Pulmonary hypertension and cardiac function in congenital diaphragmatic hernia: relationship to disease severity and outcomes

Abstract Objective To assess patterns of postnatal biventricular function and their relationship to prenatal and postnatal markers of disease severity in infants with congenital diaphragmatic hernia (CDH). To investigate clusters of micro RNA as 17-92 expressed in pulmonary hypoplasia in patients with CDH. Study Design Observational case-control study of cardiac function in infants with CDH in the first 5 days of life. Systolic and diastolic function in the right (RV) and left (LV) ventricles were assessed using speckle tracking-derived global strain and tissue Doppler imaging of myocardial velocities. In another cohort of CDH, Bronchoalveolar aspiration (BAL) fluid was obtained and metabolomic analysis was run for micro RNA 17-92 cluster. Specifically miR17, miR18a, miR19a, miR19b, miR20a, miR92 were analyzed. Correlation between cardiac function and prenatal observed:expected total fetal lung volume (TFLV), oxygenation index (OI), duration of intubation and length of stay were assessed. Results All measures of systolic and diastolic function were significantly reduced in the CDH group (n=25) compared to controls (n=20) at <48 hours, and improved by 72- 96 hours. LV global systolic longitudinal strain (LV GLS) correlated with prenatal TFLV (r2 0.32, p 0.03), OI (r2 0.35, P<0.001), duration of intubation (r2 0.24, p 0.04), and length of stay (r2 0.4, p 0.006). LV GLS at <48 hours was significantly lower in 6 CDH patients who did not survive and/or required ECMO compared to those who did not; -11.5 (5.3) % vs -16.9 (5.3) %, p 0.02. For the metabolomic analysis we found miR17, miE18a, miR19b, miR20a were found significantly higher in CDH (p<0.05) and miR19a lower (p<0.05). No significant correlation was found between miR92 expression and clinical outcomes. Conclusions Right and left ventricular function are impaired in the transitional period in infants with CDH. Early left ventricular systolic function correlates with prenatal and postnatal markers of clinical disease severity and may be an important determinant of disease severity and therapeutic target in CDH. These findings support regular assessment of cardiac function in CDH and investigational trials of targeted cardiovascular therapies. miR 17-92 cluster may represent a potential hallmark of PH in CDH however from our result doesn’t predict disease severity

Early postnatal ventricular dysfunction is associated with disease severity in patients with congenital diaphragmatic hernia

Objective: To assess patterns of postnatal ventricular function and their relationship to prenatal and postnatal markers of disease severity in infants with congenital diaphragmatic hernia (CDH). Study design: In this observational case-control study of cardiac function in infants with CDH in the first 5 days of life, systolic and diastolic function in the right ventricle (RV) and left ventricle (LV) were assessed using speckle tracking echocardiography-derived global strain and tissue Doppler imaging. Correlation between cardiac function and prenatal observed:expected total fetal lung volume (TFLV), oxygenation index (OI), duration of intubation, and hospital length of stay were assessed. Results: All measures of systolic and diastolic function were significantly reduced in the CDH group (n = 25) compared with controls (n = 20) at &lt;48 hours, and were improved by 72-120 hours. LV global systolic longitudinal strain (GLS) correlated with prenatal TFLV (R2= 0.32; P =.03), OI (R2= 0.35; P &lt;.001), duration of intubation (R2= 0.24; P =.04), and length of stay (R2= 0.4; P =.006). Mean (SD) LV GLS at &lt;48 hours was significantly lower in infants with CDH who did not survive and/or required ECMO compared with those who did not: −11.5 (5.3)% vs −16.9 (5.3)% (P =.02). Conclusions: RV and LV function are impaired in the transitional period in infants with CDH. Early LV systolic function correlates with prenatal and postnatal markers of clinical disease severity and may be an important determinant of disease severity and therapeutic target in CDH. These findings support regular assessment of cardiac function in CDH and investigational trials of targeted cardiovascular therapies

Neurocognitive assessment and sleep analysis in children with sleep-disordered breathing

Objective: To assess possible correlations between intelligence quotient (IQ) and attention deficit hyperactive disorder (ADHD) rating scale values and sleep (including cyclic alternating patterns analysis) and respiratory parameters in children with sleep-disordered breathing (SDB). Methods: Thirteen children who satisfied the criteria for primary snoring and 31 children for obstructive sleep apnea syndrome (OSAS) underwent polysomnography in a standard laboratory setting and a neurocognitive assessment. Sixty normal controls recruited from two schools underwent the neurocognitive assessment. Results: The IQ estimates of controls were higher and the ADHD rating scale scores lower than those of children with SDB. Children with OSAS had a higher REM sleep latency and arousal index as well as a lower N3 and A mean duration than children who snored. In our sample of children with SDB, the percentage of wakefulness after sleep onset, of N1, of A2, of arousal and A2 index correlated positively with global intelligence. Total and hyperactivity scores correlated positively with the A2 index. Regression analysis mostly confirmed the correlations between neurocognitive measures and sleep parameters and further demonstrated a negative correlation between the hyperactivity rating score and oxygen saturation during the night. Conclusions: Our results support the hypothesis that arousal is a defensive mechanism that may preserve cognitive function by counteracting the respiratory events, at the expense of sleep maintenance and NREM sleep instability. Significance: We believe that our study makes an interesting contribution to research on the relationship between sleep fragmentation and cognitive function. (C) 2010 International Federation of Clinical Neurophysiology

Propofol Formulation Affects Myocardial Function in Newborn Infants

This study aimed to evaluate the effects of propofol in diluted and undiluted formulations on cardiac function in infants. Infants > 30 days received propofol sedation for central line insertion. Cases were divided into two groups: those who received undiluted 1% propofol (P1%); and those who received a diluted formulation (Pd) of equal volumes propofol 1% and 0.9% NaCl. Echocardiograms were performed pre (t0)-, immediately post (t1)-, and 1-h post (t2) propofol administration. Myocardial deformation was assessed with tissue Doppler imaging (TDI) analysis and peak longitudinal strain (LS). 18 cases were included: nine (50%) P1% and nine (50%) Pd. In the P1% group, TDI velocities and LS were significantly reduced at t1 and t2. In the Pd Group, only TDI velocities in the left ventricle were reduced at t1, but not at t2. Dilution of propofol may minimize myocardial dysfunction while maintaining adequate sedation in infants. Further comparative studies are needed to investigate the safety and efficacy of this approach.status: publishe

Propofol Formulation Affects Myocardial Function in Newborn Infants.

This study aimed to evaluate the effects of propofol in diluted and undiluted formulations on cardiac function in infants. Infants > 30 days received propofol sedation for central line insertion. Cases were divided into two groups: those who received undiluted 1% propofol (P1%); and those who received a diluted formulation (Pd) of equal volumes propofol 1% and 0.9% NaCl. Echocardiograms were performed pre (t)-, immediately post (t)-, and 1-h post (t) propofol administration. Myocardial deformation was assessed with tissue Doppler imaging (TDI) analysis and peak longitudinal strain (LS). 18 cases were included: nine (50%) P1% and nine (50%) Pd. In the P1% group, TDI velocities and LS were significantly reduced at t and t. In the Pd Group, only TDI velocities in the left ventricle were reduced at t, but not at t. Dilution of propofol may minimize myocardial dysfunction while maintaining adequate sedation in infants. Further comparative studies are needed to investigate the safety and efficacy of this approach

Current Strategies to Optimize Nutrition and Growth in Newborns and Infants with Congenital Heart Disease: A Narrative Review

(1) Objective: This review aims to identify the clinical and practical barriers to optimizing nutrition in newborn infants with congenital heart disease (CHD) and to describe updated evidence-based recommendations for clinical and nutritional management of these patients in a narrative review. (2) Research Methods and Procedures: We conducted a search of the relevant literature published from 2000 to December 2021. (3) Results: CHD patients undergo several nutritional challenges related to the underlying cardiac disease anomaly, the potential increased risk of NEC, and delayed enteral feeding, resulting in inadequate energy intake and sub-optimal growth, increased morbidity and mortality. (4) Conclusions: To optimize nutrition and growth in newborn infants with CHD, standardized protocols should be implemented. Regular nutritional and growth assessment with a multi-disciplinary team is essential. We propose a decisional algorithm that may represent a potentially useful tool to guide clinicians to optimize growth and nutrition

Current Strategies to Optimize Nutrition and Growth in Newborns and Infants with Congenital Heart Disease: A Narrative Review

(1) Objective: This review aims to identify the clinical and practical barriers to optimizing nutrition in newborn infants with congenital heart disease (CHD) and to describe updated evidence-based recommendations for clinical and nutritional management of these patients in a narrative review. (2) Research Methods and Procedures: We conducted a search of the relevant literature published from 2000 to December 2021. (3) Results: CHD patients undergo several nutritional challenges related to the underlying cardiac disease anomaly, the potential increased risk of NEC, and delayed enteral feeding, resulting in inadequate energy intake and sub-optimal growth, increased morbidity and mortality. (4) Conclusions: To optimize nutrition and growth in newborn infants with CHD, standardized protocols should be implemented. Regular nutritional and growth assessment with a multi-disciplinary team is essential. We propose a decisional algorithm that may represent a potentially useful tool to guide clinicians to optimize growth and nutrition