1. RDB Jaquiss Duke University
Is “Four Stage Management” the Future of Univentricular Hearts? – Destination Therapy in the Young
RDB Jaquiss
Duke University

2. Disclosures
None

3. Current Paradigm for Palliation
Stage I – shunt/band PROTECT PVR
Stage II – superior CPC RELIEVE VOLUME LOAD
Stage III – Fontan completion RELIEVE CYANOSIS
If (when?) palliation fails - transplant

4. Normal
 (A) The normal cardiovascular circulation. The pulmonary circulation (P) is connected in series with the systemic circulation (S). The right ventricle maintains the right atrial pressure lower than the left atrial pressure, and provides enough energy to the blood to pass the pulmonary resistance. (B) The patient with a univentricular heart. The systemic and pulmonary circuits are connected in parallel, with a considerable volume overload to the single ventricle (V). The width of the line reflects the degree of volume load. There is complete admixture of systemic and pulmonary venous blood, causing arterial oxygen desaturation. (C) The Fontan circulation. The systemic and pulmonary circulations are connected in series. The right atrium (RA) or systemic veins are connected to the pulmonary artery (PA). The volume overload to the single ventricle is now less than expected for body surface area. In the absence of fenestration, there is no more admixture of systemic and pulmonary venous blood, but the systemic venous pressure is notably elevated. Ao; aorta; LA, left atrium; LV, left ventricle, RV, right ventricle.

5. Single Ventricle
 (A) The normal cardiovascular circulation. The pulmonary circulation (P) is connected in series with the systemic circulation (S). The right ventricle maintains the right atrial pressure lower than the left atrial pressure, and provides enough energy to the blood to pass the pulmonary resistance. (B) The patient with a univentricular heart. The systemic and pulmonary circuits are connected in parallel, with a considerable volume overload to the single ventricle (V). The width of the line reflects the degree of volume load. There is complete admixture of systemic and pulmonary venous blood, causing arterial oxygen desaturation. (C) The Fontan circulation. The systemic and pulmonary circulations are connected in series. The right atrium (RA) or systemic veins are connected to the pulmonary artery (PA). The volume overload to the single ventricle is now less than expected for body surface area. In the absence of fenestration, there is no more admixture of systemic and pulmonary venous blood, but the systemic venous pressure is notably elevated. Ao; aorta; LA, left atrium; LV, left ventricle, RV, right ventricle.

6. Fontan Circulation
 (A) The normal cardiovascular circulation. The pulmonary circulation (P) is connected in series with the systemic circulation (S). The right ventricle maintains the right atrial pressure lower than the left atrial pressure, and provides enough energy to the blood to pass the pulmonary resistance. (B) The patient with a univentricular heart. The systemic and pulmonary circuits are connected in parallel, with a considerable volume overload to the single ventricle (V). The width of the line reflects the degree of volume load. There is complete admixture of systemic and pulmonary venous blood, causing arterial oxygen desaturation. (C) The Fontan circulation. The systemic and pulmonary circulations are connected in series. The right atrium (RA) or systemic veins are connected to the pulmonary artery (PA). The volume overload to the single ventricle is now less than expected for body surface area. In the absence of fenestration, there is no more admixture of systemic and pulmonary venous blood, but the systemic venous pressure is notably elevated. Ao; aorta; LA, left atrium; LV, left ventricle, RV, right ventricle.

7. Fontan Circulation
 (A) The normal cardiovascular circulation. The pulmonary circulation (P) is connected in series with the systemic circulation (S). The right ventricle maintains the right atrial pressure lower than the left atrial pressure, and provides enough energy to the blood to pass the pulmonary resistance. (B) The patient with a univentricular heart. The systemic and pulmonary circuits are connected in parallel, with a considerable volume overload to the single ventricle (V). The width of the line reflects the degree of volume load. There is complete admixture of systemic and pulmonary venous blood, causing arterial oxygen desaturation. (C) The Fontan circulation. The systemic and pulmonary circulations are connected in series. The right atrium (RA) or systemic veins are connected to the pulmonary artery (PA). The volume overload to the single ventricle is now less than expected for body surface area. In the absence of fenestration, there is no more admixture of systemic and pulmonary venous blood, but the systemic venous pressure is notably elevated. Ao; aorta; LA, left atrium; LV, left ventricle, RV, right ventricle.

8. How Well and How Long Does It Work?
Conditional Freedom from Death or Transplant
5 years
94%
10 years
90%
15 years
87%
20 years
83%

9. A Very Large and Excellent Experience
Conditional Survival
Atriopulmonary
25 years
76%
Lateral Tunnel
20 years
90%
Extracardiac
13 years
97% 2

10. A Closer Look Survival Isn’t Everything

11. Problems with Fontan Circulation
Low Cardiac Output (70% of Normal at Rest)
Impaired Exercise Capacity
pre-load (PVR) determined
Thromboembolism
Arrythmias
Hepatopathy
Nephropathy
Death

12. Developments in Fontan Surgery
Description by Fontan and Baudet
Ten Commandments
Lateral Tunnel
Extracardiac
Staging
Fenestration
Fontan conversion
Intra/Extra
“Y” Grafts

13. (A) The normal cardiovascular circulation
 (A) The normal cardiovascular circulation. The pulmonary circulation (P) is connected in series with the systemic circulation (S). The right ventricle maintains the right atrial pressure lower than the left atrial pressure, and provides enough energy to the blood to pass the pulmonary resistance. (B) The patient with a univentricular heart. The systemic and pulmonary circuits are connected in parallel, with a considerable volume overload to the single ventricle (V). The width of the line reflects the degree of volume load. There is complete admixture of systemic and pulmonary venous blood, causing arterial oxygen desaturation. (C) The Fontan circulation. The systemic and pulmonary circulations are connected in series. The right atrium (RA) or systemic veins are connected to the pulmonary artery (PA). The volume overload to the single ventricle is now less than expected for body surface area. In the absence of fenestration, there is no more admixture of systemic and pulmonary venous blood, but the systemic venous pressure is notably elevated. Ao; aorta; LA, left atrium; LV, left ventricle, RV, right ventricle.
Marc Gewillig Heart 2005;91:

14. “…pattern of endothelial dysfunction-induced remodeling due to chronic, non-pulsatile flow with in situ thrombotic lesions.”

15. Fontan Circulation
 (A) The normal cardiovascular circulation. The pulmonary circulation (P) is connected in series with the systemic circulation (S). The right ventricle maintains the right atrial pressure lower than the left atrial pressure, and provides enough energy to the blood to pass the pulmonary resistance. (B) The patient with a univentricular heart. The systemic and pulmonary circuits are connected in parallel, with a considerable volume overload to the single ventricle (V). The width of the line reflects the degree of volume load. There is complete admixture of systemic and pulmonary venous blood, causing arterial oxygen desaturation. (C) The Fontan circulation. The systemic and pulmonary circulations are connected in series. The right atrium (RA) or systemic veins are connected to the pulmonary artery (PA). The volume overload to the single ventricle is now less than expected for body surface area. In the absence of fenestration, there is no more admixture of systemic and pulmonary venous blood, but the systemic venous pressure is notably elevated. Ao; aorta; LA, left atrium; LV, left ventricle, RV, right ventricle.

16. Modes of Fontan Failure
Pump failure (LV<RV)
Intrinsic, iatrogenic, arrythmic, valvular
Concept failure
“high”PVR leading to high Fontan pressure
PLE, plastic bronchitis
Cyanosis (V-V collaterals)

17. Scope of Problem STS Congenital Database 7/2010 to 6/2014
3245 “Fontan” operation (excluding conversions)
810/year entering pipeline
Scientific Registry of Transplant Recipients

18. Total Heart Transplants – U.S.

20. 47% of patients were listed as Status 2 (i.e. high PVR failure)
Status 2 – 10% waitlist mortality Status 1 (inotropes) – 40% waitlist mortality Status 1 (ventilator) – 70% waitlist mortality

21. Changing Profile of Adult Heart Transplant 2002 and 2012%
Urgency
Status 1A
660
34.8
1190
58.5 1B
723
38.2
743
36.5 2
509
26.9
102
5.0
Bridge
No VAD
1454
76.8
1194
58.7
VAD
440
23.2
841
41.3

24. Application of LVAD Pump failure (LV<RV) Concept failure
Intrinsic, iatrogenic, arrythmic, valvular
Concept failure
“high”PVR leading to high Fontan pressure
PLE, plastic bronchitis
Cyanosis (V-V collaterals)

25. “As many as two thirds of adult Fontan patients who die or require transplantation do so with preserved ventricular function.”

26. LVAD for Concept Failure – “Pulling”

27. Concept Failure – “Pushing”
“Fontan patients are doomed to a circulatory failure and many of them will require a circulatory assistance as a bridge to transplantation.”

28. RVAD vs. LVAD Pushing vs. Pulling
Power Requirements X
0.25 X
Size
Small
Smaller
Embolic Consequences
Very Bad
Bad
Duarbility
Unknown
Unknown+++

31. Fig. 2 Aortic flow and superior vena cava (SVC) pressure at various phases of the experiment (mean flow and pressure of all 4 animals). Aortic flow decreased with institution of the Fontan circulation and was restored upon activation of the VAD. SVC pressu...

34. Factors Which Will Impact Future

36. Right Heart Failure after LVAD
~10-15% temporary RVAD
~20-30% moderate RVF
Worse survival DT
Worse outcome BTT
Worse QOL

38. “It's tough to make predictions, especially about the future.”
Yogi Berra

39. Transplant vs. Fontan-RVAD
Resting Cardiac Output
Normal
Exercise Cardiac Output
Hepatic Venous Pressure
Normal to Slightly Elevated
Chronic Anticoagulation No
Yes
Power Cord
Probably
Immunosuppression Risks
Diabetes, Hypertension, Renal Failure
Additional Surgery Needed
Yes (Redo Tx)
Yes (Redo VAD or Tx)

40. Future Paradigm for Palliation
Stage I – shunt/band PROTECT PVR
Stage II – superior CPC RELIEVE VOLUME LOAD
Stage III – Fontan completion RELIEVE CYANOSIS
SET UP STAGE IV
Stage IV
A. Pump Failure leads to transplant
B. Concept Failure leads to RVAD (timing?)