Asymptomatic Double-Orifice Mitral Valve in an Elderly Male Presenting with Complete Heart Block: A Case Report

Double orifice mitral valve (DOMV) is a rare congenital cardiac defect which is usually found incidentally. A 70-year-old male with multiple chronic illnesses presented with recurrent episodes of syncope and was diagnosed with complete heart block. His echocardiogram revealed an incidental finding of a DOMV, with no signs of stenosis or regurgitation. Therefore, a permanent pacemaker implant is required to treat complete heart blockage. This case highlights the incidental discovery of a double orifice mitral valve and the diagnostic value of echocardiography. DOMV was hemodynamically insignificant; therefore, no intervention was required, and long-term monitoring was recommended to check for possible complications.

Double orifice mitral valve (DOMV) is an unusual congenital cardiac defect in which the mitral valve has two separate openings through which blood flows from the left atrium into the left ventricle. It mainly develops because of the incomplete fusion of endocardial cushions during development of the embryo 1,2 . Mostly, it is seen in association with other congenital cardiac anomalies; however, it rarely exists alone. When associated with other anomalies, the incidence is approximately 1%. When it exists alone, the incidence ranges from 0.01% to 0.1%, as evidenced by the imaging and autopsy findings 1,3 . This dual etiology indicates that DOMV is not exclusively a pediatric concern, but isolated and asymptomatic cases of DOMV may remain undetected into adulthood; therefore, the exact prevalence and natural history within the adult population remain unclear 4 . The first description of the double mitral valve was recorded in 1876 by British pathologist William Smith Greenfield. Epidemiological and clinical data for DOMV are still limited owing to its inherent rarity 5 . CASE PRESENTATION A 70-year-old Asian male having diabetes, hypertension, chronic kidney disease and liver disease, benign prostatic hyperplasia, and advanced dementia presented to the emergency department at the Tahir Heart Institute on June 24, 2025, with multiple episodes of syncope over the last past two days, along with worsening shortness of breath and body swelling for several weeks. He had a heart rate of 34 beats per minute, blood pressure of 174/97 mmHg, and oxygen saturation of 92% on room air. He had bilateral basal fine inspiratory crepitations, a variable intensity of the first heart sound, and peripheral pitting edema. His electrocardiogram (ECG) showed a heart rate of approximately 34 beats per minute with a complete heart block. The patient was immediately transferred to the cardiac catheterization laboratory where a temporary pacemaker was inserted. His troponin level and thyroid function test results were normal. Hemoglobin level was low at 9.3 g/dl, and creatinine level was elevated at 2.3 mg/dl. His echocardiogram (ECHO) revealed mild concentric left ventricular hypertrophy, normal segmental wall motion, and a DOMV without evidence of valvular stenosis or regurgitation. In the parasternal short axis view, there was a DOMV in mitral position and the valve was split into two orifices via the bridge, which was fibrous and was a normal flow of blood (Figure 1A, Video 1) and apical chamber view showing a “seagull” style of valve as well as two mitral openings for inflow are visible (Figure 1B, Video 2). He was kept under observation; however, he remained in complete heart block after the temporary pacemaker was turned off. Therefore, a permanent pacemaker implantation was planned.

The DOMV is an uncommon congenital or acquired abnormality that is often not symptomatic, particularly when isolated and not associated with significant valve dysfunction 5 . Approximately half of patients with DOMV are functionally normal and do not exhibit obvious signs or symptoms, which predisposes them to underdiagnosis in routine clinical practice 1 . As highlighted by the case reports that focus on accidental findings, DOMV is often discovered during cardiac evaluations performed for unrelated reasons 1 . Examples include detection during an abnormal ECG workup 1 or the assessment of other cardiac conditions in elderly patients 4 . The functional impact of DOMV is highly variable; in some instances, it may not cause significant hemodynamic effects or symptoms, allowing normal blood flow between the cardiac chambers. However, it can contribute to or cause significant mitral regurgitation (MR) or stenosis (MS), leading to hemodynamic compromise 5 . The pathophysiology is complex and potentially involves a variety of hemodynamic dysfunctions, such as regurgitation, stenosis, and turbulence, along with structural problems of the valve cusps and cords 1 . DOMV presents with non-specific symptomatology, with symptom severity correlating to the degree of mitral valve dysfunction and symptoms can lead to pulmonary edema, congestive heart failure, and atrial arrhythmias and severity of symptoms for isolated DOMV is depends upon the degree of blood flow turbulence and left atrial pressure 5 . The frequency and prognostic significance of DOMV in adulthood, particularly in asymptomatic cases, remain largely unknown 1 . Echo is the test of choice in patients with DOMV. Based on Echo characteristics, congenital DOMV is broadly divided into three types: eccentric, central and duplicate mitral valve 5 . The majority of cases are about 85%, which are identified from other cases by having a larger orifice as well as a lesser accessory orifice. Different classifications include complete bridging, incomplete bridging, and holes, with 15% of DOMVs having complete bridging 1,5 . For asymptomatic and isolated DOMV with no significant hemodynamic impairment active intervention is usually not required, whereas surgical repair or valve replacement is indicated when DOMV leads to severe MS or MR or when present with other significant cardiac abnormalities that require intervention 5 . The transcatheter percutaneous balloon dilation can be an option that can be used to relieve obstructions associated with mitral stenosis 5 . The selection of the surgical procedure depends on the degree of valve dysfunction, patient age, comorbidities, and surgeon’s experience 6 . Long term follow-up is crucial for all patients on DOMV to allow early detection of any potential complications 7 . Ethics Approval Statement: This case report was approved by the Institutional Review Board of Tahir Heart Institute. Patient Consent Statement: Written informed consent was obtained from the patient’s legal guardian. Data Availability Statement: Data available on request due to privacy restrictions. Conflict of Interest Disclosure: The authors declare no conflict of interest. Funding Statement: None declared. Figure 1: (A) Parasternal short-axis view showing double orifice mitral valve; (B) Apical view with two separate mitral valve openings.

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Authors Metrics & Citations Metrics Article Usage 173views 99downloads Citations Download citation Faraz Ahmad, Ans Ahmad, Ikram Ahmed. Asymptomatic Double-Orifice Mitral Valve in an Elderly Male Presenting with Complete Heart Block: A Case Report. Authorea. 30 August 2025. DOI: https://doi.org/10.22541/au.175655000.08843417/v1 DOI: https://doi.org/10.22541/au.175655000.08843417/v1 If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Simply select your manager software from the list below and click Download. For more information or tips please see 'Downloading to a citation manager' in the Help menu.