Left Ventricular Diastolic Function in Patients With Advanced Cystic Fibrosis

Koelling, Todd M.; Dec, G. William; Ginns, Leo C.; Semigran, Marc J.

doi:10.1378/chest.123.5.1488

Cited by 36 publications

(46 citation statements)

References 25 publications

(24 reference statements)

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“…Nevertheless, these findings suggest that abnormal LV performance may be due to ventricular interdependence, in which RV dilatation leads to bulging of the septum into the LV, which in turn increases LVEDD, decreases LV performance, and alters left atrium mechanics. This is in agreement with previous reports [28,29] on the cause of LV diastolic dysfunction in patients with PH.…”

supporting

confidence: 94%

“…Vizza et al [27] examined RV and LV function in patients with severe airway, parenchymal, and pulmonary vessel disease; the presence of RV dysfunction varied between 59% and 94%. Koelling et al [28] observed that RV systolic function at rest and after peak exercise was similarly compromised in 40 patients with advanced cystic fibrosis, as well as in 9 patients with moderately severe bronchiectasis. They also noticed abnormal diastolic function in patients with cystic fibrosis compared with control subjects or patients with bronchiectasis.…”

mentioning

confidence: 99%

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Impact of Cystic versus Cylindrical Types of Bronchiectasis on Pulmonary and Cardiac Functions

Hassan¹,

Ziada²,

El-hamd³

2014

OJRD

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Background: The combination of the clinical features, HRCT score and echocardiographic evidence of pulmonary hypertension help assess the prognosis in bronchiectasis. Aim: To test whether pulmonary and cardiac functions associated with bronchiectasis would differ according to its type (cystic versus cylindrical) utilizing HRCT score, PFTs and echocardiography. Patients and Methods: A cross-sectional study of patients with bronchiectasis was conducted at Chest and Internal Medicine Departments at Assiut University Hospital, Egypt. The diagnosis of bronchiectasis type was based on HRCT findings. PFTs, HRCT score and echocardiography were assessed in all cases. Results: We studied 56 patients with bronchiectasis; 31 were cystic (group A) and 25 were cylindrical (group B). Forced vital capacity (FVC%) and Dlco% were significantly lower in group A (P < 0.0001) as compared with group B; whereas FEF 25% -75% and FEV1 (0.04) were lower in group B. Global HRCT score, RVD and SPAP were significantly higher in group A (P = 0.002) and correlated with FEV1% (r = −0.51), and with SPAP (r = 0.16). Conclusions: Airway obstruction and small airway dysfunction were more significantly seen in cylindrical bronchiectasis. Patients with cystic bronchiectasis had significantly, higher global HRCT scores, RVD and SPAP. HRCT scores correlate with FEV 1% and SPAP and could be a predictor of future PH. Routine echocardiographic assessment of patients with bronchiectasis, particularly in those with cystic disease is highly recommended.

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supporting

confidence: 94%

mentioning

confidence: 99%

Impact of Cystic versus Cylindrical Types of Bronchiectasis on Pulmonary and Cardiac Functions

Hassan¹,

Ziada²,

El-hamd³

2014

OJRD

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“…Although cystic fibrosis is associated with cardiac impairment, 30,31 it is not clinically associated with hypertension, a systemic effect that would be predicted as a consequence of S1P-dependent augmentation of myogenic tone and hence peripheral resistance. However, mean arterial pressure measurements integrate several factors and mechanisms (eg, blood volume, cardiac output, total peripheral resistance).…”

Section: Discussionmentioning

confidence: 98%

“…However, mean arterial pressure measurements integrate several factors and mechanisms (eg, blood volume, cardiac output, total peripheral resistance). In addition to cardiac dysfunction, 30,31 patients with cystic fibrosis are known to have increased sweat salt loss 32 ; both are confounding factors that could ameliorate the effects of increased resistance artery tone. Nevertheless, our findings align well with previous studies reporting altered lipid metabolism in patients with cystic fibrosis (eg, accumulation of ceramide, 33 a lipid mediator involved in the sphingolipid rheostat 1 ).…”

Section: Discussionmentioning

confidence: 99%

Tumor Necrosis Factor-α–Mediated Downregulation of the Cystic Fibrosis Transmembrane Conductance Regulator Drives Pathological Sphingosine-1-Phosphate Signaling in a Mouse Model of Heart Failure

Meißner¹,

Yang²,

Kroetsch³

et al. 2012

Circulation

150

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Background-Sphingosine-1-phosphate (S1P) signaling is a central regulator of resistance artery tone. Therefore, S1P levels need to be tightly controlled through the delicate interplay of its generating enzyme sphingosine kinase 1 and its functional antagonist S1P phosphohydrolase-1. The intracellular localization of S1P phosphohydrolase-1 necessitates the import of extracellular S1P into the intracellular compartment before its degradation. The present investigation proposes that the cystic fibrosis transmembrane conductance regulator transports extracellular S1P and hence modulates microvascular S1P signaling in health and disease. Methods and Results-In cultured murine vascular smooth muscle cells in vitro and isolated murine mesenteric and posterior cerebral resistance arteries ex vivo, the cystic fibrosis transmembrane conductance regulator (1) is critical for S1P uptake; (2) modulates S1P-dependent responses; and (3) is downregulated in vitro and in vivo by tumor necrosis factor-␣, with significant functional consequences for S1P signaling and vascular tone. In heart failure, tumor necrosis factor-␣ downregulates the cystic fibrosis transmembrane conductance regulator across several organs, including the heart, lung, and brain, suggesting that it is a fundamental mechanism with implications for systemic S1P effects. Conclusions-We identify the cystic fibrosis transmembrane conductance regulator as a critical regulatory site for S1P signaling; its tumor necrosis factor-␣-dependent downregulation in heart failure underlies an enhancement in microvascular tone. This molecular mechanism potentially represents a novel and highly strategic therapeutic target for cardiovascular conditions involving inflammation. (Circulation. 2012;125:2739-2750.)Key Words: acute myocardial infarction Ⅲ hemodynamics Ⅲ myogenic vasoconstriction Ⅲ signal transduction Ⅲ vasomotor tone S phingosine-1-phosphate (S1P) is a ubiquitous signaling mediator that directs a diverse array of biological processes. 1 In the microcirculation, S1P is a potent vasoconstrictor and a central mediator regulating myogenic tone. [2][3][4][5] This confers S1P signaling with significant importance in the control of blood flow autoregulation, tissue perfusion, and systemic blood pressure. Editorial see p 2692 Clinical Perspective on p 2750The potent and pleiotropic effects of S1P are confined both spatially and temporally 6 ; however, the molecular mechanisms limiting S1P bioavailability are not completely understood. We have demonstrated that S1P phosphohydrolase-1 (SPP1), an intracellular enzyme primarily localized to the endoplasmic reticulum, 7,8 degrades extracellular S1P. 3 As a consequence, we concluded that an S1P "import" mechanism must be present in vascular smooth muscle cells. Boujaoude et al 9 have provided indirect evidence that the cystic fibrosis transmembrane conductance regulator (CFTR) could act as this S1P transporter and thereby limit S1P receptor-mediated effects. Accordingly, we have observed that CFTR inhibition specifically enhance...

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“…In some patients, cardiovascular abnormalities might play a role, as a decreased left ventricular diastolic function (in patients with advanced disease) and lower stroke volume for a given V O 2 have been reported [28,29]. …”

Section: Reasons For a Reduced Exercise Capacitymentioning

confidence: 99%

Rehabilitation in Patients with Chronic Respiratory Disease Other than Chronic Obstructive Pulmonary Disease: Exercise and Physical Activity Interventions in Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

Burtin

Hebestreit

2015

Respiration

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A relevant proportion of children and adults with cystic fibrosis (CF) have a marked decrease in exercise tolerance, which can be partly related to impaired muscle function and decreased physical activity levels in daily life, in addition to lung disease. Preliminary findings suggest that patients with non-CF bronchiectasis face the same problems. These patients might be excellent candidates for exercise and physical activity interventions. This review elaborates on the rationale for exercise training and activity behaviour changes and summarizes the existing evidence for these rehabilitation strategies in patients with bronchiectasis, both CF and non-CF bronchiectasis. Furthermore, practical considerations and safety aspects are discussed.

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Left Ventricular Diastolic Function in Patients With Advanced Cystic Fibrosis

Cited by 36 publications

References 25 publications

Impact of Cystic versus Cylindrical Types of Bronchiectasis on Pulmonary and Cardiac Functions

Impact of Cystic versus Cylindrical Types of Bronchiectasis on Pulmonary and Cardiac Functions

Tumor Necrosis Factor-α–Mediated Downregulation of the Cystic Fibrosis Transmembrane Conductance Regulator Drives Pathological Sphingosine-1-Phosphate Signaling in a Mouse Model of Heart Failure

Rehabilitation in Patients with Chronic Respiratory Disease Other than Chronic Obstructive Pulmonary Disease: Exercise and Physical Activity Interventions in Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

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