General Child Neurology
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Congenital heart disease: neurologic complications
- Updated 03.29.2024
- Released 07.12.1994
- Expires For CME 03.29.2027
Congenital heart disease: neurologic complications
Introduction
Overview
Congenital heart defects are the most common birth defects with an estimated prevalence of more than 8 to10 per 1000 live births in the United States, and they account for about 10% of all birth defects worldwide. Acute and chronic neurologic complications are not uncommon in children born with congenital heart disease: stroke, seizures, epilepsy, hemorrhage, infections and cognitive deficits. Data on developmental outcomes, mortality, and morbidity have been rapidly updated as surgical techniques improve. This article reviews the current literature and describes the different types of congenital heart defects, including genetic and environmental factors, and their associated neurologic disorders/complications. Methods for management, treatment, and prevention of the most common types of congenital heart disease are also provided.
Key points
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• Children born with congenital, or acquired, heart disease experience adverse neurologic complications in up to 25% of cases. | |
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• Changes in brain development can occur as early as the fetal period in severe heart defects, eg, TGA and HLHS, due to deficiencies in oxygen and nutrients delivered to the brain resulting in worse neurologic outcomes in patients with congenital heart diseases compared to their unaffected peers. | |
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• With the growing success of complex surgical procedures that correct congenital heart defects, infants who might have previously died are now surviving into adulthood. | |
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• Alongside improved survival rates, advancements in critical care and neuroprotective strategies and technology have altered the neurodevelopmental outcomes seen in congenital heart disease survivors. Application of noninvasive diagnostic tools, such as MRI, MRA, CT, microarray analysis, and cranial ultrasound studies in neonates, have allowed us to diagnose vascular lesions that in previous years may have been silent. |
Historical note and terminology
Aggressive surgical correction of congenital heart disease had been proposed as the future treatment of choice years before the first open heart surgery in children in 1951. Cardiopulmonary bypass with hypothermia techniques soon followed in 1953. As more difficult cases were taken to surgery and as survival rates improved, more neurologic complications were noted. It has been shown that therapeutic hypothermia, now a standard procedure in neonatal hypoxic-ischemic encephalopathy, can be applied to patients with severe congenital heart disease and anoxic injury (05). Advances in technology have altered the types of neurologic complications that are seen in survivors of congenital heart disease who would likely have died decades ago.
Clinical manifestations
Presentation and course
Table 1. Clinical Manifestations of Congenital Heart Disease
Neurologic complications of congenital heart disease | Clinical manifestations |
Stroke | • Focal hemi- or monoparesis |
Cerebral abscess | • Symptoms depend on the specific location of the abscess in the brain. |
Acute hypoxic episodes or “TET” spells | • Attacks tend to occur shortly after the child wakes up. |
Chronic hypoxia | • Mild symptoms: decreased short-term memory and difficulties with complex learning tasks. |
Bacterial endocarditis | • Fever |
Cerebral hemorrhage | • Headache |
Prognosis and complications
Up to 25% of children with congenital heart disease experience neurologic complications (26). Neonates with congenital heart disease demonstrate different neurobehavioral performance (attention, regulation, asymmetry, increased nonoptimal reflexes, lethargy, and need for handling) than typically developing neonates (18). Eighty-five percent of infants born with congenital heart disease reach adulthood (43). A meta-analysis done in 2012 estimates that the prevalence of congenital heart disease in the adult population is 3,000 per million (44). Infants with congenital heart disease have alterations in brain size at 3 months of age; however, their brain growth is similar to that of healthy term infants. The primary predictor of brain growth in the congenital heart disease population is somatic growth, which highlights the importance of optimal weight gain (35). Von Rhein and colleagues studied structural brain lesions and neurocognitive impairment in adolescents with congenital heart diseases who underwent cardiac surgery (45). In this study, 21% of the patients in the population had white matter abnormalities and volume loss on MRI most consistent with a hypoxic-ischemic brain injury. Although none of the patients in the study had cerebral palsy, 34% of the patients had mild to moderate motor deficits. Additionally, total mean IQ was lower in the studied population than in the control; the patients with congenital heart disease had an IQ score of 105 +/- 15.8 whereas the control population had an IQ score of 113 +/-10.4 p = 0.009. Also, patients with congenital heart disease and brain lesions scored significantly lower in all assessed neurodevelopmental domains, especially motor performance, visual perception, and visuomotor integration when compared to those patients with congenital heart disease and no brain lesions. A study by The Colorado Congenital Heart Disease Surveillance System (COCHD) to investigate the prevalence of mental health illness among adolescents and adults with congenital heart disease revealed that 33% of adults and 20% of adolescents with congenital heart disease are affected by mental illness. Anxiety and mood disorders were greater than three times more likely in patients with severe congenital heart disease (requiring at least two cardiac procedures) compared to patients with simple congenital heart disease. Overall, developmental disorder was the most common mental illness diagnosis affecting adolescents, whereas adults with congenital heart disease are at increased risk of heart failure and atrial arrhythmias, which are associated with worse executive function, cognitive impairments, and dementia (21).
Clinical vignette
Case 1. During her 20th week of pregnancy, a 33-year-old woman obtained a fetal ultrasound that showed a congenital heart malformation with suspected tetralogy of Fallot. Genetic evaluation revealed a deletion in the q11.2 region of 1 chromosome of the pair of chromosomes 22, consistent with DiGeorge syndrome. A child born with a ventricular septal defect was confirmed via echocardiography to have tetralogy of Fallot. Three days following uncomplicated vaginal vertex delivery, the neonate presented a sudden onset of weakness of the right arm and lower right side of the face, cortical thumbing, and persistent fisting. Deep tendon reflexes were decreased in the right biceps and right patella. On day 4 of life, the child exhibited episodic posturing of the right arm, with tonic extension and clonic activity (29).
A CT scan of the child’s head showed a hypodense, wedge-shaped region in the left middle cerebral artery distribution. There was no incidence of hemorrhage. An EEG obtained in the neonatal intensive care unit demonstrated decreased amplitude over the left hemisphere. Spike-wave discharges were noted over the left central region. MRA revealed thrombotic occlusion of the left middle cerebral artery. Following these tests, phenobarbital was administered.
Case 2. A 2020 case report by Lakhani and colleagues describes the case of a 13-year-old boy with known uncorrected tetralogy of Fallot (TOF) who presented to a cardiac center in Pakistan with shortness of breath, high-grave fever, headache, and two episodes of generalized tonic-clonic seizures with altered consciousness for three days (24). On general physical examination he was alert and oriented with no focal neurologic deficits. There was digital clubbing and cyanosis, with varying oxygen saturation from 85% to 90%. Infective endocarditis was suspected, but there were no peripheral signs. Cardiac examination revealed a displaced PMI to the left 5th intercostal space within the midclavicular line, left parasternal heave, and a harsh systolic ejection murmur of grade III/VI audible at the left sternal border and accentuated with inspiration. Labs were significant for polycythemia (hematocrit of 44.7%), leukocytosis with a neutrophilic predominance, and a positive blood culture for Escherichia coli. Echocardiogram revealed all components of uncorrected TOF with no evidence of endocarditis. CT scan of the brain confirmed diagnosis of brain abscess with a small, well-defined, ring-enhancing hypodense lesion in the right parieto-occipital region with surrounding edema. He was managed conservatively with intravenous antibiotics (vancomycin and meropenem), acetaminophen, and anticonvulsant (sodium valproate) for two weeks. Repeat imaging showed regression of in size of abscess, and repeat blood culture showed no growth. He was discharged home on two weeks of oral antibiotics (cefotaxime and metronidazole).
Biological basis
Etiology and pathogenesis
Congenital heart disease can be caused by a number of factors ranging from inherited genetic mutations or spontaneous mutations to environmental factors. The most common genetic factors that lead to heart abnormalities are outlined in Table 2 (17):
Table 2. Genetic Factors Leading to Heart Abnormalities
|
Genetic factors |
Diseases |
Most common congenital heart defects |
|
Abnormal chromosome structure (42) | ||
|
Trisomy 21 |
Down syndrome |
AVSD, VSD, ASD, PDA |
|
Trisomy 18 |
Edward syndrome |
ASD, VSD, PDA, TOF |
|
Trisomy 13 |
Patau syndrome |
ASD, VSD, PDA |
|
22q11.2 deletion |
DiGeorge syndrome |
TOF, IAA, TA, VSD |
|
Deletion from chromosome |
Williams syndrome |
Supravalvular AS, PPS |
|
Single X chromosome |
Turner syndrome |
Coarctation of Aorta, AS, BAV |
|
Gene mutations (42) | ||
|
Affected FBN1 gene |
Marfan syndrome |
Aortic aneurysm, MVP, dilated PA |
|
Mutations HRAS gene |
Costello syndrome |
HCM |
|
TBX5 gene |
Holt Oram syndrome |
ASD, VSD, PDA |
|
EVC gene |
Ellis Van Creveld syndrome |
ASD, common atrium |
|
KRAS, BRAF, PTPN11, RAF1, and SOS1 genes |
Noonan syndrome |
PS, ASD, TOF, VSD, PDA, HCM |
|
CHD7 gene |
CHARGE syndrome |
ASD, VSD, TOF |
|
KMT2D, KDM6A |
Kabuki syndrome |
ASD, VSD, TOF, Coarctation of Aorta, BAV, TGA, HLHS |
|
JAGGED1, NOTCH2 |
Alagille syndrome |
PPS, PS, TOF |
Congenital heart disease may be caused by Mendelian mutations, de novo mutations, noncoding mutations, copy number variants (SNV), translocations, or single nucleotide polymorphisms (SNP) (23; 16). There are two paradigms that attempt to explain the various mutations that lead to congenital heart disease. The direct convergence model theorizes that different risk factors impact the same genes and molecules. In the functional convergence model, congenital heart disease is caused by various risk factors, both genetic and environmental, that affect various molecules involved in a network of heart development. Through studies conducted on both human and animal models, Lage and colleagues discovered that the functional convergence model best explains the development of congenital heart disease. The researchers concluded that there was a lack of direct convergence between risk and responder datasets; more interestingly, however, they discovered functional convergence between thousands of risk factors. Although the genetic and environmental factors that cause congenital heart disease participate in many molecular pathways, they participate in a larger protein interaction network that drives the development of cardiac structures (23). At this time, we look forward to the results of functional genomic studies using single-cell RNA-sequencing, ChIP-sequencing, and ATAC-sequencing to reveal complex intrinsic genetic networks with specific cardiac lineages in early heart development (14).
Environmental factors include the following (06; 17; 42):
Table 3. Environmental Factors Leading to Heart Abnormalities
|
Environmental factors |
Specific examples |
|
Infection |
• Rubella |
|
Teratogens |
• Retinoic acid |
|
Maternal diseases |
• Diabetes |
|
Air pollution |
• Nitrogen oxides |
The most common heart defects associated with congenital heart disease are outlined in Table 4 (American Heart Association):
Table 4. Heart Defects Most Commonly Found in Congenital Heart Disease Patients
|
Most common heart defects associated with CHD |
Incidence of babies born with CHD born with these heart defects |
|
Ventricular septal defects |
14% to 16% |
Of these heart defects, some are more likely to cause neurologic complications than others. The incidence of neurologic abnormalities in various types of congenital heart disease is outlined in Table 5 (31):
Table 5. Incidence of Neurologic Abnormalities Associated with Specific Congenital Heart Defects
|
Heart defect |
Incidence of neurologic abnormalities (%) |
|
Hypoplastic left-sided heart syndrome |
29.0% |
The heart defects mentioned above can be divided into three categories: (1) left-to-right shunts, (2) obstructive lesions, and (3) those defects that cause cyanotic heart disease.
Each of the three categories and certain caveats are further outlined in Tables 6, 7, and 8. In addition, the pathogenesis and pathophysiology are included (31).
Table 6. Neurologic Impairments Associated with Left-to-Right Shunts
|
Heart defects |
Mechanism of injury |
Pathogenesis and pathophysiology |
|
Atrial septal defects |
Cerebral embolus due to: - or - • bacterial endocarditis (most common in ventricular septal defects and patent ductus arteriosus) |
• Shunt reversal causes direct communication between right side of the heart and systemic circulation. Flow reversal puts patient at a greater risk for cerebral embolus. • Vegetations of ventricular septal defects occur on the right side of the heart, making neurologic complications rare. • Vegetations of a patent ductus arteriosus can occur on the pulmonary artery side and possibly extend to the aorta. |
|
Ventricular septal defects | ||
|
Patent ductus arteriosus |
Table 7. Neurologic Impairments Associated with Obstructive Lesions
|
Heart defects |
Mechanism of injury |
Pathogenesis and pathophysiology |
|
Aortic stenosis Pulmonary stenosis Coarctation of aorta |
• All three lesions can cause bacterial endocarditis and subsequent cerebral vascular or peripheral embolization. • Aortic stenosis can cause cerebral hypoxia. • Coarctation of the aorta can cause intracranial arterial aneurysms. |
• Cerebral hypoxia is caused by diminished blood flow from decreased cardiac output. Aortic stenosis can reduce coronary artery flow, leading to an arrhythmia such as ventricular tachycardia or fibrillation. • Intracranial aneurysms from coarctation are particularly located in the anterior communication artery in the circle of Willis. |
Table 8. Neurologic Impairments Associated with Cyanotic Congenital Heart Disease
|
Heart defects |
Mechanism of injury |
Pathogenesis and pathophysiology |
|
Transposition of great arteries Tetralogy of Fallot Truncus arteriosus |
• Brain abscess. • Cerebrovascular accident. • Tetralogy of Fallot could present with risk of acute hypoxic episode or TET spells. |
• Any peripheral infections can cause a cerebrovascular accident or brain abscess because these particular heart defects allow a direct connection between systemic venous blood passing into the heart and cerebral circulation, without the lungs acting as an intervening filter. • Brain abscess (lack of pulmonary phagocytic filter of systemic bacteria) • Cerebral infarcts are marked by vascular occlusions, usually in the middle cerebral artery. Arterial thrombi are less common than venous thrombi. • Acute hypoxic episodes or TET spells result from a sudden increase in infundibular stenosis. This stenosis then increases the flow of hypoxic blood from the right ventricle through the ventricular septal defect to the aorta and into cerebral circulation. |
Epidemiology
In 2003, more than 25,000 cardiovascular operations for congenital cardiovascular defects were performed on children younger than 20 years of age (40). Cardiothoracic surgeons began operating on infants with congenital heart disease about 50 years ago, and since then mortality of this population has improved from 95% to 5% (09). Inpatient mortality rate after all types of cardiac surgery was 4.8%. Mortality risk varies for different defect types (08). The prevalence of congenital heart disease in the adult population is 3000 per million (44). The types of surgeries to correct congenital heart defects are outlined in Table 9 (33). Table 10 describes the prevention of congenital heart disease.
Table 9. Surgery to Correct Congenital Heart Defects
Total surgeries | Mortality rate | |
Total surgeries for congenital heart disease | 25,831 | 4.8% |
Prevention
Table 10. Prevention of Congenital Heart Disease
|
Method of prevention |
Risk factors |
Notes |
|
Correct underlying heart defect |
Aortic clamping release can increase risk of microemboli. |
Surgical techniques must incorporate selective antegrade or retrograde cerebral perfusion techniques to alleviate period of cerebral ischemia (13). |
|
Avoid intraoperative complications and iatrogenic problems |
• Cardiopulmonary bypass • Regional cerebral perfusion (30) | |
|
Screen microemboli |
Microemboli detected in carotid arteries can help prevent cerebral lesions (34). | |
|
Genetic screening |
Detecting a heart malformation in utero can lead to planning delivery in a center with high level neonatal care. | |
|
Iron supplementation |
Anemia can increase risk of heart problems and chronic hypoxia. | |
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Folate and B vitamin supplementation in pregnant women (40) | ||
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Physical exercise, healthy diet and weight, controlling lipid, blood pressure, and glucose levels, and cessation or not smoking |
Maintenance can prevent cardiovascular disease (33) |
Differential diagnosis
The clinical signs of a focal neurologic deficit or altered mental status with or without seizures will lead the astute clinician immediately to a workup for stroke, abscess, or hemorrhage in a patient with congenital heart disease. Certain illnesses may masquerade as stroke, hemorrhage, or abscess, and should be noted (Table 11).
Table 11. Common Illnesses That Masquerade As Stroke, Hemorrhage, or Abscess
|
Illness |
Subtypes |
|
Seizure |
With or without Todd paralysis |
|
Migraine |
Hemiplegic, ophthalmoplegic, confusion state |
|
Diabetes mellitus with ketoacidosis |
-- |
|
Lipoprotein disorders |
-- |
|
Hypoglycemia |
-- |
|
Trauma or nonaccidental trauma |
-- |
|
Postviral acute demyelinating encephalomyelitis |
-- |
|
Depression or conversion reaction |
-- |
|
Drug reaction |
-- |
In the younger pediatric age group, certain illnesses appear to have central nervous system origin and may occur in congenital heart disease patients (Table 12) (27).
Table 12. Illnesses of CNS Origin That Most Commonly Occur In Congenital Heart Disease
Common illnesses of CNS origin
|
• Paroxysmal vertigo |
Diagnostic workup
Congenital heart disease is usually not detected well with routine newborn examination, mostly because babies with congenital heart disease do not show signs of their disease at the exam. Although there has been much debate and discussion on using pulse oximetry to detect or screen for cyanotic heart disease (especially transposition of the great arteries, TGA), data suggest that even newborns with TGA can have O2 saturation levels higher than 96%, making this a very impractical screening mechanism (37).
In children confirmed to have congenital heart disease via echocardiography and electrocardiography, testing should be done to evaluate potential neurologic complications (19). Additional diagnostic tools are displayed in Table 13.
Table 13. Diagnostic Tools to Evaluate Neurologic Impairments in Congenital Heart Disease Patients
|
Test |
When to administer |
Description |
|
Cranial MRI and CT (28) |
Initial evaluation; workup planned based on results |
Evaluates for stroke and abscess |
|
Cranial ultrasound (11) |
Utilized with MRI or CT |
Detection of brain abnormalities |
|
MRA |
After MRI or CT |
Noninvasive; visualizes intracranial vasculature |
|
Conventional arteriography |
MRA normal or not diagnostic (arterial dissection or vasculitis) |
Diagnosis of arteriovenous malformations or aneurysms |
|
SPECT |
CT or MRI appear normal |
Detection of abnormal blood flow |
Based on the results of these studies, the workup for stroke and other diseases in the differential of stroke can be planned (07). MRA is a noninvasive method of visualizing the intracranial vasculature, and although conventional angiography is still the “gold standard,” MRA can largely replace invasive methods in the evaluation of ill children.
Table 14. ICD Codes Applicable for Neurologic Complications of Congenital Heart Disease [In Progress]
|
ICD-9 |
ICD-10 | |
|
Acute and subacute bacterial endocarditis |
421.0 |
I33.0 |
Management
Arrhythmia is the main reason for the hospitalization of adults with congenital heart disease and is an increasingly frequent cause of morbidity and mortality. Due to the additive effects of hypoxia, patients with congenital heart disease are susceptible to malnutrition and growth failure. Cyanotic patients with pulmonary hypertension are most severely affected (32). Care must be given to the nutritional needs of these children.
Because patients with cyanotic congenital heart disease are at a greater risk of cerebrovascular accident resulting from iron deficiency, administration of one tablet of ferrous sulfate (or gluconate) is recommended. Hemoglobin should be rechecked within 7 to 10 days. If red cell count is drastically increased, iron should be discontinued (12).
Outcomes
Table 15. Nonsurgical complications
|
Cause |
Neurologic complication |
|
Progressive hemoconcentration in high 60s to low 70s |
Cerebrovascular accident secondary to embolic phenomenon or intrinsic vascular occlusion (31) |
|
Untreated atrial defect |
Paradoxical emboli routed to the brain to cause potential neurologic sequelae |
|
Moyamoya disease |
Seizures and strokes months or years after CHD repair |
Phrenic nerve injury also may occur in 5% of congenital heart disease repairs (32).
Table 16. Neurologic complications from cardiac surgery
|
Corrective surgery |
Mechanism of injury |
Risk factors |
Clinical manifestations |
|
Coarctation of aorta (31) |
Spinal cord damage |
• Children with fewer collateral vessels • Long periods of aortic occlusion • Degree of compromise to the circulation of the spinal cord |
Can range from anterior spinal artery syndrome to complete paraplegia |
|
Cardiopulmonary bypass (22) |
Micro/macro emboli Paradoxical embolism |
• Length of time on bypass |
Postoperative stroke |
|
Shunts, prosthetic valves, conduits (31) |
Infective endocarditis |
• Susceptible cardiac or vascular substrate • Source of bacteremia |
Postoperative stroke |
|
Ductus arteriosus repair |
Horner syndrome Vocal cord paralysis | ||
|
Scoliosis repair (38) |
High risk of reoperation |
Given the high prevalence of cerebral desaturation during corrective cardiac surgeries, postoperative neurologic complications have been investigated with use of cerebral oximetry (intervention to monitor and correct desaturation). Although there are no significant differences observed for postoperative delirium and postoperative strokes, cerebral oximetry was associated with a lower incidence of postoperative cognitive decline up to three months after cardiac surgery when compared with control (no cerebral oximetry) (10).
Special considerations
Pregnancy
The benefits and risks of contraception and pregnancy in females diagnosed with congenital heart disease should be discussed with specialists in adult congenital heart disease and obstetrics and gynecology. There are certain types of contraceptives that can be hazardous to a woman’s health, causing thromboembolism or fluid retention (46). Postoperative females of childbearing age with corrected or uncorrected congenital heart defect should be aware of the hemodynamic burden of pregnancy that increases the risk of cardiovascular complications including strokes. Labor in the lateral decubitus position minimizes the hemodynamic fluctuations correlated with major uterine contractions in the supine position (01). Because thromboembolism is likely to increase during the postpartum period, patients with lesions susceptible to paradoxical embolization require meticulous leg care, use of elastic support stockings, and early ambulation (36).
Anesthesia
Medications used can have neuromuscular side effects, thereby mimicking neurologic events. Anesthetic medications should be included in the differential diagnosis of adverse events (04).
Media
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Contributors
All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.
Authors
-
Bernard L Maria MD
Dr. Maria of Thomas Jefferson University has no relevant financial relationships to disclose.
See Profile -
Kara Gay-Simon MD
Dr. Gay-Simon of Goryeb Children's Hospital has no relevant financial relationships to disclose.
See Profile
Former Authors
- Richard Young MD (original author), Kenton R Holden MD, Laura A Hatfield MD, Anne M Comi MD, Gily Raz BA BS, Asiya Khan Shakir BA BS, and Melissa A Eppinger BA
Patient Profile
- Age range of presentation
-
- 0 month to 65+ years
- Sex preponderance
-
- male=female
- Heredity
-
- Stroke itself is not hereditary. However, the prevalence of some genetic diseases--the most common of which is atrial fibrillation--are major risk factors for stroke (Baird 2010).
- Population groups selectively affected
-
- none selectively affected
- Occupation groups selectively affected
-
- none selectively affected
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