Right ventricular pressure-volume (PV) analysis characterizes ventricular systolic and diastolic properties independent of loading conditions like volume status and afterload. While long-considered the gold-standard method for quantifying myocardial chamber performance, it was traditionally only performed in highly specialized research settings. With recent advances in catheter technology and more sophisticated approaches to analyze PV data, it is now more commonly used in a variety of clinical and research settings. Herein, we review the basic techniques for PV loop measurement, analysis, and interpretation with the aim of providing readers with a deeper understanding of the strengths and limitations of PV analysis. In the second half of the review, we detail key scenarios in which right ventricular PV analysis has influenced our understanding of clinically relevant topics and where the technique can be applied to resolve additional areas of uncertainty. All told, PV analysis has an important role in advancing our understanding of right ventricular physiology and its contribution to cardiovascular function in health and disease.

Background: Coronary artery fistulas (CAFs) presenting in infancy are rare, and data regarding postclosure sequelae and follow-up are limited. Methods: A retrospective review of all the neonates and infants (<1 year) was conducted from the CAF registry for CAF treatment. The CAF type (proximal or distal), size, treatment method, and follow-up angiography were reviewed to assess outcomes and coronary remodeling. Results: Forty-eight patients were included from 20 centers. Of these, 30 were proximal and 18 had distal CAF; 39 were large, 7 medium, and 2 had small CAF. The median age and weight was 0.16 years (0.01–1) and 4.2 kg (1.7–10.6). Heart failure was noted in 28 of 48 (58%) patients. Transcatheter closure was performed in 24, surgical closure in 18, and 6 were observed medically. Procedural success was 92% and 94 % for transcatheter closure and surgical closure, respectively. Follow-up data were obtained in 34 of 48 (70%) at a median of 2.9 (0.1–18) years. Angiography to assess remodeling was available in 20 of 48 (41%). I. Optimal remodeling (n=10, 7 proximal and 3 distal CAF). II. Suboptimal remodeling (n=7) included (A) symptomatic coronary thrombosis (n=2, distal CAF), (B) asymptomatic coronary thrombosis (n=3, 1 proximal and 2 distal CAF), and (C) partial thrombosis with residual cul-de-sac (n=1, proximal CAF) and vessel irregularity with stenosis (n=1, distal CAF). Finally, (III) persistent coronary artery dilation (n=4). Antiplatelets and anticoagulation were used in 31 and 7 patients post-closure, respectively. Overall, 7 of 10 (70%) with proximal CAF had optimal remodeling, but 5 of 11 (45%) with distal CAF had suboptimal remodeling. Only 1 of 7 patients with suboptimal remodeling were on anticoagulation. Conclusions: Neonates/infants with hemodynamically significant CAF can be treated by transcatheter or surgical closure with excellent procedural success. Patients with distal CAF are at higher risk for suboptimal remodeling. Postclosure anticoagulation and follow-up coronary anatomic evaluation are warranted.

Background: Congenital heart disease (CHD) is the most common birth defect, affecting 40,000 births annually in the United States. Despite advances in medical care, CHD is often a chronic condition requiring continuous management and education. Effective care management depends on children’s understanding of their condition. This highlights the need for targeted health educational interventions to enhance health literacy among children with CHD. Objective: This scoping review aims to map and explore existing health educational interventions for children with CHD. The review identifies the types of interventions, target populations, delivery methods, and assessed outcomes. The goal is to consolidate fragmented research, identify gaps, and establish future research agendas. Methods: Comprehensive searches were conducted in February 2024 using the PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews) framework across multiple databases: APA PsycINFO, MedlinePlus via Ovid, Web of Science, ACM Digital Library, Scopus, and EBSCOhost (CINAHL Complete, CINAHL Ultimate, Health Source: Nursing/Academic Edition, and ERIC). The search covered health care, design, and human-computer interaction disciplines to capture the interdisciplinary nature of CHD health educational interventions. There was no predefined time limit due to the limited number of relevant studies. Eligible studies were in English, published in peer-reviewed journals, and focused on primary data about educational health interventions for children with CHD. We extracted and synthesized data using thematic analysis. Results: The review identified 11 studies: 9 randomized controlled trials and 2 observational studies. These used 6 educational strategies: 3D patient-specific models (n=3), habit formation interventions (n=2), empowerment-based health education programs (n=2), rehabilitation interventions (n=2), web-based portals (n=1), and videotape presentations (n=1). Interventions ranged from brief outpatient sessions to 1.5-year programs, with follow-up from none to 24 months. Studies aimed to improve coping, self-management, and knowledge for children with CHD and their families. The most frequently used assessment method was the independent samples t test (n=4) for pre- and postassessments, and all 11 studies used questionnaires, 8 of which incorporated qualitative feedback. The target participants for these interventions were children aged 13 years and older (n=3), parents (n=2), and children of various ages and their parents (n=6). Outcomes included improved children’s health literacy, reduced parental burden, and increased health care provider efficiency. Conclusions: This review underscores the critical need for tailored educational interventions for children with CHD. Current research mainly focuses on adolescents and relies heavily on parental involvement, possibly overlooking the specific needs of younger children younger than 13 years of age. It is essential to develop engaging, age-appropriate interventions that actively involve children with CHD in their health care journey. Effective health educational interventions are crucial in empowering these young patients and improving their long-term health outcomes.

Cyanosis is defined by bluish discoloration of the skin and mucosa. It is a clinical manifestation of desaturation of arterial or capillary blood and may indicate serious hemodynamic abnormality. The goal of this article is to help the reader understand the etiology and pathophysiology of cyanosis and to formulate an approach to its differential diagnosis. [ Pediatr Ann. 2015;44(2):76–80.]