Pathophysiology of pediatric heart failure with preserved ejection fraction

• Project/Area Number: 25461630
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Pediatrics
• Research Institution: Saitama Medical University
• Principal Investigator: Masutani Satoshi 埼玉医科大学, 医学部, 准教授 (10316739)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Project Status: Completed (Fiscal Year 2015)
• Budget Amount: ¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000); Fiscal Year 2015: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000); Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000); Fiscal Year 2013: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
• Keywords: 心不全 / 駆出率 / 小児 / stiffness / relaxation / elastance / reserve / Fontan / HFpEF / aldosterone / BNP / biventricular / heart failure / ejection fraction / 拡張 / 心機能

Outline of Final Research Achievements

This study has clarified the detail hemodynamics in pediatric HFpEF. Those include ventricular systolic, diastolic, and arterial stiffness at rest. Furthermore, those characteristics are associated with diastolic and catecholamic reserves, and coronary supply-demand balance.
Fontan circulation has higher diastolic and arterial stiffness, which are similar to pediatric HFpEF. However, there is marked differences in the ventricular filling pressure: high filling pressure in HFpEF and low filling pressure in good Fontan. We also clarified the good correlations between peripheral venous pressure and central venous pressure, and between arm equilibrium pressure and mean circulatory filling pressure in Fontan patients.

Research Products

• [Journal Article] Novel mechanisms for cerebral blood flow regulation in patients with congenital heart disease. (2016)
  • Author(s): Saiki H, Sugimoto M, Kuwata S, Kurishima C, Iwamoto Y, Ishido H, Masutani S, Senzaki H.
  • Journal Title: Am Heart J Volume: 172 Pages: 152-159
  • DOI: 10.1016/j.ahj.2015.11.009
  • Peer Reviewed
• [Journal Article] Ventricular fibrogenesis activity assessed by serum levels of procollagen type III N-terminal amino peptide during the staged Fontan procedure. (2016)
  • Author(s): Sugimoto M, Saiki H, Tamai A, Seki M, Inuzuka R, Masutani S, Senzaki H.
  • Journal Title: J Thorac Cardiovasc Surg Volume: Epub Issue: 6 Pages: 1518-1526
  • DOI: 10.1016/j.jtcvs.2016.01.020
  • Peer Reviewed
• [Journal Article] Vulnerability of Coronary Circulation After Norwood Operation. (2016)
  • Author(s): Saiki H, Kuwata S, Kurishima C, Masutani S, Senzaki H
  • Journal Title: Ann Thorac Surg Volume: 101 Issue: 4 Pages: 1544-1551
  • DOI: 10.1016/j.athoracsur.2015.10.077
  • Peer Reviewed
• [Journal Article] Influence of Left Ventricular Stiffness on Hemodynamics in Patients With Untreated Atrial Septal Defects (2015)
  • Author(s): Kurishima, C.Inuzuka, R.Kuwata, S.Iwamoto, Y.Sugimoto, M.Saiki, H.Ishido, H.Masutani, S.Senzaki, H.
  • Journal Title: Circulation Journal Volume: 79 Issue: 8 Pages: 1823-1827
  • DOI: 10.1253/circj.CJ-14-1351
  • NAID: 130005089353
  • ISSN: 1346-9843, 1347-4820
  • Peer Reviewed
• [Journal Article] Re: 'Post-LA space index' as a potential novel marker for the prenatal diagnosis of isolated total anomalous pulmonary venous connection (2015)
  • Author(s): Ishido, H.Masutani, S.Hishitani, T.Taketadu, M.Senzaki, H.
  • Journal Title: Ultrasound Obstet Gynecol Volume: 46 Issue: 6 Pages: 747-8
  • DOI: 10.1002/uog.15756
  • Peer Reviewed
• [Journal Article] Prevalence, implication, and determinants of worsening renal function after surgery for congenital heart disease. (2015)
  • Author(s): Saiki H, Kuwata S, Kurishima C, Iwamoto Y, Ishido H, Masutani S, Senzaki H.
  • Journal Title: Heart Vessels Volume: Epub
  • Peer Reviewed
• [Journal Article] Tailored therapy for aggressive dilatation of systemic veins and arteries may result in improved long-term Fontan circulation. (2015)
  • Author(s): Kurishima C, Saiki H, Masutani S, Senzaki H.
  • Journal Title: J Thorac Cardiovasc Surg Volume: 150 Issue: 5 Pages: 1367-1370
  • DOI: 10.1016/j.jtcvs.2015.08.002
• [Journal Article] Oxygen Supply to the Fetal Cerebral Circulation in Hypoplastic Left Heart Syndrome:A Simulation Studay Based on the Theoretical Models of Fetal Circulation (2015)
  • Author(s): Sakazaki S,Masutani S,Sugimoto M,Tamura M,Kuwata S,Kurishima C,Saiki H,Iwamoto Y,Ishido H,Senzaki H.
  • Journal Title: Pediatr Cardiol.. Volume: 36(3) Issue: 3 Pages: 677-684
  • DOI: 10.1007/s00246-014-1064-6
  • Peer Reviewed
• [Journal Article] Cystatin C and body surface area are major determinants of the ratio of N-terminal pro-brain natriuretic peptide to brain natriuretic peptide levels in children. (2014)
  • Author(s): Kurishima C, Masutani S, Kuwata S, Iwamoto Y, Saiki H, Ishido H, Tamura M, Senzaki H.
  • Journal Title: J Cardiol Volume: Epub ahead Issue: 2 Pages: 175-180
  • DOI: 10.1016/j.jjcc.2014.11.002
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Arterial stiffness in patients after Kawasaki disease without coronary artery involvement: Assessment by performing brachial ankle pulse wave velocity and cardio-ankle vascular index. (2014)
  • Author(s): Nakagawa R, Kuwata S, Kurishima C, Saiki H, Iwamoto Y, Sugimoto M, Ishido H, Masutani S, Senzaki H.
  • Journal Title: J Cardiol Volume: Epub ahead Issue: 2 Pages: 130-134
  • DOI: 10.1016/j.jjcc.2014.10.003
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Aldosterone-cortisol imbalance immediately after fontan operation with implications for abnormal fluid homeostasis. (2014)
  • Author(s): Saiki H, Kuwata S, Kurishima C, Iwamoto Y, Ishido H, Masutani S, Senzaki H.
  • Journal Title: Am J Cardiol Volume: 114 Issue: 10 Pages: 1578-83
  • DOI: 10.1016/j.amjcard.2014.08.030
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Aortic root dilatation and aortic stiffness in patients with single ventricular circulation. (2014)
  • Author(s): Kojima T, Kuwata S, Kurishima C, Iwamoto Y, Saiki H, Ishido H, Masutani S, Senzaki H.
  • Journal Title: Circ J Volume: 78 Pages: 2507-11
  • NAID: 130004677640
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Left atrial volume is superior to the ratio of the left atrium to aorta diameter for assessment of the severity of patent ductus arteriosus in extremely low birth weight infants. (2014)
  • Author(s): Toyoshima K, Masutani S, Senzaki H, Kawataki M, Itani Y.
  • Journal Title: Circ J Volume: 78 Pages: 1701-9
  • NAID: 130004926953
  • Peer Reviewed / Open Access / Acknowledgement Compliant
• [Journal Article] Cerebral circulation in patients with Fontan circulation: assessment by carotid arterial wave intensity and stiffness. (2014)
  • Author(s): Saiki H, Kurishima C, Masutani S, Senzaki H.
  • Journal Title: Ann Thorac Surg Volume: 97 Issue: 4 Pages: 1394-9
  • DOI: 10.1016/j.athoracsur.2013.10.079
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] Assessment of ventricular relaxation and stiffness using early diastolic mitral annular and inflow velocities in pediatric patients with heart disease. (2014)
  • Author(s): Masutani S, Saiki H, Kurishima C, Kuwata S, Tamura M, Senzaki H.
  • Journal Title: Heart Vessels Volume: 29 Issue: 6 Pages: 825-33
  • DOI: 10.1007/s00380-013-0422-2
  • Peer Reviewed / Acknowledgement Compliant
• [Journal Article] 心血管機能診断の重要性 (2014)
  • Author(s): 増谷 聡
  • Journal Title: 循環器内科 Volume: 75 Pages: 117-122
  • NAID: 40019956507
• [Journal Article] Heart Failure With Preserved Ejection Fraction in Children (2013)
  • Author(s): Masutani S, Saiki H, Kurishima C, et al.
  • Journal Title: Circulation Journal Volume: 77 Issue: 9 Pages: 2375-2382
  • DOI: 10.1253/circj.CJ-12-1271
  • NAID: 10031191767
  • ISSN: 1346-9843, 1347-4820
  • Peer Reviewed
• [Journal Article] beta3-Adrenergic Receptor Antagonist Improves Exercise Performance in Pacing-Induced Heart Failure. (2013)
  • Author(s): Masutani S, Cheng HJ, Morimoto A, et al.
  • Journal Title: Am J Physiol Heart Circ Physiol Volume: 305 Issue: 6 Pages: H923-H930
  • DOI: 10.1152/ajpheart.00371.2012
  • Peer Reviewed
• [Journal Article] Preserved stroke volume late after tetralogy repair, despite severe right ventricular dilatation. (2013)
  • Author(s): Masutani S, Senzaki H.
  • Journal Title: Heart Volume: 99 Issue: 24 Pages: 1875-1875
  • DOI: 10.1136/heartjnl-2012-303449
  • Peer Reviewed
• [Journal Article] Usefulness of cystatin C in the postoperative management of pediatric patients with congenital heart disease. (2013)
  • Author(s): Yana A, Masutani S, Kojima T, et al.
  • Journal Title: Circ J Volume: 77 Pages: 667-72
  • NAID: 10031138956
  • Peer Reviewed
• [Journal Article] Progressive aortic dilation and aortic stiffness in children with repaired tetralogy of Fallot. (2013)
  • Author(s): Seki M, Kurishima C, Saiki H, Masutani S, Arakawa H, Tamura M, Senzaki H.
  • Journal Title: Heart Vessels Volume: 29 Issue: 1 Pages: 83-87
  • DOI: 10.1007/s00380-013-0326-1
  • Peer Reviewed
• [Journal Article] Landiolol hydrochloride infusion for treatment of junctional ectopic tachycardia in post-operative paediatric patients with congenital heart defect. (2013)
  • Author(s): Saiki H, Nakagawa R, Ishido H, Masutani S, Senzaki H
  • Journal Title: Europace Volume: 15 Issue: 9 Pages: 1298-1303
  • DOI: 10.1093/europace/eut044
  • Peer Reviewed
• [Journal Article] Constrictive pericarditis developed after childhood repair of ventricular septal defect. (2013)
  • Author(s): Saiki H, Masutani S, Tamura M, Senzaki H.
  • Journal Title: Pediatr Int. Volume: 55 Issue: 4 Pages: 512-516
  • DOI: 10.1111/ped.12023
  • Peer Reviewed
• [Journal Article] Impaired Cerebral Perfusion After Bilateral Pulmonary Arterial Banding in Patients With Hypoplastic Left Heart Syndrome. (2013)
  • Author(s): Saiki H, Kurishima C, Masutani S, Tamura M, Senzaki H.
  • Journal Title: Ann Thorac Surg Volume: 96 Issue: 4 Pages: 1382-1388
  • DOI: 10.1016/j.athoracsur.2013.05.108
  • Peer Reviewed
• [Journal Article] Pulmonary Arterial Wall Stiffness and Its Impact on Right Ventricular Afterload in Patients With Repaired Tetralogy of Fallot. (2013)
  • Author(s): Ryo Inuzuka, Mitsuru Seki, Masaya Sugimoto, Hirofumi Saiki, Satoshi Masutani, Hideaki Senzaki
  • Journal Title: Ann Thorac Surg Volume: 96 Issue: 4 Pages: 1435-1441
  • DOI: 10.1016/j.athoracsur.2013.05.085
  • Peer Reviewed
• [Journal Article] Indomethacin for preterm infants with intracranial hemorrhage. (2013)
  • Author(s): Chiba H, Masutani S, Toyoshima K, Mori R.
  • Journal Title: Pediatr Int. Volume: 55 Issue: 1 Pages: 124-126
  • DOI: 10.1111/ped.12037
  • Peer Reviewed
• [Journal Article] Clostridium difficile Colitis Induced by Long-term Low-dosage Erythromycin. (2013)
  • Author(s): Arao M, Masutani S, Kojima T, Saiki H, Ishido H, Tamura M, Senzaki H.
  • Journal Title: Pediatr Infect Dis J. Volume: 32 Issue: 9 Pages: 1042-1043
  • DOI: 10.1097/inf.0b013e318298e00c
  • Peer Reviewed
• [Journal Article] 弁・大血管の異常 (2013)
  • Author(s): 増谷聡
  • Journal Title: 周産期医学 周産期の画像診断第2版 Volume: 43 Pages: 416-422
• [Presentation] Rest and Reserve Functions in Fontan Patients With Right Ventricular Morphology are Worse Than Those With Left Ventricular Morphology. (2015)
  • Author(s): Satoshi Masutani, Seiko Kuwata, Clara Kurishima, Yoichi Iwamoto, Hirofumi Saiki, Hirotaka Ishido, Masanori Tamura, Hideaki Senzaki.
  • Organizer: American Heart Association Scientific Session 2015
  • Place of Presentation: Orland, FL, US
  • Year and Date: 2015-11-07
  • Int'l Joint Research
• [Presentation] Right ventricular mechanics in biventricular circulation – effects of RV afterload on RV-PA coupling, RV diastolic stiffness, and RA pressure. (2015)
  • Author(s): Satoshi Masutani, Seiko Kuwata, Clara Kurishima, Yoichi Iwamoto, Hirotaka Ishido, Masanori Tamura, Hideaki Senzaki.
  • Organizer: the 11th CKJ Pediatric Heart Forum
  • Place of Presentation: Tianjin, China
  • Year and Date: 2015-10-29
  • Int'l Joint Research / Invited
• [Presentation] 二心室疾患における右室メカニクス 右室後負荷と、右室・肺動脈カップリング、拡張期スティッフネス、右房圧の連関 (2015)
  • Author(s): 増谷 聡, 金 晶恵, 桑田 聖子, 栗嶋 クララ, 岩本 洋一, 石戸 博隆, 先崎 秀明
  • Organizer: 日本小児循環器学会
  • Place of Presentation: 東京 ホテル日航東京（東京都港区）
  • Year and Date: 2015-07-16
• [Presentation] 心不全に対するβ遮断薬療法 (2015)
  • Author(s): 増谷 聡, 先崎 秀明
  • Organizer: 日本小児循環器学会
  • Place of Presentation: 東京 ホテル日航東京（東京都港区）
  • Year and Date: 2015-07-16
  • Invited
• [Presentation] Heart failure with preserved ejection fraction (HFpEF) in children. (2015)
  • Author(s): Satoshi Masutani, Hideaki Senzaki.
  • Organizer: PAS/ASPR/JPS joint symposium
  • Place of Presentation: Osaka 大阪国際会議場（大阪府大阪市）
  • Year and Date: 2015-04-17
  • Int'l Joint Research / Invited
• [Presentation] Decision-making and Therapeutic Strategy before Glenn (2015)
  • Author(s): Satoshi Masutani
  • Organizer: 第３回Mt. Fuji Network Forum
  • Place of Presentation: 日本平ホテル（静岡県静岡市）
  • Year and Date: 2015-02-27 – 2015-02-28
• [Presentation] Ventricular-Arterial Coupling Biphasically Correlates to Ventricular Diastolic Chamber Stiffness in Children−Association With Aldosterone Activation (2014)
  • Author(s): Satoshi Masutani, JeongHye Kim, Seiko Kuwata, Clara Kurishima, Hirofumi Saiki, Masanori Tamura, Hideaki Senzaki
  • Organizer: American Heart Association Scientific Session 2014
  • Place of Presentation: Chicago, USA
  • Year and Date: 2014-11-15 – 2014-11-19
• [Presentation] Failed vs Super Fontan: beyond pulmonary resistance (2014)
  • Author(s): Satoshi Masutani, Clara Kurishima, Hideaki Senzaki
  • Organizer: 第50回日本小児循環器学会
  • Place of Presentation: 岡山コンベンションセンター（岡山県岡山市）
  • Year and Date: 2014-07-03 – 2014-07-05
  • Invited
• [Presentation] Right Ventricular-Left Ventricular interaction (2014)
  • Author(s): Satoshi Masutani and Hideaki Senzaki
  • Organizer: 第50回日本小児循環器学会
  • Place of Presentation: 岡山コンベンションセンター（岡山県岡山市）
  • Year and Date: 2014-07-03 – 2014-07-05
  • Invited
• [Presentation] 心室大血管統合関係(Ees/Ea)は拡張期stiffnessを二相性に規定する (2014)
  • Author(s): 増谷聡、金 晶惠、桑田聖子、栗嶋クララ、岩本洋一、石戸博隆、先崎秀明
  • Organizer: 第50回日本小児循環器学会
  • Place of Presentation: 岡山コンベンションセンター（岡山県岡山市）
  • Year and Date: 2014-07-03 – 2014-07-05
• [Presentation] 新生児の心機能の基礎から心不全治療へ (2014)
  • Author(s): 増谷 聡
  • Organizer: 第１６回若葉小児科臨床研究会
  • Place of Presentation: Ｋｏｂｅ
  • Invited
• [Presentation] Wave Intensity Analysis of the Pulmonary Artery in Postoperative Tetralogy of Fallot: Impact of Residual Pulmonary Stenosis on Pulmonary Regurgitation and Ventricular Function. (2013)
  • Author(s): Hirofumi Saiki, Seiko Kuwata, Clara Kurishima, Yoichi Iwamoto, Hirotaka Ishido, Satoshi Masutani, Hideaki Senzaki.
  • Organizer: AHA Scientific Session.
  • Place of Presentation: Ｄａｌｌａｓ, USA
• [Presentation] Aldosterone Regulation by Angiotensin-converting Enzyme in Congenital Heart Disease: The Clinical Importance of Blocking Angiotensin-converting Enzyme in a Single Ventricular Heart. (2013)
  • Author(s): Hirofumi Saiki, Seiko Kuwata, Clara Kurishima, Yoichi Iwamoto, Hirotaka Ishido, Satoshi Masutani, Hideaki Senzaki.
  • Organizer: AHA Scientific Session.
  • Place of Presentation: Ｄａｌｌａｓ, USA
• [Presentation] Renovascular Interaction in Children With Congenital Heart Disease. (2013)
  • Author(s): Hirofumi Saiki, Hirotaka Ishido, Satoshi Masutani, Masanori Tamura, Hideaki Senzaki.
  • Organizer: AHA Scientific Session.
  • Place of Presentation: Ｄａｌｌａｓ, USA
• [Presentation] Unique Hormonal Response After Fontan Operation: Aldosterone-Cortisol Imbalance. (2013)
  • Author(s): Hirofumi Saiki, Hirotaka Ishido, Satoshi Masutani, Hideaki Senzaki.
  • Organizer: AHA Scientific Session
  • Place of Presentation: Ｄａｌｌａｓ, USA
• [Presentation] Cystatin C and Body Surface Area Are Major Determinants of the Ratio of N-Terminal Pro-Brain Natriuretic Peptide to Brain Natriuretic Peptide Levels in Pediatric Patients. (2013)
  • Author(s): Clara Kurishima, Satoshi Masutani, Seiko Kuwata, Hirofumi Saiki, Masanori Tamura, Hideaki Senzaki
  • Organizer: AHA Scientific Session
  • Place of Presentation: Ｄａｌｌａｓ, USA
• [Presentation] Relationships Between Inertial Force, Wave Intensity, and Ventricular Function in Patients With Fontan Circulation. (2013)
  • Author(s): Mitsuru Seki, Seiko Kuwata, Clara Kurishima, Hirofumi Saiki, Satoshi Masutani, Hideaki Senzaki.
  • Organizer: AHA Scientific Session
  • Place of Presentation: Ｄａｌｌａｓ, USA
• [Presentation] 最大BNPより最終BNPが、より強く予後と関連する. (2013)
  • Author(s): 増谷 聡, 玉井 明子, 桒田 聖子, 栗嶋 クララ, 川崎 秀徳, 石川 博隆, 竹田津 未生, 齋木 宏文, 先崎 秀明:
  • Organizer: 日本小児循環器学会
  • Place of Presentation: Ｔｏｋｙｏ
• [Presentation] 房室弁置換が心室弛緩・流入に及ぼす影響について (2013)
  • Author(s): 増谷 聡, 桒田 聖子, 栗嶋 クララ, 石戸 博隆, 竹田津 未生, 川崎 秀徳, 先崎 秀明
  • Organizer: 日本小児循環器学会
  • Place of Presentation: Ｔｏｋｙｏ
• [Presentation] 超低出生体重児における四腔断面からの簡単の左房容積指標の有用性 (2013)
  • Author(s): 増谷 聡, 豊島 勝昭, 山口 直人, 斎藤 朋子, 新関 昌枝, 田仲 健一, 石川 淑, 星野 陸夫, 大山 牧子, 川滝 元良, 猪谷 泰史:
  • Organizer: 日本未熟児新生児学会
  • Place of Presentation: Ｋａｎａｚａｗａ
• [Presentation] 経カテーテル心房中隔閉鎖術後の心血行動態の変化 (2013)
  • Author(s): 増谷 聡
  • Organizer: 成人先天性心疾患セミナー
  • Place of Presentation: Ｔｏｋｙｏ
  • Invited
• [Book] Congenital Heart Disease: Morphological and Functional Assessment; Assessment of Ventricular Function Using the Pressure-Volume Relationship (2015)
  • Author(s): Satoshi Masutani
  • Total Pages: 30
  • Publisher: Springer Japan
• [Book] Congenital Heart Disease (2015)
  • Author(s): Satoshi Masutani and Hideaki Senzaki (Editor: Hideaki Senzaki, Satoshi Yasukochi)
  • Total Pages: 208
  • Publisher: Springer
• [Book] 小児科学レクチャー 子どもの不定愁訴 (2014)
  • Author(s): 増谷聡
  • Total Pages: 230
  • Publisher: 総合医学社
• [Book] 心エコーハンドブック 先天性心疾患 (2013)
  • Author(s): 増谷 聡
  • Total Pages: 227
  • Publisher: 金芳堂