Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners.

Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to many cancers. Two randomized controlled trials of autologous hematopoietic stem cell transplantation (AHSCT) for SSc have shown significant improvement in organ function, quality of life and long-term survival compared to standard therapy. However, transplant-related mortality (TRM) ranged from 3-10% in patients undergoing HSCT. In SSc, the main cause of non-transplant and TRM is cardiac related. We therefore updated the previously published guidelines for cardiac evaluation, which should be performed in dedicated centers with expertize in HSCT for SSc. The current recommendations are based on pre-transplant cardiopulmonary evaluations combining pulmonary function tests, echocardiography, cardiac magnetic resonance imaging and invasive hemodynamic testing, initiated at Northwestern University (Chicago) and subsequently discussed and endorsed within the EBMT ADWP in 2016

Sometimes they come back: recurrent noncardiac right-to-left shunt after percutaneous patent foramen ovale closure.

An increasing number of patients are being evaluated for percutaneous patent foramen ovale (PFO) closure to prevent recurrent cerebrovascular events, but debate still exists on therapeutic indications and off-label closure device implantation. Pulmonary arteriovenous fistulas (PAVFs) are a rare and heterogeneous malformation prevalently associated with Rendu-Osler-Weber syndrome, and may mimic PFO right-to-left shunt (RLS), leading to unnecessary interventions and in some cases to relapses. Residual shunt is increasingly being observed both after PFO closure and PAVF embolization, even at long-term follow-up, with unclear clinical relevance. This instrumental and possibly therapeutic failure could lie in the presence of pulmonary microfistulas, either pre-existing or following the intervention. Hence, if RLS persists after optimal device placement and reasonable endothelialization time, the presence of a PAVF should be assumed and investigated; if RLS recurs after previous, negative echocontrast studies, presence of device-related complications or pulmonary microfistulas should be taken into consideration