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The Next Frontier: Ventricular Assist Devices in Children With Failing Single Ventricles


Sharon Chen, MD and Beth Kaufman, MD
Lucile Packard Children's Hospital
Stanford University School of Medicine
Stanford, CA, USA
shchen@stanford.edu bethkaufman@stanford.edu




We were honest with the parents and used terms such as "pioneering" and "innovative" to describe the operation we were offering. But we also admitted, "This surgery is still experimental. We do not know if it will work or not." Our surgeon described how he would attempt to place DG, a 1 year old with a failing single ventricle, palliated to a bidirectional cavopulmonary anastomosis, onto a ventricular assist device (VAD). The procedure would involve an extensive reconstruction of the SVC and pulmonary artery, placing a modified shunt, followed by cannulation and connection to a rotary pump. He explained the risk of anesthesia and cardiac bypass. But as for the likely success of supporting a child with a failing single ventricle with a VAD? That was much more difficult to explain and quantify.

Over a 25-year period, from 1988 - 2013, there have been 16 published cases of children with single ventricular physiology supported with VADs (Table 1). Most cases involved pulsatile devices, a variety of surgical palliations, and support time of 2 to 363 days. Of these 16 reported cases, there was an overall survival of 63% (10/16). Encouraging, yet one must speculate about the number of similar unpublished cases whom were likely unsuccessful.

Weinstein and investigators examined the Berlin EXCOR Pediatric Investigational Device Exemption study database and identified 26 children with single ventricle anatomy implanted with the Berlin EXCOR [1]. Eleven of the 26 (42%) were successfully bridged to transplant and 11 died on EXCOR support. The remaining 4 were transitioned to ECMO due to failure of the VAD to provide adequate support and all four died within 30 days. In comparison, 185 of 255 (72%) biventricular patients supported with the EXCOR were bridged to transplant or recovery during the same time period.

Ten years ago, in 2004, our institution performed one of the first pediatric Berlin EXCOR device implantations in the United States in MC, a 5-month-old with cardiomyopathy. The device had been used in Europe, however the surgeons at our institution had yet to perform this procedure in such a small infant, nor did the medical team previously manage an infant VAD. The device, ordered from Germany, arrived with labels and manuals ... printed in German. Our conversation back then with MC's parents was probably not too different from our recent conversation with DG's parents. We are offering a pioneering, innovative therapy for your child. We do not have much experience with this. The outcome is uncertain, and he could die or suffer a devastating complication. Would you like us to proceed?

According to the 2013 ISHLT annual report, 20% of pediatric heart transplant recipients were bridged with VADs or total artificial hearts [2]. Today, we can tell families that 75% of children on VADs are successfully bridged to transplant or recovery [3] and that postoperative survival is comparable to overall pediatric heart transplant survival [4]. Ten years ago, when we first started implanting VADs in children, we did not have these assurances.

Today, we are at a similar threshold, pushing the current boundaries with new applications of VAD to support children with univentricular heart disease. The challenges related to VAD support for this population are unique due to the complexity of single ventricle physiology and heterogeneous anatomy. VADs were well established in adults with biventricular circulation prior to application to children. In contrast, there are no larger population studies for single ventricle mechanical circulatory support.

Our current success in pediatric VAD support is due in part to families willing to accept the risks of a novel therapy and to medical teams willing to offer pioneering and innovative technology. The distinction between research and innovation may be challenging at these times, particularly without the infrastructure of a clinical trial. It is therefore imperative that we continue to share our successes and, perhaps more importantly, our failures with VAD support for children with single ventricle circulations, to inform the way.


TABLE 1. Case reports of pediatric single ventricle patients supported with VAD

Author

Year

Age (yrs)

Anatomy

Surgery

Device

Duration
(days)

Outcome

Lal (5)

2013

1.2

HLHS (MS/AA)

Glenn→mBT shunt

Berlin then Revolution pump

65

OHT

Brancaccio (6)

2013

2

HLHS (MA/AA)

Glenn

Berlin

2

OHT (sudden death at 6mo)

4

DIRV

Glenn

Berlin

166

Died (thromboembolic complications)

Mackling (7)

2012

4

DORV

Fontan

Berlin

363

Died (sepsis)

4

HLHS (MS/AS)

Glenn

Berlin

270

Died (resp failure)

VanderPluym (8)

2011

3

HLHS (MA, TGA)

Fontan→Glenn

Berlin

174

OHT

Pearce (9)

2009

1.3

DORV/MA/d-TGA

Central shunt

Berlin

49

OHT

Cardarelli (10)

2009

1.5

HLHS

Fontan

Berlin

179

Decannulation

Irving (11)

2009

2.9

HLHS

Glenn

Berlin

7

OHT

Chu (12)

2007

4

HLHS

Glenn

Berlin

13

Died

Calvaruso (13)

2007

10

Fontan

Berlin

7

OHT

Nathan (14)

2006

4

HLHS

Fontan

Berlin

28

OHT (died graft failure)

Frazier* (15)

2005

14

TA

Fontan

Centrifugal pump then HM I

45

OHT

Sadeghi (16)

2000

8

HLHS

Fontan

BVS 5000 (pulsatile)

8

OHT

Matsuda (17)

1988

10

Fontan

Toyobo (pulsatile)

7

Died

3

BT shunt

Toyobo (pulsatile)

5

Died

* Also reported by Russo 2008 (18)
HLHS hypoplastic left heart syndrome; MS mitral stenosis; AA aortic atresia; MA mitral atresia; AS aortic stenosis; DIRV double inlet right ventricle; DORV double outle right ventricle; TGA transposition of great arteries; TA tricuspid atresia; OHT orthotopic heart transplant

Disclosure Statement: the authors have no disclosures.


References:

  1. Weinstein S, Bello R, Pizarro C, et al.: The use of the Berlin Heart EXCOR in patients with functional single ventricle. The Journal of thoracic and cardiovascular surgery 2014;147:697-705.
  2. Dipchand AI, Kirk R, Edwards LB, et al.: The Registry of the International Society for Heart and Lung Transplantation: sixteenth official pediatric heart transplantation report--2013; focus theme: age. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2013;32:979-88.
  3. Almond CS, Morales DL, Blackstone EH, et al.: Berlin Heart EXCOR pediatric ventricular assist device for bridge to heart transplantation in US children. Circulation 2013;127:1702-11.
  4. Eghtesady P, Almond CS, Tjossem C, et al.: Post-transplant outcomes of children bridged to transplant with the Berlin Heart EXCOR Pediatric ventricular assist device. Circulation 2013;128:S24-31.
  5. Lal AK, Chen S, Maeda K, et al.: Successful bridge to transplant with a continuous flow ventricular assist device in a single ventricle patient with an aortopulmonary shunt. ASAIO J 2014;60:119-21.
  6. Brancaccio G, Gandolfo F, Carotti A, et al: Ventricular assist device in univentricular heart physiology. Interact Cardiovasc Thorac Surg 2013;16:568-9.
  7. Mackling T, Shah T, Dimas V, et al.: Management of single-ventricle patients with Berlin Heart EXCOR Ventricular Assist Device: single-center experience. Artif Organs 2012;36:555-9. 8. VanderPluym CJ, Rebeyka IM, Ross DB, Buchholz H: The use of ventricular assist devices in pediatric patients with univentricular hearts. J Thorac Cardiovasc Surg 2011;141:588-90.
  8. Pearce FB, Kirklin JK, Holman WL, et al: Successful cardiac transplant after Berlin Heart bridge in a single ventricle heart: use of aortopulmonary shunt as a supplementary source of pulmonary blood flow. J Thorac Cardiovasc Surg 2009;137:e40-2.
  9. Cardarelli MG, Salim M, Love J, et al.: Berlin heart as a bridge to recovery for a failing Fontan. Ann Thorac Surg 2009;87:943-6.
  10. Irving CA, Cassidy JV, Kirk RC, et al: Successful bridge to transplant with the Berlin Heart after cavopulmonary shunt. J Heart Lung Transplant 2009;28:399-401.
  11. Chu MW, Sharma K, Tchervenkov CI, et al.: Berlin Heart ventricular assist device in a child with hypoplastic left heart syndrome. Ann Thorac Surg 2007;83:1179-81.
  12. Calvaruso DF, Ocello S, Salviato N, et al.: Implantation of a Berlin Heart as single ventricle by-pass on Fontan circulation in univentricular heart failure. ASAIO J 2007;53:e1-2.
  13. Nathan M, Baird C, Fynn-Thompson F, et al.: Successful implantation of a Berlin heart biventricular assist device in a failing single ventricle. J Thorac Cardiovasc Surg 2006;131:1407-8.
  14. Frazier OH, Gregoric ID, Messner GN: Total circulatory support with an LVAD in an adolescent with a previous Fontan procedure. Tex Heart Inst J 2005;32:402-4.
  15. Sadeghi AM, Marelli D, Talamo M, et al: Short-term bridge to transplant using the BVS 5000 in a 22-kg child. Ann Thorac Surg 2000;70:2151-3.
  16. Matsuda H, Taenaka Y, Ohkubo N, et al.: Use of a paracorporeal pneumatic ventricular assist device for postoperative cardiogenic shock in two children with complex cardiac lesions. Artif Organs 1988;12:423-30.
  17. Russo P, Wheeler A, Russo J, et al: Use of a ventricular assist device as a bridge to transplantation in a patient with single ventricle physiology and total cavopulmonary anastomosis. Paediatr Anaesth 2008;18:320-4.



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