Pulmonary arteriovenous fistulas. Case presentations and clinical recognition

This report describes characteristic features and clinical presentation of six young patients (ranging in age from five to 25 years) with various congenital forms of pulmonary arteriovenous fistulas as well as one 21-year old patient with traumatic intrapulmonary arteriovenous fistula. The former six patients were cyanotic, had clubbing of the digits and exertional dyspnea; two, additionally, had telangiectasia. All patients underwent cardiac catheterization and pulmonary angiography. None had pulmonary hypertension. Angiographically, four had diffuse arteriovenous fistulas in both lungs (Figures 1 and 3 to 5) and two had arteriovenous fistulas localized in one pulmonary segment. Four patients were additionally studied with radionuclide angiography and pulmonary perfusion scintigraphy. In three of these, substantial right-to-left shunt in the region of the lungs was detected; the diagnosis could not be established in only one of the latter with a small traumatic intrapulmonary arteriovenous shunt of less than 10%. While noninvasive methods provide adjunctive diagnostic support, pulmonary angiography is still required to assess, with certainty, the nature and localization of the lesion. In cyanotic patients with no evidence of cardiac disease, differential diagnostic consideration should be given to pulmonary arteriovenous fistulas.     

Cirrhosis of the liver simulating congenital cyanotic heart disease

During the last 25 years, 20 patients with cirrhosis of liver with severe cyanosis and gross clubbing simulating congenital cyanotic heart disease were subjected to cardiac catheterization and angiography, splenography, liver function tests, and liver biopsy. No portopulmonary fistulas could be demonstrated. The cyanosis and clubbing were secondary to right to left intrapulmonary shunting across multiple tiny pulmonary arteriovenous fistulas. In 15 cases, selective pulmonary angiography revealed discrete arteriovenous fistulas. In five cases, the angiogram did not reveal any convincing evidence of pulmonary arteriovenous fistulas. In two of these five cases, peripheral vein contrast echocardiography demonstrated right to left intrapulmonary shunting and seems a sensitive investigation. Open lung biopsy in one case showed evidence of pulmonary arteriovenous fistulas.     

Effects of postural change on oxygen saturation and respiration in patients after the Fontan operation: platypnea and orthodeoxia

The aim of this study was to assess whether platypnea and orthodeoxia occur in Fontan patients. We divided 14 Fontan patients into 2 groups: 8 patients who had pulmonary arteriovenous fistulas and/or intra-atrial shunts (group A) and 6 patients who had neither pulmonary arteriovenous fistulas nor intra-atrial shunts (group B). They were compared with 9 controls (group C). Arterial oxygen saturation, minute ventilation per body weight and ventilatory equivalent for carbon dioxide were measured in the supine and then sitting positions. In group A, 1 patient had platypnea and 3 patients had orthodeoxia (changes in the saturation from the supine position to the sitting position were -4% to -7%) accompanied with slight hyperpnea, and all 4 patients had both pulmonary arteriovenous fistulas and intra-atrial shunts. Contrary, patients in group B had neither platypnea nor orthodeoxia. The saturation was significantly lower and the minute ventilation was significantly higher in the sitting position than in the supine position in group A (p < 0.05). The other groups showed no significant difference in the saturation or the minute ventilation between the 2 positions. All groups showed the ventilatory equivalent was significantly higher in the sitting position than in the supine position (p < 0.05 to 0.01). We demonstrated platypnea and orthodeoxia in Fontan patients with pulmonary arteriovenous fistulas and intra-atrial shunts. We believe platypnea and orthodeoxia should be regard as a complication in Fontan patients with pulmonary arteriovenous fistulas and/or intra-atrial shunts.     

Osler-Weber-Rendu disease and pulmonary arteriovenous fistulas. Deterioration and embolotherapy during pregnancy

Several reports have implicated pregnancy as a cause of deterioration in patients with pulmonary arteriovenous fistulas. We report a 27-year-old woman with multiple pulmonary arteriovenous fistulas who required coil spring embolotherapy in her 24th week of pregnancy due to a spontaneous hemothorax and hypoxemia.     

Diagnosis and treatment of pulmonary arteriovenous fistulas

Pulmonary arteriovenous fistulas are rare malformations often associated with Rendu-Osler-Weber (ROW) disease. Morbidity and mortality are significant and arise from hemorrhagic and neurological complications.We report the cases of two patients, mother and son, with earlier diagnoses of ROW disease, who suffered, respectively, a stroke and a brain abscess with massive pulmonary thromboembolism. Helicoid computed axial tomographic scans demonstrated pulmonary arteriovenous fistulas in both. Given these findings, we performed embolotherapy.     

Pulmonary arteriovenous malformations: lung transplantation as a therapeutic option.

Multiple pulmonary arteriovenous malformations (PAVM) constitute an uncommon cause of respiratory disability. They may lead to severe hypoxaemia via right-to-left shunts and are sources of substantial mortality and morbidity. Conservative surgical resection has been proposed as the treatment of choice. More recently, percutaneous balloon or coil embolization of the feeding vessels offered an efficacious and safe alternative therapy for patients whose fistulas are too numerous to excise. This study reports an unusual case of respiratory disability in a patient with multiple and microscopic pulmonary arteriovenous malformations who failed to respond to embolotherapy and who received a double lung transplantation with good initial outcome.     

A senile patient with Rendu-Osler-Weber disease who underwent resection of 4 multiple bilateral pulmonary arteriovenous fistulas]

A 70-year-old female patient with multiple bilateral pulmonary arteriovenous fistulas was referred to our hospital for its treatment. She had experienced frequent epistaxis and had nasopharyngeal telangiectasia. Her mother, sister and all four children also suffered from epistaxis. We diagnosed this patient as Rendu-Osler-Weber disease combined with multiple bilateral pulmonary arteriovenous fistulas. Her chest roentgenogram revealed 4 arteriovenous fistulas in the left lung and 2 arteriovenous fistulas in the right. On admission arterial blood gas analysis while breathing room air showed a PaO2 of 39.8 torr and an SaO2 of 75.4%. The percent of R-L shunt was calculated to be 56.5%. Because of severe hypoxemia, she underwent resection of four large fistulas in segments 3, 4, 5, and 8 of the left lung. Twenty-eight days after the operation, the PaO2 was 76.4 torr and SaO2 was 95.7%. There was no sign of enlargement in size of the remaining arteriovenous fistulas.     

Longitudinal evaluation of a patient with arteriovenous fistulas. Use of FRC-TLC shunt technique.

A patient with a brain abscess was found to have multiple pulmonary arteriovenous fistulas. Differences in arterial blood gas levels at low and high lung volumes were used to quantify shunting. Using this technique one can objectively follow progression, remission, or stabilzation of pulmonary arteriovenous fistulas.     

Successful occlusion of multiple pulmonary arteriovenous fistulas using Amplatzer vascular plugs

Pulmonary arteriovenous fistulas, or malformations, are abnormal vascular connections between a pulmonary artery and a pulmonary vein. Clinical presentation may vary from asymptomatic patients to others showing a variety of symptoms such as cyanosis, dyspnea, high output heart failure, hemoptysis and paradoxical embolization. Initially, surgical treatment was the single method of therapy until several percutaneous embolization techniques using coils, coil bags, detachable balloons and devices were established. In this report, we describe a child in whom the new Amplatzer Vascular Plug device was successfully used to close multiple, large pulmonary arteriovenous fistulas.     

Clinical spectrum of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) is an autosomal dominant, systemic fibrovascular dysplasia in which telangiectases, arteriovenous malformations, and aneurysms may be widely distributed throughout the body vasculature. Major clinical manifestations include: recurrent bleeding from mucosal telangiectases and arteriovenous malformations; hypoxemia, cerebral embolism, and brain abscess due to pulmonary arteriovenous fistulas; high-output congestive heart failure and portosystemic encephalopathy from hepatic arteriovenous malformations; and a variety of neurologic symptoms due to central nervous system angiodysplasia. Therapy is primarily supportive, consisting of iron supplementation and blood transfusion. Septal dermoplasty and oral estrogens may allow prolonged remission of epistaxis, but permanent surgical cure of gastrointestinal bleeding is rarely feasible because of diffuse angiodysplasia of the alimentary tract. Ligation, resection, or embolization may be indicated for pulmonary arteriovenous fistulas. The prognosis and survival of patients with hereditary hemorrhagic telangiectasia are favorable, providing treatable complications are accurately diagnosed.     

Circulatory studies on a case of arteriovenous aneurysm

The deleterious influence of arteriovenous fistulas upon the heart was first demonstrated by Reid¹ in a series of interesting survival experiments on animals. Following the production of large arteriovenous communications in dogs, cardiac hypertrophy and dilatation and the evidence of myocardial insufficiency developed. This investigation was prompted by Reid's clinical experience with patients with arteriovenous aneurysms. In two such cases cardiac hypertrophy and dilatation had been noted by Osler and Halsted, but no causal relationship was considered.The present knowledge of the pathological physiology associated with such fistulas is in large measure due to Reid², Holman³, Lewis and Drury,⁴ but certain of the phenomena associated with this interesting condition are still incompletely understood.The results of the study of this case added a new observation to the published data regarding arteriovenous aneurysms. In general, our findings are in accord with the excellent clinical and experimental work of Holman, but differ in several respects from the investigations of Lewis and Drury.     

The biological "scrabble" of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery

Pulmonary arteriovenous fistulas are vascular malformations, which, by virtue of producing abnormal vascular connections proximal to the units of gas exchange, result in intrapulmonary right-to-left shunting. These malformations or fistulas reflect at least in part disordered angiogenesis, and less commonly recruitment and dilation of pre-existing vascular channels. Pulmonary arteriovenous fistulas occur in a number of diverse clinical settings. Such fistulas are a well-established feature of the Weber-Osler-Rendu complex, or hereditary haemorrhagic telangiectasia, an autosomal dominant vascular dysplasia characterized by mucocutaneous telangiectasis, epistaxis, gastrointestinal haemorrhage, and arteriovenous malformations in the lung, brain, liver and elsewhere. They are also seen in the patient with acute or chronic liver disease, disease that is usually but not invariably severe, or those with non-cirrhotic portal hypertension. They may occur as congenital malformations, single or diffuse, large or small in isolation, and when large or extensive enough may result in hypoxaemia, clinical cyanosis, and heart failure. Cerebral vascular accidents are also a well-known complication of this disorder. An extensive literature has accumulated with regard to the pulmonary arteriovenous fistulas seen in the setting of the Weber-Osler-Rendu complex, and there is considerable information on the genetics, basic biology, clinical findings, complications and therapeutic interventions of these malformations in the setting of this syndrome. These issues, however, are not the primary considerations of this review, although some aspects of this fascinating disorder will be discussed later. Rather the focus will be on pulmonary arteriovenous malformations that develop in the setting of cavopulmonary surgery, and their relationship to the pulmonary arteriovenous fistulas occurring in the hepatopulmonary syndrome. The complex tapestry of these overlapping and intersecting clinical observations will be unfolded in the light of their chronology.     

Multiple pulmonary arteriovenous fistulas in childhood

Three cases of multiple pulmonary arteriovenous fistulas are described in children who presented at five months, two and nine years of age. Mass spectrometry was used to measure pulmonary blood flow and, in two cases, the intrapulmonary right-to-left shunt. The shunt fractions were 51% and 35%, with no significant change on breathing 100% oxygen. In one case, effective pulmonary blood flow was measured during cardiac catheterisation by the argon-freon rebreathing method and agreed closely with that found from the Fick, principle with measured oxygen consumption. Treatment consisted of surgical ligation of a lower lobe pulmonary artery in the youngest child, balloon embolisation in the second, and initial surgical oversewing of a single large fistula followed twenty months later by steel coil embolisation in the third. The last and oldest child is well and no longer cyanosed. The first two children died seven months after treatment with evidence of progression of their pulmonary arteriovenous fistulas. The first of these, who also had an atrial septal defect and discordant thoraco-abdominal arrangement, died of heart failure. Autopsies on both children confirmed extensive involvement of both lungs by arteriovenous fistulas. In one case who had a diffuse, telangiectatic form of pulmonary arteriovenous fistulas, microscopic serial reconstructions of lung tissue revealed that anastomoses occurred between arteries accompanying terminal bronchioles and intra-acinar arteries and adjacent veins. Occlusion of the pulmonary arteries supplying the fistulas led to extensive fibrosis within them, and was associated with enlargement of the corresponding bronchial arterial circulation.     

Bubble contrast echocardiography in detecting pulmonary arteriovenous malformations after modified Fontan operations.

The development of pulmonary arteriovenous malformations is a well-known complication after Fontan operations, and may result in significant morbidity due to increasing arterial desaturation. We compared the use of bubble contrast echocardiography and pulmonary angiography in detecting such malformations. We also examined which anatomical and haemodynamic variables were associated with their development. Our study includes 20 patients who had undergone modified Fontan procedures, 10 with atriopulmonary and 10 with total cavopulmonary connections, in Gothenburg between 1980 and 1991. All patients underwent cardiac catheterisation and pulmonary angiography. Bubble contrast echocardiography was performed at the same time, with injection of agitated polygelin colloid solution (Haemaccel, Hoechst) into the right and left pulmonary arteries, respectively. Transoesophageal echocardiography was used to detect the appearance of bubble contrast in the pulmonary venous atrium. The aim was also to evaluate the role of hepatic venous blood. Of the 20 patients, 9 (45%) had a positive contrast echocardiography study, compared with only 2 (10%) detected by pulmonary angiography. Patients with positive contrast echocardiography had a significantly lower arterial oxygen saturation than those with negative studies, both at rest (88% vs 95%, p < 0.01) and during exercise testing (78% vs 89%, p = 0.01). Bubble contrast echocardiography is much more sensitive in detecting pulmonary arteriovenous malformations than pulmonary angiography. By injecting echo contrast into the right and left pulmonary arteries, the method can be made highly selective. Pulmonary arteriovenous malformations develop much more frequently in patients with the Fontan circulation than previously reported.     

Acquired Intercostal Arteriovenous Fistulas

Acquired intercostal arteriovenous fistulas are recognized complications of traumatic injuries. In this article, transesophageal Doppler echocardiographic findings in a patient with posttraumatic acquired intercostal arteriovenous fistulas are presented. The findings have been confirmed by angiography. The demonstration of intercostal arteriovenous fistulas by transesophageal Doppler echocardiography has not been reported before.     

Evaluation of the complications of different types of right ventricular bypass by contrast echocardiography]

We evaluated 54 patients with different right heart by-pass by means of contrast echocardiography with rapid venous injection of shaken saline (3 cc, < 20 k; 6cc, > 20 k), in order to detect venous collateral circulation in partial by-pass, pulmonary arteriovenous fistulae in partial and total by-pass, and right-to-left shunt in total by-pass. Forty three patients had partial by-pass: 29 had a bidirectional cavopulmonary anastomosis with additional pulmonary flow (systemic-pulmonary anastomosis and/or restrictive anterograde ventricular flow); 10 had a partial ventricular correction (bidirectional cavopulmonary anastomosis and non restrictive anterograde ventricular flow; two had classical Glenn procedures; two had Kawashima operations (bidirectional cavopulmonary anastomosis and non restrictive anterograde ventricular flow; two had classical Glenn procedures; two had kawashima operations (bidirectional cavopulmonary anastomosis with inferior vena cava interruption), and 11 with total by-pass (10 atriopulmonary anastomosis and 1 with total cavopulmonary anastomosis). The age ranged from 2.5 to 33 years (x = 12.2 years), and the mean postoperative period was 4.3 years. Venous collateral circulation: 32/43 patients (74%) with partial by-pass, specially in those without partial biventricular correction: 29/33 patients (88%) compared to those with partial biventricular correction: 3/10 (30%) p < 0.01. Pulmonary arteriovenous fistulae: 6/43 patients (14%) with partial by-pass; 6/33 (18%) with partial biventricular correction, 0/10 patients with partial biventricular correction, and 0/11 with total by-pass. Right-to-left shunt: 2/11 patients (18%) with total by-pass, all of them with atriopulmonary anastomosis. Contrast echocardiography is an excellent noninvasive method for the initial evaluation of specific dysfunctions of different right heart by-pass. We emphasize the higher frequency of collateral venous circulation in cavopulmonary anastomosis, that would explain the dysfunction with progressive hypoxia in the evolution of these patients. Pulmonary arteriovenous fistulae were detected only in partial by-pass, without partial biventricular correction (p < 0.01). The minor frequency of these fistulae in bidirectional cavopulmonary anastomosis would be due to additional pulmonary flow.     

Bilateral multiple pulmonary arteriovenous fistulas and duplicated renal collecting system in a child with Noonan's syndrome

Noonan's syndrome involves the association of multiple congenital abnormalities, with a variety of cardiac defects. We describe here the association of Noonan's syndrome with multiple pulmonary arteriovenous fistulas and bilateral duplicated renal collecting systems. To the best of our knowledge, this is the first reported case of an association of the Noonan phenotype with pulmonary arteriovenous fistulas.     

Direct atriopulmonary anastomosis (modified Fontan) for single ventricle and its equivalents

The modified Fontan procedure is being used in an increasing number of complex cyanotic cardiac lesions with pulmonary stenosis. Seven patients aged 11 to 24 years (average 17.5 years) underwent surgery by a technique derived from the Fontan procedure: direct atriopulmonary anastomosis without a tube or valve. The tricuspid valve when patent was closed with a patch. The diagnoses were: single ventricule (4 cases), Taussig-Bing anomaly (2 cases) and tricuspid atresia (1 case). All patients had associated pulmonary stenosis with low pulmonary vascular resistances. The great vessels were in L-malposition in 3 cases. The hospital mortality was nil. Transient atrial fibrillation was observed in 2 cases and was well tolerated clinically. The follow-up period ranges from 2 months to 4 years (average 2.3 years). All patients are acyanotic with no signs of right-sided failure and in sinus rhythm. Control cardiac catheterisation and angiography were performed in 6 cases and showed good function of the anastomosis and a mean atrial pressure of 14 mmHg. Direct atriopulmonary anastomosis offers a very acceptable surgical solution to certain forms of single ventricle or equivalent with low pulmonary pressures. The short and medium term results seem to be better than those of intraventricular repair.     

Acquired Intercostal Arteriovenous Fistulas: Transesophageal Doppler Echocardiographic Diagnosis

Acquired intercostal arteriovenous fistulas are recognized complications of traumatic injuries. In this article, transesophageal Doppler echocardiographic findings in a patient with posttraumatic acquired intercostal arteriovenous fistulas are presented. The findings have been confirmed by angiography. The demonstration of intercostal arteriovenous fistulas by transesophageal Doppler echocardiography has not been reported before. (ECHOCARDIOGRAPHY, Volume 13, November 1996)     

Familial pulmonary hypertension and multiple abnormalities of large systemic arteries in Osler's disease

Two sisters with severe pulmonary hypertension and Osler's disease are described. Heart disease also occurred in other family members affected with hereditary hemorrhagic telangiectasia. Multiple abnormalities of large systemic vessels, severe intrahepatic arteriovenous fistulas and systemic-pulmonary arterial anastomoses were demonstrated in one of the patients, and we conclude that analogous manifestations may have been present in her sister and presumably in other members of the family too. The patients are discussed in relation to similar cases reported earlier and to various types of pulmonary hypertension.