53:
29:
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964:
Faria, Bebiana; von Hafe, Pedro; Ferreira, Francisco Castro; Almeida, Filipa; Dias, Geraldo; Cardoso, Filipa; Lourenço, Mário Rui; Ribeiro, SĂlvia; Lourenço, AntĂłnio (2020).
1228:
389:
Hoschtitzky, Andreas; Rowlands, Helen; Ilina, Maria; Khambadkone, Sachin; Elliott, Martin J. (2010). "Single
Ventricle Strategy for Uhl's Anomaly of the Right Ventricle".
518:
Cammalleri, Valeria; Forcina, Marco; Pugliese, Luca; Romeo, Francesco; Floris, Roberto; Chiocchi, Marcello (2019). "Uhl
Anomaly in Asymptomatic Adult Woman".
1581:
362:
Uhl, Henry (September 1952). "A previously undescribed congenital malformation of the heart: almost total absence of the myocardium of the right ventricle".
337:
and bidirectional Glenn shunt; (2) right ventricular exclusion alongside atrial septectomy as well as a bidirectional Glenn shunt (superior cavopulmonary
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by nonfunctional fibroelastic tissue that resembles parchment. As of 2010 less than 100 cases have been reported in literature.
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124:
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693:"Dysplastic conditions of the right ventricular myocardium: Uhl's anomaly vs arrhythmogenic right ventricular dysplasia"
333:. Various surgical techniques have been used, such as: (1) one-and-a-half ventricular repair alongside partial right
807:
Chounoune, Reginald; Lowry, Adam; Ramakrishnan, Karthik; Pearson, Gail D.; Moak, Jeffrey P.; Nath, Dilip S. (2018).
96:
1267:
1331:
1321:
432:
Richardson, J.D.; Teo, K.S.L.; Bertaso, A.G.; Wong., D.T.L.; Disney, P.; Worthley, S.G. (2012). "Uhl's anomaly".
85:
1451:
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139:
Although the precise cause of Uhl's anomaly is unknown, there have been reports of primary nondevelopment of
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66:
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1399:
1378:
1017:
Cardaropoli, D.; Russo, M. G.; Paladini, D.; Pisacane, C.; Caputo, S.; Giliberti, P.; Calabrò, R. (2006).
1185:
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Azhari, Nawal; Assaqqat, Mervat; Bulbul, Ziad (2002). "Successful surgical repair of Uhl's anomaly".
294:
52:
1339:
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226:
172:
128:
100:
809:"Uhl's anomaly: A one and a half ventricular repair in a patient presenting with cardiac arrest"
195:
Myocardial biopsies can confirm the diagnosis, which is often established by imaging tests like
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that was first identified by Dr. Henry Uhl in 1952. It is characterized by the absence of the
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70:
864:
Caruso, Settimo; Cannataci, Christine; Romano, Giuseppe (2021). "Case 288: Uhl
Anomaly".
163:
The pathophysiological outcome of Uhl anomaly involves compromised diastolic filling and
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is caused by a right-to-left shunting of blood through the patent oval foramen.
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attachment to the right atrioventricular junction, and normal left ventricular
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contraction. Systemic venous congestion and elevated right atrial and systemic
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Agarwal, Rakesh; Datta, Rajarshi; Saha, Manjari; Sarkar, Nirmalendu (2016).
271:
at the right ventricular free wall, lack of fibrofatty infiltration, normal
155:. A sporadic mutation could indicate that genetics is the underlying cause.
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layer, giving the right ventricular free wall a parchment-like appearance.
734:
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Gerlis, L M; Schmidt-Ott, S C; Ho, S Y; Anderson, R H (February 1, 1993).
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248:, reduced contractility and restricted ventricle filling, dilation of the
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88:. Atrial right-to-left shunting is frequently observed as the cause of
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Histopathologically, nonfunctioning fibroelastic tissue replaces the
1194:
652:"VEGF induces cardiovascular malformation and embryonic lethality"
206:
Right atrial and right ventricular dilatation is the cause of the
1150:
1117:
225:
Right ventricular and right atrial dilatation is evident on the
1210:
966:"Uhl's Anomaly: 10 Years of Follow-Up of an Unoperated Patient"
662:(5). American Society for Investigative Pathology: 1407–1416.
77:, either entirely or partially, and the replacement of the RV
607:
Uhl, Henry S.M. (April 15, 1996). "Uhl's
Anomaly Revisited".
305:, are included in the differential diagnosis of Uhl anomaly.
750:"Uhl′s anomaly: Absence of the right ventricular myocardium"
576:
Congenital and
Paediatric Acquired Heart Disease in Practice
127:
decreases. The main clinical features in older patients are
615:(8). Ovid Technologies (Wolters Kluwer Health): 1483–1484.
578:. Singapore: Springer Nature Singapore. pp. 271–273.
293:
Other anomalies that can result in RV dilatation, such as
183:(a result of ineffective right ventricular forward flow).
1019:"Prenatal echocardiography in a case of Uhl's anomaly"
36:
Uhl anomaly is inherited via autosomal dominant manner
1064:
123:
at birth, though these conditions may get better as
1521:
1467:
1407:
1398:
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1348:
1330:
1307:
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1244:
1160:
1068:
913:"Uhl's anomaly: A rare case of portal hypertension"
256:attachment to the right atrioventricular junction.
40:
21:
526:(2). Ovid Technologies (Wolters Kluwer Health).
483:(2). Cambridge University Press (CUP): 192–195.
84:Patients will typically present as infants with
214:. The lung fields of newborns with functional
1222:
8:
1582:Anomalous aortic origin of a coronary artery
650:Feucht, M.; Christ, B.; Wilting, J. (1997).
317:has become high and the newborn has severe
240:evaluation reveals a thin free wall of the
1404:
1250:
1229:
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1207:
1065:
51:
27:
18:
1034:
1023:Ultrasound in Obstetrics & Gynecology
999:
981:
946:
928:
840:
783:
765:
748:Ganczar, Joanna; English, Robert (2015).
724:
667:
325:infusion is recommended. In most cases,
574:Cheung, Yiu-fai (2023). "Uhl Anomaly".
354:
813:Journal of the Saudi Heart Association
364:Bulletin of the Johns Hopkins Hospital
321:soon after birth, a brief intravenous
819:(1). Saudi Heart Association: 52–54.
7:
520:Circulation: Cardiovascular Imaging
434:International Journal of Cardiology
1317:Sinus venosus atrial septal defect
1275:Transposition of the great vessels
281:cardiac magnetic resonance imaging
197:cardiac magnetic resonance imaging
153:vascular endothelial growth factor
14:
656:The American Journal of Pathology
303:anomalous pulmonary venous return
403:10.1016/j.athoracsur.2009.12.084
233:in the right praecordial leads.
179:newborns may develop functional
107:are used to make the diagnosis.
297:, RV arrhythmogenic dysplasia,
1350:Atrioventricular septal defect
754:Annals of Pediatric Cardiology
532:10.1161/circimaging.118.008277
391:The Annals of Thoracic Surgery
331:congestive right heart failure
1:
1292:Persistent truncus arteriosus
1263:Double outlet right ventricle
397:(6). Elsevier BV: 2076–2078.
315:pulmonary vascular resistance
220:pulmonary vascular resistance
177:pulmonary vascular resistance
125:pulmonary vascular resistance
1255:Aortopulmonary septal defect
584:10.1007/978-981-99-2862-0_26
446:10.1016/j.ijcard.2011.05.005
976:(5). Elsevier BV: 351–355.
440:(2). Elsevier BV: e36–e37.
115:Infants may exhibit severe
1629:
983:10.1016/j.case.2020.05.014
825:10.1016/j.jsha.2017.03.011
97:magnetic resonance imaging
1332:Ventricular septal defect
878:10.1148/radiol.2021192475
489:10.1017/s1047951102000410
171:are the results of right
86:right-sided heart failure
35:
26:
1608:Congenital heart defects
1238:Congenital heart defects
930:10.4103/1995-705x.201779
767:10.4103/0974-2069.149529
621:10.1161/01.cir.93.8.1483
1577:Coronary artery anomaly
923:(4). Medknow: 142–145.
477:Cardiology in the Young
343:cardiac transplantation
1400:Valvular heart disease
1379:Cyanotic heart disease
1322:Lutembacher's syndrome
279:are all indicative of
1587:Ventricular inversion
1299:Aortopulmonary window
1268:Taussig–Bing syndrome
760:(1). Medknow: 71–73.
229:. One can observe an
1386:Eisenmenger syndrome
1309:Atrial septal defect
709:10.1136/hrt.69.2.142
131:symptoms and signs.
67:cardiac malformation
1340:Tetralogy of Fallot
1246:Heart septal defect
703:(2). BMJ: 142–150.
173:ventricular failure
129:right heart failure
121:right heart failure
101:computed tomography
1161:External resources
329:are necessary for
222:appear oligaemic.
151:overexpression of
111:Signs and symptoms
1595:
1594:
1517:
1516:
1457:Ebstein's anomaly
1394:
1393:
1204:
1203:
593:978-981-99-2861-3
299:pulmonary atresia
263:of a thin-walled
238:echocardiographic
227:electrocardiogram
216:pulmonary atresia
181:pulmonary atresia
165:right ventricular
60:
59:
16:Medical condition
1620:
1572:Brugada syndrome
1567:Crisscross heart
1437:tricuspid valves
1415:pulmonary valves
1405:
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1038:
1036:10.1002/uog.2798
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231:epsilon (ε) wave
212:chest radiograph
105:echocardiography
56:
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47:Medical genetics
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1562:Cor triatriatum
1528:Underdeveloped
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905:Further reading
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335:ventriculectomy
323:prostaglandin E
311:
295:Ebstein anomaly
273:tricuspid valve
265:right ventricle
254:tricuspid valve
242:right ventricle
193:
169:venous pressure
161:
137:
113:
71:right ventricle
50:
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1069:Classification
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1061:External links
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1029:(6): 713–714.
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872:(1): 237–241.
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201:echocardiogram
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1613:Rare diseases
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1497:mitral valves
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1485:insufficiency
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1475:aortic valves
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1447:regurgitation
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1374:Cardiac shunt
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149:cardiomyocyte
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1552:Dextrocardia
1544:
1367:Consequences
1190:
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250:right atrium
244:without any
235:
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210:seen on the
208:cardiomegaly
205:
194:
162:
143:, selective
138:
114:
94:
83:
62:
61:
1545:Uhl anomaly
1170:Radiopaedia
917:Heart Views
609:Circulation
341:); and (3)
339:anastomosis
95:Typically,
63:Uhl anomaly
22:Uhl anomaly
1602:Categories
1557:Levocardia
1135:DiseasesDB
349:References
288:myocardial
283:findings.
277:myocardium
269:myocardium
261:dyskinesia
246:myocardium
79:myocardium
75:myocardium
65:is a rare
1146:SNOMED CT
1045:0960-7692
992:2468-6441
939:1995-705X
886:0033-8419
866:Radiology
833:1016-7315
776:0974-2069
717:1355-6037
629:0009-7322
540:1941-9651
497:1047-9511
454:0167-5273
411:0003-4975
327:diuretics
313:When the
309:Treatment
218:and high
191:Diagnosis
159:Mechanism
145:apoptosis
42:Specialty
1502:stenosis
1490:bicuspid
1480:stenosis
1452:stenosis
1420:stenosis
1195:Q2852241
1181:Orphanet
1053:16710881
1010:33117927
957:28400938
894:33750225
851:29296065
794:25684894
548:30700135
505:12018730
462:21640406
419:21095379
376:12978573
319:cyanosis
185:Cyanosis
141:myocytes
117:cyanosis
90:cyanosis
1442:atresia
1430:absence
1191:Scholia
1151:2829000
1129:C536932
1001:7581623
948:5363090
842:5744023
785:4322408
735:8435240
726:1024941
678:9358767
669:1858086
637:8608612
259:Global
175:. High
1280:dextro
1118:107970
1092:LA88.Y
1051:
1043:
1008:
998:
990:
955:
945:
937:
892:
884:
849:
839:
831:
792:
782:
774:
733:
723:
715:
676:
666:
635:
627:
590:
546:
538:
503:
495:
460:
452:
417:
409:
374:
301:, and
147:, and
135:Causes
103:, and
49:
1535:right
1522:Other
1408:Right
1175:24882
1140:33469
1107:Q24.8
697:Heart
73:(RV)
1540:left
1468:Left
1285:levo
1186:3403
1124:MeSH
1113:OMIM
1049:PMID
1041:ISSN
1006:PMID
988:ISSN
970:CASE
953:PMID
935:ISSN
890:PMID
882:ISSN
847:PMID
829:ISSN
790:PMID
772:ISSN
731:PMID
713:ISSN
674:PMID
633:PMID
625:ISSN
588:ISBN
544:PMID
536:ISSN
501:PMID
493:ISSN
458:PMID
450:ISSN
415:PMID
407:ISSN
372:PMID
119:and
1098:ICD
1083:ICD
1031:doi
996:PMC
978:doi
943:PMC
925:doi
874:doi
870:299
837:PMC
821:doi
780:PMC
762:doi
721:PMC
705:doi
664:PMC
660:151
617:doi
580:doi
528:doi
485:doi
442:doi
438:154
399:doi
236:An
199:or
1604::
1193::
1184::
1173::
1149::
1138::
1127::
1116::
1105::
1102:10
1090::
1087:11
1047:.
1039:.
1027:27
1025:.
1021:.
1004:.
994:.
986:.
972:.
968:.
951:.
941:.
933:.
921:17
919:.
915:.
888:.
880:.
868:.
845:.
835:.
827:.
817:30
815:.
811:.
788:.
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658:.
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631:.
623:.
613:93
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586:.
556:^
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524:12
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413:.
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395:90
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368:91
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1230:e
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1216:v
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