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. 2017 Mar 2;6(3):e003498.
doi: 10.1161/JAHA.116.003498.

Rheumatic Heart Disease Severity, Progression and Outcomes: A Multi-State Model

Jeffrey Cannon  1 , Kathryn Roberts  2 , Catherine Milne  3 , Jonathan R Carapetis  4
Affiliations

Rheumatic Heart Disease Severity, Progression and Outcomes: A Multi-State Model

Jeffrey Cannon et al. J Am Heart Assoc. .

Abstract

Background: Rheumatic heart disease (RHD) remains a disease of international importance, yet little has been published about disease progression in a contemporary patient cohort. Multi-state models provide a well-established method of estimating rates of transition between disease states, and can be used to evaluate the cost-effectiveness of potential interventions. We aimed to create a multi-state model for RHD progression using serial clinical data from a cohort of Australian patients.

Methods and results: The Northern Territory RHD register was used to identify all Indigenous residents diagnosed with RHD between the ages of 5 and 24 years in the time period 1999-2012. Disease severity over time, surgeries, and deaths were evaluated for 591 patients. Of 96 (16.2%) patients with severe RHD at diagnosis, 50% had proceeded to valve surgery by 2 years, and 10% were dead within 6 years. Of those diagnosed with moderate RHD, there was a similar chance of disease regression or progression over time. Patients with mild RHD at diagnosis were the most stable, with 64% remaining mild after 10 years; however, 11.4% progressed to severe RHD and half of these required surgery.

Conclusions: The prognosis of young Indigenous Australians diagnosed with severe RHD is bleak; interventions must focus on earlier detection and treatment if the observed natural history is to be improved. This multi-state model can be used to predict the effect of different interventions on disease progression and the associated costs.

Keywords: pediatric; prognosis; rheumatic heart disease.

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Figures

Figure 1
Figure 1
Potential health state transitions of patients on the NT RHD register. NT indicates Northern Territory; RHD, rheumatic heart disease.
Figure 2
Figure 2
Selection of RHD cases included in analysis. NT indicates Northern Territory; RHD, rheumatic heart disease.
Figure 3
Figure 3
Number and severity at diagnosis of cases of RHD diagnosed between 1999 and 2012, by age and sex. RHD indicates rheumatic heart disease.
Figure 4
Figure 4
Age of RHD patient at time of first cardiac surgery. RHD indicates rheumatic heart disease.
Figure 5
Figure 5
Age of RHD patient at time of death. RHD indicates rheumatic heart disease.
Figure 6
Figure 6
RHD prognosis over 14 years; probability that an individual will be in a particular health state over time, based on RHD severity at diagnosis. RHD indicates rheumatic heart disease.
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