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The variable natural history of idiopathic dilated cardiomyopathy
Kapil Parakh1, Michelle M Kittleson, Bettina Heidecker
1Department of Medicine, Johns Hopkins Bayview Medical Center, Baltimore, MD, USA.
Insights
Predicting long-term outcomes for idiopathic dilated cardiomyopathy (IDCM) patients is challenging. Standard clinical factors like ejection fraction and NYHA class have limited accuracy in classifying prognosis, suggesting other unappreciated factors influence IDCM progression.
Area of Science:
- Cardiology
- Internal Medicine
- Biomedical Research
Background:
- Accurate prognosis is crucial for heart failure patient management and clinical trials.
- Traditional prognostic criteria for heart failure have limitations in assessing individual long-term outcomes.
- Idiopathic dilated cardiomyopathy (IDCM) presents a variable natural history, complicating prognosis prediction.
Purpose of the Study:
- To evaluate the predictive power of clinical, hemodynamic, and echocardiographic parameters for long-term prognosis in IDCM patients.
- To assess the ability of established risk factors and medications to predict outcomes in IDCM.
- To determine the accuracy of ejection fraction and NYHA class in classifying IDCM prognosis.
Main Methods:
- A large, contemporary cohort of IDCM patients (n=171) referred to Johns Hopkins (1997-2004) was analyzed.
- Baseline clinical data, physical examinations, laboratory studies, echocardiograms, right heart catheterization, and endomyocardial biopsies were collected.
- Long-term prognosis was assessed by tracking survival-free of major adverse events (death, VAD, or transplant).
Main Results:
- Established risk factors and common heart failure medications were similar between long-term survivors (LTS) and non-long-term survivors (NLTS).
- While LTS were younger with higher ejection fraction (EF) and lower NYHA class, EF < 25% had 64% predictive value and NYHA class > 2 had 53% predictive value.
- A logistic model using these three variables misclassified 29% of patients, indicating limited predictive accuracy.
Conclusions:
- Idiopathic dilated cardiomyopathy (IDCM) demonstrates a highly variable natural history.
- Standard clinical predictors possess limited ability to categorize IDCM patients into distinct prognostic groups.
- Unappreciated host-environmental factors likely play a significant role in the biology and progression of IDCM.
Background:
Determining the prognosis of patients with heart failure is essential for patient management and clinical trial conduct. The relative value of traditional prognostic criteria remains unclear and the assessment of long-term prognosis for individual patients is problematic.
Objectives:
To determine the ability of clinical, hemodynamic and echocardiographic parameters to predict the long-term prognosis of patients with idiopathic dilated cardiomyopathy.
Methods:
We investigated the ability of clinical, hemodynamic and echocardiographic parameters to predict the long-term prognosis of individual patients in a large, representative, contemporary cohort of idiopathic dilated cardiomyopathy (IDCM) patients referred to Johns Hopkins from 1997 to 2004 for evaluation of cardiomyopathy. In all patients a baseline history was taken, and physical examination, laboratory studies, echocardiogram, right heart catheterization and endomyocardial biopsy were performed.
Results:
In 171 IDCM patients followed for a median 3.5 years, there were 50 long-term event-free survivors (LTS) (median survival 6.4 years) and 34 patients died or underwent ventricular assist device placement or transplantation within 5 years (NLTS; non-long-term survivors) (median time to event 1.83 years. Established risk factors (gender, race, presence of diabetes, serum creatinine, sodium) and the use of accepted heart failure medications (angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, beta blockers) were similar between the two groups. Although LTS had younger age, higher ejection fraction (EF) and lower New York Heart Association (NYHA) class at presentation, the positive predictive value of an EF < 25% was 64% (95% CI 41%-79%) and that of NYHA class > 2 was 53% (95% CI 36-69%). A logistic model incorporating these three variables incorrectly classified 29% of patients.
Conclusions:
IDCM exhibits a highly variable natural history and standard clinical predictors have limited ability to classify IDCM patients into broad prognostic categories. These findings suggest that there are important host-environmental factors still unappreciated in the biology of IDCM.
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