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Right Ventricular Dysfunction in Adult Severe Cystic Fibrosis

2000; Elsevier BV; Volume: 118; Issue: 4 Linguagem: Inglês

10.1378/chest.118.4.1063

1931-3543

Viorel G. Florea, N Florea, Rakesh Kumar Sharma, Andrew J.S. Coats, Derek G. Gibson, Margaret E. Hodson, Michael Y. Henein,

Tracheal and airway disorders

This study sought to assess theextent of impairment of cardiac function in adult patients withend-stage cystic fibrosis (CF) and to examine the relationship betweencardiovascular abnormalities and the degree of hypoxemia andhypercapnia. A retrospective studyin a tertiary cardiac and CF center. A total of 103 adult patients with end-stage CFawaiting lung or heart and lung transplantation (mean age [± SD],26 ± 7 years; 54 men) underwent Doppler echocardiography andarterial blood gas analysis (mean Pao2,54 ± 10 mm Hg; mean Paco2, 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 orpulmonary disease. Allpatients were in sinus rhythm with a mean tachycardia of112 ± 18 beats/min (control subjects, 76 ± 16; p < 0.0001) andhad 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 wereall within normal limits. The mean amplitude of long-axisexcursion in patients was normal at the LV site, but that of the rightventricular (RV) free wall was significantly reduced as compared withcontrol 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 tocardiac cycle length, was longer in patients than in control subjects(8.7 ± 4.8% vs 5.0 ± 3.0%, respectively; p < 0.03). Thepulmonary 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/s2; p < 0.001) wereboth lower than normal. This studyconfirms the presence of significant RV systolic and diastolicdysfunction in the setting of consistent tachycardia and increasedcardiac output in adult CF patients with severe disease. No specific LVabnormalities were detected in thesepatients.