Diagnostics, monitoring and outpatient care in children with suspected pulmonary hypertension/paediatric pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK

Abstract

Pulmonary hypertension (PH) is a condition of multiple aetiologies with underestimated prevalence and incidence. Indeed, despite access to modern therapies, pulmonary hypertensive vascular disease (PHVD) remains a progressive, usually life-limiting condition, severely impacting on the patients' well-being. We herein provide practical, expert consensus recommendations on the initial diagnostic work-up, clinical management and follow-up of children and adolescents with PH/PHVD, including a diagnostic algorithm. The major topics and methods that need to be tailored and put into context of the individual patient include PH classification, clinical signs and symptoms, basic diagnostic and advanced imaging measures (ECG, chest X-ray, transthoracic echocardiography, cardiac magnetic resonance, chest CT angiography, cardiac catheterisation, ventilationperfusion lung scan, abdominal ultrasound), lung function tests, 6 min walk and cardiopulmonary exercise testing, sleep study ( polysomnography), laboratory/ immunological tests, considerations for elective surgery/ general anaesthesia, physical education and exercise, flying on commercial airplanes, vaccinations, care of central intravenous lines and palliative care. Due to the complexity of PH/PHVD, the clinical care has to be multidisciplinary and coordinated by a dedicated specialist paediatric PH centre, not only to decrease mortality but to allow children with PH/PHVD to reach a reasonable quality of life.

Genetic testing and blood biomarkers in paediatric pulmonary hypertension. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK

“…Emerging evidence is accumulating supporting the use of genetic testing and blood biomarkers in paediatric PAH to individualise diagnostics,32 prognostics and therapy 33. In addition, genetic testing supports efficient risk stratification in family members.…”

Left Ventricular Diastolic Dysfunction in Pediatric Pulmonary Hypertension

“…17 However, the first and most frequently applied diagnostic test in suspected PH-beyond history taking, clinical examination, ECG, chest x-ray, blood plasma/serum N-terminal prohormone of brain natriuretic peptide-is the transthoracic echocardiogram. 3,5 Echocardiography in children usually allows a comprehensive assessment of the cardiovascular anatomy; it may also confirm RV or PA pressure elevation by Doppler interrogation of more than trivial tricuspid and pulmonary regurgitation and analysis of the end-systolic shape of the interventricular septum. 18 Nevertheless, over-reliance on a single echocardiographic variable and false estimation of RV systolic pressure because of poor CW-Doppler envelopes or severe tricuspid regurgitation can limit the value of the methodology for non-PH experts.…”

“…5,13 The term pediatric PAH (ie, group 1 PH) defines a subgroup of precapillary PH with an end-expiratory pulmonary artery wedge pressure (PAWP) <15 mm Hg and a PVR indexed to body surface area >3 WU m 2 . In 2011, the Pulmonary Vascular Research Institute introduced the disease entity pediatric PHVD (mean PA pressure ≥25 mm Hg; PVR index >3 WU m 2 ) that was divided into 10 main categories (Panama Classification, 2011).…”

“…1,2 Meanwhile, consensus statements have been developed in North America and Europe specifically to guide the clinical care of children with pulmonary hypertension (PH). 3,4 The challenges in the diagnosis 5 and treatment 6 of pediatric PH include early detection in the absence of specific symptoms or biomarkers, the complexity of the underlying etiologies, broad comorbidities, genetic syndromes, and the paucity of pediatric trial data to support and guide clinical management.…”

Heartbeat: Imaging complex vascular anatomy in congenital heart disease

“…An entire supplement to Heart 1 – 3) provides several articles with detailed reviews and consensus statements for management of pediatric pulmonary hypertension. A novel approach to management of pediatric patients with refractory pulmonary hypertension is creation of a shunt from the descending aorta to the left pulmonary artery to unload the suprasystemic pulmonary vascular circuit by shunting into the systemic circulation, often called a “reverse Potts shunt” because the flow direction is the opposite of the shunt created to increase pulmonary blood flow in other types of congenital heart disease.…”