Do Right Heart Hemodynamic Improvements Persist After Pulmonary Thromboendarterectomy?

The survival benefits of pulmonary thromboendarterectomy (PTE) for the treatment of chronic thromboembolic pulmonary hypertension have been well described. However, the significance of right heart hemodynamic changes and their impact on survival remains poorly understood. We sought to characterize the effects of these changes. We conducted a single center, retrospective review of 159 patients who underwent PTE between 1993 and 2015. Echocardiographic and right heart catheterization data were compared longitudinally before and after PTE in order to establish the extent of hemodynamic response to surgery. Kaplan Meier estimates were used to characterize patient survival over time. Univariable and multivariable Cox proportional hazards regression models were used to assess factors associated with long-term mortality. Among the 159 patients studied, 74 (46.5%) were male with a median age of 55 (IQR: 42-66). One-, 5-, 10-, and 15-year survival was 91.0% (95% CI: 86.6-95.6), 79.6% (73.5-86.3), 66.5% (59.2-74.7), and 56.2% (48.1-65.8). Of the 9 candidate risk factors that were evaluated, only advanced age and increased cardiopulmonary bypass time were found to be significantly associated with increased risk of mortality. Pre- and postsurgical echocardiographic imaging data, when available, revealed a median reduction in right ventricular systolic pressure of 29.0 mm Hg (P < 0.0001) and improvement of tricuspid regurgitation (P < 0.0001), both of which appeared to be sustained across long-term follow-up. Improvements in right heart hemodynamics and tricuspid valvular regurgitation persist on long term surveillance following PTE. While patient selection is often driven by the distribution of disease, close postoperative follow up may improve outcomes.

"Carcinoid-like" tricuspid valvulopathy associated with nephrogenic systemic fibrosis.

We present a case of a patient with end-stage renal disease and nephrogenic fibrosing dermopathy (NFD) whose echocardiogram demonstrated rare tricuspid valve abnormality with malcoaptation due to tethering and restricted motion of the leaflets causing severe tricuspid regurgitation. The cardiac abnormalities developed 3 years after her initial diagnosis of NFD was made by skin biopsy. The echocardiographic appearance of the tricuspid valve resembled that seen in patients with carcinoid heart disease; however, an evaluation for carcinoid tumor in our patient was negative. Myocardial biopsy performed at the time of right heart catheterization demonstrated trace gadolinium within the lysosomes of one cardiac fibroblast. This report is the first to describe the association between nephrogenic systemic fibrosis and severe valvular heart disease requiring valve replacement.

Discontinuation of antithrombotic therapy for a year or more in patients with continuous-flow left ventricular assist devices.

The recommended anticoagulation regimen during continuous-flow axial left ventricular assist device (LVAD) support is aspirin and warfarin with a targeted international normalized ratio of 2.0-3.0. We report two patients in whom recurrent gastrointestinal bleeding during LVAD support necessitated discontinuation of this anti-thrombotic regimen for a year or more. Despite this, neither patients developed thrombotic complications during 29 patient-months of follow-up. An acquired von Willebrand factor (VWF) abnormality reflected by the absence or decreased abundance of the highest molecular weight multimers was demonstrated in both patients. The gold standard test for platelet function, light transmission platelet aggregometry was measured in one patient and was normal, indicative that the predominant abnormality in the coagulation profile of these patients is an acquired VWF syndrome. Clinical trials are required to address the question whether it is safe to discontinue anticoagulation in LVAD patients with acquired VWF abnormalities.