Left Ventricular Function by Pressure‐Volume Loop Analysis before and after Percutaneous Repair of Large Atrial Septal Defects

Aim The intent of the present study was to evaluate changes in ventricular function with percutaneous closure of atrial septal defect (ASD), as it is associated with alterations in ventricular loading and function. Transcatheter occlusion of ASD imparts acute changes in volume loading of the left ventricle (LV) that obscures measurement of ventricular function by load‐dependent indices. To differentiate between changes in ventricular loading and function, load‐independent indices of ventricular function must be utilized . Methods During transcatheter occlusion of ASD, subjects underwent measurement of LV pressure and volume by the conductance catheter method. Load‐dependent indices of ventricular function included: systolic and diastolic pressures, +dP/dt max , and −dP/dt max . Load‐independent indices included: elastance and tau, the preload‐independent time constant ofisovolumic relaxation. To obtain elastance, afterload was augmented by phenylephrine bolus pre‐ and post‐device occlusion . Results In total, 29 patients (age 2–79 years) underwent ASD device occlusion (device size 12–38 mm, median 28 mm). Load‐dependent indices were obtained in all, and satisfactory pressure‐volume loops in 11. At baseline, LV end‐diastolic pressure was 5–23 mmHg (13 ± 5 mmHg) and tau was 31 ± 6 ms. Postclosure of the ASD, LV systolic and diastolic pressures rose by 10 ± 11 mmHg and 5 ± 3 mmHg, respectively (P < 0.05), and +dP/dt max rose from 1,288 ± 313 mmHg/sec to 1,415 ± 465 mmHg/sec (P < 0.05), but −dP/dt max was unchanged. Elastance significantly improved (9.4 ± 8.3 mmHg/mL vs. 13.0 ± 7.3 mmHg/mL, P < 0.05) and tau was unchanged . Conclusions Transcatheter occlusion of ASD is associated with acute improvement in load‐independent indices of systolic function in this cohort, without significant worsening of the preload‐independent index of diastolic function . (J Interven Cardiol 2014;27:204–211)Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/106889/1/joic334.pd

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

Acute Cardiovascular Manifestations in 286 Children With Multisystem Inflammatory Syndrome Associated With COVID-19 Infection in Europe

Background: The aim of the study was to document cardiovascular clinical findings, cardiac imaging, and laboratory markers in children presenting with the novel multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) infection. Methods: This real-time internet-based survey has been endorsed by the Association for European Paediatric and Congenital Cardiologists Working Groups for Cardiac Imaging and Cardiovascular Intensive Care. Children 0 to 18 years of age admitted to a hospital between February 1 and June 6, 2020, with a diagnosis of an inflammatory syndrome and acute cardiovascular complications were included. Results: A total of 286 children from 55 centers in 17 European countries were included. The median age was 8.4 years (interquartile range, 3.8-12.4 years) and 67% were boys. The most common cardiovascular complications were shock, cardiac arrhythmias, pericardial effusion, and coronary artery dilatation. Reduced left ventricular ejection fraction was present in over half of the patients, and a vast majority of children had raised cardiac troponin when checked. The biochemical markers of inflammation were raised in most patients on admission: elevated C-reactive protein, serum ferritin, procalcitonin, N-terminal pro B-type natriuretic peptide, interleukin-6 level, and D-dimers. There was a statistically significant correlation between degree of elevation in cardiac and biochemical parameters and the need for intensive care support (P<0.05). Polymerase chain reaction for severe acute respiratory syndrome coronavirus 2 was positive in 33.6%, whereas immunoglobulin M and immunoglobulin G antibodies were positive in 15.7% cases and immunoglobulin G in 43.6% cases, respectively, when checked. One child in the study cohort died. Conclusions: Cardiac involvement is common in children with multisystem inflammatory syndrome associated with the Covid-19 pandemic. The majority of children have significantly raised levels of N-terminal pro B-type natriuretic peptide, ferritin, D-dimers, and cardiac troponin in addition to high C-reactive protein and procalcitonin levels. In comparison with adults with COVID-19, mortality in children with multisystem inflammatory syndrome associated with COVID-19 is uncommon despite multisystem involvement, very elevated inflammatory markers, and the need for intensive care support.info:eu-repo/semantics/publishedVersio

Paediatric and adult congenital cardiology education and training in Europe

Background: Limited data exist on training of European paediatric and adult congenital cardiologists. Methods: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. Results: Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87-9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63-10.72 million), and one training centre per 4.29 million population (range 1.63-10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1-17), and duration of training was 3 years (range 2-5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R-2 = 0.41). Conclusion: Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.Developmen

Reference intervals for the echocardiographic measurements of the right heart in children and adolescents: a systematic review

BACKGROUND: Transthoracic echocardiography is the primary imaging modality for the diagnosis of right ventricular (RV) involvement in congenital and acquired heart diseases. There is increasing recognition of the contribution of RV dysfunction in heart diseases affecting children and adolescents, but there is insufficient information on reference intervals for the echocardiographic measurements of the right heart in children and adolescents that represent all the continental populations of the world.OBJECTIVE:The aim of this systematic review was to collate, from published studies, normative data for echocardiographic evaluation of the right heart in children and adolescents, and to identify gaps in knowledge in this field especially with respect to sub-Saharan Africans. METHODS: We performed a systematic literature search to identify studies of reference intervals for right heart measurements as determined by transthoracic echocardiography in healthy children and adolescents of school-going age. Articles were retrieved from electronic databases with a combination of search terms from the earliest date available until May 2013. RESULTS: Reference data were available for a broad range of variables. Fifty one studies out of 3096 publications were included. The sample sizes of the reference populations ranged from 13 to 2036 with ages varying from 5 to 21 years. We identified areas lacking sufficient reference data. These included reference data for determining right atrial size, tricuspid valve area, RV dimensions and areas, the RV % fractional area change, pulmonary artery pressure gradients and the right-sided haemodynamics, including the inferior vena cava dimensions and collapsibility. There were no data for sub-Saharan African children and adolescents. CONCLUSION: Reliable reference data are lacking for important echocardiographic measurements of the RV in children and adolescents, especially for sub-Saharan Africans

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

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