**5. Conclusion**

Further investigation into the ACHD population is essential in order to effectively manage their unique medical concerns as this patient group continues to expand. This investigation must occur from multiple points in order to ensure the variety of distinct challenges presented by this population are adequately addressed. Specifically, there are four areas this chapter suggests future research efforts should focus on in order to provide the most advantageous information for medical management:

• The cause of increased early mortality rates in heart transplant operations for ACHD patients. After thorough review of the current literature, it is evident that ACHD patients experience 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 better outcomes to those who undergo this type of medical management.


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

**Source Sample/study description Purpose Results**

To analyze the survival probability between different subgroups with ACHD.

To develop a risk prediction model for posttransplant in hospital mortality in heart transplant patients.

To determine if there is an associated with early death post heart transplantation in patients who possess 1 V anatomy in ACHD.

The early mortality rating associated with ACHD can primarily be attributed to the presence of primary graft failure. The frequency of primary graft failure in ACHD was 23%, versus 17% in IHD and 13% in IDCM. The following is the frequency of early mortality rates: 25% CHD, 14%

IDCM, 16% IHD.

transplantation.

outcomes.

The model determined that the ACHD diagnosis is correlated with an odds ratio of 4.18 for early in hospital mortality post heart

ACHD patients that possess 1 V anatomy are associated with a higher death incidence post heart transplantation. Transplantation registries should include specific ACHD diagnoses due to the evident difference in associated

Survival outcomes in a total of 3166 patients were included: 1888 IHD, 1223 IDCM, and 55

Adults who underwent heart transplantation in the United States between January 2007 and June 2009 were utilized to determine and validate the risk prediction model. This efficiency of this model was further assessed by evaluating the performance in patients from July 2009 to October 2010 receiving heart transplants.

A comparison among in hospital deaths between ACHD patients that possessed either 1 V or 2 V anatomy was conducted retrospectively from 1993 to 2007 through data gathered in the Nationwide Inpatient

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

ACHD.

Further investigation into the ACHD population is essential in order to effectively manage their unique medical concerns as this patient group continues to expand. This investigation must occur from multiple points in order to ensure the variety of distinct challenges presented by this population are adequately addressed. Specifically, there are four areas this chapter suggests future research efforts should focus on in order to provide the most advantageous

• The cause of increased early mortality rates in heart transplant operations for ACHD patients. After thorough review of the current literature, it is evident that ACHD patients experience

thoroughly prior to transplant consideration.

Sample (NIS).

information for medical management:

**5. Conclusion**

Paniagua Martin et al. [16]

86 Heart Transplantation

Singh et al. [33]

Karamlou et al. [34]

> • 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 transplant system.

> 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

will vastly change and improve how ACHD patients are approached from a treatment standpoint and ultimately provide more advantageous clinical options that can successfully handle the complexities presented by this population.

of heart and lung transplantation : The official publication of the international society

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

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

89

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