PMID- 25398293
OWN - NLM
STAT- MEDLINE
DCOM- 20150730
LR  - 20181113
IS  - 1613-7671 (Electronic)
IS  - 0043-5325 (Linking)
VI  - 126
IP  - 21-22
DP  - 2014 Nov
TI  - Fontan-like circulation as a criterion for heart transplantation in 
      arrhythmogenic right ventricular dysplasia.
PG  - 705-9
LID - 10.1007/s00508-014-0656-8 [doi]
AB  - BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is often associated 
      with progressive right ventricular dysfunction. Although heart transplantation 
      (HTx) is suggested in these patients, indication and optimal timing for listing 
      can be challenging. METHODS AND RESULTS: The study comprises four patients (two 
      male, range: 37-56 years) with advanced ARVD who were considered for HTx. 
      Standard inclusion criteria for HTx listing such as clinical signs, New York 
      Heart Association (NYHA) classification (II-III), and N-terminal pro-brain 
      natriuretic peptide (NT-proBNP) (3672 +/- 1407 pg/ml) were heterogeneous and did 
      not add unequivocally to decision making. In all patients, though, right heart 
      hemodynamics revealed Fontan-like circulation (FLC) with equilibrated pressure 
      tracings between the right atrium (16 +/- 4 mmHg) and the pulmonary artery (16 +/- 5 
      mmHg). In this condition, the pulmonary blood flow can be regarded as nearly 
      non-pulsatile, as it is passive and propelled by the transpulmonary gradient and 
      intrathoracic pressure alterations produced by breathing to the left atrium. 
      Based on these findings, all patients were listed for HTx and were finally 
      successfully transplanted. CONCLUSIONS: In patients with ARVD, evidence of FLC 
      may serve as an additional criterion for HTx. This applies particularly to 
      patients who do not clearly fulfill standard transplant criteria and to patients 
      with electrical instability.
FAU - Schernthaner, Christiana
AU  - Schernthaner C
AD  - Department of Cardiology, Salzburger Landeskliniken, Paracelsus Private Medical 
      University, Muellner Hauptstrasse 48, 5020, Salzburg, Austria, 
      c.schernthaner@salk.at.
FAU - Poelzl, Gerhard
AU  - Poelzl G
FAU - Strohmer, Bernhard
AU  - Strohmer B
FAU - Steinacher, Richard
AU  - Steinacher R
FAU - Granitz, Marcel
AU  - Granitz M
FAU - Altenberger, Johann
AU  - Altenberger J
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20141115
PL  - Austria
TA  - Wien Klin Wochenschr
JT  - Wiener klinische Wochenschrift
JID - 21620870R
SB  - IM
MH  - Adult
MH  - Arrhythmogenic Right Ventricular Dysplasia/*complications/diagnosis/*surgery
MH  - Diagnosis, Differential
MH  - Heart Transplantation/*methods
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Patient Selection
MH  - Treatment Outcome
MH  - Ventricular Dysfunction, Right/diagnosis/*etiology/*surgery
EDAT- 2014/11/16 06:00
MHDA- 2015/08/01 06:00
CRDT- 2014/11/16 06:00
PHST- 2014/08/30 00:00 [received]
PHST- 2014/10/19 00:00 [accepted]
PHST- 2014/11/16 06:00 [entrez]
PHST- 2014/11/16 06:00 [pubmed]
PHST- 2015/08/01 06:00 [medline]
AID - 10.1007/s00508-014-0656-8 [doi]
PST - ppublish
SO  - Wien Klin Wochenschr. 2014 Nov;126(21-22):705-9. doi: 10.1007/s00508-014-0656-8. 
      Epub 2014 Nov 15.