64:
37:
155:
330:
with repair of the heart is the only curative option. Transplantation is the final therapeutic option and only for patients with poor prognosis and quality of life. Timing and appropriateness of transplantation remain difficult decisions. 5-year and 10-year survival ranges between 70% and 80%, 50%
348:
are important for many patients with
Eisenmenger syndrome, as evidence suggests that arrhythmia-induced sudden cardiac death may be the leading cause of death among patients with the disease. These therapies generally aim to restore and maintain sinus rhythm, but the specific interventions chosen
748:
Goerler, Heidi; Simon, Andre; Gohrbandt, Bernhard; Hagl, Christian; Oppelt, Petra; Weidemann, Juergen; Haverich, Axel; Strueber, Martin (2007). "Heart-lung and lung transplantation in grown-up congenital heart disease: long-term single centre experience".
1062:
1047:
146:. Most deaths occur either during or within the first weeks after delivery. Pregnant women with Eisenmenger syndrome should be hospitalized after the 20th week of pregnancy, or earlier if clinical deterioration occurs.
835:
Stoica, S. C.; McNeil, K. D.; Perreas, K.; Sharples, L. D.; Satchithananda, D. K.; Tsui, S. S.; Large, S. R.; Wallwork, J. (2001). "Heart-lung transplantation for
Eisenmenger syndrome: early and long-term results".
411:
302:, which facilitates the identification and evaluation of shunts, anatomical defects, and ventricular function. Following diagnosis, or in some cases of inconclusive diagnosis, a
525:
Oechslin, Erwin; Mebus, Siegrun; Schulze-Neick, Ingram; Niwa, Koichiro; Trindade, Pedro T; Eicken, Andreas; Hager, Alfred; Lang, Irene; Hess, John; Kaemmerer, Harald (2010).
792:
Waddell, Thomas K.; Bennett, Leah; Kennedy, Renee; Todd, Thomas R. J.; Keshavjee, Shaf H. (2002). "Heart-lung or lung transplantation for
Eisenmenger syndrome".
306:
may be used to both confirm the diagnosis and to assess the patient's pulmonary arterial pressure, an important predictive value for prognosis and treatment.
130:
mother can cause serious complications, though successful delivery has been reported. Maternal mortality ranges from 30% to 60%, and may be attributed to
331:
and 70%, 30% and 50%, respectively. Since the average life expectancy of patients after lung transplantation is as low as 30% at 5 years, patients with
1176:
1181:
615:
416:
1171:
527:"The Adult Patient with Eisenmenger Syndrome: A Medical Update after Dana Point Part III: Specific Management and Surgical Aspects"
794:
The
Journal of Heart and Lung Transplantation: The Official Publication of the International Society for Heart Transplantation
299:
254:. Approximately 40% of patients diagnosed with Eisenmenger syndrome were also found to have these arrhythmias during routine
481:
920:"Eisenmenger Syndrome Treatment & Management: Approach Considerations, Oxygen Therapy, Pulmonary Vasodilator Therapy"
322:
is sufficient to reverse the blood flow through the defect, however, the maladaptation is considered irreversible, and a
318:
it causes significant pulmonary hypertension, it can normally be repaired through surgery, preventing the disease. After
251:
751:
European
Journal of Cardio-Thoracic Surgery: Official Journal of the European Association for Cardio-Thoracic Surgery
631:
Vongpatanasin, W.; Brickner, M. E.; Hillis, L. D.; Lange, R. A. (1998-05-01). "The
Eisenmenger syndrome in adults".
342:
Various medicines and therapies for pulmonary hypertension are under investigation for treatment of the symptoms.
323:
279:
97:
1082:
283:
105:
1186:
1166:
303:
93:
1144:
319:
250:
One of the most severe and common complications of
Eisenmenger syndrome is cardiac arrhythmia, especially
135:
109:
113:
582:
345:
327:
275:
263:
101:
680:
1066:
385:
Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Søndergaard L (April 2009). "Eisenmenger syndrome".
255:
116:
63:
262:
in patients with
Eisenmenger syndrome, compared to the general population, and can be a source of
880:
716:
362:
208:
192:
131:
123:
early in life, the incidence of heart defects progressing to
Eisenmenger syndrome has decreased.
606:
Mann, Douglas L.; Zipes, Douglas P.; Libby, Peter; Bonow, Robert O.; Braunwald, Eugene (2015).
583:"Eisenmenger syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program"
1122:
1093:
974:
900:
861:
853:
817:
809:
774:
766:
734:
708:
700:
656:
648:
611:
564:
546:
493:
462:
394:
52:
964:
956:
892:
845:
801:
758:
692:
644:
640:
554:
538:
452:
442:
203:
120:
57:
919:
218:
1133:
1016:
559:
526:
457:
430:
179:
1071:
969:
944:
849:
805:
696:
431:"PGE1 nebulisation during caesarean section for Eisenmenger's syndrome: a case report"
1160:
287:
198:
185:
158:
89:
45:
992:
V, Eisenmenger (1897). "Die angeborenen
Defekte der Kammerscheidewand des Herzens".
720:
36:
143:
1087:
501:
1117:
762:
162:
1011:
896:
542:
274:
A number of congenital heart defects can cause Eisenmenger syndrome, including
154:
1098:
1056:
945:"Pulmonary hypertension with special reference to the vasoconstrictive factor"
213:
139:
857:
813:
770:
704:
652:
550:
1128:
447:
259:
244:
238:
978:
904:
865:
821:
778:
712:
568:
497:
466:
398:
660:
1139:
960:
173:
127:
1075:
1039:
161:
of fingers in a patient with Eisenmenger syndrome. First described by
1051:
358:
229:
681:"Pulmonary Vascular Disease in Adults With Congenital Heart Disease"
30:
ES, Eisenmenger's syndrome, Eisenmenger reaction, tardive cyanosis.
169:
Signs and symptoms of Eisenmenger syndrome include the following:
153:
88:
is defined as the process in which a long-standing left-to-right
234:
136:
blood clots forming in the veins and traveling to distant sites
298:
Diagnosis of Eisenmenger syndrome is typically conducted via
679:
Diller, Gerhard-Paul; Gatzoulis, Michael A. (2007-02-27).
176:(a blue tinge to the skin resulting from lack of oxygen)
349:
will depend on the nature of the patient's arrhythmia.
482:"Pregnancy in the patient with Eisenmenger's syndrome"
480:
Makaryus AN, Forouzesh A, Johnson M (November 2006).
1029:
1108:
1033:
71:
51:
26:
21:
314:If the inciting defect in the heart is identified
165:, clubbing is also known as "Hippocratic fingers".
610:. Philadelphia, PA: Saunders. p. 1617-1618.
241:resorption and production with impaired excretion
879:Beghetti, Maurice; Galiè, Nazzareno (Mar 2009).
337:improved survival with conservative medical care
119:. Because of the advent of fetal screening with
380:
378:
885:Journal of the American College of Cardiology
365:, who first described the condition in 1897.
8:
520:
518:
112:and eventual reversal of the shunt into a
1030:
62:
44:Eisenmenger Syndrome due to a ventricular
35:
18:
968:
558:
456:
446:
258:screenings. These arrhythmias have worse
645:10.7326/0003-4819-128-9-199805010-00008
374:
223:Infections (endocarditis and pneumonia)
75:Supportive measures, transplant surgery
286:, and more complex types of acyanotic
335:related to Eisenmenger syndrome have
7:
674:
672:
670:
14:
697:10.1161/CIRCULATIONAHA.105.592386
918:Penalver, Jorge L (2019-04-15).
737:. NIH MedLine Plus. 2010-02-05.
339:compared with transplantation.
184:Swollen or clubbed fingertips (
838:The Annals of Thoracic Surgery
300:transthoracic echocardiography
1:
1177:Syndromes affecting the heart
850:10.1016/s0003-4975(01)03099-5
806:10.1016/s1053-2498(01)00420-x
1182:Syndromes affecting the lung
429:Siddiqui S, Latif N (2008).
417:Dorland's Medical Dictionary
333:reasonable functional status
252:supraventricular arrhythmias
763:10.1016/j.ejcts.2007.08.024
633:Annals of Internal Medicine
1203:
1172:Health issues in pregnancy
897:10.1016/j.jacc.2008.11.025
543:10.2174/157340310793566127
531:Current Cardiology Reviews
280:ventricular septal defects
237:(rarely) due to increased
126:Eisenmenger syndrome in a
608:Braunwald's Heart Disease
587:rarediseases.info.nih.gov
357:Eisenmenger syndrome was
180:High red blood cell count
98:ventricular septal defect
43:
34:
284:patent ductus arteriosus
191:Fainting (also known as
106:patent ductus arteriosus
448:10.1186/1752-1947-2-149
94:congenital heart defect
881:"Eisenmenger Syndrome"
735:"Eisenmenger syndrome"
412:"Eisenmenger syndrome"
320:pulmonary hypertension
204:Abnormal heart rhythms
166:
110:pulmonary hypertension
86:Eisenmenger's syndrome
949:British Heart Journal
324:heart–lung transplant
276:atrial septal defects
157:
961:10.1136/hrt.20.4.557
387:Ugeskrift for Laeger
346:Antiarrhythmic drugs
264:sudden cardiac death
104:, or less commonly,
102:atrial septal defect
82:Eisenmenger syndrome
22:Eisenmenger syndrome
361:by Paul Wood after
117:right-to-left shunt
1109:External resources
924:Medscape Reference
363:Victor Eisenmenger
209:Bleeding disorders
167:
150:Signs and symptoms
1154:
1153:
617:978-0-323-29429-4
214:Coughing up blood
140:coughing up blood
79:
78:
16:Medical condition
1194:
1031:
1019:
1008:
1002:
1001:
989:
983:
982:
972:
943:Wood, P (1958).
940:
934:
933:
931:
930:
915:
909:
908:
876:
870:
869:
844:(6): 1887–1891.
832:
826:
825:
789:
783:
782:
745:
739:
738:
731:
725:
724:
691:(8): 1039–1050.
676:
665:
664:
628:
622:
621:
603:
597:
596:
594:
593:
579:
573:
572:
562:
522:
513:
512:
510:
509:
500:. Archived from
486:Mt. Sinai J. Med
477:
471:
470:
460:
450:
426:
420:
409:
403:
402:
382:
304:cardiac catheter
121:echocardiography
96:(typically by a
67:
66:
58:Medical genetics
39:
19:
1202:
1201:
1197:
1196:
1195:
1193:
1192:
1191:
1157:
1156:
1155:
1150:
1149:
1104:
1103:
1042:
1028:
1023:
1022:
1009:
1005:
991:
990:
986:
942:
941:
937:
928:
926:
917:
916:
912:
878:
877:
873:
834:
833:
829:
791:
790:
786:
747:
746:
742:
733:
732:
728:
678:
677:
668:
630:
629:
625:
618:
605:
604:
600:
591:
589:
581:
580:
576:
524:
523:
516:
507:
505:
479:
478:
474:
428:
427:
423:
410:
406:
384:
383:
376:
371:
355:
328:lung transplant
312:
296:
272:
226:Kidney problems
219:Iron deficiency
152:
138:, hypovolemia,
132:fainting spells
61:
17:
12:
11:
5:
1200:
1198:
1190:
1189:
1187:Rare syndromes
1184:
1179:
1174:
1169:
1167:Heart diseases
1159:
1158:
1152:
1151:
1148:
1147:
1136:
1134:article/154555
1125:
1113:
1112:
1110:
1106:
1105:
1102:
1101:
1090:
1079:
1059:
1043:
1038:
1037:
1035:
1034:Classification
1027:
1026:External links
1024:
1021:
1020:
1003:
984:
935:
910:
891:(9): 733–740.
871:
827:
800:(7): 731–737.
784:
757:(6): 926–931.
740:
726:
666:
639:(9): 745–755.
623:
616:
598:
574:
537:(4): 363–372.
514:
472:
435:J Med Case Rep
421:
404:
393:(15): 1270–5.
373:
372:
370:
367:
354:
351:
311:
308:
295:
292:
271:
268:
248:
247:
242:
232:
227:
224:
221:
216:
211:
206:
201:
196:
189:
182:
177:
151:
148:
77:
76:
73:
69:
68:
55:
49:
48:
41:
40:
32:
31:
28:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
1199:
1188:
1185:
1183:
1180:
1178:
1175:
1173:
1170:
1168:
1165:
1164:
1162:
1146:
1142:
1141:
1137:
1135:
1131:
1130:
1126:
1124:
1120:
1119:
1115:
1114:
1111:
1107:
1100:
1096:
1095:
1091:
1089:
1085:
1084:
1080:
1077:
1073:
1069:
1068:
1064:
1060:
1058:
1054:
1053:
1049:
1045:
1044:
1041:
1036:
1032:
1025:
1018:
1017:Who Named It?
1014:
1013:
1007:
1004:
999:
995:
988:
985:
980:
976:
971:
966:
962:
958:
955:(4): 557–70.
954:
950:
946:
939:
936:
925:
921:
914:
911:
906:
902:
898:
894:
890:
886:
882:
875:
872:
867:
863:
859:
855:
851:
847:
843:
839:
831:
828:
823:
819:
815:
811:
807:
803:
799:
795:
788:
785:
780:
776:
772:
768:
764:
760:
756:
752:
744:
741:
736:
730:
727:
722:
718:
714:
710:
706:
702:
698:
694:
690:
686:
682:
675:
673:
671:
667:
662:
658:
654:
650:
646:
642:
638:
634:
627:
624:
619:
613:
609:
602:
599:
588:
584:
578:
575:
570:
566:
561:
556:
552:
548:
544:
540:
536:
532:
528:
521:
519:
515:
504:on 2009-03-05
503:
499:
495:
492:(7): 1033–6.
491:
487:
483:
476:
473:
468:
464:
459:
454:
449:
444:
440:
436:
432:
425:
422:
419:
418:
413:
408:
405:
400:
396:
392:
389:(in Danish).
388:
381:
379:
375:
368:
366:
364:
360:
352:
350:
347:
343:
340:
338:
334:
329:
325:
321:
317:
309:
307:
305:
301:
293:
291:
289:
288:heart disease
285:
281:
277:
269:
267:
265:
261:
257:
253:
246:
243:
240:
236:
233:
231:
228:
225:
222:
220:
217:
215:
212:
210:
207:
205:
202:
200:
199:Heart failure
197:
194:
190:
187:
183:
181:
178:
175:
172:
171:
170:
164:
160:
159:Nail clubbing
156:
149:
147:
145:
141:
137:
133:
129:
124:
122:
118:
115:
111:
107:
103:
99:
95:
91:
90:cardiac shunt
87:
83:
74:
70:
65:
59:
56:
54:
50:
47:
46:septal defect
42:
38:
33:
29:
25:
20:
1138:
1127:
1116:
1092:
1081:
1061:
1046:
1010:
1006:
997:
993:
987:
952:
948:
938:
927:. Retrieved
923:
913:
888:
884:
874:
841:
837:
830:
797:
793:
787:
754:
750:
743:
729:
688:
684:
636:
632:
626:
607:
601:
590:. Retrieved
586:
577:
534:
530:
506:. Retrieved
502:the original
489:
485:
475:
438:
434:
424:
415:
407:
390:
386:
356:
344:
341:
336:
332:
315:
313:
297:
273:
249:
168:
144:preeclampsia
125:
92:caused by a
85:
81:
80:
1118:MedlinePlus
685:Circulation
163:Hippocrates
27:Other names
1161:Categories
1094:DiseasesDB
994:Z Klin Med
929:2024-08-10
592:2021-10-25
508:2008-12-20
441:(1): 149.
369:References
245:Gallstones
1129:eMedicine
1012:synd/3034
858:0003-4975
814:1053-2498
771:1010-7940
705:0009-7322
653:0003-4819
551:1573-403X
353:Etymology
310:Treatment
294:Diagnosis
260:prognosis
239:uric acid
108:) causes
72:Treatment
53:Specialty
1140:Orphanet
1078:745.410)
979:13584643
905:19245962
866:11789765
822:12100899
779:17900918
721:10479313
713:17325254
569:22043213
498:17195894
467:18466628
399:19416617
186:clubbing
174:Cyanosis
128:pregnant
114:cyanotic
1088:D004541
1076:CDC/BPA
1000:: 1–28.
661:9556469
560:3083818
458:2405798
193:syncope
1123:007317
977:
970:491807
967:
903:
864:
856:
820:
812:
777:
769:
719:
711:
703:
659:
651:
614:
567:
557:
549:
496:
465:
455:
397:
316:before
270:Causes
230:Stroke
60:
1145:97214
1072:745.4
1057:Q21.8
717:S2CID
359:named
326:or a
1099:4143
1083:MeSH
1067:9-CM
975:PMID
901:PMID
862:PMID
854:ISSN
818:PMID
810:ISSN
775:PMID
767:ISSN
709:PMID
701:ISSN
657:PMID
649:ISSN
612:ISBN
565:PMID
547:ISSN
494:PMID
463:PMID
395:PMID
235:Gout
1063:ICD
1048:ICD
1015:at
965:PMC
957:doi
893:doi
846:doi
802:doi
759:doi
693:doi
689:115
641:doi
637:128
555:PMC
539:doi
453:PMC
443:doi
414:at
391:171
256:ECG
142:or
84:or
1163::
1143::
1132::
1121::
1097::
1086::
1070::
1055::
1052:10
998:32
996:.
973:.
963:.
953:20
951:.
947:.
922:.
899:.
889:53
887:.
883:.
860:.
852:.
842:72
840:.
816:.
808:.
798:21
796:.
773:.
765:.
755:32
753:.
715:.
707:.
699:.
687:.
683:.
669:^
655:.
647:.
635:.
585:.
563:.
553:.
545:.
533:.
529:.
517:^
490:73
488:.
484:.
461:.
451:.
437:.
433:.
377:^
290:.
282:,
278:,
266:.
134:,
100:,
1074:(
1065:-
1050:-
1040:D
981:.
959::
932:.
907:.
895::
868:.
848::
824:.
804::
781:.
761::
723:.
695::
663:.
643::
620:.
595:.
571:.
541::
535:6
511:.
469:.
445::
439:2
401:.
195:)
188:)
Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
