Intensive Care, Right Ventricular Support and Lung Transplantation in Patients with Pulmonary Hypertension

• ICU monitoring of patients with severe right-sided heart failure should include regular measurements of central venous pressure and S_cvO2.

• Key warning signs of imminent death in patients with right-sided heart failure are a decline in S_cvO2 accompanied by an increase in lactate and a decline in urine output.

• The use of right heart catheterisation or other devices to monitor haemodynamics and cardiac output should be considered in patients with severe right-sided heart failure and in complex situations.

• Patients with PAH or other forms of severe PH with right-sided heart failure requiring ICU therapy should be treated at expert centres capable of providing all treatment options, i.e. medical therapy, ECLS and advanced treatment including lung transplantation, if possible.

• Interhospital transfer should be considered on an individual basis. Some centres provide mobile units facilitating interhospital transfer with ECLS.

• ICU treatment of patients with right-sided heart failure should include treatment of underlying causes and comorbidities, supportive measures, meticulous fluid management, reduction of right ventricular afterload with drugs approved for PAH, and an individualised use of inotropes and vasopressors.

INDICATIONS AND CONTRAINDICATIONS

• Established indication: bridge to transplant in patients who have been fully evaluated and accepted for this procedure.

• Potential indications in highly selected cases:

  • – bridge to transplant decision in potentially eligible patients who have not yet been fully evaluated;

  • – bridge to recovery in patients with untreated or undertreated PAH, or in patients with a reversible cause of right ventricular failure.

• Contraindication: end-stage disease without a realistic chance for recovery or successful transplantation (futility).

CHOICE OF ECLS

• Veno-arterial ECMO and the PA-LA approach are currently the only established right ventricular support strategies, but there is rapid evolution in device technologies.

• At present, veno-arterial ECMO is the most widely used ECLS strategy.

• The PA-LA approach should be considered if the expected ECLS time is of longer duration or in children with small femoral arteries.

• The choice of ECLS depends largely on centre experience.

TIMING OF ECLS

• All forms of ECLS are associated with potentially life-threatening complications; hence, ECLS should be used only when less invasive treatment options have been exhausted.

• ECLS should be initiated when the clinical course suggests that terminal right heart failure and/or secondary organ failure is imminent despite optimised medical therapy.

• ECLS initiated in patients with advanced PH/PAH undergoing cardiopulmonary resuscitation for right-sided heart failure will rarely result in good outcomes.

ECLS AND LUNG TRANSPLANTATION

• ECLS is now an established strategy to bridge patients with right heart failure to lung transplantation.

• Centres performing lung transplantation in patients with PAH should have an established ECLS programme.

• Repeated risk assessment is pivotal to identify the appropriate time for initiating transplant evaluation.

• Established and validated risk prediction tools such as the REVEAL risk score or the ESC/ERS risk prediction strategy should be applied in patients with PAH to determine timing for referral to a transplant centre.

• Potentially eligible candidates should be referred for lung transplantation evaluation early, i.e. when they have an inadequate response to oral combination therapy, indicated by an intermediate or high risk according to the ESC/ERS risk stratification strategy or by a REVEAL risk score >7.

• Listing for lung transplantation should be considered in patients who present with a high risk of death according to the ESC/ERS risk stratification strategy or by a REVEAL risk score ≥10 despite receiving optimised medical therapy including s.c. or i.v. PCAs, as the expected mortality on medical therapy exceeds the expected mortality after bilateral lung transplantation. Depending on local circumstances, listing of patients at intermediate risk might be appropriate in some countries.

• Timing of listing must depend on expected local waiting time.

• Bilateral lung transplantation is the method of choice in patients with PH.

• There is no degree of right ventricular dysfunction that precludes bilateral lung transplantation in patients with PAH.

• Despite advances in ICU management and ECLS, the ideal recipient is an ambulant outpatient.

• Extended use of ECMO support should be considered after lung transplantation in patients with PH to prevent early graft dysfunction.

• Given the low number and high risk of lung transplants performed for PAH worldwide, this procedure should be concentrated in specialised centres.