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Transcript
Hypoplastic Left Heart Syndrome What to Expect Down the Road Edward L. Bove, MD Helen and Marvin Kirsh Professor Chair, Department of Cardiac Surgery University of Michigan No Disclosures What to Expect Down the Road Edward L. Bove, MD Helen and Marvin Kirsh Professor Chair, Department of Cardiac Surgery University of Michigan “Hypoplastic left heart syndrome is a plumbing problem…a serious plumbing problem.” -William Norwood, MD Arch reconstruction Ascending aorta Coronary artery Systemic to PA Shunt Modified BTS RV to PA conduit Superior cavopulmonary shunt BDG HFP Fontan procedure Lateral tunnel Extracardiac conduit LV hypoplasia, 64% HLHS, 52% RV hypoplasia, 36% Lateral tunnel, 92% Extra-cardiac, 8% Hirsch et al, Ann Surg 2008;248(3):1-10 Hospital survival, 96% Survival with intact Fontan, 94% Takedown, 3% (17/636); mortality, 41% (7/17) 4/10 survivors underwent later Fontan Median LOS, 10 days Morbidity Arrhythmias, 20% Prolonged pleural drainage (< 2 wk), 19% Neurologic event, 5% Median follow-up, 50 months Actuarial survival at 14 yrs, 91% Late morbidity PLE, 6% Neurologic event, 4% Arrhythmia requiring treatment, 3% Chronic systemic venous hypertension PA hypotension Reduced ventricular preload/diastolic dysfunction Elevated systemic vascular resistance Low cardiac output PLE/plastic bronchitis Liver disease/cirrhosis/coagulopathy Pulmonary thromboembolism Recurring cyanosis/pulmonary AVM’s Exercise intolerance/ventricular dysfunction Dysrhythmias Valve: neoaortic, tricuspid Arch: obstruction, dilatation Fontan pathway/pulmonary artery Stenosis Hypoplasia Thrombus Coronary artery stenosis NB male, 37 weeks, 3.4 kg Fetal diagnosis of HLHS Aortic atresia, mitral atresia Ascending aorta 2 mm Normal RV function Mildly restrictive ASD Mild TR Stable on PGE infusion The Year: 1994 Successful Norwood operation performed with 3.5 mm MBTS Stage 2 and 3 procedures completed at 6 mos and 18 mos of age Active life, star pitcher for his HS baseball team Age 17 years, c/o angina symptoms while pitching PAP 12 mmHg, no Fontan pathway obstruction PVR, 2 units Trivial TR Normal RV function No arch obstruction But…aortogram demonstrated narrowed connection into the native ascending aorta Successful revision of coronary artery connection performed Now w/o symptoms and fully active Difficulty measuring the PVR Non-pulsatile flow Low pulmonary blood flow AP collaterals Thromboembolic events Elevated antibody levels Transfusions Homograft patch material Technical difficulties Carey et al Eur J Cardiovasc Surg 1998;14:7 65% actuarial survival at 5 years, p=ns, Fontan vs non-Fontan Kanter et al, Ann Thor Surg 2011;91:823 Small increments in flow and pressure needed to be effective Absence of venous reservoir/vessel collapse Avoid obstruction to native flow Potential for competing caval flows Bridge to transplant vs destination therapy Giridharan et al JTCVS 2013;145:249 Giridharan et al, JTCVS 2013;145:249 Lacour-Gayet et al, Ann Thor Surg 2009;88:170 PHN Study 546 children with a Fontan Mean age, 12 yrs (6 – 18 yrs) Physical and psychosocial scores within 80% of normal for healthy children, but point averages below the mean for the normal population Anderson et al, JACC 2008;52:85 Variable Anderson d’Udekem % predicted peak VO2 65% + 16% 57% + 8% % predicted peak VO2 at anaerobic threshold 78% + 25% 78% + 16% ↓ percent predicted peak VO2 ↓ percent predicted peak VO2 at anaerobic threshold d’Udekem et al, JTCVS 2009;88:1961 Author IQ Uzark1 97.5 + 12.1 Goldberg2 Total Cohort HLHS Non-HLHS 101.4 + 5.4 93.8 + 7.3 107 + 7.0 Wernovsky3 95.7 + 17.4 1. Pediatrics 1998;101(4):630-3 2. J Pediatr 2000;137:646-52. 3. Circulation 2000;102:883-9 Overall, the functional outcomes of Fontan patients are excellent The majority of both parent and self-reported QOL domains are lower than those score of the reference population and their siblings, but are still within the range of normal While peak exercise parameters are below normal, the majority of Fontan patients subjectively report good QOL in most physical domains Levels of physical activity are lower than normal, but are not related to peak exercise thresholds, suggesting that these limitations are self-imposed Neurocognitive outcomes, as measured by IQ, appear to be well within the range of normal The “modern” Fontan era provides 20-25 year follow-up Majority of patients are doing well within this time frame Structural issues will be relatively few Dysrhythmias will increase over time Functional problems will become more prevalent with age The use of transplant will increase The need for assist devices will become paramount