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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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Jesse J Esch,
Pinak B Shah,
Barbara A Cockrill,
Harrison W Farber,
Michael J Landzberg,
Mandeep R Mehra,
Mary P Mullen,
Alexander R Opotowsky,
Aaron B Waxman, James E Lock,
Audrey C Marshall
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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; · 6.06 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. · 3.13 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; · 0.90 Impact Factor
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ABSTRACT: OBJECTIVES: The goal of this study was to review results of a novel management strategy intended to rehabilitate the left heart (LH) in patients with LH hypoplasia who have undergone single-ventricle palliation (SVP). BACKGROUND: Management of patients with hypoplastic LH syndrome and borderline left ventricle (LV) involves 2 options: SVP or biventricular repair. We hypothesized that staged LV recruitment and biventricular conversion may be achieved after SVP by using a strategy consisting of relief of inflow and outflow tract obstructions, resection of endocardial fibroelastosis, and promotion of flow through the LV. METHODS: Patients with hypoplastic LH and borderline LV who underwent traditional SVP (n = 34) or staged LV recruitment (n = 34) between 1995 and 2010 were retrospectively analyzed and compared with a control SVP group. RESULTS: Mean initial z-scores for LH structures before stage 1 SVP were not significantly different between groups. Mortality occurred in 4 of 34 patients after LV recruitment and in 7 of 34 after traditional SVP. LH dimension z-scores increased significantly over time after LV recruitment, whereas they declined after traditional SVP, with significant interaction between stage of palliation and treatment group. Restriction of the atrial septum (conducted in 19 of 34 patients) was the only predictor of increase in left ventricular end-diastolic volume (p < 0.001). Native biventricular circulation was achieved in 12 patients after staged LV recruitment; all of these patients had restriction at the atrial septum. CONCLUSIONS: In these patients with borderline LH disease who underwent SVP, it is possible to increase LH dimensions by using an LV recruitment strategy. In a subset of patients, this strategy allowed establishment of biventricular circulation.
Journal of the American College of Cardiology 09/2012; · 14.16 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: In congenital aortic stenosis, chronic pressure load has detrimental effects on left ventricular (LV) systolic and diastolic function. Reduction in LV pressure load with balloon aortic valvuloplasty (BAVP) may improve diastolic function.
Echocardiographic and catheterization data for 25 consecutive patients undergoing BAVP for congenital aortic stenosis were retrospectively analyzed. Median age at BAVP was 11.5 years (3.2-40.1). LV end-diastolic pressure was elevated (≥15 mm Hg) in 72% of patients, with a median of 17 mm Hg (range, 9-24). With BAVP, median aortic stenosis gradient was reduced from 63 mm Hg (range, 44-105) to 30 mm Hg (range, 10-43). Aortic regurgitation increased from trivial (none to mild) to mild (trivial to moderate). Pre-BAVP early diastolic mitral inflow velocity/tissue Doppler early diastolic velocity (E/E´) correlated with LV end-diastolic pressure (r=0.52, P=0.007). On follow-up echocardiography (median, 11 months after BAVP), aortic stenosis gradient was lower (P<0.001) and degree of AR was higher (P=0.01) compared with pre-BAVP echocardiograms. LV end-diastolic volume z-score increased (P=0.02), LV mass was unchanged, and LV mass:volume decreased (P=0.002). Mitral annular and septal E´ (P<0.001) were higher and E/E´ was lower after dilation (10.8 versus 14.2, P<0.001). Lower pre-BAVP E/E´ and lower pre-BAVP LV mass z-score were associated with lower post-BAVP E/E.
After BAVP, LV remodeling characterized by an increase in EDV and decrease in LV mass:volume occurs and echocardiographic measures of diastolic function and LV end-diastolic pressure improve in most patients. Risk factors for persistent diastolic dysfunction include higher pre-BAVP LV mass z-score and worse pre-BAVP diastolic function.
Circulation Cardiovascular Interventions 06/2012; 5(4):549-54. · 6.06 Impact Factor
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ABSTRACT: Objective. Mortality among children with congenital and acquired heart disease has decreased significantly over the past decades. We sought to determine whether the underlying problems leading to death in these patients had changed over the past decade. Methods. We reviewed medical records for 100 deaths of cardiac patients in 2004-2005 and 100 deaths in 1995-1996. Demographic, clinical, and procedural data as well as circumstances of death were collected. A consensus committee reviewed each case and sought to identify the condition leading to death. These conditions were classified as predominantly surgical or medical. Results. General patient characteristics (age, gender, cardiac history, comorbidities, proportion of surgical patients) did not change significantly between the two time periods. However, in 1995-1996, 64% of deceased surgical patients had died within 30 days of surgery. This rate was nearly halved to only 38% by 2004-2005 (P= .003). Furthermore, the conditions leading to death changed significantly: 51% of patient deaths in 1995-1996 resulted from a surgical problem, 29% from a medical condition. This ratio was reversed in 2004-2005: Only 31% of patient deaths were due to a surgical problem, while 50% of deaths resulted from a medical condition (P= .005). The most common medical conditions resulting in death were pulmonary vein stenosis, pulmonary arterial hypertension, and primary myocardial failure. Conclusions. The proportion of deaths within 30 days of cardiac surgery decreased significantly over the past decade. While surgical causes accounted for the majority of these deaths in 1995-1996, most patient deaths in 2004-2005 resulted from cardiac medical causes.
Congenital Heart Disease 05/2012; · 0.90 Impact Factor
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ABSTRACT: OBJECTIVE: Our aim was to assess acute hemodynamic changes with stent insertion, outcomes, and factors associated with increased longevity of stented small diameter homograft conduits. BACKGROUND: Right ventricle-to-pulmonary artery (RV-PA) homograft conduits are commonly used to palliate RV outflow tract obstruction. Bare metal stenting (BMS) and transcatheter pulmonary valve implantation have been shown to relieve obstructed larger diameter conduits and may delay surgical conduit reintervention. Less is known about BMS of small conduits. METHODS AND RESULTS: From 1992 to 2009, BMS was performed to relieve obstruction in 106 homograft conduits that were ≤12 mm at implant. The peak RV-PA gradient fell from 54.3 ± 17.4 mm Hg at baseline to 46.1 ± 15.2 mm Hg after balloon dilation alone and to 25.1 ± 11.4 mm Hg with stenting (all P < 0.001). Higher pre-BMS gradient and RV pressure were the only factors associated with higher post-BMS RV-PA gradient (≥30 mm Hg; both P < 0.001). There were no procedural deaths, two patients required surgical removal of embolized stents. At a median follow-up of 1.6 years, 83 conduits were replaced; freedom from conduit reoperation after BMS was 66% ± 5% at 1 year and 28% ± 5% at 3 years. Factors associated with shorter freedom from reoperation included implanted conduit diameter <10 mm (P = 0.009), higher post-stent RV-PA gradient (P = 0.026), and higher post-stent RV pressure (P < 0.01); only post-stent RV pressure remained significant on multivariable analysis (P < 0.001). CONCLUSION: BMS was acutely effective for the treatment of obstructed small diameter homograft conduits, with low morbidity. Prolongation of small diameter homograft conduit longevity with BMS may be useful in the lifetime management of conduit dysfunction in this patient population. © 2012 Wiley Periodicals, Inc.
Catheterization and Cardiovascular Interventions 03/2012; · 2.29 Impact Factor
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ABSTRACT: Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid.
We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 62±8% at 1 year and 50±8% at 5 years after pulmonary vein stent implantation. Stent placement acutely relieved focal obstruction in all veins. Of the 54 stents reexamined with catheterization, 32 underwent reintervention. Freedom from reintervention was 62±7% at 6 months and 42±7% at 1 year. Stent occlusion was documented in 9 cases and significant in-stent stenosis in 17 cases. Stent implantation diameter ≥7 mm was associated with longer freedom from reintervention (hazard ratio, 0.32; P=0.015) and from significant in-stent stenosis (hazard ratio, 0.14; P=0.002). Major acute complications occurred in 5 cases.
Transcatheter stent implantation can acutely relieve PVS in children, but reintervention is common. Larger stent lumen size at implantation is associated with longer stent patency and a lower risk of reintervention.
Circulation Cardiovascular Interventions 02/2012; 5(1):109-17. · 6.06 Impact Factor
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ABSTRACT: The Melody valve is approved for percutaneous pulmonary valve replacement in dysfunctional right ventricular outflow tracts. The function of this valve when subjected to high-pressure loads in humans is unknown. The aim of this study was to describe the immediate and short-term results of Melody valves implanted in a high-pressure environment.
Definitions of a high-pressure system were established for Melody valves implanted in the systemic (ie, aortic or mitral position) and pulmonary (ie, right ventricular outflow tract conduit or tricuspid valve annulus) circulations. Implants in these environments were ascertained from databases of the 5 centers that participated in the US Investigational Device Exemption trial. Thirty implants met the inclusion criteria: 23 pulmonary circulation implants (all in the pulmonary position) systemic circulation implants (5 in the native aortic position, 1 in a left ventricle-to-descending aorta conduit, and 1 in the mitral annulus). All pulmonary circulation implants were performed percutaneously in the catheterization laboratory. A hybrid approach (surgical exposure for transcatheter implant) was used for 4 of the aortic implants. There were no procedure-related deaths. Three patients died of nonprocedure- and nonvalve-related causes. At 1 year, freedom from moderate to severe regurgitation was 100%, and freedom from mild regurgitation was 90%. Freedom from moderate to severe stenosis was 86% at 1 year.
Short-term performance of the Melody valve in high-pressure environments is encouraging, with good valve function in all patients.
Circulation Cardiovascular Interventions 11/2011; 4(6):615-20. · 6.06 Impact Factor
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ABSTRACT: Among patients undergoing transcatheter pulmonary valve (TPV) replacement with the Melody valve, risk factors for Melody stent fracture (MSF) and right ventricular outflow tract (RVOT) reintervention have not been well defined.
From January 2007 to January 2010, 150 patients (median age, 19 years) underwent TPV implantation in the Melody valve Investigational Device Exemption trial. Existing conduit stents from a prior catheterization were present in 37 patients (25%, fractured in 12); 1 or more new prestents were placed at the TPV implant catheterization in 51 patients. During follow-up (median, 30 months), MSF was diagnosed in 39 patients. Freedom from a diagnosis of MSF was 77±4% at 14 months (after the 1-year evaluation window) and 60±9% at 39 months (3-year window). On multivariable analysis, implant within an existing stent, new prestent, or bioprosthetic valve (combined variable) was associated with longer freedom from MSF (P<0.001), whereas TPV compression (P=0.01) and apposition to the anterior chest wall (P=0.02) were associated with shorter freedom from MSF. Freedom from RVOT reintervention was 86±4% at 27 months. Among patients with a MSF, freedom from RVOT reintervention after MSF diagnosis was 49±10% at 2 years. Factors associated with reintervention were similar to those for MSF.
MSF was common after TPV implant in this multicenter experience and was more likely in patients with severely obstructed RVOT conduits and when the TPV was directly behind the anterior chest wall and/or clearly compressed. A TPV implant site protected by a prestent or bioprosthetic valve was associated with lower risk of MSF and reintervention.
Circulation Cardiovascular Interventions 11/2011; 4(6):602-14. · 6.06 Impact Factor
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ABSTRACT: We sought to determine the incidence of sudden cardiac death among patients discharged from the cardiology clinic with presumed noncardiac chest pain (CP).
The records of children >6 years of age who presented to Children's Hospital Boston between January 1, 2000, and December 31, 2009, with a complaint of CP were reviewed for demographic features, clinical characteristics, resource utilization, and presumed diagnosis. Patients were searched for in the US National Death Index and Social Security Death Index.
Data for a total of 3700 patients with CP (median age at evaluation: 13.4 years [range: 7-22.3 years]) were reviewed. The median follow-up period was 4.4 years (range: 0.5-10.4 years), for total of 17 886 patient-years of follow-up data. CP with exertion occurred in 1222 cases (33%), including 15 with associated syncope. A cardiac cause was determined in 37 cases; the remaining 3663 patients (99%) had CP of unknown (n = 1928), musculoskeletal (n = 1345), pulmonary (n = 242), gastrointestinal (n = 108), anxiety-related (n = 34), or drug-related (n = 4) origin. Emergency department visits for CP were documented for 670 patients (18%), and 263 patients (7%) had cardiology follow-up visits related to CP. There were 3 deaths, including 2 suicides and 1 spontaneous retroperitoneal hemorrhage.
CP in children is a common complaint and rarely has a cardiac cause. Review of 1 decade of cardiology visits (nearly 18 000 patient years) revealed that no patient discharged from the clinic died as a result of a cardiac condition.
PEDIATRICS 11/2011; 128(5):e1062-8. · 4.47 Impact Factor
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Lisa Bergersen,
Kimberlee Gauvreau,
Susan R Foerster,
Audrey C Marshall,
Doff B McElhinney,
Robert H Beekman,
Russel Hirsch,
Jacqueline Kreutzer,
David Balzer,
Julie Vincent,
William E Hellenbrand,
Ralf Holzer,
John P Cheatham,
John W Moore,
Grant Burch,
Laurie Armsby, James E Lock,
Kathy J Jenkins
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ABSTRACT: This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates.
The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007.
A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios.
Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41.
Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease.
09/2011; 4(9):1037-46. · 1.07 Impact Factor
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ABSTRACT: Chest pain is a common reason for referral to pediatric cardiologists and often leads to an extensive cardiac evaluation. The objective of this study is to describe current management practices in the assessment of pediatric chest pain and to determine whether a standardized care approach could reduce unnecessary testing.
We reviewed all patients, aged 7 to 21 years, presenting to our outpatient pediatric cardiology division in 2009 for evaluation of chest pain. Demographics, clinical characteristics, patient outcomes, and resource use were analyzed.
Testing included electrocardiography (ECG) in all 406 patients, echocardiography in 175 (43%), exercise stress testing in 114 (28%), event monitoring in 40 (10%), and Holter monitoring in 30 (7%). A total of 44 (11%) patients had a clinically significant medical or family history, an abnormal cardiac examination, and/or an abnormal ECG. Exertional chest pain was present in 150 (37%) patients. In the entire cohort, a cardiac etiology for chest pain was found in only 5 of 406 (1.2%) patients. Two patients had pericarditits, and 3 had arrhythmias. We developed an algorithm using pertinent history, physical examination, and ECG findings to suggest when additional testing is indicated. Applying the algorithm to this cohort could lead to an ∼20% reduction in echocardiogram and outpatient rhythm monitor use and elimination of exercise stress testing while still capturing all cardiac diagnoses.
Evaluation of pediatric chest pain is often extensive and rarely yields a cardiac etiology. Practice variation and unnecessary resource use remain concerns. Targeted testing can reduce resource use and lead to more cost-effective care.
PEDIATRICS 08/2011; 128(2):239-45. · 4.47 Impact Factor
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ABSTRACT: The late morbidity of pulmonary regurgitation has intensified the interest in valve-sparing repair of tetralogy of Fallot. This study reviewed a single institution's experience with valve-sparing repair and investigated the role of intraoperative balloon valvuloplasty.
A retrospective chart review identified 238 patients who underwent complete primary repair of tetralogy of Fallot at less than 180 days of age. Patients were divided into 4 groups on the basis of the type of right ventricular outflow tract repair: transannular patch (n = 111), commissurotomy or standard rigid dilation (n = 71), intraoperative balloon pulmonary valvuloplasty (n = 32), or no valvar intervention (n = 24).
Baseline demographic and anatomic factors differed among the 4 procedural groups with substantial overlap. Among 142 patients with pulmonary valve hypoplasia (z score, -2 to -4), 37% had valve-sparing repair. These patients had significant annular growth over time: z score increased 0.67 and 1.00 per year in the intraoperative balloon valvuloplasty (P < .001) and traditional valve-sparing (P < .001) groups, respectively. Rates of valve growth did not differ across groups, but z scores were 0.58 lower for the balloon valvuloplasty group across all time points (P = .001). Freedom from reintervention and surgery was shorter for the balloon valvuloplasty group than for the other groups (P < .001).
Patients with tetralogy of Fallot and pulmonary valve hypoplasia who undergo valve-sparing repair with intraoperative balloon valvuloplasty have significant longitudinal annular growth, with normalization of annular size over time. Despite application in patients with more hypoplastic valves, balloon valvuloplasty resulted in similar valve growth and pulmonary regurgitation as traditional methods, but higher rates of reintervention. Although the precise role of this technique needs further refinement, it is likely to be most useful in patients with moderate pulmonary stenosis and moderate pulmonary valve dysplasia.
The Journal of thoracic and cardiovascular surgery 06/2011; 142(6):1367-73. · 3.41 Impact Factor
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ABSTRACT: Pulmonary artery (PA) balloon angioplasty and/or stenting (PA rehabilitation) is one of the most common procedures performed in the cardiac catheterization laboratory, but comprehensive and consistently reported data on procedure-related adverse events (AE) are scarce.
Data were prospectively collected using a multicenter registry (Congenital Cardiac Catheterization Project on Outcomes). All cases that included balloon angioplasty and/or stent implantation in a proximal or lobar PA position were included. Multivariate analysis was used to evaluate for independent predictors of AE and need for early reintervention. Between February 2007 and December 2009, 8 institutions submitted details on 1315 procedures with a PA intervention. An AE was documented in 22% with a high severity (level 3 to 5) AE in 10% of cases. Types of AE included vascular/cardiac trauma (19%), technical AE (15%), arrhythmias (15%), hemodynamic AE (14%), bleeding via endotracheal tube/reperfusion injury (12%), and other AE (24%). AE were classified as not preventable in 50%, possibly preventable in 41%, and preventable in 9%. By multivariate analysis, independent risk factors for level 3 to 5 AE were presence of ≥2 indicators of hemodynamic vulnerability, age below 1 month, use of cutting balloons, and operator experience of <10 years. Reintervention during the study period occurred in 22% of patients undergoing PA rehabilitation.
PA rehabilitation is associated with a 10% incidence of high-level severity AE. Hemodynamic vulnerability, young age, use of cutting balloons, and lower operator experience were significant independent risk factors for procedure-related AE.
Circulation Cardiovascular Interventions 06/2011; 4(3):287-96. · 6.06 Impact Factor
