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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The type and distribution of pulmonary vascular lesions in 23 cases of primary pulmonary hypertension were reviewed. 15 cases were classified as plexogenic arteriopathy. The remaining cases displayed thromboembolic lesions. All cases showed a predominance of eccentric intimal lesions. In the plexogenic group, mixed concentric and eccentric intimal fibrosis coexisted. Therefore, in the present study, we propose an objective descriptive approach to the diagnosis of hypertensive pulmonary vascular disease, to correlate morphology and function.
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PMID:Specificity of pulmonary vascular lesions in primary pulmonary hypertension. A reappraisal. 367 95

Selected cases of severe primary pulmonary arterial hypertension and associated pulmonary vascular disease have been related to the oral ingestion of aminorex fumarate, an anorexigen obviously responsible for an epidemic of primary pulmonary hypertension in Western Europe between 1967 and 1970. This report describes a fifteen year follow-up of a female patient with aminorex fumarate related pulmonary hypertension and the uncommon finding of the formation of an excessive fusiform pulmonary trunk aneurysm in the late stage of the disease process. The progressive clinical course was followed by serial chest x-ray films and repeat right heart catheterization. The diagnosis of a main stem pulmonary artery aneurysm was noninvasively established by two-dimensional echocardiography and confirmed by contrast-enhanced computed tomography and radionuclide blood pool imaging. The patient is alive, thus no histologic correlate of this entity is available at present.
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PMID:Development of pulmonary aneurysm in primary pulmonary hypertension: a case report. 371 97

Four cases of pulmonary veno-occlusive disease are described. Two patients, who were brothers, had respiratory tract infections. The third patient had chronic active hepatitis and coeliac disease suggesting an abnormality of the immune system; the fourth patient had no obvious cause but presented initially with systemic hypertension. Three of the cases had been diagnosed initially as primary pulmonary hypertension either on open lung biopsy or clinically. In all cases the pulmonary arteries were abnormal with medial hypertrophy, intimal fibrosis and, in some cases, thrombosis in elastic pulmonary arteries. These findings suggest that pulmonary veno-occlusive disease is not confined to veins and should be considered as a widespread pulmonary vascular disease. The range of aetiological factors indicate that it should not be considered as a single disease entity.
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PMID:Pulmonary veno-occlusive disease. A report of four cases. 378 91

Pulmonary wedge angiograms have been shown to reflect the severity of pulmonary vascular disease in congenital heart disease. Thirteen pulmonary wedge angiograms with a balloon occlusion catheter were performed in 11 adult patients (five normals and six with primary pulmonary hypertension [PPH]) and their features related to the resting pulmonary artery pressure (PAP). Individual cine frames from each study were selected and digitized with a computer-assisted operator-interactive program. By fitting densitometric profiles from the vessel segments, serial arterial cross-sectional diameters were calculated from mathematically derived points. There was a strong correlation between arterial taper (T, change in vessel caliber per unit axial length) and a power function of mean PAP with T = 0.304 X PAP-0.59, R = .91, P less than .001. These results demonstrate a correlation between an angiographically derived morphologic characteristic of the pulmonary vasculature (taper) and a hemodynamic parameter (PAP) in PPH. This offers a method to follow the course of the disease and the effects of drug therapy by assessing anatomic changes in the vessels.
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PMID:Automated morphologic evaluation of pulmonary arteries in primary pulmonary hypertension. 380 57

Transplantation of the heart and both lungs is the only therapy that can be offered to certain patients with end-stage pulmonary vascular disease. Our experimental experience with the baboon is presented. Fourteen allotransplants were performed, 12 recipients (inadequately immunosuppressed with cyclosporin A and azathioprine) surviving between 4 and 29 days. In 11 cases death resulted from acute rejection which predominantly involved the lungs, the heart being spared in 10 cases; the remaining death was from bronchopneumonia. Two autotransplanted baboons survived until sacrificed at 6 months. Indications for the operation, selection of both the recipient and the donor, and recent results at other centres are briefly reviewed. It would seem that this operation is recommended in selected patients with idiopathic pulmonary hypertension or Eisenmenger's syndrome whose condition is deteriorating and in whom no other form of therapy is applicable.
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PMID:Transplantation of the heart and both lungs. Experimental and clinical experience and review of the literature. 388 28

The endothelium regulates the concentrations of several types of vasoactive substances that affect pulmonary vascular tone, and endothelia can oppose vasoconstriction in some circumstances by releasing vasodilators. To assess some of these endothelial functions in patients with pulmonary hypertension, we made measurements of selected vasoactive substances before and during attempts at pharmacologic vasodilatation. Studies were performed in 16 patients (1 1/2 to 23 years of age) with either idiopathic pulmonary hypertension (n = 11) or pulmonary hypertension as a consequence of unexpected early pulmonary vascular disease accompanying congenital heart defects (n = 5). In six of ten children, norepinephrine levels were elevated, and in two of the six, the concentrations of norepinephrine were greater in the aorta than in the pulmonary artery. In four out of 16 patients, thromboxane levels were increased, and in three of the four, the concentrations of thromboxane were greater in the aorta than in the pulmonary artery. These concentration gradients suggest pulmonary release of these vasoconstrictors. Identification of the contribution to pulmonary vasoconstriction made by changes in the endothelial metabolism of vasoactive substances may lead to a more fundamental understanding of the control of the pulmonary circulation, and hence lead to more specific modes of therapy for pulmonary hypertension.
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PMID:Endothelial function in clinical pulmonary hypertension. 389 37

We report our initial experience with three patients who received heart-lung transplants. The primary immunosuppressive agent used was cyclosporin A, although conventional drugs were also administered. In the first patient, a 45-year-old woman with primary pulmonary hypertension, acute rejection of the transplant was diagnosed 10 and 25 days after surgery but was treated successfully; this patient still had normal exercise tolerance 10 months late. The second patient, a 30-year-old man, underwent transplantation for Eisenmenger's syndrome due to atrial and ventricular septal defects. His graft was not rejected, and his condition was markedly improved eight months after surgery. The third patient, a 29-year-old woman with transposition of the great vessels and associated defects, died four days postoperatively of renal, hepatic, and pulmonary complications. We attribute our success to experience with heart-lung transplantation in primates, to the use of cyclosporin A, and to the anatomic and physiologic advantages of combined heart-lung replacement. We hope that such transplants may ultimately provide an improved outlook for selected terminally ill patients with pulmonary vascular disease and certain other intractable cardiopulmonary disorders.
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PMID:Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease. 679 24

An autopsy case of mixed connective tissue disease (MCTD) with pulmonary hypertension is presented. A 34-year-old woman suffering from arthralgia, Raynaud's phenomenon, and dyspnea of 6-years duration was diagnosed as having MCTD on the basis of a high titer (1:160,000) of serum antibody to the ribonuclease-sensitive component of extractable nuclear antigen. Examination of cardiac function revealed the complication of pulmonary hypertension. Autopsy revealed concentric intimal cellular proliferation of the small arteries and arterioles of both lungs. Typical plexiform lesions of these vessels were also observed. These findings coincide with those of plexogenic pulmonary angiopathy of primary pulmonary hypertension (PPH). This is the second autopsy case of MCTD with fatal pulmonary hypertension reported and our observations suggest that some cases with PPH who had immunological abnormalities but could not be classified as cases of classical collagen disease, may have been induced by MCTD.
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PMID:Mixed connective tissue disease with fatal pulmonary hypertension. 715 44

Visna-maedi virus induces in sheep an interstitial lung disease characterised by an accumulation of smooth muscle cells (SMC) or myomatosis. Infection by HIV-1 has been recently associated with disorders of the vessel-derived cells: primary pulmonary hypertension, coronary artery disease and smooth muscle tumors in humans. We hypothesized that, besides their regular targets (i.e. macrophages and lymphocytes), lentiviruses could infect smooth muscle cells. Smooth muscle cell cultures derived from ovine aorta were infected with visna-maedi virus strain K1514. The cultured cells were smooth muscle cells as demonstrated by their antigenic expression of alpha-actin and vimentin. The lentiviral infection of the smooth muscle cells was demonstrated by a typical cytopathic effect (syncytia), the expression of virus specific antigens, and the presence of genomic RNA detected by Northern blot analysis and RT PCR. The detection of a reverse transcriptase activity, the presence of viral RNA in supernatants of infected smooth muscle cells detected by RT PCR and their ability to infect ovine permissive fibroblasts demonstrated a productive infection. The ability of smooth muscle cells to be infected by lentiviruses may participate in the pathogenesis of the tissue damage associated with the lentiviruses such as myomatosis in sheep and vascular disease in humans.
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PMID:Ovine aortic smooth muscle cells allow the replication of visna-maedi virus in vitro. 754 8

Apparently primary pulmonary hypertension is rarely described in patients infected with the human immunodeficiency virus (HIV). We observed a case in a HIV seropositive patient with no known risk factor for pulmonary vascular disease. The characteristics of this new association are described.
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PMID:[Primary pulmonary hypertension in a HIV positive patient]. 783 52


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