Pregnancy outcomes in patients with congenital heart disease have not been fully assessed in Korea. Forty-nine pregnancies that occurred in 34 women with congenital heart disease who registered at our hospital between September 1995 and April 2006 were reviewed. Spontaneous abortions occurred in two pregnancies at 6+1 and 7 weeks, and another two underwent elective pregnancy termination. One maternal death in puerperium occurred in a woman with Eisenmenger syndrome. Maternal cardiac complications were noted in 18.4%, pulmonary edema in 16.3%, symptomatic arrhythmia in 6.1%, deterioration of New York Heart Association (NYHA) functional class by > or =2 in 2.0%, and cardiac death in 2.0%. Independent predictors of adverse maternal cardiac events were an NYHA functional class of > or =3 (odds ratio [OR], 20.3), right ventricular dilation (OR, 21.2), and pulmonary hypertension (OR, 21.8). Neonatal complications occurred in 22.4% of pregnancies and included preterm delivery (16.3%), small for gestational age (12.2%), and neonatal death (2.0%). Independent predictors of adverse neonatal events were pulmonary hypertension (OR, 6.8) and NYHA functional class > or =3 (OR, 23.0). Pregnancy in women with congenital heart disease was found to be significantly associated with maternal cardiac and neonatal complications. Pre-pregnancy counseling and multidisciplinary care involving cardiologists and obstetricians are recommended for women with congenital heart disease contemplating pregnancy.
Abortion, Spontaneous ; Adult ; Female ; Heart Defects, Congenital/complications/*physiopathology ; Humans ; Korea ; Multivariate Analysis ; Obstetrics/methods ; Odds Ratio ; Pregnancy ; Pregnancy Complications, Cardiovascular/physiopathology ; Pregnancy Outcome

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

OBJECTIVETo evaluate the value of Doppler echocardiography in the diagnosis of pulmonary artery hypertension (PAH) and the effect of PAH on left ventricular remodelling and diastolic function using Doppler echocardiography in children with congenital heart disease (CHD).

METHODSDoppler echocardiography was performed on 45 CHD children with PAH (PAH group) and the results were compared with those of 22 CHD children with normal pulmonary artery pressure (control group).

RESULTSDoppler echocardiography showed that the diameter of end-diastolic left ventricular (18.24 +/-1.71 mm vs 16.28 +/-0.52 mm), the diameter of right ventricular (12.23 +/-2.14 mm vs 8.14 +/-0.73 mm), and the pulmonary artery diameter (11.20 +/-1.35 mm vs 7.92 +/-0.21 mm ) increased significantly in the PAH group compared with those in the control group (P < 0.05). The PAH group had higher velocity of tricuspid valve regurgitation (2.56 +/-0.46 m/s) and higher pulmonary artery pressure (40.23 +/-4.56 mmHg) than the control group (P < 0.05). The PAH group had also higher mitral peak A velocity (AV, 94.56 +/-31.45 m/s vs 51.17 +/-26.67 m/s), higher mitral AV velocity-time intergrate (10.89 +/-2.73 s vs 4.94 +/-1.85 s), higher ratio of mitral AV to mitral peak E velocity (EV) (1.79 +/-0.32 vs 0.59+/-0.19 and higher ratio of mitral velocity-time intergrate of AV/EV (1.61+/-0.49 vs 0.45 +/-0.21) than the control group. The left ventricular isovolumetric relaxation time (119.86 +/-54.62 s vs 52.31 +/-28.06 s) was prolonged in the PAH group (P < 0.05). In the PAH group, there was a positive correlation between the increased pulmonary artery pressure and the ratio of mitral AV/EV (r=0.4456, P < 0.01).

CONCLUSIONSDoppler echocardiography is not only an important non-invasive diagnostic technique for PAH in children with CHD, but also a tool which can indicate the left ventricular remodelling and diastolic dysfunction induced by PAH. It is useful to evaluate the severity and the prognosis of PAH secondary to CHD.

Child ; Child, Preschool ; Echocardiography, Doppler ; methods ; Female ; Heart Defects, Congenital ; complications ; Humans ; Hypertension, Pulmonary ; diagnostic imaging ; pathology ; physiopathology ; Infant ; Infant, Newborn ; Male ; Ventricular Function, Left ; Ventricular Remodeling

OBJECTIVETo study the plasma natriuretic peptide (BNP) level in children with pulmonary artery hypertension (PAH) secondary to congenital heart disease (CHD) and its correlation with left ventricular diastolic function.

METHODSDoppler echocardiography was performed on 95 CHD children with PAH (PAH group) and on 42 CHD without PAH (control group). The plasma BNP level was measured using radioimmunity assay.

RESULTSDoppler echocardiography showed that the left ventricular end-diastolic diameter (LVDd), the right ventricular diameter end-diastolic (RVDd), and the pulmonary artery diameter (PAd) increased significantly in the PAH group compared with those in the control group (p<0.05). The PAH group had higher velocity of tricuspid regurgitation (VTR) and higher pulmonary artery systolic pressure (PASP) than the control group (p<0.05). The PAH group also had higher mitral A peak velocity (AV), higher mitral A peak velocity integral (AVI), higher E peak velocity intgral (EVI), and higher ratio of mitral AV to mitral E peak velocity (EV) and AVI/EVI ratio as well as prolonged left ventricular volumetric relaxation time than the control group. PASP was positively correlated with the AV/EV ratio (p<0.05). The plasma BNP level in the PAH group increased significantly compared with that in the control group. In the PAH group, the plasma BNP level was positively correlated with the pulmonary artery pressure and the ratio of AV/EV.

CONCLUSIONSThe left ventricular diastolic function is positively correlated with the plasma BNP level in children with PAH secondary to CHD. BNP may play an important role in the generation and development of left ventricular diastolic dysfunction induced by PAH.

Child ; Child, Preschool ; Diastole ; Female ; Heart Defects, Congenital ; complications ; Humans ; Hypertension, Pulmonary ; blood ; physiopathology ; Infant ; Infant, Newborn ; Male ; Natriuretic Peptide, Brain ; blood ; Ventricular Function, Left

OBJECTIVETo determine the relationship between the serum sulfur dioxide, homocysteine and the pulmonary arterial pressure in children with congenital heart defects who generated a pulmonary arterial hypertension syndrome (PAH-CHD), and analyze their role in the pathological process of the disease.

METHODThis was a prospective cohort study, children with systemic pulmonary shunt CHD were selected. The patients were divided into three groups: the CHD with no PAH group:n = 20, 10 males, 10 females, 5 with ventricular septal defect (VSD), 8 with atrial septal defect (ASD) and 7 with patent ductus arteriosus (PDA), mean age (1.9 ± 1.8) years; the CHD with mild PAH group:n = 20, 10 males, 10 females, 12 with VSD, 6 with ASD, and 2 with PDA, mean age (1.0 ± 0.8) year; the CHD with moderate or severe PAH group:n = 20, 8 males, 12 females, 12 with VSD, 6 with ASD, and 1 with PDA, 1 with ASD+VSD, mean age (1.8 ± 1.6) year. Twenty healthy children were enrolled from outpatient department as the control group [included 8 males, 12 females, mean age (1.9 ± 1.5) years]. The homocysteine and SO2 concentrations in the serum samples were detected by a modified high performance liquid chromatographic method with fluorescence detection (HPLC-FD), then, multiple comparisons among the groups were performed with analysis of variance, and the pearson correlation.

RESULTThe serum homocysteine concentrations were respectively (11.0 ± 2.7) , (11.7 ± 2.5), (12.0 ± 2.1), (14.3 ± 3.2) µmol/L in the control group, CHD with no PAH group, CHD with mild PAH group, and CHD with moderate or severe PAH group. According to the multiple comparisons, the CHD with moderate or severe PAH group had the highest level (P all < 0.05) .While the comparison within the control group, CHD with none PAH group, and CHD with mild PAH group, the differences were not significant (P all > 0.05). The serum sulfur dioxide strength (concentrated as SO3(2-)) were respectively (10.6 ± 2.4), (8.9 ± 2.3), (7.3 ± 2.9), (4.3 ± 2.1) µmol/L in the control group, CHD with none PAH group, CHD with mild PAH group, and CHD with moderate or severe PAH group. CHD with moderate or severe PAH group had the highest level of serum sulfur dioxide (P < 0.05) . The pearson correlation analysis indicated that in the CHD children, the serum homocysteine were positively correlated with the pulmonary arterial pressure (r = 0.481, P < 0.01), while, the sulfur dioxide were negatively correlated with pulmonary arterial pressure (r = -0.553, P < 0.01).In all children, the serum homocysteine levels were negatively correlated with the sulfur dioxide (r = -0.231, P = 0.039).

CONCLUSIONThe PAH-CHD children had higher homocysteine levels and lower sulfur dioxide levelsl, which demonstrated the disturbance of homocysteine-sulfur dioxide pathway in the sulfur containing amino acids metabolish in the disease. The homocysteine may become a biological marker which reflecting the severities of the PAH-CHD, while the sulfur dioxide can be a new target for the therapy of PAH-CHD.

Biomarkers ; blood ; Case-Control Studies ; Child, Preschool ; Ductus Arteriosus, Patent ; blood ; complications ; physiopathology ; Familial Primary Pulmonary Hypertension ; blood ; etiology ; physiopathology ; Female ; Heart Defects, Congenital ; blood ; complications ; physiopathology ; Heart Septal Defects ; blood ; complications ; physiopathology ; Hemodynamics ; Homocysteine ; blood ; Humans ; Infant ; Male ; Sulfur Dioxide ; blood

OBJECTIVETo explore the possibility and reliability of echocardiography in quantitative evaluation of pulmonary blood flow in patients with congenital heart disease (CHD).

METHODSSixty-four patients with left to right shunt congenital atrial septal defect (ASD) underwent echocardiographic examinations of the right upper and lower pulmonary vein blood flow spectrum in the four-chamber face, and the right upper pulmonary vein flow velocity time integral (VTIrupv) and right inferior pulmonary venous flow velocity time integral (VTIrlpv) were calculated according to the heart rate. The VTIrupv and VTIrlpv were compared with the pulmonary blood flow (Qp) calculated by Fick method with right heart catheterization.

RESULTSThere was a high correlation between the right lung vein flow velocity time integral measured by the catheter of transthoracic echocardiography and Qp.

CONCLUSIONThe pulmonary venous flow spectrum measured by echocardiography can be informative of the pulmonary blood flow in patients with CHD. Echocardiography may serve as a potential noninvasive technique to evaluate pulmonary blood flow in these patients.

Adolescent ; Adult ; Aged ; Echocardiography, Doppler, Color ; Female ; Heart Defects, Congenital ; complications ; diagnostic imaging ; physiopathology ; Humans ; Hypertension, Pulmonary ; etiology ; physiopathology ; Lung ; blood supply ; Male ; Middle Aged ; Regional Blood Flow ; 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

OBJECTIVETo investigate the feasibility, advantages and efficacy of implantation of left atrial and ventricular epicardial dual chamber pacemaker to treat pediatric complete atrioventricular block.

METHODEleven children with median age 4.0 years (0.5-7.6 years) diagnosed as complete atrioventricular block resisting to drug therapy received implantations of left atrial and ventricular epicardial dual chamber pacemakers. Six were male and five female. Temporal or permanent right ventricular pacing was used for all of them before implantation of left atrial and ventricular epicardial dual chamber pacemakers. Three cases showed cardiac dysfunction. Left lateral thoracotomy was performed at 4th intercoastal space along anterior axillary line under general anesthesia, the pericardium was incised vertically anterior to the phrenic nerve, two pacing leads were individually located at left atrial appendage and left ventricular lateral wall. After all the parameters were detected to be satisfactory, a pouch was made at left abdomen under coastal margin. Dual chamber pacemaker was connected with pacing leads through subcutaneous tunnels. The sizes of heart chambers, cardiac functions, parameters of pacemaker, sensitivity, pacing status, PR interval and QRS interval were closely followed up post-operatively.

RESULTImplantations of pacemakers were successful for all of the patients with no complications associated with operations. Preoperative electrocardiograms showed QRS interval (180 ± 33)ms under right ventricular pacing, it decreased to (140 ± 24)ms after implantation of left atrial and ventricular epicardial dual chamber pacemaker, significantly lower than right ventricular pacing (t = 8.8, P < 0.05) . Atrioventricular (AV) interval was set at 90 ms, PR interval (124 ± 4)ms. Echocardiograms performed within 2-3 days after implantation of left atrial and ventricular epicardial dual chamber pacemakers showed that for the 3 cases who were previously under right atrial and right ventricular dual chamber pacing presenting cardiac dysfunction, their left ventricular diastolic diameter (LVDd) decreased from (46.3 ± 12.5) (32.0-55.0) ms to (44.7 ± 12.0) (31.0-53.0) mm and left ventricular ejection fraction (LVEF) increased from 30% ± 15% (18%-47%) to 44% ± 18% (33%-65%). During 2-14 months' follow up, LVEF increased progressively which became significantly higher than before (65% ± 8% vs. 30% ± 15%, t = 5.6, P < 0.05) . Cardiac chamber sizes and left ventricular systolic function for the other 8 patients maintain normal during follow up. Pacing status and sensitivity were satisfactory for all these patients during follow up.

CONCLUSIONImplantation of left atrial and ventricular epicardial pacemaker might be considered for children diagnosed as complete atrioventricular block for whom endocardial pacemaker could not be implanted, due to its utmost protection for cardiac function with minimal injury and its ability to prevent or reverse pacemaker syndrome. Left atrial and left ventricular epicardium should be regarded as the first-choice and routine locations for epicardial pacing.

Atrioventricular Block ; etiology ; physiopathology ; therapy ; Cardiac Pacing, Artificial ; methods ; Child ; Child, Preschool ; Electrodes, Implanted ; Equipment Design ; Feasibility Studies ; Female ; Follow-Up Studies ; Heart Atria ; physiopathology ; Heart Defects, Congenital ; complications ; Heart Ventricles ; physiopathology ; Humans ; Infant ; Male ; Pacemaker, Artificial ; Stroke Volume ; Thoracotomy ; Treatment Outcome

OBJECTIVETo observe the expression of urotensin II (UII) on the lung of patients with pulmonary hypertension (PH) with congenital heart disease and investigate the meaning of this phenomenon.

METHODThirty eight patients with CHD were divided into three groups according to pulmonary arterial systolic pressure (PASP) measured in cardiac catheterization and surgery: normal pulmonary pressure group (N group, PASP < 30 mm Hg, n = 10), mild PH group (M group, PASP ≥ 30 mm Hg, n = 15), severe or moderate PH group (S group, PASP ≥ 50 mm Hg, n = 13). The expression of UII protein and UII mRNA in pulmonary arterioles were measured separately by immunohistochemical (IHC) analysis and in situ hybridization (ISH) analysis.

RESULT(1) The results of UIIIHC staining: The UII protein expression of group M was higher than that of group N (20.22 ± 3.58 vs. 14.34 ± 2.18, P < 0.01), but less than group S (20.22 ± 3.58 vs. 28.92 ± 3.22, P < 0.05). (2) The results of UIIISH mRNA staining were similar to IHC staining, the A value of group M was higher than group N (12.51 ± 2.02 vs. 8.85 ± 1.41, P < 0.05), less than that of group S(12.51 ± 2.02 vs. 25.35 ± 4.33, P < 0.01). (3) Correlation study: there was a positive correlation between the A values of UIIIHC and pulmonary hypertension (r = 0.64, P < 0.01, n = 38), a positive correlation between the A values of UIIISH and pulmonary hypertension (r = 0.58, P < 0.01, n = 38).

CONCLUSIONThere was the expression of Urotensin II protein and mRNA in the lung of pulmonary hypertension patients with congenital heart disease, and these expression may involve the formation of pulmonary hypertension of congenital heart disease.

Adolescent ; Blood Pressure ; Case-Control Studies ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; complications ; metabolism ; physiopathology ; Humans ; Hypertension, Pulmonary ; etiology ; metabolism ; physiopathology ; Immunohistochemistry ; In Situ Hybridization ; Infant ; Lung ; metabolism ; physiopathology ; Male ; Pulmonary Artery ; metabolism ; physiopathology ; RNA, Messenger ; genetics ; metabolism ; Severity of Illness Index ; Urotensins ; genetics ; metabolism

OBJECTIVETo improve the accuracy of diagnosis of heart failure (HF) has been the focus of research for a long time. The diagnosis for HF with congenital heart disease, however, is more difficult. The aim of the study was to evaluate the diagnostic criteria for HF in children and examine the value of plasma brain natriuretic peptide (BNP) and NT-proBNP for diagnosing HF in pediatric patients with congenital heart disease, and to look for the most valuable index for the diagnosis according to the multifactor analysis.

METHODSTotally 118 children with congenital heart disease were enrolled. They were diagnosed using modified Ross score, Qingdao criteria, NYU PHFI, and plasma BNP and NT-proBNP. According to modified Ross score as the referent criteria, other diagnostic criteria and plasma BNP and NT-proBNP were studied. The sensitivity, specificity and area of the ROC curve were examined. Logistic regression analysis was used to select the valuable index for diagnosing HF.

RESULTS(1) The value of each clinical criteria: 1 The sensitivity of Qingdao criteria for diagnosing HF was 47.9%. The specificity was 100% and the accuracy was 57.6%. 2 There were 52 patients younger than six months in whom 27 (51.9%) were breast fed. Only 25 children were measured with Ross score. The Ross score was positively correlated with the modified Ross score (r = 0.948). The area under the ROC curve of Ross score diagnosing HF was 0.985, and the sensitivity was 88%, while the specificity was 100%. 3 NYU PHFI score was positively correlated with the modified Ross score. The area under the ROC curve of the NYU PHFI diagnosing HF was 0.964, and the sum of sensitivity and specificity was favorite when > or = 8 was set as the cut-off point. If > 2 was set as cut-off point, it had a high sensitivity but a low specificity. The sensitivity of NYU PHFI was 100% > was set 2 as cut-point for diagnosing HF, but the specificity was 4.5%. (2) Plasma BNP and NT-proBNP were positively correlated with the modified Ross score, and increased with the severity of congestive HF. The area under the ROC curve of BNP was 0.880, and the cut-off line was > or = 349 pg/ml. The area under the ROC curve of NT-proBNP was 0.981, and the cut-off line was > or = 499 fmol/ml. (3) Logistic regression analysis showed that in multifactor analysis, only plasma concentration of NT-proBNP, dyspnea, tachycardia, tachypnea, failure to thrive were the independent predictors for diagnosing HF. (4) Plasma concentration of NT-proBNP incorporated with clinical criteria would improve its accuracy.

CONCLUSIONAll the clinical criteria commonly used were valuable for diagnosing HF in children with congenital heart disease, but each has its own limits, such as the low sensitivity of Qingdao, the low adaptation of Ross score because of the high breast-feeding rate in our country and the low specificity of NYU PHFI when > 2 was set as the cut-off point. Plasma concentrations of BNP and NT-proBNP were valuable for diagnosing HF in children with congenital heart disease, and NT-proBNP was the independent predictor for HF.

Adolescent ; Biomarkers ; blood ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; blood ; complications ; diagnostic imaging ; Heart Failure ; blood ; diagnosis ; diagnostic imaging ; etiology ; physiopathology ; Humans ; Infant ; Male ; Natriuretic Peptide, Brain ; blood ; Peptide Fragments ; blood ; Protein Precursors ; blood ; ROC Curve ; Reference Values ; Regression Analysis ; Sensitivity and Specificity ; Severity of Illness Index ; Ultrasonography ; Ventricular Function, Left