A 63-Year-Old Male with D-Transposition of the Great Arteries Who Had an Early Form of the Arterial Switch Operation

We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the mid-1960s.We review the clinical and imaging data that support our conclusion.He had a diagnostic cardiac catheterization which demonstrated severe pulmonary hypertension responsive to epoprostenol and oxygen.Our case may represent one example of the experimental surgical work done prior to Dr.Adibe Jatene’s description of thefirst successful arterial switch performed in 1975.

3.0T MR Coronary Angiography after Arterial Switch Operation for Transposition of The Great Arteries-Gd-FLASH Versus Non-Enhanced SSFP.A Feasibility Study

Background:Patency of the coronary arteries is an issue after reports of sudden cardiac death in patients with transposition of the great arteries(TGA)operated with arterial switch(ASO).Recent studies give rise to concern regarding the use of ionising radiation in congenital heart disease,and assessment of the coronary arteries with coronary MR angiography(CMRA)might be an attractive non-invasive,non-ionising imaging alternative in these patients.Theoretically,the use of 3.0T CMRA should improve the visualisation of the coronary arteries.The objective of this study was to assess feasibility of 3.0T CMRA at the coronary artery origins by comparing image quality with non-contrast CMRA in ASO TGA patients to healthy age-matched controls,and by comparing image quality with non-contrast CMRA to contrast enhanced CMRA in the patient group.Material and methods:Twelve patients,9-15 years(mean 11.9 years,standard deviation 1.5 years),and 12 age-matched controls(mean 12.7 years,standard deviation 1.7 years)were examined with 3D balanced steady-state free precession(SSFP).Nine of twelve patients had Gadolinium-enhanced fast low-angle shot(Gd-FLASH)performed after SSFP.Image quality at the coronary artery origins was evaluated subjectively with a 10 cm figurative visual analogue scale(fVAS)and objectively by signal-to-noise and contrast-to-noise ratio(SNR,CNR).Results:All,but one,coronary artery origins were identified.No significant difference in image quality scores was found between patients and controls with SSFP(mean values 6.5 cm—9.1 cm in patients and 7.0 cm—8.0 cm in controls,p-values>0.1).With SSFP,intra-observer fVAS mean score was 6.7 cm—8.6 cm and with Gd-FLASH 7.7 cm—8.7 cm.CNR was higher with Gd-FLASH(p<0.03).Intra-observer agreement index(AI)with SSFP was moderate-to-good(0.43–0.71)and with Gd-FLASH good(0.64–0.79)in all origins.Inter-observer AI was good in the left main stem(LMS)with SSFP(0.65).With Gd-FLASH inter-observer AI was good in LMS(0.78)and moderate(0.5)in the left anterior descending artery,but lacking in the other origins though with a good agreement on Bland-Altman plots.Conclusions:Our findings indicate a better,more reproducible image quality with Gd-FLASH than with non-contrast SSFP CMRA on 3.0T for evaluation of the coronary artery origins in ASO TGA children and adolescents.

Morphological Features of Complex Congenital Cardiovascular Anomalies in Fetuses:as Evaluated by Cast Models

Accurate prenatal diagnosis of complex congenital cardiovascular anomalies, vascular ones in particular, is still challenging. A fetal cardiovascular cast model can provide a copy of the cardiac chambers and great vessels with normal or pathological structures. This study was aimed to demonstrate three-dimensional anatomy of complex congenital cardiovascular anomalies in fetuses by means of corrosion casting. Twenty fetuses with prenatal-ultrasound-diagnosed complex cardiovascular anomalies were enrolled in this study（19 to 35 gestational weeks）. Fetal cardiovascular cast models were made by a corrosion casting technique. The specimens were injected with casting material via the umbilical vein, and then immersed in strong acid after casting fluid was solidified, to disclose the geometries of cardiovascular cavities. Nineteen cast models were successfully made from 20 specimens. The casts distinctly showed the morphological malformations and spatial relationship between cardiac chambers and great vessels. One hundred and eleven abnormalities were revealed by casting in the 19 specimens, including 34 abnormalities located in the cardiac chambers（3, 4 and 27 anomalies in the atria, atrioventricular valves and ventricles, respectively）, and 77 in the great vessels（28, 20, 24 and 5 anomalies in the aorta and its branches, the pulmonary artery, the ductus arteriosus and the major veins, respectively）. Corrosion casting can display three-dimensional anatomy of fetal complex cardiovascular anomalies. This improves our understanding of related pathomorphology and prenatal diagnosis.

Isolated Type C Interrupted Aortic Arch in Adult:Extra-anatomic Repair Using Circulatory Arrest

INTERRUPTED aortic arch （IAA） is a rare congenital malformation that occurs in 5.8 per million live births. 1 IAA was thought to be incompatible with life once the ductus arteriosus closed. However, ifextensive collateral circulation is p possible. We report treated successfully an unusual resent at case of with extra-anatomic under deep hypothermia drculatory arrest birth survival is IAA, who was aortic repair

Ellis-Van-Creveld Syndrome and Congenital Cardiac Anomaly: Common Atrium with Atrioventricular Canal Septal Defect

Background: Children presenting with physical features of chondro-ectodermal dysplasia (Ellis-Van Creveld syndrome) such as skeletal and joint abnormalities often have concomitant congenital cardiac anomalies. Presence of cardiorespiratory symptoms in children with Ellis-Van Craved syndrome warrants a thorough cardiologic evaluation to recognize and treat underlying congenital heart anomaly. Aim: A child with physical stigmata of Ellis-Van-Creveld syndrome is evaluated to detect an associated congenital cardiac anomaly and accomplish successful repair of the underlying cardiac lesion to reduce the cardiac related morbidity and improve the patient survival. Case Presentation: Ten years old boy with chondroectodermal dysplasia (dental anomalies, genu valgum and other skeletal abnormalities) presented with dyspnea and cyanosis. Cardiac evaluation by 2D echo revealed an atrioventricular (AV) canal septal defect with AV valve regurgitation and a common atrium. Angiocardiography showed a goose neck deformity of the left ventricular outflow tract. The Qp/Qs was 3.4: 1, with systemic arterial oxygen desaturation (SaO2 of 0.7) and O2 saturation in the common atrium was 0.7. The pulmonary venous connections to the common atrium were anomalous. Atriotomy on cardiopulmonary bypass and on a cardioplegic arrest discerned a partial AV canal septal defect with a common bridging leaflet, clefts in septal leaflets of tricuspid and mitral vlalves, an incompletely closed interventricular communication, and a common atrium with highly anomalous pulmonary venous insertions well anterior (8 cm) to vena caval orifices. Intracardiac repair was performed with two patches of Goertex to partition the common atrium into the pulmonary and systemic venous chambers after repair of the partial AV canal septal defect. Patient required only a temporary afterload reduction with enalapril;otherwise patient had an uneventful postoperative course. At a 2-year follow-up, the child was well without AV valve regurgitation and had normal biventricular function. Conclusion: A child with Ellis-Van-Creveld syndrome with skeletal abnormalities and dental anomalies had manifested with cardio-respiratory symptoms. Preoperative cardiac and intraoperative evaluation showed a common atrium with severely anomalous pulmonary venous connection and partial AV canal septal defect. Successful biventricular repair was accomplished by repairing the partial AV canal septal defect and partitioning the common atrium into left and right atrium by a complex atrial routing technique with two patches of Gore-Tex. On a follow-up at 2 years, the patient had adequate biventricular function without AV valve regurgitation. 114264

Surgical management of mediastinal tumor invading the heart and large blood vessels

Background Surgical removal plays an irreplaceable important role in mediastinal tumor therapy. With advances of anesthesia, cardiopulmonary bypass and vascular surgery technology, even tumor invading the heart and great vessels can also be removed. Patients get good results after radical surgery. Methods Review The perioperative treatment was reviewed for 36 patients with complicated mediastinal tumor involving the heart and great vessels from February 1997 to March 2014. Out of them, 2 patients underwent extracorporeal circulation; one patient underwent superior vena cava and left innominate vein replacement besides the resection of mediastinal tumor; one did superior vena cava replacement, 3 patients received partial right atrial resection, 5 did superior vena cava angioplasty, 4 did innominate vein resection, 5 did innominate vein angioplasty, 10 did partial excision and repair of pericardium, 8 did pulmonary wedge resection successfully,and 2 did lobectomy. Results All 36 procedures were completed with no perioperative death. Clinical outcome was satisfactory. Conclusions Surgical management for complicated mediastinal tumor invading the heart and great vessels is difficult and risky. Therefore, comprehensive perioperative preparation and appropriate surgical procedures are indispensable for a successful surgery and desirable therapeutic effect.