title: Reversal of pulmonary hypertension after diaphragm pacing in an adult patient with congenital central hypoventilation syndrome.
abstract: BACKGROUND
  Patients with the congenital central hypoventilation syndrome (CCHS) suffer from life-threatening hypoventilation when asleep, making them dependent on mechanical ventilation (MV) at night or during naps. State-of-art respiratory management consists of intermittent positive-pressure ventilation via a tracheotomy or mask. In some patients hypoventilation is permanent, in which case ventilatory support must be extended to the waking hours. Diaphragm pacing can prove useful in such situations.
  
  
  RESULTS
  This report describes the case of a 26-year-old woman with CCHS in whom failure to achieve adequate MV led to life-threatening pulmonary hypertension (PH), with a systolic pulmonary artery pressure (PAP) of 80 mmHg and right ventricular hypertrophy, despite optimization of all possible measures and despite extensive therapeutic education efforts. Diaphragm pacing using laparoscopically implanted intradiaphragmatic phrenic nerve stimulation electrodes corrected alveolar hypoventilation and lastingly reversed PH (systolic PAP below 40 mmHg after 2 months, sustained after 2 years). Diaphragm pacing induced shoulder pain, however, involving the chronic use of analgesics. The pacing had to be stopped for tolerance reasons after two years, leading to PH worsening and the need for diurnal MV.
  
  
  CONCLUSIONS
  Diaphragm pacing appears likely effective to restore alveolar ventilation and reverse PH in adult CCHS patients.
authors: Assistance Publique-Hôpitaux de Paris, Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Pneumologie et Réanimation Médicale, Paris, France. capucine.morelot@psl.aphp.fr
authors: Capucine
authors: C
authors: Morélot-Panzini
authors: Jesus
authors: J
authors: Gonzalez-Bermejo
authors: Christian
authors: C
authors: Straus
authors: Thomas
authors: T
authors: Similowski
mesh_terms: D000328:Adult; D000700:Analgesics / Q000627:therapeutic use; D062186:Arterial Pressure; D059350:Chronic Pain / Q000188:drug therapy / Q000209:etiology; D003964:Diaphragm / Q000294:innervation*; D004599:Electric Stimulation Therapy / Q000009:adverse effects / Q000295:instrumentation; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D017380:Hypertrophy, Right Ventricular / Q000209:etiology / Q000503:physiopathology; D007040:Hypoventilation / Q000150:complications / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D058117:Neural Prostheses; D010791:Phrenic Nerve / Q000503:physiopathology*; D011651:Pulmonary Artery / Q000503:physiopathology; D012121:Respiration, Artificial; D020069:Shoulder Pain / Q000188:drug therapy / Q000209:etiology; D020182:Sleep Apnea, Central / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000700:Analgesics