Pulmonary veno-occlusive disease in a patient with unilateral absence of right pulmonary artery

A 25-year-old patient had unilateral absence of the right pulmonary artery (UARPA) and severe left pulmonary artery hypertension. After death from congestive right heart failure, autopsy revealed histologic signs of pulmonary veno-occlusive disease (PVOD) and pulmonary hypertension (PH). An accessory arterial vessel that was thrombotically occluded was found connecting the ascending aorta and the right pulmonary hilum. There was also histologic evidence of arterial thrombi within the right lung arterial vascular bed. The PH in UARPA usually occurs very early during the course of disease. From histologic findings and medical history, it is likely that in this case, late-onset elevation of pulmonary pressures was triggered by the occurrence of PVOD. This is the first case of UARPA and PVOD--a congenital unilateral arterial malformation in the presence of bilateral involvement in a possibly acquired venous obliterative disease.     

Sildenafil as adjunct therapy to high-dose epoprostenol in a patient with pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease is refractory to medical treatment and is generally associated with a poor prognosis. Treatment with vasodilators, such as prostacyclin, of patients with PVOD is controversial because of concerns regarding hemodynamic deterioration. Although a preferential pulmonary vasodilatory effect of a specific phosphodiesterase-5 inhibitor, sildenafil, has recently been reported in patients with primary pulmonary hypertension, little information is available regarding the effect of sildenafil on patients with pulmonary veno-occlusive disease. In the present case, remarkable improvement of hemodynamics and of clinical course was produced by adjunctive use of oral sildenafil in association with intravenous high-dose epoprostenol. These findings suggest that sildenafil may be a therapeutic option in the medical treatment of pulmonary veno-occlusive disease.     

Pulmonary veno-occlusive disease as a primary cause of pulmonary hypertension in a patient with mixed connective tissue disease

Mixed connective tissue disease (MCTD) is a systemic disease seen in a group of patients with overlapping clinical features of lupus, scleroderma, polymyositis, and rheumatoid arthritis. A defining feature of MCTD is the presence of antibodies against the U1-ribonucleoprotein (U1-RNP) complex. Pulmonary hypertension is the major cause of death in MCTD. We report an autopsy case of MCTD with pulmonary hypertension. The U1-RNP antibody of this patient was 171.9 U (normal < 25.0 U). The immediate cause of death was attributed to acute pulmonary embolism at left lower lobe. A severe vasculopathy characterized by fibrotic occlusion of small veins and venules, associated with prominent capillary congestion, was consistent with pulmonary veno-occlusive disease (PVOD). This is the first case reported in which PVOD is the primary cause of pulmonary hypertension in MCTD.     

Florid pulmonary veno-occlusive disease

A young woman presented with rapidly progressive dyspnea and clinical findings strongly suggestive of primary pulmonary hypertension or possible pulmonary embolism (or both). She died of acute right-sided heart failure. A diagnosis of pulmonary veno-occlusive disease was made at autopsy. Approximately 100 cases of this disease have been reported previously in the literature. We describe a patient with a particularly florid progression of this unusual disease. Death occurred within six weeks of the onset of symptoms.     

MR imaging findings in a patient with hepatic veno-occlusive disease

We report the MRI findings in a 31-year-old woman with veno-occlusive disease. MRI demonstrated patent hepatic veins and patchy signal enhancement of the liver after gadolinium chelate injection. This enhancement was compatible with sinusoidal congestion. The diagnosis of veno-occlusive disease was confirmed by histological examination of liver biopsy. The diagnosis of veno-occlusive disease should be evoked when patchy liver enhancement suggestive of sinusoidal congestion is observed in the absence of hepatic vein thrombosis and congestive heart failure.     

Pulmonary veno-occlusive disease in a patient with a history of Hashimoto's thyroiditis

Pulmonary veno-occlusive disease (PVOD), a rapidly progressive and fatal disorder, is a rare cause of pulmonary hypertension. We report the occurrence of PVOD in a female patient with Hasimoto's thyroiditis. This report emphasises that PVOD can co-exist with Hashimoto's thyroiditis and a high index of clinical suspicion is required to confirm the diagnosis of PVOD.     

Sarcoidosis simulating pulmonary veno-occlusive disease

We describe a young woman who presented with a 6-month history of progressive pulmonary hypertension. At autopsy she was found to have noncaseating granulomas obliterating pulmonary veins, without granulomatous involvement of pulmonary arteries. Noncaseating granulomas were also present in the liver and hilar lymph nodes, confirming the diagnosis of sarcoidosis. To our knowledge this is the first reported case of pulmonary hypertension caused by sarcoidosis simulating pulmonary veno-occlusive disease.     

Prolonged survival in pulmonary veno-occlusive disease treated with nifedipine

A case of pulmonary veno-occlusive disease diagnosed by open lung biopsy is presented. The patient had a clinical and hemodynamic response to therapy with nifedipine with a significant reduction in pulmonary vascular resistance. This is the first reported case of prolonged survival in this disease resulting from vasodilator therapy with nifedipine.     

肺静脉闭塞病1例

肺静脉闭塞病（pulmonaryvenoocclusivedisease,PVOD）是一种罕见的疾病,由于PVOD和特发性肺动脉高压具有相似的临床表现、遗传背景和血流动力学,有5％～10％的患者被初步考虑为特发性肺动脉高压,PVOD必须通过活检来确定诊断,但因肺活检存在着高风险,因此,一般是通过胸部高分辨率CT（highresolutioncomputedtomography,HRCT）、动脉血气、肺功能和支气管肺泡灌洗等临床诊断PVOD。现将1例通过肺活检病理确诊病例的临床表现、影像学表现、病理结果及相关检查报道如下。     

Pulmonary venous dilatation in pulmonary veno-occlusive disease

A case of pulmonary veno-occlusive disease in an adult is presented. The diagnosis was suspected clinically and verified at cardiac catheterization. Balloon dilatation of the pulmonary veins was attempted at operation and later again in the catheterization laboratory. The result was a significant reduction in pulmonary venous to left atrial pressure gradient and angiographic evidence of increase in venous diameter. It appears that in the focal form of pulmonary venous occlusion, balloon dilatation may offer a therapeutic approach in an otherwise progressively lethal disease.     

肺静脉闭塞病的研究进展

肺动脉高压（pulmonary arterial hypertension,PAH）是由不同病因导致的、以肺动脉压力和肺血管阻力升高为特点的一组综合征。肺静脉闭塞病（Dulmonary venoocclusive disease,PVOD）是公认的PAH比较少见原因之一。     

Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin.

Our ultimate goal in treating patients is to improve their quality of life and to increase survival. The optimal treatment for primary pulmonary hypertension will continue to change as our understanding of its causes improves and as progress is made in lung transplantation. There is no one best treatment for all patients. Optimal medical and surgical treatment must be tailored to the individual with changes in therapeutic regimens based on serial evaluations. Quality of life and survival have improved with current treatments and the future should offer additional therapies-inhaled nitric oxide, endothelin receptor blockers, and other modulators of the pulmonary vascular bed-to improve further the treatment of this disease. In conclusion, although primary pulmonary hypertension, if untreated, is most often a rapidly progressive and fatal disease, recent advances in the treatment have significantly improved the outcome for patients. Although transplantation is often considered the only definitive treatment for patients with primary pulmonary hypertension, medical treatment seems to be an effective long term palliation to successful transplantation as well as a possible alternative treatment to transplantation in selected children and adults. Quality of life and cost analyses, as well as longer follow up studies are needed to determine the best treatment for patients with primary pulmonary hypertension.     

Death due to diffuse alveolar hemorrhage in a child with pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease (PVOD) occurred in a six-year-old girl. Investigation of a number of proposed causes for this uncommon condition and histologic progression of disease are documented. To our knowledge, this is the first case of PVOD in which death was due to massive alveolar hemorrhage.     

Clinical characteristics analysis of pulmonary veno-occlusive disease

<正>Objective To investigate the clinical characteristics of patients with pulmonary veno-occlusive disease(PVOD).Methods A total of 217 patients with pulmonary arterial hypertension(PAH) admitted to Fuwai hospital from 2016 to 2018 were retrospectively collected.     

A case of pulmonary veno-occlusive disease associated with systemic sclerosis

The case of a patient with pulmonary veno-occlusive disease associated with systemic sclerosis is reported. The patient presented with progressive dyspnoea. Echocardiography and cardiac catheterization study demonstrated right-sided heart failure. The CXR suggested pulmonary hypertension and interstitial pulmonary oedema. We suspected pulmonary veno-occlusive disease based on radiological and haemodynamic findings. Treatment with prednisolone resulted in a reduction in pulmonary arterial pressure and CXR findings improved 2 months later, but no further effect was observed. The patient died 7 months later and at autopsy the lungs showed prominent thickening of the interlobular septa and small branches of pulmonary veins showed intimal thickening.     

肺静脉闭塞病患者的临床特点及预后分析

目的 分析肺静脉闭塞病(PVOD)的临床表现、诊断治疗及预后情况.方法 回顾性分析2008年5月至2011年5月间同济大学附属上海市肺科医院临床诊断PVOD患者的临床特点及预后情况.结果 共5例患者被诊断为PVOD,年龄范围为12 ～42(22±12)岁,其中女性4例,男性1例.患者从首发症状至被确诊为PVOD的时间为2～50(16±20)个月,其中4例曾被误诊为特发性肺动脉高压.患者确诊时心功能均显著下降,其中3例为WHO肺高压功能分级Ⅲ级,另2例为Ⅳ级.所有患者胸部CT检查均提示双肺弥漫性毛玻璃样渗出征象,肺功能检查示肺泡弥散功能显著下降,平均肺泡一氧化碳弥散量/预计值为(38±12)％.右心导管测得患者肺动脉压力和肺血管阻力均显著升高.5例患者均接受传统和肺动脉高压靶向药物治疗.随访至今,其中4例患者因难治性右心衰竭死亡,从出现首发症状至死亡及从确诊PVOD至死亡的时间分别为5～65(27±26)个月和1 ～16(9±9)个月.结论 PVOD是罕见的恶性心肺血管疾病,易被误诊,使用现有肺动脉高压靶向治疗药物疗效不佳、预后差,应及早考虑进行肺移植治疗.