Perioperative mortality is the Achilles heel for cardiac transplantation in adults with congenital heart disease: Evidence from analysis of the UNOS registry

Abstract

Perioperative morbidity and mortality adversely affects short-term survival in ACHDR. ACHDR who survive the first post-transplant year have equivalent or better long-term survival than NCR.

Pulmonary hypertension: Barrier or just a bump in the road in transplanting adults with congenital heart disease

“…Graft failure secondary to elevated PVR, allosensitization, longer donor organ ischemic times, and post-operative bleeding are the most likely cause of death in ACHD transplants and is the driver for the mortality difference between ACHD and noncongenital transplants. 6 As described, our experience has been similar to what has been described in the literature, and for that reason we closely monitored and prepared for the possibility of right HF in the l-TGA patient. As such, we moved slowly in regards to extubation (postoperative day #2) to prevent hypoxia-induced vasoconstriction, continued invasive hemodynamic monitoring, and used inhaled nitric oxide with transition to low dose sildenafil (20 mg three times daily).…”

“…In addition, to a successful surgical operation, outcomes remain dependent on precise perioperative management. Graft failure secondary to elevated PVR, allosensitization, longer donor organ ischemic times, and post‐operative bleeding are the most likely cause of death in ACHD transplants and is the driver for the mortality difference between ACHD and noncongenital transplants …”

“…While several parameters including declining NYHA class, progressive ventricular dysfunction, elevated serum BNP reduced peak oxygen uptake (VO2 max), and low BMI have each been associated with increased mortality, the specific thresholds to drive decisions regarding timing of advanced therapies referral are lacking. Despite this, more recently, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes …”

“…Despite this, more recently, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes. [6][7][8] Pulmonary hypertension (PH) is a common barrier to HT, especially in the ACHD population as the approach to PH and HT becomes more complicated in comparison to the overall population, given the anatomic heterogeneity and lower prevalence and experience. Currently, there are only four placebo-controlled trials for ACHD patients with PH.…”

Mechanical Circulatory Support (MCS) for Primary Graft Dysfunction (PGD)

“…PGD was significantly higher in ACHDR compared to NCR (4.3% vs. 2.6%, p < 0.01); however, there was no difference in acute and chronic rejection rates [49]. PGD continues to be a significant cause of morbidity in the most recent era [54].…”