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Successful Catheter Embolization of a Pulmonary Arteriovenous Malformation in a Patient with Hereditary Pulmonary Arterial Hypertension Due to BMPR2 Mutation
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A7005 - Successful Catheter Embolization of a Pulmonary Arteriovenous Malformation in a Patient with Hereditary Pulmonary Arterial Hypertension Due to BMPR2 Mutation
Author Block: J. D'Amico1, J. Pollak2, T. Ryan1, W. H. Fares3, J. A. Wirth1; 1Maine Pulmonary Hypertension Program, Maine Medical Center, Portland, ME, United States, 2Vascular and Interventional Radiology, Yale University School of Medicine, New Haven, CT, United States, 3Pulmonary Vascular Disease Program, Yale University School of Medicine, New Haven, CT, United States.
Introduction: Hereditary Pulmonary Arterial Hypertension (HPAH) with concomitant pulmonary arteriovenous malformation (PAVM) is a rare presentation and is typically associated with Hereditary Hemorrhagic Telangiectasia (HHT) and ALK1 mutations. We present a case of a Hereditary Pulmonary Arterial Hypertension patient with pulmonary AVM who has a BMPR2 mutation without evidence of HHT. Our patient underwent successful catheter embolization of the PAVM. Case Presentation: A 64 year old male with HPAH and known BMPR2 mutation presented with recurrent non-massive hemoptysis. A pulmonary CT angiogram revealed a left lower lobe PAVM. Right heart catheterization (RHC) showed pulmonary artery pressure (PAP) 100/38 mmHg, mean PAP (mPAP) 62 mm Hg, pulmonary capillary wedge pressure (PCWP) 15 mmHg, cardiac output (CO) 8.8 L/min, cardiac index (CI) of 4.0 l/min/m2, and pulmonary vascular resistance (PVR) of 5.4 WU. He subsequently underwent pulmonary angiography with stepwise embolization of his PAVM. Post-embolization RHC showed: PAP 110/35 mmHg, mPAP 64 mmHg, PCWP 14 mmHg, CO 5.4 L/min, CI 2.6 L/min/m2 and PVR 8.9 WU. His functional class improved from WHO FC II to WHO FC I. His Six- Minute Walk Test distance improved from 418 meters to 518 meters and Borg Dyspnea Index from 4 to 2. His symptoms, functional status, exercise performance and echocardiogram have remained stable for more than 14 months post-embolization. Discussion: This is the third documented case of PAVM associated with HPAH due to BMPR2 mutation. Ours is the first report of a successful catheter embolization of a PAVM in a HPAH patient. Our patient had stable hemodynamics with improved functional status after embolotherapy. Careful catheter closure of pulmonary AVMs may be considered in select patients with Pulmonary Arterial Hypertension.
Author Block: J. D'Amico1, J. Pollak2, T. Ryan1, W. H. Fares3, J. A. Wirth1; 1Maine Pulmonary Hypertension Program, Maine Medical Center, Portland, ME, United States, 2Vascular and Interventional Radiology, Yale University School of Medicine, New Haven, CT, United States, 3Pulmonary Vascular Disease Program, Yale University School of Medicine, New Haven, CT, United States.
Introduction: Hereditary Pulmonary Arterial Hypertension (HPAH) with concomitant pulmonary arteriovenous malformation (PAVM) is a rare presentation and is typically associated with Hereditary Hemorrhagic Telangiectasia (HHT) and ALK1 mutations. We present a case of a Hereditary Pulmonary Arterial Hypertension patient with pulmonary AVM who has a BMPR2 mutation without evidence of HHT. Our patient underwent successful catheter embolization of the PAVM. Case Presentation: A 64 year old male with HPAH and known BMPR2 mutation presented with recurrent non-massive hemoptysis. A pulmonary CT angiogram revealed a left lower lobe PAVM. Right heart catheterization (RHC) showed pulmonary artery pressure (PAP) 100/38 mmHg, mean PAP (mPAP) 62 mm Hg, pulmonary capillary wedge pressure (PCWP) 15 mmHg, cardiac output (CO) 8.8 L/min, cardiac index (CI) of 4.0 l/min/m2, and pulmonary vascular resistance (PVR) of 5.4 WU. He subsequently underwent pulmonary angiography with stepwise embolization of his PAVM. Post-embolization RHC showed: PAP 110/35 mmHg, mPAP 64 mmHg, PCWP 14 mmHg, CO 5.4 L/min, CI 2.6 L/min/m2 and PVR 8.9 WU. His functional class improved from WHO FC II to WHO FC I. His Six- Minute Walk Test distance improved from 418 meters to 518 meters and Borg Dyspnea Index from 4 to 2. His symptoms, functional status, exercise performance and echocardiogram have remained stable for more than 14 months post-embolization. Discussion: This is the third documented case of PAVM associated with HPAH due to BMPR2 mutation. Ours is the first report of a successful catheter embolization of a PAVM in a HPAH patient. Our patient had stable hemodynamics with improved functional status after embolotherapy. Careful catheter closure of pulmonary AVMs may be considered in select patients with Pulmonary Arterial Hypertension.
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