Combined lung and liver transplantation for noncirrhotic portal hypertension with severe hepatopulmonary syndrome in a patient with dyskeratosis congenita

Abstract

DC is caused by defects at the level of telomere maintenance, and cells from patients with this disease have abnormally short telomeres and show premature senescence. One consequence of DC is bone marrow failure. Thus, patients with DC often require HSCT. However, HSCT does not ameliorate other DC‐related manifestations. In fact, HSCT can accelerate organ dysfunction due to treatment‐related complications, and solid organ transplantation is required in some patients with DC. In this report, we describe the clinical course of a 5‐year‐old boy who was transferred to our hospital because of progressive dyspnea, 2 years after HSCT. At admission, he had tachypnea and hypoxemia. A liver biopsy was performed for suspected HPS caused by PH, and LT was considered. Eventually, his hypoxemia worsened, and he was transferred to a PICU and started on VA ECMO. He subsequently underwent a CLLT. ECMO was stopped on post‐operative day 12, extubation was achieved on post‐operative day 29, and the patient recovered well from the surgery. Our results show that CLLT could be a life‐saving treatment option for DC patients with very severe HPS in whom a poor outcome is expected after LT.

Extracorporeal membrane oxygenation as rescue therapy in a pediatric liver transplant recipient with very severe hepatopulmonary syndrome

“…Only a few previous cases of ECMO for HPS‐associated refractory hypoxemia post‐LT in pediatric recipients have been previously reported, with ECMO duration ranging from 17–28 days 4,7,16 . Herein, we describe a pediatric liver transplant recipient with very severe HPS that experienced a delayed decompensation post‐LT requiring ECMO support for 91 days with successful decannulation after embolization of a persistent splenorenal shunt.…”

“…reported, with ECMO duration ranging from 17-28 days. 4,7,16 Herein, we describe a pediatric liver transplant recipient with very severe HPS that experienced a delayed decompensation post-LT requiring ECMO support for 91 days with successful decannulation after embolization of a persistent splenorenal shunt. Research on the role of persistent portosystemic shunts following LT in refractory HPS is limited.…”

Progression of liver disease and portal hypertension in dyskeratosis congenita and related telomere biology disorders

“…The literature on liver transplantation in patients with DC/TBD is currently still sparse with limited follow-up data. There are few case reports describing the success of liver transplantation in patients with DC and cirrhosis, HPS, and numerous comorbid conditions 30–33 . However, they only reported the short-term outcomes of liver transplant with no follow-up after.…”

“…There are few case reports describing the success of liver transplantation in patients with DC and cirrhosis, HPS, and numerous comorbid conditions. [30][31][32][33] However, they only reported the short-term outcomes of liver transplant with no followup after. In a population of patients with liver cirrhosis awaiting liver transplantation, P/LP TBD gene variants were more prevalent than in the general population and were found to be associated with lower baseline platelet and WBC counts and a longer post-transplant length of stay.…”

Outcomes following liver transplant in adults with telomere biology disorders

“…(3) There have been three previous reports describing liver transplantation in TBD, only one of which took place in an adult. (4)(5)(6) We present four additional adult cases of liver transplantation for hepatic complications of telomere biology disorders. (Table 1)…”