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December 30, 2010Circulation Heart FailureOpen Access

Pressure-Volume Relationships in Patients With Transthyretin (ATTR) Cardiac Amyloidosis Secondary to V122I Mutations and Wild-Type Transthyretin

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Population

29 subjects with ATTR cardiac amyloidosis (18 with wild-type ATTRwt and 11 with mutated ATTRm V122I)

Comparison

Echocardiography-derived pressure-volume indices… vs ATTRwt vs ATTRm and survivors vs nonsurvivors

Design

Cohort

Follow-up

mean 478 days (range, 31 to 807)

Authors

TBTaslima BhuiyanBoston UniversitySHStephen HelmkeBoston Medical CenterAPAyan R. PatelTufts University

Discussion

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Implication

May identify higher-risk ATTR amyloidosis patients; leaves open whether EF-guided interventions improve outcomes.

Structured PICO

P
Population
29 subjects with ATTR cardiac amyloidosis (18 with wild-type ATTRwt and 11 with mutated ATTRm V122I)
I
Intervention
Echocardiography-derived pressure-volume (PV) indices assessment at baseline and every 6 months for up to 2 years
C
Comparator
ATTRwt vs ATTRm (V122I) and survivors vs nonsurvivors
O
Outcome
Pressure-volume indices (end-systolic and end-diastolic PV relations, PVA(iso)) and mortality or cardiac transplantsurrogate

In ATTR cardiac amyloidosis, pressure-volume analysis reveals alterations in ventricular work that correlate with reduced survival, specifically an initial ejection fraction <50% predicting increased mortality.

Cite This Study

Bhuiyan et al. (2010) studied this question.

synapsesocial.com/papers/6a011b3b2ff633f365783aafhttps://doi.org/10.1161/circheartfailure.109.910455

Topics

CardiomyopathyCardiac amyloidosis
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Effective arterial elastance as index of arterial vascular load in humans.1992 · 838 citations
  2. 2Left ventricular interaction with arterial load studied in isolated canine ventricle1983 · 818 citations
  3. 3The Tetrameric Protein Transthyretin Dissociates to a Non-native Monomer in Solution1999 · 188 citations
  4. 4Echocardiographic findings in systemic amyloidosis: Spectrum of cardiac involvement and relation to survival1985 · 273 citations
  5. 5Systemic Cardiac Amyloidoses2009 · 778 citations