Dextro-transposition Of Great Arteries Research Articles

Transcatheter Cardiac Interventions in Neonates with Congenital Heart Disease: A Single Centre Experience

Objective: To analyze the outcome among neonates undergoing transcatheter balloon atrial septostomy for various kinds of congenital heart diseases. Study Design: Prospective longitudinal study. Place and Duration of Study: National Institute of Cardiovascular Diseases, Karachi Pakistan, from Feb 2020 to May 2021. Methodology: A total of 28 neonates of both genders with echocardiography proven diagnosis of Tricuspid Atresia with restrictive Patent Foramen Ovale, Dextro-Transposition of Great Arteries with Intact Ventricular Septum, complex cyanotic congenital heart disease with inadequate mixing or hypoplastic left heart syndrome with restrictive PFO and a non-significant Patent Ductus Arteriosus, undergoing transcatheter balloon atrial septostomy were included. Demographic data along with diagnosis, clinical presentation, echocardiographic findings, procedural outcome (successful/unsuccessful), duration of the procedure, complication (if any), post-procedural echocardiographic findings, duration of hospital stay, further treatment plan and 3-months follow up outcomes were recorded among all neonates. Results: Most common diagnosis was Dextro-Transposition of the Great Arteries with IVS observed in 14(50.0%) neonates. Procedural outcome was found to be successful among 23(82.1%) neonates. There were 19(67.9%) neonates who did not show any post-procedure complication. At 3-months follow up, improvement in blood saturation and weight gain were reported among 12(42.9%) neonates each. Overall, mortality was noted in 7(25.0%) neonates, of whom 4(57.1%) neonates were having hypoplastic left heart syndrome. Conclusion: Transcatheter atrial septostomy done in neonatal period was found to have positive outcomes. Mortality remains high among neonates undergoing transcatheter atrial septostomy having hypoplastic left heart syndrome.

Severe hypoalbuminemia complicating a successful atrial switch operation (Senning) with cardiopulmonary bypass - report on a rare case

Post-operative hypoalbuminemia has been reported in 12% of neonates and 20% of infants; 97% occurs in the first 48 hours in neonates and infants undergoing cardiac surgery with cardiopulmonary bypass (CPB). Perioperative hypoalbuminemia is not independently associated with any post-operative morbidity. However, when analysed as a continuous variable, lower serum albumin levels have been reported to be associated with increased post-operative morbidity. Pre-operative low serum albumin level is independently associated with increased odds of post-operative hypoalbuminemia, and prolonged length of hospital stay. Immediate post-CPB (0-24 hour), lower serum albumin level is independently associated with an increased duration of mechanical ventilation. There is a relative paucity of literature on the impact of hypoalbuminemia in the paediatric population. Hypoalbuminemia is a marker of poor outcome in critically ill children and those undergoing a wide range of medical interventions. Albumin infusions may be an effective therapy for fluid resuscitation and for patients with severe hypoalbuminemia. We present a successful perioperative management of a 3 months old-male child, weighing 3.5 kg with dextro-transposition of great arteries (D-TGA) with regressed LV undergoing an atrial switch (Senning) procedure, and uneventful intraoperative course i.e. easy weaning from CPB. However, in the ICU, the baby suffered an unusual complication as severe hypoproteinaemia resulting in acute kidney injury (AKI) with complete anuria, generalized anasarca, hypotension requiring inotropes and vasopressors, and difficult weaning from ventilator.

Successful conservative treatment of pulmonary thromboembolism in unrepaired d-transposition of the great arteries with pulmonary hypertension: a case report

Background: Unrepaired dextro-transposition of great arteries (D-TGA) is well known for long-term complications, including tricuspid valve regurgitation, arrhythmias, and coronary artery abnormalities. Pulmonary hypertension (PH) is a frequent, progressive, and fatal complication in unrepaired CHD. This chronic condition can induce pulmonary thromboembolism (PTE). Aim: To report the management of PTE in unrepaired D-TGA with PH. Case Presentation: We present a rare case of an eleven-year-old male in unrepaired D-TGA with PH. He came with hemoptysis with no sign of acute or chronic infection. This condition was not associated with endocarditis or tuberculosis. The patient underwent a CT-pulmonary angiography examination and found a filling defect in the apical and posterior branches of the right superior pulmonary artery, suggesting a PTE that caused pulmonary parenchymal infarction. He was treated with intravenous low molecular weight heparin (LMWH) for ten days (1 mg/kg) and tranexamic acid. On follow-up, he did not experience cardiovascular collapse or hypotension; by the day, the hemoptysis had decreased in frequency and volume. He received long-term warfarin (2 mg/day) for three months. After three months of follow-up, the PTE was not visualized from CT-pulmonary angiography. Summary: Hemoptysis is rare and life-threatening in unrepaired CHD. Heparin administration continued with warfarin, and tranexamic acid provides a good prognosis in patients with PTE with D-TGA and PH.

Half-Turned Truncal Switch Surgery for Dextro-Transposition of Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis: Role of Intraoperative Echocardiography.

Half-Turned Truncal Switch Surgery for Dextro-Transposition of Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis: Role of Intraoperative Echocardiography.

Prenatal Delineation of Coronary Anatomy in Dextro-Transposition of Great Arteries.

Background:Dextro-transposition of the great arteries (D-TGA) is the second-most common cyanotic congenital heart disease with variable coronary artery anatomy. The arterial switch procedure has revolutionized outcomes for this defect, with coronary anatomy being a key determinant of both short- and long-term outcomes following surgical repair. The assessment of coronary anatomy is usually undertaken in the postnatal period by transthoracic echocardiography, with assessment prenatally not being well studied. We sought to assess the feasibility of delineating the coronary arteries on fetal echocardiograms in a small cohort of patients followed prenatally.Methods:This was a retrospective review of fetuses with D-TGA from 2008 to 2018. Patients with prenatal diagnosis of D-TGA were reviewed for the assessment of coronary artery anatomy. Details of coronary artery anatomy diagnosed prenatally were compared with postnatal transthoracic echocardiograms and intraoperative findings.Results:Thirty-four fetuses with findings of D-TGA on prenatal echocardiograms were reviewed. 14/34 fetuses had attempted delineation of coronary artery anatomy, with average gestational age of 28 weeks (range 23–31 weeks) at the time of diagnosis. Two-dimensional and color Doppler imaging of the coronary arteries on both short and long axis images were performed, with complete delineation being possible in ~ 86% of fetuses. These findings were confirmed postnatally.Conclusions:Fetuses with D-TGA can have variable coronary artery anatomy which drives postnatal outcomes. Our study describes a cohort of patients with D-TGA wherein coronary artery anatomy was assessed. We demonstrate that coronary artery evaluation is feasible prenatally with optimal imaging techniques, being more successful after 25 weeks' gestation. The potential knowledge of dangerous variants can help with referral to centers of excellence for appropriate postnatal management and facilitate prenatal care accordingly.

Foramen ovale and ductus arteriosus hemodynamics in Dextro Transposition of Great Arteries (D-TGA) with intact ventricular septum, case report, and literature review

D-TGA is characterized by discordant Ventriculo-arterial connection. For the patient to survive there should be a mixing of the oxygenated and deoxygenated blood at the atrial, ventricular and/or the great vessel level. The hemodynamics of the PFO and ductus arteriosus in D-TGA with intact ventricular septum (VS) is complex and depends upon the relative difference between the pulmonary and systemic vascular resistance as well as the ventricular compliance. D-TGA with intact VS might be associated with persistent pulmonary hypertension as well as with low pulmonary vascular resistance leading to changes in the hemodynamics and direction of flow through the PDA and/or the PFO. The indications of balloon atrial septestomy as well as the initiation and/or the continuation of Prostaglandin infusion are conflicting issues in such cases. We are presenting a case of D-TGA with intact VS who developed steeling and signs of low systemic blood flow because of a large PDA. We reviewed the hemodynamics of PFO and DA in such cases as well as the indications of BAS and PG infusion.

An exceedingly rare association of an isolated right subclavian artery with transposition of great vessels.

Isolation of the right subclavian artery (RSCA), defined as loss of continuity between the RSCA and aorta, is a rare anomaly and its association with dextrotransposition of great arteries (D-TGA) is exceedingly unusual. We present the case of a 5-day-old neonate with cyanosis from birth and a transthoracic echocardiographic diagnosis of D-TGA, where on computed tomography angiography, the RSCA was seen arising from the proximal part of the ipsilateral pulmonary artery via a patent right ductus arteriosus.

Ex vivo cardiovascular\xa0magnetic resonance diffusion weighted imaging in congenital heart disease, an insight into the microstructures of tetralogy of Fallot, biventricular and univentricular systemic right ventricle

PurposeCommon types of congenital heart disease exhibit a variety of structural and functional variations which may be accompanied by changes in the myocardial microstructure. We aimed to compare myocardial architecture from magnetic resonance diffusion tensor imaging (DTI) in preserved pathology specimens.Materials and methodsPathology specimens (n = 24) formalin-fixed for 40.8 ± 7.9 years comprised tetralogy of Fallot (TOF, n = 10), dextro-transposition of great arteries (D-TGA, n = 8) five with ventricular septal defect (VSD), systemic right ventricle (n = 4), situs inversus totalis (SIT, n = 1) and levo-TGA (L-TGA, n = 1). Specimens were imaged using a custom spin-echo sequence and segmented automatically according to tissue volume fraction. In each specimen T1, T2, fractional anisotropy, mean diffusivity, helix angle (HA) and sheet angle (E2A) were quantified. Pathologies were compared according to their HA gradient, HA asymmetry and E2A mean value in each myocardial segment (anterior, posterior, septal and lateral walls).ResultsTOF and D-TGA with VSD had decreased helix angle gradient by − 0.34°/% and remained symmetric in the septum in comparison to D-TGA without VSD. Helix angle range was decreased by 45°. It was associated with a decreased HA gradient in the right ventricular (RV) wall, i.e. predominant circumferential myocytes. The sheet angle in the septum of TOF was opposing those of the left ventricular (LV) free wall. Univentricular systemic RV had the lowest HA gradient (− 0.43°/%) and the highest HA asymmetry (75%). HA in SIT was linear, asymmetric, and reversed with a sign change at about 70% of the depth at mid-ventricle. In L-TGA with VSD, HA was asymmetric (90%) and its gradients were decreased in the septum, anterior and lateral wall.ConclusionThe organization of the myocytes as determined by DTI differs between TOF, D-TGA, L-TGA, systemic RV and SIT specimens. These differences in cardiac structure may further enlighten our understanding of cardiac function in these diverse congenital heart diseases.

Echocardiography guided bed side balloon atrial septostomy in dextro transposed great arteries (dTGA) with intact ventricular septum (IVS): A resource limited country experience

Objective:To determine effectiveness and safety of echocardiography guided bed side Balloon Atrial Septostomy (BAS) in dextro transposition of great Arteries (dTGA) with intact ventricular septum (IVS) at a public sector tertiary care hospital Karachi, Pakistan.Methods:This case series include 40 patients with echocardiographic findings of dTGA with IVS and restricted PFO (≤ 2mm) who underwent bedside BAS at department of pediatric cardiology NICVD, Karachi, Pakistan. We recorded pre and post BAS diameter of PFO/Atrial Septal Defect (ASD), oxygen saturation (SpO2 %), and post procedure complications and outcome.Results:Median age was 16 days, Majority of them (n=23, 58%) were severely cyanosed with SpO2 of 41.4±3.4% and underwent emergency BAS and remaining underwent elective procedure. An increase in SpO2% from 46.0±6% to 81.0±3.0% (p=<0.001) and ASD size from 1.4±2.8mm to 5.45±0.4mm was observed (p=<0.001). No complication was observed in most of cases (n=28, 70%). Mean hospital stay was 3.4±1 days. Success rate was 97.5% however, one neonate died due to neonatal sepsis.Conclusion:Our study provides sufficient evidence that bed side balloon atrial septostomy is a safer technique, save a lot of time and resources which were required otherwise in transporting these patients to catheterization laboratory.

Noninvasive Nitric Oxide Therapy in Right Ventricular Systolic Dysfunction Following Arterial Switch Procedure

Right ventricular dysfunction is uncommon following surgical repair of dextrotransposition of great arteries (d-TGA) by the arterial switch procedure. Inhaled nitric oxide (iNO) is a specific pulmonary vasolidator that has proven successful in treating pulmonary hypertension when delivered via endotracheal tube. We present a five-month-old infant with d-TGA and ventricular septal defect (VSD) who developed pulmonary hypertensive crisis, causing severe right ventricular dysfunction with hemodynamic deterioration at day 6 postsurgical repair by VSD closure and arterial switch. He was successfully treated with iNO via nonrebreather face mask avoiding endotracheal reintubation. This modality should be considered in the management of such infants.

Diagnosis, classification and prognosis prediction of fetal double outlets of right ventricle by echocardiography

Objective To study the accuracy of diagnosis,anatomical classification and prognostic prediction of double outlet of right ventricle (DORV) by fetal echocardiography.Methods Fifty-nine fetuses detected and diagnosed with DORV by fetal echocardiography were investigated to analyze the anatomical characteristics,types and complicated malformations.Results Fifty-nine fetuses with DORV included 56 with atrioventricular accordance and 3 with atrioventricular discordance.Among them,there were 44 with dextro-transposition of great arteries,7 with levo-transposition,2 with normal position,6 with un-identified in early stage.SDD was the most common type in DORV for approximate 81％ (44/53).Complicated abnormalities included pulmonary artery stenosis in 21 fetuses,single atrium in 5,atrialventricular valvular stenosis in 3,and mild ascending aortic dysplasia in 2.Amniocentesis and chromosome were test in 7 fetuses including 5 fetuses with normal chromosomes,one with trisomy 21 and one with trisomy 18.Conclusions Fetal echocardiography shows high clinical value in diagnosis,classification and detecting complicated abnormalities of DORV.Careful examinations provide accurate evaluation in surgical procedure and prognosis. Key words: Ultrasonography, prenatal; Double outlet right ventricle

Arterial Switch Operation With Unidirectional Valved Patch Closure of Ventricular Septal Defect in Patients With Transposition of Great Arteries and Severe Pulmonary Hypertension

For patients with dextro-transposition of great arteries (d-TGAs), ventricular septal defect (VSD), and severe pulmonary arterial hypertension (PAH), the common surgical options are palliative arterial switch operation (ASO) or palliative atrial switch operation leaving the VSD open. We evaluated the role of ASO with VSD closure using a fenestrated unidirectional valved patch (UVP). Between July 2009 and February 2011, six patients with TGAs, VSD, and severe PAH (mean age 39.8 ± 47.4 months, median 21, range 8-132 months), weighing 10.7 ± 9.2 kg (median 8.6, range 4.3-29 kg), underwent ASO with VSD closure using our simple technique of UVP. Mean pulmonary artery systolic pressure before the operation was 106 ± 12.7 mm Hg (median 107.5, range 95-126 mm Hg) and pulmonary vascular resistance was 9.5 ± 4.22 units (median 9.5, range 6.6-17.1 Wood units). There were no deaths. All patients had a postoperative systemic arterial saturation of more than 95%, although there were frequent episodes of systemic desaturation due to right-to-left shunt across the valved VSD patch (as seen on transesophageal and transthoracic echocardiograms). Mean follow-up was 10 ± 7.6 months (median 7.5, range 1-22 months). At most recent follow-up, all patients had systemic arterial saturation of more than 95% and no right-to-left shunt through the VSD patch. In one patient, the follow-up cardiac catheterization showed a fall in pulmonary artery systolic pressure to 49 mm Hg. Arterial switch operation with UVP VSD closure is feasible with acceptable early results. It avoids complications of palliative atrial switch (arrhythmia and baffle obstruction) and partially or completely open VSD.

Cardiac magnetic resonance imaging utilization for optimal pacemaker placement in stented baffle in transposition of great arteries

In dextro-transposition of great arteries (d-TGA), especially in an adult who has undergone both Mustard operation and superior vena cava (SVC) baffle stenting, permanent pacemaker lead placement can prove to be challenging. Magnetic Resonance Imaging (MRI) can be utilized for procedural planning and locating optimal anatomical sites for lead placement. In this report, we demonstrate dualchamber permanent pacemaker (PPM) lead placement through a stented SVC baffle to the left atrium (LA) and left ventricle (LV). This was done after an MRI demonstrated stent patency and anatomical locations within the LA for potential pacing. Knowledge of SVC patency and less-thanoptimal anatomy of adults with congenital heart defects are imperative in PPM.

Utility of Intracardiac Echocardiography for Atrial Baffle Leak Closure in Repaired Transposition of the Great Arteries

A 27-year-old male with dextro-transposition of great arteries had Senning atrial switch repair in childhood and dual-chamber pacemaker placement for sinus node dysfunction in adulthood. Transthoracic echocardiography showed a lead in the systemic (anatomic right) ventricle. Multidetector computed tomography showed the lead perforating the baffle in the region of the body of the systemic venous atrium into the systemic ventricle. The lead was extracted, and a new lead was placed in the pulmonary (anatomic left) ventricle. A bidirectional baffle shunt persisted. The iatrogenic baffle leak was percutaneously closed with an Amplatzer septal occluder device using both intracardiac echocardiography (ICE) and three-dimensional transesophageal echocardiography (3D-TEE). We report the first use of ICE for baffle leak closure, which provided a good definition of the complex anatomy and guided the procedure.

SHA 19. Both sides coronary angiography and intervention using a single transradial guiding catheter

and group II (Rastelli n= 4), one patient had an arch repair and Pulmonary Artery Banding then Rastelli. Results: Thirteen patients of Taussig–Bing anomaly underwent total correction. Median age and weight were 5 ± 6 weeks, 3.4 ± 0.7 kg, respectively. Aortic arch abnormalities was present in nine patients (69%), abnormal coronary artery patterns were present in seven patients (54%), side by side great arteries in five patients (38%), dextrotransposition of great arteries in seven patients (54%) and P-malposition in one patient (7.7%). There was no early mortality (one late mortality due to left ventricle dysfunction). Four cases (two from each group) developed right ventricular outflow tract obstruction, one required re-operation. Two patients from group II developed significant left ventricular outflow tract obstruction, one required re-operation. One patient from group I underwent balloon dilatation due to residual coarctation. Conclusion: DORV–TB is often associated with other congenital cardiac anomalies. In general total repair is feasible in majority of cases with satisfactory results and improved outcome. Tracks: Pediatric Cardiology.

Late Presenters with Dextro-transposition of Great Arteries and Intact Ventricular Septum: To Train or Not to Train the Left Ventricle for Arterial Switch Operation?

We report our experience in managing late presenters (older than 4 weeks) with dextro-transposition of great arteries and intact ventricular septum (d-TGA/IVS) in an effort to achieve successful arterial switch operation (ASO) in a third world setting. We retrospectively reviewed the charts of all late presenters with d-TGA/IVS. Patients were divided into two groups: left ventricular training (LVT) group and non-left ventricular training (non-LVT) group. LVT group underwent pulmonary artery banding and Blalock-Taussig Shunt prior to ASO. Twenty-one late presenters were included in the study. In LVT group, 11 patients with median age of 6 months (range, 1-72 months) underwent LVT. Later, 8 patients with median age of 9.25 months (range, 1.33-84 months) underwent ASO. Prior to ASO, left ventricle (LV) collapse resolved in all and left ventricle to systemic pressure (LV/SP) ratio was 0.81 (range, 0.76-0.95) in 4 patients. Two patients who had LVT for < or =14 days required postoperative extracorporeal membrane oxygenation (ECMO) support due to LV dysfunction. Seven patients survived to discharge. In non-LVT group, 10 patients with median age of 2.5 months (range, 1-98 months) underwent ASO. Five patients had LV collapse, and median LV/SP ratio was 0.67 (range, 0.56-1.19) in 5 patients. Seven patients needed ECMO support. Seven patients survived to discharge. Late presenters with d-TGA/IVS, who have LV collapse on echocardiography and/or a LV/SP ratio <0.67 on cardiac catheterization, should be subjected to LVT preferably for duration of longer than 14 days in order to avoid potential ECMO use.