Total anomalous pulmonary venous connection: Outcome of postoperative pulmonary venous obstruction

ObjectivePulmonary venous obstruction (PVO) is an important cause of late mortality in total anomalous pulmonary venous connection (TAPVC). We aimed to describe current practices for the management of postoperative PVO and the efficacy of the different interventional procedures.MethodsWe conducted a retrospective international collaborative population-based study involving 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. Patients with TAPVC born between January 1, 1998, and December 31, 2004, were identified. Patients with functionally univentricular circulation or atrial isomerism were excluded. All available data and images were reviewed.ResultsOf 406 patients undergoing repair of TAPVC, 71 (17.5%) had postoperative PVO. The diagnosis was made within 6 months of surgery in 59 (83%) of the 71 patients. In 12, serial imaging documented change in appearance of the pulmonary veins. Good-sized pulmonary veins can progress to diffusely small veins and rarely atresia. Patients presenting after 6 months had less severe disease; all are alive at most recent follow-up. Fifty-six (13.8%) of 406 patients underwent intervention for postoperative PVO: 44 had surgical treatment and 12 had an initial catheter intervention. One half underwent 1 or more reinterventions. Three-year survival for patients with postoperative PVO was 58.7% (95% confidence intervals, 46.2%-69.2%) with a trend that those having a surgical strategy did better (P = .083). Risk factors for death included earlier presentation after TAPVC repair, diffusely small pulmonary veins at presentation of postoperative PVO, and an increased number of lung segments affected by obstruction.ConclusionsPostoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur

Evidence of support used for drug treatments in pediatric cardiology

Background and aims: Clinical support systems are widely used in pediatric care. The aim of this study was to assess the support for drug treatments used at pediatric cardiac wards and intensive care units in Sweden. Methods: Drug information, such as type of drug, indication, dose, and route of administration, for all in-hospital pediatric cardiac patients, was included in the study. Treatments were classified as either on-label (based on product information) or off-label. Support for off-label treatment was stratified by the use of clinical support systems (the national database on drugs, local, or other clinical experience guidelines). Results: In all, 28 patients were included in the study. The total number of drug treatments was 233, encompassing 65 different drugs. Overall, 175 (75%) treatments were off-label. A majority of off-label drug treatments were supported by other sources of information shared by experts. A total of 7% of the drug treatments were used without support. Conclusion:  Off-label drug treatment is still common in Swedish pediatric cardiac care. However, the majority of treatments were supported by the experience shared in clinical support systems. Key Points: Seventy-five percent of all prescriptions in pediatric cardiology care were off-label. A majority of patients received three or more drug treatments off-label. Use of clinical support systems and guidelines was common, but in 7% of all drug treatments, no support was found for the chosen treatment

COVID-19 FAQs in paediatric and congenital cardiology: AEPC position paper

The COVID-19 pandemic has had a huge influence in almost all areas of life, affecting societies, economics, and health care systems worldwide. The paediatric cardiology community is no exception. As the challenging battle with COVID-19 continues, professionals from the Association for the European Paediatric and Congenital Cardiology receive many questions regarding COVID-19 in a Paediatric and Congenital Cardiology setting. The aim of this paper is to present the AEPC position on frequently asked questions based on the most recent scientific data, as well as to frame a discussion on how to take care of our patients during this unprecedented crisis. As the times are changing quickly and information regarding COVID-19 is very dynamic, continuous collection of evidence will help guide constructive decision-making

Comprehensive Characterization of Arterial and Cardiac Function in Marfan Syndrome—Can Biomarkers Help Improve Outcome?

Background: Marfan Syndrome (MFS) has been associated with increased aortic stiffness and left ventricular dysfunction. The latter may be due to the underlying genotype and/or secondary to aortic stiffening (vascular-ventricular interaction). The aim of this study was to characterize arterial and cardiac function in MFS using a multimodal approach. Methods: Prospective observational study of MFS patients and healthy controls. Methods included echocardiography, ascending aortic distensibility, common carotid intima media thickness [cIMT], parameters of wave reflection, carotid-femoral pulse wave velocity [cfPWV]), reactive hyperemia index [RHI], and biomarker analysis (Olink, CVII panel). Results: We included 20 patients with MFS and 67 controls. Ascending aortic distensibility, cIMT and RHI were decreased, while all parameters of arterial wave reflection, stiffness and BNP levels were increased in the MFS group. Both systolic and diastolic function were impaired relative to controls. Within the MFS group, no significant correlation between arterial and cardiac function was identified. However, cfPWV correlated significantly with indexed left ventricular mass and volume in MFS. Bran natriuretic peptide (BNP) was the only biomarker significantly elevated in MFS following correction for age and sex. Conclusions: MFS patients have generally increased aortic stiffness, endothelial dysfunction and BNP levels while cIMT is decreased, supporting that the mechanism of general stiffening is different from acquired vascular disease. CfPWV is associated with cardiac size, blood pressure and BNP in MFS patients. These may be early markers of disease progression that are suitable for monitoring pharmacological treatment effects in MFS patients

Survey of 23 Nordic university hospitals showed that 77% lacked written procedures for measuring and interpreting blood pressure in infants

Aim This study determined the use of standardised procedures for infant noninvasive blood pressure (NIBP) measurements in the Nordic countries and aimed to identify factors included in the standardisation and interpretation of NIBP measurements in infants. Methods A cross-sectional electronic questionnaire survey was sent to 84 physicians in all 23 university hospitals in Sweden, Norway, Denmark, Finland and Iceland and was completed from February to March 2017. The survey contained respondent characteristics, the presence and description of standardised procedures for NIBP measurements, daily practice of NIBP measurements and methodological considerations and interpretation of NIBP measurements in a healthy six-month-old child. Results We received responses from 55 of 84 physicians working in all 23 Nordic university hospitals, in paediatric cardiology (n = 22), general paediatrics (n = 16), paediatric nephrology (n = 14) and other fields (n = 3). Less than a quarter (23%) said their hospital issued specific NIBP procedures relating to infants and they referred to 19 different sources of information. The factors that were most commonly assessed for interpretation were age (100%), arousal state (78%) and cuff size (76%). Conclusion Most of the university hospital units treating children lacked age-specific written procedures for measuring and interpreting infant NIBP, and there is a strong need for common Nordic guidelines.Peer reviewe

Patient empowerment and its correlates in young persons with congenital heart disease

OBJECTIVE: The objective of this study was to measure the level of empowerment and identify its correlates in young persons with congenital heart disease. STUDY DESIGN: Patients aged 14-18 years with congenital heart disease, and under active follow-up in one of four paediatric cardiology centres in Sweden were invited to participate in a cross-sectional study. A total of 202 young persons returned the questionnaires. Patient empowerment was measured with the Gothenburg Young Persons Empowerment Scale that allows the calculation of total and subscale scores. Univariate and multivariate linear regression analyses were undertaken to analyse possible correlates, including: sex, age, health behaviours, knowledge of congenital heart disease, quality of life, patient-reported health, congenital heart disease complexity, transition readiness and illness perception. RESULTS: The mean empowerment score was 54.6±10.6 (scale of 15-75). Univariate analyses showed that empowerment was associated with age, quality of life, transition readiness, illness perception, health behaviours and patient-reported health (perceived physical appearance, treatment anxiety, cognitive problems and communication issues). However, multivariable linear regression analyses identified that only transition readiness (β=0.28, P<0.001) and communication (β=0.36, P<0.001) had a positive association with patient empowerment. These variables were also significantly associated with the subscale scores of the empowerment scale of knowledge and understanding ( P<0.001), shared decision-making ( P<0.001) and enabling others ( P<0.01). The overall models' explained variance ranged from 8% to 37%. CONCLUSION: Patient empowerment was associated with transition readiness and fewer problems communicating. While it is not possible to establish the directionality of the associations, interventions looking to increase empowerment could benefit from using these variables (or measurements) for evaluation purposes.status: publishe