Considerations in Adult Congenital Heart Disease and Stroke
2020; Lippincott Williams & Wilkins; Volume: 51; Issue: 8 Linguagem: Inglês
10.1161/strokeaha.119.028605
ISSN1524-4628
AutoresNelson Lu, Jonathan Smith, Jason G. Andrade, Alana M. Flexman, Thalia S. Field,
Tópico(s)Cardiac Arrhythmias and Treatments
ResumoHomeStrokeVol. 51, No. 8Considerations in Adult Congenital Heart Disease and Stroke Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyRedditDiggEmail Jump toFree AccessReview ArticlePDF/EPUBConsiderations in Adult Congenital Heart Disease and StrokeA Case Report Nelson Lu, BScPharm Jonathan M.C. Smith, MD Jason G. Andrade, MD Alana M. Flexman, MD Thalia S. FieldMD, MHSc Nelson LuNelson Lu , Jonathan M.C. SmithJonathan M.C. Smith , Jason G. AndradeJason G. Andrade , Alana M. FlexmanAlana M. Flexman , and Thalia S. FieldThalia S. Field Correspondence to: Thalia S. Field, MD, MHSc, Gordon & Leslie Diamond Health Care Centre, 2775 Laurel St, 8th Floor, Vancouver, BC, Canada V5Z1M9. Email E-mail Address: [email protected] https://orcid.org/0000-0002-1176-0633 Originally published24 Jun 2020https://doi.org/10.1161/STROKEAHA.119.028605Stroke. 2020;51:e148–e150A 38-year-old right-handed man presented to our centre with an acute ischemic stroke. He had a history of total anomalous pulmonary venous return corrected at 7 months of age and was lost to follow-up. He was otherwise healthy with no prior history of stroke or cardiac risk factors.On arrival, his National Institutes of Health Stroke Scale was 8. He had right-sided facial droop and expressive aphasia. He was able to follow 2-step commands and was alert, but was unable to state his age or month. His pupils were equal and reactive with intact visual fields and normal extraocular movements. There was right-sided upper extremity weakness graded 2/5 on the Medical Research Council scale. His blood pressure was 159/82, with a heart rate of 41, and oxygen saturation of 100% on room air. CT head and CT angiogram showed a left middle cerebral artery M2 branch occlusion and minimal early infarct changes, with an Alberta Stroke Program Early CT Score of 8. An ECG showed newly identified atrial fibrillation.IV tPA (intravenous tissue-type plasminogen activator) was administered, and the endovascular team were alerted. Midway through the tPA infusion, before thrombectomy, he had a generalized tonic-clonic seizure requiring discontinuation of the infusion and management with a phenytoin load. He had depressed level of consciousness and was intubated for airway protection. A repeat CT head showed no intracranial hemorrhage. He subsequently underwent successful mechanical thrombectomy with achieved Thrombolysis in Cerebral Infarction score 2b reperfusion. He received vasopressor support with phenylephrine intraprocedurally.Postprocedure, he spontaneously converted to junctional rhythm with bradycardia and hypotension, which required treatment with atropine. Transesophageal echocardiogram excluded the presence of intracardiac thrombus, but demonstrated moderate left atrial and severe right atrial dilatation. While his left ventricular systolic function was normal, his right ventricle was dilated with moderately depressed systolic function. Right-to-left shunt was excluded by bubble study. Follow-up neuroimaging confirmed an established left middle cerebral artery branch infarct.He was started on oral anticoagulation with apixaban. At the time of transfer to inpatient rehabilitation, he had mild expressive dysphasia and 3/5 pyramidal arm weakness. He subsequently made a complete recovery with regards to his language, with subtle deficits of fine finger movements and pronator drift on the right within 1 month of his event.DiscussionIndividuals with congenital heart disease (CHD) are surviving into adulthood due to advancements in pediatric diagnosis and treatment. Those with complex CHD, including cyanotic lesions like unrepaired total anomalous pulmonary venous return (Table 1) are at increased risk of stroke due to a number of factors. Many congenital cardiac lesions result in abnormal flow patterns and atypical pressure gradients. Pressure and volume stress can lead to chamber dilatation and fibrosis of the myocardium which may mediate stasis and thrombus formation as well as dysrhythmias. Dysrhythmias are the most common cause of hospitalization in adults with CHD and are a common cause of death in this population. The presence of a tachyarrhythmia in this group confers a greater risk of thromboembolism than in the general population. Patients with shunt lesions may have increased risk of stroke via paradoxical embolism. The right-left shunts in cyanotic lesions, or temporary shunt reversals in the case of left-right shunts, may mediate venous-to-arterial emboli. For cyanotic CHD lesions, thrombotic risk may also be increased as a result of hyperviscosity from compensatory erythrocytosis during sustained hypoxemia.3Table 1. Stroke Risk in Adults With Congenital Heart DiseaseStudyMandalenakis et al1Lesion TypeAll CHDHigh Complexity LesionsAtrial Level ShuntAortic CoarctationDouble Inlet VentricleRisk of Ischemic Stroke—HR vs Age-Matched Healthy Control (95% CI)10.76 (8.49–13.63)12.22 (7.93–18.85)10.00 (2.02–49.55)12.86 (4.79–34.56)4.49 (1.56–12.93)Hoffmann et al2Lesion TypeTOFTGAOpen ASDFontan CirculationNon-Eisenmenger Cyanotic LesionPrevalence Estimates of Ischemic Stroke2.4%3.2%4.0%4.1%23.3%ASD indicates atrial septal defect; CHD, congenital heart disease; HR, hazard ratio; TOF, Tetralogy of Fallot; and TGA, transposition of the great arteries.Previous strokes in addition to congenital brain abnormalities may potentiate risk of seizure at or following stroke onset. Care must be taken not to overlook a potential stroke diagnosis in a younger adult with CHD presenting with a seizure, and use of antiepileptics with adverse cardiovascular effects should be avoided.4The optimal approach to hyperacute stroke management and relevant periprocedural anesthetic management during endovascular therapy for adults with CHD is not known and is not discussed in existing guidelines. The available pediatric literature is sparse with only a few reports on treatment of acute stroke in pediatric CHD.5 Bleeding risk with thrombolysis may be increased in CHD due to multifactorial coagulation abnormalities, and it is important to recognize that some of these individuals may be therapeutically anticoagulated for stroke prevention.3 There would not typically be arterial anatomic differences that would preclude endovascular therapy. However, vascular access may be complicated by recent or recurrent catheterization procedures.3Relevant information for anesthetic management for endovascular therapy includes the patient's relevant cardiac history, including prior surgical correction, intracardiac shunting, pulmonary hypertension and cardiac dysfunction, and any associated congenital syndromes. The 2018 American Heart Association/American College of Cardiology Guidelines for the Management of Adults with CHD recommend involving regional adult CHD centers with specialized anesthesiologists for patients with moderate-to-complex CHD.6For stroke prevention in adults with CHD, a multidisciplinary approach is optimal. It is important to identify patients with known CHD who have not received specialty follow-up in adulthood and arrange for referral to a regional adult CHD subspecialty clinic. This is an integral aspect of care for these patients for ongoing monitoring of heart failure and arrhythmias, among other comorbidities.6Current guideline recommendations for antithrombotics in adults with CHD are primarily based on observational literature (Table 2). While clinical risk scores such as CHADS2 and CHA2DS2-VASc are commonly used to stratify stroke risk in patients with atrial fibrillation, they may not apply in adults with CHD with atrial fibrillation/intraatrial re-entrant tachycardia.9 Some forms of complex CHD (eg, intracardiac repair, cyanosis, Fontan palliation, or systemic right ventricle) warrant antithrombotic therapy for the purposes of stroke prevention even in the absence of atrial dysrhythmia.6 While not yet reflected in published guidelines, newer retrospective and prospective observational studies examining stroke prevention for atrial arrhythmias in CHD suggest a favourable safety and short-term efficacy (1-year) profile with non-Vitamin K antagonist oral anticoagulants.10 Our patient was anticoagulated with apixaban as a multidisciplinary team decision.Table 2. Recommendations on Antithrombotics for Stroke Prevention in Adults With CHDGuidelineRecommendationLevel of Evidence2013 AHA Scientific Statement7Warfarin is recommended for ACHD with paroxysmal, persistent, or permanent AF or flutter or IART, or history of embolic strokeClass I; level C2014 PACES/HRS Expert Consensus Statement8Adults with complex CHD and sustained or recurrent IART/AF should receive long-term oral anticoagulationClass I; level BLong-term oral anticoagulation is reasonable in adults with CHD of moderate complexity and sustained or recurrent IART/AFClass IIa; level CVKA can be considered the oral anticoagulant of choice in adults with moderate or complex CHDClass IIa; level BMay be reasonable for adults with IART/AF and simple nonvalvular CHD to receive oral anticoagulant, ASA, or nothing on the basis of established scores for stroke risk and bleeding riskClass IIb; level BIn adults with simple CHD and no prosthetic heart valve or hemodynamically significant valve disease, a NOAC may be a reasonable alternative to VKA when anticoagulation is indicatedClass IIb; level CCurrently insufficient data to endorse use of NOACs in adults with Fontan surgeryClass III; level C2015 AHA Scientific Statement9Anticoagulation with warfarin is recommended in older (>40) patients with ACHD with sustained AF whether or not they meet usual criteria for AF/flutter in acquired heart disease (eg, CHADS2 scoring)Class I; level CAF indicates atrial fibrillation; AHA, American Heart Association; ASA, aspirin; CHD, congenital heart disease; IART, intraatrial re-entrant tachycardia; NOAC, non-Vitamin K antagonist oral anticoagulant; and VKA, vitamin K antagonist.Circumstances where warfarin would be preferred include recent cardiac surgery (<3 months), the presence of a mechanical heart valve, and atrioventricular valve stenosis with enlarged and diseased atria. Likewise, for cyanotic heart disease and single ventricle physiology, warfarin may be preferred as there is insufficient safety and efficacy data to support routine NOAC use. Close collaboration between subspecialty cardiologists and stroke neurologists is important for optimal selection of anticoagulation and ongoing clinical follow-up in the adult CHD population.Teaching PointsPatients with complex CHD are surviving into adulthood, have an increased risk for stroke, and it is likely that these individuals will increasingly present to stroke neurologists.Adult patients with CHD with complex disease who were lost to follow-up should be referred to subspecialty clinics for expert follow-up regardless of stroke history.Typical risk stratification tools for atrial fibrillation, such as CHADS2 and CHA2DS2-VASc, do not apply in cases of complex CHD.Nonstandard Abbreviations and AcronymsCHDcongenital heart diseaseIV tPAintravenous tissue-type plasminogen activatorSources of FundingNone.DisclosuresDr Field reports grants from Bayer Canada, personal fees from Servier, grants from Canadian Institutes of Health Research, grants from Michael Smith Foundation for Health Research, grants from Heart and Stroke Foundation of Canada, grants from American Academy of Neurology, grants from Centre for Aging and Brain Health Innovation, and grants from Vancouver Coastal Health Research Institute outside the submitted work. The other authors report no conflicts.FootnotesFor Sources of Funding and Disclosures, see page e150.Correspondence to: Thalia S. Field, MD, MHSc, Gordon & Leslie Diamond Health Care Centre, 2775 Laurel St, 8th Floor, Vancouver, BC, Canada V5Z1M9. Email thalia.[email protected]caReferences1. Mandalenakis Z, Rosengren A, Lappas G, Eriksson P, Hansson PO, Dellborg M. Ischemic stroke in cildren and young adults with congenital heart disease.J Am Heart Assoc. 2016; 5:1–8Google Scholar2. Hoffmann A, Chockalingam P, Balint OH, Dadashev A, Dimopoulos K, Engel R, Schmid M, Schwerzmann M, Gatzoulis MA, Mulder B, et al.. Cerebrovascular accidents in adult patients with congenital heart disease.Heart. 2010; 96:1223–1226. doi: 10.1136/hrt.2010.196147CrossrefMedlineGoogle Scholar3. Smith JMC, Andrade JG, Human D, Field TS. Adults With Complex Congenital Heart Disease: Cerebrovascular Considerations for the Neurologist.Front Neurol. 2019; 10:329. doi: 10.3389/fneur.2019.00329Google Scholar4. 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Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD).Heart Rhythm. 2014; 11:e102–e165. doi: 10.1016/j.hrthm.2014.05.009CrossrefMedlineGoogle Scholar9. Bhatt AB, Foster E, Kuehl K, Alpert J, Brabeck S, Crumb S, Davidson WREaring MG, Ghoshhajra BB, Karamlou T, et al.; American Heart Association Council on Clinical Cardiology. Congenital heart disease in the older adult: a scientific statement from the American Heart Association.Circulation. 2015; 131:1884–1931. doi: 10.1161/CIR.0000000000000204LinkGoogle Scholar10. Yang H, Bouma BJ, Dimopoulos K, Khairy P, Ladouceur M, Niwa K, Greutmann M, Schwerzmann M, Egbe A, Scognamiglio G, et al.. Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study.Int J Cardiol. 2020; 299:123–130. doi: 10.1016/j.ijcard.2019.06.014Google Scholar Previous Back to top Next FiguresReferencesRelatedDetails August 2020Vol 51, Issue 8Article InformationMetrics Download: 645 © 2020 American Heart Association, Inc.https://doi.org/10.1161/STROKEAHA.119.028605PMID: 32576089 Originally publishedJune 24, 2020 Keywordsseizuresthrombectomyatrial fibrillationheart defects, congenitalstrokethrombolytic therapyanticoagulantsPDF download SubjectsCongenital Heart DiseaseCerebrovascular Disease/StrokeCerebrovascular ProceduresRevascularizationIschemic Stroke
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