Successful Transcatheter Closure of a Giant Congenital Pulmonary Arteriovenous Fistula
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J INVASIVE CARDIOL 2024. doi:10.25270/jic/24.00308. Epub November 27, 2024.
A 7-year-old girl presented with cyanosis for the past 4 years. Physical examination revealed digital clubbing and cyanosis, and a rest arterial oxygen saturation level of 82%. Auscultation revealed a continuous murmur on the right side of her chest. The electrocardiogram showed a normal sinus rhythm. Transthoracic echocardiography showed dilatation of the left atrium and ventricle but no cardiac structural abnormalities. Pulmonary arteriovenous fistula (PAVF) was suspected.
Maximal intensity projection CT angiography (CTA) revealed a giant PAVF between the right inferior pulmonary artery and the right inferior pulmonary vein (RIPV), measuring 6.8 × 5.4 cm (Figure A, B). Volume-rendered CTA showed the feeding and draining vessels of the PAVF (Figure C). Selective right pulmonary angiography revealed the giant PAVF (Figure D, Video 1).
The patient was recommended for interventional closure of the PAVF. A 6-French (Fr) catheter with a 100-cm diameter was placed over a stiff exchange wire. A 125-cm 5 Fr, coaxial catheter was used to position the tip of the guiding catheter into the distal portion of the feeding artery for passage between the artery and vein. The PAVF was successfully occluded with a 12- to 14-mm Amplatzer Vascular Plug (AGA Medical Corporation) (Video 2). After the procedure, the patient's arterial oxygen saturation level returned to 96% at rest. The postoperative course was uncomplicated, and she was discharged after 5 days.
Congenital PAVF is a rare vascular disease that is classified into 3 types: simple, complex, and diffuse.1 The main clinical manifestations of PAVF include cyanosis, dyspnea, high-output heart failure, ischemic stroke, hemoptysis, and paradoxical embolization.2 If the PAVF is not diagnosed and treated, the patient can develop respiratory failure, spontaneous hemothorax, or other catastrophic complications.3 Even though pulmonary angiography is the gold standard for diagnosing PAVF, it is at risk of causing vessel injury. CTA and magnetic resonance angiography of the pulmonary arteries can clearly and entirely visualize the location, quantity, extent, and morphological structure of the PAVF. Therefore, these tools are a viable way to evaluate the diagnosis and severity, particularly in complex PAVF types.4 Early diagnosis and a multidisciplinary approach could prevent the progression of the disease and improve the prognosis of the patient.
Affiliations and Disclosures
Leizhi Ku, MD1; Yuhang Wang, MD1; Zheng Liu, MD1; Xiaojing Ma, PhD2
From the Departments of 1Radiology and 2Echocardiography, Wuhan Asia Heart Hospital Affiliated Wuhan University of Science and Technology, Wuhan, China
Dr Ku and Dr Wang contributed equally to the article.
Disclosures: The authors report no financial relationships or conflicts of interest regarding the content herein.
Funding: This work is funded by the 2021 General Project Health and Family Planning Commission of Wuhan municipality scientific research project (WX21D46); Wuhan Clinical Medical Research Center for Cardiovascular Imaging (CMRC202307).
Consent statement: The authors confirm that the patient provided informed consent for the procedures presented in this manuscript.
Address for correspondence: Xiaojing Ma, PhD, Department of Echocardiography, Wuhan Asia Heart Hospital Affiliated Wuhan University of Science and Technology, Wuha 430022, P.R. China. Email: klz1534292102@163.com.
References
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2. Guvenc O, Odemis E, Saygi M, Onalan MA. Transcatheter management of life-threatening pulmonary arteriovenous fistula with extracorporeal membrane oxygenation support in an infant. Cardiol Young. 2023;33(3):498-501. doi:10.1017/S1047951122002724
3. Shu L, Luo L, Zuo Y. Attention to pulmonary arteriovenous fistula in a case of transient hypoxemia and cerebral infarction during pregnancy: a case report and literature review. BMC Pregnancy Childbirth. 2023;23(1):626. doi:10.1186/s12884-023-05946-2
4. Wu Z, Zeng C, Wang H, Shi W, Luo X. The chief culprit of intractable hypoxemia: a case report of rare pulmonary arteriovenous fistula complicated with giant hemangioma. J Cardiothorac Surg. 2024;19(1):78. doi:10.1186/s13019-024-02521-4
