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Professor Zhang Jingfa: A case of primary pulmonary hypertension with plasma-like fundus changes in both eyes

Posting time:2022-10-06 01:47:30

Professor Zhang Jingfa: A case of primary pulmonary hypertension with plasma-like fundus changes in both eyes

Editor's note: Primary pulmonary arterial hypertension (PAH) is a persistent increase in pulmonary arterial pressure caused by an unexplained increase in pulmonary vascular resistance. In severe cases, it may lead to heart failure or even death. Ocular complications secondary to pulmonary hypertension are less reported. Recently, Professor Zhang Jingfa from the Department of Ophthalmology of Shanghai First People's Hospital published a case report entitled "Bilateral central serous chorioretinopathy-like abnormalities in a man with pulmonary arterial hypertension". This report describes a patient with PAH who developed bilateral visual acuity loss with visual distortion to central serous chorioretinopathy (CSCR)-like abnormalities and other ocular lesions, published in Frontiers in Medicine (IF 5.058). Case introduction Prof. Zhang shared a case of CSCR secondary to insufficient PAH treatment. The patient was a 45-year-old male patient with PAH who had been treated with low-dose sildenafil (2.5 mg, bid) for 6 years and developed bilateral Decreased visual acuity and visual distortion, ophthalmological examination showed CSCR-like abnormalities in both eyes and atrophy of the optic disc in the right eye. Sildenafil, a phosphodiesterase type 5 inhibitor, is a potent vasodilator that has been used to treat erectile dysfunction and PAH. Phosphodiesterase type 5 is present in choroidal and retinal vessels, and sildenafil inhibition may increase choroidal blood flow, resulting in retinal and choroidal vasodilation. The usual dose of sildenafil for the treatment of PAH is 20-80 mg/day, which is generally well tolerated. The patient visited the ophthalmology department this time, and the examination results were as follows: binocular visual acuity: right eye index/10cm, left eye 0.12; right eye corrected -3.5D without response, left eye -1D corrected to 0.15. Intraocular pressure: 19.9mmHg in the right eye and 17.4mmHg in the left eye. Eye axis: right eye 23.49mm, left eye 22.46mm. Slit-lamp examination: scleral vessels and conjunctival vessels in both eyes were markedly distended and tortuous. The anterior chamber depth was 2.39 mm in the right eye and 2.21 mm in the left eye, and the chamber angle was open. Color photo of the fundus of both eyes: a large number of speckle-like lesions in the macular area and the optic disc of the right eye can be seen. OCTA: Shows chronic CSCR-like abnormalities in both eyes, intraretinal cystoid edema and subretinal fluid accumulation, and choroidal thickening. OCT of the right eye: showed obvious optic atrophy, the thickness of the nerve fiber layer was significantly reduced, and the cup-to-disc ratio was increased. The visual field of the right eye showed a marked near-total loss of visual field defect. FA and ICGA: Both results showed delayed retinal and choroidal vascular perfusion, slow blood flow, choroidal vasodilation, tortuosity, hyperemia, and more pronounced vortex veins. Based on the patient's medical history and examination results, it was presumed that the patient had venous stasis retinopathy and choroidopathy caused by insufficient long-term low-dose sildenafil treatment, showing CSCR-like changes. In view of this, Prof. Zhang suggested that patients receive intensive PAH treatment and follow up closely for ocular complications. After 6 weeks, the patient's self-reported visual acuity improved, with a correction of 0.3 in the left eye and an index/10cm in the right eye. OCTA of both eyes showed significant reduction in macular and subretinal fluid absorption. Visual field examination revealed an improvement in the visual field of the left eye as well. Case reports suggest that PAH can lead to increased pulmonary vascular resistance, right heart failure, and death if left untreated or undertreated. Choroidal hyperemia, ciliary body detachment, central retinal vein occlusion, acute serous retinal detachment, CSCR-like lesions, macular edema, retinal neovascularization, choroidal effusion due to elevated venous pressure in the superior vena cava and ophthalmic vein in PAH patients , angle-closure glaucoma, transient myopia and other ocular complications. Patients with PAH should be closely followed and treated, and patients should be educated about the risk of potential ocular adverse effects when undertreated. The author introduces Professor Zhang Jingfa, deputy director of Shanghai Institute of Ophthalmology. Researcher, Deputy Chief Physician, Doctoral Supervisor, Postdoctoral Fellow of Harvard Medical School, Shanghai Pujiang Talent. Deputy Director (and Secretary) of Basic Research and Clinical Translation of the Fifth Committee of the Ophthalmologist Branch of the Chinese Medical Doctor Association, Deputy Director of the Youth Committee of the Ophthalmology Branch of the Chinese Geriatrics Association, and member of the Standing Committee of the Precision Medicine Professional Committee of the Shanghai Biomedical Industry Association. The clinical and scientific research direction is the pathogenesis and treatment of fundus diseases, focusing on diabetic retinopathy and age-related macular degeneration. Professor Zhou Xinrong is the chief physician of the Department of Ophthalmology, First People's Hospital Affiliated to Shanghai Jiaotong University, and a master tutor. Postdoctoral fellow at Johns Hopkins University Eye Institute, visiting scholar at University of California, San Diego Eye Institute. Member of the Evidence-Based Medicine Group of the Ophthalmology Branch of the Chinese Medical Association, engaged in basic and clinical research on glaucoma, optic nerve protection and genetics. Specializes in the diagnosis and treatment of various complex glaucoma and cataract.

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