Peripheral microvascular function is linked to cardiac involvement on cardiovascular magnetic resonance in systemic sclerosis-related pulmonary arterial hypertension.

AIMS: Systemic sclerosis (SSc) is characterized by vasculopathy, inflammation, and fibrosis, and carries one of the worst prognoses if patients also develop pulmonary arterial hypertension (PAH). Although PAH is a known prognosticator, patients with SSc-PAH demonstrate disproportionately high mortality, presumably due to cardiac involvement. In this cross-sectional study, the relationship between cardiac involvement revealed by cardiovascular magnetic resonance (CMR) and systemic microvascular disease severity measured with nailfold capillaromicroscopy (NCM) in patients with SSc-PAH is evaluated and compared with patients with idiopathic PAH (IPAH). METHODS AND RESULTS: Patients with SSc-PAH and IPAH underwent CMR, echocardiography, and NCM with post-occlusive reactivity hyperaemia (PORH) testing on the same day. CMR imaging included T2 (oedema), native, and post-contrast T1 mapping to measure the extracellular volume fraction (ECV, fibrosis) and adenosine-stress-perfusion imaging measuring the relative myocardial upslope (microvascular coronary perfusion). Measures of peripheral microvascular function were related to CMR indices of oedema, fibrosis, and myocardial perfusion. SSc-PAH patients (n = 20) had higher T2 values and a trend towards a higher ECV, compared with IPAH patients (n = 5), and a lower nailfold capillary density (NCD) and reduced capillary recruitment after PORH. NCD correlated with ECV and T2 (r = -0.443 and -0.464, respectively, P < 0.05 for both) and with markers of diastolic dysfunction on echocardiography. PORH testing, but not NCD, correlated with the relative myocardial upslope (r = 0.421, P < 0.05). CONCLUSION: SSc-PAH patients showed higher markers of cardiac fibrosis and inflammation, compared with IPAH patients. These markers correlated well with peripheral microvascular dysfunction, suggesting that SSc-driven inflammation and vasculopathy concurrently affect peripheral microcirculation and the heart. This may contribute to the disproportionate high mortality in SSc-PAH.

Microtransesophageal Echocardiographic Guidance during Percutaneous Interatrial Septal Closure without General Anaesthesia.

OBJECTIVE: To study the safety and efficacy of microtransesophageal echocardiography (micro-TEE) and TEE during percutaneous atrial septal defect (ASD) and patent foramen ovale (PFO) closure. BACKGROUND: TEE has proven to be safe during ASD and PFO closure under general anaesthesia. Micro-TEE makes it possible to perform these procedures under local anaesthesia. We are the first to describe the safety and efficacy of micro-TEE for percutaneous closure. METHODS: All consecutive patients who underwent ASD and PFO closure between 2013 and 2018 were included. The periprocedural complications were registered. Residual shunts were diagnosed using transthoracic contrast echocardiography (TTCE). All data were compared between the use of TEE or micro-TEE within the ASD and PFO groups separately. RESULTS: In total, 82 patients underwent ASD closure, 46 patients (49.1 ± 15.0 years) with TEE and 36 patients (47.8 ± 12.1 years) using micro-TEE guidance. Median device diameter was, respectively, 26 mm (range 10-40 mm) and 27 mm (range 10-35 mm). PFO closure was performed in 120 patients, 55 patients (48.6 ± 9.2 years, median device diameter 25 mm, range 23-35 mm) with TEE and 65 patients (mean age 51.0 ± 11.8 years, median device diameter 27 mm, range 23-35 mm) using micro-TEE. There were no major periprocedural complications, especially no device embolizations within all groups. Six months after closure, there was no significant difference in left-to-right shunt after ASD closure and no significant difference in right-to-left shunt after PFO closure using TEE or micro-TEE. CONCLUSION: Micro-TEE guidance without general anaesthesia during percutaneous ASD and PFO closure is as safe as TEE, without a significant difference in the residual shunt rate after closure.

Transcatheter Tricuspid Valve Interventions: An Emerging Field.

PURPOSE OF REVIEW: This review aims to provide an updated overview and a clinical perspective on novel transcatheter tricuspid valve interventions (TTVI), highlighting potential challenges and future directions. RECENT FINDINGS: Severe tricuspid regurgitation (TR) is a predictor of mortality. However, a sizeable number of patients remain untreated until the end-stage when cardiac surgery presents a prohibitive risk. The emergent need in finding a treatment for patients with TR, deemed for surgery options, has encouraged the development of TTVI. These procedures mimic classical surgery techniques and are mainly divided in four categories: annuloplasty and coaptation devices, edge-to-edge techniques and transcatheter tricuspid valve replacement. Early studies showed promising results, but long-term follow-up data are not available. For patients with severe TR and high surgical risk, several percutaneous options are available. However, these therapies are in a growing phase and bigger studies and long term follow-up are needed to prove their efficacy.

Prediction of hemodynamic improvement after pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension using non-invasive imaging.

Pulmonary endarterectomy (PEA) is the recommended treatment in chronic thromboembolic pulmonary hypertension (CTEPH). Prediction of outcome after PEA remains challenging. In search for pre-operative predictors we evaluated non-invasive parameters measured by chest CT-scan and echocardiography. Between May 2004 and January 2009, 52 consecutive patients with CTEPH who underwent PEA (59.6 % female, mean age 58.9 ± 13.4 years) were included. Prior to surgery, pulmonary artery (PA) diameter indices were calculated by chest CT scan and different echocardiographic measurements to evaluate pulmonary hypertension were obtained. Hemodynamic improvement after PEA was defined as a pulmonary vascular resistance (PVR) <500 dyn s cm(-5) and a mean pulmonary artery pressure <35 mmHg 3 days after PEA. Mortality was evaluated at day 30. Mean pulmonary artery pressure (PAP) at baseline was 40.1 ± 8.5 mmHg, with a PVR of 971 ± 420 dyn s cm(-5). Persistent pulmonary hypertension was observed in 15 patients (28.8 %). Gender, pre-operative mean PAP, PA diameter indices, and tricuspid annular plane systolic excursion were all predictors for hemodynamic improvement after PEA. The indexed PA diameter on CT was the only independent predictor for hemodynamic improvement: 19.4 ± 2.4 versus 22.9 ± 4.9 mm/m(2) in those without improvement (OR 0.76: 95 % CI 0.58-0.99, p = 0.04). All patients who died within 30 days (9.6 %) had persistent pulmonary hypertension, with a post-operative mean PAP of 51.6 ± 14.1 mmHg and PVR of 692 ± 216 dyn s cm(-5). The pre-operative PA diameter indexed for body surface area is the only independent predictor for hemodynamic improvement after PEA in CTEPH patients. In all patients who died within 30 days after PEA, persistent pulmonary hypertension was present.

Survival of transcatheter mitral valve repair compared with surgical and conservative treatment in high-surgical-risk patients.

OBJECTIVES: The goal of this study was to compare survival between transcatheter mitral valve (MV) repair using MitraClip system (Abbott Vascular, Santa Clara, California), MV-surgery, and conservative treatment in high-surgical-risk patients symptomatic with severe mitral valve regurgitation (MR). BACKGROUND: Up to 50% of patients with symptomatic severe MR are denied for surgery due to high perioperative risk. Transcatheter MV repair might be an alternative. METHODS: Consecutive patients (n = 139) treated with transcatheter MV repair were included. Comparator surgically (n = 53) and conservatively (n = 59) treated patients were identified retrospectively. Surgical risk was based on the logistic European System for Cardiac Operative Risk Evaluation (log EuroSCORE) or the presence of relevant risk factors, as judged by the heart team. RESULTS: The log EuroSCORE was higher in the transcatheter MV repair group (23.9 ± 16.1%) than in the surgically (14.2 ± 8.9%) and conservatively (18.7 ± 13.2%, p < 0.0001) treated patients. Left ventricular ejection fraction was higher in surgical patients (43.9 ± 14.4%, p = 0.003), with similar values for the transcatheter MV repair (36.8 ± 15.3%) and conservatively treated (34.5 ± 16.5%) groups. After 1 year of follow-up, the transcatheter MV repair and surgery groups showed similar survival rates (85.8% and 85.2%, respectively), whereas 67.7% of conservatively treated patients survived. The same trend was observed after the second and third years. After weighting for propensity score and controlling for risk factors, both the transcatheter MV repair (hazard ratio [HR]: 0.41, 95% confidence interval [CI]: 0.22 to 0.78, p = 0.006) and surgical (HR: 0.52, 95% CI: 0.30 to 0.88, p = 0.014) groups showed better survival than the conservatively treated group. The transcatheter MV repair and surgical groups did not differ (HR: 1.25, 95% CI: 0.72 to 2.16, p = 0.430). CONCLUSIONS: Despite a higher log EuroSCORE, high-surgical-risk patients with symptomatic severe MR treated with transcatheter MV repair show similar survival rates compared with surgically treated patients, with both displaying survival benefit compared with conservative treatment.

Percutaneous edge-to-edge mitral valve repair in high-surgical-risk patients: do we hit the target?

OBJECTIVES: This study sought to assess the feasibility and safety of percutaneous edge-to-edge mitral valve (MV) repair in patients with an unacceptably high operative risk. BACKGROUND: MV repair for mitral regurgitation (MR) can be accomplished by use of a clip that approximates the free edges of the mitral leaflets. METHODS: All patients were declined for surgery because of a high logistic EuroSCORE (>20%) or the presence of other specific surgical risk factors. Transthoracic echocardiography was performed before and 6 months after the procedure. Differences in New York Heart Association (NYHA) functional class, quality of life (QoL) using the Minnesota questionnaire, and 6-min walk test (6-MWT) distances were reported. RESULTS: Fifty-five procedures were performed in 52 patients (69.2% male, age 73.2 ± 10.1 years, logistic EuroSCORE 27.1 ± 17.0%). In 3 patients, partial clip detachment occurred; a second clip was placed successfully. One patient experienced cardiac tamponade. Two patients developed inguinal bleeding, of whom 1 needed surgery. Six patients (11.5%) died during 6-month follow-up (5 patients as a result of progressive heart failure and 1 noncardiac death). The MR grade before repair was ≥3 in 100%; after 6 months, a reduction in MR grade to ≤2 was present in 79% of the patients. Left ventricular (LV) end-diastolic diameter, LV ejection fraction, and systolic pulmonary artery pressure improved significantly. Accompanied improvements in NYHA functional class, QoL index, 6-MWT distances, and log N-terminal pro-B-type natriuretic peptide were observed. CONCLUSIONS: In a high-risk population, MR reduction can be achieved by percutaneous edge-to-edge valve repair, resulting in LV remodeling with improvement of functional capacity after 6 months.

Transapical treatment of paravalvular leaks in patients with a logistic EuroSCORE of more than 15%: acute and 3-month outcomes of a "proof of concept" study.

OBJECTIVE: We describe the short-term results of the patients who underwent transapical treatment of a paravalvular leak (PVL) in our centre. BACKGROUND: Increasing experience with transapical aortic valve implantation has inspired us to explore this approach for prosthetic paravalvular leak reduction in high risk patients. METHODS: All procedures were performed in the catheterization laboratory under general anesthesia, using a small anterolateral thoracotomy to expose the apex. Access through a 9-French sheath was necessary to introduce the Amplatzer Vascular III plug. Three-dimensional transesophageal echocardiography (3D-TEE) was used to guide the operator and evaluate the severity of regurgitation postimplantation. RESULTS: In total seven consecutive patients (mean age 72.8 ± 5.6 years, 86% male) with a history of mitral valve (n = 6) or aortic valve replacement and severe PVL, underwent transapical PVL reduction using seven plugs in total (diameter 10-14 mm). Preprocedural median logistic EuroSCORE was 28.5% (range 17.1-41.1%) and NYHA functional class was ≥3 in all patients. The procedure was successful in all patients, with a median fluoroscopic time of 18.7 min (range 10.1-29.6 min). Postprocedure 3D-TEE showed occlusion of PVL in three patients, and significant reduction in three patients. Postprocedural complication was a hematothorax requiring surgery in one patient. Median hospitalization duration after the procedure was 5 days (range 5-59 days). At 3-month follow-up one patient died, functional class and LDH did not differ significantly and there was a significant increase in hemoglobin. CONCLUSIONS: Transapical paravalvular leak reduction might be a good or rather attractive alternative in high-risk patients for major re-do cardiac surgery.

Cardiac magnetic resonance imaging showing complete resolution of subendocardial involvement in Churg-Strauss syndrome.

Churg-Strauss syndrome or allergic angiitis and granulomatosis is a rare systemic vasculitis. Cardiac involvement is the leading cause of mortality and includes eosinophilic endomyocarditis. We present a case of complete resolution of subendocardial involvement after high-dose corticosteroids, diagnosed by contrast-enhanced cardiovascular magnetic resonance imaging.

Increased prevalence of migraine in Marfan syndrome.

OBJECTIVE: A high prevalence of migraine has been described in various forms of congenital heart disease, with and without shunt. In this study we investigated the prevalence of migraine in patients with Marfan syndrome (MFS). METHODS: All 457 adult patients with MFS from the participating centres and 194 controls received a validated questionnaire about headache. Migraine was diagnosed according to the International Headache Society criteria, by three independent neurologists, blinded to patient files. RESULTS: Response rate was 68% and 56% in Marfan patients and controls, respectively. Forty percent of the 309 responding MFS patients (mean age 40+/-14 years; 51% females) and 28% of the 102 controls (mean age 43+/-15 years; 58% females), suffered from migraine (p=0.03). The prevalence of migraine with aura (MA) was 22% in MFS patients and 14% in controls (p=0.06). We found MFS to be an independent risk factor for having overall migraine (OR 1.7; 95%CI 1.1-2.8), also after adjustment for age and gender (OR 1.9; 95%CI 1.1-3.1; p=0.02) and for MA after adjustment for gender (OR 2.0; 95%CI 1.1-3.7; p=0.04). In patient with MFS, previous aortic root surgery appeared to be an independent risk factor for having MA (OR 2.2; 95%CI; 1.2.-4.0, p=0.01) adjusted for gender. CONCLUSION: MFS is an independent risk factor for having overall migraine and MA. Moreover, we found that a history of aortic root surgery seems to be associated with an increased risk of MA.

PET scintigraphy of etoposide-induced pulmonary toxicity.

A patient with chronic myelomonocytic leukemia developed drug-induced pulmonary toxicity after using low dose oral etoposide. Because etoposide-induced pulmonary toxicity is an uncommon but serious adverse event, clinicians must be vigilant about the possibility of it, so that the optimal treatment can start as soon as possible. This report demonstrates that PET scintigraphy might be a helpful tool in the early diagnosis of drug-induced pulmonary toxicity.

A pulmonary right-to-left shunt in patients with hereditary hemorrhagic telangiectasia is associated with an increased prevalence of migraine.

INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal-dominant vascular dysplasia with a high prevalence of pulmonary arteriovenous malformation (PAVM). Recent studies report an increased prevalence of migraine in patients with a cardiac right-to-left shunt. The aim of our study was to evaluate whether there is also an increased prevalence of migraine in patients with a pulmonary right-to-left shunt (PAVM). METHODS: All patients with HHT referred to our hospital till April 2004 with or without PAVM and with or without migraine were included in the study. RESULTS: In total, 538 HHT patients (41.6% men; mean age +/- SD, 39.3 +/- 18.6 years) could be included. PAVM was present in 208 patients (38.7%; mean age, 39.3 +/- 17.6 years). Significantly more women were present in the PAVM subgroup compared to the non-PAVM subgroup, 65.4% vs 53.9% (p = 0.009). Migraine occurred in 88 patients with HHT, a prevalence of 16.4%. The prevalence of migraine in women with HHT was significantly higher compared to men, 19.4% vs 12.1%, respectively (p = 0.03) The prevalence of migraine in patients with PAVM was 21.2%, which was significantly higher then in patients without PAVM, 13.3% (p = 0.02). The occurrence of PAVM in the patients with migraine is significantly higher than in those without migraine, 50.0% vs 36.4%, respectively (p = 0.02). CONCLUSION: This study showed a higher prevalence of PAVM in patients with migraine and HHT. The right-to-left shunt due to the PAVM might play a causal role in the pathogenesis of migraine in patients with HHT. This needs to be determined in further studies.