Atresia Of Coronary Sinus Research Articles

Abstract 12999: Persistent Left Superior Vena Cava Draining Directly Into Left Atrium With Occluded Coronary Sinus: A Rare Incidental Finding

Case presentation: A 43-year-old lady with pacing induced cardiomyopathy presented for pacemaker (PM) revision to biventricular PM. Medical history included complete heart block with dual-chamber PM, supraventricular tachycardia, and ventricular septal defect repair. During the procedure, numerous unsuccessful attempts were made to access the right atrium (RA) using a deflectable catheter through the left subclavian vein. Left upper extremity venography exhibited a persistent left superior vena cava (PLSVC) suspected to be entering RA through the coronary sinus (CS). Navigation of the CS was unsuccessful. As a result, the procedure was aborted, and the patient was admitted for further cardiac imaging evaluation. Transesophageal echocardiography demonstrated an abnormal blood vessel between left atrial appendage and left upper pulmonary vein. Findings confirmed with agitated saline injection into left arm. This rang a concern for unroofed coronary sinus. Surprisingly, cardiac computed tomography angiograms and left upper extremity venogram images revealed a PLSVC draining into left atrium (LA) posteriorly. Normal anatomy of the right-sided superior vena cava (RSVC). Also, a CS atresia and coronary venous drainage to LA inferiorly. Discussion: PLSVC is a rare cardiac anomaly with estimated prevalence of 0.2% - 3% among general population. Commonly PLSVC drains into RA through expanded CS and rarely into LA through unroofed CS. Uniquely like our patient, the PLSVC drains directly into the LA with atretic CS and no interatrial communication. PLSVC is usually asymptomatic and detected incidentally during invasive cardiac procedures. Despite existence of right to left shunt, patient was maintaining normal oxygen saturation even after a treadmill stress test. Crowdedness of the RSVC with previously implanted leads is likely the reason for failed passage of the catheter to RA. Cardiac arrhythmias and paradoxical embolization were reported with PLSVC.

Challenging Implants Require Tools and Techniques Not Tips and Tricks.

The EP Clinics article "How to implant CRT devices in a busy clinical practice" describes the basics of the "interventional telescoping technique". This article focuses on specific circumstances where the tools and techniques are invaluable: (1) inability to locate the coronary sinus (CS), (2) inability to advance a catheter into the CS, (3) patients with CS atresia, (4) unstable CS access, (4) angulated target veins, (5) small and/or tortuous target veins, (6) target veins into which a wire cannot be advanced, (7) target veins with a drain pipe takeoff, (8) target veins close to the CS ostium.

Incidence and appearances of coronary sinus anomalies in adults on cardiac CT.

The aim of this study was to analyze the frequency and appearances of coronary sinus (CS) anomalies on cardiac computed tomography (CT) of adult patients and to compare them with transthoracic echocardiography (TTE) findings. We retrospectively evaluated cardiac CT images for the presence of CS anomalies in 6936 adult patients who underwent imaging from April 1 2008 to March 31 2015 at our institution. We also reviewed and compared with TTE findings for the cases of CS anomalies. CS anomalies were diagnosed in 23 of the 6936 (0.33%) and included persistence of the left superior vena cava (PLSVC) in 19 cases, unroofed CS (UCS) in two, coronary artery-CS fistula in two, and CS atresia in one. TTE revealed CS dilatation in only five of the 16 cases of PLSVC and suggested CS anomaly in the two cases of coronary artery-CS fistula. The other cases of CS anomaly were detected incidentally on CT. The incidence of CS anomalies was 0.33%. Precise diagnosis of CS anomalies with TTE and the original transverse images on cardiac CT alone was difficult for some conditions. We should be alert for the presence of CS anomalies which can cause clinical or procedural complications.

Raghib Syndrome Presenting as a Cryptogenic Stroke: Role of Cardiac MRI in Accurate Diagnosis.

Raghib Syndrome is a rare developmental complex, which consists of persistence of the left superior vena cava (PLSVC) along with coronary sinus ostial atresia and atrial septal defect. This Raghib complex anomaly has also been associated with other congenital malformations including ventricular septal defects, enlargement of the tricuspid annulus, and pulmonary stenosis. Our case demonstrates an isolated PLSVC draining into the left atrium along with coronary sinus atresia in a young patient presenting with cryptogenic stroke without the atrial septal defect. Majority of the cases reported in the literature were found to have the lesion during the postmortem evaluation or were characterized at angiography and/or echocardiography. We stress the importance of modern day imaging like the computed tomography (CT) angiography and cardiac MRI in diagnosis and surgical management of such rare lesions leading to cryptogenic strokes.

Successful left-sided accessory pathway ablation without reference catheter in patient with atresia of coronary sinus and thin persistent left superior vena cava

We report a case of atrioventricular reentrant tachycardia (AVRT) with ostial atresia of the coronary sinus (CS). Without the anatomic angiography, radiofrequency (RF) energy was applied at the mitral valve annulus and the bypass tract was eliminated. After the therapy procedure, by CS angiography, we knew the persistent left superior vena cava (PLSVC), and the coronary sinus was connected with vena cava superior, very thin in a diameter. The therapy procedure was successful. The patient has remained completely symptom free.

Unroofed coronary sinus or coronary sinus on the roof? Successful implantation of biventricular implantable defibrillator in anomalous coronary venous drainage

w b p t l c t s t Unfavorable coronary sinus (CS) anatomy remains an impediment to placing the left ventricular (LV) lead for cardiac resynchronization. We successfully implanted a biventricular defibrillator in a patient with unique anomalous coronary venous drainage, not described to date. A 51-year-old man with dilated nonischemic cardiomyopathy, left bundle branch block (QRS duration 160 ms), LV dysfunction (ejection fraction 20%), and chronic systolic heart failure (NYHA Class III) underwent biventricular defibrillator implantation. After transvenous CS cannulation, angiography performed in the 30° left anterior oblique (LAO) view suggested atresia of CS in the posteroinferior portion of the right atrium (RA) with a large anomalous venous connection filling retrogradely and draining into the RA roof just posterior to and leftward of the superior vena cava/RA junction (Figure 1A). Selective cannulation of this anomalous ostium in the RA roof was achieved with a 6F steerable decapolar catheter (Biosense Webster Inc., Diamond Bar, CA, USA). Angiography (28° LAO projection) performed through the guiding sheath at this site revealed a venous tributary draining the posterolateral LV wall. An LV lead (Medtronic Inc., Minneapolis, MN, USA) was successfully placed in the targeted posterolateral venous branch (Figure 1B, arrows) with good pacing parameters and withut any evidence of phrenic nerve stimulation. The course f the lead is shown on chest X-ray film (Figure 1C, arows). Axial slice from three-dimensional (3D) spiral comuted tomography (Figure 1D) showed the course of the LV

Radiofrequency Catheter Ablation of Posterior Paraseptal Accessory Pathway with Atresia of the Coronary Sinus Ostium

Atresia of coronary sinus (CS) ostium is a rare abnormality and only a few case reports in association with electrophysiological studies. These patients have been reported to have a variety of several arrhythmogenic sources. A 42-year-old man was referred to our hospital for an electrophysiologic study because of recurrent episodes of palpitation. A 6-French decapolar catheter was introduced from the right jugular vein and advanced to the near the CS ostium. But, we could not be advance the catheter into the CS despite several attempts. On coronary angiogram, an anomalous atresia of the CS ostium was discovered. Regular tachycardia was easily induced by an atrial stimulus, we suggested the patient had an atrioventricular reentrant tachycardia. During the tachycardia, the earliest retrograde atrial activation was recorded in the 5–6 o’clock region of the mitral annulus. The ablation catheter was inserted from the right femoral artery to the accessory pathway (AP) of posterior paraseptal area. Radiofrequency energy was delivered to this site, resulting in elimination of the AP. After this application, there was persistent ventriculo-atrial dissociation and led to successful ablation of the AP. We report a rare case of the ablation procedure of posterior paraseptal AP with atresia of the CS ostium.

Successful Ablation of Left-Sided Accessory Pathways in a Patient with Coronary Sinus Atresia Using 3-Dimensional Mapping System

A 62-year-old man with recurrent paroxysmal supraventricular tachycardia was referred to our hospital for ablation. A 12-lead electrocardiogram revealed sinus rhythm with no delta waves. A 12-lead electrocardiogram during the palpitations recorded a regular, narrow QRS tachycardia at the rate of 186/min. A coronary angiography was performed after the coronary sinus could not be cannulated. On levophase coronary angiography, there was dual venous drainage into the right and left atria and no coronary sinus ostium. A regular, narrow QRS tachycardia with a cycle length of 300 ms was induced by atrial extrastimulus pacing. A transseptal catheterization was performed using the standard Brockenbrough technique. Mapping of the mitral annulus was performed during ventricular pacing, using a 7-French deflectable ablation catheter and the 3-dimensional electroanatomical mapping system. The earliest retrograde atrial activation was recorded in the posterolateral region of the mitral annulus. Though the ventriculoatrial conduction time was prolonged after radiofrequency energy was delivered to this site, the ventriculoatrial conduction after transvenous adenosine triphosphate administration was maintained. After re-mapped, the earliest retrograde atrial activation site was shifted to the left anterior region radiofrequency energy eliminated this accessory pathway. Thirty minutes after effective radiofrequency discharge, there was persistent ventriculoatrial dissociation. Six months after the procedure, the patient has been free from any symptoms.

Coronary sinus atresia and persistent left superior vena cava with the presence of thrombus complicating implantation of a left ventricular pacing lead

A 68-year-old male with heart failure and a suitable candidate for resynchronization therapy was referred to our hospital because of a failed coronary sinus (CS) lead implant. Catheterization of the CS initially also failed in our department but a left coronary angiogram revealed atresia of the CS and drainage of the coronary venous system via a persistent left superior vena cava (PLSVC). Implantation of a CS lead through the PLSVC could be accomplished after a selective angiogram, even in spite of the presence of a large thrombus at the junction of PLSVC and CS.

Inability to Cannulate the Coronary Sinus in Patients with Supraventricular Arrhythmias: Congenital and Acquired Coronary Sinus Atresia

We report 4 cases of congenital and acquired coronary sinus ostial atresia incidentally found during electrophysiologic assessment for supraventricular arrhythmias. Congenital variants consisted of coronary sinus drainage predominantly via persistent left superior vena cavae and partial coronary sinus unroofing into the left atrium. The acquired variant was inadvertently produced during surgery for cor triatriatum. A variety of electrophysiologic substrates including right and left-sided accessory pathways and both typical and atypical AV nodal reciprocating tachycardia were identified. Approaches to imaging and accessing the coronary sinus when the os cannot be cannulated are discussed, including a search for right atrial accessory venous collaterals, venography to rule-out a persistent left superior vena cava, and coronary angiography.

Cardinal vein isomerism: An embryological hypothesis to explain a persistent left superior vena cava draining into the roof of the left atrium in the absence of coronary sinus and atrial septal defect

Background: A persistent left superior vena cava (PLSVC) is a relatively frequent systemic venous anomaly associated with congenital heart defects. This anomaly has been explained with the persistence of the left superior cardinal vein. PLSVC usually drains into the right atrium, via coronary sinus, but it joins the left atrium in approximately 8% of the cases either directly in the setting of atrial isomerism, or via an unroofed coronary sinus, or through a coronary sinus type atrial septal defect. Case report: We describe a case of an adult patient with atria in the situs solitus, PLSVC draining into the left atrium, atresia of coronary sinus without atrial septal defect, and with additional cardiac anomalies (ventricular septal defect and discrete subaortic stenosis). Conclusion: A possible embryological explanation to this case rises from a right partial isomerism of the superior cardinal veins, which gives reason for both the coexistence of the PLSVC draining into the left atrium and the absence of coronary sinus, atrial septal defect, or coronary sinus ostium.