Pulmonary hypertension in the intensive care unit. 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

Acute pulmonary hypertension (PH) complicates the course of several cardiovascular, pulmonary and other systemic diseases in children. An acute rise of RV afterload, either as exacerbating chronic PH of different aetiologies (eg, idiopathic pulmonary arterial hypertension (PAH), chronic lung or congenital heart disease), or pulmonary hypertensive crisis after corrective surgery for congenital heart disease, may lead to severe circulatory compromise. Only few clinical studies provide evidence on how to best treat children with acute severe PH and decompensated RV function, that is, acute RV failure. The specific treatment in the intensive care unit should be based on the underlying pathophysiology and not only be focused on so-called 'specific' or 'tailored' drug therapy to lower RV afterload. In addition therapeutic efforts should aim to optimise RV preload, and to achieve adequate myocardial perfusion, and cardiac output. Early recognition of patients at high risk and timely initiation of appropriate therapeutic measures may prevent the development of severe cardiac dysfunction and low cardiac output. In patients not responding adequately to pharmacotherapy, (1) novel surgical and interventional techniques, temporary mechanical circulatory support with extracorporeal membrane oxygenation, (2) pumpless lung assist devices (3) and/or lung or heart-lung transplantation should be timely considered. The invasive therapeutic measures can be applied in a bridge-to-recovery or bridge-to-lung transplant strategy. This consensus statement focuses on the management of acute severe PH in the paediatric intensive care unit and provides an according treatment algorithm for clinical practice.

Treatment of children with 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

“…Therapy of group 2–5 PH is controversial and requires separate discussions. Special considerations for the treatment of PH in the acute and critical care setting,3 treatment of PAH patients with congenital heart disease (CHD)4 and therapy of infants with PH associated with acute and chronic lung disease (CLD)5 are discussed in separate articles of this special issue.…”

“…Venoarterial-ECMO as bridge to lung transplantation or bridge to recovery in severe PAH is discussed in a separate article of the European Paediatric Pulmonary Vascular Disease Network 3. A limited number of studies on the value of balloon atrial septostomy (AS) and Potts shunt in advanced paediatric PAH are available and will be discussed in the following section.…”

Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK

“…Finally, detailed recommendations on therapy are given in two documents: (1) treatment of acute PH in the intensive care unit, including ventricular assist device (VAD)/extracorporeal membrane oxygenation and lung transplantation (LuTx),28 and (2) comprehensive recommendations on mid- to long-term treatment of PH/PHVD in the inpatient and outpatient setting, including pharmacotherapy, catheter interventions and surgery 29…”

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

“…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.…”