The 53rd Annual Meeting of Japanese Society of Pediatric Cardiology and Cardiac Surgery

Presentation information

JCK E-Oral Presentation

JCK E-Oral Presentation 1 (II-JCKEOP01)

Sat. Jul 8, 2017 6:15 PM - 7:15 PM E-Oral Presentation Area (Exhibition and Event Hall)

Chair:Shiro Baba(Department of Pediatrics, Graduate School of Medicine, Kyoto University, Japan)

6:15 PM - 7:15 PM

[II-JCKEOP01-08] Utility of and hemodynamic association with alpha1 -antitrypsin clearance in Fontan patients

Satoshi Masutani, Yoichi Iwamoto, Hirotaka Ishido, Akiko Yana, Seiko Kuwata, Clara Kurishima, Hideaki Senzaki (Pediatric Cardiology, Saitama Medical University Saitama Medical Center, Japan)

【Background】Prevention and early recognition of protein-losing enteropathy (PLE) are important in Fontan patients (F). α1 -antitrypsin (AT) is an endogenous substance which is not secreted or reabsorbed in the intestine in normal condition. AT clearance (CAT) increases as PLE advances. We assessed its relation with hemodynamics and detectability of a subtle protein losing which cannot be detected by serum albumin level (Alb).
【Methods】This study included 42 F(3-24, median 7.7 years)including 2 active PLEs. We obtained CAT (mL/day): daily AT excretion (mg/day) measured by 3 days feces storage / plasma AT concentration (mg/dL) * 100. We compared CAT with Alb, and hemodynamic variables obtained by simultaneous cardiac catheterizations.
【Results】CAT were elevated (63.7, 90.6 mL/day) in 2 active PLE (both Alb 2.8 mg/dL). CAT after remission of PLE (Alb 4.1, 3.8 mg/dL) were normal (2.2, 1.2 mL/day). After exclusion of active PLEs (range 1.1-21.4, median 6.0 mL/day), lower serum Alb correlated with lower CAT (P<0.01). All three patients with Alb<3.5 mg/dL showed normal CAT <8 mL/day, and their ALB recovered without PLE therapy. All three patients with CAT >13 mL/day had ALB >4 mg/d. CAT did not correlate with the duration after F operation, CI, SaO2, Rp, HR, or CVP.
【Discussion】CAT may be useful for the differential diagnosis of hypoalbuminemia. There should be the factors other than CVP which induce intestinal protein losing. Clinical significance of subtle protein losing deserves future investigations employing a large number of patients.