Heart Transplantation in Systemic Diseases: How Far Can We Go?

Abstract

Purpose Few data are already available on the follow up of heart transplant (HTx) patients for systemic rare diseases. Aim of the study was to assess the cardiovascular, non-cardiovascular outcome and quality of life (QoL) of heart transplant patients with systemic diseases. Methods We have reviewed all the cases of HTx in rare diseases performed in our Cardiothoracic Department from 1985 to Feb 2021. QoL was assessed by SF-36 questionnaire. Results Since 1985, out of the 680 HTx performed at our Centre, 25 patients had a systemic rare disease (cardiac amyloidosis, Danon, Becker distrophy, see Fig 1) as cause of advanced heart failure (HF) needing of HTx. Mean age was 45±15 years old, mostly males (76%); 3 patients were in urgent list. The peripheral muscular involvement was present in 11 cases; however, almost all patients retained the capacity of walking. Donors mean age was 37±11 and 16 (64%) were male. Eleven (44%) were considered non-standard donors (ischemic time >4 hours in 5 (20%), cardiac arrest or LVEF<50% or coronary artery disease in 3 (12%), previous drug abuse in 2 (8%). Considering the cardiovascular outcome, the survival was 100% at 30-days, 96% and 90% at 1 and 5 years after HTx in a median follow up of 72 (range 7-318) months. Rejection >2 grade was in 6 (24%), while cardiac allograft vasculopathy in 4 (16%). Related the non-cardiovascular outcome, there was ocular and muscular worsening in 6 (24%) and 9 (36%) cases, while 3 (12%) developed severe psychosis. Lastly, the SF-36 questionnaire showed lower scores for the items related to Physical Component Summary, while similar scores compared to the general population for the Mental Component Summary. Conclusion In a median follow up of 5 years, HTx patients with systemic rare disease showed a satisfied general survival. The ocular and muscular worsening related to the systemic disease could be very frequent, conditioning the QoL. The relapse of the systemic disease is a rare event, although possible. Few data are already available on the follow up of heart transplant (HTx) patients for systemic rare diseases. Aim of the study was to assess the cardiovascular, non-cardiovascular outcome and quality of life (QoL) of heart transplant patients with systemic diseases. We have reviewed all the cases of HTx in rare diseases performed in our Cardiothoracic Department from 1985 to Feb 2021. QoL was assessed by SF-36 questionnaire. Since 1985, out of the 680 HTx performed at our Centre, 25 patients had a systemic rare disease (cardiac amyloidosis, Danon, Becker distrophy, see Fig 1) as cause of advanced heart failure (HF) needing of HTx. Mean age was 45±15 years old, mostly males (76%); 3 patients were in urgent list. The peripheral muscular involvement was present in 11 cases; however, almost all patients retained the capacity of walking. Donors mean age was 37±11 and 16 (64%) were male. Eleven (44%) were considered non-standard donors (ischemic time >4 hours in 5 (20%), cardiac arrest or LVEF<50% or coronary artery disease in 3 (12%), previous drug abuse in 2 (8%). Considering the cardiovascular outcome, the survival was 100% at 30-days, 96% and 90% at 1 and 5 years after HTx in a median follow up of 72 (range 7-318) months. Rejection >2 grade was in 6 (24%), while cardiac allograft vasculopathy in 4 (16%). Related the non-cardiovascular outcome, there was ocular and muscular worsening in 6 (24%) and 9 (36%) cases, while 3 (12%) developed severe psychosis. Lastly, the SF-36 questionnaire showed lower scores for the items related to Physical Component Summary, while similar scores compared to the general population for the Mental Component Summary. In a median follow up of 5 years, HTx patients with systemic rare disease showed a satisfied general survival. The ocular and muscular worsening related to the systemic disease could be very frequent, conditioning the QoL. The relapse of the systemic disease is a rare event, although possible.