Adverse Outcomes of Fontan

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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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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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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Stage 1 (Norwood) Neurological

Seizures 4-22%

CVA 5%

Phrenic nerve injury


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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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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

92.


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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.

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Adverse Outcomes of Fontan