Antenatal Diagnosis of a Partial Atrioventricular Canal with Ebstein's Anomaly.

The simultaneous occurrence of an atrioventricular canal defect (AVCD) and Ebstein's anomaly is extremely rare, occurring in less than 0.5% of all patients with AVCD. Only 22 cases are described in the literature. This patient's antenatal diagnosis of both Ebstein's anomaly and partial AVCD was made at 25 weeks of gestation. The delivery was organized in a tertiary center. The initial neonatal course was difficult but with adequate treatment, a rapid improvement allowed for a gap of almost 2 years before a complete surgical repair including a cone tricuspid plasty. To our knowledge, this is the first case of antenatal diagnosis, with carefully tailored delivery, neonatal care and subsequent follow-up before indication for successful surgery.

Extending percutaneous left atrial appendage closure indications using the AMPLATZER™ Cardiac Plug device in patients with persistent left atrial appendage thrombus: The thrombus trapping technique.

BACKGROUND: Percutaneous left atrial appendage (LAA) closure has emerged as an alternative therapeutic option for the prevention of embolic stroke in high-risk patients with non-valvular atrial fibrillation. The presence of thrombus in the LAA is currently a contraindication to the procedure. AIM: To describe a modified LAA closure technique that allows a safe procedure in patients with LAA thrombus. METHODS: Between May 2013 and October 2014, LAA closure was performed in three patients with LAA thrombus (mean age 73.6±14 years; two men), using a modified technique that avoids manipulation of catheters or angiography in the LAA. RESULTS: Two patients had persistent thrombus despite appropriate antithrombotic therapy, while the other patient had a contraindication to systemic anticoagulation. The procedure was successful using the modified implantation technique in all patients. The implanted device was the AMPLATZER™ Cardiac Plug (St. Jude Medical, Minneapolis, MN, USA) in one patient and the Amulet™ (St. Jude Medical, Minneapolis, MN, USA) in two patients. No periprocedural complications occurred. After a mean follow-up of 8±2 months, no deaths or late complications were observed. CONCLUSIONS: Thrombus trapping is a feasible and effective technique for performing LAA occlusion in patients with thrombus within the LAA. This modification of the implantation technique may allow LAA closure indications to be extended to include patients with LAA thrombus, who were formerly considered unsuitable.

Coronary Wall Structural Changes in Patients With Kawasaki Disease: New Insights From Optical Coherence Tomography (OCT).

BACKGROUND: Coronary artery aneurysms (CAA) are serious complications of Kawasaki disease (KD). Optical coherence tomography (OCT) is a high-resolution intracoronary imaging modality that characterizes coronary artery wall structure. The purpose of this work was to describe CAA wall sequelae after KD. METHODS AND RESULTS: KD patients scheduled for routine coronary angiography underwent OCT imaging between March 2013 and August 2014. Subjects' clinical courses, echocardiography, and coronary angiography examinations were reviewed retrospectively. OCT was performed in 18 patients aged 12.4±5.5 years, 9.0±5.1 years following onset of KD. Of those, 14 patients (77.7%) had a history of CAA (7 with giant CAA and 7 with regressed CAA at time of OCT). Intracoronary nitroglycerin was given to all patients (88.4±45.5 µg/m(2)). Mean radiation dose was 10.9±5.2 mGy/kg. One patient suffered from a transitory uneventful vasospasm at the site of a regressed CAA; otherwise no major procedural complications occurred. The most frequent abnormality observed on OCT was intimal hyperplasia (15 patients, 83.3%) seen at both aneurysmal sites and angiographically normal segments amounting to 390.8±166.0 µm for affected segments compared to 61.7±17 µm for unaffected segments (P<0.001). Disappearance of the media, and presence of fibrosis, calcifications, macrophage accumulation, neovascularization, and white thrombi were seen in 72.2%, 77.8%, 27.8%, 44.4%, and 33.3% of patients. CONCLUSIONS: In this study, OCT proved safe and insightful in the setting of KD, with the potential to add diagnostic value in the assessment of coronary abnormalities in KD. The depicted coronary structural changes correspond to histological findings previously described in KD.

Multivariable assessment of the right ventricle by echocardiography in patients with repaired tetralogy of Fallot undergoing pulmonary valve replacement: a comparative study with magnetic resonance imaging.

BACKGROUND: Evaluation of the right ventricle (RV) using transthoracic echocardiography is challenging in patients with repaired tetralogy of Fallot (rTOF). AIMS: To evaluate the accuracy of conventional echocardiographic variables and real-time three-dimensional echocardiography (RT3DE) in assessing right ventricular (RV) volumes and function compared with magnetic resonance imaging (MRI), in adult patients with rTOF and referred for pulmonary valve replacement (PVR). METHODS: Complete echocardiography was performed on 26 consecutive patients referred for PVR, before and 1 year after surgery. All variables were compared with MRI. RESULTS: Correlations between conventional variables and MRI were absent or poor when assessing RV ejection fraction (RVEF), except for fractional area of change (FAC; r=0.70, P<0.01 before PVR; r=0.68, P<0.01 after PVR) and RT3DE (r=0.96, P<0.01 before PVR; r=0.98, P<0.01 after PVR). The RV volume correlation between RT3DE and MRI was excellent before and after surgery for RV end-diastolic volume (r=0.88, P<0.01 and r=0.91, P<0.01, respectively) and RV end-systolic volume (r=0.92, P<0.01 and r=0.95, P<0.01, respectively). The accuracy of these indices, as a diagnostic test for impaired RV (<45%), was good: Youden's indexes varied from 0.47 to 0.89; areas under the receiver operating characteristic curve before and after PVR were 0.86 and 0.81 for FAC and 0.98 and 0.97 for RT3DE, respectively. CONCLUSION: Commonly used echocardiography variables, such as tricuspid annular plane systolic excursion and tricuspid annular peak systolic velocity, did not sensitively evaluate global RVEF. A global approach, that includes the whole RV and integration of its different components, was more reliable in patients with rTOF.