Kevin G. Friedman

Management of Pediatric Chest Pain Using a Standardized Assessment and Management Plan

OBJECTIVES: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome: postnatal outcomes of the first 100 patients.

Background— Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported. Methods and Results— We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth ( P <0.01). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96±4% at 5 years and 84±12% at 10 years, which was better than HLHS patients (log-rank P =0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was +1.7 (range, −1.3 to +8.2), and 80% had normal ejection fraction. Conclusions— Short- and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted. # CLINICAL PERSPECTIVE {#article-title-39}Background— Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported. Methods and Results— We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P<0.01). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96±4% at 5 years and 84±12% at 10 years, which was better than HLHS patients (log-rank P=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was +1.7 (range, −1.3 to +8.2), and 80% had normal ejection fraction. Conclusions— Short- and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted.

Resource utilization after introduction of a standardized clinical assessment and management plan.

INTRODUCTION A Standardized Clinical Assessment and Management Plan (SCAMP) is a novel quality improvement initiative that standardizes the assessment and management of all patients who carry a predefined diagnosis. Based on periodic review of systemically collected data the SCAMP is designed to be modified to improve its own algorithm. One of the objectives of a SCAMP is to identify and reduce resource utilization and patient care costs. METHODS We retrospectively reviewed resource utilization in the first 93 arterial switch operation (ASO) SCAMP patients and 186 age-matched control ASO patients. We compared diagnostic and laboratory testing obtained at the initial SCAMP clinic visit and control patient visits. To evaluate the effect of the SCAMP over time, the number of clinic visits per patient year and echocardiograms per patient year in historical control ASO patients were compared to the projected rates for ASO SCAMP participants. RESULTS Cardiac magnetic resonance imaging (MRI), stress echocardiogram, and lipid profile utilization were higher in the initial SCAMP clinic visit group than in age-matched control patients. Total echocardiogram and lung scan usage were similar. Chest X-ray and exercise stress testing were obtained less in SCAMP patients. ASO SCAMP patients are projected to have 0.5 clinic visits and 0.5 echocardiograms per year. Historical control patients had more clinic visits (1.2 vs. 0.5 visits/patient year, P<.01) and a higher echocardiogram rate (0.92 vs. 0.5 echocardiograms/patient year, P<.01) CONCLUSION Implementation of a SCAMP may initially lead to increased resource utilization, but over time resource utilization is projected to decrease.

Resource Utilization Reduction for Evaluation of Chest Pain in Pediatrics Using a Novel Standardized Clinical Assessment and Management Plan (SCAMP)

Background Chest pain is a common reason for referral to pediatric cardiologists. Although pediatric chest pain is rarely attributable to serious cardiac pathology, extensive and costly evaluation is often performed. We have implemented a standardized approach to pediatric chest pain in our pediatric cardiology clinics as part of a broader quality improvement initiative termed Standardized Clinical Assessment and Management Plans (SCAMPs). In this study, we evaluate the impact of a SCAMP for chest pain on practice variation and resource utilization. Methods and Results We compared demographic variables, clinical characteristics, and cardiac testing in a historical cohort (n=406) of patients presenting to our outpatient division for initial evaluation of chest pain in the most recent pre-SCAMP calendar year (2009) to patients enrolled in the chest pain SCAMP (n=364). Demographic variables including age at presentation, sex, and clinical characteristics were similar between groups. Adherence to the SCAMP algorithm for echocardiography was 84%. Practice variation decreased significantly after implementation of the SCAMP (P<0.001). The number of exercise stress tests obtained was significantly lower in the SCAMP-enrolled patients compared with the historic cohort (∼3% of patients versus 29%, respectively; P<0.001). Similarly, there was a 66% decrease in utilization of Holter monitors and 75% decrease in the use of long-term event monitors after implementation of the chest pain SCAMP (P=0.003 and P<0.001, respectively). The number of echocardiograms obtained was similar between groups. Conclusions Implementation of a SCAMP for evaluation of pediatric chest pain has lead to a decrease in practice variation and resource utilization. (J Am Heart Assoc. 2012;1:jah3-e000349 doi: 10.1161/JAHA.111.000349.)

Risk factors for failed staged palliation after bidirectional Glenn in infants who have undergone stage one palliation

OBJECTIVE The bidirectional Glenn procedure (BDG) is a routine intermediary step in single-ventricle palliation. In this study, we examined risk factors for death or transplant and failure to reach Fontan completion after BDG in patients, who had previously undergone stage one palliation (S1P). METHODS All patients at our institution, who underwent BDG following S1P between 2002 and 2009 (n=194), were included in the analysis. RESULTS Transplant-free survival through 18 months post BDG was 91%. Univariable competing risk analyses showed atrioventricular valve regurgitation (AVVR) >mild, age ≤ 3 months at BDG, ventricular dysfunction >mild, and prolonged hospital stay after S1P to be associated with increased risk of death or orthotopic heart transplant. Multivariable competing risk analysis through 5 years of follow-up showed >mild AVVR (hazard ratio (HR) 7.5, 95% confidence interval (CI) 3.0-18.8), prolonged hospitalization after S1P (HR 4.5, 95% CI 1.8-11.5), and age ≤ 3 months at BDG (HR 6.8, 95% CI 2.3-20.0) to be independent risk factors for death or transplant. Concomitantly, > mild AVVR and age ≤ 3 months were independently associated with an overall decreased rate of Fontan completion. CONCLUSIONS Pre-BDG AVVR, age ≤ 3 months at time of BDG, and prolonged hospitalization after S1P are independently associated with decreased successful progression of staged palliation in midterm follow-up after BDG.

Acute Outcomes after Introduction of a Standardized Clinical Assessment and Management Plan (SCAMP) for Balloon Aortic Valvuloplasty in Congenital Aortic Stenosis

INTRODUCTION 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. METHODS 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. RESULTS 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.

Prenatal diagnosis of transposition of the great arteries over a 20‐year period: improved but imperfect

To evaluate temporal trends in the prenatal diagnosis of transposition of the great arteries with intact ventricular septum (TGA/IVS) and its impact on neonatal morbidity and mortality.

Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population

Background The natural history of coronary artery aneurysms (CAA) after intravenous immunoglobulin (IVIG) treatment in the United States is not well described. We describe the natural history of CAA in US Kawasaki disease (KD) patients and identify factors associated with major adverse cardiac events (MACE) and CAA regression. Methods and Results We evaluated all KD patients with CAA at 2 centers from 1979 to 2014. Factors associated with CAA regression, maximum CA z‐score over time (zMax), and MACE were analyzed. We performed a matched analysis of treatment effect on likelihood of CAA regression. Of 2860 KD patients, 500 (17%) had CAA, including 90 with CAA z‐score >10. Most (91%) received IVIG within 10 days of illness, 32% received >1 IVIG, and 27% received adjunctive anti‐inflammatory medications. CAA regression occurred in 75%. Lack of CAA regression and higher CAA zMax were associated with earlier era, larger CAA z‐score at diagnosis, and bilateral CAA in univariate and multivariable analyses. MACE occurred in 24 (5%) patients and was associated with higher CAA z‐score at diagnosis and lack of IVIG treatment. In a subset of patients (n=132) matched by age at KD and baseline CAA z‐score, those receiving IVIG plus adjunctive medication had a CAA regression rate of 91% compared with 68% for the 3 other groups (IVIG alone, IVIG ≥2 doses, or IVIG ≥2 doses plus adjunctive medication). Conclusions CAA regression occurred in 75% of patients. CAA z‐score at diagnosis was highly predictive of outcomes, which may be improved by early IVIG treatment and adjunctive therapies.

Perinatal and Infant Outcomes of Prenatal Diagnosis of Heterotaxy Syndrome (Asplenia and Polysplenia)

Patients with heterotaxy syndrome (HS) have a range of anomalies and outcomes. There are limited data on perinatal outcomes after prenatal diagnosis. To determine the factors influencing perinatal and infant outcomes, we analyzed prenatal and postnatal variables in fetuses with HS from 1995 to 2011. Of 154 fetuses with HS, 61 (40%) had asplenia syndrome (ASP) and 93 (60%) had polysplenia syndrome (PSP). In the ASP group, 22 (36%) patients were elected for termination of pregnancy, 4 (10%) had fetal death, and 35 of 39 (90%) continued pregnancies were live born. In the PSP group, 12 (13%) patients were elected for termination of pregnancy, 5 (6%) had fetal death (4 with bradyarrhythmia), and 76 of 81 (94%) continued pregnancies were live born. Bradyarrhythmia was the only predictor of fetal death. In the live-born ASP group, 43% (15 of 35) died, 7 because of pulmonary vein stenosis, 4 postoperatively, and 4 because of noncardiac causes. In the live-born PSP group, 13% (10 of 76) died, 5 postoperatively, 2 from bradyarrhythmia, 1 from a cardiac event, and 2 from noncardiac causes. Pulmonary vein stenosis and noncardiac anomalies were independent risk factors for postnatal death. Only 8% of ASP patients achieved biventricular circulation, compared with 65% of PSP patients. In the live-born cohort, the 5-year survival rate was 53% for ASP and 86% for PSP. In conclusion, most PSP patients are currently alive with biventricular circulation in contrast with few ASP patients. Bradyarrhythmia was the only predictor of fetal death. Pulmonary vein stenosis and noncardiac anomalies were predictors of postnatal death.

Left Ventricular Remodeling and Improvement in Diastolic Function After Balloon Aortic Valvuloplasty for Congenital Aortic Stenosis

Background— 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. Methods and Results— 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. Conclusion— 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.