Congenitally corrected transposition of the great arteries (CC-TGA) is a rare congenital heart disease (CHD) characterized by atrioventricular and ventriculoarterial discordance, accounting for 0.4% of all CHD [1,2]. Although the circulation is physiologically corrected, the morphological right ventricle (RV) functions as the systemic ventricle, predisposing patients to progressive systemic ventricular dysfunction, tricuspid regurgitation (TR), and conduction abnormalities [3,4]. Associated lesions such as ventricular septal defect (VSD) and pulmonary outflow obstruction are common; however, valvular aortic stenosis (AS) is rarely reported [5-7]. We present a complex adult case of CCTGA with severe valvular AS and complete heart block managed with a tailored interventional approach.

Survival into adulthood in patients with uncorrected CCTGA and associated cardiac defects is uncommon. This is largely due to early detection and timely intervention in developed countries, as well as attrition from complications such as systemic right ventricular failure and complete heart block. However, delayed diagnosis is still encountered in developing nations.

Our patient was a 45-year-old woman with previously undiagnosed CCTGA who had successfully completed three pregnancies, including one normal vaginal delivery and two cesarean sections. The late diagnosis of such a complex congenital heart disease highlights the challenges of early detection and the possibility of missed congenital cardiac lesions at the primary healthcare level.

Another unique aspect of our case is the rare combination of defects in an adult patient with CCTGA and the individualized treatment strategy adopted. Our patient presented with multiple coexisting lesions, including severe AS, a rare association in CCTGA, with only a handful of cases reported in the literature [6,7]. Syncope secondary to complete heart block made PPI mandatory. The principal management dilemma was whether to address the associated intracardiac defects, namely the VSD and AS.

An urgent multidisciplinary team (MDT) discussion was conducted, and three treatment options were considered:

(1) Surgical aortic valve replacement, tricuspid valve repair, VSD closure, and epicardial PPI;

(2) Transvenous PPI with BAV and

(3) Transvenous PPI alone.

The MDT decided against surgical intervention because of the high operative risk associated with systemic RV dysfunction and elevated PVRI. Cardiac catheterization was therefore planned to assess the severity of AS, quantify the VSD shunt, and proceed with pacemaker implantation. Hemodynamic assessment confirmed severe AS despite systemic RV dysfunction, along with a moderate-sized VSD and elevated PVRI. Consequently, the patient underwent BAV and dual-chamber permanent pacemaker implantation.

Although BAV in adults is generally considered a palliative procedure with less favorable long-term outcomes than valve replacement, it represented a reasonable strategy in this patient. Given the reduced life expectancy associated with CCTGA and systemic RV dysfunction (8,9), BAV provided symptomatic relief while avoiding the substantial risks of surgery.

As far as closure of the VSD was concerned, although the Qp:Qs ratio was >1.5, we decided against surgical closure. The primary reasons were the elevated PVRI, which significantly increased the operative risk, and the broader concern regarding the patient’s overall life expectancy in the setting of CCTGA in an adult. In such a scenario, the long-term benefit of VSD closure would likely be limited, and the potential risks of surgery outweighed the expected gains. Therefore, a conservative strategy was deemed more appropriate [8,9].

This case illustrates a rare and complex presentation of CCTGA with severe aortic stenosis and complete heart block in an adult. Given the high surgical risk, a palliative approach with balloon aortic valvotomy and dual-chamber PPI provided effective symptomatic relief and improvement in systemic RV function. Individualized, multidisciplinary management remains essential in such challenging cases.

Adult survival with uncorrected CCTGA associated with severe valvular AS is exceedingly rare. This case highlights the diagnostic and therapeutic challenges posed by such complex anatomy and emphasizes the importance of multidisciplinary decision-making. In carefully selected high-risk patients, a tailored transcatheter approach may provide meaningful symptomatic and hemodynamic benefit while avoiding the considerable risks of surgical intervention.

The authors confirm that written informed consent was obtained from the patient for publication of this case report and any accompanying images. The patient has been informed that no personal identifiers will be disclosed, and every effort has been made to ensure confidentiality.

The authors declare that there are no conflicts of interest regarding the publication of this case report. No financial or personal relationships influenced the work reported in this manuscript.

This case highlights a rare adult presentation of uncorrected CCTGA with an unusual combination of severe valvular AS and complete heart block. It adds to the limited literature on coexisting left-sided obstructive lesions in CCTGA and illustrates the challenges of managing multiple high-risk lesions in a single patient. Importantly, it demonstrates the role of individualized, multidisciplinary decision-making and supports a tailored transcatheter strategy—BAV combined with PPI—as a feasible alternative in selected high-risk surgical candidates.