Transjugular closure of a residual septal device defect: a case report.

Background: Severe residual shunts after percutaneous closure of atrial septal defects are unusual. These patients are usually considered candidates for surgery. Case summary: We describe the transjugular closure of a residual atrial septal defect with significant left-to-right shunt due to a malpositioned large atrial septal device in a symptomatic 74-year-old female. Transjugular access was chosen first due to the unfavourable position of the device for delivery of a new one from the femoral approach. An overlapping Figulla® Flex II 27/30 mm PFO device was successfully implanted with the guidance of 3D-transoesophageal echocardiography. Discussion: This case demonstrates the safety and feasibility of transjugular access as an alternative to femoral or transhepatic approaches in patients with difficult atrial septal anatomies, who are usually referred for surgery.

To demonstrate the feasibility of the transjugular access as an alternative to femoral or transhepatic approaches in patients with complex atrial septal anatomy who are usually referred for surgery.

To emphasize the value of real-time 3D-transoesophageal echo for planning and guiding these complex interventions. It can increase success rates and decrease potential complications, thus avoiding the need for open-heart surgery.

Introduction

Percutaneous closure is the established treatment for ostium secundum atrial septal defect (ASD). However, some malaligned, post-surgical, or unusually located defects pose a challenge to the interventionist. Such defects may not be amenable to closure from the femoral venous route and are often referred for surgical closure. Transhepatic and transjugular approaches have been described to be useful in patients in whom femoral venous approach is failed or not feasible, mainly due to a congenital or acquired inferior vena cava occlusion.[1] We describe the transjugular closure of a malaligned post-device residual ASD in a symptomatic patient with patent femoral venous access.

Case presentation

A 74 year-old female, with a history of percutaneous closure of an ASD years ago and permanent atrial fibrillation, was admitted for evaluation due to dyspnoea with mild efforts and signs of right heart failure (peripheral oedema, abdominal distension) despite treatment with diuretics (furosemide, spironolactone) and low-dose beta-blockers (for rate control). She was also receiving enalapril and dabigatran.

The patient did not have a clinical report of the intervention, and follow-up revisions afterwards had been irregular. Clinical status had deteriorated progressively in the last months, and at the time of the visit she was in NYHA Class III.

The electrocardiogram showed atrial fibrillation with controlled ventricular response. A TTE confirmed a normal left ventricular function, moderate right chambers dilatation (right ventricular end-diastolic volume 116 mL/m2), the presence of a septal occluder device in the interatrial septum (IAS), and a significant left-to-right shunt in the anterior part of the device (estimated Qp:Qs 2.2:1), which showed a slight misalignment (). A moderate tricuspid regurgitation was detected, and the estimated systolic pulmonary pressure was 60 mmHg.

Transthoracic echocardiogram apical four-chamber view (left) and short-axis view (right) showing right chambers dilatation, a moderate tricuspid regurgitation and a significant left-to-right shunt in the anterior part of the interatrial septum. IAS, interatrial septum; TTE, transthoracic echocardiogram; LA, left atrium; RA, right atrium; LV, left ventricle; RV, right ventricle.

A TEE was performed to better examine the IAS and the device position, and to identify the cause of the shunt. A clear misalignment of the device was observed, with a slight protrusion towards the right atrium in the anterosuperior part (, see Supplementary material online, ). A significant left-to-right shunt was evident, but the rest of the device was correctly anchored in the septum. 3D-TEE showed a ‘watermelon slice’ morphology of the defect between the device and the septum ().

Transthoracic echocardiogram shows the misalignment of the device, which protrudes towards the right atrium in the anterosuperior part, and the left-to-right shunt. Ao, aorta; LA, left atrium; RA, right atrium; RV, right ventricle; TEE, transoesophageal echocardiogram.

Three-dimensional transthoracic echocardiogram ‘watermelon slice’ image of the residual defect.

The case was discussed in the Heart Team meeting. The patient was considered high-risk for surgery (age, pulmonary hypertension, and chronic heart failure), so a percutaneous procedure was planned. The strategy was to implant a second device between the remanent IAS and the device. Preprocedure planning, based on imaging techniques (TEE and CT), suggested that going through the defect with a wire from a femoral access would pose an extreme angle to later cross with a delivery sheath, due to the protrusion of the device into the superior part of the right atrium. Thus a good support for a controlled deployment of the new device would be difficult to achieve. So the plan was to try from the transjugular approach first, and have the femoral access prepared as an alternative in case of failure, and use deflectable sheaths and high-support wires if needed.

Under general anaesthesia, the internal right jugular access was punctured and an 8F sheath was inserted. Pulmonary pressures and cardiac output were recorded and measured (54/21 mmHg, mean 34 mmHg, cardiac output by Fick method 4.2 L/min, Qp/Qs 1.9:1) and no evidence of elevated pulmonary resistances that could contraindicate the procedure was found. Then a 260 cm long 0.035″ hydrophilic wire was advanced through the defect with the help of a 5F multipurpose catheter and positioned in the inferior left pulmonary vein, with 3D-TEE guidance. The multipurpose catheter was exchanged for a 9F sheath and a 27/30 mm Figulla® Flex II PFO device was mounted in the delivery system and advanced into the left atrium (). Careful and controlled deployment of the device was performed until a stable and adequate overlapping position was achieved (see Supplementary material online, ). TEE showed a good position of the new device, without any interference with other intracardiac structures, and a very significant reduction of the shunt (see Supplementary material online, ). After stability manoeuvres were performed (Wiggle test), the device was released. A negligible residual flow was observed with TEE. Successful haemostasis was achieved by manual compression after retrieval of the sheath.

Three-dimensional transthoracic echocardiogram device implant. Sheath through the defect (transjugular access) and sequential left disc deployment on the prior device and the remanent interatrial septum.

The patient was discharged two days after the procedure. Three months later she was in NYHA Class I, and diuretics had been reduced. A follow-up TTE showed a marked reduction of right chambers size, a mild tricuspid regurgitation and an estimated systolic pulmonary pressure of 45 mmHg. There was a very small residual shunt between both devices, no pericardial effusion and no device impingement on the aorta. A cardiac CT confirmed a correct position of both devices, which remained unchanged from the obtained at the end of the procedure ().

Final result. Fluoroscopy image of the two devices and the overlapping position (left) and computed tomography image 3 months after the procedure (right). RA, right atrium.

Discussion

Percutaneous closure of ASDs (native and residual) is considered the treatment of choice when technically suitable, as the 2020 ESC Guidelines for the management of adult congenital heart disease endorse.[2] However, some complex ASDs (postsurgical, malaligned, or in unusual locations) have been classically referred for surgery because it was thought that such defects were not amenable for percutaneous closure. There are several reports of successful device closure of postsurgical residual defects as a reliable alternative to redo surgery.[3] Interestingly, there are very few reports of percutaneous closure of severe residual defects after device implant,[4] and most of them are described in PFO anatomies, but not with ASD devices.

Beyond the technical problems that can arise during the anchoring of a second device on a prior intervention, there are some other issues with the femoral access that can challenge a successful result. Some patients may have an interrupted inferior vena cava or a chronic thrombosis that preclude the use of that conduit. In such cases the alternative access routes are the transhepatic route (mainly used in children) or the transjugular access, which has been described in selected cases with good results.[1,5] Although its use is really unusual, the transjugular route allows a different deployment angle of the device, with a very low rate of vascular complications. On the other hand, previous case reports of closure through this route mention the difficulty in maintaining a stable pulmonary venous wire position.[1] Another important point is to perform a careful unsheathing of the device in the left atrium, under fluoroscopy and TEE guidance, in order to prevent any mitral valve dysfunction.

In this particular case, the angle to access the left atrium from the inferior vena cava was almost 180°. Manoeuvres to access with a wire and then with a long sheath into the left atrium from the femoral route would probably have required specific material (deflectable sheaths, high-support wires) and techniques that can increase the risk of complications and the total time of the procedure. Transjugular access can avoid much of that angulation. It helped to perform the procedure in a very similar way as a standard one, and to achieve a final good result.

Finally, the concomitant use of real-time 3D-TEE should be routine in these complex structural cases. It facilitates the evaluation of the morphology of the defect, and thus provides clear information about the spatial relationship with the previous device.[6] It is also a very useful tool for guiding device deployment and positioning, to confirm a good result and to rule out potential complications (device malposition or embolization, interference with intracardiac structures).

Conclusion

Percutaneous closure of residual shunts of IAS devices can be safely performed with a careful planification, which is key to obtain a good result. Transjugular access can be a feasible alternative to femoral or transhepatic approaches in patients with complex atrial septal anatomy who are usually referred for open-heart surgery. Advanced imaging tools (3D-TEE, cardiac CT) are extremely helpful for planning and guiding these complex interventions. Their use could increase success rates and decrease potential complications.

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	Sequence of events
Background	Percutaneous closure of ASD several years before
Presentation	Dyspnoea with mild efforts (NYHA Class III) and right heart failure
Diagnostic tests	Transthoracic echocardiogram (TTE): significant left-to-right shunt and device misalignmenttransoesophageal echo (TEE): anterosuperior device protrusion into the right atrium, rest of the device correctly anchored
Decision	Percutaneous closure with a new device through a transjugular access
Procedure	A Figulla Flex II PFO 27/30 mm device was implanted partially overlapped with the prior one
Imaging	3D-TEE guidance confirms correct position and a trace residual shunt
Follow up	Symptoms resolution (NYHA Class I) and stable device position