Clinical and Prognostic Value of Depressive Symptoms and N-Terminal Pro-B-Type Natriuretic Peptide in Patients With Eisenmenger Syndrome.

OBJECTIVES: Eisenmenger syndrome (ES) is commonly associated with depressive symptoms and elevated N-terminal pro-B-type natriuretic peptide (NT-proBNP). We investigated the predictive value of depressive symptoms and NTproBNP levels for long-term outcomes in patients with ES. METHODS: Blood was drawn to measure NT-proBNP, and depressive symptoms were measured using the Korean version of the Beck Depression Inventory (BDI) in an outpatient clinic sample of 64 patients with ES (67% female; median age = 41.5 years [range, 21.0-74.8 years]). Cardiac events (hospitalization, emergency department visits, and cardiac death) were monitored during 3 years of follow-up. RESULTS: During the follow-up period, 15 (23.4%) patients experienced a cardiac event. The combination of depressive symptoms and NT-proBNP levels better predicted future cardiac events than either variable alone. Patients with NT-proBNP > 510 pg/ml and a total BDI score > 10 had a 9.6 times higher risk for cardiac events than did patients with NT-proBNP ≤ 510 pg/ml or total BDI score ≤ 10 (p < .001). CONCLUSIONS: Depressive symptoms and NT-proBNP levels are both associated with adverse clinical outcomes in ES.

Changes in coronary perfusion after occlusion of coronary arteries in Kawasaki disease.

PURPOSE: Myocardial infarction in children with total occlusion of a coronary artery after Kawasaki disease is rare due to multiple collateral vessels. We aimed to investigate the changes in coronary perfusion associated with coronary artery occlusion after Kawasaki disease. MATERIALS AND METHODS: Eleven patients with coronary artery occlusion after Kawasaki disease were investigated. Serial coronary angiographies after total occlusion of a coronary artery were reviewed and the changes were described in all patients with additive information collected. RESULTS: The median age at the occlusion was 5.9 years old. The interval to occlusion was 6.2±6.9 years. Four left anterior descending coronary artery total occlusions and 10 right coronary artery total occlusions were detected. Immediate coronary artery bypass graft for left anterior descending coronary artery total occlusion made right coronary total occlusion occurred in all except one patient and the intervals thereof were 1 year, 1.8 years, and 4 years. Collaterals to the left coronary artery regressed after recanalization, while new collaterals to the right coronary artery developed. In three, collaterals to the right coronary artery decreased without recanalization without clinical signs. CONCLUSION: The right coronary artery should be followed up carefully because of possible occlusion of new onset or changes in collaterals.

Amiodarone and catheter ablation as cardiac resynchronization therapy for children with dilated cardiomyopathy and wolff-Parkinson-white syndrome.

Preexcitation by accessory pathways (APs) is known to cause dyssynchrony of the ventricle, related to ventricular dysfunction. Correction of ventricular dyssynchrony can improve heart failure in cases of dilated cardiomyopathy (DCMP) with preexcitation. Here, we report the first case of a child with DCMP and Wolff-Parkinson-White (WPW) syndrome treated with amiodarone and radiofrequency catheter ablation (RFCA) in Korea. A 7-year-old boy, who suffered from DCMP and WPW syndrome, showed improved left ventricular function and clinical functional class after treatment with amiodarone to eliminate preexcitation. QRS duration and left ventricular ejection fraction (LVEF) were inversely correlated with amiodarone dosage. After confirming the reduction of preexcitation effects in DCMP, successful RFCA of the right anterior AP resulted in LVEF improvement, along with the disappearance of preexcitation. Our findings suggest that ventricular dyssynchrony, caused by preexcitation in DCMP with WPW syndrome, can worsen ventricular function and amiodarone, as well as RFCA, which should be considered as a treatment option, even in young children.

Clinical features of adult patients with Eisenmenger's syndrome in Japan and Korea.

BACKGROUND: There are few articles on mortality and morbidity of adult patients with Eisenmenger's syndrome (ES) in the current era when disease targeting therapy (DTT) has been available. METHODS AND RESULTS: 198 patients (a median age 35 years, 64% female) with ES who visited the 16 participating institutes in Japan and Korea from 1998 to 2009 were enrolled. Clinical data during adulthood were collected from each institutional chart and analyzed centrally. During a median follow-up of 8 years, 30 patients died including 14 sudden deaths. 89 patients took oral medication of DTT and clinical improvement was observed in 54 of them. However, survival rate in patients taking DTT was not different from those without (87% vs 84%, p=0.55). When the clinical data in between first and last clinic visits were compared in 85 patients, the patients with NYHA >/=III increased from 24% to 48% (p<0.001), SpO2 decreased from 89% to 85% (p=0.008) and hematocrit increased from 51.4% to 52.9% (p=0.04). Non-survivors had poorer NYHA function class, lower body weight (BW), lower body mass index (BMI), and higher serum level of Cr at the first visits than survivors. CONCLUSIONS: Long term survival and clinical status of adult patients with ES remains unsatisfactory even in the current era of DTT. Poor NYHA functional class, low BW, low BMI and high serum level of Cr were related to mortality. DTT therapy improved clinical status in many patients with Eisenmenger's syndrome, but no significant impact on survival could be shown.

Surgical strategy in patients with atrial septal defect and severe pulmonary hypertension.

The aim of the study was to review our experience with atrial septal defect (ASD) closure with a fenestrated patch in patients with severe pulmonary hypertension. Between July 2004 and February 2009, 16 patients with isolated ASD underwent closure with a fenestrated patch. All patients had a secundum type ASD and severe pulmonary hypertension. Patients ranged in age from 6 to 57 years (mean ± SD, 34.9 ± 13.5 years). The follow-up period was 9 to 59 months (mean, 34.5 ± 13.1 months). The ranges of preoperative systolic and pulmonary arterial pressures were 63 to 119 mm Hg (mean, 83.8 ± 13.9 mm Hg) and 37 to 77 mm Hg (mean, 51.1 ± 10.1 mm Hg). The ranges of preoperative values for the ratio of the pulmonary flow to the systemic flow and for pulmonary arterial resistance were 1.1 to 2.7 (mean, 1.95 ± 0.5) and 3.9 to 16.7 Wood units (mean, 9.8 ± 2.9 Wood units), respectively. There was no early or late mortality. Tricuspid annuloplasty was performed in 14 patients (87.5%). The peak tricuspid regurgitation gradient and the ratio of the systolic pulmonary artery pressure to the systemic arterial pressure were decreased in all patients. The New York Heart Association class and the grade of tricuspid regurgitation were improved in 13 patients (81.2%) and 15 patients (93.7%), respectively. ASD closure in patients with severe pulmonary hypertension can be performed safely if we create fenestration. Tricuspid annuloplasty and a Cox maze procedure may improve the clinical result. Close observation and follow-up will be needed to validate the long-term benefits.

Relation of fragmented QRS complex to right ventricular fibrosis detected by late gadolinium enhancement cardiac magnetic resonance in adults with repaired tetralogy of fallot.

Fragmented QRS (fQRS) on 12-lead electrocardiography reflects conduction delay caused by myocardial fibrosis and dysfunction. Ventricular fibrosis detected by late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is reportedly correlated with worse clinical outcomes in adults with repaired tetralogy of Fallot (TOF). The aim of this study was to assess whether the presence of fQRS is associated with right ventricular (RV) fibrosis or dysfunction in this patient group. In 37 consecutive patients (median age 30 years, median age at repair 6.6 years), the number of leads showing fQRS, defined as the presence of >2 notches on the R/S wave in ≥2 contiguous leads, was counted. RV systolic function, dilatation, and LGE score were measured using LGE CMR. Ventricular LGE was observed mainly at the previous surgical sites: the RV outflow tract (33 of 37), ventricular septal defect patch region (15 of 37), and RV anterior wall (11 of 37). Fragmented QRS was found mostly in the right and mid precordial leads. The fQRS group (n = 20) demonstrated higher RV LGE scores (p <0.001) and lower RV ejection fractions (p = 0.02) and a trend toward larger RV end-diastolic and end-systolic volumes (p = 0.12 and p = 0.06, respectively) compared to the non-fQRS group (n = 17). The number of electrocardiographic leads showing fQRS was positively correlated with RV LGE score (r = 0.75, p <0.001). The presence of fQRS remained independently associated with the presence of supramedian RV LGE score, even after adjusting for relevant parameters. In conclusion, fQRS was closely associated with more extensive RV fibrosis and dysfunction in adults with repaired tetralogy of Fallot.

Clinical features and genetic analysis of Korean patients with Loeys-Dietz syndrome.

Loeys-Dietz syndrome (LDS) is an inherited disorder that is characterized by the triad of arterial tortuosity and aneurysms, hypertelorism and a bifid uvula or cleft palate. The disease is caused by heterozygous mutations in the genes encoding transforming growth factor ß receptors 1 and 2 (TGFBR1 and TGFBR2, respectively). However, studies of patients with LDS are limited in Korea. From June 2000 to December 2010, 13 patients (10 probands) diagnosed with LDS were enrolled. The multidisciplinary data of the patients were reviewed retrospectively. The frequency of each clinical manifestation in Korean patients with LDS was compared with Western populations as described in the report by Loeys et al. Twelve (92%) of the 13 LDS patients had arterial tortuosity, 9 (69%) patients had hypertelorism and 11 (85%) patients had bifid uvula or cleft palate. Mutations in either TGFBR1 or TGFBR2 were detected in nine probands (90%). Of the mutations, five novel mutations were detected; three in TGFBR2 and two in TGFBR1. Blue sclera and atrial septal defect were not observed in the Korean patients, and the frequency of blue sclera was significantly lower in our Korean population than previously-described Western population (0 vs 40%; P=0.005). Despite the restricted number of patients in our study, we identified five novel mutations in the TGFBR1 and TGFBR2 genes and, except for blue sclera, no differences in phenotype are apparent between Korean patients and Western patients.

Retrospective analysis of the clinical manifestations and survival of Korean patients with mucopolysaccharidosis type II: emphasis on the cardiovascular complication and mortality cases.

Hunter syndrome (mucopolysaccharidosis II, MPS II) is a rare, X-linked disorder of glycosaminoglycan (GAG) catabolism caused by a deficiency in the activity of the lysosomal enzyme, iduronate-2-sulfatase (I2S). In this study, the medical records of 75 Korean patients with Hunter syndrome (74 males, 1 female) were retrospectively reviewed to investigate the frequency of organ involvement and survival at a single center. The three most common symptoms of organ involvement were hepatosplenomegaly (99%), facial dysmorphism (97%), and frequent otitis media (91%). Cardiovascular involvement was also common including valvular abnormalities (89%), left ventricular hypertrophy (68%), and hypertension (30%). The 19 patients who died had a median age of 16.8 years at the time of death. Four of them died within 1 year of the start of enzyme replacement therapy; autopsy showed myocardial infarction with severe coronary artery disease in one patient. Two other patients died due to pneumonia and sleep apnea. In one case, the cause of death was not investigated. The high incidence of hypertension, and the presence of valvular heart disease indicates that close cardiac monitoring is mandatory in all patients with Hunter syndrome, especially relatively older patients even if they are being treated with enzyme replacement therapy.

A comparison of the Ghent and revised Ghent nosologies for the diagnosis of Marfan syndrome in an adult Korean population.

Recently, a revised Ghent nosology has been established for the diagnosis of Marfan syndrome (MFS) that puts more weight on the aortic root aneurysm and ectopia lentis. We compared the application of the Ghent and revised Ghent nosologies in adult Korean patients for whom there is suspicion of MFS. From January 1995 to June 2010, we enrolled 106 patients older than 20 years for whom there was suspicion of MFS, and who had undergone genetic analysis of the fibrillin-1 gene (FBN1). Of 106 patients, 86 patients (81%) fulfilled the criteria of the Ghent nosology, and 84 patients (79%) met the criteria of the revised Ghent nosology. The two patients who met the Ghent nosology criteria, but not the criteria of the revised Ghent nosology were diagnosed with Loeys-Dietz syndrome and MASS phenotype. The level of agreement between both nosologies was very high (κ = 0.94, 95% confidence interval: 0.86 to 1.0). Marfan-like syndromes were diagnosed in 30% (6/20 patients) with negative Ghent and revised Ghent criteria and no FBN1 mutations. These results suggest that adult Korean patients who fulfill the old Ghent criteria almost all fulfill the new criteria for the diagnosis of MFS.

Omission of a prior Glenn anastomosis is a risk factor for prolonged pleural drainage after the fenestrated extracardiac conduit Fontan procedure.

OBJECTIVE: Factors related to prolonged pleural drainage after the Fontan operation have not been clearly defined. We investigated perioperative variables to establish factors predicting operative morbidity including prolonged chest tube drainage. Also, we pursued the fate of the fenestration during the follow-up period. METHODS: We retrospectively reviewed 52 patients who had undergone a fenestrated extracardiac Fontan procedure between August 1998 and June 2008. The median age at the time of surgery was 34.8 (range: 18.5 ∼ 156) months and the median body weight 13.2 kg (range: 9.5 ∼ 33). A multivariable logistic regression model was used to compare demographic, anatomic, and physiological variables for postoperative morbidity. RESULTS: Operative mortality occurred in one patient (1.9%). The mean duration of respiratory support, chest tube drainage, and hospital stay was 13 hours (range: 4 to 328 hours), six days (range: 2 to 45 days), and 16 days (range: 7 to 444 days), respectively. Statistically, an operation without previous bidirectional cavopulmonary shunt (OR 30, 95% CI 3.1 to 289) was the only independent risk factor for prolonged pleural drainage. Aortic cross-clamp time was identified as a risk factor for prolonged mechanical ventilatory support. During a median follow-up at 62 months (range: 17 to 137 months), there was one late death (1.9%). Twenty-two patients (43%) underwent intervention for fenestration closure. CONCLUSIONS: Previous bidirectional cavopulmonary shunt and shortened aortic cross-clamp time may reduce postoperative morbidity including prolonged chest tube drainage and mechanical ventilator support after the fenestrated extracardiac conduit Fontan procedure.

Outcome of single-stage repair of coarctation with ventricular septal defect.

BACKGROUND: The optimal surgical strategy for coarctation of the aorta (CoA) with ventricular septal defect (VSD) is controversial. The aim of this study is to evaluate the clinical outcome of a single-stage repair of CoA with VSD. METHODS: We reviewed 72 patients who underwent single-stage repair for CoA with VSD between January 1995 and December 2007. There were 43 males and 29 females. The median age of the patients was 28 days (range = 3 to 188) and median weight was 3.7 kg (range = 2.16 to 5.6). Deep hypothermic circulatory arrest was used in 22 patients and selective antegrade cerebral perfusion was performed in 43 patients. RESULTS: There were no operative deaths and one late death at a median follow-up of 60 months (range = 16 to 158). Postoperative complications were left main bronchus compression requiring aortopexy in one patient and recoarctation requiring balloon dilatation in one patient. Subaortic stenosis occurred in two patients and surgical repair was performed. CONCLUSIONS: One-stage simultaneous repair can be performed with a low risk at an early age.

Partially unroofed coronary sinus: MDCT and MRI findings.

OBJECTIVE: The purpose of this study was to analyze the MDCT and MRI findings in patients with unroofed coronary sinus syndrome. MATERIALS AND METHODS: This retrospective study included 11 patients with unroofed coronary sinus syndrome (10 adults, one child) without persistent left superior vena cava and one adult with communication of the left atrium and coronary sinus via an anomalous vein (unroofed coronary sinus variant). Four patients underwent contrast-enhanced ECG-gated MDCT; six, MRI; and two patients, both CT and MRI. We also measured the coronary sinus on the CT scans of 28 adults with normal cardiac anatomy and 10 adults with persistent left superior vena cava and compared the measurements with those in the patients with unroofed coronary sinus syndrome. Seven patients underwent surgical treatment of unroofed coronary sinus syndrome. RESULTS: At echocardiography, unroofed coronary sinus syndrome was not clearly discriminated from atrial septal defect in two patients and was not suspected in three patients. CT and MRI showed that 11 patients had a defect in which the coronary sinus communicated with the left atrium and that the other patient had atresia of the coronary sinus orifice with an anomalous vein connecting the coronary sinus and left atrium. In patients with unroofed coronary sinus syndrome, the mean standardized diameter of the coronary sinus according to the patient's body surface area was 15 ± 4 mm/m², similar to that of the control group with persistent left superior vena cava (15 ± 6 mm/m²; p = 0.97) and significantly greater than that of the group with normal cardiac anatomy (7 ± 2 mm/m²; p < 0.0001). CONCLUSION: CT and MRI facilitate definite diagnosis of unroofed coronary sinus syndrome.

Dual pulsed-wave Doppler tracing of right ventricular inflow and outflow: single cardiac cycle right ventricular tei index and evaluation of right ventricular function.

BACKGROUND AND OBJECTIVES: The reliability and usefulness of the right ventricular (RV) Tei index (RTX) remains controversial because it has not been possible to simultaneously measure RV inflow and outflow. However, dual pulsed-wave Doppler (DPD) enables flow velocities to be obtained at different sampling sites simultaneously. In this study we evaluated the feasibility and reliability of RTX values obtained by DPD (RTX(DPD)). SUBJECTS AND METHODS: Forty-one patients who underwent cardiac catheterization and echocardiography for RV volume or pressure overloading conditions were evaluated. Symptom-limited exercise treadmill testing with expired gas analysis was performed and maximal exercise capacity was measured. RESULTS: RTX by conventional flow Doppler (RTX(CFD), 0.262±0.164) was similar to RTX(DPD) (0.253±0.117, p=NS), whereas RTX by tissue Doppler echocardiography (RTX(TDE), 0.447±0.125) was significantly larger than RTX(DPD) (p<0.001). Based on multiple regression analysis, maximal exercise capacity was independently related to RTX(DPD) (ß=-0.60, p<0.001), mid-RV dimension (ß=-0.26, p=0.012), left ventricular ejection fraction (ß=0.22, p=0.023), and early diastolic tricuspid annular velocity (ß=0.21, p=0.048). CONCLUSION: It is feasible and reliable to evaluate RV function using RTX(DPD) values. However, to evaluate the clinical usefulness of RTX(DPD), additional studies are required with a large number of patients and long-term follow-up.

Accuracy of CoaguChek XS for point-of-care antithrombotic monitoring in children with heart disease.

The CoaguChek XS international normalized ratio (INR) assay was compared to INR assay by a standard laboratory method in children with heart disease on anticoagulant therapy. The data comprised 120 pairs of INR values for 42 patients (age <16 yr) who attended a cardiology clinic between 1 May 2007 and 30 January 2008. Parallel INR assays by the CoaguChek XS and the standard method were performed within 1 hr by a single qualified technician and the paired results were evaluated by linear regression and Bland-Altman analysis. The mean difference in the INR values was -0.08 +/- 0.04 units (p = 0.63); the difference between the two results was consistently <0.5 INR units. The slope of the regression line was 0.98 (95% CI: 0.96 to 1.01) and the y-intercept was 0.014 (95% CI: -0.01 to 0.04). In the Bland-Altman analysis, the mean difference in INR between the two methods was 0.08 units and values for 99.4% of the patients fell within the limit of agreement (-0.17 to 0.28 units). In summary, INR assays in children by the CoaguChek XS device are as accurate as the standard method, but faster and more convenient.

Clinical experience of chest pain in adults with congenital heart disease in a single tertiary center.

The aim of this study was to investigate clinical manifestation and causes of chest pain in adults with congenital heart disease. Of 966 patients in our Adult Congenital Heart Clinic, 50 patients had cyanosis (cyanosis [+]), and 916 patients did not have cyanosis (cyanosis [-]). Fifty-four patients (16 male and 38 female) with a history of chest pain requiring medical care were selected: 33 were in the cyanosis (+) group, and 21 were in the cyanosis (-) group. Medical records were reviewed. The causes of chest pain were (no. cyanosis [+], no. cyanosis [-], respectively) as follows: idiopathic (n = 19, n = 10), pulmonary hemorrhage (n = 6, n = 0), pulmonary tuberculosis (n = 2, n = 0), pulmonary thromboembolism (n = 4, n = 0), localized musculoskeletal (n = 1, n = 1), myocardial ischemia (n = 1, n = 1), and arrhythmia (n = 2, n = 9). Patients with cyanosis had much higher frequency of chest pain compared with patients without cyanosis (33 of 50 vs. 21 of 916, p < 0.0001). Chest pain in adults with congenital heart disease has diverse causes. Meticulous evaluation of chest pain in adults with congenital heart disease is required to differentiate correctable causes from idiopathic or incurable ones.

A single-center experience with intracardiac thrombosis in children with dilated cardiomyopathy.

Intracardiac thrombosis in patients with a dilated cardiomyopathy can be life threatening. This study investigated the incidence, risk factors, and outcome of intracardiac thrombosis in children with dilated cardiomyopathy. A retrospective review of the clinical records was performed in 83 children with dilated cardiomyopathy diagnosed from January 1995 to December 2008. Intracardiac thrombi were detected in 5 patients (6.0%). The intracardiac thrombi were found mainly in the left ventricle (n = 3). One patient had a thrombus in the left atrium at the time of diagnosis, and a right ventricular thrombus was found in 1 patient with unrepaired ventricular septal defect complicated by pulmonary hypertension. Intracardiac thrombosis developed during rapid deterioration of ventricular function, and all patients had a poor ejection fraction of the left ventricle. All patients were treated with heparinization, and thrombectomy was performed in 1 patient. Three patients achieved complete resolution of the thrombus without further embolic complications. Careful evaluation and aggressive anticoagulation are necessary for the prevention of intracardiac thrombosis in children with poor ventricular function, especially during rapid deterioration of ventricular function.

Closure of conduit fenestration after extracardiac Fontan procedure using Amplatzer vascular plug: comparison with detachable coil.

The objective of this study was to report the outcome of fenestration closure using either an Amplatzer Vascular Plug I or detachable coils in patients undergoing extracardiac Fontan procedure with conduit fenestration. We reviewed the medical records of 23 patients who underwent transcatheter closure of conduit fenestration with the extracardiac Fontan procedure. The median age at fenestration closure was 57 months (range 36 to 98). The median interval between the operation and the procedure was 28 months (range 8 to 68). The Amplatzer Vascular Plug I (AVP) was used in 16 patients, and detachable coils were used in 7 patients. In the coil group, we used 1 coil in 3 patients, 2 coils in patients, and 3 coils in 1 patient, all based on angiographic results. In the AVP group, we used a device that was at least 1 mm larger than the initial fenestration size. No immediate or long-term complications were associated with the procedure or with the closure devices. On final follow-up, 3 of 7 patients (43%) in the coil group had complete closure, and 17 of 17 patients (100%) in the AVP group had complete closure. The AVP showed good results for transcatheter closure of a conduit-type fenestration and was found to perform better than the detachable coil.

Factors influencing depression in adolescents with congenital heart disease.

OBJECTIVE: This study was designed to identify variables associated with depression in adolescents who underwent operation for congenital heart disease (CHD). METHODS: Data were collected from 231 adolescents, aged 13 to 18 years with CHD, during outpatient clinic follow-up after open heart surgery in 3 major cardiac centers in Korea. Adolescents completed measures of resilience, depression, and parental attitude. Their New York Heart Association functional class, CHD functional index, and noninvasive saturation of arterial oxygen were also measured. RESULTS: There were significantly positive relations between depression and the 3 variables: older age, worse New York Heart Association functional class, and higher CHD functional index. Negative relations were found between depression and higher saturation of arterial oxygen, higher academic achievements, affectionate parental attitude, and higher resilience. The multiple regression analysis also showed that 62% of the variance in depression in adolescents with CHD could be explained by resilience and parental attitude. CONCLUSION: This study demonstrated that adolescents with higher resilience and an affectionate parent were less depressed.

Outcomes of pregnancy in women with congenital heart disease: a single center experience in Korea.

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.

Outcome following surgical closure of patent ductus arteriosus in very low birth weight infants in neonatal intensive care unit.

PURPOSE: The aims of this study were to determine the factors affecting the outcome of patent ductus arteriosus ligation in very low birth weight infants (VLBWI) and demonstrate the safety of PDA ligation in VLBWI performed in the neonatal intensive care unit (NICU). MATERIALS AND METHODS: From October 1994 to July 2006, medical records of 94 VLBWI weighing <1,500 g who underwent PDA ligation in the NICU of Samsung Medical Center were reviewed retrospectively. Factors affecting the final outcome of PDA ligation were evaluated by dividing the infants into 3 groups according to mortality and major morbidities as follows: mortality group (Mo), major morbidity group (Mb), and no major morbidity group (NM). RESULTS: In the Mo group, birth weight was significantly lower and the preoperative mean FiO2 and mean dopamine dose were significantly higher than those in the other 2 groups. There was no significant difference in gestational age, incidence of RDS, number of courses of indomethacin, surgery-related factors, including weight and age at surgery, perioperative vital signs, and complications after surgery between the 3 groups. During surgery in the NICU, there were no significant hemodynamic instability or serious acute complications. CONCLUSION: The factors affecting the outcome of surgery in VLBWI are not the factors related to surgery but the preoperative conditions related to the underlying prematurity. PDA ligation of VLBWI performed in the NICU is safe without serious complications.