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J Cardiothorac Surg
J Cardiothorac Surg
Journal of Cardiothoracic Surgery
1749-8090
BioMed Central London

39261924
2985
10.1186/s13019-024-02985-4
Case Report
“One-stop” interventional therapy for quadricuspid aortic valve combined with severe coronary artery disease: a case report
Luo Cheng
Zheng Baoshi baoshizhengyx@163.com

https://ror.org/030sc3x20 grid.412594.f Cardiovascular Surgery Ward, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi China
12 9 2024
12 9 2024
2024
19 52625 4 2024
13 8 2024
© The Author(s) 2024
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ Open Access This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.
Congenital Quadricuspid Aortic Valve (QAV) malformation is a relatively rare cardiac valve malformation, especially with abnormal coronary opening and severe stenosis of Coronary Artery Disease (CAD). The patient underwent “one-stop” interventional treatment with transcatheter aortic valve replacement and percutaneous coronary stent implantation. Follow up for 12-month with good outcomes.

Keywords

One-stop
Quadricuspid aortic valve
Coronary artery disease
Transcatheter aortic valve replacement
Percutaneous coronary intervention
Teachers Foundation of GuangxiNo. 2022KY0101 No. 2022KY0101 issue-copyright-statement© BioMed Central Ltd., part of Springer Nature 2024
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pmcIntroduction

This patient’s Computed Tomography Angiography (CTA) showed that the aortic valve comprised three equal-sized leaflets and one smaller leaflet. It was a type B QAV. The left and right coronary artery openings were low and combined with severe stenosis of several coronary arteries, massive aortic regurgitation, and aortic valve had a large amount of reflux. The patient underwent “one-stop” interventional treatment with good outcomes and the symptoms improved significantly, with no cerebrovascular accident, atrioventricular block, myocardial infarction, and valve displacement. We report this case of a patient with QAV in combination with CAD, underwent one-stop treatment of Transcatheter Aortic Valve Replacement (TAVI) and Percutaneous Coronary Intervention (PCI), the results were satisfactory with a 12-month postoperative follow-up.

Patient information

The patient Female, 76 years old, was admitted to the hospital on January, 2023, with “chest tightness and tightness of breath for more than 10 years, with recurrent exacerbation for 4 days”. She had a history of “hypertension, type II diabetes, anemia, cardiac arrhythmia, thyroid dysfunction, hyperuricemia, and ectopic pregnancy surgery”.

Clinical findings and timeline

Body temperature 36.4℃, pulse 62 times/min, respiration 20 times/min, blood pressure 148/57mmHg, no obvious filling of jugular vein, symmetrical and normal chest, regular respiratory rhythm, no dry or wet rales heard in both lungs, cardiac boundary expanding to the left, no cardiac arrhythmias, grade 4/6 decreasing diastolic sigh-like murmurs can be heard between the third and fourth ribs of the left sternum, and no bilateral lower limb oedema. Laboratory tests: brain natriuretic peptide precursor: 8324.00 pg/ml, no significant abnormality in the blood routine, liver and kidney function, myocardial enzymes, electrolytes, and thyroid function. Atherosclerosis plaque in the whole right coronary segment, and plaque formation in the opening of the right descending branch. Electrocardiogram: frequent premature ventricular and sinus bradycardia. CTA show that the aortic valve is a type B QAV (Fig. 1a), the noncoronary cusp near the right coronary cusp is small, and the other three valves were essentially equal in size, the aortic valves were slightly thickened and calcified. In this setting, aortic valve calcifcation (AVC) assessed by CTA is of major interest. According to the aortic valve calcium score (Score of 0: No evidence of calcium deposits in the aortic valve; Score of 1–99: Mild calcification. Small or scattered calcium deposits present in the valve leaflets; Score of 100–399: Moderate calcification. Moderate amount of calcium deposits seen in the valve, with increased thickness and density; Score of 400 or higher: Severe calcification.) For enhanced accuracy, CTA-AVC scores indexed in this patient should be identify a severe stenosis. The diameter of the aortic annulus was 21.7 mm(Fig. 1b), the height of the ostial left coronary artery was 9.9 mm(Fig. 1c), and the height of the ostial right coronary artery was 6.6 mm (Fig. 1d). Coronary angiography showed: 70–85% irregular stenosis in the proximal to proximal middle segment of the Left Anterior Descending (LAD) coronary artery, 40% stenosis in the opening of the circumflex coronary artery, 75% in the middle segment, and 80% stenosis in the distal segment (the lumen of the distal segment was about 1.5 mm) (Fig. 2a). Echocardiography showed that the aortic valve was thickened and calcified with moderate to severe insufficiency and mild stenosis; posterior mitral valve root and annulus calcification and mild regurgitation (Fig. 2c).

Fig. 1 Preoperative CTA. a Type B quadricuspid aortic valve; b Aortic valve annulus and inner diameter; c The height of ostial left coronary artery; d The height of the ostial right coronary artery

Fig. 2 Coronary angiography and echocardiography. a Severe stenosis of the left anterior descending; b Stent placement; c Preoperative severe aortic regurgitation; d Postoperative no aortic regurgitation

Diagnostic

(1) Type B quadricuspid aortic valve, aortic valve severe insufficiency; (2) Coronary atherosclerotic heart disease; (3) Type 2 diabetes mellitus; (4) Hypertension; (5) Arrhythmias, Frequent premature ventricular beats, Sinus bradycardia; (6) Anemia; (7) Peripheral atherosclerosis.

Assessment

According to the American Society of Thoracic Surgeons (STS) score and the European System for Cardiac Operative Risk (EuroSCORE II), the patient had a high cardiac operative risk, TAVI and PCI were recommended.

Therapeutic

The procedure was performed in our cardiac catheterization operating room on January, 2023, under the monitoring of transesophageal echocardiography and Digital Subtraction Angiography (DSA), with general intravenous anesthesia, endotracheal intubation, and ventilator-assisted. First, coronary artery stent implantation through the femoral artery, one 3.0 * 30 mm drug-eluting stent was implanted in the proximal segments of LAD. Angiography showed no significant residual stenosis, and Thrombolysis in Myocardial Infarction (TIMI) blood flow was grade 3 (Fig. 2b). Through the left femoral vein, a temporary cardiac pacemaker was inserted into the right ventricle, and through the left fourth intercostal into the chest. A purse-string suture was performed in the avascular area at the apical of the heart and a guide wire. The pacing heart rate was adjusted to 180 beats per minute, and the blood pressure was adjusted to 60mmHg. The aortic valve was dilated with a balloon and retracted the balloon (Fig. 3a). J-valve NO.23 bioprosthetic aortic valves were implanted through the apical pathway (Fig. 3b). Transesophageal Echocardiography (TEE) and aortic root angiography showed that the valve was properly positioned, with good opening and closing, no perivalvular regurgitation, and good visualization of the left and right coronary arteries and the guide wire was removed (Fig. 2d), the pericardium was knotted, placed a drainage tube, and the chest is closed.

Fig. 3 Valve insertion and valve release. a valve insertion; b valve release

Follow-up and outcomes

The patient’s postoperative symptoms improved significantly, and no perivalvular leakage was found in the postoperative follow-up echocardiography. There were no complications such as cerebrovascular accidents, atrioventricular block, myocardial infarction, perivalvular leakage, or valve displacement during the perioperative period and 12-month follow-up.

Discussion

QAV was a rare congenital heart valve dysplasia with an incidence of approximately 0.0003-0.017%, slightly more in males than females [1, 2], according to Hurwitz and Roberts, quadricuspid aortic valves can be divided into seven types, of which type B (three equal large leaflets and one small valve) are the most common, and progressive aortic valve closure insufficiency is the main hemodynamic alteration and the reason for the need for surgical treatment at a later stage; simple aortic stenosis or dilatation of the ascending aorta is relatively uncommon [3, 4]. Patients with QAV have unequal size and position of the four aortic valve cusps, leading to an uneven distribution of hemodynamic stresses and incomplete or misaligned diastolic leaflets. This is thought to be an important reason for progressive aortic valve insufficiency. When the disease progresses and the leaflets are severely degenerated, surgical intervention is required, with aortic valve replacement being the most common approach [5]. Some studies report that aortic valvuloplasty is feasible, including the removal of malfunctioning valve leaflets and the “trileaflet valve” or “bicuspid valve” aortic valvuloplasty [6]. Idrees [7] described the surgical experience of 31 QAV patients, with a median follow-up time of 38 months and showing a favorable prognosis, and the other literature reports that QAV patients underwent aortic valve replacement surgery, with good cardiac function recovery and no late death [8, 9]. For elderly and high-risk patients with QAV malformations, traditional surgical aortic valve replacement may not be suitable. TAVI is considered an optional and effective surgical method [10–12].

QAV usually combined with other congenital defects, the most common being coronary artery opening malformation and dislocation, and abnormal coronary artery opening position is a high-risk factor for coronary artery obstruction during TAVI [13]. In patients with aortic valve regurgitation, the degree of valve calcification is relatively mild. The lack of leaflet calcification markers and insufficient annulus anchoring during TAVI surgery can significantly increase the risk of interventional valve displacement, perivalve leakage, and coronary artery occlusion.

The patient had a rare QAV malformation with a low position of the left and right coronary openings (both below 10 mm). The complex anatomical structure, uneven size, and shape of the aortic valve may result in inconsistent distribution of force on each valve, resulting in easy misalignment and dislocation after TAVI. It is difficult to fully visualize the four valve cusps in the two-dimensional view under DSA, which requires high requirements for optimal positioning of the valve implantation. This increases the risk of coronary ostium obstruction, for which the surgeon chose the transapical approach for J-Valve valve implantation. Compared with the transfemoral approach, the transapical approach is the only compliant TAVI route with a shorter distance to the aortic valve, providing a stable platform for TAVI and facilitating the valve crossing, determination of optimal position, and stability after valve implantation [14].

The J-Valve is an interventional valve for the treatment of aortic regurgitation. It consists of a retaining ring and a support frame with three anatomically positioned U-shaped “grippers”, which are uniquely designed to allow for precise anatomical positioning and secure anchorage during TAVI, as well as a movable interconnection between the retaining ring and the support frame. This unique design allows precise anatomical positioning and secure anchoring during TAVI, and the ring is interconnected with the support frame, the position of the valve can be adjusted up and down even after the ring is positioned in the aortic sinus, thus effectively preventing the leaflets obstructing the coronary ostium and lowering the risk of coronary artery obstruction and myocardial infarction.

There is a high incidence of comorbid CAD in patients undergoing TAVI, TAVI combined with PCI has a higher survival rate than PCI without in these patients [15]. Depending on the time of PCI, there are three different strategies: pre-TAVI, intra-TAVI, and post-TAVI. Each of the three modalitie has its own advantages and disadvantages, and the optimal modality is still unclear. TAVI combined with PCI one-stop treatment has the following advantages: (1) No additional vascular access is required; (2) Reduces the risk of vascular complications; (3) Reducing patient’s pain; (4) Rational use of medical resources. However, its radiation time and the contrast dosage are higher in one-stop treatment compared with staged strategy, which may increased risk of postoperative acute kidney insufficiency, increase the complexity of surgical operation as well as the radiation time of the surgeon [16]. In this case, the patient was treated with one-stop treatment, the time was not significantly different from the TAVI procedure alone, no postoperative acute kidney insufficiency, and the patient recovered and was discharged from the hospital on the fourth day after the procedure.

Since Cribier [17] completed the world’s first TAVI in 2002, TAVI has become an effective therapeutic option for patients with high risk of aortic valve, and the indications for the procedure are expanding as the technology continues to evolve [18], however, due to the rarity of QAV, the application of TAVI in the treatment of QAV rarely reported, and lack of experience. In this study, we report this case of a patient with QAV in combination with CAD, underwent one-stop treatment of TAVI and PCI, the results were satisfactory with a 12-months postoperative follow-up.

Acknowledgements

Not applicable.

Author contributions

Cheng Luo wrote the main manuscript text and Baoshi Zheng prepared Figures 1-3. All authors reviewed the manuscript.

Funding

This work was supported by the Enhancement Project for Teachers Foundation of Guangxi (No. 2022KY0101).

Data availability

No datasets were generated or analysed during the current study.

Declarations

Ethics approval and consent to participate

The study was approved by the Ethics Committee of the First Affiliated Hospital of Guangxi Medical University (No. 2022KY0101). The patient was anonymous and provided written informed consent before being recruited.

Consent for publication

The final version of the manuscript has been reviewed and approved by all.

Competing interests

The authors declare no competing interests.

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
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