January 15, 2024/Pediatrics/Cardiology

Exploring Long-Term Quality of Life in Patients With Congenital Heart Disease

What factors can be modified to improve outcomes?


Findings from a new study emphasize that long-term quality of life among congenital heart disease (CHD) survivors may have more to do with neurodevelopmental, psychosocial and physical factors than surgical and ICU-related variables. The authors say these findings set the stage for more patient- and parent-centered strategies during hospitalization and follow-up.


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From preventing mortality to maximizing quality of life

Outcomes in pediatric patients undergoing congenital heart surgery have shifted significantly over the past several decades, from a focus largely on preventing mortality to a focus on minimizing morbidity and maximizing quality of life. CHD remains the most common birth defect in the United States, occurring in about 1% of babies.

“Right now, there are more adult patients than pediatric patients living with congenital heart disease,” says senior author Bradley Marino, MD, MBA, Chief of Cardiology and Cardiovascular Medicine at Cleveland Clinic Children’s.

While most children who have heart surgery are surviving, their long-term quality of life is not well understood. Long-term quality of life in patients with CHD has been a research focus for Dr. Marino and was the subject of a recent study that explored the impact of surgical and ICU factors on these patients. Dr. Marino and colleagues published their findings in Pediatric Critical Care Medicine.

Looking beyond surgical complexity

This cross-sectional corollary study leveraged data from the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study. The PCQLI is a tool that Dr. Marino developed more than a decade ago that has acquired quality-of-life data on more than 3,000 patients and their parents from 19 centers in the United States and the United Kingdom. This study and others have ushered in new understanding about decreased quality of life in children following congenital heart surgeries — beyond what was previously believed to be factors linked to surgical complexity.

“Surgical complexity accounts for only about 1% to 2% of the variation of the quality-of-life score in the long term,” notes Dr. Marino, who led an analysis on surgical complexity and quality of life in 2016.

Continuing to tease out the question of what impacts long-term quality of life, the research team examined the surgical and ICU factors in children and adolescent CHD survivors (ages 8 to 18).

Their analysis included 572 electronic health records from the PCQLI registry of patients who underwent the Fontan procedure, surgery for tetralogy of Fallot or surgery for transposition of the great arteries at eight pediatric hospitals. Previous studies established that these patients have lower long-term quality of life compared with their counterparts with mild CHD (and no intervention) and kids who are heart healthy.

The researchers also added data to the initial PCQLI dataset from the patients’ medical records to examine a broader set of surgical and ICU factors.

Findings explained: Surgical and ICU variables

“When we looked at the results from our multivariable models, lowest body temperature (a surrogate for surgical complexity) and total number of cardiopulmonary bypass runs were negatively associated with PCQLI Total score,” says Dr. Marino.

ICU factors associated with lower quality of life included the cumulative days on an inotropic or vasoactive drug in the ICU as well as a neurologic deficit at discharge.


“What’s interesting,” says Dr. Marino, “is that the variance explained by all the factors in the model was 24% to 29%, but the actual ICU and surgical variables accounted for a relatively small percentage of that total variance.”

Nonsurgical and non-ICU factors such as living in a two-parent household, time since last surgery and last cardiac hospitalization, and medical care utilization (number of doctor visits in the past year and number of daily medications) had a significant impact on health-related quality of life in the study population.

Dr. Marino says this study, taken with the findings from the CHD quality-of-life predecessor studies, reinforces that neurodevelopmental and psychosocial factors play a critical role in long-term quality of life — regardless of the type of heart disease and risk category.

“Take, for example, patients with single ventricle anatomy who have undergone a Fontan procedure,” he says. “There is wide variation in their quality-of-life scores. Some of these patients will have very low quality of life and others will have very high quality of life, and we think much of that is due to specific neurodevelopmental, psychosocial, and behavioral and emotional factors.”

A focus on intervention, follow-up care

These new findings reinforce to providers and health systems that there is a need for patient- and family-centered efforts designed to build resiliency and develop coping strategies during hospitalization and follow-up care.

Complex heart surgery and a longer ICU course can put patients at risk for neurologic injury and neurodevelopmental deficits. The Neurodevelopmental Support Program housed in Cleveland Clinic Children’s Hospital for Rehabilitation collaborates closely with pediatric cardiologists to identify high-risk patients postoperatively in intensive care units and outpatient areas. The program delivers targeted interventions to manage neurodevelopmental and psychosocial issues.

“These specific neurodevelopmental and psychosocial issues do not just stop when the child becomes a young adult,” says Dr. Marino. “Without appropriate identification and intervention, they can become lifelong and significantly impact self-sufficiency while navigating the complexity of adult life.”


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