A modified noninvasive screening protocol for pulmonary hypertension in children with sickle cell disease-Who should be sent for invasive evaluation?

BACKGROUND: Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patients with sickle cell disease (SCD) tend to be overestimated if based exclusively on Doppler-derived tricuspid regurgitant velocity (TRV) as surrogate noninvasive marker with a cutoff ≥2.5 m/s. OBJECTIVES: We aimed to better define a subgroup of pediatric SCD patients who should be sent for invasive evaluation of pulmonary artery pressure (PAP) based on a modified echocardiographic PH screening protocol that implements evidence from Doppler-catheter comparative studies. STUDY DESIGN: Charts of 121 pediatric patients with stable SCD were reviewed regarding echocardiographically assessed risk for elevated PAP/PH and associated clinical characteristics. TRV cutoff was refined at ≥2.9 m/s to avoid overestimating the risk for PH. TRV was combined with additional echocardiographic parameters to avoid underestimating the PH risk. RESULTS: Ninety-one patients qualified for analysis. Based on our modified echocardiographic protocol, 5.5% of patients qualified for at least moderate risk for elevated PAP (compatible with PH) as opposed to 20.9% if based exclusively on TRV ≥2.5 m/s. These patients were older, homozygous for hemoglobin S (HbSS), and more anemic. No subject had an echocardiographic risk constellation suggesting more than mild PH. CONCLUSIONS: Our modified noninvasive screening protocol-if confirmed by invasive studies-may help to better identify a subgroup of pediatric SCD patients in whom evaluation by catheterization appears justified. Unlike estimates based on the conventional protocol, the size of the targeted subgroup compares favorably with catheterization-confirmed PH prevalence rates. Characteristics associated with an increased PH risk were also identified.

Impact of prenatal opioids on cardiac and autonomic development: systematic review and meta-analysis.

Prenatal opioid exposure has recently risen four-fold with limited data on the developmental effects on neonatal physiology. The objective of this systematic review is to develop an association between prenatal opioid exposure and fetal and neonatal cardiac and autonomic development and function. The review was conducted in accordance with PRISMA Guidelines, and searches were conducted using PubMed, Embase, CINAHL, and Web of Science between May 25 and October 27, 2020. Twenty studies fit inclusion criteria, in four categories: (1) fetal cardiac outcomes, (2) neonatal cardiac outcomes, (3) noninvasive autonomic outcomes, and (4) clinical and behavioral measures. For the meta-analysis, three studies (total of 210 subjects) were included. Effect sizes were measured as the mean difference in fetal heart rate between opioid-exposed and non-exposed groups. Mothers with prenatal opioid use had a significantly lower fetal heart rate as compared to mothers without prenatal opioid use, requiring further studies to determine clinical significance.

An international multicenter study comparing arrhythmia prevalence between the intracardiac lateral tunnel and the extracardiac conduit type of Fontan operations.

OBJECTIVE: The study objective was to determine whether the extracardiac conduit Fontan confers an arrhythmia advantage over the intracardiac lateral tunnel Fontan. METHODS: This multicenter study of 1271 patients compared bradyarrhythmia (defined as need for pacing) and tachyarrhythmia (defined as needing antiarrhythmic therapy) between 602 patients undergoing the intracardiac Fontan and 669 patients undergoing the extracardiac Fontan. The median age at the time of the Fontan procedure was 2.1 years (interquartile range, 1.6-3.2 years) for the intracardiac group and 3.0 years (interquartile range, 2.4-3.9) for the extracardiac group (P < .0001). The median follow-up was 9.2 years (interquartile range, 5-12.8) for the intracardiac group and 4.7 years (interquartile range, 2.8-7.7) for the extracardiac group (P < .0001). RESULTS: Early postoperative (<30 days) bradyarrhythmia occurred in 24 patients (4%) in the intracardiac group and 73 patients (11%) in the extracardiac group (P < .0001). Early postoperative (<30 days) tachyarrhythmia occurred in 32 patients (5%) in the intracardiac group and 53 patients (8%) in the extracardiac group (P = not significant). Late (>30 days) bradyarrhythmia occurred in 105 patients (18%) in the intracardiac group and 63 patients (9%) in the extracardiac group (P < .0001). Late (>30 days) tachyarrhythmia occurred in 58 patients (10%) in the intracardiac group and 23 patients (3%) in the extracardiac group (P < .0001). By multivariate analysis factoring time since surgery, more patients in the extracardiac group had early bradycardia (odds ratio, 2.9; 95% confidence interval, 1.8-4.6), with no difference in early tachycardia, late bradycardia, or late tachycardia. CONCLUSIONS: Overall arrhythmia burden is similar between the 2 groups, but the extracardiac Fontan group had a higher incidence of early bradyarrhythmias. There was no difference in the incidence of late tachyarrhythmias over time between the 2 operations. Therefore, the type of Fontan performed should be based on factors other than an anticipated reduction in arrhythmia burden from the extracardiac conduit.

Sudden cardiac death in children and adolescents (excluding Sudden Infant Death Syndrome).

Sudden death in the young is rare. About 25% of cases occur during sports. Most young people with sudden cardiac death (SCD) have underlying heart disease, with hypertrophic cardiomyopathy and coronary artery anomalies being commonest in most series. Arrhythmogenic right ventricular dysplasia and long QT syndrome are the most common primary arrhythmic causes of SCD. It is estimated that early cardiopulmonary resuscitation and widespread availability of automatic external defibrillators could prevent about a quarter of pediatric sudden deaths.