Paediatric Restrictive Cardiomyopathy - Diagnosis and Challenges: A report of two cases.

Restrictive cardiomyopathy is one of the rarest forms of cardiomyopathies in paediatric patients characterised by impaired myocardial relaxation or compliance with restricted ventricular filling, leading to a reduced diastolic volume with a preserved systolic function. We report 2 cases-a 5-year-old boy who presented with abdominal distension and palpitation with family history of similar complaints but no definite genetic diagnosis as yet and a 5-year-old girl who presented with chronic cough and shortness of breath. Both cases were diagnosed in a tertiary care hospital in Muscat, Oman, in 2019 and are managed supportively with regular outpatient follow-up. This is the first series of reported cases of paediatric restrictive cardiomyopathy from Oman.

Evaluation of a Standard Handover Tool at a Pediatric Tertiary Care Unit in Oman.

Background The handover system is a great communication tool physicians use to transfer and receive patients' care-related information. The introduction of structured handover tools has resulted in a dramatic reduction in hospital-acquired injuries. We hypothesize that the I-PASS handover tool will improve both written and verbal communication without compromising the handover duration. The current study aims to improve the quality of care and patient safety by evaluating the applicability of I-PASS handover in the Child Health Department at Sultan Qaboos University Hospital, Oman.  Results A total of 20 trainees were enrolled in this study. After the implementation of I-PASS, 70% (14/20) of the respondents thought that the handover was well-structured, compared to 30% (6/20) prior to the implementation of I-PASS (P = .003). Due to I-PASS, about 80% of the participants could identify deteriorating patients and around 60% were confident in addressing emergencies. The I-PASS handover technique has raised participants' satisfaction from 80% to 95%. Before I-PASS, the mean adherence rate across all 10 variables was 28.7/50 (57.4%), compared to the post-I-PASS rate of 47/50 (94%). Conclusion The I-PASS system is a feasible and flexible clinical handover tool. This study showed that I-PASS has improved on-call handovers and patient safety.

All Endocarditis Is Not Infective: Libman-Sacks Endocarditis.

Libman-Sacks endocarditis (LSE) is an uncommon disorder that might be confused with infective endocarditis. It is one of the systemic lupus erythematosus (SLE) manifestations that could present with heart failure. We report a 12-year-old girl who presented with a history of shortness of breath, joint pain for four weeks, and fever for about one week. On examination, she was pale, edematous, and febrile. Her cardiac exam revealed a pan-systolic murmur of mitral regurgitation, harsh, grade 3/6 best heard at the apex. She was diagnosed with systemic lupus erythematosus with lupus nephritis and carditis. Her echocardiography revealed severe mitral regurgitation with nodular thickening of the valve in keeping with a diagnosis of LSE. After appropriate management of her underlying disorder using immunosuppressive, we saw a dramatic clinical improvement and her heart failure symptoms resolved. This case proves that SLE can have significant cardiac involvement and a proper evaluation would help in overall management and prognosis.

Practice Variation in Establishing the Adequacy of Beta-Blockers as an Antiarrhythmic Agent in School-Aged Children and Adolescents.

BACKGROUND: Beta-blockers (BBs) are commonly prescribed to manage arrhythmias in children and adolescents without any standardised approach to establish BB adequacy. We invited all Canadian pediatric cardiologists to participate in an anonymous survey to understand practice variation in the assessment of BB adequacy in school-aged children and adolescents with arrhythmia or the potential for arrhythmia. METHODS: An electronic survey approved by the Institutional Ethics Board was distributed by e-mail to 96 Canadian pediatric cardiologists who had been active in practice for at least 1 year. Incomplete surveys were excluded. RESULTS: Forty-one cardiologists (43%) responded to all questions in the survey. Thirteen cardiologists (32%) reported always assessing BB adequacy, 17 (41%) did so only for specific arrhythmias, and 11 (27%) reported never performing such an assessment. A total of 19 cardiologists (46%) and 18 cardiologists (44%) reported using Holter monitoring and exercise testing, respectively, to assess beta receptor blockade adequacy. Thirteen cardiologists (32%) considered BB therapy adequate if Holter demonstrated a 20% decrease in heart rate (HR) from baseline, and 10 respondents (24%) defined adequate BB therapy using exercise testing as a 20% decrease in maximal HR or blood pressure from baseline. CONCLUSION: Despite wide variation in practice, Holter monitoring and exercise testing are commonly used methods to measure the adequacy of BB therapy. There are no standard criteria, but the majority (56%) reported using a 20% decrease in HR or blood pressure from the pretreatment state as a criterion for adequate BB therapy in children and adolescents with arrhythmia or the potential for arrhythmia.

CONTEXTE: Les bêtabloquants (BB) sont couramment prescrits pour traiter les arythmies chez l'enfant et l'adolescent, en l'absence de méthode standardisée permettant d'établir le caractère adéquat du traitement par ces agents. Nous avons invité tous les cardiologues pédiatriques canadiens à participer anonymement à une enquête visant à rendre compte de la variabilité observée en pratique dans l'évaluation du caractère adéquat du traitement par BB chez l'enfant d'âge scolaire ou l'adolescent atteints d'arythmie ou susceptibles de l'être. MÉTHODOLOGIE: Un questionnaire électronique approuvé par le comité d'éthique en établissement a été distribué par courriel à 96 cardiologues pédiatriques canadiens en exercice depuis au moins un an. Les questionnaires partiellement remplis ont été exclus. RÉSULTATS: Parmi les participants, 41 cardiologues (43 %) ont répondu à toutes les questions de l'enquête. D'après les réponses obtenues, 13 cardiologues (32 %) évaluent toujours le caractère adéquat du traitement par BB, 17 (41 %) l'évaluent uniquement en présence de certains types d'arythmies et 11 (27 %) ne l'évaluent jamais. Au total, parmi les cardiologues interrogés, 19 (46 %) et 18 (44 %) affirment avoir recours respectivement à l'électrocardiographie (ECG) ambulatoire et à l'épreuve d'effort pour évaluer le caractère adéquat du blocage des récepteurs bêta. Ainsi, 13 cardiologues (32 %) jugent que le traitement par BB est adéquat si l'enregistrement ECG ambulatoire démontre une diminution de 20 % de la fréquence cardiaque (FC) maximale par rapport à la valeur de référence, et 10 cardiologues (24 %) estiment qu'il en est de même si le résultat obtenu à l'épreuve d'effort démontre une diminution de 20 % de la FC maximale ou de la pression artérielle par rapport aux valeurs de référence. CONCLUSION: Malgré de grandes variations observées en pratique, l'ECG ambulatoire et l'épreuve d'effort sont des méthodes couramment utilisées pour mesurer le caractère adéquat du traitement par BB. Il n'existe pas de critères standard, mais la majorité (56 %) des répondants ont indiqué qu'une diminution de 20 % de la FC ou de la pression artérielle par rapport aux valeurs de référence obtenues avant le traitement constitue un critère du caractère adéquat du traitement par BB chez l'enfant ou l'adolescent atteints d'arythmie ou susceptibles de l'être.