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Article: Long-term outcome and cardiac arrhythmias in infants with right atrial isomerism

TitleLong-term outcome and cardiac arrhythmias in infants with right atrial isomerism
Authors
Issue Date2004
Publisher中華醫學會. The Journal's web site is located at http://zhek.periodicals.net.cn/
Citation
Zhonghua Er Ke Za Zhi. Chinese Journal Of Pediatrics, 2004, v. 42 n. 3, p. 166-171 How to Cite?
AbstractOBJECTIVE: The investigators compared the outcome of infants and children having right atrial isomerism with normal pulmonary venous drainage to those with anomalous drainage and determined factors associated with poor outcome. They further determined the prevalence of symptomatic cardiac arrhythmia in these patients and its relation to long-term morbidity and mortality. METHODS: The authors made a retrospective review of management and outcome of 116 infants and children diagnosed to have right atrial isomerism between January 1980 and December 2000. The type, timing and precipitating factors of symptomatic cardiac arrhythmia that occurred in patients, among a cohort of 85 who had or are awaiting surgical interventions, were noted. RESULTS: The 116 patients presented at a median of 1 day (range 1 day to 3.7 years) with cyanosis in the majority (96%). No interventions were planned in 31 (27%) patients who all died. The early surgical mortality for pulmonary venous repair was 25% (2/8), Fontan procedure 26% (5/19), cavopulmonary shunting 8% (1/13) and systemic-pulmonary arterial shunt insertion 2% (1/53). Late mortality was related to infection (n = 10), sudden death of unknown aetiology (n = 7) and documented arrhythmia (n = 1). Patients with obstructed anomalous pulmonary venous drainage had poor survival (P < 0.001). The mean (SEM) survival estimates for those with normal pulmonary venous drainage at 1, 5, 10 and 15 years were 81 (5)%, 67 (7)%, 60 (8)% and 43 (12)%, respectively, similar to those of patients with non-obstructed anomalous drainage (P = 0.06). Independent risk factors for mortality included pulmonary venous obstruction (relative risk RR 3.8, P = 0.001) and a single ventricle (RR 2.9, P = 0.016). Symptomatic cardiac arrhythmia occurred in 15/85 (18%) patients; 11 of whom had supraventricular tachycardia, and 1 atrial tachycardia, 1 atrial flutter, 1 ventricular tachycardia and 1 congenital complete heart block. The arrhythmias occurred before surgery in 4, early after surgery in 5, and late after surgery in 6 patients. Freedom from arrhythmia at 1, 5, 10, 15 and 20 years was (93 +/- 3)%, (86 +/- 4)%, (80 +/- 6)%, (73 +/- 9)% and (48 +/- 15)%, respectively. Logistic regression failed to identify any risk factors for symptomatic arrhythmia. CONCLUSION: The long-term outcome of infants and children with right atrial isomerism, whether associated with normal or anomalous pulmonary venous drainage, remains unfavourable. Sepsis and sudden death are major causes of late mortality. While symptomatic cardiac arrhythmias are not uncommon. They do not seem to relate to the overall high mortality and occurrence of sudden death in this patient group. Nonetheless, detailed assessment and aggressive management of cardiac arrhythmias once they occur are warranted in light of the precarious single ventricular haemodynamics.
Persistent Identifierhttp://hdl.handle.net/10722/170361
ISSN
2015 SCImago Journal Rankings: 0.146

 

DC FieldValueLanguage
dc.contributor.authorCheung, YFen_US
dc.contributor.authorCheng, YWen_US
dc.contributor.authorChau, KTen_US
dc.contributor.authorYung, TCen_US
dc.contributor.authorTong, KSen_US
dc.date.accessioned2012-10-30T06:07:48Z-
dc.date.available2012-10-30T06:07:48Z-
dc.date.issued2004en_US
dc.identifier.citationZhonghua Er Ke Za Zhi. Chinese Journal Of Pediatrics, 2004, v. 42 n. 3, p. 166-171en_US
dc.identifier.issn0578-1310en_US
dc.identifier.urihttp://hdl.handle.net/10722/170361-
dc.description.abstractOBJECTIVE: The investigators compared the outcome of infants and children having right atrial isomerism with normal pulmonary venous drainage to those with anomalous drainage and determined factors associated with poor outcome. They further determined the prevalence of symptomatic cardiac arrhythmia in these patients and its relation to long-term morbidity and mortality. METHODS: The authors made a retrospective review of management and outcome of 116 infants and children diagnosed to have right atrial isomerism between January 1980 and December 2000. The type, timing and precipitating factors of symptomatic cardiac arrhythmia that occurred in patients, among a cohort of 85 who had or are awaiting surgical interventions, were noted. RESULTS: The 116 patients presented at a median of 1 day (range 1 day to 3.7 years) with cyanosis in the majority (96%). No interventions were planned in 31 (27%) patients who all died. The early surgical mortality for pulmonary venous repair was 25% (2/8), Fontan procedure 26% (5/19), cavopulmonary shunting 8% (1/13) and systemic-pulmonary arterial shunt insertion 2% (1/53). Late mortality was related to infection (n = 10), sudden death of unknown aetiology (n = 7) and documented arrhythmia (n = 1). Patients with obstructed anomalous pulmonary venous drainage had poor survival (P < 0.001). The mean (SEM) survival estimates for those with normal pulmonary venous drainage at 1, 5, 10 and 15 years were 81 (5)%, 67 (7)%, 60 (8)% and 43 (12)%, respectively, similar to those of patients with non-obstructed anomalous drainage (P = 0.06). Independent risk factors for mortality included pulmonary venous obstruction (relative risk RR 3.8, P = 0.001) and a single ventricle (RR 2.9, P = 0.016). Symptomatic cardiac arrhythmia occurred in 15/85 (18%) patients; 11 of whom had supraventricular tachycardia, and 1 atrial tachycardia, 1 atrial flutter, 1 ventricular tachycardia and 1 congenital complete heart block. The arrhythmias occurred before surgery in 4, early after surgery in 5, and late after surgery in 6 patients. Freedom from arrhythmia at 1, 5, 10, 15 and 20 years was (93 +/- 3)%, (86 +/- 4)%, (80 +/- 6)%, (73 +/- 9)% and (48 +/- 15)%, respectively. Logistic regression failed to identify any risk factors for symptomatic arrhythmia. CONCLUSION: The long-term outcome of infants and children with right atrial isomerism, whether associated with normal or anomalous pulmonary venous drainage, remains unfavourable. Sepsis and sudden death are major causes of late mortality. While symptomatic cardiac arrhythmias are not uncommon. They do not seem to relate to the overall high mortality and occurrence of sudden death in this patient group. Nonetheless, detailed assessment and aggressive management of cardiac arrhythmias once they occur are warranted in light of the precarious single ventricular haemodynamics.en_US
dc.languageengen_US
dc.publisher中華醫學會. The Journal's web site is located at http://zhek.periodicals.net.cn/en_US
dc.relation.ispartofZhonghua er ke za zhi. Chinese journal of pediatricsen_US
dc.subject.meshArrhythmias, Cardiac - Etiology - Mortalityen_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshHeart Atria - Abnormalitiesen_US
dc.subject.meshHeart Defects, Congenital - Complications - Surgeryen_US
dc.subject.meshHumansen_US
dc.subject.meshInfanten_US
dc.subject.meshInfant, Newbornen_US
dc.subject.meshPrognosisen_US
dc.subject.meshSurvival Analysisen_US
dc.subject.meshSurvival Rateen_US
dc.titleLong-term outcome and cardiac arrhythmias in infants with right atrial isomerismen_US
dc.typeArticleen_US
dc.identifier.emailCheung, YF:xfcheung@hku.hken_US
dc.identifier.authorityCheung, YF=rp00382en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.pmid15144707-
dc.identifier.scopuseid_2-s2.0-2942638159en_US
dc.identifier.volume42en_US
dc.identifier.issue3en_US
dc.identifier.spage166en_US
dc.identifier.epage171en_US
dc.publisher.placeChinaen_US
dc.identifier.scopusauthoridCheung, YF=7202111067en_US
dc.identifier.scopusauthoridCheng, YW=7404914186en_US
dc.identifier.scopusauthoridChau, KT=35188515600en_US
dc.identifier.scopusauthoridYung, TC=9132842300en_US
dc.identifier.scopusauthoridTong, KS=7102473468en_US

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