La sindrome del cuore sinistro ipoplasico è caratterizzata da un ventricolo sinistro piccolo,
talvolta virtuale, stenosi critica o atresia delle valvole aortica e/o della mitrale e
ipoplasia dell’aorta ascendente e dell’arco dell’aorta, atrio sinistro piccolo,coartazione dell’aorta che
è la malformazione associata più frequente.
Rappresenta circa 1% di tutte le malformazioni cardiache congenite e, se non trattata,
ha la più alta mortalità nel primo mese di vita.
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Unguarded tricuspid valvar orifice in the fetus
Kenny K. Wong; Duncan I. Farquharson; Walter J. Duncan
Cardiology in the Young, October 2004, vol. 14, no. 5, pp. 557-559(3)
Publisher: Greenwich Medical Media
Abstract:
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Creazione in utero di un difetto nel setto atriale in feti con Sindrome del Cuore Sinistro Ipoplasico e setto atriale intatto o fortemente restrittivo.
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Ultrasound Obstet Gynecol 2004; 24: 629–632
Published online in Wiley InterScience (www.interscience.wiley.com). DOI: 10.1002/uog.1753
Prenatal diagnosis of Shone’s syndrome: parental counseling and clinical outcome
N. ZUCKER, A. LEVITAS and E. ZALZSTEIN
Pediatric Cardiology Unit, Department of Pediatrics, Soroka University Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel
KEYWORDS: fetal echocardiography; prenatal diagnosis; Shone’s syndrome
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La sindrome di Shone comprende quattro anomalie :
1- una membrana sopravalvolare mitralica
2- mitrale a paracadute
3- stenosi subaortica (membranosa o muscolare)
4- coartazione aortica
Esiste anche una forma incompleta in cui si riconoscono solo due o tre componenti. Ha una scarsa prognosi chirurgica con una mortalità tra il 24-27% e richiede interventi multipli con una scarsa prognosi a distanza.
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A study of congenital cardiac disease in a neonatal population – the validity of echocardiography undertaken by a neonatologist
Authors: Gregory R. Samson; Suresh R. Kumar
Source : Cardiology in the Young, December 2004, vol. 14, no. 6, pp. 585-593(9)
Publisher: Greenwich Medical Media
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Prenatal diagnosis of tetralogy of Fallot associated with
a fistula from the left coronary artery to the left atrium
Mohammed D. Khan, Sivasankaran Sivasubramonian, John M. Simpson
Department of Congenital Heart Disease, Guy’s Hospital, London, UK
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The Challenge
Long QT syndrome (LQTS) is a disorder of cardiac repolarization. It is characterized by a prolongation of the QT interval, and a predisposition to ventricular tachyarrhythmias, which are associated with syncope, arrhythmic events, and sudden cardiac death (SCD). In recent years, there have been significant advances in understanding the genetic basis of the syndrome. Newer genetic forms of LQTS have been identified, too, plus a laboratory test has become available to help in the diagnosis of the syndrome. As knowledge of LQTS continues to expand, clinical applications of this growing body of information need further study
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