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Congenital heart defects are cardiac abnormalities present at birth.  Approximately 8 out of every 1,000 infants are born with congenital heart disease.  Some defects are mild and may not even be apparent during infancy or childhood.  Others are life threatening and require surgery during infancy.  The care of infants and children with congenital heart disease has been a monumental success.

With the advent of sophisticated diagnostic imaging, advanced medical therapy, catheter-based interventional procedures, and innovative surgery, over 90% of infants born with a congenital heart defect now survive well into adulthood.

The success of caring for infants and children with congenital heart disease has created new challenges.  The unique anatomy, physiology, hemodynamics, and surgical procedures are unfamiliar to most physicians who care for adult patients. The nomenclature alone can be daunting and intimidating.  Terms such as Pott’s shunt, Mustard procedure, restrictive and nonrestricive defects are generally unfamiliar to cardiologists who care for adult patients.  In many cases patients who have undergone surgical repair of their defect are “fixed” but their hearts are not normal. 

For example significant pulmonic regurgitation often follows repair of tetralogy of Fallot.  This can lead to right heart dilatation, tricuspid regurgitation, arryhthmias, and shortness of breath decades after the operation.  Significant mitral regurgitation may follow repair of an ostium primum atrial septal defect because of the associated cleft in the anterior leaflet of the mitral valve.  In many cases the long-term sequelae following surgical or interventional catheter based repair are well recognized.  In other cases the long-term sequelae are not yet characterized.  Long-term follow-up studies will be needed to define the natural history after repair of both simple and complex defects. An awareness of associated defects is essential. For example, ascending aortic aneurysms are often associated with bicuspid aortic valves.

Given the complexity of caring for adult patients with repaired or unrepaired congenital heart defects, specialized programs for this purpose have evolved throughout the world including here, at the University of Vermont College of Medicine and Fletcher Allen Health Care.  The Adult Congenital Heart Disease and Pulmonary Hypertension Program at the University of Vermont College of Medicine and Fletcher Allen Health Care is committed to providing state-of-the-art and comprehensive care for adults with repaired or unrepaired congenital heart defects. 

Some of the defects encountered in patients followed in our program include:

• Atrial septal defect (ASD)
• Sinus venosus defect
• Ventricular septal defect (VSD)
• Partial and complete atrioventricular canal defects
• Patent ductus arteriosus (PDA)
• Bicuspid aortic valve
• Pulmonary stenosis and regurgitation
• Ebstein's anomaly of the tricuspid valve
• Coarctation of the aorta
• Tetralogy of Fallot
• Congenitally corrected and complete transpositon of the great arteries
• Persistent truncus arteriosus
• Tricuspid and/or pulmonary atresia
• Anomalous pulmonary venous connection
• Eisenmenger syndrome

In addition to the management of ongoing problems, a major focus is long-term preventive care including consultation regarding bacterial endocarditis, pregnancy, exercise and genetic risk to children. A full range of imaging techniques are available, including CVMRI, to facilitate diagnosis and management.

Pulmonary Hypertension

Pulmonary hypertension is high blood pressure in the blood vessels of the lung.  It is often a debilitating and progressive disorder that causes shortness of breath, heart failure, and premature death.  It can be primary (unknown cause) or secondary to a variety of disorders such as thromboembolic disease, valvular heart disease, lung disease, collagen vascular diseases such as scleroderma or lupus, sleep disorders such as obstructive sleep apnea, pulmonary vasculitis, and congenital heart disease. The Adult Congenital Heart Disease and Pulmonary Hypertension Program at the University of Vermont College of Medicine and Fletcher Allen Health Care is committed to the comprehensive evaluation and care of adults with primary and secondary pulmonary hypertension. The cause of the pulmonary hypertension is identified, and reversible disorders are treated.  A wide range of diagnostic modalities are available.  Patients with severe pulmonary hypertension may require specialized treatment including intravenous Flolan, subcutaneous Remodulin, or oral Tracleer.  We have considerable experience with these treatments, utilizing them for our patients when appropriate.

Genetic Heart Disease

Genetic disorders can effect the heart or circulatory system.  Some of the more common genetic diseases affecting the heart or blood vessels include Down syndrome, Marfan syndrome, hypertrophic cardiomyopathy, and hereditary hemorrhagic telangectasia.  Both the Adult Down Syndrome Program and The Adult Congenital Heart Disease and Pulmonary Hypertension Program at the University of Vermont College of Medicine and Fletcher Allen Health Care are committed to the comprehensive evaluation and care of adults with genetic forms of heart disease.  Marfan syndrome, hypertrophic cardiomyopathy and hereditary hemorrhagic telangectasia are all autosomal dominant disorders. Thus, first degree relatives of the patients have a 50% chance of also having the disorder.  When needed, we work closely with the genetics staff to evaluate both the patient and the family.  A wide range of diagnostic and therapeutic options are available for the optimal treatment and management of patients with genetic forms of heart disease.