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7/28/2019 Adverse Outcomes of Fontan
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Hypoplastic left heart
syndrome and adverseoutcomes after the Fontan
procedure
Ian Lindsay
Senior Talk6/7/13
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Outline
Single-ventricle congenital heart disease
Staged palliative repair
Short-term complications
Long-term complications
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Hypoplastic Left Heart
Syndrome Hypoplastic (rarely atretic) L-sided cardiac
structures
Left ventricle
Ascending aorta
Valvular abnormalities
Aortic valve dysplasia/hypoplasia (100%)
Mitral valve (94%)
Tricuspid valve (56%)
Pulmonary valve (11%)
Moss & Adams 2007, JACC 2012
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HLHS - Epidemiology
2-3% congenital heart disease
25-40% neonatal cardiac deaths
(untreated)
2-3 cases per 1,000 live births (USA)
16,781 US cases from 1988-2005
1.5:1 male:female
J Pediatr 2008, J Thorac Cardriovasc Surg 2010
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HLHS - Mortality
Life expectancy Untreated: 100% fatal
Surgical palliation Early 1980s:
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HLHS - Genetics
Heritable
Associated genetic syndromes
Turner Syndrome Trisomy 13, 18, partial trisomy 9
Holt-Oram
Smith-Lemli-Opitz
Jacobsen Syndrome Extra-cardiac anomalies (prognosis)
Agenesis of the corpus callosum
Diaphragmatic hernia
OmphaloceleJACC 2012, Am Heart J 1992
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HLHS - Embryology
Any change of flow into or out of the LV canimpair LV growth
Atrial septal anomalies
Small foramen ovale
Posterior deviation of the septum premum
Underlying LV genetics
Endocardial fibreolastosis Obstructive valvular abnormalities
Mitral valve (parachute, arcade)
Aortic valve (bicuspid, unicuspid)
JACC 2012, Cardiol Young 2010
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HLHS - Embryology
20 weeks 33 weeks
JACC 2012
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HLHS - Anatomy
Moss & Adams 2007
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Other single-ventricle lesions
Tricuspid atresia
Pulmonary atresia with intact ventricular
septum
Double-inlet left ventricle
Double-outlet right ventricle
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Tricuspid Atresia
Mayo Clinic
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Pulmonary Atresia
Mayo Clinic
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DILV
Rush
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DORV
Rush
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Surgical Treatment
Palliative surgeries Conceptualized in 1971 (Dr. Francois Fontan) for
tricuspid atresia
3-stage approach in early 1990s
Palliative - does not restore normal physiology
Neonatal heart transplant 17% mortality rate on pediatric cardiac transplant
waiting list (increasing) Highest solid organ waitlist mortality in US
Survival rates 30-day (80%), 1-yr (75%), 5-yr (65%), 10-yr (60%)
JACC 2012
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Palliative Surgeries
Stage 1 Norwood procedure (neonatal period)
Unobstructed flow from systemic ventricle to
systemic circulation Neo-aorta creation
Controlled source of pulmonary blood flow
BT shunt - Innominate artery to RPA
Sano - RV to PA conduit (superior in SVR trial)
Unobstructed pulmonary venous return
Resection of atrial septum
Hybrid procedure (surgery + cath)
Moss & Adams 2007, JACC 2012, NEJM 2010
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Norwood
Moss & Adams 2007
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Palliative Surgeries
Stage 2 Glenn vs Hemi-Fontan (age 4-6
months)
Superior cavopulmonary shunt (SVC to RPA)
Removal of stage 1 shunt
Decrease volume load on systemic
ventricle Decrease pressure load on pulmonary
circulation
Improved cyanosis ~80% RAMoss & Adams 2007, JACC 2012
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Bidirectional Glenn
Moss & Adams 2007
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Palliative Surgeries
Stage 3
Fontan (Age 2-5 years)
Total cavopulmonary shunt
(IVC to RPA)
Complete passive systemic venous returnto pulmonary circulation
Single ventricle pump for systemiccirculation
Acyanotic!!
Moss & Adams 2007, JACC 2012
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Standard Fontan
RadioGraph 2011
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Modified Fontan
RadioGraph 2011
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External Conduit
RadioGraph 2011
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Lateral Tunnel
RadioGraph 2011
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Fetal Cardiac Interventions
(FCI) Severe aortic stenosis (leading to HLHS)
Fetal aortic balloon valvuloplasty
Mid-gestation (dilated LV, prior to shrinking)
10-15% fetal loss
HLHS with intact atrial septum Highly lethal underlying anatomy
Percutaneous atrial puncture + dilation Ideally performed at 20-25 weeks
Due to potentially fatal hemopericardium, mostoften performed in third trimester
Fetal cardiac transplant listingCirculation 2006, J Pediatr 2005, Circulation 2004
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FCI - Aortic Valvuloplasty
J Pediatr 2005
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Short-Term Complications
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Short-term ComplicationsStage 1 (Norwood)
JACC 2012
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Short-term Complications
Stage 1 (Norwood) Cardiac
CPR 10-17%
ECMO 7-10%
Arrhythmias 14-15% (SVT, VT, heart block)
BT shunt - coronary artery steal
Pulmonary
Mechanical ventilation 3-7 days Persistent chylothorax
Bleeding, coagulopathy
Infection 10%
JACC 2012
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Short-term Complications
Stage 1 (Norwood) Neurological
Seizures 4-22%
CVA 5%
Phrenic nerve injury
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Short-term Complications
Stage 2 (Glenn) Mortality
Hospital mortality ~0%
1-year survival >95%
Cardiovascular SVC syndrome
AV node dysfunction 6-8%
Phrenic nerve injury Embolic complications (R-L shunt)
Pleural effusions 12-45%
JACC 2012
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Short-term ComplicationsStage 2 (Glenn)
JACC 2012
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Short-term Complications
Stage 3 (Fontan) Hospital survival >95%
Cardiac
Atrial arrhythmias Baffle thrombus
Pulmonary embolism
Effusions Pleural, pericardial, ascites
Addressed with fenestration
JACC 2012
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Long-term Medical Therapy
Afterload reduction PVR
Sildenafil (PDE-5 inhibitor) Increased cardiac index, exercise capacity and
myocardial performance in teens and adults
Systemic
ACE (captopril, enalapril, lisinopril) No improvement in (RCT):
Pleural effusions, exercise capacity, growth,ventricular remodeling/function, CHF
JACC 2012, Circulation 2009, Eur Heart J 2008, Eur J CT Surg 2009
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Long-term Medical Therapy
Anticoagulation Thromboembolism due to low-flow state in baffle
and underlying hypercoagulable state
Bimodal distribution First post-op year, 10 yrs post-op
Annual incidence 0.8-33%
Annual CVA incidence 1.4-19%
ASA vs heparin/warfarin prophylaxis Prospective RCT, first two years s/p Fontan
Overall rate 23% (18% vs 9%)
No statistically significant difference
Most patients on lifelong warfarin after 1st event
JACC 2012, Circulation 2008
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Long-term Complications
Congestive heart failure
Protein-losing enteropathy
Plastic bronchitis
Congestive hepatopathy
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Heart Failure
Decreased exercise tolerance Inadequate preload reserve
Inability of pulmonary vascular bed tovasodilate -> increased pressure ->reflected in Fontan circulation
Limited LV filling and inotropic
augmentation Other cardiac complications
Sudden death, arrhythmia
JACC 2012
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Protein-losing Enteropathy
Excessive GI protein loss
Due to lymphatic distention?
Prevalence 3.7-24%
10-year cumulative risk 13.4%
5-year mortality 64%
JACC 2012, Nutr Clin Pract 2012, Rychik 2007
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PLE - Pathophysiology
Nutr Clin Pract 2012
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PLE
Presentation Peripheral edema, ascites
Effusions - pleural, pericardial
GI symptoms - severe diarrhea, pain
Diagnosis Clinical presentation
Labs albumin, total protein, calcium, lymphocytes
stool alpha-1-antitrypsin
JACC 2012, Nutr Clin Pract 2012, Rychik 2007
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PLE
Pediatr Radiol 2012
Pleural Effusions Ascites, cirrhosis
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Nutr Clin Pract 2012
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Plastic Bronchitis
Long, branching bronchial casts
Recurrent
Typically presents with obstruction andrespiratory compromise/failure
Pathogenesis similar to PLE
Prevalence 4-14%
Pediatr Radiol 2012, JACC 2012
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Plastic Bronchitis
Pediatr Radiol 2012
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Plastic Bronchitis
NEJM 2002
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PB - Treatment
Mechanical extraction
Thoracic duct ligation
Cardiac transplant
Ann Thorac Surg 2012
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Congestive Hepatopathy
Previously described in adult CHF beforeadoption of aggressive medical therapy
Congestion -> fibrosis -> cirrhosis
Cirrhosis in Utah adult Fontan cohort Found in 44% of those screened (n=61)
HCC x3, adenomatosis x1
No consistent lab or clinical abnormalities No association with PLE, type of Fontan, etc.
Complicates transplant options Heart-liver transplant vs denial
Hepatology 2012
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Conclusions
Single-ventricle congenital heart diseaserequires complex, multi-staged surgicalpalliation
70% of patients born today are expected toreach adulthood and will be seen inyourprimary care clinics
Emerging evidence of multiple cardiac andextra-cardiac complications
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References
Marshall A, Tworetzky W, Bergersen L, et al. Aortic valvuloplasty in the fetus: technical characteristics of successful balloondilation. J Pediatr. 2005;147:5359.
Tworetzky W, Wilkins-Haug L, Jennings RW, et al. Balloon dilation of severe aortic stenosis in the fetus: potential for preventionof hypoplastic left heart syndrome: candidate selection, technique, and results of successful intervention. Circulation.2004;110:2125-31.
Makikallio K, McElhinney DB, Levine JC, et al. Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome:
patient selection for fetal intervention. Circulation. 2006;113:14015.
Struder S, Terry P. Images in clinical medicine: bronchial cast. NEJM. 2002;346(13):981.
Asrani S, Asrani N, Freese D, et al. Congenital heart disease and the liver. Hepatology. 2012; 56(3):1160-1169.
Gordon BM, Rodriguez S, Lee M, et al. Decreasing number of deaths of infants with hypoplastic left heart syndrome. J Pediatr.2008;153(3):354
Karamlou T, Diggs BS, Ungerleider RM, et al. Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-
year period. J Thorac Cardiovasc Surg. 2010;139(1):119.
Feinstein J, Benson W, Dubin A, et al. Hypoplastic left heart syndrome: current considerations and expectations. JACC. 2012;59(1, SupplS):S1-42.
Danford DA, Cronican P. Hypoplastic left heart syndrome: progression of left ventricular dilation and dysfunction to left ventricularhypoplasia in utero. Am Heart J. 1992;123:17123.
Lurie PR. Changing concepts of endocardial fibroelastosis. Cardiol Young. 2010;20:11523.
Ohye RG, Sleeper LA, Mahony L, et al. Comparison of shunt types in the norwood procedure for single-ventricle lesions. N Engl J
Med. 2010;362:1980
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References
Fredenburg T, Johnson T, Cohen M. The Fontan procedure: anatomy, complications and manifestations of failure. RadioGraph.2011;31:453-63.
Goldberg DJ, Szwast AL, Marino BS, et al. Abstract 2161: sildenafil improves ventricular performance in children and youngadults after the Fontan operation (abstr). Circulation 2009;120:S603-b.
Giardini A, Balducci A, Specchia S, Gargiulo G, Bonvicini M, Picchio F. Effect of sildenafil on haemodynamic response to exerciseand exercise capacity in Fontan patients. Eur Heart J 2008;29:16817.
Francis K, Bov T, De Groote K, et al. Pleural effusions, water balance mediators and the influence of lisinopril after completion ofFontan procedures. Eur J Cardiothorac Surg 2009;36:57 62.
Monagle P, Cochrane A, Roberts R, et al. Abstract 1157: a multicenter randomized trial comparing Heparin/warfarin versusaspirin as primary thromboprophylaxis for two years after Fontan procedure in children (abstr). Circulation 2008;118:S651.
Johnson J, Driscoll D, OLeary P. Protein-losing enteropathy and the Fontan operation. Nutr Clin Pract. 2012;27:375-84.
Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis. 2007;2:288-300.
Grutter G, Di Carlo D, Gandolfo F, et al. Plastic bronchitis after extracardiac Fontan operation. Ann Thorac Surg. 2012;94:860-4.
Khanna G, Bhalla S, Krishnamurthy R, et al. Extracardiac complications of the Fontan circuit. Pediatr Radiol. 2012;42:233-41.