Right ventricular overload and cardiovascular neuroendocrine derangement in systemic sclerosis

AIM: Systemic sclerosis (SSc) may be associated with right ventricular overload, secondary to pulmonary hypertension. In heart failure patients, neuroendocrine derangements can influence clinical evolution and prognosis. The aim of this study was to investigate neurohormonal control affected in SSc patients with and without right ventricular impairment. METHODS AND RESULTS: A prospective series of 28 patients with SSc was studied. In addition to conventional evaluations, extensive neuroendocrine studies were done, including assays of both the vasoconstrictor system (plasma renin activity [PRA], aldosterone and catecholamines) and vasodilatory molecules (brain natriuretic peptide [BNP] and atrial natriuretic peptide [ANP]). A significant relation was observed between echo-Doppler estimated pulmonary systolic pressure (PAP) and neurohormonal activation, in particular between PAP and BNP (R=0.58, p=0.004), ANP (R=0.65, p<0.001) and PRA (R=0.45, p=0.032). Patients with right ventricular overload (i.e., PAP>40 mmHg confirmed at cardiac catheterization) had higher levels of ANP and BNP (147±26 vs 34±6 pg/mL and 344±86 vs 30±7 pg/mL, respectively, p<0.001), PRA (6.4±1.9 vs 1.8±0.4 ng/mL/h, p<0.001) and aldosterone (257±86 vs 114±22 pg/mL, p=0.02). These patients had increased plasma noradrenaline, but not adrenaline (701±87 vs 452±66 pg/mL, p<0.001). CONCLUSION: SSc patients with right heart failure have a neurohormonal derangement, showing overactivity of the vasoconstrictive system, counteracted by oversecretion of cardiac natriuretic hormones.