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.