Elsevier

Progress in Cardiovascular Diseases

Volume 61, Issues 3–4, September–October 2018, Pages 314-319
Progress in Cardiovascular Diseases

When the Heart Is Not to Blame: Managing Lung Disease in Adult Congenital Heart Disease

https://doi.org/10.1016/j.pcad.2018.07.019Get rights and content

Abstract

It is well-recognized now that adult survivors with congenital heart disease (CHD) are at risk for non-cardiac co-morbidities and complications that can impact symptoms and clinical outcomes. Lung disease, in particular, is common in this population, but likely an under-recognized and undertreated cause for long-term morbidity. Abnormal lung function contributes to exercise intolerance and is associated with a higher risk for mortality in this population. The exact mechanisms that contribute to abnormal measurements of lung function are not entirely known, and are likely multifactorial and variable depending on the underlying CHD. Nevertheless, lung disease is a potentially modifiable risk factor in this patient population, the management of which may result in improved clinical outcomes. This review summarizes our current understanding of the prevalence, impact and management of lung disease in adults with CHD.

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.

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