• Journal of Chest Surgery
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• v.53 no.2
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• pp.79-81
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• 2020

Treatment options for children with end-stage heart failure are limited. We report the first case of a successful pediatric heart transplantation bridged with a durable left ventricular assist device in Korea. A 10-month-old female infant with dilated cardiomyopathy and left ventricular non-compaction was listed for heart transplantation. During the waiting period, the patient's status deteriorated. Therefore, we decided to provide support with a durable left ventricular assist device as a bridge to transplantation. The patient was successfully bridged to heart transplantation with effective support and without any major adverse events.

• Journal of Chest Surgery
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• v.47 no.3
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• pp.211-219
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• 2014

Background: Our objectives were to review our institutional early and midterm experience with primary tetralogy of Fallot (TOF) repair, and identify predictors of intensive care unit (ICU) morbidity. Methods: We analyzed perioperative and midterm follow-up data for all cases of primary TOF repair from 2001 to 2012. The primary endpoint was early mortality and morbidity, and the secondary endpoint was survival and functional status at follow-up. Results: Ninety-seven patients underwent primary repair. The median age was 4.9 months (range, 1 to 9 months), and the median weight was 5.3 kg (range, 3.1 to 9.8 kg). There was no early surgical mortality. The incidence of junctional ectopic tachycardia and persistent complete heart block was 2% and 1%, respectively. The median length of ICU stay was 6 days (range, 2 to 21 days), and the median duration of mechanical ventilation was 19 hours (range, 0 to 136 hours). By multiple regression analysis, age and weight were independent predictors of the length of ICU stay, while the surgical era was an independent predictor of the duration of mechanical ventilation. At the 8-year follow-up, freedom from death and re-intervention was 97% and 90%, respectively. Conclusion: Primary TOF repair is a safe procedure with low mortality and morbidity in a medium-sized program with outcomes comparable to national standards. Age and weight at the time of surgery remain significant predictors of morbidity.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.919-924
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• 2007

Pulmonary venous stenosis may be congenital or acquired. Regardless of its origin, the prognosis for patients affected with PVS remains poor. There have been many attempts to palliate PVS with little success. This report describes two patients with PVS which became evident after repair of total anomalous pulmonary venous connection. Intravascular stents were successfully implanted, but progressive restenoses in the stents occurred and eventually both of the patients died. The pertinent literature is reviewed.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.158-164
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• 2005

Purpose : We reviewed the therapeutic results of various Duct-Occlud coils(pfm AG, $K{\ddot{o}}ln$, Germany) to evaluate the efficacy of the most-recently modified Duct-Occlud coil(Nit-Occlud) in the transcatheter closure of patent ductus arteriosus(PDA), including large defects more than 4 mm in diameter. Methods : Two hundred and five patients who underwent percutaneous PDA occlusion using Duct-Occlud devices from March 1996 to December 2003 were enrolled and four types of Duct-Occlud [Standard(S), Reinforced(R), Reinforced reverse cone(RR) and Nit-Occlud(N)] were used in this study. The patients were followed up by echocardiogram and physical examination before discharge, one month, six months and 12 months after the procedure. Results : The rate of residual shunt according to the type of Duct-Occlud were as follows : S-54%, R-72%, RR-50%, N-14%(P<0.05 compared with other devices) at one month, S-25%, R-44%, RR-37%, N-0%(P<0.05 compared with other devices) at six months, S-8%, R-8%, RR-4%, N-0%(P<0.05 compared with S and R) at 12 months and later. Nit-Occlud coil showed the complete occlusion of PDA after six months of follow-up, even in 12 patients with relatively large PDA(>4 mm). Conclusion : The transcatheter closure of PDA using Duct-Occlud was an effective treatment and our study revealed that a Nit-Occlud coil which showed higher rate of occlusion even in PDA with large diameters over than 4 mm, was a more effective modality compared to previous devices.

• Journal of Chest Surgery
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• v.48 no.4
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• pp.272-276
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• 2015

Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.

• Clinical and Experimental Pediatrics
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• v.46 no.7
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• pp.627-634
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• 2003

• Clinical and Experimental Pediatrics
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• v.64 no.6
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• pp.269-279
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• 2021

Clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9 (CRISPR-Cas9) is an ancient prokaryotic defense system that precisely cuts foreign genomic DNA under the control of a small number of guide RNAs. The CRISPR-Cas9 system facilitates efficient double-stranded DNA cleavage that has been recently adopted for genome editing to create or correct inherited genetic mutations causing disease. Congenital heart disease (CHD) is generally caused by genetic mutations such as base substitutions, deletions, and insertions, which result in diverse developmental defects and remains a leading cause of birth defects. Pediatric CHD patients exhibit a spectrum of cardiac abnormalities such as septal defects, valvular defects, and abnormal chamber development. CHD onset occurs during the prenatal period and often results in early lethality during childhood. Because CRISPR-Cas9-based genome editing technology has gained considerable attention for its potential to prevent and treat diseases, we will review the CRISPR-Cas9 system as a genome editing tool and focus on its therapeutic application for CHD.

• Clinical and Experimental Pediatrics
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• v.63 no.5
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• pp.189-194
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• 2020

Background: The decision to use transannular patching (TAP) during tetralogy of Fallot (TOF) repair depends on the pulmonary valve annulus size; the z score of the pulmonary annulus is the most commonly used predictor. However, definitive results are not obtained with z scores as different z score data sets are used for different parameters. Purpose: This study aimed to identify the echocardiographic and other key factors that warranted a change in the surgical method during TOF surgery. Methods: Sixty-two patients were enrolled and divided into a pulmonary valve (PV) preservation group and a TAP group. Their medical records were reviewed. Results: The z score for PV annulus (PVA), ratio of the PVA to aortic annulus size, and ratio of PVA to descending aorta (DAO) size were significantly different between the PV preservation and TAP groups (-1.72±1.52 vs. -3.07±1.94, P=0.004; 0.62±0.12 vs. 0.50±0.14, P=0.002; and 1.32±0.32 vs. 1.07±0.36, P=0.008, respectively). For TAP repair, the PVA z score had a sensitivity of 65.4% and specificity of 73.1%, ratio of PVA to aortic annulus size had a sensitivity of 73.1% and specificity of 65.4%, and ratio of PVA to DAO size had a sensitivity of 69.2% and specificity of 57.7%. The TAP group showed more monocuspid PVs (P=0.011), while the PV preservation group showed more tricuspid PVs (P=0.027). Commissurotomy was more frequently performed in the PV preservation group than in the TAP group (P=0.001). Of patients with commissurotomy, 58% showed a PV z score<-2. Conclusion: Although various echocardiographic parameters may serve as predictors for determining surgical methods for TOF patients, the PV morphology and tissue characteristics should also be considered.

• Clinical and Experimental Emergency Medicine
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• v.5 no.4
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• pp.286-287
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• 2018

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.67-75
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• 2003

Purpose : Intravascular stent implantation for the treatment of postoperative branch pulmonary artery(PA) stenosis has been used successfully. However, the cross sectional area of contralateral branch PA does not regress in spite of the successful dilation of the stenotic branch PA after stent implantation. We analyzed the morphologic and hemodynamic factors on the size of branch PA after successful stent implantation. Methods : The subjects in our study were 23 children who had undergone stent implantaion from Jan. 1995 to Jul. 2002 in the Division of Yonsei Pediatric Cardiology. We evaluated the cross sectional area index(CSAI) of branch PA before and after stent implantation at follow-up catheterization. We also investigated factors such as residual pulmonary stenosis, pulmonary regurgitation(PR), systolic pressure of right ventricle, and lung perfusion scan. Results : The CSAI of the RPA without stenosis changed from mean $238{\pm}17mm^2/BSA$ to mean $249{\pm}20mm^2/BSA$(P=0.47), but didn't regress. The CSAI of the LPA with stenosis was increased effectively by stent implantation from the mean $102{\pm}12mm^2/BSA$ to mean $125{\pm}11mm^2/BSA$(P< 0.05). At follow up after stent implantation, the CSAI of PA is correlated with the residual PR fraction after stent implantation. Conclusion : In a group with increased residual PR, CSAI of RPA was found to be significantly increased between the pre- and post-stages of stent implantation. So, we suspect that the pulmonary regurgitation remaining after right ventricle outlet tract(RVOT) dilatation surgery is correlated with the increase in CSAI of RPA.