scholarly journals Secundum Type Atrial Septal Defect in Patients with Trisomy 21—Therapeutic Strategies, Outcome, and Survival: A Nationwide Study of the German National Registry for Congenital Heart Defects

2021 ◽  
Vol 10 (17) ◽  
pp. 3807
Author(s):  
Astrid E. Lammers ◽  
Julia Stegger ◽  
Marc-André Koerten ◽  
Paul C. Helm ◽  
Ulrike M. Bauer ◽  
...  
Keyword(s):  
Atrial Septal Defect ◽  
Congenital Heart Defects ◽  
Trisomy 21 ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Defects ◽  
Normal Population ◽  
Preschool Age ◽  
Pulmonary Vascular Disease ◽  
Asd Closure

(1) Secundum type atrial septal defect (ASD II) is usually considered a relatively benign cardiac lesion amenable to elective closure at preschool age. Patients with trisomy 21 (T21), however, are known to have a higher susceptibility for pulmonary vascular disease (PVD). Therefore, T21 children may present with clinical symptoms earlier than those without associated anomalies. In addition, early PVD may even preclude closure in selected T21 patients. (2) We performed a retrospective analysis of the German National Register for Congenital Heart Defects including T21 patients with associated isolated ASD II. We report incidence, demographics, therapeutic strategy, outcome, and survival of this cohort. (3) Of 46,628 patients included in the registry, 1549 (3.3%) had T21. Of these, 156 (49.4% female) had an isolated ASD II. Fifty-four patients (34.6%) underwent closure at 6.4 ± 9.9 years of age. Over a cumulative follow-up (FU) of 1148 patient-years, (median 7.4 years), only one patient developed Eisenmenger syndrome and five patients died. Survival of T21 patients without PVD was not statistically different to age- and gender-matched controls from the normal population (p = 0.62), whereas children with uncorrected T21/ASD II (including patients with severe PVD, in whom ASD-closure was considered contraindicated) showed a significantly higher mortality. (4) The outcome of T21-patients with ASD II and without PVD is excellent. However, PVD, either precluding ASD-closure or development of progressive PVD after ASD-closure, is associated with significant mortality in this cohort. Thus T21 patients with ASD II who fulfill general criteria for closure and without PVD should be offered defect closure analogous to patients without T21.

Atrial Septal Stent Implant: Atrial Septal Defect Creation in the Management of Complex Congenital Heart Defects in Infants

2006 ◽  
Vol 1 (3) ◽  
pp. 129-135 ◽  
Author(s):  
Glenn T. Leonard ◽  
Henri Justino ◽  
Karina M. Carlson ◽  
Joseph W. Rossano ◽  
Steven R. Neish ◽  
...  
Keyword(s):  
Atrial Septal Defect ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Defects ◽  
Defect Creation

Genetic variation in the COL6A1 region is associated with congenital heart defects in trisomy 21 (Down's syndrome)

1995 ◽  
Vol 59 (3) ◽  
pp. 253-269 ◽  
Author(s):  
G. E. DAVIES ◽  
C. M. HOWARD ◽  
M. J. FARRER ◽  
M. M. COLEMAN ◽  
L. B. BENNETT ◽  
...  
Keyword(s):  
Genetic Variation ◽  
Congenital Heart Defects ◽  
Trisomy 21 ◽  
Congenital Heart ◽  
Heart Defects ◽  
Down's Syndrome ◽  
Down’S Syndrome

scholarly journals Penanganan Perioperatif Pasien Penyakit Jantung Kongenital Dewasa dengan ASD, Suspek Hipertensi Pulmonal, LV Smallish

2017 ◽  
Vol 9 (2) ◽  
pp. 71
Author(s):  
Wisnhu Wardhana ◽  
Cindy Elfira Boom
Keyword(s):  
Intensive Care Unit ◽  
Congenital Heart Disease ◽  
Intensive Care ◽  
Heart Disease ◽  
Atrial Septal Defect ◽  
Patent Foramen Ovale ◽  
Congenital Heart ◽  
Septal Defect ◽  
Foramen Ovale ◽  
Asd Closure

Penyakit jantung kongenital dewasa / grown-up congenital heart disease   (GUCH) yang menempati urutan teratas dengan insidensi 10% dari jantung kongenital asianotik pada dewasa adalah atrial septal defect (ASD). Terapi optimal ASD masih kontroversial. Operasi direkomendasikan pada pasien usia pertengahan dan usia tua dengan pintasan kiri ke kanan yang bermakna. Komorbid yang paling sering didapatkan pada defek kongenital pada usia dewasa muda adalah gangguan hemodinamik, hipertensi pulmonal, aritmia,  penyakit kardiovaskular dan penyakit resprasi. Dilaporkan pasien perempuan usia 29 tahun dengan atrial septal defect(ASD) dengan hipertensi pulmonaldan Left Ventricle (LV) Smallishyang dilakukan operasi penututupan defek atrial atau ASD closure. Persiapan preoperasi mencakup anamnesa, pemeriksaan fisik dan pemeriksaan penunjang.Perubahan patologi utama adalah peningkatan resistensi vaskuler paru dan perubahan sekunder terhadap peningkatan aliran darah dari pintasan kiri ke kanan. Masalah yang dihadapi pada pasien  perioperasi ini adalah ukuran jantung kiri baik atrium maupun ventrikel kiri yang kecil memberikan dampak hemodinamik tidak stabil berupa aritmia dan pulmonal hipertensi saat dilakukan penutupan defek. Pemberianobat topangan jantung (nitroglyserin, milrinone, norepinephrine, adrenaline) dan pembuatan Patent Foramen Ovale (PFO) memberikan hasil hemodinamik yang stabil selama operasi dan  di ruang perawatan Intensive Care Unit (ICU).

scholarly journals Pattern and frequency of pediatric congenital heart disease at the Cardiac Research Institute of Kabul Medical University, Afghanistan

2018 ◽  
Vol 58 (3) ◽  
pp. 106-9
Author(s):  
Abdul Muhib Sharifi
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Atrial Septal Defect ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Defects ◽  
Cardiac Lesions ◽  
Medical University ◽  
Research Institute

Background Congenital heart disease (CHD) is the most common birth defect, with incidence of 0.7-0.9 live birth; it increases to 2-6% if first degree relative is affected. In Afghanistan majority of births take place at home and routine screening of neonates is not common, so true birth prevalence of CHD cannot be possibly calculated. Therefore, true prevalence of CHD in our population is unknown. Objective To verify the current pattern and frequency distribution of congenital heart disease (CHD) at the Cardiac Research Institute of Kabul Medical University. Methods  This retrospective study was conducted in children aged 0-14 years, who underwent echocardiography for possible congenital heart disease from January 2015 to December 2016. Results  Of 560 patients who underwent echocardiography, 392(70%) had cardiac lesions. Congenital cardiac lesions were found in 235 (60% of those with lesions) patients, while 157 (40%) patients had rheumatic heart disease. Patients with CHD were further subdivided into acyanotic and cyanotic groups. The majority of acyanotic group had isolated atrial septal defect (55%) while the most common lesion in the cyanotic group was Tetralogy of Fallot (42%). Conclusion Congenital heart defects are the most common heart disease in the pediatric population presenting at the Cardiac Research Institute of Kabul Medical University. Atrial septal defect (ASD) was the most common acyanotic defect, while Tetralogy of Fallot (ToF) is the most common cyanotic defect.

scholarly journals Left Ventricular Noncompaction Is More Prevalent in Ventricular Septal Defect Than Other Congenital Heart Defects: A Morphological Study

2020 ◽  
Vol 7 (4) ◽  
pp. 39
Author(s):  
Laís Costa Marques ◽  
Gabriel Romero Liguori ◽  
Ana Carolina Amarante Amarante Souza ◽  
Vera Demarchi Aiello
Keyword(s):  
Ventricular Septal Defect ◽  
Congenital Heart Defects ◽  
Morphological Study ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Defects ◽  
Atrioventricular Septal Defect ◽  
Left Ventricular ◽  
Left Ventricular Noncompaction ◽  
Ventricular Noncompaction

Left ventricular noncompaction (LVNC) is a condition characterized by prominent ventricular trabeculae and deep intertrabecular recesses and has been described as a possible substrate for arrhythmias, thromboembolism, and heart failure. Herein, we explored the prevalence of LVNC morphology among hearts with congenital heart defects (CHD). We examined 259 postnatal hearts with one of the following CHD: isolated ventricular septal defect (VSD); isolated atrial septal defect (ASD); atrioventricular septal defect (AVSD); transposition of the great arteries (TGA); isomerism of the atrial appendages (ISOM); Ebstein’s malformation (EB); Tetralogy of Fallot (TF). Eleven hearts from children who died of non-cardiovascular causes were used as controls. The thickness of the compacted and non-compacted left ventricular myocardial wall was determined and the specimens classified as presenting or not LVNC morphology according to three criteria, as proposed by Chin, Jenni, and Petersen. Normal hearts did not present LVNC, but the CHD group presented different percentages of LVNC in at least one diagnostic criterium. The prevalence of LVNC was respectively, according to Chin’s, Jenni´s and Petersen´s methods: for VSD—54.2%, 35.4%, and 12.5%; ASD—8.3%, 8.3%, and 8.3%; AVSD—2.9%, 2.9%, and 0.0%; TGA—22.6%, 17%, and 5.7%; ISOM—7.1%, 7.1%, and 7.1%; EB—28.6%, 9.5%, and 0.0%; TF—5.9%. 2.9%, and 2.9%. VSD hearts showed a significantly greater risk of presenting LVNC when compared to controls (Chin and Jenni criteria). No other CHD presented similar risk. Current results show some agreement with previous studies, such as LVNC morphology being more prevalent in VSDs. Nonetheless, this is a morphological study and cannot be correlated with symptoms or severity of the CHD.

657: Intrapartum fetal heart rate characteristics of fetuses with trisomy 21 and congenital heart defects

2011 ◽  
Vol 204 (1) ◽  
pp. S259-S260
Author(s):  
Priyadarshini Koduri ◽  
Maria Adelaida Giraldo ◽  
Phillip Shlossman ◽  
Anthony Sciscione ◽  
Vincenzo Berghella ◽  
...  
Keyword(s):  
Heart Rate ◽  
Congenital Heart Defects ◽  
Fetal Heart Rate ◽  
Trisomy 21 ◽  
Congenital Heart ◽  
Fetal Heart ◽  
Heart Defects
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