Catheterization and Cardiovascular Interventions 60:287-290 (2003) Transcatheter Closure of a Coronary Sinus Defect With an Amplatzer Septal Occluder ‘Stefano Di Bernardo, mo, Margrit Fasnacht, mo, and Felix Berger,” mo We report a successful transoatheter closure of a coronary sinus defect using an Am- blatzer septal occluder in a 9.5-year-old boy suffering from a significant volume overload ‘due to left-to-right shunt. Catheter Cardiovasc Intory 2003;60:287-200. 290 We is ne ‘Key words: coronary sinus defect heart septal defect; transcatheter closure; Amplatzer ‘optal occluder INTRODUCTION Coronary sinus (CS) defect is an unusual interatrial communication, almost always associated with a persis- tent left superior vena cava (LSVC) [1-3] and an en- Iarged coronary sinus. The hemodynamic consequence of this association is a certain degree of lefi-to-right shunt with volume overload of the right heart, ‘The management of this type of congenital anomaly is usually through surgical closure of the interattial com- ‘munication [4,5]. With the introduction of the Amplatzer septal occluder (ASO), the percutaneous transcatheter treatment of selected secundum atrial septal defects has become a standard procedure [6]. Based on the extended experience with the ASO within the last few years, other types of intracardiac defects seem also to be amenable to Percutaneous closure, We report the first successful per- cutaneous transcatheter closure of a coronary sinus de- fect with an Amplatzer septal occhider in a young boy. CASE REPORT A 9S-yearcold Caucasian boy (body weight, 31 kg: length, 145 cm) was referred to our department for ther- apy of a CS defect. The child presented with a discrete precordial bulge and a slightly hyperactive precordium. ‘The second heart sound was widely split and a 2/6 systolic murmur could be heard over the pulmonary area. Chest X-ray revealed a slightly enlarged heart and mod- erate incteased pulmonary vasculature. ECG showed an abnormal right atrial rhythm with a P-axis of -27° and right ventricular volume overload. Echocardiography demonstrated a moderate CS defect of 5 mm with sig- nificant left-to-right shunt located in the distal part of the’ roof of the CS (Fig, 1), Due to the volume overload, the end-diastolic diameter of the right ventricle in the four- chamber view was measured 39 mm, A small LSVC © 2008 Wiley-Liss, Ine drained through the CS and was also connected via an inominate vein to the superior vena cava. The dilatation of the CS was due to the volume overload of the left-to- right shunt. Because of its location at the dilated distal part of CS, the defect seemed to be suitable for a trans- catheter closure using an ASO, Right heart cardiac catheterization was performed un- der general anesthesia and under anticoagulation with 100 TU/kg of heparin. Right ventricular systolic pressure (44 mm Hg) and pulmonary artery systolic pressure (24 mm Hg) were measured. Calculation of Qp was 14, Angiography into the inominate vein showed small LSVC draining into the CS. The defect was located atthe distal part of the roof of the CS. ‘The defect could be crossed with a regular Berman balloon angiographic catheter passing through the coro- nary sinus from right atrium (RA). The balloon of the Berman angiographic catheter was then inflated in the left atrium (LA) with CO, and an angiography was performed in an LAO/RAO projection to visualize the defect (Fig. 2) For sizing of the defect, the balloon of the Berman angiographic catheter was inflated with a dye-saline so- Ition (1:5) under fluoroscopy and transesophageal echo- cardiographic (TEE) guidance. The balloon’s diameter, which could be pulled through the defect with a slight Department of Congenital Heart Dissase, University Children’s Hospital Zurich, Zurich, Switzeriand ‘Comespondence to: Dr. Felix Berges, Department of Congeaital Heart Disease, University Childen’s Hospital, Steinwiessrasse 15, ‘CH.8032 Zovich, Switeiand. E-mail: felix berger@kispvizh.eh Received 27 December 2002; Revision accepted 25 May 2003, Or 10.1002/e<.10619 Published oaline in Wiley InterScience (wwrintrciencewiley com),288 Di Bernardo ot al. tne 2, Echocardiography with color Doppler demonstrating the Coronary sinus defect (CSD) and the left-to-right shure (L-R-S). LV, lott ventricle; LVOT, left ventricular outfion tract, Fig, 2. Angiography (60° LAO) of the coronary sinus defect with @ balloon Berman catheter before device closure, tension, was measured with TEE (Fig. 3) and outside the body with the regular sizing tablet (defect diameter, 6 mm). A corresponding ASO was chosen and loaded into 2.6 Fr long introducer sheati. The tip of the sheath was located in the left rium passing from the right atrium to the CS through the coronary sinus defect. The loading and implantation procedure of an ASO has been de, scribed in details elsewhere and this procedure was fol. lowed [7,8]. A second catheter was inserted and located into the ESVC passing through the superior vena cava and the inominate vein to control the implantation procedure ‘with angiography (Fig. 4). The left side of the device was Fig. 8. Transesophageal echocardiography (54°) during bi ‘oon sizing ofthe coronary sinus defect. MV, mitral vahve, Fig. 4. Angiography (50° LAO) of the coronary sinus after dou ployment of the lt atrial disk; the right arial dick stil loaded in the long sheath attached to the implantation wire (W}, deployed in the left atrium and pulled back toward the oof of the CS. Then the connecting waist of the ASO Was deployed within the defect under guidance of TER, With withdrawal of the sheath, the right disk of the Gevice was deployed in the CS. Device position was confirmed with TEE and angiography into LSVC. No fesidual shunt or obstruction of the CS was observed, The device was then unscrewed by turing the delivery cable counterclockwise. Repeated angiography 10 mix alter releasing the device demonstrated the safe position of the ASO without obstruction of the CS (Fig. 5). ‘Twenty-four hours later, a color Doppler echocardiogray phy showed no residual shunt, laminar flow through theCoronary Sinus Defect Closure 289 Fig. 5. Angiography (60° LAO) of the coronary sinus after Im plantation of the Amplatzer septal occluder demonstrating its ‘unobstructed lumen. CS, and no interference of the left-sided disk of the ASO with left ventricular inflow (Fig. 6). DISCUSSION A coronary sinus defect results in a communication of variable size between the CS and the LA. In most cases, itis associated with a persistent LSVC and often part of a more complex cardiac malformation [1,2]. If the CS-to-LA fenestration is the single cardize defect, this anomaly is described as an unusual type of inter” atrial communication [3] with the same hemodynamic consequences. The diagnosis is usually made by echo- cardiography [9]. The therapy so far has been the domain of surgery [4.5]. Tn the last years, interventional closure of atrial septal defects with ASO has become a safe and effi- cient technique for most interatrial defects [6]. The easy, safe, and effective placement might be advanta- ‘geous especially in atypical localization with the ben ficial potential of removing and replacing the device as long as it is attached to the delivery cable. This made.the Amplatzer septal occluder the device of ‘choice in this case. ‘The enlarged CS in these types of defects made placement of the ASO easier and minimized the risk of ‘an obstruction of the CS. To avoid any obstruction with device placement within the lumen of the coro- nary sinus, the intervention should be monitored by TEE. An angiographic catheter placed within the LSVC passing through the inominate vein made se- peated angiography in the CS during the implantation Fig.6. Color Doppler echocardiography 24 hr ofter suocessful closure of the coronary sinus detect with the Amplatzor eoptal ‘occluder without any obstruction of the coronary sinus oF In- ‘erference with the left ventricular inflow. procedure very simple, without the risk of dislocation of the ASO, Interventional occlusion of CS defects might only be possible if the location of the defect is ‘lose to the mouth of the CS in the RA. In our opinion, CS obstruction is unlikely if the cross-section of the ‘configured RA disk of the ASO is measured less then third of the diameter of the CS, Most important seems to be the judgment of the flow in the CS before releasing the device; if it is turbulent and accelerated, removal of the device should be advised. So far, there are no experiences whether a device placed in a coronary sinus defect may cause supraven- ‘wicular arrhythmia. Tn our ease, no arrhythmia could be observed during or within the first 48 hr after the inter- ‘ventional procedure. Repeated 24-hr ECG reports may be necessary to screen for potential arrhythmia as a part of the long-term follow-up. To our knowledge, we desctibe the first percutaneous closure of a coronary sinus defect using the same proce- dure and the same device as for closure of secundum type defects. The excellent result without any residual shunt or obstruction of the coronary sinus demonstrates the feasibility and safety of the procedare with an Amplatzer septal occluder if the CS diameter offers space enough to configure the RA disk of the ASO to a flat disk well aligned to the roof of the CS. REFERENCES 1, Franz C, Mesnicken U, Dalichan H, Hirsch H, Abnormal comma ication between th left airium and the coronary sinus: present tion of 2 cases and review ofthe literature, Thoae Cardiovace Surg 1985;33:113-117200 Di Bernardo et al 2, Hager W, Neudeck F, Donbuijsen K, Bischoff KO, ltraial com> uniaton trough the coronary sins, Z Kardiol 1982:71:691-684, 3. 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