Melanie Vogel

Boston Children's Hospital, Boston, MA, USA

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Publications (9)35.5 Total impact

  • Article: Dilation of the ascending aorta after balloon valvuloplasty for aortic stenosis during infancy and childhood.
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    ABSTRACT: Dilation of the ascending aorta (AA) is common in patients with a bicuspid aortic valve. The natural history of the aortic root and AA and the risk factors for dilation have not been characterized in patients with congenital aortic stenosis (AS) treated with balloon valvuloplasty during childhood. The present study was performed to determine the prevalence of aortic dilation in patients with congenital AS before and up to 20 years after balloon valvuloplasty performed during childhood. In patients who underwent balloon valvuloplasty for AS at age ≤ 18 years from 1984 to 2005, the aortic diameter measurements before intervention and at 5-year intervals afterward were recorded and the Z scores calculated. Among 156 patients (median age 1.5 years at valvuloplasty), the AA Z scores were significantly larger than normal before intervention (median Z score 1.5) and at all follow-up points (all p <0.001). Using mixed modeling, with time as a categorical variable (before intervention, 5-year window, 10-year window, and so forth), the mean AA Z score was greater at all postvalvuloplasty points than before the intervention, with mean Z score increases of 1.20 at 5 years and 2.11 at 20 years (p <0.001). Moderate or greater aortic regurgitation early after valvuloplasty was associated with greater AA Z scores than mild or less aortic regurgitation, with a progressive difference over time. More significant residual AS after valvuloplasty was associated with lower AA Z scores over time. In conclusion, AA dilation is common in children with congenital AS and continues to progress over many years after balloon valvuloplasty.
    The American journal of cardiology 05/2012; 110(5):702-8. · 3.58 Impact Factor
  • Article: Ebstein's malformation of the tricuspid valve: short-term outcomes of the "cone procedure" versus conventional surgery.
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    ABSTRACT:   We report our analysis of conventional surgery and the cone procedure for Ebstein's malformation (EM) of the tricuspid valve at a single institution. Previous conventional surgery for EM, including use of bioprosthetic valves, has inherent problems especially in pediatrics. The newer cone procedure aims to construct a funnel-like valve out of native leaflets, obviating problems with artificial valves.   This is a retrospective cohort study to examine short-term outcomes of both surgeries for EM.   Nineteen patients (our initial cohort) had the cone procedure, and 13 had conventional tricuspid valve repair or replacement. No early deaths occurred in either group. Three cone and one conventional repair patients required reoperation. Two of 19 patients in the cone and one of 13 in the conventional group died suddenly >30 days after operation, assumed secondary to dysrhythmias. At discharge, by two-dimensional echocardiography, the cone group had 85% reduction in tricuspid valve regurgitation (TVR), and the conventional group had 56% reduction, P= .004. This decrease of TVR persisted to a greater extent in the cone group.   Short-term results for the cone procedure are similar to conventional surgery. The cone procedure uses autologous tissue; hypothetically, early favorable improvement in reduction of TVR should persist.
    Congenital Heart Disease 12/2011; 7(1):50-8. · 0.90 Impact Factor
  • Article: Early reintervention on the pulmonary arteries and right ventricular outflow tract after neonatal or early infant repair of truncus arteriosus using homograft conduits.
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    ABSTRACT: Patients with truncus arteriosus often require pulmonary arterial (PA) and/or right ventricular outflow tract (RVOT) reintervention within the first year of repair. However, little is known about the risk factors for early reintervention on the PAs or RVOT in this population. The objective of the present retrospective cohort study was to determine the risk factors for early PA or RVOT reintervention after repair of truncus arteriosus in neonates and young infants. Of 156 patients ascertained (median age at repair 14 days; 143 early survivors), reinterventions on the RVOT and/or PAs were performed in 109. The first reintervention was catheter therapy in 73 patients (conduit dilation/stenting in 29, PA dilation/stenting in 31, both in 13) and conduit reoperation in 36 patients. The freedom from any RVOT or PA reintervention was 68 ± 4% at 1 year and 48 ± 5% at 2 years. The factors associated with early reintervention (shorter 1-year freedom from reintervention) on univariate analysis were repair quartile, neonatal repair, smaller weight at repair, and smaller implanted conduit size. On multivariable analysis, only smaller conduit size remained significant (multivariable hazard ratio 0.66/mm, range 0.53 to 0.83; p <0.001). The freedom from conduit reoperation was 92 ± 3% at 1 year and 76 ± 4% at 2 years. Overall, the left and right PA sizes were modestly larger than normal by the 1-sample t test, and PA Z scores and the PA area index were not associated with the risk of reintervention. Early reintervention for PA and/or RVOT conduit obstruction is common after neonatal and early infant repair of truncus arteriosus using homograft conduits. A smaller conduit size was associated with early RVOT/PA reintervention. The branch PA size was normal before surgery, suggesting that the PA stenosis in these patients resulted from factors other than intrinsic stenosis or hypoplasia.
    The American journal of cardiology 07/2011; 108(1):106-13. · 3.58 Impact Factor
  • Article: Aortic stenosis and severe mitral regurgitation in the fetus resulting in giant left atrium and hydrops: pathophysiology, outcomes, and preliminary experience with pre-natal cardiac intervention.
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    ABSTRACT: The objective of this article is to review anatomic, physiologic, and clinical features of fetuses and neonates with severe mitral regurgitation (MR) in conjunction with aortic stenosis (AS) and left ventricular (LV) and left atrial (LA) dilation and to present preliminary results of pre-natal intervention for this condition. Severe fetal valvar AS with an abnormal mitral valve (MV) and MR can lead to left heart dilation, with consequent compression of the right ventricle (RV); hydrops and low cardiac output are often associated. This is a retrospective review of fetuses diagnosed with AS, severe MR, and LA dilation (2002 to 2009) and neonates with the same combination of abnormalities (1988 to 2009). Fourteen fetuses and 7 neonates were investigated. Eleven fetuses had severe hydrops; all had polyhydramnios and a structurally abnormal MV, abnormal MV inflow pattern, restrictive/intact atrial septum, retrograde flow in the transverse aortic arch, and compression of the right heart. The mean indexed RV output was 326 ± 160 ml/kg/min, lower than the normal average fetal combined ventricular output of 550 ± 150 ml/kg/min. Ten fetuses underwent pre-natal cardiac intervention: aortic valvuloplasty (n = 8) and/or atrial septal dilation/stenting (n = 5). Seven of these, and 11 overall, were live born. Nine patients died (median age 6 days), and 2 patients are currently alive. All 7 patients diagnosed in the neonatal period died (median age 1 day). Aortic stenosis associated with significant MR in the fetus can cause severe LA and LV enlargement, leading to low cardiac output and hydrops. Despite the potential advantages of early pre-natal diagnosis and both fetal and neonatal interventions, this rare complex of anomalies carries a poor prognosis.
    Journal of the American College of Cardiology 01/2011; 57(3):348-55. · 14.16 Impact Factor
  • Article: Assessment of left ventricular endocardial fibroelastosis in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome.
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    ABSTRACT: Systematic evaluation of left ventricular (LV) endocardial fibroelastosis (EFE) in the fetus has not been reported. The role of EFE in the pre- and postnatal evolution of hypoplastic left heart disease, and the implications of EFE for outcomes after prenatal intervention for fetal aortic stenosis with evolving hypoplastic left heart syndrome have also not been determined. A 4-point grading system (0-3) was devised for the assessment of fetal LV echogenicity, which was presumed to be due to EFE. Two reviewers independently graded EFE on the preintervention echocardiograms of fetuses treated with in utero aortic valvuloplasty for evolving hypoplastic left heart syndrome from 2000 to 2008. Intra- and interobserver reproducibility was determined for the EFE grade and characterization of related echocardiographic features. The relations among EFE severity, other left heart anatomic and physiologic variables, and postintervention outcomes were analyzed. The assessment and grading of EFE was possible for both observers in all 74 fetuses studied. By consensus, the EFE severity was grade 1 in 31 patients, grade 2 in 32, and grade 3 in 11. Fetuses with mild (grade 1) EFE had significantly greater maximum instantaneous aortic stenosis gradients (e.g., higher LV pressures) and less globular LV geometry than patients with grade 2 or 3 EFE on preintervention echocardiogram. The severity of EFE was not associated with the size of the aortic valve or LV. From preintervention to late gestation, the time-indexed change in LV end-diastolic volume was significantly greater in fetuses with grade 1 EFE than those with more severe EFE. Incorporation of EFE severity into our previously published threshold score improved the sensitivity and positive predictive value for the postnatal biventricular outcomes. In conclusion, echocardiographic grading of EFE is possible, with reasonable intra- and interobserver reliability in midgestation fetuses with evolving hypoplastic left heart syndrome. EFE severity corresponded to some indexes of left heart size, geometry, and function and with the probability of a biventricular outcome postnatally. Additional experience and external validation of the EFE grading scoring system are necessary.
    The American journal of cardiology 12/2010; 106(12):1792-7. · 3.58 Impact Factor
  • Article: Fetal diagnosis of interrupted aortic arch.
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    ABSTRACT: To determine the frequency of prenatal detection among liveborn patients with an interrupted aortic arch (IAA), the accuracy of prenatal diagnosis, and the anatomic features associated with IAA in the fetus. The prenatal diagnosis of an IAA is challenging. The data on the features and outcomes of fetal IAA are limited. This was a retrospective review of the fetuses and neonates diagnosed with IAA at the Children's Hospital Boston. From 1988 to 2009, 26 fetuses were diagnosed with an IAA. Of these, 21 were live born, and 5 pregnancies were terminated. Of these 21 patients, 18 were confirmed to have an IAA after birth and 3 had severe aortic coarctation. Of the 56 patients diagnosed with an IAA as neonates, 3 had a prenatal echocardiogram that did not include the correct diagnosis. Among the liveborn patients with a postnatally confirmed IAA, 24% were diagnosed prenatally, which increased from 11% during the first 7-year period to 43% more recently. Also, 15% of the prenatally diagnosed patients with IAA had a family history of structural or genetic anomalies. In fetuses with an IAA, echocardiographic Z-scores for the aortic valve and ascending aorta were significantly lower than in normal fetuses, but the left ventricular dimensions were normal. Aortopulmonary diameter ratios were abnormally low. In conclusion, although the identification of IAA on a prenatal echocardiogram can be challenging, a number of anatomic features can facilitate the diagnosis. In particular, a low aortopulmonary diameter ratio in the absence of a ventricular size discrepancy should prompt consideration of this diagnosis. Despite the diagnostic challenges, the frequency of prenatal diagnosis of the IAA is increasing.
    The American journal of cardiology 03/2010; 105(5):727-34. · 3.58 Impact Factor
  • Article: Reversible ductus arteriosus constriction due to maternal indomethacin after fetal intervention for hypoplastic left heart syndrome with intact/restrictive atrial septum.
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    ABSTRACT: Fetal cardiac intervention (FCI) has been performed at our center in selected fetuses with complex congenital heart disease since 2000. Most interventions are performed in fetuses with a ductus arteriosus (DA)-dependent circulation. Indomethacin promotes closure of the DA in newborns and in fetal life, a potentially life threatening complication in fetuses with ductus-dependent congenital heart disease. We reviewed our experience with FCI with a focus on the frequency, features, and clinical course of ductal constriction. Fetuses undergoing FCI receive comprehensive pre- and postoperative cardiac and cerebral ultrasound evaluation, approximately 24 hours before and after the procedure, including imaging of DA flow and Doppler assessment of the umbilical artery and vein, ductus venosus, and, since 2004, the middle cerebral artery. Among 113 fetuses that underwent FCI, 24 of which were older than 28 0/7 weeks gestation, 2 were found to have DA constriction due to indomethacin therapy within 24 hours of intervention. Both of these were 30-week fetuses with hypoplastic left heart syndrome and restrictive or intact atrial septum. The DA was stenotic by spectral and color Doppler, and middle cerebral and umbilical artery pulsatility indexes were depressed. After discontinuation of indomethacin, the Doppler indices improved or normalized. Close echocardiographic monitoring of fetal Doppler flow velocities is very important after fetal intervention and indomethacin treatment, as the consequences of DA constriction in a fetus with hypoplastic left heart syndrome are potentially lethal. Sonographic evaluation should include measurement of cerebral and umbilical arterial flow velocities as well as color and spectral Doppler interrogation of the DA.
    Fetal Diagnosis and Therapy 12/2009; 27(1):40-5. · 1.05 Impact Factor
  • Article: Prevalence of increased nuchal translucency in fetuses with congenital cardiac disease and a normal karyotype.
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    ABSTRACT: Our aims were to estimate the prevalence of increased nuchal translucency in fetuses with a normal karyotype that were subsequently diagnosed with congenital cardiac disease on fetal echocardiography, and to assess whether there is a link between increased nuchal translucency and specific congenital cardiac malformations. We reviewed all patients referred to King's College Hospital and the Evelina Children's Hospital in London for fetal echocardiography between January 1998 and December 2007. We investigated the proportion of chromosomally normal fetuses with congenitally malformed hearts in which nuchal thickness was increased, both overall and with specific defects. We identified 2133 fetuses with congenital cardiac disease by prenatal echocardiography. Of those, 707 were excluded due to abnormal karyotype, and 690 were excluded due to unknown karyotype. The remaining 736 were eligible for inclusion. Among 481 fetuses with documented congenital cardiac disease and normal chromosomes, making up 23% of the overall cohort, 224 had increased nuchal thickness defined as equal or greater than 2.5 millimetres, this being 0.47 of the inclusive cohort, with 95% confidence intervals from 0.42 to 0.51. These proportions were significantly higher than the expected proportion of the normal population, which was 0.05 (p < 0.001). The only diagnosis for which the proportion of fetuses with nuchal translucency measurement equal or greater than 2.5 millimetres was higher than the others was atrioventricular septal defect, with 0.62 of this cohort having abnormal values, with 95% confidence intervals from 0.47 to 0.77 (p = 0.038). We found that nearly half of prenatally diagnosed fetuses with congenitally malformed hearts, when examined ultrasonically in the first or early-second trimester, had increased nuchal thickness. We recommend, therefore, referral of all fetuses with increased nuchal translucency for fetal echocardiography.
    Cardiology in the Young 07/2009; 19(5):441-5. · 0.76 Impact Factor
  • Article: End-stage cardiac failure caused by isolated ventricular non-compaction: cardiac transplantation in a 5-year-old boy.
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    ABSTRACT: Isolated ventricular non-compaction (IVNC) is a rare congenital cardiomyopathy characterized by arrest in endomyocardial morphogenesis. Presenting features in children include systolic and diastolic ventricular dysfunction, cardiac arrhythmias, and thromboembolism. End-stage cardiac failure necessitating cardiac transplantation is described in adults. We report the unique case of a 5-year-old boy with IVNC and end-stage heart failure who underwent cardiac transplantation. Diagnostic echocardiographic features, gross cardiac morphology, and histopathologic findings are illustrated.
    The Journal of Heart and Lung Transplantation 06/2005; 24(5):618-20. · 4.33 Impact Factor