Rendu Osler Stress Echocardiography (ROSE)

Background

The hepatic involvement in Hereditary Hemorrhagic Telangiectasia (HHT) is characterized by arterio-sus-hepatic shunts that can lead to high cardiac output heart failure (HCO-HF). This develops from asymptomatic diastolic heart failure to rest pulmonary hypertension (PH), which is actually required for liver tranplantation (LT) candidacy. However, when PH is present, HCO-HF improvement after LT is uncertain since such referral may possibly occur too late.

Objectives

We hypothesized that abnormal pulmonary pressure response during exercice in HHT patients with hepatic involvement can precede rest PH can be detected during exercise echocardiography and may be partially related to rest cardiac output indexed to BSA (CO).

Methods

ROSE is a pilot study of 40 HHT patients with hepatic involvement as defined by a hepatic artery diameter ≥ 6 mm,. Median CO value divided the population in 2 groups: high cardiac output (HCO group) vs normal cardiac output (NCO group). Patients were in sinus rhythm without betablocker treatment. Stress echocardiography was performed for CO, left ventricular (LV) and atrial (LA) volumes and functions, and systolic pulmonary arterial pressure (sPAP) at rest, 20 W, and peak exercise. Endpoints were the comparison between groups of the evolution of sPAP (primary) and sPAP/CO slopes (secondary) from rest to peak exercise.

Results

Among 33 patients, we found no difference in sPAP at rest nor at peak exercise between HCO and NCO patients (Table 1). However, sPAP/CO slopes were higher in the HCO group especially in 4 patients exhibiting a greater sPAP increase for a relative smaller CO increase during exercise (Figure 1, Figure 2).

Conclusion

In HHT patients with hepatic involvement, sPAP assessment during exercise echocardiography for the identification of earlier stages of HCO-HF can be promising, especially when assessing the relationships between CO increase and sPAP increase. The sPAP/CO slope should be further investigated as an early marker nearly preceding rest PH.