Heart transplantation in children with markedly elevated pulmonary vascular resistance: Impact of right ventricular failure on outcome

doi:10.1016/j.healun.2010.12.007

The Journal of Heart and Lung Transplantation

Volume 30, Issue 6, June 2011, Pages 659-666

https://doi.org/10.1016/j.healun.2010.12.007 Get rights and content

Background

Pulmonary hypertension causes increased morbidity and mortality in adults after heart transplantation. The effect of markedly elevated pulmonary vascular resistance (PVR) on post-transplant outcomes in children has not been well described.

Methods

Outcomes were compared in a retrospective study between 58 children with an elevated PVR index (PVRI) ≥ 6 U/m² and 205 children with a PVRI < 6 U/m². Patients who did and did not respond to acute vasodilator testing and patients who underwent transplant before (pre-1995) and after (post-1995) the availability of inhaled nitric oxide (iNO) were compared.

Results

The pre-transplant diagnoses, and cardiopulmonary bypass and donor ischemic times were similar between the high and low PVRI groups. High PVRI patients were older at transplant (12 ± 6.2 vs 8 ± 7.1 years, p = 0.002). The post-transplant inotrope score was higher in the high PVRI group (12 ± 12 vs 2 ± 2, p = 0.0001) and 1-year survival was worse (76% vs 81%, p = 0.03). The PVRI fell to < 6 U/m² with acute vasodilator testing in 21 of 49 (42%) high PVRI patients. RV failure occurred in 4 (19%) of the responders and in 14 (50%) of the non-responders (p = 0.037). One responder (5%) and 4 non-responders (14%) died of RV failure. In the period after 1995, the year iNO became clinically available, the select group of high PVRI patients who received iNO preemptively had a lower incidence of post-transplant RV failure than the group that did not receive preemptive iNO (13% vs 54%, p = 0.04).

Conclusions

Pre-transplant vasodilator testing identified patients at higher risk for RV failure. Patients who did not respond to vasodilator testing had an increased incidence of RV failure and death from RV failure. Preemptive use of iNO was associated with a decreased incidence of RV failure.

Section snippets

Methods

This was a retrospective record review of all patients who had heart transplantation at the Program for Pediatric Cardiomyopathy, Heart Failure and Transplantation of the Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center, between June 1984 and January 2005. The study was approved by the Columbia University Institutional Review Board.

Pre-transplant data obtained included diagnoses, gender, age at transplantation, hemodynamic variables, and the amount

Statistical methods

Post-transplant outcomes, including incidence of RV failure, need for mechanical circulatory support (VAD or ECMO), and death from RV failure were compared between patients who had a high PVRI (≥ 6 IU) and those who had a low PVRI (< 6 IU). Additional analyses in the high PVRI group were performed to assess the influence of elevated pre-transplant PVRI, era of transplantation and post-transplant use of iNO on patient outcomes. Patients with elevated baseline PVRI were divided into 3 sub-groups

Pre-transplant characteristics

Between 1984 and 2005, 263 children and young adults received heart transplants, of which 58 (22%) had a baseline PVRI ≥ 6 IU. Table 1 compares the pre-transplant characteristics of the high and low PVRI groups. The high PVRI group was significantly older at the time of transplant and they had higher pre-transplant inotrope scores. The pre-transplant diagnoses of the high PVRI patients were congenital heart disease in 19 (33%), dilated cardiomyopathy in 26 (45%), hypertrophic cardiomyopathy in

Discussion

In this study we report an aggressive approach to the diagnosis and treatment of pulmonary hypertension in patients undergoing heart transplantation. A high proportion of patients (22%) who underwent transplant during the study period had baseline pulmonary hypertension. Survival was 71%, somewhat lower than would be expected but comparable to survival after transplant in patients with congenital heart disease.¹² Significant controversy exists regarding heart transplantation in patients with

Disclosure statement

None of the authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript or other conflicts of interest to disclose.

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Original clinical scienceHeart transplantation in children with markedly elevated pulmonary vascular resistance: Impact of right ventricular failure on outcome

Background

Methods

Results

Conclusions

Section snippets

Methods

Statistical methods

Pre-transplant characteristics

Discussion

Disclosure statement

Ann Thorac Surg

J Heart Lung Transplant

Transplant Proc

J Heart Lung Transplant

J Heart Lung Transplant

J Heart Lung Transplant

J Heart Lung Transplant

Ann Thorac Surg

J Heart Lung Transplant

Lancet

J Cardiothorac Vasc Anesth

Original clinical science
Heart transplantation in children with markedly elevated pulmonary vascular resistance: Impact of right ventricular failure on outcome