The CRASH report: emergency management dilemmas facing acute physicians in patients with pulmonary arterial hypertension

Abstract

Treatment of acute emergencies in patients with pulmonary arterial hypertension (PAH) can be challenging. In the UK and Ireland, management of adult patients with PAH is centred in eight nationally designated pulmonary hypertension (PH) centres. However, many patients live far from these centres and physicians in local hospitals are often required to manage PAH emergencies. A committee of physicians from nationally designated PH centres identified the 'most common' emergency clinical scenarios encountered in patients with PAH. Thereafter, a review of the literature was performed centred on these specified topics and a management approach was developed based on best available evidence and expert consensus. Management protocols were developed on the following PAH emergencies: chest pain (including myocardial ischaemia), right ventricular failure, arrhythmias, sepsis, haemoptysis ('CRASH'), as well as considerations relevant to surgery, anaesthesia and pregnancy. Emergencies are not uncommon in PAH. While expertise in PAH management is essential, all physicians involved in acute care should be aware of the principles of acute management of PAH emergencies. A multidisciplinary approach is necessary, with physicians from tertiary PH centres supporting care locally and planning safe transfer of patients to PH centres when appropriate.

Aortic valve replacement with pulmonary hypertension: Meta‐analysis of 70 676 patients

“…Patients with PHT undergoing SAVR also experienced increased risks of adverse events such as acute kidney injury, prolonged ventilation, and longer duration of hospital stay. These may be related to older age and higher prevalence of comorbidities (eg, COPD, CKD, peripheral vascular disease) as well as respiratory failure secondary to PHT …”

Pulmonary Hypertension Associated with Connective Tissue Disease

“…1 Intermediate and highrisk operations should be avoided unless necessary, where necessary these should be planned with the PH team, to ensure optimal pre-and peri-operative management of right ventricular function, using spinal anesthesia where possible, and often with invasive monitoring of central venous pressure. 39,40 Early mobilization postoperation is essential with early aggressive management of any complications, especially infection. In general, if the mPAP is <35 mm Hg with a cardiac index, >2.5 patients will get through major surgery.…”

Pulmonary Arterial Hypertension Emergency Complications and Evaluation

“…Patients with PAH may experience clinical worsening because of an acute harmful event, or simply disease progression, that worsens RV function causing RV distension, impaired RV adaptation, and myocardial oxygen consumption/delivery imbalance ( Price et al, 2017 ). Renal dysfunction (i.e., cardiorenal failure) commonly occurs in patients with PAH because of chronic diuretic therapy used to treat RHF.…”

“…In a study of 231 patients with PAH who were followed up for 6 years, there was an annual risk of 2.8% for both atrial fibrillation and atrial flutter ( Tongers et al, 2007 ). In advanced PAH, increased atrial stretch and myocardial fibrosis may contribute to the development of SVTs ( Price et al, 2017 ). In patients with PAH and congenital heart disease, SVTs are common and often related to previous surgical scars or right atrial dilatation.…”

“…Policies for how to treat patients with acute decompensated heart failure in the ED exist, but these focus on left heart failure ( Collins et al, 2015 ; Silvers, Howell, Kosowsky, Rokos, & Jagoda, 2007 ). A recent review addressed some issues faced by ED physicians when treating patients with PAH in the United Kingdom and Ireland, but some of the treatment guidelines are specific to those health care systems ( Price et al, 2017 ).…”