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: 5.6). 01/2007; 92(12):1879-85. DOI: 10.1136/hrt.2006.093344
Source: PubMed
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    • "These anatomic lesion often describe as atrioventricular canal that may be partial (PAVC)-openings between the left and right atrium and improper formation of mitral valve, and complete (CAVC)-there is a large hole where atria and ventricles meet with one common valve (2). Atrioventricular septal defect is associated with different defects including subaortic stenosis, ventricular hypoplasia, tetralogy of Fallot, atrial isomerism, and rare with pulmonary atresia (3). Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop with the only source of pulmonary blood flow-patent ductus arteriosus. "
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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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    • "It is often associated with other cardiac and extracardiac anomalies [1] [2]. "
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    ABSTRACT: Objective. The purpose of this study was to establish the outlook for fetuses diagnosed with complete atrioventricular septal defect (cAVSD) prenatally and its relation to additional cardiac, extracardiac, and chromosomal abnormalities. Methods. We retrospectively reviewed fetal echocardiograms diagnosed with cAVSD from January 2002 to December 2007, comparing fetuses with and without aneuploidy. Results. Complete antrioventricular septal defect was confirmed in 62 fetuses. Mean maternal age was 28.79 ± 4.78 years (range 20–38). Mean gestational age was 23.69 ± 5.48 weeks (range 12–38). Fetal karyotype was known in all fetuses. An abnormal karyotype was found in 21 fetuses. Complete AVSD occurred without any other intracardiac abnormality in 28 fetuses. Extracardiac anomalies were present in 38 fetuses. As for pregnancy outcomes, there were 36 (58%) terminations of pregnancy and 4 (6.4%) intrauterine fetal deaths. In these four fetuses, complex cAVSD was associated with atrioventricular block (one case), heterotaxy (one case), and fetal hydrops (two cases). Of the 22 live births, 5 were neonatal deaths without surgery while 17 babies underwent surgery and 13 have survived to date. The mean survival age was 53 ± 4 months (range 22–64 m). Conclusion. AVSD is associated with chromosomal, other cardiac, and extracardiac abnormalities. The detection of these abnormalities is important in order to give the best indication of the likely outcome when counselling parents.
    Obstetrics and Gynecology International 06/2009; 2009(3):958496. DOI:10.1155/2009/958496
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    ABSTRACT: During the past decades, health care of patients born with congenital heart disease (CHD) has improved substantially, leading to a growing population of adult survivors. Using the recently published and relevant data on adult CHD (ACHD), we reviewed the most common congenital heart defects and discussed important related issues. Adults with CHD most often require specialized medical or surgical care in a tertiary centre. However, this population also need local follow-up; general practitioners and other specialists therefore have to face the complexity of their disease. AREAS OF CONTROVERSIES: Management of pregnancy, non-cardiac surgery, arrhythmias and endocarditis prophylaxis may be challenging in patients with CHD and should be adapted to their condition. The present article summarizes key clinical information on ACHD for the benefit of physicians who are not specialized in this field. Areas timely for developing research Research efforts and education strategies are greatly needed in order to optimize the care of patients with ACHD.
    British Medical Bulletin 02/2008; 85(1):151-80. DOI:10.1093/bmb/ldn005 · 3.66 Impact Factor
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