Serial tissue Doppler imaging in the evaluation of bronchopulmonary dysplasia-associated pulmonary hypertension among extremely preterm infants: a prospective observational study.

Objectives: To evaluate serial tissue Doppler cardiac imaging (TDI) in the evolution of bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) among extremely preterm infants. Design: Prospective observational study. Setting: Single-center, tertiary-level neonatal intensive care unit. Patients: Infant born <28 weeks gestation. Main outcome measures: Utility of TDI in the early diagnosis and prediction of BPD-PH and optimal timing for screening of BPD-PH. Results: A total of 79 infants were included. Of them, 17 (23%) had BPD-PH. The mean gestational age was 25.9 ± 1.1 weeks, and mean birth weight was 830 ± 174 g. The BPD-PH group had a high incidence of hemodynamically significant patent ductus arteriosus (83% vs. 56%, p < 0.018), longer oxygen days (96.16 ± 68.09 vs. 59.35 ± 52.1, p < 0.008), and prolonged hospital stay (133.8 ± 45.9 vs. 106.5 ± 37.9 days, p < 0.005). The left ventricular eccentricity index (0.99 ± 0.1 vs. 1.1 ± 0.7, p < 0.01) and the ratio of acceleration time to right ventricular ejection time showed a statistically significant trend from 33 weeks (0.24 ± 0.05 vs. 0.28 ± 0.05, p < 0.05). At 33 weeks, the BPD-PH group showed prolonged isovolumetric contraction time (27.84 ± 5.5 vs. 22.77 ± 4, p < 0.001), prolonged isovolumetric relaxation time (40.3 ± 7.1 vs. 34.9 ± 5.3, p < 0.003), and abnormal myocardial performance index (0.39 ± 0.05 vs. 0.32 ± 0.03, p < 0.001). These differences persisted at 36 weeks after conceptional gestational age. Conclusions: TDI parameters are sensitive in the early evolution of BPD-PH. Diagnostic accuracy can be increased by combining the TDI parameters with conventional echocardiographic parameters. BPD-PH can be recognizable as early as 33-34 weeks of gestation.

Risk Factors Predicting the Need for Phototherapy in Glucose 6 Phosphate Dehydrogenase-Deficient Infants in a Large Retrospective Cohort Study.

INTRODUCTION: Glucose 6-phosphate dehydrogenase (G6PD) deficiency increases the risk of severe neonatal hyperbilirubinemia. This study evaluates the risk factors predicting the need for phototherapy in G6PD-deficient neonates after 72 h of age and assesses the safety of early discharge. METHODS: A retrospective cohort study of 681 full-term G6PD-deficient infants with a birth weight ≥2,500 g over 4 years was conducted. We compared the baseline characteristics, bilirubin level on day 4 (after 72 h of life), day of peak bilirubin, G6PD levels, and concomitant ABO incompatibility between the group that required phototherapy (Group A) and those who did not (Group B). RESULTS: 396 infants (58%), predominantly males, required phototherapy in the first week of life. The infants who required phototherapy had a lower median gestational age (38.3 vs. 38.7 weeks, p < 0.01) and had lower G6PD levels (2.3 ± 2.5 vs. 3 ± 3.4 IU, p < 0.05) compared to the controls. The mean day-four total serum bilirubin (TSB) levels were higher (213 ± 32 vs. 151 ± 37 µmol/L, p < 0.01), with bilirubin level peaking earlier (3 vs. 4 days of life, p < 0.01) in group A. Regression analysis identified TSB levels on day 4, Chinese race, lower gestation, and concomitant ABO incompatibility as the significant predictors for the need for phototherapy in the study population. In particular, coexisting ABO blood group incompatibility increased the risk of jaundice requiring phototherapy (OR 4.27, 95% CI: 1.98-121, p < 0.01). Day four TSB values above 180 µmol/L predicted the need for phototherapy with 86% sensitivity and 80% specificity. The findings were similar across both male and female infants with G6PD deficiency. CONCLUSION: G6PD-deficient infants with day four TSB levels of >180 µmol/L (10.5 mg/dL) and associated ABO blood group incompatibility have a higher risk of requiring phototherapy in the first week of life and should be closely monitored.

Complete Heart Block Secondary to Flecainide Toxicity: Is It Time for CYP2D6 Genotype Testing?

Flecainide acetate is a Vaughan-Williams class IC antiarrhythmic drug prescribed for the treatment of supraventricular arrhythmias. It has a narrow therapeutic index and proarrhythmic effects even at therapeutic doses. Flecainide is metabolized by a CYP2D6 enzyme that exhibits polymorphism. In this case report, we present, to our best knowledge, the first case of toxicity contributed by genetic polymorphism in an infant. Our patient with recurrent supraventricular tachycardia was treated with a therapeutic dose of flecainide but developed heart block requiring extracorporeal membrane oxygenation support and subsequent treatment with lipid emulsion therapy. He was found to have supratherapeutic serum flecainide concentration, and gene testing revealed the patient to be an intermediate metabolizer. With this case report, we reinforce the importance of evaluating the CYP2D6 genotype before drug initiation in the neonatal population and recommend regular monitoring of serum flecainide levels and electrocardiograms in these patients.

Selective Treatment of PDA in High-Risk VLBW Infants With Birth Weight ≤800 g or <27 Weeks and Short-Term Outcome: A Cohort Study.

Background: Patent ductus arteriosus (PDA) causing significant left to right shunt can increase key morbidities in preterm infants. Yet, treatment does not improve outcomes and spontaneous closure is the natural course of PDA. The Impact of PDA on 23-26-week gestation infants is uncertain. Selective treatment of such infants would likely balance outcomes. Objective: To test the hypothesis that treatment of PDA in high-risk VLBW infants [birth weight ≤800 g or gestation <27 weeks, hemodynamically significant, ductal diameter (DD, ≥1.6 mm), and mechanical ventilation] and expectant management in low-risk infants will reduce the need for treatment and surgical ligation, without altering short term morbidities. Methods: This prospective observational study was initiated subsequent to the introduction of a new treatment protocol in 2016. The 12-months before and after protocol introduction were, respectively, defined as standard and early selective treatment periods. In the early selective treatment cohort, PDA was treated with indomethacin, maximum of two courses, 1 week apart. Surgical ligation was considered after 30 days of age if indicated (DD ≥2 mm, mechanical ventilation). Primary outcomes were need for treatment and rate of ligation. Protocol compliance and secondary outcomes were documented. Results: 415 infants were studied, 202 and 213 in the standard treatment and early selective treatment cohorts, respectively. Numbers treated (per protocol) in the standard treatment and early selective treatment cohorts were 27.7 and 19.3% (56/202 and 41/213) (p = 0.049), and the respective ligation rates were 7.54 and 2.96% (P = 0.045). Secondary outcomes were comparable. Conclusion: The early selective treatment protocol reduced the rates of treatment and surgical ligation of PDA, without altering key morbidities. Further studies under a randomized control trial setting is warranted.

Congenital diaphragmatic hernia associated with oesophageal atresia and tracheo-oesophageal fistula in a low birth weight infant.

The cooccurrence of congenital diaphragmatic hernia and oesophageal atresia with distal tracheo-oesophageal fistula is very rare and carries high mortality. Very few anecdotal case reports and one case series have been reported in the literature. We report a case of a late preterm, low birth weight infant with this rare association who was successfully managed by staged surgical approach and had good outcome.

Catastrophic antiphospholipid syndrome in childhood: presentation with an inferior caval vein mass.

Venous thromboembolism remains one of the important causes of pulmonary hypertension in children. The causes of venous thromboembolism are varied and include antiphospholipid antibody syndrome (APS). Catastrophic APS (CAPS) is a severe variant of APS characterised by disseminated thrombosis and microangiopathy resulting in multiorgan failure. CAPS can occur independently (primary APS) but mainly occurs in association with systemic lupus erythematosus including the paediatric population. We report a young girl with primary CAPS who presented with pulmonary hypertension and an inferior vena cava mass masquerading as a tumour.