Left-sided surgical approach for tricuspid valve replacement in congenitally corrected transposition of the great arteries with dextrocardia and situs inversus totalis: A case report

Abstract

Background

Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital cardiac anomaly characterized by atrioventricular and ventriculoarterial discordance, resulting in a physiologically corrected circulation. When ccTGA is combined with dextrocardia and situs inversus totalis, surgical management becomes particularly challenging because of the mirror-image anatomy and altered spatial orientation.

Case presentation

A 61-year-old man presented with progressive dyspnea and was diagnosed with ccTGA accompanied by dextrocardia and situs inversus totalis. Transthoracic echocardiography revealed severe regurgitation of the systemic atrioventricular valve (morphological tricuspid valve) with dilation and systolic dysfunction of the systemic right ventricle. Comprehensive preoperative imaging confirmed atrioventricular and ventriculoarterial discordance, right-sided cardiac apex, and complete visceral inversion. Tricuspid valve replacement was performed via a median sternotomy with the surgeon standing on the patient’s left side to accommodate the mirror-image anatomy. Cardiopulmonary bypass was established safely after meticulous identification of the great vessels. A mechanical prosthetic valve was successfully implanted. The postoperative course was uneventful, and follow-up echocardiography at six months confirmed satisfactory prosthetic valve function without complications.

Conclusions

This case illustrates that tricuspid valve replacement can be safely and effectively performed in patients with ccTGA complicated by dextrocardia and situs inversus totalis. A left-sided surgical approach, combined with thorough preoperative imaging and careful intraoperative anatomical verification, may help restore operative orientation and reduce technical complexity in patients with mirror-image cardiac anatomy.

Supplementary Information

The online version contains supplementary material available at https://doi.org/10.1186/s13019-026-04150-5.

Introduction

Congenitally Corrected Transposition of the Great Arteries (ccTGA) constitutes a rare and complex congenital cardiac anomaly, representing approximately 0.05% of congenital heart defects [1, 2]. Anatomically, this condition is characterized by ventricular inversion with corresponding transposition of the atrioventricular valves, along with abnormal ventriculoarterial connections. Although characterized by dual discordance, compensatory mechanisms within the circulatory system preserve physiological oxygenation sequence [3]. This case report presents a patient with ccTGA and dextrocardia. In addition to the classic features of ccTGA, cardiac imaging revealed right-sided thoracic cardiac displacement accompanied by a mirror-image atrial arrangement: the left atrium occupies the right-sided cardiac compartment, while the right atrium situates leftward. Severe systemic atrioventricular valve dysfunction necessitated tricuspid valve replacement.

Case report

A 61-year-old man was admitted following the acute onset of dyspnea and substernal chest oppression. The patient denied gastrointestinal symptoms, headache, or dizziness. Initial evaluation at a primary care center established diagnoses of ccTGA. Electrocardiography showed a sinus rhythm (69 bpm) with no evidence of atrioventricular block. Laboratory investigations revealed a markedly elevated serum level of brain natriuretic peptide (BNP) at 840 pg/mL. Complete blood count, hepatic and renal function panels, and serum electrolyte concentrations were all within normal limits. Cranial computed tomography revealed an absence of acute infarction or intracranial hemorrhage. Furthermore, coronary computed tomography angiography illustrated no hemodynamically significant luminal stenosis. Preoperative upright posteroanterior chest radiography revealed characteristic cardiac dextroposition, indicated by a rightward orientation of the cardiac apex (Fig. 1). The results of preoperative transthoracic echocardiography are showed in Fig. 2. This echocardiographic examination demonstrated dextrocardia with situs. The aorta arised from the anatomical right ventricle, while the pulmonary artery originated from the anatomical left ventricle. Consequently, the segmental analysis revealed dual discordance: the right atrium connected to a morphological left ventricle which gave rise to the pulmonary artery, while the left atrium drained into a morphological right ventricle that supported the aorta. Significant dilation of the anatomical right ventricle was apparent, with end-diastolic and end-systolic dimensions measuring 61 mm and 48 mm. Right ventricular systolic function was globally impaired, yielding an ejection fraction of 45%. In contrast, left ventricular dimensions and systolic function remained normal. Color Doppler Flow Imaging (CDFI) revealed severe eccentric tricuspid regurgitation into the right atrium during systole, with an estimated regurgitant jet area of 19.7 cm². Computed tomography (Fig. 3) showed dextrocardia and situs inversus totalis, featuring a rightward cardiac apex and transposition of abdominal organs with a left-sided liver and right-sided spleen.

Fig. 1.

Preoperative upright posteroanterior chest radiograph

Fig. 2.

Preoperative transesophageal echocardiography with color Doppler (midesophageal tricuspid commissural view)

Fig. 3.

Preoperative computed tomography. (A) Computed tomography imaging of the chest shows dextrocardia. (B) Computed tomography imaging of the abdomen shows situs inversus totalis. (Ao, aorta; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right. ventricle.)

The surgical procedure is demonstrated in Video 1. Following endotracheal intubation under general anesthesia, median sternotomy was performed for tricuspid valve replacement (Fig. 4). After systemic heparinization, cardiopulmonary bypass was established via ascending aortic cannulation and bicaval venous drainage. Given the mirror-image anatomy caused by dextrocardia with situs inversus, the operation was intentionally performed with the surgeon standing on the patient’s left side. The tricuspid valve was deemed unsuitable for repair. Following cardiac exposure, a right atriotomy was extended toward the interatrial septum, which permitted direct confirmation of both atrioventricular and ventriculoarterial discordance. After excising the diseased tricuspid valve, a mechanical prosthesis was implanted. The patient weaned off cardiopulmonary bypass smoothly. Transesophageal echocardiography revealed normal prosthetic valve hemodynamics and absence of paravalvular regurgitation. On postoperative day one, the patient was extubated without complications. Discharge was uneventful on postoperative day five. At the six-month follow-up, echocardiography demonstrated good valve function with no complications.

Fig. 4.

Intraoperative view of the patient. Ao, aorta; LV, left ventricle; RA, right atrium; RV, right ventricle; PA, pulmonary artery; SVC, superior vena cava

Discussion

The ccTGA with dextrocardia represents a rare congenital cardiac complex malformation. The fundamental pathoanatomy of this congenital malformation consists of atrioventricular discordance combined with ventriculoarterial discordance, accompanied by displacement of the cardiac axis into the right thoracic cavity [4]. On this basis, the patient successfully underwent tricuspid valve replacement via a left-sided surgical approach. This case demonstrates the technical feasibility of a left-sided surgical approach for managing systemic atrioventricular valve regurgitation in patients with situs inversus totalis and ccTGA.

The pathophysiological significance of the tricuspid valve within this complex malformation warrants further exploration [5]. In this unique physiology, the morphological right ventricle assumes the role of the systemic pumping chamber. Chronic exposure to elevated systemic afterload promotes tricuspid annular dilation, leaflet tethering, and dysfunction of the subvalvular apparatus, ultimately leading to significant regurgitation [4, 6]. Therefore, intervention for tricuspid valve dysfunction, particularly definitive treatment with replacement surgery, is essential to correct hemodynamic derangements and preserve systemic ventricular function. The most significant challenge and unique aspect of this case originate from its distinct anatomic spatial configuration [7]. In the setting of dextrocardia with situs inversus, the heart and great vessels exhibit a mirror-image relationship, which profoundly challenges the surgeon’s conventional spatial orientation and established operative techniques. In this case, the surgeon opted to operate from the patient’s left side. This strategic positioning was intended to align the spatial relationships of the cardiac chambers and major vessels with the surgeon’s familiar anatomy.

Aortic cannulation was technically demanding due to the altered spatial relationship of the great vessels. The main pulmonary artery is typically located anterior and to the left of the aorta, but in ccTGA with dextrocardia, the spatial relationship between the two great vessels may undergo unpredictable variations such as rotation and distortion. With the surgeon standing on the patient’s left side, the vascular course appears completely reversed from the conventional perspective. To ensure safety, meticulous exploration must be performed during surgery: carefully dissecting the great vessels within the pericardium, initially identifying them through palpation of their course, wall thickness, and pulsation; more importantly In addition, coordination with the perfusionist is essential to validate aortic cannula placement by analyzing cardiopulmonary bypass pump pressure waveforms and characteristic pressure gradients in real time, thus confirming its position in the systemic aorta rather than the pulmonary artery which connects to the pulmonary circulation. Positioning of the primary surgeon on the patient’s left side thereby satisfies the demands of mirror-image operation. Third, identification and protection of critical adjacent structures are essential [8]. Meticulous care must be taken during the procedure to avoid injury to the conduction system, which could result in cardiac block or damage to the coronary arteries. In contrast to normal hearts, the conduction tissue in ccTGA is characterized by congenital rearrangement, an ectopic atrioventricular (AV) node, and an abnormally superficial His bundle coursing along the morphologic right ventricular surface close to the tricuspid annulus, while the native AV node, normally located in the AV groove anteroinferior to the coronary sinus ostium at the atrial‑ventricular septal junction, is frequently hypoplastic, atretic, or nonfunctional, with a functional accessory AV node developing as the main conduction pathway in an anomalous region between the tricuspid annulus and coronary sinus ostium and some patients presenting with dual AV nodes, all of which creates an extremely high risk of intraoperative injury and postoperative complete atrioventricular block during systemic atrioventricular (morphologic tricuspid) valve replacement. To avoid such injury, surgical suturing must strictly adhere to core principles tailored to this anomalous anatomy: annular sutures should be placed mainly in the anterior, lateral, and left‑sided safe regions of the tricuspid annulus, whereas the postero‑septal annulus (corresponding to the ectopic AV node and His bundle course) and the triangle of danger between the coronary sinus ostium and the septal leaflet insertion require extreme caution, with deep suturing, trans‑septal needle passage, and excessive electrocoagulation strictly prohibited; needle penetration is limited to the superficial endocardial layer of the annulus [9, 10].

In summary, this case of ccTGA with dextrocardia was successfully managed with tricuspid valve replacement via a left-sided approach, highlighting the necessity of anatomical adaptation during cardiac surgery for extreme anatomical variants. A profound understanding of individualized pathological anatomy, and the adaptation of standard surgical positioning are pivotal to achieving successful outcomes.

Electronic Supplementary Material

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Abbreviations

ccTGA

Congenitally corrected transposition of the great arteries

TGA

Transposition of the great arteries

TV

Tricuspid valve

RV

Right ventricle

LV

Left ventricle

BNP

Brain natriuretic peptide

CT

Computed tomography

CTA

Computed tomography angiography

CDFI

Color Doppler flow imaging

Author contributions

CY and TT were involved in the conception and design of the study. CY, TT, JL, PS and HC contributed to data collection and/or analysis. CY and TT were involved in the construction of the manuscript. PS, JL, CR and YL critically revised the manuscript for intellectual content. HC and YL were responsible for full-text proofreading. All authors read and approved the final manuscript.

Funding

This study was funded by the National Natural Science Foundation’s Excellent Young Scientist Fund and the Excellent Young Scientist Program of Capital Medical University (KCA2305), Beijing Anzhen Hospital High Level Research Funding (2024AZC1003), Beijing Municipal Science and Technology Project (Grant No. Z251100004625015), and Chinese Institutes for Medical Research, Beijing (Grant No. CX23YQA05).

Data availability

The data that support the findings of this case are available from the corresponding author upon reasonable request.

Declarations

Competing interests

The authors declare no competing interests.

Ethics approval and consent to participate

This study was approved by ethics committee of Beijing Anzhen Hospital and conducted in accordance with the Declaration of Helsinki. Written informed consent was obtained from the patient for participation in this study and for the publication of this case report and any accompanying images or videos.

Consent for publication

Written informed consent was obtained from the patient for the publication of this case report and any accompanying images or videos.