Double orifice mitral valve: rare disease with a wide range of presentation

Double orifice mitral valve (DOMV) is a rare congenital anomaly, as implied by the name, that results in two distinct orifices within the mitral valve, rather than usual single orifice. Due to its rarity, there is no established guidelines on evaluation of DOMV.

Theerasuwipakorn et al. (1) have highlighted two distinct cases of DOMV and their different outcomes. This condition is rare with an incidence of approximately 1 in 100,000. The exact embryogenesis of DOMV is unknown, but it is thought to be due to abnormal development of the endocardial cushions during embryonic life (2). Baño-Rodrigo et al. reported that an anomaly of tensor apparatus was always found in 27 post-mortem cases that included chordal ring, accessory papillary muscle, subdividing muscle ridge, fused papillary muscle, crossing chordae tendineae and central fibrous division (2).

The pathophysiology of DOMV is complex, and it may involve a combination of hemodynamic abnormalities, such as regurgitation, stenosis, and turbulence, as well as structural abnormalities, such as prolapse, redundancy, and fibrosis, of the valve leaflets and the chordae tendineae. It is commonly associated with other congenital anomalies, most commonly atrioventricular septal defects, patent ductus arteriosus or coarctation of aorta (3,4). Thus, a thorough evaluation is needed if DOMV is detected.

The diagnosis of DOMV can be challenging, as it may be mistaken for other conditions, such as mitral stenosis, mitral regurgitation, or mitral valve prolapse. Echocardiography is the gold standard for the diagnosis of DOMV, and it can provide detailed information on the anatomy, function, and severity of the valve abnormalities (5). In the current era of advanced imaging, we believe that multimodality imaging approach is essential and can be help in achieving accurate diagnosis and, more importantly, it allows for better assessment of its hemodynamic consequences and in evaluating concomitant congenital anomalies. 3D transthoracic echocardiogram (TTE) provides a comprehensive assessment of the morphology of DOMV, over and above the information obtained by 2D imaging (6,7). Cardiac magnetic resonance imaging (MRI) can also help assess DOMV especially in the setting of poor echocardiographic windows, and in addition it can better assess potential other congenital anomalies (8). The entity is not well established with cardiac MRI; however, once we use it to assist in diagnosis, it will gain traction to be used as a diagnostic tool in the evaluation of DOMV.

The management of DOMV depends on the severity of the valve dysfunction, the presence of associated congenital heart defects, and the clinical symptoms of the patient. Asymptomatic patients with mild or no valve dysfunction may be managed conservatively with regular follow-up and surveillance (9), while symptomatic patients with severe valve dysfunction or associated heart defects may require surgical intervention. The surgical options for DOMV include mitral valve repair or replacement, with the choice of procedure depending on the extent of the valve abnormalities, the age and comorbidities of the patient, and the surgeon’s experience and preference.

Acknowledgments

Funding: None.

Footnote

Provenance and Peer Review: This article was commissioned by the editorial office, AME Case Reports. The article did not undergo external peer review.

Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-23-49/coif). The authors have no conflicts of interest to declare.

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References

1. Theerasuwipakorn N, Chattranukulchai P, Satitthummanid S, et al. Double orifice mitral valve: a report of two cases with different severity and treatment. AME Case Rep 2023;7:12. [Crossref] [PubMed]
2. Baño-Rodrigo A, Van Praagh S, Trowitzsch E, et al. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol 1988;61:152-60. [Crossref] [PubMed]
3. Warnes C, Somerville J. Double mitral valve orifice in atrioventricular defects. Br Heart J 1983;49:59-64. [Crossref] [PubMed]
4. Rhissassi J, El Malki H, Benmessaoud FA, et al. Double-orifice mitral valve associated with atrioventricular canal defects. Pan Afr Med J 2016;23:199. [Crossref] [PubMed]
5. Wójcik A, Klisiewicz A, Szymański P, et al. Double-orifice mitral valve - echocardiographic findings. Kardiol Pol 2011;69:139-43. [PubMed]
6. Raja Shariff RE, Hui Beng K, Mohd Ghazi A. Acquired double-orifice mitral valve with severe mitral regurgitation. Eur Heart J Case Rep 2022;6:ytac321.
7. Pires N, Li W, Senior R, et al. Incidental finding of a double orifice mitral valve in an elderly patient: value of 3D imaging. Echo Res Pract 2017;4:K21-4. [Crossref] [PubMed]
8. Laccetta G, Napoli AM. Incidental Finding of an Isolated Double-orifice Mitral Valve in an Asymptomatic Man. J Cardiovasc Echogr 2016;26:100-3. [Crossref] [PubMed]
9. Majid AA. Double orifice mitral valve: a case report and review of management. J Cardiovasc Surg (Torino) 1991;32:837-9. [PubMed]