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Efficacy of Noninvasive Ventilation in a Patient with Hypercapnic Respiratory Failure Complicating Eisenmenger’s Syndrome [Eurasian J Pulmonol]
Eurasian J Pulmonol. 2017; 19(2): 112-114 | DOI: 10.5152/ejp.2017.82905  

Efficacy of Noninvasive Ventilation in a Patient with Hypercapnic Respiratory Failure Complicating Eisenmenger’s Syndrome

Ana Jaureguizar Oriol, Salvador Díaz- Lobato, Francisco León Román, Sagrario Mayoralas Alises
Department of Respiratory Diseases, Ramón Y Cajal University Hospital, Madrid, Spain

Eisenmenger’s syndrome is a severe type of congenital heart disease characterized by severe pulmonary arterial hypertension. In the cases that the pressure in pulmonary circulation exceeds the systemic pressure, there appears a right-to-left shunting of blood. Consequently, the syndrome exists hypoxemia and cyanosis. Hypercapnia is not common in these patients; however, it might coexist with hypoxemic failure if there are other restrictive pathologies associated. Meanwhile, it has been described high prevalence of sleep disorders in Down syndrome. There is no evidence about the role of noninvasive ventilation in the management of these patients. We present a 39-year-old man, suffering of Down and Eisenmenger’s syndrome with multiple cardiac decompensations and obstructive sleep apnea (OSA), who was admitted to hospital due to severe somnolence, edema, and dyspnea. We observed a hypercapnic respiratory acidosis that ameliorated with noninvasive ventilation (NIV). The patient returned home with nocturnal NIV as a new treatment, and no further admission to hospital was seen in the following two years. To our knowledge, this is the first report about the utility of NIV in Eisenmenger’s and Down syndrome patients.

Keywords: Congenital diseases, Eisenmenger’s syndrome, hypercapnic respiratory, failure, hypoxemia, noninvasive ventilation


Ana Jaureguizar Oriol, Salvador Díaz- Lobato, Francisco León Román, Sagrario Mayoralas Alises. Efficacy of Noninvasive Ventilation in a Patient with Hypercapnic Respiratory Failure Complicating Eisenmenger’s Syndrome. Eurasian J Pulmonol. 2017; 19(2): 112-114

Corresponding Author: Ana Jaureguizar Oriol, Spain


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