1/10. Complete atrioventricular septal defect and Ebstein's anomaly.A case child with complete atrioventricular septal defect (AVSD) and Ebstein's anomaly underwent surgical treatment at 3 months of age. She died on the third postoperative day. Postmortem examination showed complete AVSD, downward displacement of the right atrioventricular valve, left ventricular outflow tract obstruction, and hypertensive pulmonary vascular disease. association of complete AVSD and Ebstein's anomaly is a rare cardiac anomaly for which no attempt at surgical repair has previously been made. This report deals with our experience and also with the morphological features of this anomaly.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/10. Paradoxical embolization in an adult patient with cystic fibrosis.To our knowledge, we describe the first reported case of paradoxical embolization via a patent foramen ovale (PFO) in an adult with moderately severe cystic fibrosis (CF) and advanced lung disease. Fluctuating neurologic symptoms and signs suggestive of cerebrovascular disease in an adult patient with advanced CF may be due to paradoxical embolization via a PFO. The possibility of a PFO should be considered before placement of a totally implantable venous access device to avert unnecessary risk of stroke in CF patients. Further study is needed to determine whether the use of a totally implantable venous access device increases the risk of paradoxical embolization in adult CF patients with a PFO.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
3/10. Atrial septal defect with failure to thrive in infancy: hidden pulmonary vascular disease?Atrial septal defects are usually asymptomatic, and are closed surgically or by a catheter implanted device in preschool age children. Rarely, they may cause symptoms in infancy, and management at this age is debated. We report our experience of six infants who underwent surgical closure, with variable outcomes. Five of these had significant extracardiac pathology. Our data suggest the need to exclude other causes of symptoms, both cardiac and non-cardiac. The question should be asked as to whether surgery is of benefit in these children, and particularly whether they may have underlying primary pulmonary vascular disease.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
4/10. Atrial septal defect with borderline pulmonary vascular disease: surgery and long-term oral prostacyclin therapy for recalcitrant pulmonary hypertension.The hemodynamic determination of operability in atrial septal defect (ASD) with severe pulmonary hypertension is problematic. Therefore, we perform an open lung biopsy prior to the corrective surgery in cases with pulmonary vascular resistance greater than 8 units x m2 and/or pulmonary arterial peak pressure greater than 70 mmHg. We present 4 cases showing occlusion of more than 70% of the small pulmonary arteries and arterioles by musculoelastosis, thromboembolism and mixed-type (musculoelastosis and plexogenic arteriopathy) which was considered borderline in terms of operability. After complete closure of the ASD and postoperative long-term oral prostacyclin (PGI2) therapy, pulmonary artery peak pressure decreased from 110-72 (mean 84) to 105-45 (mean 74) mmHg immediately after operation and 65-40 (mean 57) mmHg after PGI2 therapy. The New York Heart association functional status of the patients improved from class II-III to class I with oral PGI2 only. Our cases demonstrate that despite more than 70% occlusion of the small pulmonary arteries and arterioles, surgery and long-term PGI2 therapy can reduce pulmonary artery pressure and improve the quality of life.- - - - - - - - - - ranking = 4keywords = vascular disease (Clic here for more details about this article) |
5/10. Lung biopsy diagnosis of operative indication in secundum atrial septal defect with severe pulmonary vascular disease.OBJECTIVE: Surgical indication was determined by lung biopsy in 91 patients with secundum atrial septal defect (ASD) and severe pulmonary hypertension > 70 mm Hg of pulmonary arterial peak pressure and/or pulmonary vascular resistance of > 8 U/m(2). methods AND RESULTS: Pulmonary vascular disease (PVD) in ASD was classified into four types: (1) Musculoelastosis consisting of longitudinal muscle bundles and elastic fibers; surgery is indicated no matter how severely the peripheral small pulmonary arteries are occluded. Surgery was performed in all of the 20 patients, and the postoperative course was uneventful. (2) Plexogenic pulmonary arteriopathy: surgery is indicated for a PVD index < or = 2.3. Surgery was performed in 25 of the 32 patients. The remaining seven patients for whom surgery was not indicated are under follow-up observation. No deaths have occurred among the 32 patients. (3) thromboembolism of small pulmonary arteries: Surgery is indicated for all such cases. Surgery was indicated in all of the five patients. (4) Mixed type of plexogenic pulmonary arteriopathy and musculoelastosis: Surgery is indicated if the collateral is not observed. Surgery was performed in 15 of the 25 patients. The remaining 10 patients for whom surgery was not indicated are under follow-up observation. Nine of these 91 patients associated with primary pulmonary hypertension were eliminated from this study. CONCLUSION: No deaths due to PVD occurred among the 82 patients who underwent lung biopsy diagnosis. Lung biopsy diagnosis is concluded to be very effective.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
6/10. Single-lung transplantation with atrial septal defect repair for Eisenmenger's syndrome.heart-lung transplantation has been used successfully for patients with pulmonary vascular disease but its application has been very limited due to the scarcity of donors. We report a patient with Eisenmenger's syndrome who underwent right single-lung transplantation with closure of atrial septal defect; postoperative convalescence was uneventful. Serial magnetic resonance imaging examinations demonstrate improved right heart function.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/10. Ostium secundum atrial septal defect in the elderly.Atrial septal defect (ASD) is one of the most common congenital cardiac anomalies in adults. life expectancy is shortened, and almost 90% of patients die by the age of 60 years. The progression of this congenital disease to congestive heart failure has been related to several factors such as the onset of pulmonary hypertension, arrhythmias, bronchopulmonary infections, or the development of other cardiovascular disease. We describe three cases of very old patients with significant ASDs and late development of symptoms. Given the higher risks and poorer long-term results of surgical closure of the defect in advanced age, the indications for such an intervention in elderly patients should be carefully evaluated.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/10. Pulmonary vascular disease in secundum atrial septal defect in childhood: an unusual evolution.Reported is a case of a 16-month-old girl with an isolated atrial septal defect in whom severe pulmonary hypertension has developed in 26 months in spite of an important functional gradient across the pulmonic valves at a first catheterization. Individual susceptibility to an increased pulmonary blood flow is evoked.- - - - - - - - - - ranking = 4keywords = vascular disease (Clic here for more details about this article) |
9/10. Pulmonary vascular disease in a child with atrial septal defect of the secundum type and type I glycogen storage disease.A rare combination of atrial septal defect of the secundum type and type I glycogen storage disease was seen with early fatal outcome due to rapidly progressive severe pulmonary vascular disease. Possible reasons for the unexpected, rapidly progressive pulmonary vascular disease are discussed.- - - - - - - - - - ranking = 6keywords = vascular disease (Clic here for more details about this article) |
10/10. Unusual longevity without surgical intervention in complete transposition of the great arteries.A case of unusual longevity to the age of 58 years is reported for a female patient with complete transposition of the great arteries. The association with a wide atrial septal defect with intact interventricular septum may have contributed to the long survival without surgery. Factors determining intercirculatory mixing and systemic oxygen saturation may be the high pulmonary flow, the location of the anatomic communication, sufficient hemoglobin concentration to allow an adequate level of systemic resistance and recirculated systemic flow, and the belated development of pulmonary vascular disease.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
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