Hostname: page-component-78c5997874-g7gxr Total loading time: 0 Render date: 2024-11-10T09:19:47.216Z Has data issue: false hasContentIssue false
  • English
  • Français

Impact of gastrointestinal comorbidities in patients with right and left atrial isomerism

Published online by Cambridge University Press:  02 September 2021

Anastasia Fotaki Open the ORCID record for Anastasia Fotaki [Opens in a new window] ,
Victoria L. Doughty ,
Winston Banya ,
Stefano Giuliani ,
Sarah Bradley  and
Julene S. Carvalho
Anastasia Fotaki*
Affiliation:
Brompton Centre for Fetal Cardiology, Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital, London SW3 6NP, UK St. George’s University Hospitals NHS Foundation Trust, St George’s Hospital, London SW17 0QT, UK
Victoria L. Doughty
Affiliation:
Brompton Centre for Fetal Cardiology, Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital, London SW3 6NP, UK
Winston Banya
Affiliation:
Department of Medical Statistics, Research & Development, Royal Brompton & Harefield NHS Foundation Trust, London SW3 6NP, UK
Stefano Giuliani
Affiliation:
Department of Neonatal & Paediatric Surgery, St. George’s University Hospitals NHS Foundation Trust, London SW17 0QT, UK
Sarah Bradley
Affiliation:
Department of Neonatal & Paediatric Surgery, St. George’s University Hospitals NHS Foundation Trust, London SW17 0QT, UK
Julene S. Carvalho
Affiliation:
Brompton Centre for Fetal Cardiology, Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital, London SW3 6NP, UK St. George’s University Hospitals NHS Foundation Trust, St George’s Hospital, London SW17 0QT, UK Cardiovascular Clinical Academic Group, Molecular and Clinical Sciences Research Institute, St. George’s, University of London, St. George’s University Hospitals NHS Foundation Trust, London SW17 0RE, UK
*
Author for correspondence: Dr A. Fotaki, Clinical Fellow in Paediatric and Fetal Cardiology, Brompton Centre for Fetal Cardiology, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. Tel: +44 (20) 7351 8361. E-mail: anastasia.fotaki@kcl.ac.uk
Get access Rights & Permissions [Opens in a new window]

Abstract

Background and aim:

Heterotaxy syndrome, being right atrial isomerism (RAI) or left atrial isomerism (LAI), often presents with Congenital Heart Disease (CHD). Intestinal abnormalities, including malrotation are common. We assessed the spectrum of gut abnormalities and their impact on medium-term outcome in a cohort of patients with fetal and postnatal diagnoses of heterotaxy syndrome.

Methods:

We reviewed the cardiology records of heterotaxy syndrome patients from two centres, regarding the presence of CHD, time for cardiac intervention, presence of gastrointestinal abnormalities, and type/time of surgery. A questionnaire about gastrointestinal status was sent to patients <18 years old. Kaplan–Meier curves were derived for survival data and freedom from intervention.

Results:

Data were included for 182 patients (49 RAI and 133 LAI) of 247 identified. Questionnaires were sent to 77 families and 47 replied. CHD was present in all RAI and 61.7% of LAI cases. Thirty-eight patients had abdominal surgery (20.9%), similar for RAI and LAI (20.4% versus 21%, p> 0.99): Ladd procedure in 17 (44.7%), non-Ladd in 12 (31.5%), and both procedures in 9 (23.7%). Ten-year freedom from Ladd procedure for all was 86% for the whole cohort (RAI = 87%; LAI = 85%, p = 0.98). Freedom from any gastrointestinal surgery at 10 years was 79% for the whole cohort (RAI = 77%; LAI = 80%, p = 0.54). Ten-year freedom from cardiac surgery was 31% for the whole cohort (RAI = 6%; LAI = 43%, p < 0.0001).

Conclusions:

In our cohort, one in five patients required abdominal surgery, mostly in their first year of life, similar for RAI and LAI. Between 1 and 10 years of follow-up, the impact of gastrointestinal abnormalities on outcome was minimal. Medium term survival was related to CHD.

Keywords

Heart defectscongenitalprenatal diagnosisfetalechocardiography
Type
Original Article
Information
Cardiology in the Young , Volume 32 , Issue 7 , July 2022 , pp. 1053 - 1060
Check for updates
Copyright
© The Author(s), 2021. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Raya, A, Izpisua Belmonte, JC. Insights into the establishment of left-right asymmetries in vertebrates. Birth Defects Res C Embryo Today 2008; 84: 81–94.CrossRefGoogle ScholarPubMed
Raya, A, Izpisua Belmonte, JC. Sequential transfer of left-right information during vertebrate embryo development. Curr Opin Genet Dev 2004; 14: 575–581.10.1016/j.gde.2004.07.011CrossRefGoogle ScholarPubMed
Lin, AE, Ticho, BS, Houde, K, Westgate, MN, Holmes, LB. Heterotaxy: associated conditions and hospital-based prevalence in newborns. Genet Med 2000; 2: 157–172.CrossRefGoogle ScholarPubMed
Salavitabar, A, Anderson, BR, Aspelund, G, Starc, TJ, Lai, WW. Heterotaxy syndrome and intestinal rotational anomalies: impact of the ladd procedure. J Pediatr Surg 2015; 50: 1695–1700.CrossRefGoogle ScholarPubMed
Yu, DC, Thiagarajan, RR, Laussen, PC, Laussen, JP, Jaksic, T, Weldon, CB. Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation. JPediatrSurg 2009; 44: 1089–1095.Google ScholarPubMed
Lim, J, McCrindle, B, Smallhorn, J, et al. Clinical features, management, and outcome of children with fetal and postnatal diagnoses of isomerism syndromes. Circulation 2005; 112: 2454–2461.CrossRefGoogle ScholarPubMed
Nakada, K, Kawaguchi, F, Wakisaka, M, Nakada, M, Enami, T, Yamate, N. Digestive tract disorders associated with asplenia/polysplenia syndrome. J Pediatr Surg 1997; 32: 91–94.CrossRefGoogle ScholarPubMed
Ditchfield, MR, Hutson, JM. Intestinal rotational abnormalities in polysplenia and asplenia syndromes. PediatrRadiol 1998; 28: 303–306.Google ScholarPubMed
Strouse, PJ. Disorders of intestinal rotation and fixation (“malrotation”). Pediatr Radiol 2004; 34: 837–851.CrossRefGoogle ScholarPubMed
Ferdman, B, States, L, Gaynor, JW, Hedrick, HL, Rychik, J. Abnormalities of intestinal rotation in patients with congenital heart disease and the heterotaxy syndrome. Congenit Heart Dis 2007; 2: 12–18.CrossRefGoogle ScholarPubMed
Tashjian, DB, Weeks, B, Brueckner, M, Touloukian, RJ. Outcomes after a Ladd procedure for intestinal malrotation with heterotaxia. J Pediatr Surg 2007; 42: 528–531.10.1016/j.jpedsurg.2006.10.060CrossRefGoogle ScholarPubMed
Graziano, K, Islam, S, Dasgupta, R, et al. Asymptomatic malrotation: diagnosis and surgical management: an american pediatric surgical association outcomes and evidence based practice committee systematic review. J Pediatr Surg 2015; 50: 1783–1790.CrossRefGoogle ScholarPubMed
Pockett, CR, Dicken, B, Rebeyka, IM, Ross, DB, Ryerson, LM. Heterotaxy syndrome: is a prophylactic ladd procedure necessary in asymptomatic patients? Pediatr Cardiol 2013; 34: 59–63.CrossRefGoogle ScholarPubMed
McVay, MR, Kokoska, ER, Jackson, RJ. Jack Barney Award. The changing spectrum of intestinal malrotation: diagnosis and management. Am J Surg 2007; 194: 712–717, discussion 718-9.CrossRefGoogle ScholarPubMed
Hill, SJ, Heiss, KF, Mittal, R, et al. Heterotaxy syndrome and malrotation: does isomerism influence risk and decision to treat. J Pediatr Surg 2014; 49: 934–937, discussion 937.CrossRefGoogle ScholarPubMed
Cohen, MS. Clarifying anatomical complexity: diagnosing heterotaxy syndrome in the fetus. Prog Pediatr Cardiol 2006; 22: 61–70.10.1016/j.ppedcard.2006.01.006CrossRefGoogle Scholar
Van Praagh, S, Santini, F, Sanders, S. Cardiac malpositions with special emphasis on visceral heterotaxy (asplenia and polysplenia syndromes). In: Keany JF, Lock JE, Fyler DC (eds). Nadas’s Pediatric Cardiology, 2nd edn. Saunders Elsevier, Philadelphia, 2006: 589–608.Google Scholar
D’Emidio, L, Carvalho, JS. Left isomerism in the fetus: is it always bad news? frequency andd outcome of cases in the ‘normal heart’. Ultrasound Obst Gyn 2007; 30: 475–475.10.1002/uog.4456CrossRefGoogle Scholar
Kloesel, B, DiNardo, JA, Body, SC. Cardiac embryology and molecular mechanisms of congenital heart disease: a primer for anesthesiologists. Anesth Analg 2016; 123: 551–569.CrossRefGoogle ScholarPubMed
Snyder, WH, Chaffin, L. Embryology and pathology of the intestinal tract: Presentation of 40 cases of malrotation, From the Surgical Service of Los Angeles Childrens Hospital and the University of Southern Californal School of Medicine, 1954, 368–379.CrossRefGoogle Scholar
Escobar-Diaz, MC, Friedman, K, Salem, Y, et al. Perinatal and infant outcomes of prenatal diagnosis of heterotaxy syndrome (asplenia and polysplenia). Am J Cardiol 2014; 114: 612–617.CrossRefGoogle ScholarPubMed
Serraf, A, Bensari, N, Houyel, L, et al. Surgical management of congenital heart defects associated with heterotaxy syndrome☆. Eur J Cardio-THORAC 2010; 38: 721–727.10.1016/j.ejcts.2010.02.044CrossRefGoogle ScholarPubMed
Pepes, S, Zidere, V, Allan, LD. Prenatal diagnosis of left atrial isomerism. Heart (British Cardiac Society) 2009; 95: 1974–1977.CrossRefGoogle ScholarPubMed
Gilljam, T, Mccrindle, BW, Smallhorn, JF, Williams, WG, Freedom, RM. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol 2000; 36: 908–916.CrossRefGoogle Scholar
White, SC, Dean, PN, McGahren, ED, Gangemi, JJ, Vergales, J. Malrotation is not associated with adverse outcomes after cardiac surgery in patients with heterotaxy syndrome. J Pediatr Surg 2018; 53: 1494–1498.10.1016/j.jpedsurg.2018.01.008CrossRefGoogle Scholar
Papillon, S, Goodhue, CJ, Zmora, O, et al. Congenital heart disease and heterotaxy: upper gastrointestinal fluoroscopy can be misleading and surgery in an asymptomatic patient is not beneficial. J Pediatr Surg 2013; 48: 164–169.CrossRefGoogle Scholar
Pockett, CR, Dicken, BJ, Rebeyka, IM, Ross, DB, Ryerson, LM. Heterotaxy syndrome and intestinal rotation abnormalities: a survey of institutional practice. J Pediatr Surg 2013; 48: 2078–2083.10.1016/j.jpedsurg.2013.03.001CrossRefGoogle ScholarPubMed
Choi, M, Borenstein, SH, Hornberger, L, Langer, JC. Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities. ArchDisChild 2005; 90: 813–815.Google ScholarPubMed
Elder, CT, Metzger, R, Arrington, C, Rollins, M, Scaife, E. The role of screening and prophylactic surgery for malrotation in heterotaxy patients. J Pediatr Surg 2014; 49: 1746–1748.CrossRefGoogle ScholarPubMed
Tan, YW, Khalil, A, Kakade, M, et al. Screening and treatment of intestinal rotational abnormalities in heterotaxy: a systematic review and Meta-Analysis. J Pediatr 2016; 171: 153–62 e1-3.CrossRefGoogle ScholarPubMed