Case Of Pulmonary Arteriovenous Fistula Research Articles

Cerebral symptoms in pulmonary arteriovenous fistula. A result of paradoxical emboli (?).

Two cases of pulmonary arteriovenous fistulas with hemiparesis and other neurologic findings, possibly from paradoxical embolization, are described. Many theories have been put forward to explain the pathophysiology of the frequently present neurologic manifestations. We regard paradoxical embolization through the fistula as the most important factor in the pathogenesis of the neurologic deficit. Due to the potential hazard of paradoxical cerebral embolization and possible subsequent brain abscess formation, surgical resection of all pulmonary arteriovenous fistulas wherever feasible is recommended.

Hereditary Telangiectasia and Pulmonary Fistula

Hereditary LEREDITARY hemorrhagic telangiectasia manifests itself by the formation of localized arteriovenous capillary connections that are tiny arteriovenous fistulas. The first comprehensive description was given by Rendu¹in 1896. Osler²in 1901 and Weber³in 1907 added to knowledge of the entity, which brought about the designation "Rendu-Osler-Weber syndrome." In 1909, Hanes⁴used the term "hereditary hemorrhagic telangiectasia," which is the preferred designation. Hodgson and colleagues⁵studied 231 members of a large family of which 91 descendants had the disease entity. Of the descendants studied, 40% had telangiectasia and 6% had pulmonary arteriovenous fistulas. In 50% of the cases of pulmonary arteriovenous fistula, telangiectasia elsewhere in the body is encountered.⁶Approximately 5% to 6% of patients with Rendu-Osler-Weber syndrome have pulmonary arteriovenous fistula.⁷ Pulmonary arteriovenous fistulas may occur in persons with or without hereditary hemorrhagic telangiectasia, but the close relationship makes it imperative that patients with this disorder be investigated

Retinal arteriovenous aneurysm in hereditary hemorrhagic telangiectasia.

The usual pattern of cutaneous, mucosal, and visceral lesions in Osler's disease, hereditary hemorrhagic telangiectasia, is known to all experienced physicians. Variations upon this common theme, rare manifestations of a rare disease, need to be recorded to make the composite picture more detailed if not complete. In addition to the widespread cutaneous and internal telangiectatic lesions in Osler's disease which are notorious as the cause of bleeding in this disorder, various other vascular anomalies may occur in larger vessels. The best known is the pulmonary arteriovenous fistula which may result in polycythemia, cyanosis, and the rare complication of brain abscess. Aneurysms of various arteries, including the hepatic artery and splenic artery, have been reported and reviewed. 1 One of us (WBB) has observed more than 40 kindreds and more than 250 patients with the disease. Only three cases of pulmonary arteriovenous fistula have been observed, a considerably lower frequency than

A case of pulmonary arterio-venous fistulae

A case of pulmonary arterio-venous fistulae is reported and the treatment described. The nomenclature of abnormalities of the vascular pattern of the lung is discussed.

Pulmonary arteriovenous fistula located by indicator dilution studies

Two cases of pulmonary arteriovenous fistula are described in which thoracotomy was performed and the lesion resected. In both patients the site of the venoarterial shunt and the absence of significant lesions in the opposite lung were demonstrated by indicator-dilution curves. During cardiac catheterization, peripheral arterial unsaturation was demonstrated and venoarterial shunts of 32 per cent of systemic flow and 53 per cent of systemic flow, respectively, were calculated.

Pulmonary arteriovenous fistula.

A proven case of Pulmonary Arteriovenous Fistula in a 54-year-old male is described. Interest lies in the relatively few recorded cases, and in the fact that a cor pulmonale was an associated finding. The diagnosis was established clinically and radiologically, and was confirmed at autopsy.

Arteriovenous Fistulas of the Lungs

Two cases of pulmonary arteriovenous fistula (or aneurysm) are presented, one of which is reported in detail. Twenty-three other cases of pulmonary arteriovenous aneurysm or fistula previously reported in the American literature are reviewed, and the clinical, laboratory, radiologic, physiologic, pathologic and hereditary features of this condition discussed. Clubbing of the fingers and toes, polycythemia and cyanosis are almost constant findings. The imnportance of differentiating this condition from polycythemia vera and congenital heart disease is emphasized.

The Angiographic Demonstration of Pulmonary Arteriovenous Fistula

Contrast angiography in the diagnosis of pulmonary arteriovenous fistula was first recorded by Smith and Horton (24) in 1939. Their patient, a forty-seven-year-old male in whom cyanosis and clubbing of the fingers had been noted since the age of twenty-four, was examined first in 1932. At that time he was thought to have polycythemia vera. Five years later a bruit was heard at the base of the right lung and a density in the right lower lobe was demonstrable roentgenographically. Following the injection of a radiopaque medium into the basilic vein to visualize the pulmonary blood vessels, roentgenograms showed the density to be a vascular tumor and the diagnosis of hemangioma of the lung acting as an arteriovenous fistula was made. The patient was not treated. The first successful surgical cure of a pulmonary arteriovenous fistula was reported by Hepburn and Dauphinee (12) in 1942. A twenty-three-year-old female complained of dizziness, faintness, and dyspnea. She was cyanotic, and was known to have had clubbing of the fingers since she was fifteen. Roentgenograms revealed a shadow in the right middle and lower lobes, which was diagnosed as a pulmonary arteriovenous fistula. A right pneumonectomy was done by Shenstone (21), and the patient made a rapid recovery, with prompt disappearance of the cyanosis and polycythemia and gradual improvement in the clubbing of the fingers. In the accompanying table are listed data on 22 cases of pulmonary arteriovenous fistula recorded in the literature accessible to us. It will be noticed that the first accurately described case was recorded by Wilkens (28) in 1918. At the postmortem examination of a twenty-three-year-old girl it was noted that two vessels which emerged from a dilated branch of the artery to the left lower lobe joined to enter the pulmonary vein just before its entrance into the left auricle. Similar changes were found in the parenchyma of the right lung. The ages in the reported cases, as shown in the table, varied from two days to fortyfive years. There were 13 males and 9 females. Some degree of cyanosis was noted in 17 cases, and clubbing of the fingers and toes was found in 15. Nine patients had small hemangiomata of the skin or petechiae. In more than half of the cases some type of murmur was heard over the affected area. Red cell counts varied from normal to 11.4 million. Hemorrhage or epistaxis was reported in 9 cases. Other symptoms were dyspnea, cough, persistent headache, and a “lightheaded feeling.” The familial character of the lesion was stressed by Goldman (10), who observed its occurrence in two brothers. Whitaker reported (27) a family history of telangiectasis accompanied in one member by a proved pulmonary arteriovenous fistula. Maier, Himmelstein, Riley, and Bunin (18) described a bacterial endarteritis in association with their case of pulmonary arteriovenous fistula.

The clinical syndrome associated with pulmonary arteriovenous fistulas, including a case report of a surgical cure

The clinical syndrome, consisting of cyanosis, clubbing of fingers, and polycythemia related to pulmonary arteriovenous fistula, is discussed and the importance of the diagnosis in view of the availability of surgical cure is emphasized. The first case with the unusual complication of collateral circulation to the thoracic wall and with surgical cure by lobectomy is presented. It is believed that this is the ninth case of pulmonary arteriovenous fistula to be recognized clinically and reported and the fifth patient to be cured by surgical treatment.