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Background: To evaluate the association between the pulmonary vein (PV) entry site morphology after total anomalous pulmonary vein repair (TAPVC) and postoperative pulmonary vein stenosis (PVS)., Methods: Computed tomography (CT) examination was performed to determine the PV entry site morphology. The width of the PV confluence was divided by the width of the left atrium (LA) to obtain the cPV/LA index. The cPV/LA index was compared between patients with and without postoperative PVS., Results: Fifty-one patients who had undergone CT after TAPVC repair were included, with a median cPV/LA index of 0.5 (interquartile range (IQR) = 0.349-0.654). Among them, 27 patients developed postoperative PVS. The median cPV/LA index after primary TAPVC repair was significantly lower in patients with PVS compared to those without PVS (0.367, IQR = 0.308-0.433 vs. 0.657, IQR = 0.571-0.783, P < 0.0001). Additionally, the cPV/LA index after surgical re-intervention for PVS was significantly smaller in patients who developed recurrent stenosis compared to those who remained free-from re-stenosis after surgical relief (0.459, IQR = 0.349-0.556; vs. 0.706, IQR = 0.628-0.810, P = 0.0045)., Conclusion: A small PV confluence width is associated with the development of postoperative PVS and recurrent stenosis after surgical relief of PVS. Our results suggest that adequate bilateral pulmonary vein lateralization during TAPVC surgery is crucial., Competing Interests: Declaration of competing interest All the authors reported no conflicts of interest., (Copyright © 2024 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

Background/purpose: Estrogen in hormone replacement therapy causes homeostatic changes. However, little is known regarding the safety of high-dose phytoestrogen on coagulation and hematological parameters in healthy postmenopausal women. This study evaluated the effects of high-dose soy isoflavone (300 mg/day) on blood pressure, hematological parameters, and coagulation functions including circulating microparticles in healthy postmenopausal women., Methods: The original study is a 2-year prospective, double-blind, placebo-controlled study. In total, 431 postmenopausal women (from 3 medical centers) were randomly assigned to receive either high-dose isoflavone or placebo for 2 years. At baseline, 6 months, 1 year, and 2 years after treatment, blood pressure, body weight, liver function tests, hematological parameters, and lipid profiles were measured. The 1(st) year blood specimens of 85 cases of 144 eligible participants (from one of the three centers) were analyzed as D-dimer, von Willebrand factor antigen, factor VII, plasminogen activator inhibitor type 1, and circulating cellular microparticles, including the measurement of monocyte, platelet, and endothelial microparticles., Results: In the isoflavone group, after 1 year, the changes in liver function tests, hematological parameters, and coagulation tests were not different from those of the control. Triglyceride levels were significantly lower after 6 months of isoflavone treatment than the placebo group, but the difference did not persist after 1 year. Endothelial microparticles increased steadily in both groups during the 1-year period but the trend was not affected by treatment., Conclusion: The results of the present study indicate that high-dose isoflavone treatment (300 mg/day) does not cause hematological abnormalities or activate coagulation factors., (Copyright © 2013. Published by Elsevier B.V.)

Background/purpose: Frailty is the core of geriatric syndromes in the elderly. However, there is no solid prevalence data in Taiwan even with the rapid growth of the elderly population. The aim of this study was to explore the prevalence of frailty defined by different instruments and to identify the factors associated with frailty in a northern Taiwan community., Methods: The 65-79-year old community-dwelling residents randomly selected from Toufen were first screened with a telephone version of the Chinese Canadian Study of Health and Aging Clinical Frailty Scale (CCSHA-CFS; level 1-7). Those who scored 3-6 with this instrument were evaluated at a local hospital with the Fried Frailty Index (FFI) and the Edmonton Frail Scale (EFS). Other baseline characteristics including health and functional performance were also evaluated., Results: Among the 2900 population representative samples, 845 (29.1%) completed the CCSHA-CFS telephone interview with the prevalence of frailty approximately 11.0% [95% confidence interval (CI) 8.9-13.1]. Among the 275 who completed assessments with FFI and EFS, prevalence of frailty was 11.3% (95% CI = 7.6-15.0) by FFI and 14.9% (95% CI = 10.7-19.1) by EFS. About 57.5% of respondents had memory impairment, 29.8% experienced pain, 25.1% experienced falls, 16.7% had depression, 14.5% had urinary incontinence, and 5.8% had polypharmacy. Being older, having more complaints with falls, pain, dysphagia, polypharmacy, depression, comorbidity, longer time for the Timed Up and Go test, less education, lower Mini-Mental State Examination score, and lower Barthel Index were associated with frailer status. In multinomial logistic regression analysis, increasing age, less education status, lower Barthel Index score and depression were positively associated with physical frailty., Conclusion: In this study, the prevalence of frailty was from 11.0% to 14.9% by different criteria and methodology. Various correlates were independently associated with frailty status. It is suggested that intervention for frailty requires an interdisciplinary approach., (Copyright © 2011 Formosan Medical Association & Elsevier. Published by Elsevier B.V. All rights reserved.)

This study reported our experience of bedside patent ductus arteriosus (PDA) ligation for prematurity in the neonatal intensive care unit (NICU). Between April 1992 and March 2006, 41 very-low-birth-weight premature infants underwent PDA ligation in the NICU. There were 18 male and 23 female infants. The mean gestational age and birth weight were 26.9 weeks and 900.9 g, respectively. Preoperatively, 25 infants were ventilator-dependent. After operation, there were five deaths caused by complications of prematurity. Surgical complications occurred in four and all recovered well after treatment. Twenty preoperatively intubated babies survived and were extubated at 21.6 +/- 12.7 days postoperatively. In conclusion, bedside PDA ligation in the NICU is safe and effective. It can avoid transportation of critically ill, very small infants. We suggest surgical closure as the primary treatment in very-low-birth-weight infants who are ventilator-dependent to avoid the possible complications of indomethacin and prolonged intubation.

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.

Background/purpose: To evaluate the value of 3D reconstructed computed tomography (CT) imaging for patients with double outlet right ventricle (DORV)., Methods: CT images were obtained preoperatively from 17 patients who ranged in age from 5 days to 5 years. Reconstructed 3D images were created using gradient-shading surface rendering, which allowed partial subtraction of the anterior sections of the virtual heart to view the interior. Interpretations of CT, echocardiography and cine-cardioangiography were compared and verified from surgical findings, autopsy, and consensus upon review of all imaging and diagnostic tests., Results: Three subaortic, seven subpulmonary, six non-committed, and one double-committed subtypes of ventricular septal defect (VSD) were observed. The 3D electron beam CT images provided good delineation of the spatial relationship inside the heart. The range of diagnostic accuracy for all VSD types in DORV was 88-100% for 3D CT, 71-94% for echocardiography, and 60-100% for cine-cardioangiography. In comparison, 3D CT offered better diagnostic accuracy for all variants of DORV., Conclusion: 3D constructed CT imaging is a good modality for differentiating VSD type in DORV. It allowed us to directly evaluate the inside of cardiac chambers for the right ventricular outlet, great arterial root, and determine the VSD relationships.

Intracardiac repair for complex congenital heart defects in premature neonates with very low birth weight (VLBW) is still a challenge to pediatric cardiac surgeons. We report the successful rerouting of cardiac type total anomalous pulmonary venous return (TAPVR) in a premature newborn (36th gestational week) with VLBW (1250 g). She had severe hypoxemia and low cardiac output despite medical treatment. Rerouting surgery of TAPVR was performed under deep hypothermia circulatory arrest at the age of 20 days. The sternum was left open and approximated 2 days later. Follow-up echocardiography showed good ventricular function without pulmonary venous obstruction. The endotracheal tube was removed 7 days postoperatively. She was then discharged without complication. In conclusion, with improved cardiopulmonary bypass technique and perioperative care, open heart surgery can be performed in premature newborns with VLBW.

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: Currently, prosthetic selection for aortic valve replacement is still debatable in pediatric patients with aortic valve disease. Ross procedure is a time-consuming and demanding technique which may produce good results. This study assessed the results obtained with various methods of right ventricle outflow tract (RVOT) reconstruction in patients receiving Ross procedure., Methods: From September 1996 to December 2003, 13 pediatric patients underwent Ross procedure for aortic valve disease. Their ages ranged from 1 month to 17 years (mean, 6.29 +/- 3.62 years; median, 7 years) and 2 patients were less than 1 year of age. Previous procedures in these patients included balloon dilation of the aortic valve in 10 and surgical aortic valvuloplasty in 2. RVOT was reconstructed with heterografts in 3, homografts in 3 or without extracardiac conduits in 7., Results: There was 1 in-hospital death (7.7%) and 1 late death from a non-cardiac cause. One patient developed infective endocarditis with periaortic abscess 2 weeks after the Ross procedure. Eleven patients were followed for a mean of 3.3 years (range, 8 months to 8 years). There was no significant pressure gradient across the neoaorta. Severe stenosis developed gradually in 3 patients who had RVOT reconstruction with heterograft conduits. All 3 had received RVOT redo operation. None of the patients who received RVOT reconstruction without extracardiac conduits had significant pulmonary stenosis but 6 had regurgitation (nil 1, mild 3, moderate 3). No redo operation was required during follow-up (range, 8 months to 4 years). All of the 11 survivors were in New York Heart Association functional class I., Conclusions: This study found satisfactory results of Ross procedure in pediatric aortic valve disease. RVOT reconstruction without extracardiac conduit is a feasible alternative despite the availability of homograft.

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.

Superior vena cava (SVC) syndrome rarely responds to conservative treatment. We report the case of a 2-month-old boy with SVC syndrome and bilateral chylothorax after surgical repair of the hemitruncus. Medical management with low-dose heparin, dipyridamole, and aspirin resulted in improvements of head swelling and chylothorax. The chyle had disappeared 46 days postoperatively. Compensatory growth of collateral vessels was also found. Although surgical repair is sometimes advocated in patients with SVC syndrome, medical treatment is an important alternative if the risk of surgery is too high.

Purpose: To investigate the usefulness of electron beam computed tomography (EBCT) in the preoperative detection and perioperative management of insidious concomitant primary tracheobronchial anomalies in patients with tetralogy of Fallot (TF)., Methods: From July 1995 to October 1999, 88 children (38 girls, 50 boys) with TF were enrolled in this study. EBCT examinations provided information needed to plan management. The final diagnoses of airway abnormalities were correlated with the findings of bronchoscopy in five patients and confirmed during surgery., Results: Fourteen (16%) of the 88 patients had associated primary tracheobronchial anomalies. Nine patients had tracheal bronchi, which were combined with tracheobronchial stenosis in two patients and with tracheal stenosis in one patient. Two patients had tracheal diverticulum, which was combined with lower tracheal stenosis in one patient. Two patients had congenital tracheal stenosis. Tracheomalasia was found in one patient. Three patients with ventilation difficulties died postoperatively. Special attention was given to the care of the diseased airways perioperatively, and the remaining 11 patients had a smooth course of hospitalization and discharge., Conclusions: Our results show that EBCT provides good delineation of both cardiac and tracheobronchial anomalies and suggest that it should be used perioperatively to detect associated airway anomalies in TF, to facilitate the design of an appropriate ventilation care strategy.

Acute respiratory distress syndrome (ARDS) carries a high mortality of about 60%. The results of conventional treatments for ARDS are poor. We report the use of extracorporeal membrane oxygenation (ECMO) to rescue a child with ARDS. The patient, a 7-year-old boy, underwent a Ross procedure and mitral valvuloplasty because of severe aortic and mild mitral regurgitation. ARDS due to massive transfusion and prolonged cardiopulmonary bypass developed in the early postoperative period. Hypoxemia persisted despite conventional treatments, including pressure-controlled ventilation and high-frequency ventilation. Finally, venovenous ECMO was used to rescue the patient. With ECMO support, gas exchange was well maintained with a lower ventilator setting, and ventilator-induced lung injuries were avoided. ECMO was used for 183 hours, at which point the boy was weaned without complications. His recovery was uneventful. At the latest follow-up, 6 months after the operation, he was in New York Heart Association function class I and had no complaints of lung disease. This case suggests that venovenous ECMO can be a rescue method for patients with ARDS that is refractory to conventional treatments.

Primary pericardial disease is rare in children. The clinical features usually reflect limited venous return and cardiac output. Tuberculous pericarditis is the leading cause of pericardial disease in developing nations. A definitive diagnosis in children is frequently difficult and the manifestations are protean. We report a 10-month-old girl with fibrinofibrous pericarditis that manifested as constrictive pericarditis with prolonged fever, hepatomegaly, edema, and poor appetite. Echocardiography showed a solid mass that originated from the thickened pericardium and compressed the whole heart. In contrast, computed tomography revealed pericardial thickening with fluid collection. The symptoms and signs dramatically improved after surgical pericardiectomy. Pathologic analysis confirmed the diagnosis of tuberculous fibrinofibrous pericarditis. The patient received a 1-year course of antituberculosis therapy and has remained symptom free for 2 years. We suggest that a discrepancy between echocardiography and computed tomography (CT) findings might indicate a diagnosis of fibrinofibrous pericarditis.

A 4-French gauge Opticath catheter, inserted into a neonate's right atrium through the right internal jugular vein, can be used to continuously monitor venous blood oxygen saturation in the right atrium. This method instantly detects subtle changes in cardiopulmonary function in small infants and neonates and enables the appropriate treatment to be given in cases of critical infantile and neonatal cardiopulmonary deterioration. This report discusses four neonatal and infantile patients who underwent continuous monitoring of venous blood oxygen saturation in the right atrium. Continuous monitoring allowed appropriate treatment to be given earlier compared to conventional monitoring methods of blood pressure and heart rate.

Unlabelled: Hypoplastic left heart syndrome is not infrequently seen in Taiwan, but all died previously. An 8 day-old-male baby suffering from hypoplastic left heart syndrome was transferred to our hospital after cardiopulmonary resuscitation. Oral prostaglandin E2 was used to maintain ductal patency and carbon dioxide was used through a respirator to control his pulmonary blood flow. He underwent Norwood operation at age 15 days under deep hypothermia and circulatory arrest. The postoperative course was smooth and occasional tachypnea was controlled by carbon dioxide inhalation through a mask. A follow up echocardiogram showed patent shunt and neoaortic pathway, good interatrial communication and mild tricuspid regurgitation. The baby survived and has fared well 2 years postoperatively., Conclusion: We recommend carbon dioxide manipulation before and after the Norwood operation for hypoplastic left heart syndrome with increased pulmonary blood flow uncontrollable by the respirator.

Two cases of double-orifice mitral valve are reported. The first case was associated with coarctation of the aorta and a large perimembranous ventricular septal defect, and the second case had an associated large patent ductus arteriosus. A bridge-type defect created two equal-sized openings in the mitral valve in case 1. In case 2, a small accessary opening was located in the posteromedial leaflet of the mitral valve. Double-orifice mitral valve is a rare anomaly associated with coarctation of the aorta, ventricular septal defect, and patent ductus arteriosus. The morphology of a double-orifice mitral valve can be clearly delineated by 2-D echocardiography.