Regulatory efforts to assess and improve the quality of pediatric cardiac surgery in New York State

Progress in Pediatric Cardiology 32 (2011) 111–119

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Regulatory efforts to assess and improve the quality of pediatric cardiac surgery in New York State Edward L. Hannan a,⁎, Kimberly S. Cozzens a, Zaza Samadashvili a, John N. Morley i,1, Roberta G. Williams b, Thomas J. Kulik c, Frederick Z. Bierman d, Carlos E. Ruiz e, George M. Alfieris f, John J. Lamberti Jr. g, Jeffrey P. Gold h a

University at Albany, State University of New York, Albany, NY, United States Univeristy of Southern California, Los Angeles, CA, United States Children's Hospital, Boston, MA, United States d Westchester Medical Center, Valhalla, NY, United States e Lenox Hill Heart and Vascular Institute, New York, NY, United States f Strong Memorial Hospital, Rochester, NY, United States g Children's Hospital of San Diego, San Diego, CA, United States h The University of Toledo, Toledo, OH, United States i Health and Hospitals Corporation, New York, NY, United States b c

a r t i c l e Keywords: Pediatric Cardiac Surgery Quality Outcomes Risk-adjustment

i n f o

a b s t r a c t The New York State Department of Health developed a Pediatric Cardiac Surgery Reporting System in 1991 that contains detailed information on demographics, diagnoses, procedures, comorbidities, complications, and discharge information for every pediatric cardiac surgery patient in the state. The Department and the Congenital Cardiac Services Subcommittee of the Department's Cardiac Advisory Committee have used data from this system to assess, assure, and improve quality of care and to generate public reports on an ongoing basis. Two reports (one covering 3 years and the other covering 4 years) have been published, and a third report, comprising the years 2006–2009, will be released shortly. These reports contain information on patient diagnoses, patient severity groups, and risk-adjusted in-hospital mortality rates for all hospitals that perform pediatric cardiac surgery. The goal of the DOH and the CAC is to improve the quality of cardiac care in NYS. Providing the hospitals and cardiac surgeons in NYS with data about their own outcomes for these procedures allows them to examine the quality of the care they provide and to identify areas that need improvement. These data are fed back to providers on an ongoing basis in addition to in the published reports. The overall results of this program of ongoing review show that significant progress is being made. The volume of surgeries and the number of hospitals where they are performed have both decreased dramatically in the years since NYS first began reporting risk-adjusted outcomes for pediatric cardiac surgery. In 1997, 16 hospitals performed 1749 pediatric cardiac surgeries. By 2009, there were only 10 hospitals performing 1304 surgeries. The average number of surgeries per hospital in 1997 was 109 compared to 139 in 2010. Many factors, including increased use of catheter-based therapies, may have contributed to the overall decline in the number of surgeries. The overall mortality rate for pediatric cardiac surgery dropped from 4.08% in 2002 to 2005 period to a mortality rate of 3.35% in 2006 to 2009 period with no major changes in overall patient risk, although part of this decrease may also be due to improvements in the field in general. A multivariable analysis demonstrated that a dedicated pediatric cardiac ICU proved to be a significant independent predictor of mortality. The methods for assessing patients' risk of in-hospital mortality have changed with each successive report, and this study describes the current method and the risk factors used in the method. The Department's other initiatives for improving quality, including consolidation of services, linking processes and structures of care to outcomes, and other regulatory actions, are also described. © 2011 Published by Elsevier Ireland Ltd.

1. Introduction and history ⁎ Corresponding author at: University at Albany, State University of New York, Department of Health Policy, Management, and Behavior, One University Place, Rensselaer, NY 12144-3456, United States. Tel.: +1 518 402 0297 (office); fax: +1 518 402 6992. E-mail address: [email protected] (E.L. Hannan). 1 At the time of the study, the affiliation was New York State Department of Health, Albany, NY, United States. 1058-9813/$ – see front matter © 2011 Published by Elsevier Ireland Ltd. doi:10.1016/j.ppedcard.2011.10.009

In this journal in 2003, the Department of Health (the DOH) and University at Albany School of Public Health described the efforts of the DOH and its Cardiac Advisory Committee (CAC) to improve the quality of pediatric cardiac surgery in New York [1]. This manuscript is an update on the efforts that have occurred since then.

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A thorough history of the adult cardiac surgery and pediatric cardiac surgery systems in New York through 2003 has been described in our earlier communication [1]. The DOH introduced the first cardiac surgery reporting system (the New York State Cardiac Surgery Reporting System, or CSRS) in the United States in 1989, and this effort led to public reporting of risk-adjusted mortality data for hospitals and surgeons for adult cardiac surgery. These data have been reported annually ever since 1989. The DOH also created a coronary angioplasty reporting system in 1992 (now called the Percutaneous Coronary Interventions Reporting System (PCIRS)) and an annual risk-adjusted hospital and cardiologist procedure outcome reports. The DOH and its CAC developed in 1991 a separate pediatric cardiac surgery reporting system that contained the unique diagnoses and procedures for pediatric patients in need of cardiac surgery. At that time, the system allowed for a single diagnosis and a single procedure code, although some procedure codes represented a combination of procedures. The first Pediatric Congenital Cardiac Surgery Report, which was based on 1997 to 1999 data, was released in 2004 [2]. In 2000, the system was expanded considerably by updating diagnosis and procedure codes, but also allowing for multiple diagnoses and procedures for each patient (see Appendices 1 and 2 for a listing of these codes). A report based on 2002 to 2005 data from this newer system was released in January 2007 [3]. The analyses in the second report were enhanced because of the more comprehensive description of each patient's diagnoses and procedures. A third report, based on data from 2006 to 2009 will be released shortly. The following is a description of the methods and statistical models used in the report, as well as the most recent efforts to improve the quality and outcomes of pediatric cardiac surgery in New York. 2. Description of the New York State department of health pediatric cardiac surgery report 2006–2009 report 2.1. Patient population The patient population for the report consists of all pediatric (age less than 18 years) patients undergoing congenital cardiac surgery in New York state hospitals who were discharged between January 1, 2006 and December 31, 2009. Patients with any non-congenital cardiac disease and patients who received a heart or lung transplant, or an artificial heart during their admission were excluded. 2.2. Risk-adjustment process for assessing hospital performance The objective of this report was to assess provider (hospital) performance on the basis of risk-adjusted short-term outcomes (in-hospital mortality) as in the previous two New York pediatric cardiac surgery (PCS) reports [2,3] and in New York's adult cardiac surgery [4] and percutaneous coronary interventions reports [5]. The following is a description of the risk-adjustment process used in the upcoming report. Hospitals in New York State where pediatric cardiac surgery is performed are required to provide information to the DOH for each patient undergoing PCS procedures. The cardiac surgery department in each hospital collects data pertaining to patients' demographic and clinical characteristics (e.g., age, sex, pediatric congenital diagnoses and comorbidities). Approximately 25 of these characteristics (or risk factors) are collected for each patient. These data, along with information about the hospital, physician, procedure performed, and the patient's status at discharge, are entered into a computer and sent to the DOH for auditing and analysis. The data accuracy is verified through the review of unusual reporting frequencies, cross-matching of pediatric cardiac surgery data with other DOH databases and a review of medical records for a selected sample of cases. These activities are extremely helpful in ensuring consistent interpretation of data elements and external generalizability across hospitals.

The outcome that is examined and reported on is death occurring during the same hospital stay in which the patient underwent a pediatric congenital cardiac surgery. An in-hospital death is defined as a patient who died subsequent to cardiac surgery during the same acute care admission or who was discharged to hospice and expires within 30 days. The risk factors in the data system are used to predict each patient's chance of dying in the hospital based on the statewide relationship between the risk factors and in-hospital mortality (expected mortality). The general idea behind the method is to identify which of the potential risk factors are significant independent predictors of mortality and include them in the final statistical model that assigns weights to this set of significant predictors. A predictor is regarded as a significant “independent” predictor if its presence in the model significantly changes the estimates of patients' chance of dying in the hospital compared to a model without that predictor in it. The method used to assess risk in this report differs somewhat from the methods used in the previous reports [3]. In those reports, patients were initially placed into 14 groups on the basis of important determinants of mortality: age, whether the heart is univentricular or biventricular, and the severity of the congenital diagnosis at admission (high, medium, low). A univentricular heart, or “single ventricle”, is a heart that has only one ventricle capable of sustaining a full cardiac output. The patients were categorized, in addition to these 14 groups, according to whether or not they had other conditions (comorbidities) that were shown to be predictors of mortality. This was done using an iterative process whereby tentative risk levels were developed using the data, and CAC members altered the risk level assignments based on their clinical knowledge, and the data were used again to examine the assignments. This process was repeated until the CAC was comfortable with the risk level assignments. When that method was tested on the 2006 to 2009 data for the most recent report, it did not have the consistent estimates across patient groups that were present in the previous report. This was likely due to the fact that some of the 14 groups had so few patients in them that their mortality rates could not be trusted to be accurate estimates. Consequently, in this report each of the important determinants of mortality (e.g., age/birth weight, univentricular/biventricular anatomy and severity of diagnosis) were treated as independent predictors of mortality in the risk-adjustment calculation, similar to the methodology used in the CSRS and PCIRS reports although the exact determinants are different. The statistical analyses show that splitting out the various patient characteristics in this new way is preferable because it allows for a more precise prediction for each individual patient. Importantly, it does not suffer from the estimation problems related to small sample sizes for some groups of patients. However, clustering cases into similar groups to look at outcomes for various types of patients is useful in assessing the performance of healthcare providers and in helping to understand the risk associated with particular cases. Therefore, Appendix 3 is also used in the report to present mortality rates for groups of patients according to age, number of ventricles and risk. Table 1 presents the statistical model that is used as a basis for calculation of each hospital's risk-adjusted mortality rate. Specifically, the statistical model in Table 1 is used to calculate the probability of in-hospital mortality for each pediatric cardiac surgery patient as a function of the factors mentioned above. The different factors and the way they are represented are as follows: Age/birth weight: age N 1 year, age b 30 days/birth weightb 2000 g, age b 30 days/birth weight 2000 g or more, age 30 days–1 year/birth weightb 2000 g, age 30 days–1 year/birth weight 2000 g or more Number of functioning ventricles: 1,2 Risk associated with diagnosis: Low, Medium, High

E.L. Hannan et al. / Progress in Pediatric Cardiology 32 (2011) 111–119 Table 1 Multivariable risk factor equation for pediatric congenital cardiac surgery in-hospital mortality in New York State, 2006–2009. Logistic regression Patient risk factor Age Greater than 1 year Less than 30 days With birthweight b 2000 g With birthweight 2000 g or more 30 days–1 year With birthweight b 2000 g With birthweight 2000 g or more Type of defect Biventricular Univentricular Risk associated with diagnosis Low Medium High Comorbidities Pre-op ventilator dependence Major extracardiac anomalies Pre-existing neurology abnormality Severe cyanosis or hypoxia Positive blood cultures Renal failure requiring dialysis Pulmonary hypertension

Prevalence (%)

Coefficient

P-value

Odds ratio

41.04

—Reference—

1.07 21.33

3.0545 1.4922

b.0001 b.0001

21.210 4.447

3.20 33.36

1.6493 0.5577

b.0001 0.0667

5.203 1.747

82.37 17.63

—Reference— 0.4426 0.0222

1.000 1.557

22.21 60.03 17.76

—Reference— 1.3242 0.0112 2.0460 b.0001

1.000 7.737 3.759

16.49 9.42 3.39

0.5207 0.5649 0.8300

0.0091 0.0082 0.0042

1.683 1.759 2.293

15.50 1.51 0.41 11.70

0.4673 0.8848 1.3483 0.4438

0.0090 0.0120 0.0221 0.0314

1.596 2.423 3.851 1.559

1.000

Intercept = − 6.3715. C-statistic = 0.854.

Comorbidities (present or not): major extracardiac anomalies, positive blood cultures, pre-existing neurologic abnormality, pre-op ventilator dependence, pulmonary hypertension, severe cyanosis or hypoxia The process undertaken to assign each patient to a risk group is described in Appendix 4. The independent importance of each of the factors can be interpreted using the odds ratios in Table 1. For each factor, the lowest level of severity is represented as the “reference” value. The odds ratios of other categories of that factor are relative to the reference value. As an example, a patient with an age between 30 days and 1 year and a birth weight less than 2000 g has an odds of dying in the hospital that are 5.203 times the odds of a patient in the reference category (age more than 1 year) dying in the hospital, assuming all other risk factors for the patients are the same. For binary risk factors (presence or absence of a condition), the odds are for a patient with the condition compared to a patient without the condition. For example, a patient with pulmonary hypertension has odds of dying in the hospital that are 1.559 times as high as a patient without pulmonary hypertension, assuming the patients have the same other risk factors represented in Table 1. The coefficients of the risk factors represented in Table 1 are used to predict the probability of in-hospital death for each patient. This is done using the formula from the statistical model (logistic regression) that was used to arrive at the set of significant risk factors and their coefficients. The predicted probabilities of mortality for all patients in a hospital are averaged to obtain the predicted or expected mortality rate (EMR) for the individual hospital. The EMR is an estimate of what the hospital's mortality rate would have been if the hospital's performance was identical to the state performance. The EMR is therefore an indicator of patient severity of illness. A hospital's EMR is contrasted with its observed mortality rate (OMR), which is the number of pediatric

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congenital cardiac surgery patients who died in that hospital divided by the total number of pediatric congenital cardiac surgery patients in that hospital. To calculate the risk-adjusted mortality rate (RAMR), the OMR is divided by the hospital's EMR. If the resulting ratio is larger than one, the hospital has a higher mortality rate than expected on the basis of the patient mix; if it is smaller than one, the hospital has a lower mortality rate than expected from its patient mix. The ratio is then multiplied by the overall statewide mortality rate (3.35% for 2006 to 2009) to obtain the hospital's risk-adjusted rate. There is no statewide EMR or RAMR, because the statewide data is not risk-adjusted. The statewide OMR (number of total cases divided by number of total deaths) serves as the basis for comparison for each hospital's EMR and RAMR rates. If the RAMR is significantly lower than the statewide mortality rate, the hospital has a better performance than the state as a whole; if, however, the RAMR is significantly higher than the statewide mortality rate, the hospital's performance is worse than the state as a whole. Significant differences, higher and lower, are identified in the report with one or two asterisks, respectively. The RAMR is used in the report as a measure of the quality of care provided by hospitals. The risk-adjusted mortality rate (RAMR) represents the best estimate, based on the associated statistical model, of what the hospital's mortality rate would have been if the hospital had a mix of patients identical to the statewide mix. Thus, the RAMR, to the extent possible, smoothes out differences among hospitals in patient severity of illness, since it calculates a mortality rate for each hospital based on an identical group of patients. There are reasons that a provider's risk-adjusted rate may not be indicative of their true quality of care. For example, extreme outcome rates may occur due to chance alone. This is particularly true for lowvolume providers, for whom very high or very low rates are more likely to occur than for high-volume providers. The expected ranges (confidence intervals) are included as part of the reported results in an attempt to prevent misinterpretation of differences caused by chance variation. Differences in hospital coding of risk factors could be an additional reason that a hospital's risk-adjusted mortality rate may not be reflective of their quality of care. The DOH monitors the quality of coded data by reviewing medical records to confirm the presence of key risk factors in each reported patient outcome. Every effort is made to review the list of risk factors used in the system to ensure that all important risk factors are included.

3. Using the pediatric cardiac surgery reports to improve quality The goal of the DOH and the CAC is to improve the quality of cardiac care in NYS. Providing the hospitals and cardiac surgeons in NYS with data about their own outcomes for these procedures allows them to examine the quality of the care they provide and to identify areas that need improvement. These data are fed back to providers on an ongoing basis in addition to in the published reports. However, the DOH and CAC also try to use the data to improve quality and outcomes. The data analyzed in this program are reviewed by the CAC, and CAC members assist with interpretation and advise the DOH regarding hospitals and surgeons that may need special attention. Committee members have also conducted site visits to particular hospitals and have recommended that some hospitals obtain the expertise of outside consultants to design improvements for their programs. The overall results of this program of ongoing review show that significant progress is being made. For example, the overall mortality rate for pediatric cardiac surgery dropped from 4.08% in 2002–2005 to 3.35% in 2006–2009 with no major changes in overall patient risk, although part of this decrease may also be due to improvements in the field in general.

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4. Efforts to link processes/structures and outcomes The DOH and CAC have also tried to identify the processes and structures of care that are most important in achieving optimal outcomes [6]. Appendix 5 contains a survey administered to the pediatric cardiac surgery programs in the state in order to identify important structures of care. When responses to the survey were linked to outcomes, two measures (a dedicated pediatric cardiac ICU and a group of nurses dedicated to pediatric cardiac patients) were each associated with significantly better outcomes. These two measures were also highly correlated with one another, and in multivariable analyses, only a dedicated pediatric cardiac ICU proved to be a significant independent predictor of mortality. 5. Consolidation of services The volume of surgeries and the number of hospitals where they are performed have both decreased dramatically in the years since NYS first began reporting risk-adjusted outcomes for pediatric cardiac surgery. In 1997, 16 hospitals performed 1749 pediatric cardiac surgeries. By 2009, there were only 10 hospitals performing 1304 surgeries. The average number of surgeries per hospital in 1997 was 109 compared to 139 in 2010. Many factors, including increased use of catheter-based therapies, may have contributed to the overall decline in the number of surgeries. The reduced number of programs is in large part a direct consequence of this declining volume and a reflection of the DOH efforts to promote robust, high-quality programs by supporting consolidation of services where appropriate. In several cases, pediatric cardiac surgery programs were closed, either voluntarily by the hospital or by the Department, after a period of low volume and an inability to increase referrals to the program. This was in part informed by an earlier study done using New York data that affirmed the importance of higher volumes in achieving low mortality [7]. Several centers found it difficult to retain a fulltime pediatric cardiac surgeon based on the case volume available. An itinerant or shared surgeon model has been attempted at some centers with mixed success. 6. Regulatory standards The New York State Department of Health has regulatory authority over hospitals that perform pediatric cardiac surgery. These regulations establish staffing requirements, minimum workload standards and structure and services requirements to “ensure both quality and economy of services.” In November 2009, title 10 section 405 was revised to codify the policy of encouraging the development and maintenance of high-quality programs with substantial volume [8]. The minimum volume requirement was increased from 50 cases per year to 75 cases. These regulations also provide a mechanism for lower volume programs to continue if they enter into a coordinated program with one or more partners, as long as the total volume exceeds 100 per year and one of the centers performs at least 75 cases annually. By requiring these programs to enter into a formal agreement covering quality improvement, peer review and coordination of care between the affiliated centers, access and distribution of services are maintained while quality is assured. There are also provisions to waive the minimum volume requirements if there are extenuating circumstances or to ensure access to care, as long as the program has good outcomes. The revised regulations also reflect lessons learned from past experience with low volume programs in their specific requirements for cardiac surgeon availability. The revised regulations allow for inclusion of detailed requirements for the availability of a cardiac surgeon in the post-operative period and during interventional pediatric cardiac catheterization.

In NYS, a certificate of need is required prior to opening new services. The criteria for determining need (709.14) were also updated in November 2009. These standards require that all other pediatric cardiac surgery programs in the planning area are performing at least 200 cases per year and that the applicant can demonstrate a projected volume of 200 cases per year individually or 50 cases per year as part of a coordinated program with another hospitals. These planning projections represent higher volume than those required to maintain services at a center already operating. 7. Future directions 7.1. Outcomes for adult congenital cardiac surgery The data collection for adult cardiac surgery patients (CSRS, as described above) was modified in July 2009, to include cardiac diagnosis for adult patients undergoing cardiac surgery for a congenital condition. This will allow for assessment of outcomes for adult congenital cardiac surgery. 7.2. Evaluation of short-term out-of hospital deaths for pediatric congenital cardiac surgery In-hospital mortality is not the ideal measure for assessing provider performance in cardiac surgery, and for the last several years in-hospital mortality/death outside the hospital within 30 days of the index procedure has been used in the Department's adult cardiac surgery and percutaneous coronary angioplasty reports [4,5]. Although it is not expected that there will be a high percentage of short-term deaths after discharge for pediatric patients, this needs to be investigated further. 8. Discussion The goal of the DOH and the CAC is to improve the quality of cardiac care in NYS. The DOH has undertaken a few new initiatives since the last report of our activities in the journal [1]. These initiatives include: • the development of a new methodology for obtaining risk-adjusted mortality rates for hospitals to assess their quality/performance; • the creation of a survey to compare processes and structures of care in pediatric cardiac surgery hospitals, and to study the relationship between these processes and structures and outcomes of care; [2] • the commitment to expand this survey in light of the recent National Quality Forum Consensus Standards Report; and, [9] • the consolidation of services in light of low volumes of some programs (based on the model of cooperative programs that was written into DOH regulations). As noted earlier, we have been exploring new ways of predicting short-term mortality for patients that would prove to be more accurate [1]. The method used in our previous reports consisted of using 14 categories of patients based on age, number of functioning ventricles, and the risk level of the combination of diagnoses the patient had, as well as a small number of comorbidities. This method did not prove to be stable when applied to our newer 2006 to 2009 dataset, probably because some of the 14 patient categories had too few patients in them to be representative of the true risk. Our solution to the problems discussed earlier with our previous statistical model was to develop a model using all of the previous important risk factors (e.g., age, number of ventricles, diagnosis risk level) in addition to birth weight combined with age as independent predictors of risk rather than to force patients into categories contained in the model. This approach is similar to the way the methodology is used in the adult cardiac and many other statistical models. The results demonstrate that the predictive ability of the new pediatric model is very good.

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It should be noted that our new method is similar to the commonly used Risk-Adjusted Classification for Congenital Heart Surgery (RACHS)-1 method [10]. The RACHS-1 method also uses age (cut at the same intervals), birth weight (cut at 2500 g), and some comorbidities (e.g., Down syndrome, major chromosomal abnormality or recognizable syndrome, and major noncardiac structural abnormality). In addition, the RACHS uses transfer from another acute care facility and male gender in their riskadjustment scheme. Another difference is that the RACHS measures risk severity using aggregations of procedural groups rather than aggregations of diagnostic groups. The CAC opted to base risk on diagnoses instead of procedures because they felt that diagnoses are a more accurate reflection of a patient's illness than the chosen treatment. It should be noted anecdotally that when we applied both the RACHS-1 and our method to our database, our method performed better, but not statistically significantly better, than the RACHS-1 method. Our ultimate goal is to identify the complex relationship between institutional volume, case complexity, processes/structures of care, and outcomes. The survey tool developed by the CAC has been helpful in clarifying some of the important determinants of quality of care and outcomes for pediatric cardiac surgery. However, these should be regarded as preliminary conclusions at the current time pending further investigation of the hospital responses and pending the expansion of the number of questions in the survey based on the more comprehensive NQF tool. We are hopeful that these investigations will lead to recommendations for hospitals regarding the procedures they should perform as a function of their annual volume and the processes and structures of care they have in place. Arriving at these recommendations will be a challenge given the multiplicity of diagnoses and procedures relative to the number of patients undergoing pediatric cardiac surgery. In summary, the DOH and its CAC are committed to a multifaceted effort to continually improve the quality of pediatric cardiac surgery in New York State. This collaboration has resulted in improved quality and outcomes during the last two decades, and we look forward to continued success in providing the best possible care in our New York pediatric cardiac surgery hospitals.

Appendix 1 (continued) 120 121 122 123 130 131 132 133 134 135 140 141 142 Anomalies of ventricular septum 150 151 152 153 154 155 Atrioventricular septal defects (AVSD) 160 163 161 162 Univentricular heart (single ventricle) 170 171 172 173 174 175 176 180 181 190 191

Appendix 1. Primary cardiac diagnosis codes NYSDOH Cardiac Advisory Committee Atrial situs anomalies 010 011 Cardiac position anomalies 020 021 022 Anomalies of pulmonary veins 100 101 102 103 104 105 106 Anomalies of atrial septum 110 111 112 113 114

Situs inversus Situs ambiguous/Heterotaxy syndrome Dextrocardia Mesocardia Ectopia cordis Partial anomalous return Total anomalous return Supracardiac Cardiac Infracardiac Mixed Pulmonary vein stenosis Cor triatriatum Secundum ASD Single atrium Unroofed coronary sinus Sinus venosus ASD PFO

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Anomalies of ventricular outflow tracts 200 201 209 202 203 204 205 206 207 208 210 211 212 220 230 231 232

Tricuspid valve Ebstein's anomaly Tricuspid stenosis Tricuspid regurgitation Straddling tricuspid valve Mitral valve Supravalvular mitral stenosis Valvular mitral stenosis Subvalvular mitral stenosis Mitral regurgitation Straddling mitral valve Papillary muscle abnormality Common AV valve abnormality Stenosis Regurgitation Malaligned Perimembranous VSD Doubly committed VSD (subarterial) Inlet VSD Muscular VSD Multiple VSDs Malalignment VSD

Partial AVSD (primum ASD) Transitional/Intermediate AV canal Complete AVSD Balanced Unbalanced

Double/Common inlet LV Double/Common inlet RV Tricuspid atresia With IVS With VSD With TGA Mitral atresia Indeterminate ventricle Hypoplastic right ventricle Pulmonary atresia with IVS Other type of hypoplastic RV Hypoplastic left ventricle Classical HLHS (aortic atresia w/ hypoplastic LV) Any other hypoplastic LV Pulmonary ventricular outflow tract Pulmonary valve stenosis Supravalvar pulmonary stenosis Subvalvular/Infundibular pulmonary stenosis Double chamber right ventricle Branch pulmonary artery stenosis Hypoplastic pulmonary arteries Pulmonary valve regurgitation Main pulmonary artery atresia Branch pulmonary artery atresia Aortic ventricular outflow tract Valvular aortic stenosis Subvalvular aortic stenosis Discrete Long segment/tunnel Supravalvular aortic stenosis Aortic valve atresia Aortic valve regurgitation Aorto-ventricular tunnel

Tetralogy of Fallot (TOF) 240 241 242

RV–PA continuity TOF with pulmonary valve atresia Absent pulmonary valve syndrome

Truncus arterious 250 251 252

Type I Type II Type III (continued on next page)

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Appendix 1 (continued)

Appendix (continued) 2 (continued)

Transposition of the great arteries (TGA) 260 261 Double outlet right ventricle (DORV) 270 271 272 273 274 Great vessel anomalies 280 281 282 283 284 297 285 286 287 288 289 290 291 292 293 294 295 296 Coronary artery anomalies 300 301 302 303 304 305 306 Cardiac rhythm anomalies 310 311 312 313 Cardiomyopathies 320 321 322 398 Acquired disease 400 401 402 403 Organ failure 820 821 Cardiac neoplasms 900 901 902 903

100–398 D-TGA Congenitally corrected transposition Subaortic VSD Subpulmonic VSD Uncommitted VSD Doubly committed VSD Restrictive VSD Aortopulmonary window Patent ductus arteriosus Origin of L/R PA from aorta Sinus of Valsalva aneurysm/fistula Aortic coarctation Hypoplastic aortic arch Aortic interruption Aortic aneurysm Ascending Descending Transverse Vascular ring Origin of LPA from RPA (PA sling) Discontinuous PAs Bronchial PA blood flow (MAPCA) Isolated LSVC Bilateral SVCs Azygous/Hemiazygous continuous IVC Other great vessel anomalies Coronary artery fistula Coronary artery sinusoids Coronary artery stenosis Coronary artery aneurysm Anomalous origin coronary Artery Atresia left main coronary artery Atresia right main coronary artery Supraventricular tachycardia Ventricular tachycardia Sinus bradycardia Heart block Hypertrophic Left ventricle Right ventricle Dilated Other diagnoses NOT listed Kawasaki's disease Endocarditis Myocarditis Traumatic Cardiac Pulmonary Atrial Ventricular Valvular Great vessel

Appendix 2. Congenital and acquired cardiac procedure codes NYSDOH Cardiac Advisory Committee 100–398

Congenital heart disease — operations with or without extracorporeal circulation

Note: extracorporeal circulation will be determined from the data element entire procedure off pump reported under Section 2. Procedural information on the front of the form. Please accurately complete this item for all appropriate cases. Anomalies of pulmonary veins 100 Repair of anomalous pulmonary venous return 101 Repair of pulmonary vein stenosis 103 Repair of partial anomalous pulmonary venous return

Anomalies of atrial septum 120 121 122 123 Atrioventricular septal defect (AVSD) 130 131 Anomalies of ventricular septum 140 141 142 Anomalies of atrioventricular valves Tricuspid valve 150 151 152 153 154 Mitral valve 160 161 Replacement 162 163 170 Anomalies of ventricular outflow tract(s) Pulmonary ventricular outflow tract 180 181 182 Pulmonary valve replacement 190 191 192 Pulmonary outflow conduit 200 201 202 210 211 212 Aortic ventricular outflow tract 220 221 230 231 235 240 241 242 243 250 251 252 255 Tetralogy of Fallot 260 261 262 263 264 265

Congenital heart disease — operations with or without extracorporeal circulation ASD closure Creation of ASD Repair of cor triatriatum PFO closure

Repair of complete AV canal Repair of partial AV canal

Repair of VSD Creation/Enlargement of VSD Fenestration of VSD patch

Repair (non-Ebstein's valve) Replacement Homograft Prosthetic Tricuspid valve closure Repair Ebstein's anomaly Resect supramitral ring Repair (including annuloplasty) Homograft Prosthetic Common AV valve repair

Pulmonary valvotomy/valvectomy Resection of subvalvular PS Repair of supravalvular PS Homograft Prosthetic Xenograft Valved Homograft Prosthetic Non-valved Transannular patch With monocusp valve Without monocusp valve Repair branch PS

Aortic valvuloplasty Aortic valvotomy Repair supravalvular AS Resection of discrete subvalvular AS Aortoventriculoplasty (Konno procedure) Aortic valve replacement Autograft (Ross procedure) Homograft Prosthetic Heterograft Aortic root replacement Autograft (Ross procedure) Homograft Prosthetic LV apex to aorta conduit Repair with pulmonary valvotomy Repair with transannular patch Repair with non-valved conduit Repair with valved conduit Homograft Prosthetic Repair with reduction/plasty of PAs Repair with pulmonary valve replacement

E.L. Hannan et al. / Progress in Pediatric Cardiology 32 (2011) 111–119 Appendix (continued) 2 (continued) 100–398 266 267 Truncus arteriosus 262 263 264 Univentricular heart (single ventricle) 270 271 272 273 274 275 Hypoplastic right ventricle 200 201 202 Transannular patch 210 211 Hypoplastic left ventricle 280 290 Transposition of great arteries or double outlet RV 310 311 312 313

Appendix (continued) 2 (continued) Congenital heart disease — operations with or without extracorporeal circulation Homograft Prosthetic Repair with non-valved conduit Repair with valved conduit Homograft Prosthetic

326 327 328 Great vessel anomalies 330 331 332 333 340 348 341 342 343 344 345 346 347 Coronary artery anomalies 350 351 352 353 Cardiomyopathies 360

361

100–398 Interval procedures 370 375 Shunts 381 382 383

Fontan operations Direct RV–PA connection Total cavopulmonary connection Lateral tunnel — nonfenestrated Lateral tunnel — fenestrated Extracardiac — nonfenestrated Extracardiac — fenestrated Septation of single ventricle

384 385 386 390 398

Valved Homograft Prosthetic Non-valved

401 402 403

With monocusp valve Without monocusp valve

Norwood Damus Kaye Stansel (DSK)

Arterial switch Senning procedure Mustard procedure Intraventricular repair of DORV

Rastelli procedure RV–PA conduit 320 321 322 325 LV–PA conduit

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Valved Homograft Prosthetic Non-valved REV operation (Modified Rastelli) Valved Homograft Prosthetic Non-valved PDA ligation Repair aortopulmonary window Reimplantation of left or right pulmonary artery Repair sinus of Valsalva aneurysm Aortic repair (coarctation or interruption) End to end anastomosis End to side anastomosis Subclavian flap angioplasty Onlay patch Interposition graft Vascular ring division Repair of PA sling Reimplantation of innominate artery Aortopexy Translocation of LCA to aorta Direct Transpulmonary tunnel (Takeuchi) Coronary artery ligation Coronary fistula ligation Left ventricular reconstruction (Batiste procedure, surgical ventricular restoration) Radical myomectomy

400–998

404 Repair of aortic deceleration injury 420 421 Other 498

Congenital heart disease — operations with or without extracorporeal circulation Pulmonary artery band Unifocalization of pulmonary vessels Central aortopulmonary shunt Blalock Taussig shunts Classical Modified Glenn shunts Unidirectional (classical) Bidirectional Bilateral bidirectional Cardiac arrhythmia surgery Other operations for congenital heart disease Acquired heart disease — operations performed with or without extracorporeal circulation Mitral valvotomy Pericardiectomy Stab wound of heart or great vessel repair (without extracorporeal circulation) Saccular aortic aneurysm

With shunt Without shunt Other operation for acquired heart disease (without extracorporeal circulation)

Valvuloplasty — single valve 500 Aortic 501 Mitral 502 Tricuspid Replacement — single valve 510–518* Ross procedure 520–528* Aortic mechanical 530–538* Aortic heterograft 540–548* Aortic homograft 550–558* Mitral mechanical 560–568* Mitral heterograft 570–578* Tricuspid mechanical 580–588* Tricuspid heterograft 590–598* Pulmonary 600–608* Mitral valve homograft Multiple valve surgery — valvuloplasty or replacement 610–618* Double, including tricuspid 620–628* Double, not including tricuspid 630–638* Triple *Reoperations: for single valve replacement or multiple valve surgery (510–638), use third digit to indicate reason for reoperation, as follows: 0 Not a reoperation 4 Failed valvuloplasty 1 Periprosthetic leak 5 Disease of another valve 2 Prosthetic endocarditis 8 Other reason 3 Prosthetic malfunction Valve conduits 660 Apical aortic conduit Note: record aortic valve and ascending aorta replacement under aneurysms. Coronary artery bypass grafts 670 Coronary artery bypass graft Please note: if you code a 670 then you must complete the CABG information under the procedural information section of the form. Other revascularization 710 Transmyocardial revascularization 715 Growth factor installation Combined CABG with other 720 Acquired ventricular septal defect 721 Resection or plication of LV aneurysm 722 Carotid endarterectomy 723 Implantation of AICD 724 Ventricular reconstruction (Batiste procedure, surgical ventricular restoration) Please note: if you code a 720–724 then you must complete the CABG information under the procedural information section of the form. (continued on next page)

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Appendix (continued) 2 (continued) 100–398 Valve surgery and CABG 740

Appendix 3. Mortality rates for various patient risk groups Congenital heart disease — operations with or without extracorporeal circulation

Mitral valve replacement plus single or multiple CABG 741 Mitral valvuloplasty plus single or multiple CABG 742 Aortic valvuloplasty or replacement plus single or multiple CABG 744 Double valvuloplasty or replacement, including tricuspid, plus single or multiple CABG 746 Other single valve surgery plus single or multiple CABG 747 Other multiple valve surgery plus single or multiple CABG Please note: if you code a 740–747 then you must complete the CABG information under the procedural information section of the form. Surgery for complication of CAD without CABG 760 Acquired ventricular septal defect 761 Resection or plication of LV aneurysm 762 Ventricular reconstruction (Batiste procedure, surgical ventricular restoration) Radiofrequency or operative ablation 770 Atrial 771 Ventricular 772 Maze procedure Aortic aneurysm repair/aortic root replacement 780 Ascending aorta, with graft, with coronary reimplantation 781 Ascending aorta, replacement or repair, without coronary reimplantation 782 Transverse aorta 783 Descending thoracic aorta (excluding acute deceleration injury) 784 Thoracoabdominal 785 Aortic root replacement or repair, with graft, with coronary reimplantation Dissecting aneurysm surgery 800 Intraluminal graft 801 Intraluminal graft with aortic valve suspension 802 Tube graft with aortic valve suspension 803 Tube graft with aortic valve replacement 818 Other dissecting aneurysm surgery Transplant procedures 820 Heart transplant 821 Heart and lung transplant 822 Lung transplant 830 Left ventricular assist device (LVAD) — extracorporeal 831 Left ventricular assist device (LVAD) — implantable 832 Right ventricular assist device (RVAD) 833 Bi-ventricular assist device (BIVAD) 834 Extracorporeal membrane oxygenation (ECMO) 840 Ventricular assist device as a destination therapy (must also code either 830 or 831) 901 Artificial heart Other 902 Pulmonary embolectomy 903 Stab wound of heart or great vessel repair (with extracorporeal circulation) 904 Removal of intracardiac tumor 905 Removal of intracardiac catheter 906 Repair of aortic deceleration injury (with aortofemoral bypass) 907 Repair of a cardiac laceration due to trauma 915 Septal myomectomy 916 Ventricular myomectomy 920 Ventricular free wall rupture 998 Other operation for acquired heart disease (with extracorporeal circulation)

Patient group Univentricle, age b 30 days High-risk Moderate-risk Univentricle, age 1–12 months Moderate to High-risk Univentricle, age ≥ 1 year Moderate to High-risk Biventricle, age b 30 days High-risk Moderate-risk Low-risk Biventricle, age ≥ 1 month High-risk, 1–12 months Moderate-risk, 1–12 months Moderate to High-risk, ≥1 year Low risk Total

Cases

Prevalence (%)

No. of deaths

Mortality rate (%)

188 117

3.64 2.27

32 18

17.02 15.38

289

5.60

11

3.81

316

6.12

4

1.27

144 634 73

2.79 12.28 1.41

22 35 1

15.28 5.52 1.37

161 1106 1061 1073 5162

3.12 21.43 20.55 20.79 –

14 20 13 3 173

8.70 1.81 1.23 0.28 3.35

Appendix 4. Assignment of risk level to patients The following is the process used to assign risk levels (e.g., Low, Medium, High) to patients. First, each pediatric diagnosis in the system was analyzed with regard to its mortality rate when it was a patient's only diagnosis and when it was one of multiple diagnoses. This information was used in conjunction with the number of functioning ventricles to assign each diagnosis a severity level of Low, Medium, or High. For univentricular patients, a high-risk patient was one who had at least one of the following diagnoses: Hypoplastic Left Ventricle, Classical HLHS or any other. Patients with a univentricular heart that did not have one of these diagnoses were classified as moderate-risk. No univentricular patients are associated with a low risk diagnosis. For biventricular patients, a high risk patient was one who had at least one of the following diagnoses: Anomalies of Pulmonary Veins — Total Anomalous Return, Infracardiac; Anomalies of Pulmonary Ventricular Outflow Tract, Main Pulmonary Artery Atresia; Tetralogy of Fallot, with Pulmonary Valve Atresia or Absent Pulmonary Valve Syndrome; Double Outlet Right Ventricle with Subaortic, Subpulmonic or Uncommitted Ventricular Septal Defect. A low risk patient was one whose only reported diagnosis was one of the following: Anomalies of the Atrial Septum — Secundum ASD, Single Atrium, Unroofed Coronary Sinus, Sinus Venosus ASD, or Patent Foramen Ovale; Anomalies of Ventricular Septum/Ventricular Septal Defect (VSD) — Perimembranous, Doubly committed, Inlet, Muscular or Multiple; Atrioventricular Septal Defects (AVSDs) — Partial Primum ASD or Complete AVSD — Balanced; Anomalies of the Pulmonary Ventricular Outflow Tract — Pulmonary Valve Stenosis, Subvalvular/Infundibular Pulmonary Stenosis or Double Chamber Right Ventricle; Anomalies of Aortic Ventricular Outflow Tract — Valvular Aortic Stenosis, Discrete Subvalvular Aortic Stenosis, or Aortic Valve Regurgitation; Great Vessel Anomalies — Aortic Coarctation. A patient with Anomalies of Pulmonary Veins — Partial Anomalous Return in conjunction with Anomalies of Atrial Septum — Sinus Venosus ASD was also at low risk. Otherwise, patients with more than one diagnosis were not considered low risk. Patients classified as moderate risk were those with any diagnosis not listed as high or low risk as described above, or with more than one low risk diagnosis. Appendix 5. Pediatric cardiac hospital survey The pediatric Subcommittee of the Cardiac Advisory Committee is requesting all facilities performing pediatric cardiac surgery in New York State complete this survey in order to look at specific features of the programs. Hospital name: Pediatric cardiac catheterization program director:

E.L. Hannan et al. / Progress in Pediatric Cardiology 32 (2011) 111–119 Pediatric cardiac surgery program director: 1. Describe the location and staffing of intensive care for pediatric cardiac surgery patients. Answer the questions below and provide additional information on a separate page if desired. a. Is there a dedicated pediatric cardiac ICU? Yes No ○ ○ b. Describe the unit's geography. Are patients situated in a specific geographic area where their care is rendered by specially trained personnel? c. How is the unit staffed? I. Is there a core group nurses dedicated to pediatric cardiac patients? Yes No ○ ○ d. Are there ongoing educational programs regarding post-op care for Yes No attendings, fellows, residents, nurses and other support personnel? ○ ○ If yes, please describe: e. Is there 24-hour/7-days-a-week attending in-house coverage in Yes No the Pediatric Intensive Care Unit (PICU)? ○ ○ I. If yes, please provide their board qualifications (i.e. pediatric, intensivist, etc.) 2. Please describe anesthesiology coverage for pediatric surgery patients. a. Do you have dedicated pediatric cardiac anesthesiologists? Yes No ○ ○ b. Is there a pediatric cardiac anesthesia call schedule? Yes No ○ ○ I. If yes, is it 24/7/365? Yes No ○ ○ c. What are the qualifications of the anesthesiologists who cover pediatric cardiac surgical cases? For example, have they done extra training in the field (i.e. fellowship) or how may experience do they have in the field? 3. Is there a single person who oversees the quality of the pediatric Yes No cardiac program? ○ ○ a. If yes, please provide name and title.

References [1] Doran DR, Roark CS, Hannan EL. Improving quality of care for pediatric cardiac surgery in New York State. Prog Pediatr Cardiol 2003;18:13–25. [2] Pediatric congenital cardiac surgery in New York State: 1997–1999. New York State Department of Health; February 2004. [3] Pediatric congenital cardiac surgery in New York State: 2002–2005. New York State Department of Health; January 2007. [4] Coronary artery bypass surgery in New York State: 2006–2008. New York State Department of Health; December 2010. [5] Percutaneous coronary angioplasty in New York State: 2006–2008. New York State Department of Health; December 2010. [6] New York State Department of Health. Pediatric cardiac hospital survey; September 15 2009.

4. Pediatric residency/fellowship in formation: a. Is there a pediatric residency program? If yes, do the pediatric residents participate in post-op-care? If so, where? Check all that apply 1) PICU 2) CVICU 3) Stepdown Unit b. Is there a pediatric cardiology fellowship program? I. How many fellows per year? II. Do the cardiology fellows participate in the ICU care?

119

Yes ○ Yes ○

No ○ No ○

□ □ □ Yes No ○ ○

Yes ○ 5. Is there a general surgery residency program? Yes ○ If yes, do the surgery residents rotate in pediatric cardiac surgery? Yes ○ 6. Is there a fellowship program in cardiovascular and thoracic surgery? Yes ○ 7. Do you have an ABTS post-graduated certified fellowship in pediatric Yes cardiac surgery? ○ 8. Is there a surgery resident of fellow “in house” with designated Yes responsibilities in the pediatric cardiac ICU? ○ 9. Do you utilize foreign medical graduates to provide support in your Yes pediatric cardiac program? ○ 10. Do you perform ECMO at your facility? Yes ○ a. If yes, do you have “rapid-deployment ECMO”? Yes ○

No ○ No ○ No ○ No ○ No ○ No ○ No ○ No ○ No ○

[7] Hannan EL, Racz M, Kavey R-E, Quaegebeur JM, Williams R. Pediatric cardiac surgery: the effect of hospital and surgeon volume on in-hospital mortality. Pediatrics 1998;101:963–9. [8] Title 10 of the Codes, Rules and Regulations of the State ofNew York. New York. State Department of Health, November, 2009. [9] National Quality Forum. National Quality Forum Consensus Standards for Pediatric Cardiac Surgery, Unpublished, http://www.qualityforum.org/projects/pediatriccardiac-surgery.aspx. Accessed 8/5/11. [10] Jenkins KJ, Gavreau K. Center-specific differences in mortality: preliminary analyses using the Risk-Adjustment in Congenital Heart Surgery (RACHS-1) method. J Thorac Cardiovasc Surg 2002;124:97–104.