Uncommon Cause of Chest Pain in a 9-Year-Old Boy with Crohn’s Disease

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Think out of the box: association of left congenital diaphragmatic hernia and abnormal origin of the right pulmonary artery

Abstract Background We report the occurrence of a severe pulmonary hypertension (PH) in a neonate affected by a left congenital diaphragmatic hernia (CDH). PH in this patient was associated with an abnormal origin of the right pulmonary artery from the right brachiocephalic artery. This malformation, sometimes named hemitruncus arteriosus, has to the best of our knowledge never been reported in association with a CDH. Case presentation A male newborn was hospitalized from birth in the neonatal intensive care unit after prenatal diagnosis of a left CDH. Ultrasound examination at 34 weeks of gestational age evaluated the observed-to-expected lung-to-head ratio at 49%. Birth occurred at 38 + 5 weeks of gestational age. Soon after admission, severe hypoxemia, i.e., preductal pulse oximetry oxygen saturation (SpO 2 ) < 80%, prompted therapeutic escalation including the use of high frequency oscillatory ventilation with fraction of inspired oxygen (FiO 2 ) 100% and inhaled nitric oxide (iNO). Echocardiography assessment revealed signs of severe PH and normal right ventricle function. Despite administration of epoprostenolol, milrinone, norepinephrine, and fluid loadings with albumin and 0.9% saline, hypoxemia remained severe, preductal SpO 2 inconsistently greater than or equal to 80-85% and post ductal SpO 2 lower on average by 15 points. This clinical status remained unchanged during the first 7 days of life. The infant’s clinical instability was incompatible with surgical intervention, while chest X-ray showed a relatively preserved lung volume, especially on the right side. This prompted an additional echocardiography, aimed at searching an explanation of this unusual evolution and found an abnormal origin of the right pulmonary artery, which was confirmed on computed tomography angiography subsequently. A change in the medical strategy was decided, with the suspension of pulmonary vasodilator treatments, the administration of diuretics, and the decrease in norepinephrine dose to decrease the systemic-to-pulmonary shunt. Progressive improvement in the infant respiratory and hemodynamic status enabled to perform CDH surgical repair 2 weeks after birth. Conclusions This case recalls the interest of systematic analysis of all potential causes of PH in a neonate with CDH, a condition frequently associated with various congenital malformations

Isolated Ventricular Inversion : A Rare Complex Congenital Heart Disease With Neonatal Cyanosis

International audienceA 1-day-old girl was referred for a cardiology consultation for a mean saturation at 80% without respiratory distress. Echocardiography showed an isolated ventricular inversion. This entity is extremely rare, with fewer than 20 cases reported. This case report describes the clinical evolution and the complex surgical management of this pathology. (Level of Difficulty: Advanced.

Réadaptation cardiaque de l’enfant et l’adulte avec une cardiopathie congénitale

International audienceAdvances in heart surgery over the past 30 years have significantly improved the prognosis of congenital heart diseases (CHD). Therefore, the epidemiology of CHD has changed dramatically with a shift of mortality from pediatrics to adulthood and an increased prevalence of complex CHD. Today, caregivers and patients focus their interests to new perspectives: improving the quality of life, practicing sports, improving psychosocial care. Cardiac rehabilitation is completely integrated in these new therapeutic strategies. The starting point is the cardiopulmonary exercise test (CPET), with the measurement of oxygen uptake, or "VO2". CPET is now recommended in the follow-up of the adults with CHD. Maximum oxygen uptake correlates to the quality of life of children and adults with CHD. The principles of the rehabilitation in patients with heart failure may usually be applied to CHD patients. Some studies in complex CHD showed improvement of VO2 and quality of life after rehabilitation, without any adverse events. However few physicians have the experience in rehabilitation among CHD patients, especially children. Randomized trials on cardiac rehabilitation in adult and pediatric CHD patients are essential to increase the level of evidence and lead to specific guidelines in this population

Atrial septal defect and exercise capacity: value of cardio-pulmonary exercise test in assessment and follow-up

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Use of treprostinil in pediatric pulmonary hypertension

International audienceAdd-on therapy with prostacyclin in pediatric refractory pulmonary hypertension poses a challenge, especially when considering continuous intravenous administration in younger children. A search for alternate routes of drug delivery has led to the clinical investigation of stable and long-acting prostacyclin analogues, such as subcutaneous treprostinil. We reported two pediatric cases of pulmonary hypertension treated with subcutaneous treprostinil and reviewed the literature on treprostinil use in children

The VE/VCO 2 slope: a useful tool to evaluate the physiological status of children with congenital heart disease

International audienceIntroduction Cardio-pulmonary exercise test (CPET) is becoming a key examination to assess physical capacity and disease severity in paediatric cardiology. The VE/VCO2 slope has been increasingly used as a surrogate marker for morbidity and mortality in adult heart failure, pulmonary arterial hypertension and for adult patients with CHD. Nevertheless, the use of the VE/VCO2 slope in children remains limited in the absence of reference values and clearly identified clinical determinants. This study aimed to compare the VE/VCO2 slope in a paediatric cohort with CHD to that of age- and gender-adjustedhealthy controls. We also intended to identify the clinical and CPET variables associated with VE/VCO2 slope in this population. Methods This cross-sectional study was carried out between November 2010 and September 2015 in two tertiary care paediatric cardiology reference centres. Results A total of 700 children were enrolled (399 CHD and 301 healthy controls). The mean VE/VCO2 slope was significantly higher in CHD than in healthy subjects (31.6±4.8 vs. 29.3±4.8; P<0.001). The VE/VCO2 slope was higher in children with significant pulmonary regurgitation, tricuspid regurgitation, right ventricular hypertension and right ventricle outflow tract (RVOT) obstacle. In the CHD group, VE/VCO2 slope increase was associated with BMI, the presence of a RVOT obstacle, the number of cardiac catheter procedures, as well as low age, FVC, tidal volume, and PetCO2. Conclusion Increased VE/VCO2 slope was predominantly in children with single ventricle and/or residual right heart abnormalities suggesting that maldistribution of pulmonary blood flow during exercise is an important CHD-unique determinant of VE/VCO2 slope

Birth during on-call period: Impact of care organization on mortality and morbidity of very premature neonates

International audienceObjectives The evidence that risks of morbidity and mortality are higher when very premature newborns are born during the on-call period is inconsistent. This study aimed to assess the impact of this situation among other determinants of outcomes, particularly newborn characteristics and care organization.Methods Observational study including all infants born < 30 weeks’ gestation in a French tertiary perinatal center between 2007 and 2020. On-call period corresponded to weekdays between 6:30 p.m. and 8:30 a.m., weekends, and public holidays. The primary endpoint was survival without severe morbidity, including grade 3–4 intraventricular hemorrhage (IVH), cystic periventricular leukomalacia, necrotizing enterocolitis, severe bronchopulmonary dysplasia (BPD), and severe retinopathy of prematurity. The relationship between admission and outcome was assessed by an adjusted odds ratio (aOR) on the propensity of being born during on-call period and expressed vs. weekday. Secondary analyses were carried out in extremely preterm newborns (<27 weeks’ gestation), in cases of early death (within 7 days), and before (2007–2013, 51.5% of the cohort) vs. after (2014–2020, 48.5% of the cohort) the implementation of a pediatrician-nurse team dedicated to newborn care in the delivery room.Results A total of 1,064 infants [27.9 (26.3; 28.9) weeks, 947 (760; 1,147) g] were included: 668 during the on-call period (63%) and 396 (37%) on weekdays. For infants born on weekdays, survival without severe morbidity was 54.5% and mortality 19.2%. During on-call, these rates were 57.3% [aOR 1.08 (0.84–1.40)] and 18.4% [aOR 0.93 (0.67–1.29)]. Comparable rates of survival without severe morbidity [aOR 1.42 (0.87–2.34)] or mortality [aOR 0.76 (0.47–1.22)] were observed in extremely preterm infants. The early death rate was 6.4% on weekdays vs. 8.2% during on-call [aOR 1.44 (0.84–2.48)]. Implementation of the dedicated team was associated with decreased rates of mortality [aOR 0.57 (0.38, 0.85)] and grade 3–4 IVH [aOR 0.48 (0.30, 0.75)], and an increased rate of severe BPD [aOR 2.16 (1.37, 3.41)], for infants born during on-call.Conclusion In this cohort, most births of very premature neonates occurred during the on-call period. A team dedicated to newborn care in the delivery room may have a favorable effect on the outcome of infants born in this situation

15‐Year trends in respiratory care of extremely preterm infants: Contributing factors and consequences on health and growth during hospitalization

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