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Objectives: This study aimed to identify clinical characteristics to differentiate multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) in Taiwan, an island with a delayed cluster of MIS-C and a high incidence of KD. Additionally, we studied risk factors for developing severe complications in patients with MIS-C., Methods: We conducted a retrospective, multicenter, cohort, and observational study that linked data on patients with MIS-C between May and December 2022 and patients with KD between 2019 and 2021 from 12 medical centers. Hemodynamic compromise, defined as the need for inotropic support or fluid challenge, was recorded in patients with MIS-C. We also evaluated maximal coronary Z-scores before treatment and one month after disease onset., Results: A total of 83 patients with MIS-C and 466 patients with KD were recruited. A 1:1 age and gender-matched comparison of 68 MIS-C and KD pairs showed that MIS-C patients had a lower percentage of positive BCG red halos, lower leukocyte/platelet counts, more gastrointestinal symptoms, and a higher risk of hemodynamic compromise. In Taiwan, 38.6% of MIS-C patients experienced hemodynamic compromise, with presence of conjunctivitis and elevated levels of procalcitonin (>1.62 ng/mL) identified as independent risk factors., Conclusion: We identified two independent risk factors associated with hemodynamic compromise in MIS-C patients. The comparison between matched MIS-C and KD patients highlighted significant differences in clinical presentations, like BCG red halos, which may aid in the differential diagnosis of the two disease entities, especially in regions with a high incidence rate of KD., Competing Interests: Declaration of competing interest None., (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background: Left ventricular outflow tract obstruction (LVOTO) re-intervention is a significant cause of morbidity and mortality in patients with coarctation of the aorta (CoA) or interrupted aortic arch (IAA) after aortoplasty., Methods: This retrospective study analyzed data from neonates with IAA/CoA who underwent biventricular repair between 2012 and 2022. LVOTO events were defined by the detection of color Doppler flow acceleration ≥3.0 m/s at the valvular, subvalvular, or supravalvular regions via transthoracic echocardiography, and the necessity for surgical or catheter intervention to relieve the obstruction., Results: Among 121 neonates with CoA/IAA, 16 (13.7%) primary aortoplasty patients developed LVOTO. Additionally, one patient (25%) who underwent a staged Yasui operation developed LVOTO due to a narrowed ventricular septal defect-pulmonary atresia tunnel. During follow-up, 58% of patients with a bicuspid valve and 25% of patients with a subaortic ridge developed LVOTO. The combination of either a bicuspid valve, subaortic ridge, or an aortic valve annulus Z-score < -3.0 predicted a high re-intervention rate (7/8 [87.5%])., Conclusions: In patients with IAA/CoA, the presence of multiple risk factors, including a bicuspid valve, subaortic ridge, and an aortic valve annulus Z-score < -3.0, is associated with a significantly increased rate of re-intervention for LVOTO., Competing Interests: Declaration of competing interest none., (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background: The incidence of atrial fibrillation/atrial flutter (AF/AFL) in general population is lower in Asia compared to Western countries. It is unclear whether a similar trend exists among adults with congenital heart disease (ACHD). We determine the profile, risk factors, and impact of AF/AFL in an Asian ACHD cohort., Methods: We included all ACHD patients diagnosed in an Asia tertiary care center between 2007 and 2018, analyzing AF (sustained and paroxysmal AF) and AFL, collectively./Purpose., Results: The study encompassed 4391 patients (55.9% women), with 81% having simple, 16.3% moderate and 2.8% severe CHD. AF/AFL was observed in 6.7% of the patients, with 54.6% having paroxysmal AF, 27.3% sustained AF, and 18.1% AFL. Incidence of AF/AFL increased with age and was higher in patients with pulmonary hypertension (PH), severe CHD, and metabolic syndrome. We found a progressive trend in the onset age of arrhythmia: AFL at a younger age, followed by paroxysmal and sustained AF. Risk factors for AF/AFL included severe and moderate CHD, PH, previous interventions, and male sex (odds ratio 11.2 and 3.15, 2.03, 1.75, and 1.71, respectively). When stratifying by CHD severity, PH and male sex were significant risk factors in simple CHD, while only PH in severe CHD. Patients with AF/AFL had a significantly lower major adverse cardiovascular events-free survival rate., Conclusions: This large ACHD cohort from Asia exhibited a high incidence of AF/AFL, similar to Western reports. The risk of AF/AFL was primarily associated with hemodynamic factors such as PH and CHD severity., Competing Interests: Declaration of competing interest None, (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background: Cardiovascular complications after Pfizer-BioNTech COVID-19 (BNT) vaccination are a concern, especially in adolescents. We analyzed the risk factors for myocarditis after BNT vaccination., Methods: We used a special evaluation protocol for all patients aged 12-18 years who presented to our emergency department with cardiovascular symptoms after BNT vaccination., Results: A total of 195 patients (109 boys and 86 girls) were enrolled. Eleven (5.6%) patients presented with arrhythmia (arrhythmia group), 14 (7.2%) had a diagnosis of pericarditis/myocarditis (the peri/myocarditis group), and the remaining 170 were controls (no cardiac involvement). Chest pain (77.6%) was the most common symptom. The median time from vaccination to symptom onset was 3 days. In the peri/myocarditis group (13 myocarditis and 1 pericarditis), the median time to the peak troponin T level was 5 days after vaccination. Abnormal electrocardiographic changes, including ST-T changes and conduction blocks, were more commonly detected in the peri/myocarditis group (85.7% vs. 12.4% in the control group, p < 0.01). Echocardiography revealed normal ventricular function in all patients. Symptoms were resolved before discharge in all, with the median duration of hospital stay being 4 days. The electrocardiography was the most appropriate screening tool for myocarditis, with a sensitivity and specificity of 85.7% and 87.6%, respectively., Conclusion: Pericarditis or myocarditis was diagnosed in 7.2% of adolescents presenting to the emergency department with cardiovascular symptoms after BNT vaccination. In addition to the troponin T level, ECG change listed above can be used as a screening tool for vaccine-induced cardiac complications., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2022 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background: There has been a remarkable increase in the number of pediatric ventricular assist device (VAD) implanted over the past decade. Asian pediatric heart centers had not participated in the multicenter registries among the Western countries. This article aimed to report the outcomes of pediatric VAD in our hospital., Methods: The study enrolled all patients aged <18 years at the time of VAD implantation in our institution between 2008 and 2021., Results: There were 33 patients with diagnosis of acute fulminant myocarditis (n = 9), congenital heart disease (n = 5), dilated cardiomyopathy (n = 16), and others. Paracorporeal continuous-flow pump was the most frequently implanted (n = 27). Most of the devices were implanted in patients with INTERMACS profile 1 (n = 24). The median duration on VAD was 22 days (range 2-254). The proportion of patients attaining positive outcomes (alive on device, bridge to transplantation or recovery) was 72.7% at 1 month, 67.7% at 3 months, and 67.7% at 6 months. Most of the deaths on device occurred within the first month post-implant (n = 9), with neurological complications being the most frequent cause of death. All recovered cases were successfully weaned off the device within the first month of implantation., Conclusion: We demonstrated a favorable outcome in pediatric patients supported with VAD at our institution., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2022 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background: A widely used method of treating left-sided arrhythmia substrates in children is retrograde transaortic ablation under fluoroscopic guidance. However, the feasibility, safety, and efficacy of this approach under zero fluoroscopy (ZF) guidance, especially the mid-term safety of anatomy and function of aortic valves, have yet to be proven., Methods: All consecutive patients who received ablation of left-sided arrhythmias between January 2012 and June 2020 and below 20 years-old were enrolled. The study group submitted to 55 ZF-guided procedures using cardiac mapping system (EnSite Precision), whereas 49 procedures were performed under fluoroscopic guidance in the control group. Echocardiographic studies took place before and 6-months after ablative procedures., Results: One-hundred-and-two patients (male, 66; female, 36) underwent a total of 104 ablative procedures. Mean procedural durations were 83.9 ± 44.4 min in the study group and 64.8 ± 29.1 min in the control group, respectively (p = .01; the 95% confidence interval, -33.57 to -4.63). Corresponding fluoroscopic times were .5 ± 2.2 min and 24.7 ± 13.9 min (p < .001; the 95% confidence interval, 20.15 to 28.22). ZF may be reasonably applied after a learning curve of 20 cases. Immediate procedural success and recurrence rates were similar in each groups. There was no detectable progression of aortic regurgitation in any of the patients during serial follow-up of echocardiography., Conclusion: ZF-guided retrograde transaortic ablation of left-sided arrhythmia substrates proved safe in children at midterm follow-up, reducing radiation exposure significantly within a learning curve of <20 cases., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2022 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center., Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed., Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era., Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2021. Published by Elsevier B.V.)

Background/purpose: Prognostic factors remain unclear in patients undergoing transcatheter implantation of Venus P-valve for their severe pulmonary regurgitation associated with native right ventricular (RV) outflow tract., Method: Between January 2017 and October 2018, we prospectively collected data of patient characteristics, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, and levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) before and 6-12 months after valve graft implantation., Results: Fifteen patients (male: 8, median age: 24.8 years) were enrolled. The procedure success rate was 100%. The median follow-up was 16.3 months without any dysfunction of the valve graft. The cohort demonstrated a significant improvement in cardiac index (from 3.3 to 3.9 L/min/m 2 ) and increase of percentage of New York Heart Association functional class I (P < 10 -3 ), reduction in RV end-diastolic volume index (P = 0.008), and reductions in NT-proBNP levels (from 78.9 to 45.8 pg/mL, P = 0.040). However, the peak oxygen consumption (VO 2 ) dropped from 50.2% to 48.5% of the predicted value. Interestingly, we determined that patients with NT-proBNP levels below 70 pg/mL and left ventricular end-diastolic pressure (LVEDP) below 11 mmHg had a significantly higher chance of exhibiting improvement in peak VO 2 compared with those without (3/4 vs 1/10, P = 0.041)., Conclusion: In the small cohort with severe pulmonary regurgitation, implantation of a Venus P-valve led to promising reductions in RV volume. However, no definite improvement in cardiopulmonary exercise capacity or RV ejection fraction was achieved. Levels of NT-proBNP and LVEDP may be helpful for refining the indications of the Venus P-valve implantation., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2020 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: Outlet-type VSD is frequently associated with aortic valve prolapse that surgery is frequently required. The literature regarding outcomes of transcatheter closure of outlet-type VSDs is scant. This study was conducted to know the safety and efficacy of transcatheter closure of outlet-type ventricular septal defects (VSDs) with Amplatzer Duct Occluder II (ADO II)., Methods: Medical records of patients underwent attempted transcatheter closure of outlet-type VSD with ADO II between October 2013 and August 2019 were retrospectively reviewed., Results: Among 49 patients, transcatheter closure was successful in 45 (91.8%; 33 males and 12 females; mean [± standard deviation] age and body weight: 15.8 (±17.7) years and 36.6 (±23.3) kg, respectively). The median VSD diameter was 4.0 mm (range: 1.2-6.0 mm). Device closure failed in four because the sheath could not be advanced through a prograde or retrograde route in one patient, occluder embolization in the two patients, and failed right ventricular disc anchoring in one patient. After a mean follow-up of 22.7 months (range: 0.3-51.1 months), only nine (20.0%) patients had increased severity in aortic regurgitation (AR) on the echocardiography. Preprocedural AR decreased in severity or even disappeared in 11 (24.4%) patients. No heart block or device failure occurred during follow-up. A trivial-to-small residual shunt was detected in 19 patients (42.2%) in the most recent echocardiography., Conclusion: Transcatheter closure of outlet-type VSDs with ADO II is feasible. Although no significant aggravation of AR was observed in the short-to-mid-term follow-up, long-term follow-up is mandatory., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest relevant to this article., (Copyright © 2020. Published by Elsevier B.V.)

Background: Intravascular leiomyomatosis (IVL) is relatively rare. The optimal surgical method and long-term outcomes are not completely understood., Methods: Medical records between 2007 and 2017 in our hospital were analyzed to identify IVL cases with surgical intervention. Their medical records, operative details, and follow-up were collected by chart review and telephone communication., Results: Eight patients with IVL were included in the study, accounting for 0.26% of all uterine leiomyoma cases. Primary IVL was confined to pelvic cavity in three patients, extended to the inferior vena cava (IVC) below renal vein in one, reached IVC and right atrium in three, and reached main pulmonary artery in one. One-stage operation was performed for seven patients. Cardiopulmonary bypass was done in four patients, and aortic cross-clamp and temporary circulatory arrest was performed in two patients. None of the four patients with intrapulmonary tumors received concomitant pulmonary tumor resection. There was no operative mortality and four morbidities, including ureter injury (2), bladder injury (1), and femoral vein thrombosis (1). During follow-up, two patients exhibited local recurrence of the tumor in the pelvis, and one patient had rapidly growing intrapulmonary tumor three months post-operatively. Intrapulmonary tumors in the other three patients remained stationary at 6, 84, and 120 months post-operatively., Conclusion: One-stage operation to completely remove IVL is feasible and with good long-term outcomes, which is recommended if the patient can tolerate the operation. Concomitant intrapulmonary tumors can be followed up watchfully except when associated with pleural effusion or the pathology indicating trend of increasing malignancy., (Copyright © 2018. Published by Elsevier B.V.)

Background/purpose: Kawasaki disease (KD) is the most common pediatric vasculitis. The study aimed to identify the risk factors of intravenous immunoglobulin (IVIG) unresponsiveness from the initial clinical parameters of the Taiwanese KD patients., Methods: We enrolled 248 KD (development dataset: 181, validation: 67) patients who received IVIG within 10 days after fever onset. IVIG unresponsiveness was defined by persistent fever beyond 24 hours after IVIG or recrudescent fever with KD symptoms., Results: From the development dataset (181 patients), IVIG unresponsiveness was noted in 22 patients (12.1%). The preIVIG levels of albumin, percentage of neutrophils, and positive lymphadenopathy were identified with highest risk for IVIG unresponsiveness. These three variables were used to construct a three-variable logistic regression model, which yielded an area under the receiver-operating-characteristics curve of 0.87. These three variables were further used to generate a composite scoring model (Formosa score) which yielded a sensitivity of 90.9% and specificity of 81.3% for a cut-off point of three or more. Validation in an independent cohort (67 KD patients) yielded sensitivity and specificity of 71.4% and 81.0%, respectively., Conclusion: We have established a simple three-variable Formosa score for KD patients to identify early those at risk of IVIG unresponsiveness for timely aggressive immunomodulation initially., (Copyright © 2015. Published by Elsevier B.V.)

Background/purpose: Endometrial hyperplasia is considered a precursor of endometrial carcinoma, but concurrent endometrial carcinoma in patients with endometrial hyperplasia is seen frequently. Our aim was to examine the risk factors for coexisting endometrial carcinoma in patients with endometrial hyperplasia., Methods: Between January 1996 and September 2006, 77 patients who underwent hysterectomy for endometrial hyperplasia were enrolled retrospectively. We divided the patients into non-endometrial carcinoma and endometrial carcinoma groups, depending on the final pathology of hysterectomy and analyzed the clinical variables of these patients., Results: The prevalence rate of concurrent endometrial carcinoma in patients with endometrial hyperplasia was 26%. Those with atypical endometrial hyperplasia had a higher rate of coexisting endometrial carcinoma (54%). In addition to cytologic atypia, body mass index (BMI) was another risk factor. All the patients with concomitant endometrial carcinoma had at least one risk factor, but almost 50% of the cases in the non-endometrial group had no risk factors. Half of the women with cytological atypia and BMI > 25 had coexisting endometrial carcinoma., Conclusion: When patients are diagnosed with endometrial hyperplasia, surgical intervention should be performed in those with cytological atypia and higher BMI because of the possibility of coexisting endometrial carcinoma.

Background/purpose: The optimal management of interrupted aortic arch (IAA) with ventricular septal defect is controversial. The aim of this study was to evaluate our 12 years of experience of surgical outcomes of one-stage and rapid two-stage total corrections of IAA with ventricular septal defect and to delineate the management of postoperative complications., Methods: We reviewed the medical charts of all patients from 1996 to 2007. Neonates with inherent complex anatomy were excluded. There were 26 patients in our series, with 11 type A and 15 type B IAA. Nineteen patients received one-stage repair and seven patients received rapid two-stage total correction. Rapid two-stage total correction was defined as two operations performed within 1 week., Results: The 1-month postoperative survival rate was 81% (21/26), with 79% (15/19) in the one-stage group, and 86% (6/7) in the rapid two-stage group. The rapid two-stage group had a shorter cardiopulmonary bypass time (160.1 +/- 58.4 vs. 216.8 +/- 73.7 minutes, p = 0.054) and aortic cross clamp (AXC) time (65.6 +/- 24.4 vs. 91.8 +/- 22.4 minutes, p = 0.022) than the one-stage group. Postoperative left ventricular outflow tract obstruction (LVOTO) and aortic arch restenosis were common in survivors, with frequencies of 48% (10/21) and 71% (15/21) respectively. Within the postoperative arch stenosis subgroup, nine out of 15 patients received balloon angioplasties, which proved effective after only one treatment. The overall late survival rate was 73% (19/26), with 68% (13/19) in the one-stage group, and 86% (6/7) in the rapid two-stage group., Conclusion: The outcome of rapid two-stage repair is comparable to that of one-stage repair. Rapid two-stage repair has the advantages of significantly shorter cardiopulmonary bypass duration and AXC time, and avoids deep hypothermic circulatory arrest. LVOTO remains an unresolved issue, and postoperative aortic arch restenosis can be dilated effectively by percutaneous balloon angioplasty.

The formation of pseudoaneurysms in patients with gynecologic malignancies is rare. We describe a patient with locally advanced cervical cancer who had life-threatening rectal bleeding due to a ruptured pseudoaneurysm of the right external iliac artery, which was successfully treated by emergent endovascular covered stent placement. This 56-year-old woman had received concurrent chemoradiation, salvage hysterectomy and systemic chemotherapy for advanced cervical cancer. About 25 months after her diagnosis of cervical cancer, she suffered from acute life-threatening rectal bleeding. Angiography revealed active extravasation from a pseudoaneurysm of the right external iliac artery. A covered stent was placed across the pseudoaneurysm via an endovascular approach to stop the bleeding. The patient recovered well without any sequelae. We believe that this technique might also be useful in other irradiated gynecologic cancer patients, especially when direct surgical repair is difficult to perform due to pelvic irradiation or tumor recurrence.

Extraperitoneal cesarean section was once used for the prevention of infection and postoperative adhesion. However, we report an unusual complication after this procedure. A 29-year-old woman had pus discharge from the anterior vaginal wall after extraperitoneal cesarean section. Broad-spectrum antibiotics failed to relieve her symptoms and vaginal culture yielded Morganella morganii . Magnetic resonance imaging, sagittal view, showed a cervical abscess measuring 5 x 5 cm with a tract extending to the anterior vagina. After performing dilation and abscess drainage via the cervical ostium, the symptoms gradually subsided with adequate antibiotic treatment. Cervical abscess may develop after extraperitoneal cesarean section and present initially as vaginal fistula. Detailed imaging study provides comprehensive anatomic information for effective management.

Coexistence of ovarian cancer and renal cell carcinoma (RCC) is extremely rare. Only one case was diagnosed in a total of 584 patients with RCC from 1982 to 2002 at our hospital. A 58-year-old woman presented with an enlarged girdle length for 3 months. Computed tomography scan showed a right cystic adnexal mass measuring 10 x 10 cm, and another tumor measuring 3 x 2 cm at the right kidney. She underwent debulking surgery and radical nephrectomy. Pathologic examination revealed right ovarian clear-cell carcinoma with peritoneal, omental, and fallopian tube metastasis, and conventional clear-cell renal carcinoma. RCC was strongly positive in epithelial membrane antigen (EMA) staining and negative in estrogen receptors (ER), progesterone receptors (PR), 34betaE12 (high molecular weight cytokeratin), and vimentin staining. Ovarian clear-cell carcinoma showed weakly positive results in EMA staining and negative results in ER, PR, 34betaE12, and vimentin staining. Although chemotherapy was given, the patient died of disseminated ovarian cancer metastasis 20 months after operation. In conclusion, coexistence of RCC and ovarian cancer is rare and the pathogenesis remains to be clarified.

Background/purpose: Aortopulmonary window (APW) is a rare congenital heart disease and surgical correction is advised as early as possible. However, few studies have focused on infants. This study sought to define the clinical features and outcomes in patients who underwent surgical repair of APW in early infancy., Methods: Between 1983 and 2004, there were 14 patients (0.15%) with APW out of 9414 patients with congenital heart disease. Ten underwent surgical repair of APW when they were younger than 4 months of age and constituted the study population., Results: There were four patients with type I APW, three with type II and three with type III. Concomitant cardiovascular anomalies were present in all patients, mainly aortic arch anomalies (80%, including right aortic arch, interrupted aortic arch [IAA] and severe coarctation of the aorta [CoA]). Patients were further grouped according to the presence (n = 5) or absence (n = 5) of IAA or severe CoA. All patients underwent surgical repair of APW with various techniques, including direct ligation, division and patch or flap closure. There was one early postoperative death. None of the survivors died during a median follow-up of 34 months (range, 8-116 months). Patients with IAA or severe CoA had longer intubation time, intensive care unit stay and hospital stay. Early reintervention was required only in patients with IAA or severe CoA (n = 3, 60%). Among them, two had significant residuals and both had type III APW., Conclusion: Long-term outcomes are generally good in patients with APW who have undergone surgical correction in early infancy. However, associated IAA or severe CoA may predict a prolonged hospital course and an increased risk of early reintervention. In patients with type III APW requiring early reintervention, significant hemodynamic residuals may be common even after reintervention.

Background and Purpose: Constrictive pericarditis (CP) is rare in children. The purpose of this study was to define the clinical manifestations and outcomes of this disease., Methods: From January 1985 through December 2003, 8 patients with surgically confirmed CP were enrolled. Data on clinical characteristics, disease manifestations, treatment, and outcomes were collected and analyzed., Results: One patient had l-transposition of the great arteries (l-TGA); the other 7 patients had no underlying cardiovascular diseases. None of the patients had direct evidence of tuberculous infection. Age at disease onset ranged from 8 months to 13 years (median, 10 years), and the interval between symptom onset and correct diagnosis ranged from 2 to 31 months (median, 12 months). Major symptoms and signs included abdominal distension, hepatomegaly, neck vein engorgement and ascites. Evidence of calcification on chest roentgenogram was found in 1 patient (12.5%). Computed tomography or magnetic resonance image studies showed pericardial thickening in 6 patients (75%). Seven patients underwent cardiac catheterization, and all hemodynamic data met the diagnostic criteria for CP. All patients with structurally normal hearts survived during a median follow-up of 31 months after pericardiectomy (range, 5 to 103 months). The patient with l-TGA died of pneumonia 1 year after pericardiectomy., Conclusions: Symptoms and signs of right heart failure were common in children with CP. The etiology of CP remains largely unknown. Patients with structurally normal hearts have good outcomes after pericardiectomy.

Cervical cancer is a serious health problem in Taiwan, with nearly 2700 women developing the disease each year. The estimated incidence of this disease from 1993 to 1997 was 21.8 per 100,000 woman-years. The lack of effective Papanicolaou smear screening systems was the major cause of the high prevalence rate before 1995. Surgery and radiotherapy are the standard treatments for early stage cervical cancer. For women with advanced stage cervical cancer, external-beam pelvic radiation followed by intracavitary radiotherapy is the treatment of choice. Although randomized clinical trials have shown that concurrent chemoradiotherapy should be regarded as the standard treatment for locally advanced cervical cancer (stage IIB to IVA), the impact of the complications associated with this treatment have not been evaluated in Taiwanese studies. Localized bulky (stage IB2 or bulky IIA) tumors are commonly treated with various combinations of chemotherapy, surgery and radiotherapy, despite unresolved concerns about the morbidity and effectiveness of this approach compared with definitive radiotherapy or radical surgery. Although about 60% of Taiwanese women with cervical cancer receive primary surgical treatment, about 20% do not receive treatment or receive non-standard therapies. Efforts are needed to increase the screening rate, to improve access to medical care, and to provide public education for patients to reduce the occurrence and mortality of cervical cancer in Taiwan.

Although enteroviruses can cause overwhelming and fatal systemic infections in neonates, such severe neonatal infections remain uncommon and rarely involve both of twin neonates at the same time. We report the cases of twin neonates who developed fever initially, and then progressed to disseminated systemic disease with marked thrombocytopenia, coagulopathy, and hepatic failure. One of the neonates died and the other survived. Both neonates were treated with intravenous immunoglobulin and maternal fresh frozen plasma was also given to the neonate who survived. Virus cultures from the nasopharynx, rectum and cerebral spinal fluid of both neonates yielded enterovirus, later typed as echovirus 30. The surviving neonate had normal development without obvious sequelae during a follow-up period of 1 year. The major determinant of the survival from severe neonatal enterovirus infection might have been the pre-existing severity of the disease before treatment, and complete recovery could be expected if the infant survived the acute stage of illness.

Background and Purpose: Early-stage bulky cervical carcinoma treated with conventional surgery or radiotherapy has a higher rate of recurrence compared to smaller tumors at the same stage. Whether neoadjuvant chemotherapy prior to radical hysterectomy can improve survival in early-stage bulky cervical carcinoma remains unclear. This study was designed to answer this question., Methods: Fifty-eight women with early-stage bulky cervical cancer were included in this retrospective study. Thirty-one had received neoadjuvant chemotherapy before radical hysterectomy, and the other 27 patients underwent surgery alone. The chemotherapeutic regimen was a combination of cisplatin, vincristine, and bleomycin with a 10-day interval for two to three courses., Results: The age, parity, and tumor diameter before treatment in the two groups were similar. The mean tumor diameter was significantly decreased after neoadjuvant chemotherapy (4.6 +/- 0.8 vs 3.4 +/- 1.5 cm, P = 0.003). Patients without neoadjuvant chemotherapy had a significantly higher incidence of parametrial invasion (14/27 vs 7/31, P = 0.022). More involved lymph nodes were found during surgery in patients without neoadjuvant chemotherapy (23.1 +/- 10.5 vs 17.4 +/- 7.1, P = 0.024), but the incidence of lymph node metastasis was not different between the two groups (18/31 vs 17/27, P = 0.71). The response rate of primary tumor to chemotherapy was 48.4% (15/31). No significant differences in clinical and pathologic parameters were found between responders and non-responders. Deep stromal invasion (> or = 3/4 thickness of cervical stroma) was the only independent prognostic factor for disease-free survival (DFS) and overall survival (OS) in the 58 patients and in the 31 patients who received neoadjuvant chemotherapy. Neither neoadjuvant chemotherapy nor the response to it was an independent prognostic factor for DFS or OS., Conclusions: Neoadjuvant chemotherapy could reduce the incidence of local invasion for bulky early-stage cervical carcinoma but did not improve the DFS or OS in our patients. Without further randomized study of the effects of neoadjuvant chemotherapy, this treatment should be chosen carefully.

Adnexal torsion is an unusual, but serious complication in pregnancy. The treatment is surgical, but this may increase the risk of pregnancy loss in the first trimester. The use of laparoscopic surgery, which is less invasive than traditional laparotomy, has been limited by diagnostic and technical difficulties including determination of ovarian tumor nature and spillage of cyst contents intraoperatively. A 25-year-old woman in her 11th week of pregnancy had acute severe left lower-abdominal pain, which was diagnosed as left ovarian teratoma with torsion. She underwent emergency laparoscopic surgery with unwinding of the twisted fallopian tube and ovary and cystectomy of the teratoma. The patient subsequently delivered a full-term baby, without complications. Accurate ultrasound and cytologic diagnoses along with copious intraoperative warm, normal saline irrigation were likely contributing factors to the successful outcome of this case.

Serum neuron-specific enolase (NSE) is a tumor marker for neuroendocrine tumors or tumors of neuroepithelial origin. While the clinical application of NSE in small cell lung cancer has been established, its role in small cell carcinoma of the uterine cervix is unknown. We examined serum NSE in six patients with cervical small cell carcinoma and 13 patients with cervical squamous cell carcinoma. Elevated serum NSE was noted in four of six patients (67%) with small cell carcinoma, but none of the 13 patients with squamous cell carcinoma. An extremely high NSE serum level (154.7 ng/mL) was found in one patient with recurrence of small cell carcinoma, suggesting its correlation with disease extension. Although further studies to evaluate its relevance to prognosis, treatment effect, and early detection of recurrence are in progress, this preliminary result seems promising.

Forty-two patients with secondary ovarian cancer from extragenital sites encountered in the past 14 years at the National Taiwan University Hospital were retrospectively analyzed. They accounted for 11.6% (42/362) of all ovarian cancers. The mean age was 43.7 years, and 32 patients were premenopausal. Gastric cancer was the most frequent primary malignancy (50.0%); other sites of extragenital primaries were colon (23.8%), breast (11.9%), rectum (4.8%), and indeterminate (9.5%). Bilateral ovarian involvement was found in 78.6% of patients. The most common presenting symptom was abdominal distension (52%). All patients received surgical intervention as the primary treatment; 30 patients had subsequent chemotherapy and four patients had postoperative radiotherapy. However, the outcome was poor; more than half of the patients died within one year, with a two-year survival rate of 22.5%.

Malignant mixed Müllerian tumor (MMMT) of the fallopian tube is an extremely rare neoplasm with a poor prognosis. To date, only 37 cases have been reported. The primary treatment for this tumor is surgery. Chemotherapy has been used with some success. The role of radiotherapy is less clear. A case of malignant mixed Müllerian tumor of the fallopian tube occurring in a 63- year- old woman, presenting with postmenopausal vaginal spotting for 7 months, is reported.

The serum copper (SCL) and zinc (SZL) levels were measured in 99 patients with cervical cancer and 50 patients with uterine myoma as controls. The mean SCL in the control group was 109.4 +/- 17.4 micrograms/ml as compared to 117.1 +/- 14.6 micrograms/dl and was not significant (NS) in 17 carcinoma in situ (CIS) patients, 142.3 +/- 14.2 micrograms/dl in 30 stage I patients (p less than 0.001), 159.0 +/- 16.6 micrograms/dl in 22 stage II patients (p less than 0.001), 171.6 +/- 25.7 micrograms/dl in 10 stage III or IV patients (p less than 0.001), and 166.2 +/- 32.2 micrograms/dl in 20 recurrent patients (p less than 0.001). The SCL returned to control level 2 weeks after surgical treatment for the stage I and II patients (mean 110.6 +/- 19.6 and 108.7 +/- 20.4 micrograms/dl, respectively, p less than 0.001). The SZL was 97.2 +/- 15.8 micrograms/dl in control patients and only showed a significant decrease in stage III or IV and recurrent patients (67.2 +/- 16.6 and 70.4 +/- 17.2 micrograms/dl, respectively). Concerning the copper/zinc ratio, the control group was 1.13 +/- 0.07 as compared to 1.17 +/- 0.07 in CIS (p = 0.06), 1.51 +/- 0.24 in stage I (p less than 0.001), 1.85 +/- 0.37 in stage II (p less than 0.001), 2.66 +/- 0.61 in stage III or IV (p less than 0.001), and 2.50 +/- 0.75 in recurrent patients (p less than 0.001). Taking mean +/- 2.5 SD of the control values as cut off points, the percentages of the recurrent patients with abnormal SCL, SZL, and a Cu/Zn ratio were 65, 30 and 90%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

The cell-mediated immunity in 19 patients with stage I and II cervical carcinoma was evaluated before and after postoperative PS-K immunotherapy. Lymphocyte subpopulations were enumerated using specific monoclonal antibodies (moAbs) of the Leu series (Leu2, Leullb, Leu15, and Leu19) and the OKT series (OKT3, and OKT4). A 4-hour 51Cr-release assay was used to measure natural killer (NK) cell activity. Serum levels of interleukin-2 (IL-2), soluble interleukin-2 receptor (IL-2R), and tumor necrosis factor (TNF) were also determined before and after postoperative PS-K immunotherapy. The results showed that the number of cells carrying the marker of a non-major histocompatibility complex (MHC), restricted cytotoxic lymphocytes in the peripheral blood and NK activity at three different effector/target ratios increased significantly after postoperative PS-K immunotherapy. It is therefore concluded that postoperative PS-K immunotherapy enhanced the NK activity in patients with cervical carcinoma, in which NK activity tended to be lower. Further clinical investigation to assess the effectiveness of PS-K immunotherapy in improving the overall survival is in progress.