Endovascular coil embolization of multiple pulmonary arteriovenous malformations in Osler-Weber-Rendu disease: A challenging pursuit

Pulmonary arterionevous malformation (PAVMs) are a rare disorder with an incidence of 2-3/1,000,000 population. Approximately 70% of cases are associated with Osler-Weber-Rendu Disease. This intrapulmonary malformation causes hypoxemia and dyspnea largely attributed to the right to left shunting. We present a case of 25-year-old male who was admitted for fever and headaches with chronic history of epistaxis, hemoptysis, cyanosis and clubbing. Central cyanocis and clubbing were evident with hypoxemia of 65%. Heart sounds and peripheral pulses were normal. There was no systolic bruit noted in the lung bases. Multiple telangiectasias were seen in the truncal area and abdomen. Chest x-ray revealed multi-chambered cardiomegaly with no opacifications. CBC showed erythrocytosis with hematocrits of 0.68-0.78. Transthoracic echocardiography showed intact interatrial and interventricular septum, with contrast study suggestive of intrapulmonary shunting. CT angiography revealed PAVMs in bilateral lung fields. Pulmonary angiography demonstrated diffuse PAVMS in the left lung with 3 large PAVMs with multiple feeders > 7mm and smaller PAVMs in the Right lower lobe. The diagnosis was Multiple Pulmonary Arteriovenous Malformation, Osler-Weber-Rendu Disease, Brain Abscess Left Temporoparietal area. Craniotomy with brain abscess evacuation was done. Left pneumonectomy or Right lobectomy was considered but was deemed unacceptable due to high morbidity and mortality. Endovascular coil embolization was done on the 3 large fistulas on the Left lobe. Post procedure, his arterial oxygenation improved to 96%. He has no recurrence of hemoptysis, no headaches and with less episodes of shortness of breath 2 months on follow-up. Diagnosing the complex diffuse intrapulmonary malformations requires a high index of suspicion among patients with chronic cyanosis, chronic history of bleeding, and brain abscess. Treatment of this condition and its associated complications remain a big challenge and should be highly individualized.

Update on the Amplatzer duct occluder: a 10-year experience in Asia.

Few data exist regarding the efficacy and safety of the Amplatzer ductal occluder (ADO) type 1 device in the Asian region. This retrospective study, conducted between August 2001 and April 2011, attempted device placement for 231 patients (165 females and 66 males) with a median age of 7.4 years (range, 3 months to 64 years) and an average weight of 19.4 kg (range, 4.1-81.0 kg). Among the patients in this study, 66 (28.6%) had pulmonary hypertension, ten (4.3%) had trisomy 21, and eight (3.5%) had other congenital cardiac anomalies. The mean narrowest patent ductus arteriosus (PDA) diameter was 4.2 mm (range, 1.3-10 mm), and the ampulla size was 9.6 mm (range, 4-20 mm). Successful implantation was achieved for 229 patients (99.1%). Complete angiographic occlusion was achieved for 201 patients (87.8%) at the end the procedure. Follow-up data were available for 129 patients (66%). At the follow-up assessment, complete echocardiographic occlusion was seen in 128 patients (99.2%) after 1 month and in 100% of the patients after 6 months. The significant morbidities involved one device embolization and one dislodgment, for which surgical retrieval was performed. No mortalities occurred during the study period, and no late clinical adverse events occurred during the follow-up period. Occlusion of the PDA using ADO is safe, effective, and applicable for a wide range of PDA sizes including large PDAs in small symptomatic infants and in adults. Good outcomes can be attributed to experience of the operators, proper patient selection, and appropriate device size selection.