Parvovirus-Mediated Fetal Cardiomyopathy With Atrioventricular Nodal Disease

Acute parvovirus B19 infection (API) in pregnancy has been associated with fetal anemia and hydrops fetalis. Direct myocardial damage from API in a fetus and an infant has been described. This report presents a case of fetal second-degree heart block and cardiomyopathy secondary to API. A 19-year-old G4P1112 (gravida 4 para 2 with 1 term delivery, 1 preterm delivery, 1 termination, and 2 living children) was referred at 20 weeks gestation for fetal bradycardia. A 2:1 atrioventricular block was identified by fetal echocardiography at 23 weeks. Hydrops developed at 25 weeks. Amniocentesis and percutaneous umbilical blood sampling demonstrated API. At 31 weeks, the patient presented with preterm labor and delivered a viable female infant, who died of poor cardiac function and arrhythmia on the first day of life. In addition to fetal anemia and hydrops fetalis, API in pregnancy may cause direct fetal myocardial damage and conduction system disease.     

Evaluation and prognosis of fetal arrhythmia]

From January 1986 to August 1990, a fetal arrhythmia was diagnosed in 97 pregnant women referred to our unit. Atrial or ventricular extrasystoles were the most frequent rhythm disturbance encountered (71%). They were always well tolerated and all disappeared during the perinatal period. Tachycardia was found in 16 fetuses; 6 had a supraventricular reentrant tachycardia, 6 an atrial chaotic tachycardia, 1 an ectopic atrial tachycardia and the remaining 3 an atrial flutter. A congenital heart malformation was present in 4 fetuses. The arrhythmia induced hydrops fetalis in 25% of cases. One hydropic fetus died in utero and another needed premature delivery. Bradycardia was diagnosed in 12 cases, 3 had benign atrial blocked extrasystoles, 3 others sinus bradycardia due to fetal distress and 6 atrio-ventricular block. Atrio-ventricular block was associated with congenital malformation in 4 cases (66%). All these fetuses were hydropic and died. The 2 fetuses without cardiac malformation tolerated well their bradycardia during fetal life. Fetal arrhythmia is not rare, but in most cases is benign. Sustained tachycardia requires prompt treatment, because when hydrops fetalis appears the prognosis is worse. The major prognostic factor for atrio-ventricular block is the association with a cardiac malformation.     

Fetal arrhythmias: Intrauterine diagnosis,treatment and prognosis

Fetal echocardiography can provide useful information for the evaluation of fetal arrhythmias. Between 1980 and 1993, 44 fetuses with arrhythmias were diagnosed in utero at 12 and 40 weeks of gestation in Kurume University Hospital. Fetal bradycardia, tachycardia and ectopic beats were revealed in 17, seven and 20 fetuses, respectively, and their clinical features and prognosis were evaluated. In the 17 fetuses with bradycardia, eight were associated with congenital heart defect, and six of these developed to fetal hydrops. Of the 17 fetuses, four died in utero, one was terminated, and six died after birth. The other six cases survived. Three of these had a pacemaker implanted after birth. In the seven fetuses with tachycardia, transplacental anti-arrhythmic drugs were administered in five cases and conversion of the arrhythmia was achieved in four. None of the cases was associated with any congenital heart defect, and none died. Three infants had paroxysmal tachycardia postnatally. In the 20 fetuses with ectopic beats, arrhythmia was observed postnatally in 10, but all of these were resolved within 3 months after birth. Fetal bradycardias carried a poor prognosis in most cases and further studies are required to establish effective treatment. Some cases of fetal tachycardia developed recurrent tachycardia postnatally. Close follow-up of the newborn is therefore necessary.     

Truncus Arteriosus: Diagnostic Accuracy,Outcomes, and Impact of Prenatal Diagnosis

Limited data exist on the impact of prenatal diagnosis and outcomes of fetal truncus arteriosus (TA). We sought to assess prenatal diagnostic accuracy and prenatal outcomes in fetuses with TA and compare postnatal outcomes in neonates with prenatally and postnatally diagnosed TA. Records were reviewed for patients diagnosed with TA in utero or at ≤60 days of life from 1992 to 2007. Forty-three (32%) of 136 TA patients had prenatal diagnosis. Five patients with TA were prenatally misdiagnosed, and 5 with other congenital heart diseases were misdiagnosed with TA prenatally. Of 28 fetuses diagnosed at <24 weeks gestation, 19 (68%) did not survive to birth because of spontaneous fetal death (n = 2) or because of elective termination (n = 17). Pregnancy termination was not more likely for fetuses with extracardiac anomalies. Of 19 live-born patients with correct prenatal diagnosis of TA, 2 (11%) died before surgery, and 4 (24%) died in the early postoperative period. All patients who died presurgically had been diagnosed prenatally. Overall, early postoperative mortality was 10%. Prenatal diagnosis of TA remains challenging and is associated with a high rate of elective termination. Fetal diagnosis was associated with younger age at repair but was not associated with improved neonatal survival.     

Is Left Ventricular Noncompaction in Children Truly an Isolated Lesion?

Left ventricular noncompaction (LVNC) is a form of cardiomyopathy resulting from a disorder of endomyocardial morphogenesis. It has been associated with significant morbidity and mortality. The aim of this study was to characterize associated cardiac findings in children with LVNC and to identify risk factors associated with increased mortality. From our echocardiography database, we identified 46 patients diagnosed with LVNC between December 1999 and February 2005. The mean age at presentation was 3.6 ± 5.6 years, and the mean duration of follow-up was 1.9 ± 2.1 years. Left ventricular ejection fraction was decreased in 24 patients (52%; mean 39.5% ± 13.1%). Thirty-six patients (78%) had associated cardiac lesions, including atrial septal defect (n = 16 [35%]), ventricular septal defect (n = 17 [37%]), patent ductus arteriosus (n = 14 [30%]), and Ebstein’s anomaly (n = 5 [11%]). Electrocardiogram abnormalities were found in 80% of patients; most commonly they included left (n = 15 [43%]) and right ventricular hypertrophy (n = 19 [54%]). Documented arrhythmias included ectopic atrial rhythm (n = 2), junctional rhythm (n = 2), supraventricular tachycardia (n = 2), and ventricular tachycardia (n = 1). Overall mortality was 20%, and there was no association with ejection fraction, morphologic defect, or arrhythmia. Mean age at diagnosis in survivors (4.5 ± 6.1 years) was higher than nonsurvivors (0.4 ± 0.7 years) (p < 0.0001). LVNC is a rarely isolated form of cardiomyopathy, and it is associated with significant additional cardiac abnormalities. Although it does not have an invariably fatal course, early presentation in infancy does carry an increased risk of mortality.     

The Myocardium of Fetuses with Endocardial Fibroelastosis Contains Fewer B and T Lymphocytes than Normal Control Myocardium

The observation that endocardial fibroelastosis (EFE) can result from an immune response to maternal autoantibody deposition in the fetal myocardium raises the possibility that the fetal immune system may contribute to the pathogenesis of idiopathic EFE and dilated cardiomyopathy (DCM). This study sought to characterize myocardial immune cell presence in fetuses and neonates with idiopathic EFE + DCM, in those with EFE + structural heart disease, and in normal control subjects. Paraffin tissue sections from fetuses identified from the pathology database were stained for B cell, T cell, macrophage, and general hematopoietic cell surface markers. Of the 14 fetuses included in the study, 5 had EFE + DCM, 4 had EFE + structural heart disease, and 5 were normal control fetuses. The EFE + DCM group had fewer B cells than the control group (0.15 vs. 0.44 cells/mm²; p = 0.005). The EFE + heart disease group had both fewer B cells (0.18 vs. 0.44 cells/mm²; p = 0.08) and T cells (0.29 vs. 0.80 cells/mm²; p = 0.04) than the control group. The CD4/CD8 ratio was similar in the EFE + DCM and EFE + heart disease groups (1.0 vs. 0.9; p = 0.17) but higher in the EFE + DCM group than in the control group (0.9 vs. 0.3; p = 0.03). The myocardium of fetuses with EFE contains fewer B and T lymphocytes than normal control fetuses.     

Pathophysiology, management, and outcomes of fetal hemodynamic instability during prenatal cardiac intervention

Prenatal cardiac intervention (PCI) may favorably alter the in utero course of some congenital heart defects. In our preliminary experience with PCI, fetal hemodynamic instability (FHI) characterized by bradycardia and ventricular dysfunction was common. This study evaluated the pathophysiology, management, and short-term outcomes of FHI during PCI for aortic stenosis with evolving hypoplastic left heart syndrome (HLHS), HLHS with restrictive atrial septum, pulmonary atresia with intact ventricular septum, and hydrops due to structural heart disease. From 2000 to 2006, 83 fetuses underwent PCI, with ventricular access in 63, atrial access in 17, and both in three. FHI occurred in 37 fetuses (45%). FHI was associated with transventricular PCI (all but one case of FHI; p < 0.001) and large hemopericardium (n=9; p=0.07). Prolonged FHI was associated with severe ventricular distortion during ventricular puncture (p = 0.06). FHI was treated with resuscitation medications in 31 of 37 fetuses and resolved in all 37. Five fetuses died within 1 d of PCI: four had FHI and one had a massive hemopericardium. FHI is common and clinically important during transventricular PCI and may be caused by a ventricular reflex or reduced cardiac output from cardiac distortion during ventricular puncture. Hemopericardium may be causative in a subset of fetuses.     

Postnatal Outcome in Patients With Fetal Tachycardia

The diagnosis and management of prenatal tachyarrhythmias is well established; however, the postnatal course and outcomes are not. The purpose of our study was to review the natural history of patients with fetal tachycardia, determine the incidence of postnatal arrhythmias, and determine whether there are factors to predict which fetuses will develop postnatal arrhythmias. A retrospective chart review of patients with fetal tachyarrhythmias investigated at British Columbia Children’s and Women’s Hospitals between 1983 and 2010 was conducted. Sixty-nine mother–fetus pairs were eligible for the study. Fifty-two had fetal supraventricular tachycardia, and 17 had fetal atrial flutter. Conversion to sinus rhythm occurred prenatally in 52 % of patients. Postnatal arrhythmia occurred in two thirds of patients, with 82 % of those cases occurring within the first 48 h of life. Hydrops fetalis, female sex, and lack of conversion to sinus rhythm was predictive of postnatal arrhythmia (P = 0.01, P = 0.01, and P = 0.001, respectively). Conversion to sinus rhythm prenatally did not predict postnatal arrhythmia. Median duration of treatment was 9 months. Two postnatal deaths of unknown etiology occurred. Two thirds of all patients with prenatal tachycardia will develop postnatal arrhythmia. Prenatal factors that predict postnatal arrhythmia include hydrops, sex, and whether or not conversion to sinus rhythm occurred prenatally. The majority of patients with postnatal arrhythmia present within 48 h of life, which has clinical implications for monitoring. Postnatal outcome is generally very good with most patients being weaned off medication in 6–12 months.     

Cisapride reduces neonatal postoperative ileus: randomised placebo controlled trial

AIM—To assess the efficacy of cisapride in reducing ileus persisting to the tenth postoperative day after neonatal abdominal surgery.METHODS—A prospective, randomised, double blind trial comparing rectal cisapride (l.4-2.3 mg/kg/day) with placebo over seven days was undertaken in 33 neonates.RESULTS—Seven of 12 (58%) patients receiving placebo and eight of 11 (73%) receiving cisapride achieved a first sustained feed during treatment. Of those receiving cisapride, the first sustained feed occurred at 2.3 days (SEM 0.6) compared with 4.7 days (SEM 0.8) with placebo. By the seventh day the mean daily net enteral balance was 69 (SEM 18) ml/kg in the cisapride subgroup and 17 (SEM 8) ml/kg for those receiving placebo. Stool was passed on 6.3 (SEM 0.4) treatment days in the cisapride subgroup compared with 4.1 (SEM 1.0) treatment days in the placebo subgroup.CONCLUSION—Cisapride is effective in neonates with a prolonged ileus after abdominal surgery.     

Congenital heart block in neonatal lupus: the pediatric cardiologist's perspective

Clinical presentation. Congenital heart block (CHB) in the absence of major structural abnormalities is associated with maternal antibodies to Ro (SS-A) and La (SS-B). CHB is most commonly diagnosed between 18 and 24 wk of gestation, and may be first, second or third degree (complete). Mortality approaches ∼20%, and most surviving children require pacemakers. Affected infants may develop cardiomyopathy. Abnormalities in the skin, liver and blood of neonates are also associated with anti-Ro/La antibodies, and are usually self-limiting; these manifestations and CHB are collectively referred to as neonatal lupus syndromes (NLS).Investigation of pathogenesis. Recent studies demonstrate that Ro/La ribonucleoproteins appear on the surface of apoptotic fetal cardiocytes and are recognized by their cognate antibodies, promoting an inflammatory response. Mice immunized with Ro/La proteins have offspring with conduction abnormalities.In vitro, human serum and IgG with anti-Ro/La antibodies affect the conducting properties of isolated animal heart tissue.Diagnostic problems. If fetal bradycardia is identified, a 2-dimensional and M-mode fetal echocardiographic and Doppler ultrasound should be obtained to determine whether there is an atrial arrhythmia or atrioventricular (AV) block, and to what degree, and whether there are major structural abnormalities of the heart. The mother’s serum should be tested by ELISA for anti-Ro and/or anti-La antibodies.Therapeutic options. To date, only anecdotal and retrospective evidence guidesin utero therapy of CHB. A prospective trial is currently underway to evaluate the efficacy of maternal oral dexamethasone in treating newly identified first, second or third degree block. Established third-degree block appears to be irreversible. Dexamethasone and sympathomimetics may be of some benefit in treating hydrops fetalis. In pregnant women with anti-Ro/La antibodies, prophylactic therapy is not indicated but serial echocardiographic analysis is strongly recommended, with emphasis on the mechanical PR interval to identify a reversible block.Conclusion. CHB occurs in ∼1–5% of pregnancies in mothers with antiRo/La antibodies, independent of the mother’s disease status, and in ∼15–20% of pregnancies following the birth of a child with NLS. Treatment of CHB identifiedin utero is not established but guidelines are provided. Serial echocardiographic monitoring of high-risk pregnancies, using the mechanical PR interval to identify first degree block, may afford the earliest opportunities for therapeutic intervention     

Perinatal Outcome in Fetuses with Heterotaxy Syndrome and Atrioventricular Block or Bradycardia

Congenital atrioventricular (AV) block is commonly associated with heterotaxy syndrome; together they have reportedly low survival rates (10–25 %). However, information about perinatal outcome and predictors of non-survival after prenatal diagnosis of this association is scarce. Therefore, we studied fetuses with heterotaxy syndrome and bradycardia or AV-block diagnosed between 1995 and 2011, and analyzed pre and post-natal variables. The primary outcome was death and the secondary outcome was pacemaker placement. Of the 154 fetuses with heterotaxy syndrome, 91 had polysplenia syndrome, 22/91(24 %) with bradycardia or AV-block. Thirteen (59 %) patients had sinus bradycardia at diagnosis, 8 (36 %) complete AV block, and 1 (5 %) second-degree AV-block. Three patients elected for termination of pregnancy (3/22, 14 %), 4 had spontaneous fetal demise (4/22, 18 %), and 15 (15/22, 68 %) were live-born. Of the fetuses with bradycardia/AV-block, 30 % presented with hydrops, 20 % had ventricular rates <55 beats/min, and 10 % had cardiac dysfunction. Excluding termination of pregnancy, 15/19 fetuses (79 %) survived to birth. Among the 15 live-born patients, 4 had bradycardia and 11 had AV-block. A further 3 patients died in infancy, all with AV-block who required pacemakers in the neonatal period. Thus, the 1-year survival rate, excluding termination of pregnancy, was 63 % (12/19). Of the remaining 12 patients, 9 required pacemaker. Predictors of perinatal death included hydrops (p < 0.0001), ventricular dysfunction (p = 0.002), prematurity (p = 0.04), and low ventricular rates (p = 0.04). In conclusion, we found a higher survival rate (63 %) than previously published in patients with heterotaxy syndrome and AV block or bradycardia diagnosed prenatally. Hydrops, cardiac dysfunction, prematurity and low ventricular rates were predictors of death.     

Changes in oxygenation and pulmonary haemodynamics in preterm infants treated with inhaled nitric oxide

AIM—To investigate changes in various cardiorespiratory variables with inhaled nitric oxide (NO), as part of a randomised controlled trial.METHODS—Infants were treated with inhaled NO for 72 hours. Changes in oxygenation were assessed using the oxygenation index (OI). Serial changes in pulmonary artery pressure (PAP) were assessed using the Doppler derived acceleration time to right ventricular ejection time ratio (AT:RVET). Doppler measurements of right ventricular output, pulmonary blood flow, and systolic PAP was performed in a subset of infants.RESULTS—Twenty infants received inhaled NO and 22 acted as controls. Infants were treated at a median dose of 5 (range 5 to 20) ppm. There was a fall in median OI by 17% in treated infants within 30 minutes of treatment. The fall in OI in treated infants was significantly different from the response in controls until 96 hours. Infants treated with inhaled NO showed a rapid response with a median rise in AT:RVET of 0.04 (range ?0.06 to 0.12) within 30 minutes. The change in AT:RVET was significantly different from controls until 4 hours. Median systolic PAP also fell in treated infants by 6.1 (range ?14.4 to ?4.4) mm Hg within 1 hour. Changes in OI were significantly associated with changes in PBF (r = 0.44), but not with changes in AT:RVET.CONCLUSION—Treatment with inhaled NO rapidly improves oxygenation and lowers PAP in preterm infants. However, these effects are transient and treatment does not influence long term outcome.     

Congenital complete heart block in the fetus: hemodynamic features, antenatal treatment, and outcome in six cases

Congenital heart block (CHB) can result in intrauterine cardiac failure leading to fetal or neonatal loss. To establish perinatal hemodynamic factors which might predict adverse outcome, six fetuses with CHB diagnosed between 20 and 30 gestational weeks were examined by echocardiography at 2-week intervals. Neonatal morbidity and outcome in infancy are detailed. The fetuses showed a significant decrease in ventricular rate (VR) with advancing gestation (60 ± 7 vs 51 ± 4 beats/min, p= 0.03). Cardiac decompensation defined as hydrops or pericardial effusion was associated with VR of lower than 55 beats/min in two fetuses. Three mothers had a therapeutic trial with a sympathomimetic and digoxin. Salbutamol increased VR 10% in one of three fetuses treated. Digoxin decreased pericardial effusion in one hydropic fetus with autoimmune myocarditis. In this fetus, poor left ventricular fractional shortening (LVFS) was accompanied with high umbilical artery resistance index (RI). High amniotic fluid erythropoietin indicated severe hypoxia preceding death. Pacemaker was indicated in all the newborns. At the age of 2 weeks all the surviving infants had tricuspid regurgitation and a shunt through foramen ovale due to asynchronized atrioventricular contraction. During the 12-month follow-up two of five surviving infants had no symptoms. One had symptomatic neonatal lupus. Two infants had patent ductus arteriosus, one with dilated cardiomyopathy. In conclusion, poor fetal outcome was associated with low VR, low LVFS, and high RI. Despite early pacing, morbidity was high in infancy due to cardiomyopathy and associated heart defects. Regular echocardiographic monitoring during pregnancy and after delivery is required in order to optimize care and timing of any interventions.     

Outcome of Intermittent Tachyarrhythmias in the Fetus

Persistent fetal tachycardias are known to have an adverse effect on fetal outcome. The outcomes of intermittent fetal tachyarrhythmias over a 12-year period at a tertiary fetal cardiology center were studied. Main outcome criteria included control of arrhythmia and death during the prenatal or postnatal period. A total of 28 fetuses had an intermittent tachyarrhythmia: 4 had intermittent atrial flutter and 24 had supraventricular tachycardia. At the time of presentation 14 fetuses were hydropic, and in 5 of the 14 an arrhythmia had not been noted prior to referral. Of the 28 fetuses, 23 were treated by drug administration to the mother. Control of arrhythmia was achieved in 10 of 11 (91%) nonhydropic fetuses and 8 of 12 (67%) hydropic fetuses, with resolution of hydrops in four cases. In the overall group there was one intrauterine death, two neonatal deaths, and one infant death, all of which occurred in the hydropic group. The arrhythmia recurred postnatally in 11 of 23 (48%) fetuses. We conclude that intermittent tachyarrhythmias may have a deleterious effect on the fetus with a significant risk of death pre- or postnatally. The fetus with nonimmune hydrops should be evaluated for a cardiac cause. Maternal antiarrhythmic therapy is indicated for intermittent fetal tachyarrhythmias. There is a high risk of recurrence of the arrhythmia during infancy, particularly if hydrops was documented during the prenatal period or if Wolff-Parkinson-White syndrome is diagnosed. Fetal echocardiography is a useful tool for diagnosis and for monitoring the progress of the fetus.     

Pretransplant increases in left ventricular volume and mass are associated with QT prolongation during pediatric liver transplantation

Structural alterations in the cirrhotic heart may contribute to electromechanical abnormalities, represented by QT prolongation. The aim of this study was to investigate the changes in QTc according to the operative stage during pediatric LT and to identify which baseline echocardiographic parameters were associated with intraoperative QTc prolongation. Data were evaluated from 39 children undergoing LT for chronic liver disease (median age 9 months). In 19 patients (48.7%), baseline QTc was prolonged ≥440 ms (462 ± 19 ms). Through the period of post‐reperfusion, QTI, QTc, and JTI progressively increased, although values partially recovered toward the end of surgery. High LVMI (≥82.51 g/m²) was associated with baseline QTc ≥ 440 ms (OR = 1.034, P = .032). In the 5 minutes post‐reperfusion stage, marked QTc prolongation (defined as QTc ≥ 500 ms; n = 24, 61.5%) was significantly associated with high EDVI (OR = 1.060, P = .027) and SVI (OR = 1.075, P = .026). In children with chronic liver disease, increased ventricular volumes and mass may increase the risk of QTc prolongation during LT, suggesting that repolarization abnormalities might be contributed by structural changes characteristic of cirrhotic cardiomyopathy.     

Frequent Ventricular Premature Beats in Children With a Structurally Normal Heart: A Cause for Reversible Left Ventricular Dysfunction?

Ventricular premature beats (VPBs) in a structurally normal heart generally are a benign condition. Rarely, however, reversible cardiomyopathy may develop. This study aimed to evaluate the incidence of cardiomyopathy among pediatric patients in a cohort with frequent VPBs and to examine the characteristics of the ventricular ectopic beats as well as therapeutic options. This study reviewed the charts of all pediatric patients between the ages of 1 day and 18 years seen at the University of Kentucky with the diagnosis of VPBs between 2003 and 2007. Frequent VPBs were defined as an ectopy burden of 5% or more in 24 h. Electrocardiograms, Holter monitors, and echocardiograms were reviewed. The review identified 28 patients (17 boys, age 13.3 ± 5.9 years, and 11 girls, age 13 ± 5.2 years) with frequent VPBs. The echocardiograms of four patients (2 boys, 14%) showed cardiomyopathy. Cardiac function normalized in all four patients, with spontaneous resolution of the VPBs (2 patients) or with antiarrhythmic therapy (2 patients). During a follow-up period of 2.7 ± 2.3 years, 32% of the patients without cardiomyopathy showed a marked spontaneous improvement in arrhythmia burden. Most of the patients showed VPBs with a left bundle branch block (LBBB) and inferior axis morphology. The most commonly associated symptoms were chest pain (17.8%) and dizziness and syncope (21.4%). Generally, VPBs in structurally normal hearts are considered benign. Rarely, a reversible cardiomyopathy can develop, requiring therapeutic intervention.     

Assessment of spontaneous baroreflex sensitivity in neonates

AIMS—To determine whether it is possible to assess baroreflex sensitivity in neonates by studying only spontaneous variations in systolic blood pressure and heart rate.METHODS—ECG and non-invasive blood pressure signals were continuously studied in 14 preterm neonates (term 29-32 weeks) and five term neonates (term 40-41 weeks). Non-invasive blood pressure measures were obtained using a Finapres placed around the child''s wrist. Both signals (ECG and blood pressure), sampled at 400 Hz, were digitised by an A/D converter and stored in a binary mode on magnetic disk. An inhouse software QRS detection algorithm was used to define R peaks of the QRS complexes with an accuracy greater than 2 ms. Four 4 minute periods were recorded in each infant. The slope of the linear regression of RR intervals versus systolic blood pressure was calculated in each period and the mean value of the four slopes was then considered as the index of baroreflex sensitivity (in ms/mm Hg) in each neonate.RESULTS—Spontaneous baroreflex sensitivity was lower in preterm neonates than in term neonates (mean(SD): 4.07 (2.19) ms/mm Hg vs 10.23 (2.92) ms/mm Hg).CONCLUSION—Baroreflex sensitivity can be assessed in term and preterm neonates by studying spontaneous variations in systolic blood pressure alone. This method could be useful for studying the ontogeny of baroreflex sensitivity and might therefore provide information about the maturation of the autonomic nervous system.     

Right Ventricular Dilatation in the Fetus: A Study of Associated Features and Outcome

Right ventricular dilatation is an infrequent finding at fetal echocardiography. Previous studies have documented an association with aortic coarctation. However, there are associations with other congenital abnormalities. We reviewed our experience of fetal right heart dilatation in order to recognize concurrent anomalies and to assess the outcome of the affected fetuses. We studied all fetuses with right ventricular dilatation over a 5-year period. We documented associated cardiac and noncardiac lesions, and outcome data were assessed in terms of the number of fetuses that were born live and the number surviving to 2 months of age. Forty-three fetuses with right heart dilatation were seen. Fifteen had associated cardiac abnormalities: most commonly coarctation (n= 4) and VSD (n= 4). Seven had associated noncardiac abnormalities. There were seven fetuses who also had chromosomal abnormalities. In total, there were three terminations of pregnancy, four intrauterine deaths, one stillbirth and 35 live-births. Twenty-eight were alive at 2 months of age (70% of the nonterminated pregnancies). Fetal right heart dilatation is frequently associated with both cardiac and noncardiac congenital abnormalities. Our outcome figures suggest a guarded prognosis be given during counseling of parents of fetuses with right heart dilatation.     

Value of Autopsy in Nonimmune Hydrops Fetalis: Series of 51 Stillborn Fetuses

Nonimmune hydrops fetalis (NIHF) is used to describe fetuses and newborns with generalized edema and cavity effusions. It is helpful to alert physicians about the presence of anemia, heart failure, and/or hypoproteinemia, but this diagnosis is frequently overlooked. We reviewed the autopsy files from 1990 to 2000, selected all cases with NIHF including clinical information (with maternal laboratory tests and ultrasound), and classified patients by etiology. Among 840 stillborn autopsies during the 11-year period, we found 51 with NIHF (6.07%). The clinical summary had mentioned hydrops in 14 patients and the etiology in another 7 by fetal ultrasonography, but without addressing the possibility of hydrops. In the remaining 30 cases neither hydrops nor an etiology was mentioned. Other pertinent diagnoses were maternal diabetes mellitus (4), congenital heart disease (3), and cystic hygroma (2). The following diagnoses were made in one instance each: cardiac tumor, twin transfusion syndrome, congenital adenomatoid malformation, syphilis, Turner syndrome, and cerebral arteriovenous malformation. Postmortem and placental examination confirmed the following etiologies: congenital infections (17); placental pathology significant enough to explain NIHF (10); cardiovascular diseases (8) (further classified as congenital heart disease [3], rhabdomyoma [1], and vascular malformations [4]); chromosomal abnormalities (6); uncontrolled maternal diabetes (4); intrathoracic lesions (2); prune-belly syndrome (2); and idiopathic NIHF (2). Only 3.9% of the cases studied had no identifiable etiology. The cause of hydrops was confirmed by autopsy in 47 fetuses (92%), which further supports the importance of performing an autopsy. Thirty-two cases (62.74%) had placental abnormalities helpful to the etiology (parvovirus, syphilis, Turner's syndrome, etc.). In 20 instances, the clinical summary had no mention of either hydrops or any of the diseases leading to it. The autopsy in conjunction with placental examination and fetal ultrasound represent the best combination to determine the etiology of NIHF among stillborn fetuses.     

Perinatal risk factors for developmental dysplasia of the hip

AIMS—To identify perinatal risk factors for developmental dysplasia of the hip (DDH) and define the risk for each factor.METHODS—In this case control study, using logistic regression analysis, all 1127 cases of isolated DDH live born in South Australia in 1986-93 and notified to the South Australian Birth Defects Register were included; controls comprised 150 130 live births in South Australia during the same period without any notified congenital abnormalities.RESULTS—Breech presentation, oligohydramnios, female sex and primiparity were confirmed as risk factors for DDH. Significant findings were an increased risk for vaginal delivery over caesarean section for breech presentation (as well as an increased risk for emergency section over elective section), high birthweight (?4000 g), postmaturity and older maternal age; multiple births and preterm births had a reduced risk. There was no increased risk for caesarean section in the absence of breech presentation. For breech presentation, the risk of DDH was estimated to be at least 2.7% for girls and 0.8% for boys; a combination of factors increased the risk.CONCLUSIONS—It is suggested that the risk factors identified be used as indications for repeat screening at 6 weeks of age and whenever possible in infancy. Other indications are family history and associated abnormalities.