J E Lock

Boston Children's Hospital, Boston, Massachusetts, United States

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Publications (325)2311.11 Total impact

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    ABSTRACT: The short-term surgical results for mixed aortic valve disease (MAVD) and the long-term effects on the left ventricle (LV) are unknown. Retrospective review identified patients with at least both moderate aortic stenosis (AS) and aortic regurgitation (AR) before surgical intervention. A one-to-one comparison cohort of patients with MAVD not referred for surgical intervention was identified. The 45 patients in this study underwent surgical management for MAVD. A control group of 45 medically managed patients with MAVD also was identified. Both groups had elevated LV end-diastolic volume (EDV), elevated LV mass, a normal LV mass:volume ratio (MVR), and a normal ejection fraction. Both groups had diastolic dysfunction shown by early diastolic pulsed-Doppler mitral inflow/early diastolic tissue Doppler velocity z-score. The LV end-diastolic pressure (EDP) was correlated with age (R = 0.4; p = 0.03) and LV MVR (R = 0.4; p = 0.03) but not with AS, AR, or the score combining gradient and LV size. As shown by 6- to 12-month postoperative echocardiograms, aortic valve gradients and AR significantly improved (gradient 65 ± 17 to 28 ± 18 mmHg, p = 0.01; median regurgitation grade moderate to mild; p < 0.01), LV EDV normalized, and LV mass significantly improved (p < 0.01). Diastolic dysfunction was unchanged. Symptoms did not correlate with any measured parameter, but the preoperative symptoms resolved. In conclusion, despite diastolic dysfunction, systolic function is invariably preserved, and symptoms are not correlated with aortic valve function or LV EDP. Current surgical practice preserves LV mechanics and results in short-term improvement in valve function and symptoms.
    Pediatric Cardiology 02/2014; · 1.20 Impact Factor
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    ABSTRACT: Peripheral and central pulmonary artery (PA) stenoses can result in right ventricular hypertension, dysfunction, and death. Percutaneous PA angioplasty and stent placement relieve obstruction acutely, but patients frequently require reintervention. Within a heterogeneous patient population with PA stents referred for catheterization because of noninvasive signs of PA obstruction, we have observed that in-stent stenosis (ISS) occurs commonly in some groups, challenging previous reports that this phenomenon occurs infrequently. We set out to evaluate the incidence and demographics of patients with previous PA stent placement who develop ISS. Consecutive patients with previously placed stents presenting for catheterization and undergoing PA angiography were reviewed (104 patients, 124 cases). We defined ISS angiographically, as a 25% narrowing of the contrast-filled lumen relative to the fluoroscopically apparent stent diameter at any site along the length of the stent. For inclusion, we required that the stenotic segment be narrower or equal in size to the distal vessel. ISS was diagnosed in 24% of patients, with the highest incidence among patients with tetralogy of Fallot and multiple aortopulmonary collaterals, Williams syndrome, or Alagille syndrome. In conclusion, ISS after PA stent placement is a more frequent problem than previously reported, and patients with inherently abnormal PAs are disproportionately affected. Increased clinical surveillance after stent placement and investigation of innovative preventive strategies may be indicated.
    The American journal of cardiology 02/2014; 113(3):541-5. · 3.58 Impact Factor
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    ABSTRACT: The options for mitral valve replacement in children with irreparable mitral valve disease have been limited to fixed-diameter prostheses that do not accommodate for somatic growth. We have modified an externally stented bovine jugular vein graft (Melody valve) for implantation in this cohort. Because it is not a fixed-diameter prosthesis, we hypothesized that the valve can be expanded in the catheterization laboratory as the child grows. The medical records of patients who had undergone Melody valve implantation in the mitral or left atrioventricular valve position from 2010 to 2013 were reviewed. Eleven patients had undergone Melody valve implantation at a median age of 7 months (range, 2-28). The techniques of valve modification and implantation included stent shortening, adding a pericardial sewing cuff, intraoperative balloon expansion, and fixation of the distal stent to the inferior left ventricle wall. The valve was competent, with a low gradient acutely postoperatively in all patients. One patient died, and one required permanent pacemaker implantation. One patient developed valve dysfunction and required explantation. Two patients without a pericardial sewing cuff developed paravalvular leaks. One patient who had not undergone distal stent fixation developed left ventricular outflow tract obstruction. Three patients who had undergone subsequent catheter-based balloon expansion of the valve have continued to demonstrate acceptable valvular function. The Melody valve has demonstrated acceptable short-term function. Implantation techniques to prevent left ventricular outflow tract obstruction (suture fixation of the distal stent) and paravalvular leaks (the addition of a pericardial cuff) should be considered. The Melody valve can be percutaneously expanded as the child grows.
    The Journal of thoracic and cardiovascular surgery 12/2013; · 3.41 Impact Factor
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    ABSTRACT: BACKGROUND: ANGIOPLASTY AND STENT PLACEMENT IN RIGHT VENTRICLE-TO-PULMONARY ARTERY (RV-PA) CONDUITS HAVE BEEN SHOWN TO PROLONG THE FUNCTIONAL LIFESPAN OF A CONDUIT. SAFETY AND EFFICACY OF ANGIOPLASTY OF OBSTRUCTED RV-PA HOMOGRAFTS USING ULTRA-NONCOMPLIANT (UNC) OR ULTRAHIGH-PRESSURE BALLOONS ARE UNKNOWN.METHODS AND RESULTS: FROM 2004 TO 2012, 70 PATIENTS UNDERWENT 76 PROCEDURES FOR ANGIOPLASTY OF RV-PA HOMOGRAFTS WITH UNCATLAS BALLOONS. THE UNC GROUP WAS COMPARED WITH A PARTIALLY CONTEMPORANEOUS CONTROL COHORT OF 81 PATIENTS WHO UNDERWENT 84 ANGIOPLASTY PROCEDURES WITH CONVENTIONAL BALLOONS. ACUTE HEMODYNAMIC CHANGES AFTER ANGIOPLASTY OF HOMOGRAFTS WITH UNC BALLOONS INCLUDED SIGNIFICANTLY REDUCED RV:AO PRESSURE RATIO (P=0.02) AND RIGHT VENTRICULAR OUTFLOW TRACT GRADIENTS (P0.001). BALLOON WAIST RESOLUTION WAS MORE FREQUENTLY ACHIEVED WITH UNC BALLOONS (P=0.04), AND BALLOON RUPTURE OCCURRED LESS OFTEN (P0.001). CONDUIT TEARS OF ANY SEVERITY OCCURRED IN 22% OF PATIENTS OVERALL AND WERE MORE COMMON IN THE UNC GROUP (P=0.001). PATIENTS WITH ANY CONDUIT TEAR HAD SIGNIFICANTLY GREATER REDUCTION IN THEIR RV:AO PRESSURE RATIO (P0.001) AND RIGHT VENTRICULAR OUTFLOW TRACT GRADIENT (P=0.004) THAN THOSE WITH NO TEAR. THERE WERE 4 UNCONFINED TEARS, ALL IN THE UNC GROUP, WITH NO ACUTE DECOMPENSATIONS OR DEATHS AND ONLY 1 PATIENT WHO REQUIRED SURGICAL MANAGEMENT.CONCLUSIONS: RV-PA conduit tears are common in patients undergoing angioplasty, but clinically important tears, which only occurred during UNC angioplasty in this series, were uncommon. UNC balloons can be used to good effect with significant reduction in right ventricular outflow tract gradient and the RV:Ao ratio when compared with conventional balloons.
    Circulation Cardiovascular Interventions 11/2013; · 6.54 Impact Factor
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    ABSTRACT: Standardization of care can reduce practice variation, optimize resource utilization, and improve clinical outcomes. We have created a standardized clinical assessment and management plan (SCAMP) for patients having balloon aortic valvuloplasty (BAV) for congenital aortic stenosis (AS). This study compares acute outcomes of BAV at our institution before and after introduction of this SCAMP. In this retrospective matched cohort study, each SCAMP patient was matched to four historical controls. Outcomes were categorized based on the combination of residual AS and aortic regurgitation (AR) as: (1) Optimal: gradient ≤35 mm Hg and trivial or no AR; (2) Adequate: gradient ≤35 mm Hg and mild AR; (3) Inadequate: gradient >35 mm Hg and/or moderate or severe AR. All 23 SCAMP patients achieved a residual AS gradient ≤35 mm Hg; the median residual AS gradient for the SCAMP group was lower (25 [10-35] mm Hg) than in matched controls (30 [0-65] mm Hg; P = 0.005). The two groups did not differ with regard to degree of AR grade after BAV. Compared with controls, SCAMP patients were more likely to have an optimal result and less likely to have an inadequate result (52% vs. 34% and 17% vs. 45%, respectively; P = 0.02) CONCLUSIONS: A SCAMP for BAV resulted in optimal acute results in half of the initial 23 patients enrolled, and outcomes in this group were better than those of matched historical controls. Whether these improved acute outcomes translate into better long-term outcomes for this patient population remains to be seen.
    Congenital Heart Disease 10/2013; · 1.01 Impact Factor
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    ABSTRACT: THE MELODY TRANSCATHETER PULMONARY VALVE (TPV) WAS APPROVED FOR IMPLANTATION IN OBSTRUCTED RIGHT VENTRICULAR OUTFLOW TRACT CONDUITS IN 2010 AFTER A MULTICENTER TRIAL DEMONSTRATING IMPROVEMENTS IN CONDUIT OBSTRUCTION, REGURGITATION, AND RIGHT VENTRICULAR PRESSURE. A RECOGNIZED RISK AND CONTRAINDICATION TO TPV IMPLANTATION IS THE DEMONSTRATION OF CORONARY ARTERY (CA) COMPRESSION DURING BALLOON ANGIOPLASTY OR STENT PLACEMENT IN THE OVERLYING CONDUIT. THIS STUDY IS THE FIRST TO CHARACTERIZE THE RISK OF CA COMPRESSION IN THIS POPULATION.METHODS AND RESULTS: FROM 2007 TO 2012, 404 PATIENTS UNDERWENT 407 CATHETERIZATIONS FOR POTENTIAL TPV IMPLANTATION (MEDIAN AGE, 18 YEARS) AT 4 CENTERS. THREE HUNDRED FORTY-THREE PATIENTS (85%) UNDERWENT VALVE IMPLANTATION. TWENTY-ONE PATIENTS (5%) HAD EVIDENCE OF CA COMPRESSION WITH SIMULTANEOUS RIGHT VENTRICULAR OUTFLOW TRACT ANGIOPLASTY AND CA ANGIOGRAPHY. SIXTY-EIGHT PATIENTS (17%) HAD ABNORMAL CA ANATOMY. FIFTEEN OF 21 (71%) PATIENTS WITH CA COMPRESSION HAD ABNORMAL CA ANATOMY. EIGHT PATIENTS WITH TETRALOGY OF FALLOT AND 7 PATIENTS WITH TRANSPOSITION OF THE GREAT ARTERIES DEMONSTRATED COMPRESSION. OF THE 34 PATIENTS WITH TETRALOGY OF FALLOT AND ABNORMAL CA, 7 (21%) DEMONSTRATED CA COMPRESSION.CONCLUSIONS: CA compression following TPV implantation can be catastrophic. CA compression was observed in 5% of patients during test balloon angioplasty. No patients in this study developed clinically apparent CA compression after TPV implantation. CA compression was significantly associated with the presence of abnormal CA anatomy, especially in patients with tetralogy of Fallot or transposition of the great arteries. Preimplantation coronary angiography with simultaneous test angioplasty is an important step to evaluate for the presence of CA compression during TPV implantation.
    Circulation Cardiovascular Interventions 09/2013; · 6.54 Impact Factor
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    ABSTRACT: BACKGROUND: ANGIOPLASTY AND STENT IMPLANTATION HAVE BECOME ACCEPTED THERAPIES FOR ISOLATED PERIPHERAL PULMONARY STENOSIS, AND HAVE BEEN SHOWN TO INCREASE VESSEL DIAMETER AND REDUCE RIGHT VENTRICULAR (RV) PRESSURE ACUTELY IN PATIENTS WITH PULMONARY ARTERY (PA) STENOSIS. THE PURPOSE OF THIS STUDY WAS TO ASSESS LONG-TERM OUTCOMES AFTER PRIMARY TRANSCATHETER THERAPY FOR PERIPHERAL PULMONARY STENOSIS.METHODS AND RESULTS: WE STUDIED 69 PATIENTS WHO UNDERWENT PRIMARY TRANSCATHETER INTERVENTION FOR SEVERE ISOLATED PERIPHERAL PULMONARY STENOSIS AT 5 YEARS OF AGE. GENETIC/SYNDROMIC DIAGNOSES INCLUDED WILLIAMS SYNDROME (N=23), NON-WILLIAMS FAMILIAL ARTERIOPATHY (N=12), AND ALAGILLE SYNDROME (N=3). AT THE INITIAL PA INTERVENTION, MEDIAN RV:AORTIC PRESSURE RATIO DECREASED FROM 1.00 TO 0.88 (MEDIAN DECREASE, 0.18; P0.001). PATIENTS WITH A HIGHER PREINTERVENTION RV:AORTIC PRESSURE RATIO HAD A GREATER REDUCTION (P0.001). DURING FOLLOW-UP (MEDIAN, 8.5 YEARS), 10 PATIENTS DIED, 5 FROM COMPLICATIONS OF PA CATHETERIZATION (ALL BEFORE 1998). THIRTEEN PATIENTS UNDERWENT SURGICAL PA INTERVENTION, MOST WITHIN 1 YEAR AND ALONG WITH REPAIR OF SUPRAVALVAR AORTIC STENOSIS. FREEDOM FROM ANY PA REINTERVENTION WAS 386% AT 1 YEAR AND 226% AT 5 YEARS. THE MEDIAN RV:AORTIC PRESSURE RATIO DECREASED FROM 1.0 AT BASELINE TO 0.53 AT THE MOST RECENT CATHETERIZATION (P0.001), AND 82% OF PATIENTS WITH AVAILABLE CLINICAL FOLLOW-UP WERE ASYMPTOMATIC.CONCLUSIONS: Transcatheter therapy for infants with severe peripheral pulmonary stenosis has become safer, regardless of genetic condition. Coupled with reintervention and surgical relief in selected cases, RV:aortic pressure ratios decrease substantially and most patients are asymptomatic at late follow-up.
    Circulation Cardiovascular Interventions 08/2013; · 6.54 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: BACKGROUND: Midaortic syndrome is often associated with refractory hypertension. The aim of our study was to better understand the short- and medium-term outcomes in this patient population utilizing a multidisciplinary management approach. METHODS: We conducted a review of patients with midaortic syndrome treated at our institution over the past 30 years. RESULTS: Fifty-three patients presented at a median age of 6.7 years (birth to 28.7 years). Thirty-five patients (66 %) underwent invasive management (percutaneous techniques: 21; surgical techniques: 5; both: 9). Percutaneous interventions were acutely successful in decreasing the gradient across the obstruction and degree of luminal stenosis. However, freedom from reintervention was 58 % at 1 year and 33 % at 5 years. Freedom from reintervention after a surgical procedure was longer: 83 % at 1 year and 72 % at 10 years. At the most recent follow-up, the majority of patients (69 %) were normotensive. The median duration between time of presentation and achievement of blood pressure control was 5.7 (0.4-21.1) years. The median number of anti-hypertensive medications was 1 (0-5). CONCLUSIONS: A multidisciplinary management strategy which couples comprehensive medical management with catheter-based and surgical interventions can lead to adequate blood pressure control and preservation of end-organ function in patients with midaortic syndrome.
    Pediatric Nephrology 06/2013; · 2.94 Impact Factor
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    ABSTRACT: BACKGROUND: PERCUTANEOUS PULMONARY VALVE IMPLANTATION USING A STENT-BASED BIOPROSTHETIC VALVE PROVIDES AN ALTERNATIVE TO SURGERY IN SELECT PATIENTS. SYSTEMIC INFECTIONS IN MELODY VALVEIMPLANTED PATIENTS WITH AND WITHOUT IDENTIFIED VALVE INVOLVEMENT HAVE BEEN REPORTED, YET THE INCIDENCE IS UNKNOWN, AND RISK FACTORS REMAIN UNIDENTIFIED.METHODS AND RESULTS: BETWEEN 2007 AND 2012, A TOTAL OF 147 CONSECUTIVE PATIENTS WITH CONGENITAL HEART DISEASE UNDERWENT MELODY PERCUTANEOUS PULMONARY VALVE IMPLANTATION AT OUR INSTITUTION. DEMOGRAPHIC AND CLINICAL VARIABLES WERE COLLECTED AT BASELINE AND AT FOLLOW-UP AND ANALYZED AS PREDICTORS. THE OCCURRENCE OF BLOODSTREAM INFECTION (BSI), DEFINED AS A BACTERIAL INFECTION TREATED WITH 4 WEEKS OF ANTIBIOTICS, SERVED AS OUR PRIMARY OUTCOME. THE MEAN AGE AT IMPLANTATION FOR THE STUDY POPULATION WAS 21.511 YEARS, AND TETRALOGY OF FALLOT WAS THE CARDIAC CONDITION IN 59%. DURING A MEDIAN FOLLOW-UP OF 19 MONTHS, 14 PATIENTS EXPERIENCED BSI (9.5%; 95% CONFIDENCE INTERVAL, 5.3%15%). OF THESE, 4 (2.7%) PATIENTS HAD MELODY VALVE ENDOCARDITIS. TWO PATIENTS DIED DURING THE EVENT, NEITHER OF WHOM HAD KNOWN VALVE INVOLVEMENT. THE MEDIAN PROCEDURE TO INFECTION TIME WAS 15 MONTHS (RANGE, 156). IN UNIVARIATE ANALYSIS, MALE SEX, PREVIOUS ENDOCARDITIS, IN SITU STENTS IN THE RIGHT VENTRICULAR OUTFLOW TRACT, AND PRESENCE OF OUTFLOW TRACT IRREGULARITIES AT THE IMPLANT SITE WERE ASSOCIATED WITH BSI OCCURRENCE.CONCLUSIONS: In this cohort, 9.5% of patients who underwent Melody percutaneous pulmonary valve implantation experienced subsequent BSI, occurring 1 to 56 months after implant, and 2.7% of patients had prosthetic endocarditis. Our findings suggest that patient and nonvalve anatomic factors may be associated with BSI after percutaneous pulmonary valve implantation.
    Circulation Cardiovascular Interventions 06/2013; · 6.54 Impact Factor
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    ABSTRACT: Variability in medical practice in the United States leads to higher costs without achieving better patient outcomes. Clinical practice guidelines, which are intended to reduce variation and improve care, have several drawbacks that limit the extent of buy-in by clinicians. In contrast, standardized clinical assessment and management plans (SCAMPs) offer a clinician-designed approach to promoting care standardization that accommodates patients' individual differences, respects providers' clinical acumen, and keeps pace with the rapid growth of medical knowledge. Since early 2009 more than 12,000 patients have been enrolled in forty-nine SCAMPs in nine states and Washington, D.C. In one example, a SCAMP was credited with increasing clinicians' rate of compliance with a recommended specialist referral for children from 19.6 percent to 75 percent. In another example, SCAMPs were associated with an 11-51 percent decrease in total medical expenses for six conditions when compared with a historical cohort. Innovative tools such as SCAMPs should be carefully examined by policy makers searching for methods to promote the delivery of high-quality, cost-effective care.
    Health Affairs 05/2013; 32(5):911-20. · 4.64 Impact Factor
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    ABSTRACT: Risk factors for developing systemic-to-pulmonary artery collaterals (SPCs) in hypoplastic left heart syndrome (HLHS) are unknown. We performed a retrospective case-control study to identify risk factors for developing a profuse SPC burden in HLHS. Angiograms of 439 patients with HLHS (performed <2 years of age) were reviewed using a previously published angiographic grading scale to identify cases (profuse SPC burden, n = 20) and controls (no or minimal SPC burden, n = 35). In univariate analyses, profuse SPC burden was associated with mitral atresia and aortic atresia subtype (MA/AA) (65% vs 14%, p <0.0001), use of a Sano shunt (70% vs 37%, p = 0.03), longer log-transformed durations of intensive care unit stay (p = 0.02), hospital stay (p = 0.002), pleural drainage (p = 0.008) after stage 1 palliation, lower oxygen saturation at discharge after stage 1 palliation (82 ± 4 vs 85 ± 4%, p = 0.03), and a history of severe shunt obstruction (37% vs 11%, p = 0.04). In a multivariate logistic regression model, profuse SPC burden was associated with MA/AA subtype (odds ratio 6.6), Sano shunt type (odds ratio 8.6), and log-transformed duration of hospital stay after stage 1 (odds ratio 7.9, model p <0.0001, area under the curve 0.88). Nonassociated parameters included fetal aortic valve dilation, severe cyanotic episodes, number of days with open sternum or number of additional exploratory thoracotomies after stage 1 palliation, pulmonary vein stenosis, and restrictive atrial septal defect. In conclusion, in the present case-control study of patients with HLHS, the development of a profuse SPC burden was associated with MA/AA subtype, Sano shunt type, and longer duration of hospital stay after stage 1 palliation.
    The American journal of cardiology 04/2013; · 3.58 Impact Factor
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    ABSTRACT: BACKGROUND: Mitral valve replacement remains the last resort for treatment of congenital mitral valve disease. Enlarging the mitral annulus at the time of mitral valve replacement may allow implantation of a larger prosthesis in children. METHODS: All mitral valve replacement procedures from 1990 to 2012 were included, and mitral annulus enlargement techniques were analyzed. A control group of patients undergoing replacement of a previously implanted mechanical mitral valve prosthesis was compared with the cohort of patients who underwent annular enlarging procedures. Primary end points were increases in annulus size and Z score, and freedom from paced rhythm. RESULTS: Among 205 mitral valve replacement procedures, 16 (8%) included techniques to upsize the mitral annulus in 15 patients, all but 1 of whom had undergone prior mitral valve replacement. These patients were compared with a control cohort of 53 patients undergoing mitral valve re-replacement without annulus enlargement. The annulus was upsized by 18% ± 11% (compared with 16% ± 20% in controls, P = .5) using open balloon dilation in 4 patients, radial annular incisions in 5 patients, and patch augmentation of the aorto-mitral continuity in 7 patients. The mean valvar diameter Z score increased from -0.2 ± 1.3 to +1.1 ± 1.4 (P < .001), compared with an increase from -0.3 ± 1.2 to 0.9 ± 1.1 in controls (P < .001). During a mean follow-up of 6.5 ± 6.4 years, 4 of 12 patients required permanent pacing in the upsizing group (predominantly with patch augmentation), versus 5 of 53 patients in the control group. CONCLUSIONS: In children undergoing mitral valve replacement, various techniques can be used to upsize the mitral annulus and allow implantation of a larger prosthesis. There is a nontrivial risk of heart block with annulus upsizing, which deserves further study.
    The Journal of thoracic and cardiovascular surgery 03/2013; · 3.41 Impact Factor
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    ABSTRACT: Midaortic syndrome (MAS) is a rare condition characterized by stenosis of the aorta and often involving renal and visceral arteries. Current therapies include medical management of associated hypertension, and interventional procedures such as angioplasty or surgical bypass. We report a 2-year-old female with severe MAS who was initially treated with angioplasty and stents in both her aorta and superior mesenteric artery (SMA). Due to the presence of long segment stenoses, her renal arteries were not amenable to surgical reconstruction and she rapidly progressed to Stage V chronic kidney disease. The patient underwent bilateral nephrectomy and renal transplantation using a donor thoracoabdominal aorta allograft to provide inflow for the kidney as well as to bypass the nearly occluded aorta. The donor SMA was used to bypass the native SMA stenosis. Postoperatively, the patient had normalization of four limb blood pressures. She weaned from five anti-hypertensive agents to monotherapy with excellent renal function. This is the first reported case of thoracoabdominal aortic bypass using allograft aorta to address MAS. This approach allowed for successful kidney transplantation with revascularization of the mesenteric, and distal aortic circulation using allograft conduit that will grow with the child, obviating the need for repeated interventional or surgical procedures.
    American Journal of Transplantation 02/2013; · 6.19 Impact Factor
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    ABSTRACT: BACKGROUND: Patients with severe pulmonary arterial hypertension (PAH) face significant morbidity and death as a consequence of progressive right heart failure. Surgical shunt placement between the left PA and descending aorta (Potts shunt) appears promising for PAH palliation in children; however, surgical mortality is likely to be unacceptably high in adults with PAH. METHODS: We describe a technique for transcatheter Potts shunt (TPS) creation by fluoroscopically guided retrograde needle perforation of the descending aorta at the site of apposition to the left PA to create a tract for deployment of a covered stent between these vessels. This covered stent-anchored by the vessel walls and surrounding tissue-serves as the shunt. RESULTS: TPS creation was considered in 7 patients and performed in 4. The procedure was technically successful in 3 patients; 1 patient died during the procedure as a result of uncontrolled hemothorax. One acute survivor, critically ill at the time of TPS creation, later died of comorbidities. The 2 mid-term survivors (follow-up of 10 and 4 months) are well at home, with symptomatic improvement and no late complications. The 3 candidate patients in whom the procedure was not performed died within 1 month of consideration, underscoring the tenuous nature of this population. CONCLUSIONS: TPS creation is feasible and may offer symptomatic relief to select patients with refractory PAH. Further study of this innovative approach is warranted.
    The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 02/2013; · 3.54 Impact Factor
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    ABSTRACT: BACKGROUND: VENTRICULAR SEPTAL RUPTURE (VSR) AFTER ACUTE MYOCARDIAL INFARCTION (AMI) IS A POTENTIALLY LETHAL MECHANICAL COMPLICATION OF ACUTE CORONARY SYNDROMES. GIVEN HIGH SURGICAL MORTALITY, TRANSCATHETER CLOSURE HAS EMERGED AS A POTENTIAL STRATEGY IN SELECTED CASES. WE REPORT OUR SINGLE-CENTER EXPERIENCE WITH DOUBLE-UMBRELLA DEVICE PERCUTANEOUS CLOSURE OF POST-AMI VSR.METHODS AND RESULTS: IN THIS SINGLE-CENTER, RETROSPECTIVE, COHORT STUDY, PATIENTS WHO UNDERWENT TRANSCATHETER CLOSURE OF POST-AMI VSR BETWEEN 1988 AND 2008 AT BOSTON CHILDRENS HOSPITAL WERE INCLUDED. DATA WERE ANALYSED ACCORDING TO WHETHER THE PATIENTS UNDERWENT DIRECT PERCUTANEOUS VSR CLOSURE OR CLOSURE OF A RESIDUAL VSR AFTER A PREVIOUS SURGICAL APPROACH. PRIMARY OUTCOME WAS MORTALITY RATE AT 30 DAYS. CLINICAL PREDICTORS OF PRIMARY OUTCOME WERE INVESTIGATED USING UNIVARIATE LOGISTIC REGRESSION. THIRTY PATIENTS WERE INCLUDED IN THE STUDY (MEAN AGE, 678 YEARS). A TOTAL OF 40 CLOSURE DEVICES WERE IMPLANTED. MAJOR PERIPROCEDURAL COMPLICATIONS OCCURRED IN 4 (13%) PATIENTS. CARDIOGENIC SHOCK, INCREASING PULMONARY/SYSTEMIC FLOW RATIO, AND THE USE OF THE NEW GENERATION (6-ARM) STARFLEX DEVICE ALL WERE ASSOCIATED WITH HIGHER RISK OF MORTALITY. THE MODEL FOR END-STAGE LIVER DISEASE EXCLUDING INTERNATIONAL NORMALIZED RATIO (MELD-XI) SCORE AT THE TIME OF VSR CLOSURE SEEMED TO BE MOST STRONGLY ASSOCIATED WITH DEATH (ODDS RATIO, 1.6; CONFIDENCE INTERVAL, 1.12.2; P0.001).CONCLUSIONS: Transcatheter closure of post-AMI VSR using CardioSEAL or STARFlex devices is feasible and effective. The MELD-XI score, a marker of multiorgan dysfunction, is a promising risk stratifier in this population of patients. Early closure of post-AMI VSR is advisable before establishment of multiorgan failure.
    Circulation Cardiovascular Interventions 01/2013; · 6.54 Impact Factor
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    ABSTRACT: : Obstruction of the superior vena cava is one of the potential complications of neonatal extracorporeal membrane oxygenation. Chylothorax is a known complication of surgery involving the thoracic cavity in children, and of extracorporeal membrane oxygenation. The aim of this study was to evaluate the association between chylothorax and superior vena cava obstruction after neonatal extracorporeal membrane oxygenation. : Twenty-two patients diagnosed with superior vena cava obstruction at ≤6 months of age (median 1.8 months) after neonatal extracorporeal membrane oxygenation were compared with a randomly selected cohort of 44 neonatal extracorporeal membrane oxygenation patients without superior vena cava obstruction. Among patients with superior vena cava obstruction, 18 underwent extracorporeal membrane oxygenation for respiratory disease and four for cardiac insufficiency. Chylothorax was more prevalent among patients with superior vena cava obstruction than controls (odds ratio 9.4 [2.2-40], p = .01) and was associated with extension of obstruction into the left innominate vein. Patients with superior vena cava obstruction were supported by extracorporeal membrane oxygenation for a longer duration than controls. Nineteen patients with superior vena cava obstruction (86%) underwent transcatheter balloon angioplasty and/or stent implantation (median 7 days after diagnosis), which decreased the superior vena cava pressure and superior vena cava-to-right atrium pressure gradient and increased the superior vena cava diameter (all p < 0.001). There were no serious procedural adverse events. Six study patients died within 30 days of the diagnosis of superior vena cava obstruction (including three of nine with chylothorax), which did not differ from controls. During a median follow-up of 2.7 yrs, two additional patients died and nine underwent 14 superior vena cava reinterventions. : Among neonates treated with extracorporeal membrane oxygenation, superior vena cava obstruction is associated with an increased risk of chylothorax. In neonates with chylothorax after extracorporeal membrane oxygenation, evaluation for superior vena cava obstruction may be warranted. Although mortality is high in this population, transcatheter treatment can relieve superior vena cava obstruction and facilitate symptomatic improvement.
    Pediatric Critical Care Medicine 01/2013; 14(1):37-43. · 2.35 Impact Factor
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    ABSTRACT: Young patients with congenital aortic valve disease are at risk of left ventricular (LV) diastolic dysfunction (DD). We evaluated LV remodeling and the prevalence of, and risk factors for, DD in patients with aortic stenosis (AS), pure aortic regurgitation (AR), and AS+AR. Patients aged 8 to 39 years with congenital AS (n = 103), AR (n = 36), or AS+AR (n = 107) were identified. Cross-sectional assessment of the LV remodeling pattern and diastolic function was performed. A diastolic function score (DFS; range 0 to 4) was assigned to each patient, with 1 point for an abnormal value in each of 4 categories: mitral inflow (E/A and E-wave deceleration time), tissue Doppler E', E/E', and left atrial volume. Patients with a DFS of ≥2 were compared to those with a DFS <2. Concentric hypertrophy was the most common remodeling pattern in those with AS (51%), mixed/physiologic hypertrophy in those with AS+AR (48%) and eccentric hypertrophy in those with AR (49%) predominated. In the entire cohort, 91 patients (37%) had a DFS of ≥2. Patients with AS or AS+AR had greater DFS than those with pure AR (p <0.001). On multivariate analysis, a greater LV mass z-score and previous aortic valve balloon dilation were associated with a DFS of ≥2. In patients with catheterization data (n = 65), E/E' correlated with LV end-diastolic pressure. Those with a DFS of ≥2 had a greater LV end-diastolic pressure and mean pulmonary artery pressure than those with a DFS <2. In conclusion, DD is common in young patients with AS and AS+AR but not in those with pure AR. A greater LV mass and previous aortic valve dilation were associated with DD.
    The American journal of cardiology 10/2012; · 3.58 Impact Factor
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    ABSTRACT: OBJECTIVES.: Guidewires used in pediatric catheterization are typically floppy, soft, or J-tipped, and are generally assumed to be atraumatic. A recent sentinel case suggested that such wires may cause clinically significant pulmonary artery (PA) injury. We sought to determine the incidence of wire-related PA injury as a cause of "idiopathic" airway bleeding (endobronchial blood) during interventional cardiac catheterization in patients with congenital heart disease. DESIGN.: The Children's Hospital Boston database of cardiac catheterizations was reviewed for adverse events (AEs) indicating possible PA injury occurring between September 2006 and August 2011. Procedure notes were reviewed, and when the clinical scenario was suggestive of wire injury or was not clear, relevant angiograms were reviewed. RESULTS.: One thousand forty-seven cases involving PA dilation were performed in the period of interest. Five cases of probable wire injury were identified, suggesting an incidence of approximately 0.5 per 100 cases. Of these five cases, trauma was judged due to floppy-tipped wires in two, soft-tipped wires in two, and a J-tipped wire in one. In three cases, the distal wire was looped such that the leading segment was stiffer than the wire tip. Clinical manifestations of wire injury comprised contrast within the airway, vessel aneurysm/tear, obstructive intimal flap, blood from the endotracheal tube, hemothorax, and wedge defect on lung scan. These injuries were relatively benign and did not result in instability or prolonged bleeding. CONCLUSIONS.: Wire injury to the PAs is relatively uncommon, although possible with even floppy-tipped wires. The mechanism and implications of such injuries are markedly different than balloon-mediated vascular tears.
    Congenital Heart Disease 10/2012; · 1.01 Impact Factor
  • [show abstract] [hide abstract]
    ABSTRACT: We report the use of a Melody valve as a surgical implant in the mitral position in 2 infants, one with severe mitral regurgitation and another with mitral stenosis, where other prostheses are not small enough to be implanted in the mitral position nor expandable as the child grows.
    The Annals of Thoracic Surgery. 10/2012; 94(4):e97–e98.

Publication Stats

9k Citations
2,311.11 Total Impact Points

Institutions

  • 1985–2014
    • Boston Children's Hospital
      • • Department of Cardiac Surgery
      • • Department of Radiology
      • • Children's Hospital Primary Care Center
      Boston, Massachusetts, United States
    • University of Minnesota Twin Cities
      • Department of Laboratory Medicine and Pathology
      Minneapolis, MN, United States
  • 2012
    • Virginia Commonwealth University
      Richmond, Virginia, United States
  • 2011
    • Riley Hospital for Children
      • Department of Cardiology
      Indianapolis, Indiana, United States
  • 2007–2011
    • Harvard University
      Cambridge, Massachusetts, United States
    • University of Virginia
      • Department of Pediatrics
      Charlottesville, VA, United States
  • 1985–2010
    • Harvard Medical School
      • Department of Pediatrics
      Boston, Massachusetts, United States
  • 2009
    • Miami Children's Hospital
      • Department of Cardiology
      Miami, FL, United States
  • 1988–2008
    • Brigham and Women's Hospital
      • Department of Medicine
      Boston, MA, United States
    • American Heart Association
      Dallas, Texas, United States
  • 2006
    • Partners HealthCare
      Boston, Massachusetts, United States
  • 2003
    • Case Western Reserve University
      • Rainbow Babies and Children's Hospital
      Cleveland, OH, United States
  • 1995
    • State University of New York Downstate Medical Center
      • Division of Pediatric Radiology
      Brooklyn, NY, United States
  • 1990
    • Medical University of South Carolina
      Charleston, South Carolina, United States
  • 1986
    • University of Massachusetts Boston
      Boston, Massachusetts, United States
  • 1984
    • University of Minnesota Medical Center, Fairview
      Minneapolis, Minnesota, United States
  • 1978–1983
    • University of Minnesota Duluth
      Duluth, Minnesota, United States