Wayne Tworetzky

Harvard Medical School, Boston, Massachusetts, United States

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Publications (89)560.23 Total impact

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    ABSTRACT: Fetal aortic valvuloplasty (FAV) has shown promise in averting the progression of fetal aortic stenosis to hypoplastic left-heart syndrome. Altered loading conditions due to valvar disease, intrinsic endomyocardial abnormalities, and procedures that alter endomyocardial mechanics may place patients with biventricular circulation (BiV) after FAV at risk of abnormal LV remodeling and function. Using the most recent echo data on BiV patients after technically successful FAV (n = 34), we evaluated LV remodeling pattern, risk factors for pathologic LV remodeling, and the association between LV remodeling pattern and LV function. Median age at follow-up was 4.7 years (range 1.0-12.5). Cardiac interventions were common. At latest follow-up, no patient had hypoplastic LV. Nineteen patients (55 %) had dilated LV, and five (16 %) patients had severely dilated LV. LV remodeling patterns were as follows: 12 (35 %) normal ventricle, 11 (32 %) mixed hypertrophy, 8 (24 %) eccentric hypertrophy or remodeling, and 3 (9 %) concentric hypertrophy. Univariate factors associated with pathologic LV remodeling were long-standing AR, ≥2 cardiac interventions, EFE resection, and aortic or mitral regurgitation ≥ moderate at most recent follow-up. In multivariate analysis, only long-standing AR fraction remained associated with pathologic remodeling. Pathologic LV remodeling was associated with depressed ejection fraction, lower septal E´, and higher E/E´. Pathologic LV remodeling, primarily eccentric or mixed hypertrophy, is common in BiV patients after FAV and is related to LV loading conditions imposed by valvar disease. Pathologic remodeling is associated with both systolic and diastolic dysfunction in this population.
    Pediatric Cardiology 05/2015; DOI:10.1007/s00246-015-1193-6 · 1.55 Impact Factor
  • American Journal of Obstetrics and Gynecology; 01/2015
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    ABSTRACT: To evaluate temporal trends in prenatal diagnosis of transposition of the great arteries with intact ventricular septum (TGA/IVS) and its impact on neonatal morbidity and mortality. Newborns with TGA/IVS referred for surgical management to our center over a 20-year period (1992 - 2011) were included. The study time was divided into 5 four-year periods, and the primary outcome was rate of prenatal diagnosis. Secondary outcomes included neonatal pre-operative status and perioperative survival. Of the 340 patients, 81 (24%) had a prenatal diagnosis. Prenatal diagnosis increased over the study period from 6% to 41% (p < 0.001). Prenatally diagnosed patients underwent a balloon atrial septostomy (BAS) earlier than postnatally diagnosed patients (0 vs. 1 day, p < 0.001) and fewer required mechanical ventilation (56% vs. 69%, p = 0.03). There were no statistically significant differences in pre-operative acidosis (16% vs. 26%, p = 0.1) and need for preoperative ECMO (2% vs. 3%, p = 1.0). There was also no significant mortality difference (1 pre-operative and no post-operative deaths among prenatally diagnosed patients, as compared to 4 pre-operative and 6 post-operative deaths among postnatally diagnosed patients). The prenatal detection rate of TGA/IVS has improved but still remains below 50%, suggesting the need for strategies to increase detection rates. The mortality rate was not statistically different between pre- and postnatally diagnosed patients; however, there were significant pre-operative differences with regard to earlier BAS and less mechanical ventilation. Ongoing study is required to elucidate whether prenatal diagnosis confers long-term benefit. This article is protected by copyright. All rights reserved.
    Ultrasound in Obstetrics and Gynecology 12/2014; 45(6). DOI:10.1002/uog.14751 · 3.14 Impact Factor
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    ABSTRACT: Objectives To better understand the natural history and spectrum of fetal aortic stenosis (AS), we aimed to 1) determine the prenatal diagnosis rate of neonates with critical AS and a biventricular (BV) outcome; and 2) describe the findings at fetal echocardiography in prenatally diagnosed patients.MethodsA multi-center, retrospective study was performed from 2000 to 2013. Neonates with critical AS who were discharged with a BV outcome were included. The prenatal diagnosis rate was compared to that reported for hypoplastic left heart syndrome (HLHS). Fetal echocardiographic findings in prenatally diagnosed patients were reviewed.ResultsOnly 10 of 117 neonates (8.5%) with critical AS and a BV outcome were diagnosed prenatally, a rate significantly lower than that for HLHS in the contemporary era (82%; p < 0.0001). Of the 10 patients diagnosed prenatally, all developed LV dysfunction by a median gestational age of 33 weeks (range, 28-35). When present, Doppler abnormalities such as retrograde flow in the aortic arch (n = 2), monophasic mitral inflow (n = 2), and left to right flow across the foramen ovale (n = 8) developed late in gestation (median 33 weeks).Conclusion The prenatal diagnosis rate among neonates with critical AS and a BV outcome is very low, likely due to a relatively normal 4-chamber view in mid-gestation with development of significant obstruction in the 3rd trimester. This natural history contrasts with that of severe mid-gestation AS with evolving HLHS and suggests that the timing in gestation of significant AS has an important impact on subsequent left heart growth in utero.
    Ultrasound in Obstetrics and Gynecology 09/2014; 45(3). DOI:10.1002/uog.14667 · 3.14 Impact Factor
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    ABSTRACT: Background-Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported. Methods and Results-We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P<0.01). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96 +/- 4% at 5 years and 84 +/- 12% at 10 years, which was better than HLHS patients (log-rank P= 0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was + 1.7 (range, -1.3 to +8.2), and 80% had normal ejection fraction. Conclusions-Short-and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted.
    Circulation 07/2014; 130(8). DOI:10.1161/CIRCULATIONAHA.114.009032 · 14.95 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; 45(6). DOI:10.1002/uog.14634 · 3.14 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 07/2014; 84(1). DOI:10.1002/ccd.25098 · 2.40 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; 31(7). DOI:10.1055/s-0034-1383884 · 1.60 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; 114(4). DOI:10.1016/j.amjcard.2014.05.042 · 3.43 Impact Factor
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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; 129(21). DOI:10.1161/01.cir.0000437597.44550.5d · 14.95 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; 114(1). DOI:10.1016/j.amjcard.2014.04.013 · 3.43 Impact Factor
  • Journal of the American College of Cardiology 04/2014; 63(12):A514. DOI:10.1016/S0735-1097(14)60514-3 · 15.34 Impact Factor
  • Journal of the American College of Cardiology; 04/2014
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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; 35(6). DOI:10.1007/s00246-014-0874-x · 1.55 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; 35(1). DOI:10.1159/000341717 · 2.30 Impact Factor
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    ABSTRACT: Purpose Transposition of the great arteries with intact ventricular septum (TGA/IVS), despite being a major heart defect, has been shown to have a low rate of prenatal diagnosis (Dx). This is likely due to the obstetric screening technique using a four-chamber view. In TGA/IVS changes occur after birth that untreated may lead to hemodynamic compromise, acidosis and death. Prenatal Dx allows for delivery site planning and timely neonatal management. The aim of this study was to evaluate the temporal trend in prenatal Dx of TGA/IVS and its impact on neonatal morbidity and mortality. Methods We included all neonates with TGA/IVS from the New England region, our natural catchment area, referred for surgical repair to Boston Children’s Hospital during the period 1992 to 2012. The 20 year study was divided into five-year periods. We compared pre and post-op variables between those diagnosed pre or postnatally. Pre-op variables included prenatal Dx, gestational age and weight at birth, age at admission, need for mechanical ventilation and ECMO, metabolic acidosis, timing of septostomy and surgery, and mortality. Post-op variables included need for ECMO, mortality and ICU and hospital stay. Results A total of 340 neonates were included. Of the 340 patients, 81(24%) had prenatal Dx at a median gestational age of 22.5 weeks (range17-38). There was an increase in the prenatal detection rate over the study period, from 6%, at the first period (1992-1996), to 42% at the last period (2008-2012) (p<0.0001). Gestational age at birth was lower in the prenatal group (38.3 vs. 38.8 weeks, p<0.05) but birth weight was similar between groups (3290 vs. 3350 gm). Age at admission (0 vs. 1.5 days, p<0.0001), at septostomy (0.3 vs.1.3 days, p<0.001) and surgery (4 vs. 4.7 days, p<0.05) were significantly lower in the prenatal Dx group. The prenatal Dx group had less metabolic acidosis (16% vs. 26%, p<0.05), need for mechanical ventilation (55% vs. 69%, p<0.05) and need for ECMO (2.5% vs. 2.7%, p=0.63) prior to surgery. The overall mortality was low at 3.2%. In the postnatal Dx group 10/259 patients died, 4 pre-op and 6 post-op. There was 1 death (pre-op) in the prenatal Dx group due to a complication related to the atrial septostomy. Hospital stay was longer (16.2 vs. 14.5 days) but ICU stay (6.2 vs. 6.7 days) was shorter for the prenatal Dx group, without significant differences between groups. Conclusion Prenatal detection rate of TGA/IVS increased significantly over the study period, but is still disappointingly low at less than 50%. Although the mortality rate was not different between pre and postnatal Dx groups, patients with prenatal Dx had significantly less metabolic acidosis and need for mechanical ventilation as well as earlier admission, septostomy and surgery.
    2013 American Academy of Pediatrics National Conference and Exhibition; 10/2013
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    ABSTRACT: Purpose Congenital atrioventricular block (AVB) as part of heterotaxy syndrome (HS) is an uncommon disease with low survival rates (10-25%), despite early diagnosis and aggressive medical management. The purpose of this study was to determine the mortality rate of patients with HS and complete AVB after prenatal diagnosis and determine the potential risk factors associated with non-survival. Methods Fetuses with HS and bradycardia or AVB diagnosed at a single large cardiac referral center, from 1995 to 2011 were included. We studied pre and post-natal variables. The primary outcome was non-survival, including termination of pregnancy, fetal demise or postnatal death. The secondary outcome was pacemaker placement. Results There were 154 fetuses with HS, 22(14.3%) with bradycardia or AVB. The median gestational age at diagnosis was 23 weeks (range 16-38). Thirteen (59%) patients had sinus bradycardia at diagnosis, 8(36%) complete AVB, and 1(5%) second degree AVB. Median ventricular rate (VR) at diagnosis among those with sinus bradycardia was 100 beats per minute (bpm) (range 60-100), decreasing to 80 bpm (55-100) before the end of pregnancy. Median VR among those with AVB was 60 bpm (50-67) at both diagnosis and before the end of the pregnancy. Fifteen patients (68%) survived to birth, with four (18%) fetal deaths and three (14%) elective termination of pregnancy. Thirty percent of fetuses presented hydrops, 20% had VR <55 bpm, and 10% presented cardiac dysfunction. Of the four fetal deaths, two had sinus bradycardia and two had AVB, all of them with hydrops. Excluding terminations, 15/19 fetuses (79%) survived to birth. Among the 15 live-born patients, 3 had bradycardia and 12(80%) had AVB. Twelve patients (80%) of the live-born cohort needed pacemaker placement. Three patients died in infancy, all with AVB and pacemakers implanted in the neonatal period. These three patients were delivered prematurely because the development of hydrops and two of them died during the first week of life. The remaining patient died at 3 months of age from non-cardiac causes. Nine out of the twelve surviving patients required pacemaker placement: eight for complete AVB [median VR of 60 bpm (30-70)], and one for sinus node dysfunction (VR 60 bpm). The overall survival rate, starting with fetal diagnosis, among those continued pregnancies was 63%(12/19) after a mean follow-up of 5.7 years (0.3-15 years). Risk factors for non-survival included hydrops (p<0.0001), prematurity (p=0.04), ventricular dysfunction (p=0.002) and VR <55 bpm (p=0.04). Conclusion In our series, patients with prenatal diagnosis with HS and AVB or bradycardia had a high survival rate (63%). Hydrops, prematurity, cardiac dysfunction and low ventricular rates were risk factors for non-survival. Fetuses with HS and bradycardia, either sinus or due to AVB, should be closely followed during pregnancy to monitor the development of hydrops.
    2013 American Academy of Pediatrics National Conference and Exhibition; 10/2013
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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; 96(4). DOI:10.1016/j.athoracsur.2013.05.060 · 3.65 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; 9(3). DOI:10.1111/chd.12120 · 1.20 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; 60(19). DOI:10.1016/j.jacc.2012.07.041 · 15.34 Impact Factor

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  • 2006–2014
    • Harvard Medical School
      • Department of Pediatrics
      Boston, Massachusetts, United States
  • 2004–2014
    • Harvard University
      Cambridge, Massachusetts, United States
  • 2003–2014
    • Boston Children's Hospital
      • Department of Cardiac Surgery
      Boston, Massachusetts, United States
  • 2012
    • University of Massachusetts Boston
      Boston, Massachusetts, United States
  • 2011
    • Brigham and Women's Hospital
      Boston, Massachusetts, United States
  • 1998–2002
    • University of California, San Francisco
      • • Division of Hospital Medicine
      • • Division of Pediatric Cardiothoracic Surgery
      • • Division of Adult Cardiothoracic Surgery
      San Francisco, California, United States