No Increase in Masked Hypertension Prevalence in Children With Sickle Cell Disease in France.

BACKGROUND: An important prevalence (32%-45%) of masked hypertension has been reported in children with sickle cell disease (SCD). Stroke screening is well established using transcranial Doppler (TCD) ultrasound. The objectives of our proof-of-concept study in childhood SCD were to evaluate the prevalence of hypertension and its relationships with cerebral vasculopathy (TCD velocity) and to further evaluate in a subgroup of children the correlations of cardiovascular autonomic nervous system indices with TCD velocity. METHODS: Ambulatory blood pressure measurement (ABPM) and TCD velocity were obtained in children with SCD and in a restricted sample, cardiac sympathovagal balance using heart rate variability analyses, baroreflex sensitivity, and pulse wave velocity were measured. RESULTS: In 41 children with SCD (median age 14.0 years, 19 girls, SS/Sß + thalassemia/SC: 33/2/6), ABPM results showed masked hypertension in 2/41 (5%, 95% confidence interval, 0-11) children, consistent with the prevalence in the general pediatric population, elevated blood pressure (BP) in 4/41 (10%) children, and a lack of a normal nocturnal dip in 19/41 children (46%). Children with increased TCD velocity had lower nocturnal dipping of systolic BP. In the 10 participants with extensive cardiovascular assessment, increased TCD velocity was associated with parasympathetic withdrawal and baroreflex failure. Exaggerated orthostatic pressor response or orthostatic hypertension was observed in 7/10 children that was linked to parasympathetic withdrawal. CONCLUSIONS: Autonomic nervous system dysfunction, namely loss of parasympathetic modulation, of SCD contributes to increase TCD velocity but is not associated with an increased prevalence of masked hypertension. CLINICAL TRIALS REGISTRATION: NCT04911049.

Diagnostic tests and subtypes of dysfunctional breathing in children with unexplained exertional dyspnea.

BACKGROUND: Inappropriate hyperventilation during exercise may be a specific subtype of dysfunctional breathing (DB). OBJECTIVE: To assess whether Nijmegen questionnaire and hyperventilation provocation test (HVPT) are able to differentiate inappropriate hyperventilation from other DB subtypes in children with unexplained exertional dyspnea, and normal spirometry and echocardiography. METHODS: The results were compared between a subgroup of 25 children with inappropriate hyperventilation (increased V'E/V'CO2 slope during a cardiopulmonary exercise test (CPET)) and an age and sex matched subgroup of 25 children with DB without hyperventilation (median age, 13.5 years; 36 girls). Anxiety was evaluated using State-Trait Anxiety Inventory for Children questionnaire. RESULTS: All children were normocapnic (at rest and peak exercise) and the children with hyperventilation had lower tidal volume/vital capacity on peak exercise (shallow breathing). The Nijmegen score correlated positively with dyspnea during the CPET and the HVPT (p = 0.001 and 0.010, respectively) and with anxiety score (p = 0.022). The proportion of children with a positive Nijmegen score (≥19) did not differ between hyperventilation (13/25) and no hyperventilation (14/25) groups (p = 0.777). Fractional end-tidal CO2 (FETCO2 ) at 5-min recovery of the HVPT was < 90% baseline in all children (25/25) of both subgroups. Likewise, there was no significant difference between the two subgroups for other indices of HVPT (FETCO2 at 3-min recovery and symptoms during the test). CONCLUSION: The validity of the Nijmegen questionnaire and the HVPT to discriminate specific subtypes of dysfunctional breathing, as well as the relevance of the inappropriate hyperventilation subtype itself may both be questioned.

Prenatal diagnosis of congenital ventricular aneurysm and diverticulum: Prenatal features and perinatal management.

Congenital ventricular diverticulum (VD) and aneurysm are rare cardiac developmental anomalies and their pathophysiology is still unclear. They present as an anomaly of the four chambers view, cardiomegaly, arrhythmia, pericardial effusion, or hydrops. They are usually isolated anomalies. Differential diagnosis between diverticulum and aneurysm is challenging during the prenatal period. Management policy is not uniform either conservative or repeated pericardial puncture. OBJECTIVE: We wanted to describe prenatal features and post-natal outcomes of fetal cardiac out pouching. METHODS: We retrospectively report 6 cases of VD and aneurysm prenatally managed in our fetal medicine unit between 2010 and 2020. All cases were evaluated from the first or second trimester of pregnancy until postnatal follow-up (3 months to 3 years). RESULTS: All six cases underwent a monthly ultrasound follow-up with spontaneous regression of pericardial effusion, and normal hemodynamics at birth No pericardial puncture was done and postnatal outcome was favorable in all cases. CONCLUSION: Based on our experience and on cases previously published, prenatal counseling should be prudent regarding the final diagnosis. Referral and monthly prenatal ultrasound follow-up, birth in a tertiary center after multidisciplinary evaluation and cardiological evaluation at birth still seem mandatory.

Interdialytic weight gain and vasculopathy in children on hemodialysis: a single center study.

BACKGROUND: Increased interdialytic weight gain (IDWG) has been associated with poor outcomes in adults, but its impact on hemodialysis vasculopathy in children is unknown. METHODS: Nineteen patients (age 9 to 19 years old) with a median hemodialysis duration of 10.4 months were enrolled. Cardiovascular evaluation included left ventricular mass index (LVMI), pulse wave velocity (PWV), and carotid intima-media thickness (cIMT) measurements. PWV and cIMT were expressed as z-scores based on reference values in healthy children. Blood pressure (BP) evaluation consisted in a 24-h ambulatory BP monitoring. Mean IDGW and residual urine output during the 6 months prior to cardiovascular examination were calculated. RESULTS: Increased cIMT, LVMI, and PWV was observed in 11 (57.9%), 7 (36.8%), and 5 (26.3%) patients respectively, while BP was normal in all patients. Median IDWG was 3.5% (1.8-6.7). Residual urine output and BP status did not significantly differ between patients with IDWG ≥ or < 4%. After linear regression, IDWG was correlated to cIMT z-score (r2 = 0.485, p = 0.001), but not to PWV z-score (r2 = 0.04, p = 0.415) and LVMI (r2 = 0.092, p = 0.206). After univariate logistic regression, IDWG ≥ 4% was significantly associated to increased cIMT (above 1.65 SDS) (odds ratio 12.25, 95% confidence interval 1.08-138.988). The trend toward an increased cIMT with IDWG ≥ 4% was observed in both patients with short and long dialysis vintage. CONCLUSIONS: High IDWG is associated with increased cIMT in hemodialyzed children independently of BP control and dialysis vintage. This observation reinforces the importance of interventions to avoid IDWG in hemodialyzed children.

Early cardiovascular manifestations in children and adolescents with autosomal dominant polycystic kidney disease: a single center study.

BACKGROUND: This study aims to describe the cardiovascular manifestations in children with autosomal dominant polycystic kidney disease (ADPKD) and detect their relation with kidney disease and type of gene mutation. METHODS: Twenty-one patients (7 to 19 years old) were included. Cardiovascular evaluation involved blood pressure (BP), indexed left ventricular mass (LVMI), pulse wave velocity (PWV), and carotid intima media thickness (cIMT) measurement. Patients were classified according to percentile reference values of these parameters in healthy children. The 95th percentile was the highest level of normal values. Glomerular filtration rate (GFR) and microalbuminuria were also measured. RESULTS: Antihypertensive treatment, large LVMI, high PWV, and increased cIMT were observed in 6 (28.6%), 2 (9.5%), 4 (19%), and 8 (38.1%) patients respectively. Antihypertensive treatment was not associated with either high PWV or increased cIMT. Linear correlation was noticed between LVMI and PWV (r2 = 0.243, p = 0.023) and also between LVMI and cIMT (r2 = 0.203, p = 0.041). The median age of patients with high PWV, increased cIMT, and large LVMI was 9.5, 13, and 18 years old. GFR was normal in all patients. Patients with increased cIMT presented higher levels of urine microalbumin to creatinine ratio (p = 0.025). Genetic mutation was available in 18 patients. Antihypertensive treatment was more frequent in patients without PKD1 missense mutation (p = 0.044). CONCLUSIONS: High PWV and increased cIMT indicating arterial stiffness and hypertrophic vasculopathy may be present in children with ADPKD regardless BP status, and prior to GFR decline, suggesting that vascular disease precedes chronic kidney disease in ADPKD.

Mazindol: a risk factor for pulmonary arterial hypertension?

Mazindol is an imidazo-isoindole derivative, a tricyclic compound and a non-amphetamine central nervous system stimulant that blocks dopamine and norepinephrine reuptake. Mazindol was withdrawn from the US and European markets in 1999 for reasons unrelated to its efficacy or safety around a time when other anorexic drugs were found to be associated with the development of pulmonary arterial hypertension (PAH). Despite the use of mazindol for decades, reports of PAH due to mazindol intake have been extremely rare. Recent interest on mazindol has emerged for the treatment of narcolepsy and attention-deficit/hyperactivity disorder. Therefore, an updated understanding of the potential benefits and risks of mazindol in these patient populations is warranted.