Pulmonary arteriovenous fistula

Stringer, C J; Stanley, Arthur L.; Bates, Richard C.; Summers, James

doi:10.1016/0002-9610(55)90580-8

Cited by 80 publications

(28 citation statements)

References 78 publications

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“…However, cerebral abscesses, cerebrovascular accidents, and transient ischaemic attacks due to paradoxical embolism through the PAVMs result in considerable morbidity and mortality. [3][4][5][6] Although individual PAVMs are amenable to surgical resection, in most individuals multiple lesions are likely with time and therefore the advent of the parenchymal-sparing embolisation techniques has transformed the outlook for patients. 7 To reduce the incidence of paradoxical embolism, embolisation of all feasible PAVMs is recommended with antibiotic prophylaxis for dental and surgical procedures in all patients with residual shunts.…”

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confidence: 99%

A hereditary haemorrhagic telangiectasia family with pulmonary involvement is unlinked to the known HHT genes, endoglin and ALK-1

Wallace

Shovlin²

2000

Thorax

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Methods-Family members were assessed clinically to assign HHT disease status and were screened for PAVMs. DNA was extracted from blood obtained from 20 individuals of known disease status. Short tandem repeat polymorphic markers spanning the intervals containing the endoglin and ALK-1 genes were amplified by the polymerase chain reaction using 33 P-labelled oligonucleotide primers, separated by denaturing polyacrylamide gel electrophoresis (PAGE), and the resultant autoradiographs were examined for allele sizes. Linkage analyses were performed using MLINK and GENE-HUNTER. Results-Twelve members spanning four generations were aVected with HHT. Two had proven PAVMs, one with a classical appearance, the other exhibiting microscopic PAVMs exacerbated by pregnancy. Two point lod and multipoint lod scores significantly excluded linkage to endoglin and ALK-1 in this pedigree. Conclusions-This study confirms the existence of a third HHT locus that accounts for disease in some HHT patients with pulmonary involvement. (Thorax 2000;55:685-690)

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confidence: 99%

A hereditary haemorrhagic telangiectasia family with pulmonary involvement is unlinked to the known HHT genes, endoglin and ALK-1

Wallace

Shovlin²

2000

Thorax

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“…A total of 208 cases of pulmonary arteriovenous fistula were reported from 1939 to 1989. [2][3][4][5][6][7][8] Of these, only 5 had multiple pulmonary arteriovenous fistulas bilaterally in the lungs. 2,3 The first 3 patients, who were infants, died of massive pulmonary hemorrhage.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4][5][6][7][8] Of these, only 5 had multiple pulmonary arteriovenous fistulas bilaterally in the lungs. 2,3 The first 3 patients, who were infants, died of massive pulmonary hemorrhage. The 2 adult patients had telangiectasia and severe central nervous system symptoms, including a 29-year-old man who died of hemothorax, and a 41-year-old man who died of coronary thrombosis.…”

Section: Discussionmentioning

confidence: 99%

“…The 2 adult patients had telangiectasia and severe central nervous system symptoms, including a 29-year-old man who died of hemothorax, and a 41-year-old man who died of coronary thrombosis. 2 In 1989, Knight et al reported a case of multiple pulmonary arteriovenous fistulas. 3 The patient was observed to have central cyanosis at the age of 5 months and diagnosed with multiple pulmonary arteriovenous fistulas.…”

Section: Discussionmentioning

confidence: 99%

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A Case of Diffuse Pulmonary Arteriovenous Fistula

et al. 1999

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ulmonary arteriovenous fistulas are abnormal connections between a pulmonary artery and vein. A close relationship between a pulmonary arteriovenous fistula and hereditary hemorrhagic telangiectasia has often been noted and the occurrence of pulmonary arteriovenous fistulas without a family history of telangiectasia is rare. The diffuse type of arteriovenous fistula is difficult to recognize, because it exhibits no abnormal findings on chest radiography or pulmonary arteriography. We report here an adult case of diffuse pulmonary arteriovenous fistulas. Case ReportA 30-year-old Japanese woman was admitted to hospital for dyspnea. She had suffered from cyanosis during infancy, and underwent corrective surgery for a large atrial septal defect (ASD) and partial anomalous pulmonary venous drainage when she was 3 years old. However, her cyanosis continued postoperatively.Her height was 156 cm, weight 47 kg, blood pressure 100/60 mmHg in both arms with a pulse of 80 /min and regular respiratory rate of 25 /min. She exhibited cyanosis and digital clubbing, but no telangiectasia. The lungs were clear to auscultation. The second heart sound was split widely and fixed, but no cardiac murmurs was audible. The electrocardiogram and chest radiograph were normal. Thoracic computed tomography showed dilated azygos and hemiazygos veins. Neurological examination was normal. Arterial blood gas analysis revealed pH 7.454, PO2 47.0, PCO2 33.1 mmHg and SaO2 84%. Transthoracic echocardiography did not reveal atrial septal defect or abnormal Japanese Circulation Journal Vol.63, June 1999 shunt flow. Contrast echocardiograms following the injection of saline agitated with small amount of air revealed a cloud of tiny bubbles appearing first in the right ventricle. After 3 s, these bubbles appeared in the left ventricle, entering through the mitral valve funnel. Angiography revealed doubled inferior vena cavas (IVC); the left IVC and left external iliac vein were connected with the hemiazygos vein, and the right IVC with the left atrium through a small orifice (Fig 1). Most of the blood from the IVCs flowed into the superior vena cava (SVC) via the distended azygos and hemiazygos veins (Fig 2). Pulmonary arteriography revealed no abnormal structures. The mean pulmonary arterial pressure was 10 mmHg. Oxygen saturations in the left upper, right upper and lower pulmonary veins were 85.6, 78.8 and 83.0%, respectively. Perfusion lung scintigraphy, using 99m Tc-macroagglutinated albumin (MAA), showed multiple segmental perfusion defects in the lung fields and accumulation in the kidneys, although ventilation lung scans revealed no defects (Fig 3). Microscopic pulmonary arteriovenous fistulas appearedJpn Circ J 1999; 63: 499 -501 (Received November 11, 1998; revised manuscript received March 2, 1999; accepted March 18, 1999) A 30-year-old Japanese woman was admitted to hospital for dyspnea. She had a history of corrective surgery for a large atrial septal defect and partial anomalous pulmonary venous drainage, which had produced cyanosis in...

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“…Pulmonary vascular lesions are known under a variety of names including pulmonary arteriovenous malformation (PAVM), pulmonary arteriovenous aneurysm (PAVA), pulmonary angioma, arteriovenous angiomatosis, cavernous or venous haemangioma, pulmonary arteriovenous fistula and pulmonary hamartoma [1][2][3][4]. The majority of authors believe that the so-called venous haemangioma, especially congenital and occurring in children, should be classified as a vascular malformation since it does not fulfil all the criteria of a true neoplasm.…”

Section: Introductionmentioning

confidence: 99%