INTRODUCTION

Ductal stenting can be a nonsurgical option and less morbid method to provide pressure and volume overload to the regressing left ventricle in late presenters of transposition of the great arteries, intact ventricular septum (TGA-IVS), prior to arterial switch operation (ASO).

CASE PRESENTATION

This is a case of an infant diagnosed with TGA-IVS beyond the neonatal period who underwent balloon atrial septostomy and ductal stenting to retrain involuted left ventricle prior to definitive ASO.

DISCUSSION

Ductal stenting is an alternative option in late presenters of transposition of the great arteries with involuted left ventricle. It provides volume loading and, to a lesser extent, pressure loading leading to left ventricular hypertrophy. Also, presence of the stented duct allows improved oxygenation eliminating the need for aortopulmonary shunt. It can be a less morbid method of left ventricular training because it avoids hemodynamic stress, pulmonary artery distortion, and neoaortic valve regurgitation.

CONCLUSION

Ductal stenting in late presenters with TGA-IVS successfully retrains the left ventricle and achieves safe late ASO.

Heart Defects, Congenital ; Transposition of Great Vessels ; Arterial Switch Operation

Congenital heart disease (CHD) is the result of a complicated interplay between genetic and non-genetic, or “environmental,†factors acting on the foetus and one of those environmental factors is maternal hyperglycemia. Maternal diabetes has teratogenic effects on the evolution of the foetal cardiovascular system; as a consequence, cardiovascular malformations are the most common anomalies in infants of diabetic mothers with transposition of the great arteries, tricuspid atresia and truncus arteriosus being some of the common cardiac malformations encountered. Thus, it is important to perform a detailed heart examination at autopsy of perinatal deaths in order to ascertain related anomalies. We present a case of stillbirth in a woman with Type 1 Diabetes mellitus on insulin therapy who claims she was unaware about her pregnancy.
Heart Defects, Congenital ; Diabetes, Gestational ; Transposition of Great Vessels

BACKGROUNDDouble outlet right ventricle (DORV) is a group of complex congenital heart abnormalities. Preoperative pulmonary hypertension (PH) is considered an important risk factor for early death during the surgical treatment of DORV. The aim of this study was to report our experience on surgical treatment of DORV complicated by PH.

METHODSFrom June 2004 to November 2016, 61 patients (36 males and 25 females) aged 2 weeks to 26 years (median: 0.67 years and interquartile range: 0.42-1.67 years) with DORV (two great arteries overriding at least 50%) complicated by PH underwent surgical treatment in our center. All patients were categorized according to surgical age and lesion type, respectively. Pulmonary artery systolic pressure (PASP), pulmonary artery diastolic pressure (PADP), and mean pulmonary artery pressure (mPAP) were measured directly before cardiopulmonary bypass (CPB) was established and after CPB was removed. An intracardiac channel procedure was performed in 37 patients, arterial switch procedure in 19 patients, Rastelli procedure in three patient, Senning procedure in one patients, and Mustard procedure in one patient. The Student's t-test and Chi-squared test were performed to evaluate clinical outcomes of the surgical timing and operation choice.

RESULTSFifty-five patients had uneventful recovery. PASP fell from 55.3 ± 11.2 mmHg to 34.7 ± 11.6 mmHg (t = 14.05, P < 0.001), PADP fell from 29.7 ± 12.5 mmHg to 18.6 ± 7.9 mmHg (t = 7.39, P < 0.001), and mPAP fell from 40.3 ± 10.6 mmHg to 25.7 ± 8.3 mmHg (t = 11.85, P < 0.001). Six (9.8%) patients died owing to complications including low cardiac output syndrome in two patients, respiratory failure in two, pulmonary hemorrhage in one, and sudden death in one patient. Pulmonary artery pressure (PAP) dropped significantly in infant and child patients. Mortality of both infants (13.9%) and adults (33.3%) was high.

CONCLUSIONSPAP of patients with DORV complicated by PH can be expected to fall significantly after surgery. An arterial switch procedure can achieve excellent results in patients with transposition of the great arteries type. Higher incidence of complications may occur in patients with ventricular septal defect (VSD) type before 1 year of age. For those with remote VSD type, VSD enlargement and right ventricle outflow tract reconstruction are usually required with acceptable results. The degree of aortic overriding does not influence surgical outcome.

Adolescent ; Adult ; Cardiac Surgical Procedures ; methods ; Child ; Child, Preschool ; Double Outlet Right Ventricle ; surgery ; Female ; Heart Septal Defects, Ventricular ; surgery ; Heart Ventricles ; surgery ; Humans ; Hypertension, Pulmonary ; complications ; Infant ; Male ; Pulmonary Valve Stenosis ; surgery ; Risk Factors ; Transposition of Great Vessels ; surgery ; Treatment Outcome ; Young Adult

Cardiac Surgical Procedures ; adverse effects ; methods ; Female ; Heart Septal Defects, Ventricular ; diagnosis ; surgery ; Humans ; Infant, Newborn ; Male ; Transposition of Great Vessels ; diagnosis ; surgery ; Treatment Outcome

The half-turned truncal switch (HTTS) operation has been reported as an alternative to the Rastelli or réparation à l'étage ventriculaire procedures. HTTS prevents left ventricular outflow tract (LVOT) obstruction in patients with complete transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and pulmonary stenosis (PS), or in those with a Taussig-Bing anomaly with PS. The advantages of the HTTS procedure are avoidance of late LVOT or right ventricular outflow tract (RVOT) obstruction, and of overstretching of the pulmonary artery. We report the case of a patient who underwent HTTS for TGA with VSD and PS, in whom there was no LVOT obstruction and only mild aortic regurgitation and mild RVOT obstruction, including observations at 12-year follow-up. Our experience with long-term follow-up of HTTS supports a solution for late complications after the Rastelli procedure.
Aortic Valve Insufficiency ; Arteries* ; Double Outlet Right Ventricle ; Follow-Up Studies* ; Heart Septal Defects, Ventricular* ; Humans ; Pulmonary Artery ; Pulmonary Valve Stenosis* ; Transposition of Great Vessels

An abnormal myocardial perfusion is not uncommon in congenital heart defects (CHD). Many case reports and prospective studies were done describing myocardial peOision scintigraphy (MPS)findings after an arterial switch operation (ASO) among transposition of the great arteries (TCA), but to the best of the authors' knowledge, none have cited MPS findings in the immediate post-operative petiod. This paper aims to show two ckfferent clinicalthuatiomts in which (MPS) was used in the assessment of myocarchalfiinction in TCA immediatelyfollowing an arterial switch operation. Two male infants; aged 2 months and 4 months; both diagnosed with transposition of the great arteries presenting with past-operative morbidities after an arterial switch operation were referred to the Department of Nuclear Medicine/or evaluation. The younger infant was ditty discharged improved with an earlier magical intervention in contrast to the other who expired Bothpatients revealed a scintigraphic picture of myocarcbal ischemia and left ventricular a54ifiniction with concomitant right ventricular hypertrophy MPS .findings and reflective clinical pictures of patients with corrected congenital disease are yet to be/illy elticidated,.from the expected natural course after the stag/Cal intervention and long term complications ofsuch cases. The two cases present an invaluable avenue of non-invasive diagnostic modality using-MPS to assess probable pathologic mechanisms that occur after an arterial switch operation, and perhaps suggest incremental value not only in the post-operative period but in the preoperative scenario in the prognostication of these patients.

Human ; Male ; Female ; Adult ; Infant ; Hypertrophy, Right Ventricular ; Transposition Of Great Vessels ; Arterial Switch Operation ; Myocardial Perfusion Imaging ; Heart Defects, Congenital ; Heart Ventricles

An abnormal myocardial perfusion is not uncommon in congenital heart defects (CHD). Many case reports and prospective studies were done describing myocardial peOision scintigraphy (MPS)findings after an arterial switch operation (ASO) among transposition of the great arteries (TCA), but to the best of the authors' knowledge, none have cited MPS findings in the immediate post-operative petiod. This paper aims to show two ckfferent clinicalthuatiomts in which (MPS) was used in the assessment of myocarchalfiinction in TCA immediatelyfollowing an arterial switch operation. Two male infants; aged 2 months and 4 months; both diagnosed with transposition of the great arteries presenting with past-operative morbidities after an arterial switch operation were referred to the Department of Nuclear Medicine/or evaluation. The younger infant was ditty discharged improved with an earlier magical intervention in contrast to the other who expired Bothpatients revealed a scintigraphic picture of myocarcbal ischemia and left ventricular a54ifiniction with concomitant right ventricular hypertrophy MPS .findings and reflective clinical pictures of patients with corrected congenital disease are yet to be/illy elticidated,.from the expected natural course after the stag/Cal intervention and long term complications ofsuch cases. The two cases present an invaluable avenue of non-invasive diagnostic modality using-MPS to assess probable pathologic mechanisms that occur after an arterial switch operation, and perhaps suggest incremental value not only in the post-operative period but in the preoperative scenario in the prognostication of these patients.

Human ; Male ; Female ; Adult ; Infant ; Hypertrophy, Right Ventricular ; Transposition Of Great Vessels ; Arterial Switch Operation ; Myocardial Perfusion Imaging ; Heart Defects, Congenital ; Heart Ventricles

A five-month-old boy who had undergone previously transcatheter balloon atrioseptostomy at 3 days of age for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis underwent pulmonary root translocation with the Lecompte maneuver. This operation has the advantages of maintaining pulmonary valve function, preserving the capacity for growth, and avoiding problems inherent to the right ventricular to pulmonary artery conduit. This patient progressed well for 9 months postoperatively and we report this case of pulmonary root translocation with the Lecompte maneuver.
Arteries* ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular* ; Humans ; Male ; Pulmonary Artery ; Pulmonary Valve ; Pulmonary Valve Stenosis* ; Transposition of Great Vessels

OBJECTIVE: To analyze the spectrum of prenatally diagnosed congenital heart disease in a Korean population with 22q11.2 deletion syndrome, and to provide guidelines for screening 22q11.2 deletion prenatally. METHODS: This retrospective study evaluated 1,137 consecutive fetuses that had prenatal genetic testing for 22q11.2 deletion because of suspected congenital heart disease between September 2002 and December 2012, at Asan Medical Center, Seoul, Korea. RESULTS: Main cardiovascular diseases in the 53 fetuses with confirmed 22q11.2 deletions were tetralogy of Fallot (n = 24, 45%), interrupted aortic arch (n = 10, 19%), ventricular septal defect (n = 5, 9%), double outlet right ventricle (n = 4, 8%), and coarctation of the aorta (n = 4, 8%). Other cardiac defects were rarely associated with 22q11.2 deletion. One fetus had persistent truncus arteriosus, one had aortic stenosis, and one had hypoplastic right heart syndrome. Two fetuses had normal intracardiac anatomy with an isolated right aortic arch, and one had an isolated bilateral superior vena cava. CONCLUSION: A variety of congenital heart diseases were seen during the prenatal period. Conotruncal cardiac defects except transposition of great arteries were strongly associated with 22q11.2 deletion. When such anomalies are diagnosed by fetal echocardiography, genetic testing for 22q11.2 deletion should be offered. Even if less frequent deletion-related cardiac defects are detected, other related anomalies, such as thymic hypoplasia or aplasia, should be evaluated to rule out a 22q11.2 deletion.
Aorta, Thoracic ; Aortic Coarctation ; Aortic Valve Stenosis ; Cardiovascular Diseases ; Chungcheongnam-do ; DiGeorge Syndrome* ; Double Outlet Right Ventricle ; Echocardiography ; Fetus ; Genetic Testing ; Heart ; Heart Defects, Congenital* ; Heart Diseases ; Heart Septal Defects, Ventricular ; In Situ Hybridization, Fluorescence ; Korea ; Mass Screening ; Retrospective Studies ; Seoul ; Tetralogy of Fallot ; Transposition of Great Vessels ; Truncus Arteriosus, Persistent ; Vena Cava, Superior

A nonrestrictive interatrial communication is mandatory in some patients with complex cyanotic heart disease. Up to the present time, Park blade atrial septostomy with a Park blade catheter is performed only under fluoroscopic guidance. We successfully monitored only under transthoracic 2-dimensional (2D) echocardiography (TTE), in a 7-week-old infant with d-Transposition of Great Arteries (d-TGA), a restrictive patent foramen ovale (PFO), and small ventricular septal defect (VSD), who presented with severe hypoxemia. TTE confirmed the position of the blade before and during pullback to the right atrium, followed by balloon septoplasty. There was an adequate enlargement of the interatrial communication with subsequent improvement in the oxygenation of the infant. This is the first reported case of the performance of blade atrial septostomy as a bedside TTE guided procedure here in the Philippines.

Human ; Male ; Infant ; Heart Septal Defects, Ventricular ; Foramen Ovale, Patent ; Philippines ; Echocardiography ; Heart Atria ; Cardiac Surgical Procedures ; Transposition Of Great Vessels ; Hypoxia