Skip to main content
Erschienen in: BMC Cardiovascular Disorders 1/2015

Open Access 01.12.2015 | Case report

Isolated double-orifice mitral valve: a case report

verfasst von: Philipp Krisai, Bastian Wein, Beat A. Kaufmann

Erschienen in: BMC Cardiovascular Disorders | Ausgabe 1/2015

download
DOWNLOAD
print
DRUCKEN
insite
SUCHEN

Abstract

Background

Double-orifice mitral valve is an extremely rare cardiac anomaly possibly originating from insufficient endocardial fusion in embryogenesis. Severe concomitant cardiac anomalies and malfunction of the valve usually lead to an early diagnosis in childhood. Therefore the prevalence of isolated double-orifice mitral valve in adulthood is not known.

Case presentation

We present the case of a 63 years old, female Caucasian patient with isolated double-orifice mitral valve diagnosed in routine echocardiographic evaluation after chemotherapy presenting without clinical symptoms.

Conclusion

Trans-thoracic echocardiography is a suitable modality to diagnose and further assess anatomical and functional properties of the anomaly. In the presence of double-orifice mitral valve concomitant cardiac anomalies and valvular stenosis or regurgitation must be excluded. If an isolated double-orifice mitral valve with no functional abnormalities is present, no further follow-up is necessary.
Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​s12872-015-0168-0) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

PK analyzed and interpreted the data, drafted the manuscript and made critical revision of the manuscript for important intellectual content. BW carried out the echocardiographic examination, made critical revision of the manuscript for important intellectual content and analyzed the data. BK carried out the echocardiographic examination, made critical revision of the manuscript for important intellectual content, analyzed the data and handled supervision. All authors read and approved the final manuscript.
Abkürzungen
DOMV
double-orifice mitral valve
TTE
trans-thoracic echocardiography

Background

Isolated double-orifice mitral valve (DOMV) is an extremely rare congenital anomaly, possibly originating from insufficient embryonic fusion of the endocardial cushions [1, 2], leading to two separate orifices of the mitral valve into the left ventricle. Because of frequent associated anomalies and impaired function [3], the anomaly is usually detected in early childhood. The prevalence and prognostic relevance of an isolated DOMV in adulthood is not known.

Case presentation

We present the case of a 63 years old Caucasian female, referred to our echocardiography lab for routine evaluation 5 years after chemotherapy including cyclophosphamid and stem cell transplantation for chronic myeloid leukemia. She was in good general condition and asymptomatic. Physical examination, electrocardiography, prior thoracic X-ray and CT-scan were unremarkable. On trans-thoracic echocardiography (TTE), performed with a Philips iE33, a DOMV could be detected in standard 2-dimensional views without stenosis or regurgitation (Fig. 1a, b, e and f, Additional file 1: Movie 1, Additional file 2: Movie 2, Additional file 3: Movie 3, Additional file 4: Movie 4 and Additional file 6: Movie 6). We classified the DOMV as a complete bridge type with a fibrous bridge separating the valve into two orifices with a slightly larger anterolateral orifice. Each orifice had its own leaflets and subvalvular apparatus, each connected to one papillary muscle resulting in a double parachute appearance in apical views (Fig. 1a and b, Additional file 1: Movie 1, Additional file 2: Movie 2 and Additional file 3: Movie 3). Two separate jets of antegrade flow into the left ventricle were detected with color Doppler flow (Fig. 1e and f, Additional file 5: Movie 5 and Additional file 6: Movie 6). We furthermore evaluated the valve with three dimensional TTE (Fig. 1c and d, Additional file 4: Movie 4), illustrating the relative dimensions of the two orifices. Interestingly, no other congenital cardiac anomaly or structural cardiopathy was found. In the absence of cardiac symptoms in this patient with a DOMV with normal function no further diagnostic or therapeutic steps were necessary. The patient was instructed to return to our clinic if any symptoms should evolve.

Discussion

First described in 1876 [4], DOMV is an extremely rare congenital anomaly, classified into three categories: complete bridge (15 %), incomplete bridge and hole type (85 %) [1]. DOMV is believed to originate from insufficient fusion of endocardial cushion, which can also be responsible for associated anomalies [2]. In a large autopsy series of patients with congenital heart disease, 1 % had a DOMV, which in the majority of cases was associated with other cardiac anomalies (atrioventricular septal defect, coarctation of the aorta, patent ductus arteriosus, interrupted aortic arch) or mitral regurgitation [3]. Because of the associated anomalies, DOMV is usually detected in early childhood but is an exceedingly rare diagnosis in adults, and thus the incidence and prognostic relevance of an isolated DOMV detected in adulthood is unknown.
As an isolated DOMV without functional abnormalities of the mitral valve does not necessarily cause clinical symptoms it is prone to be missed. Our case demonstrates a rare case of isolated DOMV without apparent clinical implications diagnosed very late at 63 years of age, underlining the need for comprehensive and accurate TTE in patients referred for “routine” exams. Furthermore we show that TTE is an appropriate diagnostic tool to not only diagnose, but also evaluate the anomaly in 2D and 3D imaging modalities.

Conclusions

Echocardiography is a suitable modality for diagnosing DOMV and offers the possibility of comprehensive anatomical and functional assessment. In the presence of DOMV concomitant cardiac anomalies and valvular stenosis or regurgitation must be excluded. If an isolated DOMV with no functional abnormalities is present, no further follow-up is necessary.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Acknowledgements

No acknowledgements.
Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://​creativecommons.​org/​licenses/​by/​4.​0/​), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://​creativecommons.​org/​publicdomain/​zero/​1.​0/​) applies to the data made available in this article, unless otherwise stated.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

PK analyzed and interpreted the data, drafted the manuscript and made critical revision of the manuscript for important intellectual content. BW carried out the echocardiographic examination, made critical revision of the manuscript for important intellectual content and analyzed the data. BK carried out the echocardiographic examination, made critical revision of the manuscript for important intellectual content, analyzed the data and handled supervision. All authors read and approved the final manuscript.
Literatur
1.
Zurück zum Zitat Trowitzsch E, Bano-Rodrigo A, Burger BM, Colan SD, Sanders SP. Two-dimensional echocardiographic findings in double orifice mitral valve. J Am Coll Cardiol. 1985;6:383–7.PubMedCrossRef Trowitzsch E, Bano-Rodrigo A, Burger BM, Colan SD, Sanders SP. Two-dimensional echocardiographic findings in double orifice mitral valve. J Am Coll Cardiol. 1985;6:383–7.PubMedCrossRef
2.
Zurück zum Zitat Das BB, Pauliks LB, Knudson OA, Kirby S, Chan K-C, Valdes-Cruz L, et al. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr. 2005;18:231–6.CrossRef Das BB, Pauliks LB, Knudson OA, Kirby S, Chan K-C, Valdes-Cruz L, et al. Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr. 2005;18:231–6.CrossRef
3.
Zurück zum Zitat Baño-Rodrigo A, Van Praagh S, Trowitzsch E, Van Praagh R. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol. 1988;61:152–60.PubMedCrossRef Baño-Rodrigo A, Van Praagh S, Trowitzsch E, Van Praagh R. Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations. Am J Cardiol. 1988;61:152–60.PubMedCrossRef
4.
Zurück zum Zitat Greenfield W. Double mitral valve. Trans Pathol Soc Lond. 1876;27:128–9. Greenfield W. Double mitral valve. Trans Pathol Soc Lond. 1876;27:128–9.
Metadaten
Titel
Isolated double-orifice mitral valve: a case report
verfasst von
Philipp Krisai
Bastian Wein
Beat A. Kaufmann
Publikationsdatum
01.12.2015
Verlag
BioMed Central
Erschienen in
BMC Cardiovascular Disorders / Ausgabe 1/2015
Elektronische ISSN: 1471-2261
DOI
https://doi.org/10.1186/s12872-015-0168-0

Weitere Artikel der Ausgabe 1/2015

BMC Cardiovascular Disorders 1/2015 Zur Ausgabe

Screening-Mammografie offenbart erhöhtes Herz-Kreislauf-Risiko

26.04.2024 Mammografie Nachrichten

Routinemäßige Mammografien helfen, Brustkrebs frühzeitig zu erkennen. Anhand der Röntgenuntersuchung lassen sich aber auch kardiovaskuläre Risikopatientinnen identifizieren. Als zuverlässiger Anhaltspunkt gilt die Verkalkung der Brustarterien.

Niedriger diastolischer Blutdruck erhöht Risiko für schwere kardiovaskuläre Komplikationen

25.04.2024 Hypotonie Nachrichten

Wenn unter einer medikamentösen Hochdrucktherapie der diastolische Blutdruck in den Keller geht, steigt das Risiko für schwere kardiovaskuläre Ereignisse: Darauf deutet eine Sekundäranalyse der SPRINT-Studie hin.

Therapiestart mit Blutdrucksenkern erhöht Frakturrisiko

25.04.2024 Hypertonie Nachrichten

Beginnen ältere Männer im Pflegeheim eine Antihypertensiva-Therapie, dann ist die Frakturrate in den folgenden 30 Tagen mehr als verdoppelt. Besonders häufig stürzen Demenzkranke und Männer, die erstmals Blutdrucksenker nehmen. Dafür spricht eine Analyse unter US-Veteranen.

Adipositas-Medikament auch gegen Schlafapnoe wirksam

24.04.2024 Adipositas Nachrichten

Der als Antidiabetikum sowie zum Gewichtsmanagement zugelassene Wirkstoff Tirzepatid hat in Studien bei adipösen Patienten auch schlafbezogene Atmungsstörungen deutlich reduziert, informiert der Hersteller in einer Vorab-Meldung zum Studienausgang.

Update Kardiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.