ABSTRACT
Objective:To analyze the characteristics and surgical outcomes of Shone′s syndrome in children, and to explore the surgical treatment strategy and technical key.Methods:Retrospective study.Children with Shone′s syndrome treated in the Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University from May 2013 to June 2019 were retrospectively analyzed for their baseline characteristics and surgical data.The data were compared by Student t-test. Results:A total of 22 children with Shone′s syndrome were recruited, involving 15 males (68.2%) and 7 females (31.8%). There were 6 cases (27.3%) of complete form of Shone′s syndrome and 16 cases (72.7%) of incomplete form.No deaths were reported.The postoperative mitral valve velocity [(149.7±38.2) cm/s vs.(234.9±34.0) cm/s, t=7.341, P<0.05], left ventricular outflow tract velocity [(202.0±105.0) cm/s vs.(328.6±120.3) cm/s, t=6.575, P<0.05] and aortic arch coarctation velocity [(186.1±60.9) cm/s vs.(347.9±100.8) cm/s, t=7.630, P<0.05]were significantly lower than those of preoperative levels.There were no complications occurred at 1-year follow-up, and 91.7% of the patients were followed up for 3 years, and 80.2% were followed up for 5 years without complications, 2 cases needed reoperation. Conclusions:Surgical treatment of Shone′s syndrome achieved satisfactory outcomes.Early diagnosis and early intervention are beneficial to children with Shone′s syndrome, although they need to be followed up and have the risk of reoperation in the long term.
ABSTRACT
Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.
Subject(s)
Adult , Female , Humans , Aortic Coarctation , Congenital, Hereditary, and Neonatal Diseases and Abnormalities , Constriction, Pathologic , Diagnosis , Follow-Up Studies , Heart , Heart Defects, Congenital , Mitral ValveABSTRACT
The parachute mitral valve is a rare group of heart valve diseases that are typically characterized by only one papillary muscle.All the chordae tendineaes are connected to the papillary muscle and shaped like a para-chute.It mainly causes mitral stenosis and occasionally leads to mitral regurgitation. Due to the early appearance of clinical symptoms and rapid development of the disease,early accurate diagnosis and surgical treatment are needed. Common surgical treatments are mitral valve repair and mitral valve replacement.Now,the progress of surgical treatment of ＂parachute－like＂ mitral valve malformation was reviewed in order to provide a meaningful reference for its surgical treatment.
ABSTRACT
Objective To explore the value of echocardiography in the diagnosis of the parachute mitral valve(PMV).Methods The echocardiographic characteristics of 12 patients with PMV between 2005 and 2012 were reviewed retrospectively,including the morphology,movement and blood flow of mitral valve apparatus,and other associated cardiac abnormalities.Results Of the mitral valve leaflets in these 12 cases,all were thickened and opened restricted in diastole.Axial view showed that in all cases mitral valves opened eccentrically and in 2 cases they didn't close tightly.Each patient only had single papillary muscle in left ventricle,which received all chords of mitral valves.Of papillary muscles in these patients,4 were located at posteromedial wall,3 at anterolateral wall,2 at middle posterior wall,2 at apical wall,and 1 at posterolateral wall.Of the 12 patients,only one were simple PMV,11 had associated cardiovascular anomalies,2 had mild regurgitation in systolic period and 11 had increased trans-valve peak flow velocity.Conclusions Echocardiography is reliable in the diagnosis of PMV,which could provide a comprehensive evaluation of valvular lesions and other associated cardiovascular anomalies.
ABSTRACT
El síndrome de Shone constituye una rara entidad cardíaca congénita que consiste en la presencia de lesiones estructurales obliterantes de hemicardio izquierdo que incluyen: anillo supravalvular, válvula mitral en paracaídas, estenosis sub-aórtica y coartación aórtica. Es poco frecuente que curse en su forma incompleta. El caso que a continuación se presenta, describe el síndrome de Shone incompleto detectado en un adulto
Shone's syndrome is a rare congenital heart disease consisting in the presence of obliterating structural lesions on the left side of the heart, including supravalvular ring, parachute mitral valve, subaortic stenosis and aortic coarctation. The incomplete form of the disease is not common. The presentation describes a case of incomplete Shone's syndrome diagnosed in adulthood
Subject(s)
Humans , Male , Adult , Cardiovascular Diseases/congenital , Cardiovascular Diseases/diagnosisABSTRACT
Double-orifice mitral valve is a rare congenital anomaly. Most cases of double-orifice mitral valve are hemodynamically normal and remain symptomless, so that it is usually discovered incidentally in autopsy or during surgical correction of a cardiovascular abnormality. Recently, however, it is increasingly recognized as such, since the echocardiography has gained wide acceptance as a non-invasive diagnostic tool by the M-mode, two-dimensional and color Doppler echocardiogram. Two separate mitral valve apparatuses can be used on the M-mode echocardiogram. In the two-dimensional echocardiography, the parasternal and subcostal short-axis views can show two separate glass-like orifices in the left ventricle, and the parasternal long-axis view as well as the apical four-chamber view can show the anomaly. And the color Doppler echocardiogram can visualize two mosaic-pattern flows between the left atrium and ventricle. We present herewith two cases of double-orifice mitral valve, as diagnosed by means of echocardiography. The first case was an isolated one with mitral stenosis, showing two parachute mitral valves. The second was associated with perimembranous ventricular septal defect, and showed the accessory mitral valve directly attached to the ventricular septum, with the chordae crossing the ventricular outflow tract.