Adults Living with Congenital Heart Defects (CHD)

We are right in the middle of Congenital Hearts Defect (CHD) Awareness Week, which runs from February 7th through 14th. I am glad to see so many posts on social media to help draw awareness! Many of those posts center around children with CHD, which is good as heart defects are the most common birth defect in the United States. But many people are unaware of how CHD affects adults. Hence, this blog post! 

In 2010, there were 1.4 million adults in the United States living with congenital heart defects (CHD). Due to increased survival, this number increases by about 5% every year. These numbers are significant considering that at one time babies born with CHD had virtually no chance of becoming an adult.

Thanks to surgical advances and research in pediatric cardiology in the past 50 years, most congenital heart defects have been able to be corrected. Today, babies born with heart defects have a much better outlook than they did 50, or even 15 years ago.

February 7-14th is CHD Awareness Week
  Photo Source: Pixabay

Some of the First Congenital Heart Surgeries

Some of the adults who are alive today are survivors of some of the first open heart surgeries done in babies born with defects. For instance, the Blalock-Thomas-Taussig procedure was developed in 1944 to help infants with cyanosis or blue-baby disease. This procedure was done primarily for those born with Tetralogy of Fallot (TOF), one of the most common congenital heart defects.

Another surgery, called the Fontan was first done in 1968. Surgeons perform this procedure on patients born with a single ventricle or a single-functioning ventricle. Today, the Fontan operation is the most common procedure performed after the age of two years. Babies born with defects requiring this type of surgery often have to go through other procedures first.

Of course, even though surgeries like those mentioned above were first performed when they were, they have gone through several modifications. Today many of these open-heart surgeries being performed offer babies born with CHD's a better quality of life which in turn helps them grow into adults with a higher quality of life than the first-time recipients of such procedures.

The Need for Continual Care

Many adults who received treatment for their heart defect as a child go into adulthood thinking that they have been cured and they no longer need to worry about their heart condition. For over half of the adults living with CHD, this couldn't be further from the truth. Depending on the complexity of the heart condition the patient was born with, sometimes they need to receive ongoing care for the rest of their lives.

There are three primary reasons that adults who need continual care often fall through the cracks: 

• They think they have been cured and they no longer need care.
• They can't afford ongoing care.
• They don't know where to go for ongoing care.  

As children, many adults with congenital heart defects saw a pediatric cardiologist and it then becomes difficult for them to make the transition from a child to an adult with CHD.

Currently, there are nearly 60 ACHD centers that have been accredited by the Adult Congenital Heart Association in the United States. 

Mayo Clinic in Rochester MN is an ACHA-accredited center for adults with CHD

Bethesda Conference Guidelines

Every year, the American College of Cardiology (ACC) conducts a meeting in Bethesda, Maryland to address important issues in cardiology. In the year 2000 during the 32nd Bethesda conference, the ACC addressed the needs of adults living with congenital heart defects. The report produced from this conference outlines many unmet health care needs facing those adults with congenital heart defects (ACHD).

The report also states that those adults born with complex heart defects should be seen at specialized ACHD care centers on a regular basis, in other words be seen by someone with experience in treating adults, not just children with CHD. 

Since a regular cardiologist has little training in the area of pediatric cardiology and virtually none when it comes to adults with CHD, the report suggests that even those adults born with simple defects be evaluated at least once at an ACHD care center.

There are cases where the ACHD cardiologist travels from an ACHD center to surrounding towns to see patients. For example, cardiologists at the Mayo Clinic in Rochester, Minnesota travel to other areas including communities in Iowa and North Dakota.

The Challenges of Living as an Adult with CHD

There are many challenges for some adults with complex CHD that healthy adults do not face.

Contraception and Pregnancy

Women born with a complex heart condition need to be careful about which kind of contraception is safe for them and also if pregnancy puts their life at risk. At one time it was not recommended for women with certain heart defects to have children. Now, however with the development of ACHD centers, some women can become pregnant but have to be followed closely by their ACHD cardiologist.

It is advisable that before a woman starts on any kind of birth control or before getting pregnant that she talks to a cardiologist who specializes in adults with CHD.

If pregnant, women with CHD should also begin to have their baby's hearts monitored between the 16th and 18th week of pregnancy. Some congenital heart defects are thought to be hereditary.

Exercise

Some adults with CHD have to be careful about how much they can tolerate when it comes to exercising. Some patients need to have weight restrictions while others are not recommended to walk or run long distances.

Psychological Issues

Being an adult with CHD can take its toll emotionally. Some adults have a fear of dying while others may have trouble being active because of their health. Others face depression and unresolved fear and pain. 

There are also physical characteristics such as scarring or being cyanotic that can be hard to deal with. Sometimes talking about their heart defect is hard to do with friends of family members. If an adult with CHD is having emotional issues because of health reasons or any reason, it is recommended they see a counselor.

Education is Crucial

All adults living with CHD should be educated about their particular defect. To live as healthy as possible it is important to be educated in the following:

• Name and details of their heart condition
• Surgeries that have been performed
• Frequency of follow-up
• Medications and side effects
• Preventing heart infections (endocarditis)
• Exercise
• Birth control and family planning
• Career planning and resources
• Insurance
• Dental care
• Symptoms to look for
• When to contact their health care provider

They also need to know that minor or major surgery can present risks for the CHD patient. Special attention is needed with anesthesia and to prevent heart infections (endocarditis). Patients on blood thinners also require special care. Ideally all surgery on these patients should be done at ACHD centers.

Adult Congenital Heart Association

The Adult Congenital Heart Association (ACHA) is a "nonprofit organization which seeks to improve the quality of life and extend the lives of congenital heart defect survivors." It was founded in 1998 by a group of adult congenital heart defect survivors and their families.

The ACHA website is a great place to go for support, education and to become an advocate of research. There is a message board which is a wonderful way to find other people with similar conditions as yours. There is also information on how to get an ACHA support group started in your area.

If you would like to read about my personal journey with CHD, here are a few blog posts I have written: 

• My Heart Story (So Far) 
• Remembering My Last Open-Heart Surgery 
• Life with a Congenital Heart Defect 
• 50 Years Ago Today: My First Open Heart Surgery 
• A Thankful Heart 
• A Very Long Pacemaker Appointment 

Me with my sister after my pacemaker lead replacement in 2021