Sinus venosus atrial septal defect (SVASD) accounts for 5-10% of all ASDs. Partial anomalous pulmonary venous connection (PAPVC) and pulmonary hypertension (PH) in patients with SVASD increases the risk for surgical or transcatheter intervention. Closure is preferred over medical therapy. Transcatheter closure is challenging due to complex anatomy, difficulty in evaluating suitability for intervention, and lack of dedicated devices. We present a systematic approach using multimodality imaging and 3D printing for transcatheter closure of superior SVASD with PAPVC using a custom-made CP Stent®. A 53-year-old with history of drug abuse presented with NYHA Class III symptoms, hypoxemia, atrial flutter, and PH (MPAP of 30 mmHg) requiring multidrug therapy. Echocardiogram raised suspicion of superior SVASD, confirmed by cardiac CT and CMR. Multidisciplinary team ruled out surgery due to high risk and recommended transcatheter closure, if feasible. CT and CMR demonstrated a 20 × 18 mm superior SVASD with PAPVC: right upper (RUPV) and middle pulmonary veins draining into the superior vena cava (SVC). To understand spatial relationships, a 3D model was printed with 1.5-mm transparent and flexible walls to deploy a nonsterile prototype of the stent in the SVC. Rotational angiogram was performed to test feasibility of in-vivo deployment in view of stent foreshortening (from 110 to 70 mm) and differential widening of the SVC: 23 mm at the junction of the innominate vein; 35 mm at the atrial end. Transcatheter closure of superior SVASD with PAPVC in patients with PH is feasible in suitable cases using a systematic approach with multi-modality imaging and 3D printing.A 53-year-old presented to the emergency department with NYHA Class III symptoms, hypoxemia, new onset atrial flutter, ventricular dysfunction, and pulmonary hypertension (PH). Echocardiogram with agitated saline raised suspicion of superior sinus venous atrial septal defect (SVASD) with venous return directly entering the left atrium (LA) and
The use of US Food and Drug Administration (FDA)‐approved drugs for the treatment of an unapproved indication or in an unapproved age group, or at doses or route of administration not indicated on the label is known as off‐label use. Off‐label use may be beneficial in circumstances when the standard‐of‐care treatment has failed, and/or no other FDA‐approved medications are available for a particular condition. In pediatric patients, off‐label use may increase the risk of adverse events as pharmacokinetic and pharmacodynamic data are limited in children. Approximately 73% of off‐label drugs currently prescribed for various conditions do not have sufficient scientific evidence for safety and efficacy. For example, β‐blockers are a class of drugs with FDA‐approval for very few indications in pediatrics but are commonly used for various off‐label indications. Interestingly, the proportion of off‐label use of β‐blockers in adults is at about 52% (66.2 million) of the total number of β‐blockers prescribed. The frequency of off‐label use of β‐blockers in children is also high with limited data on the indications as well as safety and efficacy. We present trends in off‐label use of β‐blockers in children to discuss drug safety and efficacy and include recommendations for pediatric providers.
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