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Adult Congenital Heart Disease: The New Reality

Kathryn Rouine-Rapp, MD

Professor of Anesthesia

I have nothing to disclose

Disclosures

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Outline

� Historic perspective

� Our reality

� Common lesions

� Guidelines

� Pathways to expertise

Lorraine Sweeney 1938

� PDA

� First person to survive surgery to correct CHD

� BCH

� Dr. Robert Gros

� 7 yo

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Eileen Saxon 1944

� TOF� First person to undergo BT(T) shunt � Johns Hopkins� Drs Blalock, Taussig, & Mr Thomas� Age 15 months

“switching arteries sidetracks blood and

oxygen to otherwise starved lungs”

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Our Reality

� 1-3 million adults USA and CA with CHD� 1.8 million Europe� Survival to adulthood increased from

30% in 1940s to nearly 90% today� More adults vs children with CHD� Median age 40 yrs

�FEBRUARY 23, 2014

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Our Reality

� >10000 adults with CHD� Increasing fraction of all non-cardiac

surgery� Majority underwent surgery non-

teaching hospitals� Increased morbidity and mortality

� Maxwell et al. Anesthesiology. 2013 Oct;119(4):762-9

Lesion classification

� Complexity classification

� Simple

� Moderate

� Severe

� 20-25% overall severe complexity

� 40% simple or “resolved” post intervention

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Patient status

� Unoperated� Palliated� Surgical or device correction

� Excellent uncomplicated result� Residual defect� Sequelae

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Lesions

� Common lesions

� VSD, ASD, PDA

� Pulmonary valve stenosis

� Aortic valve stenosis

� Coarctation of the aorta

� Atrioventricular-septal defects

� TOF

� TGA

ASDOne of most common defectsFour types

� Secundum (70%) , central IAS, associated MR

� Primum (15-25%), near AV valves, associated cleft MV

� Sinus venosus (10%), associated w anomalous PV

� Unroofed coronary sinus (rare)

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�http://www.doctortipster.com/wp-content/uploads/2011/07/interatrial-septal-defect2.gif

�https://apps.childrenshospital.org/clinical/mml/viewBLOB.cfm?MEDIA_ID=306

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Spectrum of severity24 yo male asx athlete, murmur detected, secundum ASD

83 yo male, in OR for CABG, new “incidental” finding on TEE immediately prior to CPB, SV ASD & anomalous RUPV

54 yo female, presented with DOE and new onset atrial fibrillation with RVR, TTE w RVD, L to R ASD flow

VSD

� Four types� Perimembranous (70%) � Muscular (20%) � Doubly-committed (subarterial)

(5%) � Inlet (5%)

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�http://206.47.151.137/bcdecker/figures/acs/part11_ch01_fig29.gif

Spectrum of severity

� Large defects = heart failure/sx

� Qp/Qs � pulmonary to systemic flow ratio

� defect size

� SVR and PVR

� PHTN

� Infective endocarditis

� Device closure or surgery

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Tetralogy of Fallot

� Most common cyanotic defect � Four lesions

� RVOTO (severity determines cyanosis)

� RVH� VSD (PM)� Overriding aorta

�http://www.heartbirthdefect.com/images/birth-defects/621x440xtetralogy-of-Fallot.jpg.pagespeed.ic.4Xfv8mG3um.jpg

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Spectrum of severity

� 61yo male for atrial flutter ablation

� Shunt placement

� Shunt revision and PM

� Surgical repair (10yo)

� Proximal LPA hypoplasia

� Aneurysmal RVOT patch, PI

� RV EF 30%

� Decreasing exercise tolerance

� Not a candidate for percutaneous intervention

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Guidelines

� 2008 ACC/AHA consensus statement

� Adults with CHD : surgical (diagnostic, interventional) procedures that require general anesthesia or conscious sedation in adults with moderate or complex CHD should be performed in a regional adult CHD center with an anesthesiologist familiar with adult CHD patients

Guidelines

� 2008 ACC/AHA consensus statement

� Adult patients with complex or high-risk CHD should be transferred to an adult CHD center for urgent or acute problems

� …and should have a cardiologist consultation prior to procedures

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Simple lesions

� Unoperated� Isolated mild

aortic or mitral valve lesion

� Isolated ASD� Small isolated VSD� Isolated mild PV

stenosis

� Operated� PDA� Secundum ASD� Sinus venosus

ASD wo residua� VSD wo residua

�Cannesson et al Anesth 2009

Training

� no established curriculum for education

� DiNardo, Baum, Andropoulous: pathways for pediatric cardiac anesthesia fellowships depend on training prior to fellowship AnesthAnalg. 2010 Apr 1;110(4):1121-5

� Inconsistent experience during adult cardiac anesthesia fellowships across USA (personal survey)

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Closed claim analysis� Factors: adverse events n = 21� 11 (52%) cardiac procedures � 10 (48%) noncardiac procedures� cardiac procedures

� surgical technique (73%) � intraoperative anesthetic care (55%)

� noncardiac cases � postoperative monitoring/care (50%)� CHD (50%) � preoperative assessment or

optimization (40%)�Maxwell BG et al. Congenit Heart Dis. 2015 Jan-Feb;10(1):21-9

Questions to consider

� Status of patient

� Unoperated, palliated, repaired

� Lesion and classification

� Simple, moderate, severe complexity

� Functional status

� NYHA

� Standard of care & experts in your group

� When to say “no”

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Summary

� Increasing population of adults with CHD who need our care

� Lesion classification

� Specific lesions

� Guidelines

� Training variability

� Reality of local care vs triage

THANK YOU

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