MCS in Special Populations:The Use of Mechanical Support in
Adults with Congenital Heart Disease
9th Annual Meeting May 15, 2015
Christina VanderPluym,MDMedical Director VAD Program
Boston Children’s Hospital
Relevant Financial Relationship Disclosure Statement
I have no significant or relevant financial disclosures related to the content of this presentation.
Christina VanderPluym M.D..
The Unknowns of VAD support for ACHD
1. How many adults with CHD need mechanical circulatory support? Who needs it and why?
2. How many adults with CHD are being supported on MCS as BTT, BTR and DT?
3. What are the outcomes of different variants of CHD with MCS as BTT and DT? Is DT possible for ACHD?
4. What are the obstacles and challenges to MCS in adults with CHD? Are they surmountable?
The Unknowns of VAD support for ACHD
1. How many adults with CHD need mechanical circulatory support? Who needs it and why?
2. How many adults with CHD are being supported on MCS as BTT, BTR and DT?
3. What are the outcomes of different variants of CHD with MCS as BTT and DT? Is DT possible for ACHD?
4. What are the obstacles and challenges to MCS in adults with CHD? Are they surmountable?
Adult Congenital Heart Disease• Wide range of hemodynamic abnormalities:
– Biventricular pressure and volume overload lesions (d-TGA, L-TGA, AVSD, TOF, etc.)
– Univentricular failure (palliated Fontan circulation)• Multiple clinical presentations:
1. Diagnosed and palliated in childhood2. Diagnosed in adulthood with late sequelae of untreated
hemodynamic abnormalities• Lesions specific complications and natural
morbidities of aging (hypertension, diabetes, hyperlipidemia)
Scope of the Problem?• Prevalence of ACHD in difficult to characterize
– No database of systematically collected population data, CDC currently funding 3 pilot projects for ACHD surveillance
• Based on Canadian studies, est. ~850 000 adults with CHD in US in 2000, with 5% increase annually, resulting in >1.3 million US adults by 2014
• Currently, more adults with CHD than children• Unclear what percentage of these patients progress to
end stage heart failure necessitating MCS [ Hoffman et al. Am Heart J. 2004, Bhatt et al. Circ 2015]
Incidence of heart failure in adults with CHD after cardiac surgery
[Norozi et al. Am J Cardiol 2006]
The Unknowns of VAD support for ACHD
1. How many adults with CHD need mechanical circulatory support? Who needs it and why?
2. How many adults with CHD are being supported on MCS as BTT, BTR and DT?
3. What are the outcomes of different variants of CHD with MCS as BTT and DT? Is DT possible for ACHD?
4. What are the obstacles and challenges to MCS in adults with CHD? Are they surmountable?
How many ACHD are being supported on MCS?
• OPTN SRTR data from 1987-2012– 47 160 adult transplant recipients, 1213 (2.6%) had CHD– Proportion of ACHD transplants recipients relative to all
adult transplant increased yearly, with concomitant increase in MCS for ACHD yearly
– MCS used in 83 patients (6.8%) with CHD as compared to 8625 (18.8%) patients without CHD
– No difference in 30d mortality between MCS and non MCS patients with ACHD, but both had higher short term mortality than adults without CHD
[Maxwell et al. European J of Cardio-thoracic Surg 2013]
Proportion of ACHD transplants relative to alltransplants
Proportion of ACHD transplants supportedWith MCS relative to all ACHD
30d mortality rate by year of transplant for all ACHD patients
[Maxwell et al. European J of Cardio-thoracic Surg 2013]
DATA SUMMARY 2008-2014 ACHD AND MCS
Intermacs DataInclusions:
Primary LVAD/BiVAD/ TAH Implants
Adults (age ≥ 19 years at implant)
Implant dates: April 21, 2008 – December 31, 2014
Follow-up date: December 31, 2014
Study Group Patients: 13288
Patient years: 16730.83
Total deaths with a device in place: 3638
Total heart transplants: 3864
Total device removal due to recovery: 171
Mean Follow-up (Months): 15.11
Total number of contributing hospitals: 154
Distribution of CHD and no CHDby device type:
Continuous and Pulsatile Devices
5.7%
Distribution of Device Type in ACHD
• Continuous Flow– 59 patients (78%)
• 95% were LVADs• 5% were BiVADs
• Pulsatile Flow– 17 patients (22%)
• 47% (8) Total artificial heart• 35% (6) BiVAD• 18% (3) LVAD
Similar distribution to adultswithout CHD
Adults without CHD had 49% with LVAD and only 24% with TAH
DemographicsAge distribution of patients with CHD and without CHD in Intermacs
• Younger population requiring VAD support as compared to adults without CHD
Distribution of Device Strategy for ACHD and non CHD patients
Survival of adults with and without CHD on all devices: June 2006- April 2014
Not Congenital, n=13212; deaths=3613
Survival of adults with and without CHD on CF device
Survival of adults with and without CHD on pulsatile flow device
The Unknowns of VAD support for ACHD
1. How many adults with CHD need mechanical circulatory support? Who needs it and why?
2. How many adults with CHD are being supported on MCS as BTT, BTR and DT?
3. What are the outcomes of different variants of CHD with MCS as BTT and DT? Is DT possible for ACHD?
4. What are the obstacles and challenges to MCS in adults with CHD? Are they surmountable?
Challenges of MCS in ACHD• Difficult to analyze and understand outcomes of VAD
support for ACHD given the heterogeneity of palliated anatomic lesions
• Physiologic burden of ACHD results in ?irreversible end organ compromise– Liver cirrhosis and coagulopathy– Ascites, compromised nutrition and cachexia with consequent poor
wound healing• Technical challenges of cannula positioning, reconfiguring
anatomy for systemic VAD (ie Fontan to Glenn)– Lessons from pediatric congenital VAD surgeons
Expanded data capture of CHD Pre-implant (primary diagnosis)
Thank you