colorado review of anesthesia for surgicenters and ... · tetralogy of fallot tricuspid atresia...

Anesthetic considerations in Adults with Congenital Heart Disease

Dr. Mark Twite MA MB BChir FRCP

Director of Congenital Cardiac Anesthesiology

Associate Professor

Department of Anesthesiology

University of Colorado, Anschutz Medical Campus &

Children’s Hospital Colorado

CRASH Vail, Colorado 2018

Colorado Review of Anesthesia for SurgiCenters and Hospitalsand Ski Holiday!

No Financial Disclosures

CRASH Vail, Colorado 2018

Colorado Review of Anesthesia for SurgiCenters and Hospitals

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Ameristar Casino, Blackhawk, CO

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Objectives

Understand and Discuss the:

1. Changing epidemiology of congenital heart disease (CHD)

2. Current outcomes for patients with CHD

3. Perioperative anesthetic approach to adults with CHD undergoing non-cardiac surgery

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• 37yr old female in Atrial Flutter presents for TEE, EP study and ablation

• Tricuspid Atresia s/p Fontan

‐ Non-alcoholic cirrhosis (elevated LFTs and low albumin)

‐ Protein losing enteropathy

‐ Hypoxia. Baseline Sats 75-85% in air. Polycythemia Hct 65

‐ Pacemaker Dual Chamber DDD at 70bpm

• Medications

‐Apixaban, Aspirin

‐L-Arginine

‐Digoxin, Quinapril, Sotalol

‐Furosemide, Spironolactone

‐Melatonin, Valium, Ambien, Tramadol

Case

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• Echocardiogram 9/2016 TTE

1. History of Tricuspid atresia, status post initial class (RA to PA)Fontan, with subsequent Fontan conversion to intra-cardiac lateral tunnel Fontan through the RA body

2. Subjectively there remains normal LV systolic function.

3. Dilated IVC without respiratory collapse. Low velocity flow within IVC suggests IVC pressure is greater than 15 mm Hg .

4. The Glenn anastomosis is not well visualized, though there is normal respiratory variation in SVC flow suggestive of no obstruction within the Fontan pathway.

5. Trivial mitral regurgitation, mild mitral stenosis (mean gradient 5.6 mmHg).

6. Mildly dilated left ventricle with subjectively normal function. Hypoplastic right ventricle is not well seen.

7. No pericardial effusion.

Case

Mark Twite, MB Anesthetic Considerations in Adults with Congenital Heart Disease

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• Cardiac Cath

CATH (Denver, 2010) no fenestration, unobstructed PA's, Fontan 11-12 mmHg, no change with volume challenge, small angiographic right to left shunt seen likely from the suture line of the lateral tunnel.

CATH (Denver, 2013) Fontan pressures 18 mmHg, PCWP 14 mmHg, small veno-venous collaterals off left innominate embolized, 100% FiO2 and improved Fontan pressure to 11 mmHg

CATH (Denver, 2016) hepatic wedge 19 mmHg, hepatic vein 15 mmHg (transhepaticgradient 4 mmHg), Fontan 13, RPA 11, LPA 12, PCWP 9. Fick CO 5.06. S/P 10ml/kg bolus PA increased to 17, PCWP to 14, Fick CO 5.74. With 100% O2, PA pressure decreased to 13, PCWP to 12, Fick CO 5.69, with 40 ppm iNO PA pressure 14 , PCWP 13, CO 5.64

Case

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• Most common congenital disorder of newborns‐ 1% of live births‐ Leading cause of infant deaths in the USA‐ Accounts for more than half of all deaths from congenital anomalies worldwide

• Estimated 1.5 million adults living in USA with CHD

• NIH funded CHD research from 2005 – 2015‐ 663 CHD research projects for a total cost of $991 million

• 70% Basic science (Cardiac developmental biology most common)• 27% Clinical• 3% Both

Congenital Heart Disease

Van der Bom Nat Rev Cardiol 2011Burns Pediatr Cardiol 2017

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Changing Epidemiology: Adults and Children in Quebec

Marelli Circulation 2007 & 2014

Median age with severe CHD

Year Median Age (yrs)

1985 11

2000 17

2010 25

All CHD

Severe CHD

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Changing Epidemiology: Mortality

Distribution of Age at Death in Patients With Congenital Heart Disease in 1987 to 1988 and 2004 to 2005. Histogram bars depict the proportion of all deaths (x-axis) according to age at death (y-axis). Bold black curves with diamonds represent the corresponding age at death distribution in the general Quebec population Khairy J Am Coll Cardiol 2010

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Classification of CHD Complexity

Simple Moderate Severe

Atrial septal defect Anomalous pulmonary venous drainage

Single ventricle palliation

Ventricular septal defect Atrioventricular canal defect

Transposition of the great arteries

Patent ductus arteriosus Coarctation of the aorta Truncus arteriosus

Tetralogy of Fallot Tricuspid atresia

Pulmonary atresia

Eisenmenger syndrome

Simple defects have a favorable natural history unless they are unrepaired with a significant L to R shunt which may then develop Eisenmenger syndrome


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Long term Survival by complexity of CHD

95% 90% 80%

Simple Moderate Severe

Atrial septal defect Anomalous pulmonary venous drainage

Single ventricle palliation

Ventricular septal defect Atrioventricular canal defect

Transposition of the great arteries

Patent ductus arteriosus Coarctation of the aorta Truncus arteriosus

Tetralogy of Fallot Tricuspid atresia

Pulmonary atresia

Eisenmenger syndrome

Long term survival > 20 years Warnes J Am Coll Cardiol 2008

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CHD Pathophysiology

Valve stenosis & regurgitation

Atrial stretch, connections, synchrony,

residual lesions

Outflow stenosis, dilation,

compression

Ventricular function, rhythm, geometry,

residual lesions

Heart Failure Arrhythmias

Pulmonary Hypertension

Cyanosis & Bleeding COMORBIDITIES

Congenital & Acquired

Lesion Specific

1. Single Ventricle

Left vs Right, PLE

Thromboembolic

Liver Dysfunction

2. Tetralogy of Fallot

PI, RV Dilation

3. TGA

Baffle stenosis, coronaries

Lesion Specific

1. Single Ventricle

Left vs Right, PLE

Thromboembolic

Liver Dysfunction


PI, RV Dilation

3. TGA


Special Situations

Pregnancy, laparoscopy, regional & neuraxial

Special Situations


Endocarditis prophylaxisEndocarditis prophylaxis

Psychosocial

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Changing Surgical techniques: TGA

1975 Jatene Arterial Switch Operation

DoubleDouble SwitchSwitchTransfer

coronary buttonsLecomptemaneuver

1960 Senning-Mustard

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Changing Surgical techniques: TA Stage 1 BT Shunt

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Changing Surgical techniques: TA Stage 2 Glenn Shunt


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Changing Surgical techniques: TA Stage 3 Fontan

AtrioPulmonary Lateral Tunnel Extracardiac

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Effects of anesthetic agents

aKetamine can depress contractility in vitro and in catecholamine depleted patients.

bDexmedetomidinecan increase MAP during loading dose administration.

Friesen SCVA 2017

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Consensus Statements

Circulation, October 2017

Circulation, January 2017

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Time-series analysis: referral to specialized ACHD

centers and ACHD patient mortality

Mylotte Circulation 2014

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Studied 482 adults with CHD from 12 centers in the USA• Most common presenting arrhythmia:

‐ Intra-atrial re-entrant tachycardia (IART) 61.6% (increased with CHD complexity)‐ Atrial fibrillation (AF) 28.8% increased with age to surpass IART at 50yrs of age‐ Focal atrial tachycardia 9.5%

Atrial Arrhythmias

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Risk factors for developing atrial arrhythmias: ‐ single ventricle‐ previous intracardiac repair‐ systemic right ventricle‐ pulmonary hypertension


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Adults with CHD and atrial arrhythmias:• x 4 increase of heart failure• x 2 increase in death

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Atrial arrhythmias frequent cause of sudden cardiac death (43%)Increasing incidence with complexity of CHD

‐ Eisenmenger‐ Transposition of great arteries (atrial switch)‐ Single ventricles lesions palliated to Fontan circulation

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Single Center study in USA of Fontan patients• 10, 20 and 30 year freedom from arrhythmias of 71%, 42% and 24%• SCD 52 of 1052 Fontan patients (5%) with 65% due to arrhythmias

Congenital Heart Disease 2017 (12) 17‐23

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Factors leading to arrhythmias in CHD

Escudero Can J Cardiol 2013

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Risk estimates for arrhythmias across CHD

PACES / HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in ACHD Heart Rhythm 2014

Sim

ple

Mod

erat

eS

ever

e

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Majority of SCD victims unrecognized with an area under the curve of 0.6 for the discriminative ability of current guidelines Critical clinical reasoning essential when deciding on ICD placement in adult CHD patients

Implantable Cardioverter-Defibrillator


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ACHD Heart Failure

Norozi Am J Cardiol 2006DiNardo Sem Cardiothoracic Vasc Anes 2012Stefanescu Curr Treat Options Cardio Med 2014

Single Ventricle palliated to Fontan

Single ventricle has both the systemic and pulmonary resistances in series

y

Tetralogy of Fallot

Pulmonary regurgitation causes RV dilation and dysfunction, abnormal septal configuration, altered RV-LV interaction and LV dysfunction

Subsystemic RV

1. D-TGA palliated with a Mustard/Senning atrial switch

2. ccTGA Cor

onar

y A

rter

y D

isea

se –

focu

s on

prev

enti

on

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Heart Liver Transplant

Transplantation February 2018 Volume 102 Number 2

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Heart Liver Transplant

UNOS Database 1987 – 2015

• 61437 Heart Tx

• 190 CHLT

• 41 had CHD

• 30 day, 1 and 5 year survival 95%, 86% and 83%

• CHLT with and without CHD comparable

• Trend towards better survival for CHLT compared with isolated Heart Tx for CHD

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CHD Pathophysiology

Valve stenosis & regurgitation

Atrial stretch, connections, synchrony,

residual lesions

Outflow stenosis, dilation,

compression

Ventricular function, rhythm, geometry,

residual lesions

Heart Failure Arrhythmias

Pulmonary Hypertension

Cyanosis & Bleeding COMORBIDITIES

Congenital & Acquired

Lesion Specific

1. Single Ventricle

Left vs Right, PLE

Thromboembolic

Liver Dysfunction


PI, RV Dilation

3. TGA


Lesion Specific

1. Single Ventricle

Left vs Right, PLE

Thromboembolic

Liver Dysfunction


PI, RV Dilation

3. TGA


Special Situations


Special Situations


Endocarditis prophylaxisEndocarditis prophylaxis

Psychosocial

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[email protected]

Thank you


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