Transcatheter Aortic Valve Implantation in a Patient with Dextrocardia Situs Inversus, and Functional Bicuspid Aortic Valve Stenosis

INTRODUCTION

Transcatheter aortic valve implantation (TAVI) is now a therapeutic option for symptomatic severe aortic stenosis.¹ Dextrocardia with situs inversus is a very rare congenital heart disease with an incidence rate of one in 12,000. This report presents the case of a 75-year-old woman who had dextrocardia situs inversus, severe aortic stenosis with functional bicuspid valve, and tortuous aorta. She underwent successful TAVI with a SAPIEN 3 valve (Edwards Lifesciences, Irvine, CA) through a transfemoral approach.

CASE REPORT

A 75-year-old woman with dextrocardia situs inversus was admitted to our hospital with dyspnea. On cardiac auscultation, the patient had a grade 3 systolic murmur predominantly located in the second left intercostal space. A standard 12-lead electrooculogram (ECG) demonstrated a sinus rhythm, a right axis deviation, and inverted p and T waves in lead I, with biphasic T waves and progressively decreasing R-wave amplitude from leads V1 to V6 (Figure 1A). Results of a repeat ECG obtained after mirror position corrected precordial lead placement revealed a normal R-wave progression in the precordial leads (Figure 1B). The ECG also showed a complete right bundle branch block. Chest radiograph demonstrates the cardiac apex and aortic arch on the right side of the chest, consistent with dextrocardia and situs inversus (Figure 1C). Echocardiography showed a severely calcified aortic valve with a mean gradient of 42 mmHg and normal left ventricular systolic function (ejection fraction of 68%). Coronary angiography revealed no significant stenosis of coronary arteries. The heart-team recommended TAVI rather than open surgery considering her age and moderate surgical mortality risk (logistic EuroSCORE 11.7% and Society of Thoracic Surgeons score 4.1%). The pre-operative computed tomography (CT) showed dextrocardia with a right-sided aorta and left-sided inferior vena cava (Figure 1D). She had type 1 functional bicuspid aortic valve stenosis with a raphe of the right and non-coronary cusps and heavily calcification over left coronary cusp (Figure 1E). The mean aortic annulus diameter was 22.5 mm, the perimeter was 72.8 mm, with an area of 397 mm² (Figure 1F). The sinus of Valsalva diameter was 30.3, 27.1, and 29.3 mm for the left, right, and noncoronary cusps, respectively (Figure 1G). The coronary height was adequate (12.3 and 12.0 mm for the left and right coronary arteries, respectively; Figure 1H-I). The aortic root angle was 68° with a 15° left anterior oblique and 8° caudal angulation of coaxial implantation plane (Figure 1J). The virtual image of a 23-mm Sapien 3 valve at 7 mm above the annulus is shown in Figure 1K. The CT showed suitable iliofemoral artery anatomy but severely tortuous descending and abdominal aorta without obvious calcification (Figure 2F-H). These anatomic characteristics revealed that a 23-mm balloon-expandable Sapien 3 valve (Edwards Lifesciences, Irvine, CA) would be the treatment option.

Figure 1.

Patient details. (A) Standard ECG. (B) ECG obtained after corrected precordial lead placement according to mirror position. (C) The chest radiograph demonstrates the cardiac apex and aortic arch on the right side of the chest. (D) 3-dimensional image of the right-sided heart. (E) CT scan showed type 1 functional bicuspid aortic valve with a raphe of the RCC and NCC. (F) Annular sizing of 22.5 mm. (G) Sinus of Valsalva sizing. (H) Left coronary height. (I) Right coronary height. (J) Implantation view. (K) Virtual image of a 23-mm Sapien 3 valve at 7 mm above the annulus (blue circle). CT, computed tomography; ECG, electrooculogram; LCC, left coronary cusp; NCC, non-coronary cusp; RCC, right coronary cusp.

Figure 2.

Procedure details. (A-B) CT scan showed severely tortuous descending and abdominal aorta in anterior posterior view (A) and lateral view (B). (C) 3-dimensional image of the tortuous aorta in RAO 40 view. (D, F) The Commander delivery system in normal usage with Edward logo faces upwards and standard articulation of catheter. (E) Edwards logo was rotated 180° downward with one marker (yellow arrow) on the upwards side. (G) Catheter articulation to the opposite side which facilitates passage through the right-sided aortic arch. (H) Positioning of the valve prosthesis with temporary pacing lead placed in the right ventricle (yellow arrow). (I) Aortogram after deployment of 23-mm Edwards S3 valve. RAO, right anterior oblique.

TAVI was performed under general anesthesia with transesophageal echocardiography (TEE) used for intraoperative guidance. A temporary pacing lead was placed in the right ventricle through left femoral vein. A 14 Fr E-sheath (Edwards Lifesciences) was inserted from the right femoral artery through Amplatz Extra-Stiff Wire (Cook Medical, Bloomington, IN). The aortic valve was crossed using an Amplatz left (AL-1) catheter and a 0.038 straight wire. Subsequently, a curved stiff Safari wire (Boston Scientific, Natick, MA, USA) was placed in LV. To prevent possible severe aortic regurgitation, balloon aortic valvuloplasty was performed with a 12 mm Mustang balloon (Boston) under rapid pacing. Because of the right-sided aortic arch in dextrocardia, we rotated the commander delivery system 180° leaving the Edward logo in a posterior direction before insertion into the E-sheath. This allows the delivery catheter to articulate toward the opposite curve and advanced the inverted aortic arch in dextrocardia (Figure 2A-2D). We have further put a marker on the upwards side of the commander delivery system which helps a clear understanding of the rotating condition of the delivery system. Because of the functional bicuspid anatomy with heavy calcification over left coronary cusp, we took two strategies to facilitate the procedure. Firstly, a 23 mm Sapien 3 valve was implanted in a high position with the bottom of the central marker upper to the base of the cusp during a brief period of rapid pacing (Figure 2H). Secondly, the valve was under-deployed (nominal volume minus 2 mL) initially. After valve implantation, a mild paravalvular aortic regurgitation from the previous left coronary cusp position was noted and post-dilatation using delivery system 23 mm-balloon minus 1 mL of contrast was performed. The final aortography and TEE showed an excellent valve position with trivial paravalvular leak (PVL). A transthoracic echocardiography 3 days later confirmed a good result with only trivial PVL. The patient was discharged 5 days later with uneventful post-TAVI course.

DISCUSSION

Dextrocardia is a very rare congenital heart disease with an incidence rate of about one in 12,000 live births.¹ To our knowledge, this is the first reported case of dextrocardia situs inversus to have TAVI in Taiwan. There were only few cases of TAVI in patients with dextrocardia situs inversus been reported.^2-13 Of this, one third received self-expansible aortic valve prosthesis (CoreValve or CoreValve Evolut R) and 2/3 with balloon-expandable devices (SAPIEN XT or SAPIEN 3 valve) (Table 1). Most of the cases (83%, 10 of the 12 cases) received TAVI from the trans-femoral vascular approach. In one-third (4 of the 12 cases) of the cases, the left and right reversed X-ray images were used to perform TAVI. Moreover, 16% (2 of the 12 cases) of the cases developed procedure-related complications, including one case of late cardiac perforation and one case of complete atrioventricular block. Compared with previous cases, the current case is the only one with function bicuspid anatomy and has the most tortuous aorta which made the TAVI intervention more complicated and challenging. In the current case, we choose to use the balloon-expandable SAPIEN 3 valve instead of the self-expanding aortic valve for two reasons. First, the patient had a severely tortuous descending and abdominal aorta, and we judged that the distal flex function of the commander delivery system of SAPIEN 3 valve would help the catheter pass through the extremely tortuous vascular route. Second, the patient had horizontal heart anatomy with an aortic root angle of 68°, which is close to the 70° upper limit of angulation for self-expanding aortic valves, such as CoreValve or Evolut R. In the case, the tip of the 36 cm Edward sheath could be advanced through and overcame the first tortuous abdominal aorta. Because there was no obvious calcification on the thoracic aortic wall, with the stiff Safari wire, the delivery system can pass smoothly through the tortuous thoracic aorta. One important issue for successful TAVI in this case is rotation of 180° with the Commander delivery system and the Edwards logo downwards before insertion into the sheath. We further put a marker on the upwards side of the delivery system which made the operator have a better understanding of the rotating condition of the delivery system when performing TAVI in patients with dextrocardia.

Table 1. Summary of case reports of TAVI in dextrocardia with situs inversus.

Author	Year	Age	Gender	Valve type	THV type	Valve size	Vascular access	Reverse image used	Complication
Weich3	2012	66	Female	Tricuspid	SAPIEN XT	23 mm	Transapical	nil	nil
Dubois4	2012	84	Female	Tricuspid	SAPIEN XT	26 mm	Trans-femoral	nil	nil
Romaguera5	2013	78	Male	Tricuspid	CoreValve	29 mm	Trans-femoral	nil	Late perforation by the pacemaker leads, possibly related to mispositioning
Good6	2014	63	Male	Tricuspid	SAPIEN XT	29 mm	Trans-femoral	nil	nil
Agrawal7	2016	84	Male	Tricuspid	SAPIEN XT	29 mm	Transapical	nil	nil
Morita8	2018	84	Male	Tricuspid	CoreValve Evolut R	26 mm	Trans-femoral	Yes	nil
Alrifai9	2018	89	Male	Tricuspid	SAPIEN 3 valve	23 mm	Trans-femoral	nil	nil
Pierpaolo12	2018	80	Male	Tricuspid	CoreValve Evolut R	29 mm	Trans-femoral	nil	nil
Overtchouk2	2018	71	Male	Bicuspid (type 0)	SAPIEN 3 valve	26 mm	Trans-femoral	nil	nil
Kawaguchi13	2018	94	Male	Tricuspid	SAPIEN XT valve	26 mm	Trans-femoral	Yes	Complete AVB developed 9 days after TAVI
Pattakos10	2019	82	Male	Tricuspid	SAPIEN 3 valve	29 mm	Trans-femoral	Yes	nil
Malasana11	2019	81	Female	Tricuspid	CoreValve Evolut R	26 mm	Trans-femoral	Yes	nil

AVB, atrioventricular block; TAVI, transcatheter aortic valve implantation; THV, transheart valve.

In this report, according to the sizing chart, the 23-mm SAPIEN 3 valve was a feasible option for a patient having a mean aortic annulus diameter of 22.5 mm with an area of 397 mm². Because of functional bicuspid aortic valve anatomy, the virtual image of a 23-mm Sapien 3 valve at 7 mm above the annulus was taken during pre-TAVI evaluation. Due to the calcified type 1 functional bicuspid aortic valve, two treatment strategies were taken to facilitate the procedure. Firstly, the Sapien 3 valve was implanted in a high position, considering the leaflet extremities rather than the annulus, and to reduce the need for permanent pacemaker.² Secondly, the SAPIEN 3 valve was under-deployed initially, i.e., nominal volume minus 2 mL. This makes the valve more compliant to avoid annular rupture. Followed by post-dilatation using the delivery system 23 mm-balloon inflated with nominal volume minus 1 mL, the final angiography showed excellent result with only trivial PVL. In conclusion, insertion of the Commander delivery system into the sheath after 180° rotation with an additional marker on the upward side of the delivery system, high implantation with initial under-deployed of the SAPIEN 3 valve facilitated valve implantation in the patient with dextrocardia situs inversus and functional bicuspid severe aortic stenosis.

LEARNING POINTS

• Dextrocardia is a very rare congenital heart disease. This is the first reported case of dextrocardia situs inversus to have TAVI in Taiwan.

• The following procedures can be followed to facilitate valve implantation in patients with dextrocardia situs inversus and functional bicuspid severe aortic stenosis: inserting the Commander delivery system into the sheath after rotation by 180°, using an additional marker on the upside of the delivery system, and ensuring high implantation with initial under-deployed of the SAPIEN 3 valve.

ETHICS STATEMENT

This study was approved by the Institutional Review Board, Chang Gung Memorial Hospital following the Helsinki Declaration (IRB: 202002092B0). The requirement for informed consent was waived.

CONFLICT OF INTEREST

All the authors declare no conflict of interest.