The Adult Congenital Heart Disease Anatomic and Physiological Classification: Associations with Clinical Outcomes in Patients with Atrial Arrhythmias.

The implications of the adult congenital heart disease anatomic and physiological classification (AP-ACHD) for risk assessment have not been adequately studied. A retrospective cohort study was conducted using data from an ongoing national, multicentre registry of patients with ACHD and atrial arrhythmias (AA) receiving apixaban (PROTECT-AR study, NCT03854149). At enrollment, patients were stratified according to Anatomic class (AnatC, range I to III) and physiological stage (PhyS, range B to D). A follow-up was conducted between May 2019 and September 2021. The primary outcome was a composite of death from any cause, any major thromboembolic event, major or clinically relevant non-major bleeding, or hospitalization. Cox proportional-hazards regression modeling was used to evaluate the risks for the outcome among AP-ACHD classes. Over a median 20-month follow-up period, 47 of 157 (29.9%) ACHD patients with AA experienced the composite outcome. Adjusted hazard ratios (aHR) with 95% confidence intervals (CI) for the outcome in PhyS C and PhyS D were 1.79 (95% CI 0.69 to 4.67) and 8.15 (95% CI 1.52 to 43.59), respectively, as compared with PhyS B. The corresponding aHRs in AnatC II and AnatC III were 1.12 (95% CI 0.37 to 3.41) and 1.06 (95% CI 0.24 to 4.63), respectively, as compared with AnatC I. In conclusion, the PhyS component of the AP-ACHD classification was an independent predictor of net adverse clinical events among ACHD patients with AA.

Rationale and design of a prospective, observational, multicentre study on the safety and efficacy of apixaban for the prevention of thromboembolism in adults with congenital heart disease and atrial arrhythmias: the PROTECT-AR study.

INTRODUCTION: The risk for stroke in adults with congenital heart disease (ACHD) is increased, especially in the setting of commonly ensuing atrial arrhythmias (AA), namely atrial fibrillation, atrial flutter or intra-atrial re-entrant tachycardia. Data are limited regarding treatment with non-vitamin K oral anticoagulants in long-term studies involving patients with ACHD and AA. METHODS AND ANALYSIS: PReventiOn of ThromboEmbolism in Adults with Congenital HearΤ disease and Atrial aRrhythmias is a prospective, multicenter, single-arm, non-interventional cohort study designed to investigate the safety and efficacy of apixaban for the prevention of thromboembolism in ACHD with AA in a 'real-world' setting. Eligible patients will be evaluated by the means of available registries and clinical counter. The study aims to accumulate approximately 500 patient-years of exposure to apixaban as part of routine care. Enrolment will take place at four ACHD centres in Greece. The first patient was enrolled in July 2019. The primary efficacy endpoint is a composite of stroke, systemic or pulmonary embolism and intracardiac thrombosis. The primary safety endpoint is major bleeding, according to the International Society on Thrombosis and Haemostasis bleeding criteria. ETHICS AND DISSEMINATION: The study protocol has been approved by the institutional review board/independent ethics committee at each site prior to study commencement. All patients will provide written informed consent. Results will be disseminated at scientific meetings and published in peer-reviewed journals. TRIAL REGISTRATION NUMBER: NCT03854149; Pre-results.

Levoatriocardinal Vein: A Rarely Recognized Cause of Recurrent Cardiac and Cerebral Thromboembolic Events.

Levoatriocardinal vein (LACV) is a rare congenital anomaly of the systemic veins. We describe the case of a 43-year-old patient who presented with a transient ischemic attack episode, desaturation, and ventricular arrhythmias. Transesophageal echocardiography diagnosed a patent foramen ovale, and magnetic resonance imaging revealed 2 small myocardial infarcts, despite normal coronaries on subsequent angiography. Echocardiography and cardiac computed tomography revealed an LACV with bidirectional flow. The patient underwent percutaneous LACV and patent foramen ovale closure, with immediate saturation increase after occlusion of the LACV. To the best of our knowledge, this is the first time an LACV is identified as the cause of recurrent thromboembolic events.

Severe thrombocytopenia after heparin therapy in a patient with unstable angina and recent stent implantation.

Heparin-induced thrombocytopenia represents a serious side effect of heparin therapy. Immune-mediated platelet activation results in thrombocytopenia, endothelial thrombin release and development of thrombosis, mainly venous. We report the case of a man with a history of coronary artery disease and recent stent implantation. This patient developed severe heparin-induced thrombocytopenia type II after low molecular weight heparin administration because of unstable angina which occurred two months after stent implantation. The patient was treated with a new anticoagulant regimen, fondaparinux sodium. There were no complications and platelet counts were restored to normal levels.