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.

Kawarabi: Administrative Structuring of a Multicenter Research Collaborative to Study Kawasaki Disease in the Arab Countries.

Kawasaki disease (KD), the leading cause of acquired heart disease in children in developed countries, merits conducting detailed studies in Arab countries. We introduce Kawarabi, as a multicenter research collaborative effort dedicated to improving diagnosis, care, and outcome of children and adults with KD in the Arab world. During the COVID-19 pandemic, there emerged a new multisystem inflammatory syndrome in children; a disease similar to KD. This highlighted the challenges that Arab physicians face in diagnosing and managing children with KD and KD-like illnesses. Kawarabi brings together experts in North America and Arab nations to study this family of diseases in a not-for-profit, voluntary scientific collaborative setting. Bylaws addressing the vision, objectives, structure, and governance of Kawarabi were established, and vetted by the 45 organizing members in 2021. An initial scientific publication showed evidence of a decreased level of awareness of the disease in the general population, as well as the lack of access to resources available for physicians caring for children with KD in Arab countries. Kawarabi has since held several educational webinars and an inaugural yearly meeting. The groundwork for future initiatives targeted at increasing awareness and understanding of the management and the long-term outcomes of children with KD in the region was established. Data on KD in the Arab world are lacking. Kawarabi is a multicenter research collaborative organization that has the unique resources, diversified ethnic makeup, and energy, to accomplish significant advances in our understanding and management of KD and its variants.

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. Due to persistent unresolved tachypnoea and tachycardia, he was referred to Sultan Qaboos University Hospital, Muscat, Oman, in 2019 for cardiac evaluation which confirmed a diagnosis of isolated CTS with severe stenosis and pulmonary hypertension. He underwent an urgent surgical excision of the membrane with uneventful recovery.

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. RESULTS: A total of 116 patients, the majority of which were female (58%), underwent 118 percutaneous procedures for VSD closure at a median age of 3.54 years (range: 0.25-33 years) and a median weight of 12 kg (range: 3.5-78 kg). The mean diameter of the VSDs as determined by transoesophageal echocardiogram was 5.6 ± 1.9 mm (n = 105). The commonest type of VSD was perimembranous (n = 75, 63.5%). Devices were successfully placed during 111 (94.1%) procedures in 109 (94.0%) patients, with the commonest device being a Amplatzer™ duct occluder I (St. Jude Medical, Little Canada, Minnesota, USA; n = 39, 35.1%). There was no mortality. Early major cardiac complications occurred in six patients (5.5%) with device embolisation being the commonest (n = 4, 3.7%). The median follow-up period was 19 months (range: 1-84 months) in 89 (81.7%) of the patients. One patient (0.9%) required a permanent pacemaker for a complete heart block. CONCLUSION: This study has demonstrated a good rate of VSD closure with low morbidity and no mortality using the percutaneous approach with different devices. Long-term follow-up is needed to specifically evaluate the function of adjacent structures and the long-term effects on conduction systems.

Pulmonary Artery Pseudoaneurysm in a Child With ß-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 ß-thalassemia major who presented with life-threatening hemoptysis due to pulmonary artery pseudoaneurysm and was successfully treated with coil embolization.

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. The first case was a 45-year-old female who presented to the Royal Hospital, Muscat, Oman, in 2014 with a RSVA in the right ventricle. The second case was a 39-year-old male who was admitted to the Sultan Qaboos University Hospital, Muscat, in 2015 with a large multifenestrated RSVA extending into the right ventricle outflow tract. Each patient underwent transcutaneous cardiac catheterisation using three-dimensional echocardiography. Both interventions were technically successful; however, the second patient required a subsequent surgery due to the continuing presence of a significant shunt. Transcatheter closure of RSVAs is an effective alternative to surgical repair, although large multifenestrated RSVAs should be repaired surgically to ensure complete closure.

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. Data from eight patients with OI and 24 healthy controls were compared using conventional and tissue Doppler echocardiography (TDE). RESULTS: The OI group had significantly lower peak early mitral valve flow velocity (P = 0.027), peak a-wave reversal in the pulmonary vein (P = 0.030) and peak early diastolic velocity of the mitral valve and upper septum (P = 0.001 each). The peak late diastolic velocities of the mitral valve (P = 0.002) and the upper septum (P = 0.037) were significantly higher in the OI group; however, the peak early/late diastolic velocity ratios of the mitral valve (P = 0.002) and upper septum (P = 0.001) were significantly lower. Left ventricular dimensions and aortic and pulmonary artery diameters were larger in the OI group when indexed for body surface area. Both groups had normal systolic cardiac function. CONCLUSION: Children with OI had normal systolic cardiac function. However, changes in myocardial tissue Doppler velocities were suggestive of early diastolic cardiac dysfunction. They also had increased left ventricular dimensions and greater vessel diameters. These findings indicate the need for early and detailed structural and functional echocardiographic assessment and follow-up of young patients with OI.

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 2.5 months duration and a history of a small perimembranous ventricular septal defect. She was diagnosed with subacute bacterial endocarditis secondary to Streptococcus mutans. The patient developed a septic pulmonary embolism; however, with the use of appropriate antimicrobial therapy, she made an uneventful recovery. Clinicians should have a high index of suspicion for IE as the possible cause of a prolonged fever, especially in the presence of congenital heart disease (CHD). Currently, IE prophylaxis is not indicated for unrepaired acyanotic CHD. Nevertheless, with the new changes in the guidelines, more prospective studies are needed to investigate the incidence of IE in such lesions, before long-term conclusions can be drawn.

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). We studied 14 patients with CAVSD who underwent either traditional double-patch technique [group 1 (n = 7)] or modified single-patch technique [group 2 (n = 7)]. Preoperative and immediate postoperative two-dimensional (2D) echocardiograms, as well as follow-up 2D and three-dimensional (3D) studies, were reviewed. For group 1, the median age at repair was 4.1 months with a median duration from surgical repair and last echocardiogram of 44 months. For group 2, the median age at repair was 3 months with a median duration from surgical repair and last echocardiogram of 28 months. The two groups had similar demographics and ventricular septal defect size before surgery. For the LAVV, no significant difference was observed with respect to LAVV annulus size, tenting height, and the size of the vena contracta. Furthermore, there was no significant difference in the 2D echocardiographic areas and volumes of the LVOT between pre-repair and immediate post-repair studies for both groups. At the last evaluation, although there had been growth of the LVOT in both groups, no significant difference between areas and volumes were observed. Areas of the LVOT measured by 3D echocardiography on the final study showed no significant statistical difference between both groups. There was good correlation of the areas measured by 2D and 3D echocardiography within each group. In this small group, modified single-patch technique does not appear to tether the LAVV or promote an increase in regurgitation. In the short term, LVOT growth is unaffected, and the repair does not promote LVOT obstruction. 3D echocardiography is useful for area measurements of the LVOT and showed good correlation with areas measured by assumption of the LVOT shape as determined using 2D techniques.