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A rare indication for lung transplantation – pulmonary alveolar microlithiasis: institutional experience of five consecutive cases
Author(s) -
Klikovits Thomas,
Slama Alexis,
Hoetzenecker Konrad,
Waseda Ryuichi,
Lambers Christopher,
Murakoezy Gabriella,
Jaksch Peter,
Aigner Clemens,
Taghavi Shahrokh,
Klepetko Walter,
Lang Gyoergy,
Hoda Mir Alireza
Publication year - 2016
Publication title -
clinical transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 76
eISSN - 1399-0012
pISSN - 0902-0063
DOI - 10.1111/ctr.12705
Subject(s) - medicine , lung transplantation , surgery , extracorporeal membrane oxygenation , transplantation , perioperative , lung , intensive care unit , retrospective cohort study , bronchiolitis obliterans
Background Pulmonary alveolar microlithiasis ( PAM ) is a rare lung disease caused by calcifications within the alveolar space. The only known effective treatment for an end‐stage PAM is lung transplantation (Lu TX ). Methods We performed a retrospective chart review of all individuals that underwent lung transplantation at our center between 1989 and 2013. Five consecutive patients with PAM were identified. Results Four females and one male with a mean age of 46.3 yr were identified. Extracorporeal membrane oxygenation ( ECMO ) support was required intraoperatively in four cases and post‐operatively in one case. Mean post‐operative intubation time was 3.3 (range, 2–5) d and mean intensive care unit ( ICU ) stay was 8.3 (range, 4–12) d. No intraoperative complications were observed. One early patient (operated in 1995) underwent acute re‐transplantation on the second post‐operative day ( POD ) and died from sepsis on the 11 POD . In one patient reperfusion edema was observed requiring a prolonged weaning process. No other severe perioperative complications were observed. Four of five patients are currently still alive with normal follow‐up parameters. No recurrence of PAM was observed. Conclusions Lung transplantation is a feasible therapy option in patients with end‐stage PAM showing good post‐operative results comparable to other indications for Lu TX .