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Fulltext Transposition of great arteries: a preliminary report on local experience with balloon atrial septostomy

Chan KC, Alwi M

Med J Malaysia, 1987 Jun;42(2):97-103.
PMID: 3503198

Eleven consecutive cases of simple transposition of great arteries were palliated by balloon atrial septostomy, The arterial saturation was improved and the gradient across the atrial septum was reduced or abolished. Improvement in the clinical status was achieved in all the patients. There was no mortality and morbidity was mild. A description of the technique as well as a brief review of the anatomy and haemodynamics of complete transposition of great arteries are the aims of this paper.

Matched MeSH terms: Transposition of Great Vessels/therapy*
A characteristic continuous wave Doppler signal in cor triatriatum?

Alwi M, Hamid ZA, Zambahari R

Br Heart J, 1992 Jul;68(1):6-8.
PMID: 1515294

Continuous wave Doppler recordings of the turbulent jet through the restrictive orifice of a left atrial partition in a patient with corrected transposition of the great arteries and cor triatriatum showed alternate bands of high intensity diastolic and low intensity systolic signals with preservation of the normal configuration of the diastolic E and A peaks. It is thought that Doppler studies in cor triatriatum will provide useful complementary haemodynamic information in the echocardiographic diagnosis of this anomaly.

Matched MeSH terms: Transposition of Great Vessels/surgery; Transposition of Great Vessels/ultrasonography
Ductal Stenting to Permit Delayed Arterial Switch Operation in a Separated Conjoined Twin

Haranal M, Leong MC, Muniandy SR, Khalid KF, Sivalingam S

Ann Thorac Surg, 2020 10;110(4):e327-e329.
PMID: 32224240 DOI: 10.1016/j.athoracsur.2020.02.039

Conjoined twins are rare congenital malformations known to be associated with cardiac abnormalities. Management of transposition of the great arteries with an intact ventricular septum in this subgroup of patients is challenging, especially in the presence of multiple comorbidities. Prevention of left ventricular involution until the patient is stable for an arterial switch operation is a real challenge. We report a case of early ductal stenting to keep the left ventricle well trained in a separated conjoined twin who eventually underwent an arterial switch operation.

Matched MeSH terms: Transposition of Great Vessels/diagnosis; Transposition of Great Vessels/surgery*
Recanalisation of the closed ductus arteriosus in a critically ill infant with transposition of the great arteries

Soo KW, Leong MC, Khalid F

Cardiol Young, 2016 Feb;26(2):371-4.
PMID: 26095661 DOI: 10.1017/S1047951115001055

We describe the case of an infant who was a late presenter of transposition of the great arteries where we proceeded with ductal stenting to improve oxygenation and left ventricle training. Stenting improved the infant's saturation while keeping the left ventricle well trained for 4 months after the procedure. This report demonstrates that intermediate-term left ventricle training can be achieved via ductal stenting.

Matched MeSH terms: Transposition of Great Vessels
Primary Arterial Switch Operation for Late Referral of Transposition of the Great Arteries with Intact Ventricular Septum in the Current Era: Do We Still Need a Rapid Two-Stage Operation?

Ota N, Sivalingam S, Pau KK, Hew CC, Dillon J, Latiff HA, et al.

World J Pediatr Congenit Heart Surg, 2018 01;9(1):74-78.
PMID: 29310554 DOI: 10.1177/2150135117743225

OBJECTIVE: We introduced primary arterial switch operation for the patient with transposition of great arteries and intact ventricular septum (TGA-IVS) who had more than 3.5 mm of posterior left ventricle (LV) wall thickness.
METHODS: Between January 2013 and June 2015, a total of 116 patients underwent arterial switch operation. Of the 116 patients, 26 with TGA-IVS underwent primary arterial switch operation at more than 30 days of age.
RESULTS: The age and body weight (mean ± SD) at the operation were 120.4 ± 93.8 days and 4.1 ±1.0 kg, respectively. There was no hospital mortality. The thickness of posterior LV wall (preoperation vs postoperation; mm) was 4.04 ± 0.71 versus 5.90 ± 1.3; P < .0001; interval: 11.8 ± 6.5 days. The left atrial pressure (mm Hg; postoperative day 0 vs 3) was 20.0 ± 3.2 versus 10.0 ± 2.0; P < .0001; and the maximum blood lactate level (mmol/dL) was 4.7 ± 1.4 versus 1.4 ± 0.3; P < .0001, which showed significant improvement in the postoperative course. All cases had delayed sternal closure. The patients who belonged to the thin LV posterior wall group (<4 mm [preoperative echo]: n = 13) had significantly longer ventilation time (days; 10.6 ± 4.8 vs 4.8 ± 1.7, P = .0039), and the intensive care unit stay (days) was 14 ± 9.2 versus 7.5 ± 3.5; P = .025, compared with thick LV wall group (≥4.0 mm: n = 13).
CONCLUSIONS: The children older than 30 days with TGA-IVS can benefit from primary arterial switch operation with acceptable results under our indication. However, we need further investigation for LV function.

Matched MeSH terms: Transposition of Great Vessels/surgery*
Ductal Stenting to Retrain the Involuted Left Ventricle in d-Transposition of the Great Arteries

Leong MC, Ahmed Alhassan AA, Sivalingam S, Alwi M

Ann Thorac Surg, 2019 09;108(3):813-819.
PMID: 30998905 DOI: 10.1016/j.athoracsur.2019.03.045

BACKGROUND: Ductal stenting is performed to retrain involuted left ventricles (LVs) in patients with d-transposition of the great arteries and intact ventricular septum (TGA-IVS). However, its efficacy is largely unknown. This study aimed to determine the safety and efficacy of ductal stenting in retraining of the involuted LV in patients with TGA-IVS.
METHODS: This was a single-center, retrospective study. Echocardiographic assessment of the LV geometry, mass, and free wall thickness was performed before stenting and before the arterial switch operation. Patients then underwent the arterial switch operation, and the postoperative outcomes were reviewed.
RESULTS: There were 11 consecutive patients (male, 81.8%; mean age at stenting, 43.11 ± 18.19 days) with TGA-IVS with involuted LV who underwent LV retraining by ductal stenting from July 2013 to December 2017. Retraining by ductus stenting failed in 4 patients (36.3%). Two patients required pulmonary artery banding, and another 2 had an LV mass index of less than 35 g/m2. Patients in the successful group had improved LV mass index from 45.14 ± 17.91 to 81.86 ± 33.11g/m2 (p = 0.023) compared with 34.50 ± 10.47 to 20.50 ± 9.88 g/m2 (p = 0.169) and improved LV geometry after ductal stenting. The failed group was associated with an increased need for extracorporeal support (14.5% vs 50%, p = 0.012). An atrial septal defect-to-interatrial septum length ratio of more than 0.38 was associated with failed LV retraining.
CONCLUSIONS: Ductal stenting is an effective method to retrain the involuted LV in TGA-IVS. A large atrial septal defect (atrial septal defect-to-interatrial septum length ratio >0.38) was associated with poor response to LV retraining.

Matched MeSH terms: Transposition of Great Vessels/mortality; Transposition of Great Vessels/surgery*
Fulltext Stenting the ductus arteriosus: Case selection, technique and possible complications

Alwi M

Ann Pediatr Cardiol, 2008 Jan;1(1):38-45.
PMID: 20300236 DOI: 10.4103/0974-2069.41054

Ductal stenting is an attractive alternative to conventional shunt surgery in duct dependent congenital heart disease as it avoids thoracotomy and its related problems. With today's generation of coronary stents which have better profile, flexibility and trackability, ductal stenting may be achieved safely and with considerably less difficulty than previously described.As in Blalock-Taussig (BT) shunt, ductal stenting is indicated mainly in duct-dependent cyanotic lesions chiefly in the neonatal period. Unlike the Patent ductus arteriosus (PDA) as an isolated lesion, the ductus in cyanotic heart disease has a remarkable morphologic variability. The ductus tends to arise more proximally under the aortic arch, giving rise to a vertical ductus or occasionally it may arise from the subclavian artery. It also tends to be long and sometimes very tortuous, rendering stent implantation technically impossible. The ductus in these patients may also insert onto one of the branch pulmonary arteries with some stenosis at the site of insertion. The ductus in Tetralogy of Fallot with pulmonary atresia (TOF-PA) tend to exhibit these morphologic features and to a lesser degree in transposition of great arteries with ventricular septal defect and pulmonary atresia (TGA-VSD-PA) and the more complex forms of univentricular hearts. In the preliminary angiographic evaluation, it is important to delineate these morphologic features as the basis for case selection.Ductal stenting may be done by the retrograde femoral artery route or the antegrade transvenous route depending on the ductus morphology and the underlying cardiac lesion. The detailed techniques and essential hardware are described. Finally, major potential complications of the procedure are described. Acute stent thrombosis is the most serious and potentially catastrophic. Emergent treatment with thrombolytic therapy and mechanical disruption of thrombus are required. With proper case selection, appropriate technique and the right hardware ductal stenting provides reasonable short-medium term palliation in duct-dependent cyanotic heart disease.

Matched MeSH terms: Transposition of Great Vessels
Initial Clinical Trial of A Novel Pulmonary Valved Conduit

Prodan Z, Mroczek T, Sivalingam S, Bennink G, Asch FM, Cox M, et al.

Semin Thorac Cardiovasc Surg, 2021 May 10.
PMID: 33984478 DOI: 10.1053/j.semtcvs.2021.03.036

Valved allografts and xenografts for reconstruction of the right ventricular outflow tract (RVOT) lack durability and do not grow. We report the first clinical use of a completely bioabsorbable valved conduit (Xeltis pulmonary valve - XPV) in children. Twelve children (six male), median age five (two to twelve) years and median weight 17 (10 to 43) kg, underwent RVOT reconstruction with the XPV. Diagnoses were: pulmonary atresia with ventricular septal defect (VSD) (n=4), tetralogy of Fallot (n=4), common arterial trunk (n=3), and transposition of the great arteries with VSD and pulmonary stenosis (n=1). All had had previous surgery, including prior RVOT conduit implantation in six. Two diameters of conduit 16mm (n=5) and 18mm (n=7) were used. At 24 months none of the patients has required surgical re-intervention, 9 of the 12 are in NYHA functional class I and three patients in NYHA class II. None of the conduits has shown evidence of progressive stenosis, dilation or aneurysm formation. Residual peak gradient of >40 mm Hg was observed in three patients, caused by kinking of the conduit at implantation in 1 and distal stenosis in the peripheral pulmonary arteries in 2 patients. Five patients developed severe pulmonary valve insufficiency (PI); the most common mechanism was prolapse of at least one of the valve leaflets. The XPV conduit is a promising innovation for RVOT reconstruction. Progressive PI requires however an improved design (geometry, thickness) of the valve leaflets.

Matched MeSH terms: Transposition of Great Vessels
Dexmedetomidine and ketamine sedation for dental extraction in children with cyanotic heart disease

Hasan MS, Chan L

J Oral Maxillofac Surg, 2014 Oct;72(10):1920.e1-4.
PMID: 24985961 DOI: 10.1016/j.joms.2014.03.032

Treating children with cyanotic congenital heart disease poses many challenges to anesthesiologists because of the multiple problems associated with the condition. The anesthetic technique and drugs used perioperatively can affect a patient's physiologic status during surgery. The adherence to certain hemodynamic objectives and the avoidance of factors that could worsen the abnormal cardiopulmonary physiology cannot be overemphasized. In the present case series, we describe the use of a dexmedetomidine-ketamine combination for dental extraction in spontaneously breathing children with cyanotic congenital heart disease. The anesthetic concerns regarding airway management, the pharmacologic effects of drugs, and maintenance of adequate hemodynamic, blood gases, and acid-base status are discussed.

Matched MeSH terms: Transposition of Great Vessels/complications