Simultaneous transcatheter management of incidental critical left main coronary artery disease in a young woman undergoing atrial septal defect device closure

Abstract

Transcatheter device closure is the preferred treatment for secundum atrial septal defect (ASD) in young adults with suitable anatomy. In this population, routine coronary angiography is generally not recommended in the absence of ischemic symptoms or conventional cardiovascular risk factors. However, coronary imaging is selectively performed during ASD intervention to delineate anomalous coronary anatomy at risk of device-related compression, and emerging evidence indicates that clinically significant coronary artery disease may occasionally coexist silently in younger adults. We report the case of a 28-year-old asymptomatic woman undergoing transcatheter closure of a large secundum ASD with deficient rims, in whom coronary angiography performed during invasive catheterization unexpectedly revealed critical left main coronary artery stenosis. Intravascular ultrasound (IVUS) confirmed severe luminal compromise, and successful IVUS-guided left main coronary stenting was performed, followed by device-assisted ASD closure in the same procedural session. Retrospective clinical assessment revealed a history of premature sudden cardiac death in a first-degree relative. This case illustrates the potential for occult yet prognostically significant coronary artery disease in young adults undergoing congenital heart interventions and supports a selective, individualized, and context-driven approach to coronary evaluation, consistent with contemporary adult congenital heart disease guidelines.

INTRODUCTION

Secundum atrial septal defect (ASD) is commonly diagnosed in young adults and is increasingly treated by transcatheter device closure. In this age group, invasive coronary angiography is generally not performed in the absence of ischemic symptoms or conventional cardiovascular risk factors, reflecting the low reported prevalence of obstructive coronary artery disease in patients with isolated ASD.[1,2]

In selected procedural contexts, however, coronary imaging may be considered during ASD device closure to delineate coronary anatomy, particularly when large occluder devices are planned or septal rims are deficient, in order to exclude coronary anomalies that could be affected by device deployment.[3] The incidental identification of clinically significant coronary artery disease under such circumstances is uncommon. We report a young woman in whom critical left main coronary artery (LMCA) disease was unexpectedly identified and managed during transcatheter ASD device closure.

CASE REPORT

A 28-year-old woman (weight 55 kg) was evaluated for an incidentally detected secundum ASD. She reported mild effort intolerance but had no history of chest pain or other conventional cardiovascular risk factors. Physical examination revealed normal blood pressure and oxygen saturation. The first heart sound was normal, and there was fixed splitting of the second heart sound with a grade 2/6 systolic murmur at the pulmonary area.

Electrocardiography demonstrated sinus rhythm with right-axis deviation and incomplete right bundle branch block, without ischemic changes [Figure 1]. Chest radiography showed evidence of right-sided volume overload. Transthoracic and transesophageal echocardiography (TEE) confirmed a large secundum ASD with right atrial and right ventricular enlargement and preserved biventricular function. Transesophageal imaging revealed a thin, aneurysmal interatrial septum with a floppy and relatively deficient posteroinferior rim, rendering device closure technically challenging [Figure 2a and b].

Figure 1.

Twelve-lead electrocardiogram showing sinus rhythm at a rate of 86 beats per minute, PR interval of 183 ms, QRS axis of 65°, and a right bundle branch block pattern, with no evidence of ischemic changes

Figure 2.

(a and b) Show transesophageal echocardiography (TEE) demonstrating a large secundum atrial septal defect (ASD) (yellow arrow) with a thin, floppy interatrial septum and a deficient posteroinferior rim. (c) Illustrates device-assisted ASD closure, with the assisting device indicated by a black arrow and the therapeutic occluder by a green arrow. (d) Shows the post-deployment TEE image confirming appropriate positioning of the ASD device (green arrow). (e) Demonstrates the post-release fluoroscopic image with the device in a satisfactory position (green arrow). (f and g) shows the follow-up transthoracic echocardiogram confirming stable device position with no residual interatrial shunt (green arrow). (g) depicts a parasternal short-axis echocardiographic view demonstrating the left main coronary artery stent in situ (red arrow)

Given the anatomy, a device-assisted device (DAD) closure technique was planned, with surgical backup available.[4] The patient was preloaded with aspirin (325 mg). Cardiac catheterization was performed under local anesthesia using dual femoral venous access and femoral arterial access. Hemodynamic assessment demonstrated normal pulmonary artery pressures (24/6 mmHg; mean 13 mmHg) and equal mean atrial pressures (3 mmHg).

As per the institutional protocol for adult ASD intervention, coronary angiography was routinely performed before device closure to delineate coronary anatomy and exclude anomalous coronary arteries that could potentially be affected by deployment of a large occluder device. Angiography unexpectedly revealed critical LMCA stenosis, with a normal dominant right coronary artery and no significant disease in the left anterior descending or circumflex arteries [Figure 3a-d]. Initially, it was felt that the left main narrowing could be due to coronary spasm, considering the low risk for atherosclerosis in her and the absence of disease elsewhere in the coronary tree. Intracoronary nitrate was administered, but there was no change in the angiographic findings. Hence, it was decided to document the disease by intravascular ultrasound (IVUS).

Figure 3.

Coronary angiography. (a) Shows a normal right-dominant coronary circulation without significant stenosis. (b-d) Demonstrate critical narrowing of the left main coronary artery in different angiographic projections, with bifurcation into the left anterior descending and left circumflex arteries; both branches show no significant luminal narrowing. (e) Shows the left main coronary artery following stent deployment with restoration of coronary flow. (f) Demonstrates the final angiographic result after post-dilatation of the left main stent, showing well-established flow

The patient was intubated and mechanically ventilated, and a loading dose of clopidogrel was administered. Following multidisciplinary Heart Team discussion involving the pediatric cardiology team, adult interventional cardiologist, cardiac surgeon, and cardiac anesthesiology team, the angiographic and IVUS findings were reviewed and both surgical and percutaneous treatment strategies were considered. Given the isolated ostial left main lesion, absence of additional significant coronary artery disease, and favorable anatomy for percutaneous coronary intervention (PCI), the consensus recommendation was to proceed with IVUS-guided PCI with surgical backup available, if required. The IVUS clearly showed more than 90% plaque burden at the ostium of the left main, and the minimum lumen area was 3.14 mm² [Figure 4a]. IVUS-guided PCI was performed. The lesion was predilated with a 3.0 mm × 10 mm noncompliant balloon, followed by implantation of a 4.0 mm × 12 mm drug-eluting stent and postdilatation with a 4.5 mm × 6 mm balloon [Figure 3e and f]. Final IVUS demonstrated optimal stent expansion, with a minimal stent area of 11.16 mm² and angiographic TIMI Grade III flow [Figure 4b-f].

Figure 4.

Intravascular ultrasound (IVUS) of the left coronary artery system. (a) Demonstrates critical luminal narrowing of the left main coronary artery. (b) Shows changes in the left main coronary artery following initial pre-dilatation. (c) Depicts the left main coronary artery ostium after stent deployment with adequate luminal expansion. (d) Demonstrates satisfactory luminal opening at the left anterior descending artery level following left main intervention. (e and f) Show the final IVUS images after post-dilatation with balloon inflation, confirming optimal expansion of the left main coronary artery and left anterior descending artery

Transcatheter ASD closure was subsequently undertaken in the same session using a 38-mm Cocoon occluder (Shahjanand Medical Technologies, India) with the DAD technique [Figure 2c]. Two deployment attempts were required to achieve stable left atrial disc alignment across the deficient posteroinferior rim. TEE confirmed secure device position and complete closure without residual shunt [Figure 2d]. The patient was extubated on the table and remained hemodynamically stable. The coronary intervention was performed by the adult interventional cardiology team, while the device-assisted ASD closure was subsequently completed by the interventional pediatric cardiology team as the part of a coordinated multidisciplinary approach.

Postprocedural echocardiography demonstrated stable ASD device position and visualization of the LMCA stent in the parasternal short-axis view [Figure 2f and g]. Retrospective history revealed sudden cardiac death in the patient’s father at 42 years of age. The patient was discharged on dual antiplatelet therapy. There was a small pseudoaneurysm at the left femoral puncture site detected after 4 weeks, which resolved with manual compression. At a 3-month follow-up, the patient remained asymptomatic. Echocardiography demonstrated stable ASD device position without residual shunt, and there was no clinical evidence of recurrent myocardial ischemia. She continues to be followed jointly by the pediatric cardiology and adult cardiology teams while receiving dual antiplatelet therapy and statin treatment.

DISCUSSION

Transcatheter device closure is the preferred treatment for secundum ASD in adults when anatomical criteria are suitable. However, large defects with deficient rims or excessive septal mobility remain technically challenging and are associated with an increased risk of device instability. In such anatomies, adjunctive techniques have been developed to facilitate controlled device deployment, among which DAD closure has demonstrated feasibility and safety in large and complex ASDs.[4] In the present case, this technique was selected because of the large defect size and rim deficiency.

The use of large occluder devices in the presence of deficient septal rims, particularly near the anterosuperior margin, raises concern for potential compression of coronary arteries coursing adjacent to the aortic root. Multiple reports have emphasized the importance of delineating coronary anatomy before or during device deployment to prevent ischemic complications related to coronary compression.[3] In this procedural context, coronary angiography serves as an anatomical safeguard rather than a screening investigation.

Coronary angiography is not routinely recommended in patients younger than 40 years undergoing ASD device closure in the absence of ischemic symptoms or conventional cardiovascular risk factors. Earlier studies evaluating patients with isolated secundum ASD reported a low prevalence of angiographically significant coronary artery disease.[1,2] Yalonetsky et al. identified angiographically confirmed atherosclerotic coronary artery disease in 1% of patients, with ASD representing one of the most common underlying congenital diagnoses. Notably, 14% of affected patients developed premature coronary artery disease before the age of 40 years, and more than one-third were asymptomatic at presentation, highlighting the potential for clinically silent but significant coronary disease in adults with congenital heart defects and supporting an age-based selective approach.[5]

However, the incidental detection of critical LMCA disease in this patient highlights the limitations of symptom-based risk stratification. Retrospective identification of premature sudden cardiac death in a first-degree relative further suggested the possibility of an underlying predisposition to premature coronary artery disease that was not apparent during the initial clinical assessment. In patients with ASD, angina correlates poorly with angiographically significant coronary artery disease, and clinically important lesions may be identified incidentally during catheterization performed for other indications.[6]

Population-based studies from Indian cohorts have demonstrated a nontrivial prevalence of premature coronary artery disease, often occurring in the absence of conventional cardiovascular risk factors.[7] These findings indicate that young age alone does not reliably exclude clinically meaningful coronary disease, particularly in regions with a high burden of early atherosclerosis.

LMCA disease, although uncommon in young adults, carries disproportionate prognostic significance because of the large myocardial territory at risk. Once significant LMCA disease is identified, accurate lesion assessment and optimization of revascularization are critical. Angiography alone may underestimate lesion severity and vessel dimensions in left main disease. IVUS enables precise evaluation of plaque burden, minimal luminal area, and stent expansion and is increasingly regarded as essential for contemporary LMCA intervention.[8] In the present case, IVUS guidance ensured optimal stent deployment before proceeding with ASD closure.

The choice of revascularization strategy for significant LMCA disease should be individualized and guided by lesion complexity, patient characteristics, and multidisciplinary assessment. In the present case, the lesion was confined to the ostium of the LMCA, without associated multivessel coronary disease and was unexpectedly identified during an ongoing congenital heart intervention. Following multidisciplinary discussion involving the congenital/pediatric cardiology, adult interventional cardiology, and cardiac surgical teams, IVUS-guided PCI was considered an appropriate treatment strategy. Contemporary evidence from randomized studies has demonstrated that, in selected patients with low-complexity LMCA disease, PCI can provide long-term outcomes comparable to surgical revascularization, particularly when guided by intravascular imaging and performed by experienced operators. In the present patient, successful IVUS-guided left main stenting allowed definitive treatment of the coronary lesion while facilitating the completion of transcatheter ASD closure during the same procedural session.[9,10]

Simultaneous transcatheter management of coronary artery disease and ASD has been reported infrequently but supports the feasibility of addressing both conditions within a single procedural setting when clinical stability and expertise permit.[11] In this patient, IVUS-guided LMCA stenting followed by device-assisted ASD closure allowed definitive treatment of both acquired and congenital pathology without the need for staged procedures or surgical intervention.

This approach aligns with contemporary guideline philosophy. The 2025 ACC/AHA Guideline for the management of adults with congenital heart disease emphasizes individualized, context-driven coronary assessment during invasive procedures rather than rigid age-based exclusion.[12] Rather than advocating routine coronary angiography in young adults, the present case illustrates how selective coronary evaluation during ASD intervention can uncover clinically significant disease and permit effective transcatheter management within the same procedural setting.

CONCLUSION

This case demonstrates that clinically silent yet prognostically significant coronary artery disease may coexist in young adults undergoing transcatheter ASD closure. Although routine coronary angiography in patients younger than 40 years is not supported, selective coronary evaluation performed within an appropriate procedural context can uncover unexpected high-risk pathology and directly influence management. Careful clinical assessment, including evaluation of cardiovascular risk factors and relevant family history, may provide additional contextual cues. When such disease is identified, contemporary transcatheter techniques allow safe and effective treatment within the same procedural setting. Overall, this case supports an individualized, anatomy- and context-driven approach to coronary assessment during congenital heart interventions rather than reliance on age-based exclusion alone.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.