Congenital Heart Defects in the United States

Gilboa, Suzanne M.; Devine, Owen; Kucik, James E.; Oster, Matthew E.; Riehle‐Colarusso, Tiffany; Nembhard, Wendy N.; Xu, Ping; Correa, Adolfo; Jenkins, Kathy J.; Marelli, Ariane J.

doi:10.1161/circulationaha.115.019307

Cited by 513 publications

(157 citation statements)

References 32 publications

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“…More than 2.4 million people are living with congenital heart disease (CHD) in the United States 1. For the population with adult CHD (ACHD), heart failure (HF) is now the leading cause of mortality2, 3 and accounts for almost one third of deaths 4.…”

Section: Introductionmentioning

confidence: 99%

Hospitalization Trends and Health Resource Use for Adult Congenital Heart Disease–Related Heart Failure

Burchill

Gao

Kovacs

et al. 2018

JAHA

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BackgroundThis study assessed trends in heart failure (HF) hospitalizations and health resource use in patients with adult congenital heart disease (ACHD).Methods and ResultsThe Nationwide Inpatient Sample was used to compare ACHD with non‐ACHD HF hospitalization and health resource trends. Health resource use was assessed using total hospital charges, hospital length of stay, and procedural burden. A total of 87 175±2676 ACHD‐related HF hospitalizations occurred between 1998 and 2011. During this time, ACHD HF hospitalizations increased 91% (4620±438–8809±740, P<0.0001) versus a 21% increase in non‐ACHD HF hospitalizations (P=0.003). ACHD HF hospitalization was associated with longer length of stay (ACHD HF versus non‐ACHD HF, 7.2±0.09 versus 6.8±0.02 days; P<0.0001), greater procedural burden, and higher charges ($81 332±$1650 versus $52 050±$379; P<0.0001). ACHD HF hospitalization charges increased 258% during the study period ($26 533±$1816 in 1998 versus $94 887±$8310 in 2011; P=0.0002), more than double that for non‐ACHD HF (P=0.04). Patients with ACHD HF hospitalized in high‐volume ACHD centers versus others were more likely to undergo invasive hemodynamic testing (30.2±0.6% versus 20.7±0.5%; P<0.0001) and to receive cardiac resynchronization/defibrillator devices (4.7±0.3% versus 1.8±0.2%; P<0.0001) and mechanical circulatory support (3.9±0.2% versus 2.4±0.2%; P<0.0001).Conclusions ACHD‐related HF hospitalizations have increased dramatically in recent years and are associated with disproportionately higher costs, procedural burden, and health resource use.

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Section: Introductionmentioning

confidence: 99%

Hospitalization Trends and Health Resource Use for Adult Congenital Heart Disease–Related Heart Failure

Burchill

Gao

Kovacs

et al. 2018

JAHA

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“…Adults with congenital heart disease (CHD) represent a growing subset of the population and are currently estimated at 1.4 million in the United States 1. In response, research on relevant outcomes, quality, and cost of care is now being pursued on a larger scale.…”

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confidence: 99%

Limited Accuracy of Administrative Data for the Identification and Classification of Adult Congenital Heart Disease

Khan

Ramsey

Ballard

et al. 2018

JAHA

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BackgroundAdministrative data sets utilize billing codes for research and quality assessment. Previous data suggest that such codes can accurately identify adults with congenital heart disease (CHD) in the cardiology clinic, but their use has yet to be validated in a larger population.Methods and ResultsAll administrative codes from an entire health system were queried for a single year. Adults with a CHD diagnosis code (International Classification of Diseases, Ninth Revision, (ICD‐9) codes 745–747) defined the cohort. A previously validated hierarchical algorithm was used to identify diagnoses and classify patients. All charts were reviewed to determine a gold standard diagnosis, and comparisons were made to determine accuracy. Of 2399 individuals identified, 206 had no CHD by the algorithm or were deemed to have an uncertain diagnosis after provider review. Of the remaining 2193, only 1069 had a confirmed CHD diagnosis, yielding overall accuracy of 48.7% (95% confidence interval, 47–51%). When limited to those with moderate or complex disease (n=484), accuracy was 77% (95% confidence interval, 74–81%). Among those with CHD, misclassification occurred in 23%. The discriminative ability of the hierarchical algorithm (C statistic: 0.79; 95% confidence interval, 0.77–0.80) improved further with the addition of age, encounter type, and provider (C statistic: 0.89; 95% confidence interval, 0.88–0.90).Conclusions ICD codes from an entire healthcare system were frequently erroneous in detecting and classifying CHD patients. Accuracy was higher for those with moderate or complex disease or when coupled with other data. These findings should be taken into account in future studies utilizing administrative data sets in CHD.

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“…Congenital heart disease (CHD) is the most common birth defect, with an estimated 2.4 million individuals living with CHD in the United States 1, 2. The leading CHD‐related cause of death after having congenital cardiac surgery is heart failure 3.…”

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confidence: 99%

Low Nasal NO in Congenital Heart Disease With Systemic Right Ventricle and Postcardiac Transplantation

Adams

Zahid

Khalifa

et al. 2017

JAHA

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BackgroundNO bioavailability has not been systematically examined in congenital heart disease (CHD). To assess NO in patients with CHD, we measured nasal NO (nNO) generated by the nasal epithelia, given blood NO is difficult to measure (half‐life, <2 ms). Given NO's role in hemodynamic regulation and the association of NO bioavailability with heart failure risk, we hypothesized NO levels may differ with varying severity of CHD physiologic characteristics.Methods and ResultsSix‐hundred eighteen subjects, 483 with CHD and 135 controls, had nNO measured noninvasively via the nares using American Thoracic Society/European Respiratory Society guidelines. Subjects were dichotomized as having low or normal nNO based on age‐specific cutoff values. Prevalence of low nNO was examined by various CHD physiologic feature types. Low nNO was more prevalent with CHD than controls (odds ratio, 2.28; P=0.001). A logistic regression model showed overall significance (P=0.035) for single ventricle, systemic right ventricle, ventricular dysfunction, oxygen desaturation, and heterotaxy predicting low nNO, with systemic right ventricle independently having twice the odds of low nNO (odds ratio, 2.04; P=0.014). Patients with low nNO had a higher risk of experiencing heart transplant or death (hazard ratio, 2.75; P=0.048), and heart transplant recipients (N=16) exhibited 5 times the odds of low nNO (69% versus 30%; odds ratio, 5.1; P=0.001).ConclusionsPatients with CHD have increased prevalence of low nNO, with highest odds seen with systemic right ventricle and heart transplant. Further studies are needed to investigate heart failure risks in patients with CHD with left versus right systemic ventricle physiologic characteristics and utility of low nNO for predicting heart failure risk.

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Congenital Heart Defects in the United States

Cited by 513 publications

References 32 publications

Hospitalization Trends and Health Resource Use for Adult Congenital Heart Disease–Related Heart Failure

Hospitalization Trends and Health Resource Use for Adult Congenital Heart Disease–Related Heart Failure

Limited Accuracy of Administrative Data for the Identification and Classification of Adult Congenital Heart Disease

Low Nasal NO in Congenital Heart Disease With Systemic Right Ventricle and Postcardiac Transplantation

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