• Clinical and Experimental Pediatrics
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• 제46권7호
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• pp.627-634
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• 2003

• Clinical and Experimental Pediatrics
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• 제63권5호
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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 Pediatrics
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• 제64권6호
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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 Emergency Medicine
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• 제5권4호
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• pp.286-287
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• 2018

• Clinical and Experimental Pediatrics
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• 제46권1호
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• pp.67-75
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• 2003

목 적 : 분지 폐동맥 협착 부위에 스텐트를 삽입하여 효과적으로 교정한 후, 이후 추적 관찰시에 협착이 없었던 반대편 분지 폐동맥 단면적이 감소하지 않고 증가하는 양상을 관찰하였다. 이에 분지 폐동맥 단면적의 변화에 영향을 끼치는 형태학적 혈역학적 요인들을 분석해 보았다. 방 법 : 1995년 1월부터 2002년 7월까지 연세대학교 심장혈관병원 소아심장과에서 좌폐동맥 분지 협착을 진단받고 스텐트 삽입을 시행받은 23명의 환아를 대상으로 하였다. 이들 환아에서 스텐트 삽입 전후로 심혈관 조영술을 통해 좌우 폐동맥 단면적의 변화, 도플러 심초음파 검사를 시행하여 폐동맥 부전을 측정하여 역류 분율의 변화를 관찰하였고, 폐관류 검사를 함께 시행하였다. 결 과 : 1) 좌폐동맥 협착으로 스텐트를 삽입한 후 좌폐동맥 지수는 $102{\pm}12mm^2/BSA$에서 $125{\pm}11mm^2/BSA$로 유의하게 증가하였다(P=0.001). 2) 협착이 없었던 우폐동맥 지수의 변화를 보면, 좌폐동맥에 스텐트를 삽입하기 전에 평균 $238{\pm}17mm^2/BSA$에서 추적 검사시 $249{\pm}20mm^2/BSA$로 유의한 증가는 없었다(P=0.474). 3) 스텐트 삽입 전후로 양측 폐동맥 지수의 합을 보면, 삽입전 $340{\pm}21mm^2/BSA$에서 추적 검사시 $374{\pm}26mm^2/BSA$로 증가하는 경향이 있었지만 유의한 차이는 없었다(P=0.09). 4) 폐동맥 폐쇄부전 역류 분율의 변화는 스텐트 삽입전 $50{\pm}5%$에서 $46{\pm}5%$로 감소하였으나, 유의한 감소량은 아니었다. 그러나, 폐동맥 폐쇄부전 역류 분율 증감 정도에 따라 세 군으로 나누어 비교해 보면, 3 군에서 우폐동맥지수는 삽입 전 $260{\pm}23mm^2/BSA$에서 추적 검사시 $325{\pm}33mm^2/BSA$으로 유의하게 증가하였다(P=0.041). 좌폐동맥 지수는 각 군에서 스텐트 삽입 전후로 증가하는 양상은 관찰되었으나, 1군에서만 유의하였고, 이는 폐동맥 폐쇄부전의 역류 분율 증감이 좌폐동맥의 단면적에 변화를 미치는 일차적 요인은 아닌 것으로 분석된다. 5) 스텐트 삽입 후 좌 우폐동맥 모두 폐동맥 폐쇄부전이 심할수록 폐동맥지수가 증가하여 유의한 양의 상관 관계를 가지고 (좌폐동맥; r=0.69, P<0.01 및 우폐동맥; r=0.53, P<0.01), 회귀분석상으로도 유의한 관계를 보였다. 또한 양측폐동맥 지수의 합도 유의한 양의 상관 관계(r=0.71, P<0.01)를 보였다. 결 론 : 협착이 있던 좌폐동맥은 스텐트의 삽입으로 교정되어 그 단면적 지수가 증가하였다. 그리고, 반대편 우폐동맥의 경우에는 잔존하는 폐동맥 폐쇄부전의 영향으로 단면적 지수가 감소하지 않고, 오히려 증가한 것을 관찰하였다. 그러나, 잔존하는 폐동맥 폐쇄부전의 장기적 예후를 고려해 볼 때, 이를 예방할 수 있는 것이 중요하다. 또한 역류 분율을 정확히 산출하기 위해 자기공명영상을 이용하는 것도 적극 검토해야 할 것이다.

• Clinical and Experimental Pediatrics
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• 제51권6호
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• pp.555-558
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• 2008

Committee for pediatric subspecialty board certification of the Korean Pediatric Society (KPA) was established for the subspecialty certification and formal training programs in 2005. Pediatric allergy and pulmonology was the first pediatric subspecialty among 9 subdivisions of KPA to petition for the certification in 2006, and 7 additional subdivisions of KPA, pediatric cardiology, pediatric endocrinology, pediatric gastroenterology and nutrition, pediatric infectious diseases, neonatology, pediatric nephrology, pediatric neurology, respectively, were followed in 2007. Finally, pediatric hemato-oncology joined this program in 2008. An overview and the future of Korean pediatric subspecialties are described.

• Korean Circulation Journal
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• 제54권2호
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• pp.91-92
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• 2024

• Clinical and Experimental Pediatrics
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• 제61권11호
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• pp.362-365
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• 2018

Purpose: This study aimed to analyze changes in QRS duration and cardiothoracic ratio (CTR) following pulmonary valve replacement (PVR) in patients with tetralogy of Fallot (TOF). Methods: Children and adolescents who had previously undergone total repair for TOF (n=67; median age, 16 years) who required elective PVR for pulmonary regurgitation and/or right ventricular out tract obstruction were included in this study. The QRS duration and CTR were measured pre- and postoperatively and postoperative changes were evaluated. Results: Following PVR, the CTR significantly decreased (pre-PVR $57.2%{\pm}6.2%$, post-PVR $53.8%{\pm}5.5%$, P=0.002). The postoperative QRS duration showed a tendency to decrease (pre-PVR $162.7{\pm}26.4$ msec, post-PVR $156.4{\pm}24.4$ msec, P=0.124). QRS duration was greater than 180 msec in 6 patients prior to PVR. Of these, 5 patients showed a decrease in QRS duration following PVR; QRS duration was less than 180 msec in 2 patients, and QRS duration remained greater than 180 msec in 3 patients, including 2 patients with diffuse postoperative right ventricular outflow tract hypokinesis. Six patients had coexisting arrhythmias before PVR; 2 patients, atrial tachycardia; 3 patients, premature ventricular contraction; and 1 patient, premature atrial contraction. None of the patients presented with arrhythmia following PVR. Conclusion: The CTR and QRS duration reduced following PVR. However, QRS duration may not decrease below 180 msec after PVR, particularly in patients with right ventricular outflow tract hypokinesis. The CTR and ECG may provide additional clinical information on changes in right ventricular volume and/or pressure in these patients.

• Clinical and Experimental Pediatrics
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• 제61권2호
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• pp.59-63
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• 2018

Purpose: Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non-ventilator-assisted groups. Methods: We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated. Results: Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non-ventilator-assisted. The patients in the home ventilator group were older ($16.25{\pm}1.85years$) than those in the nonventilator group ($14.73{\pm}1.36years$) (P=0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity ($1,038{\pm}620.41mL$) than the nonventilator group ($1,455{\pm}603.2mL$). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio ($1.7{\pm}0.44$) was lower in the home ventilator group than in the nonventilator group ($2.02{\pm}0.62$. The mitral valve annular systolic velocity was higher in the home ventilator group (estimated ${\beta}$, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions. Conclusion: Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.

• Clinical and Experimental Pediatrics
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• 제54권5호
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• pp.197-200
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• 2011

Respiratory syncytial virus (RSV) is a major cause of respiratory infection in children. Most of the pediatric population have RSV infection before the age of 2, and recurrent infections are common even within one season. Chronic lung disease, prematurity, along with congenital heart disease (CHD) are major risk factors in severe lower respiratory infection. In hemo-dynamically significant CHD patients with RSV infection, hospitalization is usually needed and the possibility of treatment in intensive care unit and the use of mechanical ventilator support are known to increase. Therefore the prevention of RSV infection in CHD patients is mandatory. The current standard for RSV prevention is immunoprophylaxis by palivizumab. Immunoprophylaxis is recommended monthly in hemodynamically significant CHD patients, up to 5 months. Motabizumab, a second generation drug and newly developing RSV vaccines are also expected to play a key role in RSV prevention in the future. The prophylaxis of RSV infection in CHD patients is cost-effective in both the medical aspect of the patients as well as the socio-economic aspect. Therefore an effort to promote prevention should be made by not only the family of the patients but also by the government.