Surveys Show How Symptoms Differ Among Adults with Congenital Heart Disease

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The number of adults with congenital heart disease (ACHD) has grown significantly over the last 20 years and is now estimated to exceed 1.2 million in the U.S. The ACHD population now exceeds the pediatric CHD population. Little data exists for ACHD specialists to use in quantifying patient-reported outcomes (PROs).

To identify symptoms that could be used to create a single disease-specific PRO to help assess quality of care, researchers including Ami B. Bhatt, MD, director of the Mass General Adult Congenital Heart Disease Program, and Mass General cardiovascular research fellow Ada Stefanescu Schmidt, MD, MSc, surveyed health care providers and patients to identify the most significant symptoms. The results were published in Circulation: Cardiovascular Quality and Outcomes.

First, ACHD physicians were surveyed and identified 39 symptoms typically discussed during ACHD patient visits.

Next, the researchers surveyed a test cohort of 124 patients to obtain their perceptions on symptom significance and frequency. The test cohort was made up of 100 non-select patients who attended the 2014 Adult Congenital Heart Disease Association meeting and 24 patients from the Center for Adults with Congenital Heart Disease at Washington University School of Medicine. In addition, a confirmatory cohort was made up of 40 patients from Mass General’s ACHD clinic during April and May of 2015.

Among the test cohort, the mean age was 43 years and two-thirds were female. They broadly represented a range of ACHD lesions.

The researchers divided the responses into seven groups ranging from low to high anatomic complexity of the lesions.

The researchers found there were a few specific symptoms rated as most troublesome across all ACHD groups. Six of the 39 symptoms were rated as being the most problematic.

The Symptoms

The patients from the Mass General cohort attributed high impact scores to the following symptoms:

• "Shortness of breath (windedness, trouble breathing) when I do things"
• "Feeling I am different than other people"
• Having to go to the hospital or emergency room
• Feeling mentally slower than other people
• Concerns with sexual function
• Bluish or dusky colored skin

“Shortness of breath (windedness, trouble breathing) when I do things” was highly rated as troublesome in all groups except one, the group with the lowest anatomic complexity, while “Bluish or dusky colored skin” was highly rated only among patients with the highest anatomic complexity.

The Mass General confirmatory cohort revealed similar results to the derivation cohort with only five of the 39 symptoms differing from the initial cohort. Importantly, the confirmatory patients enrolled at Mass General rated the symptom “Concern about my children having health problems like mine” higher than the test group.

Based on these results, the researchers found that a single PRO can be developed for ACHD patients. Despite differences in underlying diseases and treatments, there are clearly certain patient reported concerns that are most important to address with these individuals. As a next step within their research focus, the investigators will undertake focus groups and cognitive debriefing exercises across several academic ACHD programs.

From a treatment perspective, the Mass General ACHD Program has incorporated these findings into their ACHD Health and Wellness Program, run out of the MGH West Facility where physicians and nurses work with patients to address their lives in a holistic manner, emphasizing that care of these individuals means much more than addressing medical issues alone and that the patient voice can best guide patients towards an engaged and productive life with a backdrop of congenital heart disease.

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Refer a patient to the Corrigan Minehan Heart Center

Learn more about the Adult Congenital Heart Disease Program