The authors report a case of double-orifice mitral valve (DOMV) which showed
mitral stenosis and mild insufficiency. An associated anomaly was secundum
atrial septal defect. DOMV is an unusual congenital heart defect. The occurrence
of this anomaly with or without secundum atrial septal defect is very rare.
More often it is associated with other congenital malformations arising from
atrioventricular canal defects. There may be no haemodynamic consequences
but mitral insufficiency and/or stenosis may complicate this malformation. Treatment
can be summarised as abstention, surgical repair or valve replacement

Aldur, MM

Demircin, M

Dogan, OF

Guvener, M

Tunali, S

Çelik, HH

English

Via Medica Journals

Folia Morphol. Vol. 63, No. 3, pp. 351–353Copyright © 2004 Via MedicaISSN 0015–5659www.fm.viamedica.plC A S E  R E P O R T351Address for correspondence: H. Hamdi Çelik MD, PhD, Professor, Hacettepe University Faculty of Medicine, Department of Anatomy,06100, Ankara, Turkey, e-mail: hacelik@hacettepe.edu.trDouble-orifice mitral valve and an associatedmalformation: secundum atrial septal defectMetin Demircin1, Murat Guvener1, O. Faruk Dogan1, Selcuk Tunali2, M. Mustafa Aldur2,H. Hamdi Çelik21Hacettepe University, Department of Thoracic and Cardiovascular Surgery, Ankara, Turkey2Hacettepe University, Department of Anatomy, Ankara, Turkey[Received 23 December 2003; Revised 17 February 2004; Accepted 17 February 2004]The authors report a case of double-orifice mitral valve (DOMV) which showedmitral stenosis and mild insufficiency. An associated anomaly was secundumatrial septal defect. DOMV is an unusual congenital heart defect. The occur-rence of this anomaly with or without secundum atrial septal defect is very rare.More often it is associated with other congenital malformations arising fromatrioventricular canal defects. There may be no haemodynamic consequencesbut mitral insufficiency and/or stenosis may complicate this malformation. Treat-ment can be summarised as abstention, surgical repair or valve replacement.Key words: mitral valve, abnormalities, double-orifice, atrial septaldefectINTRODUCTIONDouble-orifice mitral valve (DOMV) is an unusualbut surgically important condition. It is often associ-ated with atrioventricular (AV) canal defects, althoughit can be seen either as an isolated malformation orin association with other cardiac anomalies.Double-orifice mitral value was first described byGreenfield in 1876 (guoted from [9]). Double-orifice orduplication of the mitral valve usually presents a con-genital mitral stenosis or may show normal valve func-tion [8]. Although this malformation of the valve usual-ly produces mitral stenosis and, very rarely, mitral insuf-ficiency; it may present no haemodynamic disturbance[5]. In half of the reported cases the duplication of themitral valve orifice is seen as an isolated lesion [4].We present here a case of DOMV which is sup-ported by chordea tendinea originating from thepapillary muscles and the AV wall. No mitral cleft orAV canal defect was associated with this valve, whichshowed severe mitral stenosis and mild insufficien-cy. A bridge of tissue separated both orifices, whichwere supported by leaflets that enabled the valve tobe competent.CASE REPORTA 46-year-old female patient came to the hospi-tal for the first time when she was 7 years old, com-plaining of effort dyspnoea and exercise intolerance.Echocardiography provided a diagnosis only of se-cundum type atrial septal defect (ASD). The secun-dum ASD was repaired primarily, no additional de-fect was detected during the operation and the pa-tient was discharged with excellent postoperativeprogress. Three years later she was admitted againto Hacettepe University Department of Cardiovascu-lar Surgery with dyspnoea on effort and palpitation.She was therefore diagnosed with congenital heartdisease, probably mitral stenosis. In the light of this,she was given medical treatment. Her treatment overthe following years was limited to the control of dys-352Folia Morphol., 2004, Vol. 63, No. 3pnoea. Then, in 1972, an echocardiographic exami-nation revealed that the mitral valve might havea double orifice. However, the mitral stenosis was mildand the symptoms disappeared with medical treat-ment. She was followed up for almost 30 years withthe focus on mitral stenosis. In 1994 the patient wasadmitted to hospital with refractory congestive heartfailure. The diagnosis of DOMV was established dur-ing this admission. Moderate mitral stenosis and aninsufficiency of 1–2° was detected in both mitral or-ifices on echocardiography. In the same year she gavebirth normally at full term. Following her dischargefrom the hospital after delivery, the patient’s cardi-ac status was regularly checked with echocardio-graphy. In 2001 the mitral valve was replaced be-cause of severe mitral stenosis and mitral insufficiencyof 2°, diagnosed by colour-Doppler echocardiogramand left cardiac catheterisation. The peak diastolicgradient was 24 mm Hg (with a mean of 12 mm Hg).Cardiac catheterisation revealed a peak pulmonarycapillary wedge pressure of 12 mm Hg and pulmo-nary artery systolic pressure of 35–40 mm Hg.The patient underwent surgical correction. Thetwo orifices of the mitral valve, which were support-ed by chordea tendinea originating from the papil-lary muscles and AV wall, were unequal in size. Thelarger (major) orifice was located posteriorly, andthe other (accessory) orifice anteriorly. The two ori-fices were completely bridged by fibrous tissue(Fig. 1). The area was 1.34 cm2 in the larger orificeand 0.4 cm2 in the smaller. These results supportedthe echocardiographic findings. The valvular leafletsof the larger orifice had proper chordae tendineaeand papillary muscles. Partially normal chordaetendineae originated from the anterolateral and pos-teromedial leaflets and were attached to the ante-rolateral-middle and posteromedial-middle papillarymuscles respectively (Fig. 2). In addition, the mitralvalve was highly fibrotic and also showed examplesof severe stenosis and mild insufficiency. The excisedvalve was therefore replaced with St. Jude mechan-ical prosthesis No. 31 under cardiopulmonary bypasswith moderate hypothermia (28°C).DISCUSSIONAnatomical as well as haemodynamic observationshave shown that double mitral orifices function ascompetent units. Advances in echocardiography haveallowed more frequent detection of this entity by non-invasive techniques. Double-orifice mitral valve andadditional lesions of each subdivision of the mitralvalve were clearly shown by two-dimensional andcolour-Doppler echocardiography by Segni et al. in1986 [10]. In the present case DOMV was not diag-nosed preoperatively with colour-Doppler echocardio-gram until the 3rd admission. Because experience andtechniques of echocardiography had not yet advancedsufficiently to show this anomaly with its anatomicaldetails before 1980, most cases of DOMV in litera-ture were diagnosed intra-operatively.Double-orifice mitral valve may be of functionalsignificance, causing mitral stenosis or incompetenceand is usually associated with other cardiac anoma-lies [7, 11]. The one which most often accompaniesit is endocardial cushion defect (partial persistent AVcanal) [2]. Similarly, according to Rosenberg et al. [7],25% of patients with DOMV have partial persistentAV canal and about 5% of patients with partial per-sistent AV canal have DOMV. This association sug-gests that DOMV and partial persistent AV canal haveFigure 1. The appearance of the anomaly during the operation.O1 and O2 — indicate the larger and smaller orifices respectively,B — indicates the bridge between them.Figure 2. The appearance of the removed mitral valve. APM andPPM — indicate the anterior and posterior papillary muscles re-spectively, CT — indicates the chordea tendinea, O1 and O2— indicate orifices, B indicates the bridge between them.353Metin Demircin et al., Double-orifice mitral valve and an associated malformation: secundum atrial septal defectcommon developmental bases, such as a defect inthe endocardial cushion [2]. However, in our case,there was only a secundum type atrial septal defectas an associated anomaly and this was closed in thefirst operation.According to Baño-Rodrigo et al. [2], the frequen-cy of equal-sized orifices in patients with DOMV islimited to 15%, with the remainder being unequalin size. In 48% of patients with double orifices themitral valve function was normal, while mitral steno-sis and regurgitation were seen in 26% each [9]. Inour case, therefore, giving birth normally with sucha malformation was extraordinary.The framework that makes up DOMV can be di-vided into 3 forms: a complete bridge between thetwo orifices, an incomplete bridge, and a hole formedby the congenitally abnormal valves. A completebridge (as in this case) is the structure that is leastcommonly seen [1].Rosenberg et al. [7] stated that the true develop-mental basis of duplication of the mitral valve isunknown. DOMV might be the result of foetal en-docarditis or may be a purely developmental anom-aly [8]. In the only study so far which has put forwarda logical hypothesis Lewis, after studying the Harvardcollection of pig embryos, proposed that the doublemitral valve was the result of an early arrest of devel-opment and that the accessory orifice representsa retention of the left portion of the common AV canalwith subsequent reduction of the mitral ostium andalignment with it [2].The non-cleft orifice of a DOMV is usually com-petent and rarely requires closure. The cleft, becauseit constitutes a type of parachute (single papillarymuscle) valve, should be partially closed so as to re-lieve valve incompetence without causing unduestenosis. Repair of the AV canal associated withDOMV can be achieved with a low operative mortal-ity and excellent late results [3]. Treatment may besummarised as abstention, surgical repair or valvereplacement. It may cause peculiar surgical problemswhen plastic repair of the mitral valve is needed [6].Metallic mitral valve replacement is preferable forfibrocalcific valves, mitral stenosis with insufficency,and also for giving birth to children and at a suitablepatient age, as in our case [10].ACKNOWLEDGEMENTSThese data were presented in the form of a post-er presentation at the 1st Joint Meeting of EACA& AACA, Graz, Austria, 8–11 July 2003.REFERENCES1. Ancalmo N, Ochsner JL, Mills NL, King TD (1977) Dou-ble mitral valve. Report of a case and review of theliterature. Angiology, 28: 95–100.2. Baño-Rodrigo A, Praagh SV, Trowitzsch E, Praagh RV(1988) Double-orifice mitral valve: a study of 27 post-mortem cases with developmental, diagnostic andsurgical considerations. Am J Cardiol, 61: 152–160.3. Domenico RD, Gheno G, Cucchini F (1993) Double orificein prolapsing mitral valve. Inter J Cardiol, 41: 171–172.4. Honnekeri ST, Tendolkar AG, Lokhandwala YY(1993) Double-orifice mitral and tricuspid valves in as-sociation with the Raghib complex. Ann Thorac Surg,55:  1001–1002.5. Lee CN, Danielson GK, Schaff HV, Mair DD (1985) Sur-gical treatment of double-orifice mitral valve in atrio-ventricular canal defects. J Thorac Cardiovasc Surg, 90:700–705.6. Lee DI, Ha JW, Chung B, Kim Y, Chun KJ, Rim SJ, Chung N(1999) Double-orifice mitral valve. Clin Cardiol, 22: 425.7. Rosenberg J, Roberts WC (1968) Double-orifice mitralvalve. Arch Path, 86: 77–80.8. Sasaoka T, Ohguri H, Makita Y, Kurokawa S, Izumi T(1996) Double-orifice mitral valve in an elderly patientwith tetralogy of Fallot. Jpn Heart J, 37: 503–507.9. Saylam A, Oram A, Nazli N, Yener A, Aytaç A (1976)Double orifice mitral valve associated with ostium pri-mum atrial septal defect. Turk J Pediatr, 18: 58–62.10. Segni ED, Lew S, Shapira H, Kaplinsky E (1986) Doublemitral valve orifice. Pediatr Cardiol, 6: 215–217.11. Yurdakul Y, Arsan S, Karapinar K, Tamim M, Bilgiç A(1995) Congenital double-orifice mitral valve — a casereport. Turk J Pediatr, 37: 173–176.

Double-orifice mitral valve and an associated malformation: secundum atrial septal defect

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Double-orifice mitral valve and an associated malformation: secundum atrial septal defect

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