Literature DB >> 11035678

Right ventricular dysfunction in adult severe cystic fibrosis.

V G Florea1, N D Florea, R Sharma, A J Coats, D G Gibson, M E Hodson, M Y Henein.   

Abstract

STUDY
OBJECTIVES: This study sought to assess the extent of impairment of cardiac function in adult patients with end-stage cystic fibrosis (CF) and to examine the relationship between cardiovascular abnormalities and the degree of hypoxemia and hypercapnia. DESIGN AND
SETTING: A retrospective study in a tertiary cardiac and CF center. PARTICIPANTS AND
INTERVENTIONS: A total of 103 adult patients with end-stage CF awaiting lung or heart and lung transplantation (mean age [+/- SD], 26+/-7 years; 54 men) underwent Doppler echocardiography and arterial blood gas analysis (mean PaO(2), 54+/-10 mm Hg; mean PaCO(2), 47+/-8 mm Hg). The findings were compared to those of 17 healthy control subjects (mean age, 24+/-7 years; 13 men) who had no history of cardiac or pulmonary disease. MEASUREMENTS AND
RESULTS: All patients were in sinus rhythm with a mean tachycardia of 112+/-18 beats/min (control subjects, 76+/-16; p<0.0001) and had a cardiac output of 5.3 L/min (control subjects, 4.3 L/min; p<0.04). In the patient group, the left ventricular (LV) dimensions, systolic and diastolic function, and wall thickness were all within normal limits. The mean amplitude of long-axis excursion in patients was normal at the LV site, but that of the right ventricular (RV) free wall was significantly reduced as compared with control subjects (1.6+/-0.4 vs. 2.2+/-0.4 cm, respectively; p<0.001), which was found to correlate with the degree of hypoxemia (r = 0.63; p<0.02) and hypercapnia (r = -0.68; p<0.01). RV diastolic function, which was represented by the relative isovolumic relaxation time to cardiac cycle length, was longer in patients than in control subjects (8.7+/-4.8% vs. 5.0+/-3.0%, respectively; p<0.03). The pulmonary flow acceleration time (90+/-22 vs 121+/-34 ms, respectively; p<0.01) and the systolic stroke distance (7.0+/-2.2 vs. 10.5+/-1.9 cm/s(2); p<0.001) were both lower than normal.
CONCLUSIONS: This study confirms the presence of significant RV systolic and diastolic dysfunction in the setting of consistent tachycardia and increased cardiac output in adult CF patients with severe disease. No specific LV abnormalities were detected in these patients.

Entities:  

Mesh:

Year:  2000        PMID: 11035678     DOI: 10.1378/chest.118.4.1063

Source DB:  PubMed          Journal:  Chest        ISSN: 0012-3692            Impact factor:   9.410


  14 in total

Review 1.  The heart in cystic fibrosis.

Authors:  Rowland J Bright-Thomas; A Kevin Webb
Journal:  J R Soc Med       Date:  2002       Impact factor: 5.344

2.  Ischaemic heart disease--a new issue in cystic fibrosis?

Authors:  Felicity M R Perrin; Walter Serino
Journal:  J R Soc Med       Date:  2010-07       Impact factor: 5.344

3.  Cardiomyocytes with disrupted CFTR function require CaMKII and Ca(2+)-activated Cl(-) channel activity to maintain contraction rate.

Authors:  Zachary M Sellers; Vania De Arcangelis; Yang Xiang; Philip M Best
Journal:  J Physiol       Date:  2010-05-04       Impact factor: 5.182

4.  Polysomnographic differences associated with pulmonary hypertension in patients with advanced lung disease due to cystic fibrosis.

Authors:  Don Hayes; Curt J Daniels; Stephen Kirkby; Benjamin T Kopp; Kerri L Nicholson; Ashley E Nance; Mark L Splaingard
Journal:  Lung       Date:  2014-03-27       Impact factor: 2.584

5.  Focus on echocardiographic right ventricular strain analysis in cystic fibrosis adults without cardiovascular risk factors: a case-control study.

Authors:  Edoardo Sciatti; Enrico Vizzardi; Ivano Bonadei; Francesca Valentini; Elisa Menotti; Francesco Prati; Lucia Dallapellegrina; Marialma Berlendis; Piercarlo Poli; Rita Padoan; Marco Metra
Journal:  Intern Emerg Med       Date:  2019-05-14       Impact factor: 3.397

6.  Burkholderia cepacia is associated with pulmonary hypertension and increased mortality among cystic fibrosis patients.

Authors:  Brigitte Fauroux; Nicholas Hart; Samira Belfar; Michèle Boulé; Isabelle Tillous-Borde; Damien Bonnet; Edouard Bingen; Annick Clément
Journal:  J Clin Microbiol       Date:  2004-12       Impact factor: 5.948

7.  Shortness of breath in a CF patient: not always the chest.

Authors:  Mark Ainslie; Roland Bright-Thomas
Journal:  BMJ Case Rep       Date:  2011-11-15

Review 8.  Growth hormone and exercise tolerance in patients with cystic fibrosis.

Authors:  Matthias Hütler; Ralph Beneke
Journal:  Sports Med       Date:  2004       Impact factor: 11.136

Review 9.  RV diastolic dysfunction: time to re-evaluate its importance in heart failure.

Authors:  Richard G Axell; Stephen P Hoole; James Hampton-Till; Paul A White
Journal:  Heart Fail Rev       Date:  2015-05       Impact factor: 4.214

10.  The Impact of Pulmonary Arterial Pressure on Exercise Capacity in Mild-to-Moderate Cystic Fibrosis: A Case Control Study.

Authors:  Katerina Manika; Georgia G Pitsiou; Afroditi K Boutou; Vassilis Tsaoussis; Nikolaos Chavouzis; Marina Antoniou; Maria Fotoulaki; Ioannis Stanopoulos; Ioannis Kioumis
Journal:  Pulm Med       Date:  2012-07-29
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