timing of diagnosis of associated anomalies of the coronary sinus, and the global impact on morbidity and mortality of persistent LSVC in children with congenital heart disease after cardiac surgery. Retrospective analysis of a cohort of children after cardiac surgery on bypass for congenital heart disease. Three hundred seventy-one patients were included in the study, and their median age was 2.75 years (IQR 0.65–6.63). Forty-seven children had persistent LSVC (12.7 %), and persistent LSVC was identified on echocardiography before surgery in 39 patients (83 %). In three patients (6.4 %) with persistent LSVC, significant inflow obstruction of the left ventricle developed after surgery leading to low output syndrome or secondary pulmonary hypertension. In eight patients (17 %), persistent LSVC was associated with a partially or completely unroofed coronary sinus and in two cases (4 %) with coronary sinus ostial atresia. Duration of mechanical ventilation was significantly shorter in the control group (1.2 vs. 3.0 days, p = 0.04), whereas length of stay in intensive care did not differ. Mortality was also significantly lower in the control group (2.5 vs. 10.6 %, p = 0.004). The results of study show that persistent LSVC in association with congenital cardiac malformation increases the risk of mortality in children with cardiac surgery on cardiopulmonary bypass. Recognition of a persistent LSVC and its associated anomalies is mandatory to avoid complications during or after cardiac surgery.     

Chronic Cyanosis Due to Persistent Left Superior Vena Cava Draining into the Left Atrium in the Absence of a Coronary Sinus

We report a rare case of a 5-year-old child who presented with chronic hypoxemia and a normal cardiac examination, and was found to have a persistent left superior vena cava draining directly into the left atrium. The coronary sinus was absent. This case introduces a contradiction to the generally accepted concept that this anomalous connection can only occur in the presence of a partly or completely unroofed coronary sinus. High index of suspicion is required to include this pathology in the differential diagnosis for a patient with persistent cyanosis with normal cardiac examination.     

Persistent Left Superior Vena Cava Connected to the Coronary Sinus in the Fetus: Effects on Cardiac Structure and Flow Dynamics

Ventricular size discrepancy may be due to a persistent left superior vena cava (PLSVC) in utero. We sought to investigate for differences in cardiac structure measures and hemodynamics between fetuses with isolated PLSVC connected to the coronary sinus (CS) and normal. Fetuses diagnosed with isolated PLSVC in the second and third trimester were enrolled. We defined two groups: group 1, twenty-five fetuses in the second trimester (22–27 W + 6d); group 2, twenty-two fetuses in the third trimester (28–39 W + 6d). Fifty-three fetuses without intra-cardiac or extra-cardiac anomalies and gestation age-matched were divided into normal control groups: group 3, 28 fetuses in the second trimester; group 4, 25 fetuses in the third trimester. Parameters of cardiac structure and hemodynamics were measured, including: left- and right-side heart size, the diameter of foramen ovale, aorta (AO), aortic isthmus and pulmonary artery (PA), and ratios of cardiac structure RV/LV, RA/LA and PA/AO were calculated. Hemodynamic parameters measured included: flow velocity across mitral valve, tricuspid valve and foramen ovale. In the second trimester, the ratio of RV/LV and PA/AO of the PLSVC fetuses was significantly larger than normal, while the AO diameter, aortic isthmus diameter were smaller than normal (P < 0.05). However, in the third trimester, only the ratio of PA/AO of the PLSVC fetuses was significantly larger, and the aortic isthmus diameter was still smaller than normal (P < 0.05). Isolated PLSVC connecting to the CS is associated with differences in cardiac structure size from normal. These differences appear to diminish with gestational age. A dilated CS may have an influence on development of fetal left heart structures.     

Absent Right and Persistent Left Superior Vena Cava: Fetal and Neonatal Echocardiographic Diagnosis

Absence of the right superior vena cava (SVC) is a rare event occurring in patients without congenital cardiovascular abnormalities. Since absence of the right SVC is usually clinically silent, its diagnosis is mandatory prior to invasive medical or surgical procedures. We report two cases of echocardiographic diagnosis of absence of the right SVC with persistent left SVC and a large coronary sinus in structurally normal heart in a fetus of 20 weeks’ gestation and in a newborn. The diagnosis was confirmed by transthoracic contrast echocardiography with intravenous injection of agitated saline into the right arm.     

Is Surgical Ligation of an Accessory Left Superior Vena Cava Always Safe?

In patients considered for bidirectional Glenn or Fontan procedures, the association of left superior vena cava (LSVC) with ostial atresia of the coronary sinus should be diagnosed preoperatively in order to avoid surgical division or ligation of the LSVC and the negative effect of resulting coronary venous hypertension on myocardial perfusion. This report discusses the angiographic and hemodynamic features of LSVC when it is the only drainage route from a blind coronary sinus. A retrograde flow in the LSVC seen by Doppler ultrasonography should raise the suspicion of this diagnosis.     

Transcatheter Occlusion of a Large Left Coronary Artery to Right Superior Vena Cava Fistula Using the Amplatzer Duct Occluder Device

Transcatheter embolization has become the therapy of choice for most coronary artery fistula. We report a 5.9-kg infant with a coronary artery fistula from the left coronary artery to the right superior vena cava with significant congestive heart failure. The infant underwent successful complete occlusion using the Amplatzer duct occluder.     

Superior Vena Cava and Innominate Vein Dimensions in Growing Children

Transvenous (TV) pacing and defibrillation leads are frequently implanted in children as part of treatment for various congenital and acquired rhythm abnormalities. However, the lead–vascular endothelial interaction is not a benign process and is associated with a risk of progressive venous obstruction. Often, this obstruction requires surgical or interventional relief. The risk of obstruction is related to venous diameters at implant and lead size. Since venous diameters are largely unknown at different ages, the purpose of this study was to correlate innominate vein (INN) and superior vena cava (SVC) diameters with body dimensions in growing children.     

Focal Atrial Tachycardia Originating From Inside the Inferior Vena Cava Late After Surgical Repair of Congenital Heart Defects

An 11-year-old girl presented with focal atrial tachycardia (AT) originating from inside the inferior vena cava (IVC). Sustained AT appeared 10 years after surgical repair of the congenital heart defects. Focal AT was successfully eliminated with radiofrequency application at the earliest activation site, about 8 mm below the IVC right atrium junction. These findings suggested the existence of an electrically continuous myocardial extension in the IVC and a real risk of AT originating from IVC late after cardiopulmonary bypass with IVC cannulation.     

Biatrial or Left Atrial Drainage of the Right Superior Vena Cava: Anatomic,Morphogenetic, and Surgical Considerations Report of Three New Cases and Literature Review

Since the posterior wall of the right superior vena cava (RSVC) is contiguous with the anterior wall of the right upper pulmonary veins, a localized defect in this common wall may create a cavopulmonary venous confluence without eliminating the normal connection of the same right pulmonary veins with the left atrium (LA). Through this defect, blood of the unroofed right pulmonary veins will drain into the RSVC and right atrium (RA), and blood from the RSVC may shunt into the right pulmonary veins and LA. Hemodynamically, the RSVC will become biatrial. If the RSVC blood flows preferentially into the LA, its right atrial orifice will become stenotic or even atretic. If atretic, the normally positioned RSVC will drain entirely into the LA. In this report, we present the clinical and anatomical findings of two postmortem cases with biatrial drainage of the RSVC. We also document the clinical, echocardiographic, angiocardiographic, and surgical data of a living patient with left atrial drainage of the RSVC and tetralogy of Fallot with pulmonary atresia. The relevant literature and surgical treatment are reviewed, and the morphogenesis of the biatrial and left atrial RSVC is considered.