Percutaneous repair of systemic atrioventricular valve regurgitation with the MitraClip-NT system in congenitally corrected transposition of great arteries

Abstract

In a patient with congenitally corrected transposition of the great arteries, dilatation of the atrioventricular valve annulus related to worsening of systemic ventricular function, which worsened systemic atrioventricular valve (SAVV) functional regurgitation. In this article, we report a case of successful transcatheter treatment with MitraClip (Abbott Vascular, Santa Clara, CA, USA) in a 68-year-old female patient with congenitally corrected transposition of the great arteries and severe SAVV failure using imaging modalities. The patient had been hospitalized four times within 8 months, receiving optimal medical therapy for severe SAVV regurgitation and systemic ventricular failure. In this patient, the risk associated with surgery was considered extremely high owing to severe heart failure and liver cirrhosis. We positioned two clips appropriately, side by side, in between the anterior and septal leaflets, guided by computed tomography and three-dimensional echocardiography. The procedure resulted in optimal post-procedural reduction of regurgitation without stenosis, with a good clinical outcome noted at 2-year follow-up.

Learning objective

Since surgery for adult congenital heart disease is linked to high risk, we seek less invasive treatment for such patients. In patients with congenital heart disease and severe valve regurgitation, the use of MitraClip (Abbott Vascular, Santa Clara, CA, USA) could be a feasible option for select cases.

Introduction

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital anomaly. Previously, dilatation of the atrioventricular valve annulus was observed in a patient with CCTGA related to worsening of systemic ventricular function, which worsened systemic atrioventricular valve (SAVV) functional regurgitation [1], [2]. Transcatheter mitral valve repair using the MitraClip™ (Abbott Vascular, Santa Clara, CA, USA) has emerged as a safe and effective treatment option for selected patients with significant mitral regurgitation (MR) for whom surgery is associated with a high risk of complications [3]. Surgery for adult congenital heart disease is often linked to high risk; nevertheless, several case reports have shown the effectiveness of transcatheter treatment for SAVV dysfunction in patients with CCTGA [4]. Although the use of a multiple clips procedure for the treatment of MR is becoming more common, there are no previous cases reports showing the usefulness of this approach. In this article, we present a case of successful transcatheter treatment (using two MitraClip-NT devices) in a CCTGA patient with severe SAVV failure, guided by appropriate imaging modalities.

Case report

A 68-year-old female with CCTGA and progressive dyspnea visited our cardiology department. The patient had not experienced symptoms until the age of 66 years. Initially, cardiomegaly was detected at an annual medical check-up at the age of 60 years. Subsequently, transthoracic echocardiography (TTE) revealed CCTGA. Although we initiated medical treatment after the symptoms became obvious, the patient's heart failure worsened. Consequently, she was hospitalized four times within 8 months, receiving optimal medical therapy with an angiotensin-converting enzyme inhibitor, beta blockers, and diuretics including tolvaptan. Since TTE showed severe systemic and pulmonic atrioventricular valve regurgitation (Fig. 1a; Video 1), we considered performing surgery. At the time, functional left ventricle (LV) end diastolic volume was 110 ml and ejection fraction was 38 % by TTE. Also, pre-procedural invasive hemodynamic evaluation showed the following, systolic and mean pulmonary artery pressure were 51 and 39 mmHg, mean and v-wave of pulmonary capillary wedge pressure were 27 and 39 mmHg, mean right atrial pressure was 11 mmHg, and cardiac output was 1.51 l/min/m². As a pre-procedural assessment, we performed contrast-enhanced computed tomography (CT) and transesophageal echocardiography (TEE). The latter imaging modality showed tri-leaflet SAVV without leaflet coaptations and a regurgitation jet from the center of the valve (Fig. 1b–d). CT analysis did not reveal other combined malformations. After several examinations, the cardiology team determined that surgery in this patient was associated with an extremely high risk for complications owing to severe heart failure, pulmonary hypertension (estimated pulmonary artery pressure: 74 mmHg), and liver dysfunction (Child–Pugh class B). Assessment of key heart structure positions demonstrated that the SAVV was a posterior structure of the heart, and that the relationships between the inferior vena cava, atrial septum, and SAVV appeared sufficiently durable. Hence, application of the MitraClip procedure was considered feasible in this case (Fig. 1e and f; Video 2). In the preprocedural evaluation, the anterior and septal leaflets were larger than posterior leaflet, similar to the normal tricuspid valve leaflets. We planned to grasp the anterior and septal leaflets, since a previous tricuspid clip report showed anteroseptal commissure is the most dominant implantation site, leading to a favorable reduction in regurgitation [5].

Fig. 1.

Pre-procedural assessment. (a) Baseline TTE apical four-chamber view; severe SAVV regurgitation was observed between the AL (orange arrow) and SL (blue arrow). (b) Baseline TEE X-plane color image; severe central jet. (c) Baseline TEE 3D image; tri-leaflet valve gaps; orange, blue, and green arrows denote large AL, SL, and small PL, respectively. (d) Baseline TEE 3D color image; severe SAVV regurgitation jet. (e) Pre-procedural CT images; atrial septum and atrioventricular valve relation were normal. (f) Pre-procedural CT images; Ao and PA positions were switched.

3D, three-dimensional; AL, anterior leaflet; Ao, aorta; CT, computed tomography; LA, left atrium; LAA, left atrial appendage; PA, pulmonary artery; PL, posterior leaflet; RA, right atrium; SAVV, systemic atrioventricular valve; SL, septal leaflet; TEE, transesophageal echocardiography; TTE, transthoracic echocardiography.

We prepared the patient in a normal manner and accessed the right femoral vein. The procedure was performed under general anesthesia, using TEE and a fluoroscopy guide. Following preprocedural evaluation we tried to grasp the anterior and septal leaflets to reduce regurgitation. Normally, the aortic valve and LV outflow tract are key structures for performing the procedure. Since those were not adjacent to the SAVV, we initiated the procedure with a 90-degree and X-plane zero-degree TEE view for transseptal puncture. We selected a mid-mid position in the fossa. The septal puncture height from the SAVV annulus was 41 mm. Using an M-knob and steerable guide catheter rotation, the first clip was placed on the SAVV. Using a three-dimensional (3D) live TEE image, we clearly observed the leaflet coaptation line (Fig. 2a and b; Video 3). We considered 60 degrees as a “grasp view” and 150 degrees as a “commissure-like view” to guide the procedure. To minimize space between the anterior and septal leaflets, the first clip was positioned close to the antero-septal commissure of the leaflets (Fig. 2c). After confirming the reduction of regurgitation and stability of the clip, we released the first clip. Subsequently, the TEE revealed residual moderate regurgitation, we positioned the second clip immediately adjacent to the first. After deployment of the second clip, regurgitation became trivial (Fig. 2d). The final TEE and invasive hemodynamics confirmed a significant reduction in SAVV regurgitation, with a trans-SAVV gradient of only 2 mmHg, reduction in left atrial V-wave from 23 to 16 mmHg, and increase in cardiac index from 1.7 to 2.6 l/min/m² (Fig. 2e; Video 4).

Fig. 2.

(a) Pre-procedural TEE live 3D color image; image cut leaflets (orange and blue arrows denote AL and SL, respectively) and jet. (b) TEE live 3D image of the first clip; the image clearly confirmed the presence of leaflets on both arms. (c) TEE X-plane image after the first clip grasp; red arrow denotes residual SAVV jet in the center of the valve. (d) TEE X-plane image after the second clip grasp; trivial SAVV jet, the second clip was positioned immediately adjacent to the first clip. (e) TEE 3D image after the procedure; tissue bridge between the anterior and septal leaflets (arrow). (f) TTE image at 2-year follow-up apical four-chamber view; mild SAVV regurgitation.

3D, three-dimensional; AL: anterior leaflet; SAVV, systemic atrioventricular valve; SL, septal leaflet; TEE, transesophageal echocardiography; TTE, transthoracic echocardiography.

Following the procedure, cardiac rehabilitation was performed and the symptoms improved. There were no procedure-related complications. After 1 month, functional LV volume, New York Heart Association class, and brain natriuretic peptide levels were significantly improved, from 110 to 85 ml, class 4 to 2 s, and 498 to 147 pg/dl, respectively. At 2-year follow-up, the patient had not been hospitalized. SAVV regurgitation was mild (Fig. 2f, Video 5); pulmonic AVV regurgitation was trivial; and estimated pulmonary artery pressure was 21 mmHg.

Discussion

To the best of our knowledge, this is the first report describing the use of two clips in a patient with CCTGA. The management of SAVV failure in patients with CCTGA often poses a challenge to treating physicians [2]. Although surgical treatment is an option [6], [7], transcatheter treatment is favored for patients in whom surgery is associated with a high risk of complications. In transcatheter treatment, the greatest obstacle for physicians is anatomical anomaly in patients with congenital heart disease. In the treatment of those patients, precise assessment of the clip positioning is warranted. In the present case, two key points led to successful outcome. Firstly, pre- and peri-procedural assessments using multiple imaging modalities (e.g. enhanced CT and a 3D TEE image) are important in patients with difficult anatomy. Secondly, the use of two clips to minimize residual SAVV regurgitation results in better long-term outcome.

Previous reports showed the feasibility of the MitraClip procedure in a patient with CCTGA and severe SAVV regurgitation [4], [8]. The etiology of SAVV regurgitation in patients with CCTGA is leaflet tethering due to systemic ventricle dilatation and SAVV annular dilatation. In patients with functional MR, transcatheter mitral repair using the MitraClip system is an ideal treatment for symptomatic heart failure under optimal medical therapy [9]. According to those data, the MitraClip system may be a favorable treatment option for functional severe SAVV regurgitation. Previous evidence has shown that transcatheter treatment of tricuspid regurgitation with the MitraClip system is a safe and feasible option [5]. The report showed that tricuspid valve leaflets were technically graspable, and reduction of regurgitation by one grade could improve symptoms. For accurate grasping, pre-procedural assessment of the heart structure (especially the device route and leaflets) is important. At the time of procedure simulation, 3D CT and 3D TEE were useful in analyzing the position of leaflets. Moreover, residual MR following the MitraClip procedure is a risk factor for poor clinical outcomes [10]. To obtain better clinical outcomes, a strategy involving multiple clips is becoming increasingly popular. In our procedure, we placed two clips in appropriate positions, side by side, in between the anterior and septal leaflets, and guided by 3D echocardiography. Since the positions of key structures differ in each patient with congenital heart disease, pre-procedural simulation using CT and 3D echocardiography guidance during the procedure is critical. The procedure resulted in optimal post-procedural reduction of regurgitation without stenosis, with a good outcome observed at 2-year follow-up.

Conclusion

Appropriate imaging allowed us to treat SAVV regurgitation with the MitraClip-NT system in a patient with CCTGA. The multiple MitraClip-NT approach may be a feasible option for the treatment of valve defects in select patients with congenital heart disease for whom surgery is associated with high risk of complications.

The following are the supplementary data related to this article.

Video 1

Baseline TTE image at apical four-chamber view; severe SAVV regurgitation. SAVV, systemic atrioventricular valve; TTE, transthoracic echocardiography.

Video 2

Baseline enhanced CT image. CT, computed tomography.

Video 3

Pre-procedural TEE live 3D color image; relationship between leaflets and jet. 3D, three-dimensional; TEE, transesophageal echocardiography.

Video 4

TEE 3D image after deployment of both clips; tissue bridge between the anterior and septal leaflets. 3D, three-dimensional; TEE, transesophageal echocardiography.

Video 5

TTE image at 2-year follow-up; mild SAVV regurgitation. SAVV, systemic atrioventricular valve; TTE, transthoracic echocardiography.

Funding

None.

Declaration of competing interest

Yoshihiro Morino received lecture fees from Abbott Medical. The other authors do not have any conflict of interest to declare.