Volume 15: Issue 2

Making Strides for Pediatric Pulmonary Arterial Hypertension

The evaluation and treatment of pulmonary arterial hypertension (PAH) in children represents distinct challenges. In this issue of Advances, Drs Dunbar Ivy and Usha Krishnan have done a fantastic job guest editing in a fashion that complements recently published guidelines for this important population(1) The Advances issue, along with the pediatric guidelines, offers a more comprehensive review than is typically available in more conventional efforts.(2)

Amazingly, no FDA-approved pharmacological therapies are available for PAH in children. Nonetheless, dedicated clinicians and scientists have forged ahead relying on

Pulmonary hypertension (PH) is a complex pulmonary vascular disease of multiple etiologies, characterized by inappropriate elevation of pulmonary vascular pressure and resistance that can present at any age, including during childhood. The pharmacologic care of patients with PH has seen tremendous advances in the past 20 years, improving survival and overall quality of life for patients of all ages, although mortality remains high.1 While the care of children with PH involves the same PH-specific medicines used for adults, PH-specific drugs are employed off-label due to a lack of Federal Drug Administration approval, which adds to the complexity of administration.2 Furthermore, in addition to pharmacologic therapy, multiple other factors are involved in the care of the pediatric PH patient that require careful consideration, such as differences relating to growth and developmental stages and according to PH etiology. This review will discuss some of the highly relevant pediatric-related issues outside of traditional pharmacologic factors, with a focus on the importance of a collaborative approach to care, genetic testing, and palliative care.

As with any rare disease, a low prevalence in the population is a setup for missed diagnosis. Pulmonary hypertension (PH) can occur at any age during childhood and like in adults, a delay in diagnosis is common.1 Historical outcomes for idiopathic pulmonary arterial hypertension are worse than adults, with a mean survival of 10 months after diagnosis.2 This highlights the need for accurate workup for a potential pediatric PH diagnosis. Generally the evaluation process has paralleled the adult guidelines.3 4 However, adult guidelines lack evidence-based support in pediatrics.5 Recently the American Heart Association and American Thoracic Society created guidelines for the diagnosis and management of pediatric PH.6 The guidelines offer a diagnostic algorithm needed to verify the diagnosis and work up potential etiologies (Figure 1). They provide the best evidence-based support for the workup and rationale involved. However, the guidelines are still hindered in that the majority of the evidence to support them is Level B (data derived from limited populations)6 (Table 1). This review will discuss some of the diagnostic challenges in pediatric PH.

Perioperative management of patients with pulmonary hypertension or those at risk for increased pulmonary vascular reactivity should focus on supporting the patient through the vulnerable period of physiologic derangements surrounding surgery, including acute alterations in pulmonary blood flow, altered pulmonary endothelial function following cardiopulmonary bypass, invasive mechanical ventilation, and adaptation to new hemodynamics following correction or palliation of congenital heart disease lesions. These patients require careful attention to each step of perioperative management by teams experienced in the care of pediatric patients with pulmonary hypertension. This article will focus on preoperative evaluation, pulmonary hypertensive crises, general principles of perioperative management, and specific pulmonary vasodilator therapies.

Guest Editor's Memo

Editor's Memo

Making Strides for Pediatric Pulmonary Arterial Hypertension

Advances and Aldrighetti: An Indomitable Team

PH Professional Network: Dental Care in Children With Pulmonary Hypertension

General Approach to the Care of the Child With Pulmonary Hypertension

Diagnostic Challenges in Pediatric Pulmonary Hypertension

Therapeutic Options for Childhood Pulmonary Hypertension

Perioperative Management of Pediatric Pulmonary Hypertension

Pulmonary Vascular Disease in Bronchopulmonary Dysplasia

PH Roundtable: Pediatric Pulmonary Hypertension Guidelines Highlights and Challenges

PH Grand Rounds: Persistent Pulmonary Artery Filling Defects: Looking Beyond Anticoagulation Failure

Erratum

Living with PH: Progress in Pediatric Care

Conference Abstracts

Guest Editor’s Memo

A Roundtable Discussion Addressing Patient-Centered Care and Quality of Life in Patients With Pulmonary Arterial Hypertension

Editor’s Memo

Pulmonary Hypertension Associated With Parenchymal Lung Disease