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Imaging and guiding intervention for tricuspid valve disorders using 3-dimensional transesophageal echocardiography in pediatric and congenital heart disease

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Abstract

In the pediatric and congenital heart disease (CHD) population, tricuspid valve (TV) disorders are complex due to the variable TV morphology, its sophisticated interaction with the right ventricle as well as associated congenital and acquired lesions. While surgery is the standard of care for TV dysfunction in this patient population, transcatheter treatment for bioprosthetic TV dysfunction has been performed successfully. Detailed and accurate anatomic assessment of the abnormal TV is essential in the preoperative/preprocedural planning. Three-dimensional transthoracic and 3D transesophageal echocardiography (3DTEE) provides added value to 2-dimensional imaging in the characterization of the TV to guide therapy and 3DTEE serves as an excellent tool for intraoperative assessment and procedural guidance of transcatheter treatment. Notwithstanding advances in imaging and therapy, the timing and indication for intervention for TV disorders in this population are not well defined. In this manuscript, we aim to review the available literature, provide our institutional experience with 3DTEE, and briefly discuss the perceived challenges and future directions in the assessment, surgical planning, and procedural guidance of (1) congenital TV malformations, (2) acquired TV dysfunction from transvenous pacing leads, or following cardiac surgeries, and (3) bioprosthetic TV dysfunction.

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Abbreviations

ACHD:

Adult congenital heart disease

CHD:

Congenital heart disease

RV:

Right ventricle

TV:

Tricuspid valve

TR:

Tricuspid regurgitation

3DTEE:

3-Dimensional transesophageal echocardiography

3DE:

3-Dimensional echocardiography

2DE:

2-Dimensional echocardiography

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Acknowledgements

The authors appreciate Ms. Kaitlin Doan for her assistance in editing the videos.

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The authors have not disclosed any funding.

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Authors and Affiliations

  1. Echocardiography Laboratory, The Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children’s Hospital, 6651 Main Street, MC E1920, Houston, TX, 77030, USA

    Tam T. Doan, Ricardo H. Pignatelli & Anitha Parthiban

  2. Texas Adult Congenital Heart Center, The Lillie Frank Abercrombie Section of Pediatric Cardiology, Texas Children’s Hospital, Houston, TX, 77030, USA

    Dhaval R. Parekh

  3. Department of Pediatrics, Baylor College of Medicine, Houston, TX, 77030, USA

    Tam T. Doan, Ricardo H. Pignatelli, Dhaval R. Parekh & Anitha Parthiban

  4. Department of Medicine, Baylor College of Medicine, Houston, TX, 77030, USA

    Dhaval R. Parekh

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TD: wrote the main manuscript text and prepared figures and videos. All authors reviewed, edited, and approved the final version of the manuscript.

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Correspondence to Tam T. Doan.

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Supplementary file1 Video 1. TV dysplasia with tethered septal leaflet (S) and significantly reduced motion of the leaflet edge which led to a coaptation defect with the anterior (A) and posterior leaflet (P). Patient had a successful repair with mild regurgitation and no stenosis (MP4 30673 kb)

Supplementary file2 Video 2. Ebstein anomaly with severe apical displacement of the septal leaflet and restricted motion of all 3 leaflets resulting in a large coaptation defect. The TV orifice is seen opening into the RV outflow tract. Patient had a TV replacement due to high risk condition and severely limited leaflet mobility (MP4 24397 kb)

Supplementary file3 Video 3. Ebstein anomaly with moderate apical displacement of the septal leaflet. There are 2 large anterior leaflets and good mobility of all 3 leaflets. The effective orifice is seen opening into the RV outflow tract. The patient underwent a successful Cone reconstruction of the TV (MP4 24395 kb)

Supplementary file4 Video 4. Severe TR in a patient with history of hypoplastic left heart syndrome and Fontan circulation. The anterior and posterior leaflets are fused with significant prolapse while the septal leaflet is tethered with restricted mobility, resulting in a large coaptation defect and severe TR via an eccentric jet (MP4 15866 kb)

Supplementary file5 Video 5. Transvenous dual chamber pacemaker with atrial lead curling at the TV inflow disrupting closure of the leaflets and ventricular lead crossing the TV along the septal leaflet (MP4 23724 kb)

Supplementary file6 Video 6. Ventricular pacemaker lead crossing near the posterior leaflet with significant restriction of posterior and septal leaflet resulting in a large coaptation defect (MP4 18733 kb)

Supplementary file7 Video 7. Persistent severe TR from tethered septal and posterior leaflets and densely thickened chord resulting in a very large coaptation defect (following a transvenous defibrillator lead removal) (MP4 24470 kb)

Supplementary file8 Video 8. Patient with single RV, Fontan circulation, and mechanical St Jude valve in the TV position presenting with recurrent stroke. There are significant artifacts on 2DE. The 2 discs are with normal mobility on 3DTEE, and no obvious clots noted on the valve (MP4 4028 kb)

Supplementary file9 Video 9. Konno patch dehiscence resulting in severely restricted septal leaflet, a large coaptation defect and severe regurgitation along the septal leaflet (MP4 25059 kb)

Supplementary file10 Video 10. Procedural guidance using 3DTEE during an off-label, transcatheter valve-in-valve treatment using a 29-mm Edwards valve for severe, symptomatic TR 4 years after surgical replacement of a 33-mm Epic porcine valve (MP4 95650 kb)

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Doan, T.T., Pignatelli, R.H., Parekh, D.R. et al. Imaging and guiding intervention for tricuspid valve disorders using 3-dimensional transesophageal echocardiography in pediatric and congenital heart disease. Int J Cardiovasc Imaging 39, 1855–1864 (2023). https://doi.org/10.1007/s10554-023-02898-8

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