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- Prevalence, Management, and Outcomes of Atrial Fibrillation in Paediatric Patients: Insights from a Tertiary Cardiology Centre.Publication . Constante, Andreia Duarte; Suarez, Joana; Lourenço, Guilherme; Portugal, Guilherme; Silva Cunha, Pedro; Martins Oliveira, Mário; Trigo, Conceição; Pinto, Fátima; Laranjo, SérgioAtrial fibrillation (AF) is increasingly recognised in paediatric patients, presenting unique challenges in management due to its association with various underlying heart conditions. This study aimed to evaluate the prevalence, management strategies, and outcomes of AF in this population. : A retrospective analysis was conducted at a tertiary paediatric cardiology centre, including patients aged ≤18 years diagnosed with AF between January 2015 and December 2023. The study focused on demographic details, clinical presentations, treatments, and outcomes. Descriptive statistics were employed to assess treatment efficacy, recurrence rates, and complications. : The study included 36 paediatric patients (median age: 15 years, IQR: 13-17; 58% male). Of these, 52.8% had acquired heart disease, 16.7% had congenital heart anomalies, and 16.7% presented with lone AF. The initial management strategies involved electrical cardioversion in 53.3% of patients and pharmacological conversion with amiodarone in 46.7%. Rhythm control therapy was administered to over 80% of the cohort, and 63.9% were placed on oral anticoagulation, predominantly for rheumatic and congenital heart diseases. The overall success rate of rhythm control was 96.2%, with an AF recurrence rate of 3.8%. Ischemic stroke was the most common complication, occurring in three patients, all with underlying rheumatic heart disease. : AF in paediatric patients is predominantly associated with rheumatic and congenital heart diseases, though a significant proportion of patients present with lone AF. Despite effective rhythm control in most cases, neurological complications, particularly ischemic strokes in patients with underlying heart disease, remain a critical concern. These findings underscore the need for more comprehensive studies to better understand the aetiology, risk factors, and optimal management strategies for paediatric AF.
- Cardiomyopathy. Like Mother, Like Daughter? A New Phenotype for a MYH7 MutationPublication . Abreu, S; Trigo, C; Pinto, MFWe report the case of a six-year-old girl referred to our department due to her mother's diagnosis of hypertrophic cardiomyopathy. Initial evaluation revealed restrictive physiology on echocardiography and cardiac catheterization. Genetic analysis of both mother and daughter revealed the same genetic variant at the MYH7 gene. Notably, although the genetic mutation was the same, the phenotype differed, resulting in different management and prognosis. The daughter received a heart transplant at the age of 20 due to pulmonary hypertension's early development, whereas the mother was under heart failure medical therapy. This case relevance lies in this specific mutation, which to our knowledge, has not been previously associated with restrictive phenotype, but also on the importance of early referral of family members.
- Acute Cardiovascular Manifestations in 286 Children With Multisystem Inflammatory Syndrome Associated With COVID-19 Infection in EuropePublication . Valverde, I; Singh, Y; Sanchez-de-Toledo, J; Theocharis, P; Chikermane, A; Di Filippo, S; Kuciñska, B; Mannarino, S; Tamariz-Martel, A; Gutierrez-Larraya, F; Soda, G; Vandekerckhove, K; Gonzalez-Barlatay, F; McMahon, C; Marcora, S; Napoleone, C; Duong, P; Tuo, G; Deri, A; Nepali, G; Ilina, M; Ciliberti, P; Miller, O; Iriart, X; Hubrechts, J; Kuipers, I; Sousa, A; Donti, A; Sharpe, A; Reinhardt, Z; Cairello, F; De Wolf, D; Vieira, M; Lazea, C; Gran, F; Medrano-Lopez, C; Ortiz-Garrido, A; Vukomanovic, V; Brent, B; Milanesi, O; Dewals, W; Manso, B; Valsangiacomo-Buchel, E; Francisco, A; Seghaye, MC; Loeckx, I; Rodriguez-Gonzalez, M; ReyGarcía, S; Ziesenitz, V; Bordin, G; Doros, G; Grangl, G; Fadl, S; Perminow, K; Centeno, F; Pinto, F; Niemelä, J; Kanthimathinathan, H; Randanne, P; Niszczota, C; Zuccotti, G; Gordillo, I; Obeyasekhara, M; Armstrong, C; Butler, K; Ciuffreda, M; Villar, AM; Pappula, N; Caorsi, R; Singh, D; Durairaj, S; McLeod, K; Calcagni, G; Quizad, Y; Gewillig, M; Kuijpers, T; Ataide, R; Fabi, M; Bharucha, T; Abbas, K; Magrass, S; Wong, J; Iacob, D; Balcells, J; GilVillanueva, N; Cuenca-Peiro, V; Cerovi, I; Sarfatt, A; Zaqout, M; Sanchez-Valderrabanos, E; Kelly-Geyer, J; Diogo, F; Cajgfinger, N; Françoise, M; Rueda-Nuñez, F; Gorenflo, M; Grison, A; Mihailov, D; Koestenberger, M; Alcalde, C; Trigo, C; Arola, A; Hanseus, KBackground: 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.
- Mechanical Circulatory Support in Children: Strategies, Challenges and Future DirectionsPublication . Abreu, S; Brandão, C; Trigo, C; Rodrigues, R; Pinto, F; Fragata, JIntroduction: The use of mechanical circulatory support (MCS) in the pediatric population has evolved significantly in the past 20 years, but its management still poses several challenges. We aim to describe patient characteristics, outcomes, and morbidity associated with different modalities of MCS, in a tertiary center. Methods: Retrospective analysis of data from all the children who underwent MCS between 2002 and 2018 at a pediatric cardiology unit. Results: Between 2002 and 2018, 22 devices were implanted in 20 patients. Patients were divided into three groups: Group A (n=11) extracorporeal membrane oxygenator (ECMO); Group B (n=8) pulsatile paracorporeal ventricular assist device (VAD) and group C (n=3) paracorporeal continuous flow VAD. The median age was similar in groups A and B (18 and 23 months, respectively), and higher in group C (13 years). ECMO patients were cannulated mainly as a bridge to recovery (post cardiotomy- 8) while group B and C patients were bridged to transplantation. The most frequent complications were bleeding (group A - 36%, group C - 66.6%) and thromboembolic events (group B - 50%, group C - 33.3%). As for outcomes, in group A the majority of patients (54.5%) were weaned and 27.3% died. Half of group B and all of group C patients underwent transplantation. Conclusion: Bleeding and thromboembolic events were the main complications observed. Group B showed the highest mortality, probably related to the low weight of the patients. Overall, outcomes and complications are related to the type of device and patient status and characteristics.
- Pulmonary Arteriovenous Malformation Closure in a Preterm NewbornPublication . Loureiro, P; Laranjo, S; Lage, MJ; Trigo, C; Pinto, FF; Martins, JDF
- Successful Percutaneous Treatment with the Konar MFTM-VSD Occluder in an Infant with Abernethy Syndrome. Case ReportPublication . Loureiro, P; Georgiev, S; Ewert, P; Tanase, D; Eicken, A; Kammer, B; Dalla-Pozza, RCyanosis persisting after surgical repair of complex congenital heart disease (CHD) may be related to the underlying disease. However, extracardiac causes should be also considered. We report on a patient with heterotaxy syndrome and double outlet right ventricle, in whom postoperative cyanosis was associated with an Abernethy malformation type II causing a hepatopulmonary syndrome. Despite this complex anatomy, interventional closure of the portosystemic shunt was done with a Konar MF™-VSD Occluder. The patient recovered rapidly with relief of cyanosis within one month. This case highlights the importance of a careful diagnostic assessment in patients with complex CHD, who presents cyanoses after surgical repair. In addition, it shows the feasibility and safety of a percutaneous approach with complete closure of the vascular malformation in a patient with a complex anatomy.
- Ductus Arteriosus: the Coming of Age of a Fetal VesselPublication . Laranjo, S
- Spontaneous Preterm Birth and Congenital Heart Defects: What Is Known?Publication . Laranjo, S