Pulmonary Arteriovenous Malformations

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J Am Soc Echocardiogr. 2004 Jul;17(7):775-7.
Recurrent transient ischemic attacks in a patient with intrapulmonary arteriovenous shunting detected after closure of a patent foramen ovale.
Unger P, Stoupel E, Shadfar S, Pandolfo M, Blecic S.
Department of Cardiology, Erasme Hospital, Brussels, Belgium. punger@ulb.ac.be

Paradoxical embolism through right-to-left shunts is widely accepted as a potential cause of cerebral ischemia. Contrast echocardiography is an excellent tool for detection of these shunts. The timing of the appearance of bubbles in the left atrium (ie, early vs late) allows differentiation of foramen ovale patency from intrapulmonary shunting as a result of arteriovenous malformations. We report a patient with recurrent neurologic deficit after surgical closure of a patent foramen ovale. Transesophageal echocardiography demonstrated residual right-to-left shunting from previously unrecognized pulmonary arteriovenous malformations associated with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu). This case illustrates the fact that contrast echocardiography may fail to identify intrapulmonary shunts when a resting patent foramen ovale coexists.

Echocardiography. 2003 May;20(4):391-4.
Contrast transesophageal echocardiographic detection of a pulmonary arteriovenous malformation draining into left lower pulmonary vein.
Ahmed S, Nanda NC, Nekkanti R, Yousif AM.
Division of Cardiovascular Disease, The University of Alabama at Birmingham, Birmingham, Alabama, USA.

We report the identification of a pulmonary arteriovenous malformation draining into the left lower pulmonary vein by contrast two-dimensional transesophageal echocardiography in an adult with no evidence of hereditary hemorrhagic telangiectasia. To our knowledge, this has not been reported previously. This study also emphasizes the importance of transesophageal echocardiographic examination of the left lower pulmonary vein in the detection of a pulmonary arteriovenous malformation.

Chest. 2003 Feb;123(2):351-8.
Comment in: Chest. 2003 Feb;123(2):320-2.
Contrast echocardiography remains positive after treatment of pulmonary arteriovenous malformations.
Lee WL, Graham AF, Pugash RA, Hutchison SJ, Grande P, Hyland RH, Faughnan ME.
Division of Respirology, St. Michael's Hospital, University of Toronto, ON, Canada.

STUDY OBJECTIVES: Pulmonary arteriovenous malformations (PAVMs) in patients with hereditary hemorrhagic telangiectasia (HHT) can cause hemorrhage, stroke, and cerebral abscess. Therapy consists of transcatheter embolotherapy (TCET) to occlude the PAVMs. Contrast transthoracic echocardiography (TTE) can be used to screen for PAVMs, but little is known about the performance of contrast TTE after TCET has been performed. Our objective was to determine the effect of the successful performance of TCET on the performance of contrast TTE, specifically, in what proportion of patients the findings of contrast TTE normalized or remained positive after the performance of TCET. DESIGN: Retrospective chart review. SETTING: HHT clinic at university teaching hospital. PATIENTS: Patients who have undergone TCET for the treatment of PAVMs. INTERVENTIONS: Patients were screened for PAVMs with a chest radiograph (CXR), oxygen shunt test (OST), and contrast TTE. Pulmonary angiography was recommended for patients with any positive findings on a screening test. PAVMs > or = 3 mm were occluded by TCET. Contrast TTE, OST, and CXR were performed approximately 1 month later. The results of contrast TTE before and after patients underwent TCET were compared. Measurements and results: Thirty-nine patients underwent contrast TTE prior to undergoing TCET, and 29 patients underwent contrast TTE both prior to and after undergoing TCET. In all patients, TTE findings were positive prior to TCET. All PAVMs with feeding vessels > or = 3 mm were successfully occluded based on completion angiography. After TCET, 48% of patients had no detectable residual PAVMs, and the remainder had small (ie, < 3 mm) residual PAVMs. Of the 29 patients, 90% had positive contrast TTE findings after undergoing TCET. In the subset of patients who had no residual PAVMs on the completion angiography, 80% had positive contrast TTE findings after undergoing TCET. CONCLUSIONS: In most patients, contrast TTE findings remain positive after they undergo TCET, even in patients without residual PAVMs seen on angiography. This may reflect residual PAVMs that are too small to visualize using angiography. These findings have important implications for the follow-up and management of HHT patients.

J Am Soc Echocardiogr. 2002 Sep;15(9):1012-4.
Intrapulmonary arteriovenous shunt: diagnosis by saline contrast bubbles in the pulmonary veins.
Gudavalli A, Kalaria VG, Chen X, Schwarz KQ.
Cardiology Unit, Department of Medicine, University of Rochester, 601 Elmwood Avenue, Rochester, NY 14642, USA.

A 54-year-old man with end-stage cirrhosis of the liver presented for evaluation of dyspnea. Intrapulmonary arteriovenous shunting was suspected. Transthoracic echocardiography with agitated saline contrast injection from the right antecubital vein was performed. Late arrival of saline contrast bubbles in the left atrium by pulmonary veins leading to complete left heart opacification confirmed the diagnosis of significant intrapulmonary shunting. This case and images illustrate the use of agitated saline contrast in combination with transthoracic echocardiography for the diagnosis of intrapulmonary arteriovenous fistula.

Am J Cardiol. 2002 Feb 1;89(3):281-5.
Comparison of contrast echocardiography versus cardiac catheterization for detection of pulmonary arteriovenous malformations.
Feinstein JA, Moore P, Rosenthal DN, Puchalski M, Brook MM.
Department of Pediatrics, Stanford University, Stanford, California, USA. Jeff.Feinstein@stanford.edu

Because clinical diagnosis of pulmonary arteriovenous malformations (AVMs) is difficult and cardiac catheterization invasive, contrast echo has been used to aid in the diagnosis. Correlation between contrast echo and catheterization data in these patients remains poorly defined. We examined the ability to detect pulmonary AVMs by contrast echocardiography, pulmonary angiography, and pulmonary vein saturations in patients who have undergone cavopulmonary (Glenn) shunts. Pulmonary vein saturations were obtained from catheterization reports. Blinded observers reviewed angiographic and contrast echo data retrospectively in 27 patients who had undergone a Glenn shunt and in 19 controls with biventricular hearts. Contrast echo was positive in 68 of 99 lungs (69%). Angiography showed AVMs in 65 of 98 lungs (66%). Pulmonary vein desaturation was found in 13 of 45 lungs sampled (29%). Only 10 of 38 lungs with positive contrast echo had pulmonary vein desaturation, but only 1 patient had pulmonary vein desaturation without positive contrast echo. The degree of desaturation did not correlate with severity of contrast echo return; 6 of 11 patients with 3+ contrast echo studies had normal pulmonary vein saturations. In a control group of patients with biventricular hearts, only 1 of 19 (5.3%) contrast echos was weakly positive. Contrast echo correlates poorly with angiography and pulmonary vein saturation for evaluation of AVMs. Contrast echo is extremely sensitive and often positive despite normal pulmonary vein saturation. Assessment of pulmonary vein desaturation in 100% oxygen may improve its sensitivity and correlation with contrast echo. Additional studies are needed to follow patients with mildly positive contrast echo studies to enable determination of their clinical relevance.

Am Heart J. 2001 Feb;141(2):243-6.
Contrast echocardiography for detection of pulmonary arteriovenous malformations.
Nanthakumar K, Graham AT, Robinson TI, Grande P, Pugash RA, Clarke JA, Hutchison SJ, Mandzia JL, Hyland RH, Faughnan ME.
Division of Cardiology, St Michael's Hospital, University of Toronto, 30 Bond St., Toronto M5B 1W8, Ontario, Canada.

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) lead to stroke, brain abscess, and hemorrhage in hereditary hemorrhagic telangiectasia (HHT). The current screening approach for PAVMs in HHT patients with chest radiograph (CXR) and oxygen shunt study has not been validated and is thought to be insensitive. We hypothesized that agitated saline contrast echocardiography (ECHO) would be a useful screening test for PAVMs. METHODS AND RESULTS: A total of 106 sequential HHT patients underwent screening for PAVMs with ECHO in a prospective study. If the test was positive, or if the CXR or shunt study suggested PAVMs, pulmonary angiography was performed. A positive ECHO was defined as appearance of bubbles in the left atrium after injection of agitated saline solution. A positive shunt study was defined as a partial pressure of oxygen in arterial blood <500 mm Hg while breathing 100% oxygen. The mean age was 41 years (range 15-80 years); 66% were female. Forty-four patients had positive ECHO. Forty-one of the 44 patients underwent angiography. Three patients declined further testing. Thirty-three of the 41 patients who underwent angiography were diagnosed with PAVMs. Of the 62 patients with a negative ECHO, 18 underwent angiography because of either a shunt study or CXR that was suggestive of PAVMs. Of these 18 patients, 2 had PAVMs. In the total population of 106 patients, 35 (33%) had PAVMs. ECHO was the only positive screening test in 11 of 35 (31%) patients. The diagnosis of PAVMs in these 11 patients would have otherwise been missed. CONCLUSIONS: ECHO is a useful screening tool for PAVMs in HHT.

Cardiol Young. 2001 Sep;11(5):505-11.
Bubble contrast echocardiography in detecting pulmonary arteriovenous malformations after modified Fontan operations.
Larsson ES, Solymar L, Eriksson BO, de Wahl Granelli A, Mellander M.
Department of Pediatric Cardiology, The Queen Silvia Children's Hospital, Goteborg University, Sweden. eva.stromvall-larsson@vgregion.se

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.

Mayo Clin Proc. 1999 Jul;74(7):671-80.
Comment in: Mayo Clin Proc. 1999 Dec;74(12):1305.
Pulmonary arteriovenous fistulas: Mayo Clinic experience, 1982-1997.
Swanson KL, Prakash UB, Stanson AW.
Department of Internal Medicine, Mayo Clinic, Rochester, Minn. 55905, USA.

OBJECTIVE: To describe the results of analysis of clinical, physiologic, diagnostic, and therapeutic aspects and complications in patients with pulmonary arteriovenous fistulas (PAVFs). PATIENTS AND METHODS: Retrospective review of medical records of all patients with the diagnosis of PAVF evaluated at Mayo Clinic Rochester from 1982 through 1997. Demographic characteristics, presence or absence of hereditary hemorrhagic telangiectasia, clinical features, and results of imaging studies and blood gas analyses, treatments, and complications related to PAVFs were reviewed. RESULTS: Among the 93 patients, 44 were male and 49 female. The mean age at the time of evaluation was 40 years (range, 5-83 years). Fifteen patients (16%) were asymptomatic. History of hereditary hemorrhagic telangiectasia was present in 52 patients (56%). Notable clinical findings included epistaxis in 46 (49%), hemoptysis in 14 (15%), cyanosis in 27 (29%), clubbing in 18 (19%), dyspnea in 53 (57%), and pulmonary bruits/murmurs in 32 (34%). Chest x-ray films with or without tomograms showed abnormal findings in 87 (94%), of which 68 (73%) suggested PAVF. Polycythemia was detected in 12 (13%). Pretherapy arterial PO2 measured on room air averaged 56 mm Hg (range, 32-95 mm Hg), and the posttherapy PO2 averaged 77 mm Hg (range, 46-110 mm Hg). Echocardiography with indocyanine green dye was diagnostic of extracardiac right-to-left shunt in 26 (90%) of 29 patients tested. Diagnostic studies revealed single lesions in 32 patients (34%) and multiple lesions in 61 (66%). The most prominent complications of the disease were neurologic events in 34 patients (37%). These complications included transient ischemic attacks, hemiplegia, brain abscesses, and seizures. Surgical resection alone was carried out in 18 patients (19%), embolization therapy alone in 41 (44%), and both therapies in 7 (8%). The 48 patients treated with embolization required 78 embolization sessions with more than 200 lesions occluded. Complications of treatment included postembolization hemothorax in 1 patient and right-sided hemiparesis in another patient. Follow-up disclosed that 1 patient died from PAVF-related complications. CONCLUSIONS: Among our patients with PAVFs, hereditary hemorrhagic telangiectasia was observed in more than half and neurologic complications in more than one third. Because of the considerable risk of neurologic and other complications, definitive treatment should be considered in patients with PAVFs. Embolization is currently the preferred treatment in most patients. Frequent follow-up of treated patients is necessary because PAVFs tend to increase both in number and in size over time.

J Heart Lung Transplant. 1997 Dec;16(12):1271-4.
Resolution of cavopulmonary shunt-associated pulmonary arteriovenous malformation after heart transplantation.
Graham K, Sondheimer H, Schaffer M.
Department of Pediatrics, University of Colorado School of Medicine, Denver, USA.

A child with heterotaxia, azygous continuation of an interrupted inferior vena cava, single ventricle, and pulmonary atresia underwent the Kawashima modification of the Fontan procedure, which excluded hepatic venous return from the pulmonary blood flow. After the operation, the patient had development of pulmonary arteriovenous malformations, increasing cyanosis, and ventricular dysfunction. He underwent orthotopic heart transplantation at 7 years of age with prompt resolution of the pulmonary arteriovenous malformations.

Am J Cardiol. 1991 Dec 1;68(15):1507-10.
Two-dimensional contrast echocardiography in the detection and follow-up of congenital pulmonary arteriovenous malformations.
Barzilai B, Waggoner AD, Spessert C, Picus D, Goodenberger D.
Cardiovascular, Pulmonary Division, Washington University School of Medicine, St. Louis, Missouri 63110.

Pulmonary arteriovenous (A-V) malformation is frequently a manifestation of Osler-Weber-Rendu syndrome (hereditary hemorrhagic telangiectasia). We identified 14 patients (9 men and 5 women) with A-V malformation by contrast echocardiography; 10 patients with atrial right-to-left shunt served as control subjects. Agitated saline solution (10 ml) was injected through a peripheral vein during echocardiographic imaging. The delay in the appearance of microcavitations in the left atrium was measured (in number of frames) after right atrial appearance. The degree of left ventricular opacification was graded 1 to 4+ (where 4+ = intense left ventricular endocardial outline, and 1+ = minimal opacification). Results indicated patients with A-V malformation had a significant delay (p less than 0.001) in left atrial appearance of microcavitations compared with those with atrial right-to-left shunt (66 +/- 27 vs 21 +/- 7 frames, mean +/- 1 standard deviation). In the group with A-V malformation, abnormal blood gases were present in only 6 of 14 patients and chest x-ray was positive in 7. Pulmonary angiography was performed in 11 of 14 patients with positive contrast echocardiography, and all 11 had A-V malformation identified. In patients with 3 to 4+ left ventricular opacification (n = 8), large (greater than 5 mm feeding vessel) or multiple malformations were present, whereas patients with small or isolated malformation had 1 to 2+ left ventricular opacification. Balloon occlusion of malformations was performed in all 11 of these patients; repeat contrast echocardiography revealed significant diminution of right-to-left shunt in 9, and 2 required repeat embolotherapy for an additional previously undetected A-V malformation.(ABSTRACT TRUNCATED AT 250 WORDS)

J Pediatr Gastroenterol Nutr. 1990 Jul;11(1):133-7.
Association of juvenile and adenomatous polyposis with pulmonary arteriovenous malformation and hypertrophic osteoarthropathy.
Prieto G, Polanco I, Sarria J, Larrauri J, Lassaletta L.
Unidad de Gastroenterologia y Nutricion, Hospital Infantil La Paz, Madrid, Spain.

The juvenile form is the usual type of colonic polyp seen during childhood. However, mixed forms associating juvenile and adenomatous polyps have been reported. A syndrome including the association of generalized juvenile polyposis, pulmonary arteriovenous malformation, and hypertrophic osteoarthropathy has been described in three cases; however, this is the first report of the association of mixed juvenile and adenomatous polyposis, pulmonary arteriovenous malformation, and hypertrophic osteoarthropathy.

Am Rev Respir Dis. 1986 Aug;134(2):334-9.
Pulmonary arteriovenous malformations: a critical update.
Burke CM, Safai C, Nelson DP, Raffin TA.

Although first reported 90 yr ago, the natural history of pulmonary arteriovenous malformations (PAVM) is still incompletely understood. We review historical perspectives and pathologic classification of this rare disease and identify etiologic factors and clinical features in the 450 reported cases. The many different diagnostic modalities currently available are described, and a logical sequence of investigations is suggested. Pulmonary angiography remains mandatory prior to definitive treatment. The available evidence strongly suggests that morbidity and mortality in untreated PAVM exceeds that associated with either surgical resection or embolic occlusion. Thus, definitive treatment should be offered in all cases, with the possible exception of asymptomatic patients with small lesions who have no evidence of Osler-Weber-Rendu disease. Embolotherapy is now the treatment of choice in institutions with the necessary expertise and facilities, notwithstanding the absence of long-term follow-up for patients treated this way. It is suggested that increased documentation of long-term morbidity and mortality in both treated and untreated patients with PAVM will lead to greater understanding and more effective management of this enigmatic condition.

Cardiovasc Intervent Radiol. 1985;8(3):151-5.
Cerebral embolism--first manifestation of pulmonary arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia.
Hewes RC, Auster M, White RI Jr.

Four of five patients with asymptomatic small or moderate-size pulmonary arteriovenous malformation (PAVM) presented with a paradoxical embolus and stroke. In these patients chest radiographic findings were subtle and arterial hypoxemia provided the real clue to diagnosis. Computed tomography (CT) and cerebral arteriography confirmed embolic occlusion in four of five patients. Careful family screening including posteroanterior (PA) and lateral chest radiographs and arterial oxygen determinations in sitting or standing positions are recommended for early detection of asymptomatic patients with PAVM. Early therapeutic intervention (with balloon embolotherapy) is recommended to avoid paradoxical embolization.

Gastroenterology. 1980 Jun;78(6):1566-70.
Hereditary generalized juvenile polyposis associated with pulmonary arteriovenous malformation.
Cox KL, Frates RC Jr, Wong A, Gandhi G.

A 28-yr-old mother and her 10-year-old daughter with generalized juvenile gastrointestinal polypsis, arteriovenous (AV) malformations of the lung and severe digital clubbing are described. The AV malformations were documented by pulmonary angiography in both subjects, and generalized juvenile polyposis was confirmed histologically and radiographically. Since pulmonary AV malformations associated with juvenile polyposis has not been previously reported, a new hereditary syndrome is proposed.

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