Pediatric Restrictive Cardiomyopathy Prognosis for 53 Patients with Genetic Variants

53 pediatric patients with restrictive cardiomyopathy were studied to compare outcomes between sarcomeric and non-sarcomeric genetic variants. The retrospective study found survival without transplantation was significantly worse in the sarcomeric group, which also had a higher incidence of thrombotic events. The dataset, authored by Catherine Gardin and last updated in March 2026, is available under a CC-BY-4.0 license.

Use Cases

• Predicting survival outcomes without transplantation based on genetic variant type.
• Analyzing the association between sarcomeric variants and thrombotic event frequency.
• Comparing disease severity and hemodynamic parameters between genetic variant groups.
• Modeling prognosis for pediatric restrictive cardiomyopathy patients.

Strengths

• Focus on a specific, severe pediatric condition with 53 patient records.
• Direct comparison of two genetic variant groups (sarcomeric n=26, non-sarcomeric n=27).
• Includes clinical measures such as NT-proBNP levels and hemodynamic parameters.

Limitations

• Row count and column-level documentation are unknown, requiring manual inspection after download.
• The dataset is small (348.4 KB), limiting statistical power for complex modeling.
• Data may reflect temporal and institutional bias inherent to the single retrospective study.

Provenance

Source

figshare, authored by Catherine Gardin.

Collection Method

Retrospective study of patient records.

Time Range

Patients were often diagnosed between 6 and 10 years old; study period not specified.

Freshness

Last updated 2026-03-18 07:46:36.

Geography

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