Mid-aortic syndrome (MAS) is a rare clinical entity that is characterized by coarctation involving the distal thoracic and/or abdominal aorta and its major branches accounting for 0.5–2% of all cases of coarctation of the aorta (1). Renovascular hypertension can be a significant sequelae - it is the main symptomatic presentation of this disease among children and adolescents. We describe a 9-year-old girl who presents with recurrent abdominal pain and symptomatic hypertension. Due to significant left ventricular systolic dysfunction and uncontrolled hypertension, percutaneous balloon angioplasty was performed to treat the coarctation. To our knowledge, this is the first reported case of MAS in Malaysia. This case report highlights the clinical presentation, the role of computed tomography angiogram (CTA) in the diagnosis and current options in the management of MAS.
Abdominal Pain

Hand, foot and mouth disease (HFMD) is a common viral infection among infants and children. The major causative agents of HFMD are enterovirus 71 (EV71) and coxsackievirus A16 (CVA16). Recently, coxsackievirus A6 (CVA6) infections were reported in neighboring countries. Infected infants and children may present with fever, mouth/throat ulcers, rashes and vesicles on hands and feet. Moreover, EV71 infections might cause fatal neurological complications. Since 1997, EV71 caused fatalities in Sarawak and Peninsula Malaysia. The purpose of this study was to identify and classify the viruses which detected from the patients who presenting clinical signs and symptoms of HFMD in Seri Kembangan, Malaysia. From December 2012 until July 2013, a total of 28 specimens were collected from patients with clinical case definitions of HFMD. The HFMD viruses were detected by using semi-nested reverse transcription polymerase chain reaction (snRT-PCR). The positive snRTPCR products were sequenced and phylogenetic analyses of the viruses were performed. 12 of 28 specimens (42.9%) were positive in snRT-PCR, seven are CVA6 (58.3%), two CVA16 (16.7%) and three EV71 (25%). Based on phylogenetic analysis studies, EV71 strains were identified as sub-genotype B5; CVA16 strains classified into sub-genotype B2b and B2c; CVA6 strains closely related to strains in Taiwan and Japan. In this study, HFMD in Seri Kembangan were caused by different types of Enterovirus, which were EV71, CVA6 and CVA16.

Background: The long waiting time for Tetralogy of Fallot (TOF) operation may potentially increase the risk of hypoxic insult. Therefore, the objective of this study is to determine the frequency of acute neurological complications following primary TOF repair and to identify the peri-operative risk factors and predictors for the neurological sequelae. Methods: A retrospective review of the medical and surgical notes of 68 patients who underwent TOF repair in Hospital Serdang, from January 2013 to December 2017 was done. Univariate and multivariate analyses of demographics and perioperative clinical data were performed to determine the risk for the development of acute neurological complications (ANC) among these patients. Results: ANC was reported in 13 cases (19.1%) with delirium being the most common manifestation (10/68, 14.7%), followed by seizures in 4 (5.9%) and abnormal movements in two patients (2.9%). Univariate analyses showed that the presence of right ventricular (RV) dysfunction, prolonged duration of inotropic support (≥7 days), prolonged duration of mechanical ventilation (≥7 days), longer length of ICU stays (≥7 days), and longer length of hospital stay (≥14 days), were significantly associated with the presence of ANCs (p<0.05). However, multivariate analyses did not show any significant association between these variables and the development of ANC (p>0.05). The predictors for the development of postoperative delirium were pre-operative oxygen saturation less than 75% (Odds Ratio, OR=16.90, 95% Confidence Interval, 95%CI:1.36, 209.71) and duration of ventilation of more than 7 days (OR=13.20, 95%CI: 1.20, 144.98). Conclusion: ANC following TOF repair were significantly higher in patients with RV dysfunction, in those who required a longer duration of inotropic support, mechanical ventilation, ICU and hospital stay. Low pre-operative oxygen saturation and prolonged mechanical ventilation requirement were predictors for delirium which was the commonest neurological complications observed in this study. Hence, routine screening for delirium using an objective assessment tool should be performed on these high-risk patients to enable accurate diagnosis and early intervention to improve the overall outcome of TOF surgery in this country

The present study aims to determine the limitations of traditional Jones criteria during the first episode of acute rheumatic fever (ARF) at the initial referral hospital, in a cohort of patients below 18 years old who had undergone mitral valve repair in National Heart Institute (IJN) from 2011 to 2016. Carditis followed by fever and joint involvement were the most frequent manifestations at first diagnosis. Of the 50 patients, only seven (14%) fulfilled the traditional Jones criteria for the diagnosis of the first episode of ARF. When compulsory evidence of a previous group A Beta hemolytic streptococcus (GABHS) was disregarded, this figure rose to 54%. Therefore, strict adherence to Jones criteria with absolute documentation of GABHS will lead to underdiagnoses of ARF. The application of echocardiographic diagnostic criteria of rheumatic heart disease (RHD) needs to be emphasized to allow early diagnosis and administration of secondary prophylaxis to prevent progression to severe valvular disease.