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