Pulmonary Arterial Thrombosis in Eisenmenger Syndrome Is Associated With Biventricular Dysfunction and Decreased Pulmonary Flow Velocity

Abstract

Pulmonary arterial thrombosis among adults with Eisenmenger syndrome is common and relates to older age, biventricular dysfunction, and slow pulmonary artery blood flow rather than degree of cyanosis or coagulation abnormalities. Further work to define treatment efficacy is needed.

Guidelines for the diagnosis and treatment of pulmonary hypertension

“…haemorrhage and haemoptysis [195]. No data exist on this issue and definite recommendations cannot therefore be made.…”

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

“…Abnormal flow properties secondary to PA dilatation and intimal damage may play a role in thrombogenesis. On the other hand, bleeding under oral anticoagulation or spontaneous bleeding are not uncommon and may have a significant impact on morbidity and mortality [44]. Due to this therapeutic dilemma, routine anticoagulation is not recommended in these patients in the absence of additional indications such as atrial fibrillation, pulmonary arterial thrombus or mechanical prosthetic valves [39].…”

Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease

“…239,690,691 These in situ thrombi are not thromboembolic and appear to be related to disease severity. 237 Whether pulmonary thrombi in this setting should be treated as arterial or venous and whether routine prophylactic anticoagulation with warfarin, a common practice for patients with pulmonary vascular disease of various origins, or other antiplatelet therapy should be recommended for all patients with pulmonary vascular disease associated with CHD or acquired heart disease remain unknown and clearly warrant urgent investigation. For the older patient with CHD and pulmonary vascular disease, other indications such as sustained arrhythmia or ventricular dysfunction may be present and thus dictate anticoagulation therapy independently.…”