119:
299:
depending on the amount of blood flow through the anomalous connections. In less severe cases, with smaller amounts of blood flow, diagnosis may be delayed until adulthood, when it can be confused with other causes of pulmonary hypertension. There is also evidence that a significant number of mild cases are never diagnosed, or diagnosed incidentally. It is associated with other vascular anomalies, and some genetic syndromes such as
95:
33:
347:
During embryonic development, the lungs start as outgrowths from the foregut and share its splanchnic vascular plexus. At around 25 to 27 days of gestation, there are no direct links to the heart. By 27 to 29 days of gestation, the primitive pulmonary vein begins to form as an outgrowth from the left
298:
which is a life-threatening anomaly requiring emergent surgical correction, usually diagnosed in the first few days of life. Partial anomalous venous connection may be diagnosed at any time from birth to old age. The severity of symptoms, and thus the likelihood of diagnosis, varies significantly
254:
In TAPVC without obstruction, surgical redirection can be performed within the first month of life. The operation is performed under general anesthesia. The four pulmonary veins are reconnected to the left atrium, and any associated heart defects such as atrial septal defect, ventricular septal
335:
It is sometimes treated with surgery, which involves rerouting blood from the right atrium into the left atrium with a patch or use of the Warden procedure. However, interest is increasing in catheter-based interventional approaches, as well as medical therapy for less severe cases.
916:
897:
143:. It can be a total anomalous pulmonary venous connection, wherein all four pulmonary veins are incorrectly positioned, or a partial anomalous pulmonary venous connection, wherein only some of the pulmonary veins are incorrectly positioned.
242:
TAPVC with obstruction will clinically present with cyanosis, tachypnea, dyspnea, and hypoxemia. Chest x-ray may show a ground-glass appearance due to pulmonary edema resulting from increased pressure in the obstructed pulmonary veins.
231:, which occurs when the anomalous vein enters a vessel at an acute angle and can cause pulmonary venous hypertension and cyanosis because blood cannot enter the new vein as easily.
987:
344:
To understand the different types of anomalous pulmonary venous connection, it is important to establish the background in which normal fetal vasculature develops.
1189:
1185:
295:
980:
673:"Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage"
255:
defect, patent foramen ovale, and/or patent ductus arteriosus are surgically closed. With obstruction, surgery should be undertaken emergently.
327:. Unfortunately, less invasive and expensive testing, such as transthoracic echocardiography and CT scanning are generally less sensitive.
1238:
973:
1217:
1163:
69:
1158:
753:
ElBardissi AW, Dearani JA, Suri RM, Danielson GK (March 2008). "Left-sided partial anomalous pulmonary venous connections".
636:
Ho ML, Bhalla S, Bierhals A, Gutierrez F (May 2009). "MDCT of partial anomalous pulmonary venous return (PAPVR) in adults".
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392:
1089:
831:"The chest x-ray in congenital heart disease 1. Total anomalous pulmonary venous drainage and coarctation of the aorta"
43:
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allows oxygenated blood to go from the circulation of the right heart to the systemic circulation.
180:
790:"Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection"
1150:
1146:
1046:
209:
176:
460:"The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus"
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1099:
1084:
860:
842:
811:
770:
735:
714:"Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography"
694:
653:
618:
587:"Partial anomalous pulmonary venous return presenting with adult-onset pulmonary hypertension"
567:
528:
489:
419:
appearance similar to a cottage loaf, also known as the 'snow man' sign or 'figure of 8' sign.
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1117:
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224:; and a mixed (10%) variant, which can be a combination of any of the above connections.
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830:
788:
Alsoufi B, Cai S, Van
Arsdell GS, Williams WG, Caldarone CA, Coles JG (December 2007).
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672:
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Prasad SK, Soukias N, Hornung T, Khan M, Pennell DJ, Gatzoulis MA, et al. (2004).
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or directly into the right atrium; Infradiaphragmatic (20%), where blood drains into
173:
953:
712:
Ammash NM, Seward JB, Warnes CA, Connolly HM, O'Leary PW, Danielson GK (May 1997).
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375:
221:
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be present, or else the condition is fatal due to a lack of systemic blood flow.
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239:
The diagnosis of TAPVC is dependent upon whether or not there is an obstruction.
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atrium and eventually connects with the primitive pulmonary venous system.
739:
398:
364:
889:
204:
There are four variants: Supracardiac (50%): blood drains to one of the
508:
406:
312:
94:
901:
251:
Cases of TAPVC with obstruction are considered a surgical emergency.
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is the point of return for the blood from some (but not all) of the
1023:
409:
since the overloaded pulmonary circuit can cause pulmonary edema
256:
969:
389:
Snowman sign or 'figure of 8 configuration' on chest radiograph
438:"Facts about Total Anomalous Pulmonary Venous Return or TAPVR"
26:
386:
Right ventricular hypertrophy and right axis deviation on ECG
227:
TAPVC is further characterized on the presence or absence of
102:
Illustration of total anomalous pulmonary venous connection
172:
are malpositioned and make anomalous connections to the
51:
267:
Partial anomalous pulmonary venous connection (PAPVR)
48:
In particular, it has problems with not using MEDMOS.
879:
1208:
1172:
1145:
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1116:
1098:
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1022:
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939:
883:
147:
Total anomalous pulmonary venous connection (TAPVR)
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87:
507:Byard, Roger W.; Gilbert, John D. (2005-09-01).
201:In some cases, it can be detected prenatally.
981:
548:"Total Anomalous Pulmonary Venous Connection"
509:"Total anomalous pulmonary venous connection"
273:Partial anomalous pulmonary venous connection
208:(brachiocephalic veins), azygous vein or the
8:
296:total anomalous pulmonary venous connection
277:Partial anomalous pulmonary venous drainage
152:Total anomalous pulmonary venous connection
18:Total anomalous pulmonary venous connection
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988:
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84:
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729:
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585:Sears EH, Aliotta JM, Klinger JR (2012).
513:Forensic Science, Medicine, and Pathology
483:
281:Partial anomalous pulmonary venous return
212:; Cardiac (20%), where blood drains into
70:Learn how and when to remove this message
429:
139:) is a congenital heart defect of the
1182:Anomalous pulmonary venous connection
129:Anomalous pulmonary venous connection
88:Anomalous pulmonary venous connection
7:
458:Allan LD, Sharland GK (April 2001).
1218:Cerebral arteriovenous malformation
283:) is a congenital defect where the
133:anomalous pulmonary venous drainage
1164:Persistent left superior vena cava
690:10.1161/01.cir.0000107842.29467.c5
25:
156:anomalous pulmonary venous return
137:anomalous pulmonary venous return
1159:Congenital stenosis of vena cava
807:10.1016/j.athoracsur.2007.05.046
800:(6): 2020–6, discussion 2020–6.
767:10.1016/j.athoracsur.2007.11.038
31:
829:Somerville J, Grech V (2009).
546:Stein, Patricia (March 2007).
42:comply with Knowledge (XXG)'s
40:This article needs editing to
1:
1062:Aneurysm of sinus of Valsalva
731:10.1016/S0735-1097(97)82758-1
564:10.1016/S0001-2092(07)60123-9
393:Right ventricular hypertrophy
1090:Stenosis of pulmonary artery
650:10.1097/RTI.0b013e318194c942
378:at left upper sternal border
1239:Congenital vascular defects
1255:
1210:Arteriovenous malformation
1108:Aberrant subclavian artery
259:should be given because a
311:It can be diagnosed with
101:
92:
1037:Coarctation of the aorta
1032:Patent ductus arteriosus
261:patent ductus arteriosus
189:patent ductus arteriosus
1126:Single umbilical artery
1052:Right-sided aortic arch
1042:Interrupted aortic arch
835:Images Paediatr Cardiol
604:10.4103/2045-8932.97637
357:Right ventricular heave
294:It is less severe than
476:10.1136/heart.85.4.433
154:, also known as total
1004:Vascular malformation
718:J. Am. Coll. Cardiol
525:10.1385/FSMP:1:3:215
193:atrial septal defect
181:right-to-left shunt
52:improve the content
1151:inferior vena cava
1047:Double aortic arch
940:External resources
374:Systolic ejection
352:Signs and symptoms
319:, transesophageal
210:superior vena cava
168:in which all four
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1225:
1204:
1203:
1196:Scimitar syndrome
1134:
1133:
1100:Subclavian artery
1085:Pulmonary atresia
963:
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794:Ann. Thorac. Surg
755:Ann. Thorac. Surg
413:Cottage-loaf sign
395:on echocardiogram
183:such as a patent
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125:
82:Medical condition
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72:
16:(Redirected from
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1118:Umbilical artery
1077:Pulmonary artery
1057:Overriding aorta
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1000:vascular defects
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638:J Thorac Imaging
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321:echocardiography
206:innominate veins
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113:Medical genetics
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340:Pathophysiology
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301:Turner syndrome
289:pulmonary veins
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174:systemic venous
170:pulmonary veins
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141:pulmonary veins
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44:Manual of Style
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1174:Pulmonary vein
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1016:other arteries
1012:Great arteries
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884:Classification
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876:External links
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761:(3): 1007–14.
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558:(3): 509–520.
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1067:Vascular ring
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724:(6): 1351–8.
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683:(2): 207–14.
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644:(2): 89–95.
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631:
597:(2): 250–5.
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555:
552:AORN Journal
551:
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512:
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470:(4): 433–7.
467:
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443:November 10,
441:. Retrieved
432:
412:
382:Cardiomegaly
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166:heart defect
158:, is a rare
155:
151:
150:
136:
132:
128:
127:
66:
60:October 2017
57:
50:Please help
41:
1139:Great veins
677:Circulation
417:chest X-ray
415:, that is,
325:cardiac MRI
317:angiography
285:left atrium
229:obstruction
177:circulation
997:Congenital
841:(1): 7–9.
424:References
163:congenital
949:eMedicine
847:1729-441X
591:Pulm Circ
572:0001-2092
533:1556-2891
403:tachypnea
370:S3 gallop
331:Treatment
307:Diagnosis
247:Treatment
235:Diagnosis
108:Specialty
1233:Category
1147:Superior
954:ped/2816
865:22368552
816:18036929
775:18291189
699:14718402
658:19465830
623:22837866
494:11250972
399:Cyanosis
365:split S2
160:cyanotic
1190:Partial
856:3232600
740:9137235
614:3401879
485:1729686
407:dyspnea
360:Loud S1
313:CT scan
930:747.42
926:747.41
863:
853:
845:
814:
773:
738:
697:
656:
621:
611:
570:
531:
492:
482:
376:murmur
363:Fixed
218:portal
191:or an
115:
1186:Total
1024:Aorta
911:Q26.4
907:Q26.2
464:Heart
323:, or
921:9-CM
861:PMID
843:ISSN
812:PMID
771:PMID
736:PMID
695:PMID
654:PMID
619:PMID
568:ISSN
529:ISSN
490:PMID
445:2013
275:(or
257:PGE1
196:must
179:. A
131:(or
917:ICD
898:ICD
851:PMC
802:doi
763:doi
726:doi
685:doi
681:109
646:doi
609:PMC
599:doi
560:doi
521:doi
480:PMC
472:doi
315:,
279:or
220:or
135:or
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