WordNet

desert (a cause, a country or an army), often in order to join the opposing cause, country, or army; "If soldiers deserted Hitlers army, they were shot" (同)desert
an imperfection in an object or machine; "a flaw caused the crystal to shatter"; "if there are any defects you should send it back to the manufacturer" (同)fault, flaw
an imperfection in a bodily system; "visual defects"; "this device permits detection of defects in the lungs"
a failing or deficiency; "that interpretation is an unfortunate defect of our lack of information" (同)shortcoming
of or relating to a ventricle (of the heart or brain)
of or relating to a septum (同)septate

PrepTutorEJDIC

〈C〉『欠点』,弱点,欠陥 / 〈U〉〈C〉《古》不足,欠乏 / (国・党・主義などから)離脱する,裏切って出る《+『from』+『名』》;離反して(…に)転向する《+『to』+『名』》

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/07/28 16:06:20」(JST)

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A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart.

The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes.

The membranous portion, which is close to the atrioventricular node, is most commonly affected in adults and older children in the United States.^[1] It is also the type that will most commonly require surgical intervention, comprising over 80% of cases.^[2]

Membranous ventricular septal defects are more common than muscular ventricular septal defects, and are the most common congenital cardiac anomaly.^[3]

1 Signs and symptoms
2 Cause
3 Pathophysiology
4 Diagnosis
- 4.1 Classification
  - 4.1.1 Multiple
  - 4.1.2 Type 1 (Subarterial) (Supracristal) (Conal septal defect) (Infundibular)
  - 4.1.3 Type 2 (Perimembranous) (Paramembranous) (Conoventricular)
  - 4.1.4 Type 3 (Inlet) (AV canal type)
  - 4.1.5 Type 4 (Muscular)
  - 4.1.6 Type: Gerbode (Left ventricular to right atrial communication)
5 Treatment
- 5.1 Transcatheter closure
- 5.2 Surgery
6 Epidemiology
7 See also
8 References
9 External links

Signs and symptoms

Ventricular septal defect is usually symptomless at birth. It usually manifests a few weeks after birth.

VSD is an acyanotic congenital heart defect, aka a Left-to-right shunt, so there are no signs of cyanosis in the early stage. However, uncorrected VSD can increase pulmonary resistance leading to the reversal of the shunt and corresponding cyanosis.

Pansystolic (Holosystolic) murmur along lower left sternal

border(depending upon the size of the defect) +/- palpable thrill (palpable turbulence of blood flow). Heart sounds are normal. Larger VSDs may cause a parasternal heave, a displaced apex beat (the palpable heartbeat moves laterally over time, as the heart enlarges). An infant with a large VSD will fail to thrive and become sweaty and tachypnoeic (breathe faster) with feeds.^[4]

The restrictive VSDs (smaller defects) are associated with a louder murmur and more palpable thrill (grade IV murmur). Larger defects may eventually be associated with pulmonary hypertension due to the increased blood flow. Over time this may lead to an Eisenmenger's_syndrome the original VSD operating with a left-to-right shunt, now becomes a right-to-left shunt because of the increased pressures in the pulmonary vascular bed.

Cause

Congenital VSDs are frequently associated with other congenital conditions, such as Down syndrome.^[5]

A VSD can also form a few days after a myocardial infarction^[6] (heart attack) due to mechanical tearing of the septal wall, before scar tissue forms, when macrophages start remodeling the dead heart tissue.

The cause of VSD (ventricular septal defect) includes the incomplete looping of the heart during days 24-28 of development. Faults with NKX2.5 gene can cause this.

Pathophysiology

During ventricular contraction, or systole, some of the blood from the left ventricle leaks into the right ventricle, passes through the lungs and reenters the left ventricle via the pulmonary veins and left atrium. This has two net effects. First, the circuitous refluxing of blood causes volume overload on the left ventricle. Second, because the left ventricle normally has a much higher systolic pressure (~120 mmHg) than the right ventricle (~20 mmHg), the leakage of blood into the right ventricle therefore elevates right ventricular pressure and volume, causing pulmonary hypertension with its associated symptoms.

In serious cases, the pulmonary arterial pressure can reach levels that equal the systemic pressure. This reverses the left to right shunt, so that blood then flows from the right ventricle into the left ventricle, resulting in cyanosis, as blood is by-passing the lungs for oxygenation.^[7]

This effect is more noticeable in patients with larger defects, who may present with breathlessness, poor feeding and failure to thrive in infancy. Patients with smaller defects may be asymptomatic. Four different septal defects exist, with perimembranous most common, outlet, atrioventricular, and muscular less commonly.^[8]

Diagnosis

Echocardiographic image of a moderate ventricular septal defect in the mid-muscular part of the septum. The trace in the lower left shows the flow during one complete cardiac cycle and the red mark the time in the cardiac cycle that the image was captured. Colours are used to represent the velocity of the blood. Flow is from the left ventricle (right on image) to the right ventricle (left on image). The size and position is typical for a VSD in the newborn period.

A VSD can be detected by cardiac auscultation. Classically, a VSD causes a pathognomonic holo- or pansystolic murmur. Auscultation is generally considered sufficient for detecting a significant VSD. The murmur depends on the abnormal flow of blood from the left ventricle, through the VSD, to the right ventricle. If there is not much difference in pressure between the left and right ventricles, then the flow of blood through the VSD will not be very great and the VSD may be silent. This situation occurs a) in the fetus (when the right and left ventricular pressures are essentially equal), b) for a short time after birth (before the right ventricular pressure has decreased), and c) as a late complication of unrepaired VSD. Confirmation of cardiac auscultation can be obtained by non-invasive cardiac ultrasound (echocardiography). To more accurately measure ventricular pressures, cardiac catheterization, can be performed.

Classification

Although there are several classifications for VSD, the most accepted and unified classification is that of Congenital Heart Surgery Nomenclature and Database Project.^[9] The classification is based on the location of the VSD on the right ventricular surface of the inter ventricular septum and is as follows:

Multiple

Type 1 (Subarterial) (Supracristal) (Conal septal defect) (Infundibular)

Found in 5-7% of patients, more in Asians, related to the pulmonary valve

Type 2 (Perimembranous) (Paramembranous) (Conoventricular)

Most common variety found in 70%

Type 3 (Inlet) (AV canal type)

Commonly associated with atrioventricular septal defect, found in about 5%

Type 4 (Muscular)

Located in the muscular septum, found in 20%. Can be sub classified again based on the location into anterior, apical, posterior and mid

Type: Gerbode (Left ventricular to right atrial communication)

Due to absence of Atrioventricular septum.

Heart anatomic view of right ventricle and right atrium with example ventricular septal defects
Ventricular septal defect
Figure A shows the structure and blood flow in the interior of a normal heart. Figure B shows two common locations for a ventricular septal defect. The defect allows oxygen-rich blood from the left ventricle to mix with oxygen-poor blood in the right ventricle.

Treatment

A nitinol device for closing muscular VSDs, 4 mm diameter in the centre. It is shown mounted on the catheter into which it will be withdrawn during insertion.

Most cases do not need treatment and heal at the first years of life. Treatment is either conservative or surgical. Smaller congenital VSDs often close on their own, as the heart grows, and in such cases may be treated conservatively. Some cases may necessitate surgical intervention, i.e. with the following indications:

1. Failure of congestive cardiac failure to respond to medications

2. VSD with pulmonic stenosis

3. Large VSD with pulmonary hypertension

4. VSD with aortic regurgitation

For the surgical procedure, a heart-lung machine is required and a median sternotomy is performed. Percutaneous endovascular procedures are less invasive and can be done on a beating heart, but are only suitable for certain patients. Repair of most VSDs is complicated by the fact that the conducting system of the heart is in the immediate vicinity.

Ventricular septum defect in infants is initially treated medically with cardiac glycosides (e.g., digoxin 10-20 µg/kg per day), loop diuretics (e.g., furosemide 1–3 mg/kg per day) and ACE inhibitors (e.g., captopril 0.5–2 mg/kg per day).

Transcatheter closure

A device, known as the Amplatzer muscular VSD occluder, may be used to close certain VSDs.^[10] It was initially approved in 2009.^[10] They appear to work well and be safe.^[10] The cost is also lower than having open heart surgery.^[10] The device is placed through a small cut in the groin.^[11]

The Amplatzer septal occluder was shown to have full closure of the ventricular defect within the 24 hours of placement.^[12] It has a low risk of embolism after implantation.^[13] Some tricuspid valve regurgitation was shown after the procedure that could possibly be due from the right ventricular disc.^[12] There have been some reports that the Amplatzer septal occluder may cause life-threatening erosion of the tissue inside the heart.^[14] This occurs in one percent of people implanted with the device and requires immediate open-heart surgery.^[14] This erosion occurs due to improper sizing of the device resulting with it being too large for the defect, causing rubbing of the septal tissue and erosion.^[14]

Surgery

a) Surgical closure of a Perimembranous VSD is performed on cardiopulmonary bypass with ischemic arrest. Patients are usually cooled to 28 degrees. Percutaneous Device closure of these defects is rarely performed in the United States because of the reported incidence of both early and late onset complete heart block after device closure, presumably secondary to device trauma to the AV node.

b) Surgical exposure is achieved through the right atrium. The tricuspid valve septal leaflet is retracted or incised to expose the defect margins.

c) Several patch materials are available, including native pericardium, bovine pericardium, PTFE (Gore-Tex or Impra), or Dacron.

d) Suture techniques include horizontal pledgeted mattress sutures, and running polypropylene suture.

e) Critical attention is necessary to avoid injury to the conduction system located on the left ventricular side of the interventricular septum near the papillary muscle of the conus.

f) Care is taken to avoid injury to the aortic valve with sutures.

g) Once the repair is complete, the heart is extensively deaired by venting blood through the aortic cardioplegia site, and by infusing Carbon Dioxide into the operative field to displace air.

h) Intraoperative transesophageal echocardiography is used to confirm secure closure of the VSD, normal function of the aortic and tricuspid valves, good ventricular function, and the elimination of all air from the left side of the heart.

i) The sternum, fascia and skin are closed, with potential placement of a local anesthetic infusion catheter under the fascia, to enhance postoperative pain control.

j) A video of Perimembranous VSD repair, including the operative technique, and the daily postoperative recovery, can be seen here: VSD Repair, Perimembranous Ventricular Septal Defect i

Epidemiology

VSDs are the most common congenital cardiac abnormalities. They are found in 30-60% of all newborns with a congenital heart defect, or about 2-6 per 1000 births. During heart formation, when the heart begins life as a hollow tube, it begins to partition, forming septa. If this does not occur properly it can lead to an opening being left within the ventricular septum. It is debatable whether all those defects are true heart defects, or if some of them are normal phenomena, since most of the trabecular VSDs close spontaneously.^[15] Prospective studies give a prevalence of 2-5 per 100 births of trabecular VSDs that closes shortly after birth in 80-90% of the cases.^[16]^[17]

References

^ Taylor, Michael D. "Muscular Ventricular Septal Defect". eMedicine. Medscape.
^ Waight, David J.; Bacha, Emile A.; Kahana, Madelyn; Cao, Qi-Ling; Heitschmidt, Mary; Hijazi, Ziyad M. (March 2002). "Catheter therapy of Swiss cheese ventricular septal defects using the Amplatzer muscular VSD occluder". Catheterization and Cardiovascular Interventions 55 (3): 355–361. doi:10.1002/ccd.10124. PMID 11870941.
^ Hoffman, JI; Kaplan, S (2002). "The incidence of congenital heart disease". Journal of the American College of Cardiology 39 (12): 1890–900. doi:10.1016/S0735-1097(02)01886-7. PMID 12084585.
^ Cameron P. et al: Textbook of Pediatric Emergency Medicine. p116-117 [Elsevier, 2006]
^ Wells, GL; Barker, SE; Finley, SC; Colvin, EV; Finley, WH (1994). "Congenital heart disease in infants with Down's syndrome". Southern Medical Journal 87 (7): 724–7. doi:10.1097/00007611-199407000-00010. PMID 8023205.
^ Schumacher, Kurt R. "Ventricular septal defect". NIH and US National Library of Medicine. MedlinePlus.
^ Kumar & Clark 2009
^ Mancini, Mary C. "Ventricular Septal Defect Surgery in the Pediatric Patient". eMedicine. Medscape.
^ Jacobs, Jeffrey; Mavroudis, Constantine (March 2000). "Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect". Ann Thorac Surg 69 (3): 25–35. doi:10.1016/S0003-4975(99)01270-9. PMID 10798413. |accessdate= requires |url= (help)
^ ^a ^b ^c ^d Fu, YC (February 2011). "Transcatheter device closure of muscular ventricular septal defect.". Pediatrics and neonatology 52 (1): 3–4. PMID 21385649.
^ Amplatzer septal occluder. (2013) U.S. Food and Drug Administration. Retrieved February 26, 2014, from http://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/DeviceApprovalsandClearances/Recently-ApprovedDevices/ucm083978.htm
^ ^a ^b Szkutnik et al. (2007) “Use of the Amplatzer muscular ventricular septal defect occluder for closure of perimembranous ventricular septal defects.” Heart. 93:355–358.
^ Fernando, Rajeev et al. (2013). “Patent ductus arteriosus closure using an Amplatzer™ ventricular septal defect closure device ”. Experimental & Clinical Cardiology, 18(1), e50-e54.
^ ^a ^b ^c Rare Serious Erosion Events Associated with St. Jude Amplatzer Atrial Septal Occluder (ASO). (2013, October 17). U.S. Food and Drug Administration. Retrieved February 26, 2014, from http://www.fda.gov/MedicalDevices/Safety/AlertsandNotices/ucm371145.htm?source=govdelivery&utm_medium=email&utm_source=govdelivery
^ Meberg, A; Otterstad, JE; Frøland, G; Søarland, S; Nitter-Hauge, S (1994). "Increasing incidence of ventricular septal defects caused by improved detection rate". Acta Pædiatrica 83 (6): 653–657. doi:10.1111/j.1651-2227.1994.tb13102.x.
^ Hiraishi, S; Agata, Y; Nowatari, M; Oguchi, K; Misawa, H; Hirota, H; Fujino, N; Horiguchi, Y; Yashiro, K; Nakae, S (March 1992). "Incidence and natural course of trabecular ventricular septal defect: two-dimensional echocardiography and color Doppler flow imaging study.". The Journal of pediatrics 120 (3): 409–15. doi:10.1016/s0022-3476(05)80906-0. PMID 1538287.
^ Roguin, Nathan; Du, Zhong-Dong; Barak, Mila; Nasser, Nadim; Hershkowitz, Sylvia; Milgram, Elliot (15 November 1995). "High prevalence of muscular ventricular septal defect in neonates". Journal of the American College of Cardiology 26 (6): 1545–1548. doi:10.1016/0735-1097(95)00358-4. PMID 7594083.

External links

Ventricular Septal Defect (VSD) - Stanford Children's Health
Pediatric Heart Surgery
Ventricular septal defect - American Heart Association
Ventricular Septal Defect information from Seattle Children's Hospital Heart Center
C.S. Mott Children's Hospital, Congenital Heart Center: Ventricular Septal Defect at umich.edu
Ventricular septal defect information for parents.

UpToDate Contents

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1. 幼児および小児における孤立性心室中隔欠損の病態生理および臨床的特徴 pathophysiology and clinical features of isolated ventricular septal defects in infants and children
2. 成人の心室中隔欠損症 ventricular septal defect in adults
3. 幼児および小児の孤立性心室中隔欠損のマネージメント management of isolated ventricular septal defects in infants and children
4. 心室中隔欠損の心エコー評価 echocardiographic evaluation of ventricular septal defects
5. L型大血管転位 l transposition of the great arteries

English Journal

Transposition of the great arteries.

Squarcia U, Macchi C.SourceDipartimento dell'eta evolutiva, Cattedra di Cardiologia Pediatrica, Universita degli Studi di Parma, Parma, Italy.
Current opinion in pediatrics.Curr Opin Pediatr.2011 Oct;23(5):518-22.
PURPOSE OF REVIEW: Transposition of the great arteries (TGA) is a complex congenital heart defect usually defined within the group of conotruncal defects. Some astonishing similarities between the spiral pattern of great arteries and the spiral pattern of snail shells and a possible common genetic m
PMID 21849902

A novel GATA4 mutation responsible for congenital ventricular septal defects.

Wang J, Fang M, Liu XY, Xin YF, Liu ZM, Chen XZ, Wang XZ, Fang WY, Liu X, Yang YQ.SourceDepartment of Cardiology, East Hospital, Tongji University School of Medicine, Shanghai, PR China.
International journal of molecular medicine.Int J Mol Med.2011 Oct;28(4):557-64. doi: 10.3892/ijmm.2011.715. Epub 2011 Jun 1.
Ventricular septal defect (VSD) is the most common type of cardiovascular developmental anomaly and is an important risk factor for the substantially increased morbidity and mortality in newborns. Aggregating evidence implicates genetic defects in the pathogenesis of congenital VSD. However, VSD is
PMID 21637914

Japanese Journal

Heterotaxyの診断，病態，合併症

小川潔
Pediatric Cardiology and Cardiac Surgery 30(2), 89-96, 2014
Heterotaxyは胸腹部臓器が体の左右軸に対して異常な位置関係を示すことと定義され，複合心奇形や幅広い心外病変を伴う疾患群である．右側相同では共通房室弁や円錐動脈幹異常，肺動脈狭窄／閉鎖，体・肺静脈還流異常が多く，左側相同では両側上大静脈，近位部下大静脈欠損を伴う奇静脈結合，心室中隔欠損，房室中隔欠損，左室流出路狭窄が多い．左側相同では先天性心疾患を伴わないこともあり，合併する心疾患も比較 …
NAID 130003398263

胎児心臓超音波スクリーニング検査で発見された心疾患症例と見逃された症例の検討

山下美智子,日高庸博,林周作,入江明美,宮城晶子,河津由紀子,稲村昇,萱谷太,石井桂介,光田信明
Pediatric Cardiology and Cardiac Surgery 30(2), 166-172, 2014
目的：胎児心臓超音波スクリーニングで発見された心疾患症例と見逃された症例を検討し，検査の効果と問題点を検証すること．方法：2008年4月～2012年3月に，当院で胎児超音波スクリーニングを受け分娩に至ったローリスク単胎妊婦を対象とした．検査陽性症例および偽陰性症例の内訳を診療録より後方視的に検討した．検査は妊娠18週前後，28週前後，36週前後の3回行った．結果：対象期間の検査のべ回数は9,983 …
NAID 130003398252

Transcatheter Closure of Late-Onset Residual Aortopulmonary Septal Defect Using a Muscular Ventricular Septal Occluder

Li Xiao,Zhu Da,Feng Yuan
International Heart Journal, 2014
… Late-onset residual shunt after surgical repair of aortopulmonary septal defect (APSD) is a rare event complicating the management strategy. … We report a case of successful treatment of this type of residual shunt using a transcatheter closure technique with an infrequently used muscular ventricular septal device. …
NAID 130003392061

Related Pictures

★リンクテーブル★

リンク元	「心室中隔欠損症」「VSD」「心室中隔欠損」
拡張検索	「ventricular septal defects」「atrioventricular septal defects」
関連記事	「defect」「ventricular」「septal」「septal defect」

「心室中隔欠損症」

Ventricular septal defect
	Illustration showing various forms of a ventricular septal defects.; 1. Conoventricular, malaligned; 2. Perimembranous; 3. Inlet; 4. Muscular
Classification and external resources
Specialty	Cardiac surgery
ICD-10	Q21.0
ICD-9-CM	745.4
DiseasesDB	13808
MedlinePlus	001099
eMedicine	med/3517
MeSH	C14.240.400.560.540

　　[★]

英: ventricular septal defect, VSD
同: 心室中隔欠損
関: 先天性心疾患、動脈管開存症、心房中隔欠損症

[show details]

心室中隔欠損 : 約 175,000 件
心室中隔欠損症 : 約 122,000 件

概念

心室中隔の一部に組織欠損ができ(欠損孔)、左室と右室の間に交通がある先天性心疾患.
最も頻度の高い先天性心疾患で、出生児心奇形の約30%を占める。
他の奇形を合併することがある。
乳幼児期に自然閉鎖する場合がある。

疫学

1.5-3.5/1000生産児 (PHD.382)
先天性心疾患のうち発症例は最も多いが、自然閉鎖や幼小児期の手術により、成人例の頻度は心房中隔欠損よりも少数であり、約15%である。

病型分類

Kirklinの分類で分類されているが、新たな分類を加えているものもある。欠損孔と刺激伝導系の位置関係を強調した、Sotoらの分類も知られている。

Kirklinの分類
型	欠損部		疫学	病態
I型	漏斗部欠損	高位欠損	日本人に多い。	大動脈弁逸脱(右冠尖)によるARを合併しうる。自然閉鎖は少ない
II型	膜性部欠損	中間位欠損	最多。(70%)
III型	流入部欠損	後方欠損	ダウン症に多い	左軸偏位
IV型	筋性部欠損	低位欠損	西洋人に多い。(20%)

重症度分類

	X線上心拡大	心電図	聴診	心不全症状
小欠損	-	正常	Roger雑音	-
中欠損	+	左室肥大	汎収縮期雑音	-./+
大欠損	+	両心室肥大	II音亢進	+(乳児初期)

病態生理

PHD.382

欠損孔を流れる血液量：(small VSD)欠損孔のサイズに依存。(large VSD)体循環と肺循環の血管抵抗に依存

容量負荷：RV, 肺循環, LV, LA
容量負荷　→　chamber dilation →　systolic dysfunction　→　heart failure
2歳でpulmonary vascular diseaseとなる。

心室中隔の欠損孔の大きさと肺動脈血管床の状態(左右心室間の圧較差)が病態を決定する。
左室から右室への容量負荷は肺血管を介して左心負荷につながり、肺動脈動脈圧が上昇すれば、右心負荷に繋がる。

症状

PHD.383

small VSDは無症状
10%のVSD患者はlarge defectを持っており、生後早い内から、うっ血心不全を呈する(頻呼吸、摂食不良、成長不良、頻回の下気道感染)
right-left shuntを起こせばチアノーゼ、呼吸困難
VSDのサイズに因らず、bacterial endocarditisがおこりうる。

乳幼児：生後まもなく心雑音にて発見されることが多い。欠損孔の大きさに応じて左室から右室への血流が増加し、それによる心不全徴候を示す(多呼吸、哺乳低下、体重増加不良)。
小欠損孔では自覚症状はない。
中欠損孔では反復性気道感染、息切れを認めることがある。
大欠損孔では乳児期から心不全、体重増加不良を認める。

身体所見

PHD.383

harsh holosystolic murmur at the left sternal border
murmurの部位に一致してsystolic thrillを認める。
拡張中期ランブルを心尖で聴取　→　MV経由の血流が増加したため

pulmonaru vascular diseaseが進行すると、汎収縮期雑音は減弱　→　欠損孔の圧較差が低下してくるから

RV heave, P2音増強、チアノーゼ

聴診

聴診にて胸骨左縁第 3-4 肋間に最強点(I型では第 2 肋間)を有する粗い汎収縮期雑音を聴取し、しばしば振戦を触知する。
左右短絡量が多い症例では、心尖部に III音、拡張中期ランブルを聴取する(僧帽弁口を通過する血流増大を反映)。

PHD.383

左胸骨縁：全収縮期雑音：(harsh holosystolic murmur)　←　欠損孔が小さいほど大きい
雑音がする部位：収縮期スリルを触れる
心尖部：拡張期中期ランブル音(middiastolic rumble)：僧帽弁を通過する血流が増加するため
肺高血圧症が進展すると全収縮期雑音は小さくなる。RV heave(拡張した右室の拍動)、II音亢進、チアノーゼを認めるようになる。

検査

胸部単純X線写真

小欠損孔：正常
中-大欠損孔では肺血管陰影増強、左第4弓の突出がみられる。
Eisenmenger 化すると左第2弓の著明な突出を認めるが、末梢血管影は減少する。

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12誘導心電図

小欠損孔：正常である。
中欠損孔：左室肥大
大欠損孔：左室肥大、右室肥大
Eisenmenger化すると右室肥大のみを呈する。
III型は左軸偏位を呈する。

心エコー

左室から右室へのジェットが確認される

心臓カテーテル検査

手術療法の適応について評価する；短絡率、肺体血流比、肺血管抵抗

診断

胸骨左縁の粗い汎収縮期雑音で本症を疑う。心エコー図により欠損孔、短絡血流を証明し、さらに病型、欠損孔の大きさと数、左右短絡量、肺高血圧の有無を診断する。

鑑別診断

前胸部に粗い収縮期雑音が聴取される疾患：僧帽弁閉鎖不全症、閉塞性肥大型心筋症、肺動脈弁狭窄、右室二腔症、左室右房交通症。

治療

欠損孔が小さい場合(small VSD)：心内膜炎の予防以外に特に特別な治療は不要である。しかし、血液培養陽性となる発熱や新規の雑音が聴取されたら専門医による精査を要する。(参考1)
肺体血液量比2.0以上は手術適応、1.5-2.0は症状に応じて手術を、1.5以下は手術不要。

小欠損：通常手術は不要であるが、大動脈弁逸脱を合併するものは手術が必要である。
中欠損：左右短絡率が40%以上で手術適応がある。
大欠損：心不全に対し薬物治療を行うとともに全例手術を2～3歳までに行う。

肺高血圧が遷延し、肺血管病変を呈した幼小児期以降の例では手術は禁忌である。
手術は、体外循環下のパッチ閉鎖が原則である。

自然歴

自然閉鎖から小児期に早くから心不全症状を呈し、死亡する例まで多岐にわたる。肺血管閉塞、右室流出路狭窄、大動脈弁閉鎖不全症、感染性心内膜炎などを合併することがある。

参考

1. [charged] Management of isolated ventricular septal defects in infants and children - uptodate [1]

国試

095G018：小欠損と考えられる。小欠損の場合には特に運動制限をする必要はないが、心内膜炎の予防のために予防接種などを受けることは必要となる。2007 guidelines of the American Heart Association (AHA) recommendによれば、予防的な抗菌薬の服用は推奨されていない。(参考1)
105F026、105F027、103I065