Congenital central hypoventilation syndrome and ventilatory
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Proof of cardiopulmonary exercise test (CPET) hypoxemia and hypercapnia in children with congenital central hypoventilation syndrome have been demonstrated previously (CCHS). No longitudinal studies have, however, yet been carried out comparing CPET outcomes to polysomnograms (PSG) and PHOX2Bs. In a study, the longitudinal findings of CPET were identified and compared with the results of PSG in children with CCHS.

This retrospective study was conducted in children with CCHS. CCHS genetic mutation, age of diagnosis, ventilatory support, family history, disease associations of CCHS, CPETs, and PSG parameters were recorded and analyzed.

Results:
A total of 9 patients with CCHS (46 CPETs and 46 PSGs) were enrolled. 4 (44.4%) children had polyalanine repeat mutations. The mean (SD) age at the time of diagnosis and duration of ventilatory usage were 3.2±3.4 years and 11.5±2.8 years, respectively.

--All abnormal CPETs had hypercapnia in at least 1 phase of the exercise test. Hypercapnia (12/46; 26.1%) at peak of exercise was the most common abnormality. None of the children experienced an oxygen desaturation below 90%.

--End?tidal CO2 (PetCO2) at rest and at peak exercise in the CPETs were significantly correlated with PSG TcCO2 while PetCO2 at anaerobic threshold was correlated with CO2 in pre-PSG capillary blood gas.

Conclusively, Nocturnal hypoventilation may impact the CPET results in CCHS children. Serial CPETs should be considered standard clinical care for all CCHS children.

Source: https://onlinelibrary.wiley.com/doi/abs/10.1002/ppul.25278?af=R
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