Double-Orifice Tricuspid Valve in an Infant with Multiple Noncardiac Anomalies
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ABSTRACT:
SUBMITTER: Henning R
PROVIDER: S-EPMC9270670 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Highlights•Tricuspid regurgitation with mitral valve disease is associated with poor outcomes.•Transcatheter edge-to-edge repair of the tricuspid valve can be done successfully.•Intraprocedural echocardiography is essential for transcatheter tricuspid therapies.
| S-EPMC6200677 | biostudies-other
Project description:OBJECTIVES:Pledget-assisted suture tricuspid valve annuloplasty (PASTA) is a novel technique using marketed equipment to deliver percutaneous trans-annular sutures to create a double-orifice tricuspid valve. BACKGROUND:Tricuspid regurgitation is a malignant disease with high surgical mortality and no commercially available transcatheter solution in the US. METHODS:Two iterations of PASTA were tested using trans-apical or trans-jugular access in swine. Catheters directed paired coronary guidewires to septal and lateral targets on the tricuspid annulus under fluoroscopic and echocardiographic guidance. Guidewires were electrified to traverse the annular targets and exchanged for pledgeted sutures. The sutures were drawn together and knotted, apposing septal and lateral targets, creating a double orifice tricuspid valve. RESULTS:Twenty-two pigs underwent PASTA. Annular and chamber dimensions were reduced (annular area, 10.1?±?0.8 cm2 to 3.8?±?1.5 cm2 (naïve) and 13.1?±?1.5 cm2 to 6.2?±?1.0 cm2 (diseased); septal-lateral diameter, 3.9?±?0.3 mm to 1.4?±?0.6 mm (naïve) and 4.4?±?0.4 mm to 1.7?±?1.0 mm (diseased); and right ventricular end-diastolic volume, 94?±?13 ml to 85?±?14 ml (naïve) and 157?±?25 ml to 143?±?20 ml (diseased)). MRI derived tricuspid regurgitation fraction fell from 32?±?12% to 4?±?5%. Results were sustained at 30 days. Pledget pull-through force was five-fold higher (40.6?±?11.7N vs 8.0?±?2.6N, P?<?.01) using this strategy compared to single puncture techniques used to anchor current investigational devices. Serious complications were related to apical access. CONCLUSIONS:PASTA reduces annular dimensions and tricuspid regurgitation in pigs. It may be cautiously applied to selected patients with severe tricuspid regurgitation and no options. This is the first transcatheter procedure, to our knowledge, to deliver standard pledgeted sutures to repair cardiac pathology.
| S-EPMC6078835 | biostudies-literature
Project description:Duplication of atrioventricular valves involves the mitral valve more often than the tricuspid valve and is often associated with other cardiac defects. Double-orifice tricuspid valve (DOTV) is often identified in surgery or autopsy and missed on echocardiography, as the two orifices are orthogonal to the imaging plane. If suspected on echocardiography, it masquerades as mild tricuspid hypoplasia. Three-dimensional echocardiography and magnetic resonance imaging of a DOTV are presented.
| S-EPMC6716302 | biostudies-literature
Project description:Highlights•DOMV is rarely diagnosed in adult patients.•Echocardiography can be used to find and describe the anomaly.•DOMV is often associated with other cardiac abnormalities.•This is the first report of DOMV associated with an accessory pathway.
| S-EPMC6058762 | biostudies-other
Project description: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.
| S-EPMC4408625 | biostudies-literature
Project description: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.
| S-EPMC8617400 | biostudies-literature
Project description: Not available
| S-EPMC7451879 | biostudies-literature
Project description:BackgroundCardiac lipomas are rare benign primary tumours of the heart. Due to the nature of these tumours, they are often asymptomatic and diagnosed incidentally. Whether asymptomatic patients with cardiac lipomas should perform surgery still remains controversial.Case summaryA 34-year-old Asian male who was incidentally found hyperechoic masses in the right ventricle (RV) on the transthoracic echocardiogram by annually routine physical examination was admitted to our cardiology department. His medical history was unremarkable. The repeated transthoracic and transoesophageal echocardiogram showed multiple solitary and well-demarcated masses in the RV. On the cardiac magnetic resonance imaging, four discrete masses (considering the possibility of it being a lipoma) partially occluding the right ventricular outflow tract (RVOT) were observed. During the open-heart resection surgery, it was found that the tricuspid valve and papillary muscle were covered by multiple adipose masses in the RV that arose from the interventricular septum and the free wall, resulting in partial RVOT obstruction. These excised masses were histopathologically confirmed as lipomata characterized by the mature adipocytes with entrapped myocardial cells. The patient had no cardiac abnormality in the 1-month follow-up after the surgery.DiscussionThis rare clinical case of multiple lipomata of the tricuspid valve and papillary muscle acknowledges that multimodality imaging is the cornerstone for the assessment and diagnosis. Surgery should be performed in cases of symptomatic or large lipomas as well as when a lipoma is considered to be high risk because of RVOT obstruction.
| S-EPMC8343444 | biostudies-literature
Project description:Sotos syndrome is an autosomal dominant condition, sometimes complicated with cardiovascular malformations. We report the case of a 10-year-old Japanese male with Sotos syndrome found to have double orifice mitral valve (DOMV) combined with left ventricular noncompaction (LVNC) by transthoracic echocardiography. Three-dimensional echocardiography clearly demonstrated the trabecular meshwork, two separate mitral orifices with subvalvular apparatuses, and multiple tendinous cords. To the best of our knowledge, this is the first case of Sotos syndrome associated with DOMV and LVNC. Considering that mitral valve leaflets, chordae, papillary muscles, and primitive trabeculations of the left ventricle originate from the endomyocardial cushions in the developing heart, both cardiac defects in the present case might be explained by a common developmental aberrancy of endomyocardium. Patients with Sotos syndrome should be screened for mitral valve anomaly, subvalvular apparatuses, and left ventricular myocardial function as well as structural abnormalities. <Learning objective: Sotos syndrome is sometimes complicated with cardiovascular malformations, including left ventricular noncompaction (LVNC) as reported in some previous articles. Considering the rarity of both the syndrome and LVNC, this combination might not be coincidental. It is intriguing that the present case had double orifice mitral valve in addition to LVNC, as both mitral valve leaflets and primitive trabeculations of LV might be derived in common from aberrant development of the endomyocardial cushions.>.
| S-EPMC6279981 | biostudies-literature
Project description:Double-orifice mitral valve (DOMV) is a rare congenital anomaly consisting of an accessory bridge of fibrous tissue, which divides the mitral valve (MV) into two orifices. The mitral leaflets are essentially normal in most cases, but they can be regurgitant or stenotic. It is most commonly associated with a variety of other cardiac anomalies. Isolated DOMV with normal MV function is very rare. We present here a rare case of congenital DOMV in a 25-year-old female diagnosed by real-time three-dimensional echocardiography (RT3DE). RT3DE enabled complete anatomical and functional assessment of MV apparatus. It added much valuable information over conventional 2DE that helped in establishment of the diagnosis, identification of the anatomical type, and selection of the proper management.
| S-EPMC8021078 | biostudies-literature