Download Introduction Case report Ophthalmologic Diagnosis of Exacerbation

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.