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

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

OBJECTIVEBalloon angioplasty is an alternative to surgical repair for coarctation of the aorta in children. However, its role in the treatment of neonates and infants younger than 3 months old remains controversial. The purpose of this study was to evaluate the efficacy and safety of balloon angioplasty for native coarctation by comparing children in different age groups.

METHODThis is a retrospective clinical study including 37 children treated with balloon angioplasty for native coarctation from January 2006 to December 2012. A total of 37 patients consisting of 26 boys and 11 girls underwent the procedure, with median age 10 months (range from 7 days to 6 years) and the mean body weight was 6.3 (2.5-17.0) kg. The indication of the procedure includes discrete native coarctation without aortic arch hypoplasia and a peak-to-peak systolic pressure gradient > 20 mmHg (1 mmHg = 0.133 kPa) across aortic coarctation. During one year follow-up, the approach artery injury, recoarctation and aneurysm formation were particularly assessed.

RESULTWe classified these patients into two groups according to their age. Group A consisted of 25 patients younger than 3 months and Group B of 12 patients older than 3 months. There was no significant difference between the two groups in systolic pressure gradient before balloon angioplasty (P > 0.05). The mean peak systolic gradient decreased from (38 ± 18) mmHg to (12 ± 11) mmHg immediately after angioplasty in group A and from (47 ± 18) to (17 ± 12) mmHg in group B (P = 0.000 for both). Meanwhile, the mean diameter of the coarctation segment increased from (1.8 ± 0.7) to (3.7 ± 1.1) mm after angioplasty in group A and from (2.6 ± 1.5) to (5.5 ± 1.8) mm in group B (both P = 0). The initial successful balloon angioplasty (immediate postangioplasty peak pressure gradient < 20 mmHg) was achieved in all the 37 patients; 32 patients (86.5%) have been followed up for one year. Approach arterial complications occurred in 3 patients (9.4%), all of whom were in Group A (P = 0.537). Two patients had decreased femoral artery pulse and one required surgical repair for a postoperative pseudoaneurysm at left carotid artery. At follow-up, 8 patients (25.0%) developed recoarctation, with 6 cases in Group A and 2 in Group B. There was no significant difference between groups A and B in the recoarctation rate (P = 1.000). Among them, 7 patients underwent repeat balloon angioplasty, and all showed successful relief of coarctation, and one patient required surgical repair. Two patients (2/37, 5.4%) had small aneurysms of the descending aorta immediately after balloon angioplasty, with one patient in each group (12/25 vs.1/12, P = 0.755).Late aneurysm development has not been observed in the 17 patients who have had a follow-up CTA or MRA study.

CONCLUSIONBalloon angioplasty of discrete native coarctation is effective and safe in children both younger and older than 3 months with similar incidence of approach arterial complication, recoarctation and aneurysm formation.

Angioplasty, Balloon ; Aortic Aneurysm ; epidemiology ; Aortic Coarctation ; therapy ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Heart Septal Defects ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Postoperative Complications ; epidemiology ; Recurrence ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome

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

Adolescent ; Adult ; Cardiac Catheterization ; economics ; methods ; Cardiac Surgical Procedures ; economics ; methods ; Cardiac Valve Annuloplasty ; Child ; China ; epidemiology ; Echocardiography ; Female ; Heart Septal Defects, Ventricular ; complications ; diagnostic imaging ; surgery ; Humans ; Length of Stay ; statistics & numerical data ; Male ; Operative Time ; Postoperative Complications ; epidemiology ; Septal Occluder Device ; Tricuspid Valve Insufficiency ; complications ; diagnostic imaging ; surgery ; Young Adult