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JACC Case Rep
JACC Case Rep
JACC Case Reports
2666-0849
Elsevier

S2666-0849(24)00204-3
10.1016/j.jaccas.2024.102411
102411
Mini-Focus Issue on Congenital Heart Disease
Case Report: Clinical Case
Transcatheter Edge-to-Edge Repair in Complex Cyanotic Congenital Heart Disease With a Common Atrioventricular Valve
Connaire Stephanie J. MBBCh(Hons) Stephanie.connaire@uhbw.nhs.uk
a∗
Webster Jessica R. MB BS, BSc a
Mullen Michael J. MB BS, MD b
Mullen Aigerim MD, PhD c
Szantho Gergely V. MD a
Turner Mark S. MBChB(Hons), PhD a
a Bristol Heart Institute, Bristol, United Kingdom
b Barts Health NHS Trust, London, United Kingdom
c Abbott Medical UK Ltd, Kingston Upon Thames, United Kingdom
∗ Address for correspondence: Dr Stephanie Connaire, Bristol Heart Institute, Terrell Street, Bristol BS2 8ED, United Kingdom. Stephanie.connaire@uhbw.nhs.uk
21 8 2024
21 8 2024
21 8 2024
29 16 10241126 2 2024
24 4 2024
2 5 2024
Crown Copyright © 2024 Published by Elsevier on behalf of the American College of Cardiology Foundation.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
A 21-year-old patient with complex cyanotic congenital heart disease and highly symptomatic severe common atrioventricular valve regurgitation was deemed too high risk for surgical intervention or transplantation. She successfully underwent transcatheter edge-to-edge repair with resulting considerable improvement in her symptoms, renal function, and quality of life.

Graphical Abstract

Key Words

congenital heart defect
cyanotic heart disease
valve repair
Abbreviations and Acronyms

AV atrioventricular

IVC inferior vena cava

TEE transesophageal echocardiogram

TEER transcatheter edge-to-edge repair
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pmcHistory of Presentation

A 21-year-old woman with complex cyanotic congenital heart disease was known to the congenital heart disease team. She had recurrent admissions to hospital with breathlessness and peripheral edema secondary to severe common atrioventricular (AV) valve regurgitation with escalating diuretic requirements and deteriorating renal function. She had been dependent on intravenous furosemide infusions and frequent doses of metolazone in addition to regular spironolactone. Furthermore, she had developed recurrent episodes of supraventricular tachycardia, hemoptysis, and plastic bronchitis.Learning Objectives

• To understand the current limited use of transcatheter edge-to-edge repair in congenital heart disease.

• To recognize the advantage of using both transesophageal echocardiography and intracardiac echocardiography during TEER to support a safe procedure in complex disease.

• To consider the use of TEER to maximize quality of life in young patients with atrioventricular valve regurgitation in which surgery is not a feasible option.

Echocardiography showed progression of common AV valve regurgitation from moderate to severe. She was referred for consideration of cardiac transplantation but was deemed unsuitable due to multiple risk factors, including absent right pulmonary veins and significant aortopulmonary and venovenous collaterals. AV valve surgery was deemed too high risk and likely associated with significant decompensation of single ventricular function.

Past Medical History

The congenital anatomy included dextrocardia, right atrial isomerism, complete atrioventricular septal defect, functionally single ventricle, double-outlet right ventricle with transposition of the great arteries, pulmonary atresia, and total anomalous pulmonary venous drainage. She had a duct-dependent circulation at birth and subsequently had a Blalock-Taussig shunt. At 6 months of age, the Blalock-Taussig shunt was taken down, anomalous pulmonary veins were reconstructed, and a cavopulmonary connection (Glenn) created. She had recurrent obstruction in the reconstructed right upper pulmonary vein requiring multiple balloon dilatations and surgical repair with an autologous patch at 5 years of age.

On transition to the adult service, the patient was cyanotic with percent resting saturations in the 70s on air. The right-sided pulmonary veins were occluded on magnetic resonance imaging, with common drainage of the left pulmonary veins to the atrial mass. She was known to have moderate AV valve regurgitation for a number of years throughout childhood.

Differential Diagnosis

In the context of reasonable single ventricular systolic function and severe common AV valve regurgitation, the most likely cause of her symptoms was felt to be the AV valve regurgitation. In turn, this had resulted in pulmonary edema and plastic bronchitis.

Investigations

Serial echocardiograms reported significant progression of regurgitation through the common AV valve with an estimated regurgitant volume of 159 mL (Figure 1). The systemic single ventricle was dilated with borderline low radial systolic function. There was unobstructed aortic outflow with trivial regurgitation. The cavopulmonary connection was unobstructed.Figure 1 Transthoracic Echocardiogram

Color Doppler transthoracic echocardiography apical 4-chamber view showing severe central regurgitation through the common atrioventricular valve into a common atrium.

On transesophageal echocardiography (TEE), there was a clearly defined area of coaptation failure of the bridging AV valve leaflets, which was identified as a potential target for transcatheter edge-to-edge repair (TEER) (Video 1, Figure 2).Figure 2 Transoesophageal Echocardiogram

Transesophageal echocardiogram 3-dimensional image with color flow showing the common atrioventricular valve with a central origin of regurgitation.

Previous cross-sectional imaging (computed tomography and cardiac magnetic resonance imaging) was reviewed. This confirmed that the inferior vena cava (IVC) was not interrupted, a known association of isomerism. It was important to ensure the IVC connected to the common atrium, allowing a clear route to pass the TEER delivery system. A prominent Eustachian ridge from the IVC was also noted, which became important during the procedure (Figure 3).Figure 3 Computed Tomography

Cardiac computed tomography images: (A) common atrioventricular valve with coaptation gap and (B) prominent Eustachian valve arising from the junction of the inferior vena cava connection to the atria (yellow arrows).

Management

Following extensive multidisciplinary team discussion, it was agreed that TEER would be technically feasible. TEER was therefore offered on compassionate grounds, aiming to reduce symptoms and improve quality of life. The patient was counselled on the unconventional application of this technique and understood the uncertainty regarding procedural outcome. Approval from the hospital governance team was obtained.

TEER was performed under general anesthesia using a TriClip device (Abbott) with both TEE and intracardiac echocardiography guidance. The procedural strategy included 2 XTW implants in a central position, approximating the bridging leaflets of the common AV valve with an orientation of 6 to 12 o’clock in relation to the coaptation line. Right femoral vein access was obtained, and the steerable guide catheter was advanced into the atrium. After the introduction of the delivery system through the guide, there were difficulties in flexing the shaft with the implant further towards the valve due to the prominent ridge of tissue at the IVC-atrial junction. Using TEE and intracardiac echocardiography guidance, the guide catheter was manipulated posteroinferior to this ridge, and an XTW clip was deployed in a satisfactory position with a low transvalvular inflow gradient (Video 2).

Despite an initial significant reduction in AV regurgitation, a decision was made to deploy a second implant to stabilize the first, given the excessive leaflet mobility and the large valve area (intercommissural distance 7.2 cm). As the second XTW clip was deployed in a medial position next to the first, we noted a single leaflet detachment of the first clip. This was a potential complication we had anticipated. The second clip had successfully reduced the amount of regurgitation, but a third clip was seen to be desirable to reduce the risk of further single leaflet detachment. After the third XTW implant was deployed on the lateral side from the first device, the final result demonstrated a significant reduction in common AV valve regurgitation, stable position of all 3 implants, and a well-formed tissue bridge across bridging leaflets forming a triple orifice common AV valve with maintained low inflow transvalvular gradient (Video 3).

The patient was admitted to cardiac intensive care overnight and discharged to the ward the following day. Echocardiography day 1 postprocedure reported residual moderate common AV valve regurgitation arising from the central orifice. The proximal isovelocity surface area radius was 0.65 cm, and regurgitant volume had improved substantially to 36 mL. Diuretic doses were decreased to bumetanide 2 mg twice a day and spironolactone 50 mg once a day with no reaccumulation of fluid. Renal function normalized with significant reduction in urea (from 34 mmol/L to 11 mmol/L) and creatinine (136 mmol/L to 84 mmol/L) before discharge home.

Discussion

TEER has become established in treating mitral and tricuspid regurgitation in patients with acquired heart disease deemed unsuitable for surgery. The initial indication of central, degenerative mitral valve disease is expanding to more complex anatomy. TEER in congenital heart disease remains novel and only described in a small number of case reports. In 2022, Silini and Iriart1 published a review of case reports and case series describing TEER in congenital heart disease. Eleven cases of TEER of AV valves in systemic right ventricles and 5 cases in systemic left ventricle were included. Kumar et al2 later described TEER in an adult with a double-outlet right ventricle, published in May 2023.

We believe this case report of TEER in a patient with complex cyanotic congenital heart disease further adds to the literature. Due to the lack of an atrial septum and the specific anatomy in this case (proximity of the IVC to the AV valve coaptation gap and angulation of the annular plane), the TriClip delivery system was chosen to give more support for the maneuver.

Follow-up

Six months following the procedure, this patient remains stable on significantly reduced doses of diuretic agents with no further hospital admissions. She is no longer coughing up casts and has been weaned off prednisolone for the plastic bronchitis. She is still breathless on exertion but has been able to return to some formal exercise at the gym. She is now looking into the prospect of returning back to her university course in the near future. Her quality of life has improved considerably.

Conclusions

In summary, TEER for severe common AV valve regurgitation in a patient with complex cyanotic congenital heart disease was successful, leading to an improved quality of life and avoiding repeated hospital admissions. The use of TEER in the complex congenital patient is yet to be fully understood, but we believe it should be considered in this context when surgical intervention is not a feasible option.

Funding Support and Author Disclosures

Dr M.J. Mullen has received research grants from Edwards Lifesciences and Abbot Vascular; and has received proctoring fees from Abbot Vascular. Dr A. Mullen is marketing manager at Structural Heart UK and Ireland, Abbott Medical UK. Dr Turner has been a proctor and a consultant for Abbott, Edwards Lifesciences, Medtronic, and Occlutech. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Appendix

Video 1

TEE image with X-plane through common AV valve at 60° and 150° demonstrating a clear area of leaflet coaptation failure.

Video 2

ICE imaging showing successful deployment of the first XTW implant.

Video 3

TEE of common AV valve after all implants were deployed showing a significant improvement in the extent of regurgitation.

The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.

Appendix

For supplemental videos, please see the online version of this paper.
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References

1 Silini 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 8 5 2022 100370 10.1016/j.ijcchd.2022.100370
2 Kumar P. Gordon B.M. Kheiwa A. Abudayyeh I. A case report of percutaneous MitraClip implantation in an adult with a double-outlet right ventricle Eur Heart J Case Rep 7 6 2023 ytad247 10.1093/ehjcr/ytad247
