Conceptualization and teaching health advocacy in undergraduate medical education: a document analysis.

Background: Health advocacy is considered to be a core competence for physicians, but it remains unclear how the health advocacy role, despite being described in overarching competency frameworks, is operationalized in undergraduate medical education (UME). This study aimed to identify how health advocacy is conceptualized and taught in undergraduate medical curricula.

Methods: We performed a qualitative analysis of curriculum documents from all eight medical schools in the Netherlands, all of which offered competency-based UME.

N-terminal pro-brain natriuretic peptide serum levels reflect attrition of the Fontan circulation.

Objective: N-terminal pro-brain natriuretic peptide has an established role in the diagnosis and prognosis of heart failure. In Fontan patients, this peptide is often increased, but its diagnostic value in this particular non-physiologic, univentricular circulation is unclear. We investigated whether N-terminal pro-brain natriuretic peptide represents ventricular function or other key variables in Fontan patients.

GDF-15 (Growth Differentiation Factor 15) Is Associated With Hospitalization and Mortality in Patients With a Fontan Circulation.

Background We investigated serial serum levels of GDF-15 (growth differentiation factor 15) in Fontan patients and their relation to outcome. Methods and Results In this single-center prospective study of consecutive Fontan patients, serial serum GDF-15 measurement and clinical assessment was done at baseline (n=81) and after 2 years (n=51). The association between GDF-15 and the combined end point of all-cause mortality, heart transplant listing, and Fontan-related hospitalization was investigated.

Serial cardiovascular magnetic resonance feature tracking indicates early worsening of cardiac function in Fontan patients.

Background: In Fontan patients, attrition of ventricular function is well recognized, but early detection of ventricular dysfunction is difficult. The aim of this study is to longitudinally assess ventricular strain in Fontan patients using a new method for cardiac magnetic resonance (CMR) feature tracking, and to investigate the relationship between ventricular strain and cardiac systolic function.

Methods And Results: In this prospective, standardized follow-up study in 51 Fontan patients, age ≥ 10 years, CMR and concomitant clinical assessment was done at the start of the study and after 2 years.

[Use of cannabidiol oil in children].

Useof cannabidiol oil in children The use of cannabidioloil (CBD oil), a cannabis-derived chemical, is increasing. CBD oil is freely available in the Netherlands, but its composition and quality are not monitored. However, the alternative, pharmacist-prepared oil, is more expensive and difficult to acquire.

Quality of life and sexual well-being in patients with a Fontan circulation: An explorative pilot study with a mixed method design.

Objective: To get an impression of the quality of life (QOL) and sexual well-being in the Fontan population, and to generate hypotheses for future research.

Methods: For this cross-sectional pilot study, questionnaires regarding health-related QOL, sexual function and fertility/pregnancy were completed by 21 patients with a Fontan circulation >16 years old, followed at the University Medical Center Groningen, the Netherlands. Semi-structured qualitative interviews were conducted in 8 patients.

Fontan Circulation over Time.

The unique, unphysiological Fontan circulation is associated with an impaired functional status of the patients that is suggested to deteriorate over time. Unfortunately, previous studies did not integrate both pulmonary and cardiac determinants of functional status. In addition, a comparison with the natural decrease in exercise capacity in healthy subjects (in both children and adults) is lacking.

Diminished liver microperfusion in Fontan patients: A biexponential DWI study.

It has been demonstrated that hepatic apparent diffusion coefficients (ADC) are decreasing in patients with a Fontan circulation. It remains however unclear whether this is a true decrease of molecular diffusion, or rather reflects decreased microperfusion due to decreased portal blood flow. The purpose of this study was therefore to differentiate diffusion and microperfusion using intravoxel incoherent motion (IVIM) modeled diffusion-weighted imaging (DWI) for different liver segments in patients with a Fontan circulation, compare to a control group, and relate with liver function, chronic hepatic congestion and hepatic disease.

Surgical options after Fontan failure.

Objective: The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX.

Methods: A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%).

The Fontan circulation and the liver: A magnetic resonance diffusion-weighted imaging study.

Background: Patients with a Fontan circulation tend to develop liver fibrosis, liver cirrhosis and even hepatocellular carcinoma. The aim of this study is to use the magnetic resonance technique diffusing-weighted imaging (DWI) for detecting liver fibrosis/cirrhosis in Fontan patients and to establish whether DWI results are associated with functional aspects of the Fontan circulation.

Methods: In a cross-sectional study, 59 Fontan patients were evaluated by liver DWI.

Adverse pulmonary vascular remodeling in the Fontan circulation.

Background: The Fontan circulation is a palliation for patients with a functionally univentricular heart. It is characterized by gradual attrition over time. An increase in pulmonary vascular resistance could be a key factor in the long-term failure of the Fontan circulation.

Favourable mid-term outcome after heart transplantation for late Fontan failure.

Objectives: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF.

Methods: Twenty-year multi-institutional retrospective analysis on OHT for FF.

Trends in mortality (1975-2011) after one- and two-stage Fontan surgery, including bidirectional Glenn through Fontan completion.

Objectives: Techniques and strategies in the Fontan procedure have evolved over the years, including the evolution from a one- to two-stage procedure. With such adjustments made, attention should shift towards survival analysis, including bidirectional cavopulmonary shunt (BCPS) and interstage mortality. The purpose of this study was to investigate the trends in the overall mortality of all patients who underwent a BCPS or one-stage Fontan procedure at our institution in the period of 1975-2011.