Hybrid palliation to promote growth of left ventricle and left ventricular outflow tract.

OBJECTIVES: In patients with borderline left hearts or a severe left ventricular outflow tract (LVOT) obstruction, hybrid palliation can be used to stabilize the patient and postpone biventricular repair (BVR). In this study we analyzed growth of left-sided structures and outcomes of these patients. METHODS: We conducted a retrospective cohort study including patients who received hybrid palliation between January 2010 and September 2023. Echo measurements were collected at hybrid palliation, BVR and last follow-up. Growth of left ventricular structures were analyzed. RESULTS: In 38 patients hybrid palliation was used to promote growth of left ventricular structures. In total, 15 patients received a Ross-Konno/Yasui procedure while 23 patients received conventional BVR.In patients with a conventional BVR significant increase was found in left ventricular volume indexed by BSA (LVEDVi), z-score of aortic valve (AoV) and LVOT between hybrid palliation and BVR. Mitral valve Z-score did not increase significantly. After BVR until follow-up only increase of the AoV Z-scores and LVEDVi were found significant.Of all included patients (n = 38), additional surgical procedures were necessary in 8 patients during the interstage period and 15 patients after BVR. Additional catheter interventions were needed in 14 patients in the interstage period and 15 after BVR. Six patients died, no mortality in the conventional BVR group. CONCLUSIONS: Hybrid palliation as part of a staged biventricular repair is a safe and effective initial step and promotes growth of left ventricular structures in patients with small left-sided heart structures. Close follow-up is mandatory because extra catheter or surgical interventions are frequently needed.

Young patients with cystic fibrosis demonstrate subtle alterations of the cardiovascular system.

BACKGROUND: As life expectancy increases in patients with cystic fibrosis, it is important to pay attention to extra-pulmonary comorbidities. Several studies have shown signs of myocardial dysfunction in adult patients, but little is known about onset and development of these changes over time. In this prospective study, cardiac function in children with cystic fibrosis was compared to that of healthy children. METHODS: 33 children, aged 3-12years, with cystic fibrosis were recruited from the Wilhelmina Children's hospital and 33 age-matched healthy children were selected from the WHISTLER study, a population-based cohort study. Measurements of lung function, arterial stiffness, and echocardiography (conventional measures and myocardial deformation imaging) were performed. RESULTS: There were no differences in anthropometrics, lung function and blood pressure between the two groups. The cystic fibrosis children had a higher arterial stiffness compared to the healthy children (pulse wave velocity respectively 5.76±0.57m/s versus 5.43±0.61m/s, p-value 0.049). Using conventional echocardiographic parameters for right ventricular function, Tricuspid Annular Plane Systolic Excursion) and Tissue Doppler Imaging, cystic fibrosis children had a reduced right ventricular systolic function when compared to the healthy children. After adjustment for lung function, global strains of both right and left ventricles were significantly lower in the cystic fibrosis group than in healthy children (linear regression coefficient 1.45% left ventricle, p-value 0.022 and 4.42% right ventricle, p-value <0.01). Systolic strain rate of basal segment of the left ventricle, the mid segment of the right ventricle and the apical septum were significantly lower in the cystic fibrosis children than in healthy controls. CONCLUSION: Our study suggests that already at a very young age, children with cystic fibrosis show an increased arterial stiffness and some signs of diminished both right and left ventricular function.

Cardiovascular and Metabolic Health of 74 Children From Women Previously Diagnosed With Polycystic Ovary Syndrome in Comparison With a Population-Based Reference Cohort.

Women with polycystic ovary syndrome (PCOS) have compromised cardiovascular health profiles and an increased risk of pregnancy complications. In order to evaluate potential consequences, we aim to compare the cardiovascular and metabolic health of the children from women with PCOS with a population-based reference cohort. We included children from women with PCOS between the age of 2.5 to 4 years (n = 42) and 6 to 8 years (n = 32). The reference groups consisted of 168 (3-4 years old) and 130 children (7-8 years old). In an extensive cardiovascular screening program, we measured anthropometrics and blood pressure (all children), heart function and vascular rigidity (young children), metabolic laboratory assessment and carotid intima thickness (old age-group). Results showed that young PCOS offspring have a significantly lower diastolic blood pressure (ß = 2.3 [95% confidence interval, CI: 0.5-4.0]) and higher aortic pulse pressure (ß = -1.4 [95% CI: -2.5 to -0.2]), compared to the reference population. Furthermore, a higher left ventricle internal diameter but a lower tissue Doppler imaging of the right wall in systole compared to the reference group was found. Older offspring of women with PCOS presented with a significantly lower breast and abdominal circumference, but higher triglycerides (ß = -0.1 [95% CI: -0.2 to -0.1]), LDL-cholesterol (ß = -0.4 [95% CI: -0.6 to -0.1]), and higher carotid intima-media thickness (ß = -31.7 [95% CI: -46.6 to -16.9]) compared to the reference group. In conclusion, we observe subtle but distinct cardiovascular and metabolic abnormalities already at an early age in PCOS offspring compared to a population-based reference group, despite a lower diastolic blood pressure, breast, and abdominal circumference. These preliminary findings require confirmation in independent data sets.

Feasibility and characteristics of arterial stiffness measurement in preschool children.

Background Arterial stiffness is an important predictor of cardiovascular risk in adult life. Increased arterial stiffness can also be present in children and may be associated with several other cardiovascular risk factors. Until now, however, we know little about measuring arterial stiffness in preschool children. In this study, we assessed the feasibility of measuring arterial stiffness in preschool children and explored possible determinants related to arterial stiffness at this age. Methods We studied 168 healthy children, aged 3.3-4.1 years, who were recruited from a prospective birth cohort. We measured arterial stiffness, expressed in aortic pulse wave velocity and augmentation index, using a non-invasive oscillometric device (Arteriograph). We measured anthropometry and recorded other determinants using a questionnaire. Results In 100 children (59.5%) at least one valid arterial stiffness measurement was obtained. Of these infants, 89 had at least two valid measurements and 73 infants had at least three valid measurements. The mean aortic pulse wave velocity was 5.56 m/s (SD 0.77), and the mean augmentation index of the aorta was 19.7 m/s (SD 7.0). The augmentation index was significantly inversely associated with body height, with a regression coefficient of -0.78 (m/s)/cm (95% confidence interval -1.13 to -0.42). The augmentation index was not significantly associated with age, sex or (birth) weight. Conclusion The feasibility of measuring arterial stiffness in preschool children using the Arteriograph is moderate. We identified height as the most important determinant of the augmentation index in preschool children.

Maternal body mass index, neonatal lung function and respiratory symptoms in childhood.

Recent studies have shown that maternal obesity is associated with increased risk of wheezing in the offspring. We assessed whether impaired neonatal lung function could explain this association. We measured neonatal lung function in 2606 children of our prospective birth cohort. Information about daily symptoms of wheezing was obtained using questionnaires. Consultations and prescriptions for wheezing illnesses were derived from general practitioner patient files. Higher maternal body mass index (BMI) was associated with increased risk of wheezing in the first year of life and more consultations and prescriptions for wheezing illnesses until the age of 5 years. Lung function could partially explain the association with wheezing in the first year of life. Adding respiratory resistance to the model decreased the incidence rate ratio from 1.023 (95% CI 1.008-1.039) to 1.015 (95% CI 0.998-1.032). Anthropometrics of the 5-year-olds largely explained the association with consultations. Intermediates or confounders could not explain the association with prescriptions. There is an association between higher maternal BMI and increased risk of wheezing illnesses. In the first year of life, it is largely explained by an impaired lung function in early life, especially in children of nonatopic mothers. At the age of 5 years, infant lung function is of minor influence in this association.

Life-course of cardio-respiratory associations.

BACKGROUND: Several studies have shown that raised cardiovascular risk factors are associated with an impaired lung function in adulthood. Whether this association also exists in the young is unknown. Our aim was to study the relation between blood pressure and lung function from neonatal to elderly age. STUDY DESIGN: This was a cross-sectional study in a general population cohort. METHODS: Within the Utrecht Health Project (UHP) 6673 adults (aged 18-91 years) had spirometry and blood pressure measurements taken. In the WHeezing Illnesses STudy LEidsche Rijn (WHISTLER) study, a satellite birth cohort of the UHP, blood pressure and respiratory mechanics were measured using the single occlusion technique in 755 newborns and spirometry in 382 5-year-old participants. Linear regression analyses were performed with lung function as an independent variable and blood pressure as a dependent variable in different age groups. The analyses were adjusted for age, sex, weight and height. RESULTS: In infancy a more favorable lung function (higher compliance and lower resistance) was associated with higher blood pressure. In 5-year-old children and young adults higher forced expiratory volume in 1 second (FEV1) was associated with higher systolic blood pressure (p-values < 0.05). At the age of 5 the adjusted regression coefficient for systolic blood pressure was 4.8 mmHg/L (95% confidence interval (95% CI) -0.3-9.98). The association decreased with increasing age and reversed in the age groups above 40 years to -7.3 mmHg/L (95% CI -15.5-0.9) in those aged over 70 years of age. The association with pulse pressure showed a similar pattern. CONCLUSIONS: A positive association between the mechanical properties of the respiratory system and blood pressure in childhood and young adulthood reverses in later adulthood.

Nocturnal wheeze measurement in preschool children.

RATIONALE: Wheezing is a very common symptom in preschool children. Nocturnal wheezing is present in many asthmatic patients, due to enhanced airflow limitation overnight. We assessed the prevalence of nocturnal wheezing in young children and correlated this with respiratory system resistance and history of wheezing symptoms. METHODS: Using a continuous overnight recording of respiratory sounds we analyzed wheeze rate (ratio between wheezing time and recorded breathing time), oxygen saturation and heart rate during one night in 59 three-year-old children of an ongoing birth cohort study, the WHISTLER-project. We associated the nocturnal measurements with the patient's history of wheezing symptoms and with measurement of respiratory system resistance (Rint). RESULTS: Analysis of wheeze rate was successful in 44 children. The overall wheeze rate of these children was low, with the highest wheeze rate of 0.63% measured by the tracheal sensor during expiration. In total, 21/44 children had a wheeze rate of ≥ 5% during at least 1 min. There was no statistically significant difference in wheeze rate between the children with and without a history of wheezing. The wheeze rate of the tracheal sensor had a significant correlation with Rint (correlation coefficients of inspiration and expiration: 0.308 and 0.382, P-values 0.05 and 0.01, respectively). CONCLUSIONS: Overall, the wheeze rate in young children is low, but seems to increase over nighttime. Almost 50% of the children have sporadic wheeze during the night. Although higher nocturnal wheeze rates are related to increased respiratory system resistance, it is not related to clinical wheezing symptoms.