Brief Communication
Stroke in a Case of Neonatal Lupus: An Uncommon Complication
Journal of Child Neurology 2014, Vol. 29(11) NP157-NP160 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0883073813513497 jcn.sagepub.com
Renu Suthar, MD1, Jitendra K. Sahu, MD, DM1, Manojkumar Rohit, MD, DM2, Niranjan K. Khandelwal, MD3, and Pratibha Singhi, MD1
Abstract Stroke is an extremely rare complication of congenital heart block in children. We report a 2-year-old girl with congenital complete heart block who presented with acute-onset right middle cerebral artery territory stroke. The congenital heart block was secondary to maternal lupus. Keywords congenital heart block, embolic, hemiparesis, lupus, stroke Received August 25, 2013. Received revised October 19, 2013. Accepted for publication October 28, 2013.
Congenital heart disease (cyanotic and acyanotic), valvular heart disease, myocarditis, and cardiac arrhythmia are wellknown risk factors for stroke in infants and young children; however, stroke as a complication of congenital heart block is uncommonly reported.1-5 Symptomatic congenital heart block usually presents as hydrops fetalis or heart failure in the neonate or exercise intolerance, syncope, fainting attacks in the older child.3,6 Longer pauses in congenital heart block can cause presyncope, syncope (classical Adams Stokes attacks), or even sudden cardiac death. We report a case of acute ischemic stroke in a girl with congenital heart block secondary to maternal lupus.
Case Report A 2-year-old developmentally normal girl presented with acute-onset left hemiparesis with left upper motor neuron facial nerve palsy noticed on waking up in the morning. There was no history of seizure, speech difficulty, aphasia, loss of consciousness, or gaze preference. There was no history of any preceding febrile illness, diarrhea, dehydration, or antecedent trauma. Antenatal history revealed that at 6 months of gestation, fetal bradycardia was detected and a diagnosis of congenital heart block was made. Maternal antinuclear antibodies, dsDNA, anti-Ro, and anti-La antibody were positive, though she was asymptomatic. The baby had persistent bradycardia since birth; however, she remained completely asymptomatic till now. There was no history of previous episodes of dizziness, fainting, mottling, bluish discoloration, or focal deficits. Parents did not notice any exercise intolerance or easy fatigability. She was
living with her parents; however; parents did not seek medical advice because she remained largely asymptomatic. On examination, her weight was 12 kg (at 50th centile) and height was 85 cm (at 25th centile). Her heart rate was varying between 50 and 60 beats per minute, with regular rhythm, blood pressure 100/60 mm Hg, respiratory rate was 24/minute, and grade 2 systolic murmur over the aortic area was recorded. There was no organomegaly, rash, or any neurocutaneous marker. Neurologically, she was conscious and comfortable, and her speech was normal. She had left upper motor neuron facial nerve palsy with dense left hemiplegia and normal sensory examination. Electrocardiography (ECG) showed complete (third degree) atrioventricular conduction block with narrow QRS complexes (0.06 seconds) (Figure 1). Holter monitoring showed a minimum ventricular rate of 34/minute during sleep and maximum 134/minute, narrow QRS complex suggestive of junctional escape rhythm. Echocardiography showed a normal-sized heart
1
Department of Paediatrics, Division of Pediatric Neurology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India 2 Department of Cardiology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India 3 Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India Corresponding Author: Pratibha Singhi, MD, Division of Pediatric Neurology, Advanced Pediatric Centre, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India. Email: [email protected]
Downloaded from jcn.sagepub.com at PURDUE UNIV LIBRARY TSS on May 23, 2015
NP158
Journal of Child Neurology 29(11)
Figure 1. Holter electrocardiography (ECG) of index patient with heart rate varying between 38/minute to 101/minute along with complete atrioventricular conduction block.
In view of complete atrioventricular block, a permanent dual-chamber ventricular pacemaker was implanted through transvenous route. There was no immediate postoperative complication and no recurrence of transient ischemic attack/stroke. According to her parents, she had become more active as compared to her previous physical abilities. Currently, she is 3.5 years old and she is in regular follow-up. Her right hemiparesis has improved with minimal persistent residual right-hand weakness. Her mother developed arthritis during follow-up and she was started on hydroxy-chloroquine.
Discussion Figure 2. T2-weighted axial magnetic resonance imaging (MRI) brain images (A) at level of lateral ventricles showing right-sided putamen and internal capsule infarct (open arrow). T2-weighted images at levels of basal cistern (B) showing T2 hyperintensity involving right cerebellar hemisphere suggestive of old ischemic infarct (black arrow).
with a patent foramen ovale with normal ejection fraction and there was no thrombus, valvular regurgitation, or hypokinetic segment. Brain magnetic resonance imaging (MRI) showed hypointensity involving the right putamen and internal capsule in T1-weighted images and corresponding hyperintensity in T2-weighted and fluid-attenuated inversion recovery images suggestive of acute infarct (Figure 2). In addition, MRI brain also showed old infarct in right cerebellum (Figure 2). Magnetic resonance angiography brain and neck vessel was normal. Her hemoglobin, platelet counts, serum cholesterol, iron studies, triglycerides, arterial lactate, serum homocysteine, antinuclear antibodies, and dsDNA were normal. Procoagulant workup (Factor V Leiden, lupus anticoagulant, anticardiolipin antibody, antithrombin III, protein C, and protein S levels) was normal.
This case merits attention for a variety of reasons. Transient cerebral ischemia with Stoke Adam syndrome is reported in congenital heart block, but cerebral infarcts are rarely reported as a complication of congenital heart block. Four cases of presumed embolic stroke have been reported in cases with congenital heart block either prior or subsequent to transvenous pacemaker implantation.1-3,5 Congenital heart block when diagnosed in utero presents with fetal hydrops in majority of cases; and when diagnosed later in life presents with features of low cardiac output and exertion intolerance.7 The index patient remained clinically asymptomatic prior to this episode even though she had severe bradycardia due to complete atrioventricular block. She presented with acuteonset ischemic stroke involving the right capsule-ganglionic area and presence of another old infarct in the right cerebellum suggests recurrent ischemic events in the index patient. Central nervous system complications reported in neonatal lupus includes macrocephaly, ischemic or hemorrhagic stroke, hydrocephalus, echogenic white matter, basal ganglia calcification, and central nervous system vasculopathy.8-10 Central nervous system vasculopathy involving the ganglion-thalamic
Downloaded from jcn.sagepub.com at PURDUE UNIV LIBRARY TSS on May 23, 2015
Suthar et al
NP159
region is reported in neonatal lupus with complete congenital heart block.11 Normal magnetic resonance angiography in index patient ruled out presence of vasculopathy. Presence of old infarct in the cerebellum also suggests involvement of vascular territory other than ganglion-thalamic vessels, so the possibility of cardioembolic stroke was more likely in the index patient. Presence of significant bradycardia and associated sluggish circulation might have precipitated formation of a cardiac thrombus, which could be responsible for recurrent cardioembolic stroke in the index patient. However, echocardiography was normal and a thrombus could not be identified. Congenital heart block is the most serious manifestation of neonatal lupus syndrome, in which maternal immunoglobulin IgG anti-Ro/SS-A autoantibodies crosses the placenta and injure an otherwise normally developing heart.12 The incidence of congenital heart block in an offspring of a mother with antiSSA/Ro antibodies is about 2%, whereas if the mother already had an affected child, the risk of congenital heart block in a subsequent pregnancy rises to 20%.13,14 Congenital heart block in the fetus is usually detected between 17 and 24 weeks of gestation.13 In addition, congenital heart block might be associated with fetal hydrops and stillbirth.15 The degree of heart block varies from first-degree block discovered accidentally with an ECG taken in postnatal life showing a prolonged PR interval to a complete atrioventricular block, which is most frequently recognized. Once established, complete congenital heart block is irreversible.16 Congenital heart block carries a significant morbidity and mortality (15%-30%) most often in utero or in the first few months of life: in fact, all recognized cases require a pacemaker insertion before reaching adulthood.13 Increased mortality is associated with fetal hydrops, endocardial fibroelastosis, and preterm birth
Stroke in a Case of Neonatal Lupus: An Uncommon Complication
Journal of Child Neurology 2014, Vol. 29(11) NP157-NP160 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0883073813513497 jcn.sagepub.com
Renu Suthar, MD1, Jitendra K. Sahu, MD, DM1, Manojkumar Rohit, MD, DM2, Niranjan K. Khandelwal, MD3, and Pratibha Singhi, MD1
Abstract Stroke is an extremely rare complication of congenital heart block in children. We report a 2-year-old girl with congenital complete heart block who presented with acute-onset right middle cerebral artery territory stroke. The congenital heart block was secondary to maternal lupus. Keywords congenital heart block, embolic, hemiparesis, lupus, stroke Received August 25, 2013. Received revised October 19, 2013. Accepted for publication October 28, 2013.
Congenital heart disease (cyanotic and acyanotic), valvular heart disease, myocarditis, and cardiac arrhythmia are wellknown risk factors for stroke in infants and young children; however, stroke as a complication of congenital heart block is uncommonly reported.1-5 Symptomatic congenital heart block usually presents as hydrops fetalis or heart failure in the neonate or exercise intolerance, syncope, fainting attacks in the older child.3,6 Longer pauses in congenital heart block can cause presyncope, syncope (classical Adams Stokes attacks), or even sudden cardiac death. We report a case of acute ischemic stroke in a girl with congenital heart block secondary to maternal lupus.
Case Report A 2-year-old developmentally normal girl presented with acute-onset left hemiparesis with left upper motor neuron facial nerve palsy noticed on waking up in the morning. There was no history of seizure, speech difficulty, aphasia, loss of consciousness, or gaze preference. There was no history of any preceding febrile illness, diarrhea, dehydration, or antecedent trauma. Antenatal history revealed that at 6 months of gestation, fetal bradycardia was detected and a diagnosis of congenital heart block was made. Maternal antinuclear antibodies, dsDNA, anti-Ro, and anti-La antibody were positive, though she was asymptomatic. The baby had persistent bradycardia since birth; however, she remained completely asymptomatic till now. There was no history of previous episodes of dizziness, fainting, mottling, bluish discoloration, or focal deficits. Parents did not notice any exercise intolerance or easy fatigability. She was
living with her parents; however; parents did not seek medical advice because she remained largely asymptomatic. On examination, her weight was 12 kg (at 50th centile) and height was 85 cm (at 25th centile). Her heart rate was varying between 50 and 60 beats per minute, with regular rhythm, blood pressure 100/60 mm Hg, respiratory rate was 24/minute, and grade 2 systolic murmur over the aortic area was recorded. There was no organomegaly, rash, or any neurocutaneous marker. Neurologically, she was conscious and comfortable, and her speech was normal. She had left upper motor neuron facial nerve palsy with dense left hemiplegia and normal sensory examination. Electrocardiography (ECG) showed complete (third degree) atrioventricular conduction block with narrow QRS complexes (0.06 seconds) (Figure 1). Holter monitoring showed a minimum ventricular rate of 34/minute during sleep and maximum 134/minute, narrow QRS complex suggestive of junctional escape rhythm. Echocardiography showed a normal-sized heart
1
Department of Paediatrics, Division of Pediatric Neurology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India 2 Department of Cardiology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India 3 Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India Corresponding Author: Pratibha Singhi, MD, Division of Pediatric Neurology, Advanced Pediatric Centre, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India. Email: [email protected]
Downloaded from jcn.sagepub.com at PURDUE UNIV LIBRARY TSS on May 23, 2015
NP158
Journal of Child Neurology 29(11)
Figure 1. Holter electrocardiography (ECG) of index patient with heart rate varying between 38/minute to 101/minute along with complete atrioventricular conduction block.
In view of complete atrioventricular block, a permanent dual-chamber ventricular pacemaker was implanted through transvenous route. There was no immediate postoperative complication and no recurrence of transient ischemic attack/stroke. According to her parents, she had become more active as compared to her previous physical abilities. Currently, she is 3.5 years old and she is in regular follow-up. Her right hemiparesis has improved with minimal persistent residual right-hand weakness. Her mother developed arthritis during follow-up and she was started on hydroxy-chloroquine.
Discussion Figure 2. T2-weighted axial magnetic resonance imaging (MRI) brain images (A) at level of lateral ventricles showing right-sided putamen and internal capsule infarct (open arrow). T2-weighted images at levels of basal cistern (B) showing T2 hyperintensity involving right cerebellar hemisphere suggestive of old ischemic infarct (black arrow).
with a patent foramen ovale with normal ejection fraction and there was no thrombus, valvular regurgitation, or hypokinetic segment. Brain magnetic resonance imaging (MRI) showed hypointensity involving the right putamen and internal capsule in T1-weighted images and corresponding hyperintensity in T2-weighted and fluid-attenuated inversion recovery images suggestive of acute infarct (Figure 2). In addition, MRI brain also showed old infarct in right cerebellum (Figure 2). Magnetic resonance angiography brain and neck vessel was normal. Her hemoglobin, platelet counts, serum cholesterol, iron studies, triglycerides, arterial lactate, serum homocysteine, antinuclear antibodies, and dsDNA were normal. Procoagulant workup (Factor V Leiden, lupus anticoagulant, anticardiolipin antibody, antithrombin III, protein C, and protein S levels) was normal.
This case merits attention for a variety of reasons. Transient cerebral ischemia with Stoke Adam syndrome is reported in congenital heart block, but cerebral infarcts are rarely reported as a complication of congenital heart block. Four cases of presumed embolic stroke have been reported in cases with congenital heart block either prior or subsequent to transvenous pacemaker implantation.1-3,5 Congenital heart block when diagnosed in utero presents with fetal hydrops in majority of cases; and when diagnosed later in life presents with features of low cardiac output and exertion intolerance.7 The index patient remained clinically asymptomatic prior to this episode even though she had severe bradycardia due to complete atrioventricular block. She presented with acuteonset ischemic stroke involving the right capsule-ganglionic area and presence of another old infarct in the right cerebellum suggests recurrent ischemic events in the index patient. Central nervous system complications reported in neonatal lupus includes macrocephaly, ischemic or hemorrhagic stroke, hydrocephalus, echogenic white matter, basal ganglia calcification, and central nervous system vasculopathy.8-10 Central nervous system vasculopathy involving the ganglion-thalamic
Downloaded from jcn.sagepub.com at PURDUE UNIV LIBRARY TSS on May 23, 2015
Suthar et al
NP159
region is reported in neonatal lupus with complete congenital heart block.11 Normal magnetic resonance angiography in index patient ruled out presence of vasculopathy. Presence of old infarct in the cerebellum also suggests involvement of vascular territory other than ganglion-thalamic vessels, so the possibility of cardioembolic stroke was more likely in the index patient. Presence of significant bradycardia and associated sluggish circulation might have precipitated formation of a cardiac thrombus, which could be responsible for recurrent cardioembolic stroke in the index patient. However, echocardiography was normal and a thrombus could not be identified. Congenital heart block is the most serious manifestation of neonatal lupus syndrome, in which maternal immunoglobulin IgG anti-Ro/SS-A autoantibodies crosses the placenta and injure an otherwise normally developing heart.12 The incidence of congenital heart block in an offspring of a mother with antiSSA/Ro antibodies is about 2%, whereas if the mother already had an affected child, the risk of congenital heart block in a subsequent pregnancy rises to 20%.13,14 Congenital heart block in the fetus is usually detected between 17 and 24 weeks of gestation.13 In addition, congenital heart block might be associated with fetal hydrops and stillbirth.15 The degree of heart block varies from first-degree block discovered accidentally with an ECG taken in postnatal life showing a prolonged PR interval to a complete atrioventricular block, which is most frequently recognized. Once established, complete congenital heart block is irreversible.16 Congenital heart block carries a significant morbidity and mortality (15%-30%) most often in utero or in the first few months of life: in fact, all recognized cases require a pacemaker insertion before reaching adulthood.13 Increased mortality is associated with fetal hydrops, endocardial fibroelastosis, and preterm birth
