Major Cardiac Anomalies Research Articles

OP28.04: Routine cardiac evaluation at 11-14 weeks scan

To evaluate the potential benefit of an early cardiac ultrasound assessment, included in the 11–14 weeks scan. We included in our study group 382 patients, referred to our clinic for the first trimester anomaly scan, over a period of 12 months. The gestational age at the time of examination was included in the 11–13 + 6 weeks interval and the patients' age was between 21 and 42 years, with a mean value of 33 years. We used a General Electric Voluson E8 with high-resolution transabdominal probe. Only in selected cases the transvaginal approach was necessary. A single trained operator examined all cases. The examination was limited to a maximum of 20 minutes, during which a general fetal survey was performed, with emphasis on measurement of nuchal translucency, evaluation of nasal bones, ductus venosus flow and tricuspid regurgitation. The cardiac assessment included four views (four chamber view, three vessel view, great vessels outflow tracts and aortic arch). High-density flow was used in all cases. In 74 cases (19.3%) the heart examination was not possible. In all 308 remaining patients (80.7%) the four chamber view and transvalvular flow were visualized. In 286 patients (74.8%) the three vessel flow and the “V” sign were imaged, the outflow tracts and the crossing of the great vessels in 294 cases (76.9%) and the aortic arch in 160 patients (41.8%). Our group included three cases with major cardiac anomalies, confirmed at 20 weeks scan, but suspected from the first trimester examination. The 20 minutes examination time was sufficient in all cases. The evaluation was limited by early gestational age, increased maternal abdominal adipose tissue or uterine pathology (leiomyomas and uterine scar) in which cases a transvaginal examination was helpful. Even though the diagnosis of cardiac anomaly was not made during the first trimester scan, it helped in selecting suspicious cases. The examination time is not significantly increased and the procedure was accessible to second level sonographers.

OP17.10: Prognostic value of prenatal diagnosis of conotruncal anomalies

Conotruncal anomalies (CTA) are complex but surgically correctable with good outcome, 90% have duct dependent circulation at birth and all can be diagnosed by mid gestation fetal cardiac ultrasound (FCUS). Advantage of prenatal diagnosis (PD) has been univocally shown only for transposition of the great arteries (TGA), diagnosis > 72 hours being associated with significant morbidity and mortality. All fetuses affected by CTA diagnosed by FCUS between Jan 2005 and July 2008 were assessed by complete obstetric US. Karyotype and del 22q11.2 analysis was obtained either pre or postnatally. Confirmation was obtained after birth by US or at autopsy in case of termination of pregnancy (TOP) or intrauterine death (IUD). Besides outcome of all CTA with PD, we compared outcome of cases of isolated CTA (ICTA) with and without PD. 49 fetuses (Group 1) were diagnosed as CTA. Gestational age (GA) at diagnosis was 23.7 + 6.5 weeks (range 15–37). 11 neonates (group 2) presented de novo with CTA. In group 1 diagnoses were: Fallot's tetralogy (TF) 17 (35%) TGA 15 (31%) double outlet right ventricle (DORV) 8 (16%)Truncus arteriosus (TA) 5 (10%) Interrupted aortic arch type B (IAAB) 2 (4%), pulmonary atresia VSD (PAVSD) 2 (4%). CTA was isolated in 19 (39%) Major extra cardiac anomalies were present in 28 (57%), 7 (15%) had increased nuchal translucency and 5 (11%) IUGR. Genetic abnormalities were present in 21 (43%): 14 (28.5%) had ChrA, 5 (10%) del 22q11.2, 1 had both and 1 was a CHARGE association. Group 2: 8 (73%) had TF, 2 (18%) TGA, 1 (9%) APVSD, none had abnormal chromosomes, 1 del 22q11.2. 3 had minor skeletal (1) and renal (2) associated anomalies. PD of CTA is associated with poor outcome because of associated problems and severity of CTA compared with cases presenting after birth.

P25.01: Prospective follow-up of the cases with prenatally diagnosed major cardiac anomalies

P25.01: Prospective follow-up of the cases with prenatally diagnosed major cardiac anomalies

Utility of Fetal Echocardiography After Normal Cardiac Imaging Findings on Detailed Fetal Anatomic Ultrasonography

The purpose of this study was to assess the utility of fetal echocardiography (FE) after normal fetal cardiac imaging findings during detailed fetal anatomic ultrasonography (FAU). We conducted a retrospective cohort review of obstetric ultrasonographic studies from November 2001 through July 2005. We identified women with a singleton gestation with increased risk for congenital heart disease who received FAU performed by a maternal-fetal medicine specialist at 16 to 20 weeks' gestation with subsequent FE. These records were compared with newborn outcomes. Of 789 pregnancies that had FAU and FE, 481 had satisfactory cardiac imaging. Of those, only 1 fetus had abnormal FE findings. After delivery, 4 of the 480 neonates with normal FAU and FE findings had a diagnosis of a heart defect. Fetal echocardiography does not substantially increase the detection rate of major cardiac anomalies after normal findings on detailed FAU performed by a maternal-fetal medicine specialist.

Esophageal atresia: Prognostic classification revisited

Although the Spitz classification is the most widely used prognostic classification for esophageal atresia and/or tracheoesophageal fistula (EA), its discrimination ability remains unclear. We sought to develop a more accurate prognostic classification for EA. The records of 121 consecutive infants with EA (1980-2005) were reviewed. The independent variables included 6 clinical characteristics, and the dependent variables were survival and mortality. Stepwise logistic regression analysis was used to construct models predicting mortality and create a revised prognostic classification. The discrimination abilities of the revised classification and the Spitz classification were compared using receiver-operating characteristic (ROC) curves. Birth weight and the presence of major cardiac anomalies were significant prognostic factors for mortality, and major cardiac anomalies affected mortality more than birth weight. The ROC curve for birth weight suggested that 2,000 g was an appropriate cutoff point. The Spitz classification was revised as follows: the revised class I (low-risk group) consisted of patients without major cardiac anomalies and birth weight >2,000 g; class II (moderate-risk group) consisted of patients without major cardiac abnormalities and birth weight <2,000 g; class III (relatively high-risk group) consisted of patients with major cardiac anomalies and birth weight >2,000 g; and class IV (high-risk group) consisted of patients with major cardiac anomalies and birth weight <2,000 g. The ROC comparisons showed that the revised classification provided a significant improvement (P = .049). This revised classification can improve the stratification of EA patients and be a useful predictor of survival.

Fetal cardiac scanning performed immediately following an abnormal nuchal translucency examination

To study the implications of early fetal cardiac scanning immediately following an abnormal nuchal translucency (NT) examination. Fetal cardiac scanning was performed immediately after an increased NT was observed. Scans were performed transvaginally at 11 to 14 weeks. Fetal echocardiography was repeated between 14 and 24 weeks in continuing pregnancies, or when the cardiac scanning appeared normal at 11 to 14 weeks. We performed 2513 NT examinations. An abnormal NT was observed in 135 (5.4%) patients. In addition, 65 patients with an abnormal NT were referred to us for fetal cardiac scanning from other offices. Overall, we performed 200 fetal cardiac scans between 11.2 and 13.5 weeks for an abnormal NT examination. Twelve major fetal cardiac anomalies were diagnosed between 12 and 13.5 weeks. Seven patients (58%) terminated pregnancy between 12 and 14 weeks without performing chorionic villous sampling (CVS). Five patients asked for chromosomal analysis before deciding about their pregnancy. Fetal cardiac anomalies were suspected in six additional cases, but only one of them was diagnosed. Another five minor and one major fetal cardiac anomaly were suspected at 11 to 14 weeks but diagnosed later on fetal echocardiography. Major fetal cardiac anomalies can be detected immediately following an abnormal NT examination and be useful for the patients' decisions about the management of their pregnancy.

Genotype-phenotype correlations in MYCN-related Feingold syndrome

Feingold syndrome (FS) is the most frequent cause of familial syndromic gastrointestinal atresia and follows autosomal dominant inheritance. FS is caused by germline mutations in or deletions of the MYCN gene. Previously, 12 different heterozygous MYCN mutations and two deletions containing multiple genes including MYCN were described. All these mutations result in haploinsufficiency of both the canonical MYCN protein and the shorter isoform, DeltaMYCN. We report 11 novel mutations including seven mutations in exon 2 that result in a premature termination codon (PTC) in the long MYCN transcript. Moreover, we have identified a PTC in exon 1 that only affects the DeltaMYCN isoform, without a phenotypic effect. This suggests that mutations in only DeltaMYCN do not contribute to the FS. Additionally, we found three novel deletions encompassing MYCN. Together with our previous report we now have a total of four missense mutations in the DNA binding domain, 19 PTCs of which six render the transcript subject to nonsense-mediated decay (NMD), and five larger deletions in a total of 77 patients. We have reviewed the clinical features of these patients, and found that digital anomalies, e.g., brachymesophalangy and toe syndactyly, are the most consistent features, present in 100% and 97% of the patients, respectively. Small head circumference was present in 89% of the cases. Gastrointestinal atresia remains the most important major congenital anomaly (55%), but cardiac and renal anomalies are also frequent. We suggest that the presence of brachymesophalangy and toe syndactyly in combination with microcephaly is enough to justify MYCN analysis.

Low nitric oxide: a key factor underlying copper-deficiency teratogenicity

Copper (Cu)-deficiency-induced teratogenicity is characterized by major cardiac, brain, and vascular anomalies; however, the underlying mechanisms are poorly understood. Cu deficiency decreases superoxide dismutase activity and increases superoxide anions, which can interact with nitric oxide (NO), reducing the NO pool size. Given the role of NO as a developmental signaling molecule, we tested the hypothesis that low NO levels, secondary to Cu deficiency, represent a developmental challenge. Gestation day 8.5 embryos from Cu-adequate (Cu+) or Cu-deficient (Cu−) dams were cultured for 48 h in Cu+ or Cu− medium, respectively. We report that NO levels were low in conditioned medium from Cu−/Cu− embryos and yolk sacs, compared to Cu+/Cu+ controls under basal conditions and with NO synthase (NOS) agonists. The low NO production was associated with low endothelial NOS phosphorylation at serine 1177 and cyclic guanosine-3′,5′-monophosphate (cGMP) concentrations in the Cu−/Cu− group. The altered NO levels in Cu-deficient embryos are functionally significant, as the administration of the NO donor DETA/NONOate increased cGMP and ameliorated embryo and yolk sac abnormalities. These data support the concept that Cu deficiency limits NO availability and alters NO-dependent signaling, which contributes to abnormal embryo and yolk sac development.

Laparoscopy-Assisted Gastropexy for Gastric Volvulus in a Child with Situs Inversus, Asplenia, and Major Cardiac Anomaly

The aim of this study was to report on laparoscopy-assisted gastropexy in a child with situs inversus, asplenia, and major cardiac anomaly. A 15-month-old boy presented with a sudden onset of epigastralgia, nonbilious vomiting, and severe abdominal distention. After a nasogastric tube decompression of the stomach, symptoms resolved and an upper gastrointestinal contrast study confirmed situs inversus and asplenia. Computed tomography showed hepatic symmetry. Major cardiac anomalies (e.g., single atrium, single ventricle, common atrioventricular valve, and pulmonary atresia) were also present and had been treated elsewhere by a Blalock-Taussig shunt operation, the Glenn procedure, and pulmonary artery plasty. To prevent recurrent gastric volvulus, an anterior gastropexy procedure was performed laparoscopically. The patient's weight at the time of surgery was 8.1 kg, and the operating time was 65 minutes. Cardiopulmonary status was stable during insufflation and throughout the laparoscopic procedure. Postoperative recovery was uneventful, and the patient was allowed oral fluids 1 day after surgery and an unrestricted diet on day 2. A Fontan procedure was performed 18 months later, and our patient is now 6 years old and well--with no recurrence of gastrointestinal symptoms. This is the first report about the successful application of laparoscopy for performing a gastropexy procedure in a child with gastric volvulus, situs inversus, major cardiac anomaly, and asplenia.

Modified Prognostic Criteria for Oesophageal Atresia and Tracheo-Oesophageal Fistula

With the advances in technology, the outcome of oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) has significantly changed. The aim of this study was to review the outcome of OA and TOF with respect to the various known prognostic criteria and to find out if there is a need for a further modification of the prevailing prognostic classification. The case notes of 57 newborns with OA/TOF treated between 1996 and 2004 were reviewed retrospectively. Patient demographics, associated anomalies and management were studied. The outcome was analysed with respect to different published prognostic criteria (Spitz, Waterston, Bremen and Montreal). The results in this series show identical results for Waterston class A and B as well as for the Bremen "without complications" groups. Furthermore, there was no statistically significant difference between Spitz type I (survival 100 %) and type II (survival 92.8 %) (Fisher's exact test [p = 0.259], Pearson's chi-square [p = 0.088] and Mann-Whitney test [p = 0.091]). There was, however, a significant variation (Fisher's exact test) after combining the results for Spitz type I & II and comparing them to type III (p = 0.006). On the basis of these results, a further modified prognostic criteria for infants with OA/TOF is proposed. Group A would include infants with either a single poor prognostic risk factor (i.e., weight below 1.5 kg or a major cardiac anomaly) or isolated TOF/OA. According to this study, the prognosis for such infants should be excellent (survival = 98 %). The alternate group (B) would include infants affected by both negative risk factors and TOF/OA; such infants have a poor prognosis (survival = 33 %). Survival for children with TOF/OA is not dependent on factors including birth weight, gestational age, pre/postoperative ventilation and a major cardiac anomaly taken independently. In the modified prognostic classification for OA/TOF, a low birth weight combined with cardiac malformations is associated with a poor prognosis. This alternate prognosticator offers benefits for appropriately advising parents of babies with such anomalies taking the current standards of care into consideration. It should also serve as a foundation stone for further prospective studies.

Cor Triatriatum Sinistrum: Surgical Experience in Taiwan

Cor triatriatum sinister, more commonly referred to as cor triatriatum (CT), is characterized by a common pulmonary venous chamber (proximal chamber) separated from the left atrium (distal chamber) by a fibromuscular septum. This report of our experience updates the surgical results of this anomaly in the Asian population. Between January 1992 and May 2005, nine patients with cor triatriatum underwent surgical correction at Chang Gung Memorial Hospital. We retrospectively analyzed their cardiac anatomy, clinical data, surgical procedures and follow-up data. The mean age at surgery was 260+/-215 days (range, 20-790 days), with the exception of one patient who underwent surgery at 31 years of age. Three patients had a partial anomalous pulmonary venous connection, one had a complete atrioventricular canal defect, and one had another major complex cardiac anomaly. Eight corrective operations and one palliative operation were performed. There was no surgical mortality. The mean follow-up time was 52.1+/-43.6 months (range, 17-139 months). Our findings show that surgical correction is efficient and safe for this rare cardiac anomaly.

Early childhood health, growth, and neurodevelopmental outcomes after complete repair of total anomalous pulmonary venous connection at 6 weeks or younger

This interprovincial inception cohort study explores early childhood outcomes and their operative and perioperative predictors after total anomalous pulmonary venous connection repair, simple (patent ductus arteriosus, atrial septal defect, or both) or complex (any associated major cardiac anomalies). From 1996 through 2004, a total of 41 consecutive neonates with total anomalous pulmonary venous connection underwent complete repair with deep hypothermic circulatory arrest. Multidisciplinary health and neurodevelopmental outcomes (Bayley Scales of Infant Development II [1993], Mental and Psychomotor Developmental Indices) were assessed at 18 to 24 months of age. Regression analyses explored potentially modifiable predictors of outcome. Survival after simple total anomalous pulmonary venous connection repair was 31 of 32 (97%); that after complex repair was 3 of 9 (33%). Relative risk of death was higher for complex (21.3) and obstructive (8.4) total anomalous pulmonary venous connections. Those who died had longer deep hypothermic circulatory arrest times (P < .001). For 34 survivors, Mental Developmental Index was 87 +/- 16 and Psychomotor Developmental Index was 89 +/- 13. Among survivors, 24.6% of variability in Mental Developmental Index was explained by its linear relationship with socioeconomic status, whereas 35.5% of variability in Psychomotor Developmental Index was explained by its linear relationship with weight at surgery and highest plasma lactate level after surgery. North American native peoples made up 38% of survivors, a higher than expected number. Mean developmental scores were in the low average range of normative data. Socioeconomic factors predicted mental outcome. High postoperative lactate level on day 1 predicted low motor scores. Other potentially modifiable variables will need to be explored. Further epidemiologic study relating race to occurrence of total anomalous pulmonary venous connection is suggested. Early neurodevelopmental assessment is recommended.

Nuchal Translucency and the Risk of Congenital Heart Disease

To estimate whether nuchal translucency assessment is a useful screening tool for major congenital heart disease (CHD) in the absence of aneuploidy. Unselected patients with singleton pregnancies at 10(3/7) to 13(6/7) weeks of gestation were recruited at 15 U.S. centers to undergo nuchal translucency sonography. Screening characteristics of nuchal translucency in the detection of major CHD were determined using different cutoffs (2.0 or more multiples of the median [MoM], 2.5 or more MoM, 3.0 or more MoM). A total of 34,266 euploid fetuses with cardiac outcome data were available for analysis. There were 224 cases of CHD (incidence 6.5 per 1,000), of which 52 (23.2%) were major (incidence 1.5 per 1,000). The incidence of major CHD increased with increasing nuchal translucency: 14.1 per 1,000, 33.5 per 1,000, and 49.5 per 1,000 at 2.0 or more MoM, 2.5 or more MoM, and 3.0 or more MoM cutoffs, respectively. Sensitivity, specificity, and positive predictive values were 15.4%, 98.4%, and 1.4% at 2.0 or more MoM; 13.5%, 99.4%, and 3.3% at 2.5 or more MoM; and 9.6%, 99.7%, and 5.0% at 3.0 or more MoM. Nuchal translucency of 2.5 or more MoM (99th percentile) had a likelihood ratio (95% confidence interval) of 22.5 (11.4-45.5) for major CHD. Based on our data, for every 100 patients referred for fetal echocardiography with a nuchal translucency of 99th percentile or more, three will have a major cardiac anomaly. Nuchal translucency sonography in the first trimester lacks the characteristics of a good screening tool for major CHD in a large unselected population. However, nuchal translucency of 2.5 or more MoM (99th percentile or more) should be considered an indication for fetal echocardiography. II.

Study of 24 cases with congenital esophageal atresia: What are the risk factors?

Recovery from esophageal atresia (EA) and tracheoesophageal fistula (TEF) has improved markedly over the years. But postoperative complications, however, have remained. This study evaluates recovery, preoperative, and postoperative status of patients with EA/TEF. A retrospective study review was undertaken in 24 patients with EA/TEF after primary anastomosis (January 1975 through September 2003). There were no patients who had major cardiac anomalies or trisomy 18. In total, 17 of 24 (70.8%, group A) patients have survived and seven (29.2%, group B) have died. Birthweight and Apgar Scores in group A were significantly higher than in group B. The ratio of GAP (the distance of the location of the blind pouch from the ends of the upper and lower esophagus) to body length in group B was significantly higher than in group A. The birthweight and Apgar Scores in group A were significantly higher than in group B. When the authors compared their sample of cases by means of the Waterston classification, the Montreal classification and the Spitz classification, there were statistically significant differences between the results using the Waterston classification and the results using to the Spitz classification. For the cases of EA surgery that were examined, the authors concluded that bodyweight at birth and the existence of pre-surgery respiratory system complications have a significant effect on post-surgery recovery, and that results appear to indicate the importance of classification using the Waterston classification and Spitz classification as a means of assessing the degree of risk. Results also appeared to indicate that the control of Respiratory Distress Syndrome throughout both the pre-surgery and post-surgery periods is critical.

Initial nonoperative management and delayed closure for treatment of giant omphaloceles

Purpose Traditional treatment of giant omphaloceles with silo closure has been associated with respiratory insufficiency, hemodynamic compromise, dehiscence, and inability to close the abdomen with subsequent death. To minimize such complications, initial nonoperative management with delayed closure of the defect has been used. Methods Between January 1981 and December 2002, 111 patients with omphaloceles were treated. Twenty-two patients with giant omphaloceles (19 containing liver) underwent initial nonoperative management consisting of silver sulfadiazine dressing changes. After pulmonary and other comorbidities stabilized, the contents were gradually reduced with a loose elastic bandage, and delayed closure was planned at 6 to 12 months. The medical records of these 22 patients were retrospectively reviewed to determine the efficacy and safety of this technique in the setting of severe associated anomalies. Those 15 patients (n = 15) from the latter 10 years were further reviewed to determine additional end points (length of hospital stay, length of intensive care unit stay, duration of mechanical ventilation, time to feed, time to closure, and type of closure). Results Of the 15 patients treated during the latter 10 years, mean gestational age and birth weight were 38 ± 1.4 weeks and 3.1 ± 0.57 kg, respectively. Median length of stay after birth was 20 days (range, 5-239 days). Median time to full diet was 8 days (range, 4-80 days). Four patients were discharged on oral feedings only, 7 with combination oral/gavage, and 4 with tube feedings. Pulmonary hypoplasia or pulmonary hypertension was present in 11 (50%) of 22 patients. There were 11 patients with major cardiac anomalies, 14 with a patent ductus arteriosus, and 8 with a patent foramen ovale. Three early complications (2 ruptured sacs and 1 bleeding sac) and 1 late complication (gastric necrosis) occurred in the initial nonoperative period. In addition, 4 patients were treated for line sepsis, 1 patient for acute renal insufficiency, and 1 for aspiration pneumonia. Three patients required tracheostomy and were discharged with home ventilators. There were no complications associated with the use of silver sulfadiazine. Of the 22 patients, 16 have undergone delayed repair, 2 did not require repair, 1 is awaiting repair, 2 died before closure, and 1 was lost to follow-up. Delayed closure was achieved at a median age of 14 months (range, 2-28 months) and mean weight of 8.8 ± 3.3 kg. Four patients required implantation of mesh for definitive closure. Median postoperative length of stay was 4 days (range, 2-21 days). Postoperative complications included prolonged ileus, recurrent ventral hernia, and prolonged intubation. Overall mortality rate was 9.1%. One death occurred after diaphragmatic hernia repair, and 1 death was from overwhelming sepsis in the patient with a late gastric perforation. Conclusion The use of silver sulfadiazine dressing changes for initial nonoperative management of giant omphaloceles is a safe and effective bridge to delayed closure. We recommend this method as initial nonoperative management given the high incidence of associated cardiopulmonary malformations because it may facilitate enteral feeding, minimize respiratory compromise, and reduce morbidity and mortality.

ADHERENCE BETWEEN PLACENTA AND OMPHALOCELE SAC

The baby who had placental adherence between fetal site of placenta and omphalocele sac was presented. A 21 years old woman, gravida 2, parity 0, abortion 1 was admitted to the Selcuk University, Medical Faculty of Meram, Department of Obstetrics and Gynecologic, because of beginning the labor. Omphalocele was seen with ultrasonographie. Cesarean section was preferred according to desire of the family. A placental adherence between fetal site of placenta and omphalocele sac was detected during cesarean section. This adherence was divided and the baby was delivered. But baby died because of major cardiac anomalies. Anomalies such as omphalocel may be together with adherence between omphalocele sac and placenta or membranes.

Comparative studies on diagnosis between angiocardiography and surgery in isolated dextrocardia

目的 比较单发右位心的心血管造影与外科术后诊断,评价心血管造影诊断结果的准确性,探讨最佳的造影方法及投照体位.方法27例先天性单发右位心病例接受外科姑息性或根治手术治疗,回顾性分析其心血管造影资料(常规左、右室造影,其中8例行选择性心房造影,18例加行双斜位),根据先天性心脏病节段分析法原则,将其与外科术后诊断进行对比研究.结果心血管造影诊断与外科术后诊断结果对比显示22例诊断基本一致,5例不一致,包括1例解剖校正型大动脉错位误诊为功能校正型大动脉错位,1例完全性大动脉错位误诊为功能校正型大动脉错位,1例单心室误诊为右室双出口(合并心房不定位误诊为心房正位),2例解剖左室双出口误诊为功能校正型大动脉错位.结论单发右位心并复杂的心血管病畸形,因解剖复杂,在常规行双侧心室造影和正侧位造影基础上加行选择心房造影和双斜位造影,有利于达到准确诊断的目的。

Twenty years experience of surgical aortic valvotomy for critical aortic stenosis in early infancy☆

To examine early and long-term results of surgical aortic valvotomy in neonates and infants aged less than 3 months. A review of all 34 neonates (n=26) and young infants (n=8) aged 1-62 days undergoing primary open valvotomy for aortic valve stenosis between 1983 and 2003 was carried out. Associated major cardiac anomalies were endocardial fibroelastasis (n=8), aortic coarctation (n=3), subvalvular aortic stenosis (n=2), and ventricular septal defect (n=1). Risk factors for early mortality were estimated. Current information was available for 31 patients for a follow-up of 115+/-67 months. Kaplan-Meier method was used to estimate freedom from reintervention. Two neonates died early: operative mortality of 6% (2/34). Risk factors for early mortality were associated endocardial fibroelastosis, monocuspid aortic valve and impaired left ventricular function. No patient died late. Seven patients needed reintervention for re-aortic stenosis (n=5) or aortic insufficiency (n=2), i.e., re-valvotomy (n=3), valve replacement (n=2), Ross procedure (n=1), and balloon valvuloplasty (n=1). Freedom from reintervention was 85.1+/-6.9%, 78.0+/-9.35%, and 53.5+/-15.9% at 5, 10, and 15 years, respectively. Primary surgical aortic valvotomy in early infancy carries a low early and late mortality, a low occurrence of significant aortic regurgitation and a low early recurrence of aortic stenosis. In great majority of cases, reintervention can be delayed to allow implantation of an adult-sized prosthesis, when required.

1264: The role of Doppler ultrasound in obstetrics

1264: The role of Doppler ultrasound in obstetrics

Conjoined twins—past, present, and future

Background/Purpose Conjoined twins are rare and complex anomalies of the newborn. They require a highly experienced team and a center equipped to deal with such challenging anatomy. Method A review of 31 sets of conjoined twins was managed by one team over a 15-year period. The spectrum of the anomalies, management strategies, and outcomes will be analyzed with future recommendations. Results Ten sets of complete conjoined twins and two sets of parasitic conjoined twins were successfully separated. Tissue expanders and prosthetic meshes were not required. A separation of one set of craniopagus parasiticus conjoined twins was attempted, but abandoned owing to major vascular and brain sharing. The remaining 18 sets were not separable owing to major cardiac anomalies and/or sharing, and all died within several weeks of birth. Conclusion Careful planning, a multidisciplinary approach, rehearsal, and experience are important factors in dealing with conjoined twins. Tissue expanders and prosthetic meshes are not required in most cases. Selective abortion or fetal intervention may play a role in the future.