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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.
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.
Fulltext Right-sided patent ductus arteriosus with bovine aortic arch, tetralogy of Fallot, dextrocardia and situs inversus

Sayuti KA, Shamsuddin AM, Mamat AZ, Zain MRM, Ramli N, Mokhtar AM, et al.

J Surg Case Rep, 2021 Jul;2021(7):rjab307.
PMID: 34316346 DOI: 10.1093/jscr/rjab307

A neonate with cyanosis at birth was found to have a rare type of tetralogy of Fallot. Echocardiography showed dextrocardia, left aortic arch with constricting and tortuous patent ductus arteriosus (PDA). Computed tomography angiography thorax revealed visceroatrial situs inversus, mirror image dextrocardia, tetralogy anatomy and tortuous right PDA arising from bovine brachiocephalic artery. In view of severe cyanosis, emergency division of PDA and modified right Blalock-Taussig shunt through median sternotomy were performed under cardiopulmonary bypass. Post-operatively, the sternum was left open and he developed pulmonary overcirculation requiring prolonged ICU stay. He was discharged well at Day 26 post surgery. This case highlights a rare association tetralogy of Fallot, dextrocardia and situs inversus, with concomitant unilateral right PDA and bovine aortic arch.
Fulltext Humanitarian Mission in Pediatric Cardiothoracic Surgery: A Recipient's Perspective

Mohd Zain MR, Shamsuddin AM, Mamat AZ, Mokhtar AM, Ali S, Chen YC, et al.

Front Pediatr, 2019;7:230.
PMID: 31231625 DOI: 10.3389/fped.2019.00230

Introduction: Pediatric cardiac surgical mission programs are deemed as common practice, especially in developing nations funded by international non-governmental organizations (NGOs). This article presents and discusses the results and strategies implemented by this partnership, aiming at achieving the autonomy of the local center by this collaboration. Materials and Methods: A retrospective review was conducted on patients with congenital heart disease who underwent surgical intervention from the beginning of the NGO collaboration (September 2015) until November 2018 in an existing cardiac center. In between those visits, any congenital heart disease patient with Risk Adjustment Congenital Heart Surgery (RACHS)-1 Category 1-3 would be discussed in a local multi-disciplinary meeting with regards to the feasibility of the surgery being performed by the local members. Results: A total of 60 operations were performed during the trips. Throughout the visit, 46% (28) of the operations were performed by the local surgeon, with or without assistance from the visiting surgeon. Between September 2015 and November 2018, 27 cases were also performed by the local team independently. For the 27 cases performed by the local team independently, the median age of the patient was 42 days (ranging from 14 days to 20 years old), with median body weight of 3.2 kg (ranging from 2.8 to 64 kg). Conclusion: Humanitarian pediatric cardiac surgical missions are safe to be done for the population in need. In order to achieve autonomy, continuous efforts by both teams are crucial, as the cooperation by the two parties ensures that the objectives are achieved.