Heung Jae Lee

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.

Mri of Total Anomalous Pulmonary Venous Connections

Objective A prospective MR study was undertaken in 13 patients with total anomalous pulmonary venous connection (TAPVC) to determine the value of MR in its detection. Materials and Methods Echocardiography was performed in all cases and cardiac angiography was performed in eight patients before MRI. Results Magnetic resonance detected TAPVC in four cases without prior echocardiographic and angiographic diagnosis. In seven surgically proven cases, the diagnostic accuracy of preoperative MR, echocardiography, and cardiac angiography was 100 (7 of 7), 57 (4 of 7), and 25% (1 of 4), respectively. In the remaining cases including three cases for postoperative evaluation, MR findings correlated well with those of echocardiography or angiography. The combination of axial and coronal MRI visualized 96% of the individual anomalous pulmonary veins and 100% of the common pulmonary veins. Stenosis of a common pulmonary vein (three cases) or the superior vena cava (one case) was identified on MR in all cases. Conclusion Magnetic resonance is an effective modality in depicting TAPVCs.

Epidemiologic picture of Kawasaki disease in Korea, 2000–2002

Background : The aim of this study was to investigate the incidence rate and epidemiologic patterns of Kawasaki disease in Korea for a 3 year period during 2000 to 2002.

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 ≥2 in 2.0%, and cardiac death in 2.0%. Independent predictors of adverse maternal cardiac events were an NYHA functional class of ≥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 ≥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.

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.

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 patients 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.

MR imaging of congenital heart diseases in adolescents and adults.

Echocardiography and catheterization angiography suffer certain limitations in the evaluation of congenital heart diseases in adults, though these are overcome by MRI, in which a wide field-of view, unlimited multiplanar imaging capability and three-dimensional contrast-enhanced MR angiography techniques are used. In adults, recently introduced fast imaging techniques provide cardiac MR images of sufficient quality and with less artifacts. Ventricular volume, ejection fraction, and vascular flow measurements, including pressure gradients and pulmonary-to-systemic flow ratio, can be calculated or obtained using fast cine MRI, phase-contrast MR flow-velocity mapping, and semiautomatic analysis software. MRI is superior to echocardiography in diagnosing partial anomalous pulmonary venous connection, unroofed coronary sinus, anomalies of the pulmonary arteries, aorta and systemic veins, complex heart diseases, and postsurgical sequelae. Biventricular function is reliably evaluated with cine MRI after repair of te ralogy of Fallot, and Sennings and Mustards operations. MRI has an important and growing role in the morphologic and functional assessment of congenital heart diseases in adolescents and adults.

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.

Somatostatin for postoperative chylothorax after surgery for children with congenital heart disease.

Chylothorax is a rare but serious postoperative condition with a high rate of morbidity that may lead to death of children with congenital heart disease. Here we reviewed nine consecutive cases with chylothorax in infants and children following cardiac surgery from March 2002 to February 2003. Somatostatin was added to conservative treatment proctocol to increase effectiveness of therapy in all cases. The duration of somatostatin therapy varied from 7 to 32 days. All cases of chylothorax were successfully treated with intravenous infusion of somatostatin as an adjunctive treatment. Even though two cases showed rebound phenomena, we avoided any surgical procedure in the nine patients who treated with conservative management combined with somatostatin. No significant side effects of somatostatin were observed. It seems that somatostatin is effective, noninvasive and safe therapeutic modality. It can be used as an adjunctive treatment to conservative management to control postoperative chylothorax in children with congenital heart disease.

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.