Woong-Han Kim

Outcome of 200 patients after an extracardiac Fontan procedure

OBJECTIVES Despite the known advantages of the extracardiac conduit Fontan procedure, the long-term outcomes related to the longevity of the conduit and anticoagulation therapy have not been determined. The purpose of this study was to evaluate the outcome of hospital survivors with an extracardiac Fontan circulation. METHODS AND RESULTS Between 1996 and 2006, 200 patients had the extracardiac conduit Fontan operation. The median age at operation was 3.4 years. Most patients (89.5%) underwent a bidirectional cavopulmonary shunt. Fenestration was required in 85 patients. Overall, the 10-year survival was 92.4% +/- 2.1%. Multivariate analysis identified severe infection during the early postoperative period and a high pulmonary arterial pressure during the preoperative period as independent risk factors for patient mortality. The Kaplan-Meier estimate for freedom from reoperation was 82.4% +/- 4.1% at 10 years. Arrhythmia occurred in 32 patients after the Fontan operation; freedom from arrhythmia was 85.1% +/- 4.4% at 10 years. The risk factors for arrhythmia were the heterotaxy syndrome, follow-up duration, and age at Fontan operation. Freedom from thromboembolism at 10 years was 92.9% +/- 1.9%. Among all of the patients, 95.2% were classified in New York Heart Association class I. CONCLUSIONS The results of this study showed that during 10 years of follow-up, the overall survival and the functional status of the survivors after an extracardiac Fontan procedure were satisfactory. We might infer that fenestration provided benefit inasmuch as the high-risk Fontan patients fenestrated had similar outcomes to those not fenestrated, who were presumably low risk. The incidence of late death, reoperation, obstruction of the cavopulmonary pathway, arrhythmias, and thromboembolism was low.

Diagnostic Dilemma of Pseudoprogression in the Treatment of Newly Diagnosed Glioblastomas: The Role of Assessing Relative Cerebral Blood Flow Volume and Oxygen-6-Methylguanine-DNA Methyltransferase Promoter Methylation Status

BACKGROUND AND PURPOSE: Methylation of the MGMT gene promoter is associated with a favorable prognosis in adult patients with GBM treated with TMZ. We determined the incidence of pseudoprogression according to the MGMT methylation status and the potential value of DSC perfusion MR images for predicting pseudoprogression. MATERIALS AND METHODS: New or enlarged enhancing lesions after CCRT in adult patients with newly diagnosed GBMs were prospectively assessed by measuring their rCBV by using DSC perfusion MR images. Tumor tissue was assayed to determine MGMT promoter methylation status. All patients were regularly followed up at an interval of 2 months by MR images, including DSC perfusion MR images. RESULTS: Ninety eligible patients were enrolled in this study. After CCRT, new or enlarged enhanced lesions were found in 59 of 90 patients, which were subsequently classified as pseudoprogression (26 patients, 28.9%) and real progression (33 patients, 36.7%). Overall, there was a significant difference in the mean rCBV between pseudoprogression and real tumor progression (P = .003). The ROC curve revealed that an rCBV ratio >1.47 had an 81.5% sensitivity and a 77.8% specificity. The unmethylated MGMT promoter group had a significant difference of mean rCBV between pseudoprogression and real progression (P = .009), though the methylated MGMT promoter group had no significant difference (P = .258). CONCLUSIONS: The current study suggests that rCBV measured by DSC perfusion MR images has a differential impact on the predictability of pseudoprogression in patients with GBM.

Long-term results of the arterial switch operation for ventriculo-arterial discordance

OBJECTIVES The arterial switch operation (ASO) has become the standard surgical procedure for transposition of the great arteries (TGA) or variants with an excellent early outcome. However, there are concerns regarding neopulmonary stenosis, neoaortic regurgitation (neoAR) associated with neoaortic root dilatation and coronary artery disease. METHODS A total of 220 early survivors of the ASO were included in this retrospective study between November 1987 and June 2011. The median age and weight at operation were 13 days (0-1768 days) and 3.52 kg (1.69-19 kg), respectively. The indications for the ASO included TGA with intact ventricular septum in 113 patients, TGA with ventricular septal defect in 90 and Taussig-Bing anomaly in 17 patients. The median follow-up period was 103.2 months (0.4-277.4 months). Statistical analyses with the Kaplan-Meier and Cox proportional hazards models were performed. RESULTS The actuarial late survival rate and freedom from reoperation at 23 years were 96.6 ± 1.3 and 81.9 ± 3.8%, respectively. Twenty-four (10.9%) patients underwent reoperations for right ventricular outflow tract obstruction in 10 patients, neoAR in four and coronary artery stenosis in three, etc. Freedom from neoAR of Grades IV, III and II at 23 years was 90.2 ± 6.6, 70.9 ± 9.6 and 20.3 ± 5.5%, respectively. The risk factors for neoAR were size discrepancy of the great arteries, aortic root dilatation after the ASO and follow-up duration after the ASO. NeoAR was significantly correlated with the size of aortic sinus and aortic sinotubular junction over time. Freedom from pulmonary stenosis (PS) of ≥ 36 and ≥ 20 mmHg at 23 years was 34.8 ± 18.0 and 17.7 ± 9.6%, respectively. The risk factors for PS were Taussig-Bing and arch anomalies. Coronary artery evaluation was performed in 95 (43.2%) patients with angiography, computed tomography or single-photon emission computed tomography, and five (5.3%) patients had abnormal coronary morphology or perfusion. Three patients underwent reoperation for coronary artery stenosis, and two had reversible perfusion defects in various regions, which were clinically not significant. Freedom from coronary events was 88.1 ± 6.4% at 22 years. A risk factor for coronary events was the single coronary artery. CONCLUSIONS The survival and functional outcomes of the ASO were excellent in the long-term. Strict serial surveillance is required to evaluate the long-term functional outcome of the ASO, particularly in a high-risk anatomy.

The long-term result of total repair for tetralogy of Fallot

OBJECTIVE The objective of this study was to evaluate the long-term outcome of total repair for tetralogy of Fallot. METHODS Between April 1986 and December 2007, a total of 734 patients underwent total repair for tetralogy of Fallot. There were 444 males and 290 females. The median age and weight were 17.2 months (0.4-329.6 months) and 9.5kg (2.6-53.5kg). The median follow-up duration was 150.2 months (1.9-356.2 months). RESULTS There were 27 early deaths (3.7%) and 13 late deaths. A longer cardiopulmonary bypass time and the use of total circulatory arrest were risk factors for early death. The overall survival rate was 94.8%, 92.8% and 92.8% at 10, 20 and 25 years, respectively. The presence of pulmonary atresia was a risk factor for long-term survival. Re-operation or re-intervention was required in 224 patients (31.7%). The most common causes of re-operation or re-intervention were pulmonary regurgitation in 109 patients and branch pulmonary artery stenosis in 127 patients. Freedom from re-operation or re-intervention rate was 81.5%, 68.9% and 46.6% at 5, 10 and 20 years, respectively. Reconstruction of the right ventricular outflow tract with other than non-trans-annular repair and branch pulmonary arterioplasty at the time of total repair were the risk factors for late re-operation or re-intervention. The use of a monocusp patch was not associated with early mortality or re-operation. At the latest follow-up, most patients were in the New York Heart Association functional class 1 or 2. CONCLUSIONS The long-term outcome of total repair for tetralogy of Fallot was satisfactory. A longer cardiopulmonary bypass time and the use of deep hypothermic circulatory arrest were associated with early mortality. The patients with pulmonary atresia have poorer late survival. Preservation of the pulmonary annulus can reduce the re-operation rate. A small pulmonary artery that requires augmentation may increase the risk of re-operation. The use of a monocusp in patients who underwent trans-annular repair has no benefit for early survival, the postoperative recovery and avoidance of re-operation. Age was not a risk factor for early mortality and re-operation.

Undifferentiated embryonal sarcoma of the liver: US and CT findings

Six cases of undifferentiated embryonal sarcoma (UES) were reviewed to determine their characteristic features on ultrasonography (US) (n=5) and computed tomography (CT) (n=6). US demonstrated a single large, echogenic mass with some anechoic spaces. Contrast-enhanced CT scan revealed a well-demarcated low-attenuation mass with hyperdense septations of variable shape and thickness. Discrepancy of internal architecture on US and CT was one of the important characteristics of UES. CT numbers were 25–47 HU in low-attenuation areas. Enhancing peripheral rim was found in four cases and some solid portions at the periphery or adjacent to the septa were found in all cases. Two patients who had follow-up US and CT without treatment showed enhancing solid portions, changing to hypodense as the tumor grew. When compared with the pathologic findings, US showed a more accurate representation of internal architecture than did CT. Familiarity with these US and CT findings of UES of the liver will be helpful in the differential diagnosis of primary hepatic tumors in childhood.

Sinus node dysfunction after Fontan modifications—influence of surgical method

BACKGROUND Sinus node dysfunction (SND) is reported to be a troublesome complication following various types of Fontan operations. The correlation of post-Fontan SND with surgical methods was evaluated in this study. METHODS By reviewing the medical records, surface ECGs, and Holter monitoring, the range of heart rate (HR) and the risk of SND at intermediate term after Fontan type operation (follow up: 41.3+/-13.1 months) were analyzed between two age matched groups of patients, consisting of the extracardiac conduit group (EC, n=33) and the lateral tunneling group (LT, n=35). RESULTS Junctional rhythm was observed in nine out of 35 patients in LT and five out of 33 patients in EC during the follow-up period. Resting HR was faster in EC than that in LT (108+/-15 vs. 82+/-21, P<0.001). Average and maximal HR in Holter monitoring were also faster in EC than those in LT. SND was found in 13 cases (10 in LT, three in EC) during follow-up and one required pacemaker implantation. In the case of situs solitus heart, SND was less frequent in EC than in LT (0/16 vs. 8/26, P=0.01). In the case of heterotaxy syndrome, SND occurred in similar number of cases (3/17 vs. 2/9). The staged approach to Fontan completion did not influence SND. LT repair was the only factor causing sinus node dysfunction according to multivariate logistic regression (P=0.03, OR 5.96). CONCLUSIONS Lateral tunnel type surgical repair was more likely to lead to the development of sinus node dysfunction than extracardiac conduit operation. In the case of heterotaxy syndrome, surgical method had no significant influence.

Twin AV Node and Induced Supraventricular Tachycardia in Fontan Palliation Patients

Introduction: The coexistence of two distinct atrioventricular (AV) nodes has been described in congenital heart disease requiring Fontan type palliation. The purpose of this study was to evaluate the occurrence of twin AV node according to anatomical subgroups, and to determine its relation to tachycardia.

Congenital intrahepatic portohepatic venous shunt: treatment with coil embolisation.

Abstract Congenital abnormalities of the portal venous system are rare. There are few radiological descriptions of intrahepatic portosystemic venous shunt detected in the perinatal period. We report a congenital portosystemic shunt that was detected by US and treated with coil embolisation in the neonatal period.

Mid-term results of bioprosthetic pulmonary valve replacement in pulmonary regurgitation after tetralogy of Fallot repair

OBJECTIVES Pulmonary valve replacement (PVR) is performed to reduce right ventricular (RV) volume overload, resulting in improved ventricular function and clinical status. Significant pulmonary regurgitation (PR) after tetralogy of Fallot (TOF) repair could result in RV dysfunction, exercise intolerance, arrhythmia and sudden death. The present study was conducted to investigate the mid-term clinical outcomes of PVR after TOF repair. METHODS Between 2001 and 2010, we retrospectively reviewed the outcomes of 131 (89 males and 42 females) PVRs with PR or pulmonary steno-insufficiency after TOF repair. PVR was performed at a mean age of 14.8±6.7 years. The mean interval from total correction of TOF to PVR was 12.5±5.2 years. Surgical indications of PVR were more than moderate PR with/without pulmonary stenosis, right ventricle dilatation, right ventricle dysfunction and reduced exercise capacity. Hancock II (n=58), Carpentier-Edwards Perimount (n=49) and St Jude Biocor (n=35) bioprosthetic valves were used. The mean z-score at implantation was 1.1±0.8. The mean valve size implanted was 25.1±1.5 mm. RESULTS There was no early or late mortality in this study. RV end-diastolic and end-systolic volume indices (from 111.3±34.7 to 64.6±23.6, P<0.01) (preoperative n=70, postoperative n=17) were markedly decreased PVR during the 13.2±16.1 months follow-up period. Eleven patients (male=10, female=1) required a repeat PVR operation due to prosthetic valve failure. The rate of freedom from reoperation at 10 years was 66.4±4.4%. Implanted valve type (Carpentier-Edwards bovine valve), young age, and large-sized valve implantation (z-score>2.0) were risk factors for a repeat PVR in the univariate analysis. There was no risk factor in the multivariable analysis. CONCLUSIONS PVR reduced the RV volume and improved the RV function within the first postoperative year. The rate of freedom from reoperation during the 10-year follow-up period in our series was acceptable. However, a longer follow-up will be necessary to determine the long-term outcomes of bioprosthetic valves in PVR.

Hydrothorax in a patient with Denys-Drash syndrome associated with a diaphragmatic defect

The Wilms tumor suppressor gene, WT1, plays an important role in the development of the urogenital system and the gonads, and clinical syndromes associated with WT1 mutations, such as WAGR syndrome, Denys-Drash syndrome and Frasier syndrome, typically manifest as renal and genitourinary abnormalities. WT1 may also play an important role in the development of the diaphragm, and recently several papers have reported an association between WT1 mutations and diaphragmatic hernias. In addition, WT1 mutations were also detected in some patients with Meacham syndrome, a rare malformation syndrome comprising congenital diaphragmatic hernia, double vagina, sex reversal, and cardiac malformations. Here, we report a case of an infant with typical clinical features of Deny-Drash syndrome and a heterozygous missense mutation, Arg366His, in the WT1 gene, in whom a diaphragm defect was detected after starting peritoneal dialysis. Diaphragmatic defects are rare but may be considered as clinical manifestations of WT1 mutation syndromes. In addition, we suggest that WT1 abnormalities should be suspected in patients with chronic renal failure who develop hydrothorax after peritoneal dialysis, especially in those with genitourinary abnormalities.