1/5. 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 = 1keywords = vascular disease (Clic here for more details about this article) |
2/5. Cerebrovascular disease associated with sickle cell pulmonary hypertension.In patients with sickle cell disease, anemia is a recognized risk factor for stroke, death, and the development of pulmonary hypertension. We have proposed that hemolytic anemia results in endothelial dysfunction and vascular instability and can ultimately lead to a proliferative vasculopathy leading to pulmonary hypertension. Consistent with this mechanism of disease, we now report a case series of six patients with obliterative central nervous system vasculopathy who also have pulmonary hypertension and high hemolytic rate. These patients, identified in the course of a prospective screening study for pulmonary hypertension, presented with neurological symptoms prompting neuroimaging studies. Compared to 164 other patients of similar age in the screened population, those with newly diagnosed or clinically active cerebrovascular disease have significantly lower hemoglobin levels and higher levels of lactate dehydrogenase. A review of the literature suggests that many clinical, epidemiological, and physiological features of the arteriopathy of pulmonary hypertension closely overlap with those of stroke in sickle cell disease, both known to involve proliferative vascular intimal and smooth muscle hypertrophy and thrombosis. These cases suggest that cerebrovascular disease and pulmonary hypertension in sickle cell disease share common mechanisms, in particular, reduced nitric oxide bioactivity associated with particularly high-grade hemolysis. Clinicians should suspect occult cerebrovascular disease in sickle cell patients with pulmonary hypertension.- - - - - - - - - - ranking = 0.77777777777778keywords = vascular disease (Clic here for more details about this article) |
3/5. Hypertrophic spinal pachymeningitis presenting as compressive myelopathy: a case report.Hypertrophic pachymeningitis (HP) is a rare chronic inflammatory process that causes thickening of the dura leading to compressive myelopathy. HP has diverse etiologies like infections, chronic inflammatory processes, collagen vascular diseases and malignancy. We report one such case of HP who presented with compressive myelopathy, underwent decompressive surgery and died due to complications of surgery with the original disease process.- - - - - - - - - - ranking = 0.11111111111111keywords = vascular disease (Clic here for more details about this article) |
4/5. Genitourinary neurofibromatosis in childhood.Neurofibromatosis in children occurs uncommonly and involvement of the bladder is exceedingly rare. The syndrome is transmitted as an autosomal dominant trait and is characterized by cutaneous pigmentation and tumors of neural crest origin. Hypertension has been documented in association with neurofibromatosis in the form of renovascular disease in children and with pheochromocytomas in adults. We report on 2 children with extensive neurofibromas of the bladder, upper tract damage and genital enlargement. One case was misdiagnosed as posterior urethral valves and the other case as clitoral hypertrophy. The literature of urogenital neurofibromatosis is reviewed with particular attention to bladder lesions, hypertension, malignant potential and treatment options.- - - - - - - - - - ranking = 0.11111111111111keywords = vascular disease (Clic here for more details about this article) |
5/5. Progressive pulmonary vascular disease after surgery in a case of patent ductus arteriosus with pulmonary hypertension.In a 15-month-old girl with patent ductus arteriosus (PDA) with pulmonary hypertension, division of the PDA was undertaken, but she died of heart failure 7 months post-operatively. Morphometric study of biopsy and autopsy lungs showed that medial hypertrophy and pulmonary intimal lesions developed markedly after surgery. Severe pulmonary hypertension and hypoxemia were present preoperatively. The pulmonary hypertension remaining postoperatively and aggravated pulmonary hypoxemia are thought to have caused postoperative constriction of the pulmonary vessels and to bring about unusual medial hypertrophy. Since it is known that marked hypertrophy of the media can easily cause vasospasms, it is thought that, in the present case, the smooth muscle cells of the media became necrotic, which brought about damage to endothelial cells. Such damage, in turn, led to the development of occlusive pulmonary vascular disease.- - - - - - - - - - ranking = 0.55555555555556keywords = vascular disease (Clic here for more details about this article) |