1/10. A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease.INTRODUCTION: Pulmonary arteriovenous malformations are a common cause of progressive cyanosis in children after cavopulmonary anastomoses. We analyzed the pulmonary histologic characteristics from children in whom pulmonary arteriovenous malformations developed after procedures that resulted in pulmonary arterial blood flow devoid of hepatic venous effluent. methods: We performed routine histologic studies, immunohistochemical staining, and electron microscopic analysis of peripheral lung biopsy specimens from 2 children with angiographically proven pulmonary arteriovenous malformations. Microvessel density was determined with a computer-assisted, morphometric analysis system. RESULTS: Histologic examination demonstrated large, dilated blood vessels ("lakes") and clustered, smaller vessels ("chains") in the pulmonary parenchyma. Microvessel density was significantly greater in these patients than in age-matched controls (P =.01). immunohistochemistry demonstrated uniform staining for type IV collagen and alpha-smooth muscle actin, weak staining for the endothelial marker CD31 (cluster of differentiation, PECAM-1), and negative staining for proliferating cell nuclear antigen. Electron microscopy revealed endothelial irregularity, a disorganized basement membrane, and increased numbers of collagen and actin filaments beneath the endothelium. CONCLUSIONS: This study represents an attempt to characterize the histologic features of pulmonary arteriovenous malformations in children with congenital heart disease who have pulmonary arterial blood flow devoid of hepatic venous effluent. The histologic correlate of this condition appears to be greatly increased numbers of thin-walled vessels. immunohistochemistry suggests that the rate of cellular proliferation is not increased in these lesions. The development of these techniques may provide a standardized histologic approach for this condition and aid in understanding its etiology.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/10. Abernethy malformation: one of the etiologies of hepatopulmonary syndrome.hepatopulmonary syndrome (HPS) is the clinical relationship between hepatic disease and the existence of pulmonary vascular dilatations, which can result in a range of arterial oxygenation abnormalities. It is probably caused by an alteration in the synthesis or metabolism of vasoactive pulmonary substances at a hepatic level, leading to vasodilatation of pulmonary vessels and diffusion perfusion defects. The Abernethy malformation is characterized by the congenital diversion of portal blood away from the liver, by either end-to-side or side-to-side shunt. Here, we report on a 5-year-and-11-month-old-boy who had started cyanosis at age 4 years and 11 months, and did not have any other pulmonary or cardiac signs or symptoms. In the investigation, arterial blood gases revealed a PaO(2) of 41.4 mm Hg. The chest x-ray film and echo Doppler cardiography were normal. Nuclear scanning with technetium 99m-labeled macroaggregated albumin showed the presence of arteriovenous shunt, at 47%. Abdominal echography revealed Abernethy malformation with an absence of portal vein. We concluded that the patient had HPS caused by Abernethy malformation. The possible mechanism is that in this malformation, there is a deviation in the blood that comes from the spleen to the vena cava without passing through the liver, so there is no metabolism of some substances which can be responsible for the imbalance between the vasodilatation and the vasoconstriction of the pulmonary circulation. Abernethy malformation must be included as one of the etiologies of hepatopulmonary syndrome. This is the first case described in the literature with this form of presentation.- - - - - - - - - - ranking = 0.2keywords = vessel (Clic here for more details about this article) |
3/10. A high-titer, high-thermal-amplitude autoanti-B associated with acrocyanosis but no obvious hemolytic anemia.BACKGROUND: ABO autoantibodies are rare. Most reported examples have been antibodies with 4 degrees C titers not greater than 256 in patients without apparent hemolytic anemia. Most high-titer, high-thermal-amplitude, complement-activating cold agglutinins are associated with hemolytic anemia. STUDY DESIGN AND methods: A 52-year-old man presented with acrocyanosis and mild small-vessel brain disease, but no evidence of obvious hemolytic anemia. Regular plasmapheresis treatment was helpful in relieving the clinical symptoms associated with acrocyanosis. Serologic methods were used to study the patient's RBCs and sera. RESULTS: The patient's RBCs were strongly reactive with anti-C3 and anti-IgM and weakly reactive with anti-IgA. The patient's serum contained a high-titer, high-thermal-amplitude, IgMkappa autoanti-B, capable of activating complement in vitro. CONCLUSION: A patient with a powerful ABO autoantibody is described. This patient had acrocyanosis but did not appear to have an obvious hemolytic anemia. This case is a good example of the lack of correlation between in vitro serologic tests and in vivo reactions in individual patients.- - - - - - - - - - ranking = 0.2keywords = vessel (Clic here for more details about this article) |
4/10. Respiratory symptoms secondary to aortopulmonary collateral vessels in tetralogy of Fallot absent pulmonary valve syndrome.Hemodynamically significant systemic-to-pulmonary artery collaterals may represent an underappreciated cause of cardiorespiratory compromise in tetralogy of Fallot with absent pulmonary valve (TOF/APV). We retrospectively reviewed the angiographic, magnetic resonance imaging, operative, and autopsy reports of the 50 patients with TOF/APV managed at our institution in the past 10 years and demonstrated that at least 7 of 50 patients (14%) had significant aortopulmonary collaterals.- - - - - - - - - - ranking = 0.8keywords = vessel (Clic here for more details about this article) |
5/10. Hypoxia and cyanosis in alpha-1-antitrypsin deficiency. Cirrhosis as an unusual etiologic factor.A patient with type PiZZ alpha-1-antitrypsin deficiency was found to have severe hypoxia despite normal pulmonary function testing and a normal chest radiograph. A nuclear medicine ventilation-perfusion study revealed a right-to-left shunt. Computed tomography showed minimal bleb formation, no diffuse changes, and hepatic changes of cirrhosis with portal hypertension. No nodular pulmonary masses or enlarged peripheral pulmonary vessels were found. The diagnosis of diffuse intrapulmonic arteriovenous shunts ("pulmonary spiders of cirrhosis") was suggested and then confirmed with a dynamic radionuclide flow study.- - - - - - - - - - ranking = 0.2keywords = vessel (Clic here for more details about this article) |
6/10. The Rombo syndrome: a familial disorder with vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis.This hitherto unknown and dominantly inherited disorder is characterized by vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis. It has been present in the family reported for at least four generations. The lesions become visible in late childhood and are most pronounced on the face. Basal cell carcinomas may develop around the age of 35. Histological observations during the early stage include irregularly distributed and atrophic hair follicles, milia, dilated dermal vessels, lack of elastin or elastin in clumps. After light irradiation a tendency to increased repair activity was observed both in epidermis and in the dermal fibroblasts. On exposure to cold the change in the skin temperature of the hands compared with that in the controls was insignificant. The response to adrenalin iontophoresis was weak.- - - - - - - - - - ranking = 0.2keywords = vessel (Clic here for more details about this article) |
7/10. Direct communication of the right pulmonary artery with the left atrium in an infant.An 8-month-old Japanese girl with cyanosis and evidence of a round shadow connected with the right cardiac border on the chest x-ray film was diagnosed as a case of direct communication of the right pulmonary artery with the left atrium (RPA-LA communication) following cardiac catheterization and angiocardiography. She had a history of transient congestive heart failure in the early neonatal period. A secundum atrial septal defect and a two-lobed right lung were also present. The patient became asymptomatic after a successful ligation of the anomalous connecting vessel.- - - - - - - - - - ranking = 0.2keywords = vessel (Clic here for more details about this article) |
8/10. Increasing cyanosis early after cavopulmonary connection caused by abnormal systemic venous channels.OBJECTIVE--To show that abnormal systemic venous channels in patients who undergo cavopulmonary anastomoses can become manifest and haemodynamically important only after surgery despite detailed preoperative investigation. DESIGN--Descriptive study of patients fulfilling the above criteria selected from hospital records over the past three years. SETTING--A tertiary referral centre. patients--Of the three cases identified, two were isomeric, one with left atrial isomerism and hemiazygos continuation of the inferior vena cava who underwent bilateral bidirectional Glenn anastomoses and one with right isomerism who underwent total cavopulmonary anastomosis. Case 3 had absent left atrioventricular connection with a hypoplastic left lung and underwent a classic right Glenn procedure. All three cases presented with progressive cyanosis in the early postoperative period. INTERVENTIONS AND RESULTS--Postoperative angiography in case 1 showed a remnant of a left inferior vena cava draining to the atrium to have become grossly dilated causing cyanosis, which resolved after redirection of this vessel and of the hepatic veins into the right pulmonary artery with an intra-atrial baffle. cyanosis in case 2 was caused by intra-hepatic shunting to a hepatic vein draining to the left of the intra-atrial baffle. The diagnosis was made at necropsy, being overlooked on postoperative angiography. Repeat angiography in case 3 showed progressive dilatation of a small left superior vena cava to coronary sinus. Test occlusion with a view to embolisation revealed hitherto an undemonstrated hemiazygos continuation of inferior caval to brachiocephalic vein. The patient underwent surgical ligation of these two venous channels. CONCLUSIONS--Despite appropriate investigation some "abnormal" venous pathways manifest themselves, dilate, and become haemodynamically important only after surgical cavopulmonary anastomoses. In the presence of early postoperative cyanosis "new" systemic venous collateral channels should be considered as a possible cause, which may require reintervention.- - - - - - - - - - ranking = 0.2keywords = vessel (Clic here for more details about this article) |
9/10. A patch technique for reconstruction of stenotic pulmonary bifurcation in a Rastelli-type operation after palliations for cyanotic congenital heart disease. A report of two cases.A Rastelli-type operation was successfully performed on two patients with cyanotic congenital heart disease with pulmonary artesia (PA) and severe stenosis or obstruction of the pulmonary bifurcation, following the improvement of a pulmonary artery index (PA index) after palliative systemic pulmonary shunts. It is a point that stenotic or obstructive pulmonary bifurcation was reconstructed by a patch technique. An extracardiac conduit was used a combination of a bicuspid valved pericardial roll and a prosthetic vessel graft. In a 5-year-old male with an extreme type of tetralogy of fallot associated with PA and patent ductus arteriosus (PDA), the enlargement of stenotic pulmonary bifurcation was attempted using a brimshaped equine pericardial patch in order to get sufficient suture margin and be safely anastomosed with a conduit. In a 6-year-old female with complete transposition of the great arteries, PA, PDA and right aortic arch (RAA), an obstructive pulmonary bifurcation behind the RAA was enlarged using a brimshaped equine pericardial patch. The attachment of the conduit was shifted to the left pulmonary artery to avoid compression of an extracardiac conduit and pulmonary artery behind RAA. Reconstruction of the pulmonary bifurcation using an equine pericardial patch is useful to anastomose an extracardiac conduit and the stenotic or obstructive pulmonary bifurcation.- - - - - - - - - - ranking = 0.2keywords = vessel (Clic here for more details about this article) |
10/10. Cyanotic Raynaud's phenomenon with conventional but not with liposomal amphotericin b: three case reports.Despite its common adverse effects intravenous (i.v.) amphotericin b is an indispensable antifungal drug in childhood oncology. We report here on three cases of painful cyanotic Raynaud's phenomenon after i.v. administration or inhalation of amphotericin b. A liposomal i.v. preparation of amphotericin b was well tolerated by the infants. Spasms of peripheral vessels mediated by thromboxane a2 could be responsible for the Raynaud's phenomenon. Hence, inhibitors of prostaglandin synthesis are suggested for therapy.- - - - - - - - - - ranking = 0.2keywords = vessel (Clic here for more details about this article) |
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