Iatrogenic aortic valve fenestration rupture following coronary angiography: a case report

Abstract

Background

Aortic valve fenestration (AVF) is a relatively common anatomical variation, while its rupture is a rare cause of aortic regurgitation (AR), especially following coronary angiography. This case report highlights the importance of echocardiographic evaluation and multidisciplinary discussion for the differential diagnosis of fibroelastoma or endocarditis.

Case summary

A 66-year-old man presented with acute shortness of breath following coronary angiography for an inferior ST-elevation myocardial infarction. Echocardiography revealed severe aortic regurgitation associated with a filamentous mobile structure on the non-coronary cusp. A multidisciplinary discussion was conducted to determine the cause of the AR, leading to the patient undergoing aortic valve replacement. A gross inspection of the valve confirmed AVF rupture.

Conclusion

Iatrogenic AVF rupture following coronary angiography is an exceptionally rare complication. Thorough echocardiographic evaluation plays a significant role in differentiating AVF rupture from other valve pathologies, such as fibroelastoma, Lambl’s excrescence, or endocarditis. Echocardiographic features, including a very thin, linear, and flail-like mobile lesion, the paracommissural location, frequent involvement of the non-coronary cusp, and an eccentric jet of the AR, are suggestive of AVF rupture. While medical history and clinical examination contribute to the diagnostic process, definitive diagnosis relies on surgery and anatomopathology.

Introduction

Aortic valve fenestration (AVF) is a common anatomical variation characterized by a small oval aperture located under the free edge of an aortic cusp (Figure 1). While AVF rupture can lead to aortic regurgitation (AR), it is classically secondary to spontaneous or traumatic causes and is rarely severe.

This case report aims to describe the management of a patient who experienced AVF rupture following coronary angiography, emphasizing the importance of a thorough echocardiographic evaluation for differential diagnosis with fibroelastoma or infective endocarditis (IE).

Summary figure

Case description

A 66-year-old man with a past medical history of sleep apnoea syndrome and junctional tachycardia ablation presented with an acute AR following a coronary angiography. His current treatment included aspirin 75 mg, clopidogrel 75 mg, bisoprolol 5 mg, atorvastatin 80 mg, and ramipril 1.25 mg. A few days prior, he experienced an inferior ST-elevation myocardial infarction, leading to an urgent percutaneous coronary intervention (PCI). Upon admission, physical examination was normal, and no valvular abnormality was reported on the initial transthoracic echocardiography (TTE). A complete occlusion of the distal right coronary artery (RCA) was identified, and a stent was implanted (Guide catheter JR40, Stent Resolute Onyx 4.5 mm × 12 mm, duration of the procedure 38 min). The proximal RCA had a shepherd’s crook configuration but no difficulty was encountered during catheter engagement. Shortly after the procedure, the patient complained of shortness of breath and a short decrescendo early diastolic aortic murmur was detected. The NT-pro-brain natriuretic peptide level rose to 1784 pg/mL, chest X-ray was normal and a computed tomography scan revealed no aortic dissection or pulmonary embolism. A second TTE revealed an unexpected moderate-to-severe AR (vena contracta width 0.6 cm, Jet width > 50% of outflow tract, Pressure Half-Time 340 ms) associated with an abnormal mobile structure on the aortic valve (AV). Additionally, the inferior wall was hypokinetic, without left ventricle dilatation and the ejection fraction was 45%. A subsequent transoesophageal echocardiography (TOE) in mid-oesophageal view detected a fine and filamentous 17 mm mobile lesion on the non-coronary cusp (NCC), responsible for a coaptation defect with severe eccentric AR. The AV was tricuspid and no aortic root dilatation was observed. The rest of the assessment was unremarkable, with no clinical or laboratory evidence in favour of IE. A multidisciplinary discussion was conducted to determine the cause (infective or tumoral) of this AR and the patient was finally transferred to our cardiac surgery ward for further exploration and management.

On the day of surgery, intraoperative TOE (X8-2t Probe of Ultrasound Epiq 7, Philips, Hamburg, Germany) in mid-oesophageal long and short axis views confirmed the presence of the previously observed mobile structure on the AV, which had not evolved in size or appearance (Figure 2). Following median sternotomy, cardiopulmonary bypass, cross-clamping, and cardioplegic arrest were instituted. Aortotomy was performed and gross inspection of the AV confirmed the prolapse with the presence of an AVF rupture of the NCC (Figures 3). The other two cusps presented small AVFs and no evidence of endocarditis was observed. Because of the severity of the rupture, the presence of fenestrations on other cusps and the patient’s age, a valve-sparing strategy was preferred, and the injured valve was replaced with a n° 25 bioprothesis (Carpentier-Edwards Perimount ThermaFix, Edwards Lifesciences, Irvine, CA, USA). Anatomopathological analysis did not reveal any infectious or tumoral origin. Regarding antithrombotic therapy, the clopidogrel was discontinued 5 days before surgery, and was not resumed after. Postoperatively, heparin was administered and warfarin was promptly initiated, for 3 months. The postoperative course was uneventful and he was discharged from the hospital after 12 days.

Discussion

AVF is a frequent anatomical variation (present in 56% of cases) that occurs more frequently in men, with its frequency increasing with age.¹ Although AVF typically does not cause regurgitation, it may be implicated in some cases of severe AR after spontaneous or traumatic fenestration rupture.² AVF rupture is an exceptional but severe complication of coronary angiography, with only a few cases reported in the literature.^3,4 Other AV lesions following PCI, such as laceration, tear, or cusp perforation with a stent,^5–9 have been reported. In cases of AV injury and severe AR, patients typically present with dyspnoea, and surgery (replacement or repair) is indicated. In this particular case, the shepherd’s crook configuration of the proximal RCA might have led to repetitive contacts of the guidewire on the delicate tissue of the fenestrated valve, resulting in its rupture.

The preoperative diagnosis of AVF rupture is complex and relies on the clinical context and echocardiography. Based on this clinical case and other reports, proposed criteria suggesting AVF rupture include a very thin, linear, and flail-like mobile lesion measuring less than 20 mm, the paracommissural location of this lesion, and frequent involvement of the NCC with a prolapse associated with an eccentric jet of the AR. However, these proposed criteria are not pathognomonic, and the main challenge lies in the differential diagnosis with fibroelastoma, Lambl’s excrescence, and vegetation of endocarditis. Each of these lesions has characteristic echocardiographic features, but making the diagnosis solely with TOE may be complex, particularly if the lesion is small. Lambl’s excrescence is a degenerative filamentous strand of valvular leaflets that appears as very thin and lint-like mobile threads arising from the free borders or ventricular surfaces of aortic leaflets in echocardiography. Fibroelastoma, on the other hand, is a benign tumour originating from the endocardium of the arterial or ventricular side of the valves. It is usually found to be pedunculated and larger than Lambl’s excrescence in echocardiography. Although fibroelastoma and Lambl’s excrescence are small (<20 mm) and rarely cause severe AR, they can both lead to stroke and systemic embolism, justifying cardiac surgery. Vegetations of endocarditis seen on echocardiography are irregularly shaped lesions of variable size with independent oscillatory motion. Moreover, the presence of complications, such as valvular perforations with AR or perivalvar extension (abscess, fistulae), strongly suggests IE.¹⁰ The presence of positive blood cultures represents the other major criterion for this diagnostic (Table 1).

Although TOE is commonly used for preoperative assessment of aortic valvular lesions, its ability to provide precise resolution is limited, particularly when dealing with smaller lesions. In this regard, cardiovascular magnetic resonance imaging offers a non-invasive alternative that can provide detailed images of valve anatomy and enable quantitative evaluation of AR. Nonetheless, it is important to note that no imaging exams can achieve perfect discrimination of these small lesions, and definitive confirmation of the diagnosis relies on surgical intervention and subsequent anatomopathological analysis. Nevertheless, the presence of complications or indirect echographic signs, coupled with a thorough review of the patient’s medical history and clinical examination, can contribute to the diagnostic process.

Iatrogenic AVF rupture following coronary angiography is an exceptionally rare but severe complication. This case report underscores the importance of considering AVF rupture as a potential cause of severe AR in patients undergoing coronary angiography. The diagnosis of AVF rupture can be challenging, requiring a comprehensive evaluation, including echocardiography and consideration of differential diagnoses such as fibroelastoma or endocarditis. Prompt recognition and accurate diagnosis are crucial for guiding appropriate management, which may involve surgical intervention.

Lead author biography

Olivier Montandrau is anaesthesist and intensivist specialised in cardiac and vascular surgery at the Montsouris Institute in Paris where he has worked for 7 years. He has particular competences in echocardiography in cardiac surgery and in circulatory support devices. He is also member of the French College of Anaesthesia and Intensive Care (CFAR) and the European Society of Cardiology (ESC).

Supplementary material

Supplementary material is available at European Heart Journal – Case Reports online.

Acknowledgements

The authors want to thank Dr Alain Berrebi for his expertise in echocardiography; Prof. Marc Beaussier and Dr Konstantinos Zannis for their support and ever-informative advice.

Consent: The authors confirm that written consent for submission and publication of this case report including images and associated text has been obtained from the patient in accordance with COPE guidelines.

Funding: None.

Data availability

No new data were generated or analysed in support of this research.

References

Author notes