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Objectives: Elderly patients with advanced non-small lung cancer (aNSCLC) represent a high-risk patient population due to disease burden, comorbidities, and performance status, particularly after progressing on first-line therapy. Among elderly patients who receive second-line therapy, treatment related toxicities can have substantial impact on both clinical and economic outcomes. This study assessed the impact of severe adverse events (AEs) during second-line therapy on overall survival (OS) and all-cause heathcare costs in elderly with aNSCLC., Materials and Methods: Patients with aNSCLC aged ≥65 years who initiated second-line chemotherapy/targeted therapy were identified in the SEER-Medicare database (2007-2011). Fifty-seven AEs were identified by literature review and consultation with two oncologists. Severe AEs were defined as AEs that required a hospitalization and were operationalized based on AE diagnosis(es) recorded during hospitalizations. OS post-second-line initiation and healthcare costs during second-line were compared between patients with and without severe AEs., Results: Among 3967 patients initiating second-line therapy, 1624 (41%) had ≥1 severe AE, where hypertension (26%), anemia (24%), and pneumonia (23%) were most commonly reported. Patients with and without severe AEs had similar demographic and cancer characteristics at diagnosis and similar second-line treatment regimens, but patients with severe AEs had more comorbidities at second-line initiation. Median OS was lower in patients with versus without severe AEs (6 vs. 11 months). After multivariate adjustment, hazard of death was more than twice higher in patients with versus without severe AEs (adjusted hazard ratio [HR] 2.31, 95% CI 2.16-2.47). Healthcare costs were more than twice higher in patients with versus without severe AEs ($16,135 vs. $7559 per-patient-per-month)., Conclusion: Severe AEs among elderly patients with aNSCLC treated with second-line chemotherapy/targeted therapy were found to be associated with decreased OS and increased healthcare costs. Results suggest a potential link between severe AEs in second-line treated aNSCLC elderly and patient survival and economic burden to the healthcare system., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Purpose: To describe the patient characteristics, treatments, disease monitoring, and kidney function of patients with sporadic angiomyolipoma (sAML), stratified by the number and size of renal angiomyolipomas (AMLs)., Methods: Single-center retrospective analysis of patients with sAML treated from 1990 to 2015 in a dedicated clinic for inheritable tumor syndromes in a tertiary referral center from the Netherlands. Patients' first AML assessment at the clinic was defined as the index date. Patient characteristics were measured at the index date. Treatments, disease monitoring, and kidney function were measured post-index date., Results: The study sample included 53 patients followed for a total of 184.6 patient-years. At the index date, the largest AML was ≥ 3.5 cm for 26 patients and < 3.5 cm for 27 patients (including six patients with five or more AMLs of < 3.5 cm). As compared to patients with AMLs < 3.5 cm, patients with largest AML ≥ 3.5 cm had higher frequency of pre-index bleeding episodes (31 vs. 4%), pre-index hypertension (35 vs. 15%), post-index nephrectomy (19 vs. 4%), post-index embolization (8 vs. 0%), and post-index renal scans (1.14 vs. 0.74 scans/year). Kidney impairment was especially pronounced in young adults with AML ≥ 3.5 cm. On average, patients with sAML developed chronic kidney disease stage two earlier than the general Dutch population (age 42 vs. 55 years), but later than the patients with tuberous sclerosis complex (35 years)., Conclusions: Patients with sAML, especially those with larger AMLs, have high disease burden.

Background: Access to specialty care is critical for patients with advanced stage lung cancer. This study assessed access to cancer specialists and cancer treatment in a broad population of patients with advanced stage lung cancer., Materials and Methods: Two study samples were extracted from 2 claims databases and analyzed independently: patients aged ≥ 18 years with de novo diagnosis of metastatic lung cancer in the MarketScan database between 2008 and 2014 (commercially insured adult patients; n = 22,268); and patients aged ≥ 65 years in the Surveillance, Epidemiology, and End Results-Medicare database with a diagnosis of advanced non-small-cell lung cancer between 2007 and 2011 (Medicare-insured elderly patients; n = 9651). The study period spanned from 6 weeks before the first lung biopsy tied to the initial lung cancer diagnosis until the end of continuous health insurance enrollment, or data availability, or death., Results: Among the commercially insured adults (MarketScan), most patients were seen by a cancer specialist within a month of first lung biopsy (80%), 12% were never seen by a cancer specialist, and 6% did not receive cancer-directed therapy. Among the Medicare-insured elderly patients (SEER-Medicare), the proportions were 79%, 4%, and 10%, respectively. Patients seen by a cancer specialist were more likely to receive cancer-directed therapy (95% vs. 92%, P < .001 and 92% vs. 38%, P < .001, respectively)., Conclusion: Between 4% and 12% of patients with advanced stage lung cancer do not have appropriate access to cancer specialist, which appears to negatively affect access to optimal and timely treatment., (Published by Elsevier Inc.)

The prevalence rate of cancer among adult patients with congenital heart disease (CHD) in North America has not been previously described. The Quebec adult CHD database was used to determine the prevalence rate of cancer among adult patients with CHD measured as the number of adults with CHD and cancer alive in 2005 per 1,000 adults with CHD. This prevalence rate was compared with the prevalence rate of cancer in the general population of adults in Canada. Types of cancer among the CHD group were described by gender and age. Adult patients with CHD had a 1.6 to 2 times higher prevalence of cancer at 2, 5, and 10 years for both men and women. Overall, men had a greater prevalence of total cancers in all-time durations than did women. Breast, colon, and prostate cancer were the most common cancers reported in adults with CHD. In conclusion, we observed an increased prevalence of cancer among the adult CHD population of Quebec compared with the general Canadian population., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Trastuzumab reduces the risk of relapse in women with HER2-positive non-metastatic breast cancer, but little information exists on the timing of trastuzumab initiation. The study investigated the impact of delaying the initiation of adjuvant trastuzumab therapy for >6 months after the breast cancer diagnosis on time to relapse, overall survival (OS), and relapse-free survival (RFS) among patients with non-metastatic breast cancer. Adult women with non-metastatic breast cancer who initiated trastuzumab adjuvant therapy without receiving any neoadjuvant therapy were selected from the US Department of Defense health claims database from 01/2003 to 12/2012. Two study cohorts were defined based on the time from breast cancer diagnosis to trastuzumab initiation: >6 months and ≤6 months. The impact of delaying trastuzumab initiation on time to relapse, OS, and RFS was estimated using Cox regression models adjusted for potential confounders. Of 2749 women in the study sample, 79.9 % initiated adjuvant trastuzumab within ≤6 months of diagnosis and 20.1 % initiated adjuvant trastuzumab >6 months after diagnosis. After adjusting for confounders, patients who initiated trastuzumab >6 months after the breast cancer diagnosis had a higher risk of relapse, death, or relapse/death than those who initiated trastuzumab within ≤6 months of diagnosis (hazard ratios [95 % CIs]: 1.51 [1.22-1.87], 1.54 [1.12-2.12], and 1.43 [1.16-1.75]; respectively). The results of this population-based study suggest that delays of >6 months in the initiation of trastuzumab among HER2-positive non-metastatic breast cancer patients are associated with a higher risk of relapse and shorter OS and RFS.

Daclatasvir (DCV) is a potent, pangenotypic nonstructural protein 5A inhibitor with demonstrated antiviral efficacy when combined with sofosbuvir (SOF) or simeprevir (SMV) with or without ribavirin (RBV) in patients with chronic hepatitis C virus (HCV) infection. Herein, we report efficacy and safety data for DCV-based all-oral antiviral therapy in liver transplantation (LT) recipients with severe recurrent HCV. DCV at 60 mg/day was administered for up to 24 weeks as part of a compassionate use protocol. The study included 97 LT recipients with a mean age of 59.3 ± 8.2 years; 93% had genotype 1 HCV and 31% had biopsy-proven cirrhosis between the time of LT and the initiation of DCV. The mean Model for End-Stage Liver Disease (MELD) score was 13.0 ± 6.0, and the proportion with Child-Turcotte-Pugh (CTP) A/B/C was 51%/31%/12%, respectively. Mean HCV RNA at DCV initiation was 14.3 × 6 log10 IU/mL, and 37% had severe cholestatic HCV infection. Antiviral regimens were selected by the local investigator and included DCV+SOF (n = 77), DCV+SMV (n = 18), and DCV+SMV+SOF (n = 2); 35% overall received RBV. At the end of treatment (EOT) and 12 weeks after EOT, 88 (91%) and 84 (87%) patients, respectively, were HCV RNA negative or had levels <43 IU/mL. CTP and MELD scores significantly improved between DCV-based treatment initiation and last contact. Three virological breakthroughs and 2 relapses occurred in patients treated with DCV+SMV with or without RBV. None of the 8 patient deaths (6 during and 2 after therapy) were attributed to therapy. In conclusion, DCV-based all-oral antiviral therapy was well tolerated and resulted in a high sustained virological response in LT recipients with severe recurrent HCV infection. Most treated patients experienced stabilization or improvement in their clinical status., (© 2016 American Association for the Study of Liver Diseases.)

Purpose: The NSABP Trial B-31 and NCCTG Trial N9831 (B-31/N9831 trials, Romond et al. in N Engl J Med 353:1673-84, 2005. doi:10.1056/NEJMoa052122; Perez et al. in J Clin Oncol 32:3744-52, 2014. doi:10.1200/JCO.2014.55.5730) established the efficacy of adjuvant trastuzumab for patients with HER2-positive early stage breast cancer. We aimed to estimate the overall survival (OS) and relapse-free survival (RFS) of HER2-positive non-metastatic breast cancer patients treated with adjuvant trastuzumab in a clinical practice setting in the United States., Methods: Adult women initiating adjuvant trastuzumab within 1 year of breast cancer surgery were identified in the health claims database of the US Department of Defense (01/2003-12/2012). OS and RFS unadjusted rates at 4 and 6 years after the first trastuzumab treatment following the breast cancer diagnosis were estimated from Kaplan-Meier analyses., Results: The study sample included 3188 women followed for a median of 3.3 years after trastuzumab initiation and treated continuously with trastuzumab for a median of 12 months. The OS rates (95 % confidence intervals) at 4 and 6 years were 90.0 % (88.6-91.2) and 87.1 (85.3-88.6), respectively. The corresponding RFS rates were 75.8 % (74.0-77.5) and 72.7 (70.7-74.7), respectively. The OS and RFS rates at 6 years reported in the B-31/N9831 trials were 89.8 and 81.4 %, respectively., Conclusions: OS rates estimated in this study were in range with those estimated in the B-31/N9831 trials, while RFS rates were lower. However, patients in the B-31/N9831 trials were younger and possibly had fewer comorbidities than patients in the current study; these differences were not adjusted for in the crude OS and RFS analyses.

Background: The burden of low-dose ionizing radiation (LDIR) exposure from medical procedures among individuals with congenital heart disease (CHD) is unknown. In this longitudinal population-based study, we sought to determine exposure to LDIR-related cardiac imaging and therapeutic procedures in children and adults with CHD., Methods and Results: In an analysis of the Quebec CHD database, exposure to the following LDIR-related cardiac procedures was recorded: catheter-based diagnostic procedures, structural heart interventions, coronary interventions, computed tomography scans of the chest, nuclear procedures, and pacemaker/implantable cardioverter-defibrillator insertion and repair. From 1990 to 2005, there were 16 253 LDIR-exposed patients with CHD with 317 988 patient-years of available follow-up. The total number of LDIR-related procedures increased from 18.5 to 51.9 per 1000 CHD patients per year (P<0.0001). This increase was attributable to increases in rates per 1000 CHD patients in diagnostic cardiac catheterizations (11.7 to 13.7 per 1000), structural heart interventions (1.0 to 5.2 per 1000), coronary interventions (1.0 to 2.4 per 1000), pacemaker/implantable cardioverter-defibrillator insertions (1.6 to 4.4 per 1000), nuclear procedures (4.2 to 13.8 per 1000), and computed tomography scans of the chest (2.5 to 12.3 per 1000). Over time, among children with CHD, the median age at first LDIR procedure decreased from 5.0 years to 9.6 months. Severity of CHD significantly predicted extent of exposure., Conclusions: From 1990 to 2005, patients with CHD were exposed to increasing numbers of LDIR-emitting cardiac procedures. This exposure occurred at progressively younger ages. These findings provide an important perspective on longitudinal LDIR exposure in this at-risk population., (© 2015 American Heart Association, Inc.)

Background: Psoriasis's effect on diabetes onset is well documented, but its effect on course of diabetes is poorly understood., Objective: We sought to compare risks of developing microvascular and macrovascular complications between diabetic patients with and without psoriasis., Methods: Adults with 2 or more diabetes diagnoses selected from MarketScan databases (Truven Health Analytics Inc, Ann Arbor, MI) (2000-2006) were classified into 2 cohorts: 2 or more psoriasis diagnoses and without psoriasis diagnosis. Patients with psoriasis were matched using propensity score, and exactly matched using age, sex, and diabetes characteristics with patients without psoriasis. Outcomes were compared between cohorts using Cox regression models., Results: In all, 6164 diabetic patients with psoriasis (27% moderate to severe) were matched to 6164 diabetic patients without psoriasis. Patients with psoriasis were significantly more likely to develop microvascular events than patients without psoriasis overall (hazard ratio [HR] 1.14, P < .001) and by psoriasis severity (mild: HR 1.13, P = .004; moderate to severe: HR 1.16, P = .038). Risk of macrovascular events was higher for patients without psoriasis overall (HR 1.13, P = .001) and those with mild psoriasis (HR 1.15, P = .003), but not for moderate to severe cases (HR 1.10, P = .210)., Limitations: Psoriasis to diabetes association may be underestimated., Conclusion: Among diabetic patients, psoriasis is generally associated with higher rates of microvascular and macrovascular complications. Greater psoriasis severity did not increase risk of diabetic complications., (Copyright © 2015 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Objectives: Patient-centered care and patient satisfaction represent key dimensions of health care quality. This is relevant for the growing number of patients with life-long conditions. In the present study, our goal was to examine clinicians' attitudes and behavior with respect to patient satisfaction in adult congenital heart disease outpatient clinics., Methods: A 34-question survey was developed to assess adult congenital heart disease clinicians' awareness, attitudes, and behavior relative to patient satisfaction and administered in-person or online to clinicians from the largely U.S.-based Adult Congenital Heart Association's database of adult congenital heart disease health care providers., Results: Overall, 267 questionnaires were filled out: 108 were collected in person (79% response rate) and 159 online (17.5% response rate). Responses were received from physicians (161); nurses (73); physician assistants (20); and others (13). Although 85% of clinicians believed it was important to inquire about patient satisfaction, only 28% reported routinely inquiring about this dimension of care. Only 34% claimed they had adequate training to cope with varying levels of patient satisfaction, 44% stated that their department utilized patient satisfaction surveys, and 37% received feedback from the hospital management in the preceding 12 months. In multivariate analyses, clinicians that received feedback from the hospital management and had adequate training were more likely to inquire about patient satisfaction., Conclusion: Although patient satisfaction is perceived as an important dimension of quality care by adult congenital heart disease clinicians, most of them reported insufficient institutional support to achieve this. Our findings suggest that clinicians would benefit from health care organizations engaging them in the delivery of this dimension of health care quality., (© 2014 Wiley Periodicals, Inc.)

Objective: The Universal Child Care Benefit (UCCB) is a 2006 Canadian federal policy of income supplementation that provides parents with $100 monthly in Canadian dollars for each child aged <6years. The study main objective was to estimate the causal effect of UCCB on self-reported food insecurity overall and in vulnerable subgroups., Method: The Canadian Community Health Survey (2001-2009) was used to conduct a difference-in-differences (DID) regression analysis for the effect of the UCCB on self-reported food insecurity. Respondents were ages ≥12 in families with at least one child aged <6years (UCCB-eligible, n=22,737) or a child aged 6-11 but no child <6years (control group, n=17,664)., Results: Over the study period 16.3% of respondents experienced some level of food insecurity. Overall, UCCB reduced the proportion of respondents reporting food insecurity by 2.4% (95% CI: -4.0%, -0.9%). There was a significantly stronger impact on food insecurity for respondents from households with yearly income below the population median (-4.3%, 95% CI: -7.2%, -1.4%) and respondents from single parent families (-5.4%, 95% CI: -10.3%, -0.6%)., Conclusion: We found that a relatively small monthly income supplementation results in a significant reduction in food insecurity at the population level, with larger effects in vulnerable groups., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: The use of bevacizumab in advanced non-squamous non-small cell lung cancer (NSCLC) is controversial among elderly patients. This study aimed to compare overall survival for Medicare patients diagnosed with NSCLC and treated with either first-line bevacizumab-carboplatin-paclitaxel (BCP) or carboplatin-paclitaxel (CP)., Methods: Patients ≥ 65 years old, first diagnosed with non-squamous NSCLC stage IIIB/IV between 2006 and 2009, and treated with either first-line BCP or CP, were selected from the SEER-Medicare database that links cancer registry and US Medicare claims data. Kaplan-Meier estimates were used to evaluate survival. Multivariable Cox proportional hazards models were used to compare the effect of BCP versus CP on the hazard of death. Age-stratified analyses were conducted for patients aged 65-74 and ≥ 75 years., Results: Of 1706 patients in the study sample, 592 (34.7%) received BCP and 1114 (65.3%) received CP; 692 (40.6%) were ≥ 75 years. Adjusted median survival time in the BCP versus CP cohorts was 10.5 versus 8.5 months (p = 0.008). The difference in median survival favoring the BCP cohort was statistically significant for both patients aged ≥ 75 years (2.8 months, p = 0.019), and patients aged 65-74 years (1.5 months, p = 0.018). The adjusted hazard of death did not differ between the cohorts (HR: 0.96, 95% CI: 0.86-1.06); however, during the first year of follow-up, when most deaths (>60%) occurred, the hazard of death was 18% lower for the BCP cohort (HR: 0.82, 95% CI: 0.71-0.94). BCP patients also had 18% fewer hospital admissions than CP patients (adjusted incidence rate ratio (IRR): 0.82, 95% CI: 0.72-0.94) and 23% fewer inpatient days (IRR: 0.77, 95% CI: 0.65-0.91)., Conclusions: In this retrospective analysis of Medicare patients in the SEER database, first-line therapy with BCP was associated with longer survival and fewer hospitalizations than CP., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Objective: Chronic myeloid leukemia (CML) treatment relies on tyrosine kinase inhibitors (TKIs), but their use can be associated with low-grade adverse events (AEs). This analysis aimed to identify the low-grade AEs which significantly impact the Health Related Quality of Life (HRQoL) of CML patients in chronic phase (CP) and to compare the incidence of such AEs among nilotinib- and imatinib-treated patients., Research Design and Methods: Data from the 48 month ENESTnd trial were used (N = 593 patients). HRQoL was assessed using generic (SF-36) and leukemia-specific (FACT-Leu) HRQoL surveys. AEs were categorized into 26 system organ classes., Results: In the adjusted regression model, five low-grade AE categories - gastrointestinal disorders, blood and lymphatic system disorders, general disorders and administration site conditions, musculoskeletal disorders, and psychiatric disorders - significantly impaired at least one HRQoL score. The incidence rate of these five AE categories was either significantly lower for nilotinib than imatinib or not different between the two drugs. The AE categories with lower incidence for both nilotinib 300 mg BID and 400 mg BID versus imatinib 400 mg daily were gastrointestinal, blood and lymphatic system, and musculoskeletal; nilotinib 300 mg BID had lower incidence than imatinib for general disorders., Limitations: Low-grade AEs were grouped and analyzed by system organ class category, so the effect of some rare individual AEs on HRQoL may have been missed., Conclusions: The impact of low-grade AEs on HRQoL should be taken into account, along with other factors, when selecting the optimal treatment for patients newly diagnosed with CML-CP.

Objective: To describe the distribution of costs and to identify the drivers of high costs among adult patients with type 2 diabetes mellitus (T2DM) receiving oral hypoglycemic agents., Methods: T2DM patients using oral hypoglycemic agents and having HbA1c test data were identified from the Truven MarketScan databases of Commercial and Medicare Supplemental insurance claims (2004-2010). All-cause and diabetes-related annual direct healthcare costs were measured and reported by cost components. The 25% most costly patients in the study sample were defined as high-cost patients. Drivers of high costs were identified in multivariate logistic regressions., Results: Total 1-year all-cause costs for the 4104 study patients were $55,599,311 (mean cost per patient = $13,548). Diabetes-related costs accounted for 33.8% of all-cause costs (mean cost per patient = $4583). Medical service costs accounted for the majority of all-cause and diabetes-related total costs (63.7% and 59.5%, respectively), with a minority of patients incurring >80% of these costs (23.5% and 14.7%, respectively). Within the medical claims, inpatient admission for diabetes-complications was the strongest cost driver for both all-cause (OR = 13.5, 95% CI = 8.1-23.6) and diabetes-related costs (OR = 9.7, 95% CI = 6.3-15.1), with macrovascular complications accounting for most inpatient admissions. Other cost drivers included heavier hypoglycemic agent use, diabetes complications, and chronic diseases., Limitations: The study reports a conservative estimate for the relative share of diabetes-related costs relative to total cost. The findings of this study apply mainly to T2DM patients under 65 years of age., Conclusions: Among the T2DM patients receiving oral hypoglycemic agents, 23.5% of patients incurred 80% of the all-cause healthcare costs, with these costs being driven by inpatient admissions, complications of diabetes, and chronic diseases. Interventions targeting inpatient admissions and/or complications of diabetes may contribute to the decrease of the diabetes economic burden.

Background: Our objective was to obtain contemporary lifetime estimates of congenital heart disease (CHD) prevalence using population-based data sources up to year 2010., Methods and Results: The Quebec CHD database contains 28 years of longitudinal data on all individuals with CHD from 1983 to 2010. Severe CHD was defined as tetralogy of Fallot, truncus arteriosus, transposition complexes, endocardial cushion defects, and univentricular hearts. We used latent class bayesian models combining case definitions from physician claims, hospitalization, and surgical data to obtain point and interval prevalence estimates of CHD in the first year of life, in children (<18 years of age) and in adults. We identified 107 559 CHD patients from 1983 to 2010. Prevalence of CHD in the first year of life was 8.21 per 1000 live births (95% confidence interval, 7.47-9.02) from 1998 to 2005. In 2010, overall prevalence of CHD was 13.11 per 1000 (95% confidence interval, 12.43-13.81) in children and 6.12 per 1000 (95% confidence interval, 5.69-6.57) in adults. CHD prevalence increased by 11% in children and 57% in adults from 2000 to 2010. Prevalence in the severe CHD subgroup increased by 19% (95% confidence interval, 17%-21%) in children and 55% (51%-62%) in adults. By 2010, adults accounted for 66% of the entire CHD population., Conclusions: With an increase of >50% in CHD prevalence since 2000, by 2010 adults accounted for two thirds of patients with severe and other forms of CHD in the general population. Our findings should inform allocation of resources and the planning of workforce needs for the predominantly adult CHD population., (© 2014 American Heart Association, Inc.)

Background: Clinical guidelines recommend specialized care for adult congenital heart disease (ACHD) patients. In reality, few patients receive such dedicated care. We sought to examine the impact of specialized care on ACHD patient mortality., Methods and Results: We examined referral rates to specialized ACHD centers and ACHD patient mortality rates between 1990 and 2005 in the population-based Quebec Congenital Heart Disease database (n=71 467). This period covers several years before and after the publication of guidelines endorsing specialized care for ACHD patients. A time-series design, based on Joinpoint and Poisson regression analyses, was used to assess the changes in annual referral and patient mortality rates. The association between specialized ACHD care and all-cause mortality was assessed in both case-control and cohort studies. The time-series analysis demonstrated a significant increase in referral rates to specialized ACHD centers in 1997 (rate ratio, +7.4%; 95% confidence interval [CI], +6.6% to +8.2%). In parallel, a significant reduction in expected ACHD patient mortality was observed after year 2000 (rate ratio, -5.0%;95% CI, -10.8% to -0.8%). In exploratory post hoc cohort and case-control analyses, specialized ACHD care was independently associated with reduced mortality (hazard ratio, 0.78; 95% CI, 0.65-0.94) and a reduced odds of death (adjusted odds ratio, 0.82; 95% CI, 0.08-0.97), respectively. This effect was predominantly driven by patients with severe congenital heart disease (hazard ratio, 0.38; 95% CI, 0.22-0.67)., Conclusions: A significant increase in referrals to specialized ACHD centers followed the introduction of the clinical guidelines. Moreover, referral to specialized ACHD care was independently associated with a significant mortality reduction. Our findings support a model of specialized care for all ACHD patients.

Background: The most common congenital anomaly in adults is secundum, which can be closed using a surgical or transcatheter approach. Despite the growing use of transcatheter ASD closure, few studies have examined the cost-effectiveness of this strategy. We sought to compare the long-term cost effectiveness of transcatheter and surgical closure of secundum in adults., Methods: A decision-analytic model was used with all clinical outcome parameter estimates obtained from the province-wide Québec Congenital Heart Disease Database. Costs were obtained from a single academic centre (Canadian dollars). A cost-effectiveness analysis using a discrete event Monte Carlo simulation model from the perspective of a single third party payer and multiple sensitivity analyses were performed. Patients were followed for a maximum of 5 years after ASD closure., Results: Between l998 and 2005, we identified 718 adults (n=335 transcatheter; n=383 surgical) who underwent ASD closure in Quebec. The 5-year cost of surgical closure was $15,304 SD $4581 versus $11,060 SD $5169 for the transcatheter alternative. At 5 years, transcatheter closure was marginally more effective than surgery (4.683 SD 0.379 life-years versus 4.618 SD 0.638 life-years). Probabilistic sensitivity analyses demonstrated that transcatheter ASD closure was a dominant strategy with an 80% probability of cost savings and equal or greater efficacy compared to surgical treatment., Conclusion: Although definitive conclusions are limited given the observational nature of the primary data sources, transcatheter ASD closure appeared to be a cost-effective strategy associated with slightly improved clinical outcomes and reduced costs compared to surgical closure at 5-years follow-up., (Copyright © 2013. Published by Elsevier Ireland Ltd.)

Background: The American Heart Association guidelines for prevention of infective endocarditis (IE) in 2007 reduced the groups of congenital heart disease (CHD) patients for whom antibiotic prophylaxis was indicated. The evidence base in CHD patients is limited. We sought to determine the risk of IE in children with CHD., Methods and Results: We performed a population-based analysis to determine the cumulative incidence and predictors of IE in children (0-18 years) with CHD by the use of the Quebec CHD Database from 1988 to 2010. In 47 518 children with CHD followed for 458 109 patient-years, 185 cases of IE were observed. Cumulative incidence of IE was estimated in the subset of 34 279 children with CHD followed since birth, in whom the risk of IE up to 18 years of age was 6.1/1000 children (95% confidence interval, 5.0-7.5). In a nested case-control analysis, the following CHD lesions were at highest risk of IE in comparison with atrial septal defects (adjusted rate ratio, 95% confidence interval): cyanotic CHD (6.44, 3.95-10.50), endocardial cushion defects (5.47, 2.89-10.36), and left-sided lesions (1.88, 1.01-3.49). Cardiac surgery within 6 months (5.34, 2.49-11.43) and an age of <3 years (3.53, 2.51-4.96; reference, ages 6-18) also conferred an elevated risk of IE., Conclusions: In a large population-based cohort of children with CHD, we documented the cumulative incidence of IE and associated factors. These findings help identify groups of patients who are at the highest risk of developing IE.

Objectives: The study objectives were to analyze sex differences in hospital mortality of adult patients with congenital heart disease (CHD) and to determine the impact of health services associated with pregnancy on outcomes in women., Background: The determinants of sex differences in the demographic distribution of CHD are poorly understood., Methods: The Quebec CHD database and the Dutch CONCOR (CONgenital CORvitia) registry were used to identify patients with CHD aged 18 to 65 years who were hospitalized between 1996 and 2005. Regression analyses were used to compare 30-day in-hospital mortality in men versus women and in women aged 18 to 45 years with versus without a pregnancy history, after adjustment for age, CHD severity, comorbidities, and admission diagnosis., Results: Of 39,776 patients followed for 259,741 patient years, 19,099 patients (48%) had 54,195 admissions (62% among women). In those aged 18 to 45 years, 30-day in-hospital mortality was higher in men compared with women with nonpregnancy admissions (adjusted rate ratio: 1.36; 95% confidence interval: 1.02 to 1.81). The adjusted rate ratio for 30-day in-hospital mortality in women with a pregnancy history compared with those without was 0.49 (95% confidence interval: 0.24 to 0.99). A history of pregnancy was not associated with an overall increase in medical encounters., Conclusions: We demonstrated a protective effect of sex on in-hospital mortality in women with CHD of reproductive age that did not correlate with increased medical surveillance. Future studies need to explore other mechanisms to account for our observations. Understanding the determinants of the sex distribution of adults with CHD is important for our ability to predict demographic changes in the population with CHD., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Introduction: This study compared progression, progression-free survival (PFS), overall survival (OS), and treatment changes among chronic myelogenous leukemia patients in chronic phase (CML-CP) receiving nilotinib or dasatinib as second-line therapy., Patients and Methods: Information on CML-CP patients switched from imatinib to nilotinib or dasatinib as second-line therapy was collected retrospectively from 122 US hematologists and oncologists through an online medical chart review. Progression, PFS, and OS were compared using multivariate Cox proportional hazard models, and treatment changes using chi-square tests., Results: Of 597 imatinib resistant or intolerant patients, 301 initiated nilotinib and 296 dasatinib as second-line therapy. Nilotinib was associated with a lower risk of progression (hazard ratio [HR] = 0.27; p = 0.021) and longer PFS (HR = 0.48; p = 0.030) than dasatinib, with a median follow-up time of 11 months for nilotinib and 10 months for dasatinib. Nilotinib patients had a lower estimated hazard of mortality than dasatinib patients, though not statistically significant (HR = 0.46; p = 0.067). When treatment changes were classified by the physicians' justifications, fewer nilotinib patients had treatment changes due to ineffectiveness (2.0% vs. 5.1%, p = 0.041) or drug holidays due to intolerance (0.0% vs. 1.7%, p = 0.024) than dasatinib patients., Conclusions: Among CML-CP patients in this retrospective chart review who switched from imatinib to either nilotinib or dasatinib, nilotinib was associated with a significantly lower risk of progression and longer PFS than dasatinib. Nilotinib patients were also less likely than dasatinib patients to subsequently have treatment changes due to ineffectiveness or drug holidays due to intolerance. These findings could be subject to unobserved confounders.

Objectives: The purpose of this study was to assess the comparative effectiveness and long-term safety of transcatheter versus surgical closure of secundum atrial septal defects (ASD) in adults., Background: Transcatheter ASD closure has largely replaced surgery in most industrialized countries, but long-term data comparing the 2 techniques are limited., Methods: We performed a retrospective population-based cohort study of all patients, ages 18 to 75 years, who had surgical or transcatheter ASD closure in Québec, Canada's second-largest province, using provincial administrative databases. Primary outcomes were long-term (5-year) reintervention and all-cause mortality. Secondary outcomes were short-term (1-year) onset of congestive heart failure, stroke, or transient ischemic attack, and markers of health service use., Results: Of the 718 ASD closures performed between 1988 and 2005, 383 were surgical and 335 were transcatheter. The long-term reintervention rate was higher in patients with transcatheter ASD closure (7.9% vs. 0.3% at 5 years, p = 0.0038), but the majority of these reinterventions occurred in the first year. Long-term mortality with the transcatheter technique was not inferior to surgical ASD closure (5.3% vs. 6.3% at 5 years, p = 1.00). Secondary outcomes were similar in the 2 groups., Conclusions: Transcatheter ASD closure is associated with a higher long-term reintervention rate and long-term mortality that is not inferior to surgery. Overall, these data support the current practice of using transcatheter ASD closure in the majority of eligible patients and support the decision to intervene on ASD with significant shunts before symptoms become evident., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Controversy continues concerning the choice of rhythm control vs rate control treatment strategies for atrial fibrillation (AF). A recent clinical trial showed no difference in 5-year mortality between the 2 treatments. We aimed to determine whether the 2 strategies have similar effectiveness when applied to a general population of patients with AF with longer follow-up., Methods: We used population-based administrative databases from Quebec, Canada, from 1999 to 2007 to select patients 66 years or older hospitalized with an AF diagnosis who did not have AF-related drug prescriptions in the year before the admission but received a prescription within 7 days of discharge. Patients were followed until death or administrative censoring. Mortality was analyzed by multivariable Cox regression., Results: Among 26,130 patients followed for a mean (SD) period of 3.1 years (2.3 years), there were 13,237 deaths (49.5%). After adjusting for covariates, we found that the effect of rhythm vs rate control drugs changed over time: after a small increase in mortality for patients treated with rhythm control in the 6 months following treatment initiation (hazard ratio [HR], 1.07; 95% CI, 1.01-1.14), the mortality was similar between the 2 groups until year 4 but decreased steadily in the rhythm control group after year 5 (HR, 0.89; 95% CI, 0.81-0.96; and HR, 0.77; 95% CI, 0.62-0.95, after 5 and 8 years, respectively)., Conclusions: In this population-based sample of patients with AF, we found little difference in mortality within 4 years of treatment initiation between patients with AF initiating rhythm control therapy vs those initiating rate control therapy. However, rhythm control therapy seems to be superior in the long-term.

Background: Aortic coarctation (CoA) is reported to predispose to coronary artery disease (CAD). However, our clinical observations do not support this premise. Our objectives were to describe the prevalence of CAD among adults with CoA and to determine whether CoA is an independent predictor of CAD or premature CAD., Methods and Results: The study population was derived from the Quebec Congenital Heart Disease Database. We compared patients with CoA and those with a ventricular septal defect, who are not known to be at increased risk of CAD. The prevalence of CAD in patients with CoA compared with those with ventricular septal defect was determined. We then used a nested case-control design to determine whether CoA independently predicted for the development of CAD. Of 756 patients with CoA who were alive in 2005, 37 had a history of CAD compared with 224 of 6481 patients with ventricular septal defect (4.9% versus 3.5%; P=0.04). Male sex (odds ratio [OR], 2.13; 95% confidence interval [CI], 1.62-2.80), hypertension (OR, 1.95; 95% CI, 1.44-2.64), diabetes mellitus (OR, 1.68; 95% CI, 1.09-2.58), age (OR per 10-year increase, 2.28; 95% CI, 2.09-2.48), and hyperlipidemia (OR, 11.58; 95% CI, 5.75-23.3) all independently predicted for the development of CAD. CoA did not independently predict for the development of CAD (OR, 1.04; 95% CI, 0.68-1.57) or premature CAD (OR for CoA versus ventricular septal defect, 1.44; 95% CI, 0.79-2.64) after adjustment for other factors., Conclusions: Although traditional cardiovascular risk factors independently predicted for the development of CAD, the diagnosis of CoA alone did not. Our findings suggest that cardiovascular outcomes of these patients may be improved with tight risk factor control.

Objective: The instrumental variable (IV) method can remove bias because of unobserved confounding, but it is unclear to what extent the choice of the IV may affect the results. We compared the estimates obtained with different provider-based IVs in a real-life observational comparative drug effectiveness study., Study Design and Setting: We assessed the effectiveness of rhythm vs. rate control treatment in reducing 5-years mortality in a population-based cohort of patients with atrial fibrillation. We compared the IV treatment effect estimates obtained from two-stage least square regression models using nine alternative provider-based IVs defined at either hospital or physician level., Results: All nine IVs reduced the covariate imbalance between the treatment groups. Yet, there were large variations in both the point estimates and the width of the confidence intervals obtained with alternative IVs. Relative to the physician-based IVs, the hospital-based IVs were stronger, had smaller variance, and produced less extreme point estimates., Conclusions: The IV estimates of treatment effect may vary considerably depending on the IV definition. Choosing the strongest IV could reduce the variance of the IV estimates., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Instrumental variable (IV) methods based on the physician's prescribing preference may remove bias due to unobserved confounding in pharmacoepidemiologic studies. However, IV estimates, originally defined as the treatment prescribed for a single previous patient of a given physician, show important variance inflation. The authors proposed and validated in simulations a new method to reduce the variance of IV estimates even when physicians' preferences change over time. First, a potential "change-time," after which the physician's preference has changed, was estimated for each physician. Next, all patients of a given physician were divided into 2 homogeneous subsets: those treated before the change-time versus those treated after the change-time. The new IV was defined as the proportion of all previous patients in a corresponding homogeneous subset who were prescribed a specific drug. In simulations, all alternative IV estimators avoided strong bias of the conventional estimates. The change-time method reduced the standard deviation of the estimates by approximately 30% relative to the original previous patient-based IV. In an empirical example, the proposed IV correlated better with the actual treatment and yielded smaller standard errors than alternative IV estimators. Therefore, the new method improved the overall accuracy of IV estimates in studies with unobserved confounding and time-varying prescribing preferences.

Background: The congenital heart disease population is aging. We hypothesized that changes in rates of congenital, valvular, and noncongenital surgical operations in congenital heart patients varied with age and disease severity over the last two decades., Methods: We performed time trend analysis using a Quebec congenital heart disease database constructed from administrative data. We included congenital heart patients of all ages having cardiac surgical operations. Heart lesions were classified as "severe" and "other." Cardiac surgical operations were grouped as congenital, valvular (including aortic), and noncongenital (arrhythmia surgery, coronary artery bypass grafting, and cardiac transplants). An adapted Aristotle score was developed to classify procedures based on surgical risk. Yearly surgical rates were measured as surgical operations per 1,000 person-years and analyzed over time using Poisson regression models stratified by age, lesion severity, and cardiac surgery category., Results: From 1988 to 2005 we followed 71,979 patients for 1,009,430 person-years. We identified 17,444 cardiac surgical operations. There was a 31% increase in volumes and a 5% increase in surgical rates over time. In children, congenital surgical operations remained constant, accounting for 80% of all surgical operations. In adults, valvular operations were the most common type of surgical operations, increasing from 42% to 63% of all procedures over time. Rates of valvular operations increased significantly in all adult subgroups and in children with severe lesions., Conclusions: The need for valvular interventions has increased in the last two decades in congenital heart disease patients. These findings should be taken into account when allocating resources that will optimize outcomes for this growing population., (Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to characterize temporal trends in all-cause mortality in patients with congenital heart disease (CHD)., Background: Historically, most deaths in patients with CHD occurred in early childhood. Notable advances have since been achieved that may impact on mortality trends., Methods: We conducted a population-based cohort study of patients with CHD in Quebec, Canada, from July 1987 to June 2005. A total of 8,561 deaths occurred in 71,686 patients with CHD followed for 982,363 patient-years., Results: The proportion of infant and childhood deaths markedly declined from 1987 to 2005, with a reduction in mortality that exceeded that of the general population. Distribution of age at death transitioned from a bimodal to unimodal, albeit skewed, pattern, more closely approximating the general population. Overall, mortality decreased by 31% (mortality rate ratio: 0.69, 95% confidence interval [CI]: 0.61 to 0.79) in the last (2002 to 2005) relative to the first (1987 to 1990) period of observation. Mortality rates decreased in all age groups below 65 years, with the largest reduction in infants (mortality rate ratio: 0.23, 95% CI: 0.12 to 0.47). In adults 18 to 64 years, the mortality reduction (mortality rate ratio: 0.84, 95% CI: 0.73 to 0.97) paralleled the general population. Gains in survival were mostly driven by reduced mortality in severe forms of CHD, particularly in children (mortality rate ratio: 0.33, 95% CI: 0.19 to 0.60), and were consistent across most subtypes., Conclusions: Deaths in CHD have shifted away from infants and towards adults, with a steady increase in age at death and decreasing mortality., (Copyright © 2010 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate whether a 12-week home-based exercise program is more effective than usual care for alleviating depressive symptomology in the postpartum., Methods: Eighty-eight women experiencing postpartum depressed mood were randomly assigned to a 12-week home-based exercise program or usual care. Outcomes assessed immediately post-treatment and 3-months post-treatment were the Hamilton Rating Scale for Depression (HAM-D) and Edinburgh Postnatal Depression Scale (EPDS)., Results: In the intention-to-treat analysis, the effect of the intervention on EPDS did not change from 3 to 6 months evaluations, but was modified by the baseline EPDS score, with subjects with greater depression at baseline (EPDS > 13) in the intervention group having a significantly lower postbaseline EPDS score compared with the usual care group (mean difference 4.06 points, 95%CI 1.51-6.61, p < 0.001). After adjusting for baseline HAM-D, subjects in the intervention group had a significantly lower HAM-D score at post-treatment compared with subjects in the usual care group (mean difference 1.83 points, 95%CI 0.24-3.41, p = 0.02). The difference in HAM-D became non-significant at 3-months post-treatment., Conclusions: Home-based exercise is a feasible nonpharmacological intervention with the potential to alleviate postpartum depressive symptoms, especially in women with higher initial depressed mood scores as measured by the EPDS. These findings may guide the design of future exercise clinical trials with postpartum depressed women.

Purpose: Instrumental variables (IV) methodology removes bias due to unobserved confounding by replacing in the analysis the treatment with another variable--the instrument--that is well correlated with the treatment and independent of confounders. Recently, physician drug preference, operationalized as the treatment prescribed to the previous patient of the same physician, was proposed as an instrument in database studies comparing two competing drugs. We assessed, in simulations, how the performance of the IV estimates depends on the strength of this instrument., Methods: The 'physician preference' instrument correlates well with the treatment only if physician preferences affect the treatment received by a large fraction of patients. Yet, often there is a subgroup of patients whose treatment cannot be affected by physician's preferences. The larger this subgroup is, the weaker the instrument. We investigated the impact of weakening this instrument on the performance of IV estimates, by comparing risk difference estimates from the conventional and IV analyses in the presence of an unobserved confounder, for both continuous and binary outcomes., Results: The IV estimates were uniformly less biased than the conventional estimates, but had higher variance. Accordingly, the bias-variance trade-off favors the IV estimates only when physician' preference is a strong instrument. Still, the coverage rate of the 95%CI for the IV estimates was very close to the nominal 95%, while it was consistently lower for the conventional estimates., Conclusion: Researchers should consider using the 'physician preference' instrument when comparing two competing drugs, but should be aware of the underlying assumptions., ((c) 2009 John Wiley & Sons, Ltd.)

Guidelines published in 2001 recommended 1 regional adult congenital heart disease (ACHD) center per 3 to 10 million population. Our objective was to determine if published guidelines on the numbers of regional ACHD centers are sufficient to meet the needs of adults with congenital heart disease in the general population. Population data were examined to evaluate the recommendations for the number of regional centers. We extrapolated a population prevalence of 4.09 per 1,000 adults corresponding to 847,896 and 87,375 patients with ACHD in the United States and Canada, respectively. We reviewed the information currently available on the numbers of ACHD facilities of any kind indexed to continental populations. We examined the distribution of disease and health services in pediatric and adult populations and examined the evidence for pressure points during the transition process. Published data on 6 of the largest regional ACHD centers were used to model regional center care. We reviewed determinants and recommendations for follow-up in regional centers. We explore 3 scenarios of referral patterns to regional centers, examining their impact of the number of centers required per country population. In conclusion, we demonstrate that 1 regional ACHD center for a population of 2.0 million adults appears to be closer to what is required for improving access to specialized care for patients with ACHD in the United States and Canada.

Objective: To describe treatment adherence to a multimodal integrated program for patients with fibromyalgia (FM), identify predictors of adherence to treatment recommendations, and examine the relationship between adherence and patient outcomes., Methods: Sixty-three patients with FM were followed while participating in a 3-month outpatient program including physiotherapy, occupational therapy, nursing, and cognitive-behavior therapy. Patients completed a battery of psychosocial questionnaires pre- and post-treatment. At the end of each month of the treatment, patients completed 2 adherence questionnaires (for general and specific adherence) and 1 questionnaire about barriers to adherence to treatment. Generalized estimating equations extension of multivariable linear regression analyses for repeated measures examined predictors of general and specific adherence. Conventional linear regression analyses examined the relationships of general adherence with post-treatment FM disability and pain intensity., Results: In general, adherence to treatment recommendations was good (mean general adherence score of 62 points, on a 0 to 100 scale), with no significant changes in mean level of general or specific adherence over the 3-month period. The main predictor for both general and specific adherence was barriers to adherence to treatment. Increased general adherence was significantly associated with lower pain at post-treatment., Conclusion: The items described in the questionnaire for barriers to treatment are the main problem when it comes to adhering to a multimodal treatment program for FM. Healthcare professionals are advised to discuss these barriers directly with patients and assist in overcoming them.

Background: Little is known about the factors that predispose children with congenital heart disease (CHD) to readmission soon after hospital discharge. We sought to determine rates and risk factors for hospital readmission within 1 month among children with CHD., Methods: Data were obtained from administrative databases that record all hospitalizations within the province of Quebec. We included children 0 to 17 years with CHD who were hospitalized with a cardiac diagnosis (an "index hospitalization") between 1990 and 2005. Cox proportional hazards analysis was used to identify predictors of readmission., Results: Among 3675 hospitalizations, there were 518 readmissions (15%) within 31 days. Median time to readmission was 12 days (interquartile range 5-19 days). Readmissions occurred in the same hospital as the index hospitalization 86% of the time. The most common diagnoses at readmission were cardiac (59%) and respiratory illnesses (12%). Independent predictors of readmission were severe CHD lesion, younger age, Friday or Saturday discharge, having an emergency department visit within the preceding 6 months, length of index hospitalization > 14 days, and multiple (> or = 4) diagnoses (either cardiac diagnoses or comorbid conditions). Having an invasive procedure during the index hospitalization reduced the likelihood of readmission., Conclusions: Readmission occurred after 1 in 7 hospitalizations. Infants and patients with severe CHD lesions were among those more likely to be readmitted. The risk of readmission may be reduced by avoiding hospital discharge at the beginning of a weekend. Further studies are needed to determine if targeted interventions will reduce readmission rates in high-risk patients.

Background: People aged 65 years or more represent a growing group of emergency department users. We investigated whether characteristics of primary care (accessibility and continuity) are associated with emergency department use by elderly people in both urban and rural areas., Methods: We conducted a cross-sectional study using information for a random sample of 95,173 people aged 65 years or more drawn from provincial administrative databases in Quebec for 2000 and 2001. We obtained data on the patients' age, sex, comorbidity, rate of emergency department use (number of days on which a visit was made to an emergency department per 1000 days at risk [i.e., alive and not in hospital] during the 2-year study period), use of hospital and ambulatory physician services, residence (urban v. rural), socioeconomic status, access (physician: population ratio, presence of primary physician) and continuity of primary care., Results: After adjusting for age, sex and comorbidity, we found that an increased rate of emergency department use was associated with lack of a primary physician (adjusted rate ratio [RR] 1.45, 95% confidence interval [CI] 1.41-1.49) and low or medium (v. high) levels of continuity of care with a primary physician (adjusted RR 1.46, 95% CI 1.44-1.48, and 1.27, 95% CI 1.25-1.29, respectively). Other significant predictors of increased use of emergency department services were residence in a rural area, low socioeconomic status and residence in a region with a higher physician:population ratio. Among the patients who had a primary physician, continuity of care had a stronger protective effect in urban than in rural areas., Interpretation: Having a primary physician and greater continuity of care with this physician are factors associated with decreased emergency department use by elderly people, particularly those living in urban areas.

Objective: To evaluate alternative approaches to correct for bias due to inaccurate diagnostic criteria in database studies of associations., Study Design and Settings: A simulation study of a hypothetical cohort of 10,000 subjects selected based on database-derived diagnostic criteria with positive predictive value (PPV) of either 53% or 80%. Analyses focus on the putative association between a drug and the time to a negative outcome. The association is confounded for "false positive" subjects, where the drug acts as a marker for unobserved frailty. First, we estimate the conventional multivariable Cox's Model 1. We then assume having in-depth evaluation of a fraction of subjects, which permits estimating the probabilities of having the disease for all subjects in the cohort. Alternative correction methods use the estimated probability as a confounder (Model 2), a modifier of the drug effect (Model 3), or an importance weight (Model 4)., Results: With a PPV of 53%, Models 1 and 2 induced about 50% underestimation bias for the drug effect. Interaction-based Model 3 yielded the least biased estimates (25% bias), whereas weighting by probability (Model 4) resulted in slightly more biased (33%), but more stable estimates., Conclusion: Proposed methods help reducing bias due to sample contamination.

The number of adults with congenital heart disease (CHD) is increasing. However, rates of health care resource utilization in this population are unknown. The objectives of this study were to describe the use of general health care resources in adults with CHD and to examine the impact of CHD severity on resource utilization. The study consisted of adults alive in 1996 who had > or = 1 diagnosis of a CHD lesion conforming to the International Classification of Disease, Ninth Revision, in the physician's claims database of the province of Quebec from 1983 to 2000. From 1996 to 2000, rates of health care utilization were measured. The impact of the severity of CHD on the use of health care resources was determined using multivariate models to adjust for age, gender, Charlson co-morbidity score, and duration of follow-up. The study population consisted of 22,096 adults with CHD (42% men). From 1996 to 2000, 87% received outpatient care from specialists, 68% visited emergency rooms, 51% were hospitalized, and 16% were admitted to critical care units. Patients with severe CHD had higher adjusted rates of outpatient cardiologist care (rate ratio [RR] 2.24, 95% confidence interval [CI] 2.06 to 2.45), emergency department utilization (RR 1.09, 95% CI 1.03 to 1.17), hospitalization (RR 1.30, 95% CI 1.19 to 1.43), and days in critical care (RR 2.12, 95% CI 1.80 to 2.50) than patients with other congenital cardiac lesions. Hospitalization rates were higher than in the general Quebec adult population (RR 2.08, 95% CI 2.00 to 2.17). In conclusion, adults with CHD have high rates of health care resource utilization, particularly those with severe lesions. Appropriate resource allocation is required to serve this growing population.