Single ventricle defects are a group of complex congenital heart conditions that require lifelong medical care. The Adult Congenital Heart Program at UK Gill Heart & Vascular Institute provides advanced, comprehensive care for adults with single ventricle defects in Central Kentucky.
A typical heart has two upper chambers called atria that receive blood and two lower pumping chambers called ventricles. Usually, oxygen-poor blood travels from the right atrium to the right ventricle to the lungs. Next, oxygen-rich blood flows from the lungs to the left atrium and then to the left ventricle. Finally, the left ventricle pumps blood out of the heart to the rest of the body.
In people with a single ventricle defect, only one ventricle develops. The other is either missing or underdeveloped, which interferes with normal heart pumping and causes oxygen-poor and oxygen-rich blood to mix in the heart.
Types of single ventricle defects include:
- Double inlet left ventricle: The right and left atria are connected to the left ventricle, leading to poor development of the right ventricle.
- Ebstein anomaly: The tricuspid valve develops lower than usual in the right ventricle, causing a small right ventricle and a poorly functioning tricuspid valve.
- Hypoplastic left heart syndrome (HLHS): The left side of the heart is underdeveloped, including the aorta, left ventricle and multiple valves.
- Pulmonary atresia: The pulmonary valve doesn’t develop, affecting blood flow into the lungs. The right ventricle and tricuspid valve may also be underdeveloped.
- Tricuspid atresia: The tricuspid valve between the right atrium and right ventricle is missing or underdeveloped, and the right ventricle is small.
Patients with single ventricles may experience different symptoms based on the type of defect they have. Symptoms are often present from birth but may develop soon after. A common early symptom is cyanosis, a blue coloring to the lips or skin caused by low oxygen levels.
Adults with a single ventricle defect may also experience:
- Abnormal heart rhythms, or arrhythmias
- Congestive heart failure
- Fatigue
- Hoarseness or voice changes due to frequent larynx issues
- Long-term cyanosis
- Lung conditions
- Kidney issues
- Shorter than average height
- Swelling in the abdomen or legs due to fluid retention
With treatment, most people with single ventricle defects can live a full life, but they may have physical restrictions and require medications. Overall, they may have a reduced life expectancy. However, with medical advances and consistent care, many patients who have surgery in childhood live three to five decades beyond their surgery.
Genetic abnormalities cause certain types of single ventricle defects. But in many cases, the cause of the heart defect is unknown.
Risk factors for developing a single ventricle heart defect may include:
- Increasing parental age
- Influenza or rubella during pregnancy
- Pregestational diabetes
- Prenatal exposure to certain chemicals or drugs, including anticonvulsant medication, certain treatments for bacterial infections, industrial chemicals, lithium, marijuana and some anti-inflammatory drugs
Children with congenital heart disease become adults with congenital heart disease (CHD). Ninety percent of children born with CHD have a normal life expectancy due to improved surgical interventions and better medical management. At all ages, close monitoring and expert care for CHD patients are essential. Despite this, studies indicate that 2 out of 3 young adults with CHD don’t transition from pediatric to adult CHD care. Not getting continued cardiac care can lead to a variety of health problems and poor outcomes.
To guide teens and their families through the transition, UK Gill Heart & Vascular Institute team members talk about the transition early and often. Starting at age 15, teens with CHD begin transition education. One Wednesday every month, a special transition clinic staffed by adult congenital heart disease providers, is held to meet with teens and their families to discuss important topics, including:
- Abilities and limitations experienced as an adult with CHD
- Lifelong need for proper CHD monitoring and treatment
- What adult CHD care may involve over the years
Additionally, once you transition to adult care with the Adult Congenital Heart Program at UK Gill Heart & Vascular Institute, we work hard to enable you to keep your appointments and testing on track. We also work hard to empower you with knowledge and assist you in being your best advocate to live your best life. If you miss an appointment, we will call to reschedule.
During your first visit to the UK Gill Heart & Vascular Institute’s Adult Congenital Heart Program, you’ll meet our clinic nurse, social worker and other members of your care team. You’ll also undergo imaging exams, which may include an echocardiogram or a cardiac MRI.
Once imaging is complete, you will meet with an adult congenital cardiologist and other team members for up to one hour. You’ll discuss the results of your echocardiogram and your health history and symptoms. You will also get in-depth education about your diagnosis, how it may affect you moving forward and possible treatment options. The meeting is also a great opportunity to ask any questions about living with CHD.
When coming to your appointment:
- Bring a list of any medications or supplements you take and surgeries you’ve undergone.
- Dress comfortably.
- Have your insurance information on hand. UK HealthCare accepts most insurers, and financial assistance is available.
- You may want a family member or friend with you to take notes or help ask questions.
Where we are located
The UK Gill Heart & Vascular Institute is located at 800 Rose St. in Pavilion G, on the first floor of UK Albert B. Chandler Hospital. We will mail you a map and directions when your appointment is scheduled.
Patient drop-off
Patients can be dropped off in front of the main entrance to Albert B. Chandler Hospital at 1000 S. Limestone. If you are dropped off at the main entrance, you’ll enter the revolving doors on the ground floor of the hospital. Take the main stairs just to the left (or elevator across the atrium) to the first floor of Pavilion A.
After climbing the stairs or taking the elevator, turn left. You’ll pass the Kentucky Wall and Kentucky Children’s Hospital on your right. Continue to the end of the hall until it comes to a T shape. Turn left and the Gill clinic entrance is across from an open area housing the organ donor wall.
Parking
It is easiest to park in the UK HealthCare Parking Garage, across from the hospital at 110 Transcript Ave.
There are two paths from this garage to the Gill clinic:
- You may take the free shuttle from Level A of the parking garage. You’ll exit the shuttle at the Pavilion A/ Pavilion G stop in front of the main entrance to the hospital. After climbing the stairs or taking the elevator, turn left. You’ll pass the Kentucky Wall and Kentucky Children’s Hospital on your right. Continue to the end of the hall until it comes to a T shape. Turn left and the Gill clinic entrance is across from an open area housing the organ donor wall.
- From Level C of the parking garage you may walk or take a golf cart across the pedway to the first floor of Pavilion A. Turn left past the help desk and walk away from the dining hall. You’ll pass the Kentucky Wall and Kentucky Children’s Hospital on your right. Continue to the end of the hall until it comes to a T shape. Turn left and the Gill clinic entrance is across from an open area housing the organ donor wall.
If you need help finding your way, information desks are located inside the main entrances to Pavilion A, both on the ground floor and at the end of the pedway on the first floor. You may also call the information desk at 859-323-5816.
Clinical trials provide access to novel treatments for those in the adult CHD program at UK Gill Heart & Vascular Institute. Through our Cardiology Clinical Research Center, patients benefit from advances in valve replacement and other therapies before they become standard treatment. Your care team will notify you if you qualify for an ongoing clinical trial.
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