Device closure is recommended as a Class I and Level B indication for ostium secundum atrial septal defect (OS ASD) with appropriate anatomy without significant pulmonary arterial hypertension [1]. The anatomy of the atrial defect is the most important factor when deciding between surgery and transcatheter closure [2]. Closing an eccentric OS ASD defect eccentric appliance with a large appliance is difficult and requires technical modifications to avoid unnecessary complications [3-5]. When the ASD has misaligned, eccentric, or deficient rims, cabling of the left superior pulmonary vein, negotiation of the sheath and dilator, and release of the left atrial disc are difficult and technical modifications are required for successful deployment of the device [5].
This case was previously presented as an e-poster entitled “Device closure of an eccentric atrial septal defect using a large device” at CSI Frankfurt 2022 (June 22-25, 2022).
A 45-year-old woman with atrial fibrillation was admitted for transcatheter closure of an atrial septal defect. Transesophageal echocardiography (TEE) showed an ASD size of 30 mm in four-chamber view and 21 mm in bicaval view. The total length of the interatrial septum was 6 cm. The defect had a deficient retro-aortic rim; the rim of the superior vena cava (SVC) was 8 mm. Therefore, the defect was considered eccentric, with the location of the defect viewed in relation to the length of the interatrial septum (Figure 1A). The pulmonary veins drained into the left atrium without any turbulent flow as seen in TEE. The right ventricular systolic pressure was 41 mmHg. Coronary angiography was normal. Informed consent was taken and the heart team was consulted.
The defect was attempted to plug through the femoral access using a 34mm Lifetech ASD device (Lifetech Scientific Co., Shenzhen, China) using the dedicated 14 Fr sheath. procedure was performed under fluoroscopic and TEE control. We noticed the torturous course of the Terumo 0.035 inch x 260 cm guide wire combo (Terumo Corporation, Tokyo, Japan) and the right Judkins (JR) 5 Fr diagnostic catheter when attempting to wire the left superior pulmonary vein, which which was mainly due to the cranial position of the catheter more towards the junction of the SVC and the right atrium (SVC-RA) due to the eccentric defect, i.e. the interatrial septal length of 60 mm, the defect of 30 mm and the edge of the SVC of 8 mm (Figure 2A). The 14-Fr sheath and its dilator were advanced from the groin until the combo reached the level of the hepatic vein in the inferior vena cava. The dilator was replaced with a 5-Fr JR diagnostic catheter, and this combo was advanced into the left atrium over the Terumo guidewire in place of the extra stiff Amplatzer guidewire (Boston Scientific, Marlborough, MA ) to avoid injury. It was noticed in the TEE that the combination of the 14-Fr sheath and the diagnostic 5-Fr was not central to the fault at all. Therefore, the upper end of the delivery sheath was parked in the left atrium before loading the ASD device into the delivery sheath. Left atrial disc release was initiated in the left atrium rather than the left superior pulmonary vein (Figure 2B). Despite this precaution, we noticed a pericardial effusion before plugging the defect probably due to the lesion of the SVC-RA junction due to the more cranial position of the sheath and the partially freed left atrial disc near the SVC-RA junction. (Picture 1B). The device was properly seated on the ASD when it was pulled down over the defect (Figures 1 C, 1D, 2C). The pericardial effusion progressed to a depth of 10 mm within one hour after device implantation (Figure 1D). A pigtail aspiration was initiated (Figure 2D).
A follow-up contrast-enhanced computed tomography (CECT) scan performed the next morning revealed a 2.1 cm contained tear in the SVC-RA junction, and the left superior pulmonary vein was found to be tortuous with no apparent injury. A unit of fresh blood was donated. One gram of tranexamic acid was given intravenously followed by 500 mg every eight hours for the next two days. We had a second aspiration of 400ml after 36 hours and a third aspiration of 200ml after 48 hours. CECT showed significant left pleural effusion which was detected when the patient was assessed for room air desaturation on the second day of the procedure. Insertion of the intercostal drainage tube showed 400 ml of hemorrhagic effusion which subsided after an additional 48 hours (Figure 3). Right ventricular failure and elevated right ventricular pressure have been managed with diuretics, digoxin, and pulmonary vasodilators. She was successfully discharged on the sixth day after catheter closure. This patient has completed the six-month follow-up and is asymptomatic. Now she is taking metoprolol succinate, digoxin and rivaroxaban 10mg for atrial fibrillation.
Balloon sizing of the defect and left atrial appendage closure were not performed in this case due to financial constraints.
When an atrial septal defect is large, has peripheral insufficiency, is fenestrated, and there are multiple atrial septal defects in the same patient, the septal defect is called complex atrial septal defect which causes problems during transcatheter closure. Malzahn et al. device closure of large atrial anomalies in 275 consecutive patients [6]. The incidence of periprocedural complications was noted in 20.4% of cases and pericardial effusion was noted in 5.5% of cases. In an adult, an ASD larger than 25mm is called a large defect. [7]. The device is said to be large when the size of the device is greater than 30 mm; the rims are said to be adequate when they measure at least 5 mm. In our case, the retro-aortic labrum was deficient, which is acceptable for transcatheter closure in most studies. Some of the complications such as arrhythmia, pericardial effusion, device embolization, cardiac perforation, cardioembolic stroke and hemoglobinuria are immediate. A rapid diagnosis of these rare complications using 2D echo, TEE or CECT is necessary for appropriate management by a conservative or surgical approach, as observed in a study by Batta et al. [8]. Thanopoulos et al. in one study showed that ASDs with isolated rim deficiency can be safely closed without any significant complications if proper techniques are used [9]. Device closure of an ASD with complex anatomy is possible using several modified techniques [5]. Rigid sheath with dilator negotiation in such situations can cause cardiac perforation in cases of larger defects, such as in our case, which require close monitoring during the procedure using both TEE and fluoroscopy [10]. There may be cardiac perforation when negotiating the anchoring sheath of the device through the defect. Abnormal course of the guide wire when parking the extra-rigid guide wire in the left superior pulmonary vein can cause injury to the pulmonary vein which can manifest as pleural effusion as in our case [6]. However, close monitoring and appropriate conservative management can sometimes save the patient undergoing heart surgery. [11]. Although it is rare to encounter distant cardiac erosion despite successful closure of an atrial septal defect, regular follow-up of patients is essential as observed in a study by Zhang and Ding [12].
Device closure of an eccentric and large atrial septal defect can be difficult. Patients should be closely monitored during and after the procedure. Most of the time, a minor cardiac perforation can be managed conservatively or surgically without explantation of the device if the alignment of the device is correct.