Clinical problem In vivo skeletal muscle energetics during exercise - PowerPoint PPT Presentation

4/21/2018 Clinical problem In vivo skeletal muscle energetics during exercise in pulmonary arterial hypertension JDL. Vos, BSc 1 • MG. Haarman, MD 1 • AJ. Sibeijn-Kuiper 2 • RMF. Berger, MD, PhD 1 TP. Willems, MD, PhD 3 • JAL. Jeneson, PhD 2 1 Center for Congenital Heart Diseases, 1 Department of Pediatric Cardiology 2 NeuroImaging Center, Division of Neurosciences 3 Department of Radiology D'Alonzo, CHEST 1987 University Medical Center Groningen University Medical Center Groningen Scientific problem Study Design (1) iPAH Control Population (n) 3 6 Age (y) median (range) 14,1 (13,1 – 18,0) 13,6 (10,5 – 18,0) Sex (f) 3 5 Height (cm) 155 – 169 150 – 177 Pulmonary dysfunction Cardiac dysfunction Muscular dysfunction? Weight (kg) 37,0 – 70,0 35,0 – 62,0 • Decreased muscle strength BMI 15,2 – 24,5 15,6 – 20,8 (Pepplinkhuizen, submitted) • Decreased microcirculation/vessels WHO-FC I, II, III, IV 0,3,0,0 6,0,0,0 ( Potus, AJRCCM 2014) • Fiber type shift I -> II (Batt, AJRCMB 2014) • Decreased mitochondrial density and structure (Pflieger, Circulation 2013) University Medical Center Groningen University Medical Center Groningen 1

4/21/2018 Study Design (2) Study Design (3) Spirometry - Lung capacity In-vivo 31 P-MR Spectroscopy of quadriceps muscle - Max. ventilation - Muscular energy reserve - Muscular pH CPET - Pulmonary efficiency - Recovery rate - Cardiac output & HR - Exercise capacity University Medical Center Groningen University Medical Center Groningen In vivo 31 P-MRS In vivo 31 P-MRS pH pH Energy reserve Energy reserve Recovery time Brussels, Acad Radiol 2015 Video shown with permission of the person ìnvolved University Medical Center Groningen University Medical Center Groningen 2

4/21/2018 Results (1) Spirometry Results (2) CPET reference normal University Medical Center Groningen University Medical Center Groningen Results (3) in vivo muscle energetics Results (3) post-exercise recovery Recovery time baseline end of exercise University Medical Center Groningen University Medical Center Groningen 3

4/21/2018 Conclusion (1) Thank you Dutch National Referral Center for Pediatric PH Center for Congenital Heart Diseases UMCG Department of Neuroscience Rolf Berger, MD PhD Jeroen Jeneson, PhD Guido Bossers, MD Anita Sibeijn-Kuiper Graziella Eshuis, BSc Gert ter Horst, PhD Pulmonary function Cardiac function Muscular function Diederik van der Feen, MD Marlies Haarman, MD Department of Radiology High energy reserve Theresia Kazemier, MSc Neutral pH values Tineke Willems, MD PhD Annemarie Koop, MD Normal recovery time Floris-Jan Ridderbos, MD University Medical Center Groningen University Medical Center Groningen 4