PMID- 31262606
OWN - NLM
STAT- MEDLINE
DCOM- 20200625
LR  - 20200625
IS  - 1874-1754 (Electronic)
IS  - 0167-5273 (Linking)
VI  - 292
DP  - 2019 Oct 1
TI  - Surgical septal myectomy for relief of dynamic obstruction in Anderson-Fabry 
      Disease.
PG  - 91-94
LID - S0167-5273(19)32010-8 [pii]
LID - 10.1016/j.ijcard.2019.06.042 [doi]
AB  - Patients with Anderson-Fabry Disease (AFD) and severe left ventricular 
      hypertrophy complicated by left ventricular outflow tract (LVOT) obstruction may 
      benefit from surgical septal myectomy (SSM). Mid- and late outcomes following 
      surgery have not been established, and we sought to better characterize 
      postoperative outcomes following septal myectomy. Between January 2011 and June 
      2017, 7 patients (6 females) with AFD underwent SSM. The median (range) age at 
      the time of surgery was 53 (37-72) years; 4 patients had a positive family 
      history of AFD and a preoperative diagnosis of AFD. Extracardiac features 
      suggestive of AFD were present in 3 patients and all but 1 (female) had reduced 
      alpha-galactosidase A activity. All patients had severe left ventricular hypertrophy 
      and LVOT obstruction on transthoracic echocardiography. Preoperatively, all 
      patients were severely symptomatic with New York Heart Association (NYHA) class 
      III symptoms. There was no early mortality following surgery. The median 
      in-hospital length of stay was 5 (4-7) days with 6 patients reporting NYHA class 
      II or less symptoms at 3 month follow-up. Long-term outcomes were favorable with 
      4 patients reporting sustained NYHA class II or less symptoms, but 2 patients had 
      recurrence of NYHA class III symptoms without evidence of recurrent LVOT 
      obstruction. In conclusion, SSM appears to provide favorable short- and long-term 
      relief of severe, symptomatic LVOT obstruction but may not alter progression of 
      Fabry cardiomyopathy.
CI  - Copyright (c) 2019 Elsevier B.V. All rights reserved.
FAU - Meghji, Zahara
AU  - Meghji Z
AD  - Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, United States 
      of America.
FAU - Nguyen, Anita
AU  - Nguyen A
AD  - Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, United States 
      of America.
FAU - Miranda, William R
AU  - Miranda WR
AD  - Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, United States 
      of America.
FAU - Geske, Jeffrey B
AU  - Geske JB
AD  - Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, United States 
      of America.
FAU - Schaff, Hartzell V
AU  - Schaff HV
AD  - Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, United States 
      of America.
FAU - Peck, Dawn S
AU  - Peck DS
AD  - Department of Biomedical Genetics, Mayo Clinic, Rochester, MN, United States of 
      America.
FAU - Newman, Darrell B
AU  - Newman DB
AD  - Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, United States 
      of America. Electronic address: Newman.Darrell@mayo.edu.
LA  - eng
PT  - Journal Article
DEP - 20190619
PL  - Netherlands
TA  - Int J Cardiol
JT  - International journal of cardiology
JID - 8200291
SB  - IM
MH  - Adult
MH  - Aged
MH  - Cardiac Surgical Procedures/methods
MH  - Cardiomyopathy, Hypertrophic/*etiology/*surgery
MH  - Fabry Disease/complications
MH  - Female
MH  - Heart Septum/*surgery
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Severity of Illness Index
MH  - Treatment Outcome
MH  - Ventricular Outflow Obstruction/etiology/*surgery
OTO - NOTNLM
OT  - Fabry
OT  - Hypertrophic cardiomyopathy
OT  - Myectomy
OT  - Obstruction
EDAT- 2019/07/03 06:00
MHDA- 2020/06/26 06:00
CRDT- 2019/07/03 06:00
PHST- 2019/04/16 00:00 [received]
PHST- 2019/05/23 00:00 [revised]
PHST- 2019/06/18 00:00 [accepted]
PHST- 2019/07/03 06:00 [pubmed]
PHST- 2020/06/26 06:00 [medline]
PHST- 2019/07/03 06:00 [entrez]
AID - S0167-5273(19)32010-8 [pii]
AID - 10.1016/j.ijcard.2019.06.042 [doi]
PST - ppublish
SO  - Int J Cardiol. 2019 Oct 1;292:91-94. doi: 10.1016/j.ijcard.2019.06.042. Epub 2019 
      Jun 19.