aaim15 - congenital heart disease...• describe the commonly encountered forms of adult congenital...

Long Term Outcomes & Issues for Adults with

Congenital Heart DiseaseJoseph D Kay MD FACC

Associate Professor of Medicine & Pediatrics

Colorado’s Adult and Teen Congenital Heart (CATCH)

Program Director

Med/Peds Residency Associate Program Director

October 23rd, 2015

Financial Disclosures

Actelion – Research Grants

(Dose not pertain to Talk)

Outline• Describe the stable incidence but changing prevalence in the population of

ACHD from epidemiologic studies (understanding increasing importance)

• Describe the commonly encountered forms of adult congenital heart

disease (CHD), including those that may be initially detected in adulthood

– Emphasis on septal defects, coarctation of the aorta, tetralogy of Fallot, transposition of the great vessels

– Discuss appropriate follow-up care for adults with CHD, including

optimal studies for following adult patients

• Describe late complications and long-term morbidity and mortality risks, and how to best detect/prevent such occurrences

– Residual mortality risks after complete structural/hemodynamic

correction

• Discuss risks of re-operation and co-morbid heart conditions (hypertensive

heart disease, coronary artery disease) in adult CHD patients

CardiologyEpidemiology - Changing Picture Of

Congenital Heart Disease

Marelli, A. Circulation. 2007;115:163-172.

Changing Prevalence of Adult Congenital Heart Disease

(AHCD)

Marelli, A. Circulation. 2007;115:163-172.

Changing Mortality in Congenital

Heart Disease (CHD)

Khairy et al., JACC 56(14) Sept 2010

Estimated Disease Prevalence

Assuming incidence of 0.52% (Newcastle)

Estimated # of ACHD survivors

• Canada: 1996 = 94,000 2006 = 124,000

• UK: Annual estimated increase >1,600/yr

• US: Annual estimated increase > 8,960/yr

32nd Bethesda Conf. JACC vol.37(5).

2001

Mortality rate in CONCOR patients and in the general Dutch population by decade in 2007.

Verheugt C L et al. Eur Heart J 2010;31:1220-1229

Causes of, and age at, death in CONCOR patients (n = 197).

Verheugt C L et al. Eur Heart J

2010;31:1220-1229

Straight Forward Congenital Lesions Expected to be Cared for By All

Cardiologists

Lesions Congenital Cardiologists Should See (Minimum of every 2 years for Moderate, Annual for Severely Copmplex)

Atrial Septal Defects(ASD)

Types of ASD: ECHO View

• A - Sinus venosus defect

• B - Secundum ASD

• C - Primum ASD

• D - Unroofed Coronary sinus (not shown)

Park MK, Ped. Card. For Pract.; 1996

Long Term Survival After Atrial Septal Defect Closure

Subjective Health Late After ASD Closure

Ventricular Septal Defects(VSD)

Types of Ventricular Septal

defects

• Top - 4 segments of

the normal right and

left ventricles

• Bottom - 4 types of

VSDs

Long Term Mortality After

Ventricular Septal Defect Closure

M. Menting et. al. JACC (65) 2015; 1941-51

Late Events After Ventricular Septal Defect Closure

M. Menting et. al. JACC (65) 2015; 1941-51

Coarctation of the Aorta

Coarctation Surgical RepairVarious forms of Rapair

Top – End to end

Middle – Subclavian flap

Bottom – Dacron patch

Left – Extended end to end

Right – Interposition graft

Complications with Both Approaches

Pseudoaneurysm After Stenting

Restenosis and Aneurysm

After surgery

Forbes TJ. JACC; 58(25) 2011:2664-74

Catheter Intervention – vs – Surgery for Native Coarctation : 2002-2009

Forbes TJ. JACC; 58(25) 2011:2664-74

Surgery –vs- Catheterization:Acute Outcomes

Forbes TJ. JACC; 58(25) 2011:2664-74

Intermediate Follow Up

Forbes TJ. JACC; 58(25) 2011:2664-74

Intermediate Outcomes (cont.)

Forbes TJ. JACC; 58(25) 2011:2664-74

Coarctation Long Term Surgical Outcomes

Cohen M et al. Circulation 1989;80:80-845

Coarctation: Long Term Survival Update - 2013

Brown ML et al., JACC 2013 (62):1020-25

Later survival after Coarctationrepair by Age at time of Surgery


Freedom from reoperation based

on age and Coarctation Repair


Causes of Death – Late after surgery

Cohen M et al. Circulation 1989;80:80-845

Patient Example

• 30 year old with history of bicuspid aortic valve and coarctation of the aorta

– s/p subclavian flap repair of his coarctation age 5.

– Balloon angioplasty for recurrent stenosis age 6-8

– Repeat surgery with resection of narrowing and placement of interposition graft age 11

• Followed up by cardiology. Note by cardiologist in Kaiser system 2/2014 mentioned echo looks fine and no hypertension, with normal lipids. Pt complains of atypcial left sided chest pain. Follow up in 1 year – reassured …

• Presents 1/24/2015 with hemoptysis

CT after CXR

Pt Course

• Transferred urgently to UCH, in shock.

• Considered minimally invasive covered stent.

• Decided to proceed to OR – sudden drop of BP when moving pt – placed on peripheral bypass

• Required resection of LUL of lung, and new interposition graft

• Hospital for about a 1 month but recoverd

High Increased Risk of Aortic Pseudoaneurysm with Gortex

Patch Repair – 63 patients

Cramer J. et al. Pediatr. Cardiol (2013) 34:296-301

ACC/AHA guidelines For repaired Coarctation, recommend regular CT/CMRI without symptoms

Incidence of HTN – late after surgical repair


Incidence of Hypertension Late After Successful CoA Repair – Meta Analysis

• 26 articles included

1987-2012

• 35% incidence of

HTN (25% - 68%)

• Conflicting data on

beta blockers –vs-

ACE-I or ARB

• High incidence of

endothelial

dysfunction & CITCanniffe, C. Int Journal Cardio. In press

10 % Incidence of Intracranial Aneurysms in

Coarctation Patients (5 x population incidence)

Connelly HM. Et.al. Mayo Clinic Proc.2003;78:1491-99

Incidence of Intracranial Aneurysms In

PTs with Coarctation (ICA) – Study 2

• OSU Screened 43 Adults with repaired coarctation CT /MR

• Found 5 patiens (11% ) with ICA

• Only risk in this group was age

– Avg 45.6 in ICA pts –vs- 30.89 in no ICA (p =

0.0003)

– Trend with HTN but not significant (p = 0.167)

Concordant with Previously published Research

on Incidence late after coarctation repairCook et. al. Congenit Heart Dis. 2012 Dec 27. doi:

10.1111/chd.12024. [Epub ahead of print]

Coarctation - Facts• Associated with diffuse arteriopathy of varying

degree – Abnormal vascular reactivity in many

– 70-85% with associated Bicuspid Aortopathy

– Medial necrosis of aorta in many

– Intracranial Berry aneurysms incidence 3-5 x normal population

– Increased arterial and central aortic stiffness in majority

• Older age of repair associate with increase HTN

late ( > 5 y/o)

• Young age ( < 12 m/o) associated with

increased risk of renarrowing

Professional Society Guidelines of when to treat

ESC Guidelines 2010CATCHNET

Guidelines 2009AHA Guidelines

2008

Guidelines Follow up Recommendations

ESC 2010

1. Minimum follow up every 2 years in GUCH center

2. Treat of HTN, exertional HTN itself is debated

3. Regular CT/MR/angiography of aorta

4. Be aware of Berry aneurysm risk, but do not rec. Routine screening

5. No SBE afer 6 mo

CATCHNET 2009

1. Regular F/U with ACHD cardiologist

2. Periodic imaging of the aorta (MRI preferred, CT with devices or stents)

3. Clinically assess for arm to leg gradient, headaches, valve function

4. Do not address routine CNS aneurysm screening

5. No SBE

1. Yearly ACHD f/u if surgical or catheter repair

2. Careful appearance of resting or exercise HTN

3. MRI/CT imaging minimum Q 5 yrs

4. Mention CT/MR of brain

5. Regular stress testing (IIb)

AHA 2008

Coarctations - Conclusion• Regular follow up remains critical !!!

• Younger surgical intervention may affect the late complication incidence

– ? Lower late HTN in adults in the future

– Possible increase in recurrent CoA

• Newer less invasive approaches maybe available, with new safer devices world wide soon

– Need longer term f/u

– Should compare surgery to Stenting more carefully.

• Further research in ICA screening needed

• Larger registry and multi-center trials helping to increase statistical power to provide more information

– NCDR IMPACT registry : First abstracts at ACC 2013

Tetralogy of Fallot (TOF)

Tetralogy of Fallot(MOST COMMON CYANOTIC LESION)

1. Pulmonary and

subpulmonary

stenosis

2. Conal septal VSD

3. Aortic over ride of

RV

4. RV hypertrophyRA

RV

LA

LV

First performed in 1945

Benefits:

– Pressure restrictive (prevents

PVOD)

– Controlled pulmonary blood

flow (less CHF)

Problems:

– Prone to thrombosis

– Prone to kinking

– “Steal” phenomenon from

vertebral artery

– Long term decreased BP in

ipsilateral arm

Blalock-Taussig Shunt (classic)

“Complete Repair”Trans-annular patch

• Trans-annular incision & Subvalular resection

• Patch closure of VSD


Repair of choice

Usually done in infancy

Problems:

– Pulmonary insufficiency with

late RV Failure (need muliple

PV replacements in future)

– Late arrhythmias (VT, Afib,

heart Block)

Transannular “Repair”


Used in cases of coronary artery crossing the RVOT

Problems:

– Conduits don’t grow

– Prone to stenosis

– Even when placed in adults, last only 15-20 years then the valve wears out.

– Important to document type and size of valve

Conduit Repair

Late Survival of TOF

Cuypers JA. Circulation.2014;130:1944-53

Indication for PVR in Adults Repaired TOF

Effect of isolated PR: Symptoms

Shimasaki et al, TCVS 1984

72 patients


Effect of PR post TOF repair: Exercise capacity

Carvalho et al, Heart 1992

12 patients

12 years old


Mortality of PVR

Oeschlin et al, JTCVS 1999 Yemets et al, ATS 1997

10 year actuarial survival 95 ± 3%

60 patients

33 years old

85 patients

9 years old


Effect of PVR on Symptoms

Discigil et al, J Thorac Cardiovasc Surg 2001


Longevity of PVR

Yemets et al, ATS 1997

86 ± 7% at 10 years85 patients

9 years old

• 16 or 18mm Modified Contegra Bovine Jugular Vein Valve

• Platinum Iridium Frame– 28mm length

– Crimped down to 6mm, re-expanded 18mm up to 22mm

TPV ProgramMelody® Transcatheter Pulmonary Valve

Melody®

TPV is Intended to:

• Relieve conduit stenosis without inducing regurgitation

• Restore and maintain pulmonary valve competence

… with the goal of extending RV to PA conduit life

Event Free survival late after TOF repair


Copyright ©2010 American Heart Association

Khairy, P (AARCC investigators). Circulation 2010;122:868-875

Prevalence of Tachyarrhythmias in Surgically repaired Tetralogy of Fallot According to Age (USA)

Arrhythmia Burden Late After TOF (Netherlands)


. 6

Toronto 70 pt TOF cohort – Arrhythmias Pre & Post PVR

Change in incidence of clinical

arrhythmia after PVR. AF/fib

indicates atrial flutter or

fibrillation; VT, ventricular

tachycardia. Dashed area

represents de novo arrhythmia

after PVR.

Circulation. 103(20):2489-2494, May 22, 2001.

Difference in VT s/p PVR with and without RVOT Cryoablation

Freedom from recurrent preexisting atrial flutter or monomorphic ventricular tachycardia after PVR. Solid

line represents patients with preoperative arrhythmia who underwent concomitant intraoperative

ablation therapy. Dashed line represents patients with preoperative arrhythmia who did not undergo

concomitant intraoperative cryoablation therapy.

Circulation. 103(20):2489-2494, May 22, 2001.

Complete Transposition of the Great Vessels

Complete Transposition of the

Great Arteries - Definitions• D-TGA

• Atrial to ventricular concordance with ventricular to arterial discordance

• Complete Transposition of the Great Arteries

• Simple TGA – No associated defects

• Complex TGA –Associated defects (VSD, PS, etc)

RA

RV

AO

PA

LA

LV

Historical Treatments For

Complete Transposition• 1950 – Alfred Blalock & C Rollins Hanlon develop

surgical atrial septectomy• 1959 – Ake Senning developed the atrial switch with

autologous material

• 1964 – William Mustard developed the atrial switch with prosthetic material

• 1966 – William Rashkind developed the balloon atrial septostomy

• Mid 1970’’’’s – Prostaglandin E1 accepted as routine. • 1975 – Adib Domingos Jatene performed the arterial

switch operation (ASO)

• Mid 1980’’’’s – ASO adopted as the surgery of choice (2 stage if performed after 2 weeks of age

Atrial Switch(Senning & Mustard Procedure)

PVA

RV

AO

PA

SVA

LV

Overall Survival After Mustard’s Palliation

Gelatt M, JACC 21(1)1997:194-201

Roos-Hesselink, J.W et al. Eur Heart J 2004 25:1264-1270;

Long Term Survival and Event Free

Survival With Need of Intervention

Atrial Switch – Late Complications

1. Systemic Right ventricular dysfunction

2. Tricuspid valve (systemic AV valve)

regurgitation

3. LVOT obstruction

4. SVC and IVC baffle obstruction

5. Baffle leak

6. Sick sinus syndrome

7. Re-entry atrial tachycardias

8. Sudden death

Complication rates for Mustard –vs- Senning

Khairy P., Cariol Young 2004;14:284-292

Incidence of Baffle Complications

(Mustard)

0

10

20

30

40

50

60

70

Roos-

Hesselink

et. al.

Hagler et. Al Takahashi

et. Al.

Baffle Obstruction

Baffle Leak

Perc

en

tag

e

Roos-Hesselink J.W., Eur Heart Jour. 2004;25,1264-1270

Hagler D., Circulation. 1978; 57(6); 1214-1219

Takahashi M., Circulation supp2. 1977 56(3) 11-85-90

Sinus Node Dysfunction

Gelatt M, JACC 21(1)1997:194-201

Risk Of Re-Entry Atrial Tachycardia

Over Time After the Mustard

Gelatt M, JACC 21(1)1997:194-201

Arterial Switch Operation

Adib Domingos Jatene

Surgery of the Chest, 5th ed. 1990. W,B Saunders

Late Problems After ASO

1. Coronary Problems

2. Supra-valvular Pulmonary Artery Stenosis

3. Aortic Root dilation/Aortic Insufficiency

4. ? Arrhythmis

New Anatomic Problems for ASO

The Coronary Artery Anatomy

Wernovsky G., Coronary Artery Dis 1993;4:148-157

Coronary Patterns of Risk

Pasquali SK., Circulation.2002;106:2575-2580

Pulmonary Artery Stenosis

• Incidence reported from 10-14%

• Severe obstruction requiring intervention 4-5%

• Included supra valvular and branches

• Branches “stretched” over the Ascending aorta (? Long term effect into adulthood)

Losay J. Circulation. 2001;104(supp I):I-121-I-126

Sidi D. Circulation. 1987;2, 429-435

Wernovsky G. Circulation 1988; 6, 1333-1344

Value Based Care:When all is said and done do

ACHD Programs Impact ACHD Patient Outcomes?

Answer: Yes !

(Assuming Programs are as good as those in Quebec)

Referrals to ACHD Centers –Quebec 1990 - 2005

Darren Mylotte et. al. Circulation. published online March 3, 2014;

Hazard Risk on Mortality of ACHD patients in Quebec

Darren Mylotte et. al. Circulation. published online March 3, 2014

ACHD Survival Based Referral

Conclusions

• Expansion of Dedicated ACHD programs is

needed to meet the growing need of moderate

and complex ACHD survivors

• Complete Care for these patients requires a

variety of providers, not just the ACHD

cardiologist

• Such programs REDUCE MORTALITY !!!

• ACHD teams need to reach out to Community

providers to get this folks into appropriate care

Buy in from both Pediatric & Adult

Programs is CriticalUniversity of Colorado

HospitalChildren’s Hospital

Colorado

aaim15 - congenital heart disease...• describe the commonly encountered forms of adult congenital...

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