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

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

Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 +/- 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.
Adult ; Aged ; Connective Tissue Diseases/complications ; Data Collection ; *Databases, Factual ; Familial Primary Pulmonary Hypertension/*epidemiology/mortality/therapy ; Female ; Heart Defects, Congenital/complications ; Humans ; Internet ; Male ; Middle Aged ; Prognosis ; Prospective Studies ; Pulmonary Artery/*physiopathology ; *Registries ; Republic of Korea/epidemiology ; Survival Rate ; Young Adult

OBJECTIVEBranch pulmonary artery stenosis is one of the common congenital heart disease. Stent implantation to relieve branch pulmonary artery stenosis (BPAS) is an alternative to failed surgical or balloon angioplasty. The aim of this study was to explore the indication, methods and complications of using balloon expandable stent placement to treat branch pulmonary artery stenosis, and evaluate the results of stent implantation in the treatment of branch pulmonary artery stenosis.

METHODFrom August 2005 to December 2012, 19 patients underwent an attempt at stent implantation. The median age of those patients was 9.1 years (range 4.0-15.0 years). The median weight was 31.7 kg (range 17.0-60.5 kg); 14/19 patients underwent post surgical repair of tetralogy of Fallot, one patient received post surgical repair of pulmonary atresia with ventricular septal defect, one patient underwent post surgical repair of pulmonary atresia with intact septum, one with native left BPAS, and one was after surgical repair of aortopulmonary window and the other truncus arteriosus. CP stent and NuMED Balloon-in-Balloon catheter were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the inner balloon and outer balloon was inflated successively to expand the stent to desired diameter. Statistical analysis was performed with the unpaired Student t test.

RESULTA total of 26 stents were implanted successfully in 19 patients. The systolic gradient across the stenosis fell from a median of (36.0 ± 18.3) to (3.8 ± 3.4) mmHg (P < 0.01, 1 mmHg = 0.133 kPa) and the diameter of the narrowest segment improved from (6.0 ± 1.9) to (11.6 ± 3.1) mm (P < 0.01). The right ventricle to aortic pressure ratio fell from 0.68 to 0.49 (P < 0.01). Complications included the following: two stents were malpositioned in the right ventricular outflow tract and one balloon ruptured when dilated the hole of the stent. No other complications occurred. All patients were followed up for 6 months to 6.0 (2.5 ± 1.8) years. One patient underwent stent re-dilation in order to accommodate somatic growth two years later.

CONCLUSIONBalloon expandable stents are safe and effective in relieving BPAS. Stent implantation should be considered the treatment of choice for most patients with BPAS. Stents placed into growing children will require further dilation to keep up with normal somatic growth. Intermediate and long-term follow up studies have shown excellent results after further dilation over time.

Adolescent ; Angioplasty, Balloon ; methods ; Blood Vessel Prosthesis Implantation ; Child ; Child, Preschool ; Constriction, Pathologic ; surgery ; Female ; Heart Defects, Congenital ; complications ; surgery ; Humans ; Male ; Postoperative Complications ; epidemiology ; surgery ; Pulmonary Artery ; pathology ; surgery ; Pulmonary Valve Stenosis ; etiology ; surgery ; Retrospective Studies ; Stents ; Treatment Outcome ; Vascular Surgical Procedures ; methods

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

Right-sided infective endocarditis (RIE) occurs predominantly in intravenous drug users in western countries, and it has a relatively good prognosis. Clinical features and prognosis of RIE occurring in non-drug users are not well known. We investigated the clinical findings of RIE in non-drug users. We retrospectively reviewed 345 cases diagnosed with IE. Cases with RIE or left-sided infective endocarditis (LIE) defined by the vegetation site were included and cases having no vegetation or both-side vegetation were excluded. Clinical findings and in-hospital outcome of RIE were compared to those of LIE. Among the 245 cases, 39 (16%) cases had RIE and 206 (84%) cases had LIE. RIE patients were younger (40+/-19 yr vs 50+/-18 yr, P=0.004), and had a higher incidence of congenital heart disease (CHD) (36% vs 13%, P<0.001) and central venous catheter (CVC) (21% vs 4%, P=0.001) compared to LIE patients. A large vegetation was more common in RIE (33% vs 9%, P<0.001). Staphylococcus aureus was the most common cause of RIE, while Streptococcus viridans were the most common cause of LIE. In-hospital mortality and cardiac surgery were not different between the two groups. CHD and use of CVC were common in non-drug users with RIE. The short-term clinical outcome of RIE is not different from that of LIE.
Adult ; Aged ; Central Venous Catheters/microbiology ; Echocardiography ; Endocarditis, Bacterial/*diagnosis/microbiology/mortality ; Female ; Heart Defects, Congenital/complications/epidemiology ; Hospital Mortality ; Humans ; Incidence ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Staphylococcus aureus/isolation & purification ; Viridans Streptococci/isolation & purification ; Young Adult

Animals ; Aortic Valve ; surgery ; Aortic Valve Stenosis ; congenital ; therapy ; Balloon Valvuloplasty ; methods ; Cardiac Surgical Procedures ; adverse effects ; methods ; Catheterization ; adverse effects ; methods ; Female ; Fetal Diseases ; therapy ; Fetal Heart ; surgery ; Heart Defects, Congenital ; therapy ; Humans ; Postoperative Complications ; epidemiology ; Pregnancy ; Pregnancy Trimester, Second ; Ultrasonography, Interventional ; methods

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