Abstract
BackgroundThere is growing recognition of hypertension in a significant proportion of children with ADPKD. In this study, we assessed blood pressure and cardiovascular status in children with ADPKD.MethodsA prospective two-centre observational study of children (< 18 years) with ADPKD was compared against age- and BMI-matched healthy controls. Children underwent peripheral BP (pBP) measured using an aneroid sphygmomanometer and auscultation, 24-h ambulatory BP monitoring (ABPM), non-invasive central BP (cBP) measurement, carotid-femoral pulse wave velocity (PWVcf) measured using applanation tonometry and measurement of indexed left ventricular mass (LVMI) using echocardiography. This study received independent ethical approval.ResultsForty-seven children with ADPKD and 49 healthy controls were recruited (median age 11 years vs. 12 years). Children with ADPKD had significantly higher systolic pBP (mean 112 ± 13.5 mmHg vs. 104 ± 11 mmHg, p < 0.001), higher systolic cBP (mean 97 ± 12.8 mmHg vs. 87 ± 9.8 mmHg, p < 0.001) and lower pulse pressure amplification ratio (1.59 ± 0.2 vs. 1.67 ± 0.1, p = 0.04) compared to healthy children. Thirty-five percent of children with ADPKD showed a lack of appropriate nocturnal dipping on 24-h ABPM. There was no difference in PWVcf between children with ADPKD and healthy children (mean 5.74 ± 1 m/s vs. 5.57 ± 0.9 m/s, p = 0.46). Those with ADPKD had a significantly higher LVMI (mean 30.4 ± 6.6 g/m2.7 vs. 26.2 ± 6.2 g/m2.7, p = 0.01).ConclusionsThese data highlight the high prevalence of hypertension in children with ADPKD, also demonstrating early cardiovascular dysfunction with increased LVMI and reduced PP amplification despite preserved PWVcf, when compared with healthy peers. These early cardiovascular abnormalities are likely to be amenable to antihypertensive therapy, reinforcing the need for routine screening of children with ADPKD.
Highlights
Autosomal dominant polycystic kidney disease (ADPKD) was traditionally considered an ‘adult’ disease [1], but recent evidence from multiple studies has shown a significant prevalence of hypertension and other features suggestive of early cardiac damage during childhood [2,3,4]
We have identified that those with ADPKD have higher mean peripheral and central blood pressures, compared to healthy children
That 8 children in our study were on anti-hypertensive medication suggests that the true mean BP values may be even greater in the ADPKD group, had they not been on anti-hypertensives at the time of the study
Summary
Autosomal dominant polycystic kidney disease (ADPKD) was traditionally considered an ‘adult’ disease [1], but recent evidence from multiple studies has shown a significant prevalence of hypertension and other features suggestive of early cardiac damage during childhood [2,3,4]. Some studies (including the largest single study published to date) report higher prevalence of hypertension in children with ADPKD, up to 35% [8]. Those with ADPKD had a significantly higher LVMI (mean 30.4 ± 6.6 g/m2.7 vs 26.2 ± 6.2 g/ m2.7, p = 0.01) Conclusions These data highlight the high prevalence of hypertension in children with ADPKD, demonstrating early cardiovascular dysfunction with increased LVMI and reduced PP amplification despite preserved PWVcf, when compared with healthy peers. These early cardiovascular abnormalities are likely to be amenable to antihypertensive therapy, reinforcing the need for routine screening of children with ADPKD
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