Publications by authors named "Trisha V Vigneswaran"

16 Publications

  • Page 1 of 1

Reference Ranges for Pulsed-Wave Doppler of the Fetal Cardiac Inflow and Outflow Tracts from 13 to 36 Weeks' Gestation.

J Am Soc Echocardiogr 2021 May 3. Epub 2021 May 3.

Medway Fetal and Maternal Medicine Centre, Medway Maritime Hospital, Gillingham, United Kingdom; Institute of Medical Sciences, Canterbury Christ Church University, Chatham, United Kingdom.

Background: Doppler assessment of ventricular filling and outflow tract velocities is an integral part of fetal echocardiography, to assess diastolic function, systolic function, and outflow tract obstruction. There is a paucity of prospective data from a large sample of normal fetuses in the published literature. The authors report reference ranges for pulsed-wave Doppler flow of the mitral valve, tricuspid valve, aortic valve, and pulmonary valve, as well as heart rate, in a large number of fetuses prospectively examined at a single tertiary fetal cardiology center.

Methods: The study population comprised 7,885 fetuses at 13 to 36 weeks' gestation with no detectable abnormalities from pregnancies resulting in normal live births. Prospective pulsed-wave Doppler blood flow measurements were taken of the mitral, tricuspid, aortic, and pulmonary valves. The fetal heart rate was recorded at the time of each assessment. Regression analysis, with polynomial terms to assess for linear and nonlinear contributors, was used to establish the relationship between each measurement and gestational age.

Results: The measurement for each cardiac Doppler measurement was expressed as a Z score (difference between observed and expected values divided by the fitted SD corrected for gestational age) and percentile. Analysis included calculation of gestation-specific SDs. Regression equations are provided for the cardiac inflow and outflow tracts.

Conclusions: This study establishes reference ranges for fetal cardiac Doppler measurements and heart rate between 13 to 36 weeks' gestation that may be useful in clinical practice.
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http://dx.doi.org/10.1016/j.echo.2021.04.017DOI Listing
May 2021

Postnatal impact of a prenatally diagnosed double aortic arch.

Arch Dis Child 2021 Jun 28;106(6):564-569. Epub 2020 Oct 28.

Paediatric Intensive Care Unit, Evelina London Children's Hospital, London, UK.

Background: A double aortic arch (DAA) is increasingly identified before birth; however, there are no published data describing the postnatal outcome of a large prenatal cohort.

Objective: To describe the associations, symptoms and impact of prenatally diagnosed DAA.

Methods: Retrospective review of consecutive cases seen at two fetal cardiology units from 2014 to 2019. Clinical records including symptoms and assessment of tracheobronchial compression using flexible bronchoscopy were reviewed. Moderate-severe tracheal compression was defined as >75% occlusion of the lumen.

Results: There were 50 cases identified prenatally and 48 with postnatal follow-up. Array comparative genomic hybridisation (aCGH) was abnormal in 2/50 (4%), aCGH was normal in 33/50 (66%) and of those reviewed after birth, 13 were phenotypically normal. After birth, there was a complete DAA with patency of both arches in 8/48 (17%) and in 40/48 (83%) there was a segment of the left arch which was a non-patent, ligamentous connection.Stridor was present in 6/48 (13%) on the day of birth. Tracheo-oesophageal compressive symptoms/signs were present in 31/48 (65%) patients at median age of 59 days (IQR 9-182 days). Tracheal/carinal compression was present in 40/45 (88%) cases. Seven of 17 (41%) asymptomatic cases demonstrated moderate-severe tracheal compression. All morphologies of DAA caused symptoms and morphology type was not predictive of significant tracheal compression (p=0.3).

Conclusions: Genetic testing should be offered following detection of double aortic arch. Early signs of tracheal compression are common and therefore delivery where onsite neonatal support is available is recommended. Significant tracheal compression may be present even in the absence of symptoms.
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http://dx.doi.org/10.1136/archdischild-2020-318946DOI Listing
June 2021

Speckle-Tracking Echocardiography for the Assessment of Atrial Function during Fetal Life.

J Am Soc Echocardiogr 2020 11 20;33(11):1391-1399. Epub 2020 Aug 20.

Fetal Cardiology Unit, Department of Congenital Heart Disease, Evelina London Children's Healthcare, Guy's and St. Thomas' NHS Foundation Trust, London, United Kingdom.

Introduction: Speckle-tracking echocardiography has become a major tool in the evaluation of heart function. Atrial strain has emerged as an important component in the assessment of cardiac function, but there is a paucity of prenatal data. The aim of this study was to describe our initial experience of measurement of atrial strain in fetuses, with respect to both feasibility and the strain patterns observed.

Methods: Four-chamber Digital Imaging and Communications in Medicine loops were acquired prospectively for deformation imaging. Fifty-three normal fetuses with no morphologic or functional abnormalities were selected for analysis. The three strain components of atrial cycle for both left atrium (LA) and right atrium (RA) were acquired-reservoir (LAres or RAres), conduit, and contraction (LAct or RAct)-and are expressed as a percentage. Ratios of these components were calculated. Simple linear regression was used to analyze how the dependent variables changed according to gestational age and frame rate.

Results: The median gestational age was 30 weeks (range, 23-35), and the frame rate was 74 frames per second (fps; range, 35-121). Left atrial strain was feasible in 48/53 (91%), and right atrial strain in 46/53 (87%) of cases. The onset of LA contraction could be identified on the strain curves in 32 of 48 (67%) cases, and of the RA in 17 of 46 (37%) cases. The values of RAres and RAct were higher compared with those of LAres and LAct (33.9% vs 30.3%, P = .014; and 21.5% vs 16.8%, P = .005), and the contraction:reservoir ratio was also higher for RA (0.63 vs 0.55 for LA, P = .003). Higher values for LAres, LAct, RAres, and RAct were associated with higher frame rate (P = .007, .020, .049, and .012, respectively). The onset of LA contraction was better identified with a higher frame rate (mean 77 vs 59 fps when not seen, P = .007). A higher LA contraction:reservoir ratio was associated with a lower gestational age (P = .042).

Conclusion: Measurement of atrial strain is feasible in the fetal heart. The values are influenced by gestational age and frame rate, so it is necessary to account for these variables. Comparison of left versus right atrial strain values contrasts with those observed postnatally. Atrial function merits further study during fetal life, to aid understanding of maturational changes and disease states.
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http://dx.doi.org/10.1016/j.echo.2020.06.011DOI Listing
November 2020

Fetal Speckle-Tracking: Impact of Angle of Insonation and Frame Rate on Global Longitudinal Strain.

J Am Soc Echocardiogr 2020 09 15;33(9):1141-1146.e2. Epub 2020 May 15.

Fetal Cardiology Unit, Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's & St. Thomas' NHS Trust, London, United Kingdom; Harris Birthright Centre for Fetal Medicine, Fetal Medicine Research Institute, King's College Hospital, London, United Kingdom; School of Biomedical Engineering and Imaging Sciences, King's College London, London, United Kingdom. Electronic address:

Background: There is a growing body of research on fetal speckle-tracking echocardiography because it is considered to be an angle-independent modality. The primary aim of this study was to investigate whether angle of insonation and acquisition frame rate (FR) influence left ventricular endocardial global longitudinal peak strain (GLS) in the fetus.

Methods: Four-chamber views of 122 healthy fetuses were studied at three different angles of insonation (apex up/down, apex oblique, and apex perpendicular) at high and low acoustic FRs. GLS was calculated, and a linear mixed-model analysis was used for analysis. Six hundred fifty-six fetal echocardiographic clips were analyzed (288 in the second trimester, at a median gestation of 21 weeks [interquartile range (IQR), 1 week], and 368 in the third trimester, at a median gestation of 36 weeks [IQR, 2 weeks]).

Results: Angle of insonation and FRs were significant determinants of GLS. Ventricular septum perpendicular to the ultrasound beam was associated with higher (more negative) GLS compared with apex up/down (at high FR: -21.8% vs -19.7%, P < .001; at low FR: -24.1% vs -21.4%, P < .001). Higher frames per second (FPS; median 149 FPS [IQR, 33 FPS] = 61 frames per cycle [FPC] [IQR, 17 FPC]) compared with lower FPS (median 51 FPS [IQR, 15 FPS] = 22 FPC [IQR, 7 FPC]) at the same insonation angle resulted in lower GLS (apex up/down: -19.7% vs -21.4%, P < .001; apex oblique: -21.2% vs -22.7%, P < .001; apex perpendicular: -21.8% vs -24.1%, P < .001).

Conclusions: The present findings show that insonation angle and FR influence GLS significantly. These factors need to be considered when comparing studies with different acquisition protocols, when establishing normative values, and when interpreting pathology. Speckle-tracking echocardiography cannot be considered an angle-independent modality during fetal life.
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http://dx.doi.org/10.1016/j.echo.2020.03.013DOI Listing
September 2020

Early Postnatal Echocardiography in Neonates with a Prenatal Suspicion of Coarctation of the Aorta.

Pediatr Cardiol 2020 Apr 7;41(4):772-780. Epub 2020 Feb 7.

Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's & St Thomas' Hospitals, London, SE1 7EH, UK.

Coarctation of the aorta (COA) is suspected prenatally when there is ventricular asymmetry, arterial disproportion, and hypoplasia of the aortic arch/isthmus. The presence of fetal shunts creates difficulty in prenatal confirmation of the diagnosis so serial echocardiography after birth is necessary to confirm or refute the diagnosis. The first neonatal echocardiogram in prenatally suspected cases of COA was assessed for prediction of neonatal COA repair (NCOAR). This included morphological assessment, measurement of the aortic arch and calculation of the distal arch index (DAI = distance between left common carotid and left subclavian artery/diameter of the distal arch). NCOAR was undertaken in 23/60 (38%) cases. Transverse arch, aortic isthmus z-score, and DAI had an area under the receiver operator curve of 0.88 (95% CI 0.77-0.98), 0.86 (95% CI 0.75-0.96), and 0.84 (95% CI 0.74-0.95), respectively for the prediction of NCOAR. Using transverse arch z-score threshold < - 3 gave sensitivity 100%, NPV: 100%, specificity 76%; aortic isthmus z-score  < - 3: NPV 92%, specificity 62% and DAI > 1.4: NPV 88%, specificity 78%. The size of the distal aortic arch in infants with a common origin of the innominate artery and left common carotid artery who did not require COA repair was similar to the NCOAR cases (p = 0.22). The early postnatal assessment of the size and morphology of the aortic arch can assist in risk stratification for development of neonatal COA. The branching pattern of the head/neck vessels impacts on the size of the distal aortic arch adding to the complexity of predicting COA based on vessel size.
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http://dx.doi.org/10.1007/s00246-020-02310-5DOI Listing
April 2020

Initial Experience of Superb Microvascular Imaging for Key Cardiac Views in Foetal Assessment before 15 Weeks Gestation.

Fetal Diagn Ther 2020 9;47(4):268-276. Epub 2019 Oct 9.

Harris Birthright Research Centre for Fetal Medicine, King's College Hospital, London, United Kingdom,

Background: In the first trimester, ultrasound confirmation of normal or abnormal cardiac anatomy is difficult. B-mode and colour flow Doppler (CFD) are used to assess the foetal heart. Superb microvascular imaging (SMI) can visualise blood flow within the heart and vessels in early gestation.

Objective: We report an initial experience of SMI for visualisation of normal and abnormal cardiac anatomy in the first trimester.

Methods: Transabdominal foetal echocardiography was performed between 11 + 6 and 14 + 3 weeks (Aplio 500 US system, Toshiba Medical Systems, Tokyo, Japan) from January 2017 to December 2017. All scans were performed at a tertiary foetal cardiology unit. To assess the potential utility of the technique for early gestation screening, normal scans were reviewed by foetal medicine trainees with respect to the B-mode, CFD and SMI. Three key views were selected to compare modalities: the 4-chamber view, outflow tracts and the 3-vessel and trachea view (VTV). Visualisation rates of key echocardiographic features of significant cardiac abnormalities by SMI were reviewed.

Results: Fifty-five normal echocardiograms and 34 cardiac abnormalities were included. In the normal heart, when B-mode, CFD and SMI were assessed separately, SMI had the highest rate of visualisation of 4-chamber, outflow tracts and 3-VTV (93, 85 and 83%, respectively). Intra-observer reliability was moderate for SMI of the 3 standard views (kappa 1, 0.64 and 0.64); inter-observer for 4-chamber and outflow tract views was moderate (kappa 0.64 and 0.77). In 29/34 abnormal cases, SMI showed key features, enhancing greyscale visualisation.

Conclusion: SMI has potential to become a useful, complementary modality for early foetal echocardiography. Further prospective studies are warranted to establish the place of the technique in assessment of the first trimester foetal heart.
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http://dx.doi.org/10.1159/000502839DOI Listing
January 2021

Prenatal incidence of isolated right aortic arch and double aortic arch.

J Matern Fetal Neonatal Med 2019 Oct 16:1-6. Epub 2019 Oct 16.

Harris Birthright Centre for Fetal Medicine, Fetal Medicine Research Institute, King's College Hospital , London , UK.

To define the incidence of variants of aortic arch sidedness in fetuses undergoing routine first trimester ultrasound examination. The data for this study were derived from prospective routine ultrasound examination at 11 to 13 weeks' gestation in singleton pregnancies examined in a local population between January 2014 and March 2018. We examined the incidence of isolated right aortic arch (RAA) and double aortic arch (DAA) in the local, screened population and compared the groups with and without these abnormalities. The study population of 33,202 pregnancies included 18 (5.4 per 10,000) cases with isolated RAA and 5 (1.5 per 10,000) with DAA. In the group with isolated RAA or DAA, compared to those without, the median maternal age was higher and the incidence of conceptions from fertilization (IVF) was eight-fold higher. The prevalence of 22q11microdeletion was 5% in patients with RAA from this local population. The incidence of isolated RAA and DAA in a local population undergoing routine first-trimester ultrasound examination is 2-3-fold higher than that reported in postnatal studies and the risk for these abnormalities is substantially increased in fetuses conceived by IVF.
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http://dx.doi.org/10.1080/14767058.2019.1676413DOI Listing
October 2019

Prenatal diagnosis and clinical implications of an apparently isolated right aortic arch.

Prenat Diagn 2018 12;38(13):1055-1061

Harris Birthright Centre for Fetal Medicine, Fetal Medicine Research Institute, King's College Hospital, London, UK.

Objective: To define the associations of a prenatally diagnosed, apparently isolated right aortic arch (RAA) with chromosomal or genetic abnormalities and tracheal compression.

Methods: This was a retrospective study of apparently isolated RAA assessed by fetal cardiologists and fetal medicine specialists at Kings College Hospital, London between 2000 and 2017.

Results: The search identified 138 cases of apparently isolated RAA. Invasive testing was performed in 75, and chromosomal or genetic anomalies were identified in 16 (22%), and the most common was 22q11 microdeletion. An aberrant left subclavian artery was seen in 51% of cases. Symptoms of a vascular ring were present in 24 of 97 (25%) children who were reviewed after birth. Bronchoscopy was performed in 33 children, and significant tracheal compression was diagnosed in 28, including 18 of 19 symptomatic and 10 of 14 asymptomatic children.

Conclusions: An apparently isolated RAA is associated with a high incidence of chromosomal or genetic abnormalities and a high incidence of tracheal compression in symptomatic and asymptomatic patients. Prenatal counselling for genetic associations and postnatal airway assessment in the context of the vascular anatomy is recommended.
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http://dx.doi.org/10.1002/pd.5388DOI Listing
December 2018

Reference Ranges for the Size of the Fetal Cardiac Outflow Tracts From 13 to 36 Weeks Gestation: A Single-Center Study of Over 7000 Cases.

Circ Cardiovasc Imaging 2018 07;11(7):e007575

Harris Birthright Centre for Fetal Medicine, Fetal Medicine Research Institute, King's College Hospital NHS Foundation Trust, London, United Kingdom (T.V.V., R.A., A.S., M.C., L.D.A., K.H.N., V.Z., J.M.S.).

Background: Assessment of the outflow tract views is an integral part of routine fetal cardiac scanning. For some congenital heart defects, notably coarctation of the aorta, pulmonary valve stenosis, and aortic valve stenosis, the size of vessels is important both for diagnosis and prognosis. Existing reference ranges of fetal outflow tracts are derived from a small number of cases.

Methods And Results: The study population comprised 7945 fetuses at 13 to 36 weeks' gestation with no detectable abnormalities from pregnancies resulting in normal live births. Prospective measurements were taken of (1) the aortic and pulmonary valves in diastole at the largest diameter with the valve closed, (2) the distal transverse aortic arch on the 3 vessel and trachea view beyond the trachea at the distal point at its widest systolic diameter, and (3) the arterial duct on the 3 vessel and trachea view at its widest systolic diameter. Regression analysis, with polynomial terms to assess for linear and nonlinear contributors, was used to establish the relationship between each measurement and gestational age. The measurement for each cardiac diameter was expressed as a z score (difference between observed and expected value divided by the fitted SD corrected for gestational age) and percentile. Analysis included calculation of gestation-specific SDs. Regression equations are provided for the cardiac outflow tracts and for the distal transverse aortic arch:arterial duct ratio.

Conclusions: The study established reference ranges for fetal outflow tract measurements at 13 to 36 weeks' gestation that are useful in clinical practice.
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http://dx.doi.org/10.1161/CIRCIMAGING.118.007575DOI Listing
July 2018

Effect of Prenatal Laterality Disturbance and Its Accompanying Anomalies on Survival.

Am J Cardiol 2018 08 20;122(4):663-671. Epub 2018 Jun 20.

Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's & St Thomas' Hospitals, London SE1 7EH, United Kingdom.

In this retrospective, observational study of fetuses diagnosed with a laterality disturbance we describe the findings and outcome of fetuses diagnosed between 1980 and 2017 at a tertiary fetal-pediatric cardiology unit. In addition we sought to identify features which impact on outcome. Left atrial isomerism (LAI) was diagnosed in 177 babies and right atrial isomerism (RAI) in 100. Major structural heart disease was present in all cases of RAI and 91% with LAI. Complete heart block (CHB) was present in 40% of LAI. For surviving live-born infants a biventricular circulation was feasible in 3% with RAI and 43% with LAI. The median survival for live-borns with LAI was 13 months (range 0 to 272 months) and for RAI 19 months (range 0.3 to 292 months). The median postnatal survival with CHB was 0.2 months (range 0 to 228 months) compared to 44 months with sinus rhythm (interquartile range 0 to 272 months; p <0.0001). The 5-year survival was 1980 to 1989, RAI 0%, LAI 0%; 1990 to 1999, RAI 62%, LAI 54%; 2000 to 2009, RAI 59%, LAI 53%; 2010 to 2017, RAI 67%, LAI 75% by era. The rate of intrauterine death remained. Risk factors for death/transplantation for RAI were total anomalous pulmonary venous drainage, left heart obstruction (hazard ratios 2.7, p = 0.048; 5.8, p = 0.03) and for LAI: CHB, anomalous pulmonary venous drainage and right heart obstruction (hazard ratios 11.5, 6.2, 3.8, respectively (p = 0.008, p = 0.003, p <0.001)). In conclusion, laterality disturbances represent a complex form of congenital heart disease and although survival is improved, it remains poor especially in the presence of anomalous pulmonary venous drainage, stenotic and/or atretic valves, and CHB.
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http://dx.doi.org/10.1016/j.amjcard.2018.04.040DOI Listing
August 2018

Correlation of Symptoms with Bronchoscopic Findings in Children with a Prenatal Diagnosis of a Right Aortic Arch and Left Arterial Duct.

Pediatr Cardiol 2018 Apr 6;39(4):665-673. Epub 2018 Jan 6.

Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's & St Thomas' NHS Trust, London, SE1 7EH, UK.

A right aortic arch (RAA) with a left arterial duct (LAD) together encircle the trachea and have the potential to cause tracheobronchial compression and published guidelines recommend bronchoscopy in symptomatic patients. The aim of the study was to describe the incidence of tracheal compression in a cohort of prenatally diagnosed RAA and LAD. Retrospective review of clinical course and imaging of prenatal cases of RAA and LAD assessed with flexible bronchoscopy over an 11-year period. 34 cases of prenatally diagnosed RAA with LAD underwent bronchoscopy at median age of 9 months (range 0.4-123) of whom 11 had respiratory symptoms and 23 were asymptomatic. In the neonatal period, three cases demonstrated respiratory symptoms. An aberrant left subclavian artery (ALSA) was identified in 29 cases. Pulsatile tracheal compression was identified in 32/34 (94%) cases and two cases showed normal tracheal appearances. Significant tracheal compression (> 70% occlusion) was present in 25/34 (74%) cases of which 16 were asymptomatic. Significant carinal compression (> 70% occlusion) was identified in 14/34 (42%) cases, an ALSA was observed in 13/14. Surgical relief of a vascular ring has been performed in 27 (79%) cases at a median age of 15 months (range 0.6-128 months). At surgery, a fibrous remnant of an atretic left aortic arch was identified in 11/27 (41%) cases. Significant tracheal compression may be present in infants even without symptoms. If early relief of airway compression is to be achieved to promote normal development of tracheal cartilage, early bronchoscopy should be considered.
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http://dx.doi.org/10.1007/s00246-017-1804-5DOI Listing
April 2018

Hypertension in Coarctation of the Aorta: Challenges in Diagnosis in Children.

Pediatr Cardiol 2018 Jan 17;39(1):1-10. Epub 2017 Oct 17.

Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's & St Thomas' Hospitals, London, SE1 7EH, UK.

Evidence indicates that patients with coarctation of the aorta (COA) suffer from increased cardiovascular morbidity and mortality in later life despite successful repair of COA in childhood. Systolic arterial hypertension is common, presenting in up to one-third of patients, and is regarded as the main driver of premature cardiovascular events in this group of patients. In this review, we discuss the prevalence and pathophysiology of hypertension in children following successful COA repair with no residual arch obstruction. The challenges in accurate blood pressure assessment at this early phase are considered and non-invasive measures of central blood pressure are discussed. Although the pathways for investigations in adults are well defined, we highlight the need to address the issues of cardiovascular surveillance in children and describe techniques which can provide complementary information for cardiovascular assessment in this group of patients such that timely treatment can occur.
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http://dx.doi.org/10.1007/s00246-017-1739-xDOI Listing
January 2018

Persistently elevated nuchal translucency and the fetal heart.

J Matern Fetal Neonatal Med 2018 Sep 4;31(18):2376-2380. Epub 2017 Jul 4.

a Harris Birthright Research Centre for Fetal Medicine, Fetal Medicine Research Institute, King's College Hospital , Denmark Hill, London , UK.

Objective: To describe the outcome of fifteen cases with an elevated nuchal translucency (NT) which persisted into the second trimester as nuchal edema (NE) >6 mm whom underwent fetal echocardiography.

Materials And Methods: Cases were identified following retrospective review of cardiac and genetic findings in fetuses with NE.

Results: Minor congenital heart disease was identified in 3/15 by the second trimester. Agenesis of the ductus venosus was evident in four. Pulmonary valve stenosis was diagnosed in one fetus at the 20-week scan and hypertrophic cardiomyopathy in one. However, hypertrophic cardiomyopathy or pulmonary valve stenosis was present after birth in all surviving cases by 3 months of age. On the basis of intention to treat, 11/12 survived to delivery and 9/12 survived to 28 days. There were 6 deaths before 14 months of age as a result of severe hypertrophic cardiomyopathy. Noonan syndrome was confirmed with genetic testing in 11/15 cases.

Conclusions: All fetuses with NT and NE had evidence of congenital heart disease at birth, and therefore, late gestation and postnatal review is recommended even when second trimester echocardiogram is considered normal. There is a high prevalence of Noonan syndrome and targeted genetic analysis should be considered. The outcome in these cases is poor.
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http://dx.doi.org/10.1080/14767058.2017.1342804DOI Listing
September 2018

Usefulness of the Prenatal Echocardiogram in Fetuses With Isolated Transposition of the Great Arteries to Predict the Need for Balloon Atrial Septostomy.

Am J Cardiol 2017 05 9;119(9):1463-1467. Epub 2017 Feb 9.

Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's & St Thomas' NHS Foundation Trust, London, United Kingdom.

The outcome of the arterial switch operation for transposition of the great arteries (TGA) is excellent, but there is still associated preoperative mortality. Hypoxemia due to inadequate mixing of the pulmonary and systemic circulations may be implicated. Prediction of early hypoxemia by prenatal echocardiographic criteria has proved difficult. We aimed to identify prenatal echocardiographic features that may predict the need for emergency balloon atrial septostomy (BAS) in isolated TGA. Third trimester fetal echocardiograms of the last 40 cases of isolated TGA were reviewed without knowledge of the postnatal outcome. Measurements of the arterial valves, arterial duct, total septal length (TSL), and foramen ovale (FO) length were made, in addition to a subjective assessment of the atrial septum. The first postnatal echocardiogram and charts were reviewed. Comparison with 40 gestation-matched control fetuses was performed. The FO length in normal fetuses was not significantly different from those with TGA who did not require an emergency BAS but was significantly smaller in fetuses with TGA who required an emergency BAS (p = 0.01). An emergency BAS was required in 12 of 40 cases. All 3 cases with limited movement of the atrial septum required emergency BAS. A hypermobile atrial septum was observed in 10 cases and was not associated with emergency BAS (p = 0.8). The FO:TSL was significantly smaller in those who required an emergency BAS with good predictive value (area under the receiver operating characteristics curve: 0.80). The sensitivity for FO:TSL <0.5 was 99%. There was no significant difference in arterial duct, pulmonary valve, or branch pulmonary artery diameters between those cases requiring emergency BAS and those who did not. In conclusion, the likelihood of an emergency BAS is increased by FO:TSL <0.5 and a fixed appearance of the flap valve. Hypermobile and/or aneurysmal atrial septum did not indicate inadequate postnatal mixing in our group.
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http://dx.doi.org/10.1016/j.amjcard.2017.01.017DOI Listing
May 2017

Parvovirus B19 myocarditis in children: an observational study.

Arch Dis Child 2016 Feb 27;101(2):177-80. Epub 2015 Nov 27.

Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

Background: The advent of PCR testing for the presence of viral genomes has led to the identification of parvovirus B19 (PVB19) as a causative agent of myocarditis.

Methods: The clinical presentation, course and outcome of children with PVB19 myocarditis was ascertained through a retrospective review. The PVB19 viral genome was detected by PCR from whole blood or endomyocardial biopsy specimens in patients presenting with new onset heart failure.

Results: Seventeen patients presented at a median age of 1.3 years (range: 0.4-15.4 years) in cardiac failure with a mean fractional shortening of 15±3%. Eleven patients required mechanical ventilation and intravenous inotropes and seven required extra-corporeal mechanical oxygenation. Four of the five deaths occurred in patients who had a short prodromal illness of less than 48 hours. All patients with ST segment elevation died (n=4). All non-fulminant cases survived. Event-free survival occurred in 11/17 (65%) patients. Five (29%) patients died and one patient underwent heart transplantation. Complete recovery of cardiac function occurred within a median of 12 months (range: 1-48) in five patients. There was incomplete recovery in five patients and one patient had persistent dilated cardiomyopathy.

Conclusions: PVB19 can cause a devastating myocarditis in children. Children with fulminant myocarditis, ST segment changes or a short prodrome have the worst outcome. Transplantation may be considered, but is rarely required in the acute period if mechanical circulatory support is utilised. If the initial presentation is survived, recovery of the myocardium can occur even in those who had fulminant myocarditis.
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http://dx.doi.org/10.1136/archdischild-2014-308080DOI Listing
February 2016

Correlation of maternal flecainide concentrations and therapeutic effect in fetal supraventricular tachycardia.

Heart Rhythm 2014 Nov 25;11(11):2047-53. Epub 2014 Jul 25.

Department of Congenital Heart Disease, Evelina London Children's Hospital, Guy's and St. Thomas' Hospitals, London, United Kingdom.

Background: Transplacental flecainide is an established therapy for fetal supraventricular tachycardia (SVT), but there is a paucity of data regarding the dose-response relationship.

Objective: The purpose of this study was to investigate the relationship between maternal flecainide concentrations, arrhythmia control, and adverse fetal effects in fetal SVT.

Methods: Fetuses with SVT treated with transplacental flecainide at our tertiary fetal cardiology unit between 1997 and 2012 were retrospectively studied. The maternal trough flecainide concentrations throughout treatment were collated, and clinical notes were reviewed to ascertain the response to therapy and fetal outcome.

Results: Thirty-three fetuses were treated at a median (range) gestation of 28 weeks (20-38 weeks). Median fetal heart rate was 250/min (range 207-316/min). One patient was lost to follow-up, and this fetus was excluded from further analysis. In total, 25 of 32 fetuses (78%) converted to sinus rhythm. Median time to conversion to sinus rhythm was 3 days (range 2-12 days). Median flecainide concentration was 460 μg/L (range 250-866 μg/L) at conversion to sinus rhythm. Flecainide concentrations were not significantly different between responders and nonresponders (P = .849). Twelve of 14 hydropic and 13 of 18 nonhydropic fetuses converted to sinus rhythm with similar flecainide concentrations (P = .316). No fetus achieved cardioversion with a maternal serum flecainide concentration <250 μg/L. No fetus died while being treated with flecainide.

Conclusion: The clinical response to flecainide appears good, even in hydropic fetuses. Trough maternal flecainide concentrations, once therapeutic, do not predict cardioversion in the fetus with SVT. Flecainide therapy appears both safe and effective for the fetus when monitored appropriately.
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http://dx.doi.org/10.1016/j.hrthm.2014.07.031DOI Listing
November 2014