Pulmonary arteriovenous malformations (PAVMs) are uncommon, predominantly congenital direct fistulous connections between the pulmonary arteries and pulmonary veins, resulting in a right to left shunt. Patients with PAVMs are usually asymptomatic with lesions detected incidentally when radiological imaging is performed for other indications. In this review, we discuss the classification and radiological features of PAVMs as well as their treatment and follow-up options, with a particular focus on percutaneous endovascular techniques and the evolution of the available equipment for treatment.
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http://dx.doi.org/10.3390/jcm11216282 | DOI Listing |
Gen Thorac Cardiovasc Surg Cases
December 2024
Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, 606-8507, Japan.
Background: Lung transplantation is a viable lifesaving option for patients with diffuse pulmonary arteriovenous malformations (AVMs). We present a case of diffuse pulmonary AVMs associated with juvenile polyposis and hereditary hemorrhagic telangiectasia (JP-HHT) that was successfully managed by lung transplantation.
Case Presentation: A 19-year-old woman developed severe hypoxemia due to pulmonary AVMs diagnosed at 4 years of age.
Int J Cardiol Congenit Heart Dis
June 2024
University of Sydney, Sydney, Australia.
Introduction: Rates of morbidity and mortality are high in the setting of Fontan physiology and effective medical therapies are not well-established. Clinical trials assessing phosophodiesterase-5-inhibitors, such as sildenafil, have not demonstrated major benefit in patients with a Fontan-type circulation but have only included stable, well-functioning people.
Methods: We sought to retrospectively characterize the people followed by the ANZ Fontan Registry prescribed sildenafil >30 days post Fontan-surgery.
Cureus
December 2024
Department of Cardiology, Japanese Red Cross Maebashi Hospital, Maebashi, JPN.
When encountering severe hypoxemia that does not respond to oxygen supplementation, it is essential to consider underlying right-to-left shunting. Among various diagnostic approaches, the microbubble test via transthoracic echocardiography (TTE) is a simple, noninvasive method for detecting pulmonary arteriovenous shunts, particularly in hepatopulmonary syndrome (HPS). Although microbubbles are usually administered peripherally, using a Swan-Ganz (SG) catheter to inject microbubbles directly into the pulmonary artery may provide even more definitive diagnostic information.
View Article and Find Full Text PDFCurr Probl Diagn Radiol
December 2024
Department of Radiology, Corewell Health William Beaumont University Hospital, Royal Oak, MI, USA.
The diagnostic algorithm for histoplasmosis highlights the importance of imaging and emphasizes the role of the radiologist in the diagnostic workup. Here we describe a case series of patients with a novel sign of lung involvement in histoplasmosis which we have coined the Pseudo-Pulmonary Arteriovenous Malformation (PAVM) sign, the usage of which would help in the imaging diagnosis of histoplasmosis aid by distinguishing it from PAVMs. PAVMs carry risk for serious complications such as systemic emboli and may require treatment; whereas, histoplasmomas do not.
View Article and Find Full Text PDFEur J Intern Med
December 2024
HHT Unit. Hospital Universitari Bellvitge, Barcelona, Spain; Internal Medicine Department. Hospital Universitari Bellvitge, Barcelona, Spain; Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain; Clinical Sciences Department. Faculty of Medicine and Health Sciences. Universitat de Barcelona, Barcelona, Spain; Center for Biomedical Research in Obesity and Nutrition Physiopathology Network (CIBEROBN). Carlos III Health Institute, Madrid, Spain. Electronic address:
Objectives: To describe the incidence of pulmonary arteriovenous malformations (PAVM)-related complications, right-to-left shunt (RLS) progression at transthoracic contrast echocardiography (TTCE) and development of treatable PAVM during long-term follow-up in hereditary hemorrhagic telangiectasia (HHT) patients with RLS grades 0-1 at screening TTCE.
Methods: Observational prospective study including adult HHT patients with grades 0-1 RLS at screening TTCE. Those requiring previous embolization of PAVM were excluded.
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