Abstract
Background
Coexisting coronary artery disease (CAD) and congenital heart disease (CHD) is rare in adults and poses complex management challenges requiring multidisciplinary coordination.
Case Summary
A 79-year-old man with a history of CAD and stenting was referred for evaluation given worsening dyspnea. Coronary angiography revealed severe ostial left circumflex stenosis and moderate left main disease. In addition, computed tomography demonstrated a previously undiagnosed sinus venosus atrial septal defect with partial anomalous pulmonary venous return. Multidisciplinary evaluation at a partnering CHD program recommended staged percutaneous revascularization and transcatheter closure of the defect instead of surgical repair on cardiopulmonary bypass. The patient successfully underwent complex stenting of the CAD and subsequent transcatheter closure of the sinus venosus atrial septal defect with implantation of a 5.7-cm, 10-zig G-Armor covered stent within the superior vena cava. The procedures were well tolerated without complications, and the patient reported improvement in symptoms on follow-up evaluation.
Take-Home Messages
Multidisciplinary percutaneous strategies can achieve effective revascularization and repair of complex coexisting CAD and CHD, avoiding high-risk surgical intervention. Growth in the complexity of CHD patients mandates expanded understanding of comprehensive percutaneous approaches. The hub-spoke model of adult CHD care can optimize treatment for these patients.
Key words: atrial septal defect, congenital heart disease, percutaneous coronary intervention
Visual Summary
Advances in pediatric and adult cardiovascular care have resulted in a growing population of adults with congenital heart disease (CHD). Many of these patients now survive into middle and older age and can develop acquired cardiovascular conditions such as coronary artery disease (CAD). The coexistence of CAD and previously undiagnosed CHD presents unique diagnostic and therapeutic challenges. Sinus venosus atrial septal defects (SVASDs), frequently associated with partial anomalous pulmonary venous return (PAPVR), may remain clinically silent until adulthood, when symptoms such as dyspnea or exercise intolerance prompt further investigation. In some cases, these defects are incidentally identified during evaluation for unrelated cardiac conditions, including surgical planning for coronary revascularization.
Management of patients with concurrent CAD and CHD requires careful integration of multimodality imaging, invasive hemodynamic assessment, and multidisciplinary collaboration. The evaluating team should ideally include adult and congenital interventional cardiologists, as well as adult and congenital surgeons. Higher level coordination between these specialists is best facilitated by trained adult congenital heart disease (ACHD) specialists. While surgical repair was historically the standard approach for treatment of nearly all heart disease, evolving percutaneous techniques now permit minimally invasive management of both coronary and congenital pathology in selected patients. Given the limited number of accredited ACHD centers and the growing geographic separation between patients and specialized care, innovative delivery models are increasingly necessary. Hub-spoke ACHD frameworks extend subspecialty expertise beyond tertiary centers by enabling the coordinated multidisciplinary evaluation needed for complex interventions.
Case Summary
A 79-year-old man presented with significant shortness of breath and decrease in stamina. The patient underwent bifurcation stenting of the first diagonal branch and the left anterior descending artery (LAD) with minimal protrusion into the left main artery (LM). He later developed progressive angina and exertional dyspnea. Repeat coronary angiography demonstrated severe (90%) ostial left circumflex artery (LCx) stenosis and moderate LM disease (Video 1). The patient was initially referred for surgical coronary artery bypass.
Take-Home Messages
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Multidisciplinary, staged percutaneous strategies can achieve effective revascularization and repair of complex coexisting CHD, avoiding high-risk surgical intervention.
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Growth in complexity in CHD patients mandates expanded understanding of comprehensive percutaneous approaches.
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The hub-spoke model of adult CHD care can facilitate coordinated treatment for these patients.
Preoperative contrast computed tomography demonstrated the undiagnosed findings of the right and left superior vena cava (SVC) and an SVASD with PAPVR of the right upper and middle pulmonary veins draining into the right SVC (Video 2). The SVASD resulted in a communication between the left and right atria, causing a physiology similar to an atrial septal defect. The patient was referred for CHD consultation with a partnering ACHD-accredited center, and the multidisciplinary heart team recommended staged percutaneous management of both issues. Percutaneous coronary intervention using a reverse culotte technique with intravascular ultrasound (IVUS) guidance achieved optimal revascularization of the LM-LCx bifurcation. Subsequently, transcatheter closure of the SVASD was performed using a covered stent deployed from the right SVC into the right atrium. Both procedures were uneventful, and the patient reported significant symptomatic improvement at immediate postprocedure follow-up and 1 year later.
Procedural Steps
A diagnostic cardiac catheterization revealed normal right- and left-sided filling pressures with mildly elevated mean pulmonary artery pressure (33 mm Hg) and pulmonary vascular resistance of 2.5 WU, consistent with mild precapillary pulmonary hypertension.1 Oximetry demonstrated a significant step-up from the high SVC (66%) to the right atrium (84%), with a calculated pulmonary-to-systemic flow ratio of 1.9, indicating a hemodynamically significant left-to-right shunt.
Percutaneous coronary intervention of the LM-LCx bifurcation was performed with IVUS guidance. IVUS of the LM and LAD demonstrated mild proximal underexpansion of the stent, with minimal protrusion into the distal LM and no significant neointimal hyperplasia. The LM segment stent was underexpanded with calcific atherosclerotic disease. LCx and LM bifurcation was stented using the reverse culotte technique: The LM stent strut was wired into the LCx and dilated, and the LM was stented into the proximal LCx with a 3.5-mm Onyx Frontier stent (Medtronic). Final optimization included sequential postdilation, kissing balloon inflation, and proximal optimization (Figure 1).
Figure 1.
Stenting of the Left Main Artery Into the Proximal Left Circumflex Artery Using a 3.5-mm Onyx Frontier Stent With Sequential Postdilation and Kissing Balloon Inflation
Repeat IVUS demonstrated well-expanded and well-apposed stents with minimal stent area >10 mm2. Final angiography confirmed no residual stenosis and TIMI distal flow grade 3. The patient tolerated the procedure without complication.
Four weeks after percutaneous coronary intervention, the patient underwent transcatheter closure of the SVASD. Venous access was obtained via the right femoral vein and right internal jugular vein. A 0.035-inch Rosen wire (Cook Medical) was advanced from the right femoral vein and subsequently snared from the right internal jugular vein using a 20-mm En Snare catheter (Merit Medical), creating a stable veno-veno loop. A 20 mm × 3 cm Z-Med II balloon (B. Braun) was advanced over the wire into the right SVC and inflated to 4 atm to determine the exact size of the vessel. During inflation of the balloon within the right SVC, selective angiography via a 7-F wedge catheter positioned in the right upper pulmonary vein confirmed unobstructed pulmonary venous drainage (Video 3). Transesophageal echocardiography confirmed the absence of pulmonary venous obstruction.
A 5.7-cm, 10-zig G-Armor covered stent (B. Braun) was mounted on an 18 mm × 4 cm balloon-in-balloon catheter. A 22-F 65-cm DrySeal sheath (W.L. Gore) was inserted into the right femoral vein and was advanced carefully over the wire to the mid right SVC. The covered stent was positioned across the SVASD, where it was implanted from the SVC into the right atrium, successfully excluding the defect with only trivial residual shunting (Video 4).
Given mild protrusion of the stent into the right atrium, the proximal segment was further dilated using a 23 mm × 3 cm Tyshak II balloon catheter (B. Braun), achieving complete elimination of the residual shunt (Video 5). Follow-up angiography and transesophageal echocardiography confirmed stable stent position, unobstructed pulmonary venous return, and no residual atrial-level shunting, completing the procedure without complication (Video 6).
Potential Pitfalls
In patients with significant LM or bifurcation coronary disease, inadequate lesion preparation, suboptimal stent expansion, or incomplete intravascular imaging guidance may lead to restenosis, stent thrombosis, or ischemic complications, particularly in elderly patients with heavily calcified vessels. In this population, careful use of IVUS to optimize stent sizing and expansion is critical. Additionally, sequencing of interventions is important, as untreated ischemia or unstable coronary anatomy at the time of the congenital cardiac intervention may increase procedural risk owing to hemodynamic instability or arrhythmia during the prolonged procedure.
One of the most critical complications of transcatheter SVASD closure using covered stents is inadvertent obstruction of the anomalous pulmonary veins as they enter the distal right SVC–right atrial junction. Inadequate delineation of pulmonary venous anatomy, precisely as to its entry into the right SVC, may result in partial or complete pulmonary venous occlusion, leading to pulmonary congestion, hypoxemia, or pulmonary hypertension. Preprocedural computed tomography, intraprocedural selective pulmonary venous angiography, and transesophageal echocardiographic guidance are essential to confirm unobstructed venous return before and after stent deployment.
Stent migration within the SVC has been reported in 0% to 12.5% of small case series and has appeared to decrease over time with newer equipment and techniques.2 Large sheath delivery also carries a risk of venous injury, thrombosis, or access-site complications, particularly in elderly patients with fragile vasculature. Incomplete defect coverage may lead to residual shunting, while excessive protrusion of the stent into the right atrium can interfere with atrial flow dynamics or future transvenous access. Stent migration or embolization, although uncommon, remains a catastrophic potential complication and underscores the importance of stable wire positioning and controlled deployment techniques.
Conclusions
This case highlights the complexity of managing coexisting CAD and ACHD. Multimodality imaging and invasive hemodynamic assessment were critical in defining both the severity of coronary disease and the physiological impact of the SVASD with PAPVR. A staged percutaneous strategy allowed definitive management of both conditions while avoiding the morbidity of combined surgical revascularization and congenital repair. This case underscores the value of multidisciplinary collaboration between the ACHD and CHD teams, including the surgical and interventional operators, in delivering tailored, minimally invasive care. Integration of a hub-spoke ACHD care model between the 2 programs was central to the efficient and coordinated management of this patient. Shared electronic medical record access and structured referral pathways enabled rapid transmission of imaging and operative history to the ACHD-accredited hub. Multidisciplinary case review allowed timely consensus decision-making without duplicative testing or delays in care, underscoring the need for collaboration between accredited ACHD centers and institutions without ACHD services.
Visual Summary.
Percutaneous Revascularization of the Left Main–Left Circumflex Bifurcation Was Performed Using a Delayed Reverse Culotte Technique
Intravascular ultrasound confirmed underexpanded proximal left anterior descending artery and calcified left main artery with minimum lumen area >10 mm2. The left circumflex artery was stented (3.5-mm Onyx Frontier) into the left main, followed by left anterior descending rewiring, kissing balloon inflations, and left main proximal optimization. Final intravascular ultrasound showed well-expanded, well-apposed stents with TIMI flow grade 3 and no residual stenosis. Initial injection into the right superior vena cava demonstrates shunting into the left atrium. A 10-zig G-Armor covered stent on BIB balloon was deployed across the right superior vena cava–right atrium, successfully excluding the atrial septal defect. Postdilation with a 23-mm balloon achieved full expansion with no residual shunt.
Funding Support and Author Disclosures
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
Appendix
For supplemental videos, please see the online version of this paper.
Appendix
Coronary Angiography Demonstrated Severe (90%) Ostial LCx Stenosis and Moderate LM Disease.
Preoperative Contrast Computed Tomography Demonstrated the Undiagnosed Findings of Right and Left SVC and an SVASD With PAPVR of the Right Upper and Middle Pulmonary Veins Draining Into the Right SVC
Balloon Sizing of the Right SVC Was Performed With a 20 mm × 3 cm Z-Med II Balloon Inflated to 4 atm, While Selective Right Upper Pulmonary Vein Angiography Confirmed Preserved Pulmonary Venous Drainage
Position of the Covered Stent Across the SVASD, Ensuring Distance From the Azygous, Where It Was Implanted From the SVC Into the Right Atrium, Successfully Excluding the Defect
Further Dilation of Proximal Stent Segment Using a 23 mm × 3 cm Tyshak II Balloon Catheter With Complete Elimination of the Residual Shunt
Follow-Up Right Pulmonary Arteriogram and Transesophageal Echocardiography Confirming Stable Stent Position, Unobstructed Pulmonary Venous Return, and No Residual Atrial-Level Shunt
References
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Associated Data
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Supplementary Materials
Coronary Angiography Demonstrated Severe (90%) Ostial LCx Stenosis and Moderate LM Disease.
Preoperative Contrast Computed Tomography Demonstrated the Undiagnosed Findings of Right and Left SVC and an SVASD With PAPVR of the Right Upper and Middle Pulmonary Veins Draining Into the Right SVC
Balloon Sizing of the Right SVC Was Performed With a 20 mm × 3 cm Z-Med II Balloon Inflated to 4 atm, While Selective Right Upper Pulmonary Vein Angiography Confirmed Preserved Pulmonary Venous Drainage
Position of the Covered Stent Across the SVASD, Ensuring Distance From the Azygous, Where It Was Implanted From the SVC Into the Right Atrium, Successfully Excluding the Defect
Further Dilation of Proximal Stent Segment Using a 23 mm × 3 cm Tyshak II Balloon Catheter With Complete Elimination of the Residual Shunt
Follow-Up Right Pulmonary Arteriogram and Transesophageal Echocardiography Confirming Stable Stent Position, Unobstructed Pulmonary Venous Return, and No Residual Atrial-Level Shunt