Atrioventricular septal defect: from fetus to adult.

Royal Belfast Hospital for Sick Children, Belfast, Northern Ireland, BT12 6BE, UK.
Heart (British Cardiac Society) (Impact Factor: 6.02). 01/2007; 92(12):1879-85. DOI: 10.1136/hrt.2006.093344
Source: PubMed
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    ABSTRACT: Cardiac affection is a common issue in the Down syndrome (DS) population, in the form of both congenital and acquired heart disorders. There are different types of congenital heart disease (CHD) in children with Down syndrome (DS). Echocardiography plays an important role in the detection of both structural and functional abnormalities in this group of patients. Fetal echocardiography can help in the early recognition of Down syndrome by detecting soft markers of DS, but its main role is to define the exact nature of the suspected cardiac problem in the fetus. Postnatal echocardiography is mandatory in the first month of life for all neonates with DS. It is also indicated before any cardiac surgery and for serial follow-up after cardiac surgery. In this Lecture, we discuss the types and mechanism of cardiac abnormalities in DS children and the role of both fetal and postnatal echocardiography in the detection of these abnormalities.
    First International Pediatric Conference, International Hospital of Bahrain, Kingdom of Bahrain, Mabama, Regency InterContinental Hotel, Bahrain; 10/2013
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    ABSTRACT: Atrioventricular septal defect with common atrioventricular junction is a rare adult congenital cardiac syndrome. This occurrence with prolonged survival is exceptionally rare. We present the case of a patient who presented with this defect with common atrioventricular junction who survived to the age of 32. We describe a 32-year-old man with atrioventricular septal defect with common atrioventricular junction guarded by a common valve. His history, clinical course, and anatomic findings are discussed along with the factors which may have contributed to his longevity, which is unique in the medical literature. His management reflected the state of medical knowledge at the time when he presented, and although alternate approaches may have been utilized if the patient presented today. We discuss the findings, frequency, classifi cation, and management of congenital defects. Development of embryonic structure is altered by interaction between genetics and environmental factors toward a rare associated of congenital cardiac defects-complex congenital heart disease. This case demonstrates that patients with very complex congenital cardiac disease may survive to adulthood, presenting challenges in both medical and surgical treatment.
    Acta Informatica Medica 12/2013; 21(4):293-294. DOI:10.5455/aim.2013.21.293-294
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    ABSTRACT: Atrioventricular valve regurgitation (AVVR) is a clinically important element of the common atrioventricular canal defect. Cardiac preload and afterload increase from prenatal to postnatal life. These hemodynamic changes may increase the degree of regurgitation and affect management and prognosis. We sought to investigate the frequency of change in degree of AVVR from fetal to postnatal life in this patient population. Subjects who underwent both fetal and postnatal echocardiography within 4 weeks of life between January 2008 and September 2010 were included in the study. Degree of AVVR was assessed by color Doppler imaging and scored as 0 (no regurgitation), 1 (hemodynamically insignificant regurgitation), and 2 (hemodynamically important regurgitation). Forty-nine subjects were included. Mean gestational age at fetal echocardiogram was 34 ± 2.8 weeks; age at postnatal echocardiogram was a median of <24 h of age (range 0-24). After birth, 69 % subjects had no change, 8 % of subjects had a decrease, and 22 % subjects had an increase in AVVR grade. Five patients progressed from a fetal score 0 or 1 to postnatal score 2. Neither trisomy 21 nor heterotaxy syndrome were risk factors for progression of AVVR. In patients with AV canal defects, 90 % demonstrate no hemodynamically significant change in AVVR from fetal to postnatal life, whereas 10 % display a hemodynamically significant change. AVVR appreciated in utero is predictive of neonatal regurgitation in the majority of patients. These findings have implications for the counseling and management of the fetus with AV canal defect.
    Pediatric Cardiology 05/2013; 34(8). DOI:10.1007/s00246-013-0711-7 · 1.55 Impact Factor


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