Reliability of ultrasound in the prenatal diagnosis of urinary tract abnormalities

To investigate the ability of ultrasonography to detect urinary tract abnormalities prenatally, we reviewed the records of 26 pregnancies diagnosed by prenatal ultrasound to have urinary tract anomalies. We compared the prenatal diagnoses with postnatal renal and urinary tract pathology. This comparison showed different degrees of agreement for different prenatal diagnoses (2 individuals had more than one diagnosis): 4 of 8 for multicystic kidneys, 1 of 1 for polycystic kidney disease, 1 of 2 for renal agenesis, 6 of 7 for ureteropelvic junction obstruction, 1 of 3 for posterior urethral valves, 4 of 5 for no pathology noted, and 2 of 2 for other abnormalities. Prenatal diagnosis and postantal findings were in agreement in 68% of cases. Varying levels of diagnostic reliability should be considered when managing pregnancies complicated by fetal urinary tract abnormalities and subsequent postnatal evaluation and diagnosis. Further efforts are needed to improve on the techniques and reliability of prenatal diagnosis of urinary tract abnormalities.     

Emergency surgery for obstructing colorectal cancers: a comparison between right-sided and left-sided lesions

BACKGROUND:

Fifteen to twenty percent of patients with primary colorectal cancers present with intestinal obstruction. Traditionally, different approaches have been used in the management of right-sided and left-sided colonic obstruction. Recently, single-stage resection with primary anastomosis in left colonic obstruction has been shown to have good results. The objective of this study was to compare the operative results of patients who had emergency operations for right-sided and left-sided obstructions from primary colorectal cancers.

STUDY DESIGN:

This is a retrospective study including 243 patients who underwent emergency operations for obstructing colorectal cancers from 1989 to 1997. Primary resection of the tumor-bearing segment followed by primary anastomosis was attempted when the conditions were feasible. The operative results of patients with right-sided tumors were compared with those of patients with left-sided tumors.

RESULTS:

One hundred seven patients had obstruction at or proximal to the splenic flexure (right-sided lesions), and 136 had lesions distal to the splenic flexure (left-sided lesions). The primary resection rate was 91.8%. Of the 223 patients with primary resection, primary anastomosis was possible in 197 patients. Among the 101 primary anastomoses in patients with left-sided obstruction, segmental resection with on-table lavage was performed in 75 patients and subtotal colectomy was performed in 26. The overall operative mortality rate was 9.4%, although that of the patients with primary resection and anastomosis was 8.1%. The anastomotic leakage rate for those with primary resection and anastomosis was 6.1%. There were no differences in the mortality or leakage rates between patients with right-sided and left-sided lesions (mortality: 7.3% versus 8.9%, P = 0.79; leakage: 5.2% versus 6.9%, P = 0.77). Colocolonic anastomosis did not show a significant difference in leakage rate when compared with ileocolonic anastomosis (6.1% versus 6.0%, P = 1.0).

CONCLUSIONS:

This study showed that primary resection and anastomosis for left-sided malignant obstruction, either by segmental resection with on-table lavage or subtotal colectomy, was not more hazardous than primary anastomosis for right-sided obstruction. The single-stage procedure should be the objective for the treatment of patients with obstructing colorectal cancers, except when patients are hemodynamically unstable during surgery or when the condition of the bowel is not optimal for primary anastomosis.     

Cardiac morbidity and mortality in women with ductal carcinoma in situ of the breast treated with breast conservation therapy

Left-sided breast irradiation has been associated with increased risk of cardiac morbidity and mortality in some studies. This study examines the cardiac toxicity of irradiation in left- versus right-sided patients with ductal carcinoma in situ (DCIS). The medical records of 129 patients with DCIS treated with breast conservation therapy (BCT) at the Moffitt Cancer Center from 1986 to 2002 were reviewed and data regarding subsequent breast cancer and cardiac events were recorded. There were 59 left-sided and 70 right-sided patients treated. Mean age was 55 years. At 8 years, there was no significant difference observed between right- and left-sided breast cancer patients in the development of coronary artery disease, myocardial infarction, congestive heart failure, arrhythmia, valvular disease, cardiomyopathy, or cardiac-related death. Among those patients with left-sided breast cancer, 13.5% of patients developed a cardiovascular event compared to 7% of right-sided patients (p = 0.25). The overall survival at 8 years was 96% and the relapse-free survival was 85%. There were no significant differences in cardiac mortality or morbidity between right- and left-sided DCIS patients treated with BCT. Longer follow-up will be required to ascertain whether late events are more prevalent in left-sided patients.     

Congenital diaphragmatic hernia and hydrops: a lethal association?

Background/Purpose: Nonimmune hydrops in the fetus is a finding that often portends death. The association and prognosis of fetuses with congenital diaphragmatic hernia (CDH) and hydrops is not known. Methods: A retrospective review of all prenatally diagnosed cases and referrals of CDH was performed. Variables analyzed included gestational age at diagnosis and delivery, side of hernia, presence of associated anomalies and hydrops, and neonatal outcome. Results: Since 1993, 474 prenatal referrals for CDH have been made. One hundred seventy-five were evaluated; 15 fetuses had hydrops (9%). Five patients had CDH, hydrops, and associated lethal anomalies. In the remaining 10 patients, 6 of the diaphragmatic defects were right-sided and 4 were left-sided. All except one had a major portion of the liver herniated into the chest. Six fetuses had prenatal intervention. Five neonates died shortly after birth. There were 5 long-term survivors; all received prenatal intervention. Conclusions: The association of CDH and hydrops is rare but often results in fatality. Hydrops appears to be associated with liver in the hernia, right-sided lesions, and lethal anomalies. Fetal intervention can be performed successfully in patients with CDH and hydrops, and may improve long-term survival rate in this group.     

Renal duplications: the impact of perinatal ultrasound on diagnosis and management.

Perinatal urology is a rapidly expanding field in pediatric urology bringing significant changes in the diagnosis and management of congenital uropathies. Renal duplication was detected by antenatal and perinatal ultrasound in 19 cases (22 duplicated systems). In most cases, no precise diagnosis could be established in utero (4 of 13), but this lack of accuracy is of little clinical significance since all newborns with suspected uropathies undergo complete urological investigations in the perinatal period. Voiding cystogram demonstrated reflux in 11 of 22 cases. On postnatal urography a functioning upper pole was demonstrated in 11 of 22 kidneys. There were 12 ureteroceles: in 8 a nonfunctioning upper pole was removed, while 3 were endoscopically incised with good functional improvement and 1 was reimplanted. Early recognition of congenital uropathies in association with duplication by prenatal ultrasound significantly improves patient care allowing early postnatal relief of obstruction by less invasive methods and before infection or persistent obstruction worsens the prognosis.     

Unilateral multicystic dysplastic kidney in infants exposed to antiepileptic drugs during pregnancy

Prenatal exposure to antiepileptic drugs (AEDs) increases the risk of major congenital malformations (MCM) in the fetus. AED-related abnormalities include heart and neural tube defects, cleft palate, and urogenital abnormalities. Among the various congenital anomalies of the kidney and urinary tract (CAKUT), multicystic dysplastic kidney (MCDK) disease is one of the most severe expressions. Although prenatal ultrasound (US) examination has increased the prenatal diagnosis of MCDK, the pathogenesis is still unclear. We report on four cases of MCDK in infants of epileptic women treated with AEDs during pregnancy. From October 2003 to June 2006, we observed four infants with unilateral MCDK born to epileptic women. Three patients were considered to have typical features of multicystic dysplastic kidney, and one infant was operated because of a cystic pelvic mass in the absence of a kidney in the left flank. The macroscopic appearance of this mass showed an ectopic multicystic kidney confirmed by histological findings. All patients have been studied by US scans, voiding cystourethrogram (VCUG), and radionuclide screening isotope imaging. The prenatal exposure to AEDs increases the risk of major congenital malformations from the background risk of 1–2% to 4–9%. AEDs may determine a defect in apoptosis regulation that could lead to abnormal nephrogenesis, causing MCDK. Carbamazepine (CBZ) and phenobarbital (PHB) during pregnancy should be used at the lowest dosage compatible with maternal disease. The reduction, or even suspension, of drug dosage should be achieved from the periconceptional period to the first 8 weeks of gestation to avoid any interference with organogenesis.     

Conservative treatment of ureteropelvic junction obstruction in children with antenatal diagnosis of hydronephrosis: lessons learned after 16 years of follow-up

OBJECTIVES: We attempted to define predictive factors for surgery in children with antenatal diagnosis of hydronephrosis that led to postnatal diagnosis of ureteropelvic junction (UPJ) obstruction. METHODS: We retrospectively evaluated our 16-yr experience (1988-2003) with 343 children (260 male and 83 female) with antenatal diagnosis of hydronephrosis that led to postnatal diagnosis of UPJ obstruction and who were followed conservatively. Right-sided hydronephrosis was present in 110 and left-sided in 233 children. According to the Society for Fetal Urology (SFU) classification none had grade 0 of postnatal hydronephrosis, 20 had grade 1, 118 grade 2, 147 grade 3, and the remaining 58 children grade 4 postnatal hydronephrosis. Relative renal function (RRF) on radionuclide scans revealed 235 children with RRF>40%, 68 with RRF between 30% and 40%, and 40 patients with RRF<30%. Renal function deterioration >5% was the main indication for surgery. Commercially available software GraphPad Prism 4.0 (GraphPad prism, Prism 4 for Windows, version 4) using the Fisher exact test was used for statistical evaluation. RESULTS: Surgical correction was needed in 179 children (52.2%) during the course of conservative management. The average age at surgery was 10.6 mo (range, 1 mo to 7 yr). Of those, 50% underwent surgery during the first 2 yr of life and the majority of the remaining patients underwent surgery between the 2 and 4 yr of age; only two patients required surgery later on. Univariate analysis revealed that child sex, side of hydronephrosis, and SFU grade of prenatal hydronephrosis were not significant predictive factors for surgery. However, SFU grade 3-4 of postnatal hydronephrosis (p<0.0001; odds ratio, 0.06281) and RRF<40% (p<0.0001; odds ratio, 0.1022) were significant independent risk factors for surgery. CONCLUSION: In contrast with previous publications by others and by us these data show that >50% of children with antenatal diagnosis of UPJ obstruction in this series required surgical correction while on conservative protocol. SFU grade 3-4 of postnatal hydronephrosis and RRF<40% are significant independent predictive factors for surgery.     

The congenital diaphragmatic hernia composite prognostic index correlates with survival in left-sided congenital diaphragmatic hernia

PurposeWe developed the congenital diaphragmatic hernia congenital prognostic index (CDH-CPI) to incorporate all known prognostic variables into a single composite index to improve prognostic accuracy. The purpose of this study is to examine the ability of the CDH-CPI to predict survival in patients with left-sided congenital diaphragmatic hernia and to determine if the index has a stronger correlation with survival than each of the individual components.MethodsA retrospective review of patients with left-sided congenital diaphragmatic hernia between 2004 and 2010 was conducted. Ten prenatal parameters of the CDH-CPI were collected, total score was tabulated, and patients stratified according to total score and survival.ResultsSixty-four patients with a prenatal diagnosis of left-sided congenital diaphragmatic hernia were identified. Patients with a CDH-CPI score of 8 or higher had a significantly higher survival than patients with a CDH-CPI score of lower than 8. The CDH-CPI has the strongest correlation with survival compared with the individual parameters measured. The CDH-CPI correlates with extracorporeal membrane oxygenation use, and 75% of patients with a score of 5 or lower were placed on extracorporeal membrane oxygenation.ConclusionsThe CDH-CPI accurately stratifies survival in left-sided congenital diaphragmatic hernia. The amalgamation of 10 prenatal parameters of the CDH-CPI may be a better prenatal predictor than any single prognostic variable currently used.     

Airway obstructions associated with congenital heart disease in infancy

Significant airway obstruction may complicate the natural and surgical history of infants with congenital heart diseases. Airway obstruction occurred in 12 infants who had operations for congenital heart disease. In all cases tracheography demonstrated the cause of airway obstruction. Significant mortality (5/12, 41.7%) and morbidity in this group of infants were the result of airway obstruction. To reduce the complications caused by airway obstruction in infants with congenital heart disease, we recommend (1) preoperative identification of patients with potential airway obstruction, (2) preoperative tracheography in high-risk infants, (3) appropriate choice of the surgical procedure, especially when insertion of a prosthetic conduit is required, (4) early recognition of the problem during the postoperative period, (5) prompt diagnosis by postoperative tracheography, with or without angiography, and (6) therapy directed at the cause of airway obstruction.     

Redefining outcomes in right congenital diaphragmatic hernia

Purpose

Available data comparing the management and outcome of right-sided (R-CDH) vs left-sided congenital diaphragmatic hernia (L-CDH) are inconsistent. Large-volume CDH studies are limited by small numbers of R-CDH or are confounded by compilations from multiple institutions with multiple treatment strategies. Consequently, they are underpowered to draw conclusions. To define the behavior and outcomes of R-CDH better, we report the largest single-institution series of R-CDH and ask if factors traditionally linked to poor prognosis in L-CDH were applicable to R-CDH.

Methods

We reviewed a single institution's experience with 267 consecutive evaluable neonates with unilateral CDH repaired from 1990 to 2006, with specific focus on R-CDH. χ² tests were performed for disease-related categorical variables. Two-tailed unpaired t tests were used for continuous variables. Factors associated with morbidity and survival were determined by univariate regression. Statistical significance was set at P < .05.

Results

Forty right-sided (15%) and 227 (85%) left-sided cases of CDH were identified. Prenatal diagnosis was made in 20 right-sided vs 170 left-sided defects (50% vs 75%, P < .01). Survival was 22 of 40 in R-CDH compared with 175 of 227 in L-CDH (55% vs 77%, P < .01). Extracorporeal membrane oxygenation was required in 16 right-sided and 33 left-sided cases (40% vs 15%, P < .001). A diaphragmatic patch was used in 22 of 29 right-sided compared with 82 of 199 left-sided repairs (76% vs 41%, P < .01); rates of abdominal wall prosthesis were also higher in right-sided hernias (38% vs 19%, P < .05). No differences were detected in right-sided vs left-sided recurrences (14% vs 8%, P = .38), mean time from birth to operation (5.3 vs 4.8 days, P = .80), or presence of cardiac anomalies (15% vs 12%, P = .63). Morbidity persisting beyond 6 months of age was present in 16 of 22 R-CDH survivors compared with 76 of 175 L-CDH survivors (73% vs 43%, P > .05). Among R-CDHs, prenatal diagnosis was the only factor to predict survival by univariate regression (P < .01). Use of a prosthesis in the diaphragm (P < .05) for R-CDH repair correlated with morbidity.

Conclusion

Although previous reports suggest that associated anomalies, need for extracorporeal membrane oxygenation, and time to repair can influence L-CDH survival, these data do not support extrapolation to R-CDH survival. Right-sided CDH carries a disproportionately high morbidity and mortality. Prenatal diagnosis was the only factor predictive of R-CDH survival. Morbidity may correlate with use of prosthetic material for R-CDH repair. Right-sided CDH is a unique disease that may require a modified antenatal consultation.     

Impact of prenatal diagnosis on the morbidity associated with ureterocele management

PURPOSE: We postulated that prenatal detection of ureteroceles has a positive impact on the natural history and clinical outcome of ureteroceles in duplex system. MATERIALS AND METHODS: Between 1992 and 2000, 95 children underwent surgery for a ureterocele in a duplex system. We evaluated the impact of prenatal diagnosis in 40 cases versus postnatal diagnosis in 55 on morbidity, as measured by postoperative urinary tract infection and secondary procedures, while controlling for ureterocele type and the initial surgical approach. RESULTS: Mean followup in the 2 groups was 3.9 years. Preoperatively the reflux rate was 51% in the prenatal and 66% in the postnatal groups. Preoperatively urinary tract infections were less common in the prenatal group (12% versus 84%). Mean age at initial intervention in prenatally and postnatally diagnosed patients was 6 and 31 months, respectively. Postoperatively the urinary tract infection rate was double in postnatally diagnosed patients. Overall postoperatively reflux was similar in the 2 groups and grades III to V reflux with urinary tract infection accounted for 14 of the 21 secondary bladder procedures (67%). After initial endoscopic decompression none of the prenatally diagnosed patients with intravesical ureteroceles required reoperation, whereas 6 (50%) with extravesical ureteroceles required reoperation. All 10 prenatally diagnosed extravesical ureteroceles treated with partial nephrectomy were cured. Overall the secondary procedure rate in the postnatal group was higher than in the prenatal group (46% versus 20%, p = 0.02). Also, there was a difference in the reoperation rate in the endoscopic decompression group according to mode of presentation (p = 0.03) and a difference when comparing endoscopic treatment with partial nephrectomy in all patients (p = 0.02). CONCLUSIONS: Prenatal diagnosis decreases morbidity and potential adverse outcomes related to infection. Overall prenatal diagnosis is associated with a decreased rate of secondary procedures independent of the type of ureterocele. Prenatally diagnosed intravesical ureteroceles may be cured by endoscopic incision alone but for extravesical ureteroceles partial nephrectomy appears to be more definitive.     

体外循环心脏手术相关因素致肌钙蛋白T变化的研究

目的 观察体外循环心脏手术相关因素致心肌细胞的损害程度,通过心肌肌钙蛋白Ｔ变化找出其相对规律及数据,为临床提供心肌保护措施及理论根据。方法 随机选择３７例心脏手术,其中心脏瓣膜替换术１５例,先天性心脏病１５例,冠状动脉搭桥７例,于手术前、停机时、术后５ｈ、术后第１天、术后第２天抽 血检测肌钙蛋白Ｔ浓度的变化经统计学处理,ｑ检验进行对比研究。结果 （１）主动脉阻断时间〉６０ｍｉｎ组术后５小时肌钙蛋白     

Fetal intervention for myelomeningocele: effect on postnatal bladder function.

PURPOSE: Myelomeningocele is the most common congenital malformation of the central nervous system noted on prenatal ultrasound. Due to its significant postnatal sequelae, treatment in utero could potentially have a profound impact on the newborn. Others have reported fetal surgical techniques for in utero repair of myelomeningocele and its potential benefits on motor and neurological function. We report our urodynamic findings in the newborn after in utero repair of spina bifida in an effort to characterize postnatal bladder function. MATERIALS AND METHODS: A retrospective review of the fetal surgery database at University of California San Francisco was performed identifying patients with a diagnosis of myelomeningocele. Prenatal surgical repair of myelomeningocele was considered if a normal karyotype was present, no other significant congenital anomalies were evident and gestational age was less than 24 weeks. The spinal defects were in the lumbar or lumbosacral region. All surgery was performed before 24 weeks of gestations. RESULTS: Fetal surgery to correct myelomeningocele was performed in 6 patients. All patients were born premature at 32 weeks of gestation or less. Videourodynamics performed at age 1 month in 4 patients indicated decreased bladder capacity for weight, increased detrusor storage pressures and significant post-void residual. Hydronephrosis was demonstrated in 4 patients on renal/bladder ultrasound, and moderate vesicoureteral reflux was seen in 3. CONCLUSIONS: Patients with spinal bifida treated in utero appear to have the same changes in urodynamic parameters and anatomical abnormalities in the urinary tract as other children with spinal defects who have undergone standard postnatal care. In utero treatment of spinal bifida may expose the newborn to the effects of prematurity. The long-term effects on bladder function in the fetus after in utero repair of myelomeningocele remain unknown. A randomized controlled trial is necessary to evaluate long-term bladder function as well as other outcome variables in this experimental approach to patients with myelomeningocele.     

Prenatal treatment of congenital adrenal hyperplasia.

PURPOSE: The relevant aspects of congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH-21), the single most common cause of female pseudohermaphroditism, are reviewed to understand the benefits and risks of prenatal intervention. Timely diagnosis is important, since infants with this condition may suffer adrenal insufficiency which carries a high mortality rate. MATERIALS AND METHODS: Infants suspected of having CAH-21 should undergo radioimmunoassay of serum 17-hydroxyprogesterone, karyotype and pelvic/abdominal ultrasound at a minimum. Treatment with glucocorticoid and mineralocorticoid supplements should be instituted immediately. Surgical correction of genitourinary tract anomalies should be performed by a pediatric urologist experienced in this area. RESULTS: Proper postnatal medical and surgical management of CAH-21 will allow the patient to thrive. Many women with classic CAH-21 have now conceived and delivered healthy children. Prenatal diagnosis, now most often done by molecular genetic techniques, is feasible and often done in conjunction with prenatal treatment of the at risk mother. CONCLUSIONS: CAH-21 has been well characterized. The benefit of prenatal therapy is to ameliorate potentially genital ambiguity in affected female subjects. The risks of unnecessarily treating unaffected pregnancies, which now seem small, may not be fully elucidated for many years. Prenatal treatment must be done under careful, centralized and ideally long-term medical supervision.     

Congenital duodenal obstruction.

Over the period 1980-1989, 49 patients with congenital duodenal obstruction due to duodenal atresia, duodenal stenosis or annular pancreas were treated. There were three deaths, all related to associated severe congenital anomalies and none related to surgery for duodenal obstruction; 78 per cent of the patients had associated congenital anomalies which, in some instances, complicated their management. Prenatal diagnosis by ultrasonography was made in 18 per cent of the patients and these patients had operations relatively earlier.     

Congenital diaphragmatic hernia: a meta-analysis of mortality factors

PURPOSE: The aim of this study was to review all available studies reported in the English-language literature from 1975 through 1998, and by meta-analysis assess the importance of prenatal diagnosis, associated malformations, side of hernia, timing of surgery, and study population on mortality rates in patients with congenital diaphragmatic hernia (CDH). METHODS: One-hundred-two studies were identified, and 51 studies (2,980 patients) fulfilled the prespecified inclusion criteria. Studies were grouped according to study population into: (I) fetuses diagnosed prenatally; (II) neonates admitted to a treatment center; and (III) population-based studies. RESULTS: Pooled total mortality rate was significantly higher in category I than in category III (75.6% v 58.2%, P < .001). Pooled hidden postnatal mortality rate (deaths before admittance to a treatment center) in population-based studies was 34.9%. Prenatally diagnosed patients in both category II and III had significantly higher mortality rates than those diagnosed postnatally. Mortality rates were significantly higher among CDH infants with associated major malformations compared with isolated CDH in all 3 categories. An increased mortality rate in right-sided CDH was found in category II and III. CONCLUSIONS: Prenatal diagnosis of CDH, presence of associated major malformations, and the study population have a major influence on mortality rate. The very high mortality rate in studies of fetuses with a prenatal diagnosis of CDH should be taken into account in prenatal counselling.     

Prenatal diagnosis of inborn errors of metabolism with renal manifestations

Prenatal diagnosis has become increasingly important for the prevention of kidney disease due to inborn errors of metabolism. The indications for prenatal diagnosis in a specific disease depend on the degree of renal involvement and the concomitant pathology of other organs, but especially of the central nervous system. The availability of successful postnatal therapy reduces the necessity for prenatal diagnosis. The determination of enzyme activities, the demonstration of storage products in fetal tissues and the analysis of amniotic fluid components are the biochemical methods most frequently used for prenatal detection. Recently, recombinant DNA techniques have enlarged the spectrum of inherited disorders that can be diagnosed before birth.     

Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: a 13-year experience

OBJECTIVE: In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period. METHODS: The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed. RESULTS: The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1%) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9%, 54.7%, and 54.7% for the repair of congenital heart disease and lung transplantation group and 77.8%, 51.9%, and 38.9% for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9%, 51.4%, and 51.4% for the repair of congenital heart disease and lung transplantation group and 66.5%, 66.5%, and 60% for the heart-lung transplantation group, respectively. CONCLUSION: Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.     

Congenital duodenal obstruction: the impact of an antenatal diagnosis

In a series of 34 infants with congenital duodenal obstruction, 15 were diagnosed by antenatal ultrasound between the fifth month of gestation and term. Fourteen patients were available for follow-up. In 10 of 14 cases, patients were available for follow-up. In 10 of 14 cases, parents were informed of the diagnosis antenatally and, in 9, were advised of the possibility of surgery in the neonatal period. Parents felt the information helped them prepare for the surgical and medical interventions necessary in the postnatal management of their infants. The mean birth weight and gestational age of these infants were comparable with those diagnosed postnatally. Associated congenital anomalies were more common in the patients with a postnatal diagnosis. In the total group of 34 infants with duodenal obstruction, 32 had surgical repair with a survival rate of 94%. Surgical procedures were comparable between the two groups. Surgical intervention occurred earlier in the neonatal period when an antenatal diagnosis was made. Overall survival in the entire series was 88% with four deaths attributed to severe associated congenital anomalies. We conclude that (1) the antenatal diagnosis of duodenal obstruction influences parents positively in coping with the anomaly, and (2) although surgery was performed sooner, the outcome of infants with duodenal obstruction was not changed by providing an antenatal diagnosis.