Dextrocardia ; epidemiology ; Ductus Arteriosus, Patent ; epidemiology ; Female ; Heart Defects, Congenital ; epidemiology ; Heart Septal Defects, Ventricular ; epidemiology ; Humans ; Infant ; Male ; New Guinea

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

Objective: To explore the current situation of fetal heart defects in Yunnan Province and surrounding high altitude areas and the social factors affecting pregnancy outcome. Methods: This is a retrospective study. Pregnant woman who underwent fetal echocardiography and diagnosed as fetal cardiac defects in Yunnan Fuwai Cardiovascular Hospital from June 2017 to January 2021 were included. According to the clinical prognostic risk scoring system and grading criteria of fetal cardiac birth defects, the cases were divided into grade Ⅰ to Ⅳ. The disease distribution and proportion of each prognostic grade, pregnancy outcomes were analyzed and compared. The cases were divided into continued pregnancy group and terminated pregnancy group according to pregnancy outcome. The social factors that may affect the selection of pregnancy outcomes were analyzed by multivariate logistic regression analysis. Results: A total of 4 929 fetal echocardiography examination data were collected, and 4 464 cases (90.57%) were from Yunnan Province and surrounding high altitude areas. 2 166 cases of heart defects were finally analyzed, including 998 cases of congenital heart disease (CHD), 93 cases of cardiac tumors, cardiomyopathy and arrhythmia, 1 075 cases of foramen ovale, ductus arteriosus abnormalities and normal variations. The pregnant women were (29.2±5.0) years old with (25.6±3.8) gestational weeks. The number of cases with prognostic grade from Ⅰ to Ⅳ was 1 037 (47.88%), 620 (28.62%), 314 (14.50%), and 44 (2.03%), respectively. And 151 cases (6.97%) were not classified. The cases of normal variation and thin aortic arch development accounted for 42.66% (924/2 166), 5.22% (113/2 166), respectively. The top 3 diseases of grade Ⅱ were ventricular septal defect, coarctation of aorta and mild-moderate pulmonary stenosis, respectively, and their distribution was 11.63% (252/2 166), 3.92% (85/2 166) and 2.35% (51/2 166) respectively in all cases of heart defects, and 25.25% (252/998), 8.52% (85/998) and 5.11% (51/998) respectively in cases of CHD. Among the cases rated as grade Ⅲ and Ⅳ, most of them were complicated congenital heart disease, and the disease types are scattered. The more common cases in grade Ⅲ were complete transposition of great arteries (accounting for 2.40% (52/2 166) of all cases with heart defects, 5.21% (52/998) of all cases with CHD) and pulmonary artery occlusion (type Ⅰ to Ⅲ) with ventricular septal defect (accounting for 2.17% (47/2 166) of all cases with heart defects, and 4.71% (47/998) of all cases with CHD). In grade Ⅳ, single ventricle (0.74% (16/2 166) of all cases with heart defects, 1.60% (16/998) of all cases with CHD) and left ventricular dysplasia syndrome (0.65% (14/2 166) of all cases with heart defects, 1.40% (14/998) of all cases with CHD) are more common. A total of 1 084 cases were successfully followed up, and 675 cases were born, 392 cases were terminated, spontaneous abortion occurred in 17 cases. The proportion of terminated pregnancy cases was significantly increased from grade Ⅰ to Ⅳ, accounting for 5.24% (21/401), 27.78% (70/252), 89.54% (214/239) and 95.56% (43/45), respectively. Among the terminated pregnancy cases, those with grade Ⅲ accounted for the highest proportion (54.59% (214/392)). The distribution of terminated pregnancy cases was mainly complex congenital malformations or diseases with very poor prognosis (pregnancy outcome grade Ⅲ and Ⅳ), and proportion of terminated pregnancy with pregnancy outcome grade Ⅰ and Ⅱ cases (normal variation or good prognosis) accounted for 5.36% (21/392) and 17.86% (70/392), respectively. The results of multivariate logistic regression analysis showed that pregnant women with low education (high school and below: OR=2.73, 95%CI 1.26-5.93, P<0.001; illiteracy: OR=3.27, 95%CI 1.29-7.10, P<0.001) and low family income (Annual income<100 000 yuan: OR=2.47, 95%CI 1.69-5.12, P<0.001) were more likely to choose termination of pregnancy in case of fetal heart defect. Conclusion: In Yunnan province and the surrounding high altitude areas, the disease distribution of fetal heart defect is mainly simple or low-risk disease, but the complex malformation, especially the disease with poor pregnancy outcome, accounts for a relative high proportion. Pregnancy termination also occurs in some cases with good pregnancy outcome. The education level and family income of pregnant women may affect their choice of pregnancy outcome in case of fetal heart defect.
Pregnancy ; Female ; Humans ; Young Adult ; Adult ; Retrospective Studies ; Altitude ; China/epidemiology* ; Heart Defects, Congenital/diagnostic imaging* ; Heart Septal Defects, Ventricular ; Echocardiography ; Fetal Heart/diagnostic imaging*

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

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

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

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

In order to review the epidemiological data of cardiovascular diseases, statistical observation was made on the 7,271 patients with cardiovascular diseases who were treated in the Department of Internal Medicine, Han-Yang University Hospital during the 10 year period from May 1972 to December 1981. A. General Findings (1) Cardiovascular disease was seen in 18.6% out of a total 39,004 medical inpatients treated during this 10 year time span. (2) Incidences of cardiovascular disease seemed to increase in frequency in consecutive years, especially incidence of coronary heart disease and arrhythmia. (3) Incidence of cardiovascular disease occurred most frequently to patients in their fifth decade, and next most frequently to those in their sixth and fourth decade, in that order. (4) The overall ratio of male to female incidence of cardiovascular disease was 1:1. B. Specific Epidemiology (1) Hypertension was the most common form of cardiovascular problem, accounting for 52.9% of patients with cardiovascular disease and 9.9% of the total medical in-patients population. Incidence of hypertension occured most frequently in the fifth decade of life. It occured next most in the sixth and fourth decades, in that order. The male to female ratio of incidence of hypertension was 1:1. Patients with malignant hypertension made up 5.9% all hypertension patients. (2) Patients with rheumatic heart disease made up 6% of those patients with cardiovascular diseases. Male to female ratio of incidence of rheumatic heart disease was 1:2.4. The most common form of this disease was mitral valvular disease, but isolated aortic valvular disease proved to be rather uncommon in this study in Korea. (3) Coronary heart disease made up 15.5 % of all incidence of cardiovascular diseases. It occured most frequently to patients in their fifth and sixth decades, and in a male to female ratio of 1.5 to 1. (4) Congenital heart disease made of 0.9% of the total incidence of cardiovascular diseases with ventricular septal defect as the most common form of this disease. (5) The remaining forms of cardiovascular diseases diagnosed during the time span of this study were arrhythmia 16.8%, cor pulmonale 1.4%, cardiomyopathy 1.3%, peripheral vascular disease 1.0%, pericarditis 1.0%, thyrotoxic heart disease 0.8%, endocarditis 0.5%, postpartum cardiomyopathy 0.3%, myxedematous heart disease 0.1%, and miscellaneous heart ailments 1.5%.
Arrhythmias, Cardiac ; Cardiomyopathies ; Cardiovascular Diseases* ; Coronary Disease ; Endocarditis ; Epidemiology ; Female ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Heart Septal Defects, Ventricular ; Humans ; Hypertension ; Hypertension, Malignant ; Incidence ; Inpatients ; Internal Medicine ; Korea* ; Male ; Pericarditis ; Peripheral Vascular Diseases ; Postpartum Period ; Pulmonary Heart Disease ; Rheumatic Heart Disease

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

OBJECTIVETo evaluate the prevalence and risk factors of arrhythmia after transcatheter closure of ventricular septal defect (VSD) in children.

METHODSA total 1 069 children (583 males, mean age (7.7 ± 3.6) years) underwent transcatheter closure of VSD from January 2002 to December 2010 in our hospital were enrolled and retrospectively analyzed.VSD diameters were (4.0 ± 1.8)mm, 336 cases accompanied membranous aneurysm. Electrocardiogram were performed at 1, 3 days after the procedure.Once arrhythmias recorded, electrocardiogram was performed daily till discharge. All cases were followed up by ECG at 1, 3, 6, 12 months after the procedure in outpatient department and then in a year interval. The risk factors were identified by multivariable logistical analysis.

RESULTSAll VSDs were closed successfully and the diameters of occluder was (7.2 ± 2.1)mm. The median follow-up time was 2.2 (1.0-4.2) years. Mortality was zero during follow up.Incidence of early ( < 1 month) post-procedure arrhythmias was 24.6 % (263 cases), and severe arrhythmias were recorded in 50 cases (4.7%). There were 43 late ( ≥ 1 month) post-procedure arrhythmias (4.0%) including 4 (0.4%) complete atrioventricular block. Multivariable logistic analysis revealed that VSD treated with thin-waist-big-side occluder (OR = 2.426, 95%CI:1.835-3.208, P < 0.001) , male gender (OR = 1.267, 95%CI:1.055-1.523, P = 0.011) were the risk factors while higher body weight (OR = 0.838, 95%CI:0.737-0.951, P = 0.006) was protective factor for early onset arrhythmia. Placement of asymmetrical occluder (OR = 4.777, 95%CI:2.079-10.978, P < 0.001) , longer procedure time (OR = 1.011, 95%CI:1.002-1.020, P = 0.012) , occluder from foreign countries (OR = 2.621, 95%CI:1.143-6.014, P = 0.021) were the risks factors for early onset severe conduct block. Treatment with thin-waist-big-side occluder (OR = 2.654, 95%CI: 1.042-6.760, P = 0.041) was the risk factor while higher body weight (OR = 0.373, 95%CI:0.159-0.875, P = 0.023) was a protective factor for late onset conduct block.

CONCLUSIONSArrhythmia after transcatheter closure of VSD is common in children, and late onset severe conduct block is rare. The weight of patients should not too light and symmetrical occluder should be chosen if possible in the transcatheter closure VSD procedure to minimize the risk of late onset conduct block.

Arrhythmias, Cardiac ; epidemiology ; Atrioventricular Block ; Body Weight ; Cardiac Catheterization ; Child ; Child, Preschool ; Electrocardiography ; Female ; Heart Septal Defects, Ventricular ; epidemiology ; Hospitals ; Humans ; Male ; Prevalence ; Protective Factors ; Retrospective Studies ; Risk Factors