A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report.

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment. CASE PRESENTATION: We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months. CONCLUSIONS: Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers.

Cryoballoon ablation for persistent atrial fibrillation in patients without left atrial fibrosis.

INTRODUCTION: The role of cryoballoon (CB) pulmonary vein isolation (PVI) for patients with persistent atrial fibrillation (AF) is controversial, since long-term success can be poor. We performed left atrial voltage mapping before CB PVI and determined AF-free survival depending on the extent of low-voltage areas (LVAs). METHODS AND RESULTS: We consecutively enrolled 60 patients with persistent AF (average age, 60.6 ± 12.9 years; CHA2 DS 2 VASc score, 2.3 ± 1.6; and left atrial size 46.0 ± 5.2 mm) who were planned for CB PVI. Before ablation, we performed left atrial voltage mapping (Abbott EnSite Precision or Velocity). LVAs were defined if local bipolar signal amplitudes were less than 0.5 mV during sinus rhythm. Thirty-seven patients did not show significant LVAs (<10%), while 12 patients had LVAs between 10% and 30% and 11 patients showed substantial LVAs greater than 30% of the left atrial area. CB PVI could be successfully performed in all patients. A 7-day holter monitoring was obtained 3, 6, and 12 months after ablation. After a 12-month follow-up time, 83.8% of patients without LVAs (<10%) were free of atrial fibrillation, while 50.0% of patients with 10% to 30% LVAs and 9.1% of patients with LVAs more than 30% had stable sinus rhythm. The degree of atrial fibrosis correlated with the risk of AF recurrence. CONCLUSION: In patients with persistent AF undergoing CB PVI, the extent of left atrial LVAs predicts an AF-free survival. CB PVI seems to be a highly effective treatment for patients with persistent AF without atrial fibrosis.

Prognostic Value of Atrial Fibrillation Inducibility in Patients Without History of Clinical Atrial Fibrillation.

PURPOSE: During invasive electrophysiological studies (EPS), atrial fibrillation (AF) can be induced in patients without a history of AF. However, the prognostic value is not well evaluated in this population. Our aim was to investigate whether AF inducibility in those patients is associated with future clinical episodes of AF; whether non-inducibility is predictive of freedom from new-onset AF and finally, to examine clinical factors associated with inducibility. METHODS: Medical records from patients undergoing EPS between the years 2011 and 2014 were analysed retrospectively with 62 patients matching our inclusion criteria. Patients were divided into subgroups according to their inducibility status and underwent follow-up. Patients were assessed by a structured telephone interview, data from the further treating physicians and ECG recordings. RESULTS: AF was inducible in 19 patients ("induction group") and not inducible in the remaining 43 ("control group"). Inducibility was associated with a higher age (p=0.002), lower GFR (p=0.002), higher CHAD2S2-VASc score (p=0.004) and diagnosis of mitral (p=0.014), tricuspid (p=0.017) and pulmonary (p=0.026) valve insufficiency. Three months after EPS, 89.5% of all inducible patients were free of diagnosed AF, in contrast to 100% of those without inducibility (p=0.031). At three years, no significant difference was left (p=0.162). CONCLUSION: AF inducibility was found more often in an older population with cardiac comorbidities. While inducibility was associated with an increased rate of diagnosed new-onset clinical AF in the months after testing, non-inducibility seemed to be associated with freedom from AF at least in the short to medium term. However, there was no significant difference in the long term follow-up.

Circulating MicroRNA-21 Correlates With Left Atrial Low-Voltage Areas and Is Associated With Procedure Outcome in Patients Undergoing Atrial Fibrillation Ablation.

BACKGROUND: Atrial fibrosis is a hallmark of arrhythmogenic structural remodeling in patients with persistent atrial fibrillation (AF) and is negatively correlated with procedure outcome in patients undergoing ablation. However, noninvasive methods to determine the extent of atrial fibrosis are limited. Here, we used microRNA (miRNA) expression analysis to detect markers of left atrial low-voltage areas (LVAs) in patients with persistent AF undergoing catheter ablation. METHODS: We performed 3-dimensional voltage mapping in 102 patients (average age 62.1±13.1 years, CHA2DS2-VASc score of 2.3±1.6, LA size 41.5±5.7 mm) undergoing ablation for persistent AF and determined the extent of left atrial low-voltage. LVAs were defined if bipolar electrogram amplitudes were <0.5 mV during sinus rhythm. Before ablation, we obtained a blood sample, isolated miRNAs, and profiled them on a miRCURY LNA Universal RT microRNA PCR Human panel. RESULTS: Sixty-nine miRNAs were identified in all samples, with an average of 123 miRNAs detectable per sample. We found that the serum concentration of miR-21, a miRNA that has been previously linked to cardiac fibrosis development, was strongly associated with the extent of LVAs determined by voltage mapping. We could confirm that LVAs were negatively correlated with ablation success in a 1-year follow-up. In addition, miR-21 serum levels were associated with AF-free survival after catheter ablation. CONCLUSIONS: Circulating miR-21 correlates with left atrial LVAs and is associated with procedure outcome in patients with persistent AF undergoing ablation.