Catheter-Based Techniques for Addressing Atrioventricular Valve Regurgitation in Adult Congenital Heart Disease Patients: A Descriptive Cohort

Abstract

Introduction: Increasing survival of adult congenital heart disease (ACHD) patients comes at the price of a range of late complications-arrhythmias, heart failure, and valvular dysfunction. Transcatheter valve interventions have become a legitimate alternative to conventional surgical treatment in selected acquired heart disease patients. However, literature on technical aspects, hemodynamic effects, and clinical outcomes of percutaneous atrioventricular (AV) valve interventions in ACHD patients is scarce.

Method: This is a descriptive cohort from CAHAL (Center of Congenital Heart Disease Amsterdam-Leiden). ACHD patients with severe AV valve regurgitation who underwent a transcatheter intervention in the period 2020-2022 were included. Demographic, clinical, procedural, and follow-up data were collected from patient records.

Results: Five ACHD patients with severe or torrential AV valve regurgitation are described. Two patients underwent a transcatheter edge-to-edge repair (TEER), one patient underwent a valve-in-valve procedure, one patient received a Cardioband system, and one patient received both a Cardioband system and TEER. No periprocedural complications occurred. Post-procedural AV valve regurgitation as well as NYHA functional class improved in all patients. The median post-procedural NYHA functional class improved from 3.0 (IQR [2.5-4.0]) to 2.0 (IQR [1.5-2.5]). One patient died 9 months after the procedure due to advanced heart failure with multiorgan dysfunction.

Conclusion: Transcatheter valve repair is feasible and safe in selected complex ACHD patients. A dedicated heart team is essential for determining an individualized treatment strategy as well as pre- and periprocedural imaging to address the underlying mechanism(s) of AV regurgitation and guide the transcatheter intervention. Long-term follow-up is essential to evaluate the clinical outcomes of transcatheter AV valve repair in ACHD patients.

Keywords: Cardioband; adult congenital heart disease (ACHD); atrioventricular (AV) regurgitation; hybrid; transcatheter edge-to-edge repair (TEER); transcatheter valve repair; valve-in-valve (ViV).

Figure 1

Atrioventricular (AV) valve regurgitation before and after transcatheter valve intervention. Each line represents a single patient (A–E corresponding to patient A–E, respectively).

Figure 2

NYHA functional class before and after the transcatheter valve intervention, as well as at latest available follow-up. Each line represents a single patient (A–E corresponding to patient A–E, respectively).

Figure 3

(A) Color Doppler apical four-chamber view shows torrential tricuspid regurgitation (TR) with a wide vena contracta (arrow) and flow disturbance filling the enlarged right atrium. (B) Continuous wave doppler showing a dense TR signal with elevated right ventricular pressures. (C) Noncoaptation of the tricuspid valve (TV) leaflets (arrow) and annulus dilatation (dash line, 53 mm) is seen. (D) The hepatic vein Doppler demonstrates a pattern in atrial fibrillation with a prominent and late peaking systolic reversal (SR) wave. The only forward flow is evident in diastole (D-wave). (E) Two TR jets (a vena contracta of 4 and 7 mm, respectively, ERO of 90 mm², regurgitant volume of 98 mL) are evident during transesophageal imaging. (F) Left anterior oblique and (G) right superior oblique fluoroscopic views showing patent right coronary artery (asterisk) and 18 anchors (arrows) between the two TV commissures allowing the Cardioband to significantly reduce the annulus dimensions. (H) Transesophageal echocardiography showing significant reduction in TR after the Cardioband annulus reduction procedure (appreciate the difference with panel (E), vena contracta of 3 and 5 mm, respectively, ERO 35 mm², regurgitant volume of 41 mL).

Figure 4

(A) Color Doppler apical four-chamber view shows severe tricuspid regurgitation (TR) with a wide vena contracta (arrow) and a systolic jet reaching the roof of the enlarged right atrium (RA). (B) Color Doppler showing turbulent inflow through the tricuspid valve (TV) bioprosthesis and aliasing, mean gradient was elevated at 4–5 mmHg. (C) Continuous wave Doppler showing a dense TR signal with low velocity. (D) Calcified and degenerated tricuspid bioprosthesis (arrow). (E) Axial slice through a computed tomography (CT) scan at the level of the right ventricle shows a giant RA and an intra-atrial septum deviation towards the left atrium (arrow), partially suppressing it. (F) Sagittal CT slice shows the inflow angle of the inferior vena cava-right RA and the RA-TV. Note the distended hepatic vein (HV). (G,H) Right anterior oblique fluoroscopy projections show the expansion of the Sapien 3 valve (asterisk) using the ring of the degenerated bioprosthesis as the reference and the final result, respectively. (I,J) Apical four-chamber color Doppler views showing normal function of the valve-in-valve bioprosthesis (appreciate the difference with (A,B), respectively). (K,L) View of the valve-in-valve bioprosthesis in systole (closed) and diastole (open), respectively.

Figure 5

(A) Distance between the right internal jugular vein and tricuspid valve (TV) measured on a computed tomography (CT) scan in a coronal plane (arrow). (B) Severely dilated mono-atrium (asterisk) on CT scan in a sagittal plane. (C) Implantation of the second XTW clip (note the first XTW clip already released) under transesophageal echocardiography (TEE) guidance. (D) Preprocedural TEE imaging of the torrential (IV+) TR. (E) Periprocedural TEE showing TR after placing the first XTW clip. (F) TEE showing the significant reduction in TR after placing the second XTW clip to grade I–II.

Figure 6

(A,B) Computed tomography (CT) scan of the thoracic cavity with the anatomical position of the dilated functional mono-atrium (asterisk) against the right thoracic wall (panel A) and bidirectional bilateral Glenn shunt (panel B) (LPA = left pulmonary artery, RPA = right pulmonary artery, LSVC = left superior vena cava, RSVC = right superior vena cava). (C) Pre-procedural transesophageal echocardiography (TEE) which visualized severe common atrioventricular (AV) valve regurgitation. (D) Anteroposterior fluoroscopic view of the two XTW MitraClips positioned in the AV valve. At the same time, it is appreciated that the delivery system is positioned through the fifth intercostal space after a right mini-lateral thoracotomy. (E) Moderate AV regurgitation after the hybrid procedure visualized with TEE.

Figure 7

(A) Color Doppler modified parasternal right ventricular (RV) inflow view shows severe tricuspid regurgitation (TR) with a wide vena contracta (arrow) and leakage jet reaching the inferior vena cave (IVC). (B) Apical four-chamber view shows tricuspid valve (TV) annulus dilatation (dash line, 46 mm) and impingement by the RV pacemaker lead (arrow). (C) Left inferior oblique fluoroscopic view of the Cardioband (arrow) annulus reduction procedure (asterisk). (D) Anteroposterior fluoroscopic view shows the two XTW clips (arrows) implanted. (E,F) Modified apical four chamber color Doppler views showing mild (-moderate) residual TR and inflow through the TV (mean gradient of 3 mmHg).