Idiopathic Pulmonary Arteriovenous Malformation: A Case Report

Pulmonary arteriovenous malformation (PAVM) is a rare abnormal connection between a pulmonary vein and a pulmonary artery, resulting in blood bypassing the capillary bed and causing right-to-left shunting. We aim to report a case of idiopathic PAVM managed with coil embolization to highlight this ra...

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Main Authors: Abdul Samad, Farah Naz, Nauman Ismat Butt, Zubair Ashraf, Muhammad Sohail Ajmal Ghoauri, Ayesha Zahid
Format: Article
Language:English
Published: Jaypee Brothers Medical Publisher 2024-09-01
Series:Indian Journal of Respiratory Care
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Online Access:https://www.ijrc.in/doi/IJRC/pdf/10.5005/jp-journals-11010-1128
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author Abdul Samad
Farah Naz
Nauman Ismat Butt
Zubair Ashraf
Muhammad Sohail Ajmal Ghoauri
Ayesha Zahid
author_facet Abdul Samad
Farah Naz
Nauman Ismat Butt
Zubair Ashraf
Muhammad Sohail Ajmal Ghoauri
Ayesha Zahid
author_sort Abdul Samad
collection DOAJ
description Pulmonary arteriovenous malformation (PAVM) is a rare abnormal connection between a pulmonary vein and a pulmonary artery, resulting in blood bypassing the capillary bed and causing right-to-left shunting. We aim to report a case of idiopathic PAVM managed with coil embolization to highlight this rare disease. A 15-year-old girl presented with a 2-year history of progressively worsening shortness of breath on exertion and intermittent self-limiting hemoptysis. She had cyanosis with an oxygen saturation of 86% on room air. No telangiectasia was seen. Chest X-ray revealed nonhomogeneous opacifications, most marked in the right lower zone. High-resolution computed tomography (HRCT) chest demonstrated centrilobular nodules, some with a branching pattern in the anterior segment of the right upper lobe and apical segments of both the right upper and lower lobes; interlobar and intralobar septal thickening along with a prominent bronchovascular bundle in the right upper and lower lobes. CT pulmonary angiography revealed multiple dilated and enlarged serpiginous vessels in the right lung; in the arterial phase, simultaneous opacification of arteries and veins demonstrated a connection between the pulmonary artery and veins, consistent with the diffuse type of PAVM. A diagnosis of idiopathic diffuse PAVM was made, and three sessions of coil embolization were performed over a period of 1 year. By the 12-month follow-up, she was stable and asymptomatic. PAVM is a rare occurrence, and early screening with prompt management is required to improve prognosis. Our patient, who presented with shortness of breath and hemoptysis, was diagnosed with idiopathic PAVM. She refused surgery and was managed with coil embolization, which resulted in marked improvement. The differential diagnosis of shortness of breath includes respiratory, cardiac, and hematological diseases. A high index of clinical suspicion is required to timely diagnose PAVM to reduce complications and morbidity. PAVM is best managed by a multidisciplinary team encompassing an internist, pulmonologist, interventional radiologist, and cardiothoracic surgeon.
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spelling doaj-art-712a3daa3e9d48e3aaf97d668f552f162025-08-20T03:17:58ZengJaypee Brothers Medical PublisherIndian Journal of Respiratory Care2277-90192321-48992024-09-0113320220510.5005/jp-journals-11010-112812Idiopathic Pulmonary Arteriovenous Malformation: A Case ReportAbdul Samad0Farah Naz1Nauman Ismat Butt2Zubair Ashraf3Muhammad Sohail Ajmal Ghoauri4Ayesha Zahid5Department of Pulmonology, Bahawal Victoria Hospital; Quaid-e-Azam Medical College, Bahawalpur, Punjab, PakistanDepartment of Pulmonology, Bahawal Victoria Hospital; Quaid-e-Azam Medical College, Bahawalpur, Punjab, PakistanNauman Ismat Butt, Department of Medicine and Allied, Azra Naheed Medical College, Superior University, Lahore, Punjab, Pakistan, Phone: +923454651049Department of Pulmonology, Bahawal Victoria Hospital; Quaid-e-Azam Medical College, Bahawalpur, Punjab, PakistanDepartment of Neurology, Bahawal Victoria Hospital; Quaid-e-Azam Medical College, Bahawalpur, Punjab, PakistanDepartment of Pulmonology, Bahawal Victoria Hospital; Quaid-e-Azam Medical College, Bahawalpur, Punjab, PakistanPulmonary arteriovenous malformation (PAVM) is a rare abnormal connection between a pulmonary vein and a pulmonary artery, resulting in blood bypassing the capillary bed and causing right-to-left shunting. We aim to report a case of idiopathic PAVM managed with coil embolization to highlight this rare disease. A 15-year-old girl presented with a 2-year history of progressively worsening shortness of breath on exertion and intermittent self-limiting hemoptysis. She had cyanosis with an oxygen saturation of 86% on room air. No telangiectasia was seen. Chest X-ray revealed nonhomogeneous opacifications, most marked in the right lower zone. High-resolution computed tomography (HRCT) chest demonstrated centrilobular nodules, some with a branching pattern in the anterior segment of the right upper lobe and apical segments of both the right upper and lower lobes; interlobar and intralobar septal thickening along with a prominent bronchovascular bundle in the right upper and lower lobes. CT pulmonary angiography revealed multiple dilated and enlarged serpiginous vessels in the right lung; in the arterial phase, simultaneous opacification of arteries and veins demonstrated a connection between the pulmonary artery and veins, consistent with the diffuse type of PAVM. A diagnosis of idiopathic diffuse PAVM was made, and three sessions of coil embolization were performed over a period of 1 year. By the 12-month follow-up, she was stable and asymptomatic. PAVM is a rare occurrence, and early screening with prompt management is required to improve prognosis. Our patient, who presented with shortness of breath and hemoptysis, was diagnosed with idiopathic PAVM. She refused surgery and was managed with coil embolization, which resulted in marked improvement. The differential diagnosis of shortness of breath includes respiratory, cardiac, and hematological diseases. A high index of clinical suspicion is required to timely diagnose PAVM to reduce complications and morbidity. PAVM is best managed by a multidisciplinary team encompassing an internist, pulmonologist, interventional radiologist, and cardiothoracic surgeon.https://www.ijrc.in/doi/IJRC/pdf/10.5005/jp-journals-11010-1128case reportcoil embolizationdyspneahemoptysispulmonary arteriovenous malformation
spellingShingle Abdul Samad
Farah Naz
Nauman Ismat Butt
Zubair Ashraf
Muhammad Sohail Ajmal Ghoauri
Ayesha Zahid
Idiopathic Pulmonary Arteriovenous Malformation: A Case Report
Indian Journal of Respiratory Care
case report
coil embolization
dyspnea
hemoptysis
pulmonary arteriovenous malformation
title Idiopathic Pulmonary Arteriovenous Malformation: A Case Report
title_full Idiopathic Pulmonary Arteriovenous Malformation: A Case Report
title_fullStr Idiopathic Pulmonary Arteriovenous Malformation: A Case Report
title_full_unstemmed Idiopathic Pulmonary Arteriovenous Malformation: A Case Report
title_short Idiopathic Pulmonary Arteriovenous Malformation: A Case Report
title_sort idiopathic pulmonary arteriovenous malformation a case report
topic case report
coil embolization
dyspnea
hemoptysis
pulmonary arteriovenous malformation
url https://www.ijrc.in/doi/IJRC/pdf/10.5005/jp-journals-11010-1128
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AT naumanismatbutt idiopathicpulmonaryarteriovenousmalformationacasereport
AT zubairashraf idiopathicpulmonaryarteriovenousmalformationacasereport
AT muhammadsohailajmalghoauri idiopathicpulmonaryarteriovenousmalformationacasereport
AT ayeshazahid idiopathicpulmonaryarteriovenousmalformationacasereport