1/7. gadolinium-enhanced magnetic resonance angiography in scimitar syndrome: diagnosis and postoperative evaluation.We report the 1st magnetic resonance demonstration of both an anomalous pulmonary venous drainage and systemic arterial supply in a patient with scimitar syndrome. With its superior imaging capabilities, gadolinium-enhanced magnetic resonance angiography provides a powerful diagnostic tool for this complex congenital lesion and offers the possibility of surgical repair and follow-up without conventional cardiac catheterization.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
2/7. Retrospective prenatal diagnosis of scimitar syndrome aided by three-dimensional power Doppler imaging.scimitar syndrome is a rare malformation of the arterial supply and venous drainage of the lung. We report the case of a fetus that presented with cardiac asymmetry and malposition of the fetal heart. Postnatally, scimitar syndrome was confirmed at cardiac catheterization. Retrospective reconstruction of three-dimensional power Doppler volumes, obtained during fetal life, allowed direct visualization of the abnormal aortopulmonary collateral vessel. This had not been seen on conventional scans. This case demonstrates many of the strengths of three-dimensional sonographic techniques for the delineation of complex vascular anatomy. It confirms that a prospective diagnosis of scimitar syndrome should be possible during fetal life.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
3/7. Acute pulmonary edema caused by epoprostenol infusion in a child with scimitar syndrome and pulmonary hypertension.INTRODUCTION: Intravenous epoprostenol is frequently administered in adults and children for treatment of pulmonary hypertension. Although generally safe, pulmonary edema has been described in a few case reports of adult patients with pulmonary veno-occlusive disease. CASE REPORT: We present an infant who had an operation for scimitar syndrome and abnormal drainage of the right pulmonary veins into the inferior vena cava who developed pulmonary edema while receiving a prostacyclin infusion. The typical partial anomalous pulmonary venous drainage was operatively corrected at 6 days of age, and an accompanying coarctation was resected. At 7 months of age, diagnostic cardiac catheterization was performed to evaluate suspected pulmonary hypertension. Pulmonary pressure was elevated to supra-systemic values, and obstructed venous drainage of the right hypoplastic lung was demonstrated. To decrease pulmonary hypertension during weaning and extubation, epoprostenol infusion was initiated. Sixty minutes after extubation, massive acute pulmonary edema lead to reintubation. Mean airway pressure of 16 mm Hg (21 mbar) with pure oxygen ventilation was initially required, with an oxygenation index of 14, a ventilation index of 36, and an alveolar-arterial oxygen tension difference of 541 mm Hg. After discontinuation of epoprostenol, weaning and extubation was successful. CONCLUSION: pulmonary edema caused by prostacyclin infusion in patients with impaired postcapillary pulmonary drainage may also be encountered in children and has to be anticipated.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
4/7. Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome.Scimitar vein draining to the left atrium is the rarest of the pulmonary venolobar anomalies with less than a dozen reported cases. A case is reported of a patient whose plain film radiographic findings showed a typical scimitar vein but whose echocardiography showed normal pulmonary venous drainage. cardiac catheterization confirmed drainage of the scimitar vein to the left atrium and systemic arterial supply which was embolized. The case described is used to review the history of "scimitar syndrome" with reflections on the significant contributions of Halasz and colleagues, who in 1956 helped define the anatomy, and Neill and colleagues, who in 1960 described the familial occurrence and clinical spectrum of the condition.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
5/7. scimitar syndrome with absence of the right pulmonary artery: a case with volume-induced, reversible, left-sided pulmonary hypertension.An infant with scimitar syndrome, absent right pulmonary artery, and systemic blood supply to the right lung presented in severe cardiac failure. cardiac catheterization revealed suprasystemic pressure of the left pulmonary artery and a high pulmonary vascular resistance. Right-sided pneumonectomy abolished cardiac failure and normalized both pulmonary artery pressure and resistance. Pure volume load affecting one lung--as in this case through absence of the right pulmonary artery plus additional left-to-right shunt from a systemic collateral--can lead to pulmonary hypertension. Early operative intervention can reverse this process and prevent pulmonary vascular disease.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
6/7. An infant with the scimitar syndrome and pulmonary hypertension; successful surgical intervention.The case of an infant with the scimitar syndrome and pulmonary hypertension is presented. After surgical intervention with ligation of the abnormal arterial supply to the hypoplastic lung, the pulmonary hypertension disappeared. This was proven by cardiac catheterization after the operation. The abnormal drainage from the right lung into the inferior vena cava was not corrected. The child has had no complaints since except for recurrent pulmonary infections.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
7/7. scimitar syndrome complicated with right pneumonectomy syndrome: report of one case.A 2-month-old infant, suffering from respiratory distress, was suspected to have scimitar syndrome from a chest roentgenogram and was diagnosed by cardiac catheterization. ligation of the aberrant feeding arteries and right lung resection was performed at three and four months of age, respectively. However, the postoperative course was complicated with right pneumonectomy syndrome requiring continuous artificial ventilation. With supportive management, the patient improved gradually and was eventually weaned off the ventilator at three years of age.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |