Systolic doming or ‘hockey-stick sign’ of the aortic valve in rheumatic valve disease

Although rheumatic changes are well-described in atrioventricular valves (e.g., the mitral valve), they are less well recognized in arterial valves such as the aortic valve.

Diastolic doming, or the ‘hockey-stick’ phenomenon of the rheumatic mitral valve has previously been described, which occurs secondary to fusion of the leaflet commissures, as well as relatively better mobility of the base of the leaflet than the free margin of the valve. This entity, however, has not been previously described for the aortic valve.

Herein, we present images of a rheumatic aortic valve, which demonstrate moderate aortic regurgitation with mild fusion of the left/right-commissure in a tricuspid aortic valve. Although the valve is opening without a significant transvalvular gradient, we observed an unusual systolic doming [aortic valve doming (AVD)] of the aortic valve cusps, resembling the ‘hockey stick sign’ of the mitral valve (Figure 1).

Figure 1 Transesophageal echocardiography. (A) Doming of the posterior leaflet of the aortic valve in systole (arrow). (B) Aortic valve in diastole. (C) Aortic valve opening—short axis. (D) Commissural fusion of the left/right-commissure (arrow). (E) Central regurgitant jet of the aortic valve (long axis/color Doppler). (F) Central regurgitant jet of the aortic valve (short axis/color Doppler).

Although the valve appeared tricuspid, the fusion of the left/right-commissure could be easily mistaken for a very asymmetric phenotype bicuspid aortic valve, such as the forme fruste bicuspid aortic valve (1,2). These valves are sometimes difficult to distinguish from tricuspid aortic valves. However, in this image, the fusion of the commissure was acquired, rather than a pre-existing congenital malformation.

Moreover, in bicuspid- or unicuspid aortic valves, the regurgitant jet is mostly eccentric (3) (Figure 2), which is secondary to a fused cusp prolapse (4), whereas in this case the jet is central (Figure 1). This observation thus further underlines the notion that the commissural fusion is related to rheumatic heart disease rather than a pre-existing bicuspid- or unicuspid aortic valve morphology (5,6). Also, the sinuses appear rather symmetric, which would also make a bicuspid aortic valve less likely (1).

Figure 2 Transesophageal echocardiography (long axis/color Doppler): typical eccentric jet in a bicuspid aortic valve due to anterior fused cusp prolapse.

Similar to the mitral valve, the commissural fusion of the tricuspid aortic valve leads to restriction of leaflet mobility of the free margins, with better mobility of the base of the leaflets towards the valve hinges of the aortic valve. As in the diastolic doming of the mitral valve, in the aortic valve it leads to systolic doming during the ejection phase of the ventricle. In this aortic valve, the regurgitation was likely related to a combination of leaflet restriction, as well as annular dilation, which measures 25 mm, thus a combination of El Khoury type I & III aortic regurgitation (7,8).

The findings of systolic doming or hockey stick sign of the rheumatic aortic valve have not been described before, although systolic doming of bicuspid aortic valves has been described and the same mechanism also applies to unicuspid valves (Figure 3). We highlight the concept however, that although atrioventricular- and arterial valves are different morphologically, the underlying principles that guide the mechanism for dysfunction, pathology and surgical repair are the same. The disease process of rheumatic heart disease therefore should affect both valves in a similar fashion, and the ‘hockey stick sign’ of the aortic valve, thus also suggests rheumatic involvement of the valve.

Figure 3 Transesophageal echocardiography (long axis): systolic doming (arrows) of the aortic valve in a (A) bicuspid aortic valve and (B) unicuspid aortic valve.

Acknowledgments

Funding: None.

Footnote

Provenance and Peer Review: This article was commissioned by the editorial office, Journal of Visualized Surgery. The article has undergone external peer review.

Peer Review File: Available at https://jovs.amegroups.com/article/view/10.21037/jovs-24-11/prf

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jovs.amegroups.com/article/view/10.21037/jovs-24-11/coif). J.J. serves as an unpaid editorial board member of Journal of Visualized Surgery from October 2023 to September 2025. S.M.S. is a consultant to Artivion, Abbott, and the Japanese Organization for Medical Device Development (JOMDD). The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was conducted in accordance with the Helsinki Declaration (as revised in 2013). Our institution does not require IRBs for case reports or images, which obtain no identifying patient information, as in this manuscript. Written informed consent was obtained for the publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

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