Hypertension: An Important but Reversible Cause of Systolic Dysfunction in Pediatrics

A. M. Kamsheh, K. E. Meyers, R. A. Palermo,D. S. Burstein, J. B. Edelson, K. Y. Lin,K. Maeda,J. W. Rossano, C. A. Wittlieb-Weber,M. J. O'Connor

JOURNAL OF HEART AND LUNG TRANSPLANTATION(2022)

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Abstract

Purpose

Cardiac dysfunction due to hypertension (CDHTN) in pediatrics is not well described. The purpose of the study is to describe the presentation and outcomes of pediatric CDHTN.

Methods

Patients ≤ 21 years with CDHTN between January 2005 and September 2020 at our institution were reviewed. Inclusion criteria were systolic dysfunction (shortening fraction (SF) <28% or ejection fraction <50%) without another cause of dysfunction, blood pressure (BP) >95th %ile for age and height within 3 months of diagnosis and physician judgment that dysfunction was secondary to hypertension. Demographics, clinical characteristics, echocardiographic findings and outcomes were described and examined using Chi-squared and Wilcoxon rank-sum tests.

Results

There were 34 patients included, with median age 10.9 (IQR 0.3-17.0) years. Due to a bimodal age distribution, patients were divided into groups <1 year (n=12) and >1 year (n=22) for outcome analysis. Causes of hypertension included medical renal disease (n=20, 59%), renovascular disease (n=6, 17%), essential (n=5, 15%), multifactorial (n=2, 6%) and medication effect (n=1, 3%). Peak systolic and diastolic BP at diagnosis was >99th %ile in 97% and 68% of patients, respectively. Echocardiography demonstrated mild LV dilation (median LV end diastolic z-score 2.6) and mild LV hypertrophy (median LV mass z-score 2.3). Patients were often symptomatic at diagnosis (n=21, 62%) and required admission (n=28, 82%), but a minority required intubation (n=9, 26%) or inotropic support (n=15, 44%). No patients received mechanical circulatory support (MCS), and only one patient was listed for transplant. Survival was high (n=33, 97%) over 3.5 (IQR 2.0-6.3) years follow up. Despite a trend toward worse function at diagnosis (SF 19% vs 23%, p=0.07), patients <1 year had shorter duration of dysfunction (12 vs 218 days, p=0.003) and better function at last follow up (SF 36% vs 29%, p=0.002). Overall, 92% of infants and 68% of older patients had resolution of systolic dysfunction.

Conclusion

Hypertension is an important cause of systolic dysfunction with a bimodal age distribution in children. Outcomes are favorable with low mortality and a high proportion of patients with resolution of systolic dysfunction, particularly those <1 year. Identification of hypertension as the cause of dysfunction is vital, as these patients do not often require MCS or transplantation.
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Endothelial Dysfunction
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