Complete free wall isolation of arrhythmogenic persistent left superior vena cava.

INTRODUCTION: Persistent left superior vena cava (PLSVC) is one of the major sources of triggers and drivers of atrial fibrillation (AF). There has been no established PLSVC ablation procedure to eliminate the arrhythmogenicity along the entire length of PLSVC. METHODS AND RESULTS: A 70-year-old woman with a history of two previous catheter ablations for AF, mitral valvuloplasty, and an unroofed coronary sinus-type atrial septal defect closure underwent the redo AF ablations. The AF trigger and driver were identified within the patient's enlarged PLSVC. The AF was treated by complete PLSVC free wall isolation. CONCLUSION: Complete PLSVC free wall isolation may be an effective ablation method to eliminate the arrhythmogenicity along the entire length of the PLSVC.

Elucidating left atrial electrical potential with microelectrode catheter: A case of coronary sinus ostial atresia with small persistent left superior vena cava.

A 51-year-old woman presented with recurring palpitations. Electrocardiography revealed narrow QRS tachycardia with short RP configuration. Computed tomography showed coronary sinus (CS) ostial atresia along with a small persistent left superior vena cava (PLSVC). Electrophysiological study identified the retrograde earliest atrial activation site (EAAS) at the CS ostium without decremental properties, and para-Hisian pacing suggested retrograde atrioventricular nodal conduction. Using a 1.6-Fr microelectrode catheter distally placed in the CS via the PLSVC, EAAS was confirmed within the left atrium, not the CS ostium. Transseptal approach revealed a left lateral accessory pathway, which was successfully eliminated.

Prenatal Diagnosis and Postnatal Outcomes of Persistent Left Superior Vena Cava Associated With Mild Narrow Aorta: A Cohort Study.

PURPOSE: To explore prenatal ultrasonic features and prognosis of the persistent left superior vena cava (PLSVC) complicated with mild narrow aorta. MATERIALS AND METHODS: A retrospective study was conducted involving 1348 fetuses diagnosed with PLSVC prenatally between January 2016 and December 2019. Forty-five fetuses with PLSVC associated with mild narrow aorta were selected from the cohort as the study group and 79 fetuses with isolated PLSCV were recruited randomly as the control group. All clinical and ultrasound results, including images and parameters of cardiac structures, were reviewed retrospectively. General conditions, ultrasound (US) measurements, and fetal prognosis were compared between the groups. RESULTS: Aorta valve diameter (AOD), Z-score of aorta valve (AODz-score), aortic isthmus diameter (AOIsD), and pulmonary diameter (PAD)/AOD were significantly different in study group than control group no matter in the second or third trimester. Thirty-eight fetuses in study group were born with favorable outcomes after long-term follow-up. A total of 13.16% (5/38) remain mild narrow aorta and 3 of them showed smaller left ventricle after 3 years follow up. Prenatal AODz-score in infants remains mild narrow aorta after 2 years aged was higher than ones' aorta return to normal (P = .01), especially when AODz-score >1.725. Moreover, when prenatal ratio of AOIsD/left subclavian artery was <1.12, it was more likely that the aorta would remain mildly narrow at age 2. CONCLUSION: Fetuses diagnosed with PLSVC with mild narrow aorta had favorable prognosis. AODz-score and AOIsD/left subclavian artery may be two predictors that reveal the risk of a mildly narrowed aorta remaining after birth.

Persistant Left Superior Vena Cava with and Without Right Superior Vena Cava: Significance of Prenatal Diagnosis.

This study aims to define the associated anomalies with PLSVC, and to compare single PLSVC and bilateral superior vena cava in terms of accompanying anomalies and pregnancy outcomes. This was a retrospective study of the fetuses diagnosed with single and/or bilateral SVC at a tertiary fetal medicine center during 8 years. We detected 16 cases of single PLSVC and 84 cases of bilateral SVC. We found an association between the PLSVC and cardiac and extracardiac anomalies. Comparison between single PLSVC and BSVC cases revealed significant differences in the occurrence of heterotaxy and right isomerism. The study highlights the importance of prenatal diagnosis in PLSVC cases. Isolated PLSVC with situs solitus may be considered a benign finding, but larger studies are needed to understand the clinical implications of PLSVC in relation to chromosomal anomalies. Routine screening protocols should include three-vessel and trachea views to detect PLSVC.

Association of Prenatally Diagnosed Isolated Single Left Superior Vena Cava and Postnatal Development of Coarctation of the Aorta.

To report the prevalence of coarctation of the aorta (CoA) in fetuses with single left superior vena cava (SL-SVC) and to evaluate changes in echocardiographic measurements. Additionally, to report the prevalence of associated malformations. Retrospective observational study of fetuses diagnosed with SL-SVC between 2012 and 2021 at a tertiary fetal cardiology unit. In fetuses without intracardiac abnormalities, Z-scores of the ventricles, great arteries, and Doppler flow patterns are reported. We identified 47 fetuses with SL-SVC of which 8/47 (17%) had abnormal intracardiac anatomy. One fetus was lost to follow-up. Of those with normal intracardiac anatomy and postnatal follow-up (38), karyotype abnormalities were confirmed in 2/38 (5%) and ECA in 8/38 (21%). 33/38 were live-born. None developed CoA postnatally. Paired analysis of Z-scores between early and late scans of 24 fetuses showed that diameters of the right heart structures and Doppler flows of tricuspid valve increased significantly during pregnancy, while the left heart structures and flow patterns did not change. The median risk of CoA did not change between the early and the late scan. We did not observe CoA in this cohort. A degree of ventricular asymmetry was present, but this was due to right heart dominance rather than hypoplasia of left heart structures. This likely reflects redistribution of blood and does not appear to confer increased risk of CoA. Predictive models of the postnatal development of CoA which set the dimensions of right and left heart structures in relation might not be applicable in this situation.

Minimally invasive redo tricuspid valve replacement in patient with persistent left superior vena cava.

INTRODUCTION: The management of cardiopulmonary bypass (CPB) is still challenging in certain circumstances, especially for patients with anatomical variations. This challenge is even harder for reoperations, which are associated with increased morbidity and mortality risk. CASE REPORT: We describe a minimally invasive, beating-heart redo tricuspid valve replacement in a 71-years old woman with persistent left superior vena cava. DISCUSSION: Preoperative planning via CT-scan, teamwork and custom-made management of CPB are crucial for reoperations with anatomical variations. The perfusionist has a pivotal role in constructing and managing the CPB. CONCLUSION: We describe a strategy achieving the benefits of minimally invasive endoscopic and beating-heart surgery (avoidance of resternotomy risk and associated morbidity, right ventricular protection) in reoperative tricuspid surgery with persistent upper left vena cava.

Retrograde fast pathway cryoablation inside the coronary sinus for slow-fast atrioventricular nodal reentrant tachycardia in a patient with persistent left superior vena cava.

INTRODUCTION: Persistent left superior vena cava (PLSVC) is accompanied by enlarged coronary sinus (CS) and deformation of the triangle of Koch. This makes anatomical evaluation of the atrioventricular (AV) nodal pathways difficult. METHODS: We attempted cryoablation of retrograde fast pathway located in the enlarged CS roof of PLSVC for slow-fast AV nodal reentrant tachycardia (AVNRT) induced by inadvertent antegrade fast pathway elimination during ablation of left atrial tachycardia. RESULTS: Slow-fast AVNRT was successfully eliminated without AV block progression. CONCLUSIONS: This is the first case of successful retrograde fast pathway ablation of the CS ostial roof for slow-fast AVNRT with PLSVC.

Pulsed-field-ablation for the treatment of atrial fibrillation in patients with congenital anomalies of cardiac veins.

INTRODUCTION: Anomalous cardiac veins are not rare and pulmonary vein (PV) isolation for atrial fibrillation (AF) treatment should include these veins. Pulsed-field ablation (PFA) is a novel technology for AF ablation with excellent efficacy and safety profile. In this case series, we describe our first experience of isolation of anomalous cardiac veins using PFA in patients with AF. METHODS: We report a series of patients with congenital anomalies of the cardiac veins and AF, treated with PFA. All patients underwent cardiac computed tomography for procedural planning. RESULTS: We included five patients (four males). Anomalous cardiac veins included a connection of a left common ostium to the coronary sinus, a partial and complete drainage of the right superior PV into the superior vena cava (SVC) with and without additional atrial septal defect, a persistent left SVC and an anomalous posterior PV. All anomalous PVs were isolated using PFA. No phrenic nerve palsy or other complications occurred. PFA of an abnormal right superior PV draining into the distal SVC was possible without affecting the sinus node. After a median of 4 months, four patients were free of recurrence. One patient had recurrent AF and perimitral reentry tachycardia, probably facilitated by PFA in the mitral isthmus region during isolation of an anomalous connection of the left common ostium to the coronary sinus. CONCLUSIONS: Using systematic preprocedural imaging and three-dimensional-electroanatomic mapping, the currently available PFA system seems well suited, efficient, and versatile for the treatment of AF in patients with anomalous cardiac veins.

Right sided approach for left bundle branch pacing using lumen-less lead: Technical considerations and follow-up outcome.

INTRODUCTION: Left bundle branch pacing has gained significant momentum in the last few years. The procedure involves deploying the lead deep inside the interventricular septum through left subclavian vein. We aimed at analyzing the feasibility, efficacy and long-term outcome of left bundle branch pacing (LBBP) using lumen-less lead through the right subclavian vein. METHODS: This was a retrospective-institutional, single center observational study done in consecutive patients who underwent LBBP using 3830 selectsecuretm lead. Left subclavian venous access was the primary strategy for lead implantation. Patients requiring right sided approach due to venous obstruction or persistent left superior-vena-cava (PLSVC) for LBBP were included in the study. RESULTS: Right sided approach was successful in 16 out of 19 (84%) attempted patients. C315-His catheter was used in all patients without modifying its curvature. PLSVC (n = 7), left venous obstruction (n = 7), right sided device upgradation (n = 1) and left pocket infection (n = 1) were the reasons for right sided approach. Mean follow-up duration was 17 ± 12 months. LBBP resulted in reduction in QRS duration from 137.3 ± 37.8 ms to 122.3 ± 9.5 ms (p -.13) and increase in LV ejection fraction from 46.2 ± 16.3% to 54.4 ± 11.6% (p -.11). The mean fluoroscopy duration and radiation dose were significantly high in right sided approach (n = 16) as compared to left sided approach (n = 293). In patients requiring cardiac-resynchronization therapy (CRT), right sided LBBP resulted in reduction in QRS duration from 171.8 ± 18.5 to 125.5 ± 11.9 ms (p -.0001) and increase in LVEF from 29.1 ± 3.8 to 45.1 ± 11.9% (p -.005). CONCLUSION: Right sided LBBP is feasible, safe and effective in patients requiring pacing for symptomatic bradyarrhythmia and CRT. Further development in dedicated tools for right-sided approach would help in reducing the fluoroscopy-duration and radiation-dose.

Pre-procedural imaging and atrial tachycardia ablation in a patient with complex congenital heart disease.

Atrial tachycardias (AT) are common cardiac arrhythmia disorder for congenital heart disease (CHD). The anatomic substrate that surgical suture lines, scar tissue, or prosthetic material may cause pre-existing atrial conduction abnormalities which leads to the underlying mechanism of reentrant ATs. Radiofrequency Catheter ablation (RFCA) is used in the treatment of atrial tachycardia in CHD patients. However venous system abnormalities may complicate the procedure. We report that ablation of a case with atrial tachycardia with challenging anatomy (persistent left superior vena cava draining into the left atrium, coronary sinus agenesis, inferior vena cava (IVC) agenesis, azygos system drained to the superior vena cava, and repaired ventricular septal defect). This case report discusses the key points of access to cardiac chambers and mapping in very rare challenging anatomy.

Catheter ablation of the left-sided variant of right top pulmonary vein in a case with persistent left superior vena cava.

A 50-year-old woman underwent catheter ablation for atrial fibrillation. Preoperative computed tomography revealed a left-sided variant of the right top pulmonary vein (PV) and a persistent left superior vena cava. The right top PV was successfully isolated through a wide antral circumferential ablation line simultaneously with the right PVs.

Persistent left superior vena cava isolation in patients with atrial fibrillation: Selective or empirical?

BACKGROUND: Persistent left superior vena cava (PLSVC) is the most prevalent form of thoracic venous abnormality and can serve as a significant arrhythmogenic source in atrial fibrillation (AF). METHODS AND RESULTS: Among the 3950 patients who underwent radiofrequency ablation for AF between September 2014 to April 2020, 17 patients (mean age 59.4 ± 8.0 years, 64.7% male) with PLSVC were identified. Among them, nine patients (52.9%) had a prior history of pulmonary vein isolation (PVI) alone. Eight out of nine patients who experienced AF recurrence underwent PLSVC isolation with or without pulmonary vein (PV) reconnection. For the remaining eight patients (47.1%), PVI plus PLSVC isolation were performed during the index procedure. Ectopy originating from PLSVC was documented in 11 patients (64.7%) and successful PLSVC isolation was achieved in 16 patients (94.1%). After a median follow-up of 28.3 months, freedom from AF/ atrial tachycardia (AT) was observed in 13 patients (76.5%). CONCLUSION: Empirical PLSVC isolation beyond PVI appears to be a feasible and safe strategy to prevent AF recurrence in patients with concomitant PLSVC.

A rare complication of double prosthetic valve endocarditis; reconstructive surgical treatment of mitral-aortic intervalvular fibrosa pseudoaneurysm and left atrial fistula.

Mitral aortic intervalvular fibrosa or aorto-mitral curtain is a fibrous avascular skeletal structure located between the anterior leaflet of the mitral valve and the non-coronary and left coronary cusps of the aortic valve. Mitral and aortic valve endocarditis are rarely accompanied by mitral aortic intervalvular fibrosa pseudoaneurysm and left atrial fistula of the aorta. Pseudoaneurysm of mitral aortic intervalvular fibrosa is a fatal complication that can occur after valvular surgery, valvular endocarditis, or blunt trauma. In this article, reconstructive surgical management with the Commando technique of a case who developed mitral-aortic intervalvular fibrosa pseudoaneurysm to left atrial fistula after aortic and mitral prosthetic valve endocarditis is described. The important feature of this article is that it is a first in the literature as it is accompanied by persistent left superior vena cava.

Catheter fragment removal from a persistent left superior vena cava in a pediatric patient.

Tunneled central venous catheters and ports provide a long-term method of delivering nutrition, hydration, or medications in children. When these devices are no longer needed, it is best practice to remove them entirely. Complications associated with having long-term venous access devices or the process of device removal include site infections, venous thrombosis or occlusion, device fracture, and possible migration of fractured fragments. We present a case of catheter fragmentation that occurred in a pediatric patient during removal of a 3-year-old left chest port that had been placed into a left superior vena cava (SVC).

Persistent left superior vena cava without right superior vena cava during fetal life.

Enlarged coronary sinus detected during cardiac examination in the prenatal period is the first finding that raises suspicion for persistent left superior vena cava. In this report, a patient was presented who was referred with the prediagnosis of cor triatriatum sinister in the antenatal period and diagnosed with isolated persistent left superior vena cava (SVC).

Coronary Sinus Defect, Premature Restriction of Foramen Ovale and Cysto-Colic Peritoneal Band.

Background: Unroofed coronary sinus is a congenital cardiac anomaly usually associated with persistent left superior vena cava. Premature restriction or closure of foramen ovale is described in association with hypoplastic left heart syndrome. Abdominal peritoneal bands when present manifest clinically. Case report: A 27 years, gravida 2, presented with intrauterine fetal death at 24 weeks gestation due to fetal congestive cardiac failure, cardiomegaly and hydrops. Perinatal autopsy showed absent coronary sinus with cardiac veins draining directly into the heart. There was no persistent left superior vena cava. The foramen ovale was restricted prematurely. The ductus arteriosus was present and non-restrictive. Abdomen showed a cysto-colic peritoneal band. Conclusion: This is the first report showing a triad of (1) complete absence of coronary sinus without left superior vena cava (type-II); (2) premature restriction of foramen ovale without hypoplastic left heart; and (3) a cysto-colic peritoneal band between the gall bladder and colon.

Anatomical variations in coronary venous drainage: Challenges and solutions in delivering cardiac resynchronization therapy.

AIMS: To investigate the abnormalities of the coronary venous system in candidates for cardiac resynchronization therapy (CRT) and describe methods for circumventing the resulting difficulties. METHODS: From four implanting institutes, data of all CRT implants between October 2008 and October 2020 were screened for abnormal cardiac venous anatomy, defined as an anatomical variation not conforming to the accepted 'normal' anatomy. Patient demographics, procedural detail, and subsequent left ventricle (LV) lead pacing indices were collected. RESULTS: From a total of 3548 CRT implants, 15 (0.42%) patients (80% male) of 72.2 ± 10.6 years in age with an LV ejection fraction of 34 ± 10.3% were identified to have had an abnormal cardiac venous anatomy over the study period. There were 13 cases of persistent left side superior vena cava (pLSVC), five of which had coronary sinus ostium atresia (CSOA) including two with an "unroofed" coronary sinus (CS); one patient had a unique anomalous origin of the CS and one patient had an isolated CSOA. In total 14 patients (60% repeat attempt) had successful percutaneous implant under general anesthesia (46.7%) via the cephalic vein (59.1%), using the femoral approach (53.3%) for levophase venography and/or pull-through, including one case of endocardial LV implant. Pacing follow-up over 37.64 ± 37.6 months demonstrated LV lead threshold between 0.62 and 2.9 volts (pulsewidth 0.4-1.5 ms) in all cases; five patients died within 2.92 ± 1.6 years of a successful implant. CONCLUSION: CRT devices can be implanted percutaneously even in the presence of substantial abnormalities of coronary venous anatomy. Alternative routes of venous access may be required.

Persistent left superior vena cava transvenous lead extraction: A European experience.

BACKGROUND: Transvenous lead extraction (TLE) is rising in parallel to cardiac implantable electronic device implantations. Persistent left side superior vena cava (PLSVC) is a relatively common anatomical variant in the healthy population; TLE in patients with a PLSVC is rare. METHOD: Data were collated from 6 European TLE institutes of 10 patients who had undergone lead extraction with a PLSVC. Patient demographics, procedural challenges and outcomes were reported. RESULTS: Ten patients aged 73.4 ± 7.8 years (60% male) underwent TLE of 20 leads (3 left ventricle, 10 right ventricle, 7 right atrium) with dwell time of 82.95 ± 39.1 months. Of the 10 cases, 4 had an infection indication and 5 were biventricular system extractions; 25% of the extracted leads were defibrillator leads. The majority of the procedures were completed in the cardiac catheterization suite (80%) under general anaesthesia (60%) by cardiologists (80%) using a rotational powered sheath (65%). The Tandem approach was used successfully in 3 cases. Complete procedural success was obtained in 100% of cases in the absence of complications within 127.4 ± 74.7 min. There was no 30-day mortality. CONCLUSION: TLE in PLSVC is feasible albeit rare. Standard extraction techniques in experienced hands are associated with favorable outcomes; the Tandem procedure may be an additional technique to improve the safety and efficacy of TLE in PLSVC.

Single left superior vena cava: antenatal diagnosis, associated anomalies and outcomes.

OBJECTIVES: To describe the associated cardiac and extracardiac findings and estimate the prevalence of single left superior vena cava (LSVC) among fetuses referred for fetal echocardiography. METHODS: This was a retrospective case series of fetuses diagnosed with situs solitus and single LSVC at the Brompton Centre for Fetal Cardiology, London, UK, from October 2006 to December 2020. Prenatal and postnatal outcome data were collected. Prenatal diagnosis was based on abnormal vessel alignment at the three-vessel view and/or three-vessel-and-trachea view, showing a vessel to the left of the pulmonary artery (i.e. the LSVC) and absence of the usual vessel to the right of the ascending aorta (i.e. the right superior vena cava), and further visualization of the LSVC draining into the coronary sinus. RESULTS: Of 19 968 fetal echocardiograms performed during the study period, 34 cases of single LSVC were identified (a prevalence of 0.17%). Of these, 32 pregnancies had a live birth, one was lost to follow-up and one resulted in intrauterine demise. Single LSVC was isolated in 79.4% of cases. No major congenital heart disease was identified. One fetus showed mild isthmus hypoplasia, with no aortic coarctation postnatally. Two fetuses had umbilical vessel abnormalities. A genetic abnormality was found in one case (15q24.1-q24.2 deletion). CONCLUSIONS: Antenatal diagnosis of single LSVC in the setting of situs solitus is usually a benign isolated finding. Nevertheless, investigation of other cardiac, extracardiac and genetic disorders should be considered. © 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Feasibility and safety of left atrial access for ablation of atrial fibrillation in patients with persistent left superior vena cava.

BACKGROUND: In patients with persistent left superior vena cava (PLSVC) ablation procedures can be challenging. We sought to determine the feasibility and safety of left atrial ablations in patients with PLSVC, especially when PLSVC is unknown prior to the ablation procedure. METHODS AND RESULTS: In this retrospective analysis 15 adult patients (mean age 64.6 ± 14.5 years, 53.3% male) with PLSVC undergoing 27 ablation procedures for atrial fibrillation or left atrial flutter were included. In 5 (33.3%) patients PLSVC was only discovered during the procedure. Transseptal puncture (TSP) was declared "difficult" by the ablating physician in 13 of 27 (48.2%) procedures and was not successfully completed in the first attempt in two patients with known PLSVC. Once TSP was successfully completed, all relevant structures were reached both during mapping and ablation in all procedures independent of whether PLSVC was known prior to the procedure. One major complication (3.7%) occurred in 27 procedures in a patient with known PLSVC. In the patients with unknown PLSVC no complication occurred. CONCLUSION: In experienced hands, left atrial access and ablation in patients with PLSVC is feasible and safe, particularly with regard to patients in whom the PLSVC is unknown prior to the ablation procedure.