Congenital heart defect (CHD) is common in infants with Down syndrome (DS), which is the principle cause of mortality. However, there is no data available for the frequency and types of CHD in infants with DS in Korea. We investigated the frequency of CHD in infants with DS in Korea. After the survey on birth defects was conducted throughout the country, the prevalence of CHD in DS in 2005-2006 was calculated. This study was conducted based on the medical insurance claims database of the National Health Insurance Corporation. The number of total births in Korea was 888,263 in 2005-2006; of them, 25,975 cases of birth defects were identified. The prevalence of DS was 4.4 per 10,000 total births, accounting for 1.5% of all birth defects. Of the 394 infants with DS, 224 (56.9%) had a CHD. Atrial septal defect was the most common defect accounting for 30.5% of DS followed by ventricular septal defect (19.3%), patent duct arteriosus (17.5%), and atrioventricular septal defect (9.4%). Our study will be helpful to demonstrate the current status of DS and to identify the distribution of CHD in infants with DS in Korea.
Adult ; Asian Continental Ancestry Group ; Birth Weight ; Chromosome Aberrations ; Databases, Factual ; Down Syndrome/*complications ; Ductus Arteriosus, Patent/epidemiology ; Female ; Gestational Age ; Heart Defects, Congenital/*epidemiology/etiology ; Heart Septal Defects/epidemiology ; Heart Septal Defects, Atrial/epidemiology ; Heart Septal Defects, Ventricular/epidemiology ; Humans ; Male ; Prevalence ; Republic of Korea/epidemiology

INTRODUCTIONOrthodeoxia is a rare clinical syndrome characterised by dyspnoea and arterial deoxygenation that accompanies a change from a supine to erect position.

CLINICAL PICTUREWe describe an unusual case of "paradoxical orthodeoxia" in a 70-year-old man with a thoracic aortic aneurysm: arterial desaturation when supine that improved when erect.

TREATMENT AND OUTCOMENon-invasive imaging revealed compression of the left pulmonary artery by the aneurysm (thoracic computed tomography) and patent foramen ovale (transesophageal echocardiography). Nuclear studies show decreased relative left lung perfusion attributable to the former, and right-to-left atrial shunt attributable to the latter. The degree of right-to-left shunt increases in the supine position: nuclear pulmonary shunt study shows shunt extent of 21% when supine versus 10% erect.

CONCLUSIONA physioanatomical explanation is proposed.

Aged ; Aortic Aneurysm, Thoracic ; epidemiology ; Dyspnea ; etiology ; Echocardiography, Transesophageal ; Female ; Heart Septal Defects, Atrial ; epidemiology ; Humans ; Oxygen ; blood ; Posture ; physiology ; Supine Position ; physiology

We investigated the livebirths prevalence and occurrence pattern of birth defects in Korea. After the survey on birth defects was done in 2,348 medical institutions around the nation, the birth defect prevalence of livebirths in 2005-2006 was calculated. This study was based on the medical insurance claims database of the National Health Insurance Corporation. The number of livebirths in Korea was 883,184 from 2005-2006, and 25,335 cases of birth defects were notified to our study, equivalent to a prevalence of 286.9 per 10,000 livebirths. Anomalies of the circulatory system were the most common defects, accounting for 43.4% of birth defects with a prevalence of 124.5 per 10,000 livebirths. It was followed by the musculoskeletal system anomalies, the digestive system anomalies, and the urinary system anomalies. The five major birth defects based on the ranking of prevalence were atrial septal defect, ventricular septal defect, hydronephrosis, patent ductus arteriosus, and cleft lip/palate. Birth defects in livebirths were associated with a high proportion of low birthweight, prematurity, multiple births and advanced maternal age. The prevalence of birth defects in Korea is similar to or lower than those reported in developed countries. Our study suggests baseline data to explain the current status of birth defects and to establish a registry system of birth defects in Korea.
Adult ; Asian Continental Ancestry Group ; Cleft Lip/epidemiology ; Cleft Palate/epidemiology ; Congenital Abnormalities/*epidemiology ; Databases, Factual ; Ductus Arteriosus, Patent/epidemiology ; Female ; Gestational Age ; Heart Septal Defects, Atrial/epidemiology ; Heart Septal Defects, Ventricular/epidemiology ; Humans ; Hydronephrosis/epidemiology ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Live Birth ; Male ; Maternal Age ; Musculoskeletal Abnormalities/epidemiology ; Pregnancy ; Prevalence ; Republic of Korea/epidemiology

OBJECTIVEIn this study, we attempted to observe the prevalence and risk factors of atrial tachyarrhythmias (AT) before and after transcatheter closure of atrial septal defect (ASD).

METHODS264 adult patients aged over 40 years (67 men and 197 women) who underwent transcatheter closure of ASD between September, 1997 and December, 2005 were included in this study. Incidence of preoperative and postoperative AT was analyzed, risk factors for AT were determined with multivariate stepwise logistic regression analysis.

RESULTSIncidence of AT before closure was 9.1% (24/264). Twenty-nine patients (11.0 percent) developed AT after transcatheter closure (24 atrial fibrillation, 1 paroxysmal flutter, 4 paroxysmal atrial arrhythmia). The prevalence in patients of 40 to 49 years, 50 to 59 years and above 60 years was 4.3%, 14.6% and 26.3%, respectively. Most patients with atrial fibrillation were symptomatic. Compared to patients without AT, patients developed AT after closure were significantly older (53.0 +/- 7.6 years vs. 47.8 +/- 6.6 years, P < 0.01) and had larger defects (23.5 +/- 5.7 mm vs. 21.3 +/- 5.2 mm, P > 0.05), higher systolic pulmonary pressure (38.4 +/- 13.1 vs. 34.1 +/- 10.1, P < 0.05), larger left atrium dimension [(38.0 +/- 3.9) mm Hg (1 mm Hg = 0.133 kPa) vs. (33.6 +/- 4.4) mm Hg, P < 0.01], larger end diastolic right ventricular dimension [(34.7 +/- 5.9) mm vs. (32.1 +/- 6.8) mm, P > 0.05], higher incidence of tricuspid regurgitation (96.6% vs. 75.3%, P = 0.01), higher incidence of preoperative AT (51.7% vs. 3.8%, P < 0.01) and higher incidence of hypertension (27.6% vs. 10.2%, P = 0.013). Multivariate analysis showed that older age [odds ratio (OR) 2.659, 95 percent confidence interval (CI) 1.080 to 6.547, P < 0.05], presence of preoperative AT (OR 54.311, CI 9.819 to 300.395, P < 0.01), and left atrial enlargement (OR 8.529 per 10 mm increment, CI 2.162 to 33.643, P < 0.01) were independent predictors of AT after closure.

CONCLUSIONSIncidence of AT was similar before and after percutaneous closure in patients with atrial septal defects aged 40 years and over. The risk of AT is related to the age at the time of transcatheter closure, the presence of preoperative AT and enlarged left atria.

Adult ; Age Factors ; Aged ; Balloon Occlusion ; Female ; Heart Septal Defects, Atrial ; etiology ; therapy ; Humans ; Incidence ; Male ; Middle Aged ; Risk Factors ; Tachycardia ; epidemiology ; Treatment Outcome

OBJECTIVETo report the results of transcatheter interventional therapy (TIT) of congenital heart disease (CHD) register from 23 medical centers in China.

METHODIn this retrospective multicenter registry study, clinical data from 5808 patients who underwent TIT between January 2008 to December 2010 in 23 Chinese medical centers in 14 cities were analyzed.

RESULTSProcedure was successful in 5720 cases (98.5%), success rate was 99.5% for PDA, 98.8% for ASD, 97.4% for VSD and 98.5% for pulmonary stenosis (PS). Multivariate regression analysis showed that PDA size and procedure time, age and procedure time, distance from VSD to AV were significantly associated with the procedure success rate of PDA, ASD and VSD closure, respectively. Early complications occurred in 306 cases (5.3%), 36 cases (0.6%) experienced major complications including device embolization in 7 cases, serious aorta regurgitation in 5 cases, serious tricuspid regurgitation in 4 cases, tricuspid stenosis in 2 cases, heart block (HB) in 13 cases (2 in ASD and 11 in VSD), cardiac tamponade in 2 cases (1 ASD and 1 PS) and hemolysis in 3 cases. Procedure time and PDA size, ASD size, device size, age and PS degree were risk factors related to the occurrence of the early complications for PDA, ASD and VSD closure and PBPV respectively. The median follow-up time was 15 months (range 1-36 months). The complete closure rate during follow up was 100% for ASD, PDA and VSD and the pressure gradient in PS decreased to normal range in all PS patients. Late complications occurred in 15 cases (0.2%), of which 3 cases needed surgery intervention and permanent pacemaker was implanted in 1 patient. There was no death during procedure and at follow-up period.

CONCLUSIONSTIT of CHD offers encouraging results in China. Follow up is warranted to monitor the occurrence of serious complications, especially late complications.

Adolescent ; Adult ; Cardiac Catheterization ; Child ; Child, Preschool ; China ; epidemiology ; Ductus Arteriosus, Patent ; surgery ; Female ; Heart Defects, Congenital ; epidemiology ; surgery ; Heart Septal Defects, Atrial ; surgery ; Heart Septal Defects, Ventricular ; surgery ; Humans ; Infant ; Male ; Middle Aged ; Pulmonary Valve Stenosis ; surgery ; Registries ; Retrospective Studies ; Young Adult

OBJECTIVETo analyze risk factors of pulmonary arterial hypertension (PAH) in patients with atrial septal defect (ASD) patients living at above 2000 m high altitude area.

METHODSWe retrospectively analyzed the incidence of PAH in 526 ASD patients out of 1178 congenital heart disease patients who were hospitalized in Qinghai cardiovascular hospital between January 2007 to December 2009 and explored the risk factors including gander, age, altitude, defect size and nationalities for developing PAH in ASD patients using binary logistic regression.

RESULTSPAH prevalence was 68.4% (360/526) in ASD patients. The risk factors of developing PAH in these ASD patients were defect size (OR: 1.200, 95%CI: 1.156 - 1.246, P = 0.000), age (OR: 1.027, 95%CI: 1.003 - 1.052, P = 0.025) and altitude (OR: 1.389, 95%CI: 1.001 - 1.637, P = 0.043) while gender and nationality were not risk factors for PAH. The incidence of developing PAH increased with aging (P = 0.000). The standardized ratio of PAH at ≥ 3500 m was 74.8% which was significantly higher than that at 2000 - 2499 m altitude (66.2%, P = 0.005) and at 2500 - 3499 m altitude (66.9%, P = 0.005).

CONCLUSIONSThe risk for developing PAH is high in patients living at high altitude area. The risk factors of developing PAH in ASD patients living at high altitude are defect size, age and altitude.

Adolescent ; Adult ; Aged ; Altitude ; Child ; Child, Preschool ; Female ; Heart Septal Defects, Atrial ; complications ; epidemiology ; Humans ; Hypertension, Pulmonary ; complications ; epidemiology ; Incidence ; Infant ; Male ; Middle Aged ; Retrospective Studies ; Risk Factors ; Young Adult

PURPOSE: We evaluated the hemodynamic statuses of patients after partial closure of atrial septal defects with fenestration due to pulmonary hypertension. MATERIALS AND METHODS: Seventeen adult patients underwent partial atrial septal defect closure and follow-up cardiac catheterization. We analyzed hemodynamic data and clinical parameters before and after closure. RESULTS: The median age at closure was 29 years old. The baseline Qp/Qs was 1.9+/-0.6. The median interval from the operation to the cardiac catheterization was 27 months. The CT ratio decreased from 0.55+/-0.07 to 0.48+/-0.06 (p<0.05). The mean pulmonary arterial pressure decreased from 50.0+/-11.5 mm Hg to 32.5+/-14.4 mm Hg (p<0.05), and the pulmonary resistance index decreased from 9.2+/-3.6 Wood units*m2 to 6.3+/-3.8 Wood units*m2 (p<0.05). Eleven patients (64.7%) continued to exhibit high pulmonary resistance (over 3.0 Wood units*m2) after closure. These patients had significantly higher pulmonary resistance indices and mean pulmonary arterial pressures based on oxygen testing before the partial closures (p<0.05). However, no significant predictors of post-closure pulmonary hypertension were identified. CONCLUSION: Despite improvement in symptoms and hemodynamics after partial closure of an atrial septal defect, pulmonary hypertension should be monitored carefully.
Adult ; Aged ; Cardiac Catheterization/*adverse effects ; Female ; Follow-Up Studies ; Heart Defects, Congenital/epidemiology/*physiopathology/*surgery ; Heart Septal Defects, Atrial/surgery ; Hemodynamics/*physiology ; Humans ; Hypertension, Pulmonary/diagnosis/epidemiology/*physiopathology ; Male ; Middle Aged ; Oxygen ; Postoperative Complications/diagnosis/epidemiology/*physiopathology ; Retrospective Studies ; Treatment Outcome ; Young Adult