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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Management of respiratory failure &#40;RF&#41; in pulmonary hypertension &#40;PH&#41; is a complex subject&#44; since positive pressure ventilation &#40;PPV&#41; can reduce right ventricular &#40;RV&#41; output&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a> During PPV there is an increase in intrathoracic pressure&#44; increasing right atrial pressure&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> This leads to lower venous return and consequently to decreased RV preload and output&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Furthermore&#44; pulmonary vascular resistance &#40;PVR&#41;&#44; the main determinant of RV afterload&#44; is directly affected by changes in lung volume&#44; since when the lung is hyper-inflated&#44; alveolar distension occurs&#44; leading to compression of the alveolar vessels&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a> On the other hand&#44; low lung volumes result in terminal airway collapse and hypoxic vasoconstriction&#44; and parenchymal vessels also become more tortuous and predisposed to collapse&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a> All the above factors tend to reduce left ventricular output&#44; leading to systemic hypotension&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Protective ventilation may reduce the negative effects of significant changes in lung volume&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Epoprostenol is a potent vasodilator that is used in more severe cases of PH&#46; One of its main side effects is systemic hypotension&#44; which can be enhanced by PPV&#44; as previously described&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Santos et al&#46; recently showed that parenteral prostanoids are underused in group 1 PH&#44; in only 20&#37; of PH patients at the time of death in their study&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> As RF is an important concern in advanced PH&#44; we conducted a retrospective analysis of RF treatment and its impact in advanced PH patients who began epoprostenol as salvage therapy in the intensive care unit of Centro Hospitalar Universit&#225;rio Lisboa Norte&#44; Lisbon&#44; Portugal&#44; over a two-year period&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Ten patients were included&#44; mean age 65 years&#44; 50&#37; female&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Six patients had group 1 PH &#40;four idiopathic PH&#44; one congenital heart disease and one associated with schistosomiasis&#41;&#44; and four had group 4 PH&#46; All presented mean pulmonary artery pressure over 35 mmHg on right heart catheterization &#40;mean 51 mmHg&#41;&#46; Mean estimated pulmonary artery systolic pressure on the last echocardiographic assessment was 97 mmHg&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Most of these patients were in New York Heart Association functional class IV&#44; with worsening RF&#44; and most &#40;60&#37;&#41; were on long-term oxygen therapy&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Previous medication included ambrisentan &#40;four patients&#41;&#44; bosentan &#40;three&#41;&#44; macitentan &#40;two&#41;&#44; sildenafil &#40;five&#41; and riociguat &#40;four&#41;&#46; Epoprostenol was initiated as salvage therapy and dosages were titrated to &#8805;11 ng&#47;kg&#47;min in six patients&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Initially&#44; partial RF was present in eight patients and the other two rapidly developed general RF&#46; Three of the partial RF patients required high-flow oxygen therapy &#40;HFOT&#41; with flow 50 l&#47;min and fraction of inspired oxygen 100&#37;&#46; The two patients with general RF began non-invasive ventilation &#40;NIV&#41; and were titrated to inspiratory positive airway pressure &#40;IPAP&#41; 30 cmH<span class="elsevierStyleInf">2</span>O and expiratory positive airway pressure &#40;EPAP&#41; 10 cmH<span class="elsevierStyleInf">2</span>O in one&#44; and IPAP 24 cmH<span class="elsevierStyleInf">2</span>O and EPAP 12 cmH<span class="elsevierStyleInf">2</span>O in the other &#40;this patient alternated NIV with HFOT&#41;&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">There were four deaths&#44; three due to hemodynamic collapse and one due to nosocomial infection&#46; All of these four patients had group 1 PH&#46; It was not possible to titrate epoprostenol dosage to 11 ng&#47;kg&#47;min in three of the patients who died&#46; The two patients who were on NIV died&#44; as did two of the patients on HFOT due to progression of partial RF&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">This study illustrates the limitations of RF treatment in patients with PH on epoprostenol therapy&#44; particularly those on NIV &#40;due its deleterious hemodynamic effects on already hypotensive patients&#41;&#46; In fact&#44; all patients on NIV died&#44; probably due to underlying disease&#44; severity of RF and worsening of RV failure due to increased positive airway pressure&#46; Of the three patients who were only on HFOT&#44; only one survived&#44; but HFOT may be an interesting option in PH patients&#44; especially at the beginning of RF development&#46; More studies are needed to validate the possible usefulness of HFOT in PH and to establish an appropriate NIV management approach&#44; in particular the use of protective ventilation&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Authorship</span><p id="par0060" class="elsevierStylePara elsevierViewall">Fernanda Paula&#44; Paula Pinto and Filipe Froes participated in the clinical management of the patients&#46; Miguel Filipe Guia analyzed patients&#8217; clinical data&#44; particularly on classification of pulmonary hypertension&#44; oxygen and ventilator strategies&#44; and their outcomes&#46; Miguel Filipe Guia also performed a short review of respiratory failure management in pulmonary hypertension and interpreted the results based on that review&#46; Fernanda Paula&#44; Paula Pinto and Filipe Froes participated in the revision of the present work&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">The present letter has four authors since all four made essential contributions to it&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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Letter to the Editor
Respiratory failure in pulmonary hypertension patients
Insuficiência respiratória na hipertensão pulmonar
Miguel Filipe Guiaa,
Corresponding author
miguelguia7@gmail.com

Corresponding author.
, Fernanda Paulab, Paula Pintob,c, Filipe Froesb,c
a Pulmonology Department, Hospital Professor Doutor Fernando da Fonseca, Amadora, Lisbon, Portugal
b Departamento de Tórax do Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal
c ISAMB, Faculdade de Medicina de Lisboa, Lisbon, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Management of respiratory failure &#40;RF&#41; in pulmonary hypertension &#40;PH&#41; is a complex subject&#44; since positive pressure ventilation &#40;PPV&#41; can reduce right ventricular &#40;RV&#41; output&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a> During PPV there is an increase in intrathoracic pressure&#44; increasing right atrial pressure&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> This leads to lower venous return and consequently to decreased RV preload and output&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Furthermore&#44; pulmonary vascular resistance &#40;PVR&#41;&#44; the main determinant of RV afterload&#44; is directly affected by changes in lung volume&#44; since when the lung is hyper-inflated&#44; alveolar distension occurs&#44; leading to compression of the alveolar vessels&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a> On the other hand&#44; low lung volumes result in terminal airway collapse and hypoxic vasoconstriction&#44; and parenchymal vessels also become more tortuous and predisposed to collapse&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a> All the above factors tend to reduce left ventricular output&#44; leading to systemic hypotension&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Protective ventilation may reduce the negative effects of significant changes in lung volume&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Epoprostenol is a potent vasodilator that is used in more severe cases of PH&#46; One of its main side effects is systemic hypotension&#44; which can be enhanced by PPV&#44; as previously described&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Santos et al&#46; recently showed that parenteral prostanoids are underused in group 1 PH&#44; in only 20&#37; of PH patients at the time of death in their study&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> As RF is an important concern in advanced PH&#44; we conducted a retrospective analysis of RF treatment and its impact in advanced PH patients who began epoprostenol as salvage therapy in the intensive care unit of Centro Hospitalar Universit&#225;rio Lisboa Norte&#44; Lisbon&#44; Portugal&#44; over a two-year period&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Ten patients were included&#44; mean age 65 years&#44; 50&#37; female&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Six patients had group 1 PH &#40;four idiopathic PH&#44; one congenital heart disease and one associated with schistosomiasis&#41;&#44; and four had group 4 PH&#46; All presented mean pulmonary artery pressure over 35 mmHg on right heart catheterization &#40;mean 51 mmHg&#41;&#46; Mean estimated pulmonary artery systolic pressure on the last echocardiographic assessment was 97 mmHg&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Most of these patients were in New York Heart Association functional class IV&#44; with worsening RF&#44; and most &#40;60&#37;&#41; were on long-term oxygen therapy&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Previous medication included ambrisentan &#40;four patients&#41;&#44; bosentan &#40;three&#41;&#44; macitentan &#40;two&#41;&#44; sildenafil &#40;five&#41; and riociguat &#40;four&#41;&#46; Epoprostenol was initiated as salvage therapy and dosages were titrated to &#8805;11 ng&#47;kg&#47;min in six patients&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Initially&#44; partial RF was present in eight patients and the other two rapidly developed general RF&#46; Three of the partial RF patients required high-flow oxygen therapy &#40;HFOT&#41; with flow 50 l&#47;min and fraction of inspired oxygen 100&#37;&#46; The two patients with general RF began non-invasive ventilation &#40;NIV&#41; and were titrated to inspiratory positive airway pressure &#40;IPAP&#41; 30 cmH<span class="elsevierStyleInf">2</span>O and expiratory positive airway pressure &#40;EPAP&#41; 10 cmH<span class="elsevierStyleInf">2</span>O in one&#44; and IPAP 24 cmH<span class="elsevierStyleInf">2</span>O and EPAP 12 cmH<span class="elsevierStyleInf">2</span>O in the other &#40;this patient alternated NIV with HFOT&#41;&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">There were four deaths&#44; three due to hemodynamic collapse and one due to nosocomial infection&#46; All of these four patients had group 1 PH&#46; It was not possible to titrate epoprostenol dosage to 11 ng&#47;kg&#47;min in three of the patients who died&#46; The two patients who were on NIV died&#44; as did two of the patients on HFOT due to progression of partial RF&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">This study illustrates the limitations of RF treatment in patients with PH on epoprostenol therapy&#44; particularly those on NIV &#40;due its deleterious hemodynamic effects on already hypotensive patients&#41;&#46; In fact&#44; all patients on NIV died&#44; probably due to underlying disease&#44; severity of RF and worsening of RV failure due to increased positive airway pressure&#46; Of the three patients who were only on HFOT&#44; only one survived&#44; but HFOT may be an interesting option in PH patients&#44; especially at the beginning of RF development&#46; More studies are needed to validate the possible usefulness of HFOT in PH and to establish an appropriate NIV management approach&#44; in particular the use of protective ventilation&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Authorship</span><p id="par0060" class="elsevierStylePara elsevierViewall">Fernanda Paula&#44; Paula Pinto and Filipe Froes participated in the clinical management of the patients&#46; Miguel Filipe Guia analyzed patients&#8217; clinical data&#44; particularly on classification of pulmonary hypertension&#44; oxygen and ventilator strategies&#44; and their outcomes&#46; Miguel Filipe Guia also performed a short review of respiratory failure management in pulmonary hypertension and interpreted the results based on that review&#46; Fernanda Paula&#44; Paula Pinto and Filipe Froes participated in the revision of the present work&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">The present letter has four authors since all four made essential contributions to it&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Conflicts of interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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Revista Portuguesa de Cardiologia (English edition)
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