Does interatrial communication affect post-operative course of children undergoing tetralogy of Fallot repair? Single centre retrospective cohort study: propensity score matching

Introduction: During tetralogy of Fallot repair, leaving or even create an interatrial communication may facilitate post-operative course particularly with right ventricle restrictive physiology. The aim of our study is to assess the influence of atrial communication on post-operative course of tetralogy of Fallot repair. Methods: Retrospectively, we studied all children who had tetralogy of Fallot repair (2003–2018). We divided them into two groups: tetralogy of Fallot repair with interatrial communication (TOFASD) group and tetralogy of Fallot repair with intact atrial septum (TOFIAS) group. We performed propensity match score for specific pre- or intra-operative variables and compared groups for post-operative outcome variables. Secondarily, we looked for right ventricle restrictive physiology incidence and influence of early repair performed before 3 months of age on post-operative course. Results: One hundred and sixty children underwent tetralogy of Fallot repair including (93) cases of TOFIAS (58%) and (67) cases of TOFASD (42%). With propensity matching score, 52 patients from each group were compared. Post-operative course was indifferent in term of positive pressure ventilation time, vasoactive inotropic score, creatinine and lactic acid levels, duration and amount of chest drainage and length of intensive care unit and hospital stay. Right ventricle restrictive physiology occurred in 38% of patients with no effects on outcome. 12/104 patients (12%) with early repair needed longer pressure ventilation time (p = 0.003) and intensive care unit stay (p = 0.02). Conclusion: Leaving interatrial communication in tetralogy of Fallot repair did not affect post-operative course. As well, right ventricle restrictive physiology did not affect post-operative course. Infants undergoing early tetralogy of Fallot repair may require longer duration of positive pressure ventilation time and intensive care unit stay.

The Importance of Multimodality Imaging in the Diagnosis and Management of Patients with Infiltrative Cardiomyopathies: An Update

Infiltrative cardiomyopathies (ICMs) comprise a broad spectrum of inherited and acquired conditions (mainly amyloidosis, sarcoidosis, and hemochromatosis), where the progressive buildup of abnormal substances within the myocardium results in left ventricular hypertrophy and manifests as restrictive physiology. Noninvasive multimodality imaging has gradually eliminated endomyocardial biopsy from the diagnostic workup of infiltrative cardiac deposition diseases. However, even with modern imaging techniques’ widespread availability, these pathologies persist in being largely under- or misdiagnosed. Considering the advent of novel, revolutionary pharmacotherapies for cardiac amyloidosis, the archetypal example of ICM, a standardized diagnostic approach is warranted. Therefore, this review aims to emphasize the importance of contemporary cardiac imaging in identifying specific ICM and improving outcomes via the prompt initiation of a targeted treatment.

P4165Influence of restrictive right ventricular physiology in outcomes after transcatheter pulmonary valve implantation in adult patients

Background A restrictive right ventricular (RV) physiology (r-RVP) is present in almost half of the patients with repaired tetralogy of Fallot (ToF), but its effect on later patients' clinical status is controversial. Purpose Evaluate the outcomes after transcatheter pulmonary valve implantation (TPVI) in patients with restrictive versus non-restrictive RV. Methods Single-center retrospective study of patients who underwent TPVI for pulmonary regurgitation between 2008 and 2017. Electrocardiogram, transthoracic echocardiogram, cardiac magnetic resonance and cardio-pulmonary exercise test were performed previous to TPVI and repeated 12 months after. For statistical analysis restrictive physiology was defined as the presence of end-diastolic forward flow (EDFF) and RVEDVi <150ml/m2. Results Fifty-nine patients, 55 with tetralogy of Fallot, were included. R-RVP was present in 23 patients (39.0%). At the baseline, the majority of the patients were symptomatic, in NYHA functional class II–III (n=48, 81.4%) and with reduced cardiopulmonary performance (VO2max = 20.5mL/min/kg), both worse among patients with r-RVP (p=0.018 and p=0.018). During a mean follow-up of 446.4±226.1 days after TPVI one patient died. BNP levels were not significantly different from baseline. Despite self-reported functional capacity and exercise capacity improvement after TPVI (p<0.001), all parameters remained lower in r-RVP group. One year after TPVI, pulmonary and tricuspid regurgitation decrease significantly, as well as RVEDV, in both groups. However, RV systolic function remained unchanged in both groups. Regarding to the dysrhythmic profile, all patients with need for an implantable cardiac defibrillator and seven of the eight patients requiring radiofrequency ablation for sustained supraventricular arrhythmias during follow-up were in the group without r-RVP (p=0.038). Kaplan-Meier survival curve Conclusion TPVI had a positive impact in clinical status in a cohort of patients with predominant PR regardless the presence of restrictive physiology. Moreover, besides higher VO2max and anaerobic threshold in the non-restrictive group, the magnitude of the improvement was not statistically different between the two groups. Nevertheless, in patients without r-RVP, a peculiar caution has to be focused regarding rhythmic issue during follow-up, despite the TPVI.

The genetics of interstitial lung diseases

Interstitial lung diseases (ILDs) are a set of heterogeneous lung diseases characterised by inflammation and, in some cases, fibrosis. These lung conditions lead to dyspnoea, cough, abnormalities in gas exchange, restrictive physiology (characterised by decreased lung volumes), hypoxaemia and, if progressive, respiratory failure. In some cases, ILDs can be caused by systemic diseases or environmental exposures. The ability to treat or cure these ILDs varies based on the subtype and in many cases lung transplantation remains the only curative therapy. There is a growing body of evidence that both common and rare genetic variants contribute to the development and clinical manifestation of many of the ILDs. Here, we review the current understanding of genetic risk and ILD.