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Categorization of heterotaxy disorders on cardiac magnetic resonance imaging: a single institution experience

Session Poster session 2

Speaker Sundar Veerappan

Event : EuroCMR 2019

  • Topic : imaging
  • Sub-topic : Imaging - Other
  • Session type : Poster Session

Authors : S Veerappan (Bristol,GB), S Moharem-Elgamal (Bristol,GB), M Hamilton (Bristol,GB)

S Veerappan1 , S Moharem-Elgamal1 , M Hamilton1 , 1Bristol Heart Institute - Bristol - United Kingdom of Great Britain & Northern Ireland ,

European Heart Journal - Cardiovascular Imaging ( 2019 ) 20 ( Supplement 2 ), ii379

Background/Introduction: Heterotaxy syndrome is a disorder of the left-right axis determination during early fetal development.  These are usually categorised into "left" or "right" atrial isomerism based on the atrial appendage morphology with classical patterns of cardiac and abdominal abnormalities described. Recent studies have demonstrated that these associates are breached in up to 21% of patients with laterality disorders.

Purpose: The aim of this retrospective study was to utilise cardiac magnetic resonance imaging to describe the cardiac and extracardiac manifestations of patients with heterotaxy syndrome in our single tertiary institution.

Methods and Results: A search of all the MRI reports over the last 10 years in our institution was undertaken for key phrases like "heterotaxy", "isomerism", "interrupted IVC" and "anomalous pulmonary vein". A total of 29 patients were identified with a median age of 23 years (range 7 -69 years). There was no significant gender difference with 51% being female.

There were 16 patients who had "left" atrial isomerism. 5 patients (31%) had no significant associated congenital cardiac abnormality. 2 patients (13%) required a pacemaker for complete heart block, with a further 5 patients (31%) having atrial tachycardia/fibrillation. 7 patients (44%) had a right arch, 2 had circumflex arch and 1 had a double arch. One of the patients demonstrated normal, non-isomeric bronchopulmonary branching pattern. Only 38% of the patients demonstrated classical polysplenia. There was a wide variability in the location of the liver (Left – 2 patients, Midline – 8 patients and Right – 5 patients). The stomach was located on the left in 56% of patients with the rest being on the right.

All of the 13 patients with "right" atrial isomerism had undergone single ventricle palliation. 2 patients (15%) had atrial tachycardia and 1 patient (7.7%) had AVRT. 54% of patients had a right arch. 92% of the patients demonstrated the classical association of asplenia.  There was again a wide variability in the location of the liver with midline being most common (8 patients). The stomach was more likely to be located on the left, (8 patients, 62%).

Conclusion: In our population, patients with "left" atrial isomerism were more likely to not follow the classical cardiac and extracardiac associations of this condition. They were also more likely to have an associated atrial tachycardia. The prevalence of aortic arch variations in the heterotaxy group was also much higher than in the normal population.

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