Overview of Adult Congenital Heart Disease
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Overview of Adult CHD

Congenital Heart Disease is the #1 birth defect in the United States, affecting one out of 120 babies born. The past 60 years have brought dramatic surgical and medical advances allowing more than 90% of these children to live into adulthood.

The result of these amazing medical triumphs is that for the first time in history there are more adults than children living with congenital heart disease (CHD) in the United States. These numbers are expected to keep growing by about 5% each year. It is estimated that there are approximately 1 million adults and 800,000 children in the U. S. with CHD and around 30,000 new diagnoses each year.

The health concerns of patients with adult congenital heart disease may be grouped into the following categories:

1. Patients with Congenital Heart Disease whose childhood corrections require revision
2. Patients with newly recognized congenital heart defects or who were not treated in childhood
3. Patients with new rhythm disturbances and/or the need for pacemakers
4. Patients with pregnancy issues
5. Patients with exercise and training issues
6. Patients with adult cardiovascular or other age-related health concerns

The uniquely wide spectrum of cardiac defects affects a complex population of patients that can be categorized by varying degrees of severity and prognosis. The American College of Cardiology Task Force 1 of the 32nd Bethesda Conference developed a classification schema to categorize patients according to the disease severity. There are three classes or categories: simple lesions, moderate lesions, and complex lesions (see Group 1, 2, 3 below) with recommendations for follow-up for each.

A major issue for these adults is the common misperception that these surgeries are "curative". The 32nd Bethesda Conference recommends that all survivors of congenital heart disease be seen at least once in adulthood by an Adult Congenital Heart Disease Center and most will require regular follow-up cardiac care. This is because most of these patients, with the exception of those with a ligated and divided ductus arteriosus, have been shown to be at risk of residua and sequelae.

In 2006, the National Heart, Lung and Blood Institute (NHLBI) Working Group on Research in Adult Congenital Heart Disease proposed that a systematic study network for Adult Congenital Heart Disease (ACHD) be established, similar to the NHLBI-funded Pediatric Heart Network. The NHLBI Working Group specifically recommended:

> Establishing outreach programs to identify the "lost" ACHD population
> Forming a network of specialized ACHD regional centers to develop
- Databases to track outcomes, formulate best practices, etc.
- Develop environment for training and continuing education
- Design and conduct multi-center clinical research
> Developing technology to support hemodynamic and functional modeling of individual patients, to improve understanding of cardiac status and plan future therapy
> Providing consensus on training requirements
> Identify funding for clinical and research training that is needed to provide enough physicians to treat these patients

A major emphasis in the coming years will be the development of a national ACHD database to be the catalyst for hypothesis-driven research to advance the evidenced-based medical care for this unique and challenging population.

Group 1 Simple Heart Disease
These patients can usually be cared for in the general medical community

Native conditions:
- Isolated congenital aortic valve disease
- Isolated congenital mitral valve disease (except parachute valve, cleft leaflet)
- Isolated patent foramen ovale or small atrial septal defect
- Isolated small ventricular septal defect (no associated lesions)
- Mild pulmonic stenosis

Repaired conditions:
- Previously ligated or occluded ductus arteriosus
- Repaired secundum or sinus venosus atrial septal defect without residua
- Repaired ventricular septal defect without residua

Group 2 Congenital Heart Disease of Moderate Severity
These patients should be seen periodically at regional congenital heart centers

- Aorto-left ventricular fistulae
- Anomalous pulmonary venous drainage (partial or total)
- Atrioventricular canal defects (partial or complete)
- Coarctation of the aorta
- Ebstein's Anomaly
- Infundibular right ventricular outflow obstruction of significance
- Ostium primum atrial septal defect
- Patent ductus arteriosus (not closed)
- Pulmonary valve regurgitation (moderate to severe)
- Pulmonic valve stenosis (moderate to severe)
- Sinus of Valsalva fistula/aneurysm
- Sinus venosus atrial septal defect
- Subvalvar or supravalvar aortic stenosis (except HOCM = hypertrophic obstructive cardiomyopathy
- Tetralogy of Fallot
- Ventricular septal defect with
--- Absent valve or valves
--- Aortic regurgitation
--- Coarctation of the aorta
--- Mitral Disease
--- Right ventricular outflow tract obstruction
--- Straddling tricuspid/mitral valve
--- Subaortic stenosis

Group 3 Congenital Heart Disease of Great Complexity
These patients should be seen regularly at adult congenital heart disease centers

- Conduits, valved or nonvalved
- Cyanotic congenital heart disease (all forms)
- Double-outlet ventricle
- Eisenmenger syndrome
- Fontan procedure
- Mitral Atresia
- Single Ventricle (also called double inlet or outlet, common or primitive)
- Pulmonary Atresia (all forms)
- Pulmonary vascular obstructive diseases
- Transposition of the Great Arteries - D Type
- Transposition of the Great Arteries - L Type (Congenitally Corrected TGA)
- Tricuspid Atresia Truncus arteriosus/hemitruncus
- Other abnormalities of atrioventricular or ventriculoarterial connection not included above (i.e. criss-cross heart, isomerism, heterotaxy syndromes)