WordNet

having three cusps or points (especially a molar tooth); "tricuspid molar"; "tricuspid valve" (同)tricuspidate
an abnormal condition in which a normal opening or tube in the body (as the urethra) is closed or absent

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(歯が)三尖の / (歯の)三尖頭

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/09/07 17:03:01」(JST)

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Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing. It causes the heart to be unable to properly oxygenate the rest of the blood in the body. Because of this, the body does not have enough oxygen to live, so other defects must occur to maintain blood flow. Because of the lack of an A-V connection, an atrial septal defect (ASD) must be present to fill the left ventricle with blood. Also, since there is a lack of a right ventricle there must be a way to pump blood into the pulmonary arteries, and this is accomplished by a ventricular septal defect (VSD).

An atrial septal defect (ASD) and a ventricular septal defect (VSD) must BOTH be present to maintain blood flow-from the right atrium, the blood must flow through the ASD to the left atrium to the left ventricle and through the VSD to the right ventricle to allow access to the lungs

Clinical manifestations

progressive cyanosis
poor feeding
tachypnea over the first 2 weeks of life
holosystolic murmur due to the VSD
left axis deviation on electrocardiography and left ventricular hypertrophy (since it must pump blood to both the pulmonary and systemic systems)
normal heart size

Treatment

PGE1 to maintain patent ductus arteriosus
modified Blalock-Taussig shunt to maintain pulmonary blood flow by placing a Gore-Tex conduit between the subclavian artery and the pulmonary artery.
cavopulmonary anastomosis (hemi-Fontan or bidirectional Glenn) to provide stable pulmonary flow
Fontan procedure to redirect inferior vena cava and hepatic vein flow into the pulmonary circulation

External links

Tricuspid Atresia (TA) - Stanford Children's Health
Tricuspid Atresia information from Seattle Children's Hospital Heart Center

UpToDate Contents

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1. 三尖弁（TV）閉鎖症 tricuspid valve tv atresia
2. 純型肺動脈閉鎖症（PA/IVS） pulmonary atresia with intact ventricular septum pa ivs
3. 房室（AV）管欠損のマネージメントおよび転帰 management and outcome of atrioventricular av canal defects
4. Pulmonic stenosis (PS) in neonates, infants, and children
5. 肺閉鎖症および主要大動脈肺動脈側副動脈（TOF/ PA/ MAPCA）を伴うファロー四徴症 tetralogy of fallot with pulmonary atresia and major aortopulmonary collateral arteries tof pa mapcas

English Journal

The Association of H1N1 Pandemic Influenza with Congenital Anomaly Prevalence in Europe: An Ecological Time Series Study.

Luteijn JM1, Addor MC, Arriola L, Bianchi F, Garne E, Khoshnood B, Nelen V, Neville A, Queisser-Luft A, Rankin J, Rounding C, Verellen-Dumoulin C, de Walle H, Wellesley D, Wreyford B, Yevtushok L, de Jong-van den Berg L, Morris J, Dolk H.
Epidemiology (Cambridge, Mass.).Epidemiology.2015 Aug 28. [Epub ahead of print]
BACKGROUND: In the context of the European Surveillance of Congenital Anomalies (EUROCAT) surveillance response to the 2009 influenza pandemic, we sought to establish whether there was a detectable increase of congenital anomaly prevalence among pregnancies exposed to influenza seasons in general, a
PMID 26327589

Surgical management of symptomatic neonates with Ebstein's anomaly: choice of operation.

Knott-Craig CJ1, Kumar TK1, Arevalo AR, Joshi VM2.
Cardiology in the young.Cardiol Young.2015 Aug;25(6):1119-23. doi: 10.1017/S1047951114001747. Epub 2014 Sep 24.
OBJECTIVE: Symptomatic neonates with Ebstein's anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset.METHODS: A total of 32 consecutive symptomatic neonates with Ebstein
PMID 25248286

Limb ischaemia and below-knee amputation following life-saving patent ductus arteriosus stent in a critically ill infant.

Bharmanee A1, Gowda S1, Singh HR1.
Cardiology in the young.Cardiol Young.2015 Aug;25(6):1206-9. doi: 10.1017/S104795111400167X. Epub 2014 Sep 9.
Limb ischaemia is a rare but catastrophic complication related to cardiac catheterisation. We report an infant weighing 3 kg with unrepaired tricuspid atresia type 1b, small patent ductus arteriosus, and ventricular septal defect presenting with cardiogenic shock owing to progressively reduced pulmo
PMID 25200991

Japanese Journal

The Single Center Experience of Pacing Device Implantation in Patients with Complex Congenital Heart Disease

Manaka Tetsuyuki,Shoda Morio,Ejima Koichiro,Yashiro Bun,Yagishita Daigo,Wakisaka Osamu,Hagiwara Nobuhisa,Takeuchi Daiji,Toyohara Keiko
Journal of Arrhythmia 27(Supplement), OP46_1-OP46_1, 2011
… The underlying heart diseases were tricuspid atresia (n=2) and single RV (n=1) both after Fontan operation, single LV (n=1) underwent the septation, truncus arteiosus communis underwent Rastelli (n=1), transposition of great ateries (TGA) (n=9) and corrected TGA (n=12; …
NAID 130002130183

Rudimentary right ventricle to pulmonary artery shunt in the Norwood procedure

TATEISHI Atsushi,KAWADA Masaaki,MORITA Hideki,TAKEUCHI Mamoru,TAGA Naoyuki,OTSUKA Yoji,OKADA Osamu,KATAOKA Koichi
General thoracic and cardiovascular surgery 58(12), 633-635, 2010-12-10
NAID 10027671616

三尖弁閉鎖症を合併した歯科治療患者の鎮静管理経験 : 近赤外線酸素モニターの応用

四戸豊,小川さおり,坂本望,佐藤健一,佐藤雅仁,城茂治
岩手医科大学歯学雑誌 34(2), 51-58, 2009-08-20
… We experienced intravenous sedation management of a patient who had the tricuspid atresia (TA, 1b) and also investigated the change in intracerebral oxygen environment during intravenous sedation using a near-infrared oxygen monitor (NIRO). … He was diagnosed with tricuspid atresia just after birth. … Tricuspid atresia is a disease with a poor prognosis. …
NAID 110007339201

Related Pictures

★リンクテーブル★

リンク元	「三尖弁閉鎖症」「単心室症」「TA」
関連記事	「atresia」「tricuspid」

「三尖弁閉鎖症」

Tricuspid atresia
	Anterior (frontal) view of the opened heart. White arrows indicate normal blood flow. (Tricuspid valve labeled at bottom left.)
Classification and external resources
Specialty	medical genetics
ICD-10	Q22.4
ICD-9-CM	746.1
OMIM	605067
MedlinePlus	001110
eMedicine	med/2313
MeSH	D018785

　　[★]

英: tricuspid atresia TA
同: 三尖弁閉鎖
関: 先天性心疾患、三尖弁、単心室

まとめ

先天的に三尖弁が閉鎖している疾患であり、大血管転位の有無、肺動脈狭窄/閉塞などのバリエーションがあるが、共通して言えることは肺循環、体循環の血流が左心室のみから駆出されることにより、左室肥大、チアノーゼを呈する疾患ということである。治療は肺動脈血流量のコントロール(BAS/Blalock-Hanlon手術, PA banding)をした後に、根治的にFontan手術を行う。(YN.C-126 SSUR.372)