Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary 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

Invasive assessment of haemodynamics (ventricular, pulmonary) and testing of acute vasoreactivity in the catheterisation laboratory remain the gold standard for the diagnosis of pulmonary hypertension (PH) and pulmonary hypertensive vascular disease. However, these measurements and the interpretation thereof are challenging due to the heterogeneous aetiology of PH in childhood and potentially confounding factors in the catheterisation laboratory. Patients with pulmonary arterial hypertension (PAH) associated with congenital heart disease who have a cardiovascular shunt need to undergo a completely different catheterisation approach than those with idiopathic PAH lacking an anatomical cardiovascular defect. Diagnostic cardiac catheterisation of children with suspected PH usually includes right and left heart catheterisation, particularly for the initial assessment (ie, at the time of diagnosis), and should be performed in experienced centres only. Here, we present graded consensus recommendations for the invasive evaluation of children with PH including those with pulmonary hypertensive vascular disease and/or ventricular dysfunction. Based on the limited published studies and our own experience we suggest a structured catheterisation protocol and two separate definitions of positive acute vasoreactivity testing (AVT): (1) AVT to assess prognosis and indication for specific PH therapy, and (2) AVT to assess operability of PAH

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

“…The full assessment of invasive haemodynamics (right and left heart catheterisation), including ventricular performance and advanced non-invasive imaging, should be performed prior to the initiation of therapy by means of echocardiography,71 cardiac catheterization72 and cardiac MRI 73…”

“…Due to the important therapeutic implications, the complexity and specific risks and benefits of the procedures, separate articles in this issue are dedicated to cMRI73 and cardiac catheterisation72 including acute vasodilator testing 34–39…”

“…If PH/PHVD is severe and the patient presents severely ill in overt heart failure and/or pulmonary vascular crisis, cardiac catheterisation may be postponed due to its inherent risks and pharmacotherapy including intravenous prostanoids started immediately 72 75. Assessment may be postponed until the patient is clinically stabilised.…”

Left Ventricular Diastolic Dysfunction in Pediatric Pulmonary Hypertension

“…Of note, all of the PH patients had been intubated and ventilated during cardiac catheterization and echocardiographyan approach that is not followed by many PH centers, and is not recommended by the European Pediatric Pulmonary Vascular Disease Network in most instances, 15 because of the artificial hemodynamics and the risk of cardiovascular collapse with general anesthesia in PH patients. 16 The lack of both a standardized anesthesia protocol and a complete left heart hemodynamics (PAWP but not always left atrial pressure, LV enddiastolic pressure) may have had impact on the data gathered, but anesthesia probably have influenced both invasive and noninvasive data in a similar way.…”

“…15 Although the definite diagnosis of PH and PHVD is currently made by cardiac catheterization, 15,16 magnetic resonance imaging and chest computer tomography have become essential noninvasive imaging modalities in the management of PH. 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.…”