Upload
others
View
1
Download
0
Embed Size (px)
Citation preview
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
Brown ML et al., JACC 2013 (62):1020-25
Freedom from reoperation based
on age and Coarctation Repair
Brown ML et al., JACC 2013 (62):1020-25
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
Brown ML et al., JACC 2013 (62):1020-25
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
First performed in 1955
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”
First performed in 1965
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
Indication for PVR in Adults Repaired TOF
Effect of PR post TOF repair: Exercise capacity
Carvalho et al, Heart 1992
12 patients
12 years old
Indication for PVR in Adults Repaired TOF
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
Indication for PVR in Adults Repaired TOF
Effect of PVR on Symptoms
Discigil et al, J Thorac Cardiovasc Surg 2001
Indication for PVR in Adults Repaired TOF
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
Cuypers JA. Circulation.2014;130:1944-53
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)
Cuypers JA. Circulation.2014;130:1944-53
. 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