Double orifice mitral valve in a patient with bicuspid aortic valve and coarctation of the aorta: A rare presentation (original) (raw)
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Double Orifice Mitral Valve and Bicuspid Aortic Valve: Pieces of the Same Single Puzzle?
Case Reports in Cardiology, 2015
Double orifice mitral valve is a very rare congenital abnormality. Well known associations of this pathology with other congenital lesions point to a complex and central pathophysiological mechanism leading to a sequence of pathologies. These associations have long been realized and arbitrarily defined as Shone complex. We would like to present a 21-year-old patient with double orifice mitral valve associated with bicuspid aortic valve, with a brief review of the literature on possible central mechanisms leading to different subsets of congenital abnormalities involving these two.
Double orifice mitral valve: A case report
Vojnosanitetski pregled, 2016
Introduction. Double orifice mitrol valve (DOMV) is a very rare congenital heart defect. Case report. We reported 20-year-old male referred to our center due to evaluation of his cardiologic status. He was operated on shortly after birth for a tracheoesophageal fistula. Accidentally, echocardiography examination at the age of 4 years revealed double orifice mitral valve (DOMV) without the presence of mitral regurgitation, as well as mitral stenosis, with normal dimensions of all cardiac chambers. The patient was asymptomatic, even more he was a kick boxer. His physical finding was normal. Electrocardiography showed regular sinus rhythm, incomplete right bundle branch block. Transthoracic echocardiography (TTE) examination revealed the normal size of the left atrial, mitral leaflets were slightly more redundant. The left and right heart chambers, aorta, tricuspid valve and pulmonary artery valve were normal. During TTE examination on a short axis view two asymmetric mitral orifices w...
The American Journal of Cardiology, 2012
Although bicuspid aortic valve occurs in an estimated 1% of adults and mitral valve prolapse in an estimated 5% of adults, occurrence of the 2 in the same patient is infrequent. During examination of operatively excised aortic and mitral valves because of dysfunction (stenosis and/or regurgitation), we encountered 16 patients who had congenitally bicuspid aortic valves associated with various types of dysfunctioning mitral valves. Eleven of the 16 patients had aortic stenosis (AS): 5 of them also had mitral stenosis, of rheumatic origin in 4 and secondary to mitral annular calcium in 1; the other 6 with aortic stenosis had pure mitral regurgitation (MR) secondary to mitral valve prolapse in 3, to ischemia in 2, and to unclear origin in 1. Of the 5 patients with pure aortic regurgitation, each also had pure mitral regurgitation: in 1 secondary to mitral valve prolapse and in 4 secondary to infective endocarditis. In conclusion, various types of mitral dysfunction severe enough to warrant mitral valve replacement occur in patients with bicuspid aortic valves. A proper search for mitral valve dysfunction in patients with bicuspid aortic valves appears warranted.
Revista Portuguesa De Cardiologia Orgao Oficial Da Sociedade Portuguesa De Cardiologia Portuguese Journal of Cardiology an Official Journal of the Portuguese Society of Cardiology, 2004
We report a case of an asymptomatic adult patient, with several congenital malformations including an infrequent variant of double orifice mitral valve, postductal aortic coarctation, bicuspid aortic valve and an aneurysm of the right Valsalva sinus. The loss of support of the right coronary cusp of the aortic valve caused major aortic regurgitation. With the exception of the mitral valve, which was left untouched because it was neither stenotic nor regurgitant, all the other abnormalities were successfully corrected, in a two-step surgical approach. Rev Port Cardiol 2004; 23 (2) : 233-236 Palavras-Chave Cardiopatia congénita; Duplo orifício valvular mitral; Coarctação da aorta; Bicuspidia aórtica; Aneurisma de seio de Valsalva; Insuficiência aórtica
Congenital Double-Orifice Mitral Valve
Japanese Heart Journal, 1980
A case of double-orifice mitral valve (duplication of the mitral valve) in an 18-month-old baby is presented. The valve showed significant regurgitation and was replaced with a No.20 Lillehei-Kaster mitral prosthesis. Patient is kept on dipyridamole (Persantin) 10mg/Kg and aspirin 50mg/Kg daily to avoid postoperative thromboembolic complications. It is emphasized that valve replacement should be the treatment of choice in severe mitral regurgitation associated with double-orifice mitral valve.
Double orifice mitral valve: a report of two cases with different severity and treatment
AME case reports, 2023
Background: Double orifice mitral valve (DOMV), a rare congenital heart disease, is characterized by a 2-orifice MV separated by a tissue bridge, causing a spectacles-like morphology. DOMV can present with various severity ranging from asymptomatic to severe valvular dysfunction including mitral regurgitation (MR) and mitral stenosis (MS), as well as symptoms from coexisting congenital anomalies. Echocardiography is the mainstay of the investigation for a DOMV. We described two cases with DOMV who presented with different disease severity resulting in different treatment decisions. Case Description: In the first case, a 52-year-old woman presented with overt left-sided heart failure. The echocardiogram revealed DOMV with ruptured chordae tendineae of the anterior mitral valve leaflet (AMVL) causing severe MR which led the patient to undergo surgical MV replacement. Intraoperative findings confirmed a diagnosis of DOMV. After surgery, the patient could perform daily activities and light exercises without recurrent heart failure. In the second case, on the other hand, a 36-year-old woman was incidentally diagnosed with DOMV from an echocardiographic workup for symptomatic premature ventricular contraction (PVC). After controlled PVC with radiofrequency ablation, her symptom completely resolved and DOMV was classified as asymptomatic which led to the decision of a watchful waiting strategy. Conclusions: These cases highlight the diversity of DOMV manifestations and the importance of appropriate investigations, particularly echocardiography, to evaluate valvular pathology and contemplating the treatment strategy.
European Journal of Echocardiography, 2009
Double-orifice mitral valve (DOMV) is a rare congenital malformation characterized by two separate valve orifices of varying sizes in association with the abnormalities of the subvalvular apparatus. It has been reported to be associated with a variety of other congenital malformations. We report a rare case of incomplete form of Shone's complex composed of a complete bridge-type DOMV and bicuspid aortic valve, well demonstrated by both transthoracic and transoesophageal echocardiography and cardiac magnetic resonance imaging.
A case report of double-orifice tricuspid valve
International Journal of Cardiology, 1993
A heart specimen with double-orifice tricuspid valve is described. The tricuspid valve was divided into the anterior and posterior orifices by a bridge of leaflet tissue. The valve was stenotic because of the arcade deformity of the anterior papillary muscle to which the bridging leaflet tissue had short chordal insertions. The anterior orifice was regurgitant as a result of a deficient septal leaflet. Clinical correlation was obtained by magnetic resonance imaging of the specimen. The functional significance of the malformed valve was assessed by three-dimensional reconstruction of the two-dimensional magnetic resonance images.
Double-Orifice Tricuspid Valve: A Case Report and Literature Review
Echocardiography, 2015
Duplication of an atrioventricular valve is a rare congenital anomaly that usually involves the mitral rather than the tricuspid valve (TV). Isolated appearances of a double-orifice TV (DOTV) are extremely rare and in most cases are associated with other congenital anomalies. We report a rare case of an HIVseropositive male with a DOTV, divided right atrium and Wolff-Parkinson-White syndrome admitted to our hospital with palpitations and dyspnea. After medical therapy, the patient's symptoms were alleviated. We summarized the characteristics of the DOTV and reviewed the literature.