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Adult Congenital Heart Disease
Published in Takahiro Shiota, 3D Echocardiography, 2020
Pastora Gallego, Silvia Montserrat
Systematic evaluation of the congenitally malformed MV using segmental echocardiographic analysis is recommended to assist precise communication and optimal surgical management. Supravalvar region: Mitral ring in Shone syndrome (Figures 16.18 and 16.19, ).Annulus: The annulus of the MV leaflet may be normal, dilated, hypoplastic, or in extreme cases atretic.Leaflets and commissures: Carpentier classification (1976, revised 1998) is a surgical classification system to specifically facilitate the development of tailored techniques for congenital MV repair. It was based on the “predominant lesion,” as it was also observed that multisegment pathology was the most prevalent.
Electrocardiogram, chest radiograph, and ancillary investigations
Published in Neeraj Parakh, Ravi S. Math, Vivek Chaturvedi, Mitral Stenosis, 2018
Arun Sharma, Kanika Bhambri, Gurpreet S. Gulati, Neeraj Parakh
Causes of congenital MS may include association as a part of Shone’s syndrome (supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation of aorta), cor-triatriatum (Figure 7.16) or—rarely—pulmonary venous stenosis, which may present with similar findings as that of MS. Computed tomography or MRI are useful modalities where echocardiography is inadequate to provide the diagnosis.
Cardiovascular system
Published in Brian J Pollard, Gareth Kitchen, Handbook of Clinical Anaesthesia, 2017
Redmond P Tully, Robert Turner
More than 90% of cases are rheumatic in origin. Other causes include severe mitral annular calcification, infective endocarditis, inflammatory conditions (SLE and RA), left sided carcinoid syndrome, congenital causes (cor triatriatum, Shone’s syndrome, mucopolysaccharidosis) and iatrogenic (methysergide or ergot alkaloid valvulopathy).
A national registry-based study of surgery and demography comparing internationally adopted and children born in Sweden with cleft lip and/or palate
Published in Journal of Plastic Surgery and Hand Surgery, 2023
Johnna Sahlsten Schölin, Sara Rizell, Anna Paganini, Hans Mark
Among children born in Sweden with UCLP and BCLP, congenital heart malformations such as Fallot’s triad, mitral valve defects, ventricular and atrial septal defects, and pulmonary atresia were recorded for 21 children. Genital and kidney malformations were registered in six and five children, respectively. Eight children were reported as having dysmelia as well as poly- and syndactyly. Other conditions that were reported in the range of one or two Swe-born children were craniosynostosis, holoprosencephaly, corpus callosum agenesis, coloboma, lymphatic malformation, laryngomalacia, esophageal malformation, choanal atresia, clubfoot, and metabolic disorders. Syndromes and chromosomal aberrations such as Van der Woude syndrome, Wolff-Hirschorns syndrome, Charge syndrome, Shone syndrome, Goldenhaar syndrome, Saethre-Chotzen, 1p22.2 deletion syndrome, 1p36 deletion syndrome, 22q11 deletion syndrome, and trisomy 18 and 21 were registered in 19 Swedish born children.
Diagnosis of Shone syndrome on CT angiography in an adult
Published in Acta Cardiologica, 2021
Vineeta Ojha, Akash Vadher, S. H. Chandrashekhara, Amarinder Singh Malhi, Sanjeev Kumar
Shone syndrome is a rare congenital condition with multilevel obstructive lesions of the left heart, with an incidence of 0.6% of all congenital heart diseases. The complete Shone’s complex includes all the four lesions, namely mitral supravalvular membrane, parachute mitral valve (chordae from both leaflets attached to single papillary muscle), subaortic membrane and coarctation of aorta. There can be incomplete forms with a lesser number of lesions as well. Patients can have a broad spectrum of symptoms, treatments, and outcomes depending on the severity and number of defects. Patients usually present during childhood. Incidental diagnosis in adulthood, as in our patient, is rare. It is imperative to look for other components of this rare syndrome on CT angiography for accurate diagnosis and appropriate treatment. This case also highlights the role of CT angiography for the assessment of aortic lesions, especially, level and morphology of coarctation in patients with Shone’s complex.
Healthcare supply chain management: Towards 3D MRI aorta model with CPU-GPU parallel architecture for medical manufacture
Published in Cogent Medicine, 2021
Houneida Sakly, Mourad Said, Moncef Tagina
A 10-year-old child with congenital valve and valvular aortic stenosis on sensitive MRI and coarctation (operated and dilated) in the sense of shone syndrome underwent a retrospective cardiac MRI scan with 74 sequences and 3057 scans.