I-Seok Kang

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.

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.

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.

Fragmented QRS complex in adult patients with Ebstein anomaly and its association with arrhythmic risk and the severity of the anomaly.

Background—Fragmented QRS complex (fQRS) on 12-lead ECG, a marker of myocardial scar, is a predictor of arrhythmic events in patients with ischemic and nonischemic cardiomyopathy. We investigated whether the presence of fQRS is associated with the severity of the anomaly and with increased arrhythmic events in adult patients with Ebstein anomaly (EA). Methods and Results—In 51 consecutive adult patients with EA (median age, 37 years; 18 males), the severity index of EA calculated from echocardiographic data and clinical arrhythmic events were analyzed. The extent of fQRS in each patient was measured by counting the number of ECG leads showing fQRS. There were 35 (68.6%) patients with fQRS (fQRS group) and 16 (31.4%) patients without fQRS (non-fQRS group). fQRS was observed more frequently in the inferior (n=26) and precordial (n=25) leads versus the lateral leads (n=5). The patients in the fQRS group had a worse functional class, greater cardiothoracic ratios, more severe tricuspid regurgitation, larger atrialized right ventricular areas, higher EA severity scores, and more frequent arrhythmic events compared with those in the non-fQRS group. The atrialized right ventricular area showed a positive correlation with the fQRS extent (r=0.51; P<0.001). In multivariable Cox regression models, the presence of fQRS was independently associated with arrhythmic events (P=0.036). Conclusions—Fragmented QRS on 12-lead ECG was associated with larger atrialized right ventricular area and an increased risk of arrhythmic events in adult patients with EA.

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.

Clinical and genetic analysis of Korean patients with Marfan syndrome: possible ethnic differences in clinical manifestation

Yoo E‐H, Woo H, Ki C‐S, Lee HJ, Kim D‐K, Kang I‐S, Park P, Sung K, Lee CS, Chung T‐Y, Moon JR, Han H, Lee S‐T, Kim J‐W. Clinical and genetic analysis of Korean patients with Marfan syndrome: possible ethnic differences in clinical manifestation.

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.

Interstage mortality for functional single ventricle with heterotaxy syndrome: a retrospective study of the clinical experience of a single tertiary center

BackgroundIn spite of improved survival after palliation for single ventricle, interstage mortality for a single ventricle with heterotaxy syndrome is unknown. The purpose of this study was to quantify interstage mortality and influence mortality risk factors.MethodsFrom November 1994 until February 2012, all patients that had a functional single ventricle and heterotaxy syndrome who underwent palliative operations at our center were included. Patients with hypoplastic left heart syndrome and operative mortality cases were excluded. The factors that influenced interstage mortality were determined by multivariate Cox analysis.ResultsThere were 16 patients with interstage mortality (41.0%), much higher than the non-heterotaxy group (vs. 11.3%, P = 0.001, OR = 5.478). The major presumptive causes of death were infection or sepsis (37.5%) and unknown sudden death (31.3%). When we compared the survival group and the mortality group with heterotaxy syndrome, Blalock-Taussig shunt as a 1st palliation is most common for both groups but there were more for the mortality group (81.2% vs. 52.2%), and there were more with bidirectional cavo-pulmonary shunt as a 1st palliation in the survival group (10 patients vs. 2 patients). The existence of pulmonary vein stenosis at initial diagnosis was more common for the mortality group. In multivariate Cox analysis, however, the duration of hospitalization at palliation, the duration of intensive care unit stay after palliation and the existence of pulmonary vein stenosis at diagnosis were significant risk factors.ConclusionInterstage mortality for a functional single ventricle with heterotaxy syndrome is significantly higher than for non-heterotaxy syndrome. Therefore more attention should be given to the prevention of interstage mortality in these patients with risk factors.

Clinical outcomes of slide tracheoplasty in congenital tracheal stenosis

OBJECTIVES Treatment of long-segment congenital tracheal stenosis (CTS) remains challenging. Recently, slide tracheoplasty has become the standard approach in many centres. The aim of this study was to evaluate the clinical outcomes of slide tracheoplasty. METHODS Between 2004 and 2011, 18 patients underwent slide tracheoplasty in our centre. The median patient age was 2.5 months (range, 18 days-4 years) and the median body weight was 4.2 (range, 2.2-17.7) kg at operation. Eleven (61%) patients were on a mechanical ventilator prior to surgery. The median stenotic segment estimated by a computed tomography scan was 52% of the length of total trachea (range, 18-84%). Five (28%) patients had proximal bronchial stenosis, 3 (17%) had tracheal bronchus, 2 (11%) had tracheobronchomalacia and 1 (6%) had agenesis of the right lung. Thirteen (72%) patients had a combined cardiac anomaly, including 8 patients with a pulmonary artery sling. Ten (56%) patients had associated extracardiac anomalies. Slide tracheoplasty was performed on cardiopulmonary bypass in all patients, and cardiac lesions were corrected. RESULTS There was no early death. The patient with agenesis of the right lung died of left bronchial stenosis 3 months after the surgery. Two (11%) patients were reoperated on for tracheal restenosis. In the other 15 patients, the median duration of ventilator support was 8 (range, 5-34) days and the median duration of hospitalization was 31 (range, 12-79) days. During the follow-up (median duration of 17 months; range, 2-77 months), 13 (72%) patients were symptom-free and 2 (11%) underwent tracheostomy for tracheomalacia. CONCLUSIONS Based on this study, slide tracheoplasty seems to be an effective technique for CTS. However, shortening of the trachea after reconstruction may give rise to recurrent obstruction.