Figure 1. Clinical photograph, fundus photographs, and fluorescein angiogram. A, External photograph demonstrating bilateral dilated episcleral vessels. B, Montage fundus photograph of the right eye showing rare, subtle, scattered, temporal, peripheral, intraretinal hemorrhages (arrows). C, Montage fundus photograph of the left eye showing a temporal, peripheral, intraretinal hemorrhage (arrow). D, Fluorescein angiogram of the right eye showing mild vessel tortuosity, microaneurysms (arrows), and areas of capillary leakage (arrowhead) in the temporal periphery.
Figure 2. Spectral-domain optical coherence tomographic images. OD indicates right eye; OS, left eye. A, Serous macular detachment in both eyes at the initial visit. B, Complete resolution of serous macular detachments 7 months after systemic treatment.
Skondra D, Chang GC, Farber HW, Eliott D. Ophthalmologic Diagnosis of Exacerbation of Idiopathic Pulmonary Arterial Hypertension. Arch Ophthalmol. 2012;130(12):1619-1621. doi:10.1001/archophthalmol.2012.1802
Author Affiliations: Retina Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School (Drs Skondra, Chang, and Eliott) and Pulmonary Hypertension Center, Boston Medical Center, Boston University School of Medicine (Dr Farber), Boston.
Idiopathic pulmonary arterial hypertension (IPAH; formerly known as primary pulmonary hypertension) is a rare condition usually affecting young to middle-aged women in whom 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.2- 6 Herein, we illustrate a case in which ocular findings were the initial manifestation of clinically significant deterioration in a patient with IPAH.
A 28-year-old woman had blurred vision and metamorphopsia for 5 days, affecting the right eye more than the left. Her ocular history was unremarkable. Her 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 OD and 20/20 − 2 OS. On anterior segment examination, bilateral dilated episcleral vessels were noted and intraocular pressures were normal (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 and C). 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 (Heidelberg Engineering) revealed serous macular detachment in the right eye greater than in the left (Figure 2A). B-scan ultrasonography did not show choroidal thickening.
Given the recent onset of ocular symptoms and findings that were consistent with elevated systemic venous pressure indicating worsening of IPAH, a right heart catheterization was performed and revealed that mean pulmonary artery pressure had increased by 27% compared with the previous measurement 9 months earlier (76 vs 60 mm Hg, respectively; reference range, 12-20 mm Hg). The right atrium and right ventricle pressures were also increased by 20% and 33%, respectively. The patient was diagnosed as having a severe exacerbation of IPAH and right heart failure, and she was admitted for aggressive treatment of fluid overload and IPAH.
Seven months later, her visual symptoms had resolved and visual acuity was 20/20 − 1 OD and 20/20 OS. She had improved episcleral vessels and fewer intraretinal hemorrhages. Spectral-domain optical coherence tomography showed complete resolution of the serous macular detachments (Figure 2B).
We report an unusual case of bilateral retinal microvascular abnormalities and serous macular detachment as the manifesting signs of exacerbation of IPAH. Idiopathic pulmonary arterial hypertension is rarely associated with ocular manifestations including central retinal vein occlusion, open-angle glaucoma, choroidal effusion, exudative retinal detachment, serous macular detachment, and diffuse macular edema.2- 6 These manifestations are related to elevated episcleral, retinal, and choroidal venous pressure secondary to elevated systemic venous pressure.
Peripheral microaneurysms and mild capillary leakage with normal venous filling on fluorescein angiography 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 initial manifestation of clinically significant exacerbation of IPAH warranting aggressive systemic treatment. Recognition of these subtle findings in our patient led to early intervention and treatment of severe IPAH 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.
Correspondence: Dr Eliott, Retina Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St, Boston, MA 02114 (firstname.lastname@example.org).
Conflict of Interest Disclosures: None reported.