Is heart transplantation for complex congenital heart disease a good option? A 25-year single centre experience

OBJECTIVES Heart transplantation (HTx) in patients with complex congenital heart disease (CHD) is a challenge because of structural anomalies and multiple previous procedures. We analysed our results in adult and paediatric patients to evaluate outcome and assess risk factors affecting mortality. ME...

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Veröffentlicht in:European journal of cardio-thoracic surgery 2013-03, Vol.43 (3), p.605-611
Hauptverfasser: Seddio, Francesco, Gorislavets, Natalyia, Iacovoni, Attilio, Cugola, Diego, Fontana, Alessandra, Galletti, Lorenzo, Terzi, Amedeo, Ferrazzi, Paolo
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container_end_page 611
container_issue 3
container_start_page 605
container_title European journal of cardio-thoracic surgery
container_volume 43
creator Seddio, Francesco
Gorislavets, Natalyia
Iacovoni, Attilio
Cugola, Diego
Fontana, Alessandra
Galletti, Lorenzo
Terzi, Amedeo
Ferrazzi, Paolo
description OBJECTIVES Heart transplantation (HTx) in patients with complex congenital heart disease (CHD) is a challenge because of structural anomalies and multiple previous procedures. We analysed our results in adult and paediatric patients to evaluate outcome and assess risk factors affecting mortality. METHODS Between 1985 and 2011, among 839 patients who underwent HTx, 85 received transplantation for end-stage CHD. Patients were divided into four age subgroups: 18 years (33 patients, Group IV) and into two time periods: 1985-2000 (47 patients) and 2001-2011 (38 patients). Anatomical diagnoses were single-ventricle defect in 37 patients (44%) and two-ventricle defect in 48 patients (56%). Seventy-three patients (86%) had undergone one or more cardiac surgical procedures prior to HTx (mean 2.4 ± 0.9). Twenty-two of them were suffering from Fontan failure. Mean pulmonary artery pressure was 25.2 ± 14.2 mmHg. Mean transpulmonary gradient was 9.4 ± 6.9 mmHg. RESULTS Mean follow-up after HTx was 7.8 ± 6.8 years. Survival at 1 month was 37.7% in Group I, 85.8% in Group II, 96.8% in Group II and 98.4% in Group IV and was significantly worse in younger recipients. Overall 30-day mortality was 17.6%. Currently 56 patients (65.8%) are alive. Overall survival at 1, 5, 10 and 15 years is 83-, 73-, 67- and 58%, respectively. There were 14 late deaths. Univariate analysis found that risk factors for early and late death were those related to recipient illness, such as pre-transplant creatinine, intravenous inotropic drugs, intravenous diuretics, mechanical ventilation and presence of protein-losing enteropathy (PLE). Multivariate analysis for all events (early and late deaths) identified preoperative mechanical ventilation as an independent risk factor for mortality. Number of previous procedures did not influence survival. Previous Fontan procedure did not increase mortality. We documented the reversibility of PLE in survivors. CONCLUSIONS We demonstrated that heart transplantation for patients with CHD can be performed with the expectation of excellent results. Previous procedures, including the Fontan operation, do not reduce survival. Mortality is related to preoperative patient condition. We advocate early referral of complex CHD patients for transplant assessment and for inclusion in waiting lists before the detrimental effects of end-stage failure manifest thems
doi_str_mv 10.1093/ejcts/ezs350
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A 25-year single centre experience</title><source>Oxford University Press Journals All Titles (1996-Current)</source><source>MEDLINE</source><source>EZB-FREE-00999 freely available EZB journals</source><source>Alma/SFX Local Collection</source><creator>Seddio, Francesco ; Gorislavets, Natalyia ; Iacovoni, Attilio ; Cugola, Diego ; Fontana, Alessandra ; Galletti, Lorenzo ; Terzi, Amedeo ; Ferrazzi, Paolo</creator><creatorcontrib>Seddio, Francesco ; Gorislavets, Natalyia ; Iacovoni, Attilio ; Cugola, Diego ; Fontana, Alessandra ; Galletti, Lorenzo ; Terzi, Amedeo ; Ferrazzi, Paolo</creatorcontrib><description>OBJECTIVES Heart transplantation (HTx) in patients with complex congenital heart disease (CHD) is a challenge because of structural anomalies and multiple previous procedures. We analysed our results in adult and paediatric patients to evaluate outcome and assess risk factors affecting mortality. METHODS Between 1985 and 2011, among 839 patients who underwent HTx, 85 received transplantation for end-stage CHD. Patients were divided into four age subgroups: &lt;1 year (8 patients, Group I), 1-10 years (20 patients, Group II), 11-18 years (24 patients, Group III) and &gt;18 years (33 patients, Group IV) and into two time periods: 1985-2000 (47 patients) and 2001-2011 (38 patients). Anatomical diagnoses were single-ventricle defect in 37 patients (44%) and two-ventricle defect in 48 patients (56%). Seventy-three patients (86%) had undergone one or more cardiac surgical procedures prior to HTx (mean 2.4 ± 0.9). Twenty-two of them were suffering from Fontan failure. Mean pulmonary artery pressure was 25.2 ± 14.2 mmHg. Mean transpulmonary gradient was 9.4 ± 6.9 mmHg. RESULTS Mean follow-up after HTx was 7.8 ± 6.8 years. Survival at 1 month was 37.7% in Group I, 85.8% in Group II, 96.8% in Group II and 98.4% in Group IV and was significantly worse in younger recipients. Overall 30-day mortality was 17.6%. Currently 56 patients (65.8%) are alive. Overall survival at 1, 5, 10 and 15 years is 83-, 73-, 67- and 58%, respectively. There were 14 late deaths. Univariate analysis found that risk factors for early and late death were those related to recipient illness, such as pre-transplant creatinine, intravenous inotropic drugs, intravenous diuretics, mechanical ventilation and presence of protein-losing enteropathy (PLE). Multivariate analysis for all events (early and late deaths) identified preoperative mechanical ventilation as an independent risk factor for mortality. Number of previous procedures did not influence survival. Previous Fontan procedure did not increase mortality. We documented the reversibility of PLE in survivors. CONCLUSIONS We demonstrated that heart transplantation for patients with CHD can be performed with the expectation of excellent results. Previous procedures, including the Fontan operation, do not reduce survival. 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All rights reserved. 2012</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c361t-63dac59ce63a003690e99136b5bde1e2cb137df9630ed6a91ccee64936d1c1843</citedby><cites>FETCH-LOGICAL-c361t-63dac59ce63a003690e99136b5bde1e2cb137df9630ed6a91ccee64936d1c1843</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,1578,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22733841$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Seddio, Francesco</creatorcontrib><creatorcontrib>Gorislavets, Natalyia</creatorcontrib><creatorcontrib>Iacovoni, Attilio</creatorcontrib><creatorcontrib>Cugola, Diego</creatorcontrib><creatorcontrib>Fontana, Alessandra</creatorcontrib><creatorcontrib>Galletti, Lorenzo</creatorcontrib><creatorcontrib>Terzi, Amedeo</creatorcontrib><creatorcontrib>Ferrazzi, Paolo</creatorcontrib><title>Is heart transplantation for complex congenital heart disease a good option? A 25-year single centre experience</title><title>European journal of cardio-thoracic surgery</title><addtitle>Eur J Cardiothorac Surg</addtitle><description>OBJECTIVES Heart transplantation (HTx) in patients with complex congenital heart disease (CHD) is a challenge because of structural anomalies and multiple previous procedures. We analysed our results in adult and paediatric patients to evaluate outcome and assess risk factors affecting mortality. METHODS Between 1985 and 2011, among 839 patients who underwent HTx, 85 received transplantation for end-stage CHD. Patients were divided into four age subgroups: &lt;1 year (8 patients, Group I), 1-10 years (20 patients, Group II), 11-18 years (24 patients, Group III) and &gt;18 years (33 patients, Group IV) and into two time periods: 1985-2000 (47 patients) and 2001-2011 (38 patients). Anatomical diagnoses were single-ventricle defect in 37 patients (44%) and two-ventricle defect in 48 patients (56%). Seventy-three patients (86%) had undergone one or more cardiac surgical procedures prior to HTx (mean 2.4 ± 0.9). Twenty-two of them were suffering from Fontan failure. Mean pulmonary artery pressure was 25.2 ± 14.2 mmHg. Mean transpulmonary gradient was 9.4 ± 6.9 mmHg. RESULTS Mean follow-up after HTx was 7.8 ± 6.8 years. Survival at 1 month was 37.7% in Group I, 85.8% in Group II, 96.8% in Group II and 98.4% in Group IV and was significantly worse in younger recipients. Overall 30-day mortality was 17.6%. Currently 56 patients (65.8%) are alive. Overall survival at 1, 5, 10 and 15 years is 83-, 73-, 67- and 58%, respectively. There were 14 late deaths. Univariate analysis found that risk factors for early and late death were those related to recipient illness, such as pre-transplant creatinine, intravenous inotropic drugs, intravenous diuretics, mechanical ventilation and presence of protein-losing enteropathy (PLE). Multivariate analysis for all events (early and late deaths) identified preoperative mechanical ventilation as an independent risk factor for mortality. Number of previous procedures did not influence survival. Previous Fontan procedure did not increase mortality. We documented the reversibility of PLE in survivors. CONCLUSIONS We demonstrated that heart transplantation for patients with CHD can be performed with the expectation of excellent results. Previous procedures, including the Fontan operation, do not reduce survival. Mortality is related to preoperative patient condition. 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A 25-year single centre experience</atitle><jtitle>European journal of cardio-thoracic surgery</jtitle><addtitle>Eur J Cardiothorac Surg</addtitle><date>2013-03</date><risdate>2013</risdate><volume>43</volume><issue>3</issue><spage>605</spage><epage>611</epage><pages>605-611</pages><issn>1010-7940</issn><eissn>1873-734X</eissn><abstract>OBJECTIVES Heart transplantation (HTx) in patients with complex congenital heart disease (CHD) is a challenge because of structural anomalies and multiple previous procedures. We analysed our results in adult and paediatric patients to evaluate outcome and assess risk factors affecting mortality. METHODS Between 1985 and 2011, among 839 patients who underwent HTx, 85 received transplantation for end-stage CHD. Patients were divided into four age subgroups: &lt;1 year (8 patients, Group I), 1-10 years (20 patients, Group II), 11-18 years (24 patients, Group III) and &gt;18 years (33 patients, Group IV) and into two time periods: 1985-2000 (47 patients) and 2001-2011 (38 patients). Anatomical diagnoses were single-ventricle defect in 37 patients (44%) and two-ventricle defect in 48 patients (56%). Seventy-three patients (86%) had undergone one or more cardiac surgical procedures prior to HTx (mean 2.4 ± 0.9). Twenty-two of them were suffering from Fontan failure. Mean pulmonary artery pressure was 25.2 ± 14.2 mmHg. Mean transpulmonary gradient was 9.4 ± 6.9 mmHg. RESULTS Mean follow-up after HTx was 7.8 ± 6.8 years. Survival at 1 month was 37.7% in Group I, 85.8% in Group II, 96.8% in Group II and 98.4% in Group IV and was significantly worse in younger recipients. Overall 30-day mortality was 17.6%. Currently 56 patients (65.8%) are alive. Overall survival at 1, 5, 10 and 15 years is 83-, 73-, 67- and 58%, respectively. There were 14 late deaths. Univariate analysis found that risk factors for early and late death were those related to recipient illness, such as pre-transplant creatinine, intravenous inotropic drugs, intravenous diuretics, mechanical ventilation and presence of protein-losing enteropathy (PLE). Multivariate analysis for all events (early and late deaths) identified preoperative mechanical ventilation as an independent risk factor for mortality. Number of previous procedures did not influence survival. Previous Fontan procedure did not increase mortality. We documented the reversibility of PLE in survivors. CONCLUSIONS We demonstrated that heart transplantation for patients with CHD can be performed with the expectation of excellent results. Previous procedures, including the Fontan operation, do not reduce survival. Mortality is related to preoperative patient condition. We advocate early referral of complex CHD patients for transplant assessment and for inclusion in waiting lists before the detrimental effects of end-stage failure manifest themselves.</abstract><cop>Germany</cop><pub>Oxford University Press</pub><pmid>22733841</pmid><doi>10.1093/ejcts/ezs350</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record>
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source Oxford University Press Journals All Titles (1996-Current); MEDLINE; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection
subjects Adolescent
Adult
Age Factors
Analysis of Variance
Child
Child, Preschool
Female
Fontan Procedure
Heart Defects, Congenital - epidemiology
Heart Defects, Congenital - surgery
Heart Transplantation - methods
Heart Transplantation - mortality
Humans
Infant
Italy - epidemiology
Kaplan-Meier Estimate
Male
Middle Aged
Retrospective Studies
Risk Factors
Treatment Outcome
title Is heart transplantation for complex congenital heart disease a good option? A 25-year single centre experience
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