Adult Congenital Heart Disease

Management & Personalized Treatment Plans

With advances in medicine and surgical techniques, the majority of children born with congenital/structural heart conditions now live to adulthood. The most important thing is to have providers who will understand your cardiac history, regularly monitor you for ongoing risks and complications that can arise, and stay on top of current health recommendations. Our multidisciplinary team has built our Adult Congenital Heart Disease Program to ensure that patients who survive into adulthood receive the appropriate patient-centered care that they deserve.

There is no single way of treating every patient. Patient-centered care is provided to respect a patient's values, preferences, and expressed needs. It allows for coordination and integration of care, education to facilitate autonomy, and eases access to high quality and effective medical care.

Our patients with adult congenital heart conditions receive care in a timely fashion and are cared for by a team of experts who understand the complexity of congenital heart conditions. We work with a multidisciplinary team that consists:

primary care providers,
interventional specialists,
electrical specialists,
cardiothoracic surgeons, OB/GYN providers,
high risk maternal-fetal medicine specialists,
heart failure and transplant cardiologists,
cardiac genetics,
social workers,
inpatient providers,
palliative care teams, and
other providers through the pediatric and adult hospitals.

Our multidisciplinary approach ensures that patients receive the care they need, with the provider they need, and in the appropriate location for their needs. We have the ability to see patients throughout several hospital systems and outreach locations.

What Is Adult Congenital Heart Disease?

Congenital diseases or birth defects mean that a person is born with that disease. Congenital diseases or defects develop when a fetus is still developing; they don’t develop later in life. Congenital heart disease happens when someone is born with defects in parts of their heart or in the blood vessels around their heart. Even though a baby is born with congenital heart disease, this disease may not be diagnosed until later in life.

Adult congenital heart disease is common. About one in every 150 adults has congenital heart disease.

Adult Congenital Heart Disease & Pulmonary Hypertension

Some patients with congenital heart disease will develop pulmonary hypertension (high blood pressure in the lungs) that can lead to other health problems and can affect how your heart works. Our adult congenital heart disease providers also work closely with University of Utah Health's Pulmonary Hypertension Program to provide coordinated care for these patients.

Adult Congenital Heart Disease & Heart Transplant

Patients with congenital heart conditions may eventually require a heart transplant. There are several reasons why this may occur and your adult congenital cardiologist may not have any additional surgeries or procedures that would be beneficial and a heart transplant may be the best option for your condition. Heart transplants are generally deemed as a last resort treatment option since the surgery is very involved and carries significant risk to our patient.

Conditions We Treat

We treat the following conditions:

Obstructive defects
- Aortic valve stenosis (AS)
- Pulmonary valve stenosis (PS)
- Bicuspid aortic valve
- Tricuspid valve stenosis/dysplasia
- Mitral valve stenosis
- Interrupted aortic arch
- Subaortic stenosis
- Coarctation of the aorta (coarct)
Septal defects
- Atrial Septal Defect (AVSD, AV canal, endocardial cushion defect)
- Ventricular Septal Defect (VSD)
Cyanotic defects
- Total anomalous pulmonary venous return (TAPVR)
- Partial anomalous pulmonary venous return (PAPVR)
- Truncus arteriosus
Patent ductus arteriosus
Tetralogy of fallot
Ebstein’s anomaly
D-transposition of the great arteries (D-TGA)
Congenitally corrected transposition of the great arteries (L-TGA)
Aortopathy/Connective tissue disorders
- Marfan syndrome
- Loeys-Dietz syndrome
- SMAD3
- Familial aortopathies
- Ehlers-Danlos Syndrome (vascular type, classic type, hypermobility, if known aortic dilation or mitral valve prolapse)

We also treat single ventricle conditions including the following:

Tricuspid atresia
Double-inlet left ventricle
Heterotaxy syndromes
Hypoplastic left heart syndrome
Pulmonary atresia
Unbalanced AV septal defects
s/p Fontan procedure

Find a Heart Specialist

Heart Disease & Pregnancy

We provide comprehensive obstetric and gynecologic care for women with congenital heart conditions. We encourage all women with congenital heart conditions to establish care with a primary OB/GYN and meet with a maternal-fetal medicine provider (high risk OB/GYN) before they consider getting pregnant.

Your OB/GYN will be able to discuss appropriate birth control methods and sexual function as it relates to your congenital heart condition. He or she will also discuss recommendations with your adult congenital cardiologist to ensure safety and efficacy in relation to your congenital heart condition. We encourage our female patients to use appropriate contraception and wait to plan their

pregnancy until after they meet with their primary adult congenital cardiologist and consult with a maternal-fetal medicine specialist. We also offer access to certified genetic counselors and physicians who specialize in genetics.

Women who wish to prevent or delay pregnancy are encouraged to use a highly effective form of contraception, such as long-active reversible contraception (LARC). These methods include intrauterine devices (IUDs) and subdermal (injected under the skin) progesterone devices. These types of contraceptives are not harmful for women with congenital heart conditions. If you decline a LARC method, you will be counseled on other contraceptive options.

Our OB/GYN and maternal-fetal medicine providers will work closely with the adult congenital cardiologist to provide individual consultation based on each patient’s:

specific heart issue,
functional status,
genetic counseling,
recurrence risk of congenital heart conditions,
optimization of medical status before, during and after pregnancy, and
other factors regarding risks of pregnancy to the patient or fetus.

Some of our patients do come to us with an unplanned pregnancy and will be counseled about all of their options.

During your pregnancy, our multidisciplinary team will work together with you to:

optimize your care,
establish plans for timing, location, and mode of delivery, and
optimize post-delivery care.

The adult congenital cardiology team will be notified when you are admitted and will be available for inpatient consultation when needed. We will be available for phone consultation for all other locations.

Psychological Support

Adults with congenital heart conditions are often not only coping with their cardiac diagnosis, but sometimes struggle with their medical diagnosis both psychologically and socially. When a need for mental health services are found, or are expressed by our patient, our social work team will be consulted to provide an initial psychosocial and mental health assessment. The social worker will educate and assist in facilitating an appropriate mental health referral for patients based on need, location, and insurance coverage.

Patient Resources

Adult Congenital Heart Association (ACHA)

American College of Cardiology

American Heart Association (AHA)

Marfan Foundation

Utah American Heart Association

ACHD Sub-Specialty Fellowship Training

Due to the complexity of adult patients with congenital heart disease, it is recommended that most ACHD patients follow with providers who have specific training and knowledge to care for the complex conditions. “Adult Congenital Heart Disease” fellowship and subsequent board certification ensures that providers caring for this population have the necessary training and experience. Our program offers a 2-year fellowship position for providers who have already trained in pediatric or adult cardiology to gain this extra knowledge and experience caring for patients with congenital heart conditions.