Panja, Manotosh; Panja, Madhumanti; Singhi, Anil Kumar1,
Belle Vue Clinic, Medica Super Specialty Hospital, Kolkata, West Bengal, India
1Department of Pediatric and Congenital Heart Disease, Medica Super Specialty Hospital, Kolkata, West Bengal, India
Address for correspondence: Dr. Anil Kumar Singhi, MD, FNB, Senior Consultant and Head, Department of Pediatric and Congenital Heart Disease, Medica Super Specialty Hospital, Kolkata - 700 099, West Bengal, India. E-mail: [email protected]
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A 19-year-old man presented with a history of worsening cyanosis since birth and frequent episodes of hemoptysis. His oxygen saturation (SpO2) was 84%. The heart structurally was normal. Computed tomographic pulmonary angiogram showed multiple pulmonary arteriovenous fistula (PAVF) in both lungs. Among those, the right lower lobe and the left upper lobe fistulae were large [Figure 1a]. In the first intervention, the right lower lobe fistula was closed with a 16 mm Amplatzer Vascular Plug-II (AVP-II, Abbott, Plymouth, MN, USA) [Figure 1b]. Similarly, a 12 mm AVP-II was deployed in the left upper lobe PAVF [Figure 1c]. After the intervention, the SpO2 improved to 98%. Eleven months later, the patient presented with a lowering of the SpO2 (90%) attributed to additional right-sided PAVF becoming significant [Figure 2a]. In the second-stage intervention, a 6 mm AVP-II was deployed in the right lower lobe and another 6 mm AVP-II was deployed in the right upper lobe [Figure 2b-d]. One fistula in the right middle lobe was closed with Gianturco coils (Cook Medical, Bloomington, IN, USA) [Figure 2e]. One year later, the young man again had mild effort intolerance and desaturation (SpO2 88%). In the third procedure, the left lower lobe large PAVF was closed with a 16 mm Vascular Amplatzer Plug and the large fistula at the mid-zone of the left lobe was dealt with 14 mm AVP-II [Figure 3a-c]. An additional 6 mm AVP-II was deployed in a smaller fistula in the left lower zone [Figure 3d]. The SpO2 became 100% after the final procedure and cyanosis fully disappeared even after exercise. The patient was doing well after 8 years of follow-up after the third procedure. Bilateral PAVF presents with recurrent hemoptysis, severe clubbing, and cyanosis. The PAVF can persist after initial treatment or due to either recanalization of the initial vessel, incomplete treatment, or interval reperfusion via the accessory vessel.[1] The closure of PAVF using various types of plugs such as Amplatzer Vascular Plugs in the index case or with coils is useful for complete occlusion. Successful closure of bilateral large size arteriovenous fistula results in long-term durable resolution.
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Figure 1: (a) Selective angiogram of the right lower pulmonary artery branch showing large pulmonary arteriovenous fistula (white arrow). (b) Complete occlusion of the fistula with Amplatzer Vascular Plug-II (yellow arrow). (c) Digital subtraction angiogram of the left upper lobe branch pulmonary artery showing Amplatzer Vascular Plug-II device in situ (green arrow) and no significant fistula in the left middle pulmonary artery branch
Figure 2: (a) Selective angiogram during the second-stage procedure showing pulmonary arteriovenous fistula filling the right lower pulmonary vein (blue arrow). (b) Complete occlusion of the fistula (a) with Amplatzer Vascular Plug-II (yellow arrow). (c) Deployment of Amplatzer Vascular Plug-II in the right middle lobe pulmonary artery branch fistula (light green arrow) with complete occlusion (d) seen in the subsequent angiogram (dark green arrow). (e) Coil occlusion of right mid-zone pulmonary arteriovenous fistula (white arrow). Three Amplatzer Vascular Plug-II occluders placed in different pulmonary arteriovenous fistula are marked with a red, blue, and sky-blue arrow
Figure 3: (a) Selective angiogram in the left lower lobe pulmonary artery branch showing large pulmonary arteriovenous fistula (blue arrow) which was occluded with the Amplatzer Vascular Plug-II device (b, green arrow). (c) Selective angiogram of the left mid-zone branch showing pulmonary arteriovenous fistula (white arrow) which was closed with another Amplatzer Vascular Plug-II device (d, yellow arrow). (d) Four different sized Amplatzer Vascular Plug-II occluders placed in the left lung pulmonary arteriovenous fistula marked with yellow, pink, green, and red arrows
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
REFERENCE
1. Khurshid I, Downie GH. Pulmonary arteriovenous malformation. Postgrad Med J 2002;78:191-7
