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Original article
Physical and neurodevelopmental outcomes in children with single-ventricle circulation Joseph Davidson,1 Paul Gringras,2 Charlie Fairhurst,2 John Simpson3 1
King’s College London School of Medicine, London, UK 2 Department of Paediatric Neurology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Fiundation Trust, London, UK 3 Department of Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Fiundation Trust, London, UK Correspondence to Professor John Simpson, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London SE1 7EH, UK; [email protected] Received 3 April 2014 Revised 13 November 2014 Accepted 14 November 2014 Published Online First 5 December 2014
ABSTRACT Objective To investigate longer-term physical and neurodevelopmental outcomes of patients with hypoplastic left heart syndrome (HLHS) compared with other patients with functionally single-ventricle circulation surviving beyond the age of 10 years. Design A retrospective, observational study from a UK tertiary centre for paediatric cardiology. Results 58 patients with HLHS and 44 non-HLHS patients with single-ventricle physiology were included. Subjective reduction in exercise tolerance was reported in 72% (95% CI 61% to 84%) of patients with HLHS and 45% (31% to 60%) non-HLHS patients. Compared with non-HLHS patients, educational concerns were reported more frequently in patients with HLHS, 41% (29% to 54%) vs 23% (10% to 35%), as was a diagnosis of a behaviour disorder (autism or attention deficit hyperactivity disorder) 12% (4% to 21%) vs 0%, and referral to other specialist services 67% (55% to 79%) vs 48% (33% to 63%). Conclusions Within a group of young people with complex congenital heart disease, those with HLHS are likely to have worse physical, psychological and educational outcomes.
What is already known on this topic? ▸ More children with complex heart disease are surviving through to later childhood and adult life. ▸ Patients with congenital heart disease are known to be at risk for adverse developmental and behavioural outcome. ▸ Within the complex congenital heart disease population, there is a clinical suspicion of worse outcome among the hypoplastic left heart syndrome (HLHS) group.
What this study adds? ▸ Significant incidence of physical and developmental morbidity in patients managed towards a single-ventricle circulation. ▸ A higher incidence of behaviour disorders, educational concerns and subspecialty referrals was observed within the HLHS group compared with other functionally single-ventricle groups.
INTRODUCTION Background
To cite: Davidson J, Gringras P, Fairhurst C, et al. Arch Dis Child 2015;100:449–453.
Hypoplastic left heart syndrome (HLHS) is a congenital cardiac defect, with an incidence of approximately 1/4400 live births,1 in which left heart structures are underdeveloped and cannot support the systemic arterial circulation. Incorporation of cardiac views into mid-trimester anomaly scans means that the majority of cases of HLHS are now diagnosed during fetal life.2 In specialist centres, survival rates have improved dramatically, from a baseline of zero in the 1980s to 65% at age 5 and 55% at age 10 currently.3 This is due to the introduction of programmes of staged surgical palliation or transplantation. In the UK, the Norwood procedure, or its modifications,4–7 has become the initial management of choice. The eventual circulation leaves the systemic veins draining directly to the pulmonary arteries, and the right ventricle pumps systemic arterial blood (total cavopulmonary connection (TCPC)) in a functionally singleventricle circulation. TCPC and single-ventricle physiology is also the final mode of palliation for other severe forms of congenital heart disease (CHD) where a two-ventricle circulation cannot be achieved. HLHS has some specific considerations with respect to brain development. In HLHS, blood delivery to the fetal brain is supported by the right
ventricle pumping blood through the arterial duct. Brain perfusion is therefore retrograde through the aortic arch, in contrast to the normal antegrade flow. Previous data, based on MRI assessment of brain volume and cerebral metabolism, have suggested that fetuses with HLHS may be at particular risk for abnormal neurodevelopment.8 In patients with CHD with functional singleventricle circulation, cognitive development has been shown to be within normal limits in most cases, but, as a group, is lower than the normal population.9 Physical functional status and physical quality-of-life measures have been shown to be reduced compared with healthy control populations.10 11 Furthermore, data from the ongoing Single Ventricle Trial group have very clearly shown poorer neurodevelopment as measured by the psychomotor development index, motor development index of the Bayley development scores at 14 months.12 13 A clinical impression has developed among paediatric cardiologists that patients with HLHS may fare worse from a behavioural and neurodevelopmental perspective,14–17 even when compared with other forms of CHD managed towards a single-ventricle palliation. Many cases of complex CHD are recognised during fetal life, and a frequent parental concern is about the potential
Davidson J, et al. Arch Dis Child 2015;100:449–453. doi:10.1136/archdischild-2014-306449
449
Downloaded from http://adc.bmj.com/ on November 14, 2015 - Published by group.bmj.com
Original article neurodevelopmental and behavioural associations of CHD. There is, as such, a need for more data regarding the long-term status of these children.
Objectives The aim of this retrospective study was to report physical, psychological and educational outcomes of 10-year-old to 18-year-old patients with HLHS compared with non-HLHS patients who had also undergone TCPC.
Statistical methods Results were displayed with absolute numbers within group and percentages, with 95% CIs individually calculated by 1-propotion Z-intervals. Statistical significance of results was tested by twotailed Fisher’s exact test or, where group numbers allowed, χ2 analysis, with a value of p
Original article
Physical and neurodevelopmental outcomes in children with single-ventricle circulation Joseph Davidson,1 Paul Gringras,2 Charlie Fairhurst,2 John Simpson3 1
King’s College London School of Medicine, London, UK 2 Department of Paediatric Neurology, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Fiundation Trust, London, UK 3 Department of Congenital Heart Disease, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Fiundation Trust, London, UK Correspondence to Professor John Simpson, Evelina London Children’s Hospital, Guy’s and St Thomas’ NHS Foundation Trust, London SE1 7EH, UK; [email protected] Received 3 April 2014 Revised 13 November 2014 Accepted 14 November 2014 Published Online First 5 December 2014
ABSTRACT Objective To investigate longer-term physical and neurodevelopmental outcomes of patients with hypoplastic left heart syndrome (HLHS) compared with other patients with functionally single-ventricle circulation surviving beyond the age of 10 years. Design A retrospective, observational study from a UK tertiary centre for paediatric cardiology. Results 58 patients with HLHS and 44 non-HLHS patients with single-ventricle physiology were included. Subjective reduction in exercise tolerance was reported in 72% (95% CI 61% to 84%) of patients with HLHS and 45% (31% to 60%) non-HLHS patients. Compared with non-HLHS patients, educational concerns were reported more frequently in patients with HLHS, 41% (29% to 54%) vs 23% (10% to 35%), as was a diagnosis of a behaviour disorder (autism or attention deficit hyperactivity disorder) 12% (4% to 21%) vs 0%, and referral to other specialist services 67% (55% to 79%) vs 48% (33% to 63%). Conclusions Within a group of young people with complex congenital heart disease, those with HLHS are likely to have worse physical, psychological and educational outcomes.
What is already known on this topic? ▸ More children with complex heart disease are surviving through to later childhood and adult life. ▸ Patients with congenital heart disease are known to be at risk for adverse developmental and behavioural outcome. ▸ Within the complex congenital heart disease population, there is a clinical suspicion of worse outcome among the hypoplastic left heart syndrome (HLHS) group.
What this study adds? ▸ Significant incidence of physical and developmental morbidity in patients managed towards a single-ventricle circulation. ▸ A higher incidence of behaviour disorders, educational concerns and subspecialty referrals was observed within the HLHS group compared with other functionally single-ventricle groups.
INTRODUCTION Background
To cite: Davidson J, Gringras P, Fairhurst C, et al. Arch Dis Child 2015;100:449–453.
Hypoplastic left heart syndrome (HLHS) is a congenital cardiac defect, with an incidence of approximately 1/4400 live births,1 in which left heart structures are underdeveloped and cannot support the systemic arterial circulation. Incorporation of cardiac views into mid-trimester anomaly scans means that the majority of cases of HLHS are now diagnosed during fetal life.2 In specialist centres, survival rates have improved dramatically, from a baseline of zero in the 1980s to 65% at age 5 and 55% at age 10 currently.3 This is due to the introduction of programmes of staged surgical palliation or transplantation. In the UK, the Norwood procedure, or its modifications,4–7 has become the initial management of choice. The eventual circulation leaves the systemic veins draining directly to the pulmonary arteries, and the right ventricle pumps systemic arterial blood (total cavopulmonary connection (TCPC)) in a functionally singleventricle circulation. TCPC and single-ventricle physiology is also the final mode of palliation for other severe forms of congenital heart disease (CHD) where a two-ventricle circulation cannot be achieved. HLHS has some specific considerations with respect to brain development. In HLHS, blood delivery to the fetal brain is supported by the right
ventricle pumping blood through the arterial duct. Brain perfusion is therefore retrograde through the aortic arch, in contrast to the normal antegrade flow. Previous data, based on MRI assessment of brain volume and cerebral metabolism, have suggested that fetuses with HLHS may be at particular risk for abnormal neurodevelopment.8 In patients with CHD with functional singleventricle circulation, cognitive development has been shown to be within normal limits in most cases, but, as a group, is lower than the normal population.9 Physical functional status and physical quality-of-life measures have been shown to be reduced compared with healthy control populations.10 11 Furthermore, data from the ongoing Single Ventricle Trial group have very clearly shown poorer neurodevelopment as measured by the psychomotor development index, motor development index of the Bayley development scores at 14 months.12 13 A clinical impression has developed among paediatric cardiologists that patients with HLHS may fare worse from a behavioural and neurodevelopmental perspective,14–17 even when compared with other forms of CHD managed towards a single-ventricle palliation. Many cases of complex CHD are recognised during fetal life, and a frequent parental concern is about the potential
Davidson J, et al. Arch Dis Child 2015;100:449–453. doi:10.1136/archdischild-2014-306449
449
Downloaded from http://adc.bmj.com/ on November 14, 2015 - Published by group.bmj.com
Original article neurodevelopmental and behavioural associations of CHD. There is, as such, a need for more data regarding the long-term status of these children.
Objectives The aim of this retrospective study was to report physical, psychological and educational outcomes of 10-year-old to 18-year-old patients with HLHS compared with non-HLHS patients who had also undergone TCPC.
Statistical methods Results were displayed with absolute numbers within group and percentages, with 95% CIs individually calculated by 1-propotion Z-intervals. Statistical significance of results was tested by twotailed Fisher’s exact test or, where group numbers allowed, χ2 analysis, with a value of p
