Aortic Dilatation in Children and Young People With ADPKD.

Introduction: Aortic root dilatation is a reported cardiovascular sequela seen in children and young people (CYP) with chronic kidney disease (CKD) but has yet to be described in those with autosomal dominant polycystic kidney disease (ADPKD). Methods: Single center, cross-sectional study in a dedicated ADPKD clinic. Echocardiograms were evaluated for the presence of dilatation (defined by a z-score ≥2 [≥99th percentile] SDs from the mean) at 4 standardized locations, namely the aortic valve annulus, sinuses of Valsalva (SoV), sinotubular junction (STJ), and the ascending aorta. Measurements were compared with a control group to assess prevalence, severity, and determinants of aortic dilatation. Results: Ninety-seven children, median age (interquartile range) of 9.3 (6.1, 13.6) years were compared with 19 controls without ADPKD or other CKD. The prevalence of dilatation ranged from 5.2% to 17% in ADPKD, depending on anatomical location with no aortic dilatation identified in the control group. In multivariable regression, aortic root dilatation was significantly associated with cyst burden at the aortic valve annulus and SoV (ß = 0.42 and ß = 0.39, both P < 0.001), with age at SoV (ß = -0.26, P = 0.02), systolic blood pressure (SBP) z-score at SoV (ß = -0.20, P = 0.04) and left ventricular mass index (LVMI) at SoV and STJ (ß = 0.24, P = 0.02 and ß = 0.25, P = 0.03, respectively) following adjustment for age, sex (male or female), body mass index (BMI) z-score, estimated glomerular filtration rate (eGFR), SBP z-score, and LVMI. Conclusion: Our data suggests increased prevalence of aortic root and ascending aortic dilatation in CYP with ADPKD compared with controls. Further studies are needed to understand the pathogenesis and its contribution to the high cardiovascular morbidity in ADPKD.

Reliability of systolic blood pressure measured by parents in young children at home using a hand held doppler device and aneroid sphygmomanometer.

OBJECTIVE: We report data regarding systolic BP monitoring in children aged <5 years performed over a 2-week period by parents at home using a hand-held doppler device and aneroid sphygmomanometer for SBP measurements (HDBPM). Our objectives were to compare health professional measured office systolic BP by doppler device (Office-SBP Doppler ) with parent measured home systolic BP using the same doppler device (Home-SBP Doppler ). We also report data evaluating reliability and optimal number of days of measurement required. DESIGN AND METHODS: We taught parents to measure systolic BP and assessed their technique using a hand-held doppler device and aneroid sphygmomanometer. We requested parents to perform three consecutive BP measurements twice daily (ideally morning and evening around similar times) when the child was awake, settled and cooperative. RESULTS: Over a 3-year period, data from 48 of 62 children who underwent HDBPM measurements were evaluated with median (IQR) age of 1.9 (0.9, 3.6) years, 27 (56%) boys and 14 (29%) on antihypertensive medication. Office-SBP Doppler was 2.9 ±â€Š8.9 mmHg [95% confidence interval (CI), -14.4 to 20.4, P  = 0.026] higher than Home-SBP Doppler . Mean Home-SBP Doppler between Week-1 and Week-2 monitoring was similar -0.45 ±â€Š3.5 mmHg (95% CI, -7.35 to 6.45, P  = 0.41). Morning HDBPM measurements were lower than evening with a mean difference of -2.77 ±â€Š3.92 mmHg, P  < 0.001). Over Week-1, mean Home-SBP Doppler was closer to mean Office-SBP Doppler with increasing cumulative days of monitoring and with smaller standard deviations suggesting that readings become more reliable from day 4 onwards. CONCLUSIONS: HDBPM is a reliable method for measuring systolic BP in young children with BP levels measured by parents comparable to those performed by health professional in clinic. HDBPM technique described here and performed by parents over a 7-day period with a minimum of 4-days, offers a reliable and reproducible technique to measure blood pressure at home.

A meta-analysis of the haemodynamics of primary hypertension in children and adults.

We performed a systematic review and meta-analysis to determine the relative contributions of elevated cardiac output and systemic vascular resistance to hypertension in children and adults. This included 27 studies on 11 765 hypertensive and normotensive children and adults in whom cardiac output was measured. Cardiac output but not systemic vascular resistance was elevated in hypertensive compared to normotensive children and young adults (difference in means 1.15 [0.78-1.52] l/min, P < 0.001). In older hypertensive adults, both were elevated compared to normotensive individuals (0.40 [0.26-0.55] l/min, P < 0.001 and 3.21 [1.91-4.51] mmHg min/l, P < 0.001 for cardiac output and systemic vascular resistance, respectively). The main haemodynamic alteration in primary hypertension (including obesity-hypertension) in both children and young to middle-aged adults is an elevation of cardiac output. With longer duration and greater severity of hypertension there may be progression from a 'cardiac' to a 'vascular' phenotype with increased systemic vascular resistance.

Demographics of childhood hypertension in the UK: a report from the Southeast England.

We aimed to describe hypertensive phenotype and demographic characteristics in children and adolescents referred to our paediatric hypertension service. We compared age, ethnicity and BMI in primary hypertension (PH) compared to those with secondary hypertension (SH) and white coat hypertension (WCH). Demographic and anthropometric data were collected for children and adolescents up to age 18 referred to our service for evaluation of suspected hypertension over a 6 year period. Office blood pressure (BP) and out of office BP were performed. Patients were categorised as normotensive (normal office and out of office BP), WCH (abnormal office BP, normal out of office BP), PH (both office and out of office BP abnormal, no underlying cause identified) and SH (both office and out of office BP abnormal, with a secondary cause identified). 548 children and adolescents with mean ± SD age of 10.1 ± 5.8 years and 58.2% girls. Fifty seven percent (n = 314) were hypertensive; of these, 47 (15%), 84 (27%) and 183 (58%) had WCH, PH and SH, respectively. SH presented throughout childhood, whereas PH and WCH peaked in adolescence. Non-White ethnicity was more prevalent within those diagnosed with PH than both the background population and those diagnosed with SH. Higher BMI z-scores were observed in those with PH compared to SH. Hypertensive children <6 years are most likely to have SH and have negligible rates of WCH and PH. PH accounted for 27% of hypertension diagnoses in children and adolescents, with the highest prevalence in adolescence, those of non-White Ethnicity and with excess weight.

Hypertension in Children and Young Adults.

Primary hypertension (PH) is most common during adolescence with increasing prevalence globally, alongside the epidemic of obesity. Unlike in adults, there are no data on children with uncontrolled hypertension and their future risk of hard cardiovascular and cerebrovascular outcomes. However, hypertension in childhood is linked to hypertensive-mediated organ damage (HMOD) which is often reversible if treated appropriately. Despite differing guidelines regarding the threshold for defining hypertension, there is consensus that early recognition and prompt management with lifestyle modification escalating to antihypertensive medication is required to ameliorate adverse outcomes. Unfortunately, many unknowns remain regarding pathophysiology and optimum treatment of childhood hypertension.

Haemodynamics of Hypertension in Children.

PURPOSE OF REVIEW: To review the haemodynamic characteristics of paediatric hypertension. RECENT FINDINGS: Pulsatile components of blood pressure are determined by left ventricular dynamics, aortic stiffness, systemic vascular resistance and wave propagation phenomena. Recent studies delineating these factors have identified haemodynamic mechanisms contributing to primary hypertension in children. Studies to date suggest a role of cardiac over activity, characterized by increased heart rate and left ventricular ejection, and increased aortic stiffness as the main haemodynamic determinants of primary hypertension in children.

Renal disease in pregnancy: Fetal, neonatal and long-term outcomes.

Renal disease in women of childbearing age is estimated to be approximately 3%; consequently, renal disease is not an uncommon comorbidity in pregnancy. There has been considerable evidence published over the last 20 years to suggest that renal disease in pregnancy is associated with higher maternal, fetal, and offspring morbidity. Studies published are largely heterogeneous; include unmatched cohort studies; and focus on early neonatal outcomes such as prematurity, small for gestational age, and neonatal unit admission. There appears to be an inverse relationship between maternal renal function and likelihood of neonatal morbidity using these outcome measures. Overall though, data regarding medium-to long-term outcomes for children born to mothers with renal disease are scarce. However, in view of emerging epidemiological evidence regarding cardiovascular programming in intrauterine life in those born premature or small for gestational age, it is likely that this population of children remain at high risk of cardiovascular disease as adults. The scope of this review is to amalgamate and summarize existing evidence regarding the outcomes of infants born to mothers with renal disease. Focus will be given to pregnancy-related acute kidney injury, chronic kidney disease, dialysis, and transplantation.