1/15. 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) |
2/15. Progressive pulmonary vascular disease after pulmonary artery banding and total correction in a case of ventricular septal defect and pulmonary hypertension.A 7-month-old infant with ventricular septal defect and pulmonary hypertension underwent pulmonary artery banding, which resulted in a decrease in the pulmonary arterial peak pressure from 102 to 54 mmHg. lung biopsy findings showed at most an early grade 3 Heath-Edwards classification, and an index of pulmonary vascular disease of 1.4, both of which indicated operability for total correction. Small pulmonary arteries less than 100 microns in diameter, however, showed marked hydropic changes in the medial smooth muscle cells. Total correction was performed at the age of 2 years, but the pulmonary arterial pressure failed to decrease. A lung biopsy taken just after the closure of the ventricular septal defect contraindicated operability due to progressive pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.4. The patient died at 8 months after the operation, and an autopsy revealed still more advanced pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.8. The pathogenesis of arterial changes is discussed.- - - - - - - - - - ranking = 9keywords = vascular disease (Clic here for more details about this article) |
3/15. Palliative arterial repair for transposition, ventricular septal defect, and pulmonary vascular disease.Failure to repair transposition of the great arteries and ventricular septal defect in the young infant results in the early development of pulmonary vascular occlusive disease. Complete repair, preferably by an arterial switch procedure and ventricular septal defect closure, may then not be possible. We report a palliative arterial switch procedure in a 5 1/2-year-old patient with transposition, ventricular septal defect, and severe pulmonary vascular obstructive disease in whom progressive hypoxemia and exercise intolerance developed. An arterial repair without ventricular septal defect closure was performed. After the operation, the child's systemic arterial oxygen saturation and exercise tolerance have substantially improved. Although the progression of pulmonary vascular disease may not be altered, arterial repair can provide effective palliation in this subset of patients.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
4/15. Tissue-specific mosaicism for trisomy 21 and congenital heart disease.Cytogenetic studies in a girl with ventricular septal defect and mosaicism for trisomy 21 showed that trisomy was present in most cells from the myocardium and lung but in only a minority from the skin and lymphocytes. These findings emphasize the importance of tissue-specific mosaicism as a cause of certain cardiovascular diseases.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/15. Peripartum cardiomyopathy with recovery in a patient with coincidental Eisenmenger ventricular septal defect.Severe pulmonary vascular disease was found during pregnancy in a 31 year old woman with a large ventricular septal defect. Three months after delivery peripartum cardiomyopathy with severe biventricular failure developed. This was a chance association. Recovery of ventricular function was slow but was complete two years later. There was no advance in the pulmonary vascular disease.- - - - - - - - - - ranking = 2keywords = vascular disease (Clic here for more details about this article) |
6/15. Post-mortem magnetic resonance imaging provides the diagnosis following aortic rupture in an infant with treated aortic interruption.In the United Kingdom, there has been a steady decline in the number of conventional autopsies performed in children. For cardiovascular disease, structural and not pathological considerations are often more important in defining the cause of death. magnetic resonance imaging is now often used in the assessment of congenital cardiac malformations during life. In this case report, we demonstrate how post-mortem magnetic resonance imaging was able to establish the diagnosis of aortic rupture in a patient who had undergone surgical repair of aortic interruption. In this patient, the parents had declined conventional histopathological autopsy, but were happy to consent to magnetic resonance post-mortem assessment.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/15. Palliative Mustard or Rastelli operation in complete transposition of the great arteries. Option decided by lung biopsy.A case history is reported of a patient with complete transposition of the great arteries and a ventricular septal defect in whom pulmonary hypertension developed, with a pulmonary resistence of 10 units M.2. Because of the possibility that the pulmonary vascular obstructive disease might have been of recent onset and that a high hematocrit value of 82 percent may have interfered with the precise determination of pulmonary resistance, an open lung biopsy was performed. The histologic findings suggested that the vascular disease was as yet reversible. Because of these findings and the aforementioned considerations, a Rastelli operation was performed instead of a palliative Mustard procedure. At present, 6 months postoperatively, the patient is asymptomatic.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/15. Pulmonary vascular disease in children with congenital cardiovascular malformations. Etiologic considerations.A detailed clinical analysis of 16 patients with various forms of congenital cardiac malformations with the potential for large left-to-right shunts who developed extreme pulmonary hypertension in infancy and early childhood has been carried out. The study plan was to include only patients with sufficiently long follow up to allow analysis of their course in both the prepubescent and postpubescent periods. In early life these patients were all characterized by lack of evidence for significant development of the expected left-to-right shunt indicating inappropriate maturation of their pulmonary vascular circulation. Throughout all phases of their life, they were significantly polycythemic in comparison with a control group but this polycythemia, representative of peripheral systemic hypoxemia, increased markedly with the acquisition of puberty in all patients. These two findings indicate both an early abnormality, suggestively primary, of the pulmonary vasculature and a late progression of the disease associated with pubertal maturation.- - - - - - - - - - ranking = 4keywords = vascular disease (Clic here for more details about this article) |
9/15. Surgical treatment of infantile lobar emphysema in cardiovascular disease with left-to-right shunts.Serious symptoms associated with the lobar emphysema in congenital heart diseases with left-to-right shunt disappeared after radical operation for cardiac lesion. The study of seven autopsied cases revealed that the lobar emphysema resulted from check valve mechanism created by compression of the bronchi by distended pulmonary artery. Eight cases of our experience of lobar emphysema disappeared shortly after the operation for congenital heart disease with left-to-right shunt seems to indicate infantile lobar emphysema in congenital heart diseases with left-to-right shunt should not be treated by resection but rather by radical operation for cardiac lesion as soon as possible even in infancy.- - - - - - - - - - ranking = 4keywords = vascular disease (Clic here for more details about this article) |
10/15. Severe pulmonary vascular disease consisting of ventricular septal defect and patent ductus arteriosus. A case diagnosed as the indication for total correction by the hemodynamic study.A 12-year-old girl with ventricular septal defect, patent ductus arteriosus and pulmonary hypertension was hemodynamically diagnosed as the indication for total correction by the initial cardiac catheterization. However, histologic assessment by lung biopsy revealed the terminal stage of plexogenic pulmonary arteriopathy, i.e. plexiform lesions, dilatation lesions and fibrinoid necrosis of the media which denied the possibility of corrective surgery. We would like to stress that the open lung biopsy should be performed when the hemodynamic study indicates marginal operability, especially in older children and adults in order to investigate the grade of occlusive pulmonary vascular disease.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
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