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Factors associated with in utero fetal demise (IUFD) of fetuses that have underlying cardiac pathologies are largely unknown. This case-control study aimed to define the prevalence of IUFD in fetuses with a diagnosis of cardiac pathologies and to identify prenatal predictors of IUFD. Between January 2004 and December 2010, 74 IUFD cases [4.6 %; 95 % confidence interval (CI) 3.7-5.8 %] were identified from 1,584 cases with a diagnosis of structural or functional cardiac lesions in the Hospital for Sick Children database. The cases were divided into right-sided (N = 28), left-sided (N = 23), great artery (N = 8), and miscellaneous (N = 15) groups. The control subjects (1:1 ratio) were fetuses that had cardiac pathology diagnosed within 48 h of the IUFD case. Multivariable regression models were used to determine echocardiographic predictors of IUFD. The prevalence of IUFD was greatest in hypertrophic cardiomyopathy (8/16, 50 %) and Ebstein's anomaly/tricuspid dysplasia (4/15, 27 %) and lowest in transposition of the great arteries (2/85, 1 %). The findings showed IUFD to be associated with hydrops in 17 (23 %) of the 74 cases and arrhythmia in 11 (15 %) of the 74 cases. The factors identified by univariable logistic regression analyses were right ventricular dysfunction [odds ratio (OR) 2.7; p = 0.001], left ventricular dysfunction (OR 1.8; p = 0.007), umbilical vein pulsations (OR 10.9; p = 0.002), and abnormal ductus venosus flow (OR 3.3; p = 0.01). The factors associated with IUFD in multivariable logistic regression models were cardiomegaly (OR 5.6; p = 0.01), hydrops (OR 29.5; p = 0.001), pericardial effusion (OR 4.1; p = 0.06), and extracardiac abnormalities (OR 7.2; p < 0.001). The prevalence of IUFD is greatest in conditions affecting the ventricular myocardium. The onset of IUFD appears to be related initially to right ventricular dysfunction. Closer surveillance is recommended for lesions at risk of IUFD.

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/mm(2); p = 0.005). The EFE + heart disease group had both fewer B cells (0.18 vs. 0.44 cells/mm(2); p = 0.08) and T cells (0.29 vs. 0.80 cells/mm(2); 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.

Objective: To study the clinical course and outcome of fetal sinus bradycardia (SB) due to maternal antibody-induced sinus node dysfunction., Methods: We reviewed the maternal, prenatal, and postnatal findings of fetuses with SB associated with elevated maternal anti-SSA/Ro and anti-SSB/La antibodies., Results: Of the 6 cases diagnosed prenatally, 3 had isolated SB persisting after birth and had a good prognosis. Three fetuses with SB and severe myocardial involvement (congenital complete heart block and/or endocardial fibroelastosis) succumbed in utero in spite of treatment. Postmortem histopathology in 1 fetus showed inflammatory destruction of the sinus and atrioventricular nodes. SB was detected incidentally in a 7-year-old girl. She had intermittent heart block with progressive sinus arrest requiring permanent pacemaker., Conclusion: Fetal SB associated with maternal autoantibodies may persist in childhood, with a good prognosis in the absence of widespread cardiac involvement.

Objective: To establish gestational age-specific reference values in healthy singleton fetuses, we prospectively assessed the evolution of diastolic longitudinal wall-motion velocities by spectral Doppler tissue imaging., Methods: Early (Ea) and late diastolic (Aa) peak Doppler tissue imaging velocities were analyzed in 114 fetuses (age range: 14-42 weeks) at the base of right ventricular free wall, ventricular septum, and left ventricular free wall and compared with early (E) and late (A) diastolic peak Doppler inflow velocities., Results: A linear increase in Ea, Aa, and Ea/Aa ratio was documented at all sites with advancing gestation. Likewise, the peak E flow velocities of both atrioventricular valves and the tricuspid peak A flow velocity increased. The ratio of peak E/Ea velocities decreased exponentially as a result of a more rapid increase in Ea than E, to reach a stable E/Ea relationship only in the early third trimester., Conclusions: There was a strong positive correlation between Ea and Aa velocities and gestational age indicating improved diastolic myocardial lengthening with advancing gestation. Reference charts for Doppler tissue imaging velocities were established that will allow identification of fetal diastolic function abnormalities.

The occurrence of a maternal and fetal tachyarrhythmia together in pregnancy is exceedingly rare. We report a case of a persistent fetal atrial ectopic tachycardia occurring in conjunction with a maternal atrial tachycardia with left ventricular systolic dysfunction. Amiodarone was effective in treating both maternal and fetal arrhythmias. [ABSTRACT FROM AUTHOR]

Background--Fetal tachyarrhythmia may result in low cardiac output and death. Consequently, antiarrhythmic treatment is offered in most affected pregnancies. We compared 3 drugs commonly used to control supraventricular tachycardia (SVT) and atrial flutter (AF). Methods and Results--We reviewed 159 consecutive referrals with fetal SVT (n= 114) and AF (n=45). Of these, 75 fetuses with SVT and 36 with AF were treated nonrandomly with transplacental flecainide (n = 35), sotalol (n = 52), or digoxin (n=24) as a first-line agent. Prenatal treatment failure was associated with an incessant versus intermittent arrhythmia pattern (n = 85; hazard ratio [HR] = 3.1; P<0.001) and, for SVT, with fetal hydrops (n = 28; HR=1.8; P=0.04). Atrial flutter had a lower rate of conversion to sinus rhythm before delivery than SVT (HR=2.0; P=0.005). Cardioversion at 5 and 10 days occurred in 50% and 63% of treated SVT cases, respectively, but in only 25% and 41% of treated AF cases. Sotalol was associated with higher rates of prenatal AF termination than digoxin (HR=5.4; P=0.05) or flecainide (HR=7.4; P=0.03). If incessant AF/SVT persisted to day 5 (n=45), median ventricular rates declined more with flecainide (-22%) and digoxin (-13%) than with sotalol (-5%; PC0.00I). Flecainide (HR = 2.1; P=0.02) and digoxin (HR=2.9; P=0.01) were also associated with a higher rate of conversion of fetal SVT to a normal rhythm over time. No serious drug-related adverse events were observed, but arrhythmia-related mortality was 5%. Conclusion--Flecainide and digoxin were superior to sotalol in converting SVT to a normal rhythm and in slowing both AF and SVT to better-tolerated ventricular rates and therefore might be considered first to treat significant fetal tachyarrhythmia. [ABSTRACT FROM AUTHOR]

Objectives: We prospectively examined the prevalence and outcome of untreated fetal atrioventricular (AV) prolongation in the presence of maternal anti-Ro antibodies. Background: It has been suggested that antibody-mediated congenital complete atrioventricular block (CAVB) may be preventable if detected and treated early when low-grade block is present. With this rationale in mind, dexamethasone has been advocated by others to treat prolonged fetal AV conduction >2 z-scores, consistent with first-degree heart block. Methods: Between July 2003 and June 2009, 165 fetuses of 142 anti-Ro/La antibody–positive women were referred to our center for serial echocardiography. Our protocol included weekly evaluation of the fetal AV conduction between 19 (range 17 to 23) and 24 (range 23 to 35) gestational weeks. AV times were compared with institutional reference data and with post-natal electrocardiograms. Results: Of 150 fetuses with persistently normal AV conduction throughout the observation period, a diagnosis of CAVB was subsequently made in 1 at 28 weeks, after the serial evaluation had ended. Of 15 untreated fetuses either with AV prolongation between 2 and 6 z-scores or with type 1 second-degree block, progressive heart block developed in none of them. Three of these 15 fetuses (20%) had a neonatal diagnosis of first-degree block that spontaneously resolved (n = 2) or has not progressed (n = 1) on follow-up examinations. No other cardiac complications were detected. Conclusions: Fetal AV prolongation did not predict progressive heart block to birth. Our findings question the rationale of a management strategy that relies on the early identification and treatment of fetal AV prolongation to prevent CAVB. [ABSTRACT FROM AUTHOR]