Acute and chronic dissection of pulmonary artery: new challenges in pulmonary arterial hypertension?

Abstract

Right ventricular failure is a leading cause of mortality in patients with pulmonary arterial hypertension (PAH). However, up to 25% of such patients die unexpectedly, without warning signs of hemodynamical decompensation. We previously documented that pulmonary artery (PA) dilatation significantly increases the risk of those deaths. Some of them may be due to dissection of PA resulting in cardiac tamponade. However, direct confirmation of this mechanism is difficult as most of such deaths occur outside hospitals. We present 4 patients with severe PAH and PA dilatation in whom PA dissection has been confirmed. Three patients had IPAH, one had PAH associated with congenital heart disease. All patients had mean pulmonary artery pressure (PAP) > 50 mmHg at diagnosis and dissection occurred late in the course of apparently well controlled disease (6 to 14 years). Several clinical elements were common to our patients - high systolic PAP, long lasting PH, progressive dilatation of PA to more than 50 mm with chest pain prior to dissection. However, clinical course followed three different patterns: sudden death due to cardiac tamponade, hemopericarditis caused by blood leaking from dissected aneurysm with imminent but not immediate cardiac tamponade, or chronic asymptomatic PA dissection. Indeed, two of our patients are alive and on lung transplantation waiting list for more than 2 years now. Further research is needed to suggest optimal management strategies for patients with stable PAH but significantly dilated proximal pulmonary arteries or confirmed PA dissection depending on the clinical presentation and expected outcome.

A case of giant main pulmonary artery aneurysm associated with complicated congenital heart disease and simultaneous chronic pulmonary artery dissection

“…Together with our patients, only 14 cases of chronic PA dissection have been reported in patients with PH [9]. There are some reports regarding PAA without PAD and CHD [1,10], PAA with CHD [11], and PAA with acute and chronic PAD [1,12].…”

Simultaneous pulmonary artery and Stanford type B aortic dissections via the ductus arteriosus

“…Pulmonary artery dissection is an exceedingly rare diagnosis that is typically lethal and is most often encountered in patients with history pulmonary hypertension, congenital heart disease, and/or prior pulmonary artery procedural intervention [1] . Less than 100 cases of pulmonary artery dissections have been reported in the literature, and the diagnosis is more often made postmortem as it can lead to pulmonary arterial rupture [1] , [2] , [3] . The main pulmonary artery is the most common location for pulmonary artery dissection, and is often involved in isolation [1] .…”

Potential Molecular Pathways Related to Pulmonary Artery Aneurysm Development: Lessons to Learn from the Aorta

“…Although an increased PA diameter has been shown to be an independent risk factor for sudden cardiac death in PAH patients [8], there are no necropsy registries allowing for the confirmation of PA dissection being the main cause of sudden death in this population. Furthermore, there are some case series of patients with confirmed PA dissection in whom a conservative strategy was chosen with an acceptable long-term survival and who presented with some PA branches dissection without a severely dilated main PA [9,10]. Furthermore, there are other conditions that could be responsible of an unexpected death in PAH patients, such as left main coronary artery compression by a PAA, which could induce myocardial ischemia and fatal ventricular arrhythmias [11].…”

“…PA dilatation is related to an increased risk of sudden cardiac death [8] and among patients with PAA a non-significant higher proportion of sudden cardiac death has been described [1]. This increased risk of sudden cardiac death may be related to the presence of life-threatening complications such as PA dissection [9,10] and left main coronary artery compression [11].…”