Jane E. Crosson, MD Director, Pediatric Electrophysiology and Adult Congenital Heart Disease...
-
Upload
darrell-robbins -
Category
Documents
-
view
223 -
download
4
Transcript of Jane E. Crosson, MD Director, Pediatric Electrophysiology and Adult Congenital Heart Disease...
Jane E. Crosson, MDDirector, Pediatric Electrophysiology and Adult Congenital Heart Disease Program
◦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
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
• 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
Aortic atresia/severe stenosis
Mitral atresia/stenosis
Hypoplastic LV Arch obstruction Ductal and PFO
dependent
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
Double-inlet single left ventricle Unbalanced AV canal Primitive heart with single atrium and
ventricle (“frog heart”)
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
AV valve regurgitation
Aortic regurgitation Sub-aortic
obstruction Arch obstruction
PA hypoplasia, obstruction, compression by aorta
Pulmonary venous obstruction
Baffle leaks
Surgical re-intervention in ~25% of Fontan patients
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’
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
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
Copyright ©2000 American Academy of Pediatrics
Mahle, W. T. et al. Pediatrics 2000;105:1082-1089
Neurodevelopmental Outcome in HLHS
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%)
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
Harrison
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 transplantAdult patients with single ventricle are
the most poorly insured and most in need of services
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
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
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.
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