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PRF29110.1177/0267659113496580PerfusionPark et al.
Original Paper
Perfusion
2014, Vol 29(1) 8288
The Author(s) 2013
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DOI: 10.1177/0267659113496580
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Introduction
Approximately 1 in 100,000 children are diagnosed each
year with symptoms of cardiomyopathy in the USA.1
Dilated cardiomyopathy (DCM) is the primary indication, accounting for 65% of heart transplants in children
11-17 years of age.2 Children diagnosed with DCM and
decompensated heart failure represent a high-risk subgroup, often benefiting from optimal heart failure management and prompt evaluation for a heart transplant.
According to Organ Procurement and Transplantation
Network, in 2011, 375 heart transplants were performed
in pediatric patients.3 Unfortunately, children awaiting a
heart transplant are subject to the highest waiting list
mortality in solid-organ transplant medicine in the
USA.4 Almond et al. state that 17% of children under the
age of 18 years in the USA died while awaiting heart
transplantation in the years 1999-2006.4 It is imperative
1Division
Park et al.
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Figure 1A depicts the SynCardia Total Artificial Heart. The TAH-t is comprised of two semi-rigid polyurethane ventricles of 70 ml volume with a
flexible, multi-layered, polyurethane diaphragm separating the blood path from the pneumatic chamber. Quick-connect inflow sewing cuffs attach the
artificial ventricles at the 27 mm mechanical valve to the remaining atria. Quick-connect outflow sewing grafts attach the artificial ventricles at the 25
mm mechanical valve to the pulmonary artery and aorta. Percutaneous conduits protrude from the pneumatic chamber, exiting through the upper
right quadrant of the abdominal wall and attaching to 7-foot-long drivelines that connect the ventricles with the external driver and console.
Figure 1B illustrates in situ implantation of the TAH-t complete with connection of the atrial cuffs and outflow graft to and from the ventricles and
their respective anatomic structures, as well as the blood flow path. Images Courtesy: syncardia.com
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Perfusion 29(1)
The cardiac cycle of the SynCardia Total Artificial Heart operates by shuttling pressurized air in and out of the pneumatic chambers of the left and
right ventricles in synchronization through the attached percutaneous drivelines connected to the external driver and console. Figure 2A displays the
systolic phase at the beginning when air is applied from the external driver (1) to the pneumatic chambers of the ventricles. When the sub-diaphragm
ventricular pressures exceed the pulmonary and aortic pressures, the 25 mm outlet valves will open (2) and the diaphragms will displace upward
until they reach maximum distension and all ventricular blood is ejected (3). The build-up of pressure is measured and the active tracing is displayed
on the driver monitor. The accumulated pressurized air inside the ventricles is then released back along the drivelines to the console and exhausted
into the atmosphere at the end of the systolic cycle and the beginning of the diastolic filling cycle. Transition from the systolic to diastolic phase
is shown in Figure 2B; air is exhausted from the pneumatic chambers (1) of the ventricles back to the console to allow room for blood to fill the
ventricles.Ventricular filling begins when the sub-diaphragm ventricular pressures decrease below the right and left atrial pressures and the 27 mm
inlet valves open to allow blood to displace the diaphragms downward (2). The resulting displaced air along the drivelines from ventricular filling is
measured by the console and estimates ventricular stroke volume. The active tracing is displayed on the driver monitor. A small vacuum which draws
up to 15 mmHg during diastole can be applied from the console to help in diaphragm displacement and facilitate ventricular filling. Images Courtesy:
syncardia.com
Case Report
A 14-year-old male was admitted with DCM, severe
biventricular dysfunction, and ventricular arrhythmias. He initially presented to outside facilities with a
3-week history of cough, shortness of breath, upper
respiratory tract symptoms and fever. The patient was
an otherwise healthy adolescent with a past medical
history of asthma. The patient was transferred to this
institution for further evaluation following the identification of severely depressed cardiac failure and
life-threatening ventricular arrhythmias. An echocardiogram revealed biventricular dysfunction with an
ejection fraction of 15-17% and multiple clots were
present in the left ventricle and left ventricular outflow tract (Figure 3). Advanced heart failure medical
optimization was promptly initiated with intravenous
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Park et al.
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Following median sternotomy, all structures are identified and isolated. The drivelines for both ventricles are passed subcutaneously before
heparinization of the patient. The ventricles are positioned lateral to the sternotomy and well covered. Following systemic heparinization, CPB is
instituted and the heart is fibrillated. Dissection around the aorta and pulmonary artery is limited to the proximal portion of the aorta in anticipation
of later transplantation. The ventriculectomy is significantly different from that of transplantation. The annuluses of the tricuspid and mitral valves are
preserved. An incision is made on the ventricular side of the atrioventricular (AV) groove of the right ventricle, extending anteriorly across the right
ventricular outflow tract and just proximal to the pulmonary valve (Figure 6A, B). Posteriorly, the excision extends to the interventricular septum
and across the septum on the left ventricular side of the AV groove, preserving the entirety of the mitral annulus. The anterior and posterior lines
of incision are dissected to the level of the pulmonary bifurcation. The excess muscle is trimmed down to near the AV valves. The great vessels are
then separated (Figure 6C). The coronary sinus is over-sewn. Both atrial cuff complexes are encircled with Teflon felt buttresses to strengthen the
anastomosis and quick connect (Figure 6D). Hemostasis is checked with the plastic tester, which fits within the quick connect. The pulmonary and
aortic anastomoses are completed (Figure 6D). The conduits are cut to the appropriate length, usually 3 to 5 cm, and sewn in place. Following this,
using the aortic tester, they are inserted into both quick connects and checked for leaks. Once hemostasis of all suture lines has been established,
TAH-t insertion begins with placement of the left ventricle (Figure 6E). A careful assessment of the relationship of ventricles to their respective
atrial cuffs and outflow grafts should be considered prior to completing the atrial connection. The left ventricle should be filled with saline through
the aortic valve while the patient is placed in a steep Trendelenburg position and the aorta is vented. The right ventricle is filled by restoring the
caval blood flow to the atrium and air is evacuated via the pulmonary outflow graft. The TAH-t is started on low rate and pressure settings until
satisfactory de-airing of the ventricles is complete.17 Images Courtesy: syncardia.com
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Park et al.
Figure 6A and B. (A) 3D Reconstruction of cardiopulmonary structures, skeletal structures and TAH device in situ.
(B) 3D reconstruction with osseous structures subtracted.
Conclusion
Cardiomyopathy in infants and children with heart disease is one of the most common causes for death.1 An
estimated 1,000-5,000 new cases of cardiomyopathy are
diagnosed each year in pediatrics.2 DCM is the leading
cause for heart transplantation among pediatric patients.
Survival of these children depends on prompt accurate
diagnosis and aggressive heart failure therapy. Some children respond to conventional heart failure treatment
while others further decompensate, requiring mechanical circulatory support and heart transplantation.5
Unfortunately, children listed for heart transplantation
in the USA face the highest waiting list mortality irrespective of age.4,5 The emergent deployment of pediatric
cardiac assist technologies has been shown to benefit the
pediatric patient in decompensated heart failure with
multi-organ dysfunction while awaiting a transplant. In
selected pediatric patients, the TAH-t is the assist device
of choice for decompensatory biventricular heart failure.
Commonly encountered VAD issues, such as valverelated problems, left ventricular clot formation, right
heart failure, arrhythmias and the need for inotropes, are
eliminated with the TAH-t.7,9-11 A limiting factor for the
use of the TAH-t in children is the recommended minimum body surface area of 1.7 m2 for proper fit and function. Currently, there are a small number of certified
pediatric centers, with our center being the third transplant center to implant a TAH-t in a child.12 The need for
modification of the device in children with complex congenital anatomies and the absence of current anticoagulation protocols for children make increased pediatric
implants a challenge. Quick transition to ambulation and
robust diets are critical milestones for all TAH-t patients
and can directly impact quality of life and functional
88
Funding
This research received no specific grant from any funding
agency in the public, commercial or not-for-profit sectors.
References
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Cardiomyopathy
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Perfusion 29(1)
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The SynCardia freedom driver: a portable driver for discharge home with the total artificial heart. J Heart Lung
Transplant 2011; 30: 844845.
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technique for the CardioWest total artificial heart. Ann
Thorac Surg 1999; 68: 698704.
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