**4. Current outcomes for adults with congenital heart disease**

#### **4.1. Mechanical circulatory support**

In the treatment of heart failure, the emergence of mechanical circulatory assist devices has become a widely accepted option for individuals who either do not meet the transplant criteria or as a bridge to transplantation. Despite their widespread use in non-ACHD patients, mechanical circulatory assist devices are not as easily applied to the ACHD population because many of these patients present with anatomical challenges such as single ventricles, vascular reconstruction of major arteries, and systemic right ventricles [2]. The complexity of anatomical variants in addition to the presence of comorbidities contributes to a higher perioperative complication rate compared with the non-ACHD population. These adverse events include higher rates of hepatic dysfunction, respiratory failure, renal dysfunction requiring dialysis, and sustained cardiac arrhythmias [13]. When compared with a matched non-ACHD cohort, Cedars et al. found that early survival in the first 5 months post-implantation was worse in the ACHD population but comparable thereafter, and functional status and quality of life parameters were similar in both groups. They attributed these findings to the operative and perioperative factors unique to the ACHD population, particularly anatomic issues and increased likelihood of having previous sternotomies. Overall, results suggest that MCS is a good option for ACHD patients with advanced heart failure despite increased peri-operative complications and mortality as a bridge to transplant and may be a viable option as destination therapy in the future. Outcomes after MCS implantation are shown in **Table 1**.

**Source Study description Purpose Results**

To compare mortality between ACHD and non ACHD patients after device implantation.

Surgical Management for Advanced Heart Failure in Adults with Congenital Heart Disease

To collect data on utilizing LVAD's as bridge to transplantation devices in patients with previously palliated transposition of great

To analyze waitlist outcomes for ACHD versus non ACHD patients in heart transplantation.

To provide data for ACHD patients with VAD

implantation.

A case study that discusses the outcome of an LVAD implantation in an ACHD patient with a failing Fontan circulation as bridge to transplant

therapy.

arteries.

The survival rate was similar between ACHD and non-ACHD patients with

83

http://dx.doi.org/10.5772/intechopen.78218

Heart failure symptoms improved in all patients; therefore, a VAD may be a suitable treatment option in bridge to transplant for patients who are

Adults with CHD were much less likely to have a VAD (5 versus 14%) and were more likely to be given a lower urgency status. These patients were also more likely to experience cardiovascular related death with waiting to undergo heart transplantation (60 versus 40%). The utilization of VAD's should be explored to determine if survival for ACHD patients can be improved.

Five patients survived to discharge: one patient was successfully transplanted, one patient survived 262 days; one patient received 988 days of therapy while awaiting transplantation as of December 1, 2012; and two patients who received VADs as destination therapy received 577 and 493 days and were still alive as of December 1, 2012. VAD implantation is a viable option for therapy in ACHD patients in either bridge to transplant or bridge to

This case report suggests that LVAD's can become useful in patients with ACHD, particularly those with failing Fontan circulation as either bridge to transplant or bridge to

destination therapy.

destination therapy.

LVAD's.

severely ill.

Data entered into the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) from June 2006 to December 2015 was utilized. The 126 ACHD patients were categorized as follows: 63 systemic morphologic left ventricle, 45 systemic morphologic right ventricle, and 17 single ventricle.

systemic right ventricular failure after a Mustard operation were implanted with

314 of which were diagnoses with ACHD was conducted to identify key differences in listing status and outcomes.

of six ACHD patients who underwent VAD implantation.

An ACHD patient with failing Fontan circulation was implanted with an LVAD device and went on to have a successful heart transplantation

5 months later.

a HeartMate II VAD.

Maly et al. [17] Five adult patients with

Everitt et al. [18] An analysis of 9722 adults,

Shah et al. [19] A retrospective analysis

Newcomb et al.

[20]

VanderPluym et al. [13]

#### **4.2. Transplantation**

ACHD patients experience a variety of disadvantages when seeking transplantation as a treatment for their heart failure. Issues such as anatomical concerns and immune status can impact their ability for transplant candidacy significantly. If these factors do not influence their ability to be placed on the transplant registry, the ACHD population experiences a higher waitlist mortality than non-ACHD patients. This can be attributed to factors such as ACHD patients typically being of a younger age and less likely to utilize mechanical circulatory assist devices due to clinical barriers. As a result, they may experience longer wait list periods, a greater incidence of death while waiting for a transplant, or delisting [5].

The outcomes for ACHD patients that are successfully transplanted vary depending on short-term versus long-term comparisons and are shown in **Table 2**. Short-term outcomes for ACHD patients, similar to outcomes after MCS, are worse than when compared to non-ACHD patients: 20–30% mortality at 30 days mortality [1]. This increased mortality rate can potentially be explained by unique challenges associated with the ACHD population such as anatomical concerns and longer times of ischemia during surgery due to the need for reconstruction during the transplant [15]. One study by Paniagua Martn et al. [16] suggests that the cause for this difference can be attributed to a higher incidence of primary graft failure in ACHD patients. Despite increased peri-operative mortality, the long-term survival for ACHD patients is outstanding, with a median survival of greater than 20 years [2].


**4. Current outcomes for adults with congenital heart disease**

In the treatment of heart failure, the emergence of mechanical circulatory assist devices has become a widely accepted option for individuals who either do not meet the transplant criteria or as a bridge to transplantation. Despite their widespread use in non-ACHD patients, mechanical circulatory assist devices are not as easily applied to the ACHD population because many of these patients present with anatomical challenges such as single ventricles, vascular reconstruction of major arteries, and systemic right ventricles [2]. The complexity of anatomical variants in addition to the presence of comorbidities contributes to a higher perioperative complication rate compared with the non-ACHD population. These adverse events include higher rates of hepatic dysfunction, respiratory failure, renal dysfunction requiring dialysis, and sustained cardiac arrhythmias [13]. When compared with a matched non-ACHD cohort, Cedars et al. found that early survival in the first 5 months post-implantation was worse in the ACHD population but comparable thereafter, and functional status and quality of life parameters were similar in both groups. They attributed these findings to the operative and perioperative factors unique to the ACHD population, particularly anatomic issues and increased likelihood of having previous sternotomies. Overall, results suggest that MCS is a good option for ACHD patients with advanced heart failure despite increased peri-operative complications and mortality as a bridge to transplant and may be a viable option as destina-

tion therapy in the future. Outcomes after MCS implantation are shown in **Table 1**.

incidence of death while waiting for a transplant, or delisting [5].

patients is outstanding, with a median survival of greater than 20 years [2].

ACHD patients experience a variety of disadvantages when seeking transplantation as a treatment for their heart failure. Issues such as anatomical concerns and immune status can impact their ability for transplant candidacy significantly. If these factors do not influence their ability to be placed on the transplant registry, the ACHD population experiences a higher waitlist mortality than non-ACHD patients. This can be attributed to factors such as ACHD patients typically being of a younger age and less likely to utilize mechanical circulatory assist devices due to clinical barriers. As a result, they may experience longer wait list periods, a greater

The outcomes for ACHD patients that are successfully transplanted vary depending on short-term versus long-term comparisons and are shown in **Table 2**. Short-term outcomes for ACHD patients, similar to outcomes after MCS, are worse than when compared to non-ACHD patients: 20–30% mortality at 30 days mortality [1]. This increased mortality rate can potentially be explained by unique challenges associated with the ACHD population such as anatomical concerns and longer times of ischemia during surgery due to the need for reconstruction during the transplant [15]. One study by Paniagua Martn et al. [16] suggests that the cause for this difference can be attributed to a higher incidence of primary graft failure in ACHD patients. Despite increased peri-operative mortality, the long-term survival for ACHD

**4.1. Mechanical circulatory support**

82 Heart Transplantation

**4.2. Transplantation**


**Source Sample/study description Purpose Results**

To evaluate the post transplantation prognosis in

Surgical Management for Advanced Heart Failure in Adults with Congenital Heart Disease

To evaluate the survival outcomes for patients post heart transplantation.

To analyze the survival rate of adult patients with CHD post cardiac transplantation in comparison in those without CHD.

To evaluate the survival of adults with CHD after listing and transplantation.

To evaluate the survival outcomes of cardiac transplantation in adults with CHD in a contemporary

To investigate outcomes and risk factors for mortality and retransplantation for the ACHD population in comparison to the non-ACHD population.

To examine survival, causes of death and predictors of early (<1 year), midterm (1–5 years) and later (0.5 years) mortality in ACHD patients who received cardiac transplants.

cohort.

The 30-day mortality rate is elevated in the ACHD population: 16 versus 6%. However, there is not a statistical significance in the 5 and 10-year survival rates for ACHD patients in comparison to

http://dx.doi.org/10.5772/intechopen.78218

85

Having a diagnosis of ACHD is one of the most powerful predictors of 1-year mortality. But at 10 years it is associated with a marked survival advantage conditional on a 3-year survival independent of age.

The survival rate for patients with ACHD post-transplantation was 79% at 1 year and 60% at 5 years. A difference between this population and the control populations was

The early mortality rate (30 day) among ACHD patients was high (reoperation 18.9 versus 9.6%; nonreoperation 16.6 versus 6.3%), but at 10 years the survival rate was equivalent with non-ACHD patients (53.8 versus 53.6%)

There was no significant difference in survival of ACHD versus non-ACHD at 30 days (89 versus 92%), 1 year (84 versus 86%), or 5 years

The overall post-transplantation mortality and retransplantation rates were significantly higher for patients with ACHD mainly due to

Early mortality rates for the ACHD population was high in comparison to the non-ACHD transplant recipients (10 versus 4%). The long-term survival rates for ACHD patients who survived the early hazard phase was superior to the

non-ACHD patients.

not present.

(70 versus 72%).

an early hazard phase.

non-ACHD patients.

adults with CHD.

Patel et al. [27] Data reported to UNOS from

had CHD.

controls.

with CHD.

A review of heart

Taylor et al. [28]

Lamour et al. [29]

Davies et al. [8]

Bhama et al. [30]

Karamlou et al. [31]

Burchill et al. [32]

1987 to 2006 was reviewed and categorized to compare adults with CHD versus other diagnoses in heart transplantations. 2% of the individuals in this study period

Data from heart transplantations performed from 2001 to 2003 was utilized to calculate survival rates by the Kaplan–Meier method. Adults with CHD represented 2.7% of the cohort.

The post-transplantation outcomes for 24 adults with CHD were analyzed utilizing the Kaplan–Meier statistical method to estimate survival functions for patients with CHD versus all others and patients with CHD versus matched

A retrospective study of patients listed for primary transplantation between 1995 and 2009 was conducted. 2.5% of these patients were adults

A retrospective analysis was conducted from January 2001 to February 2011. 19 patients with ACHD were compared to 428 patients with non-ACHD who underwent transplantation.

transplantation patients from 1990 to 2008 reported to UNOS was conducted. A total of 8496 patients were evaluated, of which 575 had ACHD.

A retrospective study was conducted on patients who were identified in the registry of ISHLT between 1985 and 2010. The Kaplan–Meier method was used to conduct a survival comparison. 2.2% of patients transplanted in this cohort had a

diagnosis of ACHD.

**Table 1.** Outcomes after mechanical circulatory support device implantation in adults with congenital heart disease.



**Source Sample/study description Purpose Results**

**Source Study description Purpose Results**

A case study of LVAD implantation in an ACHD

To explain the case reports of two ACHD patients who received LVAD bridge to transplant therapy.

To determine the relationship that exists between VAD implantation and successful transplantation in patients that are listed for heart transplant.

A case report of three ACHD patients that underwent LVAD implantation.

To analyze the pretransplant effects of mechanical circulatory support on posttransplant outcomes in the ACHD

population.

**Table 1.** Outcomes after mechanical circulatory support device implantation in adults with congenital heart disease.

patient.

which LVAD implantation was utilized as therapy option in an ACHD patient that presented with failure of the systemic

successfully bridged to transplantation utilizing LVAD therapy. Their deterioration leading to the need for transplant can be attributed to their deteriorating right ventricular failure.

ACHD patients reported in the UNOS database from 1985 to 2010 in which these patients were compared to non –ACHD patients in terms of VAD use at listing, listing status, status upgrades and reasons for

had congenitally corrected transposition of the great arteries underwent LVAD implantation as a therapy option for their end stage heart

Data collected from September 1987 to September 2012 by the Scientific Registry of Transplant Recipients was utilized to compare the following between MCS and non-MCS ACHD patient populations: procedural, outcome and survival.

Morris et al. [21] A presentation of a case in

84 Heart Transplantation

ventricle.

Stewart et al. [22] Two ACHD patients were

Gelow et al. [23] A retrospective study of 1250

upgrades.

Joyce et al. [24] Three adult patients that

failure.

To explore data on outcomes of cardiac transplantation in the ACHD patient group.

Operative mortality for ACHD patients following cardiac transplantation is higher than for other diagnostic groups. However, long term survival is noted to be good and comparable to non

Utilization of the LVAD therapy significantly improved the patients cyanosis and ventricular function. This suggests that patients with ACHD could benefit from utilizing

This report suggests that LVAD therapy is a viable option for bridge to transplantation in ACHD patients that present with right ventricular

It was noted that the use of VAD's in ACHD patient was less at both the time of listing and transplantation. This decreased usage of VAD therapy in ACHD patients contributes to lower listing status and organ

LVAD therapy.

failure.

allocation.

LVAD implantation can be successfully completed in ACHD patients when placed under echocardiographic guidance. This offers an additional therapy option

In the ACHD patient population, those with MCS are associated with higher transfusion rates and length of stay however, they do not have less favorable outcomes post-transplant when compared to non-MCS ACHD

for ACHD patients.

patients.

ACHD patients.

Outcomes were reviewed from 38 cardiac transplants performed in 37 patients from 1988 to 2009 using medical records and transplant databases. 41% had univentricular and 59% had biventricular physiology.

Irving et al. [26]

Maxwell et al.

[25]


higher early mortality rate post heart transplantation in comparison to non-ACHD patients. However, at this point in time little research has been focused on identifying the clinical source for this mortality contrast. It is essential that research efforts focus on seeking out the root of this disparity in order to work towards minimizing the presence of this current complex outcome. Doing so will supply the medical community with more accurate predictors of mortality when seeking heart transplantation as treatment for these patients and provide

Surgical Management for Advanced Heart Failure in Adults with Congenital Heart Disease

http://dx.doi.org/10.5772/intechopen.78218

87

• Determining the appropriate timing/type of interventions to utilize for this clinically diverse group. Due to the clinical diversity that exists within the ACHD patient populations applying standardized treatment regimens remains challenging. Case studies exploring how to effectively manage different anatomical morphologies currently exist but this aspect of research still remains relatively unexamined and information specifically regarding timing is rather limited. Increasing the knowledge in terms of how to effectively approach treatment in ACHD patients in terms of when and how to intervene will assist in decreasing the complexity of approaching a therapy regimen and provide stronger evidence to provide

• Re-evaluating how ACHD patients are listed into the transplant registries. With the current listing guidelines ACHD patients are at a significant disadvantage in terms of their likelihood of being successfully transplanted. As of now, ACHD patients are more likely to experience a lower listing status with their initial listing than non-ACHD patients. In addition, ACHD patients experience a high rate of delisting after 1 year due to a decline in their worsening condition. These patients are placed at an even further disadvantage because they may not be candidates for mechanical circulatory support due to anatomical constraints. Therefore, they are unable to utilize the placement of these devices to prolong their survival to successfully reach transplantation, or utilize the benefits of attaining a higher listing status associated with these interventional therapies. The current listing criteria for heart transplantation is a cause of serious concern when considering ethical and effective medical management for patients with ACHD. There is an urgent need for re-evaluation of these current guidelines to occur in order to take into consideration the unique medical challenges presented by this growing population that will continue to rely on heart trans-

• Exploring the use of MCS as destination therapy in addition to bridge to transplantation. The utilization of these devices for treatment in ACHD patients has previously focused on their usage as bridge to transplant therapy. However, with the increasing demand for heart transplantation, it is imperative that other therapy options are considered for ACHD patients. More recently, the use of MCS has been considered as destination therapy for this group of patients. Current research indicates that there is potential for pursuing this line of treatment option for a variety of ACHD subgroups. Doing so would provide an effective treatment option for these patients and relieve some of the current burden on the

It is evident that the ACHD population presents with a variety of unique challenges and considerations that still need to be explored. Addressing each of these areas mentioned above

better outcomes to those who undergo this type of medical management.

the best possible clinical outcomes for these patients.

plantation as one of their main treatment possibilities in the future.

transplant system.

**Table 2.** Outcomes after heart transplantation in adults with congenital heart disease.

Regardless of this data, outcomes for patients with ACHD after transplantation vary depending on their initial diagnosis. As there are a variety of clinical manifestations of ACHD, assessing prognostic values remains challenging and therefore individuals should be evaluated thoroughly prior to transplant consideration.
