Pulmonary function and exercise capacity in survivors of congenital diaphragmatic hernia

M G Peetsold1, H A Heij, A F Nagelkerke

  • 1Dept of Paediatrics, VU University Medical Centre, P.O. Box 7057, 1007 MB Amsterdam, The Netherlands. m.peetsold@vumc.nl

Insights

Survivors of high-risk congenital diaphragmatic hernia (CDH) often have mild lung function deficits. Gastro-oesophageal reflux disease may contribute to these pulmonary issues in children with CDH.

Area of Science:

  • Pediatric Pulmonology
  • Congenital Abnormalities
  • Respiratory Medicine

Background:

  • Congenital diaphragmatic hernia (CDH) is linked to underdeveloped lungs and high blood pressure in the lungs.
  • Early respiratory distress defines high-risk CDH, necessitating long-term outcome assessment.

Purpose of the Study:

  • To evaluate pulmonary function and exercise capacity in children and adolescents with high-risk CDH.
  • To identify early factors influencing pulmonary outcomes in CDH survivors.
  • To correlate findings with the initial severity of CDH.

Main Methods:

  • Pulmonary function tests (spirometry, lung volumes) and maximal cardiopulmonary exercise testing (CPET) were performed on 53 high-risk CDH survivors (mean age 11.9 years).
  • Results were compared to a matched healthy control group.
  • Linear regression analyzed determinants of pulmonary function.

Main Results:

  • CDH survivors exhibited significantly reduced FEV(1), FVC, FEV(1)/FVC, and peak expiratory flow compared to controls.
  • A higher residual volume/total lung capacity ratio was observed in CDH survivors.
  • Gastro-oesophageal reflux disease was identified as an independent factor for decreased FEV(1) and FVC.

Conclusions:

  • High-risk CDH survivors present with mild to moderate pulmonary function abnormalities.
  • Pulmonary issues may be associated with early-life gastro-oesophageal reflux disease.
  • Exercise capacity and gas exchange were generally normal, suggesting minimal physical impairment for most survivors.

Related Concept Videos

Pulmonary Function Tests01:25

Pulmonary Function Tests

Pulmonary Function Tests (PFTs)
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
Respiratory Volumes and Capacities I01:26

Respiratory Volumes and Capacities I

Assessing the respiratory rate and rhythm for a complete minute is crucial for evaluating the breathing pattern. Even a minor increase in the patient's average respiratory rate, by as little as three to five breaths per minute, is an early and vital indicator of respiratory distress. Patients with a respiratory rate exceeding twenty-four breaths per minute require close monitoring to determine the physiological alterations. This careful observation is essential for prompt recognition and...
Respiratory Capacities01:24

Respiratory Capacities

Respiratory capacities are crucial indicators of lung function, representing the maximum amount of air an individual's respiratory system can handle during various breathing phases.
One key metric is the Inspiratory Capacity (IC), which represents the maximum amount of air that can be inhaled with full effort. IC is calculated by summing the tidal volume and inspiratory reserve volume, typically ranging from 2.4 to 3.6 liters.
The Functional Residual Capacity (FRC) represents the air in the...
Lung Capacity01:47

Lung Capacity

The air in the lungs is measured in volumes and capacities. Lung volume measures reflect the amount of air taken in, released, or left over after a lung function, like a single inhalation. Lung capacity measures are sums of two or more lung volume measures.
Respiratory Volumes and Capacities01:22

Respiratory Volumes and Capacities

The respiratory system is responsible for the intake of oxygen and the expulsion of carbon dioxide from the body. Respiratory volumes describe the volume of air in the lungs at different phases of the respiratory cycle. Tidal volume is the air breathed in and out during normal, quiet breathing. Inspiratory reserve volume is the air that can be forcefully inspired beyond the tidal volume. In contrast, expiratory reserve volume refers to the air that can be expelled from the lungs after a normal...
Pulmonary Cycle: Exhalation01:17

Pulmonary Cycle: Exhalation

In terms of human respiration, the act of expelling air, known as exhalation (or expiration), operates on the principle of pressure gradients. During expiration, the pressure within the lungs exceeds that of the surrounding atmosphere. Under normal conditions, quiet breathing involves passive exhalation and is free of muscular contractions. This is because the exhalation process is driven by the natural elastic recoil of the lungs and chest wall, both of which have an inherent tendency to...