Wayne Tworetzky

Harvard Medical School, Boston, Massachusetts, United States

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Publications (80)452.49 Total impact

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    ABSTRACT: -Fetal aortic valvuloplasty (FAV) can be performed for severe mid-gestation aortic stenosis (AS) in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after FAV. The postnatal outcomes and survival of the BV patients, compared to those managed as HLHS, have not been reported.
    Circulation 07/2014; · 15.20 Impact Factor
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    ABSTRACT: Objectives We investigated perinatal outcomes after fetal diagnosis of single ventricle cardiac defects. ‘Single ventricle’ was defined as a dominant RV or LV, in which biventricular circulation was not possible.Methods We reviewed patients with a fetal diagnosis of single ventricle cardiac defect at one institution from 1995–2008. Diagnoses such as double-inlet left ventricle, tricuspid atresia, pulmonary atresia with intact ventricular septum and severe RV hypoplasia, and hypoplastic left heart syndrome (HLHS) were included. HLHS patients were prenatally identified as ‘standard risk’ and ‘high risk’ groups (HLHS with highly restrictive or intact atrial septum, mitral stenosis with aortic atresia, and/or LV coronary artery sinusoids). Patients with an address outside the U.S., heterotaxy syndrome, and referrals for fetal intervention were excluded.ResultsWe identified 312 prenatally diagnosed single ventricle cardiac defects (208 dominant RV; 104 dominant LV). Most (96%) of dominant RV patients had HLHS. There were 98 (31%) elective pregnancy terminations, 12 (4%) spontaneous fetal demises, 12 (4%) prenatal lost to follow-up and 190 (61%) live born. Of the 199 patients with a fetal echocardiogram at <24 weeks, there were 97 (49%) elective pregnancy terminations. There were no differences in prenatal outcome between dominant RV vs. dominant LV (p = 0.9). Of 190 live born infants, 5 received comfort care. With ~7 average years of follow-up through Fontan completion, there was lower transplant free survival in dominant RV versus dominant LV defects (‘standard risk’ HLHS odds ratio 3.0, p = 0.01; ‘high risk’ HLHS odds ratio 8.8, p < 0.001).Conclusions Whereas the prenatal outcomes of single ventricle cardiac defects were similar, postnatal intermediate-term survival favored those with dominant LV. Prenatally identified ‘high risk’ HLHS was associated with the lowest transplant free survival.
    Ultrasound in Obstetrics and Gynecology 07/2014; · 3.56 Impact Factor
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    ABSTRACT: Introduction Prenatal diagnosis provides valuable information regarding a variety of congenital heart defects. Some defects occur early in gestation with little change throughout pregnancy, whereas others evolve during mid and late gestation. Fetal cardiac intervention (FCI) affords the opportunity to interrupt progression of disease in this latter category, resulting in improved perinatal and lifelong outcomes. Aim This chapter addresses three lesions for which percutaneous FCI can be utilized: (1) aortic stenosis with evolving hypoplastic left heart syndrome, for which aortic valvuloplasty may prevent left ventricular hypoplasia and has yielded a biventricular circulation in approximately one third of cases; (2) hypoplastic left heart syndrome with intact atrial septum, for which relief of atrial restriction has potential to improve perinatal survival; and (3) pulmonary atresia with intact ventricular septum and evolving right ventricular hypoplasia, for which pulmonary valvuloplasty has resulted in a biventricular circulation in the majority of patients. The pathophysiology, rationale for intervention, patient selection criteria, procedural technique, and outcomes for each lesion will be reviewed. This chapter will also review complications of FCI and their treatment, and maternal and fetal anesthesia specific to FCI. The importance of a specialized center with experience managing infants delivered after FCI will also be addressed.
    American Journal of Perinatology 06/2014; · 1.57 Impact Factor
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    ABSTRACT: Patients with heterotaxy syndrome (HS) have a range of anomalies and outcomes. There are limited data on perinatal outcomes after prenatal diagnosis. To determine the factors influencing perinatal and infant outcomes, we analyzed prenatal and postnatal variables in fetuses with HS from 1995 to 2011. Of 154 fetuses with HS, 61 (40%) had asplenia syndrome (ASP) and 93 (60%) had polysplenia syndrome (PSP). In the ASP group, 22 (36%) patients were elected for termination of pregnancy, 4 (10%) had fetal death, and 35 of 39 (90%) continued pregnancies were live born. In the PSP group, 12 (13%) patients were elected for termination of pregnancy, 5 (6%) had fetal death (4 with bradyarrhythmia), and 76 of 81 (94%) continued pregnancies were live born. Bradyarrhythmia was the only predictor of fetal death. In the live-born ASP group, 43% (15 of 35) died, 7 because of pulmonary vein stenosis, 4 postoperatively, and 4 because of noncardiac causes. In the live-born PSP group, 13% (10 of 76) died, 5 postoperatively, 2 from bradyarrhythmia, 1 from a cardiac event, and 2 from noncardiac causes. Pulmonary vein stenosis and noncardiac anomalies were independent risk factors for postnatal death. Only 8% of ASP patients achieved biventricular circulation, compared with 65% of PSP patients. In the live-born cohort, the 5-year survival rate was 53% for ASP and 86% for PSP. In conclusion, most PSP patients are currently alive with biventricular circulation in contrast with few ASP patients. Bradyarrhythmia was the only predictor of fetal death. Pulmonary vein stenosis and noncardiac anomalies were predictors of postnatal death.
    The American journal of cardiology. 06/2014;
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    ABSTRACT: The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin-twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.
    Circulation 04/2014; · 15.20 Impact Factor
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    ABSTRACT: Fetal aortic balloon valvuloplasty (FAV) has shown promise in averting progression of midgestation aortic stenosis (AS) to hypoplastic left heart syndrome in a subset of patients. Patients who achieve biventricular circulation after FAV frequently have left ventricular (LV) diastolic dysfunction (DD). This study evaluates DD in fetuses with AS by comparing echocardiographic indices of LV diastolic function in fetuses underwent FAV (n = 20) with controls (n = 40) and evaluates for LV factors associated with DD in patients with FAV. We also compared pre-FAV and post-FAV DD variables (n = 16). Median gestational age (24 weeks, range 18 to 29 weeks) and fetal heart rate were similar between FAV and controls. Compared with controls, patients with FAV had universally abnormal LV diastolic parameters including fused mitral inflow E and A waves (p = 0.008), higher E velocity (p <0.001), shorter mitral inflow time (p = 0.001), lower LV lateral annulus E' (p <0.001), septal E' (p = 0.003), and higher E/E' (p <0.001) than controls. Patients with FAV had abnormal right ventricular mechanics with higher tricuspid inflow E velocity (p <0.001) and shorter tricuspid inflow time (p = 0.03). Worse LV diastolic function (lower LV E') was associated with higher endocardial fibroelastosis grade (r = 0.74, p <0.001), large LV volume (r = 0.55, p = 0.013), and sphericity (r = 0.58, p = 0.009) and with lower LV pressure by mitral regurgitation jet (r = -0.68, p <0.001). Post-FAV, fewer patients had fused mitral inflow E and A than pre-FAV (p = 0.05) and septal E' was higher (=0.04). In conclusion, fetuses with midgestation AS have evidence of marked DD. Worse DD is associated with larger, more spherical LV, with more extensive endocardial fibroelastosis and lower LV pressure.
    The American journal of cardiology 04/2014; · 3.58 Impact Factor
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    ABSTRACT: 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.
    Pediatric Cardiology 02/2014; · 1.20 Impact Factor
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    ABSTRACT: Objective: The impact of prenatal intervention on fetal cardiac function has not been well defined. We assessed standard ventricular function parameters and strain in fetuses with evolving hypoplastic left heart syndrome (HLHS) treated with fetal aortic valvuloplasty (fAVP). Methods: Fetuses with valvar aortic stenosis that underwent fAVP were studied. Echocardiographic images prior to intervention (Pre), within 1 week after fAVP (Post), and at the last prenatal follow-up examination (FU) were analyzed. Left ventricular (LV) circumferential (LVCS) and longitudinal strain (LVLS), right ventricular (RV) longitudinal strain (RVLS), and LV end-diastolic dimension Z-scores (LVIDD-Z) were documented and compared according to postnatal outcome. Results: Among 57 fetuses studied, the postnatal outcome was biventricular in 23 and univentricular in 34. Prior to fAVP, strain was <4 in most cases, regardless of outcome. Biventricular fetuses had higher LVCS and LVLS segmental strain than univentricular fetuses. Among fetuses with a biventricular outcome, LVCS and LVLS increased as LVIDD-Z decreased in late gestation, whereas LVCS and LVLS remained <4 in univentricular fetuses, although the LVIDD-Z decreased to <0 in all cases. Septal RVLS increased after fAVP in the biventricular but not the univentricular outcome group. Conclusion: In utero aortic valve dilation appears to have a beneficial effect on both LV and RV function in some fetuses with evolving HLHS. © 2013 S. Karger AG, Basel.
    Fetal Diagnosis and Therapy 11/2013; · 1.90 Impact Factor
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    ABSTRACT: Patients with borderline small left heart (LH) structures who initially undergo single ventricle palliation (SVP) may eventually become candidates for biventricular conversion (BC). The purpose of this study was to describe our surgical experience with BC in patients with small LH. We reviewed our institution's records for patients who underwent BC after an initial SVP between 1995 and 2012. Patients underwent an aortopulmonary amalgamation procedure as a part of their initial palliation. Data on imaging, BC operative details, and re-interventions after BC were collected. Twenty-eight patients underwent BC. Twenty patients had hypoplastic left heart syndrome (HLHS), 7 patients had unbalanced common atrioventricular canal (uCAVC), and 1 had interrupted aortic arch with VSD. Stage of palliation at BC was stage 1 in 6 patients (21.4%), bidirectional Glenn in 19 (67.9%), and Fontan in 3 (10.7%). Prior to BC, the median left ventricular end-diastolic volume (LVEDV) by echocardiography was 58.1 mL/m(2) in the HLHS group and 28.1 mL/m(2) in the uCAVC group. After BC, the LVEDV increased to 91.3 mL/m(2) in the HLHS group and 58.5 mL/m(2) in the uCAVC group (p < 0.05 compared with pre-BC in both groups). Right ventricular pressure was less than half systemic in 8 patients (53.3% of those measured). Seventeen patients (61%) have required either catheter-based or surgical re-intervention. Twenty-five patients (89.3%) were alive at a median follow-up of 2.6 years. Biventricular conversion can be applied to patients with HLHS and uCAVC and borderline LH with acceptable short-term results. Left heart size increases after BC, but follow-up for potential left atrial hypertension is warranted.
    The Annals of thoracic surgery 07/2013; · 3.45 Impact Factor
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    ABSTRACT: Surgical management of tetralogy of Fallot (TOF) is increasingly moving toward valve-sparing approaches rather than transannular patch (TAP). We evaluate whether fetal pulmonary valve (PV) size is predictive of postnatal course and surgical approach in TOF. In this retrospective study, fetal and postnatal demographic, clinical, and echocardiographic data on 66 patients diagnosed prenatally with TOF were collected. We compared those with midgestation PV z-score > -3.5 to those with z-score ≤-3.5. We analyzed fetal and postnatal PV size and growth and outcomes between groups RESULTS: Gestational age at first fetal echo was 23 weeks (range 18-28). PV diameter and z-score on midgestation echo were 3.5 mm (1.3-6.0) and -2.8 (-0.5 to -6.0) respectively. Patients with PV z-score ≤ -3.5 on first fetal echo had smaller PV diameter (4.5 vs. 5.0 mm, P = .047) and PV z-score (-3.8 vs. -2.8, P < .001) in late gestation and at time of surgery (6.0 mm vs. 7.0 mm, P = .01; z-score = -2.9 vs. -1.7, P = .007). Similarly, those with smaller fetal PV z-score had smaller main and branch pulmonary arteries at time of surgery. PV growth rate over gestation was similar between groups, while after-birth PV growth rate was lower in those with smaller PV (0 mm/month vs. 0.6 mm/month, P = .002). Those with smaller pulmonary valve were more likely to be cyanotic (P = .05), to undergo surgery at <1 month (P < .01), and to have a TAP repair (P = .01). Among patients undergoing valve-sparing repair, those with smaller PV underwent more reinterventions for residual valvar PS (P < .01). Midgestation fetal PV size is predictive of postnatal PV and PA size in TOF. Midgestation PV size has implications for timing and type of surgical management as well as for need for reintervention in valve-sparing repair patients and is therefore important to consider in prenatal counseling for TOF fetuses.
    Congenital Heart Disease 07/2013; · 1.01 Impact Factor
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    ABSTRACT: The objective of this study was to describe our single-institution experience with prenatal atrial septal stent placement for fetuses with hypoplastic left heart syndrome and an intact atrial septum (HLHS/IAS). Infants born with HLHS/IAS are at high risk for neonatal death, despite maximal postnatal therapy. Prenatal atrial septoplasty by static balloon dilation has been effective in decompressing the left atrium in utero, but several factors have limited the size of septal defects. We attempted to overcome the limitations of balloon septoplasty using transcatheter atrial septal stents. All records from our institution of fetuses with HLHS/IAS that underwent prenatal atrial septal stent placement were reviewed, including operative notes and echocardiograms. Nine fetuses between 24 and 31 weeks gestation with HLHS/IAS underwent attempted fetal atrial septal stent placement. A stent was deployed across the atrial septum in five fetuses, with four fetuses demonstrating flow across the stent at the time of intervention. In four cases, stent placement failed due to malposition or embolization, but in three of the four cases, atrial balloon septoplasty at the same in-utero procedure successfully and acutely decompressed the left atrium. There were no maternal complications. There was one fetal demise. The remaining 8 fetuses survived to delivery, but four died in the neonatal period (2 of which had been stented). Ultrasound-guided atrial septal stent placement is feasible in some fetuses with HLHS/IAS. Visualization of the septum and catheter tip is critical to technical success. Additional experience is necessary to determine the clinical impact of this intervention. © 2013 Wiley Periodicals, Inc.
    Catheterization and Cardiovascular Interventions 06/2013; · 2.51 Impact Factor
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    ABSTRACT: OBJECTIVES: The goal of this study was to review results of a novel management strategy intended to rehabilitate the left heart (LH) in patients with LH hypoplasia who have undergone single-ventricle palliation (SVP). BACKGROUND: Management of patients with hypoplastic LH syndrome and borderline left ventricle (LV) involves 2 options: SVP or biventricular repair. We hypothesized that staged LV recruitment and biventricular conversion may be achieved after SVP by using a strategy consisting of relief of inflow and outflow tract obstructions, resection of endocardial fibroelastosis, and promotion of flow through the LV. METHODS: Patients with hypoplastic LH and borderline LV who underwent traditional SVP (n = 34) or staged LV recruitment (n = 34) between 1995 and 2010 were retrospectively analyzed and compared with a control SVP group. RESULTS: Mean initial z-scores for LH structures before stage 1 SVP were not significantly different between groups. Mortality occurred in 4 of 34 patients after LV recruitment and in 7 of 34 after traditional SVP. LH dimension z-scores increased significantly over time after LV recruitment, whereas they declined after traditional SVP, with significant interaction between stage of palliation and treatment group. Restriction of the atrial septum (conducted in 19 of 34 patients) was the only predictor of increase in left ventricular end-diastolic volume (p < 0.001). Native biventricular circulation was achieved in 12 patients after staged LV recruitment; all of these patients had restriction at the atrial septum. CONCLUSIONS: In these patients with borderline LH disease who underwent SVP, it is possible to increase LH dimensions by using an LV recruitment strategy. In a subset of patients, this strategy allowed establishment of biventricular circulation.
    Journal of the American College of Cardiology 09/2012; · 14.09 Impact Factor
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    ABSTRACT: Neurologic impairment is a major complication of complex congenital heart disease (CHD). A growing body of evidence suggests that neurologic dysfunction may be present in a significant proportion of this high-risk population in the early newborn period prior to surgical interventions. We recently provided the first evidence that brain growth impairment in fetuses with complex CHD has its origins in utero. Here, we extend these observations by characterizing global and regional brain development in fetuses with hypoplastic left heart syndrome (HLHS), one of the most severe forms of CHD. Using advanced magnetic resonance imaging techniques, we compared in vivo brain growth in 18 fetuses with HLHS and 30 control fetuses from 25.4-37.0 weeks of gestation. Our findings demonstrate a progressive third trimester fall-off in cortical gray and white matter volumes (P < 0.001), and subcortical gray matter (P < 0.05) in fetuses with HLHS. Significant delays in cortical gyrification were also evident in HLHS fetuses (P < 0.001). In the HLHS fetus, local cortical folding delays were detected as early as 25 weeks in the frontal, parietal, calcarine, temporal, and collateral regions and appear to precede volumetric brain growth disturbances, which may be an early marker of elevated risk for third trimester brain growth failure.
    Cerebral Cortex 09/2012; · 8.31 Impact Factor
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    ABSTRACT: Objective Neonatal congenital tricuspid valve (TV) dysplasia and/or displacement (Ebstein's malformation) with severe tricuspid regurgitation (TR) is a challenging condition in which outcomes are frequently poor. Little is known about left ventricular (LV) function during the perinatal period in patients with congenital TV disease. The objective of this study was to evaluate LV function in fetuses with congenital TV anomalies associated with significant TR.Methods Serial fetal echocardiograms in 16 fetuses with congenital TV dysplasia and/or displacement (five neonatal survivors and 11 fetal or neonatal deaths) were reviewed. LV stroke volume, LV end-diastolic volume (LVEDV), LV end-diastolic dimension (LVIDd), the LV eccentricity index, thoracic and cardiac areas and the cardiothoracic area ratio (CTAR), the right atrium area index, and LV longitudinal strains were compared according to gestational age and clinical outcome.ResultsThe gestational age-adjusted LVEDV (Z-score) was lower in late gestation (−1.2 ± 1.2 at last examination ≥ 28 weeks) than earlier in gestation (0.3 ± 1.5 at last examination < 28 weeks) and LV output was lower than reported late-gestation normal values. LV short-axis dimension correlated with LV volume and CTAR. LV mid-septal strain was lower than the normal average of fetal mid-septal strain and correlated with the LV eccentricity index. Among these parameters, only the LV eccentricity index differed between survivors and non-survivors.ConclusionLV function and anatomy are abnormal in fetuses with severe congenital TV anomalies and may be important contributors to outcome. Copyright © 2012 ISUOG. Published by John Wiley & Sons, Ltd.
    Ultrasound in Obstetrics and Gynecology 06/2012; 40(1):55 - 61. · 3.56 Impact Factor
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    ABSTRACT: In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.
    Journal of the American College of Cardiology 01/2012; 59(1 Suppl):S1-42. · 14.09 Impact Factor
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    ABSTRACT: Echocardiography with speckle tracking is a novel, angle-independent technique for assessing global and regional cardiac function. Normal data on longitudinal strain have been reported for human fetuses. The aim of this study was to define circumferential left ventricular (LV) strain in a normal fetal population. Singleton fetuses between 17 and 42 weeks in gestational age with no adverse maternal health issues or fetal abnormalities were studied. Regional and averaged cardiac strain were measured using syngo Velocity Vector Imaging software. Data from 81 fetuses were analyzed (mean gestational age, 29.2 ± 5.7 weeks). Overall, average midventricular circumferential strain was 18.7 ± 3.3%, LV longitudinal strain was 15.2 ± 2.7%, and right ventricular longitudinal strain was 16.0 ± 3.3%, with no correlation with gestational age. This is the first study to report normal fetal LV circumferential strain. These data may be useful as a reference for assessing fetal cardiac function. The retrospective study design and relatively low frame rates used in this study were important limitations.
    Journal of the American Society of Echocardiography: official publication of the American Society of Echocardiography 01/2012; 25(1):105-11. · 2.98 Impact Factor
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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. · 1.01 Impact Factor
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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
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    ABSTRACT: Fetal aortic balloon valvuloplasty (FAV) has shown promise in altering in utero progression of aortic stenosis to hypoplastic left heart syndrome. In patients who achieve a biventricular circulation after FAV, left ventricular (LV) compliance may be impaired. Echocardiographic indexes of diastolic function were compared between patients with biventricular circulation after FAV, congenital aortic stenosis (AS), and age-matched controls. In the neonatal period, patients with FAV had similar LV, aortic, and mitral valve dimensions but more evidence of endocardial fibroelastosis than patients with AS. Patients with FAV underwent more postnatal cardiac interventions than patients with AS (p = 0.007). Mitral annular early diastolic tissue velocity (E') was lower in patients with FAV and those with AS and controls in the neonatal period and over follow-up (p <0.001). Septal E' was similar among all 3 groups in the neonatal period. In follow-up patients, with FAV had lower septal E' than patients with AS or controls (p <0.001). Early mitral inflow velocity/E' was higher in patients with FAV as neonates and at follow-up (p <0.001). Mitral inflow pulse-wave Doppler-derived indexes of diastolic function were similar between groups. In conclusion, echocardiographic evidence of LV diastolic dysfunction is common in patients with biventricular circulation after FAV and persists in short-term follow-up. LV diastolic dysfunction in this unique population may have important implications on long-term risk of left atrial and subsequent pulmonary hypertension.
    The American journal of cardiology 05/2011; 108(4):556-60. · 3.58 Impact Factor
  • Prenatal Diagnosis 03/2011; 31(3):249-51. · 2.68 Impact Factor

Publication Stats

2k Citations
452.49 Total Impact Points

Institutions

  • 2004–2014
    • Harvard Medical School
      • Department of Pediatrics
      Boston, Massachusetts, United States
  • 2003–2014
    • Boston Children's Hospital
      • Department of Cardiac Surgery
      Boston, Massachusetts, United States
  • 2011
    • Brigham and Women's Hospital
      Boston, Massachusetts, United States
  • 2000–2010
    • University of California, San Francisco
      • Division of Pediatric Cardiothoracic Surgery
      San Francisco, California, United States
  • 1999
    • CSU Mentor
      Long Beach, California, United States