Congenital heart disease: neurologic complications

General Child Neurology

Updated 05.21.2025
Released 07.12.1994
Expires For CME 05.21.2028

Congenital heart disease: neurologic complications

Authors: Lindsey McPhillips DO, Kara Gay-Simon MD, Bernard L Maria MD

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Editor: Bernard L Maria MD

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Congenital heart disease: neurologic complications

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Introduction

Overview

Congenital heart disease is one of the most common birth defects worldwide, affecting 8 to 10 in every 1000 live births. Although approximately 50% of these cases will require surgical intervention in their lifetime, 25% are critical and will require life-saving measures within the first year of life (06). In the last 50 years, there have been significant advancements involving diagnostic testing, medical management, and surgical techniques that have greatly improved both the quality of life and life expectancy for this patient population. The physical, emotional, and financial burdens from congenital heart disease, however, are extensive due to the multitude of co-morbidities and complications associated with the primary diagnosis and medical care. Neurologic diseases, such as strokes, seizures, and neurodevelopmental disorders, are among the leading complications seen in patients with congenital heart disease. Great efforts are being made in the prevention, detection, and management of these neurologic sequelae. In this article, the authors provide an overview of congenital heart disease, discuss associated neurologic complications, review current neuroprotective strategies, and review diagnostic tools and management strategies for the treatment of these neurologic complications.

Key points

	• Seizures, strokes, and neurodevelopmental disabilities are among the leading neurologic complications in patients with congenital heart disease.
	• The onset for neurologic sequelae can occur as early as fetal life due to impaired cerebral blood flow and oxygen delivery and extend into adulthood, with the greatest risk being in the perioperative periods.
	• Neurologic complications can lead to short- or long-term developmental delays, disorders, or disabilities, such as motor deficits, speech/language deficits, feeding difficulties, cognitive impairments, behavioral and psychosocial issues, and executive decision impairments.
	• Neuroprotective strategies are targeted at improving timing of diagnosis, optimizing hemodynamic monitoring, improving cardiopulmonary bypass techniques, and reducing neuroinflammation.

Historical note and terminology

Congenital heart disease includes any structural heart abnormality present at birth. Table 1 lists the global prevalence of various congenital heart disease subtypes. These subtypes are often classified as mild, moderate, or severe based on complexity as defined in Table 2 (29). The most common types of congenital heart disease are mild forms, such as ventricular septal defects, atrial septal defects, and patent ductus arteriosus. Critical congenital heart disease comprises 25% of all cases and includes tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome. Ultrasonography is the diagnostic modality most often used in cardiology to diagnose congenital heart disease. Although ultrasound imaging of the heart was first reported in the 1950s, echocardiography was not established in clinical practice until 20 years later (12). This imaging modality has continued to evolve and expand to include Doppler ultrasound, transesophageal echocardiography, fetal echocardiography, 3D echocardiography, and intravascular ultrasound. Beyond ultrasonography, there is also advanced imaging, such as cardiac MRI and cardiac CT. All these imaging modalities have improved the timing and accuracy for diagnosing congenital heart disease. This has allowed for prenatal planning and timely interventions that have ultimately improved patients’ outcomes.

Initially, despite being able to diagnose the congenital heart defect, many infants with moderate to complex heart disease were only offered palliative care as surgical intervention was not an option. This all changed with the invention of the heart-lung machine in the 1950s, which we now know as cardiopulmonary bypass. The ability to perform intracardiac surgery with cardiopulmonary bypass allowed for surgical techniques to be discovered and refined, including the arterial switch operation for transposition of the great arteries and staged palliation for hypoplastic left heart syndrome. Then in the 1980s, cardiac surgeries were successfully performed on neonates. Coinciding with these surgical milestones were advancements in the cardiac catheterization lab, including device closures of septal defects, valve replacements, and angioplasties. Lastly, perioperative procedures regarding anesthesia and monitoring, as well as pre- and postoperative neonatal and pediatric critical care, continued to evolve with a unified goal to reduce the morbidity and mortality associated with congenital heart disease. Although survival continues to improve, there remains a significant risk for co-morbidities, including neurologic sequelae, which increases with the complexity of the congenital heart disease subtype.

Table 1. Global Prevalence of Congenital Heart Disease by Subtype

Congenital heart disease subtype	Global birth prevalence
Ventricular septal defect	35.6%
Atrial septal defect	15.4%
Patent ductus arteriosus	10.2%
Pulmonary stenosis	6.2%
Tetralogy of Fallot	4.4%
Transposition of the great arteries	3.8%
Atrioventricular septal defect	3.6%
Coarctation of the aorta	3.6%
Aortic stenosis	2.3%
Hypoplastic left heart syndrome	2.6%
Tricuspid atresia	1.1%
Pulmonary atresia	1.3%
Total anomalous pulmonary venous return	1.5%
Truncus arteriosus	1%
Ebstein anomaly	0.5%
Interrupted aortic arch	0.6%
(16)

Table 2. Complexity of Congenital Heart Disease Based on Subtype

Complexity	Congenital heart disease subtype
Mild	Ventricular septal defect
	Atrial septal defect
	Patent ductus arteriosus
	Pulmonary stenosis (mild)
Moderate	Atrioventricular canal defect
	Coarctation of the aorta
	Ebstein anomaly
	Pulmonary stenosis (moderate to severe)
	Tetralogy of Fallot
Severe	Single ventricle (hypoplastic left heart syndrome)
	Pulmonary atresia
	Transposition of the great arteries
	Tricuspid atresia
	Truncus arteriosus
	Interrupted aortic arch

Clinical manifestations

Presentation and course

The onset of neurologic sequelae related to congenital heart disease is variable and has been proven in some congenital heart lesions to be present as early as fetal life. Alterations in fetal cerebral blood flow and impairment in oxygen delivery are related to poor brain growth and development. Fetal and neonatal brain MRIs have shown disruptions in the micro and macrostructures of the grey and white matter, as well as a reduction in fetal brain volume. The incidence of microcephaly in newborns with unrepaired congenital heart disease has been reported at 33%, and the presence of neurobehavioral abnormalities, including motor deficits, feeding difficulties, alterations in suck reflex, and EEG abnormalities, in up to 50% (15). There are other factors to consider during fetal life besides the congenital heart lesion, including genetics, placental abnormalities, and environmental exposures (25). The physiological transition in the immediate postnatal period is another critical time for neurologic complications to occur. It has been reported that 25% to 40% of term neonates with congenital heart disease have documented pre-operative brain injury; however, a vast majority are clinically silent (02; 21). The most significant factor that increases the risk for neurologic complications is surgical repair. Exposure to anesthesia, need for cardiopulmonary bypass, deep hypothermia techniques, and alterations in hemodynamics all play a role in neurologic outcomes. It is during surgery that the patient is at great risk for cerebral macro and micro thrombosis and cerebral hypoperfusion, which can both result in strokes and seizures. These risks extend into the immediate postoperative period due to the need for mechanical circulatory support, fluctuations in hemodynamics, presence of a systemic inflammatory response, and risk for infection. Approximately 50% to 75% of repaired congenital heart disease cases are diagnosed with a new brain injury in the intraoperative or postoperative period. One third develop white matter injury, 10% are found to have embolic or ischemic strokes, and 5% to 20% have EEG-confirmed seizures (24; 21).

Although the risk for neurologic sequelae is lower in patients with congenital heart disease who have not required surgical intervention, the incidence is still greater than the general population (19). The mechanism for neurologic complications is largely based on the type of cardiac defect and its associated effects on hemodynamics.

Congenital heart disease neurologic impairments

From (Menkes et al 2006)

Patients with unrepaired cyanotic congenital heart disease survive in a state of chronic hypoxemia, which leads to polycythemia, erythrocytosis, and vascular dysfunction (09). The presence of right-to-left shunting allows for paradoxical embolization and risk for stroke. Cyanotic congenital heart disease carries a 10-fold higher risk for strokes compared to other congenital heart disease subtypes (26). Although less common, patients with predominately left-to-right shunts in acyanotic congenital heart disease can intermittently shunt right-to-left during times of increased right-sided pressure, also allowing for paradoxical embolization. Obstructive congenital heart disease lesions, such as aortic stenosis or coarctation of the aorta, carry a risk for low cardiac output and hypoperfusion, which can result in cerebral tissue hypoxia and vascular dysfunction. The risk for stroke is greatest in the left-sided obstructive lesions compared to right-sided, likely related to the impact sheer stress has on formation of embolisms and the direct exposure to systemic circulation (01).

Regarding the risk for infection, unrepaired shunts, valvar disease, and repaired congenital heart disease with residual lesions are a nidus for vegetations that can result in bacterial endocarditis. It has been reported that 20% to 40% of cases of bacterial endocarditis have neurologic complications, with 5% to 10% of these complications being moderate to severe (07). These complications include mycotic aneurysms, cerebral abscesses, stroke, meningitis, and encephalopathy. Cases of bacterial endocarditis with neurologic sequelae have the highest mortality, reported at 20% initially and increasing to 50% at 5-year follow-up (07). Unrepaired or partially repaired patients with cyanotic congenital heart disease have the highest risk for developing brain abscesses. Right-to-left shunting allows bacteria access to cerebral circulation, and chronic tissue hypoxia promotes bacterial overgrowth. Although the overall incidence of a brain abscess is low, cyanotic congenital heart disease is associated with 30% of these cases (27).

Table 3 outlines these neurologic complications as well as the clinical signs and symptoms to monitor. All the neurologic complications can lead to short- or long-term developmental delays, disorders, or disabilities. These can manifest as motor deficits, speech and language deficits, feeding difficulties, cognitive impairments, behavioral and psychosocial issues, and executive decision impairments (19).

Table 3. Neurologic Complications Associated with Congenital Heart Disease

Neurologic complications	Clinical manifestations
Stroke (20; 21)	• Focal hemi- or monoparesis • Partial or generalized seizures • Somnolence • Aphasia • Abnormal tone or dystonia • Coma • Oculomotor palsies • Hypotonia • Memory loss • Confusion • Tremor • Incoordination • Behavioral or personality changes
Cerebral abscess (27)	• Headache • Fever • Drowsiness • Confusion • Hemiparesis • Speech difficulties • Seizures • Stiff neck
Bacterial endocarditis (23)	• Fever • Weight loss • Heart murmur • Coughing • Malaise • Chills • Anorexia
Cerebral hemorrhage (02; 21)	• Headache • Irritability • Meningismus • Emesis • Cranial nerve abnormalities • Coma • Seizures • Hemiparesis • Aphasia • Macrocephaly • Lethargy
Neurodevelopmental disorders (19)	• Motor deficits • Speech and language deficits • Feeding difficulties • Cognitive impairments • Behavioral and psychosocial issues • Executive decision impairments

Prognosis and complications

At present, the survival rate at 1 year of age is 97% versus 75% for non-critical versus critical congenital heart disease. By 18 years of age, the survival rate is 85% versus 69% for non-critical versus critical congenital heart disease, and by 35 years of age, the overall survival rate is 81% (06). The prognosis for congenital heart disease patients with neurologic complications is variable and, in many cases, difficult to predict. The patients at greatest risk for morbidity and mortality are those with moderate to severe congenital heart disease (19). These same patients have a greater incidence of neurologic complications, as shown in Table 4 (22). The incidence of perioperative clinical seizures and EEG abnormalities is 5% to 10% and 20%, respectively (10; 05). Long-term follow-up has shown that these patients are at risk for developing epilepsy. Factors that increase this risk include number of cardiac interventions, congenital brain abnormalities, prematurity, stroke, and age at the time of surgery. Patients with congenital heart disease are almost four times more likely to have epilepsy than the general population (31). A population-based cohort study from Denmark found an overall incidence of epilepsy of 5% in patients with congenital heart disease, regardless of severity or need for intervention (14).

The most common and arguably most life-altering morbidity associated with congenital heart disease is neurodevelopmental disorders. At least one neurodevelopmental disability can be found in 50% of patients with congenital heart disease (19). As with the other neurologic complications, the patients at greatest risk are those with the most complex congenital heart disease. These disabilities can range from mild to severe, and their presence may not become evident until later in life. There are several factors that increase the risk for the number and severity of neurodevelopmental disabilities, including prematurity, surgical repair, genetic syndromes, neuroimaging abnormalities, perioperative seizures, history of mechanical support, history of cardiopulmonary resuscitation, and length of hospital stay. Guidelines have been established for early detection and intervention with the goal of optimizing neurodevelopmental outcomes. Developmental surveillance and screening in this patient population should start during infancy and extend into adulthood. Most pediatric surgical centers now have dedicated cardiac neurodevelopmental programs to implement these guidelines. Therapeutic interventions aim to improve academic, behavioral, psychosocial, and adaptive behaviors throughout life (19).

Table 4. Incidence of Neurologic Abnormalities Associated with Congenital Heart Disease

Congenital heart disease subtype	Incidence of neurologic abnormalities
Hypoplastic left-sided heart syndrome	29.0%
Transposition of great arteries	23.0%
Atrial septal defects	11.0%
Patent ductus arteriosus	11.0%
Tetralogy of Fallot	8.7%
Ventricular septal defects	8.7%
Coarctation of aorta	5.4%
Truncus stenosis	5.4%
Aortic stenosis	0% to 5.0%

Biological basis

Etiology and pathogenesis

The underlying cause of congenital heart disease remains largely unknown. Genetics and environmental exposure are likely the two greatest factors. Genetic testing has become standard of care and is generally recommended at the time of diagnosis, whether it be prenatally or postnatally. Karyotype, FISH, chromosomal microarray, and whole genome sequencing are some of the testing options that are offered. It is estimated that up to one third of congenital heart disease cases are associated with a genetic cause (30). Genetic syndromes, independent of the presence of congenital heart disease, are often associated with neurologic disorders and neurodevelopmental disabilities. In these cases, it is difficult to discern what contribution the underlying congenital heart disease may have had to neurologic complications versus the genetic syndrome. Table 5 lists common genetic syndromes and their associated congenital heart disease subtypes (11). Table 6 lists environmental exposures linked to increased risk for congenital heart disease.

Table 5. Genetic Syndromes and Associated Congenital Heart Disease

Genetic abnormality	Genetic syndrome	Congenital heart defects
Trisomy 21	Down syndrome	AVSD, VSD, ASD, PDA, TOF
Trisomy 18	Edward syndrome	ASD, VSD, PDA, TOF
Trisomy 13	Patau syndrome	ASD, VSD, PDA
22q11 deletion	DiGeorge syndrome	TOF, IAA, TA, VSD
7q11.23 deletion	Williams-Beuren syndrome	Supravalvular AS, stenosis of aorta, coronary arteries, pulmonary valve
XO	Turner syndrome	COA, AS, BAV, HLHS, PAPVR
HRAS gene	Costello syndrome	HCM, PS
TBX5 gene	Holt Oram syndrome	ASD, VSD, AVSD, PDA
EVC gene	Ellis Van Creveld syndrome	ASD, VSD, AVSD
RAS-MAPK pathway	Noonan syndrome	PS, ASD, PDA, HCM
	CHARGE syndrome	ASD, VSD, TOF PDA, AVSD
JAGGED1, NOTCH2	Alagille syndrome	PPS, PS, TOF VSD, ASD
VSD: ventricular septal defects; ASD: atrial septal defects; PDA: patent ductus arteriosus; TOF: tetralogy of Fallot; TGA: transposition of the great arteries; HLHS: hypoplastic left heart syndrome

Table 6. Environmental Factors Linked to Congenital Heart Disease

Environmental factors	Specific examples
Infection	• Rubella • Syphilis • Zika
Teratogens	• Retinoic acid • NSAID • Lithium • Dilantin • Anticonvulsants
Maternal diseases	• Diabetes • Systemic lupus erythematosus • Nutritional deficiencies (folate, vitamin A) • Smoking • Alcoholism
Air pollution	• Particulate matter • Nitrogen dioxide • Lead • Ozone • Sulfur dioxide

Prevention

There has been increasing interest in the discovery and refinement of neuroprotective strategies for patients with congenital heart disease (Table 7). Given that neurologic sequelae can be present as early as fetal life, some of these strategies can be implemented in the prenatal period. Early detection of congenital heart disease allows for prenatal planning and coordination of care for the mother and the fetus with a multidisciplinary team, including obstetrics, maternal fetal medicine, pediatric cardiology, and cardiothoracic surgery. Fetal echocardiography provides a detailed anatomic evaluation of cardiac structures. This imaging modality has its limitations, however, due to maternal body habitus, fetal position, technician expertise, and access to healthcare. Prenatal planning is a necessity with complex congenital heart disease as these fetuses require delivery at a tertiary care center, possible need for fetal therapeutic interventions, and access to highly specialized cardiologists, neonatologists, and cardiothoracic surgeons in the immediate postnatal period. As previously stated, the physiologic transition that occurs after delivery is a critical time for neonates with congenital heart disease. Limiting the duration of hemodynamic instability has been shown to reduce the risk for preoperative brain injury. Interventions, such as balloon atrial septostomy, initiation of medications (ie, intravenous prostaglandin), and access to cardiopulmonary support can prevent end-organ damage, which ultimately impacts timing of surgical repair (28).

Other perioperative neuroprotective strategies include hemodynamic monitoring, tight glycemic control, and avoidance of anemia. Near-infrared spectroscopy has now become the standard of care in critical care units and operating rooms. Near-infrared spectroscopy is a continuous monitor of cerebral and splanchnic oxygenation, allowing for early detection of hypo- or hyperoxia and timely adjustments to medical management. Tight glycemic control and avoidance of significant hypoglycemia has been associated with improved cognitive and motor outcomes. Lastly, severe anemia impairs oxygen delivery and can be avoided with blood transfusions and supplementation (ie, iron). Further studies are needed to determine the hematocrit cutoff level at which transfusion would be recommended. It has been reported that levels less than 24% are associated with poorer psychomotor development index scores. Blood transfusions come with risks, such as immune reactions; therefore, autologous umbilical cord blood transfusions are being studied in neonates.

Medications with possible neuroprotective properties are being investigated. Corticosteroids have long been used in the perioperative period to reduce systemic inflammation; however, they remain controversial as studies have not consistently shown benefit. Erythropoietin promotes red blood cell production and is also involved in cellular protection from oxidative stress. It is currently being studied in the congenital heart disease population and in infants with hypoxic ischemic encephalopathy. Dexmedetomidine is a selective alpha-2 adrenergic agonist that can reduce anesthesia-related neurotoxicity as well as neuroinflammation. Lastly, allopurinol likely has neuroprotective properties as it has been shown to reduce free oxygen radicals in neonates with hypoxic ischemic encephalopathy. This has potential in the congenital heart disease population; however, more studies are needed to confirm its impact on outcomes (13).

Cardiopulmonary bypass is a necessity for most surgical techniques yet remains a significant risk factor for neurologic complications. Optimization of regional cerebral perfusion, cooling strategies, cardiopulmonary bypass flow rates, pH control, and approaches to reperfusion are all constantly being reviewed and revised to improve outcomes (28).

Table 7. Neuroprotective Strategies for Congenital Heart Disease

Method of prevention	Risk factors	Notes
Prenatal planning and detection	Delay in diagnosis; increased length of time with hemodynamic instability; access to specialized care	Fetal echocardiography allows for early diagnosis.
		Determines need for delivery at specialized tertiary care centers.
		Planning for postnatal access to the appropriate medical and surgical care (IV prostaglandin, balloon atrial septostomy, etc).
Hematocrit monitoring	Anemia impairs oxygen delivery.	Treat deficiencies (ie iron, folate, B12) and give blood transfusions as needed.
Use of neuroprotective medications (corticosteroids, erythropoietin, dexmedetomidine, allopurinol)	Neuroinflammation, anesthesia neurotoxicity, oxidative stress	Allows for cellular protection, reduces inflammation, reduces free oxygen radical; studies needed to prove benefit.
Optimize cardiopulmonary bypass techniques	Cerebral embolization, inflammation, and hypoperfusion	Optimization of regional cerebral perfusion, cooling strategies, cardiopulmonary bypass flow rates, pH control, and reperfusion strategies.

Diagnostic workup

Table 10. Diagnostic Tools to Evaluate Neurologic Impairments in Congenital Heart Disease Patients

Test	When to administer	Description
Cranial MRI and CT (18)	Initial evaluation; workup planned based on results	Evaluates for stroke and abscess
Cranial ultrasound (08)	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. MRA is a noninvasive method of visualizing intracranial vasculature, and although conventional angiography is still the “gold standard,” MRA can largely replace invasive methods in the evaluation of ill children.

Table 11. ICD Codes Applicable for Neurologic Complications of Congenital Heart Disease

ICD-10

Acute and subacute bacterial endocarditis
Acute, but ill-defined, cerebrovascular disease
Bulbus cordis anomalies and anomalies of cardiac septal closure
Cardiac dysrhythmias
Conduction disorders
Heart failure
Ill-defined descriptions and complications of heart disease
Intracranial and intraspinal abscess
Occlusion of cerebral arteries
Other and ill-defined cerebrovascular disease
Other congenital anomalies of the circulatory system
Other congenital anomalies of the heart
Other diseases of endocardium, valve disorders
Secondary cardiomyopathy unspecified
Transient cerebral ischemia

I33.0
I64
Q20.9
I49.9
I45.9
I50.9
I51
G06
I66.9
I678-9
Q28.9
Q24.9
I38
I42.9
G45.9

Special considerations

Pregnancy

Most women with congenital heart disease will reach childbearing age due to improvements in long-term survival. A multidisciplinary team, including obstetrics, maternal fetal medicine, and cardiology, is needed to optimize outcomes for both the mother and baby. Women with complex congenital heart disease are at greatest risk for complications and require meticulous preconception planning. Hemodynamic changes during pregnancy include a 40% increase in blood volume and 30% to 50% increase in cardiac output, which increases the risk for heart failure. The threshold for conduction disturbances is lowered in pregnancy, including ventricular arrhythmias and reentrant supraventricular tachycardia (04). Poor ventricular function as well as arrhythmias are well-known risk factors for clot formation and strokes. Women with mechanical valves need to be closely monitored and appropriately anticoagulated as the most life-threatening complication in these cases is valve thrombosis. Pregnancy is already a prothrombotic state that contributes to the risk for thrombosis. In cases in which the mother requires surgical intervention for valve thrombosis, fetal mortality has been reported up to 33% (04).

Adult congenital heart disease

The long-term survival rates for adults with congenital heart disease have been steadily increasing due to advancements in diagnostic modalities, medical management, and surgical techniques over the last 50 years. Approximately 90% of patients with mild, 75% of patients with moderate, and 40% of patients with complex congenital heart disease are surviving to 60 years old (03). Currently, there are more adults living with congenital heart disease than there are children, which has been challenging. Patients with adult congenital heart disease, specifically those with an atrial septal defect, Fontan circulation, unrepaired cyanotic congenital heart disease, or Eisenmenger syndrome, have an increased risk for stroke and post-stroke mortality compared to the general population (03). Thromboembolic complications are 10 to 100 times more common in patients with adult congenital heart disease, specifically those with dilated chambers, tachyarrhythmias, intracardiac prosthetic material, pacemaker or defibrillator leads, or intracardiac shunts (03). Guidelines are being established for the prevention and management of these complications as well as other forms of acquired heart disease in the adult congenital heart disease population. Lastly, although overall survival has been improving, there has been no significant change in neurodevelopmental outcomes. Neuropsychological deficits, including executive function, impact the ability for some patients with adult congenital heart disease to function independently. This is likely contributing to poorer academic achievements, lower employment rates, and lower Health-Related Quality of Life (HRQOL) scores in patients with adult congenital heart disease compared to the general population (28).

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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

Lindsey McPhillips DO

Dr. McPhillips of Atlantic Medical Group has no relevant financial relationships to disclose.
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Kara Gay-Simon MD

Dr. Gay-Simon of Nicklaus Children's Hospital has no relevant financial relationships to disclose.
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Bernard L Maria MD

Dr. Maria of Thomas Jefferson University has no relevant financial relationships to disclose.
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Editor

Bernard L Maria MD

Dr. Maria of Thomas Jefferson University has no relevant financial relationships to disclose.
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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 months to 65+ years
Sex preponderance: None
Heredity: None
Population groups selectively affected: No population group selectively affected
Occupation groups selectively affected: No occupation group selectively affected

ICD & OMIM codes

OMIM: Alagille syndrome: #118450

CHARGE syndrome: #214800

Costello syndrome: #218040

DiGeorge syndrome: #188400

Down syndrome: #190685

Edward syndrome: #147791

Ellis-Van Creveld syndrome: #225500

Holt Oram syndrome: 142900

Noonan syndrome: 163950

Patau syndrome: 264480

Turner syndrome: 300082

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Congenital heart disease: neurologic complications

Differential Diagnosis

stroke
abscess
hemorrhage
seizures with or without a Todd paralysis
migraine (especially hemiplegic, ophthalmoplegic, or confusional state)
- diabetes mellitus with ketoacidosis
- lipoprotein disorders
- hypoglycemia
- trauma including non-accidental trauma
- post-viral acute demyelinating encephalomyelitis
- depression or conversion reaction
- drug reaction
- paroxysmal vertigo
- myoclonus
- tics
- breath-holding spells
- panic attacks
- narcolepsy
- febrile seizures
- toxic-metabolic encephalopathy
- hypertensive encephalopathy
- encephalitis
- syncope
- gastroesophageal reflux
- dystonia

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	--

Congenital heart disease: neurologic complications

Cite this article

Introduction

Overview

Key points

Historical note and terminology

Table 1. Global Prevalence of Congenital Heart Disease by Subtype

Table 2. Complexity of Congenital Heart Disease Based on Subtype

Clinical manifestations

Presentation and course

Table 3. Neurologic Complications Associated with Congenital Heart Disease

Prognosis and complications

Table 4. Incidence of Neurologic Abnormalities Associated with Congenital Heart Disease

Biological basis

Etiology and pathogenesis

Table 5. Genetic Syndromes and Associated Congenital Heart Disease

Table 6. Environmental Factors Linked to Congenital Heart Disease

Prevention

Table 7. Neuroprotective Strategies for Congenital Heart Disease

Differential diagnosis

Table 8. Common Illnesses That Masquerade as Stroke, Hemorrhage, or Abscess

Table 9. Illnesses of CNS Origin That Most Commonly Occur in Congenital Heart Disease

Common illnesses of CNS origin

Diagnostic workup

Table 10. Diagnostic Tools to Evaluate Neurologic Impairments in Congenital Heart Disease Patients

Table 11. ICD Codes Applicable for Neurologic Complications of Congenital Heart Disease

Special considerations

Pregnancy

Adult congenital heart disease

Media

References

Contributors

Authors

Editor

Former Authors

Patient Profile

ICD & OMIM codes

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