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Bosentan for pulmonary hypertension secondary to idiopathic pulmonary fibrosis.

Onda N, Tanaka Y, Hino M, Gemma A - Respir Med Case Rep (2014)

Bottom Line: We describe the case of an 81-year-old woman with idiopathic pulmonary fibrosis (IPF), who was admitted to our hospital due to aggravation of dyspnea and decreased oxygen saturation, as well as onset of orthopnea and rapidly progressing edema.Diuretic therapy was started, but the patient's condition showed poor improvement.Bosentan appears to be efficacious in some patients with pulmonary hypertension secondary to idiopathic interstitial pneumonitis.

View Article: PubMed Central - PubMed

Affiliation: Nippon Medical School, Chiba Hokusoh Hospital, Respiratory Disease Center, Japan.

ABSTRACT
Pulmonary hypertension is a poor prognostic factor in patients with interstitial lung disease. No established treatment exists for pulmonary hypertension secondary to interstitial pneumonia. We describe the case of an 81-year-old woman with idiopathic pulmonary fibrosis (IPF), who was admitted to our hospital due to aggravation of dyspnea and decreased oxygen saturation, as well as onset of orthopnea and rapidly progressing edema. The transthoracic echocardiography and right heart catheterization showed the mean pulmonary artery pressure was 39 mmHg and the mean pulmonary capillary wedge pressure was 9 mmHg. After various examinations, the diagnoses of pulmonary hypertension (PH) due to IPF and of congestive heart failure secondary to PH were established. Diuretic therapy was started, but the patient's condition showed poor improvement. Subsequent initiation of oral bosentan therapy led to improvement in symptoms and findings. At the follow-up assessment one year later her pulmonary function showed no significant changes and no apparent worsening of arterial blood gases, with evident improvement of PH, WHO functional class, maximum exercise tolerance on treadmill exercise testing, right heart catheterization, and transthoracic echocardiography. This report describes a case of successful treatment with bosentan for severe pulmonary hypertension in a patient with idiopathic pulmonary fibrosis. We also present a review of the literature on treatment of pulmonary hypertension in patients with chronic lung disease. Bosentan appears to be efficacious in some patients with pulmonary hypertension secondary to idiopathic interstitial pneumonitis.

No MeSH data available.


Related in: MedlinePlus

Images from transthoracic echocardiography. (a). Transthoracic echocardiography after admission in Oct. 2013 Upper panels: The right ventricle was enlarged and the interventricular septum was straightened or bowed into the left ventricle. Middle panels: Severe tricuspid regurgitation (TR) was detected. TR pressure gradient was 86.6 mmHg Lower panel: Mild pulmonary regurgitation (PR) was detected. PR pressure gradient was 16.1 mmHg Total ejection isovolumetric (Tei) index was 0.44 and tricuspid annular plane systolic excursion (TAPSE) was 1.13 cm (b). Transthoracic echocardiography at 1 year from start of bosentan therapy Upper panels: Findings improved compared with Fig. 2a. Middle panels: Severe TR was noted. TR pressure gradient was 48.2 mmHg Lower panel: Mild PR was noted. PR pressure gradient was too small to measure. Tei index was 0.40 and tricuspid annular plane systolic excursion (TAPSE) was 1.6 cm.
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fig2: Images from transthoracic echocardiography. (a). Transthoracic echocardiography after admission in Oct. 2013 Upper panels: The right ventricle was enlarged and the interventricular septum was straightened or bowed into the left ventricle. Middle panels: Severe tricuspid regurgitation (TR) was detected. TR pressure gradient was 86.6 mmHg Lower panel: Mild pulmonary regurgitation (PR) was detected. PR pressure gradient was 16.1 mmHg Total ejection isovolumetric (Tei) index was 0.44 and tricuspid annular plane systolic excursion (TAPSE) was 1.13 cm (b). Transthoracic echocardiography at 1 year from start of bosentan therapy Upper panels: Findings improved compared with Fig. 2a. Middle panels: Severe TR was noted. TR pressure gradient was 48.2 mmHg Lower panel: Mild PR was noted. PR pressure gradient was too small to measure. Tei index was 0.40 and tricuspid annular plane systolic excursion (TAPSE) was 1.6 cm.

Mentions: Exacerbation of IPF was unlikely because the only significant finding from imaging examination and blood tests was elevated KL-6 that was attributed to influence of diuretic therapy given since before her admission and influence of congestion (Table 1 and Fig. 1b). In addition, after admission, transthoracic echocardiography and right heart catheterization were performed. The transthoracic echocardiographic finding suggested severe PH and a congestive condition (TR pressure gradient was 86.6 mmHg and PR pressure gradient was 16.1 mmHg total ejection isovolumetric (Tei) index was 0.44 and tricuspid annular plane systolic excursion (TAPSE) was 1.13 cm). (Fig. 2a).


Bosentan for pulmonary hypertension secondary to idiopathic pulmonary fibrosis.

Onda N, Tanaka Y, Hino M, Gemma A - Respir Med Case Rep (2014)

Images from transthoracic echocardiography. (a). Transthoracic echocardiography after admission in Oct. 2013 Upper panels: The right ventricle was enlarged and the interventricular septum was straightened or bowed into the left ventricle. Middle panels: Severe tricuspid regurgitation (TR) was detected. TR pressure gradient was 86.6 mmHg Lower panel: Mild pulmonary regurgitation (PR) was detected. PR pressure gradient was 16.1 mmHg Total ejection isovolumetric (Tei) index was 0.44 and tricuspid annular plane systolic excursion (TAPSE) was 1.13 cm (b). Transthoracic echocardiography at 1 year from start of bosentan therapy Upper panels: Findings improved compared with Fig. 2a. Middle panels: Severe TR was noted. TR pressure gradient was 48.2 mmHg Lower panel: Mild PR was noted. PR pressure gradient was too small to measure. Tei index was 0.40 and tricuspid annular plane systolic excursion (TAPSE) was 1.6 cm.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

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Show All Figures
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fig2: Images from transthoracic echocardiography. (a). Transthoracic echocardiography after admission in Oct. 2013 Upper panels: The right ventricle was enlarged and the interventricular septum was straightened or bowed into the left ventricle. Middle panels: Severe tricuspid regurgitation (TR) was detected. TR pressure gradient was 86.6 mmHg Lower panel: Mild pulmonary regurgitation (PR) was detected. PR pressure gradient was 16.1 mmHg Total ejection isovolumetric (Tei) index was 0.44 and tricuspid annular plane systolic excursion (TAPSE) was 1.13 cm (b). Transthoracic echocardiography at 1 year from start of bosentan therapy Upper panels: Findings improved compared with Fig. 2a. Middle panels: Severe TR was noted. TR pressure gradient was 48.2 mmHg Lower panel: Mild PR was noted. PR pressure gradient was too small to measure. Tei index was 0.40 and tricuspid annular plane systolic excursion (TAPSE) was 1.6 cm.
Mentions: Exacerbation of IPF was unlikely because the only significant finding from imaging examination and blood tests was elevated KL-6 that was attributed to influence of diuretic therapy given since before her admission and influence of congestion (Table 1 and Fig. 1b). In addition, after admission, transthoracic echocardiography and right heart catheterization were performed. The transthoracic echocardiographic finding suggested severe PH and a congestive condition (TR pressure gradient was 86.6 mmHg and PR pressure gradient was 16.1 mmHg total ejection isovolumetric (Tei) index was 0.44 and tricuspid annular plane systolic excursion (TAPSE) was 1.13 cm). (Fig. 2a).

Bottom Line: We describe the case of an 81-year-old woman with idiopathic pulmonary fibrosis (IPF), who was admitted to our hospital due to aggravation of dyspnea and decreased oxygen saturation, as well as onset of orthopnea and rapidly progressing edema.Diuretic therapy was started, but the patient's condition showed poor improvement.Bosentan appears to be efficacious in some patients with pulmonary hypertension secondary to idiopathic interstitial pneumonitis.

View Article: PubMed Central - PubMed

Affiliation: Nippon Medical School, Chiba Hokusoh Hospital, Respiratory Disease Center, Japan.

ABSTRACT
Pulmonary hypertension is a poor prognostic factor in patients with interstitial lung disease. No established treatment exists for pulmonary hypertension secondary to interstitial pneumonia. We describe the case of an 81-year-old woman with idiopathic pulmonary fibrosis (IPF), who was admitted to our hospital due to aggravation of dyspnea and decreased oxygen saturation, as well as onset of orthopnea and rapidly progressing edema. The transthoracic echocardiography and right heart catheterization showed the mean pulmonary artery pressure was 39 mmHg and the mean pulmonary capillary wedge pressure was 9 mmHg. After various examinations, the diagnoses of pulmonary hypertension (PH) due to IPF and of congestive heart failure secondary to PH were established. Diuretic therapy was started, but the patient's condition showed poor improvement. Subsequent initiation of oral bosentan therapy led to improvement in symptoms and findings. At the follow-up assessment one year later her pulmonary function showed no significant changes and no apparent worsening of arterial blood gases, with evident improvement of PH, WHO functional class, maximum exercise tolerance on treadmill exercise testing, right heart catheterization, and transthoracic echocardiography. This report describes a case of successful treatment with bosentan for severe pulmonary hypertension in a patient with idiopathic pulmonary fibrosis. We also present a review of the literature on treatment of pulmonary hypertension in patients with chronic lung disease. Bosentan appears to be efficacious in some patients with pulmonary hypertension secondary to idiopathic interstitial pneumonitis.

No MeSH data available.


Related in: MedlinePlus