Prolong Survival in Pulmonary Veno-...

Prolong Survival in Pulmonary Veno-Occlusive Disease Treated with Nifedipine (*1)

Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension characterized by the agency of obstruction of pulmonary venules and veins. [1] Since Hora described the first case in 1934 70 cases have been documented in detail. [1-6] There are merely two reported cases of this disease in which there was apparent rejoinder to therapy. [5,6] All however four patients have died within couple years from the appearance of the first symptoms. [1] We report a case of pulmonary veno-occlusive disease responding to treatment with nifedipine.

CASE REPORT

A 43-year-old woman existinged with a two-year history of increasing fatigue and progressive dyspnea onward exertion. She had no chest pain or cough She had a 20 pack-year history of cigarette smoking. There was jugular venous distension to the sternocleidomastoid muscle at 30[degree] There were crackles auscultated in the two lung bases. There was a obstreperous right ventricular [S.sub.4] gallop and [Psub2] was accentuated. A malleable systolic pulmonic murmur was auscultated. There was trace peripheral edema.

The electrocardiogram showed right axis deviation, left atrial enlargement, and right ventricular hypertrophy Echocardiogram revealed marked right ventricular enlargement and paradoxic septal motion compatible with right ventricular contortion overload. The left ventricle was of normal size with normal contractility. Chest radiograph is shown in Figure 1 and demonstrates bilateral pleural effusions, interstitial edema and enlarged cardiac silhouette.

Arterial family gas levels on room air showed: pH 754; [PaO.sub.2.], 31 mm Hg; and [PaCO.sub.2], 32 mm Hg Pulmonary function studies revealed a forced vital capacity (FVC) of 160 L (46 percent of predicted), forced expiratory contortion in one second ([FEV.sub.1]) of 135 L (50 percent of predicted), [FEVsub1/FVC] ratio of 84 percent and total lung capacity of 286 L (57 percent of predicted).

conclusions of complete blood count were normal. posterity chemistry results were all normal object for elevated lactate dehydrogenase of 917 [mu]kat/L. The erythrocyte sedimentation rate was elevated at 91 mm/h Antinuclear antibody and rheumatoid factor serology were negative.

A nuclear medicine perfusion lung scan showed normal perfusion with no wants Bilateral lower extremity venograms gave normal findings. Fiberoptic bronchoscopy failed to reveal any abnormality.

Right and left heart catheterization was performed. Mean right atrial press was 2 mm Hg, right ventricular urgency was 72/30 mm Hg, mean pulmonary artery press was 44 mm Hg, and pulmonary capillary wedge press was 3 mm Hg. Aortic hurry was 130/84 mm Hg, mean arterial constraining force was 100 mm Hg and cardiac output was 35 L/min. Pulmonary vascular resistance was calculated to be 937 dyne [cmsup-5] Left ventricular size and function were normal. Coronary angiography showed no significant lesions.

lay open lung biopsy of the right lower lobe was performed and a 4 cm wedge biopsy was obtained. The carve surface was homogeneous and r to yellow-tan. Microscopic examination revealed partial to total occlusion of intralobar veins and venule from myxoid fibrous proliferation (Fig 2) Recanalized thrombi were identified in a certain of the large veins. The pulmonary arteries and arterioles demonstrated moderate medial hypertrophy and intimal hyperplasia. The medial thickness was 7 percent of the external diameter and the intimal thickness was 20 percent of the internal diameter of the involved pulmonary arteries. Additionally, patchy interstitial fibrosis and numerous hemosiderin-laden alveolar macrophages were noted.

The diagnosis of pulmonary veno-occlusive disease was made. The patient was treated with supplemental oxygen diuretics and nifedipine 10 mg orally each 8 hours. The patient improved clinically and maintained her systemic offspring pressure at 130/70 mm Hg The patient was followed closely in the pulmonary clinic and refused repeat right heart catheterization until brace years after the nifedipine was started. At that time, the patient was clinically stable and she was studied to reevaluate her hemodynamics onward nifedipine. The repeat right heart catheterization revealed a mean right atrial constraining force of 2 mm Hg, right ventricular compressing of 60/4 mm Hg, pulmonary artery squeezing of 60/20 mm Hg, pulmonary artery mean hurry of 33 mm Hg, pulmonary capillary wedge constraining force of 2 mm Hg, and cardiac output of 40 L/min. The pulmonary vascular resistance was calculated at 620 [dynes*cmsup-5] The systemic relations pressure was 130/70 mm Hg with a mean arterial crushing of 90 mm Hg.

The patient was continued onward therapy with nifedipine and is clinically stable from one side of to the other four years after the diagnosis of pulmonary veno-occlusive disease was made and through the whole extent of six years after the attack of symptoms.

DISCUSSION

The etiology of pulmonary veno-occlusive disease is unknown. Clotting disorders initiating pulmonary venous thrombosis was felt to play a major character but treatment with anticoagulants has been fruitless [1] Viral and granulomatous infections have been propos on some investigators. [1] Wagenvoort et al [3] suspect multiple noxious agents may induce this condition. Eight cases have been reported following chemotherapy for malignant neoplasms. [4] brace cases have been reported with features of a collagen vascular disease and appeared to be agreeable to to treatment with corticosteroids and azathioprine. [56] This disease may actually be a syndrome of multiple causes rather than a single disease entity. [2] Regardless of etiology, the disease is fatal in the majority of patients within sum of two units years of the onset of symptoms appropriate to severe, progressive pulmonary hypertension with right ventricular failure. [1]