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 ...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.展开更多
<strong>Background:</strong> <span style="font-family:;" "="">Children presenting with physical features of chondro-ectodermal</span><span style="font-family:;&...<strong>Background:</strong> <span style="font-family:;" "="">Children presenting with physical features of chondro-ectodermal</span><span style="font-family:;" "=""> 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. <b>Aim:</b> A child with physical stigmata of Ellis-Van-Creveld syndrome is evaluated to detect an associated congenital cardiac anomaly and <span>accomplish successful repair of the underlying cardiac lesion to reduce the cardiac</span> related morbidity and improve the patient survival. <b>Case Presentation:</b> Ten year</span><span style="font-family:;" "="">s</span><span style="font-family:;" "=""> 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. <span>The Qp/Qs was 3.4: 1, with systemic arterial oxygen desaturation (SaO<sub>2</sub> of 0.7) </span>and O<sub>2</sub> 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</span><span style="font-family:;" "=""> </span><span style="font-family:;" "="">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</span><span style="font-family:;" "="">-</span><span style="font-family:;" "="">year follow-up, the child was well without AV valve regurgitation and had normal <span>biventricular function. <b>Conclusion:</b> A child with Ellis-Van-Creveld syndrome</span> 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 <span>the common atrium into left and right atrium by a complex atrial routing tech<span>nique with two patches of Gore-Tex. On a follow-up at 2 years</span></span></span><span style="font-family:;" "="">, </span><span style="font-family:;" "="">the patient had</span><span style="font-family:;" "=""> adequate biventricular function without AV valve regurgitation.</span> <div class="__kindeditor_paste__" style="position:absolute;width:1px;height:1px;overflow:hidden;left:-1981px;top:0px;white-space:nowrap;"> <table width="100%" border="0" cellpadding="0" cellspacing="1" bgcolor="#cacfd2" style="border:0px solid #CCCCCC;line-height:25px;width:1041px;color:#000000;font-family:宋体, Arial, sans-serif;"> <tbody> <tr style="background-color:#FAFBFD;"> <td style="text-align:center;font-size:14px;vertical-align:middle;"> <div align="center"> 114264<strong></strong> </div> </td> </tr> </tbody> </table> </div>展开更多
文摘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.
文摘<strong>Background:</strong> <span style="font-family:;" "="">Children presenting with physical features of chondro-ectodermal</span><span style="font-family:;" "=""> 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. <b>Aim:</b> A child with physical stigmata of Ellis-Van-Creveld syndrome is evaluated to detect an associated congenital cardiac anomaly and <span>accomplish successful repair of the underlying cardiac lesion to reduce the cardiac</span> related morbidity and improve the patient survival. <b>Case Presentation:</b> Ten year</span><span style="font-family:;" "="">s</span><span style="font-family:;" "=""> 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. <span>The Qp/Qs was 3.4: 1, with systemic arterial oxygen desaturation (SaO<sub>2</sub> of 0.7) </span>and O<sub>2</sub> 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</span><span style="font-family:;" "=""> </span><span style="font-family:;" "="">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</span><span style="font-family:;" "="">-</span><span style="font-family:;" "="">year follow-up, the child was well without AV valve regurgitation and had normal <span>biventricular function. <b>Conclusion:</b> A child with Ellis-Van-Creveld syndrome</span> 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 <span>the common atrium into left and right atrium by a complex atrial routing tech<span>nique with two patches of Gore-Tex. On a follow-up at 2 years</span></span></span><span style="font-family:;" "="">, </span><span style="font-family:;" "="">the patient had</span><span style="font-family:;" "=""> adequate biventricular function without AV valve regurgitation.</span> <div class="__kindeditor_paste__" style="position:absolute;width:1px;height:1px;overflow:hidden;left:-1981px;top:0px;white-space:nowrap;"> <table width="100%" border="0" cellpadding="0" cellspacing="1" bgcolor="#cacfd2" style="border:0px solid #CCCCCC;line-height:25px;width:1041px;color:#000000;font-family:宋体, Arial, sans-serif;"> <tbody> <tr style="background-color:#FAFBFD;"> <td style="text-align:center;font-size:14px;vertical-align:middle;"> <div align="center"> 114264<strong></strong> </div> </td> </tr> </tbody> </table> </div>
