Mechanical Circulatory Support for Patients With Adult Congenital Heart Disease

Abstract

Advances in surgical and medical care of children born with heart defects have led to the emergence of a unique subgroup of young adults known as adults with congenital heart disease (ACHD). Heart failure (HF) is the leading cause of mortality and morbidity in this subset. Management of HF is challenging in these patients owing to inherent structural variations with their associated physiological consequences. Heart transplantation is of limited utility in this group either because of donor shortage or associated comorbidities that make these patients ineligible for transplantation. Mechanical circulatory support (MCS) devices have evolved as an alternative treatment modality in supporting the failing myocardium of this population, but are often used less frequently than in those with a structurally normal heart because of the unique anatomical and physiological variations. These variations create a need to gather adequate knowledge on how best to support the hearts of ACHD patients in order to reduce mortality and morbidity. This review presents clinical experience with MCS in ACHD patients.

Advances in the surgical and medical care of children born with congenital heart disease (CHD) has led to the emergence of a subset of young patients with heart diseases, known as adults with CHD (ACHD).¹ Compared with the normal population, ACHD patients are more vulnerable to heart failure (HF), and a large population-based study showed chronic HF as the leading cause of mortality in the ACHD population² (Figure 1). In addition, ACHD patients pose a significant challenge to medical treatment modalities compared with those with a structurally normal heart.³ Thus, many ACHD patients are waiting for heart transplants, but the shortage of donors has made this treatment strategy difficult. Often pulmonary vascular disease, high levels of allo-sensitization, or comorbidities such as liver cirrhosis make some of these cases ineligible for transplantation.⁴

Figure 1.

Mortality in adult congenital heart diseases: CONCOR registry. AoS/BAV, aortic stenosis/bicuspid aortic valve; ASD, atrial septal defect; AVSD, atrioventricular septal defect; cc-TGA, congenitally corrected transposition of the great arteries; CoA, aortic coarctation; DORV, double outlet right ventricle; Ebstein, Ebstein’s anomaly; Marfan, Marfan syndrome; PA, pulmonary atresia associated with ventricular septal defect; PS, pulmonary stenosis; TA, tricuspid atresia; TGA, transposition of the great arteries; ToF, tetralogy of Fallot; UVH/DILV, univentricular heart/double inlet left ventricle; VSD, ventricular septal defect. (Reproduced with permission from Verheugt CL, et al.²)

Mechanical circulatory support (MCS) has emerged as an alternative method to support the failing myocardium in ACHD patients awaiting transplant, or it can be used as a definitive treatment modality. MCS acts by unloading the failing ventricle, thereby reducing myocardial oxygen demand, enhancing recovery, and enabling adequate systemic perfusion. Despite advances in device configuration, surgical techniques, and management, MCS is used less often in ACHD patients than in patients with acquired heart disease because of the inherent anatomic and physiological variations in the former.⁵ These variations create a need to gather adequate knowledge on how best to support the hearts of ACHD patients in order to reduce mortality and morbidity. This review presents clinical experience with MCS in ACHD patients.

HF in ACHD

Most patients who underwent repair for CHD in childhood have a risk of developing HF during adult life. A recent review by Stout et al clearly describes the possible mechanisms of late ventricular dysfunction in patients with repaired CHD.⁶ Further, there is a >20-fold increased risk of death in HF patients with CHD than in those with a structurally normal heart. The potential mechanisms of HF among ACHD patients include myocardial injury during prior procedures, ongoing ventricular overload (volume or pressure) due to residual lesions, altered coronary perfusion, pre-existing ventricular non-compaction, altered anatomy, and neurohormonal activation.^7,8 Patients who have a morphological right ventricle (RV) as a systemic ventricle (i.e., patients who underwent an atrial switch procedure for dextro-transposition of the great arteries or those who underwent a physiologic repair for congenitally corrected transposition of the great arteries [ccTGA]) are at a particularly high risk of late ventricular dysfunction. Furthermore, development of myocardial fibrosis, as seen in patients with repaired tetralogy of Fallot or in the RV in the systemic circulation, is strongly associated with the development of late ventricular dysfunction.^9–11

The Fontan procedure is performed for various types of functional single ventricle lesions. Thus, by the time patients who have undergone a successful palliation procedure for a single functional ventricle reach adulthood, they have a Fontan circulation where systemic venous circulation lacks a pump and depends totally on low pulmonary vascular resistance. Because the Fontan circulation has no subpulmonary ventricle to drive the systemic venous circulation, it is inevitably susceptible to subtle anatomic or physiologic changes over the years, resulting in a failing Fontan circulation. The mechanisms underlying a failing Fontan circulation are complex and multifactorial.^12,13 In the setting of the classic Fontan configuration, a massively dilated right atrium, which makes the circulation energy inefficient, and refractory atrial arrhythmias are the main causes of failure. Some patients may be candidates for a conversion procedure to the extracardiac Fontan configuration. The contemporary form of the Fontan procedure, total cavopulmonary connection (TCPC) with either an extracardiac graft or a lateral tunnel, has been popular since the 1990 s and most patients who are currently reaching adulthood have a TCPC configuration. The mechanisms responsible for the failing TCPC-type Fontan circulation include progressive increases in pulmonary vascular resistance and Fontan pressure, systolic or diastolic dysfunction of the systemic ventricle, mechanical obstruction of the Fontan pathway either from anatomic distortion or failed reconstruction, or thrombosis and subclinical pulmonary emboli. In addition, some patients experience clinical deterioration because of protein-losing Enteropathy or plastic bronchitis even though the Fontan pressure remains unchanged.

MCS Strategies

MCS can be used as a bridge to decision, bridge to recovery, bridge to bridge (to subsequent long-term MCS), and bridge to transplant (BTT), or as destination therapy (DT). MCS can be used over the short, medium or long term, as described in Table 1.

Table 1. Classification of Mechanical Circulatory Support

Short term (Days–weeks)	Mid and long term (Weeks–years)
Extracorporeal membrane oxygenation	Pulsatile flow devices
Centrifugal ventricular assist devices	EXCOR
ROTAFLOW	PVAD/IVAD
PediMag	Continuous flow devices
CentriMag (St. Jude, Minneapolis, MN, USA)	DuraHeart
Impella	HeartWare HVAD
Tandem Heart	HeartMate II
Intra-aortic balloon pump	Jarvik 2000
	SynCardia TAH

HVAD, Heartware ventricular assist device; IVAD, implantable ventricular assist device; PVAD, percutaneous ventricular assist device; TAH, total artificial heart.

The Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) scale is a helpful tool to stratify HF patients with ACHD requiring MCS. Using the INTERMACS scale, patients are classified based on the severity of symptoms and the trajectory of decline over time, which helps prognosticate those with advanced HF receiving MCS.^2,14

Extracorporeal membrane oxygenation (ECMO) can be established either percutaneously or by an open technique (cut down). Cannulation can be either in the neck (internal jugular vein and the common carotid artery), groin (femoral vessels), or central (right atrial appendage and the aorta). Patients with venous anomalies and prior cavopulmonary anastomosis require special attention with regard to cannulation strategies.¹⁵

Ventricular assist devices (VADs) have been described as “mechanical pumps that take over the function of the damaged ventricle and restore normal hemodynamics and end-organ blood flow”.¹⁶ Inherent advantages of VADs include small priming volume, fewer anticoagulation and blood products, with a subsequent reduction in the infection rate and sensitization,^14,17 the fact that patients can be extubated, and early mobilization. The classification of VADs¹⁸ is summarized in Table 2.

Table 2. Ventricular Assist Device Classification

Basis	Type
Ventricle supported	LVAD, RVAD, BiVAD
Duration of support	Short term, intermediate, long termA
Purpose of support	BRT, BTT, DT
Device location relative to the body	Paracorporeal, extracorporeal, extracorporealB
Generation of the device	First, second, third
Drive mechanism	Pneumatically driven, electrically driven
Pump mechanism	Axial, centrifugal
Flow characteristics	Continuous-flow devices, pulsatile-flow devices

^AShort term support ranges from 6 h to 7 days (e.g., Thoratec percutaneous ventricular assist device [PVAD], Levotronics Centrimag), intermediate support ranges from 7 days to 1 year (e.g., Thoratec PVAD, HeartMate II, Heartware), and long-term support is for >1 year (e.g., HeartMate II, Heartware). ^BExamples of paracorporeal-, extracorporeal-, and intracorporeal-located devices are the Thoratec PVAD, Levotronics Centrimag, and HeartMate II (Heartware), respectively. BiVAD, biventricular assist device; BRT, bridge to recovery; BTT, bridge to transplant; DT, destination therapy; LVAD, left ventricle assist device; RVAD, right ventricle assist device.

VADs are indicated in the case of a failing systemic ventricle (morphological right, morphological left, or single ventricle) refractory to optimal medical management with suboptimal circulation before end organ damage occurs. In recent years, VAD support to determine candidacy for heart transplantation (bridge to candidacy) has become more common. Hence, VAD therapy no longer needs to be a “bridge”, but simply a mode of treating medically resistant HF (chronic VAD therapy). VAD in ACHD may help treat transplant-limiting pulmonary hypertension¹⁹ in patients who experience long transplant wait times, and serve as DT when there are contraindications to transplantation.²⁰ Extreme prematurity, low birth weight (<2.5 kg), significant neurologic impairment, a barrier to adequate anticoagulation, a constellation of congenital anomalies with poor prognosis, and major chromosomal aberrations generally preclude VAD therapy.

VAD implantation in CHD is different than in the structurally normal heart due to the altered mediastinal anatomy and cardiac physiology secondary to previous multiple operations, which may include systemic-pulmonary artery shunts and a disconnected inferior vena cava after a Glenn shunt or Fontan operations. Anatomic considerations related to prior complex repairs, abnormal size and location of the aorta, orientation of the ventricular chambers, heart position in the chest, devices designed for a morphologic left ventricle, and comorbidities of coagulopathy, malnutrition, and end organ dysfunction make VAD implantation technically challenging. Other considerations include the thickness of the ventricles, semilunar valve regurgitation and intracardiac shunts.²¹

Numerous publications have reported details of surgical implantation. Either epicardial or transesophageal echocardiography is used to assist with cannula placement. The RV free wall and diaphragm surface are both alternative options in order to best orient the inflow cannula towards the tricuspid valve. Regardless of the epicardial entrance point, implantation of devices into a systemic RV may require resection of trabeculae to provide unobstructed inflow. To avoid issues with damage to the liver or bowel, or chamber compression upon closing the chest, the device may be placed back-to-front, closer to the midline, or through the right chest. Non-sternotomy approaches and non-aortic outflow graft positions have also been used. Prêtre et al described the creation of a VAD-driven subpulmonic chamber following take-down of a Fontan circuit.²² Others have described total artificial heart (TAH) implantation in Fontan patients requiring the creation of neo right atria, which can be problematic due to the lack of normal compliance.^23,24

The TAH (SynCardia Systems, Tucson, AZ, USA) consists of right- and left-sided pumps and can be used as a BTT or as DT. TAH pumps can be configured in a variety of ways to addresses various physiologic (1 or 2 ventricles) and anatomical variations peculiar to CHD. The TAH provides optimal hemodynamic support in CHD patients with residual lesions compared with a VAD or biventricular assist device (BiVAD) alone; however, implantation of TAH is much more challenging than VAD implantation, especially in CHD patients considering the wide range of anatomical variations. One of the major advances in the TAH implantation is the development of patient-specific virtual implantation based on cross-sectional imaging studies.²⁵

An important change in VAD technology is the introduction of continuous-flow (CF) VADs. These can be implanted intracorporeally, which also allows for outpatient care. Over the past few decades there has been a major shift in use from pulsatile VAD to CFVAD, and studies have reported superior survival rates for CFVAD vs. pulsatile left ventricular assist devises (LVAD).^26,27 Percutaneous VADs are another significant technological innovation in VADs, and these are increasingly being used as short-term support in patients with critical cardiogenic shock and postcardiotomy failure.^28,29 Tandem Heart (TandemLife, Pittsburgh, PA, USA) is a percutaneous VAD that uses cannulas placed in the right atrium or trans-septally to facilitate the direct unloading of either the right or left heart.³⁰ Tandem Heart systems can also be used to establish short-term ECMO circuits under different circumstances of cardiopulmonary failure.³¹

MCS in ACHD Patients With Biventricular Physiology

MCS is challenging in ACHD patients with biventricular physiology because of ventricular morphology, residual lesions, systemic venous abnormalities, limited vascular access, previous multiple surgeries (sternotomies, thoracotomies), pulmonary hypertension, aortopulmonary collaterals, coagulopathy, and end organ dysfunction.

There are limited data on postoperative ECMO in ACHD patients as well as ECMO survival. The underlying cardiac anatomy, along with the associated comorbidities, complicates the decision to use ECMO in ACHD patients.³² A study from the Mayo Clinic showed a survival rate at discharge of 46% in high-risk ACHD patients requiring postcardiotomy ECMO support.³³ Similarly, discharge survival rates in the studies of Maybauer et al³⁴ and Schmidt et al³⁵ were 50% and 42%, respectively. In an analysis of Extracorporeal Life Support Organization data from ACHD patients requiring ECMO, Paden et al reported a 39% survival rate.³⁶

Compared with ECMO, the use of VADs in ACHD patients is encouraging. A systematic review of durable MCS in teenagers and ACHD revealed the frequent utilization of MCS in patients with ACHD.³⁷ Short-term survival rates in published series are approximately 70%, and the use of durable MCS as a BTT was 77%³⁷ (Figure 2). In an INTERMACS analysis by VanderPluym et al, 21% of cases were successfully bridged to transplant and 51% were alive with the devices.³⁸ That analysis also showed the promising role of LVAD for the treatment of HF in ACHD, with similar survival rates to those for non-ACHD patients³⁸ (Figure 3).

Figure 2.

Durable mechanical circulatory support in teenagers and adults with congenital heart disease. (A) Clinical course and vital status (at the time of report) by type of prior surgical repair. *The vital status was unknown for 1 patient each from the atrial switch and Fontan groups. (B) Comparison of assist device types between 1999–2010 and 2011 onwards. Intermacs, Interagency Registry for Mechanically Assisted Circulatory Support; TAH, total artificial heart. (Reproduced with permission from Steiner JM, et al.³⁷)

Figure 3.

Outcomes following implantation of mechanical circulatory support (MCS) in adults with congenital heart disease (ACHD): analysis of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS). Kaplan-Meier survival curves are shown for (A) ACHD vs. non-ACHD, (B) ACHD vs. Non-ACHD for left ventricular assist devices (LVAD), and (C) ACHD vs. non-ACHD for biventricular assist devices (BiVAD) and the total artificial heart (TAH). (Reproduced with permission from VanderPluym CJ, et al.³⁸)

Shah et al emphasized the use of VADs as either BTT or DT in patients with ACHD.³⁹ The SynCardia TAH has now been implanted in over 50 patients <21 years of age and in more than 20 patients with ACHD.⁴⁰ From1986 to 2012, there have been 24 implants in this patient group, accounting for approximately 2% of all implants. The median age of patients was 28 years (range 13–56 years), median support time was 24 days (range 1–359 days), and overall survival was 62%.⁴⁰ Clinical experience with the use of TAH in CHD is summarized in Table 3.^24,41,42 TAH appears to be a good option for end-stage congenital heart patients who require multiple procedures so that a VAD can be implanted.⁴² The use of the EXCOR heart (Berlin Heart, The Woodlands, TX, USA) is limited in ACHD patients. Fishberger et al demonstrated the usefulness of percutaneous RV support (Impella; Abiomed, Danvers, MA, USA) in providing hemodynamic stability during mapping and ablation of intra-atrial re-entrant tachycardia in patients after Mustard operation for TGA.⁴³

Table 3. Clinical Experience With the Total Artificial Heart in Patients With Congenital Heart Disease

References	Anatomy	Duration of support (days)	Surgical consideration	Outcome
Morales et al41	Dextrocardia, ccTGA status post Rastelli with severe AS, obstruction of LV-PA conduit	160	Separation of right and left pumps with parallel orientation	Bridge to transplant
Rossano et al24	PAt/IVS, status post Fontan	61	Creation of “neo-right atrium”	Bridge to transplant
Si et al42	Dextrocardia, ccTGA, VSD, PS, Ebstenoid TV, status post Senning-Rastelli with severe TR	8	Reversal of Senning	Bridge to transplant

AS, aortic stenosis; CcTGA, congenitally corrected transposition of the great arteries; LV, left ventricle; PA, pulmonary artery; PAt/IVS, pulmonary atresia with intact ventricular septum; PS, pulmonary stenosis; TR, Tricuspid regurgitation; TV, tricuspid valve; VSD, ventricular septal defect.

VAD to support a failing systemic RV with previous Senning or Mustard operation for TGA or ccTGA as a BTT is growing. Studies have shown that implantation of a VAD in a failing systemic RV has the potential to improve physiology and can be used as a bridge to heart transplantation.^44–46

Complex ACHD is not a contraindication to the use of MCS and should be considered as a treatment option early in the care of these patients. INTERMACS scores have trended towards lower acuity, suggesting implantation earlier in the course of advanced HF, as has been recommended for patients with acquired disease. LVADs have shown promise in treating HF in ACHD patients, with similar survival benefits as in non-ACHD patients. The role of VADs as DT is increasing. The use of VADs to support a failing systemic RV is encouraging. Virtual implantation techniques using cross-sectional imaging may significantly alter device selection in ACHD patients.

MCS in ACHD Patients With Single Ventricle Physiology

The most important factor predicting the anticipated survival for any single ventricle patient requiring MCS is the stage of palliation.⁴⁷ A patient’s current anatomy will affect their response to MCS.⁴⁸ These patients typically have altered mediastinal anatomy due to numerous previous sternotomies, are chronically anticoagulated, and have hepatic and renal dysfunction related to the obligate elevated central venous pressure associated with long-standing single ventricle physiology and diastolic dysfunction with preserved systolic function.^49,50

ECMO support in failing Fontan circulation is challenging and carries high mortality and morbidity rates. A survival to discharge rate of 35% was reported by Rood et al in Fontan failures supported by venoarterial ECMO.⁵¹ ECMO can be used in an acute setting as a bridge to decision or bridge to recovery. The stage of Fontan failure and patient selection determine the success of ECMO support in Fontan failure. The use of ECMO in advanced stages of Fontan failure is associated with even higher mortality rates due to the associate end organ damage.⁴⁷

There is growing evidence that VAD support can be successful in selected patients with a failing Fontan circulation.^52,53 However, it remains unclear when VAD implantation should be considered and what percentage of patients will benefit from a systemic VAD alone. Patients with isolated or predominant ventricular systolic dysfunction are likely to benefit from VAD alone, but such cases among patients with a failing Fontan circulation are rare. A VAD will surely not help the clinical situation if the end-diastolic pressure of the systemic ventricle is not high (at least >12 mmHg). However, patients with Fontan repair accounted for 14% of cases in the study of Steiner et al,³⁷ which showed increasing use of durable VADs in patients with ACHD with a short-term survival rate of 70%. Various clinical experiences with VAD in the failing Fontan circulation are summarized in Table 4.^{22–24,30,52,54–73} Fontan failure patients with marked end organ dysfunction, protein-losing enteropathy, and/or plastic bronchitis who are otherwise not good transplant candidates may benefit from TAH.⁴⁰ The feasibility of using a TAH in the failing Fontan circulation was demonstrated in a recent case report by Rossano et al.²⁴

Table 4. Clinical Experience With Ventricle Assist Devices in the Failing Fontan Circulation

References	Age (years)	Device	Duration of support (days)	Outcome
Matsuda et al54	10	Toyobo (pulsatile)	7	Death
Sadeghi et al55	8	BVS 5000 (pulsatile)	8	Transplant
Frazier et al56	14	Centrifugal pump then Heartmate	45	Transplant
Nathan et al57	4	EXCOR (BiVAD)	48	Transplant, died, MOF
Newcomb et al58	22	Thoratec	150	Transplant
Calvaruso et al59	10	EXCOR	7	Transplant
Ricci et al30	15	Tandem Heart	10	Died
Russo et al60	14	Heartmate	45	Transplant
Prêtre et al22	27	EXCOR (LVAD)	334	Alive
Cardarelli et al61	1.5	EXCOR (LVAD)	180	Death, non-cardiac
Morales et al62	15	Heartmate II (LVAD)	72	Transplant
VanderPluym et al63	3	EXCOR (LVAD)	174	Transplant
Mackling et al64	4	EXCOR (LVAD)	363	Death, sepsis
Sanders et al71	16	EXCOR (LVAD)	2	Transplant
VanderPluym et al72	2	EXCOR+CMag (BiVAD)	54	Death
Valeske et al23	19	EXCOR (BiVAD)	23	Transplant
Niebler et al65	4	Heartware	148	Transplant
Rossano et al24	13	SynCardia, TAH	61	Transplant
Hoganson et al73	4	EXCOR (LVAD)	26	Transplant
Halaweish et al52	14	EXCOR (LVAD)	179	Transplant
Arnaoutakis et al66	18	HVAD (LVAD)	NA	Transplant
Arnaoutakis et al66	14	SynCardia TAH	NA	Transplant
Arnaoutakis et al66	5	EXCOR (LVAD)	NA	Transplant
Arnaoutakis et al66	23	Thoratec (LVAD)	NA	Death
Poh et al67	NA	EXCOR (LVAD)	16	Death
Poh et al67	NA	EXCOR (LVAD)	17	Death
Poh et al67	NA	EXCOR (LVAD)	NA	Transplant
Poh et al67	NA	EXCOR (LVAD)	6	Transplant, death
Poh et al67	NA	EXCOR (LVAD)	150	Transplant
Woods et al68	11	HVAD (LVAD)	148	Transplant
Woods et al68	13	HVAD (LVAD)	272	Transplant
Woods et al68	17	HVAD (LVAD)	271	Transplant
Lorts et al69	22	HeartMate III (LVAD)	NA	Ongoing
Chen et al70	11	Thoratec (LVAD)	35	Transplant
Chen et al70	10	HVAD (LVAD)	76	Transplant
Chen et al70	10	HVAD (LVAD)	165	Transplant
Chen et al70	21	HVAD (LVAD)	69	Transplant

NA, not available. Other abbreviations as in Tables 1,2.

Fontan failure may be independent of ventricular function and often is driven by elevated pulmonary vascular resistance or pressure. Hence, VAD may or may not help in these patients, and cavopulmonary support may be necessary. Prêtre et al reported the first clinical experience with isolated cavopulmonary support in a case of failing Fontan as a BTT.²²

There are substantial research efforts into developing MCS for the failing Fontan circulation. Our group previously showed that a percutaneously implanted microaxial pump (Impella) for cavopulmonary assist in the failing classic Fontan physiology lowers the Fontan pressure, attenuates systemic venous congestion, and augments systemic oxygen delivery.⁷⁴ Wang et al used a percutaneous Wang-Zwische double-lumen cannula (DLC) for cavopulmonary support for 2 h in a failing Fontan sheep model.⁷⁵ Derk et al showed the feasibility of an axial pump (Jarvik 2000) to restore baseline hemodynamics and cardiac output in Fontan circulation in a pig model.⁷⁶ Rodefeld et al used a 3-dimensional computational model and showed that a single viscous Impella pump augments cavopulmonary blood flow and can be used as a bridge to recovery or transplant in patients with established univentricular Fontan circulation.⁷⁷ Our group is currently investigating a novel multilumen cannula to support the failing Fontan circulation by a computational fluid dynamic simulation model.⁷⁸

Improvements in palliation of single ventricle physiology have given rise to an increase in the number of single ventricle patients susceptible to HF later in life, which necessitates the use of some form of MCS. Supporting the failing myocardium in single ventricle patients is a complex challenging problem. Even though the literature shows the feasibility of MCS in patients with a failing single ventricle, the outcomes are mixed: MCS outcomes are better in early stages of failure than in the advanced stage. With technological advances in MCS devices and better understanding of single ventricle pathophysiology, more research is needed to determine the medium- and long-term outcomes of MCS in these patients.

Conclusions

With the advent of MCS devices it has become feasible to support ACHD hearts despite the inherent surgical challenges present in this population. The earlier MCS devices used as a salvage had suboptimal outcomes. Recent advances in MCS technology have provided more compact, durable, and better-profile devices. The use of short-term MCS devices has been extended to high-risk catheter laboratory procedures or cardiac interventions. Percutaneous MCS, the most recent advancement in MCS, can now be used in the resuscitation of patients in cardiogenic shock. Outcomes of long-term MCS in the ACHD population are encouraging and may be improved further when timing is optimized. The subgroup of ACHD patients with failing Fontan circulation is an area of ongoing clinical research. Even though the failing Fontan subgroup represents a significant proportion of the ACHD population, the best available modality to support these hearts is yet to be determined. With further research and technological advancements, it may be possible to improve prognosis in this subset of patients.

Acknowledgement

None.

Conflict of Interest

None declared.

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