Daytime predictors of nocturnal hypercapnic hypoventilation in children with neuromuscular disorders—The Holy Grail

Trucco, Federica

doi:10.1002/ppul.25888

Cited by 1 publication

(1 citation statement)

References 22 publications

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“…This presents a challenge, on top of the limited access to PSG, in that implementation of clinical guideline recommendations of surveillance PSG at the time of diagnosis of a NMD [5] or after a patient reaches a certain clinical state is not practical. There have been a wide range of approaches to assess-ment and management of hypoventilation in pediatric patients with neuromuscular disease; [6][7][8][9] however, more recent guidelines have shifted towards taking a more proactive approach, focusing on awareness of when a patient is at risk for hypoventilation and when it is important to obtain a PSG in order to maximize diagnostic yield [10][11][12][13][14][15][16] so that early therapeutic intervention can occur.…”

Section: Introductionmentioning

confidence: 99%

Respiratory Insufficiency in Neuromuscular Disease (RIND): A Delphi Study to Establish Consensus Criteria to Define and Diagnose Hypoventilation in Pediatric Neuromuscular Disease

Mayer,

Amin,

Sawnani

et al. 2023

Journal of Neuromuscular Diseases

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Chronic respiratory failure is a common endpoint in the loss of respiratory muscle function in patients with progressive neuromuscular disease (NMD). Identifying the onset of hypoventilation is critical to allow for the timely introduction of ventilator support and effectively manage respiratory failure [1–3]. While there are accepted criteria governing the diagnosis of hypoventilation during polysomnography (PSG) [4], there is concern that criteria are insufficient for identifying hypoventilation in the earlier stages of respiratory insufficiency related to NMD. The purpose of this project was to identify more sensitive criteria for identifying hypoventilation. Methods: Fifteen pediatric pulmonologists with broad experience in managing patients with NMD, 10 of whom were board certified in and practice sleep medicine, were assembled and performed a review of the pertinent literature and a two-round Delphi process with 6 domains (Table 1). Results: Within the 6 domains there were three pertinent items per domain (Table 2). There was clear agreement on findings on history (morning headaches) and pulmonary function testing (FVC < 50% or awake TcCO2 > 45 mmHg) indicating a high concern for nocturnal hypoventilation. There was close agreement on the definitions for nocturnal hypercapnia and hypoxemia. PSG criteria were identified that indicate a patient is likely in the transitional phase from adequate ventilation to hypoventilation. Discussion: We identified a set of clinical criteria that may allow for more sensitive diagnosis of hypoventilation in NMD and earlier initiation of non-invasive ventilation leading to a reduction in the respiratory morbidity in progressive NMD. These criteria need to be further and more broadly validated prospectively to confirm their utility.

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