Pulmonary hypertension (PHTN) is a common pathology in pediatrics, arising from a diverse array of etiologies and manifesting in equally diverse patient populations. The inpatient management of these infants and children may be complicated by dynamic and at times severe increases in pulmonary vascular resistance (PVR) and right ventricular (RV) afterload. Yet absent are cognitively accessible heuristics in the field whereby providers can reconcile the various clinical manifestations they observe with an understanding of the cardiac physiology at play, and therefore, appropriate physiology-driven interventions. Described herein is a framework for understanding the pathophysiology of four clinical phenotypes which are driven by two echocardiographic patient characteristics: the presence or absence of an atrial communication and the capacity of the right ventricle to maintain ventricular-vascular coupling. Application of this paradigm may facilitate accurate interpretation of observed clinical data, and alignment of treatment strategies with the underlying pathophysiology.
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Obstetrics and Gynecology