Invasive haemodynamics predict outcomes in paediatric pulmonary artery hypertension.

BACKGROUND: Invasive haemodynamics are often performed for initiating and guiding pulmonary artery hypertension therapy. Little is known about the predictive value of invasive haemodynamic indices for long-term outcomes in children with pulmonary artery hypertension. We aimed to evaluate invasive haemodynamic data to help predict outcomes in paediatric pulmonary artery hypertension. METHODS: Patients with pulmonary artery hypertension who underwent cardiac catheterisation (2006-2019) at a single centre were included. Invasive haemodynamic data from the first cardiac catheterisation and clinical outcomes were reviewed. The combined adverse outcome was defined as pericardial effusion (due to right ventricle failure), creation of a shunt for pulmonary artery hypertension (atrial septal defect or reverse Pott's shunt), lung transplant, or death. RESULTS: Among 46 patients with a median [interquartile range (IQR)] age of 13.2 [4.1-44.7] months, 76% had CHD. Median mean pulmonary artery pressure was 37 [28-52] mmHg and indexed pulmonary vascular resistance was 6.2 [3.6-10] Woods units × m2. Median pulmonary artery pulsatility index was 4.0 [3.0-4.7] and right ventricular stroke work index was 915 [715-1734] mmHg mL/m2. After a median follow-up of 2.4 years, nine patients had a combined adverse outcome (two had a pericardial effusion, one underwent atrial level shunt, one underwent reverse Pott's shunt, and six died). Patients with an adverse outcome had higher systolic and mean pulmonary artery pressures, higher diastolic and transpulmonary pressure gradients, higher indexed pulmonary vascular resistance, higher pulmonary artery elastance, and higher right ventricular stroke work index (p < 0.05 each). CONCLUSION: Invasive haemodynamics (especially mean pulmonary artery pressure and diastolic pressure gradient) obtained at first cardiac catheterisation in children with pulmonary artery hypertension predicts outcomes.

Sinus bradycardia in a toddler with multisystem inflammatory syndrome in children (MIS-C) related to COVID-19.

This report documents a case of sinus bradycardia in a hospitalised 27-month-old girl with a history of moderate persistent asthma, recent suspected viral respiratory infection and suspicion for multisystem inflammatory syndrome in children (MIS-C). This patient developed profound sinus bradycardia during her hospitalisation despite an overall well clinical appearance and good outcome. Reports of bradycardia related to COVID-19 infection are few but growing in number. In this article, we discuss what has been observed in the literature about bradycardia in relation to COVID-19 and MIS-C. We also propose sinus bradycardia as a potential sign of MIS-C with recent respiratory symptoms, which would warrant close follow-up of such patients.

Outcomes of Surgical Repair of Vascular Rings and Slings in Children: A Word for the Asymptomatic.

Vascular rings (VRs) are rare aortic arch anomalies that may present with a wide variety of symptoms related to esophageal and/or airway compression. We reviewed our surgical experience in both symptomatic and asymptomatic children. All children (n = 58) who underwent surgical repair of VRs or slings (mean age 27.4 ± 45.60 months; 36 males [62%]) between March 2000 and April 2020 were included. The most common anatomic variant was a right aortic arch (RAA) with aberrant left subclavian artery (ALSCA) (n = 29; 50%). Kommerell's diverticulum was present in 23 of these patients (79%). The second most common variant was a double aortic arch (n = 22; 38%), followed by pulmonary artery sling (n = 4; 6%), RAA with mirror image branching and left ligamentum arteriosum (n = 3; 5.2%), and left aortic arch (LAA) with aberrant right subclavian artery (n = 1; 1.7%). One patient had a double ring with pulmonary artery sling and RAA with ALSCA. Symptoms were present in 42 patients (72%). Left lateral thoracotomy was the approach in 50 patients (86%), while sternotomy was used in 8 (14%). Symptomatic improvement occurred in the majority of symptomatic patients (93%). There was one perioperative mortality (1.7%) in the symptomatic group which was non-VR related. Morbidities included recurrent laryngeal nerve injury in three patients (5.2%) and transient chylothorax in two (3.4%). Persistence/recurrence of symptoms resulted in one early and one late reoperation. The mean follow-up was 3 ± 5 years. In the current era, VR repair in children including asymptomatic ones can be performed with excellent results. We recommend complete repair of RAA with aberrant LSCA by resection of Kommerell's diverticulum and translocation of the ALSCA to avoid recurrence.

Pediatric SubQ-ICD implantation, a single center review of the inter-muscular technique.

INTRODUCTION: Pediatric patients with cardiomyopathies are at risk for sudden death and may need implantable cardioverter defibrillators (ICD's), but given their small size and duration of use, children are at increased risk for complications associated with ICD use. The subcutaneous ICD presents a favorable option for children without pacing indications. Unfortunately, initial pediatric studies have demonstrated a high complication rate, likely due to the 3-incision technique employed. MATERIAL AND METHODS: Patients with ICD but no pacing indication were retrospectively reviewed after implantation of subcutaneous ICD via the two-incision technique. In half of the patients, 10-J impedance test was also performed to compare with impedance obtained after defibrillation threshold testing with 65-J. RESULTS: Twelve patients were included. The median age was 14 years (range 10-16 years) with eight males included (72.7%). The median weight was 55 kg (range 29 kg-75.1 kg). Follow-up had a median of 11.5 months (range 2-27 months). The median body mass index was 18.4 kg/m squared (range 15.5-27.9 kg/m squared). One patient suffered a minor complication after tearing off the incisional adhesive strips early and required a non-invasive repair in clinic. Shock impedance had a median of 55 J (range 48-68 J). There was one appropriate shock/charge and no inappropriate shocks during follow-up. CONCLUSION: The two-incision, intermuscular technique appears to have a lower acute complication rate than prior reports, in our cohort of 12 pediatric patients.

Feasibility of intraoperative aortic root imaging in congenital heart surgery.

BACKGROUND: Intraoperative vascular imaging is a useful tool to evaluate coronary ostia in congenital heart patients with suboptimal preoperative imaging findings. Additionally, vascular imaging has potential value for visualization of the ascending aortic intima media thickness (IMT) in certain clinical scenarios. This study sought to evaluate the feasibility of intraoperative vascular imaging of coronary ostia and IMT during congenital heart surgery. METHODS: We describe the technique for performance of intraoperative vascular imaging by a pediatric cardiologist using a high-resolution linear sequential array transducer. RESULTS: Intraoperative vascular imaging was obtained on seven patients. Coronary ostia were normal in all except one. This patient had congenital stenosis of the left coronary ostium discovered during intraoperative imaging and confirmed by the surgeon. In another patient with Williams syndrome, the IMT was noted to be prominent. CONCLUSIONS: It is feasible to perform intraoperative vascular imaging in less than 5 minutes of operator's time and provides superior visualization of the coronary ostia and IMT.

Anomalous Aortic Origin of the Right Pulmonary Artery From the Ascending Aorta With Spontaneous Dissection and Thrombosis.

Anomalous aortic origin of the pulmonary artery is rare. It can affect either of the main branches and can be an important cause of neonatal respiratory distress. Early diagnosis and surgical repair is associated with improved survival and long-term outcomes.

Evaluation and Monitoring of Pulmonary Hypertension in Neonates With Congenital Diaphragmatic Hernia.

PURPOSE OF REVIEW: This review aims to describe the assessment of pulmonary hypertension and ventricular function in neonates with congenital diaphragmatic hernia and the long-term follow-up of their pulmonary vascular disease. RECENT FINDINGS: In 2015, the pediatric pulmonary hypertension guidelines from the American Heart Association and American Thoracic Society suggested class I level of evidence B guidelines for routine evaluation of patients with congenital diaphragmatic hernia, including longitudinal care in an interdisciplinary pulmonary hypertension program and following the recommendations offered for all children with pulmonary hypertension. Congenital diaphragmatic hernia causes compression of the lungs during critical stages of fetal development and results in lung hypoplasia. As a result, there is abnormal development of pulmonary vasculature that leads to post-natal pulmonary hypertension and increased afterload to the right ventricle. Left ventricular filling is affected by decreased pre-load and mechanical compression by abdominal content leading to decreased systemic perfusion. Persistent pulmonary hypertension after surgical repair of congenital diaphragmatic hernia is associated with increased mortality. Assessment and monitoring of pulmonary hypertension and ventricular function in this population of neonates is crucial to determine response to medical treatment, the need for extracorporeal membrane oxygenation, and the timing of surgical repair.

Mortality after hematopoietic stem cell transplantation for severe mucopolysaccharidosis type I: the 30-year University of Minnesota experience.

BACKGROUND AND AIM: Mucopolysaccharidosis IH (MPS IH, Hurler syndrome) naturally leads to death within the first decade of life, primarily from cardiac and pulmonary causes. To determine how hematopoietic stem cell transplantation (HSCT) has altered mortality, we analyzed our institution's 30-year experience of patients with MPS IH undergoing HSCT. METHODS: Using chart review and the National Death Index, we determined survival status of 134 patients (males = 69) with MPS IH transplanted between 9/16/1983 and 7/25/2013 on 12/31/2013. Analysis included descriptive statistics, Kaplan-Meier curves, and regression analysis by Cox proportional hazards model. RESULTS: Overall survival (95% CI) at one- and 25-years was 70% (62-78%) and 37% (19-55%), respectively. From 2004 onward, overall survival at one- and 8-years was 84% (73-96%) and 81% (69-94%), respectively, compared to 65% (55-74%) and 57% (47-67%) prior to 2004 (Log-rank p = 0.032). Regardless of era, male survival was significantly better than female (HR 0.40, [95% CI: 0.21-0.74], p = 0.004). The cumulative incidence of death (95% CI) at 25 years was 63% (45-81%); incidence of pulmonary-related death was the highest at 27% (10-41%) compared to 8% (0.3-16%) for cardiac, 12% (6-17%) for infectious disease, and 16% (3-27%) from other complications. CONCLUSIONS: HSCT has increased survival in MPS IH beyond the third decade of life and decreased the incidence of cardiac mortality, but deaths after the third year post-HSCT occur in excess of expected US mortality. It is important to determine if improved transplant strategies since 2004 result in better long-term survival in the current patient population.

A Randomized Controlled Trial of Glycerin Suppositories During Phototherapy in Premature Neonates.

OBJECTIVE: To determine if glycerin suppositories were effective in reducing total duration of phototherapy in premature neonates. We hypothesized that glycerin suppositories would have no effect on phototherapy duration or total serum bilirubin levels. DESIGN: Prospective randomized controlled double-blinded trial. SETTING: Level IV NICU. PARTICIPANTS: Neonates born between 30 weeks, 0 days and 34 weeks, 6 days gestational age who developed physiologic hyperbilirubinemia needing phototherapy. METHODS: Neonates were randomized to the no-suppository group or to the suppository group. Neonates were randomized to receive glycerin suppositories every 8 hours while under phototherapy or to a sham group. The primary outcome was total hours of phototherapy. Secondary outcomes included peak total serum bilirubin levels, time from start to discontinuation of phototherapy, rate of decline in bilirubin levels, repeat episodes of phototherapy, and number of stools while the neonates received phototherapy. RESULTS: A total of 39 neonates were assigned to the no-suppository group and 40 to the suppository group. Withholding suppositories was not inferior to providing suppositories. The total hours of phototherapy were not longer (i.e., noninferior) among neonates not provided suppositories (61 ± 53 hours) than among those given suppositories (72 ± 49 hours). There were no differences in peak bilirubin levels, rate of bilirubin decline, or repeat episodes of phototherapy. CONCLUSION: Routine use of glycerin suppositories among preterm neonates who receive phototherapy does not affect bilirubin levels or phototherapy duration.