Dextrocardia
Introduction
Dextrocardia is a rare condition (around 1 in 12,000 births) that can be associated with a wider Heterotaxy Syndrome that can affect the heart, lungs, liver, spleen and intestines. Children with dextrocardia require referral to multiple specialities and consideration for antibacterial prophylaxis if they are at risk of functional hyposplenia or asplenia.
Dextrocardia is a rare condition (around 1 in 12,000 births) that can be associated with a wider Heterotaxy Syndrome that can affect the heart, lungs, liver, spleen and intestines. Children with dextrocardia require referral to multiple specialities and consideration for antibacterial prophylaxis if they are at risk of functional hyposplenia or asplenia.
Scope
This guideline applies to all paediatric patients in the region.
This guideline applies to all paediatric patients in the region.
Purpose
The purpose of this guideline is to provide a standardised approach to the initial investigation and referral of dextrocardia
The purpose of this guideline is to provide a standardised approach to the initial investigation and referral of dextrocardia
Definitions
Dextrocardia
More than half of the heart is situated in the right side of the chest.
Heterotaxy Syndrome
Abnormal arrangement of organs (This encompasses situs inversus
Dextrocardia
More than half of the heart is situated in the right side of the chest.
Heterotaxy Syndrome
Abnormal arrangement of organs (This encompasses situs inversus
Summary
Investigations
Cardiac
Pre and post-ductal saturations, blood pressure and an ECG should be performed pending outpatient referral to paediatric cardiology. 90% of patients with dextrocardia will have an otherwise structurally normal heart.
Some patients will have been diagnosed antenatally and had a fetal echocardiogram. These patients may not require further cardiac evaluation, discuss with paediatric cardiology if uncertain.
Respiratory
Dextrocardia is associated with Primary Cilliary Dyskinesia (PCD) and patients are at risk of recurrent respiratory and ENT infections. 50% of patients with PCD have abnormal positioning of other organs. Refer to the PCD service as an outpatient.
Immunology
Patients are at risk of asplenia or functional hyposplenia. Start Penicillin V (as per BNFc) if asplenia or polysplenia is present, or if blood film has incidentally detected Howell-Jolly Bodies (N.B. blood film does not need to be done routinely by referring team). Start prophylactic antibiotics if there will be a significant delay in getting the results. Patients will require enhanced vaccination programme. Seek advice from and refer to tertiary immunology service.
Gastrointestinal
Ideally, perform an abdominal ultrasound prior to discharge. Otherwise, can be performed soon after birth or arranged to be done at the tertiary centre when has first speciality appointment. The purpose of the ultrasound is to look for asplenia or polysplenia. If there is a delay in obtaining results or if the child is sent home prior to the scan, start prophylactic antibiotics and provide safety net advice.
Dextrocardia can be associated with biliary atresia. Refer to Paediatric Hepatology as needed.
Dextrocardia is associated with malrotation, urgent referral to Paediatric Surgeons if bilious vomiting present. (Refer to regional bilious vomiting guideline.) Provide safety net advice to parents regarding signs and symptoms of malrotation.
Other systems
Can be associated with urogenital, muskulosketal and neurological (absent corpus callosum) problems. Refer to the relevant speciality as needed.
Cardiac
Pre and post-ductal saturations, blood pressure and an ECG should be performed pending outpatient referral to paediatric cardiology. 90% of patients with dextrocardia will have an otherwise structurally normal heart.
Some patients will have been diagnosed antenatally and had a fetal echocardiogram. These patients may not require further cardiac evaluation, discuss with paediatric cardiology if uncertain.
Respiratory
Dextrocardia is associated with Primary Cilliary Dyskinesia (PCD) and patients are at risk of recurrent respiratory and ENT infections. 50% of patients with PCD have abnormal positioning of other organs. Refer to the PCD service as an outpatient.
Immunology
Patients are at risk of asplenia or functional hyposplenia. Start Penicillin V (as per BNFc) if asplenia or polysplenia is present, or if blood film has incidentally detected Howell-Jolly Bodies (N.B. blood film does not need to be done routinely by referring team). Start prophylactic antibiotics if there will be a significant delay in getting the results. Patients will require enhanced vaccination programme. Seek advice from and refer to tertiary immunology service.
- Patients may be at risk of severe and life-threatening sepsis. Provide safety-net advice and consider providing open access with guidance on management or providing an Asplenia Card).
- The immunology and respiratory teams will endeavour to see the patient on the same day, however this cannot be guaranteed. We recommend using a single referral letter to immunology, PCD service and cardiology to avoid duplication.
Gastrointestinal
Ideally, perform an abdominal ultrasound prior to discharge. Otherwise, can be performed soon after birth or arranged to be done at the tertiary centre when has first speciality appointment. The purpose of the ultrasound is to look for asplenia or polysplenia. If there is a delay in obtaining results or if the child is sent home prior to the scan, start prophylactic antibiotics and provide safety net advice.
Dextrocardia can be associated with biliary atresia. Refer to Paediatric Hepatology as needed.
Dextrocardia is associated with malrotation, urgent referral to Paediatric Surgeons if bilious vomiting present. (Refer to regional bilious vomiting guideline.) Provide safety net advice to parents regarding signs and symptoms of malrotation.
Other systems
Can be associated with urogenital, muskulosketal and neurological (absent corpus callosum) problems. Refer to the relevant speciality as needed.
Implementation
This guideline will be made available regionally on the PIER Website. Local leads for critically ill children will disseminate guideline and raise awareness locally
This guideline will be made available regionally on the PIER Website. Local leads for critically ill children will disseminate guideline and raise awareness locally
Process for Monitoring Effectiveness
The Wessex Paediatric Critical Care Network will review problems associated with a failure to comply with this guideline through its regional governance process
The Wessex Paediatric Critical Care Network will review problems associated with a failure to comply with this guideline through its regional governance process
References
- Lucas JS, Burgess A, Mitchison HM on behalf of the National PCD Service, UK, et al Diagnosis and management of primary ciliary dyskinesia. Archives of Disease in Childhood 2014;99:850-856.
- Wessex Care Pathway for Term Infants Referred with Bilious Vomiting for Exclusion of Malrotation. http://www.sort.nhs.uk/Media/SONeT/Guidelines/NetworkClinical/Wessex%20Bilious%20Vomiting%20Pathway%20-%20Malrotation%20guideline%20final%20version%201.3%20(1).pdf
- Chin AJ, Heterotaxy Syndrome and Primary Ciliary Dyskinesia https://emedicine.medscape.com/article/896757-overview#a4 (Accessed February 2018)
- Children’s Hospital of Philadelphia. Heterotaxy Syndrome (Isomerism) http://www.chop.edu/conditions-diseases/heterotaxy-syndrome-isomerism (Accessed February 2018)
- Di Sabatino A, Carsetti R, Corazza GR. Post-splenectomy and hyposplenic states. Lancet. 2011 Jul 2;378(9785):86-97. doi: 10.1016/S0140-6736(10)61493-6. Epub 2011 Apr 5.
- de Porto AP, Lammers AJ, Bennink RJ, ten Berge IJ, Speelman P, Hoekstra JB. Assessment of splenicfunction. Eur J Clin Microbiol Infect Dis. 2010 Dec;29(12):1465-73. doi: 10.1007/s10096-010-1049-1. Epub 2010 Sep 19.
- Bohun CM1, Potts JE, Casey BM, Sandor GG. A population-based study of cardiac malformations and outcomes associated with dextrocardia. Am J Cardiol. 2007 Jul 15;100(2):305-9. Epub 2007 May 25.
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Document Version:
1.0 Lead Author: Dr Reynella Morenas (Consultant Paediatrician, HHFT) Additional Authors Dr Daniel Owens (Paediatric Registrar, Wessex Region) |
Approving Networks:
Wessex Cardiac Network Wessex Respiratory Network Wessex Immunology Network Date of Approval: 01/2020 Review Date: 01/2023 |
PIER Contact |
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