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Cardiac dysfunction is the main mechanism of aggravation of exercise intolerance in homozygous sickle cell disease patients

Session Interplay between exercise and cardiac pathology

Speaker Alexandre Ceccaldi

Event : ESC Congress 2016

  • Topic : preventive cardiology
  • Sub-topic : Exercise Testing
  • Session type : Moderated Posters

Authors : A Ceccaldi (Paris,FR), S Hatem (Paris,FR), F Lionnet (Paris,FR), K Stankovic Stojanovic (Paris,FR), R Isnard (Paris,FR), N Hammoudi (Paris,FR)

A. Ceccaldi1 , S. Hatem1 , F. Lionnet2 , K. Stankovic Stojanovic2 , R. Isnard1 , N. Hammoudi1 , 1Hospital Pitie-Salpetriere, Cardiology - Paris - France , 2Hospital Tenon - Paris - France ,

European Heart Journal ( 2016 ) 37 ( Abstract Supplement ), 26

Background: Sickle cell disease (SCD) is the most frequent genetic hemoglobinopathy worldwide associated with a marked exercise intolerance. Mechanisms underlying this exercise limitation are multifactorial, complex and difficult to discriminate in clinical practice.

Purpose: To examine the role of cardiac dysfunction in the exercise limitation in homozygous SCD patients.

Methods: 60 SCD patients (32.9±10.6 years, 36 women) with dyspnea and 20 controls matched for age and gender underwent a stress echocardiography combined with gas exchange measurements during a symptom-limited exercise ramp protocol. Peak exercise oxygen uptake indexed to weight (VO2) and peak cardiac index (Ci) were measured. The difference between arterial and venous oxygen content (Ca-vO2) was calculated using Fick principle.

Results: Compared to controls, SCA patients exhibited a low peak VO2 (19.6±4.1 vs. 36.4±9.5 ml/min/kg; p<0.001), a dramatic decrease in Ca-vO2 (7.5±1.5 vs. 15.8±3.1 ml/dl; p<0.0001) whereas Ci was markedly increased (9.6±1.6 vs. 8.4±1.6 l/min/m2; p=0.005) suggesting a compensatory mechanism of the reduced peripheral oxygen transfer.

The SCD patients were next classified in tertiles according to peak VO2. While hemoglobin level and Ca-vO2 were similar, SCD patients in the lower tertile of VO2 exhibited a reduced peak Ci (Table 1). In multivariable analysis, older age and left atrial function as assessed by peak longitudinal strain were independently related to exertional intolerance.

Conclusion: The exercise intolerance of SCD patients is mainly related to the impairment of Ca-vO2 which is compensated by a high cardiac output.

However, the aggravation of exertional intolerance in SCD is due to the alteration of cardiac function related to ageing and to left atrial function deterioration.

Table 1
Lower VO2 tertileOther VO2 tertilesp value
Age (years)40.3±10.129.2±8.80.0001
Peak Cardiac index (l/min/m2)8.6±1.010.1±1.70.0001
Peak Ca-vO2 (ml/dl)7.0±1.47.8±1.50.07
Hemoglobin (g/dl)8.3±1.38.8±1.20.13
LV diastolic volume index (ml/m2)91.9±23.092.2±21.50.95
LV ejection fraction at rest (%)60.7±7.962.2±5.10.46
Cardiac index at rest (l/min/m2)4.5±0.94.3±0.80.38
Tricuspid velocity at rest (m/s)2.5±0.32.4±0.20.28
Left atrial volume index at rest (ml/m2)54.7±16.944.0±11.30.02
Left atrial longitudinal strain at rest (%)30.7±5.438.9±7.2<0.0001
VO2 <16.1 ml/min/kg in women (n=12), VO2 <19.2 ml/min/kg in men (n=8). VO2 ≥16.1 ml/min/kg in women (n=24), VO2 ≥19.2 ml/min/kg in men (n=16).

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