Hybrid coronary revascularization vs. percutaneous coronary interventions for multivessel coronary artery disease

OBJECTIVE Hybrid coronary revascularization(HCR)combines a minimally invasive surgical approach to the left anterior descending(LAD)artery with percutaneous coronary intervention(PCI)for non-LAD diseased coronary arteries.It is associated with shorter hospital lengths of stay and recovery times than conventional coronary artery bypass surgery,but there is little information comparing it to isolated PCI for multivessel disease.Our objective is to compare long-term outcomes of HCR and PCI for patients with multivessel disease.METHODS This cohort study used data from New York’s cardiac surgery and PCI registries in 2010−2016 to examine mortality and repeat revascularization rates for patients with multivessel coronary artery disease who underwent HCR and PCI.Cox proportional hazards methods were used to reduce selection bias.Patients were followed for a median of four years.RESULTS There was a total of 335 HCR patients(1.2%)and 25,557 PCI patients(98.8%)after exclusions.There was no difference in 6-year risk adjusted survival between HCR and PCI patients 83.17%vs.81.65%,adjusted hazard ratio(aHR)=0.90(95%CI:0.67−1.20).However,HCR patients were more likely to be free from repeat revascularization in the LAD artery(91.13%vs.83.59%,aHR=0.51(95%CI:0.34−0.77)).CONCLUSIONS For patients with multi-vessel coronary artery disease,HCR is rarely performed.There are no differences in mortality rates after four years,but HCR is associated with lower repeat revascularization rates in the LAD artery,presumably due to better longevity in left arterial mammary grafts.

Progressive familial intrahepatic cholestasis—farnesoid X receptor deficiency due to NR1H4 mutation:A case report

BACKGROUND Functioning farnesoid X receptor(FXR;encoded by NR1H4)is key to normal bile acid homeostasis.Biallelic mutations in NR1H4 are reported in a few children with intrahepatic cholestasis.We describe a boy with progressive familial intrahepatic cholestasis and homozygous mutation in NR1H4.CASE SUMMARY A boy had severe neonatal cholestasis with moderate hypercholanemia and persistently elevated alpha-fetoprotein.Despite medical treatment,coagulopathy was uncontrollable,prompting liver transplantation at age 8 mo with incidental splenectomy.The patient experienced catch-up growth with good liver function and did not develop allograft steatosis.However,1 year after transplant,he died from an acute infection,considered secondary to immunosuppression and asplenia.A homozygous protein-truncating mutation,c.547C>T,p.(Arg183Ter),was subsequently identified in NR1H4,and both parents were shown to be heterozygous carriers.Absence of FXR and of bile salt export pump expression was confirmed by immunostaining of explanted liver.CONCLUSION Severe cholestasis with persistently high alpha-fetoprotein and modest elevation of serum bile acid levels may suggest FXR deficiency.Some patients with FXR deficiency may not develop allograft steatosis and may respond well to liver transplantation.