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J Pediatr. 2019 Apr;207:42-48. doi: 10.1016/j.jpeds.2018.10.064. Epub 2018 Dec 7.

Characterization of Post-Thrombotic Syndrome in Children with Cardiac Disease.

Author information

1
Labatt Family Heart Centre, University of Toronto, The Hospital for Sick Children, Toronto, ON, Canada. Electronic address: cedric.manlhiot@sickkids.ca.
2
Labatt Family Heart Centre, University of Toronto, The Hospital for Sick Children, Toronto, ON, Canada.
3
Division of Haematology/Oncology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, ON, Canada.

Abstract

OBJECTIVE:

To assess the validity of existing clinical scales assessing the presence of physical and functional abnormalities for diagnosing post-thrombotic syndrome (PTS) in children, including specific evaluation of use in children with congenital heart disease (CHD).

STUDY DESIGN:

One hundred children aged >2 years (average age, 6 years), including 33 with CHD and previously proven extremity deep vein thrombosis (DVT), 37 with CHD and no previous DVT, and 30 healthy siblings, were blindly assessed for PTS using the modified Villalta Scale (MVS). All patients aged <6 years underwent neurodevelopmental testing and an age-appropriate quality of life assessment.

RESULTS:

The MVS identified mild PTS in 20 children and moderate PTS in 1 child (including 14 of 33 [42%] in the CHD/DVT group, 5 of 37 [14%] in the CHD/no DVT group, and 2 of 30 controls [7%]). The diagnosis of PTS was confirmed clinically in 14 patients, all of whom had previous thrombosis and 1 of whom was MVS-negative. MVS had an accuracy of 91% and performed reasonably well as a screening tool but poorly as a diagnostic tool. MVS reliability was acceptable. Children with PTS had similar quality of life as those without PTS but had higher rates of neurodevelopmental delays in gross motor skills (70% vs 24%; P = .02) and problem-solving indicators (60% vs 15%; P = .008).

CONCLUSIONS:

Using the MVS scale for PTS screening in children with CHD is feasible and reliable, and the scale has good correlation with a clinical diagnosis of PTS despite a high prevalence of false-positive findings. Further research is needed to determine the clinical relevance of PTS in this population.

KEYWORDS:

congenital heart disease; instrument validation; neurodevelopment; pediatrics

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