Mechanical Circulatory Support in Children -- CTSNet FAQs
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Pediatric Cardiac Surgery FAQs
Section Editor: Tom R. Karl, M.D.


Mechanical Circulatory Support in Children
Pedro J del Nido


   

1.

What are some of the technical pitfalls in the first stage palliative procedure for hypoplastic left heart syndrome as described by Norwood?

The majority of children who require mechanical circulatory support do so following cardiac surgery. The indications are most often poor contractile function or low cardiac output, inadequate to maintain kidney or other organ function. It is important in this setting to search actively for residual defects that may explain the low cardiac output, since complete correction offers the best chance for successful weaning from mechanical support. If cardiac function does not recover, then circulatory support can be continued while awaiting transplantation in those children deemed candidates. Less frequently, the indication for circulatory assistance is pulmonary dysfunction with inadequate ventilation or oxygenation despite mechanical ventilatory support. In this situation, a complete extracorporeal membrane oxygenation (ECMO) circuit including an oxygenator is required. Another important indication for mechanic al circulatory support is resuscitation following cardiac arrest not responsive to conventional CPR. If the arrest is witnessed, and effective CPR started promptly, circulatory support as part of the resuscitation has resulted in relatively high survival probability (50% to 70%) despite periods of CPR lasting up to 90 minutes.
 

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2.

What are the most important sequelae or events that can occur following successful initial palliation and before subsequent reconstructive surgery?

Because long-term circulatory support (beyond one to two weeks) without a high incidence of complications is not yet possible in children, the primary goals of mechanical support are to maintain organ perfusion while the heart recovers or while awaiting a heart transplant. Experience with mechanical support in children has shown that the majority of patients who eventually are weaned successfully from support have significant recovery of cardiac function 4 8 to 72 hours after extracorporeal circulation is started. If little or not recovery of cardiac function has been observed by 72 hours, then transplantation should be considered for children who are deemed candidates.
 

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3.

What are the relative advantages of the bi-directional Glenn anastomosis and the hemi-Fontan procedure?

Adults frequently require mechanical circulatory support as a consequence of coronary artery disease. Whether support is required after myocardial infarction or after coronary artery bypass grafting, left ventricular dysfunction is the predominant physiologic abnormality. Because of this, intraaortic balloon counterpulsation (IABP) or left ventricular assist devices (LVAD) are frequently successful in treating adult patients that require mechanical circulatory support. Children with complex heart disease more often have biventricular cardiac failure, pulmonary hypertension, and hypoxemia as predominant features. For adequate support, biventricular assist devices (BVAD) or extracorporeal membrane oxygenation (ECMO) are often required. Some pediatric patients with cardiac defects such as anomalous left coronary artery from the pulmonary artery and cardiomyopathy with predominant left ventricular dysfunction a re well supported with LVAD. Size considerations are also important in pediatric patients requiring mechanical circulatory support. Both ECMO and VAD can be used to support even the smallest neonates. Priming volumes for ECMO tend to be somewhat larger with the smallest circuits requiring approximately 350 mm, while the smallest VAD priming is less than 150 mm.
 

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4.

What are the currently available options for mechanical circulatory support in children?

ECMO and VAD are both used successfully in pediatric patients. A typical ECMO circuit employs a roller pump, a membrane oxygenator, a heating element, and a servo-regulatory mechanism for controlling circuit flow and preventing collapse of the cardiac structures against the venous cannulae. VAD circuits do not contain an oxygenator. Cannulation can be performed for right ventricular (RVAD), left ventricular (LVAD), or biventricular support (BVAD). The absence of an oxygenator simplifies the circuit, and reduces the required priming volumes. On the other hand, the absence of an oxygenator makes VAD somewhat less flexible. The occurrence of biventricular failure or pulmonary failure might necessitate changing the type of VAD or converting support to ECMO.
 

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5.

What type of ventricular assist devices exist for pediatric patients?

The majority of published experience with VAD in children has involved the use of a centrifugal non-pulsatile pump with a variety of pump head sizes. The circuit is simple, with an intake port, an arterial perfusion port, and the appropriate tubing to complete the circuit. There is a flow probe in the input tubing to monitor flow, and since these pumps are not occlusive, to detect flow reversal when weaning from support. Heparin-bonded tubing to decrease t he risk of thrombus formation has also been advocated. There are currently no approved devices available in the United States that use a pulsatile perfusion pump. Pulsatile systems are used in Europe and are under investigation in the United States. One of the major obstacles in pediatric patients has been the requirement for a variety of stroke volumes that would serve patients ranging in size from infants to young adults. Adult-size pulsatile systems have been used in the United States to support children as young as ten years of age in the 40-50 kg range.
 

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6.

How long can circulatory support be continued?

The main limitation to long-term circulatory support in children is the need for immobilization and the onset of complications related to extracorporeal circulation, including bleeding and infection. In most series using either ECMO circuits or VADs for cardiac support, the incidence of serious complications requiring discontinuation of support begins to rise after one week of support. There are reports of successful support of children with cardiac failure for periods up to four to five weeks, however, these are rare cases and in most instances, cardiac transplantation was required to wean off mechanical circulatory support.
 

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7.

What are the outcomes and complications for mechanical circulatory support in children with cardiac disease?

The results for ECMO for all indications are maintained by the Extracorporeal Life Support Organization (ELSO). ELSO maintains a large database that covers a variety of features for each ECMO case that is performed by participating institution. A similar database for VAD in children is currently being developed at the Royal Children's Hospital in Melbourne, Australia. Extracorporeal membrane oxygenation (ECMO) has been the most widely-used method of circulatory support in children with cardiac dysfunction. For pediatric cardiac patients, the probability of survival with ECMO support has consistently been in the 40-50% range. The group with the worst outcome are patients that are unable to be weaned from bypass following cardiac surgery, with the exception of infants who have the best survival chances because of an anomalous left coronary artery from the pulmonary artery. Children placed on ECMO support emergently for cardiac arrest also have a better outlook, with reported survival probability of 50-70%. The results for children supported with VAD are similar to those in children supported with ECMO, in the 40-50% range. The most frequent complication in pediatric patients is hemorrhage related to the need for ongoing anticoagulation in patients in the immediate postoperative period. Pulmonary edema from left ventricular distension and high left a trial pressures can also occur if left ventricular decompression is not achieved. Although the incidence is relatively low, central nervous system complications including stroke and cerebral hemorrhage can also occur. One of the most common late complications is sepsis, frequently from gram negative enteric bacteria, which increases in incidence by seven to ten days of support. Mechanical complications related to malfunction of the circuit are relatively rare.
 

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8.

What are the future directions of mechanical circulatory support in children?

The development of pulsatile VAD systems for children is ongoing and a system is likely to become available in the next two to three years in the United States. Some devices currently available in Europe provide pumps with a variety of stroke volumes with a minimum volume as low as 9 mm. Such pulsatile systems may provide an advantage in the maintenance of end organ function over non-pulsatile systems during prolonged periods of support. Another evolving area is in the development of totally implantable systems for chronic support in children, similar to currently available devices in adults such as the TCI Heartmate. These devices use an electrical pump in which energy is provided by an external induction coil. New devices will also have to address features that are unique to pediatric circulatory support such as size constraints and the need to support chronic circulatory failure in children with univentricular physiology after the Fontan procedure.
 

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Additional Pediatric Cardiac Surgery FAQs

* Pediatric Cardiopulmonary Transplantation
* Mechanical Circulatory Support in Children
* Bidirectional Cavopulmonary Shunt
* Hypoplastic Left Heart Syndrome