Right Ventricular Dysfunction in Adult Severe Cystic Fibrosis

Florea, Viorel G.; Florea, Natalia; Sharma, Rakesh; Coats, Andrew J.S.; Gibson, D G; Hodson, Margaret E.; Henein, Michael Y.

doi:10.1378/chest.118.4.1063

Cited by 68 publications

(53 citation statements)

References 25 publications

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“…PHT develops secondary to primary diseases of the lung as a consequence of the pulmonary vasoconstriction caused by chronic hypoxia (18). Supporting the other studies of patients with CF, we observed a strong inverse correlation between signs of PHT (pulmonary blood flow acceleration time and RV dimension) and PaO 2 and SaO 2 (10,12). In previous studies in patients with CF, the correlation between PHT and PaCO 2 has been inconsistent (12), but we observed that PaCO 2 was correlated negatively with pulmonary blood flow acceleration time and positively with the RV dimension.…”

Section: Discussionsupporting

confidence: 89%

“…At present, assessment of right-sided cardiac function is a relatively easy process since the pulmonary artery systolic pressure (PASP) can be measured from the tricuspid regurgitant jet velocity using noninvasive Doppler echocardiography (38). More recent studies have demonstrated the usefulness of this noninvasive technique in the assessment of PHT in adult patients with CF and severe lung disease (10,12,22).…”

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confidence: 99%

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Burkholderia cepaciaIs Associated with Pulmonary Hypertension and Increased Mortality among Cystic Fibrosis Patients

et al. 2004

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The aim of the study was to evaluate the impact of Burkholderia cepacia on cardiovascular status and mortality in cystic fibrosis. Seven patients infected with B. cepacia were matched with 31 patients not infected with this organism for gender, age, height, weight, genotype, and percent predicted forced expiratory volume in one second, partial arterial oxygen pressure, and pancreatic sufficiency status. The pulmonary artery systolic pressure, as assessed by transthoracic echocardiography, was significantly higher in patients infected with B. cepacia (61.3 ؎ 17.2 mm Hg) than in controls (37.3 ؎ 13.9 mm Hg; P ‫؍‬ 0.02), and the mean acceleration time was significantly lower (77 ؎ 33 ms versus 108 ؎ 25 ms; P ‫؍‬ 0.02). The 6-month mortality was significantly higher in patients infected with B. cepacia (57% versus 16%; P ‫؍‬ 0.02). Six of the seven patients infected with B. cepacia harbored the same ribotype (genomovar II, B. multivorans). Pulmonary hypertension was significantly more frequent in patients infected by B. cepacia and could contribute to the increased mortality rate.

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Section: Discussionsupporting

confidence: 89%

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confidence: 99%

Burkholderia cepaciaIs Associated with Pulmonary Hypertension and Increased Mortality among Cystic Fibrosis Patients

et al. 2004

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“…The fact that CF is associated with a strong impact on Ppa and right ventricle function had long been demonstrated [6][7][8], bearing a strongly negative prognostic value [10,12]. Their exact frequency is not known.…”

Section: Discussionmentioning

confidence: 99%

“…The reasons for this choice were as follows. First, CF is a widely distributed chronic obstructive lung disease in which PH and right-sided cardiac failure have long been described [6][7][8][9]. Although PH seems to be relatively rare in this context, it is recognised as a very significant clinical issue with a heavy impact on the prognosis [10][11][12].…”

mentioning

confidence: 99%

Pulmonary vascular dysfunction in end-stage cystic fibrosis: role of NF-κB and endothelin-1

Henno¹,

Maurey²,

Danel³

et al. 2009

Eur Respir J

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Pulmonary hypertension is rare in chronic respiratory diseases but has a strong impact on the prognosis and is partly underlined by factors other than hypoxaemia. The aim of the present study was to assess the potential role of endothelin-1 (ET-1) and nuclear factor (NF)-kB vasoconstrictive pathways in pulmonary hypertension.The effects of ET-1 receptors blockers (BQ 123 and 788) and of genistein were assessed on response to acetylcholine of pulmonary vascular rings from cystic fibrosis (CF) lung transplant recipients (n523). NF-kB and ET-1 receptor expression was immunodetected in pulmonary arteries and quantitated using Western blotting. ET-1 vascular content was quantitated using ELISA.In total, 14 out of 23 subjects exhibited strongly impaired pulmonary vasodilation (p,0.01 versus nine out of 23 subjects with a normal response) associated with an activation of ET-1 receptors A and NF-kB pathways. Genistein restored vasodilation in subjects with an abnormal response.Pulmonary vascular dysfunction is frequent in end-stage CF, involving the NF-kB pathway and that of ET-1 through ET-1 receptor A (ETAR). These data leave a conceptual place for ETAR blockers and isoflavones in the management of the devastating vascular complication of chronic obstructive respiratory diseases such as CF.

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“…All invasive and noninvasive techniques evaluating RV performance have important limitations due to the complex geometry of the right ventricle [3][4][5][6][7][8][9][10][11][12][13][14][15]. The introduction of Doppler measurement of myocardial wall velocities (tissue Doppler imaging; TDI) and the recently developed strain rate imaging (SRI) technique have made a more adequate assessment of global and regional systolic and diastolic RV function possible [16][17][18][19][20][21][22][23].…”

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confidence: 99%

Assessment of right ventricular function by strain rate imaging in chronic obstructive pulmonary disease

et al. 2006

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The purpose of the current study was to compare right ventricular (RV) myocardial wall velocities (tissue Doppler imaging) and strain rate imaging (SRI) parameters with conventional echocardiographic indices evaluating RV function in chronic obstructive pulmonary disease (COPD) patients.In total, 39 patients with COPD and 22 healthy subjects were included in the current study. Seventeen patients had pulmonary artery pressure ,35 mmHg (group I) and 22 patients had pulmonary artery pressure .35 mmHg (group II). Tissue Doppler imaging, strain and strain rate (SR) values were obtained from RV free wall (FW) and interventricular septum. Respiratory function tests were performed (forced expiratory volume in one second/vital capacity (FEV1/VC) and carbon monoxide diffusion lung capacity per unit of alveolar volume (DL,CO/VA)).Strain/SR values were reduced in all segments of group II patients compared with group I patients and controls with lowest values at basal FW site. A significant relationship was shown between peak systolic SR at basal FW site and radionuclide RV ejection fraction. A significant relationship was shown between peak systolic SR at basal FW site and DL,CO/VA and FEV1/VC.In conclusion, in chronic obstructive pulmonary disease patients, strain rate imaging parameters can determine right ventricular dysfunction that is complementary to conventional echocardiographic indices and is correlated with pulmonary hypertension and respiratory function tests.

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Right Ventricular Dysfunction in Adult Severe Cystic Fibrosis

Cited by 68 publications

References 25 publications

Burkholderia cepaciaIs Associated with Pulmonary Hypertension and Increased Mortality among Cystic Fibrosis Patients

Burkholderia cepaciaIs Associated with Pulmonary Hypertension and Increased Mortality among Cystic Fibrosis Patients

Pulmonary vascular dysfunction in end-stage cystic fibrosis: role of NF-κB and endothelin-1

Assessment of right ventricular function by strain rate imaging in chronic obstructive pulmonary disease

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