http://www.jhltonline.org
ORIGINAL CLINICAL SCIENCE
An inpatient rehabilitation program utilizing standardized care pathways after paracorporeal ventricular assist device placement in children Seth A. Hollander, MD,a Amanda J. Hollander, DPT, PCS,b Sandra Rizzuto, OTD,b Olaf Reinhartz, MD,c Katsuhide Maeda, MD,c and David N. Rosenthal, MDa From the aDepartment of Pediatrics (Cardiology), Stanford University Medical Center; bRehabilitation Services, Lucile Packard Children's Hospital; and the cDepartment of Cardiovascular Surgery, Stanford University Medical Center, Palo Alto, California.
KEYWORDS: pediatrics; heart transplant; rehabilitation; physical therapy; occupational therapy; ventricular assist device
BACKGROUND: Structured rehabilitation programs in adults after ventricular assist device (VAD) placement result in improvements in physical function and exercise capacity, and have been shown to improve survival and accelerate post-transplant recovery. The objective of this study was to determine the safety and feasibility of an acute inpatient rehabilitation program for children utilizing standardized, age-appropriate, family-centered care pathways after paracorporeal VAD placement in both the ICU and acute-care inpatient settings. METHODS: Between November 12, 2010 and March 15, 2013, 17 patients were referred to therapy after VAD implantation, 14 of whom were medically stable enough to participate. Beginning in the ICU, a structured physical and occupational therapy program was implemented utilizing novel age-appropriate, standardized care pathways for infants (age o1 year) and children (age 1 to 12 years). The infant and child pathways consisted of 8 and 10 goals, respectively. Retrospective review was conducted to ascertain the number of phases achieved per patient. Adverse events, defined as bleeding, physiologic instability, stroke, or device disruption during therapy, were also analyzed. RESULTS: The median age was 1.1 (range 0.5 to 14.4) years in the 14 patients considered medically stable enough to participate in rehabilitation. Nine of them were female. Eight patients participated in the infant standardized care pathway (SCP) and 6 participated in the child SCP. Seven patients were on biventricular support. Twelve patients were transplanted and survived. Two patients died while awaiting transplantation. There were 1,473 total days on the VAD (range 40 to 229 days). The median time to extubation was 2 days (range 1 to 8) and the median ICU stay was 6.5 days (range 3 to 152). Eleven patients achieved all goals of the SCP, including all of the patients in the child group. For the infant group, 5 patients achieved all goals of the SCP (range 5 to 8), and all but 1 patient achieved at least 7 goals of the SCP. There were no adverse events related to therapy. CONCLUSIONS: Standardized, family-centered inpatient rehabilitation care paths are safe for infants and children after paracorporeal device placement. Structured rehabilitation goals can be achieved by the majority of pediatric patients during VAD support. Early mobilization and inpatient rehabilitation in this cohort promotes normalization of function while awaiting cardiac transplantation. J Heart Lung Transplant ]]]];]:]]]–]]] r 2014 International Society for Heart and Lung Transplantation. All rights reserved.
Reprint requests: Seth A. Hollander, MD, Department of Pediatric Cardiology, Lucile Packard Children’s Hospital, Stanford University Medical Center, 750 Welch Road, Suite 321, Palo Alto, CA 94304. Telephone: 650-736-8600. Fax: 650-497-8422. E-mail address: [email protected]
Each year, approximately 400 pediatric heart transplants are performed worldwide.1 With an increased demand for donor hearts, but a relatively stagnant donor supply, ventricular assist devices (VADs) in children allow for
1053-2498/$ - see front matter r 2014 International Society for Heart and Lung Transplantation. All rights reserved. http://dx.doi.org/10.1016/j.healun.2013.12.009
2
The Journal of Heart and Lung Transplantation, Vol ], No ], Month ]]]]
pediatric patients with end-stage heart failure to survive longer while waiting for an appropriate donor organ to become available.2–5 At present, the Thoratec PVAD and the Berlin Heart EXCOR are the most commonly used devices for infants and children. Both devices consist of paracorporeal pneumatic pumps that require tethering to a large driver, and neither are suitable for outpatient use. As a result, children who require mechanical ventricular support are subject to prolonged hospital stays, which exposes them to significant morbidity, including physical deconditioning, deficits in activities of daily living, delays in cognitive and social development, and depression.6,7 Early management of VAD patients has frequently consisted of prolonged intubations and long-term stays in the intensive care unit (ICU) often accompanied by bed-rest and limitations in physical activity, despite the documented benefits of early mobilization and rehabilitation after other forms of cardiac surgery.8,9 As experience with management of patients with VADs has increased over time, early mobilization and ambulation is now increasingly recognized as a critical component of patient recovery after VAD implantation.10,11 Structured rehabilitation programs in adults after VAD placement have been shown to result in improvements in physical function and exercise capacity, as well as improved survival and accelerated post-transplant recovery.12–15 Despite the challenges of pre-existing deconditioning from congestive heart failure (CHF), fixed cardiac output, and attachment to mechanical equipment, including ventilators and VAD percutaneous drive-lines, the safety of early mobilization and intense rehabilitation in adults after VAD implantation, starting in the ICU setting, is also well established.16,17 The adult rehabilitation experience, however, cannot necessarily be extrapolated to the pediatric population, as there are unique challenges to the rehabilitation of children after VAD implantation. Whereas adults have the option of receiving the HeartMate II, an intracorporeal continuousflow device, most children who require mechanical support require placement of pulsatile paracorporeal devices, such as the Thoratec PVAD or Berlin Heart EXCOR, presenting the challenge of needing to work around externalized pumps and cumbersome medical equipment. Furthermore, pulsatile paracorporeal devices carry an increased risk of thrombosis and thromboembolism, thereby requiring higher levels of anti-coagulation while in use. Neurologic dysfunction has been observed in 29% of Berlin Heart recipients.2 Last, patients’ unwillingness to follow directions and parental hesitation can further compound the risk of accidental extubation, falls and device disruption. One patient death secondary to accidental device dislodgement during play has been reported.2 Despite increasing recognition of its importance, the safety and feasibility of implementing an inpatient rehabilitation program in pediatric paracorporeal VAD recipients has not been systematically studied. In November 2010, we developed a comprehensive interdisciplinary care program that included standardized, age-appropriate, family-centered rehabilitation care paths for pediatric VAD patients awaiting heart transplantation. The goal of the program was to
provide safe and effective inpatient rehabilitation for both the purposes of conditioning as well as supporting normal development. The objective of the present study was to determine the safety and feasibility of an acute inpatient rehabilitation program for children utilizing age-appropriate, family-centered, standardized care pathways after paracorporeal VAD placement in both the ICU and acute-care inpatient settings.
Methods A retrospective review was conducted on all patients who received paracorporeal VADs between the initiation of the standardized care pathway (SCP) on November 12, 2010 and March 15, 2013. Seventeen patients received pulsatile paracorporeal VADs during the study period, 14 of whom were medically stable enough to participate in the SCP. All patients remained as inpatients until transplant.
Therapeutic strategy Beginning in the ICU prior to extubation and continuing until the time of transplant, a structured physical and occupational therapy intervention was implemented utilizing novel, age-appropriate SCPs for infants (o1 year) and children (age 1 to 12 years). The SCP focused on therapeutic regimens individualized for patient’s age, abilities, interests and temperament. Rehabilitative exercises were woven into normal activities (e.g., obstacle courses and modified sports to improve balance and motor planning, stimulating floor-play to promote infant development). During 4 or 5 1hour sessions per week, patients received intensive therapy by specially trained physical and occupational therapists. In general, one therapist was exclusively responsible for ensuring VADs, lines and tubes did not dislodge, whereas the second therapist supervised therapeutic activities. In addition, the nursing staff and parents were trained and expected to provide rehabilitation throughout the day as prescribed by the rehabilitation team. A goal was considered achieved when the patient was observed to perform that activity consistently according to the rehabilitation staff.
Standardized care pathway The infant and child pathways consisted of 8 and 10 goals, respectively, which focused on implementing a daily schedule, therapeutic exercise and activities, family participation and off-unit excursions (Tables 1 and 2). Toddlers who were not yet toilettrained were expected to complete 9 goals of the child pathway, minus toilet transfers. Patients who were too medically unstable to tolerate therapy were considered ineligible for the SCP.
Adverse events Adverse events were defined as falls, pre-syncope, syncope, accidental extubation, bleeding, hemodynamic instability, stroke, or device disruption during or as a direct result of therapy.
Analysis Retrospective chart review was conducted to ascertain the number of phases achieved per patient. In addition, baseline demographics, survival data, total VAD days and time between transplant and
Hollander et al.
Post-VAD Rehabilitation in Children
3
Table 1 Standardized Care Pathway for Rehabilitation of Infants After Paracorporeal VAD Implantation
Table 3 Baseline Characteristics of Patients Participating in the SCP (n ¼ 14)
Age-appropriate goals:
Demographics
1. Positioning and range of motion 2. Tolerance to therapeutic handling and interaction 3. Tolerance to upright position in McLaren reclining
Median age (years) Female gender Treatment age group Infant Child/adolescent Indication for VAD Dilated cardiomyopathy Congenital heart disease Transplant with rejection VAD type Berlin Heart EXCOR Thoratec PVAD Biventricular support Survived to discharge Total VAD days
bouncy chair 4. Parent holding 5. Appropriate developmental play/program (to be determined
by PT/OT) 6. Participation in morning, afternoon and bedtime routine 7. Wagon rides, outings to pre-school if appropriate 8. Floor-time play activities PT/OT, physical therapy/occupational therapy; VAD, ventricular assist device.
discharge were recorded and analyzed. Adverse events were also analyzed. The study was approved by the institutional review board of Stanford University.
Results Baseline demographics Baseline data are summarized in Table 3. Seventeen patients received pneumatic paracorporeal VADs during the study period. Fifteen patients received a Berlin Heart EXCOR (4 with left VADs [LVADs], 9 with biventricular VADs [BiVADs] and 2 with single systemic ventricle/Glenn physiology devices) and 2 patients received a Thoratec PVAD (1 with LVAD, 1 with BiVAD). Nine patients were female and 8 were infants. The mean age at implant was 1.1 years (range 0.5 to 14.4 years). There were 1,473 total days on the VAD. The median time from VAD to transplant was 115.5 (range 40 to 229) days and the median time from transplant to discharge was 41 (range 12 to 170) days. The median time to extubation after transplant was 2 (range 1 to 8) days and the median ICU stay was 6.5 (range 3 to 152) Table 2 Standardized Care Pathway for Rehabilitation of Children 41 Year of Age After Paracorporeal VAD Implantation Age-appropriate goals: 1. Establish daily schedule 2. If intubated and alert, work on tolerance to upright
position (bed in chair position) Therapeutic exercise program Edge of bed sitting Out of bed to chair Toilet/commode transfers Wearing regular clothes (out of hospital gown) Participation in ADLs (e.g., grooming, brushing teeth, personal hygiene) 9. Ambulation 10. Outings to school, PT/OT gym, playroom 3. 4. 5. 6. 7. 8.
ADLs, activities of daily living; PT/OT, physical therapy/occupational therapy; VAD, ventricular assist device.
Median post-transplant days to extubation (n ¼ 12) Median post-transplant ICU days (n ¼ 12) Post transplant hospital days (n ¼ 12) Total hospital days (n ¼ 13)
1.05 (range 0.5–14.4) 9 (64%) 8 (57%) 6 (43%) 11 (79%) 2 (14%) 1 (7%) 12 (86%) 2 (14%) 7 (50%) 12 (86%) 1,473 (median 115.5) (range 40–229) 2 (range 1–8) 6.5 (range 3–152) 41 (range 12–170) 2,701 (median 184.5) (range 81–342)
ICU, intensive care unit; PVAD, pediatric ventricular assist device; SCP, standardized care pathway; VAD, ventricular assist device.
days. There were a total of 2,701 hospital days, with a median stay of 184.5 (range 81 to 342) days.
Participation/completion Of the 17 patients with implants, 14 were medically stable enough to participate in the SCP. Three patients were too medically unstable to participate, all of whom died prior to discharge. Two of these patients had single-ventricle (Glenn) physiology devices and died from complications related to acute respiratory distress syndrome (ARDS) and failing hemodynamics on Days 3 and 4 post-operatively. A third patient with trisomy 21 and an atrioventricular septal defect died of multi-system organ failure secondary to overwhelming sepsis on Day 31 post-operatively. Of the 14 patients who participated, 11 achieved all the SCP goals, including all of the patients in the child age group. Individual patient results are summarized in Table 4. In the infant group, 5 of 8 patients achieved all goals of the SCP (range 5 to 8) and 7 of 8 patients achieved at least 7 goals of the SCP. Of the 3 infants who did not complete all goals of the SCP, the primary barriers to completion were cannula infection in 1 patient, repeated respiratory infections/ARDS in 1 patient, and intolerance of handling in 1 patient. Of those patients who were able to complete the SCP, 3 were able to ride an adaptive tricycle with hand and foot petals, and 6 of 7 surviving participants o1 year
The Journal of Heart and Lung Transplantation, Vol ], No ], Month ]]]]
4 Table 4
Individual Patient Data
Days between Post-Tx Age at VAD VAD and time to Patient Indication placement extubation (in years) BiVAD? Tx number Gender for VAD
PostTx ICU days
Days between Tx and discharge
Total Goals hospital days Pathway achieved Outcome
1
M
DCM
1.7
Yes
192
2
6
41
299
Child
9/9a
2
M
CHD
2.9
Yes
222
1
3
47
342
Child
9/9a
3
F
CHD
0.6
No
40
7
18
42
139
Infant
5/8
4
F
DCM
8.5
No
229
1
9
14
261
Child
10/10
5
M
DCM
0.8
Yes
121
3
5
19
231
Infant
8/8
6
F
DCM
0.6
No
124
1
6
45
182
Infant
8/8
7
M
DCM
0.1
No
60
2
7
21
81
Infant
7/8
8
F
DCM
7.4
No
74
1
4
22
99
Child
10/10
9
F
DCM
0.3
Yes
71
2
4
12
87
Infant
8/8
10
F
DCM
0.4
No
118
1
9
16
159
Infant
8/8
11
F
DCM
0.5
Yes
109
8
40
57
218
Infant
8/8
12
F
DCM
14.4
No
113
3
152
170
287
Child
10/10
13
M
Tx
12
Yes
NA
NA
NA
NA
129
Child
10/10
Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Died
F
rejection DCM
Yes
NA
NA
NA
NA
187
Infant
7/8
Died
14
0.9
BiVAD, biventricular assist device; CHD, chronic heart disease; DCM, dilated cardiomyopathy; ICU, intensive care unit; NA, not available; Tx, transplant; VAD, ventricular assist device. a Commode transfer excluded from the SCP for these patients, as they were not yet toilet-trained.
of age were able to tolerate physical therapy in the prone position (Figures 1 and 2). All of the 5 surviving participants 41 year of age were able to attend hospital school regularly.
Adverse events There were no adverse events associated with therapy. One patient had a pulmonary hemorrhage on Day 7 post-implant, necessitating re-intubation that was temporally unrelated to therapy. One patient had an ascending cannula infection that pre-dated the start of her therapy and persisted until
transplant. This patient was able to achieve all goals of the SCP, but she eventually required strict bed rest for several weeks leading to transplant and was unable to sustain her rehabilitation achievements. The remaining surviving patients were able to sustain their achieved goals of the SCP until the time of transplant.
Post-transplant ICU course The median time to extubation in the study group was 2 (range 1 to 8) days and the median ICU stay was 6.5 (range 3 to 152) days. For all patients transplanted at our institution
Hollander et al.
Post-VAD Rehabilitation in Children
Figure 1 A patient supported with the Berlin Heart EXCOR LVAD riding an adaptive tricycle to facilitate age-appropriate functional skills.
during the study period, the median time to extubation was 2 (range 0 to 53) days and the median ICU stay was 7 (range 3 to 152) days (n ¼ 35, p ¼ not statistically significant [NS]).
Outcomes Of the 14 participants, 12 were transplanted and subsequently discharged, and 2 patients died while awaiting transplant.
Discussion An acute inpatient rehabilitation program utilizing ageappropriate, family-centered SCPs after paracorporeal VAD placement in children is safe and feasible. In this study, 14 of 17 patients were able to participate in a staged SCP after VAD implantation and 11 were able to complete all goals of the care pathway. For all but 1 patient, therapeutic achievements were sustained until the time of transplant. After 1,473 total VAD days, there were no adverse events related to therapy.
Figure 2 An infant supported by the Berlin Heart EXCOR LVAD undergoing physical therapy in the prone position.
5 We found that achieving all goals of the SCP was more successful in the 41-year age group. Although all patients in this age group were able to achieve all goals of the SCP, only 5 of 8 infants were able to do so. Barriers to achieving all goals of the SCP in 2 patients were primarily related to patient irritability and intolerance of handling, which in 1 patient worsened significantly after a hemorrhagic stroke. A third patient achieved 7 of 8 goals of the SCP but could not complete the seventh phase (outings to pre-school) secondary to requiring isolation for presumed viral illness. This patient eventually had a major hypoxic brain injury and died before transplantation. The results of our study corroborate earlier reports demonstrating that early, progressive mobilization of patients after VAD implantation in adults is safe and can be initiated in the ICU setting.14,16,18 de Jonge et al reported that adults who engaged in intense rehabilitation after surgery showed improved exercise capacity and were able to perform normal activities of daily living by 12 weeks post-operatively.19 More recent studies of adults after LVAD placement have also shown that patients are capable of tolerating 3 hours of therapy 6 days a week, resulting in functional gains comparable to other types of cardiac surgery.12 Although not a formalized metric in this study, there was a notable contrast between the number of VAD days and short post-transplant time to extubation. The findings suggest that even patients with long inpatient VAD courses approach their transplant surgery in a state conducive to rapid post-operative recovery. All but 2 of the patients who survived to discharge were extubated within 72 hours of their transplant, and 5 were extubated within 24 hours of surgery. Of the 2 patients intubated for 472 hours, 1 (Patient 3) had achieved the fewest goals of the SCP pre-transplant, and also had a relatively short VAD course (40 days). The other patient with a prolonged intubation course (Patient 11) had an ascending infection of her Berlin Heart cannulae. Concerns for cannula dislodgement precipitated strict bed rest for several weeks leading up to the time of her transplant, which further led to regression in achievement of therapy milestones and sub-optimal conditioning at the time of transplant surgery. For the remainder of patients, the time to extubation and duration of ICU stay were similar to those in patients at our institution who were transplanted without VAD support, suggesting that structured rehabilitation may help VAD-assisted patients maintain a level of conditioning that allows them to be well prepared for transplant surgery despite a prolonged pretransplant hospitalization course and mobility limitations. It is also notable that several patients exceeded the expectations of the SCP without complication. Additional therapeutic activities included swings and slides and riding on an adaptive tricycle to facilitate age-appropriate functional skills. Infants were able to tolerate prone positioning despite externalized cannulae. The results of our study demonstrate that intensive rehabilitation and the facilitation of age-appropriate activities is safe when supervised by expert personnel. At Stanford, a specialized rehabilitation team comfortable with VAD management designs and oversees all aspects of the
6
The Journal of Heart and Lung Transplantation, Vol ], No ], Month ]]]]
rehabilitation program after VAD implantation. Under such supervision, there have been no adverse events associated with therapy despite the patients’ need for intense anticoagulation and dealing with the risks associated with mobilizing patients with paracorporeal pumps. This study is limited by its small sample size and descriptive nature. It is also limited by the subjective nature of determining SCP-phase achievements, as interpreted by the rehabilitation team. Furthermore, the lack of an appropriate control group limits one’s ability to analyze the effects of structured rehabilitation on post-operative outcomes. Nevertheless, our study underscores the importance of post-implantation rehabilitation in pediatric patients receiving VADs, and also demonstrates that a structured rehabilitation program utilizing age-appropriate, familycentered SCPs is safe and feasible in this population.
Disclosure statement D.N.R. was a member of the clinical events committee for the Berlin Heart EXCOR IDE Trial. The remaining authors have no conflicts of interest to disclose. All photographic images were provided with explicit written permission from the families of the patients.
References 1. Kirk R, Dipchand AI, Edwards LB, et al. The registry of the International Society for Heart and Lung Transplantation: fifteenth pediatric heart transplantation report—2012. J Heart Lung Transplant 2012;31:1065-72. 2. 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. 3. Hsu KH, Huang SC, Chou NH, et al. Ventricular assist device application as a bridge to pediatric heart transplantation: a single center’s experience. Transplant Proc 2012;44:883-5. 4. Almond CS, Buchholz H, Massicotte P, et al. Berlin Heart EXCOR Pediatric Ventricular Assist Device Investigational Device Exemption Study: study design and rationale. Am Heart J 2011;162:425-35.
5. Morales DL, Almond CS, Jaquiss RD, et al. Bridging children of all sizes to cardiac transplantation: the initial multicenter North American experience with the Berlin Heart EXCOR ventricular assist device. J Heart Lung Transplant 2011;30:1-8. 6. Serrano-Ikkos E, Lask B, Whitehead B. Psychosocial morbidity in children, and their families, awaiting heart or heart–lung transplantation. J Psychosom Res 1997;42:253-60. 7. Stein ML, Bruno JL, Konopacki KL, et al. Cognitive outcomes in pediatric heart transplant recipients bridged to transplantation with ventricular assist devices. J Heart Lung Transplant 2013;32:212-20. 8. Westerdahl E, Moller M. Physiotherapy-supervised mobilization and exercise following cardiac surgery: a national questionnaire survey in Sweden. J Cardiothorac Surg 2010;5:67. 9. Genc A. Early mobilization of the critically ill patients: towards standardization. Crit Care Med 2012;40:1346-7. 10. Freeman R, Maley K. Mobilization of intensive care cardiac surgery patients on mechanical circulatory support. Crit Care Nurs Q 2013; 36:73-88. 11. Ueno A, Tomizawa Y. Cardiac rehabilitation and artificial heart devices. J Artif Org 2009;12:90-7. 12. Nguyen E, Stein J. Functional outcomes of adults with left ventricular assist devices receiving inpatient rehabilitation. PM & R 2013;5: 99-103. 13. Reedy JE, Swartz MT, Lohmann DP, et al. The importance of patient mobility with ventricular assist device support. ASAIO J 1992;38: M151-3. 14. English ML, Speed J. Effectiveness of acute inpatient rehabilitation after left ventricular assist device placement. Am J Phys Med Rehabil 2013;92:621-6. 15. Corra U, Pistono M, Mezzani A, et al. Cardiovascular prevention and rehabilitation for patients with ventricular assist device from exercise therapy to long-term therapy. Part I: exercise therapy. Monaldi Arch Chest Dis 2011;76:27-32. 16. Morrone TM, Buck LA, Catanese KA, et al. Early progressive mobilization of patients with left ventricular assist devices is safe and optimizes recovery before heart transplantation. J Heart Lung Transplant 1996;15:423-9. 17. Perme CS, Southard RE, Joyce DL, et al. Early mobilization of LVAD recipients who require prolonged mechanical ventilation. Tex Heart Inst J 2006;33:130-3. 18. Stiller K. Physiotherapy in intensive care: towards an evidence-based practice. Chest 2000;118:1801-13. 19. de Jonge N, Kirkels H, Lahpor JR, et al. Exercise performance in patients with end-stage heart failure after implantation of a left ventricular assist device and after heart transplantation: an outlook for permanent assisting? J Am Coll Cardiol 2001;37:1794-9.
ORIGINAL CLINICAL SCIENCE
An inpatient rehabilitation program utilizing standardized care pathways after paracorporeal ventricular assist device placement in children Seth A. Hollander, MD,a Amanda J. Hollander, DPT, PCS,b Sandra Rizzuto, OTD,b Olaf Reinhartz, MD,c Katsuhide Maeda, MD,c and David N. Rosenthal, MDa From the aDepartment of Pediatrics (Cardiology), Stanford University Medical Center; bRehabilitation Services, Lucile Packard Children's Hospital; and the cDepartment of Cardiovascular Surgery, Stanford University Medical Center, Palo Alto, California.
KEYWORDS: pediatrics; heart transplant; rehabilitation; physical therapy; occupational therapy; ventricular assist device
BACKGROUND: Structured rehabilitation programs in adults after ventricular assist device (VAD) placement result in improvements in physical function and exercise capacity, and have been shown to improve survival and accelerate post-transplant recovery. The objective of this study was to determine the safety and feasibility of an acute inpatient rehabilitation program for children utilizing standardized, age-appropriate, family-centered care pathways after paracorporeal VAD placement in both the ICU and acute-care inpatient settings. METHODS: Between November 12, 2010 and March 15, 2013, 17 patients were referred to therapy after VAD implantation, 14 of whom were medically stable enough to participate. Beginning in the ICU, a structured physical and occupational therapy program was implemented utilizing novel age-appropriate, standardized care pathways for infants (age o1 year) and children (age 1 to 12 years). The infant and child pathways consisted of 8 and 10 goals, respectively. Retrospective review was conducted to ascertain the number of phases achieved per patient. Adverse events, defined as bleeding, physiologic instability, stroke, or device disruption during therapy, were also analyzed. RESULTS: The median age was 1.1 (range 0.5 to 14.4) years in the 14 patients considered medically stable enough to participate in rehabilitation. Nine of them were female. Eight patients participated in the infant standardized care pathway (SCP) and 6 participated in the child SCP. Seven patients were on biventricular support. Twelve patients were transplanted and survived. Two patients died while awaiting transplantation. There were 1,473 total days on the VAD (range 40 to 229 days). The median time to extubation was 2 days (range 1 to 8) and the median ICU stay was 6.5 days (range 3 to 152). Eleven patients achieved all goals of the SCP, including all of the patients in the child group. For the infant group, 5 patients achieved all goals of the SCP (range 5 to 8), and all but 1 patient achieved at least 7 goals of the SCP. There were no adverse events related to therapy. CONCLUSIONS: Standardized, family-centered inpatient rehabilitation care paths are safe for infants and children after paracorporeal device placement. Structured rehabilitation goals can be achieved by the majority of pediatric patients during VAD support. Early mobilization and inpatient rehabilitation in this cohort promotes normalization of function while awaiting cardiac transplantation. J Heart Lung Transplant ]]]];]:]]]–]]] r 2014 International Society for Heart and Lung Transplantation. All rights reserved.
Reprint requests: Seth A. Hollander, MD, Department of Pediatric Cardiology, Lucile Packard Children’s Hospital, Stanford University Medical Center, 750 Welch Road, Suite 321, Palo Alto, CA 94304. Telephone: 650-736-8600. Fax: 650-497-8422. E-mail address: [email protected]
Each year, approximately 400 pediatric heart transplants are performed worldwide.1 With an increased demand for donor hearts, but a relatively stagnant donor supply, ventricular assist devices (VADs) in children allow for
1053-2498/$ - see front matter r 2014 International Society for Heart and Lung Transplantation. All rights reserved. http://dx.doi.org/10.1016/j.healun.2013.12.009
2
The Journal of Heart and Lung Transplantation, Vol ], No ], Month ]]]]
pediatric patients with end-stage heart failure to survive longer while waiting for an appropriate donor organ to become available.2–5 At present, the Thoratec PVAD and the Berlin Heart EXCOR are the most commonly used devices for infants and children. Both devices consist of paracorporeal pneumatic pumps that require tethering to a large driver, and neither are suitable for outpatient use. As a result, children who require mechanical ventricular support are subject to prolonged hospital stays, which exposes them to significant morbidity, including physical deconditioning, deficits in activities of daily living, delays in cognitive and social development, and depression.6,7 Early management of VAD patients has frequently consisted of prolonged intubations and long-term stays in the intensive care unit (ICU) often accompanied by bed-rest and limitations in physical activity, despite the documented benefits of early mobilization and rehabilitation after other forms of cardiac surgery.8,9 As experience with management of patients with VADs has increased over time, early mobilization and ambulation is now increasingly recognized as a critical component of patient recovery after VAD implantation.10,11 Structured rehabilitation programs in adults after VAD placement have been shown to result in improvements in physical function and exercise capacity, as well as improved survival and accelerated post-transplant recovery.12–15 Despite the challenges of pre-existing deconditioning from congestive heart failure (CHF), fixed cardiac output, and attachment to mechanical equipment, including ventilators and VAD percutaneous drive-lines, the safety of early mobilization and intense rehabilitation in adults after VAD implantation, starting in the ICU setting, is also well established.16,17 The adult rehabilitation experience, however, cannot necessarily be extrapolated to the pediatric population, as there are unique challenges to the rehabilitation of children after VAD implantation. Whereas adults have the option of receiving the HeartMate II, an intracorporeal continuousflow device, most children who require mechanical support require placement of pulsatile paracorporeal devices, such as the Thoratec PVAD or Berlin Heart EXCOR, presenting the challenge of needing to work around externalized pumps and cumbersome medical equipment. Furthermore, pulsatile paracorporeal devices carry an increased risk of thrombosis and thromboembolism, thereby requiring higher levels of anti-coagulation while in use. Neurologic dysfunction has been observed in 29% of Berlin Heart recipients.2 Last, patients’ unwillingness to follow directions and parental hesitation can further compound the risk of accidental extubation, falls and device disruption. One patient death secondary to accidental device dislodgement during play has been reported.2 Despite increasing recognition of its importance, the safety and feasibility of implementing an inpatient rehabilitation program in pediatric paracorporeal VAD recipients has not been systematically studied. In November 2010, we developed a comprehensive interdisciplinary care program that included standardized, age-appropriate, family-centered rehabilitation care paths for pediatric VAD patients awaiting heart transplantation. The goal of the program was to
provide safe and effective inpatient rehabilitation for both the purposes of conditioning as well as supporting normal development. The objective of the present study was to determine the safety and feasibility of an acute inpatient rehabilitation program for children utilizing age-appropriate, family-centered, standardized care pathways after paracorporeal VAD placement in both the ICU and acute-care inpatient settings.
Methods A retrospective review was conducted on all patients who received paracorporeal VADs between the initiation of the standardized care pathway (SCP) on November 12, 2010 and March 15, 2013. Seventeen patients received pulsatile paracorporeal VADs during the study period, 14 of whom were medically stable enough to participate in the SCP. All patients remained as inpatients until transplant.
Therapeutic strategy Beginning in the ICU prior to extubation and continuing until the time of transplant, a structured physical and occupational therapy intervention was implemented utilizing novel, age-appropriate SCPs for infants (o1 year) and children (age 1 to 12 years). The SCP focused on therapeutic regimens individualized for patient’s age, abilities, interests and temperament. Rehabilitative exercises were woven into normal activities (e.g., obstacle courses and modified sports to improve balance and motor planning, stimulating floor-play to promote infant development). During 4 or 5 1hour sessions per week, patients received intensive therapy by specially trained physical and occupational therapists. In general, one therapist was exclusively responsible for ensuring VADs, lines and tubes did not dislodge, whereas the second therapist supervised therapeutic activities. In addition, the nursing staff and parents were trained and expected to provide rehabilitation throughout the day as prescribed by the rehabilitation team. A goal was considered achieved when the patient was observed to perform that activity consistently according to the rehabilitation staff.
Standardized care pathway The infant and child pathways consisted of 8 and 10 goals, respectively, which focused on implementing a daily schedule, therapeutic exercise and activities, family participation and off-unit excursions (Tables 1 and 2). Toddlers who were not yet toilettrained were expected to complete 9 goals of the child pathway, minus toilet transfers. Patients who were too medically unstable to tolerate therapy were considered ineligible for the SCP.
Adverse events Adverse events were defined as falls, pre-syncope, syncope, accidental extubation, bleeding, hemodynamic instability, stroke, or device disruption during or as a direct result of therapy.
Analysis Retrospective chart review was conducted to ascertain the number of phases achieved per patient. In addition, baseline demographics, survival data, total VAD days and time between transplant and
Hollander et al.
Post-VAD Rehabilitation in Children
3
Table 1 Standardized Care Pathway for Rehabilitation of Infants After Paracorporeal VAD Implantation
Table 3 Baseline Characteristics of Patients Participating in the SCP (n ¼ 14)
Age-appropriate goals:
Demographics
1. Positioning and range of motion 2. Tolerance to therapeutic handling and interaction 3. Tolerance to upright position in McLaren reclining
Median age (years) Female gender Treatment age group Infant Child/adolescent Indication for VAD Dilated cardiomyopathy Congenital heart disease Transplant with rejection VAD type Berlin Heart EXCOR Thoratec PVAD Biventricular support Survived to discharge Total VAD days
bouncy chair 4. Parent holding 5. Appropriate developmental play/program (to be determined
by PT/OT) 6. Participation in morning, afternoon and bedtime routine 7. Wagon rides, outings to pre-school if appropriate 8. Floor-time play activities PT/OT, physical therapy/occupational therapy; VAD, ventricular assist device.
discharge were recorded and analyzed. Adverse events were also analyzed. The study was approved by the institutional review board of Stanford University.
Results Baseline demographics Baseline data are summarized in Table 3. Seventeen patients received pneumatic paracorporeal VADs during the study period. Fifteen patients received a Berlin Heart EXCOR (4 with left VADs [LVADs], 9 with biventricular VADs [BiVADs] and 2 with single systemic ventricle/Glenn physiology devices) and 2 patients received a Thoratec PVAD (1 with LVAD, 1 with BiVAD). Nine patients were female and 8 were infants. The mean age at implant was 1.1 years (range 0.5 to 14.4 years). There were 1,473 total days on the VAD. The median time from VAD to transplant was 115.5 (range 40 to 229) days and the median time from transplant to discharge was 41 (range 12 to 170) days. The median time to extubation after transplant was 2 (range 1 to 8) days and the median ICU stay was 6.5 (range 3 to 152) Table 2 Standardized Care Pathway for Rehabilitation of Children 41 Year of Age After Paracorporeal VAD Implantation Age-appropriate goals: 1. Establish daily schedule 2. If intubated and alert, work on tolerance to upright
position (bed in chair position) Therapeutic exercise program Edge of bed sitting Out of bed to chair Toilet/commode transfers Wearing regular clothes (out of hospital gown) Participation in ADLs (e.g., grooming, brushing teeth, personal hygiene) 9. Ambulation 10. Outings to school, PT/OT gym, playroom 3. 4. 5. 6. 7. 8.
ADLs, activities of daily living; PT/OT, physical therapy/occupational therapy; VAD, ventricular assist device.
Median post-transplant days to extubation (n ¼ 12) Median post-transplant ICU days (n ¼ 12) Post transplant hospital days (n ¼ 12) Total hospital days (n ¼ 13)
1.05 (range 0.5–14.4) 9 (64%) 8 (57%) 6 (43%) 11 (79%) 2 (14%) 1 (7%) 12 (86%) 2 (14%) 7 (50%) 12 (86%) 1,473 (median 115.5) (range 40–229) 2 (range 1–8) 6.5 (range 3–152) 41 (range 12–170) 2,701 (median 184.5) (range 81–342)
ICU, intensive care unit; PVAD, pediatric ventricular assist device; SCP, standardized care pathway; VAD, ventricular assist device.
days. There were a total of 2,701 hospital days, with a median stay of 184.5 (range 81 to 342) days.
Participation/completion Of the 17 patients with implants, 14 were medically stable enough to participate in the SCP. Three patients were too medically unstable to participate, all of whom died prior to discharge. Two of these patients had single-ventricle (Glenn) physiology devices and died from complications related to acute respiratory distress syndrome (ARDS) and failing hemodynamics on Days 3 and 4 post-operatively. A third patient with trisomy 21 and an atrioventricular septal defect died of multi-system organ failure secondary to overwhelming sepsis on Day 31 post-operatively. Of the 14 patients who participated, 11 achieved all the SCP goals, including all of the patients in the child age group. Individual patient results are summarized in Table 4. In the infant group, 5 of 8 patients achieved all goals of the SCP (range 5 to 8) and 7 of 8 patients achieved at least 7 goals of the SCP. Of the 3 infants who did not complete all goals of the SCP, the primary barriers to completion were cannula infection in 1 patient, repeated respiratory infections/ARDS in 1 patient, and intolerance of handling in 1 patient. Of those patients who were able to complete the SCP, 3 were able to ride an adaptive tricycle with hand and foot petals, and 6 of 7 surviving participants o1 year
The Journal of Heart and Lung Transplantation, Vol ], No ], Month ]]]]
4 Table 4
Individual Patient Data
Days between Post-Tx Age at VAD VAD and time to Patient Indication placement extubation (in years) BiVAD? Tx number Gender for VAD
PostTx ICU days
Days between Tx and discharge
Total Goals hospital days Pathway achieved Outcome
1
M
DCM
1.7
Yes
192
2
6
41
299
Child
9/9a
2
M
CHD
2.9
Yes
222
1
3
47
342
Child
9/9a
3
F
CHD
0.6
No
40
7
18
42
139
Infant
5/8
4
F
DCM
8.5
No
229
1
9
14
261
Child
10/10
5
M
DCM
0.8
Yes
121
3
5
19
231
Infant
8/8
6
F
DCM
0.6
No
124
1
6
45
182
Infant
8/8
7
M
DCM
0.1
No
60
2
7
21
81
Infant
7/8
8
F
DCM
7.4
No
74
1
4
22
99
Child
10/10
9
F
DCM
0.3
Yes
71
2
4
12
87
Infant
8/8
10
F
DCM
0.4
No
118
1
9
16
159
Infant
8/8
11
F
DCM
0.5
Yes
109
8
40
57
218
Infant
8/8
12
F
DCM
14.4
No
113
3
152
170
287
Child
10/10
13
M
Tx
12
Yes
NA
NA
NA
NA
129
Child
10/10
Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Transplanted and discharged Died
F
rejection DCM
Yes
NA
NA
NA
NA
187
Infant
7/8
Died
14
0.9
BiVAD, biventricular assist device; CHD, chronic heart disease; DCM, dilated cardiomyopathy; ICU, intensive care unit; NA, not available; Tx, transplant; VAD, ventricular assist device. a Commode transfer excluded from the SCP for these patients, as they were not yet toilet-trained.
of age were able to tolerate physical therapy in the prone position (Figures 1 and 2). All of the 5 surviving participants 41 year of age were able to attend hospital school regularly.
Adverse events There were no adverse events associated with therapy. One patient had a pulmonary hemorrhage on Day 7 post-implant, necessitating re-intubation that was temporally unrelated to therapy. One patient had an ascending cannula infection that pre-dated the start of her therapy and persisted until
transplant. This patient was able to achieve all goals of the SCP, but she eventually required strict bed rest for several weeks leading to transplant and was unable to sustain her rehabilitation achievements. The remaining surviving patients were able to sustain their achieved goals of the SCP until the time of transplant.
Post-transplant ICU course The median time to extubation in the study group was 2 (range 1 to 8) days and the median ICU stay was 6.5 (range 3 to 152) days. For all patients transplanted at our institution
Hollander et al.
Post-VAD Rehabilitation in Children
Figure 1 A patient supported with the Berlin Heart EXCOR LVAD riding an adaptive tricycle to facilitate age-appropriate functional skills.
during the study period, the median time to extubation was 2 (range 0 to 53) days and the median ICU stay was 7 (range 3 to 152) days (n ¼ 35, p ¼ not statistically significant [NS]).
Outcomes Of the 14 participants, 12 were transplanted and subsequently discharged, and 2 patients died while awaiting transplant.
Discussion An acute inpatient rehabilitation program utilizing ageappropriate, family-centered SCPs after paracorporeal VAD placement in children is safe and feasible. In this study, 14 of 17 patients were able to participate in a staged SCP after VAD implantation and 11 were able to complete all goals of the care pathway. For all but 1 patient, therapeutic achievements were sustained until the time of transplant. After 1,473 total VAD days, there were no adverse events related to therapy.
Figure 2 An infant supported by the Berlin Heart EXCOR LVAD undergoing physical therapy in the prone position.
5 We found that achieving all goals of the SCP was more successful in the 41-year age group. Although all patients in this age group were able to achieve all goals of the SCP, only 5 of 8 infants were able to do so. Barriers to achieving all goals of the SCP in 2 patients were primarily related to patient irritability and intolerance of handling, which in 1 patient worsened significantly after a hemorrhagic stroke. A third patient achieved 7 of 8 goals of the SCP but could not complete the seventh phase (outings to pre-school) secondary to requiring isolation for presumed viral illness. This patient eventually had a major hypoxic brain injury and died before transplantation. The results of our study corroborate earlier reports demonstrating that early, progressive mobilization of patients after VAD implantation in adults is safe and can be initiated in the ICU setting.14,16,18 de Jonge et al reported that adults who engaged in intense rehabilitation after surgery showed improved exercise capacity and were able to perform normal activities of daily living by 12 weeks post-operatively.19 More recent studies of adults after LVAD placement have also shown that patients are capable of tolerating 3 hours of therapy 6 days a week, resulting in functional gains comparable to other types of cardiac surgery.12 Although not a formalized metric in this study, there was a notable contrast between the number of VAD days and short post-transplant time to extubation. The findings suggest that even patients with long inpatient VAD courses approach their transplant surgery in a state conducive to rapid post-operative recovery. All but 2 of the patients who survived to discharge were extubated within 72 hours of their transplant, and 5 were extubated within 24 hours of surgery. Of the 2 patients intubated for 472 hours, 1 (Patient 3) had achieved the fewest goals of the SCP pre-transplant, and also had a relatively short VAD course (40 days). The other patient with a prolonged intubation course (Patient 11) had an ascending infection of her Berlin Heart cannulae. Concerns for cannula dislodgement precipitated strict bed rest for several weeks leading up to the time of her transplant, which further led to regression in achievement of therapy milestones and sub-optimal conditioning at the time of transplant surgery. For the remainder of patients, the time to extubation and duration of ICU stay were similar to those in patients at our institution who were transplanted without VAD support, suggesting that structured rehabilitation may help VAD-assisted patients maintain a level of conditioning that allows them to be well prepared for transplant surgery despite a prolonged pretransplant hospitalization course and mobility limitations. It is also notable that several patients exceeded the expectations of the SCP without complication. Additional therapeutic activities included swings and slides and riding on an adaptive tricycle to facilitate age-appropriate functional skills. Infants were able to tolerate prone positioning despite externalized cannulae. The results of our study demonstrate that intensive rehabilitation and the facilitation of age-appropriate activities is safe when supervised by expert personnel. At Stanford, a specialized rehabilitation team comfortable with VAD management designs and oversees all aspects of the
6
The Journal of Heart and Lung Transplantation, Vol ], No ], Month ]]]]
rehabilitation program after VAD implantation. Under such supervision, there have been no adverse events associated with therapy despite the patients’ need for intense anticoagulation and dealing with the risks associated with mobilizing patients with paracorporeal pumps. This study is limited by its small sample size and descriptive nature. It is also limited by the subjective nature of determining SCP-phase achievements, as interpreted by the rehabilitation team. Furthermore, the lack of an appropriate control group limits one’s ability to analyze the effects of structured rehabilitation on post-operative outcomes. Nevertheless, our study underscores the importance of post-implantation rehabilitation in pediatric patients receiving VADs, and also demonstrates that a structured rehabilitation program utilizing age-appropriate, familycentered SCPs is safe and feasible in this population.
Disclosure statement D.N.R. was a member of the clinical events committee for the Berlin Heart EXCOR IDE Trial. The remaining authors have no conflicts of interest to disclose. All photographic images were provided with explicit written permission from the families of the patients.
References 1. Kirk R, Dipchand AI, Edwards LB, et al. The registry of the International Society for Heart and Lung Transplantation: fifteenth pediatric heart transplantation report—2012. J Heart Lung Transplant 2012;31:1065-72. 2. 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. 3. Hsu KH, Huang SC, Chou NH, et al. Ventricular assist device application as a bridge to pediatric heart transplantation: a single center’s experience. Transplant Proc 2012;44:883-5. 4. Almond CS, Buchholz H, Massicotte P, et al. Berlin Heart EXCOR Pediatric Ventricular Assist Device Investigational Device Exemption Study: study design and rationale. Am Heart J 2011;162:425-35.
5. Morales DL, Almond CS, Jaquiss RD, et al. Bridging children of all sizes to cardiac transplantation: the initial multicenter North American experience with the Berlin Heart EXCOR ventricular assist device. J Heart Lung Transplant 2011;30:1-8. 6. Serrano-Ikkos E, Lask B, Whitehead B. Psychosocial morbidity in children, and their families, awaiting heart or heart–lung transplantation. J Psychosom Res 1997;42:253-60. 7. Stein ML, Bruno JL, Konopacki KL, et al. Cognitive outcomes in pediatric heart transplant recipients bridged to transplantation with ventricular assist devices. J Heart Lung Transplant 2013;32:212-20. 8. Westerdahl E, Moller M. Physiotherapy-supervised mobilization and exercise following cardiac surgery: a national questionnaire survey in Sweden. J Cardiothorac Surg 2010;5:67. 9. Genc A. Early mobilization of the critically ill patients: towards standardization. Crit Care Med 2012;40:1346-7. 10. Freeman R, Maley K. Mobilization of intensive care cardiac surgery patients on mechanical circulatory support. Crit Care Nurs Q 2013; 36:73-88. 11. Ueno A, Tomizawa Y. Cardiac rehabilitation and artificial heart devices. J Artif Org 2009;12:90-7. 12. Nguyen E, Stein J. Functional outcomes of adults with left ventricular assist devices receiving inpatient rehabilitation. PM & R 2013;5: 99-103. 13. Reedy JE, Swartz MT, Lohmann DP, et al. The importance of patient mobility with ventricular assist device support. ASAIO J 1992;38: M151-3. 14. English ML, Speed J. Effectiveness of acute inpatient rehabilitation after left ventricular assist device placement. Am J Phys Med Rehabil 2013;92:621-6. 15. Corra U, Pistono M, Mezzani A, et al. Cardiovascular prevention and rehabilitation for patients with ventricular assist device from exercise therapy to long-term therapy. Part I: exercise therapy. Monaldi Arch Chest Dis 2011;76:27-32. 16. Morrone TM, Buck LA, Catanese KA, et al. Early progressive mobilization of patients with left ventricular assist devices is safe and optimizes recovery before heart transplantation. J Heart Lung Transplant 1996;15:423-9. 17. Perme CS, Southard RE, Joyce DL, et al. Early mobilization of LVAD recipients who require prolonged mechanical ventilation. Tex Heart Inst J 2006;33:130-3. 18. Stiller K. Physiotherapy in intensive care: towards an evidence-based practice. Chest 2000;118:1801-13. 19. de Jonge N, Kirkels H, Lahpor JR, et al. Exercise performance in patients with end-stage heart failure after implantation of a left ventricular assist device and after heart transplantation: an outlook for permanent assisting? J Am Coll Cardiol 2001;37:1794-9.
