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Ophthalmologic Diagnosis of Exacerbation of Idiopathic Pulmonary Arterial Hypertension Dimitra Skondra MD 1, Harrison W. Farber MD 2, Dean Eliott MD 1 1. Massachusetts Eye & Ear Infirmary, Harvard Medical School, Boston, Massachusetts 2. Pulmonary Hypertension Center, Boston Medical Center, Boston University School of Medicine Introduction Idiopathic pulmonary arterial hypertension (IPAH; formerly known as primary pulmonary hypertension) is a rare condition usually affecting young-middle aged women, in which idiopathic obliteration of pulmonary arterioles results in increased pulmonary artery pressure and pulmonary vascular resistance leading to right heart failure and subsequent elevation in systemic venous pressure (1). Ocular abnormalities have rarely been reported with IPAH including central retinal vein occlusion, open angle glaucoma, choroidal effusion, exudative retinal detachment, serous macular detachment and diffuse macular edema (2-4). Fluorescein angiography showed normal choroidal and retinal perfusion, scattered microaneurysms and areas of mild capillary leakage in the temporal periphery in both eyes (Figure 1D). Spectral domain optical coherence tomography (OCT, Heidelberg) revealed serous macular detachment in the right eye greater than left (Figure 2A). Figure 2 A. OCT demonstrating macular serous detachment in both eyes at presentation B. OCT showing complete resolution of macular detachments seven months after systemic treatment Discussion Here we illustrate a case in which ocular findings were the presenting manifestation of clinically significant deterioration in a patient with IPAH. • IPAH is rarely associated with ocular manifestations (2-4) which are related to elevated episcleral, retinal and choroidal venous pressure secondary to elevated systemic venous pressure. Case report A 28 year-old woman presented with blurred vision and metamorphopsia for five days affecting the right eye more than the left. Past ocular history was unremarkable. Medical history was negative for diabetes and hypertension and was significant for IPAH diagnosed 3 years prior and treated with continuous intravenous infusion of epoprostenol via an indwelling catheter and a portable infusion pump. On examination, best corrected visual acuity was 20/32+2 in the right eye and 20/20-2 in the left eye. On anterior segment examination, bilateral dilated episcleral vessels were noted (Figure 1A). Fundus examination revealed peripapillary myelinated nerve fibers in the right eye and rare, small intraretinal hemorrhages in the temporal periphery bilaterally (Figure 1B, C). Seven months later, visual acuity was improved, she had fewer intraretinal hemorrhages and OCT showed complete resolution of the serous macular detachments in both eyes. Figure 1 A. External photograph demonstrating bilateral dilated episcleral vessels B-C. Montage fundus photographs showing rare, subtle, intraretinal hemorrhages in temporal periphery in both eyes (arrow) D. Fluorescein angiography of right eye showing mild vessel tortuosity, microaneurysms (arrow) and areas of capillary leakage (arrow head) in the temporal periphery Right heart catheterization was performed and revealed increased mean pulmonary artery pressure by 27% compared to the previous measurement 9 months earlier (76mm Hg versus 60mm Hg previously, normal: 12-20mm Hg). The right atrium and right ventricle pressures were also increased by 20% and 33%, respectively. She was diagnosed with severe exacerbation of IPAH and was admitted for aggressive systemic treatment • Peripheral microaneurysms and mild capillary leakage have not been previously described in patients with IPAH. We believe that these abnormalities are precursors of central retinal vein occlusion and are secondary to increased retinal venous pressure causing chronic focal venous stasis, capillary damage, and focal breakdown of the blood-retina barrier. • To our knowledge, this is the first report of ocular findings as the presenting manifestation of clinically significant exacerbation of IPAH warranting aggressive systemic treatment. • Recognition of these subtle findings in our patient lead to early intervention and treatment of severe pulmonary hypertension before the development of more serious sequelae. Ocular manifestations should be recognized by clinicians, and systemic medical evaluation should be considered to prevent life- and vision-threatening complications. References: 1. Farber HW, Loscalzo J. Mechanisms of Disease: Pulmonary arterial hypertension. N Engl J Med. 2004; 351:1655-1665. 2. Akduman L, et al Uveal effusion syndrome associated with primary pulmonary hypertension and vomiting. Am J Ophthalmol. 1996 May; 121(5):578-80. 3. Saran BR, Brucker AJ, Bandello F, Verougstraete C. Familial primary pulmonary hypertension and associated ocular findings. Retina. 2001; 21(1):34-9. 4.Senthil S et al. Secondary open-angle glaucoma and central retinal vein occlusion in a patient with primary pulmonary hypertension. Ophthalmic Surg Lasers Imaging. 2009 Jan-Feb;40(1):50-3. Methods • Single case study • A 28 year-old woman presented with blurred vision and metamorphopsia for five days affecting the right eye more than the left. • Medical history was negative for diabetes and hypertension and was significant for idiopathic pulmonary arterial hypertension (IPAH) diagnosed 3 years prior and treated with continuous intravenous infusion of epoprostenol via an indwelling catheter and a portable infusion pump. Past ocular history was unremarkable. Results • Best corrected visual acuity was 20/32+2 in the right eye and 20/20-2 in the left eye. On anterior segment examination, bilateral dilated episcleral vessels were noted and intraocular pressures were normal. Fundus examination revealed peripapillary myelinated nerve fibers in the right eye and rare, small intraretinal hemorrhages in the temporal periphery bilaterally. • Fluorescein angiography showed normal choroidal and retinal perfusion, scattered microaneurysms and areas of mild capillary leakage in the temporal periphery in both eyes. Spectral domain optical coherence tomography (Heidelberg, OCT) revealed serous macular detachment in the right eye greater than left. B-scan ultrasonography did not show choroidal thickening. • Right heart catheterization was performed and revealed increased mean pulmonary artery pressure by 27% compared to the previous measurement 9 months earlier (76mm Hg versus 60mm Hg previously, normal: 12-20mm Hg). The right atrium and right ventricle pressures were also increased by 20% and 33%, respectively. • The patient was diagnosed with severe exacerbation of pulmonary hypertension and right heart failure and was admitted for aggressive treatment of fluid overload and pulmonary hypertension. Results A. External photograph demonstrating bilateral dilated episcleral vessels B-C. Montage fundus photographs showing rare, subtle, intraretinal hemorrhages in temporal periphery in both eyes (arrow) D. Fluorescein angiography of right eye showing mild vessel tortuosity, microaneurysms (arrow) and areas of capillary leakage (arrow head) in the temporal periphery Results • • Seven months later, her visual symptoms had resolved and visual acuity was 20/20-1 in the right eye and 20/20 in the left eye. She had improved episcleral vessels and fewer intraretinal hemorrhages. OCT showed complete resolution of the serous macular detachments A. OCT demonstrating macular serous detachment in both eyes at presentation B. OCT showing complete resolution of macular detachments seven months after systemic treatment Conclusions • IPAH is a rare condition usually affecting young-middle aged women, in which idiopathic obliteration of pulmonary arterioles results in increased pulmonary artery pressure and pulmonary vascular resistance leading to right heart failure and subsequent elevation in systemic venous pressure (1). • IPAH is rarely associated with ocular manifestations manifestations including central retinal vein occlusion, open angle glaucoma, choroidal effusion, exudative retinal (2-6) which are related to elevated episcleral, retinal and choroidal venous pressure secondary to elevated systemic venous pressure. • Peripheral microaneurysms and mild capillary leakage have not been previously described in patients with IPAH. We believe that these abnormalities are precursors of central retinal vein occlusion and are secondary to increased retinal venous pressure causing chronic focal venous stasis, capillary damage, and focal breakdown of the blood-retina barrier. • To our knowledge, this is the first report of ocular findings as the presenting manifestation of clinically significant exacerbation of IPAH warranting aggressive systemic treatment. • Recognition of these subtle findings in our patient lead to early intervention and treatment of severe pulmonary hypertension before the development of more serious sequelae. Ocular manifestations should be recognized by clinicians, and systemic medical evaluation should be considered to prevent life- and vision-threatening complications. References 1. Farber HW, Loscalzo J. Mechanisms of Disease: Pulmonary arterial hypertension. N Engl J Med. 2004; 351:1655-1665. 2. Akduman L, Del Priore LV, Kaplan HJ, Meredith T. Uveal effusion syndrome associated with primary pulmonary hypertension and vomiting. Am J Ophthalmol. 1996 May; 121(5):578-80. 3. Yang S, Jeong J, Kim JG, Yoon YH. Progressive venous stasis retinopathy and open angle glaucoma associated with primary pulmonary hypertension. Ophthalmic Surg Lasers Imaging. 2006 May-Jun;37(3):230-3. 4. Senthil S, Kaur B, Jalali S, Garudadri C. Secondary open-angle glaucoma and central retinal vein occlusion in a patient with primary pulmonary hypertension. Ophthalmic Surg Lasers Imaging. 2009 Jan-Feb;40(1):50-3. 5. Saran BR, Brucker AJ, Bandello F, Verougstraete C. Familial primary pulmonary hypertension and associated ocular findings. Retina. 2001; 21(1):34-9. 6. Hammond CJ, Chauhan DS, Stanford MS. Pulmonary hypertension and macular edema responsive to acetazolamide. Arch Ophthalmol. 1998 Nov;116(11):1535-6.