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.

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.

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.

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.

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.

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.

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.