OBJECTIVETo study the concurrence of congenital heart disease and hypospadias and the relationship between the two diseases.

METHODSWe investigated the incidence and types of congenital heart disease accompanied by hypospadias in male children received in our hospital from January 2002 to December 2012, compared them with those in the general population, and analyzed the correlation of different types of heart disease with the incidence rate of hypospadias.

RESULTSOf the 7 385 male children with congenital heart disease, 134 (1.81%) were found with hypospadias, with a significantly higher morbidity than in the general population (0.33% -0.40%) (P < 0.01). The incidence rates of hypospadias were significantly higher in the groups of ventricular septal defect (65/3 275, 1.98%), Fallot's tetralogy (17/770, 2.21%), macroangiopathy (15/788, 1.90%) and other congenital heart abnormalities (21/972, 2.16%) than in the atrial septal defect (10/1 015, 0.99%) and patent ductus arteriosus (6/565, 1.06%) groups (P < 0.05). There were no statistically significant differences in the type of hypospadias among different heart disease groups (P > 0.05).

CONCLUSIONHypospadias is a common concurrent condition in male children with congenital heart disease. The incidence rate of hypospadias is related with the type of congenital heart disease, and the two conditions may have some common pathogenic or susceptive factors.

Child ; Child, Preschool ; Heart Defects, Congenital ; complications ; epidemiology ; Heart Diseases ; complications ; congenital ; epidemiology ; Humans ; Hypospadias ; complications ; epidemiology ; Incidence ; Infant ; Male

Abnormalities, Multiple ; epidemiology ; Adolescent ; Child ; Child, Preschool ; Heart Defects, Congenital ; complications ; Humans ; Infant ; Infant, Newborn

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

OBJECTIVETo analyze the relationship between cleft severity and incidence of associated heart defect in children with isolated cleft palate (CP), as well as the characteristics of the heart defect.

METHODSFrom Aug 2008 and Dec 2009, a total 416 children with CP underwent echocardiogram, and were divided into complete and incomplete CP groups. Then each group was further classified as unilateral or bilateral groups. Incomplete CP was subdivided into submucous cleft palate, soft palate cleft, hard and soft palate cleft. The associated heart defects were recorded and analyzed in each group. The data were analyzed statistically using SPSS 13.0. Chi-square test was used to compare the incidence between groups.

RESULTSIn the series of 416 patients, 46 (11.1%) children were found to have an associated congenital heart disease. The incidence of heart defect was 9.9% (38/384) in the incomplete cleft group, and 25% (8/32) in the complete cleft group, showing a significant difference between the two groups ( F = 6.852, P < 0.05). Atrial septal defect was the most common heart defect, which accounted for 52.2% (24/46) of all associated heart malformations.

CONCLUSIONSCompared to incomplete cleft palate, complete cleft palate has a higher risk of heart defect. Cleft severity may be a predictor for congenital heart diseases in cleft palate. Routine echocardiogram should be considered in CP patients.

Chi-Square Distribution ; Child ; Child, Preschool ; Cleft Palate ; complications ; Female ; Heart Defects, Congenital ; complications ; epidemiology ; Humans ; Incidence ; Infant ; Infant, Newborn ; Male

PURPOSE: Aortic dissection (AoD) is one of the most common catastrophes involving the aorta. Nevertheless, early diagnosis remains to be a challenge in the Emergency Department (ED), particularly in young individuals. In this study, we attempted to identify the characteristics of acute AoD among young individuals, particular in patients with Marfan syndrome. MATERIALS AND METHODS: This was an retrospective chart-review study conducted in a tertiary referring hospital. The hospital database was queried for the combination of AoD and patients under age of 40 years. The medical charts were reviewed to obtain demographic data, clinical data and laboratory characteristics by using a standardized data collection sheet. A comparison between Marfan syndrome and non-Marfan syndrome patients was performed. RESULTS: During the 10-years period, 18 of 344 patients with acute AoD were younger than 40 years-old. Patients with Marfan syndrome developed acute AoD at a younger age than patients without Marfan syndrome. The mean diastolic blood pressure was significantly lower in patients with Marfan syndrome upon presenting to the ED than those without. Patients with Marfan syndrome had trends toward higher risk of development of type A AoD, increased recurrence rate and higher mortality rate than those without. However, statistical significance was not present. CONCLUSION: ED physicians should have high alert to acute AoD in young patients presenting with severe unexplained chest and back pain, particularly in those patients with a history of heart diseases, hypertension, and Marfan syndrome or featuring Marfanoid habitus. Acute coronary syndrome, unexplained abdominal symptoms, and sudden cardiac arrest could be the initial manifestation of AoD in young patients. A low threshold to perform enhanced computed tomography may facilitate early diagnosis and timely treatment in this patient population.
Adult ; Aneurysm, Dissecting/*complications/epidemiology/*pathology ; Aortic Aneurysm/complications/epidemiology/*pathology ; Asian Continental Ancestry Group ; Female ; Heart Defects, Congenital/complications/physiopathology ; Humans ; Hypertension/complications/physiopathology ; Male ; Marfan Syndrome/*complications/*physiopathology ; Middle Aged ; Retrospective Studies ; Young Adult

Laryngeal edema developed in 10.1% of studied patients with congenital heart disease after cardiac surgery. The 181 patients were divided into two groups; those with laryngeal edema (group 1) and those without laryngeal edema (group 2). The mean ages in group 1 and 2 were 10 and 22.9 months. Group 1 patients were younger on average than those of group 2 (p< 0.05). The differences in the cardiopulmonary bypass time and anesthesia time between the two groups were not statistically significant. The duration of intubations and ventilatory support before and after the onset of laryngeal edema and the period of the ICU stay were longer in group 1 than in group 2 (p< 0.05). A predictor of postextubation laryngeal edema was not found in our patients from above mentioned parameters. We conclude that the higher incidence of laryngeal edema may be due to young age (most were under 1 year of age), and duration of intubation and ventilatory support.
Adolescent ; Child ; Child, Preschool ; Heart Defects, Congenital/*surgery ; Human ; Incidence ; Infant ; Infant, Newborn ; Intubation, Intratracheal/*adverse effects ; Laryngeal Edema/epidemiology/*etiology ; Postoperative Care ; Postoperative Complications ; Risk Factors

Objective: To analyze the classification, diagnosis and treatment status of patients with pulmonary hypertension (PH) in Yunnan province. Methods: This was a retrospective study. Hospitalized patients with PH at Yan'an Affiliated Hospital of Kunming Medical University from January 2012 to December 2019 were enrolled. The clinical data of enrolled patients, including demographic data, comorbidities, targeted drug therapy, echocardiography and right heart catheterization results, were obtained through the electronic medical record system. The composition ratio of PH, diagnosis and treatment were analyzed. Results: A total of 13 590 patients with PH were enrolled, accounting for 3.09% (13 590/440 056) of the total number of hospitalizations during the same period. The composition of PH was predominantly pulmonary arterial hypertension (PAH) (55.50% (7 542/13 590)), followed by pulmonary hypertension (PH) caused by left heart disease (24.16% (3 284/13 590)). Among them, PAH could be subdivided into four types: idiopathic pulmonary arterial hypertension (IPAH), PAH associated with connective tissue disease, PAH associated with portal hypertension, and PAH associated with congenital heart disease (CHD-PAH), with CHD-PAH as the predominating type (98.09% (7 398/7 542). Patients with PAH were predominantly adolescents. In hospitalized patients with PH, from 2012 to 2019, the proportion of children and adolescents showed a decreasing trend from year to year, and the proportion of middle-aged and older adults showed a significant increasing trend, and the proportion of female patients showed a gradual decreasing trend, and the proportion of patients with comorbid hypertension, diabetes mellitus, coronary artery disease, arrhythmia, and pneumonia showed an increasing trend. A total of 1 034 patients (7.61% (1 034/13 590)) underwent right heart catheterization. The concordance rate between echocardiographic and right heart catheterization findings was (86.98% (875/1 006)). A total of 2 574 (18.94%) of PH patients were treated with PAH targeted drugs, of which 58.16% (1 497/2 574) were treated with monotherapy. Among the PH patients treated with PAH targeted drugs, the majority of patients were PAH patients (86.44% (2 225/2 574)), and 83.53% (2 150/2 574) patients treated with PAH targeted drugs were CHD-PAH. Conclusions: Hospitalized PH patients in our center between 2012 and 2019 are predominantly CHD-PAH, and the proportion of patients receiving right heart catheterization and targeted drug therapy is relatively low. The percentage of middle-aged and elderly PH patients shows an increasing trend from year to year, as well as the percentage of those with concomitant comorbidities.
Child ; Aged ; Adolescent ; Middle Aged ; Humans ; Female ; Hypertension, Pulmonary/therapy* ; Retrospective Studies ; China/epidemiology* ; Familial Primary Pulmonary Hypertension ; Pulmonary Arterial Hypertension/complications* ; Heart Defects, Congenital

Objective: To analyze the classification, diagnosis and treatment status of patients with pulmonary hypertension (PH) in Yunnan province. Methods: This was a retrospective study. Hospitalized patients with PH at Yan'an Affiliated Hospital of Kunming Medical University from January 2012 to December 2019 were enrolled. The clinical data of enrolled patients, including demographic data, comorbidities, targeted drug therapy, echocardiography and right heart catheterization results, were obtained through the electronic medical record system. The composition ratio of PH, diagnosis and treatment were analyzed. Results: A total of 13 590 patients with PH were enrolled, accounting for 3.09% (13 590/440 056) of the total number of hospitalizations during the same period. The composition of PH was predominantly pulmonary arterial hypertension (PAH) (55.50% (7 542/13 590)), followed by pulmonary hypertension (PH) caused by left heart disease (24.16% (3 284/13 590)). Among them, PAH could be subdivided into four types: idiopathic pulmonary arterial hypertension (IPAH), PAH associated with connective tissue disease, PAH associated with portal hypertension, and PAH associated with congenital heart disease (CHD-PAH), with CHD-PAH as the predominating type (98.09% (7 398/7 542). Patients with PAH were predominantly adolescents. In hospitalized patients with PH, from 2012 to 2019, the proportion of children and adolescents showed a decreasing trend from year to year, and the proportion of middle-aged and older adults showed a significant increasing trend, and the proportion of female patients showed a gradual decreasing trend, and the proportion of patients with comorbid hypertension, diabetes mellitus, coronary artery disease, arrhythmia, and pneumonia showed an increasing trend. A total of 1 034 patients (7.61% (1 034/13 590)) underwent right heart catheterization. The concordance rate between echocardiographic and right heart catheterization findings was (86.98% (875/1 006)). A total of 2 574 (18.94%) of PH patients were treated with PAH targeted drugs, of which 58.16% (1 497/2 574) were treated with monotherapy. Among the PH patients treated with PAH targeted drugs, the majority of patients were PAH patients (86.44% (2 225/2 574)), and 83.53% (2 150/2 574) patients treated with PAH targeted drugs were CHD-PAH. Conclusions: Hospitalized PH patients in our center between 2012 and 2019 are predominantly CHD-PAH, and the proportion of patients receiving right heart catheterization and targeted drug therapy is relatively low. The percentage of middle-aged and elderly PH patients shows an increasing trend from year to year, as well as the percentage of those with concomitant comorbidities.
Child ; Aged ; Adolescent ; Middle Aged ; Humans ; Female ; Hypertension, Pulmonary/therapy* ; Retrospective Studies ; China/epidemiology* ; Familial Primary Pulmonary Hypertension ; Pulmonary Arterial Hypertension/complications* ; Heart Defects, Congenital

Radiofrequency catheter ablation (RFCA) has recently become a management option for pediatric tachycardia. We reviewed the records of a total of 100 patients (aged 10 months to 19 yr) who had undergone RFCA, from March 2000 to June 2004. Types of arrhythmia (age, acute success rate) were as follows: atrioventricular reentrant tachycardia (AVRT, 9.0+/-3.7 yr, 66/67), atrioventricular nodal reentrant tachycardia (AVNRT, 13+/-2.5 yr, 16/16), ectopic atrial tachycardia (6.4+/-3.3 yr, 5/5), junctional ectopic tachycardia (10 month, 1/1), ventricular tachycardia (12+/-4.9 yr, 6/6), postsurgical intraatrial reentrant tachycardia (15.6+/-4.1 yr, 2/3), twin node tachycardia (4 yr, 0/1), and His bundle ablation (9 yr, 1/1). The age of AVNRT was older than that of AVRT (p=0.002). Associated cardiac disease was detected in 17 patients, including 6 univentricular patients, and 3 Ebstein's anomaly patients. RFCA for multiple accessory pathways required longer fluoroscopic times than did the single accessory pathway (53.9+/-4.8 vs. 36.2+/-24.1 min; p=0.03), and was associated with a higher recurrence rate (3/9 vs. 3/53; p=0.03). Regardless of the presence or absence of cardiac diseases, the overall acute success rate was 97% without major complications, the recurrence rate was 8.2%, and the final success rate was 97%. This experience confirmed the efficacy and safety of RFCA in the management of tachycardia in children.
Catheter Ablation/*statistics and numerical data ; Child ; Comorbidity ; Female ; Heart Defects, Congenital/*epidemiology/*surgery ; Humans ; Korea/epidemiology ; Male ; Pilot Projects ; Postoperative Complications/epidemiology ; Prevalence ; Retrospective Studies ; Risk Assessment/*methods ; Risk Factors ; Tachycardia/*epidemiology/*surgery ; 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