Key result
Familial primary pulmonary hypertension links to ocular complications like venous stasis retinopathy and choroidal detachments.
Why the study?
Familial primary pulmonary hypertension is a rare fatal disease causing elevated venous pressure and right heart failure, with unclear associated ocular findings.
Case Report (n=3)
Elevated venous pressure in familial primary pulmonary hypertension can lead to delayed choroidal perfusion, resulting in venous stasis retinopathy and choroidal detachments.
Venous stasis retinopathy or choroidal detachments may signal elevated venous pressure in PPH; leaves open whether routine screening alters outcomes in this rare disease.
BACKGROUND: Familial primary pulmonary hypertension (PPH) is a rare, fatal, autosomal dominant disease that results in right heart failure from idiopathic obliteration of the pulmonary arteries. Patients develop stagnation of venous blood flow and elevated venous pressure. METHODS: The authors retrospectively reviewed the clinical records of three patients diagnosed with PPH that was confirmed on the basis of physical examination, chest X-ray, electrocardiogram, and echocardiogram. Cardiac catheterization excluded cardiac shunt and other secondary causes of pulmonary hypertension. RESULTS: Two patients presented with a clinical picture resembling venous stasis retinopathy, and one with bilateral choroidal detachments. Two patients had delayed choroidal filling on fluorescein angiography, which was confirmed in one patient with indocyanine green videoangiography. CONCLUSIONS: Elevated venous pressure found in PPH is responsible for the delayed choroidal perfusion and the reduced venous blood outflow. This explains the clinical findings of venous stasis retinopathy and choroidal detachments seen in these patients.
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Saran et al. (2001) conducted a case report in Familial primary pulmonary hypertension (PPH) (n=3). Familial primary pulmonary hypertension was evaluated on Ocular findings (venous stasis retinopathy, choroidal detachments, delayed choroidal filling). Familial primary pulmonary hypertension was associated with venous stasis retinopathy (2 patients), bilateral choroidal detachments (1 patient), and delayed choroidal filling (2 patients).
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