Bennett P. Samuel

Pulmonary arterial hypertension (PAH) is a severe and progressive disease. Treatment options include anti-PAH medications, continuous intravenous therapies, and diuretics. Lung transplant is required in many cases. Atrial septostomy is an under recognized option in symptomatic patients on maximal PAH therapy. However, creating a sustainable and restrictive atrial communication is challenging with existing devices. We describe emergency use of the Occlutech^® Atrial Flow Regulator, a novel device, in a 35-year-old female with PAH supported on veno-arterial extracorporeal membrane oxygenation after postpartum decompensation.

Fontan fenestration allows right-to-left shunting increasing cardiac output and oxygen delivery. Increased shunting occurs as cardiac function and ventricular end-diastolic pressures improve, potentially decreasing oxygen saturation. Complete closure may result in impaired Fontan haemodynamics and low cardiac output; however, there are no dedicated devices to reduce fenestration size. We describe Fontan fenestration size reduction using the Atrial Flow Regulator.

Beginning with the discovery of X-rays to the development of three-dimensional (3D) imaging, improvements in acquisition, post-processing, and visualization have provided clinicians with detailed information for increasingly accurate medical diagnosis and clinical management. This paper highlights advances in imaging technologies for congenital heart disease (CHD), medical adoption, and future developments required to improve pre-procedural and intra-procedural guidance.

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.

Surgical management of complex congenital heart disease (CHD) is challenging. Three‐dimensional (3D) printing can improve multidisciplinary team decision‐making, patient and family understanding, and education of medical professionals. We describe 3D printing for surgical management of five patients with complex CHD. The anatomical details of the 3D printed models were instrumental in planning surgical techniques especially in determining between single ventricle, 1.5 ventricle, and biventricular repair.

Article Citation: Vishal Kaley, Nagib Dahdah, Amal El-Sisi, Jochen Grohmann, Eric Rosenthal, Matthew Jones, Gareth Morgan, Nicholas Hayes, Ashish Shah, Cemsit Karakurt, Masood Sadiq, Matthias Sigler, Hans Figulla, Michael Becker, Nikolaus Haas, Eustaquio Onorato, Angel Puentes Rico, Supaporn Roymanee, Anselm Uebing, Walter Wiebe, Bennett Samuel, Ziyad Hijazi, and Joseph Vettukattil (2019) Atrial Septal Defect–Associated Pulmonary Hypertension: Outcomes of Closure With a Fenestrated Device. Advances in Pulmonary Hypertension: 2019, Vol. 18, No. 1, pp. 4-9. https://doi.org/10.21693/1933-088X-18.1.4

Background Physician burnout has many undesirable consequences, including negative impact on patient care delivery and physician career satisfaction. Electronic health records (EHRs) may exacerbate burnout by increasing physician workload. Objective To determine burnout in adult congenital heart disease (ACHD) specialists by assessing stress associated with EHRs. Design Electronic survey study of ACHD providers. Setting Canada and United States. Participants Three hundred eighty‐three ACHD specialists listed on the Adult Congenital Heart Association directory between February and April 2017. Outcome Measures Burnout was measured using the Maslach Burnout Inventory (MBI) to understand factors contributing to work life and EHR satisfaction. Chi‐square and Wilcoxon Rank Sum tests were used for statistical analysis. Results Of the 383 invited participants, 110 (28.7%) completed surveys with the majority (n = 88, 80.7%) reporting from an academic medical center. Burnout was defined as high scores on the emotional exhaustion and/or depersonalization MBI subscales. When comparing the 40% (n = 44) that met criteria for burnout with those that did not, there was strong disagreement that a reasonable amount of time is spent on clerical tasks related to direct (P = .0043) or indirect (P = .0004) patient care. There was strong disagreement that EHRs increased efficiency (P = .006) or the patient portal improved patient care (P = .0215). Finally, physicians who met criteria for burnout had lower personal accomplishment scores (P = .0355). Conclusions Our results suggest time spent on EHRs creates clerical burden exacerbating ACHD physician burnout. The high levels of emotional exhaustion may decrease quality of ACHD care by directing focus away from physician‐patient interaction. Health care systems must develop best practice for EHR design and implementation to optimize patient advocacy and care, and decrease physician burnout.