Search

Key Clinical Message Systemic to pulmonary venous collaterals (SPVCs) are common findings in patients with Fontan and may be responsible for desaturation in this group of patients. In selected cases, percutaneous closure of SPVCs with coils or vascular plugs could be offered as a safe and effective treatment modality for these patients. Abstract A 27‐year‐old female patient who had operated primarily with Glenn operation and she underwent later a lateral tunnel Fontan operation. After 5 years, she presented with desaturation due to significant systemic to pulmonary venous collateral development, and a decision was made to proceed with interventional occlusion. Afterward, the arterial saturations increased.

Key Clinical Message Descending aorta to right atrial (RA) fistula is a rare and distinct clinical entity mimicking patent ductus arteriosus (PDA) and it may lead to rapid development of pulmonary vascular disease. Correct diagnosis and treatment, especially in the presence of other congenital heart defects, is very important. Interventional management is the treatment of choice. Abstract We present a case report of a trisomy 21 infant with atrial and ventricular septal defects and small patent ductus arteriosus (PDA) complicated by the presence of descending aorta to right atrial (RA) fistula with large left to right shunt leading to rapid increase in pulmonary vascular resistance. Transcatheter occlusion of the fistula followed by closure of the PDA with Nit‐Occlud coil systems led to decreased pulmonary pressure and resistance permitting successful surgical repair of the patient's intracardiac defects with good outcome over 3 years of follow‐up. Descending aorta to RA fistula is a rare and distinct clinical entity mimicking PDA and its correct diagnosis and treatment, especially in the presence of other congenital heart defects, is very important as it may lead to rapid development of pulmonary vascular disease.

Key Clinical Message Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital malformation and it needs surgical management. Consequences of this condition affect lead to pulmonary hypertension and severe pulmonary vascular disease. Abstract Anomalous origin of right pulmonary artery from the ascending aorta is a rare congenital heart malformation that results in early infant mortality affecting the right pulmonary artery more than the left. These patients are at risk for the early development of significant pulmonary hypertension. The surgical management during the early period of life is imperative.

The real-world implementation of heart failure (HF) guidelines remains unclear. Our present systematic review and meta-analysis aimed to examine the rate of HF patients receiving guideline-directed treatment to identify the proportion of under-treatment patients and those who are treated with optimal doses, to evaluate the correlation of under-treatment patients' characteristics with the prescribed therapy, and finally, to evaluate the combined effect of the above on incidental mortality and rehospitalization. We conducted a systematic review of the literature indexed in Medline. We screened 1224 papers and excluded 1166 as they did not meet the inclusion criteria. Of the remaining 58 papers, which were evaluated by studying the full text, 11 papers that referred to 45866 patients were finally studied in this work. Angiotensin-Converting-Enzyme Inhibitor (ACEI) and Angiotensin II-Receptor Blocker (ARB) use was estimated to be 80.9% (95% CI: 73.9%, 86.4%), β-blockers’ use was 78% (95% CI: 70.4%, 84.1%), Mineralocorticoid Receptor Antagonists' use was 47.4% (95% Cl 41.6%, 53.4%), and cardiac resynchronization therapy's use was 5.8% (95% Cl 3.4%, 9.6%). Meta-regression analysis showed that prescription of more than the half of target dose of ACEI/ARBs was found to be associated with reduced all-cause mortality (Z = −3.61, P = 0.0003), while the relationship with β-blockers was borderline (Z = −1.56, P = 0.11). A satisfactory adherence to the prescription of guideline-recommended treatment in patients with HF was observed. However, the under titration of the life-saving HF drugs need to be improved as only ultimate adherence to guideline-directed treatments may lead to the reduction of HF burden.

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.