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Double-outlet right atrium is an extremely rare congenital cardiac defect, in which the right atrium drains into both ventricles. Here in, we present a peculiar case involving an 8-year-old female with double-outlet right atrium characterized by three distinct atrioventricular valves and a membranous ventricular septal defect. This is associated with right atrium to left ventricular flow through the accessory atrioventricular valve while maintaining adequate size of the right ventricle. Additionally, we include a comprehensive review of the literature to underscore the variability in clinical presentation and management strategies.

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Shone's syndrome (SS) is a rare congenital cardiac anomaly characterized by a spectrum of developmental abnormalities. It predominantly presents as consisting of a variety of left ventricular inflow and outflow tract lesions, with inflow tract lesions typically including parachute mitral valve and supravalvular mitral ring. However, reports of SS involving double-orifice mitral valve are scarce.

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The double-orifice tricuspid valve (DOTV) is a rare lesion. We present a series of three patients encountered at the Stollery Children's Hospital with a diagnosis of DOTV on 2D and/or 3D echocardiography. The patient's medical records are reviewed for presentation history, investigative findings, and clinical course. We discuss the cases in the context of a complete review of all literature documenting cases of DOTV. In the majority of cases, the lesion is relatively benign, with little change in valve function over the short to medium term, and outcomes are determined largely by significant concomitant heart defects.

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Double-orifice mitral valve (DOMV), a rare congenital heart disease, is the inspiration behind the transcatheter edge-to-edge repair (TEER). Here we report a successful TEER case in a patient with DOMV with severe regurgitation. The patient's symptoms were greatly improved after the procedure.

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Double orifice mitral valve is a rare congenital anomaly that is often associated with other congenital cardiac abnormalities. Patients may present with valvular dysfunction or may be asymptomatic and have an incidental diagnosis of double outlet mitral valve. Whether symptoms due to this pathology are present or not it is important to accurately characterize this lesion. Three-dimensional imaging is a powerful diagnostic modality for diagnosing and characterizing lesions such as this as highlighted in this case image series where we describe how three-dimensional transesophageal echocardiography was utilized to characterize both complete and incomplete bridge type double orifice mitral valve.

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INTRODUCTION: Double-orifice mitral valve or left atrioventricular valve is a rare congenital cardiac anomaly that may be associated with an atrioventricular septal defect. The surgical management of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect is highly challenging with acceptable clinical outcomes. This meta-analysis is aimed to evaluate the surgical outcomes of double-orifice mitral valve/double-orifice left atrioventricular valve repair in patients with atrioventricular septal defect. METHODS AND RESULTS: A total of eight studies were retrieved from the literature by searching through PubMed, Google Scholar, Embase, and Cochrane databases. Using Bayesian hierarchical models, we estimated the pooled proportion of incidence of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect as 4.88% in patients who underwent surgical repair (7 studies; 3295 patients; 95% credible interval [CI] 4.2-5.7%). As compared to pre-operative regurgitation, the pooled proportions of post-operative regurgitation were significantly low in patients with moderate status: 5.1 versus 26.39% and severe status: 5.7 versus 29.38% [8 studies; 171 patients]. Moreover, the heterogeneity test revealed consistency in the data (p < 0.05). Lastly, the pooled estimated proportions of early and late mortality following surgical interventions were low, that is, 5 and 7.4%, respectively. CONCLUSION: The surgical management of moderate to severe regurgitation showed corrective benefits post-operatively and was associated with low incidence of early mortality and re-operation.

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Background: Double orifice mitral valve (DOMV), a rare congenital heart disease, is characterized by a 2-orifice mitral valve (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.

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Ellis-van Creveld syndrome is a rare autosomal recessive disorder caused by mutations in the EVC and EVC2 genes. The four principal manifestations are chondrodysplasia, polydactyly, ectodermal dysplasia, and congenital heart defects. We describe the case of a 7-year-old girl with Ellis-van Creveld Syndrome with the diagnosis of common atrium and partial atrioventricular septal defect. She underwent a successful surgical repair, and intraoperatively, a double orifice mitral valve was diagnosed as well. The correct diagnosis of this disorder in early life is essential in the overall prognosis of the syndrome. Clinical follow-up at regular intervals is very important in these patients to institute proper managements and prevent further complications.

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OBJECTIVE: Transcatheter mitral valve repair with the MitraClip is used for the symptomatic management of mitral regurgitation (MR). The challenge is reducing MR while avoiding an elevated mitral valve gradient (MVG). This study assesses how multiple MitraClips used to treat MR can affect valve performance. METHODS: Six porcine mitral valves were assessed using an in vitro left heart simulator in the native, moderate-to-severe MR, and severe MR cases. MR cases were tested in the no-MitraClip, 1-MitraClip, and 2-MitraClip configurations. Mitral regurgitant fraction (MRF), MVG, and effective orifice area (EOA) were quantified. RESULTS: Native MRF, MVG, and EOA were 14.22%, 2.59 mm Hg, and 1.64 cm2, respectively. For moderate-to-severe MR, MRF, MVG, and EOA were 34.07%, 3.31 mm Hg, and 2.22 cm2, respectively. Compared with the no-MitraClip case, 1 MitraClip decreased MRF to 18.57% (P < .0001) and EOA to 1.50 cm2 (P = .0002). MVG remained statistically unchanged (3.44 mm Hg). Two MitraClips decreased MRF to 14.26% (P < .0001) and EOA to 1.36 cm2 (P = .0001). MVG remained unchanged (3.29 mm Hg). For severe MR, MRF, MVG, and EOA were 59.79%, 4.98 mm Hg, and 2.73 cm2, respectively. Compared with the no-MitraClip case, 1 MitraClip decreased MRF to 30.72% (P < .0001) and EOA to 1.82 cm2 (P < .0001); MVG remained unchanged (4.03 mm Hg). MVG remained statistically unchanged. Two MitraClips decreased MRF to 23.10% (P < .0001) and EOA to 1.58 cm2 (P < .0001); MVG remained statistically unchanged (3.82 mm Hg). Both MR models yielded no statistical difference between 1 and 2 MitraClips. CONCLUSIONS: There is limited concern regarding elevation of MVG when reducing MR using 1 or 2 MitraClips, although 2 MitraClips did not significantly continue to reduce MRF.

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Double orifice mitral valve (DOMV) is a rare congenital anomaly that is often associated with cardiac malformation. Valve dysfunction usually presents in childhood; therefore, most cases are diagnosed with DOMV in childhood. Its prevalence and prognostic relevance in adulthood are unknown. Here, we report a case of a 38-year-old woman who presented to the outpatient clinic with an abnormal electrocardiogram and was found to have an isolated double orifice mitral valve malformation on transthoracic echocardiography. Echocardiography is the diagnostic tool of choice for patients with double orifice mitral valves. We should familiarize ourselves with the echocardiographic features of DOMV in order to improve the detection.

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Atypical Shone's complex is a rare congenital anomaly involving a left-sided obstructive lesion of two or three cardiovascular levels. A 70-year-old man with dyspnea on exertion was diagnosed with severe aortic stenosis (AS) with a bicuspid valve, complicated by severe aortic coarctation (CoA) and a double-orifice mitral valve. He underwent surgery for AS and CoA in one session. It is important to search for complicated malformations, even in cases of bicuspid aortic valve found in old age.

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Double-orifice mitral valve is a rare congenital anomaly being associated with other cardiac defects and rarely presented in isolation. Valve function can be preserved for long and it is usually an incidental finding. We present an unusual case of double-orifice mitral valve with mitral regurgitation in a middle-aged man associated with the atrial septal defect, highlighting the role of three-dimensional transesophageal echocardiography.

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BACKGROUND: Patients with both double orifice mitral valve (DOMV) and bicuspid aortic valve (BAV) malformation are rare. Although DOMV or BAV can be detected in some genetic syndromes, it has not been reported to simultaneously appear in Turner syndrome (TS). TrueVue, TouchVue, and TrueVue Glass are the latest technologies in advanced three-dimensional echocardiography (3DE), which is an important information supplement to two-dimensional echocardiography (2DE) for the diagnosis of congenital cardiac malformations. Herein we report the novel use of the above-mentioned technologies in the diagnosis and evaluation of a rare, combined valve malformation. Meanwhile, we also reviewed the literature for cases involving both DOMV and BAV and their association with various genetic syndromes. CASE PRESENTATION: We present the case of a 5-year-old girl diagnosed with TS because of a developmental delay. DOMV and BAV were found through echocardiographic examination. Three-dimensional transthoracic echocardiography as well as a series of novel advanced techniques were applied to clearly display the spatial structure of all tiers of the mitral valve apparatus, aortic valve, and arch to facilitate an accurate diagnosis. CONCLUSIONS: This is the first case in which both DOMV and BAV were associated with TS. Innovative TrueVue and TrueVue Glass offer unprecedented photographic stereoscopic images, while TouchVue technology greatly improved the ultrasonic diagnostic workflow and the diagnostic performance of rare valve malformations by adding virtual light sources to display realistic light-shadow effects.

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This is a case of a female infant with bilateral coronary ostial atresia associated with pulmonary atresia and ventricular septal defect. She developed coronary ischemia at 1-month of age, when she underwent an aortopulmonary shunt and an aorta-right ventricle shunt. The double-orifice tricuspid valve was separating the right ventricle from the left ventricle. She required extracorporeal cardiopulmonary support because of ventricular dysfunction and mitral regurgitation. Although she was temporarily weaned off the support after mitral valvuloplasty, she died from multiple organ failure. To the best of our knowledge, bilateral coronary ostial atresia associated with pulmonary atresia with ventricular septal defect has not been reported previously.

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INTRODUCTION: Mal-alignment between the inter-atrial septum (IAS) and the inter-ventricular septum (IVS) during cardiac embryogenesis results in abnormal atrioventricular (AV) connections ranging from doubleoulet left atrium (DOLA) to double outlet right atrium (DORA)1. CASE REPORT: We report DORA resulting from partial override of the interatrial septum (IAS) across the cleft between the bridging leaflets of the left atrioventricular (AV) valve. RESULTS: Successful surgical management with preservation of the orifice is described, leading to an unusual type of double orifice left AV valve. DISCUSSION: The mal-alignment of the interatrial septum, resulting in it bridging the bridging leaflets, leads to an additional orifice between the RA and the LV and a unique form of right to left shunt. DORA can cause cyanosis depending on the size of the shunt and the compliance of the ventricles. Our patient was acyanotic, possibly due to the small size of the orifice and low LV compliance secondary to hypertrophy of the LV. CONCLUSION: our case adds to the spectrum of atrial septal mal-alignment variant of DORA in the setting of AVSD without atrial and ventricular components, with RA to LV communication occurring via the smaller orifice of a left atrioventricular valve divided by the abnormal insertion of a partially overriding, mal-aligned atrial septum; a mechanism not described previously - the 'bridge' across the bridging leaflets!

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A 37-year-old man was transferred to our level I trauma center after a road traffic accident, presenting with right acetabular fracture, multiple rib fractures, epidural hemorrhage, and liver contusion. Severe traumatic tricuspid regurgitation was also discovered during the work-up for surgery. Our initial attempt at acetabular surgery failed when the patient experienced near cardiac arrest during anesthetic induction. It was hence decided that tricuspid valve repair should precede orthopedic surgery. Minimally invasive tricuspid valve repair using the double orifice technique was successfully performed. Subsequently, acetabular surgery was performed and he was discharged 35 days post-trauma without any complications.