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Late recovery of ventricular function in children with idiopathic dilated cardiomyopathy

¹Division of Cardiology, Childrens Hospital Los Angeles, Department of Pediatrics, CA 90054-0070, USA.

American Heart Journal

|July 30, 1999

Insights

Complete recovery of left ventricular (LV) function is achievable in children with idiopathic dilated cardiomyopathy (IDC). While some see improvement within a year, most patients require longer follow-up for normal LV function.

Area of Science:

Pediatric Cardiology
Cardiovascular Research
Pediatric Critical Care Medicine

Background:

Idiopathic dilated cardiomyopathy (IDC) in children presents a variable prognosis.
Poor left ventricular (LV) function recovery correlates with high mortality.
Complete normalization of LV performance in pediatric IDC remains understudied.

Purpose of the Study:

To investigate the possibility and timeline of complete recovery of LV function in pediatric IDC.
To identify factors associated with LV function normalization in children with IDC.

Main Methods:

Retrospective review of clinical data and echocardiograms for 63 children diagnosed with IDC.
Comparison of patients who achieved normal LV fractional shortening (LVFS) with those who did not.
Analysis of LV dimensions and LVFS changes over time.

Main Results:

Sixteen children (Group 1) demonstrated progressive LVFS improvement to normal ranges, unlike 47 children (Group 2) with persistent LV dysfunction.
Group 1 showed significant improvement in LVFS (13.6% to 33.7%) and normalization of LV dimensions (z-score from 6.9 to 1.3).
Recovery to normal LV function was observed between 0.3 and 14 years, with a mean follow-up of 4.5 years.

Conclusions:

Complete recovery of LV function is a possible outcome for children diagnosed with IDC.
While some children experience rapid improvement, the majority require extended follow-up periods for full functional recovery.
Normalization of LV dimensions accompanies functional recovery in pediatric IDC patients.

Read full analysis

Abstract

Background:

The prognosis for children with idiopathic dilated cardiomyopathy (IDC) is variable. Patients who fail to exhibit improvement in left ventricular (LV) function have a high 1-year mortality rate, whereas improvement in LV fractional shortening (LVFS) to >15% is associated with better survival. However, complete recovery of LV performance to normal has not been examined.

Methods And Results:

The clinical features and echocardiograms of 63 children with IDC were reviewed. Sixteen patients (group 1) were identified who demonstrated progressive improvement in LVFS, ultimately recovering to within the normal range. They were compared with 47 patients (group 2) in whom LVFS remained depressed. Group 1 LVFS at first examination was 13.6% +/- 5.1%, z = -10.8 +/- 4.0, and improved to within the normal range (33.7% +/- 3.4%, z = -0.9 +/- 1. 4, P <.001). Group 2 initial LVFS was 13.6 +/- 2.3, z = -8.9 +/- 3.2 and did not change significantly (15.7% +/- 7.3%, z = -7.3 +/- 1.6). The LV was dilated at initial examination in all patients (z = 6.9 +/- 3.0). Recovery in group 1 was associated with a decrease in LV dimension to within the normal range (z = 1.3 +/- 1.6, P <.001), whereas the LV dimension in group 2 patients remained increased (z = 6.2 +/- 3.4). The mean follow-up time at which LV function was noted to be normal was 4.5 +/- 3.6 years (range 0.3 to 14 years). The total duration of follow-up was 6.5 +/- 5.2 years (range 1 to 16 years).

Conclusions:

Complete recovery of LV function is possible in children with IDC. Recovery may occur within the first year after initial examination in some patients, but longer periods are needed in the majority of patients in whom LV function ultimately returned to normal.

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