First Reported Native Transcatheter Tricuspid Valve Replacement in Congenitally Corrected Transposition of the Great Arteries

Abstract

Background

Congenitally corrected transposition of the great arteries (CC-TGA) can lead to severe systemic tricuspid regurgitation (TR). There is limited evidence on transcatheter tricuspid valve replacement (TTVR) in these patients.

Case Summary

A 59-year-old woman with CC-TGA presented with symptomatic severe systemic TR. She was at high risk for surgery, and transcatheter interventions were considered by our heart team. TTVR on the systemic tricuspid valve was performed using a Sapien M3 valve (Edwards Lifesciences), and the systemic TR was eliminated. There were no complications, with significant symptomatic improvement at the 30-day follow-up.

Discussion

Transcatheter therapies in high–surgical risk adult CC-TGA patients are being increasingly investigated. This case shows that TTVR using a Sapien M3 valve is feasible in CC-TGA patients with symptomatic severe systemic TR.

Take-Home Message

TTVR with the Sapien M3 valve is a feasible and safe alternative in high–surgical risk CC-TGA patients with symptomatic systemic TR.

Visual Summary

History of Present Illness

A 59-year-old woman with a history of congenitally corrected transposition of the great arteries (CC-TGA) was referred for severe symptomatic systemic tricuspid regurgitation (TR). For 1 year, the patient experienced worsening fatigue, dyspnea and lower extremity edema, consistent with NYHA functional class IV symptoms. On examination, she had a grade 3/6 holosystolic murmur best appreciated at the cardiac apex, with grade 1 bilateral, pitting lower extremity edema.

Past Medical History

The patient also had a history of heart failure reduced ejection fraction, coronary artery disease, paroxysmal atrial fibrillation, and chronic obstructive pulmonary disease with use of home oxygen. She was an active smoker with a 40 pack-year history.

Differential Diagnosis

In patients with CC-TGA and worsening heart failure symptoms, the differential diagnosis includes worsening systemic ventricular function, complete heart block, new arrythmias, or valvular abnormalities. Systemic ventricular failure and severe systemic TR are the most common causes of morbidity and mortality in these patients.

Investigations

Chest radiography and electrocardiogram

Chest x-ray revealed cardiomegaly with mild pulmonary edema (Figure 1). A 12-lead electrocardiogram showed normal sinus rhythm with slight QRS prolongation (QRS duration: 110 ms) without evidence of conduction disease (Figure 2).

Figure 1.

Baseline Chest X-Ray

Figure 2.

Baseline 12-Lead Electrocardiogram

Transthoracic echocardiography

Transthoracic echocardiography (TTE) showed mild to moderate systemic ventricular dysfunction and pulmonic ventricular ejection fraction of 55% to 60%. TTE also showed severe systemic TR (Figure 3) and a tricuspid valve mean gradient of 3.1 mm Hg.

Figure 3.

Baseline Transthoracic Echocardiogram

Transesophageal echocardiography

Transesophageal echocardiography (TEE) showed moderate systemic ventricular dysfunction. Severe systemic TR with restricted posterior leaflet motion was also noted (Figure 4). The regurgitant systemic tricuspid volume was 100 mL, tricuspid proximal isovelocity surface area was 1.1 cm, and tricuspid effective regurgitant orifice area was 0.63 cm².

Figure 4.

Baseline Transesophageal Echocardiogram

Cardiac computed tomography

Cardiac computed tomography denoted moderate systemic ventricular dilation and dysfunction with normal pulmonic ventricular function. The systemic tricuspid annulus maximal anterior-posterior diameter and maximal intercommissural distance were 36.5 mm and 32.5 mm, respectively (Figure 5). The systemic tricuspid annulus perimeter and area were 118.3 mm and 990 mm², respectively. There was severe calcification of the systemic tricuspid's posterior leaflet. Assuming a balloon-expandable virtual valve with a 29-mm diameter and 22.5-mm height, the neo–systemic ventricular outflow tract was 660 mm² (Figure 6).

Figure 5.

Baseline Systemic Tricuspid Valve Annulus on Cardiac Computed Tomography

Figure 6.

Neo–Systemic Ventricular Outflow Tract on Cardiac Computed Tomography

Management

Intervention on the systemic tricuspid valve was considered after a sufficient trial of diuretics. Transcatheter tricuspid intervention was considered because the patient was a high-risk surgical candidate. Tricuspid transcatheter edge-to-edge repair (T-TEER) was not possible because of severe posterior leaflet calcification. Transcatheter tricuspid valve replacement (TTVR) using the Evoque (Edwards Lifesciences) was not possible given concerns about manipulation in the left atrium and valve delivery with transseptal access. TTVR with the Sapien M3 (Edwards Lifesciences) was then considered, and possible challenges were discussed. Tricuspid annulus sizing was appropriate for a 29-mm M3 valve, and the neo–systemic ventricular outflow tract was sufficient to prevent iatrogenic systemic ventricular outflow tract obstruction during TTVR. The systemic tricuspid chordal apparatus seemed adequate to allow delivery of the docking system. Therefore, we proceeded with TTVR using the M3 valve.

The procedure was performed under general anesthesia with TEE and fluoroscopic guidance. Access to the right common femoral vein, left common femoral vein, and left common femoral artery was obtained using ultrasound guidance with sheath placement in each vessel. A 6-F pigtail catheter was placed into the arterial access to monitor aortic pressure. Right common femoral vein perclosure was performed using a Perclose Prostyle device (Abbott Vascular), and the right common femoral vein sheath was upsized to a 24-F Dryseal sheath (Gore Medical). Via the right femoral venous access, an Astato XS 20 guidewire (Asahi Intecc) and 8.5-F Agilis catheter (Abbott Vascular) were used to cross the interatrial septum. An exchange length j-wire and 6-F Multipurpose A2 were then placed to locate the left upper pulmonary vein. An Amplatz extra-stiff wire (Boston Scientific) was then anchored in the pulmonary vein. This system was then removed, and a 28-F Edwards guide sheath and dilator (Edwards Lifesciences) were advanced into the left atrium. The dock delivery system (DDS) was then advanced to provide encircling capture of the tricuspid's anterior and posterior chords just below the systemic tricuspid annulus. Initial attempts were made to start encircling at the septal-anterior commissure, however that required excessive manipulation of the DDS; hence, we reapproached through the septal-posterior commissure. The chordal apparatus was captured with TEE guidance (Figure 7). The proximal portion of the DDS was then atrialized, and the leak guard was released into the left atrium. The DDS was then removed, and the dock was crossed with a 6-F pigtail catheter. A Safari guidewire (Boston Scientific) was then secured in the systemic ventricle apex, and the 29-mm M3 valve delivery system was advanced over this guidewire. The 29-mm M3 valve was deployed during a period of rapid pacing via temporary venous pacemaker inserted in the left femoral venous access site. After appearing to have sufficient M3 valve placement on TEE and fluoroscopy, the M3 delivery system and Safari guidewire were removed. The transseptal access site was closed with a 25-mm Gore Septal Occluder device (Gore Medical). TEE showed no significant TR, with a minimal tricuspid mean gradient. All access sites were closed by Perclose suture (Abbott Vascular), with good hemostasis.

Visual Summary.

Native Transcatheter Tricuspid Valve Replacement in a Patient With Congenitally Corrected Transposition of the Great Arteries

3D = 3-dimensional; CC-TGA = congenitally corrected transposition of the great arteries; TEE = transesophageal echocardiography; TTVR = transcatheter tricuspid valve replacement.

Figure 7.

Intraoperative Transesophageal Echocardiography and Fluoroscopy Images

(A) TEE of encircling. (B) Fluoroscopy of encircling. (C) TEE after M3 deployment. (D) Fluoroscopy after M3 deployment. TEE = transesophageal echocardiography.

Outcome and Follow-Up

The patient's postoperative course was unremarkable. Postoperative TTE showed a well-positioned bioprosthetic tricuspid without TR or tricuspid paravalvular leak. The tricuspid mean gradient was 8.0 mm Hg. Systemic and pulmonic ventricular function were unchanged. The patient was discharged on postoperative day 1 without complications. At the 30-day follow-up, she was doing well with NYHA functional class II symptoms, and her lower extremity edema had significantly improved. TTE at the 30-day follow-up was similar to TTE on discharge and showed good prosthetic valve function.

Discussion

CC-TGA is a rare type of congenital heart disease (CHD) occurring during embryologic formation.¹ While surgical correction typically involves either an anatomic or physiologic surgical repair, the timing of surgery is variable, and some patients do not undergo surgery in childhood.² Uncorrected adult CC-TGA patients are at a higher risk of developing congestive heart failure because of worsening systemic ventricular dysfunction, which can lead to systemic TR.^3,4 Although surgical systemic tricuspid replacement can be considered in adults, long-term survival is limited, especially in patients with systemic ventricular dysfunction.⁵ Transcatheter tricuspid intervention is of increasing interest in CC-TGA patients, however more investigation is needed.⁶

Various transcatheter systemic tricuspid interventions have been explored among CC-TGA patients. Among high–surgical risk CC-TGA patients with moderate to severe systemic TR, T-TEER using an off-label Mitraclip (Abbott Vascular) has been shown to reduce symptom severity and TR severity for up to 1 year.⁷ While these results are encouraging, T-TEER only partially reduces TR severity and cannot be used in patients with anatomy unsuitable for T-TEER. Valve-in-valve TTVR using an off-label Sapien 3 (Edwards Lifesciences) has been reported in a CC-TGA patient with degeneration of a bioprosthetic systemic tricuspid, however this requires a previous surgical bioprosthetic valve to anchor the valve.⁸ Dedicated devices for transcatheter mitral valve replacement and TTVR have provided promising results in terms of reducing valve regurgitation and reducing symptoms.^9,10 The M3 and Evoque platforms are now commonly used for transcatheter mitral valve replacement and TTVR, respectively, and their use will likely be explored in CHD patients as they age. To the best of our knowledge, this is the first reported experience of native TTVR using an M3 valve in a CC-TGA patient.

Although the M3 valve was deemed to be a better option compared to the Evoque valve, there were also concerns about using the M3 valve, as it is not typically used for TTVR. More specifically, there were concerns about the approach to encircling without causing systemic ventricular perforation. As previously discussed, initiation of encircling was originally attempted at the septal-anterior commissure, but encircling was eventually approached from the septal-posterior commissure to enhance DDS manipulation. Despite the tricuspid morphology of the valve, there were no technical issues with M3 encircling or valve deployment. Moreover, there were no complications with the case, and the systemic TR was eliminated with good bioprosthetic valve function. Additionally, ventricular function remained unchanged after valve deployment. The patient was noted to be doing well at the 30-day follow-up. While this case had favorable procedural and follow-up outcomes, operators should take each patient's unique anatomy into careful consideration when using these more novel transcatheter therapies in adult CHD patients.

Further investigation into transcatheter systemic tricuspid intervention is needed for CC-TGA patients. Moreover, the use of transcatheter tricuspid intervention in adult CHD patients should take the patient's unique anatomy into consideration. This case illustrates the efficacy and safety of using the M3 valve for native TTVR in CC-TGA to highlight an additional option for operators.

Conclusions

This case highlights how the M3 platform can be used for TTVR in a patient with CC-TGA with a favorable outcome. In the future, this may provide another option for CC-TGA patients who need systemic tricuspid valve replacement.

Take-Home Message

• •Transcatheter tricuspid valve replacement with the Sapien M3 valve is a feasible and safe alternative in high–surgical risk patients with congenitally corrected transposition of the great arteries and symptomatic systemic tricuspid regurgitation.

Funding Support and Author Disclosures

Drs Shekiladze, Gleason, Greenbaum, and Babaliaros have received institutional research support from Abbott Vascular, Ancora Heart, Edwards Lifesciences, Gore Medical, JenaValve, Medtronic, Polares Medical, Transmural Systems, and 4C Medical. Dr Greenbaum has served as a consultant to Abbott Vascular, Edwards Lifesciences, Excision Medical, and Medtronic; and has equity interests in Transmural Systems and Excision Medical. Dr Babaliaros has served as a consultant to Abbott Vascular, Edwards Lifesciences, and Medtronic; and has equity interest in Transmural Systems. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.