1993
DOI: 10.1136/adc.68.1.97
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Pulmonary veno-occlusive disease: diagnosis during life in four patients.

Abstract: Pulmonary veno-occlusive disease is a rare form of primary pulmonary hypertension of unknown aetiology. Four On clinical examination he was a healthy boy without cyanosis who had neither tachycardia nor tachypnoea at rest. He was hypertensive with a blood pressure of 150/80 mm Hg. A right ventricular heave was present. Cardiac auscultation showed an accentuated pulmonary component of the second heart sound but no murmurs, and normal breath sounds were heard on auscultation of his chest. Chest radiography sho… Show more

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Cited by 13 publications
(8 citation statements)
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“…Electrocardiography typically demonstrates right axis deviation and right ventricular hypertrophy. Echocardiography (M-mode, Doppler, and two-dimensional) reveals PAH and helps to exclude an underlying left-sided cardiac structural lesion (such as mitral stenosis or myxoma) or left ventricular dysfunction (7)(8)(9)(10)16,41,42). It is estimated that in 5%-25% of patients with PVOD, their condition is misdiagnosed as idiopathic pulmonary hypertension or pulmonary thromboembolic disease (1,7,8,10,11,41,43).…”
Section: Deceptive Clinical Presentationmentioning
confidence: 99%
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“…Electrocardiography typically demonstrates right axis deviation and right ventricular hypertrophy. Echocardiography (M-mode, Doppler, and two-dimensional) reveals PAH and helps to exclude an underlying left-sided cardiac structural lesion (such as mitral stenosis or myxoma) or left ventricular dysfunction (7)(8)(9)(10)16,41,42). It is estimated that in 5%-25% of patients with PVOD, their condition is misdiagnosed as idiopathic pulmonary hypertension or pulmonary thromboembolic disease (1,7,8,10,11,41,43).…”
Section: Deceptive Clinical Presentationmentioning
confidence: 99%
“…PVOD affects patients with a wide range of ages, from 9 days to 60 -70 years old, but it is chiefly reported in children and young adults (1,6). In the 30%-50% of patients who are less than 20 years of age, there is no sexual predilection, but among adult patients, men are affected twice as frequently as women (5,(7)(8)(9). The duration of illness before death is usually 2 years from initial presentation; pediatric patients tend to experience a more rapid, relentless progression of disease, which leads to rightsided heart failure and death in only a few short months (7)(8)(9)(10)(11).…”
Section: Introductionmentioning
confidence: 99%
“…The differential diagnosis of PH also includes obstructive processes of the intrapulmonary vessels, such as peripheral arterial stenoses, recurrent thromboembolism, stenoses of the terminal preatrial veins, vasculitides, veno-occlusive disease, and others (2,10,14). For evaluation for these conditions, a gradual use of the following investigations is nesessary: thorough laboratory tests for infections, inflammation, coagulation, and immunologieal processes; chest radiography and CT, pulmonary perfusion scintigraphy and angiography (4,10,14). All these investigations were normal in our patient.…”
Section: Discussionmentioning
confidence: 99%
“…Several regimen have been suggested for the treatment of PVOD, with rather inconsistent responses; these included various combinations of digoxin, calcium-channel-blockers and other vasodilatators, cortieosteroids, anticoagulants, oxygen, diuretics and immunosuppressives (2,3,4,10). Our patient received gradually all but the last three medications.…”
Section: Discussionmentioning
confidence: 99%
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