Management of Acutely Decompensated Pulmonary Hypertension

Abstract

Pulmonary arterial hypertension is a severe life-threatening condition associated with increased pulmonary vascular resistance and resulting right heart dysfunction. Admission to intensive care unit with acutely decompensated right heart failure is a significant negative prognostic event with a high risk of multisystem organ dysfunction and death. Presentations are heterogenous and may combine signs of both diastolic and systolic dysfunction complicating management. Renal dysfunction is often present, but other organ systems can be involved resulting in findings such as acute hepatic dysfunction or bowel wall congestion and ischemia. The goals of therapy are to rapidly reverse ventriculo-arterial decoupling and reduce right ventricular afterload to prevent progression to refractory or irreversible right heart failure. Triggering events must be investigated for and addressed urgently if identified. Volume status management is critical and both noninvasive and invasive testing can aid in prognostication and guide management, including the use of inotropes and vasopressors. In cases of refractory right heart dysfunction, consideration of urgent lung transplantation and mechanical circulatory support is necessary. These patients should be managed at expert centers in an intensive care setting with a multidisciplinary team of practitioners experienced in the management of right heart dysfunction given the high short- and long-term mortality resulting from acute decompensated right heart failure.

Approach to Decompensated Right Heart Failure in the Acute Setting

“…Medically optimizing perfusion and cardiac output with the use of inotropes and vasopressors and promptly treating hypoxia to avoid intubation is also necessary. Furthermore, the provider needs to consider mechanical circulatory support, such as VA-ECMO or RVADS, or lung transplantation in cases of refractory RHF despite maximal medical therapy [34].…”

“…Dobutamine is one of the preferred inotropes in patients with RV failure because it amplifies myocardial contractility while also reducing both right and left ventricular afterload [1,10]. However, dobutamine has chronotropic properties and can induce tachycardia at higher doses (>10.0 mcg/kg/min), which can be detrimental to diastolic filling time, particularly in patients with PAH, and arrhythmias [4,9,10,34].…”

“…Patients with atrial tachyarrhythmias may benefit from electrical cardioversion, as the dysrhythmia can lead to worsening right ventricular cardiac output due to the loss of atrial kick [17], although large-scale studies on its use have yet to be studied in RV failure [4]. Amiodarone and digoxin are the leading choices for the restoration of sinus rhythm [34,37]. Radiofrequency ablation is another option for patients with recurrent SVT [1].…”

“…Radiofrequency ablation is another option for patients with recurrent SVT [1]. Rate control with beta blockers or calcium channel blockers can worsen cardiac output and is generally not recommended [17,34]. In patients with right-bundle branch block and RV dysfunction, AV pacing may enhance RV performance [4].…”