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

Heterotaxy syndrome (HS) is a congenital disorder resulting from an abnormal arrangement of visceral organs across the normal left-right axis in the embryonic period. HS is usually associated with multiple anomalies, including defects of the major cardiovascular system and the extracardiovascular system such as intestinal malrotation, abnormal lung lobulation, bronchus anomalies, and pancreatic dysplasia. Although pancreatic dysplasia is occasionally accompanied with HS, the occurrence of diabetes mellitus (DM) due to pancreatic dysplasia in HS is rarely reported. We here report a case involving 13-year-old girl with DM caused by agenesis of the dorsal pancreas and HS diagnosed on the basis of the presence of a double-outlet right ventricle with bilateral pulmonary stenosis and intestinal malrotation with duodenal cyst. Timely diagnosis and treatment with insulin improved glycemic control.
Adolescent ; Bronchi ; Cardiovascular System ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Diabetes Mellitus* ; Diagnosis ; Double Outlet Right Ventricle ; Female ; Heterotaxy Syndrome* ; Humans ; Insulin ; Lung ; Pancreas* ; Pulmonary Valve Stenosis

No abstract available.
Arterial Switch Operation* ; Arteries* ; Catheter Ablation* ; Catheters* ; Double Outlet Right Ventricle* ; Humans ; Tachycardia, Ventricular*

Double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis (PS) are complex heart diseases, the treatment of which remains a surgical challenge. The Rastelli procedure is still the most commonly performed treatment. Aortic root translocation including an arterial switch operation is advantageous anatomically since it has a lower possibility of conduit blockage and the left ventricle outflow tract remains straight. This study reports successful aortic root transpositions in two patients, one with DORV with VSD and PS and one with TGA with VSD and PS. Both patients were discharged without postoperative complications.
Arterial Switch Operation ; Arteries* ; Double Outlet Right Ventricle* ; Heart Diseases ; Heart Septal Defects, Ventricular* ; Heart Ventricles ; Humans ; Postoperative Complications ; Pulmonary Valve Stenosis*

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