In this issue of the European Respiratory Journal, HELD et al. [2] report on 18 patients with severe pulmonary hypertension due to alveolar hypoventilation, who were markedly improved after 3 months of noninvasive bi-level positive-pressure ventilation (NIPPV). Mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) were 50 mmHg and 6–7 Wood units, respectively, at baselin...

Two children aged 1 and 20 months developed alveolar hypoventilation syndrome. They suffered severe apnoeic episodes and periodically required assisted ventilation. Their ventilatory response to carbon dioxide was lower than that of normal children and the transcutaneous oxygen tension during sleep was well below the normal range. Treatment with medroxyprogesterone acetate resulted in an improv...

Arterial blood gas analysis was performed before and after 60 to 90 s of voluntary hyperventilation in 27 consecutive patients with occlusive sleep apnea syndrome (OSA) and daytime hypercapnia. The percentage of fall in PaCO2 from baseline was examined in relationship to age, body mass index, sleep-disordered breathing indices, and pulmonary function variables. In 14 subjects without airflow ob...

Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr Mokhlesi has received consultant fees from Philips/Respironics. Drs Tulaimat and Parthasarathy have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.Reproduction of this article ...

Abstract Introduction Central Core Myopathy (CCM) is a rare genetic neuromuscular disorder involving mutation in the ‘Ryanodine Receptor’ (RyR1) gene, also known as gatekeeper of calcium within muscle cells. CCM typically manifests slowly progressive proximal weakness Currently, there limited data regarding management nocturnal hypoventilation adults with disorders. Report case(s) We present 62...