When the Heart Is Not to Blame: Managing Lung Disease in Adult Congenital Heart Disease
Section snippets
Prevalence
Airway abnormalities due to vascular compression and congenital pulmonary malformations can be present in up to 5–10% of patients born with CHD, and can contribute to chronic lung disease later in life.6., 7., 8., 9. However, the majority of adults with CHD have no overt history of lung disease, yet demonstrate a relatively high prevalence of abnormalities in lung function when measured with spirometry.10., 11., 12., 13. When spirometry is performed during routine cardiopulmonary exercise
Pathophysiology
The etiology of the restrictive spirometry pattern in this population is likely multifactorial, and the risk varies depending on a patient's age, underlying CHD, history of cardiac intervention and other co-morbid risk factors. Low FVC is suggestive of restrictive lung disease, which is comprised of a heterogeneous group of conditions characterized by reduced lung volume. However, there is a paucity of studies that have actually correlated abnormal spirometry in ACHD with measurements of total
Impact on clinical outcomes
Exertional dyspnea or fatigue is one of the most frequent causes of reduced quality of life in patients with CHD.39., 40., 41. In a study of 78 patients with various CHD diagnoses, up to 62% of patients reported some limitation in physical activities and 11% were unable to work and experienced limitation in all activities.41 Understanding the causes of exercise intolerance is a critical part to the comprehensive care of this growing population.
While decreased exercise capacity in ACHD is often
Evaluation and management
Assessment of lung function should be considered in any symptomatic ACHD patient with dyspnea or exercise intolerance. Given the high prevalence of abnormal lung function and the prognostic value of FVC measurements, lung function assessment should be considered part of the routine and serial follow-up of all ACHD patients, even those without a history of chronic lung disease.
A spirometric assessment can be performed as part of the initial assessment of lung function, and is typically performed
Conclusion
Abnormal lung function is a common extracardiac complication in ACHD. Spirometry often demonstrates a restrictive pattern with low FVC. A clear association between abnormal lung function and adverse outcomes has been demonstrated in ACHD patients, including a negative impact on exercise capacity and increased risk for hospitalization and mortality. However, further studies are needed to better elucidate the pathophysiology of abnormal lung function in this heterogeneous population, as well as
Funding
There was no external funding for this project.
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Conflicts of Interest: None of the authors have any potential conflicts of interest, including financial interests or relationships to industry, relevant to the subject matter or materials discussed in the manuscript.