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Congenital Heart Disease: Outcome in Patients with Single Ventricle PowerPoint Presentation

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Description : Check out this medical presentation on Aortic Regurgitation, which is titled "Congenital Heart Disease: Outcome in Patients with Single Ventricle", to know about congenital heart disease and patients with single ventricle.

Tags : Aortic Regurgitation, Congenital Heart Disease, Patients, Single Ventricle

Published on : Jun 02, 2021 | Views : 1111 | Downloads : 8

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Slide 1-

Congenital Heart Disease: Outcome in Patients with Single Ventricle Jane E. Crosson, MD Director, Pediatric Electrophysiology and Adult Congenital Heart Disease Program
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Characterized by only one usable ventricle Final common pathway: Fontan procedure Surgical palliation that relies on “downhill” flow from veins to pulmonary arteries, with no pump to the lungs Long-term complications are numerous Single Ventricle: The most severe form of congenital heart disease
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Tricuspid atresia Variable presentation at birth Eventual palliation Fontan procedure Best long-term survival of all single ventricles, due to systemic left ventricle Hypoplastic left heart syndrome Includes aortic and mitral atresia, most severe form of HLHS Need multiple surgeries Survival rate has improved over time Relies on systemic right ventricle RV failure, tricuspid regurgitation common Most common forms of SV
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Tricuspid Atresia: Anatomic Variation Also called Hypoplastic Right Heart Syndrome Atretic tricuspid valve Small right ventricle fed via a VSD Obligate right to left flow across atrial septum Normal or malposed great arteries +/- PS, AS, coarctation
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Hypoplastic Left Heart Syndrome Aortic atresia/severe stenosis Mitral atresia/stenosis Hypoplastic LV Arch obstruction Ductal and PFO dependent
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Pulmonary atresia with intact ventricular septum Some can undergo 2 ventricular repair, if RV large enough, and no coronary arteries are dependent on the high pressure RV Those with very small RV, and RV dependent coronary circulation, require Fontan palliation Major risk: ischemia with sudden death More common forms of SV
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Double-inlet single left ventricle Unbalanced AV canal Primitive heart with single atrium and ventricle (“frog heart”) Other forms of SV
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Modified Fontan: Extracardiac Conduit Replaced lateral tunnel by late ‘90s in most centers Advantages: Can be done without circ arrest (or even CPB) Best fluid dynamics No intra-atrial suture line Disadvantages: Fixed conduit size Epicardial pacing only
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AV valve regurgitation Aortic regurgitation Sub-aortic obstruction Arch obstruction PA hypoplasia, obstruction, compression by aorta Pulmonary venous obstruction Baffle leaks What Can Go Wrong: Plumbing Surgical re-intervention in ~25% of Fontan patients
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Arrhythmias Heart block Sinus node dysfunction Atrial arrhythmias Ventricular arrhythmia Ventricular dysfunction Right ventricle especially vulnerable Causes: chronic cyanosis, chronic volume overload, dyssynchrony Thrombus, PE AP collaterals Pulmonary AVMs Chronic effusions Protein-losing enteropathy ‘Cirrhosis’ What Can Go Wrong: Physiology
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Single ventricle long-term follow-up
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Actuarial survival in hypoplastic left heart syndrome. McGuirk S P et al. Heart 2006;92:364-370 ©2006 by BMJ Publishing Group Ltd and British Cardiovascular Society
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Improved early survival in HLHS McGuirk S P et al. Heart 2006;92:364-370 ©2006 by BMJ Publishing Group Ltd and British Cardiovascular Society
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Copyright ©2000 American Academy of Pediatrics Mahle, W. T. et al. Pediatrics 2000;105:1082-1089 Neurodevelopmental Outcome in HLHS
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Health Excellent 52 (45.2%) Good 40 (34.8%) Fair 14 (12.2%) Poor 10 (8.7%) Limitations to activity None 59 (51.3%) Slight 43 (37.4%) Significant 12 (10.4%) Severe 2 (1.7%) Parental assessment of functional status of children with HLHS
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Copyright ©2000 American Academy of Pediatrics Mahle, W. T. et al. Pediatrics 2000;105:1082-1089 Number of cardiac medications required in children with HLHS
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Harrison
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Ventricular ejection fraction in Fontan patients
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Short and long term needs of Fontan patients Top-notch medical care Early school readiness intervention Life-long access to Adult Congenital Heart Disease experts Funds for pacemakers, repeat surgeries Job training Option for heart transplant Adult patients with single ventricle are the most poorly insured and most in need of services
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Almost all arrhythmias can be well-controlled with medication, catheter ablation of the arrhythmia focus, pacemaker and/or defibrillator (ICD) No limitations to work or school for most patients Those with pacemakers and ICDs have some limitations regarding work environment No extremely physical work No close exposure to high-voltage Cardiac Rhythm Issues in Pediatrics
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Rare in children as compared to adults 1.3 to 8.5 per 100,000 patient-years Often 2ndary to hypertrophic cardiomyopathy, myocarditis or congenital heart disease SIDS/airway most common cause in infants Most common primary arrhythmias with risk of SCD are: Long QT syndrome Catecholaminergic polymorphic ventricular tachycardia Arrhythmogenic right ventricular cardiomyopathy Brugada syndrome Sudden Cardiac Death in Children
Slide 22-

Overall survival rate for out-of hospital cardiac arrest is low, ~10%, including SIDS As with adults, witnessed arrest has better outcome; unwitnessed arrest survival rate ~0% If successfully resuscitated, cognitive sequelae can be significant, including lifelong need for complete support. Sudden Cardiac Death in Children
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Access to medication, catheter techniques and devices as needed Time off from work/school to see physicians/ have procedures Rare need for disability unless associated with co-morbidities such as congenital heart disease (not uncommon association), or sequelae from SCD resuscitation Cardiac Rhythm Issues in Pediatrics: Patient Needs