Purpose: We aimed to define the characteristics and outcomes of ARVC patients who underwent OHT and combined heart-liver transplantation (OHT-OLT) at a U.S. tertiary academic medical center.
Methods: We performed a retrospective analysis of 14 patients in the Penn ARVC Transplant Database. Thirteen patients underwent OHT or OHT-OLT between May 2011 and August 2017. Demographics, pre-transplant characteristics, and survival data were collected. Normality was not assumed in the continuous data fields given the small sample size. Descriptive statistics were computed as medians and interquartile ranges (IQRs). The Wilcoxon rank-sum test and Fisher’s exact test were used for inferential statistical testing of continuous and categorical variables, respectively.
Results: Features of the study population are presented in Table 1. Nine of 13 patients underwent OHT and 4 underwent OHT-OLT. One patient died awaiting transplant. The median ages at diagnosis and listing for transplant were similar. Although not statistically significant, there were trends toward longer diagnosis to listing (127.3 months vs. 48.5 months) and diagnosis to transplant times (130.9 months vs. 51.4 months) in the OHT compared to OHT-OLT group. The post-transplant survival was 100% for both OHT and OHT-OLT with median post-transplant follow-up times of 4.1 years and 2.1 years, respectively.
Table 1: Characteristics and Outcomes by Transplant Type
| Variables | Heart | Heart-Liver | p-value |
| N | 9 | 4 | |
| Age at Diagnosis (years), median (IQR) | 50.0 (21.0, 55.5) | 49.5 (34.5, 55.0) | 0.88 |
| Age at Listing (years), median (IQR) | 59.0 (55.0, 61.0) | 55.9 (38.0, 59.5) | 0.44 |
| Sex | 1.00 | ||
| Female | 3 (33%) | 1 ( 25%) | |
| Male | 6 (67%) | 3 ( 75%) | |
| BMI ( kg/m2), median (IQR) | 23.4 (20.6, 28.8) | 27.5 (26.9, 27.7) | 0.26 |
| Creatinine (mg/dL), median (IQR) | 1.1 (1.0, 1.3) | 1.2 (1.2, 2.0) | 0.31 |
| MELD, median (IQR) | 13.0 (12.0, 15.0) | 18.5 (13.5, 19.0) | 0.33 |
| SVR (dyn-s/cm2), median (IQR | 2014.5 (1222,5, 2864.0) | 1264.0 (1255.0, 1760) | 0.31 |
| Transplant Status | 0.41 | ||
| 1A | 0 (0%) | 1 ( 25%) | |
| 1B | 3 (33%) | 1 (25%) | |
| 1B by exception | 5 (56%) | 1 (25%) | |
| 2 | 1 (11%) | 1(25%) | |
| Diagnosis to Listing (months), median (IQR) | 127.3 (49.1, 153.5) | 48.5 (27.7, 77.8) | 0.12 |
| Diagnosis to Transplant (months), median (IQR) | 130.9 (56.6, 154,8) | 51.4 (40.6, 58.3) | 0.17 |
| Time on Wait List (days), median (IQR) | 110.0 (59.0, 154.0) | 74.5 (27.5, 588.5) | 0.76 |
| Survival? | 0.31 | ||
| No | 0 (0%) | 1 ( 25%) | |
| Yes | 9 (100%) | 3 (75%) | |
| Post Transplant Follow-up (years), median (IQR) | 4.1, (1.9,5.0) | 2.1 (2.1, 5.4) | 0.78 |