- Email: [email protected]
Long-term cognitive outcome in teenage survivors of arrhythmic cardiac arrest
Resuscitation (2008) 77, 46—50
available at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/resuscitation
CLINICAL PAPER
Long-term cognitive outcome in teenage survivors of arrhythmic cardiac arrest夽 Agnieszka Maryniak a,∗, Alicja Bielawska a, Franciszek Walczak b, ekawek a, Łukasz Szumowski b, Katarzyna Bieganowska a, Joanna R˛ zbieta Szymaniak a, Maria Knecht a Monika Paszke a, El˙ a b
Children’s Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw, Poland
Received 2 July 2007; received in revised form 5 September 2007; accepted 31 October 2007
KEYWORDS Sudden cardiac arrest; Ventricular arrhythmias; Neurocognitive function
夽
Summary Background: Sudden cardiac arrest (SCA) can be the first sign of ventricular arrhythmia in teenagers. Neurocognitive problems are common after successful resuscitation. We studied cognitive function in teenage survivors of SCA, including emotional status and coping ability. Method: Ten SCA survivors, aged 11—19 years, had neuropsychological tests within a few weeks of resuscitation. Awareness status, orientation, episodic and semantic memory, basic auditoryvisual functions, praxis and speech, short-term memory, ability to learn new verbal and visual material were assessed. These tests were repeated at about 6 months. Results: Eight patients had an initial assessment; one boy remained in a coma and one was making simple emotional contact, revealing intensified mixed aphasia and dyskinesia. Six patients had severe disturbances of memory, motor functions and praxis. After 6 months, four patients had no neurocognitive disturbance. Four patients had memory impairment making school education difficult. Two patients were totally dependent on caregivers. Because of the absence of symptoms before SCA, and amnesia relating to the SCA episodes, patients had problems accepting their heart problems and limitations resulting from it. Conclusion: Teenagers surviving SCA have significant neurcognitive and psychological problems. They need psychological care and guidance in understanding their condition. © 2007 Elsevier Ireland Ltd. All rights reserved.
A Spanish translated version of the summary of this article appears as Appendix in the final online version at doi:10.1016/j.resuscitation.2007.10.024. ∗ Corresponding author. E-mail address: [email protected] (A. Maryniak).
Teenagers make up only about 0.5% of sudden cardiac arrest (SCA) patients.1,2 SCA is caused by arrhythmias including the Wolff-Parkinson-White syndrome (WPW). Genetic causes include long-QT syndromes, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia and arrythmogenic right ventricular cardiomyopathy.3,4
0300-9572/$ — see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.resuscitation.2007.10.024
Long-term cognitive outcome in teenage survivors of arrhythmic CA Neurocognitive problems are common in survivors of cardiac arrest. The duration of brain hypoxaemia, the patient’s age and pre-existing medical problems are important determinants of neurocognitive outcome. Children may have greater neurocognitive dysfunction after SCA as the developing brain is especially sensitive to hypoxia.5,6 On the other hand, SCA in teenagers usually occurs in previously healthy individuals with no previous neurocognitive deficit. We have studied neurocognitive function shortly after resuscitation and at 6 months in teenage survivors of SCA, including emotional status and coping ability.
Material and methods Teenager SCA survivors hospitalised between 2003 and 2006, and who attended two specialist arrhythmia clinics treatment were studied. Subjects were referred for a cardiology assessment by the centres where initial resuscitation had taken place. The time between SCA to admission to our clinics and first neuropsychological tests varied from 5 days to 2 months. Information about the original SCA and resuscitation (bystander resuscitation, paramedics, hospital) was obtained from medical records. Awareness status, orientation, and depending on the patient’s condition, episodic and semantic memory, basic audio-visual functions, praxis and speech, short-term memory, ability to learn new verbal and visual material were assessed. The tests of higher cognitive processes developed by A.R. Łuria (Polish adaptation7 ) were used they enable determination of whether a patient suffers from disturbances with the features of combativeness, apraxis or aphasia. In addition the Auditory-Verbal Learning Test, Benton Visual Retention Test and Rey’s Complex Figure Test were used. These tests have Polish validation for a given age group. The patient’s general condition was classified using Cerebral Performance Categories.8 Scoring is on a five-point scale: 1 = full independence, ability to function at school or in a professional environment in a way which is appropriate for a given age, with possible slight neurological deficits. 2 = dysfunctions of moderate intensification which limit capability, but not prevent from self-care in everyday life and some professional activity. 3 = complete dependence on carers. 4 = vegetative state, coma. 5 = death. Testing was repeated at about 6 months. All the patients are still under observation (duration varying from 6 months
Table 1
1 2 3 4 5 6 7 8 9 10
47
to 4 years). Information about their school results, possible problems with emotional or social functions, and rehabilitation progress is still being collected.
Results The group consisted of eight boys and two girls, aged from 11 to 19 years (Table 1). Six of them were diagnosed with Wolff-Parkinson-White (WPW) syndrome and four of them with long-QT syndrome. One girl (patient 4) had a previous history of arrhythmias and was being investigated for this at the time of her SCA. SCA was the first sign of cardiac disease in the others. The patients with WPW were treated by radiofrequency ablation of their atrioventricular accessory pathway. The patients with long-QT syndrome had a cardioverter-defibrillator implanted.
Circumstances of cardiac arrest and resuscitation According to information provided by witnesses, three patients (patients 4,5,8) reported feeling unwell (weakness, vertigo, palpitation) just before SCA. In two of them (patients 4,8) cardiac arrest occurred while being transported by the family to hospital (including one case of cardiac arrest in the admission room). In four cases (patients 1,2,3,7) there are no data regarding subjective patients’ feelings and symptoms preceding cardiac arrest as these situations occurred outside the home witnessed by bystanders. Retrograde amnesia of events preceding cardiac arrest meant that patients themselves could not provider this information. In three boys (patients 6,9,10) cardiac arrest was sudden, after physical effort and these patients did not report feeling unwell. In a female patient in whom cardiac arrest occurred in hospital just as she arrived with her family, resuscitation was promptly initiated. In the remaining patients cardiac arrest was outside hospital. In four cases resuscitation was initiated by witnesses. In five cases resuscitation only started when an ambulance crew arrived on the scene. This group probably had a longer period of brain hypoxia.
Cognitive function Neurological disorders and/or significant deficits in cognitive functions (Table 2 shows detailed results) were observed
Patient characteristics Sex
Age (years, months)
Syndrome
Resuscitation
Treatment
1st exam
Time of observation
F M M F M M M M M M
14, 12, 17, 16, 19, 16, 19, 18, 11, 11,
Long-QT Long-QT Long-QT Long-QT WPW WPW WPW WPW WPW WPW
Bystander Paramedics Bystander Hospital Paramedics Paramedics Paramedics Bystander Bystander Paramedics
ICD ICD ICD ICD RF ablation RF ablation RF ablation RF ablation RF ablation RF ablation
3 weeks 2 weeks 4 weeks 6 days 10 days 2 weeks 7 weeks 8 days 2 weeks 8 weeks
6 months 7 months 9 months 4 years 18 months 12 months 2 years 18 months 12 months 4 years
9 3 9 0 1 8 5 8 4 1
48
A. Maryniak et al.
Table 2 Sex
1 2 3 4 5 6 7 8 9 10
F M M F M M M M M M
Results of first (1) and second (2) neuropsychological examinations Age (years, months)
14, 12, 17, 16, 19, 16, 19, 18, 11, 11,
9 3 9 0 1 8 5 8 4 1
CPC
Aphasia
Apraxia
AVLT
Rey’s CFT
Benton’s VTRT
1
2
1
2
1
2
1
2
1
2
1
2
2 1 1 2 2 2 3 2 2 4
2 1 1 1 2 2 3 1 1 3
No No No No Yes No Yes No Yes X
No No No No No No Yes No No X
No No No No Yes Yes X No No X
No No No No Yes No X No No X
—— + ++ ++ —— —— X + — X
— + ++ ++ — — X + — X
—— + ++ — — — X —— —— X
—— ++ ++ + — — X + — X
—— + + — — — X — — X
—— + + + — — X + — X
CPC, cerebral performance categories. X, examination not done. — —, result <10 centile; —, result >10—30 centile; +, result >30—70 centile; ++, result >70 centile.
in eight patients during the first examination after SCA. One boy (patient 10) remained in a coma (CPC 4), one (patient 7) was making simple emotional contact, revealing intensified mixed aphasia and dyskinesia which disabled independent movements (CPC 3). No disturbances of cognitive functions were observed in two boys (patients 2,3 — CPC 1). Memory, speech and praxis disturbances of different intensification were observed in the remaining six patients (patients 1,4,5,6,8,9 — CPC 2). Fresh memory disturbances (problems with memorising current events, new material, both visual and verbal) were predominant, as well as problems with semantic memory—–updating information learned before SCA. Two boys (patients 5,6) experienced problems regarding episodic memory, including facts about themselves and their family. Deficits in speech, found in three patients (patients 5,7,9), were manifested as semantic aphasia (problems updating words). Intensified disturbances of praxis (performing targeted complex movements) were observed in two patients (patients 5,6) and they limited patients’ independence regarding self-care such as dressing and eating. Patient 8 also manifested symptoms of the frontal syndrome, such as impulsiveness, curtailing the time spent in social contacts, no adequate emotional reactions and lack of criticism towards his own behaviour. At 6th month, in two patients (patients 4,8) the deficits previously observed had subsided and patients had fully recovered. Some improvement was seen in four patients (patients 1,5,6,9,) in terms of cognitive functions; however, these were still not characteristic for their age. These current deficits (mainly regarding memory) made school education and everyday life difficult. The boy in a coma (patient 10) during the first examination regained consciousness but was quadriplegic and mute. His emotional reactions were appropriate. He answered questions using his eyes showing good reception of stimuli and their correct understanding. These are signs of a locked-in syndrome. In the period between the first and the second examination the two patients (patients 7,10) with the most severe status underwent intensive rehabilitation. This rehabilitation is still continuing. Long-term follow-up, which in one case has lasted two years, in another four years, reveals slow improvement of their condition. Both patients are still
fully dependent on the constant care of other people. The remaining subjects have returned to the activities typical for their age and continue their education. Five patients (1,5,6,8,9) experienced subjective difficulties, feelings of decreased cognitive functions and their learning requires more effort and time, and their achievements are poorer than before SCA.
Emotional status Subjects did not manifest symptoms of increased anxiety or fear after SCA. This is probably connected with the fact that all of them experienced retrograde and anterograde amnesia from several hours to 2 days before SCA and for several days after regaining consciousness. The patients were told about their situation by their families and doctors, therefore they did not feel under threat. However, they experienced some disorientation, had problems with cognitive understanding of their condition, the necessity to perform a planned procedure, its character and meaning. Before SCA these patients had been unaware of their heart disease, had not experienced any previous symptoms and were considered to be healthy and thought they were healthy. Their condition after SCA including problems regarding range of movements, speech, also did not indicate, in their subjective view, a cardiology problem and was more frequently connected with trauma. Understanding the situation was especially difficult for six patients with fresh memory disturbances as they did not remember the advice and explanations they received in the early period after SCA, and their experience and feelings were contradictory to information given by doctors and family (e.g. a 16-yearold boy [patient 6] claimed he was in hospital due to his leg disease as he was suffering pain connected with the site of injection made during his radiofrequency ablation. He did not remember information about his heart disease and a procedure). In a control examination the group with WPW who had undergone radiofrequency ablation did not show any worrying symptoms regarding emotional functions. In some cases parents reported problems regarding their limitations (e.g.
Long-term cognitive outcome in teenage survivors of arrhythmic CA physical activity, sports, choice of further education or job). For example, one boy in this group (patient 8) underwent SCA in WPW syndrome 2 months before beginning studies at a fireman’s college. He had to change his educational and professional plans. It was very difficult for him and induced rebellion and potentially dangerous behaviour, such as intensive physical exercise to prove he was healthy. In the case of patients with an implanted cardioverterdefibrillator (ICD) the situation was different. The constant presence of a device resulted in limitations and experience of the ICD intervention (twice in one boy [patient3], once in the second [patient 2], while in the two remaining ones— –without shocks) constituted confirmation of disease and risk. Increased anxiety and concentration on body signals were observed in all these patients. Follow-up observation indicates gradual adaptation to disease.
Discussion We have analysed neurocognitive functions in teenage survivors of SCA. Most experienced significant neurocognitive dysfunctions during the first days and weeks following SCA. The problems observed were connected with physical skills, audio-visual function and speech, and different aspects of memory. At 6th month there was significant improvement in terms of speech, audio-visual function and movements; however, in some patients memory disturbances remained. Despite improvement since the initial test, memory skills were below normal for age. Memory deficits are common in SCA survivors. This may be due to the sensitivity of the hippocampus to hypoxia.9 These deficits are often permanent.10—12 This may have a significantly negative influence on the further development of teenagers undergoing education. Memory disturbance limits the possibilities of effective learning and decreases the level of school achievement. Two boys (patients 7,10) with severe neurological disturbances who have been followed-up for several years show very slow improvement. It is likely that they will always be reliant on carers. We do not have detailed information about the duration of cardiac arrest. It is likely that it was the longest in patients who had to wait for an ambulance crew to arrive and start resuscitation. This group includes two boys with permanent severe brain damage and two other patients with significant memory disturbances. Two out four patients who had immediate bystander resuscitation made a complete recovery. In most cases our patients do not have any conscious memories of their SCA. They did not feel or do not remember feeling fear, helplessness, they do not know what symptoms preceded their loss of consciousness. Their knowledge about what happened comes from other people. If they were ill previously, they are able to make connections between their current condition and known information about the disease. Teenagers who regain consciousness following SCA have more problems than adults in understanding the situation they are experiencing. Sudden cardiac arrest was the first symptom of the disease in almost all patients in the analysed group. Therefore, they had thought they were completely healthy and others had thought the same. For a young person
49
cardiac disease is an abstraction which remains outside their experience, it is not a problem they meet among their peers, it is a problem connected with adults, with older people. Our patients and their families were not aware that a child may experience SCA. Therefore it is difficult for these patients to understand what really happened, what the reason and consequences for what happened are, and what the real risk to their lives was. On one hand, they do not feel appropriate fear, but on the other hand, it may hamper acceptance of the need for hospitalisation, any treatment (especially when ICD implantation is necessary) and limitations regarding their lifestyle. Our study indicates that these patients need psychological care. During the first weeks or months after SCA they need adequate description of their situation, such that does not provoke feelings of excessive danger but does present their current health status. Moreover, limitations due to memory disturbances and other cognitive functions should be considered. Information given in the early period may not be understood appropriately or permanently recorded in the memory, therefore, it may be necessary to repeat it several times. In some cases patients will need help coming to terms with their illness. In the cases of patients with an ICD, long-term regular psychological examinations are indicated as possible ICD interventions may be a risk to the emotional status of those patients, resulting in the development of anxiety or depression disorders.
Limitations of the study The main limitation of the study is a small number of subjects. Moreover, the group was varied in terms of patients’ age and duration of time between SCA and the first examination. Consequently, statistical analyses and generalisation of observations are not possible and therefore the study should be treated as a preliminary exploration of this subject.
Conflict of interest None.
References 1. Kuisma M, Suominen P, Korpela R. Paediatric out-of-hospital cardiac arrest—–epidemiology and outcome. Resuscitation 1995;30:141—50. ˚ , B˚ 2. Engdahl J, Axelsson A ang A, Karlson BW, Herlitz J. The epidemiology of cardiac arrest in children and young adults. Resuscitation 2003;58:131—8. 3. Puranik R, Chow CK, Duflou JA, Kilborn MJ, McGuire MA. Sudden death in the young. Heart Rhythm 2005;2:1277—82. 4. Gilbert-Barness E, Barness LA. Pathogenesis of cardiac conduction disorders in children genetic and histopathologic aspects. Am J Med Genet A 2006;140(19):1993—2006. 5. Anderson V, Northam E, Hendy J, Wrennall J. Developmental neuropsychology. A clinical approach. Hove: Psychology Press; 2001. 6. Jennett B. Epidemiology, causes and prognosis of the vegetative state. In: Proceedings from European Conference on Brain Injure Rehabilitation—–Children and Adults. 1998.
50 7. Łucki W. Zestaw pr´ ob do badania proces´ ow poznawczych u pacjent´ ow z uszkodzeniami m´ ozgu. Warszawa: Polskie Towarzystwo Psychologiczne; 1995. 8. Cummins RO, Chamberlain DA, Abramson NS, et al. Recommended guidelines for uniform reporting of data from out-of-hospital cardiac arrest: the Utstein style. Ann Emerg Med 1991;20:861—74. 9. Allen JS, Tranel D, Bruss J, Damasio H. Correlations between regional brain volumes and memory performance in anoxia. J Clin Exp Neuropsychol 2006;28(4):457—76.
A. Maryniak et al. 10. Sauv´ e MJ, Walker JA, Massa SM, Winkle RA, Scheinman MM. Patterns of cognitive recovery in sudden cardiac arrest survivors: the pilot study. Heart Lung 1996;25:172—81. 11. O’Reilly SM, Grubb NR, O’Carroll RE. In-hospital cardiac arrest leads to chronic memory impairment. Resuscitation 2003;58:73—9. 12. Drysdale EE, Grubb NR, Fox KAA, O’Carroll RE. Chronicity of memory impairment in long-term our-of-hospital cardiac arrest survivors. Resuscitation 2000;47:27—32.
available at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/resuscitation
CLINICAL PAPER
Long-term cognitive outcome in teenage survivors of arrhythmic cardiac arrest夽 Agnieszka Maryniak a,∗, Alicja Bielawska a, Franciszek Walczak b, ekawek a, Łukasz Szumowski b, Katarzyna Bieganowska a, Joanna R˛ zbieta Szymaniak a, Maria Knecht a Monika Paszke a, El˙ a b
Children’s Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland Institute of Cardiology, ul. Alpejska 42, 04-628 Warsaw, Poland
Received 2 July 2007; received in revised form 5 September 2007; accepted 31 October 2007
KEYWORDS Sudden cardiac arrest; Ventricular arrhythmias; Neurocognitive function
夽
Summary Background: Sudden cardiac arrest (SCA) can be the first sign of ventricular arrhythmia in teenagers. Neurocognitive problems are common after successful resuscitation. We studied cognitive function in teenage survivors of SCA, including emotional status and coping ability. Method: Ten SCA survivors, aged 11—19 years, had neuropsychological tests within a few weeks of resuscitation. Awareness status, orientation, episodic and semantic memory, basic auditoryvisual functions, praxis and speech, short-term memory, ability to learn new verbal and visual material were assessed. These tests were repeated at about 6 months. Results: Eight patients had an initial assessment; one boy remained in a coma and one was making simple emotional contact, revealing intensified mixed aphasia and dyskinesia. Six patients had severe disturbances of memory, motor functions and praxis. After 6 months, four patients had no neurocognitive disturbance. Four patients had memory impairment making school education difficult. Two patients were totally dependent on caregivers. Because of the absence of symptoms before SCA, and amnesia relating to the SCA episodes, patients had problems accepting their heart problems and limitations resulting from it. Conclusion: Teenagers surviving SCA have significant neurcognitive and psychological problems. They need psychological care and guidance in understanding their condition. © 2007 Elsevier Ireland Ltd. All rights reserved.
A Spanish translated version of the summary of this article appears as Appendix in the final online version at doi:10.1016/j.resuscitation.2007.10.024. ∗ Corresponding author. E-mail address: [email protected] (A. Maryniak).
Teenagers make up only about 0.5% of sudden cardiac arrest (SCA) patients.1,2 SCA is caused by arrhythmias including the Wolff-Parkinson-White syndrome (WPW). Genetic causes include long-QT syndromes, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia and arrythmogenic right ventricular cardiomyopathy.3,4
0300-9572/$ — see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.resuscitation.2007.10.024
Long-term cognitive outcome in teenage survivors of arrhythmic CA Neurocognitive problems are common in survivors of cardiac arrest. The duration of brain hypoxaemia, the patient’s age and pre-existing medical problems are important determinants of neurocognitive outcome. Children may have greater neurocognitive dysfunction after SCA as the developing brain is especially sensitive to hypoxia.5,6 On the other hand, SCA in teenagers usually occurs in previously healthy individuals with no previous neurocognitive deficit. We have studied neurocognitive function shortly after resuscitation and at 6 months in teenage survivors of SCA, including emotional status and coping ability.
Material and methods Teenager SCA survivors hospitalised between 2003 and 2006, and who attended two specialist arrhythmia clinics treatment were studied. Subjects were referred for a cardiology assessment by the centres where initial resuscitation had taken place. The time between SCA to admission to our clinics and first neuropsychological tests varied from 5 days to 2 months. Information about the original SCA and resuscitation (bystander resuscitation, paramedics, hospital) was obtained from medical records. Awareness status, orientation, and depending on the patient’s condition, episodic and semantic memory, basic audio-visual functions, praxis and speech, short-term memory, ability to learn new verbal and visual material were assessed. The tests of higher cognitive processes developed by A.R. Łuria (Polish adaptation7 ) were used they enable determination of whether a patient suffers from disturbances with the features of combativeness, apraxis or aphasia. In addition the Auditory-Verbal Learning Test, Benton Visual Retention Test and Rey’s Complex Figure Test were used. These tests have Polish validation for a given age group. The patient’s general condition was classified using Cerebral Performance Categories.8 Scoring is on a five-point scale: 1 = full independence, ability to function at school or in a professional environment in a way which is appropriate for a given age, with possible slight neurological deficits. 2 = dysfunctions of moderate intensification which limit capability, but not prevent from self-care in everyday life and some professional activity. 3 = complete dependence on carers. 4 = vegetative state, coma. 5 = death. Testing was repeated at about 6 months. All the patients are still under observation (duration varying from 6 months
Table 1
1 2 3 4 5 6 7 8 9 10
47
to 4 years). Information about their school results, possible problems with emotional or social functions, and rehabilitation progress is still being collected.
Results The group consisted of eight boys and two girls, aged from 11 to 19 years (Table 1). Six of them were diagnosed with Wolff-Parkinson-White (WPW) syndrome and four of them with long-QT syndrome. One girl (patient 4) had a previous history of arrhythmias and was being investigated for this at the time of her SCA. SCA was the first sign of cardiac disease in the others. The patients with WPW were treated by radiofrequency ablation of their atrioventricular accessory pathway. The patients with long-QT syndrome had a cardioverter-defibrillator implanted.
Circumstances of cardiac arrest and resuscitation According to information provided by witnesses, three patients (patients 4,5,8) reported feeling unwell (weakness, vertigo, palpitation) just before SCA. In two of them (patients 4,8) cardiac arrest occurred while being transported by the family to hospital (including one case of cardiac arrest in the admission room). In four cases (patients 1,2,3,7) there are no data regarding subjective patients’ feelings and symptoms preceding cardiac arrest as these situations occurred outside the home witnessed by bystanders. Retrograde amnesia of events preceding cardiac arrest meant that patients themselves could not provider this information. In three boys (patients 6,9,10) cardiac arrest was sudden, after physical effort and these patients did not report feeling unwell. In a female patient in whom cardiac arrest occurred in hospital just as she arrived with her family, resuscitation was promptly initiated. In the remaining patients cardiac arrest was outside hospital. In four cases resuscitation was initiated by witnesses. In five cases resuscitation only started when an ambulance crew arrived on the scene. This group probably had a longer period of brain hypoxia.
Cognitive function Neurological disorders and/or significant deficits in cognitive functions (Table 2 shows detailed results) were observed
Patient characteristics Sex
Age (years, months)
Syndrome
Resuscitation
Treatment
1st exam
Time of observation
F M M F M M M M M M
14, 12, 17, 16, 19, 16, 19, 18, 11, 11,
Long-QT Long-QT Long-QT Long-QT WPW WPW WPW WPW WPW WPW
Bystander Paramedics Bystander Hospital Paramedics Paramedics Paramedics Bystander Bystander Paramedics
ICD ICD ICD ICD RF ablation RF ablation RF ablation RF ablation RF ablation RF ablation
3 weeks 2 weeks 4 weeks 6 days 10 days 2 weeks 7 weeks 8 days 2 weeks 8 weeks
6 months 7 months 9 months 4 years 18 months 12 months 2 years 18 months 12 months 4 years
9 3 9 0 1 8 5 8 4 1
48
A. Maryniak et al.
Table 2 Sex
1 2 3 4 5 6 7 8 9 10
F M M F M M M M M M
Results of first (1) and second (2) neuropsychological examinations Age (years, months)
14, 12, 17, 16, 19, 16, 19, 18, 11, 11,
9 3 9 0 1 8 5 8 4 1
CPC
Aphasia
Apraxia
AVLT
Rey’s CFT
Benton’s VTRT
1
2
1
2
1
2
1
2
1
2
1
2
2 1 1 2 2 2 3 2 2 4
2 1 1 1 2 2 3 1 1 3
No No No No Yes No Yes No Yes X
No No No No No No Yes No No X
No No No No Yes Yes X No No X
No No No No Yes No X No No X
—— + ++ ++ —— —— X + — X
— + ++ ++ — — X + — X
—— + ++ — — — X —— —— X
—— ++ ++ + — — X + — X
—— + + — — — X — — X
—— + + + — — X + — X
CPC, cerebral performance categories. X, examination not done. — —, result <10 centile; —, result >10—30 centile; +, result >30—70 centile; ++, result >70 centile.
in eight patients during the first examination after SCA. One boy (patient 10) remained in a coma (CPC 4), one (patient 7) was making simple emotional contact, revealing intensified mixed aphasia and dyskinesia which disabled independent movements (CPC 3). No disturbances of cognitive functions were observed in two boys (patients 2,3 — CPC 1). Memory, speech and praxis disturbances of different intensification were observed in the remaining six patients (patients 1,4,5,6,8,9 — CPC 2). Fresh memory disturbances (problems with memorising current events, new material, both visual and verbal) were predominant, as well as problems with semantic memory—–updating information learned before SCA. Two boys (patients 5,6) experienced problems regarding episodic memory, including facts about themselves and their family. Deficits in speech, found in three patients (patients 5,7,9), were manifested as semantic aphasia (problems updating words). Intensified disturbances of praxis (performing targeted complex movements) were observed in two patients (patients 5,6) and they limited patients’ independence regarding self-care such as dressing and eating. Patient 8 also manifested symptoms of the frontal syndrome, such as impulsiveness, curtailing the time spent in social contacts, no adequate emotional reactions and lack of criticism towards his own behaviour. At 6th month, in two patients (patients 4,8) the deficits previously observed had subsided and patients had fully recovered. Some improvement was seen in four patients (patients 1,5,6,9,) in terms of cognitive functions; however, these were still not characteristic for their age. These current deficits (mainly regarding memory) made school education and everyday life difficult. The boy in a coma (patient 10) during the first examination regained consciousness but was quadriplegic and mute. His emotional reactions were appropriate. He answered questions using his eyes showing good reception of stimuli and their correct understanding. These are signs of a locked-in syndrome. In the period between the first and the second examination the two patients (patients 7,10) with the most severe status underwent intensive rehabilitation. This rehabilitation is still continuing. Long-term follow-up, which in one case has lasted two years, in another four years, reveals slow improvement of their condition. Both patients are still
fully dependent on the constant care of other people. The remaining subjects have returned to the activities typical for their age and continue their education. Five patients (1,5,6,8,9) experienced subjective difficulties, feelings of decreased cognitive functions and their learning requires more effort and time, and their achievements are poorer than before SCA.
Emotional status Subjects did not manifest symptoms of increased anxiety or fear after SCA. This is probably connected with the fact that all of them experienced retrograde and anterograde amnesia from several hours to 2 days before SCA and for several days after regaining consciousness. The patients were told about their situation by their families and doctors, therefore they did not feel under threat. However, they experienced some disorientation, had problems with cognitive understanding of their condition, the necessity to perform a planned procedure, its character and meaning. Before SCA these patients had been unaware of their heart disease, had not experienced any previous symptoms and were considered to be healthy and thought they were healthy. Their condition after SCA including problems regarding range of movements, speech, also did not indicate, in their subjective view, a cardiology problem and was more frequently connected with trauma. Understanding the situation was especially difficult for six patients with fresh memory disturbances as they did not remember the advice and explanations they received in the early period after SCA, and their experience and feelings were contradictory to information given by doctors and family (e.g. a 16-yearold boy [patient 6] claimed he was in hospital due to his leg disease as he was suffering pain connected with the site of injection made during his radiofrequency ablation. He did not remember information about his heart disease and a procedure). In a control examination the group with WPW who had undergone radiofrequency ablation did not show any worrying symptoms regarding emotional functions. In some cases parents reported problems regarding their limitations (e.g.
Long-term cognitive outcome in teenage survivors of arrhythmic CA physical activity, sports, choice of further education or job). For example, one boy in this group (patient 8) underwent SCA in WPW syndrome 2 months before beginning studies at a fireman’s college. He had to change his educational and professional plans. It was very difficult for him and induced rebellion and potentially dangerous behaviour, such as intensive physical exercise to prove he was healthy. In the case of patients with an implanted cardioverterdefibrillator (ICD) the situation was different. The constant presence of a device resulted in limitations and experience of the ICD intervention (twice in one boy [patient3], once in the second [patient 2], while in the two remaining ones— –without shocks) constituted confirmation of disease and risk. Increased anxiety and concentration on body signals were observed in all these patients. Follow-up observation indicates gradual adaptation to disease.
Discussion We have analysed neurocognitive functions in teenage survivors of SCA. Most experienced significant neurocognitive dysfunctions during the first days and weeks following SCA. The problems observed were connected with physical skills, audio-visual function and speech, and different aspects of memory. At 6th month there was significant improvement in terms of speech, audio-visual function and movements; however, in some patients memory disturbances remained. Despite improvement since the initial test, memory skills were below normal for age. Memory deficits are common in SCA survivors. This may be due to the sensitivity of the hippocampus to hypoxia.9 These deficits are often permanent.10—12 This may have a significantly negative influence on the further development of teenagers undergoing education. Memory disturbance limits the possibilities of effective learning and decreases the level of school achievement. Two boys (patients 7,10) with severe neurological disturbances who have been followed-up for several years show very slow improvement. It is likely that they will always be reliant on carers. We do not have detailed information about the duration of cardiac arrest. It is likely that it was the longest in patients who had to wait for an ambulance crew to arrive and start resuscitation. This group includes two boys with permanent severe brain damage and two other patients with significant memory disturbances. Two out four patients who had immediate bystander resuscitation made a complete recovery. In most cases our patients do not have any conscious memories of their SCA. They did not feel or do not remember feeling fear, helplessness, they do not know what symptoms preceded their loss of consciousness. Their knowledge about what happened comes from other people. If they were ill previously, they are able to make connections between their current condition and known information about the disease. Teenagers who regain consciousness following SCA have more problems than adults in understanding the situation they are experiencing. Sudden cardiac arrest was the first symptom of the disease in almost all patients in the analysed group. Therefore, they had thought they were completely healthy and others had thought the same. For a young person
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cardiac disease is an abstraction which remains outside their experience, it is not a problem they meet among their peers, it is a problem connected with adults, with older people. Our patients and their families were not aware that a child may experience SCA. Therefore it is difficult for these patients to understand what really happened, what the reason and consequences for what happened are, and what the real risk to their lives was. On one hand, they do not feel appropriate fear, but on the other hand, it may hamper acceptance of the need for hospitalisation, any treatment (especially when ICD implantation is necessary) and limitations regarding their lifestyle. Our study indicates that these patients need psychological care. During the first weeks or months after SCA they need adequate description of their situation, such that does not provoke feelings of excessive danger but does present their current health status. Moreover, limitations due to memory disturbances and other cognitive functions should be considered. Information given in the early period may not be understood appropriately or permanently recorded in the memory, therefore, it may be necessary to repeat it several times. In some cases patients will need help coming to terms with their illness. In the cases of patients with an ICD, long-term regular psychological examinations are indicated as possible ICD interventions may be a risk to the emotional status of those patients, resulting in the development of anxiety or depression disorders.
Limitations of the study The main limitation of the study is a small number of subjects. Moreover, the group was varied in terms of patients’ age and duration of time between SCA and the first examination. Consequently, statistical analyses and generalisation of observations are not possible and therefore the study should be treated as a preliminary exploration of this subject.
Conflict of interest None.
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