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The Journal of thoracic and cardiovascular surgery 10/2012; 144(4):880-1. · 3.41 Impact Factor
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ABSTRACT: We have previously shown that surgical Technical Performance Scores (TPS) are important predictors of early postoperative morbidity across a wide spectrum of procedures and that intraoperative recognition and intervention of residual defects resulted in improved outcomes. We hypothesized that these scores would also be important predictors of midterm outcomes.
Neonates and infants aged younger 6 months were prospectively followed from the index surgery for a minimum of 1 year. The TPS were calculated using previously published criteria, including intraoperative course, predischarge echocardiograms or catheterizations, and clinical data, and graded as optimal, adequate, or inadequate. Case complexity was determined by the Risk Adjustment for Congenital Heart Surgery-1 category. The primary outcome was mortality, and the secondary outcome was the need for unplanned reinterventions. Outcomes were analyzed using nonparametric methods and a logistic regression model.
A total of 166 patients were included in our study, with 7 early deaths. The remaining 159 patients (Risk Adjustment for Congenital Heart Surgery-1 category 4-6, 76 [48%]; neonates, 78 [49%]) were followed for a minimum of 1 year after surgery. There were 14 late deaths or late transplantations and 55 late reinterventions. On univariate analysis, the TPS were associated with mortality (P < .001) and reintervention (P = .04). On logistic regression analysis, inadequate TPS was associated with late mortality (P < .001; odds ratio, 7.2; 95% confidence interval, 2.2-23.6), and Risk Adjustment for Congenital Heart Surgery-1 category (P = .004; odds ratio, 3.7; 1.5-8.8) at index surgery was associated with need for late unplanned reintervention.
Technical performance affects midterm survival after infant heart surgery. Inadequate TPS can be used to prospectively identify patients at ongoing risk of demise and the need for reintervention. An aggressive approach to diagnosing and treating residual lesions at the initial operation is warranted.
The Journal of thoracic and cardiovascular surgery 08/2012; 144(5):1095-1101.e7. · 3.41 Impact Factor
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ABSTRACT: Stenting of the systemic ventricular outflow tract and creation or enlargement of a ventricular septal defect using stents are potential therapeutic options in rare patients with congenital heart disease who develop significant ventricular hypertension due to outflow obstruction. These stents may be at increased risk of fracture because of exposure to cyclic compressive stresses. The objective of this study was to evaluate the safety and efficacy of this intervention and to determine the incidence of stent fracture and other adverse outcomes after placement of intraventricular or interventricular stents for this indication.
Between 1992 and 2010, 27 patients underwent bare-metal stent placement in the ventricular septum or subvalvar systemic outflow tract, using 1 of the following 3 delivery approaches: (1) percutaneous (n=18), (2) intraoperative (n=8), and (3) hybrid (n=1). The median ventricular-to-aortic systolic pressure ratio decreased from 1.7 (1.3-2.6) to 1.1 (0.9-1.8) (P<0.001). Survival free from surgical reintervention for outflow obstruction was 92% at 1 year and 66% at 3 years. Stent fracture was diagnosed in 11 patients (41%) a median of 2.6 years (0.03-7.8 years) after stent placement and was always associated with recurrent obstruction. Survival free from diagnosis of stent fracture was 84% at 1 year and 73% at 3 years. Other adverse events included complete heart block (n=2) and increased atrioventricular valve regurgitation requiring surgical intervention (n=2).
Transcatheter, intraoperative, or hybrid stenting of the ventricular septum or systemic outflow tract is feasible and effective in the short term. Stent fractures were common during follow-up and associated with recurrent obstruction.
Circulation Cardiovascular Interventions 07/2012; 5(4):570-81. · 6.06 Impact Factor
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ABSTRACT: Risk factors for mortality after neonatal cardiac surgery have been seldom studied. We sought to identify contemporary risk factors for mortality and the impact of surgical technical performance on surgical outcomes after neonatal cardiac surgery.
We conducted a matched case-control study comparing 56 neonates who died after cardiac surgery (2002-2008) with 56 survivors matched by surgical procedure and year of surgery. Surgical efficacy for repair or palliation was graded using a reliable simple surgical technical score. Patient and surgical characteristics were compared for the survivors and nonsurvivors using paired analyses.
There was no significant difference between patients who died and their matched controls in terms of age, Aristotle score, Risk Adjustment in Congenital Heart Surgery-1 category, and single versus biventricular repair. When compared with survivors, patients who died were more likely to be premature (41% vs 5%, P < .001), to weigh less than 2.5 kg (25% vs 9%, P = .05), and to have inadequate surgical repair or palliation (55% vs 9%, P < .001). Cardiopulmonary bypass time was longer for the patients who died (median, 159 vs 133 minutes, P = .002). Highest postoperative lactate (median, 9.0 vs 6.0, P < .001), use of extracorporeal membrane oxygenation (71% vs 13%, P < .001), and reoperation during the same admission (75% vs 2%, P < .001) were also associated with death. In multivariable analysis, inadequate surgical repair or palliation (odds ratio, 11, P = .02) and need for postoperative extracorporeal membrane oxygenation (odds ratio, 5.1, P = .009) were the only risk factors associated with hospital death.
Our study highlights the need for optimal technical performance to minimize neonatal deaths. This has important implications when sustaining or developing a pediatric cardiac program.
The Journal of thoracic and cardiovascular surgery 03/2012; 144(5):1119-24. · 3.41 Impact Factor
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Circulation 02/2012; 125(5):e322-4. · 14.74 Impact Factor
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ABSTRACT: The aim of this study was to determine whether there are identifiable factors associated with increased risk for aortic arch reintervention in patients who have undergone balloon aortoplasty (BD) for aortic arch obstruction (COA) after the Norwood procedure (NP).
BD has been shown to be an effective acute therapy for COA after the NP. However, recurrent obstruction requiring repeat intervention is not uncommon.
All patients who underwent BD as the initial intervention for COA after the NP from to January 1993 to May 2009 were retrospectively analyzed (n = 116).
The median age at initial BD was 4.5 months. The median follow-up period was 3.4 years. Procedures were considered acutely successful in 92% of patients, with a median gradient reduction overall from 24 to 3 mm Hg (p < 0.0001) and a COA diameter increase of 52% (p < 0.0001). By Kaplan-Meier analysis, freedom from reintervention was 69% at 1 year and 58% at 5 years, and freedom from reoperation was 82% at 1 year and 79% at 5 years. By Cox regression analysis, proximal arch obstruction, age <3 months at BD, moderate or greater ventricular dysfunction, moderate or greater atrioventricular valve regurgitation on pre-catheterization echocardiography, and higher post-BD coarctation gradient were associated with shorter freedom from reoperation.
Despite a high acute success rate, a significant proportion of patients treated with BD for post-NP COA underwent reintervention during follow-up. The risk for arch reintervention is highest in patients with proximal arch obstruction, those age <3 months at the time of BD, and those with less successful acute results.
Journal of the American College of Cardiology 08/2011; 58(8):868-76. · 14.16 Impact Factor
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ABSTRACT: Our objective was to define the relationship between surgical technical performance score, intraoperative adverse events, and major postoperative adverse events in complex pediatric cardiac repairs.
Infants younger than 6 months were prospectively followed up until discharge from the hospital. Technical performance scores were graded as optimal, adequate, or inadequate based on discharge echocardiograms and need for reintervention after initial surgery. Case complexity was determined by Risk Adjustment in Congenital Heart Surgery (RACHS-1) category, and preoperative illness severity was assessed by Pediatric Risk of Mortality (PRISM) III score. Intraoperative adverse events were prospectively monitored. Outcomes were analyzed using nonparametric methods and a logistic regression model.
A total of 166 patients (RACHS 4-6 [49%]), neonates [50%]) were observed. Sixty-one (37%) had at least 1 intraoperative adverse event, and 47 (28.3%) had at least 1 major postoperative adverse event. There was no correlation between intraoperative adverse events and RACHS, preoperative PRISM III, technical performance score, or postoperative adverse events on multivariate analysis. For the entire cohort, better technical performance score resulted in lower postoperative adverse events, lower postoperative PRISM, and lower length of stay and ventilation time (P < .001). Patients requiring intraoperative revisions fared as well as patients without, provided the technical score was at least adequate.
In neonatal and infant open heart repairs, technical performance score is one of the main predictors of postoperative morbidity. Outcomes are not affected by intraoperative adverse events, including surgical revisions, provided technical performance score is at least adequate.
The Journal of thoracic and cardiovascular surgery 08/2011; 142(5):1098-107, 1107.e1-5. · 3.41 Impact Factor
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ABSTRACT: Clinical indications and outcomes for children requiring multiple extracorporeal membrane oxygenation (ECMO) runs during a single hospital admission are rarely reported. We review indications, cannulation techniques, and outcomes for infants requiring multiple ECMO runs after congenital heart surgery.
Demographic and ECMO-related data for all children requiring multiple ECMO runs during a single hospital admission from 1995 to 2008 were extracted from our institution's ECMO database. Survival was defined as survival to hospital discharge. Demographic and ECMO data were compared between survivors and nonsurvivors using the Mann-Whitney test for continuous and Fisher's exact test for categorical data.
Twenty children (5% of 391 ECMO runs) with a median age of 5 days (range, 4 to 180 days) and weight of 3.22 kg required repeat ECMO support for persistent cardiopulmonary dysfunction despite successful weaning from an index ECMO run. Of the two-ventricle repair group, tetralogy of Fallot, truncus arteriosus, and transposition constituted the majority of cardiac diagnoses (75%), whereas single-ventricle physiology made up the rest (25%). The median total ECMO duration was 191 hours (range, 77 to 485 hours), and median hospital length of stay was 41 days (range, 6 to 297 days). Five (25%) patients survived to discharge. When survivors were compared with nonsurvivors, there were no significant differences in demographics, single or biventricular repair, indication for ECMO support, or need for surgical reoperation while on ECMO. However, the incidence of renal failure was higher in nonsurvivors than in survivors after multiple ECMO runs (12 versus 2.0; p=0.03).
Survival in children undergoing congenital heart surgery requiring multiple ECMO runs is low, but 1 in 4 patients will survive to discharge. If a correctable lesion or ventricular impairment is present, repeat ECMO support may be justified. Development of renal failure is associated with nonsurvival in these patients.
The Annals of thoracic surgery 06/2011; 91(6):1949-55. · 3.74 Impact Factor
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ABSTRACT: Staged left ventricular rehabilitation is a novel surgical approach in patients undergoing single ventricle palliation for borderline hypoplastic left cardiac disease, in an attempt to salvage the left ventricle. The procedure includes resection of endocardial fibroelastosis from the left ventricular free wall and apex. We hypothesised that endocardial fibroelastosis removal may significantly affect ventricular conduction and myocardial electrical characteristics.
This study included 27 patients with borderline hypoplastic left cardiac syndrome who underwent staged left ventricle rehabilitation with endocardial fibroelastosis resection following single ventricle palliation. The effect on electrical synchrony was measured by ventricular depolarisation timing (QRS duration) on electrocardiogram. Patients were evaluated for a change in QRS duration before and after fibroelastosis removal and at most recent follow-up.
The QRS change in the immediate period after endocardial fibroelastosis resection ranged from -16 to 36 milliseconds with a median of 0 (p = 0.09). However, long-term conduction delay was common in 44% (12/27) of patients having a QRS duration greater than 98th percentile for the age at the most recent electrocardiogram. Only one patient had QRS duration greater than 98th percentile before any surgical procedure. Two patients developed left bundle branch block and one developed right bundle branch block with left, but anterior-fascicular block. Overall, the QRS duration correlated with left ventricular size (R = 0.54, p = 0.006) at the most recent electrocardiogram.
Electrical dyssynchrony is a common finding in patients undergoing staged left ventricular rehabilitation after single ventricle palliation; however, it is not acutely related to surgical endocardial resection. Left ventricular size is correlated with QRS duration. Diligent follow-up is required to evaluate the effects of left ventricular growth and consideration of resynchronisation in this population.
Cardiology in the Young 10/2010; 20(5):516-21. · 0.76 Impact Factor
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ABSTRACT: Although pulmonary valve replacement (PVR) is effective in reducing right ventricular (RV) volume overload in patients with chronic pulmonary regurgitation, persistent RV dysfunction and subsequent adverse clinical outcomes have been reported. This trial was conducted to investigate whether the addition of surgical RV remodeling with exclusion of scar tissue to PVR would result in improved RV function and laboratory and clinical parameters, as compared with PVR alone.
Between February 2004 and October 2008, 64 patients who underwent RV outflow tract procedures in early childhood had more than or equal to moderate pulmonary regurgitation, and fulfilled defined criteria for PVR were randomly assigned to undergo either PVR alone (n=34) or PVR with surgical RV remodeling (n=30). No significant difference was observed in the primary outcome (change in RV ejection fraction, -2±7% in the PVR alone group and -1±7% in the PVR with RV remodeling group; P=0.38) or in any of the secondary outcomes at 6-month postoperative follow-up. Multivariable analysis of the entire cohort identified preoperative RV end-systolic volume index <90 mL/m(2) and QRS duration <140 ms to be associated with optimal postoperative outcome (normal RV size and function), and RV ejection fraction <45% and QRS duration ≥160 ms to be associated with suboptimal postoperative outcome (RV dilatation and dysfunction).
The addition of surgical remodeling of the RV to PVR in patients with chronic pulmonary regurgitation did not result in a measurable early benefit. Referral to PVR based on QRS duration, RV end-systolic volume, or RV ejection fraction may be beneficial. Clinical Trial Registration-URL: http://www.clinicaltrials.gov. Unique identifier: NCT00112320.
Circulation 09/2010; 122(11 Suppl):S201-8. · 14.74 Impact Factor
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Jonathan R Kaltman,
Dean B Andropoulos,
Paul A Checchia,
J William Gaynor,
Timothy M Hoffman,
Peter C Laussen,
Richard G Ohye,
Gail D Pearson,
Frank Pigula,
James Tweddell,
Gil Wernovsky, Pedro Del Nido
Circulation 06/2010; 121(25):2766-72. · 14.74 Impact Factor
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ABSTRACT: The hemodynamics after Fontan surgery are notable for hypertension and dilation of the right atrium (RA). The effect of this stress on atrial cytoarchitecture has not been systematically studied and might be relevant to arrhythmias and their treatment. Morphologic and histopathologic analyses were performed on tissue from the RA and left atrium (LA) from autopsy specimens of Fontan hearts (n = 47). The findings were compared to those from control samples from young patients with normal atrial hemodynamics (n = 15). Most Fontan specimens were from young patients who died after a relatively short duration of Fontan physiology. The tissues were analyzed for wall thickness, fibrosis content, and fibrosis pattern. The mean wall thickness for the RA (3.0 +/- 1.0 mm) and LA (2.3 +/- 0.6 mm) in the Fontan hearts was significantly greater than that in the control hearts (RA, 1.8 +/- 0.4 mm; LA, 1.8 +/- 0.5 mm; p <0.001 and p = 0.024, respectively). The predictors for thickening of the RA included (1) older age at Fontan surgery, (2) older age at death, and (3) longer duration of Fontan circulation. The Fontan hearts and control hearts exhibited nearly identical fibrosis patterns in the RA and LA. Neither wall thickness nor fibrosis varied with the underlying heart defect or style of Fontan connection. In conclusion, atrial remodeling after Fontan surgery for univentricular heart physiology involves increased wall thickness in both the RA and LA. Interstitial fibrosis was also observed in the Fontan atria; however, because a similar pattern was present in the control tissue, this likely represented normal fibroelastic atrial structure, rather than a specific response to Fontan hemodynamics. The degree of wall thickening observed in the Fontan atria was not so excessive as to preclude transmural lesions during catheter or surgical ablation of reentrant arrhythmias.
The American journal of cardiology 12/2009; 104(12):1737-42. · 3.58 Impact Factor
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Antonio L Perez,
Estanislao Bachrach,
Ben M W Illigens,
Susan J Jun,
Eric Bagden,
Leta Steffen,
Alan Flint,
Francis X McGowan, Pedro Del Nido,
Enca Montecino-Rodriguez,
James G Tidball,
Louis M Kunkel
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ABSTRACT: Cell-based therapy is a possible avenue for the treatment of Duchenne muscular dystrophy (DMD), an X-linked skeletal muscle-wasting disease. We have demonstrated that cultured myogenic progenitors derived from the adult skeletal muscle side population can engraft into dystrophic fibers of non-irradiated, non-chemically injured mouse models of DMD (mdx(5cv)) after intravenous and intraarterial transplantation, with engraftment rates approaching 10%. In an effort to elucidate the cell-surface markers that promote progenitor cell extravasation and engraftment after systemic transplantation, we found that expression of the chemokine receptor CXCR4, whose ligand SDF-1 is overexpressed in dystrophic muscle, enhances the extravasation of these cultured progenitor cells into skeletal muscle after intraarterial transplantation. At 1 day post-transplantation, mice that received CXCR4-positive enhanced green fluorescent protein (eGFP)-positive cultured cells derived from the skeletal muscle side population displayed significantly higher amounts of eGFP-positive mononuclear cells in quadriceps and tibialis anterior than mice that received CXCR4-negative eGFP-positive cells derived from the same cultured population. At 30 days posttransplantation, significantly higher engraftment rates of donor cells were observed in mice that received CXCR4-positive cells compared with mice transplanted with CXCR4-negative fractions. Our data suggest that CXCR4 expression by muscle progenitor cells increases their extravasation into skeletal muscle shortly after transplantation. Furthermore, this enhanced extravasation likely promotes higher donor cell engraftment rates over time.
Muscle & Nerve 10/2009; 40(4):562-72. · 2.37 Impact Factor
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ABSTRACT: Late complications of the Fontan operation represent a significant management challenge. Failing Fontan patients have two modes of presentation: impaired ventricular function (IVF) and those with preserved ventricular function (PVF) but with failing Fontan physiology (protein-losing enteropathy [PLE] and plastic bronchitis [PB]). This study evaluated whether failing Fontan patients referred for heart transplantation had a different outcome based on the mode of presentation.
The medical records of all Fontan patients evaluated for heart transplantation at a single institution from 1994 to 2008 were retrospectively reviewed. Demographic, hemodynamic, and laboratory data were collected. Patients were stratified into an IVF or PVF group by echocardiographic criteria. Descriptive statistics and Kaplan-Meier analysis were used for hypothesis testing.
Thirty-four Fontan patients were evaluated for heart transplantation. According to echo description of systolic function, 18 were categorized as IVF and 16 as PVF. The IVF group had a significantly lower cardiac index and venous oxygen saturation, and significantly higher systemic vascular resistance vs the PVF group (p < 0.05). PLE or PB was present in 13 PVF patients and none in the IVF group. Twenty patients underwent transplantation, with similar rates amongst the IVF and PVF groups. Within 1 year from evaluation, 2 IVG patients and 7 PVF patients had died (p = 0.052).
Failing Fontan patients with PVF have decreased overall survival independent of whether they underwent transplantation. This trend indicates a need to improve the management and timing for transplantation amongst this population.
The Annals of thoracic surgery 08/2009; 88(2):558-63; discussion 563-4. · 3.74 Impact Factor
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ABSTRACT: Experimental autoimmune myocarditis (EAM) is mediated by myocardial infiltration by myosin-specific T cells secreting inflammatory cytokines.
To clarify the role of cytokines in EAM, we compared STAT 6-deficient ((-/-)) with STAT 4(-/-) and wild-type (BALB/CJ) mice following immunization with cardiac myosin peptide (614-629).
Wild-type mice developed severe disease with a small increase in severity in STAT 6(-/-) mice, while STAT 4(-/-) mice were resistant to EAM. STAT 6(-/-) mice had increased splenocyte proliferation and INF-gamma production versus wild type, while STAT 4(-/-) mice had decreased proliferation and INF-gamma. Following oral administration of myosin (614-629), tolerization was induced in wild-type mice evidenced by amelioration of myocarditis and up-regulation of IL-4. Adoptive transfer of splenocytes from orally tolerized mice resulted in inhibition of disease in STAT 6(-/-) mice.
These results demonstrate that oral tolerization ameliorates EAM in BALB/CJ mice and indicate a down-regulatory role for STAT 6 genes.
Journal of Clinical Immunology 05/2009; 29(4):434-43. · 3.08 Impact Factor
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Carole A Warnes,
Roberta G Williams,
Thomas M Bashore,
John S Child,
Heidi M Connolly,
Joseph A Dearani, Pedro Del Nido,
James W Fasules,
Thomas P Graham,
Ziyad M Hijazi,
Sharon A Hunt,
Mary Etta King,
Michael J Landzberg,
Pamela D Miner,
Martha J Radford,
Edward P Walsh,
Gary D Webb
Journal of the American College of Cardiology 01/2009; 52(23):1890-947. · 14.16 Impact Factor
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Carole A Warnes,
Roberta G Williams,
Thomas M Bashore,
John S Child,
Heidi M Connolly,
Joseph A Dearani, Pedro del Nido,
James W Fasules,
Thomas P Graham,
Ziyad M Hijazi, [......],
Steven M Ettinger,
Jonathan L Halperin,
Harlan M Krumholz,
Frederick G Kushner,
Bruce W Lytle,
Rick A Nishimura,
Richard L Page,
Barbara Riegel,
Lynn G Tarkington,
Clyde W Yancy
Journal of the American College of Cardiology 01/2009; 52(23):e143-263. · 14.16 Impact Factor
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Carole A Warnes,
Roberta G Williams,
Thomas M Bashore,
John S Child,
Heidi M Connolly,
Joseph A Dearani, Pedro del Nido,
James W Fasules,
Thomas P Graham,
Ziyad M Hijazi,
Sharon A Hunt,
Mary Etta King,
Michael J Landzberg,
Pamela D Miner,
Martha J Radford,
Edward P Walsh,
Gary D Webb
Circulation 12/2008; 118(23):e714-833. · 14.74 Impact Factor
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Carole A Warnes,
Roberta G Williams,
Thomas M Bashore,
John S Child,
Heidi M Connolly,
Joseph A Dearani, Pedro Del Nido,
James W Fasules,
Thomas P Graham,
Ziyad M Hijazi,
Sharon A Hunt,
Mary Etta King,
Michael J Landzberg,
Pamela D Miner,
Martha J Radford,
Edward P Walsh,
Gary D Webb
Circulation 12/2008; 118(23):2395-451. · 14.74 Impact Factor
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ABSTRACT: To characterize mortality and morbidity outcomes in patients undergoing surgical Fontan conversion.
Indications for and anticipated clinical outcomes associated with Fontan conversion are controversial.
A retrospective single-center cohort study of consecutive patients undergoing Fontan conversion between 1990 and 2006 stratified according to concomitant arrhythmia surgery.
Forty patients underwent Fontan conversion at a median age of 19.0 years and were followed for 4.2 years. Six (15%) died, two perioperatively, three early postoperatively, and one following heart transplant. Older age was a univariate risk factor. Major perioperative complications occurred in 9 of 35 (26%) early survivors. Patients with concomitant arrhythmia surgery (N=21) were older at conversion and had longer interoperative intervals. They experienced a reduction in prevalence (95% vs. 28%, P<0.0001) and severity (severity score 7.3 vs. 3.3, P=0.001) of atrial tachyarrhythmias, in contrast to patients without arrhythmia surgery (47% vs. 53%; 3.3 vs. 3.9, P=NS). NYHA functional class improved in both groups. In a subgroup (N=14) with non-urgent late postoperative catheterization, filling pressures were unchanged from preoperative values.
Mortality and morbidity after Fontan conversion are substantial, but survivors experience a subjective improvement in functional status. Concomitant arrhythmia surgery reduces the arrhythmia burden without a detectable increase in complication rates.
International journal of cardiology 08/2008; 137(3):260-6. · 7.08 Impact Factor
