WordNet

toward or on the right; also used figuratively; "he looked right and left"; "the party has moved right"
(frequently plural) the interest possessed by law or custom in some intangible thing; "mineral rights"; "film rights"
anything in accord with principles of justice; "he feels he is in the right"; "the rightfulness of his claim" (同)rightfulness
a turn toward the side of the body that is on the south when the person is facing east; "take a right at the corner"
the hand that is on the right side of the body; "he writes with his right hand but pitches with his left"; "hit him with quick rights to the body" (同)right_hand
those who support political or social or economic conservatism; those who believe that things are better left unchanged (同)right wing
an abstract idea of that which is due to a person or governmental body by law or tradition or nature; "they are endowed by their Creator with certain unalienable Rights"; "Certain rights can never be granted to the government but must be kept in the hands
location near or direction toward the right side; i.e. the side to the south when a person or object faces east; "he stood on the right"
make reparations or amends for; "right a wrongs done to the victims of the Holocaust" (同)compensate, redress, correct
correct in opinion or judgment; "time proved him right" (同)correct
exactly; "he fell flop on his face" (同)flop
an interjection expressing agreement (同)right on
intended for the right hand; "a right-hand glove" (同)right-hand
(of the side of cloth or clothing) facing or intended to face outward; "the right side of the cloth showed the pattern"; "be sure your shirt is right side out"
being or located on or directed toward the side of the body to the east when facing north; "my right hand"; "right center field"; "a right-hand turn"; "the right bank of a river is the bank on your right side when you are facing downstream"
completely; "she felt right at home"; "he fell right into the trap"
having the axis perpendicular to the base; "a right angle"
immediately; "she called right after dinner"
in conformance with justice or law or morality; "do the right thing and confess"
in or into a satisfactory condition; "things are right again now"; "put things right"
of or belonging to the political or intellectual right
precisely, exactly; "stand right here!"
put in or restore to an upright position; "They righted the sailboat that had capsized"
regain an upright or proper position; "The capsized boat righted again"
a plane figure with rounded sides curving inward at the top and intersecting at the bottom; conventionally used on playing cards and valentines; "he drew a heart and called it a valentine"
the courage to carry on; "he kept fighting on pure spunk"; "you havent got the heart for baseball" (同)mettle, nerve, spunk
an inclination or tendency of a certain kind; "he had a change of heart" (同)spirit
a playing card in the major suit that has one or more red hearts on it; "he led the queen of hearts"; "hearts were trumps"
the locus of feelings and intuitions; "in your heart you know it is true"; "her story would melt your bosom" (同)bosom
the hollow muscular organ located behind the sternum and between the lungs; its rhythmic contractions move the blood through the body; "he stood still, his heart thumping wildly" (同)pump, ticker
a firm rather dry variety meat (usually beef or veal); "a five-pound beef heart will serve six"
able to perceive sound
an opportunity to state your case and be heard; "they condemned him without a hearing"; "he saw that he had lost his audience" (同)audience
(law) a proceeding (usually by a court) where evidence is taken for the purpose of determining an issue of fact and reaching a decision based on that evidence
the ability to hear; the auditory faculty; "his hearing was impaired" (同)audition, auditory sense, sense of hearing, auditory modality
a session (of a committee or grand jury) in which witnesses are called and testimony is taken; "the investigative committee will hold hearings in Chicago"
connect or secure to; "They rigged the bomb to the ignition"
formation of masts, spars, sails, etc., on a vessel (同)rigging
gear (including necessary machinery) for a particular enterprise
manipulate in a fraudulent manner; "rig prices" (同)manipulate
arrange the outcome of by means of deceit; "rig an election" (同)set_up
equip with sails or masts; "rig a ship" (同)set, set_up
receive a communication from someone; "We heard nothing from our son for five years"
examine or hear (evidence or a case) by judicial process; "The jury had heard all the evidence"; "The case will be tried in California" (同)try
perceive (sound) via the auditory sense

PrepTutorEJDIC

《名試の前にのみ用いて》『右の』,右側の / 《しばしば『R-』》(政治上)『右派の』,保守的な / 〈U〉《the~》『右』,右側,右方 / 〈C〉『右手』(right hand) / 〈U〉《集合的に》《しばしばthe R-》(政治上の)右派,保守派 / 〈C〉(野球の)右翼(right field) / 『右に』,右側に,右方に
《補語にのみ用いて》(道徳的に)『正しい』,正当な,正義の(just) / (人,人の判断・意見などが)『間偉いのない』,正しい(correct) / (事実に合って)『正しい』,正確な / 適切な,ぴったりの / (精神が)健全な,まともな;(心身が)健康な / 表の,正面の / 〈U〉(道徳的な)『正しさ』,正義;正しい行い / 〈C〉〈U〉(法律上・道徳上の)権利 / 〈U〉〈C〉《複数形で》(政治的・社会的な)人権,権利 / 《話》『まっすぐに』;まともに(directly) / 《話》『きっかり』,ちょうど(exactly) / 正確に,間違いなく(correctly) / (道徳的に)正しく,公正に / すっかり,完全に(completely);ずっと(all the way) / 適切に,ふさわしく;都合よく,うまく / 《おもに米話》すぐに,ただちに / 《特定の称号に付けて》非常に(very) / …‘を'正しい位置(状態)に戻す,立ち直らせる,整とんする / 〈誤りなど〉‘を'正す
〈C〉『心臓』;胸 / 〈C〉(感情の中心をなす)『心』,気持ち / 〈U〉愛情,同情 / 〈U〉『勇気』,元気,熱意 / 《the ~》『中心』,内部,(物事の)本質,核心 / 〈C〉ハート形の物;(カードの)ハートの札
〈U〉『聴力』,聴覚〈U〉〈C〉(…を)『聞くこと』,(…の)聞き取り《+『of』+『名』》・〈C〉聞いてもらう機会,聞いてやること・聴取・聴聞会〈C〉(法廷などでの)尋問,審問,;(刑罰の減免を求める)釈明,意見陳述 / 〈U〉聞こえる距離(範囲)
〈船〉‘を'艤装(ぎそう)する / (マストなどを)〈船〉‘に'つける《+『名』+『with』+『名』》;(マストに)〈帆〉‘を'つける《+『名』+『to』+『名』》 / …‘に'装備をつける;…‘を'装備する《+『up』+『名,』+『名』+『up』》 / 船の装備(帆装),艤装(ぎそう) / (特定の目的のための)道具,用具 / 《英話》衣服,服装(clothing)
〈他〉〈相場など〉を不正な手段であやつる
〈音〉‘が'『聞こえる』,‘を'聞く(進行形にできない) / 〈人の言うこと〉‘に'『耳を傾ける』,‘を'注意深く聞く(listen);(裁判官などが)…の申し立てを聞く / 〈うわさ・ニュースなど〉‘を'『耳にしている』,聞いている / 『耳が聞こえる』 / 《しばしば命令形で》耳を傾ける,注意を向ける / 『うわさ』(『消息』)『を聞く』

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/05/31 23:39:51」(JST)

wiki en

Hypoplastic right heart syndrome is a congenital heart defect in which the right atrium and right ventricle are underdeveloped. This defect causes inadequate blood flow to the lungs and thus, a blue or cyanotic infant.[3]

Anatomy

A healthy heart has four valves, separated by flaps that open and close to control blood flow between the chambers. When the heart beats, oxygen-poor blood enters the right atrium. The blood then flows into the right ventricle, where it enters the pulmonary artery to travel to the lungs for oxygen. Oxygen-rich blood returns to the left atrium, where it then travels into the left ventricle. The left ventricle pushes the oxygenated blood into the aorta to be circulated to the rest of the body.^[1]

The heart is a mesoderm-derived organ; Mesoderm is the middle germ layer of an embryo, whose formation is regulated by various genes. Initially, the most important is expression of Nkx2.5, CR1, pitx2, anf and mhc2a, is responsible for differentiation of the types of cardiomyocytes to determine which part of the heart they go to.[5] In a later in differentiation, activation of hand1, hand2 and other genes was revealed to help in development. [5] Expression of these genes expression is regulated by various processes, including transcription and growth factors, as well as proteins like fibrillin, Wnt, BMP2, BMP 4, BMP5, BMP7, which aid in different heart development features like the valves and septum, and other substances, such as retinoid and folic acid.[5] Crucial steps in heart formation are development of the ventricles and atrium formation, as well as septation and valve formation. Any disturbances of such processes may lead to various congenital heart diseases and defects that could be initiated by various genetic, epigenetic or environmental factors. The most common heart malformations from genetic or epigenetic problems are: stenosis of the aorta and pulmonary trunk, which is a narrowing of the vessels, atrial and/or ventricular septal defect, tricuspid atresia, hypoplastic left and right heart. When you have hypoplastic right or left heart more than one of these problems occur together [5]

Pathogenesis

When the right side of the heart is more underdeveloped than the left side, this is known as hypoplastic right heart syndrome. HRHS is known for the pulmonary atresia valves, the tricuspid valve, and the hypoplastic pulmonary artery fail to form properly. HRHS also causes the right ventricle to be a fair amount smaller than the left side.^[2]

In people with hypoplastic right heart syndrome, the heart is not able to adequately pump blood to the lungs. The result of this is an inadequate supply of oxygenated blood to be circulated to the body. The severity of underdevelopment varies for each individual. A special team of pediatric cardiologists is required to develop a treatment plan.

Diagnosis and prevalence

Hypoplastic right heart syndrome is less common than hypoplastic left heart syndrome which occurs in 4 out of every 10,000 births. [3].^[3] This rare anomaly requires prenatal diagnosis since it needs immediate and emergency treatment. Pregnant women whose pregnancy is complicated with this anomaly should be referred to a level 3 hospital with pediatric cardiology and pediatric cardiothoracic surgical team.[3]

It can be associated with aortic stenosis.^[4]

Probable causes

The Notch-signaling pathway is involved in multiple processes during heart development, along with Wnt signaling.[6] Cardiomyocyte differentiation, patterning of the different cardiac regions, valve development, ventricular trabeculation, and outflow tract development have all been shown to depend on the activity of specific Notch-signaling elements.[6] The importance of Notch signaling in human disease is evident from the discovery that many mutations in components of this pathway are inherited in several genetic and acquired disorders, though one cannot acquire hypoplastic right heart syndrome.[6] Some of the mutations are thought to be on chromosome 2.[9] Other deletions are thought to be chromosomal arms 9q34 and 20p12, which contain NOTCH1 and JAG1 and deletion of band q22 or q24.[6] Wnt signaling also plays a role along with Notch 1,2 and JAG in the formation of cardiac tissues and heart development.[2] When there are mutations in the Notch and Wnt pathways, Notch and Wnt are either not signaled or inhibited.[2] These pathways have to signal proteins and other pathways, like Tbx2, 3 and 20, Bmp2, 10, and Hey1 and 2 which play a major role in different parts of the heart developing. When these proteins and other pathways aren’t signaled or are inhibited abnormalities form[6]

Treatment

There is no cure for hypoplastic right heart syndrome. A three-stage surgical procedure is commonly used to treat the condition. The surgeries rearrange the blood flow within the heart and allow the left ventricles to do the work for the underdeveloped right side of the heart. The three surgeries are spread out over the patients first few years of life. The first procedure, called the Norwood procedure, is typically done within the first few days or weeks of life. The second procedure, called the Glenn procedure, is usually performed between four and twelve months of age. The last surgery, known as the Fontan procedure, is typically performed between the ages of 18 months and three years. These surgeries change the blood flow to the lungs so that there is always oxygenated blood. The surgeries are a temporary fix from 15–30 years in which a patient will have to have a heart transplant.[3] In a stage 1 Norwood procedure for hypoplastic right heart, the main pulmonary artery is separated from the left and right portions of the pulmonary artery and joined with the upper portion of the aorta.[7] The proximal pulmonary artery is connected to the hypoplastic aortic arch, while the narrowed segment of the aorta is repaired. An aortopulmonary shunt is created to connect the aorta to the main pulmonary artery to provide pulmonary blood flow to the lungs.[7] The Glen procedure disconnects the superior vena cava from the heart and connects it to the right pulmonary artery so deoxygenated blood from the upper body goes directly to the lungs.[10] The Fontan procedure done usually after the patient is two years old, disconnects the inferior vena cava from the heart and connects it directly with the other pulmonary artery so that deoxygenated blood from the lower body then is sent directly to the lungs.[1]

Symptoms

Common symptoms include a grayish-blue (cyanosis) coloration to the skin, lips, fingernails and other parts of the body.^[2] Other pronounced symptoms can be rapid/difficulty breathing, poor feeding, cold hands or feet, or being inactive and drowsy. "In a baby with hypoplastic left heart syndrome, if the natural connections between the heart's left and right sides (foramen oval and ductus arteriosus) are allowed to close, he or she may go into shock." Signs of shock can include cool or clammy skin, a weak or rapid pulse, and dilated pupils.^[5]

Diagnosis

HLHS is most commonly diagnosed before birth with the use of a fetal echocardiogram (ultrasound), as sometimes diagnosis can occur after birth. "Diagnosis of HLHS may require some of these tests: electrocardiogram (ECG; a record of the electro activity of the heart), a chest X-ray, cardiac catheterization (a thin tube is inserted into the heart through a vein through the umbilical cord), or a cardiac MRI (a three dimensional image of the heart)."^[6]

Follow-up care

With a series of operations or even a heart transplant, a newborn can be treated but not be cured. Young individuals who have undergone reconstructive surgery must refer to a cardiologist who is experienced in congenital heart diseases,^[6] "Children with HLHS are at an increased level for developing endocarditis." Kids that have been diagnosed with HRHS must limit the physical activity they participate in to their own endurance level.^[7]

References

^ Heart Contraction and Blood Flow. National Institutes of Health.
^ ^a ^b CHD-UK, Hypoplastic Right heart Syndrome (HRHS), http://www.chd-uk.co.uk/types-of-chd-and-operations/hypoplastic-right-heart-syndrome-hrhs/, 2007-2015. Web. 25 April 2015
^ Paulick J, Tennstedt C, Schwabe M, Körner H, Bommer C, Chaoui R (May 2004). "Prenatal diagnosis of an isochromosome 5p in a fetus with increased nuchal translucency thickness and pulmonary atresia with hypoplastic right heart at 14 weeks". Prenat. Diagn. 24 (5): 371–4. doi:10.1002/pd.877. PMID 15164412.
^ Sharma J, Friedman D, Schiller M, Flynn P, Alonso ML (December 1997). "Aortic stenosis in hypoplastic right heart syndrome, associated with interstitial deletion of chromosome 2". Int. J. Cardiol. 62 (3): 199–202. doi:10.1016/S0167-5273(97)00255-6. PMID 9476678.
^ Mayo Foundation for Medical Education and Research, Hypoplastic Left Heart Syndrome, http://www.mayoclinic.org/diseases-conditions/hypoplastic-left-heart-syndrome/basics/symptoms/con-20031294?reDate=25042015, 1998-2015. Web. 12 April 2015
^ ^a ^b Reviewed by David J. Goldberg, MD, About Hypoplastic Left Heart Syndrome (HLHS), http://www.chop.edu/conditions-diseases/hypoplastic-left-heart-syndrome-hlhs/about#.VSppX7PnF_sY, 2013-2015. Web. 12 April 2015
^ American Heart Association, Single Ventricle Defects, http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefaects/AboutCongenitalHeartDefects/Single-Ventricle-Defects_UCM_307037_Article.jsp, 2015. Web. 12 April 2015

^[1]

^[2]

^[3]

^[4]

^[5]

^[6]

^[7]

^ .Fontan F, Baudet E (1971). "Surgical repair of tricuspid atresia". Thorax 26 (3): 240–8. doi:10.1136/thx.26.3.240. PMC 1019078. PMID 5089489.
^ Gillers, Benjamin S., Aditi Chiplunkar, Haytham Aly, Tomas Valenta, Konrad Basler, Vincent M. Christoffels, Igor R Efimov, Bastiaan J Boukens, and Stacey Rentschler. 2014. “Canonical Wnt Signaling Regulates Atrioventricular Junction Programming and Electrophysiological Properties.” P. 1-39. DOI: 10.1161/CIRCRESAHA.116.304731
^ Hajrije Ismaili, Lindita Kryeziu, Burbuqe Skenderi Mustafa, Ramush Bejiqi. 2010. Early Human Development. Hypoplastic Right Heart Syndrome. 86: S41. doi:10.1016/j.earlhumdev
^ . Kobylińska J1, Dworzański W, Cendrowska-Pinkosz M, Dworzańska A, Hermanowicz-Dryka T, Kiszka J, Starosławska E, Burdan F. Sept 2013. Morphological and molecular bases of cardiac development. 10;67:950-7. doi: 10.5604/17322693.1066060
^ MacGrogan Donal, Meritxell Nus, José Luis de la Pompa. 2010. Current Topics in Developmental Biology. Notch Signaling. 92: 333–365. doi:10.1016/S0070- 2153(10)92011-5
^ Norwood, WI; Lang, P; Casteneda, AR; Campbell, DN (October 1981). "Experience with operations for hypoplastic left heart syndrome.". The Journal of thoracic and cardiovascular surgery 82 (4): 511–9. PMID 6168869.
^ Yuan SM, Jing H (2009). "Palliative procedures for congenital heart defects". Arch Cardiovasc Dis 102 (6-7): 549–57. doi:10.1016/j.acvd.2009.04.011. PMID 19664575.

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 新生児におけるチアノーゼの心臓の原因 cardiac causes of cyanosis in the newborn
2. Anesthesia for adults with congenital heart disease undergoing noncardiac surgery
3. Eisenmenger症候群の評価および予後 evaluation and prognosis of eisenmenger syndrome
4. 重症先天性心疾患を有する新生児の識別 identifying newborns with critical congenital heart disease
5. 右室心筋梗塞 right ventricular myocardial infarction

English Journal

Right Ventricular Mechanical Dyssynchrony and Asymmetric Contraction in Hypoplastic Heart Syndrome are Associated with Tricuspid Regurgitation.

Bharucha T, Khan R, Mertens L, Friedberg MK.SourceLabatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada.
Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.J Am Soc Echocardiogr.2013 Oct;26(10):1214-20. doi: 10.1016/j.echo.2013.06.015. Epub 2013 Jul 19.
BACKGROUND: Right ventricular (RV) dysfunction and tricuspid regurgitation (TR) are associated with adverse outcome in hypoplastic left heart syndrome (HLHS) but remain poorly understood. The aim of this study was to assess RV longitudinal strain and mechanical dyssynchrony in HLHS in relation to TR
PMID 23876994

Right ventricular and tricuspid valve remodeling after bidirectional cavopulmonary anastomosis.

Ugaki S, Khoo NS, Ross DB, Rebeyka IM, Adatia I.SourceDivision of Pediatric Cardiac Surgery and Cardiology and Critical Care, Stollery Children's Hospital, University of Alberta, Mazankowski Heart Institute.
Circulation journal : official journal of the Japanese Circulation Society.Circ J.2013 Sep 25;77(10):2514-8. Epub 2013 Jul 20.
Background: There are few investigations of the changes in tricuspid valve (TV) and right ventricular (RV) morphology following bidirectional cavopulmonary anastomosis (BCPA). Methods and Results: The 2-D echocardiograms of 35 children (male, n=23; female, n=12; median age, 6 months; range, 3-10
PMID 23877731

Noninvasive Estimation of Ventricular Filling Pressures in Patients with Single Right Ventricles.

Husain N, Gokhale J, Nicholson L, Cheatham JP, Holzer RJ, Cua CL.SourceFrom The Heart Center, Nationwide Children's Hospital, Columbus, Ohio. Electronic address: naziahusain@gmail.com.
Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.J Am Soc Echocardiogr.2013 Sep 12. pii: S0894-7317(13)00628-7. doi: 10.1016/j.echo.2013.08.002. [Epub ahead of print]
BACKGROUND: Increased ventricular end-diastolic pressure (VEDP) is a known risk factor for morbidity and mortality in patients with single-ventricle physiology. Spectral Doppler tissue imaging (DTI) has been shown to modestly correlate with direct measurement of VEDP. Strain rate (SR) values, obtain
PMID 24035700

Japanese Journal

Norwood procedure with non-valved right ventricle to pulmonary artery shunt improves ventricular energetics despite the presence of diastolic regurgitation : a theoretical analysis

SHIMIZU Shuji,UNE Dai,SHISHIDO Toshiaki,KAMIYA Atsunori,KAWADA Toru,SANO Shunji,SUGIMACHI Masaru
The journal of physiological sciences : JPS 61(6), 457-465, 2011-11-01
NAID 10030350907

Real-time three-dimensional color Doppler fetal echocardiographic features of congenital heart disease

HATA Toshiyuki,DAI Shu-Yan,INUBASHIRI Eisuke,KANENISHI Kenji,TANAKA Hirokazu,YANAGIHARA Toshihiro,NUKAZAWA Takashi
The journal of obstetrics and gynaecology research 34(4), 670-673, 2008-08-31
NAID 10025337955