advancesin%achd · 2015-09-24 · marcg cribbs,md%% director,alabama%adult%congenitalheart%program%...

Marc G Cribbs, MD Director, Alabama Adult Congenital Heart Program 2015 Southeastern Pediatric Cardiology Society

September 26th, 2015

Advances in ACHD a narrative of thinking outside the box

§  I have no relevant financial relationships with any commercial interests to disclose.

“Congenital affections of the heart are of only limited clinical interest,

as in a large proportion of cases the anomaly is not compatible with life,

and in the others nothing can be done to remedy the defect

or even to relieve the symptoms.” Sir William Osler: The Principles and Practice of Medicine. 1894

§  34 year old male with tetralogy of Fallot

§  Status post: ü  Right Blalock Taussig shunt (age 6 mo) ü  Left BT shunt (3 yrs) ü  Transannular patch & VSD closure (6 yrs)

§  Presents with NYHA class III-‐IV

symptoms

Case 1: AH

§  Severe pulmonary insufficiency & RV dilation (age 11 yrs) §  Surgery recommended but… §  …lost to follow-‐up.

§  AICD for primary prevention (age 28) §  LVEF 25% §  Lost to follow-‐up

§  Past Medical History also includes…

Case 1: additional history

BMI = 41

§  Referred for heart transplant §  Quickly deemed ineligible due to:

§  Morbid obesity §  Continued tobacco abuse (1 ppd since 12 yrs of age) §  Medical non-‐compliance

§  Referred to the ACHD program for further management

Case 1: AH

§  Growing number of Adults with Congenital Heart Disease

§  Lost to follow-‐up: an ongoing problem

§  Care of the ACHD patient §  Alabama Adult Congenital Heart Program Alabama Adult Congenital Heart Program

Advances in ACHD: a narrative of thinking outside the box

The growing number of Adults with Congenital Heart Disease

Incidence of CHD

Expanding Population of Adolescents & Adults with CHD

Advanced Post-‐Op Care Early Complete

Repair Diagnostic Advances

Increased Early Survival Improved Peri-‐Op

Survival

Increased Mid & Late-‐Term Survival

Improved Surgical Techniques

0.4—1.2% of all live births §  #1 congenital malformation §  Blamed in 20% of miscarriages

1980 –

1970 –

1960 –

1940 –

0 10 20 30 40

Percent Survival to 18 Years Old

Decade Born with

CHD

50 60 70 80 90 100

20

JACC. 2001;37(5):1170-‐1175

1990

Circulation 2010.122:2264-‐2272

40

80

75

90+

Survival to 18 years of age with CHD

% Survival

Survival is expected – from the crib to old age

JACC. 2006; 47(4): 701-‐707

Percentage of Adults with Congenital Heart Disease

PEDIATRIC ADULT

2010

1965

2000

30%

65% 50%

70%

35% 50%

20,000 NEW ACHD patients per year

Circulation. 2015; 131: 1884-‐1931

ASD

AVSD

BAV

Coarctation

Chest pain

ccTGA

dTGA s/p Mustard

Ebstein

Fontan

Marfan, CTD

Pulm Stenosis

Tetralogy

Truncus

VSD

Alabama Adult Congenital Heart Program distribution of patients

Tetralogy of Fallot

dTGA

Fontan

Alabama ACHD Program

1560 pt visits (2014-‐2015)

…60% of patients are Lost to Follow-‐up

by age 18.

Circulation. 2009;120:302-‐309

“I have a history of tetralogy of Fallot repair and didn’t think I’d need another surgery…”

§  Misconception the disease has been “cured”

§  Lack of cardiac symptoms

§  Lack of appropriate healthcare access

Lost to follow-‐up: why does it happen?

Circulation. 2010; 122:868-‐875

Tachy-‐Arrhythmia by age in Tetralogy of Fallot

Step 1 Step 2

Step 4 Step 3

Medical School

Fellowship #2: Adult Congenital Cardiology

Fellowship #1: Adult Cardiology Pediatric Cardiology

Residency: Medicine/Pediatrics Internal Medicine Pediatrics

4 years 7-‐8 years 11-‐12 years 13-‐14 years

Number of years after college

Training: Adult Congenital Cardiology

Cost: ~$10,000.00

Lost to Follow-‐up why does it matter?

EHJ. 2010.31:1220-‐29.

Non Cardiac (23%) M

ultip

le (3

.6%)

Arrhythmia (21.9%)

Heart Failure (24.5%)

Vascular (14.3%)

Many of these can be prevented IF

UNinterrupted care is maintained

Therefore, exploring innovative ways to improve ACHD care is necessary

Care of the ACHD patient

Case 1: AH

Back to …

§  32 yo with tetralogy of Fallot status post:

ü  Right BT shunt ü  Left BT shunt ü  Trans-‐annular patch, VSD closure

§  History of: ü  Morbid Obesity Tobacco Abuse ü  Non-‐compliance AICD ü  NYHA class III-‐IV

§  Referred to the ACHD Program for further management

Right Ventricle: RV EDVi 649ml (250ml/m2); RVEF 17% Left Ventricle: LV EDV 299ml, LV ESV 236ml; LVEF 21% Cardiac CT

Dilated MPA to ~32mm Proximal narrowing of the RPA and LPA Cardiac CT

§  Case management conference §  Surgical pulmonary valve replacement?

Case 1: Decision making

§  Length of Stay > 7 days: 19.7%

§  Major Adverse Event (MAE): 20.5% §  Re-‐exploration Deep sternal infection Stroke §  Renal failure Ventilation > 48 hours

§  Hospital Mortality: 3.49%

Ann Thorac Surg. 2013; 95: 1377-‐82

§  AH’s Past Medical History: §  Male §  NYHA functional class III – IV §  Severe restrictive lung disease

§  Tobacco abuse

§  Agreed patient was High Risk for surgical PVR

§  Continue medical vs. “Other” management? §  Bilateral branch PA

percutaneous PVR pursued


Disclaimer: The procedure presented here is not included in the Medtronic Melody Valve IFU (Information for Use). IRB approval and appropriate consent was obtained.

Pre-‐procedure angiography

Right Pulmonary Artery Left Pulmonary Artery

Post-‐procedure angiography

Right Pulmonary Artery Left Pulmonary Artery

§  No pulmonary insufficiency

§  NYHA functional class II-‐III

§  Tobacco use: §  Still smoking ½ ppd

Case 1: Follow-‐up visit

Heart Failure (24.5%)

On to Case 2

Case 2: TH

§  45 year old male with dTGA: ü  S/P Mustard procedure ü  Recent Sinus Node Dysfunction and

Ventricular Tachycardia s/p pacemaker/AICD

§  Presents with progressive dyspnea

despite maximal medical therapy

Mustard: Despite excellent early outcomes…

…there remains a concerning incidence of long-‐term complications

§  Sinus node dysfunction (>50%) §  Arrhythmia (IART; ~30%) §  Baffle leaks (25%) §  Baffle obstruction (20%) §  Tricuspid Insufficiency §  Right ventricular failure

§  Primary long-‐term concern

§  Sudden death (6 – 17%) §  Tachyarrhythmia and CHF §  During exercise (81%)

Treatment of Systemic (RV) dysfunction

§  Conventional heart failure meds §  Do they help?

§  Tricuspid valve replacement §  Outcomes dependent upon good RV function

§  Heart transplant

§  Cardiac Resynchronization Therapy (CRT) §  Could it help the Systemic RV?

Cardiac MRI PRIOR to AICD

Systemic (Right) Ventricle EF <20% Severe tricuspid insufficiency

§  Patient not interested in cardiac transplant

§  Case management conference §  Tricuspid valve replacement felt to be high risk for

questionable reward §  Continue medical vs. “Other” management?


§  8 patients §  Age 6.9 to 29.2 years

§  Congenital heart disease with a Systemic RV

§  Mustard or Senning: 4 patients

§  QRS duration 161 + 21 msec

JACC. 2004;44:1927 – 31 §  QRS interval:

§  Decreased from 161 to 116msec (p<0.005)

§  RVEF%: §  Increased from 41.5 to 45.5 (p<0.04)

§  NYHA functional class: §  Improved from II to I (p<0.008)

CRT in Congenital Heart Disease

Heart Rhythm. 2014;11:e102–e165

§  Continue medical vs. “Other” management? §  Cardiac Resynchronization Therapy pursued


Transvenous leads to the left atrium and left ventricle Surgically placed leads to the systemic right ventricle

§  ECG: QRS 166 msec §  Previously, 188 msec

§  Systemic RVEF% unchanged

§  Improved shortness of breath and a 15 pound weight loss

§  NYHA functional class II-‐III

Case 2: Follow-‐up visit

Alabama Adult Congenital Heart Program And lastly…

Alabama adult congenital patients

Estimated 9,000 adults with congenital heart disease Where are they now?

60% of patients are Lost to follow-‐up…

§  Team: §  ACHD Physicians §  ACHD RNs §  ACHD Imaging (echo, CT, MRI) §  Interventional Cardiology §  Electrophysiology §  Heart Failure/Transplant §  CV Surgery §  Maternal Fetal Medicine

MFM & ACHD multidisciplinary team

Cardiology physicians Maternal Fetal Medicine physicians

OB Anesthesia physicians OB Anesthesia nurses Labor & Delivery nurses

§  Meet each month

§  Discuss each patient who is currently pregnant

§  Review history, recent imaging, and exam

§  Formulate optimal plan of care and delivery

Alabama Adult Congenital Heart Program

The Kirklin Clinic

Birmingham

Huntsville

Montgomery

Mobile

Alabama ACHD clinic locations

§  Growing number of Adults with Congenital Heart Disease

§  Lost to follow-‐up: an ongoing problem

§  Care of the ACHD patient §  Alabama Adult Congenital Heart Program Alabama Adult Congenital Heart Program

Advances in ACHD: a narrative of thinking outside the box

“Congenital affections of the heart are of only limited clinical interest,

as in a large proportion of cases the anomaly is not compatible with life,

and in the others nothing can be done to remedy the defect

or even to relieve the symptoms.” Sir William Osler: The Principles and Practice of Medicine. 1894

“Congenital affections of the heart are of significant clinical interest,

as in a large proportion of cases the anomaly is compatible with life,

and much can be done to remedy the defect and

to relieve the symptoms.”

§  Dr. Larry Markham §  Director, Vanderbilt Adult Congenital Heart Program

§  Dr. Curt Daniels §  Director, Columbus Ohio Adult Congenital Heart (COACH) program

§  Dr. Ali Zaidi §  Director, Montefiore Adult Congenital Heart program

§  Dr. Sarah Cribbs §  Med/Peds and Sports Medicine, UAB

Acknowledgements

Marc G Cribbs, MD Director, Alabama Adult Congenital Heart Program 2015 Southeastern Pediatric Cardiology Society

September 26th, 2015

Advances in ACHD a narrative of thinking outside the box Thank you

advancesin%achd · 2015-09-24 · marcg cribbs,md%% director,alabama%adult%congenitalheart%program%...

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