Pulmonary veno-occlusive disease (PVOD) is a rare still life-threatening disease.

Pulmonary veno-occlusive disease (PVOD) is a rare still life-threatening disease. Although prostacyclin (PG[I.sub.2]) attenuates pulmonary hypertension and improves the prognosis in patients with primary pulmonary hypertension, little information is available regarding the result of PG[I.sub.2] on patients with PVOD This report describes a patient with austere PVOD who showed marked improvement in exercise capacity and pulmonary hemodynamics with continuous IV PG[I.sub.2] treatment. Furthermore, he experienced no clinical ends for 12 months and survived for 25 month after the initiation of PG[I.sub.2] therapy. These proceeds suggest that continuous IV PG[I.sub.2] therapy may help as a bridge to transplantation in a certain quantity of cases of PVOD.

first note of the scale words: prostacyclin; pulmonary veno-occlusive disease

Abbreviations: PG[I.sub.2] = prostacyclin; PPH = primary pulmonary hypertension; PVOD = pulmonary veno-occlusive disease

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Pulmonary veno-occlusive disease (PVOD) describes the disease in a subset of patients with pulmonary hypertension histologically characterized through fibrotic occlusion of the smaller pulmonary veins. PVOD is an unwonted form of unexplained pulmonary hypertension that is rarely diagnosed during life and is generally associated with a progressively deteriorating course. In greatest in quantity cases, PVOD is refractory to medical treatment and patients require transplantation, although occasional instances of favorable rejoinders to some agents have been reported. (12) Continuous IV administration of prostacyclin (PG[I.sub.2]) has been established as a treatment for primary pulmonary hypertension (PPH) (34) However, the meaning of PG[I.sub.2] on PVOD remains unclear. We report upon a patient with PVOD who showed marked improvement in exercise capacity, pulmonary hemodynamics, and probably prognosis after PG[I.sub.2] therapy was initiated.

CASE REPORT

A 30-year-old man with of the present day York Heart Association functional class IV was hospitalized with chief complaints of elision and dyspnea, which had begun in November 1998 He had no medical or family history of note. He had smok the same pack of cigarettes per day for 10 years. He was noted to have jugular vein dilatation, a boisterous pulmonary component of S2, an S3 gallop, right ventricular lift, and hepatomegaly. Arterial vital current gas analysis (room air) revealed a pH of 745 P[Osub2] of 660 mm Hg and PC[Osub2] of 267 mm Hg The ECG showed right ventricular hypertrophy The chest radiograph revealed prominent pulmonary arteries and interstitial shadows in the right lower lung field, indicating pulmonary congestion. The springs of a workup for secondary causes of pulmonary hypertension were normal. Pulmonary function exhibitions showed reduced diffusing capacity of the lung for carbon monoxide (400% of predicted). Ventilation-perfusion images revealed normal ventilation and diffuse focal areas of hypoperfusion. Right heart catheterization revealed plain pulmonary arterial hypertension (mean pulmonary arterial squeezing 70 mm Hg; pulmonary vascular resistance, 151 wood-land U; mean right atrial urgency 17 mm Hg; pulmonary capillary wedge urgency 10 mm Hg; cardiac output 397 L/min). Ischemic heart disease, valvular disease, and cardiomyopathy were exclud at echocardiography and cardiac catheterization. Based onward these findings and the nearness of pulmonary congestion, PVOD, a subtype of PPH was violently suspected. Continuous IV PG[I.sub.2] was immediately begun at 1 ng/kg/min and was progressively titrated through 10 ng/kg/min in a week, because the patient's general condition deteriorated critically. Thereafter, the PG[I.sub.2] dosage was gradually increased by means of 1 ng/kg/min per week. brace episodes of pulmonary edema occurr during follow-up However, the patient's symptoms and chest radiographic findings were significantly improved when the dose of PG[I.sub.2] was titrated to 29 ng/kg/min. Furthermore, his 6-min walk distance increased from 90 to 450 m and his mean pulmonary artery crushing decreased from 70 to 36 mm Hg He was discharged from the hospital about 4 month after the start of PG[I.sub.2] administration.

Although the patient go [i]or[/i] come backed to work and was intrust with an agencyed in desk work, he was rehospitalized about 12 month after discharge. His general condition worsened despite intensive care. Finally, his respiratory state deteriorated, and marked interstitial shadows appeared in the chest radiograph and CT scan (Fig 1) We tried several agents, as it is as prednisolone, dobutamine, diuretics, and nitric oxide inhalation, still the patient died of respiratory failure about 2 years after the initiation of continuous IV PG[I.sub.2].

[FIGURE 1 OMITTED]

At autopsy, gros examination of the lung revealed accurate pulmonary edema. Histologic sections from the lung demonstrated the typical changes of PVOD Diffuse stenosis of the pulmonary veins and venule by way of fibrous tissue, thickened media of the veins, and interstitial edema accrueed in a thickening of the lobular septa, engorged and tortuous alveolar capillaries, and hemosiderosis in each section. Pulmonary arterioles exhibited hard medial hypertrophy, but plexiform lesions were absent (Fig 2) The final clinical diagnosis of PVOD was made from these autopsy findings.

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