*Purpose: Severe pulmonary arterial hypertension (PAH) as defined by a pulmonary artery systolic pressure (PASP) >50mmHg is associated with high peri-operative mortality and thus is usually considered a contraindication to kidney transplantation. In this preliminary study, we describe the pre-kidney transplant management and post-transplant outcomes in patients with severe PAH using a multidisciplinary care team approach.

*Methods: Between November 2013 and August 2020 we identified all patients with severe PAH on initial pre-transplant workup who underwent ultrafiltration (UF) and/or medical therapy for PAH prior to kidney transplant. After transplant we evaluated their peri-operative course, renal function, graft and patient survival.

*Results: Fourteen patients (mean age=56±12years, AA race: 8/14; 57%) were diagnosed with severe PAH on pre transplant screening echocardiogram. These findings were confirmed by right heart catheterization (RHC) (mean PASP 63.4±15mmHg). These patients were also noted to have an elevated pulmonary capillary wedge pressure (mean PCWP 23.4±6.4mmHg) and right atrial pressure (mean RA 12.1±3.6mmHg). Based upon a diagnosis of mixed PAH with a post-capillary component, patients were subjected to aggressive mechanical ultrafiltration (UF; mean 4.6±1.6 sessions) with an average weight loss of 5.5±2.1kg at the end of the UF sessions. Twelve (out of 14; 86%) patients underwent repeat RHC and were noted to have a marked decline in PASP from an average of 65±16 to 33±12 mmHg, in PCWP from 23.5±7 to 7.4±3.4 mmHg and in RA pressure from 12.1±3.6 to 3.17±2.0 mmHg. Four (29%) patients were on vasodilator (phosphodiesterase type 5; PDE5 inhibitor therapy) prior to UF and remained on those after UF and through transplant. Majority of these patients received a deceased donor kidney transplant (12/14; 86%). Two (14%) of the patients had a planned one-day ICU stay after surgery, six (42%) had a 30-day readmission and nine (64%) of the patients had delayed graft function. The mean eGFR at 3, 6, 9 and 12 months was 70.69±27, 70.23±27, 74.92±30 and 73.09±32 ml/min/1.73m². At a mean follow-up of 28±21 months post-transplant both graft and patient survival are 100%.

*Conclusions: In this small single center study, we report that severe PAH should not be considered an absolute contra-indication to kidney transplantation. A better elucidation of the etiology of PAH with a RHC should be considered. Post-capillary PAH can be a significant and common contributor to elevations in PASP and mean PAP especially in the dialysis population. Using a multidisciplinary approach, PAH could be improved with optimal volume removal and PDE5 inhibitor therapy leading to a complication free peri-operative period and a successful post-transplant outcome.

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