MyMedR

Anomalous right pulmonary artery from the aorta

Wong A, Mokhtar S, Rasool A

Images Paediatr Cardiol, 2006 Jan;8(1):1-4.

Anomalous origin of the right pulmonary artery is a rare form of congenital heart disease. It usually presents in early infancy with heart failure and rapid development of pulmonary hypertension. There are about 131 cases reported in the literature and we report one such case.

Fulltext Hydroxyzine-induced supraventricular tachycardia in a nine-year-old child

Wong AR, Rasool AH

Singapore Med J, 2004 Feb;45(2):90-2.

PMID: 14985850

Hydroxyzine is a first generation antihistamine widely used in the paediatric population for a variety of conditions. A nine-year-old girl presented with supraventricular tachycardia while on clinical doses of hydroxyzine for pruritus. On arrival at the hospital, she was diaphoretic, with cool peripheries, poor peripheral pulses and a heart rate of 250/minute. There was a history of three palpitation episodes with chest tightness during the five months she was taking hydroxyzine. The supraventricular tachycardia eventually reverted to sinus rhythm with intravenous verapamil. Relevant cardiac examination and investigations had not shown any cardiac abnormalities. After discontinuing hydroxyzine, she had no further episodes of supraventricular tachycardia. To our knowledge, this is the first report of hydroxyzine induced-supraventricular tachycardia in the medical literature.

Clinical and genetic analysis of long QT syndrome in two Malay children

Wong AR, Zilfalil BA, Bhuiyan ZA

Med J Malaysia, 2019 08;74(4):341-343.

PMID: 31424047

Long QT syndrome (LQTS) is predominantly a genetic cardiac arrhythmia disorder. We report here our study on long QT syndrome from two children from Kelantan, Malaysia. Clinical and genetic findings of these two unrelated Malay children with LQTS is discussed. We found a Long QT, type 1 causal mutation, p.Ile567Thr in the KCNQ1 gene in the first child. A pathogenic mutation could not be detected in the second child, explaining the heterogeneity of this disease.

Fulltext Arterial stiffness during acute and recovery phases of children with rheumatic fever

Ibrahim NN, Jaafar H, Rasool AH, Wong AR

Med J Malaysia, 2016 02;71(1):23-5.

PMID: 27130739

Acute rheumatic fever (ARF) is associated with systemic inflammation and arterial stiffness during the acute stage. It has not been reported if arterial stiffness remains after recovery. The aim of this study was to determine the arterial stiffness during acute stage and 6 months after recovery from ARF. Arterial stiffness was assessed by carotid femoral pulse wave velocity (PWV) in 23 ARF patients during the acute stage of ARF and 6 months later. Simultaneously, erythrocyte sedimentation rate (ESR) and other anthropometric measurements were taken during both stages. There was a significant reduction in PWV; 6.5 (6.0, 7.45) m/s to 5.9 (5.38, 6.48) m/s, p=0.003 6 months after the acute stage of ARF. Similarly, ESR was also significantly reduced from 92.0 (37.5, 110.50) mm/hr to 7.0 (5.0, 16.0) mm/hr, p=0.001. In conclusion, arterial stiffness improved 6 months after the acute stage with routine aspirin treatment; this correlates well with the reduction in systemic inflammation.

An Unusual Type of Totally Anomalous Pulmonary Venous Connection to the Superior Cavoatrial Junction

Shamsuddin AM, Wong AR, Anderson RH, Corno AF

World J Pediatr Congenit Heart Surg, 2016 Jul;7(4):490-3.

PMID: 26795906 DOI: 10.1177/2150135115603330

A neonate with cyanosis at birth was found to have a rare type of totally anomalous pulmonary venous connection. The pulmonary veins entered a confluence posterior to the left atrial wall, which drained into the right superior cavoatrial junction. There were no other major structural defects and no evidence of isomerism. Because of the severe cyanosis, and the restrictive nature of the interatrial communication, we performed balloon atrioseptostomy to improve oxygenation. We then achieved successful surgical repair when the baby was aged 7 months.

Hansenula anomala infection in a neonate

Wong AR, Ibrahim H, Van Rostenberghe H, Ishak Z, Radzi MJ

J Paediatr Child Health, 2000 Dec;36(6):609-10.

PMID: 11115044

We present an unusual neonatal fungal infection, Hansenula anomala in a very low birthweight infant who underwent abdominal surgery for an omphalocele. Despite treatment with adequate doses of amphotericin B, the yeast continued to grow from the blood culture, and was only eradicated with the use of oral ketoconazole.

Arterial compliance and vitamin E blood levels with a self emulsifying preparation of tocotrienol rich vitamin E

Rasool AH, Rahman AR, Yuen KH, Wong AR

Arch Pharm Res, 2008 Sep;31(9):1212-7.

PMID: 18806966 DOI: 10.1007/s12272-001-1291-5

The tocotrienol vitamin E has potent antioxidant property, however absorption is low due to high lipid solubility. A self emulsifying preparation of tocotrienol rich vitamin E (SF-TRE) had been reported to increase their bioavailability. This randomized, placebo controlled, blinded end point clinical study aimed to determine the effects of 50, 100 and 200 mg daily of SF-TRE and placebo for two months on arterial compliance and vitamin E blood levels. Assessment of arterial compliance by carotid femoral pulse wave velocity (PWV) and augmentation index (AI), plasma vitamin E, serum total cholesterol and low density lipoprotein cholesterol were taken before and after 2 months' treatment in 36 healthy males. Un-supplemented tocotrienol levels were low, after treatment, all SF-TRE treated groups had significantly higher plasma alpha, delta and delta tocotrienol concentrations compared to placebo. Augmentation index change from baseline to end of treatment for groups placebo, 50, 100, and 200 mg were 2.22+/-1.54, -6.59+/-2.84, -8.72+/-3.77, and -6.27+/-2.67% respectively (p=0.049, 0.049, and 0.047 respectively). Groups 100 and 200 mg showed significant improvement after treatment with pulse wave velocity reductions of 0.77 m/s and 0.65 m/s respectively (p=0.007 and p=0.002). There was no effect of SF-TRE on serum lipids. We conclude that there was a trend towards improvement in arterial compliance with 2 months' of SF-TRE.

Left aorto-ventricular tunnel: A differential diagnosis to aortic regurgitation

Wong AR, Amelia A, Mohd Zain MR, Sayuti KA

Med J Malaysia, 2022 Jan;77(1):101-103.

PMID: 35087005

Left aorto-ventricular tunnel is a rare congenital heart disorder. A then 19-year-old young lady was referred to our clinic for chronic rheumatic heart disease with aortic regurgitation. However, because of an almost continuous murmur, and an unusual transthoracic echo, she underwent various investigations, finally revealing a rare type 2 left aorto-ventricular tunnel. She had been variously diagnosed as chronic rheumatic heart disease with aortic regurgitation, bicuspid aortic valve with aortic regurgitation, and then possibly coronary arterial (cameral) fistula (based on 2D transthoracic echo) and finally left aorto-ventricular tunnel. This is the first case described in South East Asia who is asymptomatic, apart from a murmur, and her long journey to this final diagnosis illustrates the need to "chase shadows" on echoes, to come to a proper conclusion for the patient.

Fulltext "Fishing for lines": removal of endovascular catheters from the cardiovascular system

Wong AR, Suhaimi H, Ridzuan MA, Rizal MM, Saedah A

Singapore Med J, 2009 Oct;50(10):e358-61.

PMID: 19907875

We present two infants whose endovascular lines were accidentally cut or fractured, and had to be retrieved via transcatheter means in the cardiac catheterisation laboratory. The first case was a two-month-old infant with transposition of the great arteries, requiring an emergency balloon atrial septostomy. An indwelling vascular catheter that was placed in the right femoral vein was accidentally cut and had migrated into the inferior vena cava, before being retrieved. The second case was a one-week-old neonate who presented with pneumonia at birth, and had a long intravenous catheter placed in the left saphenous vein, which became fractured, and subsequently migrated into the heart. This case presented as a pulmonary embolus with haemodynamic instability, as the catheter had partially obstructed the right ventricular outflow tract. This was later retrieved via transcatheter means.

Extensive facial and orbital infantile hemangiomas associated with high intraocular pressure

Shatriah I, Norazizah MA, Wan-Hitam WH, Wong AR, Yunus R, Leo SW

Pediatr Dermatol, 2013 Jan-Feb;30(1):151-4.

PMID: 22329437 DOI: 10.1111/j.1525-1470.2011.01618.x

High intraocular pressure is a rare ophthalmic condition associated with infantile hemangiomas that involves the orbit, eyelid, or both. Here, we describe a patient with extensive facial and orbital infantile hemangiomas associated with high intraocular pressure in the affected eye. The prompt management of this challenging condition is essential.

Fulltext Older Age Group in Pregnancy is Associated with Increased Arterial Stiffness

Salmi AA, Nor Aliza AG, Nik Zaki NM, Wong AR, Aida HR

Med J Malaysia, 2010 Jun;65(2):115-8.

PMID: 23756794 MyJurnal

Arterial stiffness is an index of vascular health; normal pregnancy is associated with reduced arterial stiffness. This cross sectional study compared arterial stiffness in older (≥35 years) and the younger (≤34 years) age groups of pregnant women. Arterial stiffness was assessed noninvasively in 66 pregnant women between 23 - 32 weeks gestation (41 women ≤ 34 years, 25 women ≥ 35 years) using the parameters pulse wave analysis and pulse wave velocity. Blood pressure (BP), body mass index (BMI), serum total cholesterol (TC) and fasting blood glucose (FBS) were also recorded. Mean ages of the younger and older age groups were 27.6±0.62 and 39.3±0.58 years; no significant difference was seen between the groups in their BMI, TC, FBS, SBP, DBP and gestational age. The older age group of women have increased arterial stiffness (augmentation index 19.4±1.9% vs 13.2±1.6%, p=0.015) and aortic stiffness (pulse wave velocity 8.7±0.3 vs 7.7±0.2 m/s, p=0.004) compared to the younger women. Linear regression analysis showed a positive significant correlation between age and augmentation index (R=0.278, p=0.026), and pulse wave velocity (R=0.350, p=0.004). We conclude that older pregnant women has increased arterial stiffness compared to a younger age group of pregnant women suggesting that vascular changes due to ageing occurs in pregnancy despite cardiovascular adaptations occurring in pregnancy.

Fulltext Pulmonary haemosiderosis with juvenile idiopathic arthritis in a Malaysian child

Wong AR, Noor AS, Rasool AH, Quah BS, Roberton D

Med J Malaysia, 2007 Oct;62(4):352-4.

PMID: 18551948 MyJurnal

A rare case of childhood pulmonary haemosiderosis with juvenile idiopathic arthritis is discussed, with particular reference to treatment with hydroxychloroquine and sildenafil for pulmonary hypertension which occurs secondary to this disease.

Method optimization on the use of postocclusive hyperemia model to assess microvascular function

Yvonne-Tee GB, Rasool AH, Halim AS, Wong AR, Rahman AR

Clin. Hemorheol. Microcirc., 2008;38(2):119-33.

PMID: 18198413

Recent development had allowed non-invasive assessment of microvascular function in vivo; however, the method has not been fully optimized and standardized. In this study, we aimed to characterize the "effective" occlusion duration needed to elicit sufficient postocclusive hyperemia (PORH) responses in forearm skin using laser Doppler fluximetry (LDF), in subjects with differing age, gender and menstrual phases.

Method optimization to assess endothelial function in females-duration of glyceryl trinitrate effect

Ibrahim NN, Rasool AH, Wong AR, Rahman AR

Methods Find Exp Clin Pharmacol, 2007 Jun;29(5):349-52.

PMID: 17805437

Pulse-wave analysis (PWA) combined with pharmacological challenges has recently been used as a method to measure endothelial function. This involved administration of glyceryl trinitrate (GTN), followed by salbutamol as endothelium-independent and -dependent vasodilators, respectively. The duration of GTN effect needs to be established before the administration of salbutamol. Baseline augmentation index (AIx) and pulse-wave velocity (PWV) measurements were taken in 11 healthy female subjects (mean age 23.27 +/- 3.66 years). Sublingual GTN 0.5 mg was administered for 3 min, followed by AIx and PWV measurements every 5 min till 20 min and then every 10 min until 40 min post-GTN. Maximum change in AIx post-GTN was at 3 min with a mean change from the baseline of -17.86% +/- 4.40% (p < 0.001). There were no significant changes noted after 30 and 40 min with mean change being -0.82% +/- 2.61% and 0.14% +/- 3.20%, respectively (p > 0.05). Significant changes in PWV were noted at 5 and 10 min with the mean change of -0.33 +/- 0.36 m/s and -0.33 +/- 0.35 m/s, respectively (p = 0.01). There were no further changes noted at 15 min and thereafter (p > 0.05). A duration of at least 30 min after GTN is required for AIx and PWV values to reach their baseline. Thus, the administration of salbutamol should be given only after 30 min of sublingual GTN for the assessment of endothelial function.

Sildenafil as treatment for Human Immunodeficiency Virus-related pulmonary hypertension in a child

Wong AR, Rasool AH, Abidin NZ, Noor AR, Quah BS

J Paediatr Child Health, 2006 Mar;42(3):147-8.

PMID: 16509918

Human Immunodeficiency Virus (HIV)-related pulmonary hypertension is a relatively rare disease that can affect HIV sufferers. This is almost always associated with a poor outcome and death. An 18 month-old girl, probably the youngest on record, was diagnosed to have pulmonary hypertension (PHT) and retrospectively found to have HIV infection. Sildenafil was used to control her PHT and she remains alive even after 2 years.

Surgery for doubly committed ventricular septal defects

Shamsuddin AM, Chen YC, Wong AR, Le TP, Anderson RH, Corno AF

Interact Cardiovasc Thorac Surg, 2016 Aug;23(2):231-4.

PMID: 27170744 DOI: 10.1093/icvts/ivw129

OBJECTIVES: Doubly committed ventricular septal defects (VSDs) account for up to almost one-third of isolated ventricular septal defects in Asian countries, compared with only 1/20th in western populations. In our surgical experience, this type of defect accounted for almost three-quarters of our practice. To date, patch closure has been considered the gold standard for surgical treatment of these lesions. Our objectives are to evaluate the indications and examine the outcomes of surgery for doubly committed VSDs.

METHODS: Between October 2013, when our service of paediatric cardiac surgery was opened, and December 2014, 24 patients were referred for surgical closure of VSDs. Among them, 17 patients (71%), with the median age of 6 years, ranging from 2 to 9 years, and with a median body weight of 19 kg, ranging from 11 to 56 kg, underwent surgical repair for doubly committed defects. In terms of size, the defect was considered moderate in 4 and large in 13. Aortic valvular regurgitation (AoVR) was present in 11 patients (65%) preoperatively, with associated malformations found in 14 (82%), with 5 patients (29%) having two or more associated defects.

RESULTS: After surgery, there was trivial residual shunting in 2 patients (12%). AoVR persisted in 6 (35%), reducing to trivial in 5 (29%) and mild in 1 (6%). Mean stays in the intensive care unit and hospital were 2.6 ± 1.2 days, ranging from 2 to 7 days, and 6.8 ± 0.8 days, ranging from 6 to 9 days, respectively. The mean follow-up was 14 ± 4 months, ranging from 6 to 20 months, with no early or late deaths and without clinical deterioration.

CONCLUSIONS: The incidence of doubly committed lesions is high in our experience, frequently associated with AoVR and other associated malformation. Early detection is crucial to prevent further progression of the disease. Patch closure remains the gold standard in management, not least since it allows simultaneous repair of associated intracardiac defects.

Fulltext Normothermia for pediatric and congenital heart surgery: an expanded horizon

Shamsuddin AM, Nikman AM, Ali S, Zain MR, Wong AR, Corno AF

Front Pediatr, 2015;3:23.

PMID: 25973411 DOI: 10.3389/fped.2015.00023

Cardiopulmonary bypass (CPB) in pediatric cardiac surgery is generally performed with hypothermia, flow reduction and hemodilution. From October 2013 to December 2014, 55 patients, median age 6 years (range 2 months to 52 years), median weight 18.5 kg (range 3.2-57 kg), underwent surgery with normothermic high flow CPB in a new unit. There were no early or late deaths. Fifty patients (90.9%) were extubated within 3 h, 3 (5.5%) within 24 h, and 2 (3.6%) within 48 h. Twenty-four patients (43.6%) did not require inotropic support, 31 (56.4%) received dopamine or dobutamine: 21 ≤5 mcg/kg/min, 8 5-10 mcg/kg/min, and 2 >10 mcg/kg/min. Two patients (6.5%) required noradrenaline 0.05-0.1 mcg/kg/min. On arrival to ICU and after 3 and 6 h and 8:00 a.m. the next morning, mean lactate levels were 1.9 ± 09, 2.0 ± 1.2, 1.6 ± 0.8, and 1.4 ± 0.7 mmol/L (0.6-5.2 mmol/L), respectively. From arrival to ICU to 8:00 a.m. the next morning mean urine output was 3.8 ± 1.5 mL/kg/h (0.7-7.6 mL/kg/h), and mean chest drainage was 0.6 ± 0.5 mL/kg/h (0.1-2.3 mL/kg/h). Mean ICU and hospital stay were 2.7 ± 1.4 days (2-8 days) and 7.2 ± 2.2 days (4-15 days), respectively. In conclusion, normothermic high flow CPB allows pediatric and congenital heart surgery with favorable outcomes even in a new unit. The immediate post-operative period is characterized by low requirement for inotropic and respiratory support, low lactate production, adequate urine output, minimal drainage from the chest drains, short ICU, and hospital stay.

Endothelial nitric oxide synthase G894T gene polymorphism and response to skin reactive hyperemia

Rasool AH, Ghazali DM, Abdullah H, Halim AS, Wong AR

Microvasc Res, 2009 Sep;78(2):230-4.

PMID: 19481100 DOI: 10.1016/j.mvr.2009.05.005

Post occlusive skin reactive hyperemia (PORH) is a tool used to assess microcirculation. Endothelial nitric oxide synthase (eNOS) mediates nitric oxide (NO) production; polymorphism of the eNOS gene may affect response to the PORH process. This study aims to determine whether eNOS G894T gene polymorphism affects response to skin PORH. 230 normotensive male and females between 18 and 40 years participated in this cross-sectional study. 170 subjects were of the homozygous GG genotype, whereas 60 were of the GT genotype. Skin PORH was performed by occlusion of the upper arm at 200 mm Hg for 3 min. Skin perfusion and temperature were monitored before, during and after occlusion release using the laser Doppler fluximetry. There were no significant differences between genotypes in their baseline blood pressure, serum cholesterol, BMI and age. Maximum change in perfusion after occlusion release (PORHmax) for the GG and GT genotypes were not significantly different at 50.15+/-1.29 vs. 47.92+/-2.17 AU; ANCOVA, p=0.351. Peak perfusion (PORHpeak) were also not significantly different between the two genotypes (61.23+/-1.36 vs. 57.72+/-2.32 AU; p=0.169). Minimum baseline perfusion were however higher in the GG compared to the GT genotype (10.83+/-0.29 vs. 9.61+/-0.50, p=0.029). We conclude that microvascular reactivity, assessed by change in perfusion after temporary ischemia was not significantly different between the GG and GT genotypes of the eNOS G894T gene. eNOS 894T allele carriers however, have lower baseline perfusion compared to the homozygous G894 allele carrier.

Dose dependent elevation of plasma tocotrienol levels and its effect on arterial compliance, plasma total antioxidant status, and lipid profile in healthy humans supplemented with tocotrienol rich vitamin E

Rasool AH, Yuen KH, Yusoff K, Wong AR, Rahman AR

J Nutr Sci Vitaminol (Tokyo), 2006 Dec;52(6):473-8.

PMID: 17330512

Tocotrienols are a class of vitamin E reported to be potent antioxidants, besides having the ability to inhibit the HMG-CoA reductase enzyme. This study assessed the effects of 3 doses of tocotrienol-rich vitamin E (TRE) on plasma tocotrienol isomer concentration, arterial compliance, plasma total antioxidant status (TAS), aortic systolic blood pressure (ASBP), serum total cholesterol (TC) and low density lipoprotein cholesterol (LDL-C) in healthy males.

METHODOLOGY: This randomised, blinded end-point, placebo-controlled clinical trial with a parallel design involved 36 healthy male subjects who took either an oral placebo or TRE at doses of 80, 160 or 320 mg daily for 2 mo. Baseline and end-of-treatment measurements of vitamin E concentration, arterial compliance [assessed by aortic femoral pulse wave velocity (PWV) and augmentation index (AI)], ASBP, plasma TAS, serum TC and LDL-C were taken.

RESULTS: Baseline tocotrienol isomer concentrations were low and not detectable in some subjects. Upon supplementation, all TRE-treated groups showed significant difference from placebo for their change in alpha, gamma and delta tocotrienol concentrations from baseline to end of treatment. There was a linear dose and blood level relationship for all the isomers. There was no significant difference between groups for their change in PWV, AI, plasma TAS, ASBP, TC or LDL-C from baseline to end of treatment. Groups 160 mg (p = 0.024) and 320 mg (p = 0.049) showed significant reductions in their ASBP. Group 320 mg showed a significant 9.2% improvement in TAS.

CONCLUSION: TRE at doses up to 320 mg daily were well tolerated. Treatment significantly increased alpha, delta, and gamma tocotrienol concentrations but did not significantly affect arterial compliance, plasma TAS, serum TC or LDL-C levels in normal subjects.

Device closure of patent ductus arteriosus in Marfan patients: safety and effect on the aortic root diameter

Zanjani KS, Wong AR, Sadiq M, Weber HS, Cutler NG

Congenit Heart Dis, 2010 Sep-Oct;5(5):439-43.

PMID: 21087429 DOI: 10.1111/j.1747-0803.2010.00448.x

Objectives: There is little evidence in the medical literature about safety of PDA device closure and its effects on aortic root diameter in Marfan patients. We reported on nine Marfan patients whose ducts were occluded by coil or Amplatzer Duct Occluder.
Patients and interventions: Two patients had aneurismal type E ducts which were closed by a single coil, respectively. The others had conical ducts for which even Amplatzer occluder, stiff, or double coils were used.
Results: No untoward consequence was found during a median follow up period of 18.5 months (range 3 months to 6.33 years). Two patients had small residual shunts. The observed change in the mean ratio of aortic root diameter to its estimated value was statistically insignificant in seven patients who had complete data on their aortic root diameter before PDA closure and during the follow up.
Conclusions: We concluded that PDA device closure in Marfan patients is safe. Risk of residual shunt is higher even after closure of small ducts.

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