Abnormal Spirometry in Congenital Heart Disease

A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life

“…Development of the lungs starts in the last trimester of pregnancy and 95% of the alveolar surface is formed after birth: physical activity, somatic growth, and pulmonary blood supply strongly influence lung growth and development. 15 Unfortunately, Fontan patients experience all the conditions that could deteriorate alveolar growth, such as long and multiple hospitalizations, iatrogenic damage to the pulmonary vascular system In fact, regarding the limitation to exercise, our analysis showed that the patients with an inadequate ventilatory function were restricted on the cardiac level, rarely having a decreased breathing reserve. Therefore, we can conclude that a decreased FEV 1 relevantly exacerbates cardiac limitation to exercise.…”

“…34 However, all the described predictors of FEV 1 together accounted only for 22% of FEV 1 's variation. 15 Unfortunately, Fontan patients experience all the conditions that could deteriorate alveolar growth, such as long and multiple hospitalizations, iatrogenic damage to the pulmonary vascular system can conclude that a decreased FEV 1 relevantly exacerbates cardiac limitation to exercise. This suggests that other, and more generally applicable factors, which were not considered in our analysis, exist additionally.…”

“…7,8 In UVH, the single ventricle is the systemic ventricle and the passive pulmonary blood flow 9 is substantially enhanced by the ventilatory movements and by the skeletal muscular pump of the veins. 15 Therefore, this analysis investigates the extent of ventilatory impairment in Fontan patients, its predictors in the clinical/surgical history and its consequences on the subsequent outcomes in terms of exercise capacity and quality of life (QoL). 13 A reduced lung perfusion can lead to a reduced preload of the single ventricle.…”

Resting respiratory lung volumes are “healthier” than exercise respiratory volumes in different types of palliated or corrected congenital heart disease

“…Short‐term alterations of respiratory mechanics after palliative or corrective surgery have been extensively investigated; however, data on long‐term changes of respiratory mechanics, based on a complete assessment of lung volumes and simultaneous exercise tests, are scarce. Lung function is often assessed by the flow‐volume curve and not by measurements of complete lung volumes nor gas diffusion, which could help to better understand the underlying mechanisms of lung diseases, such as the role of respiratory muscles and early development factors related to pulmonary blood flow 11 . Identification of the mechanism could help to target functional respiratory intervention, which in turn may have beneficial effects on both the right and the left ventricular function 12,13 …”

“…Lung function is often assessed by the flow-volume curve and not by measurements of complete lung volumes nor gas diffusion, which could help to better understand the underlying mechanisms of lung diseases, such as the role of respiratory muscles and early development factors related to pulmonary blood flow. 11 Identification of the mechanism could help to target functional respiratory intervention, which in turn may have beneficial effects on both the right and the left ventricular function. 12,13 The primary objective of this study was to determine the longterm and severity of respiratory functional impairment in patients with different forms of CHD with prior surgical correction/palliation.…”