PMID- 29693942
OWN - NLM
STAT- MEDLINE
DCOM- 20180816
LR  - 20221207
IS  - 0021-4892 (Print)
IS  - 0021-4892 (Linking)
VI  - 66
IP  - 5
DP  - 2017 May
TI  - Intraoperative Aortic Dissection during Lung Transplantation in a Patient with 
      Alpha-1 Antitrypsin Deficiency.
PG  - 530-534
AB  - Alpha-1 antitrypsin deficiency (AATD) is an inherited disorder affecting the 
      lung, liver, and rarely skin. The most frequent features of AATD consist of 
      chronic lung disorders related to protease-antiprotease imbalance in the 
      respiratory system, to which lung transplantation is frequently indicated. We 
      describe a case of aortic dissection in a 55-year-old male who underwent left 
      single lung transplantation for respiratory failure due to AATD-related 
      emphysema. Extracorporeal membrane oxygenation (ECMO) was indicated during the 
      procedure, and an arterial cannula was placed into the descending aorta and a 
      venous cannula was placed into the right femoral vein. Bronchial and vascular 
      anastomoses were finished without any problems and ECMO was weaned off However, 
      transesophageal echocardiography carried out at the end of the operation showed a 
      dissected descending aorta. Alpha-1 antitrypsin (AAT) is the major serum 
      inhibitor of seine proteinases, which enzymatically destroys collagen and 
      elastin. Degeneration of connective tissues, in particular elastic tissues, is 
      established in AATD, and decreased stiffness of the aorta due to degradation of 
      elastic fibers has also been reported in AATD. In this patient, reduced AAT 
      activity might have boosted the enzymatic destruction of his arterial walls, 
      leading to enhanced vulnerability to aortic dissections.
FAU - Tatsumi, Kenichiro
AU  - Tatsumi K
FAU - Hamai, Yusuke
AU  - Hamai Y
FAU - Mizota, Toshiyuki
AU  - Mizota T
FAU - Fukuda, Kazuhiko
AU  - Fukuda K
LA  - eng
LA  - jpn
PT  - Case Reports
PT  - Journal Article
PL  - Japan
TA  - Masui
JT  - Masui. The Japanese journal of anesthesiology
JID - 0413707
RN  - alpha-1-Antitrypsin Deficiency, Autosomal Recessive
SB  - IM
MH  - Aortic Dissection
MH  - Aorta/*surgery
MH  - Humans
MH  - Intraoperative Complications
MH  - *Lung Transplantation
MH  - Male
MH  - Middle Aged
MH  - Pulmonary Emphysema/etiology/*surgery
MH  - alpha 1-Antitrypsin Deficiency/*complications
EDAT- 2018/04/26 06:00
MHDA- 2018/08/17 06:00
CRDT- 2018/04/26 06:00
PHST- 2018/04/26 06:00 [entrez]
PHST- 2018/04/26 06:00 [pubmed]
PHST- 2018/08/17 06:00 [medline]
PST - ppublish
SO  - Masui. 2017 May;66(5):530-534.