Transcatheter Edge-to-Edge Repair of Common Atrioventricular Valve Regurgitation in the Setting of a Lateral Tunnel Fontan Circulation

•Percutaneous repair of severely regurgitant atrioventricular valves in the setting of a Fontan circulation utilizing Tri-Clip, even in the setting of an atrioventricular septal defect, is feasible.•Percutaneous options hold promise as a low-risk repair strategy in this highly complex population, which generally has a high surgical risk. Most children with univentricular physiology now survive to adulthood by undergoing a series of operations that involve directly connecting the inferior and superior vena cavae to the pulmonary arteries so that deoxygenated blood is directed to the lungs and oxygenated blood returns to the single ventricular chamber. This "Fontan physiology" is fragile, and problems such as severe valve regurgitation markedly impede the circulation and are associated with poor survival. There are emerging case reports of percutaneous strategies being employed in patients living with Fontan circulation or non-Fontan univentricular physiology with a mitral or tricuspid valve as their single atrioventricular (AV) valve with severe regurgitation.1Silini A. Iriart X. Percutaneous edge-to-edge repair in congenital heart disease: preliminary results of a promising new technique.Int J Cardiol Congenit Heart Dis. 2022; 8100370Google Scholar, 2Donovan R.J. Lim S. Davidson W.R. Case report: percutaneous mitral valve repair in a patient with Fontan repair for a single functional ventricle.J Heart Lung Transplant. 2020; 39: S239Abstract Full Text PDF Scopus (0) Google Scholar, 3Blusztein D. Mahadevan S. Transcatheter edge-to-edge repair of systemic tricuspid valve in extracardiac Fontan circulation first in Human.J Am Coll Cardiol Case Rep. 2022; 4: 221-225Google Scholar, 4Haeffele C.L. Lui G.K. Peng L. Chan F. Sharma R.P. First described mitral clip in an adult extracardiac Fontan patient: a case report.Eur Heart J Case Rep. 2023; 7ytac479Google Scholar, 5Alshawabkeh L. Mahmud E. Reeves R. Percutaneous mitral valve repair in adults with congenital heart disease: report of the first case-series.Catheter Cardiovasc Interv. 2021; 97: 542-548Crossref PubMed Scopus (4) Google Scholar There is one report of a person with a non-Fontan univentricular circulation with an unbalanced AV septal defect undergoing transcatheter edge-to-edge repair (TEER) with the MitraClip system.6Tan W. Calfon Press M. Lluri G. Aboulhosn J. Percutaneous edge-to-edge repair for common atrioventricular valve regurgitation in a patient with heterotaxy syndrome, single ventricle physiology, and unbalanced atrioventricular septal defect.Catheter Cardiovasc Interv. 2020; 96: 384-388Crossref PubMed Scopus (2) Google Scholar We successfully undertook TEER for severe AV valve regurgitation related to an AV septal defect in the setting of a lateral tunnel Fontan circulation in a 36-year-old woman. She was born with an unbalanced complete AV septal defect with a dominant double outlet right ventricle and dual superior vena cavae. She had a neonatal pulmonary band followed by bidirectional Glenn shunts, AV valve repair with annuloplasty, and intracardiac lateral tunnel-type Fontan operation using a Teflon baffle at age 7 years. There was moderate regurgitation of the AV valve for many years, but it had worsened to very severe over 2 years (Figure 1A). Her surgical risk was further increased because of iatrogenic phrenic nerve palsy and low body weight. Baseline oxygen saturations were 93% to 95% and peak oxygen uptake was 14.8 mL/kg/min with N-terminal pro b-type natriuretic peptide of 1200 ng/L. Ventricular ejection fraction was 40%, measured at cardiac magnetic resonance imaging. Procedural planning with both in vivo simulation and a 3-dimensional-printed heart model (Figure 1B) demonstrated that it was feasible to reduce the degree of AV valve regurgitation by clipping the superior and inferior bridging leaflets (shown in Figure 1C and D) with transvenous access and intracardiac baffle puncture to access the pulmonary venous atrium. TEER was performed via a right transfemoral venous approach using a TriClip G4 system (Abbott, Chicago, IL). To access the atrium, the intracardiac baffle was punctured high and posteriorly, with passage of a BRK transeptal needle (Abbott, Chicago, IL) through atrial tissue rather than through the polymer (expanded polytetrafluoroethylene) component of the baffle (Figure 2). This position was chosen as it allowed enough height to position the system within the pulmonary venous atrium and grasp the valve leaflets. The baffle puncture was serially dilated with 6.0 and 8.0 mm-complaint balloons to facilitate passage of the Steerable Guiding Catheter of the TriClip G4 system. A TriClip XTW prosthesis was then steered toward the common AV valve under transesophageal echocardiographic guidance using combined steering of the Steerable Guiding Catheter and the Clip delivery systems. A single TriClip G4 XTW device was deployed, with independent sequential grasping of the inferior leaflet, followed by the superior leaflet, at the site of maximal inferior leaflet prolapse. Optimization of leaflet grasp was performed for the superior and inferior leaflets, respectively. Valvular regurgitation was reduced from torrential to moderate regurgitation with a mean diastolic gradient of 2 mmHg. The baffle puncture had contracted to 4 mm by conclusion, and oxygen saturations were 91% to 93%, so we opted not to proceed with fenestration closure. At the most recent follow-up of 6 months postprocedure, the patient was well with a marked reduction in the degree of valvar regurgitation, now graded as moderate (Figure 1A) with low normal ventricular contraction by echocardiography. She was New York Heart Association Class I with oxygen saturations of 94% and N-terminal pro b-type natriuretic peptide having fallen to 620 ng/L. She declined a repeat cardiopulmonary exercise test and cardiac magnetic resonance imaging. Informed consent was obtained prior to the publication of this work. The authors have no funding to report.