@Article{chd.12606,
AUTHOR = {Jonathan N. Menachem, Edo Y. Birati, Payman Zamani, Anjali T. Owens, Pavan Atluri, Christian A. Bermudez, David Drajpuch, Stephanie Fuller, Yuli Y. Kim, Christopher E. Mascio, Vikram Palanivel, J. Eduardo Rame, Joyce Wald, Michael A. Acker, Jeremy A. Mazurek},
TITLE = {Pulmonary hypertension: Barrier or just a bump in the road in transplanting adults with congenital heart disease},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {13},
YEAR = {2018},
NUMBER = {4},
PAGES = {492--498},
URL = {http://www.techscience.com/schd/v13n4/39024},
ISSN = {3071-1738},
ABSTRACT = {Background: Heart failure (HF) is the leading cause of death in adults with congenital heart disease
(ACHD). Identification of disease progression and timing of referral for advanced therapies is often
delayed. However, increased awareness and understanding of ACHD and improvements in the
approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common
barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience. However, in some cases, PH is a result of
elevated systemic filling pressures and low output.
Methods: We describe the approach used to successfully transplant an ACHD patient with severe
pre-HT PH performing HT alone. We review the literature and describe the one patient’s journey
from primarily palliative, to a combined heart-lung transplant candidate, to successful HT patient.
Results: We discuss the methodology used to successfully transplant a patient, with significantly
elevated pulmonary pressures and an initial pulmonary vascular resistance (PVR)> 13 Wood units.
Conclusions: There are a number of complexities associated with the ACHD population and it is
of utmost importance to carefully identify the underlying hemodynamic milieu and inform the
appropriate treatment course in order to have successful transplant outcomes.},
DOI = {10.1111/chd.12606}
}