Atypical pulmonary artery sling with diffuse-type pulmonary arteriovenous fistula.
(17/5022)
The case of a cyanotic infant with a rare combination of atypical pulmonary artery sling, imperforate anus, absence of the left kidney, interruption of the inferior vena cava, left side hemihypertrophy and diffuse-type pulmonary arteriovenous fistula is described. The clinical features were confusing, because of compounding abnormalities involving the respiratory tract and pulmonary circulation. The diagnostic approach to the etiology of cyanosis is discussed and the embryonic origin of pulmonary artery sling is reviewed. (+info)
The functional anatomy of the bronchial circulation of the domestic fowl.
(18/5022)
The bronchial circulation was studied in 25 adult domestic fowls. The right and left bronchial arteries originated caudal to the syrinx from a bronchoesophageal artery which is a branch of the right common carotid artery. Each bronchial artery ramified on the wall of the extrapulmonary part of the corresponding primary bronchus and finally anastomosed directly with a branch of the pulmonary artery at the hilus of the lung. Thr bronchial artery did not accompany the intrapulmonary part of the primary bronchus. The branches of each bronchial artery formed an anastomosing network on the wall of the extrapulmonary part of the primary bronchus. The calibre of the bronchial artery at its anastomosis with the branch of the pulmonary artery was greater than at its origin from the bronchoesophageal artery. Intravenous injections of Lycopodium spores indicated that the blood flows from the pulmonary artery into the bronchial artery. Small bronchial veins drained the extrapulmonary part of the primary bronchus into the pulmonary vein and the oesophageal veins. The intrapulmonary part of the primary bronchus was supplied by branches of the pulmonary artery and drained by tributaries of the pulmonary vein. The blood supply to the primary bronchus could constitute a shunt capable of passing blood from the pulmonary artery into the pulmonary vein without going through the exchange tissue. The parabronchial (atrial) muscles received a blood supply directly from the exchange tissue via septal venules which formed a network underneath the muscle bundles, without actually penetrating between the muscle cells. These venules drained into atrial veins which were tributaries of the pulmonary vein. The atrial muscles probably also received oxygen by direct diffusion from the parabronchial lumen. The pleura was supplied by the oesophageal branches of the bronchoesophageal artery, and by small twigs from the internal thoracic and intercostal arteries. (+info)
We used transgenic mice in which the promoter sequence for connexin 43 linked to a lacZ reporter was expressed in neural crest but not myocardial cells to document the pattern of cardiac neural crest cells in the caudal pharyngeal arches and cardiac outflow tract. Expression of lacZ was strikingly similar to that of cardiac neural crest cells in quail-chick chimeras. By using this transgenic mouse line to compare cardiac neural crest involvement in cardiac outflow septation and aortic arch artery development in mouse and chick, we were able to note differences and similarities in their cardiovascular development. Similar to neural crest cells in the chick, lacZ-positive cells formed a sheath around the persisting aortic arch arteries, comprised the aorticopulmonary septation complex, were located at the site of final fusion of the conal cushions, and populated the cardiac ganglia. In quail-chick chimeras generated for this study, neural crest cells entered the outflow tract by two pathways, submyocardially and subendocardially. In the mouse only the subendocardial population of lacZ-positive cells could be seen as the cells entered the outflow tract. In addition lacZ-positive cells completely surrounded the aortic sac prior to septation, while in the chick, neural crest cells were scattered around the aortic sac with the bulk of cells distributed in the bridging portion of the aorticopulmonary septation complex. In the chick, submyocardial populations of neural crest cells assembled on opposite sides of the aortic sac and entered the conotruncal ridges. Even though the aortic sac in the mouse was initially surrounded by lacZ-positive cells, the two outflow vessels that resulted from its septation showed differential lacZ expression. The ascending aorta was invested by lacZ-positive cells while the pulmonary trunk was devoid of lacZ staining. In the chick, both of these vessels were invested by neural crest cells, but the cells arrived secondarily by displacement from the aortic arch arteries during vessel elongation. This may indicate a difference in derivation of the pulmonary trunk in the mouse or a difference in distribution of cardiac neural crest cells. An independent mouse neural crest marker is needed to confirm whether the differences are indeed due to species differences in cardiovascular and/or neural crest development. Nevertheless, with the differences noted, we believe that this mouse model faithfully represents the location of cardiac neural crest cells. The similarities in location of lacZ-expressing cells in the mouse to that of cardiac neural crest cells in the chick suggest that this mouse is a good model for studying mammalian cardiac neural crest and that the mammalian cardiac neural crest performs functions similar to those shown for chick. (+info)
Spiral computed tomographic scanning and magnetic resonance angiography for the diagnosis of pulmonary embolism.
(20/5022)
PURPOSE: To compare prospectively the accuracy of spiral computed tomography (CT) with that of ventilation-perfusion scintigraphy for diagnosing pulmonary embolism. MATERIALS AND METHODS: Within 48 hours of presentation, 142 patients suspected of having pulmonary embolism underwent spiral CT, scintigraphy, and (when indicated) pulmonary angiography. Pulmonary angiography was attempted if interpretations of spiral CT scans and of scintigrams were discordant or indeterminate and intermediate-probability, respectively. RESULTS: In the 139 patients who completed the study, interpretations of spiral CT scans and of scintigrams were concordant in 103 patients (29 with embolism, 74 without). In 20 patients, intermediate-probability scintigrams were interpreted (six with embolism at angiography, 14 without); diagnosis with spiral CT was correct in 16. Interpretations of spiral CT scans and those of scintigrams were discordant in 12 cases; diagnosis with spiral CT was correct in 11 cases and that with scintigraphy was correct in one. Spiral CT and scintigraphic scans of four patients with embolism did not show embolism. Sensitivities, specificities, and kappa values with spiral CT and scintigraphy were 87%, 95%, and 0.85 and 65%, 94%, and 0.61, respectively. CONCLUSION: In cases of pulmonary embolism, sensitivity of spiral CT is greater than that of scintigraphy. Interobserver agreement is better with spiral CT. (+info)
Pulmonary artery pressure variation in patients with connective tissue disease: 24 hour ambulatory pulmonary artery pressure monitoring.
(21/5022)
BACKGROUND: The specific contribution of secondary pulmonary hypertension to the morbidity and mortality of patients with underlying lung disease can be difficult to assess from single measurements of pulmonary artery pressure. We have studied patients with secondary pulmonary hypertension using an ambulatory system for measuring continuous pulmonary artery pressure (PAP). We chose to study patients with connective tissue disease because they represent a group at high risk of pulmonary vascular disease, but with little disturbance of lung function. METHODS: Six patients (five with progressive systemic sclerosis and one with systemic lupus erythematosis) were studied. They underwent preliminary cardiopulmonary investigations followed by Doppler echocardiography, right heart catheterisation, and ambulatory pulmonary artery pressure monitoring to measure changes in pressure over a 24 hour period including during a formal exercise test. RESULTS: All patients had pulmonary hypertension as measured by Doppler echocardiography with estimated pulmonary artery systolic pressures of 40-100 mm Hg. Pulmonary function testing revealed virtually normal spirometric values (mean FEV1 86.9% predicted) but marked reduction in CO gas transfer factor (KCO 57.8% predicted). Exercise responses were impaired with mean VO2max 50.6% predicted. Ambulatory PAP monitoring indicated significant changes in pressures with variation in posture and activity throughout 24 hours. Resting PAP did not predict the change in PAP seen on exercise. CONCLUSION: Conventional methods of assessment of the pulmonary circulation based on single measurements in the supine position may underestimate the stresses faced by the right side of the circulation. This ambulatory system allows monitoring of pulmonary haemodynamics continuously over 24 hours during normal activities of daily living. These measurements may increase our understanding of the contribution made by secondary pulmonary hypertension to the morbidity and mortality of the underlying lung disease. (+info)
Flitting radiographic shadows: an unusual presentation of cancer in the lungs.
(22/5022)
Tumour involvement of pulmonary blood vessels occurs frequently in advanced lung cancer and occasionally may cause pulmonary infarction. A case is reported of diffuse obstruction of pulmonary arteries by cancer in which no primary tumour was found, and which presented as flitting radiographic opacities due to pulmonary infarction. (+info)
Prostanoid receptors involved in the relaxation of human pulmonary vessels.
(23/5022)
1. To characterize the prostanoid receptors on human pulmonary smooth muscle involved in vasodilatations, isolated arteries and veins were contracted with norepinephrine (10 microM) and vessels were subsequently challenged with different prostanoid-receptor agonists in the absence or presence of selective antagonists. 2. Prostaglandin D2 (PGD2) and the selective DP-receptor agonist, BW245C, induced relaxations in the contracted human pulmonary venous preparations. The pD2 values were: 6.88+/-0.11 (n=17) and 7.31+/-0.12 (n=5), respectively. The relaxant responses induced by PGD2 were reduced by the selective DP-receptor antagonist, BWA868C, and the estimated pA2 value was 7.84+/-0.16 (n=4). PGD2 and BW245C did not relax contracted human pulmonary arteries. 3. The selective IP-receptor agonists, iloprost and cicaprost, both induced relaxations in the contracted human vascular preparations. The pD2 values for iloprost were: 7.84+/-0.08 (n=6) and 8.25+/-0.06 (n=4) and for cicaprost: 8.06+/-0.12 (n=5) and 8.11+/-0.09 (n=5) in arteries and veins respectively. 4. Prostaglandin E2 (PGE2) and the EP2/EP3-receptor agonist, misoprostol, partially relaxed the contracted venous preparations and the pD2 values were: 8.10+/-0.15 (n=15) and 6.24+/-0.33 (n=3), respectively. These relaxations suggest the presence of an EP receptor in the human pulmonary veins. The contracted human pulmonary arteries did not relax when challenged with PGE2. 5. In human pulmonary venous preparations, the PGE2-induced relaxations were neither modified by treatment with TP/EP4-receptor antagonist, AH23848B (10 and 30 microM, n=6), nor by the DP/EP1/EP2-receptor antagonist, AH6809 (3 microM, n=6). 6. These data suggest that the relaxation induced by prostanoids involved DP-, IP-receptors and to a lesser extent an EP-receptor on human pulmonary venous smooth muscle. In contrast, only the IP-receptor is involved in the prostanoid induced relaxations on human pulmonary arterial smooth muscle. (+info)
Combined inhaled nitric oxide and inhaled prostacyclin during experimental chronic pulmonary hypertension.
(24/5022)
Inhaled nitric oxide (NO) and inhaled prostacyclin (PGI2) produce selective reductions in pulmonary vascular resistance (PVR) through differing mechanisms. NO decreases PVR via cGMP, and PGI2 produces pulmonary vasodilation via cAMP. As a general pharmacological principle, two drugs that produce similar effects via different mechanisms should have additive or synergistic effects when combined. We designed this study to investigate whether combined inhaled NO and PGI2 therapy results in additive effects during chronic pulmonary hypertension in the rat. Monocrotaline injected 4 wk before study produced pulmonary hypertension in all animals. Inhaled NO (20 parts/million) reversibly and selectively decreased pulmonary artery pressure (Ppa) with a mean reduction of 18%. Four concentrations of PGI2 were administered via inhalation (5, 10, 20, and 80 microg/ml), both alone and combined with inhaled NO. Inhaled PGI2 alone decreased Ppa in a dose-dependent manner with no change in mean systemic arterial pressure. Combined inhaled NO and PGI2 selectively and significantly decreased Ppa more did than either drug alone. The effects were additive at the lower concentrations of PGI2 (5, 10, and 20 microg/ml). The combination of inhaled NO and inhaled PGI2 may be useful in the management of pulmonary hypertension. (+info)