Carcinoma of the proximal bile ducts.

The records of 31 patients with carcinoma of the proximal bile ducts were reviewed and the operative procedures, results, causes of deaths and autopsy findings were analyzed and correlated. Ten poor risk patients were treated by external drainage and died of jaundice or abscesses of the liver within six months. However, one patient survived 13 months after effective bilateral drainage. Twelve patients underwent intubation through the tumor into the hepatic ducts with or without postoperative irradiation. Four patients with irradiation survived 42, 15, 15 and ten months, while eight patients without irradiation died within six months. Autopsy findings of two patients who survived 15 months revealed metastases to the various organs. Four patients underwent resection. A patient who underwent resection of the common hepatic duct and hepaticoduodenostomy died of ascending cholangitis nine months postoperatively, while a patient treated by resection of the common hepatic duct with hepaticojejunostomy died of a recurrence 25 months postoperatively. Two patients underwent left hepatic lobectomy and resection of the right hepatic duct followed by hepatojejunostomy. One patient survived 25 months and died of a reccurrence, while the other patient died of abscesses of the liver ten months postoperatively.     

Sequential Doppler sonographic studies of embolization in a patient with hepatic involvement in hereditary hemorrhagic telangiectasia: correlation with angiographic findings.

A 41-year-old man was admitted for symptoms of progressive congestive heart failure. His family history and the results of a physical examination were highly suggestive of Osler-Weber-Rendu disease (hereditary hemorrhagic telangiectasia, HHT). Cardiac catheterization and hepatic angiography demonstrated HHT with left-to-right shunting from the liver. The patient underwent transcatheter arterial embolization (TAE) of the right hepatic artery. We performed both Doppler sonography and angiography before and after TAE. The treatment improved the clinical manifestations of congestive heart failure, including the edema of the leg and dyspnea. Doppler sonographic studies also showed an increased resistive index in the right hepatic artery and decreased flow volumes and velocities in the right and middle hepatic veins, respectively, after treatment. Corresponding changes on angiography after TAE showed decreased right hepatic arterial flow and nonopacified branches distal to the coils, disappearance of the mottled hepatogram in the right lobe, reduction of contrast agent staining, and enhanced calibers in the right and middle hepatic veins. This case illustrates that qualitative and quantitative studies with duplex and color Doppler ultrasound can be used to detect or define the extent of hepatic involvement in HHT patients before TAE, monitor hemodynamic changes of the intrahepatic vasculature after TAE, evaluate the efficacy of treatment, and possibly obviate the need for repeated angiography for diagnosis only.     

Pregnancy and liver transplantation

To define the risks and outcomes associated with pregnancy and liver transplantation, we reviewed our experience in managing eight pregnant women who had undergone orthotopic liver transplantation. Seven patients conceived after transplantation; the interval from transplantation to conception ranged from 3 weeks to 24 months. One patient received an allograft at 26 weeks' gestation for hepatic failure secondary to acute fulminant hepatitis B. Of the seven patients who conceived after transplantation, six had live births and one electively terminated her pregnancy. Five patients developed worsening hypertension and/or preeclampsia. Three patients developed severe preeclampsia and required delivery. One patient suffered acute allograft rejection during pregnancy which was successfully treated with corticosteroids. Two patients had persistent elevation of serum transaminases and two had severe anemia. The mean gestational age at delivery was 32.8 weeks. Of the six live births to women who conceived after transplantation, five infants survived and are well and one infant died. There were no congenital anomalies. All mothers are alive at this time. Pregnancy in recipients of hepatic allografts is associated with good perinatal outcome, but there is an increased risk of preeclampsia, worsening hypertension, and preterm delivery. Pregnancy does not appear to have a deleterious effect on hepatic graft function or survival. Joint management of these patients by a transplant specialist and a perinatologist is essential.     

The hospital course of acute myocardial infarction in the Tainan area.

The hospital records of 204 patients with acute myocardial infarction (AMI) admitted from August 1988 through July 1990 were reviewed. Of these, 138 patients who were admitted within 24 hours after onset of symptoms were enrolled in this retrospective study. In 138 patients (110 men, 28 women; aged 62.6 +/- 11.3 years), the mean prehospital time was 4.8 +/- 4.5 hours (median 3.0, range 0.5 to 24.0). Ninety-nine of these 138 patients were classified as an early presentation group (less than 6 hours); their mean prehospital time was 2.5 +/- 1.3 hours. Of these, 60 patients received thrombolytic therapy with intravenous streptokinase (SK), and their prehospital time was 2.3 +/- 1.2 hours, with mean time to SK therapy of 3.7 +/- 1.5 hours. The in-hospital mortality of these 60 patients was 16.7%. The remaining 39 patients, without SK therapy, had a higher in-hospital mortality (28.2%); their mean prehospital time was 2.7 +/- 1.3 hours. For the late presentation group (greater than or equal to 6 hours), the mean prehospital time was 10.6 +/- 4.3 hours and the in-hospital mortality was 7.7%. The frequency of severe congestive heart failure (Killip class III or IV) at admission was significantly higher in patients with early presentation, than with late presentation (30.7% vs 7.6%, p less than 0.05). The overall causes of death included congestive heart failure (79%), ventricular arrhythmia (8%) and underlying medical illness (13%). In conclusion, 43.4% of the AMI patients in this study received SK therapy, within a mean time of 3.7 +/- 1.5 hours after onset.(ABSTRACT TRUNCATED AT 250 WORDS)     

Clinical features of rheumatic carditis in adolescents and adults

A retrospective study on the clinical features of acute rheumatic fever (RF) with carditis in 45 patients, 15 years of age and older (range 15-61), is described. Nineteen patients were seen at initial attack; the remaining 26 patients (28 recurrences) were admitted because of RF recurrence. Of the total 47 episodes, carditis was manifested by a significant murmur without previous RF or any known rheumatic heart disease in 40%; change in the character of a murmur under observation or the appearance of a new murmur in 15%; and acute pericarditis in 19%. Congestive heart failure of recent onset was noted in 85% of the episodes, while classical polyarthritis or polyarthralgia occurred in 57%. The aortic valve was newly damaged or preexisting aortic regurgitation worsened in 12 episodes. Twenty-nine patients were followed for 1/2-16 years. Of 11 patients with an initial attack who were maintained on regular prophylaxis (intramuscular benzathine penicillin 1,200,000 units every 4 weeks), the mitral valve murmur disappeared in 3 and none of the patients had recurrence. Of the remaining 18 patients with no or incomplete prophylaxis, 1 died from congestive heart failure 6 months later, and 3 had recurrences with involvement of the aortic valve. In conclusion, RF with carditis in adolescents and adults in our practice at the National Taiwan University Hospital is likely to show more cardiac problems and complications than that seen in the West. Although active rheumatic carditis among this patient population is relatively uncommon, recurrences with subsequent involvement of the aortic valve (aortic regurgitation) do occur.(ABSTRACT TRUNCATED AT 250 WORDS)     

Ascites: a portent of cardiopulmonary complications in the preeclamptic patient with the syndrome of hemolysis, elevated liver enzymes, and low platelets.

OBJECTIVE: Maternal ascites is frequently found at cesarean delivery in patients with severe preeclampsia or eclampsia expressed as hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). We attempted to determine whether large-volume maternal ascites present at cesarean delivery in HELLP syndrome patients is correlated with disease severity or with any specific form of increased maternal morbidity. METHODS: For this retrospective case series, we reviewed the medical records of 190 patients and noted the presence or absence of large-volume maternal ascites, peripartum complications, laboratory data, and specific operative techniques. RESULTS: The incidence of large-volume ascites in patients with HELLP syndrome who underwent abdominal delivery was approximately 10% in classes 1, 2, and 3. Compared with HELLP syndrome patients without ascites, those with HELLP-associated ascites at surgery had a significant sixfold increase in the incidence of congestive heart failure and a ninefold increase in the incidence of adult respiratory distress syndrome, both of which usually became clinically apparent within 24 hours postpartum. Those HELLP syndrome patients without ascites at surgery developed congestive heart failure or adult respiratory distress syndrome infrequently, and more than 24 hours postoperatively. CONCLUSION: Cautious fluid administration and observation for cardiopulmonary deterioration are crucial in management of the critically ill, high-risk group of HELLP syndrome patients with large-volume ascites.     

Treatment and outcome of congenital diaphragmatic hernia.

BACKGROUND AND PURPOSE: Congenital diaphragmatic hernia (CDH) is a challenging condition and is associated with a high mortality rate; optimal therapy remains unclear. This retrospective study describes the clinical characteristics of treatment and outcome in 48 infants with CDH. METHODS: Twenty-eight male (58%) and 20 female (42%) infants with CDH were treated from 1987 through 1998. The goals of the ventilator strategy were permissive hypercapnea (PaCO2 < or = 55 mm Hg) and avoidance of hyperventilation. Infants were initially ventilated with an intermittent mandatory rate of 40 to 60 per minute, peak inspiratory pressure of 20 to 25 cm H2O, and positive end-expiratory pressure of 5 cm H2O. High-frequency positive pressure ventilation was used if hypoxemia or severe hypercapnea (PaCO2 > 60 mm Hg) occurred. Most infants underwent repair after 3 days of age and only four infants underwent early repair within 24 hours of birth. A prophylactic chest tube was placed in the ipsilateral hemithorax postoperatively in all patients treated before 1996. The severity of respiratory distress was estimated by alveolar-arterial oxygen difference, oxygenation index, and alveolar-arterial ratio. RESULTS: Forty-six patients presented with Bochdalek CDH, and two with Morgangni CDH. Antenatal diagnosis was made in 10 cases. Respiratory distress was the major manifestation and usually occurred immediately after birth. Six cases were diagnosed several months after birth and presented mainly with gastrointestinal symptoms. Eleven patients died before surgery and 37 patients underwent surgical repair. Two infants died postoperatively because of congestive heart failure and tension pneumothorax, respectively. The overall mortality rate was 27%. The major causes of mortality were severe respiratory failure, persistent pulmonary hypertension, pneumothorax, and associated anomalies. CONCLUSION: Nearly 75% of patients in this series survived. This suggests that noninvasive respiratory care combined with delayed surgery may be an acceptable strategy for the treatment of CDH, and can be used in most medical institutions without equipment for extracorporeal membrane oxygenation therapy.     

Systemic tocolysis for premature labor is associated with an increased incidence of pulmonary edema in the presence of maternal infection

Our hypothesis is that systemic tocolysis of patients in premature labor is associated with a higher incidence of pulmonary edema in the presence of maternal infection. Over a 64-month period, medical records of all patients with a diagnosis at discharge of pulmonary edema or congestive heart failure were reviewed. There were 27 cases of pulmonary edema, 16 of which (59.3%) were associated with treatment of preterm labor. The incidence of pulmonary edema in patients receiving systemic tocolysis for treatment of preterm labor was significantly higher than that in our general obstetric population (3.04% versus 0.05%). Of the 527 patients receiving tocolysis, there was evidence of maternal infection in 52. The incidence of pulmonary edema was higher in the presence of maternal infection than in its absence (11/52 or 21% versus 5/475 or 1%, p = 0.0000). We conclude that there is a very strong association between the development of pulmonary edema and the presence of maternal infection in patients being treated for premature labor with systemic tocolysis.     

Congenital hepatic arteriovenous malformation: an unusual cause of neonatal persistent pulmonary hypertension.

Congenital hepatic arteriovenous malformations are rare anomalies, which typically present in infancy with congestive heart failure, anemia, and hepatomegaly. Morbidity and mortality is high if the condition is not recognized and treated promptly. Hepatic arteriovenous malformation associated with persistent pulmonary hypertension of the newborn has been reported in two cases in the literature. We report a neonate who was referred for management of persistent pulmonary hypertension and was subsequently diagnosed with a large hepatic arteriovenous malformation. He underwent coil embolization following which pulmonary hypertension resolved.     

Risk for postoperative congestive heart failure

To identify predictors of postoperative congestive heart failure (CHF), a high-risk population, mainly hypertensive and diabetic patients undergoing elective general operations, was studied. Of the 254 patients, 6 per cent had postoperative CHF. Among patients with preoperative cardiac disease (that is, previous myocardial infarction, valvular disease or CHF), 17 per cent had postoperative CHF; in contrast with less than 1 per cent of those without cardiac disease (p less than 0.001). Patients with diabetes were also at high risk (12 versus 2 per cent, p less than 0.005), particularly those with cardiac disease. Patients who had equal to or greater than 40 millimeters of mercury increases or decreases intraoperatively in mean arterial pressure in relation to preoperative baseline had increased postoperative failure rates (p less than 0.02). Of note, postoperative failure rates were highest among patients with less than 500 milliliters per hour of net intake (input and output) (p less than 0.03). Risk for postoperative CHF was restricted to patients with preoperative symptomatic cardiac disease and was especially high if patients also had diabetes. Intraoperative fluctuations in mean arterial pressure increased the probability of postoperative failure, while the intraoperative administration of higher net volumes of fluid was associated with decreased risk.     

Risk factors for mortality in primary isolated coronary artery bypass grafting surgery.

BACKGROUND AND PURPOSE: Identifying the risk factors for mortality in coronary artery bypass grafting (CABG) surgery is important to improve surgical results. The purpose of this study was to identify the risk factors for mortality in primary isolated CABG in a series of Taiwanese patients. METHODS: Medical records of 914 patients who underwent primary isolated CABG surgery in Veterans General Hospital-Taipei during the period from January 1, 1991, to December 31, 1995, were reviewed. Eighteen clinical and seven operative variables were included in the univariate and multivariate analyses to identify the determinants of mortality in CABG surgery. RESULTS: Thirty-one patients (3.4%) died within 30 days after surgery; 41 (4.5%) died during hospitalization for the procedure. After univariate and multivariate analyses, emergency surgery, history of myocardial infarction, concomitant peripheral artery occlusive disease (PAOD), and prolonged cardiopulmonary bypass (CPB) were found to be significant determinants of mortality in primary isolated CABG. Other variables, including age, sex, angina class, NYHA class, diabetes mellitus, the number of anastomoses, aortic cross-clamp time, stenosis of the left main coronary artery, the number of stenotic coronary arteries, history of congestive heart failure, and a left ventricular ejection fraction less than 35%, were not significant determinants of mortality. Patients undergoing emergency surgery were found to be at highest risk of mortality. CONCLUSIONS: Patients undergoing emergency surgery who had a history of myocardial infarction, concomitant PAOD, or prolonged CPB were at higher risk of mortality in CABG surgery. More comprehensive techniques in myocardial protection, surgical procedures, and postoperative care should be used in the treatment of high-risk patients to reduce mortality.     

Aortico-left ventricular tunnel associated with single coronary artery in an infant.

Aortico-left ventricular tunnel (ALVT) is a rare congenital malformation in which an abnormal communication between the aorta and the left ventricle (LV) bypasses the aortic valve. In infants, it usually presents with congestive heart failure. The clinical presentations mimic aortic regurgitation or ruptured sinus of Valsalva aneurysm into the LV. Progressive aortic regurgitation is a common problem in patients with ALVT. ALVT associated with a single coronary artery is extremely rare. We report the case of an infant who had congestive heart failure caused by ALVT. The diagnosis was made by echocardiography and angiography. In addition, a single coronary artery from the noncoronary cusp was found during surgery. Congestive heart failure resolved after successful surgical repair and he was asymptomatic throughout 2 years of follow-up. This case illustrates that early diagnosis of ALVT and corrective surgery can prevent aortic valve incompetence and is associated with a good clinical outcome.     

Pregnancy in a patient with aneurysms of the right coronary artery and an arterioventricular fistula. A case report

A woman with aneurysms of the right coronary artery and right coronary sinus of Valsalva and a right coronary arterioventricular fistula developed angina during pregnancy. Lumbar epidural anesthesia was utilized during induction of labor and cesarean delivery. The patient had no intrapartum evidence of ischemia or congestive heart failure, and she recovered without an immediate recurrence of angina.     

Clinical features and outcome analysis of infective endocarditis in elderly patients.

BACKGROUND AND PURPOSE: Diagnosis and management of infective endocarditis (IE) in elderly patients remains a difficult problem. This study evaluated the clinical and microbiologic characteristics and outcome of IE in elderly patients. METHODS: From 1996 to 2002, clinical and microbiologic data from 67 patients with IE aged > or = 65 years at National Taiwan University Hospital were reviewed and analyzed. RESULTS: The median age of the 67 patients was 74 years (range, 66 to 95 years). Staphylococci and streptococci were the 2 leading etiologies of IE. Forty seven of the cases were defined as community-acquired. IE had not been diagnosed at admission in nearly two-thirds of cases. Of the 20 episodes of nosocomial IE, the median interval to development of IE after hospitalization was 30 days. Staphylococci constituted 90% of causative pathogens in nosocomial IE. The in-hospital mortality rate was 29.9%. Univariate analysis revealed that staphylococci as the causative pathogen and nosocomial IE were predictors of fatal outcome (p <0.05). When significant variables related to a fatal outcome on univariate analysis, i.e., staphylococcal IE, nosocomial IE, acute renal failure and cardiac complications, were entered in the multivariate analysis, acute renal failure and development of congestive heart failure or new conduction disturbance were both significantly associated with in-hospital death. CONCLUSIONS: Diagnosis of IE in the elderly was often delayed and a substantial proportion of patients died during hospitalization. Staphylococcal IE and nosocomial IE were significant predictors of in-hospital death.     

Junctional ectopic tachycardia in infancy: report of two cases.

Junctional ectopic tachycardia (JET) in infancy is one of the serious arrhythmias which can be fatal. Typical features of JET include rapid and irregular heart beats with atrioventricular dissociation. Two cases of JET are reported: Case 1 was a 35-week-gestational age newborn who was found to have hydropsy and fetal tachycardia at the 21st week of gestational age. Antiarrhythmic agents including digoxin, propranolol and verapamil were administered to his mother to treat the fetal arrhythmia without success. JET was recognized at birth which was spontaneously converted into a sinus rhythm at 1 month of age. The maternal history revealed that two previous pregnancies ended in hydrops fetalis, and one of these was documented to have fetal tachycardia. Case 2 was a 6-month-old male infant with JET and congestive heart failure. After failure of various antiarrhythmic agents, amiodarone finally slowed down his heart rate and controlled his congestive heart failure.     

Outcome of infants born at 21-28 weeks' gestation in an inner-city hospital over an eight-year period

Outcome of all live births at 21-28 weeks' gestation (GA) and/or <1250 g birth weight (BW) over an eight-year period were reviewed and compared with other outcome data. Causes of mortality were ascertained for all deaths. Significant morbidities were reviewed among <25 weeks GA and/or <800 g survivors over a two-year period. NICU admissions log book, monthly morbidity-mortality reports, medical records and autopsy findings were used to obtain outcome data. A total of 23,046 infants were born alive during the study period, including 339 (1.47%) at 21-28 weeks or <1250 g. Eighty of the 339 (23.6%) died. Of these 80 deaths 32.5% were extremely immature, 15% died from RDS, 15% from infection, 10% from pulmonary hemorrhage and 8% had lethal malformations. Survival outcome in each weight group except <500 g were similar to that reported by the Pediatrix Medical Group (2003-04) and 2002 US National Vital Statistics. Of the extremely low gestational age infants, 79% survived to discharge, and among these, the incidence of serious neuro-morbidity, severe chronic lung disease or retinopathy of prematurity requiring treatment was 52%; 18.5% had more than one major morbidity.     

Intraparenchymal brain hemorrhage and remote soft tissue arteriovenous malformation in a newborn infant.

Congenital arteriovenous malformations (AVMs) often present with congestive heart failure. Such pathologic vascular structures typically occur in cranial, hepatic, or pulmonary locations and are usually associated with overlying external visible, tactile, or audible abnormalities. These vascular anomalies may also be associated with such complications as thromboembolic events, coagulopathy, and localized hemorrhage. We present a newborn infant with an occult but hemodynamically significant parascapular AVM who presented with an intraparenchymal brain hemorrhage, which we suspect to be a remote complication of the AVM.     

卡托普利治疗心力衰竭55例临床观察

目的:观察卡托普利治疗心力衰竭的临床疗效。方法:55例确诊为心力衰竭的患者,在常规利尿剂、洋地黄及硝酸盐制剂治疗的基础上加用卡托普利。起始量为6.25mg(2次/天),直到最大耐受剂量或推荐靶剂量50mg(3次/天),定期门诊随访,坚持服药3个月。治疗前及治疗3个月后复查超声心动围观察左室舒张末内径(LVDD)和左室射血分数(LVEF),观察临床疗效。结果:卡托普利治疗3个月后,心力衰竭患者心功能明显改善,总有效率为90.5%,超声心动图显示左室舒张末期内径缩小,左室射血分数升高。结论:长期应用卡托普利治疗心力衰竭可改善心功能及心肌重构,提高生活质量,改善预后。     

92例单脐动脉胎儿的结局

目的 探讨与单脐动脉并发的其他胎儿畸形及妊娠结局,为孕期咨询和处理提供依据.方法 对2007年9月至2009年7月之间在本院诊断并分娩的92例单脐动脉胎儿的结局进行回顾分析及随访.结果 25292例分娩的孕妇中确诊单脐动脉共92例,发生率为0.36％,其中53例(57.6％)新生儿无畸形存活,14例(15.2％)带畸形生存,围产儿死亡25例(27.2％).92例单脐动脉胎儿中伴畸形者共36例(39.1％),其中单发畸形29例(占80.6％),多发畸形7例(占19.4％).心脏畸形发生率位居首位,其他畸形依次为中枢神经系统、消化系统、运动系统及泌尿系统的畸形.单脐动脉孕妇同意选择胎儿染色体检查共33例,其中3例有染色体畸形(9.1％).92例中伴发小于胎龄儿者23例(25.0％),其中78.3％(18/23)的小于胎龄儿死亡,明显高于同期非单脐动脉小于胎龄儿的病死率4.0％ (24/597)(x2=181.71,P＜0.01).结论 单脐动脉易伴发其他先天畸形,孕期B超发现单脐动脉后,需要进一步寻查其他畸形,进行胎儿超声心动、胎儿染色体核型分析等检查,伴发严重胎儿生长受限,是胎儿不良结局的重要指标.     

Unresectable hepatic metastases from carcinoma of the colon and rectum

To alter the dismal prognosis of multiple unresectable metastases to the liver from carcinoma of the colon and rectum, 30 patients underwent hepatic dearterialization (ligation of the hepatic artery, transection of hepatic ligaments and cholecystectomy) and distal hepatic artery cannulation with prolonged infusion chemotherapy by a portable infusion pump followed by systemic intravenous chemotherapy. Involvement of the liver by carcinoma was less than 50 per cent in 16 and more than 50 per cent in 14 patients. The results of follow-up examinations, LFT, CEA and CT scan studies showed more than 50 per cent regression of the tumor and a decrease in alkaline phosphatase values and CEA in 29 patients (97 per cent); six had complete regression of tumor. The duration of response ranged from five to 39 months with the median of 17 or more months. The results of sequential LFT showed immediate increase in liver enzymes with return to normal in seven to 14 days. The mean CEA value decreased by 69 per cent within the first week and further decreased by 88 per cent in two months at the end of infusion chemotherapy. The over-all and adjusted survival rates from diagnosis were 79 and 91 per cent at 12 months; 56 and 81 per cent at 18 months, and 40 and 65 per cent at 24 months. The over-all and adjusted median survival rate after the treatment was 17 and 23 months, respectively. Of the 14 patients who failed this program, 11 had recurrences at sites other than the liver, with hepatic disease in remission in the majority. Of the 17 patients who died, six died of causes unrelated to the recurrence of disease. Thus, hepatic dearterialization and infusion regional chemotherapy can "effectively" control the hepatic disease and increase over-all survival time from three to six months to 23 months. However, recurrences of extrahepatic carcinoma and other causes are responsible for death and the over-all guarded prognosis.