Care of the Adult Congenital Heart Disease Patient in the United States: A Summary of the Current System

Abstract

With improvements in care, there has been exponential growth in the population of adult congenital heart disease (ACHD) patients. We sought to assess the availability of specialized ACHD care in the United States. We analyzed the Adult Congenital Heart Association's ACHD clinic directory for information on patient volume, provider training, and other characteristics. The information is self-reported and unverified. The ACHD directory included 72 programs in the United States. Across programs, the majority of patients (33%) seen were between 21 and 30 years old. Program directors had between 2 and 50 years (median 15) of ACHD experience and had dedicated between 10 and 100% (median 30%) of their clinical time to ACHD. There were 2,800 ACHD operations performed per year, ranging from 0 to 230 (median 28) per program. There were between 0 and 5 cardiac surgeons (median 2) involved per program. Each surgeon averaged 20 ACHD operations per year. The growing ACHD population is a largely underserved group. Few programs in the United States provide specialized care, and this care is variably conducted within pediatric and/or adult facilities. These data should serve as a stimulus to improving accessibility of services for this vulnerable population.

Characterization and Functionality of Cardiac Progenitor Cells in Congenital Heart Patients

“…The number of congenital heart patients living presently outnumbers new congenital cardiac diagnoses, occurring in 35 000 to 40 000 infants per year in the United States. 1 Whereas adult heart failure is often ischemic in origin, the origin of pediatric heart failure is more varied, including a spectrum of cardiomyopathies, congenital heart defects (CHDs), and arrhythmias. 2 A novel emerging treatment for pediatric heart failure is cellular cardiomyoplasty whereby stem cells are delivered to the dysfunctional myocardium, an approach attempted thus far only in adults.…”

Emerging Research Directions in Adult Congenital Heart Disease

“…A few studies use administrative data to evaluate this population, but available clinical and anatomic information is limited. As only a minority of ACHD patients receive care in ACHD specialty centers, little is actually known about pregnancy experience and outcomes for most adults with CHD (66). …”

Congenital Heart Defects in the United States

“…This corresponds to the typical time of transition from pediatric to adult oriented medical care during which pediatric patients can be lost to follow-up and fail to undergo a successful transition. 28,29 A long lapse in medical care may result in adverse outcomes, since adults with CHD (ACHD) must be monitored frequently for the development of complications from their structural heart defects, such as arrhythmias and pulmonary hypertension. 29,30 As of 2010, the consensus among the ACHD community was that the ACHD clinics in the US at that time were inadequate to meet the needs of this population.…”

“…28,29 A long lapse in medical care may result in adverse outcomes, since adults with CHD (ACHD) must be monitored frequently for the development of complications from their structural heart defects, such as arrhythmias and pulmonary hypertension. 29,30 As of 2010, the consensus among the ACHD community was that the ACHD clinics in the US at that time were inadequate to meet the needs of this population. 29 The treatment recommendations from the 32 nd Bethesda Conference were that adults with moderate or complex CHD should be followed in a regional ACHD center at least once or twice each year.…”

“…29,30 As of 2010, the consensus among the ACHD community was that the ACHD clinics in the US at that time were inadequate to meet the needs of this population. 29 The treatment recommendations from the 32 nd Bethesda Conference were that adults with moderate or complex CHD should be followed in a regional ACHD center at least once or twice each year. 16 Based on the 2009 estimate of nearly 860,000 adults with CHD in the US in the year 2000, and assuming that 50% of them were seen at least once each year in an ACHD regional center, it was estimated that 150 large regional centers would be required to care for the ACHD population in the US.…”