Heterotaxy syndrome and malrotation: Does isomerism influence risk and decision to treat

Published:February 03, 2014DOI:



      Controversy remains regarding the management of the asymptomatic heterotaxy syndrome (HS) patient with suspected intestinal rotational abnormalities. We evaluated the outcomes for our HS population to identify frequency of malrotation and identify characteristics of children who might benefit from expectant management.


      After IRB approval, a retrospective review of all patients treated for HS at a large tertiary care children’s hospital between January 2008 and June 2012 was performed. For the purpose of this paper, malrotation was defined as an operative note that described the presence of Ladd’s bands and a narrow mesentery.


      Thirty-eight patients with HS were identified, including 18 who underwent abdominal exploration. Left atrial isomerisation (LAI) was identified in 13 individuals, and right atrial isomerisation (RAI) was noted in 25. The rate of surgical intervention did not vary between the 2 groups (54%). Malrotation was found in 8 patients: one with LAI and 7 with RAI. This difference in incidence was statistically significant (p = 0.04).


      These data suggest that the direction of atrial isomerisation influences the likelihood of true malrotation, where RAI patients are more likely to be malrotated. Given the inherent risk of surgery on this medically fragile patient population, surgeons should consider expectant management for asymptomatic LAI patients.

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        • Polhemus D.W.
        • Schafer W.B.
        Congenital absence of the spleen; syndrome with atrioventricularis and situs inversus; case reports and review of the literature.
        Pediatrics. 1952; 9: 696-708
        • Ditchfield M.R.
        • Hutson J.M.
        Intestinal rotational abnormalities in polysplenia and asplenia syndromes.
        Pediatr Radiol. 1998; 28: 303-306
        • Nakada K.
        • Kawaguchi F.
        • Wakisaka M.
        • et al.
        Digestive tract disorders associated with asplenia/polysplenia syndrome.
        J Pediatr Surg. 1997; 32: 91-94
        • Yu D.C.
        • Thiagarajan R.R.
        • Laussen P.C.
        • et al.
        Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation.
        J Pediatr Surg. 2009; 44 ([discussion 1095]): 1089-1095
        • Pockett C.R.
        • Dicken B.
        • Rebeyka I.M.
        • et al.
        Heterotaxy syndrome: is a prophylactic Ladd procedure necessary in asymptomatic patients?.
        Pediatr Cardiol. 2013; 34: 59-63
        • Tashjian D.B.
        • Weeks B.
        • Brueckner M.
        • et al.
        Outcomes after a Ladd procedure for intestinal malrotation with heterotaxia.
        J Pediatr Surg. 2007; 42: 528-531
        • Choi M.
        • Borenstein S.H.
        • Hornberger L.
        • et al.
        Heterotaxia syndrome: the role of screening for intestinal rotation abnormalities.
        Arch Dis Child. 2005; 90: 813-815
        • Gillory L.A.
        • Megison M.L.
        • Harmon C.M.
        • et al.
        Laparoscopic surgery in children with congenital heart disease.
        J Pediatr Surg. 2012; 47: 1084-1088
        • Wulkan M.L.
        • Vasudevan S.A.
        Is end-tidal CO2 an accurate measure of arterial CO2 during laparoscopic procedures in children and neonates with cyanotic congenital heart disease?.
        J Pediatr Surg. 2001; 36: 1234-1236
        • Slater B.
        • Rangel S.
        • Ramamoorthy C.
        • et al.
        Outcomes after laparoscopic surgery in neonates with hypoplastic heart left heart syndrome.
        J Pediatr Surg. 2007; 42: 1118-1121
        • Cribbs R.K.
        • Heiss K.F.
        • Clabby M.L.
        • et al.
        Gastric fundoplication is effective in promoting weight gain in children with severe congenital heart defects.
        J Pediatr Surg. 2008; 43: 283-289
        • Mariano E.R.
        • Boltz M.G.
        • Albanese C.T.
        • et al.
        Anesthetic management of infants with palliated hypoplastic left heart syndrome undergoing laparoscopic nissen fundoplication.
        Anesth Analg. 2005; 100: 1631-1633
        • Fenton K.N.
        • Siewers R.D.
        • Rebovich B.
        • et al.
        Interim mortality in infants with systemic-to-pulmonary artery shunts.
        Ann Thorac Surg. 2003; 76 ([discussion 156–157]): 152-156
        • Hashmi A.
        • Abu-Sulaiman R.
        • McCrindle B.W.
        • et al.
        Management and outcomes of right atrial isomerism: a 26-year experience.
        J Am Coll Cardiol. 1998; 31: 1120-1126
        • Gilljam T.
        • McCrindle B.W.
        • Smallhorn J.F.
        • et al.
        Outcomes of left atrial isomerism over a 28-year period at a single institution.
        J Am Coll Cardiol. 2000; 36: 908-916
        • Bartz P.J.
        • Driscoll D.J.
        • Dearani J.A.
        • et al.
        Early and late results of the modified fontan operation for heterotaxy syndrome 30 years of experience in 142 patients.
        J Am Coll Cardiol. 2006; 48: 2301-2305
        • Anagnostopoulos P.V.
        • Pearl J.M.
        • Octave C.
        • et al.
        Improved current era outcomes in patients with heterotaxy syndromes.
        Eur J Cardiothorac Surg. 2009; 35 ([discussion 877–878]): 871-877
        • Swenson O.
        • Ladd W.E.
        Surgical emergencies of the alimentary tract of the newborn.
        N Engl J Med. 1945; 233: 660-663
        • Papillon S.
        • Goodhue C.J.
        • 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
        • Biko D.M.
        • Anupindi S.A.
        • Hanhan S.B.
        • et al.
        Assessment of recurrent abdominal symptoms after Ladd procedure: clinical and radiographic correlation.
        J Pediatr Surg. 2011; 46: 1720-1725
        • Angenete E.
        • Jacobsson A.
        • Gellerstedt M.
        • et al.
        Effect of laparoscopy on the risk of small-bowel obstruction: a population-based register study.
        Arch Surg. 2012; 147: 359-365
        • Gutt C.N.
        • Oniu T.
        • Schemmer P.
        • et al.
        Fewer adhesions induced by laparoscopic surgery?.
        Surg Endosc. 2004; 18: 898-906
        • Dowson H.M.
        • Bong J.J.
        • Lovell D.P.
        • et al.
        Reduced adhesion formation following laparoscopic versus open colorectal surgery.
        Br J Surg. 2008; 95: 909-914