Biomedical subjects

L D Allan

Publications and source records attributed to L D Allan.

At least 19 recordsLinked to original sources

PubMed2006

Right aortic arch in the fetus.

OBJECTIVE: To examine our experience of the detection of a right aortic arch in the fetus over an 8-year period. METHODS: Between February 1998 and December 2005, all patients prospectively diagnosed with a right aortic arch at our center were identified from our database and the videotape reviewed. In addition, the videotapes of 300 normal and 110 abnormal arbitrarily selected fetal echocardiograms, as well as 123 cases of tetralogy of Fallot and nine of a common arterial trunk were reviewed. Data including indication for fetal echocardiography, gestational age at diagnosis, karyotype, nuchal translucency measurement and outcome were collected. RESULTS: A right aortic arch was diagnosed prospectively in 55 fetuses and in a further 20 on retrospective videotape review. There were 21 examples of isolated aortic arch and four thought to have a double arch. A right arch was found in association with additional intracardiac malformations in 50 cases. The detection rate of a right aortic arch increased over the study period. The majority of patients were referred for a suspicion of congenital heart disease on obstetric scanning. Mean gestational age at diagnosis was 21 weeks. The karyotype including 22q11 status was known in 45/75 cases. There were 23 confirmed karyotypic anomalies, 12 of which were 22q11 deletions, occurring in 2/25 of the isolated group and 10/48 of the complex group, with a further two complex cases that were likely to have had 22q11 microdeletions. There were 29 pregnancy interruptions, four intrauterine deaths, 31 live births, four neonatal deaths and three patients lost to follow-up. The remaining four pregnancies are continuing. Of the four with suspected double arch, three were confirmed postnatally. CONCLUSION: The diagnosis of a right-sided aortic arch can be made by fetal echocardiography, either as an isolated lesion or in association with other cardiac malformations, from as early as 12 weeks' gestation. It can be difficult to distinguish from a double arch. Its increasing incidence over time in our series probably indicates that the diagnosis was previously overlooked. Karyotyping in the absence of other abnormal findings may be unnecessary in every case, but establishment of 22q11 microdeletion status in those cases with other anomalies is important.

Aorta, Thoracic↗

PubMed2005

Fetal echocardiography in trisomy 18.

BACKGROUND: Previously reported pathological series suggest that cardiac malformations are universal in trisomy 18. We examined our experience of fetal echocardiography in trisomy 18 for comparison. METHODS: Of 255 fetuses with trisomy 18 detected in our centre between January 1999 and June 2004, 174 were evaluated using fetal echocardiography. Our results were compared to four previous echocardiographic and four autopsy series, comprising 89 and 110 patients, respectively. RESULTS: Of these 174 fetuses, 114 were examined between 10 and 14 weeks gestation and the remainder between 15 and 33 weeks. An increased nuchal translucency measurement was the reason for referral in most of the early cases and extracardiac anomalies in the remainder. Images were non-diagnostic in 12 cases (7%), all examined at <15 weeks gestation. Abnormal cardiac findings were detected in 118 of the remaining 162 fetuses (73%), including 15 with functional anomalies. The various heart malformations included ventricular septal defects, tetralogy of Fallot, left heart disease, and atrioventricular septal defects. In all series used for comparison, a similar diversity of disease was seen. In pathological series of trisomy 18, structural heart malformations were found in all cases, but some had lesions which would not be detectable echocardiographically in the fetus. CONCLUSION: Abnormal cardiac findings are detectable echocardiographically in the majority of cases of trisomy 18 examined during fetal life, but not in all. A wide spectrum of heart defects is seen. Diagnosis of heart malformations can be made reliably, even in the first trimester at the time of nuchal translucency measurement.

Chromosomes, Human, Pair 18↗

PubMed2005

Likelihood ratio for trisomy 21 in fetuses with tricuspid regurgitation at the 11 to 13 + 6-week scan.

OBJECTIVE: To determine the likelihood ratio for trisomy 21 in fetuses with tricuspid regurgitation at the 11 to 13 + 6-week scan. METHODS: Fetal echocardiography was carried out by specialist pediatric cardiologists in 742 singleton pregnancies at 11 to 13 + 6 weeks' gestation and pulsed wave Doppler was used to ascertain the presence or absence of tricuspid regurgitation. To avoid confusion with other adjacent signals, a strict definition of tricuspid regurgitation was used, in that it had to occupy at least half of systole and reach a velocity of over 80 cm/s. The fetal crown-rump length (CRL) and the nuchal translucency (NT) thickness were measured and the presence of any congenital heart abnormality noted. Follow-up of the pregnancy was carried out to determine the presence of chromosomal abnormalities. The likelihood ratio for trisomy 21 in fetuses with and without tricuspid regurgitation was determined. RESULTS: The tricuspid valve was successfully examined in 718 (96.8%) cases. Tricuspid regurgitation was present in 39 (8.5%) of the 458 chromosomally normal fetuses, in 82 (65.1%) of the 126 with trisomy 21, in 44 (53.0%) of the 83 with trisomy 18 or 13, and in 11 (21.6%) of the 51 with other chromosomal defects. The prevalence of tricuspid regurgitation was also associated with fetal CRL, delta NT and the presence of cardiac defects. Logistic regression analysis, irrespective of cardiac defects, demonstrated that in the chromosomally normal fetuses significant independent prediction of the likelihood of tricuspid regurgitation was provided by fetal delta NT (odds ratio (OR), 1.26; 95% CI, 1.34-1.41; P < 0.0001), while in trisomy 21 fetuses prediction was provided by CRL (OR, 0.94; 95% CI, 0.89-0.99; P = 0.021). The likelihood ratio for trisomy 21 for tricuspid regurgitation was derived by dividing the likelihood in trisomy 21 by that in normal fetuses. In the chromosomally normal fetuses, the prevalence of tricuspid regurgitation in those with cardiac defects was 46.9% and 5.6% in those without cardiac defects, and the likelihood ratio of tricuspid regurgitation for cardiac defects was 8.4. CONCLUSION: At 11 to 13 + 6 weeks' gestation, there is a high association between tricuspid regurgitation and trisomy 21, as well as other chromosomal defects. The prevalence of tricuspid regurgitation increases with fetal NT thickness and is substantially higher in those with, than those without, a cardiac defect.

Crown-Rump Length↗

PubMed2004

Doppler assessment of cardiac function at 11-14 weeks' gestation in fetuses with normal and increased nuchal translucency.

OBJECTIVE: To investigate cardiac dysfunction as a potential underlying mechanism for increased nuchal translucency (NT) in fetuses with chromosomal abnormality or heart defects. METHODS: Myocardial performance index (MPI) and atrioventricular valve E/A ratios for both sides of the heart were measured by Doppler echocardiography in fetuses at 11-14 weeks' gestation. The study groups consisted of 159 normal control fetuses, 199 otherwise normal fetuses but with increased NT > or = 4 mm, 142 fetuses with trisomy 21, 58 with trisomy 18, 19 with trisomy 13, 37 with Turner's syndrome and 24 with isolated heart defects. Groups were compared using Student's t-test and confidence intervals for differences between groups were calculated. RESULTS: Otherwise normal fetuses with increased NT showed no difference in any of the cardiac Doppler parameters from normal controls. Mean E/A ratio was slightly but significantly increased in trisomy 21 fetuses compared with normal controls (0.604 vs. 0.578 on the right, P = 0.011; 0.581 vs. 0.542 on the left, P = 0.0001). E/A ratio was not significantly different between any of the other groups and the normals but there was a small increase in absolute E-wave velocity in trisomy 18 fetuses. MPI was significantly decreased in trisomy 21 fetuses, (0.330 vs. 0.378, P = 0.002 on the left) and also in Turner's syndrome fetuses (0.301 vs. 0.352 on the right, P = 0.04; 0.320 vs. 0.378 on the left, P = 0.034) implying better performance, but not in the other groups. CONCLUSIONS: The magnitude and/or direction of the differences shown do not support a major role for cardiac functional abnormality in the development of NT. Important cardiac dysfunction could not be demonstrated in association with increased NT in normal or abnormal fetuses.

Chromosome Aberrations↗

PubMed2003

Turner's syndrome in fetal life.

OBJECTIVE: To compare the incidence and type of heart disease found in association with 45X karyotype in fetal life with postnatal life and to examine the outcome after fetal diagnosis. METHODS: Fifty-three fetuses with a 45X karyotype were examined echocardiographically over a 4-year period between 1999 and 2002. Of these, 47 were referred because of increased nuchal translucency (NT). RESULTS: A cardiac abnormality was detected in 33/53 (62.2%) fetuses. The most common diagnosis was coarctation of the aorta in 24/53 (45.3%) fetuses, followed by the hypoplastic left heart syndrome (HLHS) in 7/53 (13.2%) fetuses. The mean NT was significantly higher in fetuses with a heart defect than in those with normal echocardiography. Termination of pregnancy was carried out in 45/53 (84.9%) fetuses and intrauterine death occurred in six cases. Two of four fetuses with a mosaic karyotype are currently alive. CONCLUSION: Turner's syndrome is associated with a higher incidence of heart defects detected prenatally when compared to postnatal reports. The commonest associated heart defects detected prenatally are HLHS and coarctation of the aorta, in contrast to postnatal life where a bicuspid aortic valve is the most common diagnosis. The typical intrauterine presentation of Turner's syndrome with a markedly increased NT or with hydrops and with a typical 45X karyotype has an extremely poor prognosis for intrauterine survival.

Aortic Coarctation↗

PubMed2003

Tricuspid regurgitation in the diagnosis of chromosomal anomalies in the fetus at 11-14 weeks of gestation.

OBJECTIVE: To analyse patient data to elucidate the apparent association between an abnormal karyotype and tricuspid regurgitation found during fetal echocardiography at early gestations. SETTING: Tertiary referral centre for fetal medicine and cardiology. METHODS: Fetuses between 11 and 14 weeks' gestation were selected for detailed echocardiography. Referral reasons were increased nuchal translucency, a suspected cardiac or extracardiac malformation, and a family history of cardiac malformation. INTERVENTION: The fetus was imaged transabdominally. The four chamber view, outflow tracts, arterial duct, and aortic arch were assessed on cross sectional imaging and colour flow mapping. Pulsed Doppler of the atrioventricular valves was recorded if possible. Subsequently, the fetal karyotype was ascertained by chorionic villous sampling. RESULTS: Pulsed Doppler recording of the tricuspid valve was obtained for 262 fetuses. Tricuspid regurgitation was present in 70 (27%) of these, of whom 58 (83%) proved to have karyotype anomalies. In contrast, 68 (35%) of those without tricuspid regurgitation were found to have karyotype anomalies (95% confidence interval 36% to 59%, p < 0.001). Structural heart defects were detected in 34 of the 58 (59%) with tricuspid regurgitation and in 22 (32%) of those without. The chromosome defect most frequently found to be associated with tricuspid regurgitation was trisomy 21, but all types of karyotypic anomalies were seen in association. CONCLUSION: A careful search for tricuspid regurgitation is an important aspect of the evaluation of the early fetus, as this is frequently a marker for chromosomal defects even in the absence of structural heart disease.

Chorionic Villi Sampling↗

PubMed2003

Proposal for a new definition of congenital complete atrioventricular block.

The classic old definition of congenital heart block by Yater (1929) is still generally accepted: 'Heart block established in a young patient. There must be some evidence of the existence of the slow pulse at a fairly early age and absence of a history of any infection which might cause the condition after birth: notably diphtheria, rheumatic fever, chorea and congenital syphilis'. However, other definitions are used. We systematically reviewed 1825 cases from 38 separate studies. We conclude that complete AV blocks detected in utero in the absence of structural abnormalities differ from blocks detected later in life with respect to pathogenesis (they are generally associated with maternal anti-Ro/SSA antibodies), poorer childhood prognosis, increased risk of developing late-onset dilated cardiomyopathy, different maternal clinical features and increased risk of recurrence in future pregnancies. For these reasons we propose a new modern definition of congenital complete AV block which might be acceptable to cardiologists, rheumatologists, pediatricians and obstetricians: 'an AV block is defined as congenital if it is diagnosed in utero, at birth or within the neonatal period (0-27 days after birth)'.

Autoantibodies↗

PubMed2002

Fetal cardiac abnormalities identified prior to 14 weeks' gestation.

OBJECTIVE: An increasing number of patients are presenting at early gestational age as being at high risk for congenital heart disease, as a result of ultrasound screening by nuchal translucency. The feasibility and accuracy of fetal echocardiography was assessed in a series of pregnancies studied before 14 weeks' gestation. METHODS: Echocardiography was attempted in 478 fetuses of crown-rump length 40.0-85.0 mm (median, 60.3 mm) with increased nuchal translucency, suspected abnormalities on routine scan or a family history of heart defect. The findings were related to results of autopsy, karyotyping, later scans and postnatal follow-up. RESULTS: Satisfactory images were obtained transabdominally in 402/478 (84.1%) and transvaginally in a further 13 patients. Cardiac defects were confidently identified in 60 fetuses and abnormalities of uncertain significance (isolated ventricular or great artery disproportion, or tricuspid regurgitation) were observed in a further 49. Defects were suspected in an additional 20 fetuses, and 286 were passed as normal. The karyotype was subsequently demonstrated to be abnormal in 70/286 (24.5%) fetuses with normal echocardiograms, and in 94/129 (72.9%) with abnormal or suspicious cardiac findings. Validation of the scan findings was possible in 241 fetuses. Normal heart structure was confirmed in 204 fetuses, and previously unsuspected cardiac abnormalities revealed in nine. Heart defects were verified in 28 fetuses, but five of these had important additional findings. There were false positive findings in three fetuses. CONCLUSIONS: Fetal echocardiography is feasible prior to 14 weeks' gestation. Cardiac defects, when present, may be identified or suspected in the majority of cases. In the risk group studied, heart defects were frequently a manifestation of chromosomal abnormality.

Chromosome Aberrations↗

PubMed2001

Patterns of pulmonary venous flow in the fetus with disease of the left heart.

A restrictive atrial septal defect is a risk factor for mortality in some forms of left heart disease, and can be predicted from the patterns of flow in the pulmonary veins in fetal life. Our objective was to document the patterns of pulmonary venous flow of blood in different forms of left heart disease in the fetus. The pulmonary venous flow profile was documented in 61 cases. The systolic, diastolic, and reverse waves were measured and compared to normal. Profiles of venous flow suggestive of restriction at the atrial septum were found in 32 of 61 cases using the ratio of systolic to diastolic waves, and 26 cases using the height of the reverse wave. A restrictive atrial septal defect was found most commonly in fetuses with critical aortic stenosis or the hypoplastic left heart syndrome, and was less frequently seen in the setting of mitral atresia or coarctation. In order to plan appropriate postnatal management, examination of the profiles of pulmonary venous flow is an essential part of the evaluation of any form of left heart disease detected in the fetus.

Blood Flow Velocity↗

PubMed2001

The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus.

BACKGROUND: Infants with isolated totally anomalous pulmonary venous return often present severely decompensated, such that they are at high risk for surgical repair. On the other hand, if surgical repair can be safely accomplished, the outlook is usually good. Thus prenatal diagnosis would be expected to improve the prognosis for the affected child. OBJECTIVE: To describe the features of isolated totally anomalous pulmonary venous drainage in the fetus. DESIGN: Four fetuses with isolated totally anomalous pulmonary venous connection were identified and the echocardiographic images reviewed. Measurements of the atrial and ventricular chambers and both great arteries were made and compared with normal values. SETTING: Referral centre for fetal echocardiography. RESULTS: There were two cases of drainage to the coronary sinus, one to the right superior vena cava, and one to the inferior vena cava. Right heart dilatation relative to left heart structures was a feature of two cases early on, and became evident in some ratios late in pregnancy in the remaining two. CONCLUSIONS: Ventricular and great arterial disproportion in the fetus can indicate a diagnosis of totally anomalous pulmonary venous connection above the diaphragm. However, in the presence of an atrial septal defect or with infradiaphragmatic drainage, right heart dilatation may not occur until late in pregnancy. The diagnosis of totally anomalous pulmonary venous drainage in fetal life can only be reliably excluded by direct examination of pulmonary venous blood flow entering the left atrium on colour or pulsed flow mapping.

Echocardiography, Doppler, Color↗

PubMed2000

World experience of percutaneous ultrasound-guided balloon valvuloplasty in human fetuses with severe aortic valve obstruction.

Prenatal alleviation of severe fetal aortic valve obstructions by percutaneous ultrasound-guided balloon valvuloplasty has been performed to improve the fate of affected fetuses. The purpose of this study was to analyze the current world experience of these procedures in human fetuses. Data from 12 human fetuses were available for analysis. The mean gestational age at intervention was 29.2 weeks (range 27 to 33). The mean time period between initial presentation and intervention was 3.3 weeks (range 3 days to 9 weeks). Technically successful balloon valvuloplasties were achieved in 7 fetuses, none of whom had an atretic valve. Only 1 of these fetuses remains alive today. Of the 5 remaining technical failures, 1 patient with severe aortic stenosis underwent successful postnatal intervention and remains alive. Six patients who survived prenatal intervention died from cardiac dysfunction or at surgery in the first days or weeks after delivery. Four fetuses died early within 24 hours after the procedure, 1 from a bleeding complication, 2 from persistent bradycardias, and 1 at valvotomy after emergency delivery. Thus, the early clinical experience of percutaneous ultrasound-guided fetal balloon valvuloplasty in human fetuses with severe aortic valve obstruction has been poor due to selection of severe cases, technical problems during the procedure, and high postnatal operative mortality in fetuses who survived gestation. Improved patient selection and technical modifications in interventional methods may hold promise to improve outcome in future cases.

Aortic Valve↗

PubMed2000

The outcome of fetal congenital heart disease.

Series reporting congenital heart disease detected in utero all indicate a different spectrum and severity of disease than is seen in postnatal series, with a tendency toward more severe disease with a poorer outcome. This is largely because of the manner in which cases are selected for fetal echocardiography. As most forms of heart disease occur in otherwise normal pregnancies with no high risk features, detection of these cases is dependent on the skill of the ultrasonographer performing general obstetric scanning. Although detection of even major malformations seen in the four chamber view, is still less than perfect, it is improving. In the future, it is expected that malformations will be detected in earlier pregnancy and examination will include evaluation of great artery structure. This will give more parents the option of termination of pregnancy in complex disease. Alternatively, in continuing pregnancies, the outcome for the affected neonate will be improved by ideal perinatal care.

Decision Making↗

PubMed2000

A practical approach to fetal heart scanning.

Evaluation of the fetal heart can be readily incorporated into the obstetric ultrasound examination and need not add more than a few minutes to the examination. Correct analysis of the four-chamber view and both outflow tracts will exclude the majority of serious congenital heart disease. Where cardiac malformations are identified during pregnancy, parents should be referred to a pediatric cardiologist with expertise and experience of fetal heart scanning for further counseling. This allows for management of the pregnancy to be tailored to the parents wishes and the type of malformation found.

Diagnosis, Differential↗

PubMed2000

Intracardiac pressures in the human fetus.

OBJECTIVE: To obtain normal values for intracardiac pressures in the human fetus. DESIGN: Intracardiac pressures were measured directly in the four chambers of the human fetal heart during clinically indicated invasive obstetric procedures. SETTING: Department of fetal medicine in a tertiary referral centre. PATIENTS: 39 fetuses between 16 and 29 weeks of gestation. RESULTS: The ventricular waveforms obtained were similar to those found in postnatal life. There was an increase in ventricular systolic and end diastolic pressures with advancing gestation. There was no difference between left and right ventricular pressures. Atrial pressures were equal and remained constant in the gestational age range studied. CONCLUSIONS: Fetal cardiovascular pressure measurements in the normal fetus assist in understanding the fetal circulation, and provide a basis for the assessment of cases of congenital heart disease that may be amenable to intrauterine treatment.

Female↗

PubMed1999

Atrioventricular septal defect in the fetus.

OBJECTIVE: I sought to study the spectrum of disease and outcome seen with atrioventricular septal defect in fetal life and to analyze the current rate of prenatal detection of this malformation. STUDY DESIGN: All cases of atrioventricular septal defect detected prenatally or in infants were ascertained between 1994 and 1998. RESULTS: An atrioventricular septal defect was detected in 49 fetuses and in a further 63 infants during this 5-year period. Among the 49 fetuses the atrioventricular septal defect was the only heart malformation in 18 (with Down syndrome in 13/18) and associated with a heterotaxia syndrome in 22, left ventricular malformations in 8, and the tetralogy of Fallot in 1. There were 23 survivors among the 49 fetuses. CONCLUSION: Despite the fact that an atrioventricular septal defect is detectable by 4-chamber view screening and this view is recommended as part of obstetric ultrasonographic evaluation, the rate of detection of this lesion prenatally is currently <50%. The recognition of this lesion has important implications for the fetus.

Down Syndrome↗

PubMed1999

Pattern of pulmonary venous blood flow in the hypoplastic left heart syndrome in the fetus.

OBJECTIVE: To determine whether restriction at the atrial septum in the newborn with hypoplastic left heart syndrome can be predicted accurately by examining the pattern of pulmonary venous flow in the fetus. A restrictive atrial septum can contribute to haemodynamic instability before surgery for this lesion and has been associated with an increased mortality. DESIGN: Pulmonary venous pulsed Doppler tracings were compared between fetuses with hypoplastic left heart syndrome and controls. The size of the atrial septal defect on the postnatal echocardiogram was graded according to the degree of restriction. Pulsed Doppler tracings of pulmonary venous blood flow were obtained in 18 fetuses with left atrial outflow atresia and compared with 77 controls, adjusted for gestational age. Postnatal echocardiograms were available for analysis in 13 of 18 neonates. SETTING: A tertiary referral centre for fetal cardiology and paediatric cardiac surgery. RESULTS: Fetuses with hypoplastic left heart syndrome were different from controls in all pulmonary vein indices measured. As assessed from the postnatal echocardiogram, there were seven fetuses with a restrictive atrial septum. In these fetuses, the systolic flow velocity (p < 0.01), S/D ratio (p < 0.01), and peak reversal wave (p < 0.001) in the pulmonary vein tracing showed a good correlation with the degree of restriction. CONCLUSIONS: The Doppler pattern of pulmonary venous flow in the fetus with hypoplastic left heart syndrome appears to be a reliable predictor of restriction of the atrial septum in the neonate. This may help in the immediate post-delivery management of these infants before surgery.

Analysis of Variance↗

PubMed1998

Usefulness of preoperative echocardiography in predicting left ventricular outflow obstruction after primary repair of interrupted aortic arch with ventricular septal defect.

Residual left ventricular outflow tract (LVOT) obstruction is a significant problem after repair of interrupted aortic arch (IAA) and ventricular septal defect. Resection of subaortic tissue at the time of primary repair, however, is associated with increased morbidity and mortality. We reviewed the preoperative echocardiograms and the postoperative clinical course and echocardiograms of 23 consecutive patients who underwent primary repair of IAA without widening of the subaortic region. Nine patients (39%) developed significant LVOT obstruction (pressure gradient >40 mm Hg). LVOT obstruction was noted postoperatively in 7 of 9 patients by 1 month, 8 of 9 by 2 months, and 9 of 9 by 1 year. On retrospective analysis of the preoperative echocardiograms, the indexed cross-sectional area of the LVOT, the subaortic diameter index, and the subaortic diameter Z score were all significantly smaller in those requiring reintervention (p <0.04, p <0.05, p <0.05, respectively). Of these, indexed cross-sectional area had the least reproducibility and subaortic diameter index the most (coefficient of variation of 26.3% vs 11.2%). In conclusion, most patients who develop significant LVOT obstruction after repair of IAA do so within 1 month of operation. Although subaortic indexed cross-sectional area is the most sensitive predictor of LVOT obstruction after primary repair of IAA, other more simple standardized measurements of the subaortic diameter were comparably predictive and had better reproducibility.

Adolescent↗

PubMed1998

Outcome after prenatal diagnosis of the hypoplastic left heart syndrome.

OBJECTIVE: To derive accurate survival figures in the current surgical era for counselling in early pregnancy after the diagnosis of fetal hypoplastic left heart syndrome. SETTING: A tertiary referral centre for paediatric cardiology and cardiac surgery. DESIGN: A retrospective study of the outcome in all cases of hypoplastic left heart syndrome presenting in fetal life between mid-1993 and the end of 1996. PATIENTS: The diagnosis was made in 30 fetuses. In four of 12 identified before 24 weeks' gestation the mothers chose to terminate the pregnancy. There was an intention to treat in 24 of the remaining fetuses. MAIN OUTCOME MEASURE: Survival to six months of postnatal life. RESULTS: Of the 24 infants, five were not offered Norwood stage 1 because of trisomy 18 (n = 2), unfavourable cardiac anatomy (n = 2), or neurological impairment (n = 1). One further infant did not survive to cardiac surgery after gastrointestinal surgery. Of the remaining 18 patients, eight had features that were considered to increase the risk of surgical repair. Of the 18 patients who underwent Norwood stage 1, there were nine survivors. There was a survival rate of 70% in infants undergoing surgery with no complicating features, a 50% survival of the all surgical candidates, and 37.5% survival from an intention to treat position. CONCLUSIONS: At the initial diagnosis of fetal hypoplastic left heart syndrome, the overall survival appears to be less than 40%. Evaluation must include detailed extracardiac and intracardiac assessment to predict the risks of surgical treatment. Prenatal counselling can be modified as pregnancy advances, depending on the detection or exclusion of complicating factors.

Female↗