SAT-062 National PA Registry as a Platform for Standardized Clinical Practice of Primary Aldosteronism in Japan

Abstract

Background: Primary aldosteronism (PA) is a representative cause of endocrine hypertension characterized by an excess production of aldosterone, hypertension, and cardiovascular complications. Although clinical practice guidelines have been published, diagnostic steps have not been standardized and heterogeneous clinical practice between centers remain to be the major issue of public health in Japan. Aim: To develop PA registry and to produce evidence for clinical practice of PA. Methods: PA registry was developed by Japan PA Study (JPAS) as one of the Incurable Disease Platform Project by the Japan Agency of Medical Research and Development (AMED) and the Ministry of Health, Labor and Welfare in Japan. 2850 PA patients who underwent AVS between 2006 and 2016 in 28 referral centers were registered. Major clinical questions were selected. Results: Evidence produced was as follows. 1) Prevalence of cardiovascular diseases was higher in PA than essential hypertension. Hypokalemia, unilateral subtype and/or PAC greater than 12.5ng/dl were at greater risk of cardiovascular diseases. 2) Since clinical manifestation of PA in elderly was similar to that in younger patients, diagnostic process should essentially follow the same steps. 3) Saline infusion test as a confirmatory test was also useful for subtype diagnosis. 4) More than 90% of PA patients with normokalemia and bilateral disease on CT had bilateral subtype on AVS and needed AVS less weakly. Those aged more than 35 yrs. with marked PA could be spared AVS. 5) While biochemical benefit after ADX was achieved solely with LI greater than 4 of AVS, clinical benefit was largely affected by clinical findings such as age, BMI, and blood pressure. 6) Since prevalence of cortisol co-secretion was high in PA with adrenal tumor smaller than 2cm, DEX suppression test was mandatory. LI greater than 4 was applicable for PA subtype diagnosis even in patients with cortisol co-secretion, but not in those with overt Cushing syndrome. 7) ADX provided superior results in correcting hypertension and hypokalemia than medication in unilateral PA. Conclusions: National PA registry was developed for standardized clinical practice of PA in Japan. Various evidence for elaboration and simplification of clinical practice guideline of PA was created by excluding institutional bias. The PA registry has started to be operated as a part of more comprehensive registry by Japan Rare Adrenal Disease Study (JRAS) for a sustainable platform of future research and development. (Supported by AMED for the Practical Research Project for Rare/Intractable Disease under Grant Number JP17ek0109122; JP18ek0109352).