Division of Cardiovascular Diseases Strategic Plan

Goals in Cardiovascular Clinical Problems or Disease States

2.1c. Improve evidence-based care and treatment of adults with congenital heart disease

Table of Contents

• Overview
• Strategies to Accomplish This Goal May Entail
  • Basic Research
  • Translational Research
  • Clinical Research
• Contributing Sources

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Overview

Major improvements in diagnosis, medical care and surgical techniques have radically changed the prognosis of patients with congenital heart disease. Currently there are more adults than children living with congenital heart disease, and the number is expected to grow at a rate of about 5 percent per year. The clinical management of these patients is becoming a focus of both pediatric and adult CV specialists as a new adult congenital heart disease subspecialty emerges. The field is at the point now where clear research questions can be articulated, but few resources have been directed to answering these questions. Conducting research is complicated by the absence of standard (if any) clinical follow-up for patients, the variety of malformations, the effects of surgical care and management strategy, and the long lag time between intervention and adverse outcome.

Strategies to Accomplish this Goal May Entail:

Basic Research:

• Use biomedical engineering tools to develop applications with direct clinical relevance to adult congenital heart disease, such as biological pacemakers, tissue-engineered valves, and right ventricular assist devices.
• Develop strategies to improve the durability of prosthetic materials.
• Develop molecular and cellular strategies for myocardial dysfunction and preservation of pacing cells.
• Expand our understanding by using animal models of the long-term effects of peri-operative factors such as myocardial preservation, hypothermia, and hematocrit.

Translational Research:

• Improve imaging and modeling of right ventricular performance.
• Identify biomarkers of arrhythmia risk.
• Identify clinical, imaging, genetic and other markers of CV risk and benefit.

Clinical Research:

• Determine the incidence and effect of complications in this heterogeneous population, such as arrhythmias, heart failure, endocarditis, thrombosis, pulmonary hypertension, neurocognitive deficits, protein-losing enteropathy, and surgical conduit obstruction.
• Develop effective preemptive, diagnostic and therapeutic strategies to prevent ischemic events, sudden cardiac death, heart failure, and thromboembolic complications in adults after repair of congenital heart disease.
• Evaluate the timing of surgical procedures and other perioperative variables to determine their effect on long-term outcome.
• Determine the impact of medications, lifestyle factors (smoking, diet, exercise), pregnancy, and normal aging on health in adults with congenital heart disease.
• Determine factors that affect the long-term function of cardiac circulation dependent on systemic right ventricles or single ventricles.
• Develop novel clinical research strategies, such as improved statistical techniques for “small n” clinical trials, novel research mechanisms, or focused collaborative strategies with existing programs such as the CTSA program.
• Explore the use of electronic medical records to identify patients and link clinical and research data, respectful of issues of patient confidentiality and informed consent.

Contributing Sources:

• NHLBI Strategic Plan Goals 1, 2, and 3
• NHLBI Plan Strategies 2 and 4
• Recommendations from the NHLBI Strategic Plan Level One Final Reports:
  • Arrhythmias
  • Valvular and Congenital Heart Disease
  • Regenerative and Reparative Medicine
  • Emerging and Evolving Technologies
• NHLBI Workshops and Working Groups:
  • Modifiers of Arrhythmias – New Targets for Therapy and Prevention, September 14, 2006.
  • Recognition and Treatment of Rare Inherited Arrhythmias, September 14-15, 2006.
  • Research in Adult Congenital Heart Disease, September 2004; Report: Williams et al., J Am. Coll. Cardiol., 2006;21 (47)701-7.
  • 32nd Bethesda Conference: Care of the Adult with Congenital Heart Disease, October 2-3, 2000. Proceedings of the 32nd Bethesda Conference: Care of the Adult with Congenital Heart Disease J. Am. Coll. Cardiol., 2001;37:1161-98.
• Other
  • American College of Cardiology Foundation: Task Force 9: Training in the Care of Adult Patients with Congenital Heart Disease. Child et al., J. Am. Coll. Cardiol., 2008;51(3)389-393.
  • Congressional Language (Appropriations, 2007)

September 2008