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Background: Right ventricular outflow tract obstruction relief is one of the major procedures during the total correction of tetralogy of Fallot (TOF). Pulmonary insufficiency (PI) is usually inevitable after a transannular incision with a patch repair is performed. Therefore, some surgeons advocate to place a monocusp valve within the transannular patch (TAP) in order to decrease the severity of the PI. However, the monocusp valve seemed not be very effective in some patients who underwent the complete TOF repair., Methods: Patients who had the classic form of TOF between January 2009 and January 2017 and underwent the corrective surgery with a TAP by the same cardiovascular surgeon were identified for further analysis. Clinical information including demographics at operation, perioperative data, and postoperative outcome were collected retrospectively and compared between the group with and without a monocusp valve., Results: A total of 24 TOF cases were included in the final analysis, and 16 (66.7%) patients received a monocusp valve placement. The patients' characteristics before and during the surgery were similar between the two groups. The median duration of chest tube drainage after the total correction in the monocusp group was longer than those without the valve (p = 0.04). There was no difference in the immediate postoperative data, including the inflammation/infection status, the duration of mechanical ventilation, and the length of ICU and hospital stay., Conclusion: Implantation of a monocusp valve during the total TOF correction using a TAP did not bring benefit to improve the immediate postoperative outcomes, especially the duration of the pleural drainage. Further study with a prospective design and a larger number of cases is needed., Competing Interests: Conflicts of interest: The authors declare that they have no conflicts of interest related to the subject matter or materials discussed in this article., (Copyright © 2021, the Chinese Medical Association.)

Background: Marfan syndrome is an inherited connective tissue disease that causes aortic root dilatation and dissection and requires surgical intervention. Apart from emergent surgery for aortic dissection or aortic aneurysmal rupture, prophylactic surgical intervention can also be administered, depending on the severity of aortic root dilatation. The direct relationship between surgical intervention and aortic regurgitation was seldom mentioned in previous studies., Methods: A retrospective cohort study was designed to determine the clinical presentations of prophylactic surgery in patients with Marfan syndrome. Between January 2009 and May 2019, 112 patients, adolescents and young adults, treated in the Department of Pediatric Cardiology of Taipei Veterans General Hospital, were enrolled. All patients' sex, body measurements, echocardiography reports, and surgical notes were collected for statistical analysis., Results: Among the participants, nine patients (8%) underwent the Bentall procedure, and the other 103 did not receive surgical intervention. The operation group had a larger aortic root size (4.89 vs 2.86 cm, p < 0.001), more dilated left ventricle (4.81 vs 4.1 cm, p = 0.002), and higher prevalence of moderate and severe aortic regurgitation (66% vs 1%, p < 0.001) than the nonoperation group., Conclusion: Among adolescents and young adults with Marfan syndrome, echocardiographic presentation of aortic root dilatation, left ventricular dilatation, and significant aortic regurgitation was significantly associated with prophylactic surgical intervention. According to the study, significant aortic regurgitation should also be considered as an important indication for prophylactic surgery., Competing Interests: Conflicts of interest: The authors declare that they have no conflicts of interest related to the subject matter or materials discussed in this article., (Copyright © 2021, the Chinese Medical Association.)

Background: Fabry disease is an X-linked inherited lysosomal storage disease that can be treated with the enzymes of agalsidase beta (Fabrazyme) and agalsidase alfa (Replagal). Since June 2009, viral contamination of Genzyme's production facility has resulted in a worldwide shortage of agalsidase beta, leading to the switch to agalsidase alfa for patients with Fabry disease in Taiwan., Methods: The medical records were retrospectively reviewed for nine male patients with Fabry disease from the start of agalsidase beta treatment until the switch to agalsidase alfa for at least 1 year., Results: After 12-112 months of enzyme replacement therapy (ERT), decreased plasma globotriaosylsphingosine (lyso-Gb3) was found in five out of seven patients, indicating improvement in disease severity. Among the six patients with available echocardiographic data at baseline and after ERT, all six experienced reductions of left ventricular mass index. Renal function, including microalbuminuria and estimated glomerular filtration rate, showed stability after ERT. Mainz Severity Score Index scores revealed that all nine patients remained stable at 12 months after switching to agalsidase alfa. ERT improved or stabilized cardiac status and stabilized renal function, while reducing plasma lyso-Gb3. ERT was well tolerated, even among the three patients who had hypersensitivity reactions., Conclusion: The switch of ERT from agalsidase beta to agalsidase alfa appears to be safe after 1 year of follow-up for Taiwanese patients with Fabry disease., (Copyright © 2013. Published by Elsevier B.V.)

Background: The objective of this retrospective cohort study was to compare the patent ductus arteriosus (PDA) closure rate with different routes (intravenous and enteral) of indomethacin treatment and neonatal outcomes., Methods: Infants with a birthweight < 1,000 g born between July 1997 and June 2007 at Taipei Veterans General Hospital and who received indomethacin treatment for PDA were included in the study. Outcome measures were ductal closure rate and neonatal outcomes., Results: Of 41 extremely-low-birth-weight infants with PDA, 3 infants had spontaneous closure and 3 died before treatment. Of the remaining 35 infants, 13 received enteral ethanol solution of indomethacin and 22 received the intravenous (IV) form. The total closure rates of the IV and enteral groups were 81.8% and 76.9%, respectively. There were no significant differences in the incidence of impaired renal function, necrotizing enterocolitis, chronic lung disease or severe retinopathy of prematurity between the 2 groups., Conclusion: Our results suggest that ethanol-based indomethacin is an effective alternative to IV indomethacin for the pharmacological closure of PDA in extremely-low-birth-weight infants.

Balloon valvuloplasty became the treatment of choice for valvular pulmonary stenosis following its first description in 1982 by Kan et al, and has almost replaced surgical pulmonary valvotomy in the present day. It is a safe and effective method for children for relief of right ventricular obstruction. The results of the procedure are excellent, without significant complications. This report describes the case of a 12-year-old boy who received successful balloon valvuloplasty for critical pulmonary valve stenosis complicated by an episode of acute pulmonary hemorrhage. Because of cyanosis, hypotension and bradycardia, he received emergent endotracheal intubation with 100% oxygen supplement and the highest infusion rate of inotropic agents. Venoarterial mode extracorporeal membrane oxygenation was indicated for life support due to the persistent high oxygenation index. Extracorporeal membrane oxygenation played a key role in the survival of this patient during the course of treatment.

Background: Transcatheter closure of a secundum atrial septal defect (ASD) has become an effective method for surgical treatment. In this study, we evaluated the feasibility and accuracy of intracardiac echocardiography (ICE) for sizing ASDs compared with conventional methods., Methods: Between January 2004 and December 2006, 270 patients underwent transcatheter closure of secundum ASD by using septal occluders. For 142 patients, the procedure was guided by transesophageal echocardiography (TEE), and for 128 patients by ICE. We compared the maximal diameters of the ASDs obtained during angiocardiography, transthoracic echocardiography (TTE), ICE, and TEE with balloon-stretched sizes ascertained by using a sizing plate., Results: ASD diameters measured with the sizing plate were significantly correlated with those measured with ICE (r = 0.963), TEE (r = 0.912), angiography (r = 0.88), and TTE (r = 0.85). The predicted stretched diameter of the ASDs, i.e. (nonstretched diameter measured with ICE x 1.07) + 3.23 mm, agreed well with that measured by using a sizing plate (Ri = 0.974)., Conclusion: ASD diameters measured with ICE correlated with sizing-plate measurements better than those determined with TEE, angiography or TTE.

Background: Kawasaki disease (KD) is an acute febrile vasculitis and may cause coronary artery abnormalities. Due to the higher incidence in Asian countries, most pediatricians in Taiwan are familiar with KD. However, there are still some patients being diagnosed 10 days after the onset of the illness and not receiving a highly effective therapy. In this study, we analyzed the risk factors and clinical manifestations of patients with a delayed diagnosis of KD., Methods: A retrospective review was made of the medical records of the patients diagnosed with KD at our institution between January 1996 and December 2005. The patients were divided into 2 groups: early-diagnosis group (EDG: diagnosis was made within 10 days after the onset of the fever) and delayed-diagnosis group (DDG: diagnosis was made 10 days after the onset of the fever)., Results: Fourteen of a total of 78 children (17.9%) were grouped into the DDG group, and 64 into the EDG group. There were no statistical differences between the 2 groups in terms of age, gender, number of antibiotics used, day of the first medical visit, total days of skin rash, conjunctivitis, mucosa changes, lymphadenopathy or laboratory examinations except for the higher white blood cell count and serum immunoglobulin G level in the DDG group. The patients in the EDG group had a clustered onset of symptoms as compared to the DDG group with a dispersed and late onset of symptoms. There was a higher risk of coronary artery abnormalities in the DDG group than the EDG group (42.9% vs. 14.1%; p = 0.036), and in the patients with KD who were younger than 1 year (29.0% vs. 12.7%; p = 0.043)., Conclusion: Patients with delayed diagnosis of KD were associated with higher risk of developing coronary arterial lesions. It is necessary to develop a diagnostic test for KD and provide more education to health care providers for early recognition of KD.

Background: In Taiwan, children with malignancies are treated under the protocols of the Taiwan Pediatric Oncology Group (TPOG). The purpose of this study was to determine the change in left ventricular (LV) function in pediatric patients undergoing chemotherapy., Methods: A total of 19 pediatric patients (mean age, 12.5 +/- 4.6 years; 11 males, 8 females) were enrolled. We divided the patients into 2 groups: (1) osteogenic sarcoma (OGS) group (n = 12; Group I); and (2) non-osteogenic sarcoma (non-OGS) group (n = 7; Group II). The accumulated dosages of anthracycline in Group I and II patients were 144.3 +/- 56.4 mg/M2 and 131.7 +/- 105.5 mg/M2 (p = 0.735), respectively. The children received echocardiography to investigate the parameters of LV systolic function, LV diastolic function, and myocardial performance index (MPI) after the entire chemotherapy course., Results: Higher E/A ratio (1.71 +/- 0.37), shorter isovolumic relaxation time (IRT, 42 +/- 19.14 ms), and shorter deceleration time (DT, 140.3 +/- 40.6 ms) were demonstrated in these patients. There was no statistically significant difference in the E/A ratio and DT between the 2 groups. Group I children were older (14.4 +/- 3.7 vs. 9.3 +/- 4.5 years; p = 0.015) and had lower MPI (0.20 +/- 0.02 vs. 0.28 +/- 0.07; p = 0.031) than Group II children., Conclusion: The children treated with chemotherapy using the TPOG protocol had a shorter IRT, higher E/A ratio and shorter DT. No significant evidence of anthracycline-related cardiotoxicity was found in any of the children in this study undergoing chemotherapy with the TPOG protocol.

Background: Hypoxic events and cardiac arrest may cause brain damage in critical infants. This study investigated cerebral tissue oxygenation and oxygen extraction in a piglet model of hypoxic events, cardiac arrest and effects of resuscitation., Methods: For the hypoxia experiment, anesthetized newborn piglets were randomized to a hypoxia group (n = 8) with decreasing ventilatory rate to 0, and a control group (n = 8) with no hypoxic conditions. Regional cerebral tissue oxygen saturation (rScO2, detected by near-infrared spectroscopy) and oxygen saturation were recorded every 5 minutes for 100 minutes. Fractional cerebral tissue oxygen extraction (FTOE) was calculated as (arterial oxygen saturation [SaO2] - rScO2)/SaO2. For the resuscitation experiment, animals were grouped as hypoxia-no CPR (n = 4), control-no CPR (n = 4), and control-CPR (n = 4) after cardiac arrest. Standard cardiopulmonary resuscitation (CPR) was performed on the control-CPR group and observed for 30 minutes., Results: Immediate and significant changes in rScO2, and gradual changes in FTOE were observed during the hypoxia experiment. In the resuscitation experiment, no significant differences in rScO2 were found between groups. However, the highest FTOE was observed in the control-CPR group., Conclusion: Noninvasive monitoring of rScO2 and evaluating FTOE changes during hypoxia and resuscitation may help clinicians evaluate brain tissue oxygenation and viability.

The incidence of multifocal atrial tachycardia (MAT) is very low and accounts for less than 1% of supraventricular tachycardia in infants and children. In this report, the clinical characteristics, medical treatment and outcome of MAT in 2 children are described. The first patient presented with tachycardia and respiratory failure since the day after birth. First, he received amiodarone, propranolol, and digoxin and then amiodarone alone. The heart rhythm converted to sinus rhythm 2 weeks after hospitalization. Although nonsustained MAT was occasionally observed when the infant suffered from pulmonary infection, the frequency of recurrent MAT decreased as the infant grew up. The second patient was a 5-year-old girl. She had congenital heart disease with double outlets of right ventricle (DORV), patent ductus arteriosus, coarctation of aorta, and ventricular and atrial septal defects. She underwent total correction at the age of 4 years. MAT was noted 3 months after the operation with the presentation of congestive heart failure. The heart rate slowed down and returned to normal sinus rhythm within several hours after amiodarone use. The symptoms and signs of congestive heart failure also disappeared. The patient took amiodarone regularly, and no tachycardia was detected during the follow-up period. MAT is considered to be a relatively benign arrhythmia with likely good outcome if there is no severe underlying illness. It can be well controlled under appropriate drugs, and a long period of follow-up is suggested. If pharmacologic intervention is required, we suggest that amiodarone may be an excellent choice.

We describe a little girl with tetralogy of Fallot (TOF) who was found to have a huge peripheral primitive neuroectodermal tumor (pPNET) when she developed progressive difficulty in standing and crawling at the age of 11 months. The tumor was located in the left paraspinal region (T4-T12), with intraspinal extension causing severe compression. Nine days after surgical decompression with laminectomy, chemotherapy was initiated with alternative courses of vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide every 3 weeks. The muscle power in her legs recovered substantially after 2 courses of chemotherapy. Although peripheral blood for cytogenetic study revealed no chromosome abnormality, recent cytogenetic analysis has revealed a high frequency of reciprocal translocation t(11;22)(q24;q12) detected in pPNET and a strong association between TOF and chromosome 22q11 microdeletion (del 22q11). Both genetic defects involve chromosome 22q in the close region. This case report illustrates the necessity of investigating for cytogenetic change in chromosome 22 and close follow-up due to the possibility of subsequent development of malignancies in patients with TOF.

Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and total correction is the definitive treatment. Chest tube drainage of pleural effusion (PE) is essential after surgery. Prolonged PE (> 7 days) is one of the complications; it may increase hospital stay and the risks of morbidity and mortality. The aim of this study was to investigate and analyze the possible risk factors for prolonged PE after total correction of TOF., Methods: Thirty-seven patients who received total correction of TOF between July 1999 and April 2001 were included in this study. They were divided into 2 groups according to the duration of chest tube drainage for postoperative PE: Group I had postoperative PE < or = 7 days; Group II had postoperative PE > 7 days. Detailed records were taken on patients' demographic characteristics, blood parameters, surgery, electrocardiographic and radiologic data, and angiographic and echocardiographic findings. The data of the 2 groups were compared using the Wilcoxon rank-sum test and Fisher's exact test. Risk factors were analyzed by logistic regression and model selection., Results: Of the 37 patients, 16 were male and 21 were female. There were 32 patients (86.5%) in Group I and 5 (13.5%) in Group II. Mean patient age at repair was 1.82 +/- 1.29 years (range, 0.53-3.11 years). Significant differences (p < 0.05) between the 2 groups were noted for gender, age at repair, body weight, presence of wound infection, duration on heart-lung machine (bypass time), oxygen saturation before surgery, duration of endotracheal intubation, length of hospital stay, and Nakata index. These risk factors were analyzed by logistic regression and model selection. Two models were set up: Model 1--oxygen saturation before surgery, presence of wound infection, age at repair; Model 2--oxygen saturation before surgery, presence of wound infection., Conclusion: Prolonged PE is a significant morbidity after TOF repair. The risk factors for prolonged PE are gender, age at repair, body weight, bypass time, low oxygen saturation before surgery, wound infection after surgery, duration of endotracheal intubation, length of hospital stay, and Nakata index. Oxygen saturation before surgery and wound infection were major risk factors while age at repair was a confounder.