Clinical Monitoring of Sacrococcygeal Teratoma.

Background: Sacrococcygeal teratomas (SCT) are often highly vascularized and may result in high-output cardiac failure, polyhydramnios, fetal hydrops, and demise. Delivery is guided by the SCT to fetus volume ratio (SCTratio), SCT growth rate, and cardiac output indexed for weight (CCOi).

Methods: We compared measurements and outcome in 12 consecutive fetuses referred with SCT.

In utero intervention for severe congenital heart disease.

The aim of foetal cardiac therapy is to treat an abnormality at the developmental stage so that the process of cardiac growth, which is complex and relies on the volume and direction of circulating blood as well as genetic determinants, can continue. In reality, most cardiac interventions are palliative; hence, major abnormalities are still present at birth. Nevertheless, tangible benefits following successful foetal intervention include improved haemodynamics and reduction in secondary damage leading to better postnatal outcomes.

Long-term outcomes for monochorionic twins after laser therapy in twin-to-twin transfusion syndrome.

Twin-to-twin transfusion syndrome typically occurs in the second trimester in 10-15% of monochorionic twin pregnancies. Vascular anastomoses of monochorionic placentae are the underlying cause of the development of the syndrome. If a blood flow imbalance occurs, one fetus becomes the so-called donor twin and the other the recipient.

Advances in fetal echocardiography.

The development of fetal echocardiography and success in prenatal cardiac screening programs over the past 30 years has been driven by technical innovation and influenced by the different approaches of the various specialties practicing it. Screening for congenital heart defects no longer focuses on examining a limited number of pregnant women thought to be at increased risk, but instead forms an integrated part of a high-quality anatomical ultrasound performed in the second trimester using the 'five-transverse view' protocol. A prenatal diagnosis is feasible in almost all cardiac lesions and the advantages to parents and to health professionals are well recognized.

The Influence of Blood Pressure on Fetal Aortic Distensibility: An Animal Validation Study.

Background/aims: Aortic distension waveforms describe the change in diameter or cross-sectional area over the cardiac cycle. We aimed to validate the association of aortic fractional area change (AFAC) with blood pressure (BP) in a fetal lamb model.

Methods: Four pregnant ewes underwent open fetal surgery under general anesthesia at 107-120 gestational days.

Acardiac twin pregnancies part IV: Acardiac onset from unequal embryonic splitting simulated by a fetoplacental resistance model.

Background: Benirschke postulated that acardiac twinning occurs when markedly unequal embryonic splitting combines with arterioarterial (AA) and venovenous placental anastomoses. We tested this hypothesis by model simulations and by comparison of outcomes with 18 "pseudo-" (twin fetus with beating heart but otherwise with clear signs of an acardiac) and 3 "normal" acardiac cases.

Methods: The smaller/larger cell volume ratio at embryonic splitting becomes the smaller/larger embryonic/fetal blood volume ratio (a).

Acardiac twin pregnancies part II: Fetal risk of chorangioma and sacrococcygeal teratoma predicted by pump/acardiac umbilical vein diameters.

Background: We recently published pump/acardiac umbilical venous diameter (UVD) ratios, representing the pump twin's excess cardiac output fraction, of 27 acardiac twin pregnancies. There was a clear separation between the 17 pump twins that had life-threatening complications and the 10 that did not. The hypothesis of this study is that placental chorangioma and sacrococcygeal teratoma (SCT), tumors whose perfusion also causes high-output complications, have the same fetal outcome as pump twins when perfusion of the tumor requires the same excess cardiac output fraction.

Fetal cardiovascular hemodynamics in twin-twin transfusion syndrome.

Twin-twin transfusion syndrome (TTTS) complicates 10-15% of monochorionic-diamniotic (MCDA) pregnancies. It originates from unbalanced transfer of fluid and vasoactive mediators from one twin to its co-twin via placental anastomoses. This results in hypovolemia in the donor and hypervolemia and vasoconstriction in the recipient twin.

Foetal cardiac function: assessing new technologies.

Assessment of foetal cardiac function is more challenging than in the adult, in whom emerging technologies are tested. The postnatal cardio-respiratory interaction is replaced by the cardio-placental circulation and impedance of the brain, and distal vascular beds play an important role in modulating flow to enable its redistribution in the foetal body. Prenatal specialists, comprising obstetricians and cardiologists, have tested a variety of traditional methodologies, as well as non-Doppler offline ultrasound methods in the foetus.

Prenatal screening for major congenital heart disease: assessing performance by combining national cardiac audit with maternity data.

Objective: Determine maternity hospital and lesion-specific prenatal detection rates of major congenital heart disease (mCHD) for hospitals referring prenatally and postnatally to one Congenital Cardiac Centre, and assess interhospital relative performance (relative risk, RR).

Methods: We manually linked maternity data (3 hospitals prospectively and another 16 retrospectively) with admissions, fetal diagnostic and surgical cardiac data from one Congenital Cardiac Centre. This Centre submits verified information to National Institute for Cardiovascular Outcomes Research (NICOR-Congenital), which publishes aggregate antenatal diagnosis data from infant surgical procedures.

The cost-effectiveness of prenatal detection for congenital heart disease using telemedicine screening.

We estimated the longer-term cost-effectiveness of using telemedicine screening for prenatal detection of congenital heart disease (CHD). One hospital in south-east England with a telemedicine service was connected to a fetal cardiology unit in London. A UK health service perspective was adopted.

Ventricular strain changes in monochorionic twins with and without twin-to-twin transfusion syndrome.

Objective: The objective of the study was to investigate whether vector velocity imaging (VVI), a non-Doppler speckle tracking ultrasound technology, is feasible in twin pregnancies and can aid management of twin-twin transfusion syndrome (TTTS).

Study Design: Twenty-seven women pregnant with monochorionic diamniotic twins affected by TTTS and 28 monochorionic pregnancies that did not develop TTTS were included in a prospective case-control study at a fetal medicine center. Fetal echocardiograms were recorded with dummy electrocardiography to retain original frame rates when exported for offline speckle tracking analysis using Syngo-VVI software (Siemens Corp, Munich, Germany).

Myocardial strain abnormalities in fetal congenital heart disease assessed by speckle tracking echocardiography.

Objectives: To compare myocardial deformation patterns in fetuses with congenital heart disease (CHD) with our reference range using speckle tracking echocardiography.

Methods: We prospectively stored and analyzed 4-chamber loops of 28 fetuses with CHD (median gestation 27 weeks, range 20.9-37.

Isolated atrioventricular block in the fetus: a retrospective, multinational, multicenter study of 175 patients.

Background: Isolated complete atrioventricular block in the fetus is a rare but potentially lethal condition in which the effect of steroid treatment on outcome is unclear. The objective of this work was to study risk factors associated with death and the influence of steroid treatment on outcome.

Methods And Results: We studied 175 fetuses diagnosed with second- or third-degree atrioventricular block (2000-2007) retrospectively in a multinational, multicenter setting.

Cost implications of introducing a telecardiology service to support fetal ultrasound screening.

A district hospital in south-east England used a telecardiology service for fetal cardiac diagnosis alongside an existing arrangement for referring pregnant women directly to perinatal cardiologists in London for detailed fetal echocardiography. Women were identified for referral according to local protocols when having a second trimester anomaly scan. For the telemedicine referrals, the sonographers video-recorded images from the anomaly scans for transmission during monthly videoconferences.

Morphological and physiological predictors of fetal aortic coarctation.

Background: Prenatal diagnosis of aortic coarctation suffers from high false-negative rates at screening and poor specificity.

Methods And Results: This retrospective study tested the applicability of published aortic arch and ductal Z scores (measured just before the descending aorta in the 3-vessel and tracheal view) and their ratio on 200 consecutive normal controls at a median of 22+/-0 gestational weeks (range, 15+/-4 to 38+/-4 weeks). Second, this study tested the ability of serial Z scores to distinguish fetuses with coarctation within a cohort with ventricular and/or great arterial disproportion detected at screening or fetal echocardiography.

Morphologic and functional predictors of eventual circulation in the fetus with pulmonary atresia or critical pulmonary stenosis with intact septum.

Objectives: The purpose of this study was to determine the morphologic and physiological predictors of post-natal surgical pathway in a longitudinal series of fetuses with pulmonary atresia with intact ventricular septum (PAIVS) and/or critical pulmonary stenosis with reversal of ductal flow (CPS) using statistical modeling.

Background: Pulmonary atresia with intact ventricular septum is rarely associated with chromosomal or extra cardiac malformations, so decisions about continuing a pregnancy are strongly influenced by the prediction of univentricular (UV) or biventricular (BV) circulation.

Methods: Predictive scores were derived, using a combination of z-scores of fetal cardiac measurements (for femoral length) and tricuspid/mitral valve (TV/MV) ratios, to facilitate early prediction of UV or BV circulation in 21 fetuses with PAIVS (18 fetuses) or CPS (3 fetuses) between 1998 and 2004.

Early environmental influences on vascular development.

The "fetal origins hypothesis" proposes that a fetus' adaptation to its intrauterine environment and postnatal stressors may have life-long consequences and that the fetal response to an environmental challenge may result in programming of different organ systems, depending on the timing of the insult. Growth restriction in the last trimester of pregnancy has been associated with later cardiovascular disease, not only through disturbances of normal cardiovascular and neuro-hormonal control mechanisms, but also because the "catch-up growth" observed in growth restricted infants disturbs the "thrifty phenotype" that permits an individual to live within its predicted environment. Problems in testing the fetal programming hypothesis include the measurement of an individual's failure to achieve their growth potential and the absence of an effective early measurement of later cardiovascular risk.

In-utero intervention for severe congenital heart disease.

The concept of fetal therapy is well established for many disorders diagnosed before birth but practical issues regarding its introduction into clinical practice are more difficult. Cardiac malformations are common, with major lesions affecting about 3.5 per thousand pregnancies; however, only a small proportion of these is likely to benefit from an intrauterine intervention.

Telemedicine in pediatric and perinatal cardiology: economic evaluation of a service in English hospitals.

Objectives: Pediatric cardiology has an expanding role in fetal and pediatric screening. The aims of this study were to observe how district hospitals use a pediatric telecardiology service, and to compare the costs and outcomes of patients referred to specialists by means of this service or conventionally.

Methods: A telemedicine service was set up between a pediatric cardiac center in London and four district hospitals for referrals of second trimester women, newborn babies, and older children.