CHRONIC OCULAR ISCHEMIA ASSOCIATED WITH PRIMARY PULMONARY HYPERTENSION Harry M. Engel, MD, Thomas L. Slamovits, MD

A 45-year-old man developed macular capillary drop-out and progressive retinal ischemia with neovascular glaucoma well before recognition of his underlying primary pulmonary hypertension.

From the Department of Ophthalmology and Visual Science, The Albert Einstein College of Medicine/ Montefiore Medical Center, Bronx, New York.

lesterol level of 210 mg/dL with a high-density lipoprotein value of 23 mg/dL and a triglyceride level of 383 mg/dL, suggesting a high-risk profile for coronary disease. The leukocyte count, differential blood cell count, and blood smears were normal. The erythrocyte sedimentation rate was 5 mm/h. Results of tests for antinuclear antibodies, anti-DNA antibodies, rapid plasma reagin, titers of antibody to Borrelia burgdorferi, complement, and proteins C and S, screening for hepatitis and anticardiolipin antibodies, and a Raji cell assay were all normal. Findings of serum chemistry screening including glucose levels were all normal. Noninvasive carotid studies (Doppler/duplex ultrasonography) showed no significant flow abnormalities. A heart/lung evaluation was recommended, but the patient did not pursue an assessment. Rubeosis iridis developed in the left eye 2 months later with subsequent neovascular glaucoma despite panretinal photocoagulation. The right eye had no additional retinopathy or neovascularization. One year later, the patient was hospitalized for an acute cyanotic syncopal episode. He related progressive exercise intolerance with respiratory distress after walking three to four blocks over the year. An electrocardiogram revealed a right ventricular strain pattern. Cardiac catheterization demonstrated pulmonary artery pressure of 118/48 mmHg and normal left ventricular function. With a diagnosis of primary pulmonary hypertension, he died of right heart failure while awaiting a lung transplant. Autopsy findings confirmed right atrial and ventricular dilatation. Mild left ventricular hypertrophy and mild atherosclerosis of the coronary vessels were also noted. The mitral and tricuspid valves had no disease; no congenital cardiac defects were present. Passive congestion was found in the liver and spleen. There was no evidence of vasculitis or embolic or carotid disease.

Case Report A 45-year-old man had blurred vision in his left eye after a flu–like illness. An ophthalmologist detected cotton-wool spots in his left retina and suspected retinal vasculitis. He was referred to our department after 4 months of persistent visual disturbance. He had a history of labile hypertension and was receiving theophylline treatment for mild asthma. He denied shortness of breath except for a recent 2-week episode of bronchitis. On his own initiative, he had been taking a baby aspirin daily for several years. He said that results of recent electrocardiography and chest roentgenography were normal. He was a robust appearing man. Visual acuity was 20/25 in the right eye and 20/40 in the left eye. A 1⫹ afferent defect of the left pupil was noted. Ocular motility, slit-lamp examination results, and orbital findings were normal. Intraocular pressure was 12 mmHg in both eyes. Ophthalmoscopy of the right eye revealed marked arteriolar narrowing and several scattered cotton-wool spots in the posterior pole. In the left eye, numerous small dot retinal hemorrhages were noted, particularly in the periphery. Neither eye had advanced arteriosclerosis or retinal thickening. Fluorescein angiography confirmed extensive capillary occlusion and microvascular disease in the posterior pole of both eyes (Fig. 1.) The retinal microvascular alterations (especially the midperipheral hemorrhages and microaneurysms) suggested an ocular ischemic syndrome. A systemic evaluation was recommended for possible carotid artery disease, an autoimmune process, or a microvascular disorder. Blood pressure measured 150/130 mmHg, which fell to 120/90 mmHg after a brief rest. Heart rate was 92 beats/min and regular. There was no heart murmur. Slight hepatomegaly was noted. Despite a high hematocrit of 49.5%, the polycythemia was reported as factitious because of decreased plasma volume. The hypertension was deemed labile. Notable laboratory findings were a cho-

Discussion This case is noteworthy in that retinal ischemia and neovascular glaucoma developed well before discovery of the underlying pulmonary hypertension and cardiac disease. The patient, a mild asthmatic, did not complain of more labored respirations on numerous follow-up visits to our ophthalmologists and to a specialist in hematology. The insidious onset of dyspnea, the predominant presenting symptom in primary pul-

Supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York, NY. The authors have no proprietary interests. Reprint requests: Harry M. Engel, MD, Department of Ophthalmology, Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467; e-mail: [email protected]

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Fig. 1. Fluorescein angiography of the left macula in the early phase (A) and in the venous phase (B) confirmed widespread retinal vascular occlusion and capillary bed damage. The microcirculation shows extensive capillary irregularity, tortuosity, and dropout above and below the horizontal raphe. Hemorrhages, exudates and edema, and a pattern of distribution characteristic of acute branch venous occlusive disease are absent.

monary hypertension in an otherwise healthy patient, is well known. Dyspnea develops secondary to cor pulmonale, which generally is the cause of death. Fatigue, angina, and syncope are also common symptoms. In early pulmonary hypertension, cardiac output is relatively normal. Over time, cardiac output becomes progressively reduced with resultant hypoxemia and signs of peripheral venous congestion and edema. The average period from the onset of symptoms to diagnosis of primary pulmonary hypertension is 2.5 years.1 Primary pulmonary hypertension can occur at all ages, although it is most common in young women in their 20s and 30s. The cause and pathogenesis remain unclear and are unrelated to asthma and hypertension. Three histologic subtypes have been identified (plexogenic, thrombotic, and venoocclusive); however, none of the histopathologic patterns are pathognomonic. (The autopsy revealed no particular histologic subtype in this case.) Labile hypertension is not a recognized feature of pulmonary hypertension. Secondary causes of chronic pulmonary hypertension include congenital and valvular heart disease, primary myocardial disease, obstructive, interstitial, and granulomatous lung disease, intravenous drug abuse, parasitic disease of the lung, sickle cell anemia, and chronic liver disease.2 Asthma is not a known cause of pulmonary hypertension and possibly obscured the patient’s own recognition of his diminishing exercise tolerance. Labile hypertension is not a recognized feature of pulmonary hypertension. Chronic ocular ischemia associated with pulmonary hypertension induces venous stasis, peripheral microvascular disease (microaneurysms, multiple small blot hemorrhages, and capillary dilation), and rubeosis iridis.3 These alterations are also typical of the early vasculopathy recognized in carotid occlusive disease.4 Recent reports describing ocular disease in primary pulmonary hypertension have underscored the devel-

opment of central retinal vein occlusion and choroidal detachments.5,6 Polycythemia, secondary to the depressed pulmonary function in pulmonary hypertension, may contribute to retinal vascular occlusion. In this case, the elevated hematocrit was attributed to volume contraction rather than elevated erythrocyte mass. Speculatively, this was stress related, as was his hypertension. Spurious polycythemia may be seen in many smokers and in moderate hypertension. The failure to consider the patient’s erythrocytosis as being related to systemic hypoxia underscores the insidious onset of cor pulmonale. The presence of hepatomegaly possibly represented early peripheral venous congestion. It is plausible that chronic hypoxia secondary to pulmonary hypertension and intensified by episodes of asthma and hypertension-induced vascular spasm combined to damage the retinal capillary bed. To our knowledge, early extensive nonexudative disruption of posterior retinal capillaries has not been previously described in pulmonary hypertension, but it is consistent with chronic ocular ischemia, as in carotid insufficiency. Acute and chronic hypertension may induce multifocal posterior retinal arteriolar spasm and injury, with characteristic cotton-wool spots and retinal whitening. Multiple and contiguous branch retinal vein occlusions may produce widespread disruption of macular and peripheral capillaries. Posterior retinal microvascular disease is rarely associated with vascular disease in other organ systems in the absence of systemic disease or vasculitis.7 Idiopathic recurrent branch artery occlusion is uncommon. Subtle cardiac defects including patent foramen ovale and interatrial septal defects may contribute to branch arteriole occlusions in individuals predisposed to coagulation abnormalities. The posterior retina is particularly vulnerable to microemboli, because its arterioles and capillaries do not anastomose. Cardiopulmonary disease must be strongly considered in

CHRONIC OCULAR ISCHEMIA ASSOCIATED WITH PRIMARY PULMONARY HYPERTENSION

patients with unexplained progressive central or peripheral injury to the retinal capillary bed. Had it been heeded, our recommendation to the patient to undergo a thorough cardiac evaluation would have led to an earlier diagnosis. Medical treatment of primary pulmonary hypertension is generally ineffective. Lung or heart–lung transplantation is the accepted therapy. Key words: cor pulmonale, Eisenmenger syndrome, ocular ischemia, primary pulmonary hypertension, retinal ischemia. References 1.

Rich S. Primary pulmonary hypertension: a national prospective study. Ann Intern Med 1987;107:216–223.

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Rich S. Primary pulmonary hypertension. Prog Cardiovasc Dis 1988;31:205–238. Harino S, Motokura M, Nishikawa N, et al. Chronic ocular ischemia associated with the Eisenmenger’s syndrome. Am J Ophthalmol 1994;117:302–307. Michelson PE, Knox DL, Green WR. Ischemic ocular inflammation: a clinical pathologic case report. Arch Ophthalmol 1971;86:274–280. Rodriquez N, Elliot D. Bilateral central retinal vein occlusion in Eisenmenger syndrome. Am J Ophthalmol 2001;132:268– 269. Saran BR, Brucker AJ, Bandello F, et al. Familial primary pulmonary hypertension and associated ocular findings. Retina 2001;21:34–39. Grand MG, Kaine J, Fulling K, et al. Cerebroretinal vasculopathy, a new hereditary syndrome. Ophthalmology 1988;95: 649–659.

Chronic ocular ischemia associated with primary pulmonary hypertension.

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