دورية أكاديمية
أعرض في EDS

Post-operative Morbidity and Mortality After Fontan Procedure in Patients with Heterotaxy and Other Situs Anomalies.

التفاصيل البيبلوغرافية
العنوان: Post-operative Morbidity and Mortality After Fontan Procedure in Patients with Heterotaxy and Other Situs Anomalies.
المؤلفون: Duong SQ; Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, 750 Welch Road, Suite 305, Palo Alto, CA, USA. sonduong@stanford.edu., Zaniletti I; Children's Hospital Association, Lenexa, KS, USA., Lopez L; Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, 750 Welch Road, Suite 305, Palo Alto, CA, USA., Sutherland SM; Division of Pediatric Nephrology, Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, USA., Shin AY; Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, 750 Welch Road, Suite 305, Palo Alto, CA, USA., Collins RT 2nd; Division of Pediatric Cardiology, Department of Pediatrics, Stanford University School of Medicine, 750 Welch Road, Suite 305, Palo Alto, CA, USA.; Stanford Cardiovascular Institute, Stanford University School of Medicine, Palo Alto, CA, USA.; Division of Cardiovascular Medicine, Department of Internal Medicine, Stanford University School of Medicine, Palo Alto, CA, USA.
المصدر: Pediatric cardiology [Pediatr Cardiol] 2022 Jun; Vol. 43 (5), pp. 952-959. Date of Electronic Publication: 2022 Jan 22.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Springer Verlag Country of Publication: United States NLM ID: 8003849 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1432-1971 (Electronic) Linking ISSN: 01720643 NLM ISO Abbreviation: Pediatr Cardiol Subsets: MEDLINE
أسماء مطبوعة: Publication: New York Ny : Springer Verlag
Original Publication: [New York, Springer-Verlag]
مواضيع طبية MeSH: Fontan Procedure*/adverse effects , Fontan Procedure*/methods , Heart Defects, Congenital*/complications , Heterotaxy Syndrome* , Situs Inversus*/complications , Situs Inversus*/surgery, Child ; Humans ; Morbidity ; Treatment Outcome
مستخلص: Heterotaxy is a complex, multisystem disorder associated with single ventricle heart disease and decreased survival. Ciliary dysfunction is common in heterotaxy and other situs abnormalities (H/SA) and may increase post-operative complications. We hypothesized that patients with H/SA have increased respiratory and renal morbidities and increased in-hospital mortality after Fontan procedure. We queried the Pediatric Health Information System database for hospitalizations with ICD-9/10 codes for Fontan procedure in patients aged 1 through 11 years from 2004 to 2019. H/SA was identified by codes for dextrocardia, situs inversus, asplenia/polysplenia, or atrial isomerism and compared to non-H/SA controls. Outcomes were in-hospital mortality or heart transplantation, ECMO, hemodialysis, length of stay (LOS), and mechanical ventilation or vasoactive medication use ≥ 4 days. We adjusted estimates with multivariable logistic regression. Of 7897 patients at 50 centers, 1366 (17%) met criteria for H/SA. H/SA had worse outcomes for all study measures: death/transplantation (1.9 vs 1.1%, OR 1.74 (95% CI 1.01-3.03); p = 0.047), ECMO (3.7 vs 2.3%, OR 1.74 (1.28-2.35); p < 0.001), hemodialysis (2.1 vs 1.2%, OR 1.66 (1.06-2.59); p = 0.026), prolonged mechanical ventilation (13.2% vs 7.6%, OR 1.85 (1.53-2.25); p < 0.001) and vasoactive medication use (29.4 vs 19.7%, OR 1.65 (1.43-1.90), and longer LOS (11 (8-17) vs 9 (7-14) days; p < 0.001). H/SA is associated with increased cardiovascular, renal, and respiratory morbidity, as well as in-hospital mortality after Fontan procedure. Attention to renal and respiratory needs may improve outcomes in this difficult population. The relationship between ciliary dysfunction and lung and renal morbidity should be explored further.
(© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
References: Lin AE, Krikov S, Riehle-Colarusso T, Frías JL, Belmont J, Anderka M et al (2014) Laterality defects in the national birth defects prevention study (1998–2007): birth prevalence and descriptive epidemiology. Am J Med Genet A 164a:2581–2591. (PMID: 10.1002/ajmg.a.36695)
Gentles TL, Mayer JE Jr, Gauvreau K, Newburger JW, Lock JE, Kupferschmid JP et al (1997) Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg 114:376–391. (PMID: 10.1016/S0022-5223(97)70183-1)
Banka P, Adar A, Schaetzle B, Sleeper LA, Emani S, Geva T (2020) Changes in prognosis of heterotaxy syndrome over time. Pediatrics 146:1. https://doi.org/10.1542/peds.2019-3345. (PMID: 10.1542/peds.2019-3345)
Patel A, Costello JM, Backer CL, Pasquali SK, Hill KD, Wallace AS et al (2016) Prevalence of noncardiac and genetic abnormalities in neonates undergoing cardiac operations: analysis of the Society of Thoracic Surgeons congenital heart surgery database. Ann Thorac Surg 102:1607–1614. (PMID: 10.1016/j.athoracsur.2016.04.008)
Loomba RS, Aggarwal S, Gupta N, Buelow M, Alla V, Arora RR et al (2016) Arrhythmias in adult congenital patients with bodily isomerism. Pediatr Cardiol 37:330–337. (PMID: 10.1007/s00246-015-1281-7)
Niu MC, Dickerson HA, Moore JA, de la Uz C, Valdés SO, Kim JJ et al (2018) Heterotaxy syndrome and associated arrhythmias in pediatric patients. Heart Rhythm 15:548–554. (PMID: 10.1016/j.hrthm.2017.11.013)
Ware SM, Aygun MG, Hildebrandt F (2011) Spectrum of clinical diseases caused by disorders of primary cilia. Proc Am Thorac Soc 8:444–450. (PMID: 10.1513/pats.201103-025SD)
Garrod AS, Zahid M, Tian X, Francis RJ, Khalifa O, Devine W et al (2014) Airway ciliary dysfunction and sinopulmonary symptoms in patients with congenital heart disease. Ann Am Thorac Soc 11:1426–1432. (PMID: 10.1513/AnnalsATS.201405-222OC)
Alsoufi B, McCracken C, Schlosser B, Sachdeva R, Well A, Kogon B et al (2016) Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome. J Thorac Cardiovasc Surg 151:1369-1377e2. (PMID: 10.1016/j.jtcvs.2016.01.054)
Harden B, Tian X, Giese R, Nakhleh N, Kureshi S, Francis R et al (2014) Increased postoperative respiratory complications in heterotaxy congenital heart disease patients with respiratory ciliary dysfunction. J Thorac Cardiovasc Surg 147:1291–1298. (PMID: 10.1016/j.jtcvs.2013.06.018)
Swisher M, Jonas R, Tian X, Lee ES, Lo CW, Leatherbury L (2011) Increased postoperative and respiratory complications in patients with congenital heart disease associated with heterotaxy. J Thorac Cardiovasc Surg 141(637–44):644.
Marathe SP, Zannino D, Cao JY, du Plessis K, Marathe SS, Ayer J et al (2020) Heterotaxy is not a risk factor for adverse long-term outcomes after fontan completion. Ann Thorac Surg 110:646–653. (PMID: 10.1016/j.athoracsur.2019.11.015)
Callahan TJ, Bauck AE, Bertoch D, Brown J, Khare R, Ryan PB et al (2017) A comparison of data quality assessment checks in six data sharing networks. EGEMS (Washington, DC) 5:8.
Bhaskar J, Galati JC, Brooks P, Oppido G, Konstantinov IE, Brizard CP et al (2015) Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery. J Thorac Cardiovasc Surg 149:1509–1513. (PMID: 10.1016/j.jtcvs.2015.01.038)
Song J, Kang IS, Huh J, Lee OJ, Kim G, Jun TG et al (2013) Interstage mortality for functional single ventricle with heterotaxy syndrome: a retrospective study of the clinical experience of a single tertiary center. J Cardiothorac Surg 8:93. (PMID: 10.1186/1749-8090-8-93)
Khan MS, Bryant R 3rd, Kim SH, Hill KD, Jacobs JP, Jacobs ML et al (2015) Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg 99:2134–2139. (PMID: 10.1016/j.athoracsur.2015.02.035)
Ota N, Fujimoto Y, Hirose K, Tosaka Y, Nakata T, Ide Y et al (2010) Improving results of atrioventricular valve repair in challenging patients with heterotaxy syndrome. Cardiol Young 20:60–65. (PMID: 10.1017/S1047951109990965)
Takeuchi K, McGowan FX Jr, Bacha EA, Mayer JE Jr, Zurakowski D, Otaki M et al (2006) Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect. Ann Thorac Surg 82:146–152. (PMID: 10.1016/j.athoracsur.2006.02.007)
Marathe SP, Cao JY, Celermajer D, Ayer J, Sholler GF, d’Udekem Y et al (2020) Outcomes of the fontan operation for patients with heterotaxy: a meta-analysis of 848 patients. Ann Thorac Surg 110:307–315. (PMID: 10.1016/j.athoracsur.2019.11.027)
Stamm C, Friehs I, Duebener LF, Zurakowski D, Mayer JE, Jonas RA et al (2002) Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg 74:1967–1977. (PMID: 10.1016/S0003-4975(02)04124-3)
Jacobs JP, Pasquali SK, Morales DL, Jacobs ML, Mavroudis C, Chai PJ et al (2011) Heterotaxy: lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons. World J Pediatr Congenital Heart Surg 2:278–286. (PMID: 10.1177/2150135110397670)
Shekerdemian LS, Bush A, Shore DF, Lincoln C, Redington AN (1997) Cardiopulmonary interactions after Fontan operations: augmentation of cardiac output using negative pressure ventilation. Circulation 96:3934–3942. (PMID: 10.1161/01.CIR.96.11.3934)
Nakhleh N, Francis R, Giese RA, Tian X, Li Y, Zariwala MA et al (2012) High prevalence of respiratory ciliary dysfunction in congenital heart disease patients with heterotaxy. Circulation 125:2232–2242. (PMID: 10.1161/CIRCULATIONAHA.111.079780)
Duong SQ, Godown J, Soslow JH, Thurm C, Hall M, Sainathan S et al (2019) Increased mortality, morbidities, and costs after heart transplantation in heterotaxy syndrome and other complex situs arrangements. J Thorac Cardiovasc Surg 157:730-740.e11. (PMID: 10.1016/j.jtcvs.2018.11.022)
Algaze CA, Koth AM, Faberowski LW, Hanley FL, Krawczeski CD, Axelrod DM (2017) Acute kidney injury in patients undergoing the extracardiac fontan operation with and without the use of cardiopulmonary bypass. Pediatr Crit Care Med 18:34–43. (PMID: 10.1097/PCC.0000000000000984)
Bartz PJ, Driscoll DJ, Dearani JA, Puga FJ, Danielson GK, O’Leary PW et al (2006) Early and late results of the modified fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol 48:2301–2305. (PMID: 10.1016/j.jacc.2006.07.053)
Esch JJ, Salvin JM, Thiagarajan RR, Del Nido PJ, Rajagopal SK (2015) Acute kidney injury after Fontan completion: risk factors and outcomes. J Thorac Cardiovasc Surg 150:190–197. (PMID: 10.1016/j.jtcvs.2015.04.011)
Ticho BS, Goldstein AM, Van Praagh R (2000) Extracardiac anomalies in the heterotaxy syndromes with focus on anomalies of midline-associated structures. Am J Cardiol 85:729–734. (PMID: 10.1016/S0002-9149(99)00849-8)
Blum M, Vick P (2015) Left-right asymmetry: cilia and calcium revisited. Curr Biol 25:R205–R207. (PMID: 10.1016/j.cub.2015.01.031)
Vetrini F, D’Alessandro LC, Akdemir ZC, Braxton A, Azamian MS, Eldomery MK et al (2016) Bi-allelic mutations in PKD1L1 are associated with laterality defects in humans. Am J Hum Genet 99:886–893. (PMID: 10.1016/j.ajhg.2016.07.011)
Olson AJ, Krentz AD, Finta KM, Okorie UC, Haws RM (2019) Thoraco-abdominal abnormalities in Bardet-Biedl syndrome: situs inversus and heterotaxy. J Pediatr 204:31–37. (PMID: 10.1016/j.jpeds.2018.08.068)
Lopez KN, Marengo LK, Canfield MA, Belmont JW, Dickerson HA (2015) Racial disparities in heterotaxy syndrome. Birth Defects Res A 103:941–950. (PMID: 10.1002/bdra.23416)
Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A (2008) Prevalence of congenital heart defects in metropolitan Atlanta, 1998–2005. J Pediatr 153:807–813. (PMID: 10.1016/j.jpeds.2008.05.059)
Pan JY, Lin CC, Wu CJ, Chang JP (2016) Early and intermediate-term results of the extracardiac conduit total cavopulmonary connection for functional single-ventricle hearts. J Formos Med Assoc 115:318–324. (PMID: 10.1016/j.jfma.2015.12.011)
Nakano T, Kado H, Tatewaki H, Hinokiyama K, Oda S, Ushinohama H et al (2015) Results of extracardiac conduit total cavopulmonary connection in 500 patients. Eur J Cardiothorac Surg 48:825–832. (PMID: 10.1093/ejcts/ezv072)
Kim SJ, Kim WH, Lim HG, Lee CH, Lee JY (2006) Improving results of the Fontan procedure in patients with heterotaxy syndrome. Ann Thorac Surg 82:1245–1251. (PMID: 10.1016/j.athoracsur.2006.04.082)
Pasquali SK, Peterson ED, Jacobs JP, He X, Li JS, Jacobs ML et al (2013) Differential case ascertainment in clinical registry versus administrative data and impact on outcomes assessment for pediatric cardiac operations. Ann Thorac Surg 95:197–203. (PMID: 10.1016/j.athoracsur.2012.08.074)
Loomba RS, Pelech AN, Shah PH, Anderson RH (2016) Determining bronchial morphology for the purposes of segregating so-called heterotaxy. Cardiol Young 26:725–737. (PMID: 10.1017/S1047951115001195)
Tremblay C, Loomba RS, Frommelt PC, Perrin D, Spicer DE, Backer C et al (2017) Segregating bodily isomerism or heterotaxy: potential echocardiographic correlations of morphological findings. Cardiol Young 27:1470–1480. (PMID: 10.1017/S104795111700049X)
Yim D, Nagata H, Lam CZ, Grosse-Wortmann L, Seed M, Jaeggi E et al (2018) Disharmonious patterns of heterotaxy and isomerism: how often are the classic patterns breached? Circ Cardiovasc Imaging 11:e006917. (PMID: 10.1161/CIRCIMAGING.117.006917)
فهرسة مساهمة: Keywords: Atrial isomerism; Congenital Heart Disease; Fontan; Heterotaxy; Post-operative outcomes
تواريخ الأحداث: Date Created: 20220122 Date Completed: 20220517 Latest Revision: 20220517
رمز التحديث: 20221213
DOI: 10.1007/s00246-021-02804-w
PMID: 35064275
قاعدة البيانات: MEDLINE
الوصف
تدمد:1432-1971
DOI:10.1007/s00246-021-02804-w