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      Transseptal access and pulmonary vein isolation via internal jugular veins for persistent atrial fibrillation treatment in a patient with left atrial isomerism, sinus node dysfunction, and interrupted inferior vena cava: The usefulness of robotic magnetic navigation

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          Abstract

          Introduction Atrial isomerism is a form of heterotaxy, which is defined as an abnormal arrangement of the internal thoracic-abdominal organs across the left-right axis of the body caused by disruption of left-right axis orientation during early embryonic development. Cardiac malformations are a major component of heterotaxy syndrome. Abnormal cardiac development typically leads to atrial isomerism, resulting in either bilateral paired right atria (right atrial isomerism) or paired left atria (left atrial isomerism; LAI). 1 , 2 Among the various kinds of congenital heart disease, atrial isomerism is rare. It occurs in approximately 1 per 10,000 to 40,000 live births. 3 LAI results in 2 left sides with bilateral morphologic left atria (LA) and left atrial appendages. Systemic venous abnormalities, such as interruption of the inferior vena cava (IVC), occurs in 80% of patients with LAI, who have subsequent drainage of the interrupted IVC into the azygos vein and, from there, to the atrium via the superior vena cava (SVC). 4 In patients with LAI the sinus node may be either absent or hypoplasic. 5 As a result, patients with LAI are more susceptible to sinus node dysfunction (SND) and atrial fibrillation (AF). 6 We now present a case report of pulmonary vein (PV) isolation to treat persistent AF in a patient with LAI, SND, and interrupted IVC. Methods Preprocedure findings A 31-year-old male patient presented with palpitations and dyspnea on exercise in February 2016. The patient had no significant past medical history and no family history of heart diseases. He has never smoked, and he denied alcohol intake and use of recreational drugs. The presenting electrocardiogram showed AF with ventricular heart rate around 85 beats per minute (bpm) and QRS duration 95 ms (Figure 1A). A 48-hour Holter showed AF 100% of the time with average heart rate of 85 bpm (range, 48–231 bpm). An echocardiogram showed normal size and function of the left and right ventricles; both atria with normal size and structure; and no valvular disease. The patient initiated beta blocker treatment for rate control and oral anticoagulation with apixaban. In April 2016 he underwent electrical cardioversion. Postcardioversion electrocardiogram showed junctional bradycardia that terminated into a low atrium rhythm at 75 bpm (Figure 1B). He remained asymptomatic for several months, having AF recurrence with need of cardioversion in May 2017 and February 2018. No antiarrhythmic drugs were attempted owing to a baseline low atrium bradycardia. The most recent cardioversion was successful for 1 week. The patient was very symptomatic while in AF and was scheduled for an AF ablation. Figure 1 A: The presenting electrocardiogram (ECG) showed atrial fibrillation with heart rate around 85 beats per minute (bpm), QRS 95 ms. B: Postcardioversion ECG showed junctional rhythm that terminated into a low atrium rhythm at 75 bpm. C: A right anterior oblique fluoroscopic projection showed a duodecapolar catheter positioned along the right atrium and coronary sinus. The angiogram revealed the intracardiac echocardiography (ICE) catheter and the long introducer to be outside the heart silhouette (black arrows). D: A left anterior oblique fluoroscopic projection showed the ICE catheter and the long introducer to be positioned posteriorly to the heart in the azygos vein. A written informed consent was obtained for the procedure. The presenting rhythm was AF. Access was obtained in the right femoral vein with ultrasound guidance using the modified Seldinger technique. Three short sheaths were introduced into the vein (8F, 8.5F, and 8.5F). A 7F split duodecapolar catheter (Livewire Duo Deca, 60 mm split, 2-8-2 mm; Abbott, Lake Bluff, IL) was advanced into the heart and placed into the coronary sinus. An 8F intracardiac echocardiography (ICE) catheter (Accuson Accunav; Siemens, Erlangen, DE) was introduced up into the heart, but it was noted that there was difficulty manipulating the catheter and visualizing heart structures. The short 8.5F short sheath was exchanged over a wire for an 8.5F long steerable sheath (Agilis NST; Abbott). The sheath was placed in what was thought to be the mid-right atrium and an angiogram was performed through the sheath. The angiogram showed an interrupted IVC with a large azygos continuation into the SVC (Figure 1C and D). Owing to the need for further anatomic evaluation prior to proceeding with the ablation, the procedure was aborted. A postprocedure chest magnetic resonance imaging showed both ventricles with normal size and function. The right-sided atrium showed characteristics of a morphologically LA. The bilateral subclavian, brachiocephalic, and SVC veins appeared to be widely patent. A large azygos vein was seen draining into the SVC. There was an IVC interruption with azygos continuation. The IVC was interrupted over 3 cm of the intrahepatic portion and there was right-sided polysplenia (Figure 2). Figure 2 Magnetic resonance imaging (MRI) showed an interruption of the inferior vena cava over 3 cm of the intrahepatic portion with azygos continuation (arrow) and right-sided polysplenia in a frontal plane (A) and in a sagittal plane (C). The MRI showed a large azygos vein draining into the superior vena cava (arrow) in both the frontal plane (B) and the sagittal plane (D). With the diagnosis of LAI with interrupted IVC, the patient was scheduled for a new AF ablation procedure via the right internal jugular (IJ) vein. Transseptal access via internal jugular veins A written informed consent was obtained. The procedure was performed under general anesthesia. Firstly, 2 right femoral venous accesses were obtained using the modified Seldinger technique. A 7F deflectable octapolar catheter (D-type curve; Biosense Webster, Irvine, CA) was advanced from the right femoral vein through the azygos vein, SVC, and right atrium and was placed in the right ventricle (RV). A 7F split duodecapolar catheter (Livewire Duo Deca, 60 mm split, 2-8-2 mm; Abbott) was advanced from the right femoral vein following the RV catheter and was placed into the coronary sinus. A left IJ venous access was obtained to advance an 8F ICE catheter (Accuson Accunav; Siemens) to the mid-RA (Figure 3A). Right IJ venous access was obtained using the modified Seldinger technique and an 8.5F short sheath was introduced into the vein. Transseptal catheterization was performed under fluoroscopic and ICE guidance. Heparin bolus and continuous intravenous infusion were given prior transseptal puncture. The sheath in the right IJ vein was exchanged over a wire for an 8.5F long steerable guiding sheath (Torflex Supracross Superior Access Sheath; Baylis Medical, Montreal, Canada) (Figure 3B). The long steerable sheath was advanced to the posterior portion of the tricuspid annulus and was deflected, maintaining the relative position of the sheath with the tip oriented in the 10 o’clock position from the operator’s view (leftward and anterior). Next, the long sheath was gently manipulated with counterclockwise rotation and was withdrawn approximately 2 cm, gradually changing the position of the sheath with the tip oriented to the 8 o’clock position (leftward and posterior). Once the long sheath reached the fossa ovalis, the deflection of the sheath was adjusted until an adequate interatrial septum tenting was visualized on ICE (Figure 3C). The transseptal access was performed using a radiofrequency needle (NRG; Baylis Medical). The position of the long steerable sheath was maintained and the transseptal needle was advanced until the sheath’s tip. Radiofrequency power was delivered at 10 watts. Successful transseptal puncture was achieved at the first attempt, with no complications. Once the transseptal access was obtained, the long steerable sheath was advanced over the transseptal needle to the LA (Figure 3D and E). The transseptal needle was exchanged for an 8F irrigated ablation catheter (ThermoCool RMT; Biosense Webster) and was positioned in the LA (Figure 3F). The heparin infusion rate was adjusted to maintain an activated clotting time between 350 and 400 seconds throughout the procedure. Figure 3 A, B: Right anterior oblique (RAO) fluoroscopic images. A 7F deflectable octapolar catheter was placed in the right ventricle (RV) from the right femoral vein through the azygos vein, superior vena cava, and right atrium (RA) (blue arrow); a 7F duodecapolar catheter was positioned along the RA and coronary sinus following the RV catheter course (green arrow); and an 8F intracardiac echocardiography (ICE) catheter was positioned in the mid-RA through the left internal jugular (IJ) vein (black arrow) (A). An 8.5F long steerable guiding sheath (Torflex Supracross Superior Access Sheath; Baylis Medical, Montreal, Canada) (red arrow) was positioned in the RA through the right IJ (B). C: ICE image showing atrial septum tenting with the long steerable sheath in the fossa ovalis previous to the transseptal access. D: ICE image: transseptal access was obtained with a radiofrequency needle (NRG; Baylis Medical) and the long steerable sheath was advanced into the left atrium (LA). E: RAO fluoroscopic image showing the long steerable sheath (red arrow) advanced into the LA through the transseptal access from the right IJ. F: RAO fluoroscopic image shows an 8F irrigated ablation catheter (ThermoCool RMT; Biosense Webster, Irvine, CA) (red arrow) positioned in the right superior pulmonary vein. G–J: Voltage map of the LA in the posteroanterior (G), anteroposterior (H), RAO (I), and left anterior oblique (J) views showed no significant areas of scar. Normal voltage range in atrial fibrillation was considered to be between 0.05 and 0.5 mV, shown in the right superior corner of each image. Bilateral radiofrequency wide-area circumferential ablation lesions and carinal lines (red marks) were performed to obtain isolation of all 4 pulmonary veins. An 8F irrigated ablation catheter (ThermoCool RMT; Biosense Webster) and RMN system (Stereotaxis, St Louis, MO) were used for both mapping and ablation. PV isolation The pulmonary veins and LA sites were mapped, making use of a 3D electroanatomic ablation mapping system (Carto 3; Biosense Webster). Voltage above 0.5 mV was considered normal owing to the presence of AF while mapping. Voltage less than 0.05 mV was representative of scar tissue. 7 An 8F irrigated ablation catheter (ThermoCool RMT; Biosense Webster) was used to construct a point-by-point map of the LA and PV. A robotic magnetic navigation (RMN) system (Stereotaxis, St Louis, MO) was used for both mapping and ablation. A 312-point voltage map of the LA using a fill threshold of 10 mm showed no significant areas of low voltage. Ablation was performed using radiofrequency energy. Applications were delivered via a generator (Stockert, Freiburg, Germany). Bilateral radiofrequency wide-area circumferential ablation lesions and carinal lines were performed on the ostia of the PV. A routinely high-power approach was used with a radiofrequency power set at 45 watts, independent of the location in the LA. The irrigation flow was 17 mL/min. Esophageal temperature was monitored continuously during the entire procedure. There were no difficulties in moving the catheter during both mapping and ablation. Successful isolation of all 4 PV was obtained (Figure 3G–J). Results Heart rhythm evaluation After achieving PV isolation, external cardioversion at 200 joules terminated the AF into a junctional bradycardia at 45 bpm. Atrial burst pacing showed an atrioventricular node Wenckebach cycle length <350 ms. H-V interval was normal. Entrance and exit block were demonstrated in all 4 PV. Rapid atrial burst pacing failed to induce any arrhythmia. The patient remained in junctional rhythm after 45 minutes post ablation. A temporary screw-in lead was placed in the RV through the right IJ for temporary pacing. After a 24-hour period of observation, the patient remained in a low atrial rhythm at 70 bpm alternating with some episodes of junctional bradycardia at 45 bpm. A permanent dual-chamber pacemaker (Azure XT DR; Medtronic, Minneapolis, MN) was successfully implanted through the left axillary vein 24 hours after the AF ablation. The pacemaker settings were programmed in DDD mode at 50–130 bpm with the “Managed Ventricular Pacing” feature, a paced A-V delay of 180 ms, and a sensed A-V delay of 150 ms. After 6 months of follow-up the patient has been completely asymptomatic. The pacemaker check showed no episodes of AF, 2.5% of atrial pacing, and no ventricular pacing needs. Oral anticoagulation was stopped 3 months after the procedure. Safety and adverse events No complications occurred either at the access sites or secondary to the transseptal access. The total radiation time for the AF ablation procedure was 33 minutes and the radiation dose was 4476 μGy·m2. Discussion We herein described a patient with LAI and SND presenting with symptomatic and persistent AF. The incidence of AF in these patients has been reported to be around 10%. Moreover, the rising prevalence of SND with age in these patients increases the risk of developing AF. On the other hand, bradyarrhythmias are frequent and highly complex in LAI patients; therefore the use of antiarrhythmic treatment alone is limited and catheter ablation should be considered. However, the interruption of the IVC is highly frequent in LAI; consequently, a standard transseptal approach via the femoral vein is not possible in these patients. 4 The Supplementary Material section provides a discussion about transseptal access from the right IJ, PV isolation with radiofrequency ablation and RMN, and SND management. Conclusion In summary, to the best of our knowledge, this is the first case report of the following: (1) successful transseptal access via right IJ vein with a steerable sheath and a radiofrequency needle in a patient with the combination of LAI, interrupted IVC, and SND; and (2) successful PV isolation via right IJ vein using radiofrequency ablation with RMN and no recurrence of AF after 6 months of follow-up. Key Teaching Points • In patients with left atrial isomerism and interrupted inferior vena cava, successful transseptal access can be obtained with a steerable sheath and a radiofrequency needle through the right internal jugular vein. • Robotic magnetic navigation offers advantages for catheter manipulation for both mapping and ablation within the left atrium in patients with interrupted vena cava with an internal jugular vein approach. • When planning the procedural approach of pulmonary vein isolation, it is important to consider that sinus node dysfunction is frequent in patients with left atrial isomerism.

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          Most cited references 7

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          Heterotaxy: associated conditions and hospital-based prevalence in newborns.

           A E Lin,  B Ticho,  K Houde (2015)
          To provide insight into the possible etiology and prevalence of heterotaxy, we studied conditions associated with heterotaxy in a consecutive hospital population of newborns. From 1972 to March, 1999 (except February 16, 1972 to December 31, 1978), 58 cases of heterotaxy were ascertained from a cohort of 201,084 births in the ongoing Active Malformation Surveillance Program at the Brigham and Women's Hospital. This registry includes livebirths, stillbirths, and elective abortions. Prevalence among nontransfers (i.e., patients whose mothers had planned delivery at this hospital) was calculated as approximately 1 per 10,000 total births (20 of 201,084). We analyzed a total of 58 patients consisting of 20 (34%) nontransfers and 38 (66%) transfers. Patients were categorized by spleen status as having asplenia (7 nontransfers, 25 total), polysplenia (8, 20), right spleen (4, 11), normal left (0, 1), and unknown (1, 0). Among the 20 nontransfer and 59 total heterotaxy patients, the following associated medical conditions were present: chromosome abnormality (1 nontransfer, 2 total), suspected Mendelian or chromosome microdeletion disorder (1 nontransfer, 6 total), and maternal insulin-dependent diabetes mellitus (1 nontransfer, 2 total). There were 6 twins (1 member each from 6 twin pairs including 1 dizygous, 4 monozygous, 1 conjoined; 2 were nontransfers). An associated condition occurred in 5 (25%) nontransfer and 16 (28%) total patients, or among 10 of 53 singleton births (19%). Although most cases of heterotaxy in this series were sporadic events, an associated condition was present in about one-fourth of the cases. Not all of these conditions would be considered causative etiologies. Based on this small series alone, maternal insulin-dependent diabetes cannot be viewed as a risk factor for heterotaxy. However, the specific association of diabetes with polysplenia with/without left atrial isomerism is noteworthy, and adds weight to animal and epidemiologic case-control data.
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            Atrial isomerism in the heterotaxy syndromes with asplenia, or polysplenia, or normally formed spleen: an erroneous concept.

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              The diverse cardiac morphology seen in hearts with isomerism of the atrial appendages with reference to the disposition of the specialised conduction system.

              Congenital cardiac malformations which include isomerism of the atrial appendages are amongst the most challenging of problems for diagnosis and also for medical and surgical management. The nomenclature for pathological description is controversial, but difficulties can be overcome by the use of a segmental approach. Such an approach sets out the morphology and the topology of the chambers of the heart, together with the types and modes of the atrioventricular, ventriculo-arterial, and venous connections. We have applied this method to a study of 35 hearts known to have isomerism of the atrial appendages. We have already published accounts of 27 of these cases, but these were reviewed for this study in the light of our increased awareness of the implications of isomerism, and 8 new cases were added. After examining, or re-examining, the morphology of every heart in detail, we grouped them together according to their ventricular topology and modes of atrioventricular connection. Then we studied the course of the specialised conduction system, by the use of the light microscope, first in each individual case, and then together in their groups. We conclude that the pathways for atrioventricular conduction in hearts with isomerism of the atrial appendages are conditioned both by ventricular topology, and by the atrioventricular connections. Based on our experience, we have been able to establish guidelines that direct the clinician to the likely location of the conduction tissues.
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                Author and article information

                Contributors
                Journal
                HeartRhythm Case Rep
                HeartRhythm Case Rep
                HeartRhythm Case Reports
                Elsevier
                2214-0271
                09 January 2020
                April 2020
                09 January 2020
                : 6
                : 4
                : 210-214
                Affiliations
                []Harrington Heart and Vascular Institute, University Hospitals Cleveland Medical Center, Cleveland, Ohio
                []Division of Pediatric Cardiology, Rainbow Babies and Children’s Hospital, The Congenital Heart Collaborative, Cleveland, Ohio
                Author notes
                [] Address reprint requests and correspondence: Dr Jaime Hernandez-Ojeda, Harrington Heart and Vascular Institute, University Hospitals Cleveland Medical Center, 11100 Euclid Ave, Cleveland, OH 44106. jaheroj@ 123456gmail.com
                Article
                S2214-0271(19)30196-4
                10.1016/j.hrcr.2019.12.015
                7156985
                © 2020 Heart Rhythm Society. Published by Elsevier Inc.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                Categories
                Case Report

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