A Pediatric Cardiology Outreach Clinic in a Local Hospital: An Experience Review from South A'Sharqiyah, Oman.

Objectives: Specialized pediatric cardiology clinics conducted in local hospitals are an important part of delivering specialized care to patients close to their homes. This study aimed to review our experience with a specialized pediatric cardiology outreach clinic at Jaalan Bani Bu Ali Hospital, South A'Sharqiyah, Oman.

Methods: Patient records for each individual, seen in the outreach clinic between March 2018 and June 2022, were reviewed to determine demographic information, reason for referral, underlying diagnosis, and clinic visit outcomes.

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.

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.

Cardio-protective effect of regular transfusion in children with non-transfusion dependent thalassemia (NTDT): A cohort study.

Background And Aim Of The Work: Cardiac complications occur in patients with non-transfusion dependent thalassemia (NTDT). The study aimed to evaluate transfusion effect on systolic and diastolic cardiac function in young NTDT patients.  Methods:  Study design: Cohort study.

Cor Triatriatum Sinistrum: A rare disease with a common presentation.

Cor triatriatum sinistrum (CTS) is a rare congenital cardiac anomaly characterised by an abnormal septum within the left atrium impairing blood flow to the left ventricle. We report the case of a two-month-old male infant who presented with symptoms of heart failure since the age of two weeks. He was admitted to a local hospital and was managed with antibiotics because of the impression of pneumonia.

Percutaneous Closure of Ventricular Septal Defects in 116 Patients: Experience with different devices.

Objectives: This study aimed to review the experience with percutaneous closure of ventricular septal defects (VSDs) at the National Heart Center (NHC) in Muscat, Oman.

Methods: This retrospective study was conducted from November 2008 to December 2017. Patients' electronic medical records were reviewed to identify their clinical, imaging and interventional data before and after the procedure and on the last follow-up.

Pulmonary Artery Pseudoaneurysm in a Child With β-Thalassemia Major.

Pulmonary artery aneurysms and pseudoaneurysms are rare vascular anomalies in children that can lead to massive hemoptysis resulting in severe morbidity and even mortality. High level of clinical suspicion, timely diagnosis, and prompt management are important for a better outcome. Here, we report a case of a 14-year-old adolescent with β-thalassemia major who presented with life-threatening hemoptysis due to pulmonary artery pseudoaneurysm and was successfully treated with coil embolization.

Phenotypic spectrum of ALPK3-related cardiomyopathy.

Cardiomyopathies are clinically heterogeneous disorders and are the leading cause of cardiovascular morbidity and mortality. Different etiologies have a significant impact on prognosis. Recently, novel biallelic loss-of-function pathogenic variants in alpha-kinase 3 (ALPK3) were implicated in causing early-onset pediatric cardiomyopathy (cardiomyopathy, familial hypertrophic 27; OMIM 618052).

Mutation in mitochondrial complex IV subunit COX5A causes pulmonary arterial hypertension, lactic acidemia, and failure to thrive.

COX5A is a nuclear-encoded subunit of mitochondrial respiratory chain complex IV (cytochrome c oxidase). We present patients with a homozygous pathogenic variant in the COX5A gene. Clinical details of two affected siblings suffering from early-onset pulmonary arterial hypertension, lactic acidemia, failure to thrive, and isolated complex IV deficiency are presented.

Transcatheter Closure of Ruptured Sinus of Valsalva Aneurysm: Report of two cases.

A ruptured sinus of Valsalva aneurysm (RSVA) is a rare cardiac anomaly. Traditionally, RSVAs were repaired surgically; however, percutaneous transcatheter closure is the current treatment of choice. We report two cases of RSVA which were closed using this approach.

Echocardiographic Evidence of Early Diastolic Dysfunction in Asymptomatic Children with Osteogenesis Imperfecta.

Objectives: Structural and functional cardiovascular abnormalities have been reported in adults with osteogenesis imperfecta (OI); however, there is a lack of paediatric literature on this topic. This study aimed to investigate cardiovascular abnormalities in children with OI in comparison to a control group.

Methods: This case-control study was conducted at the Sultan Qaboos University Hospital in Muscat, Oman, between May 2013 and August 2014.

Echocardiography in PICU: when the heart sees what is invisible to the eye.

Objective: Echocardiography has become an indispensable bedside diagnostic tool in the realm of pediatric intensive care units (PICU). It has proven to be an influential factor in the formula of clinical decision-making. This study aimed to delineate the impact of echocardiography on the management of critically ill pediatric patients in the PICU at Sultan Qaboos University Hospital, Oman.

Changing Survival Rate of Infants Born Before 26 Gestational Weeks: Single-centre study.

Objectives: This study aimed to evaluate the changing survival rate and morbidities among infants born before 26 gestational weeks at the Sultan Qaboos University Hospital (SQUH) in Muscat, Oman.

Methods: This retrospective study assessed the mortality and morbidities of all premature infants born alive at 23-26 gestational weeks at SQUH between June 2006 and May 2013. Infants referred to SQUH within 72 hours of birth during this period were also included.

Hypertrophic Obstructive Cardiomyopathy in An Infant With Hemophagocytic Lymphohistiocytosis; Answer to a Riddle.

Familial hemophagocytic lymphohistiocytosis (FHLH) is a hereditary hyperinflammatory condition with T-cell and macrophage activation. Treatment consists of immunosuppressive therapy plus bone marrow transplantation. Cardiac manifestations of FHLH were scarcely mentioned in the literature with conflicting pathophysiological explanations.

Percutaneous transhepatic stenting of a restrictive atrial septal communication in hypoplastic left heart syndrome.

The postnatal survival of patients with congenital cardiac defects such as hypoplastic left heart syndrome (HLHS) is dependent on the patency of the ductus arteriosus and the presence of an unrestrictive atrial septal defect (ASD). We report a six week old infant with HLHS and tricuspid valve regurgitation with restrictive ASD. Transfemoral balloon atrial septostomy provided temporary relief but further attempts were not possible.

Complicated subacute bacterial endocarditis in a patient with ventricular septal defect.

Infective endocarditis (IE) is an uncommon but life-threatening infection. Despite advances in management, it still causes high morbidity and mortality. We report the case of an 8-year-old girl who presented with a prolonged fever of 2.

Successful Treatment of Cardiomyopathy due to Very Long-Chain Acyl-CoA Dehydrogenase Deficiency: First Case Report from Oman with Literature Review.

Very long-chain acyl-CoA dehydrogenase deficiency (MIM 201475) is a severe defect of mitochondrial energy production from oxidation of very long-chain fatty acids. This inherited metabolic disorder often presents in early neonatal period with episodes of symptomatic hypoglycemia usually responding well to intravenous glucose infusion. These babies are often discharged without establishment of diagnosis but return by 2-5 months of age with severe and progressive cardiac failure due to hypertrophic cardiomyopathy with or without hepatic failure and steatosis.

Comparison of two surgical techniques for complete atrioventricular septal defect repair using two- and three-dimensional echocardiography.

Different surgical techniques for complete atrioventricular septal defect (CAVSD) repair have been described, with the double-patch technique being most frequently employed. More recently a newer technique using a modified single-patch repair has been advocated. We hypothesized that the modified single-patch technique would result in an increased incidence of the two major post-repair comorbidities, namely, distortion of the left AV valve (LAVV) leaflets and narrowing of the left-ventricular outflow tract (LVOT).

Coarctation of the aorta, known yet can be missed.

The clinical presentation of coarctation of aorta (CoA) is well known; however, it is the most common congenital heart disease in the newborn period to be missed, with significant mortality and morbidity associated with missing the diagnosis. We report a 20-day-old newborn boy who presented with congestive heart failure and weak femoral pulses. Chest X-ray (CXR) showed cardiomegaly and pulmonary edema and electrocardiography (ECG) showed extreme right axis deviation and absent left ventricular forces in the left precordial leads.