The authors report the cases of two neonates with retro-tracheal left pulmonary arteries(pulmonary artery sling). In the first case, there was rapid onset of cardiac failure with signs of airway compression. Echocardi...The authors report the cases of two neonates with retro-tracheal left pulmonary arteries(pulmonary artery sling). In the first case, there was rapid onset of cardiac failure with signs of airway compression. Echocardiography showed the vascular anomaly associated with a large ventricular septal defect(VSD). After standard radiological investigation and bronchoscopy to exclude an associated tracheo-bronchial malformation, the VSD was repaired surgically with reimplantation of the left pulmonary artery. Unfortunately, the patient died of major airways obstruction in the postoperative period. Autopsy showed tracheo-bronchial anomalies which had not been diagnosed preoperatively. The second patient presented with hypoventilation of the right lung. After echocardiographic diagnosis of the anomaly, a thorough investigation(thoracic CT, helicoidal scan, bronchoscopy)was carried out and no associated bronchial malformations were observed. Reimplantation of the left pulmonary artery was successful and the postoperative course was uneventful. Retro-tracheal left pulmonary artery is a rare malformation which is difficult to diagnose. It requires extensive pulmonary investigations and a multi-disciplinary approach. The prognosis is poor when there are associated tracheo-bronchial malformations.展开更多
Objective: We evaluated the cardiovascular pathologic condition in the recipient twin in twin- to- twin transfusion syndrome and the influence of amnioreduction. Study design: Fetal echocardiograms and medical records...Objective: We evaluated the cardiovascular pathologic condition in the recipient twin in twin- to- twin transfusion syndrome and the influence of amnioreduction. Study design: Fetal echocardiograms and medical records of 54 pregnancies that were complicated by twin-to-twin transfusion syndrome were reviewed. Recipient twin right and left ventricular wall thickness, diameters, systolic and diastolic function, valve regurgitation, and structural cardiac defects were assessed at examination and after amnioreduction. Results: At examination (n = 28 pregnancies), cardiomegaly because of right ventricular and/or left ventricular hypertrophy was observed in 58% of recipient twins, and biventricular hypertrophy was observed in 33% of recipient twins, without ventricular dilation. Biventricular diastolic dysfunction was present in two thirds of recipient twins, and right ventricular systolic dysfunction and significant atrioventricular valve regurgitation was observed in one third of recipient twins. Serial assessment (n = 21 pregnancies) revealed progressive biventricular hypertrophy and right ventricular systolic and biventricular diastolic dysfunction in most recipient twins. Steeper progression of hypertrophy, diastolic dysfunction, and structural or functional right ventricular outflow disease (20% incidence) were associated with an increased perinatal mortality rate. Conclusion: In twin- to- twin transfusion syndrome, the recipient twin has progressive biventricular hypertrophy with predominant right ventricular systolic and biventricular diastolic dysfunction. Despite amnioreduction, the cardiovascular disease persists and even progresses in many recipient twins.展开更多
文摘The authors report the cases of two neonates with retro-tracheal left pulmonary arteries(pulmonary artery sling). In the first case, there was rapid onset of cardiac failure with signs of airway compression. Echocardiography showed the vascular anomaly associated with a large ventricular septal defect(VSD). After standard radiological investigation and bronchoscopy to exclude an associated tracheo-bronchial malformation, the VSD was repaired surgically with reimplantation of the left pulmonary artery. Unfortunately, the patient died of major airways obstruction in the postoperative period. Autopsy showed tracheo-bronchial anomalies which had not been diagnosed preoperatively. The second patient presented with hypoventilation of the right lung. After echocardiographic diagnosis of the anomaly, a thorough investigation(thoracic CT, helicoidal scan, bronchoscopy)was carried out and no associated bronchial malformations were observed. Reimplantation of the left pulmonary artery was successful and the postoperative course was uneventful. Retro-tracheal left pulmonary artery is a rare malformation which is difficult to diagnose. It requires extensive pulmonary investigations and a multi-disciplinary approach. The prognosis is poor when there are associated tracheo-bronchial malformations.
文摘Objective: We evaluated the cardiovascular pathologic condition in the recipient twin in twin- to- twin transfusion syndrome and the influence of amnioreduction. Study design: Fetal echocardiograms and medical records of 54 pregnancies that were complicated by twin-to-twin transfusion syndrome were reviewed. Recipient twin right and left ventricular wall thickness, diameters, systolic and diastolic function, valve regurgitation, and structural cardiac defects were assessed at examination and after amnioreduction. Results: At examination (n = 28 pregnancies), cardiomegaly because of right ventricular and/or left ventricular hypertrophy was observed in 58% of recipient twins, and biventricular hypertrophy was observed in 33% of recipient twins, without ventricular dilation. Biventricular diastolic dysfunction was present in two thirds of recipient twins, and right ventricular systolic dysfunction and significant atrioventricular valve regurgitation was observed in one third of recipient twins. Serial assessment (n = 21 pregnancies) revealed progressive biventricular hypertrophy and right ventricular systolic and biventricular diastolic dysfunction in most recipient twins. Steeper progression of hypertrophy, diastolic dysfunction, and structural or functional right ventricular outflow disease (20% incidence) were associated with an increased perinatal mortality rate. Conclusion: In twin- to- twin transfusion syndrome, the recipient twin has progressive biventricular hypertrophy with predominant right ventricular systolic and biventricular diastolic dysfunction. Despite amnioreduction, the cardiovascular disease persists and even progresses in many recipient twins.
