Vasoresponsiveness of Sarcoidosis-Associated Pulmonary Hypertension

Preston, Ioana R.; Klinger, James R.; Landzberg, Michael J.; Houtchens, Jeanne; Nelson, David G.; Hill, Nicholas S.

doi:10.1378/chest.120.3.866

Cited by 116 publications

(109 citation statements)

References 21 publications

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“…Diverse pathologic processes in- cluding fibrotic destruction of the pulmonary parenchyma, vascular remodeling related to chronic hypoxia, and intravascular or extravascular granuloma invasion or compression of pulmonary arteries likely contribute to a final common clinical presentation of PH. The absence of expected radiographic and pathologic findings in some patients with sarcoidosis and PH have led several authors 16,25 to propose alternative mechanisms such as a pulmonary vasculopathy characterized by endothelial dysfunction. An imbalance in vasoconstrictor and vasodilatory mediators among patients with sarcoidosis and PH has also been shown.…”

Section: Discussionmentioning

confidence: 99%

“…Fisher et al 20 observed an average reduction in PVR of 45% in response to short-term epoprostenol administration among seven patients. Preston and coworkers 25 described eight patients with radiographic stage III or IV disease, four of whom had an acute Ն 20% decrease in MPAP with the use of inhaled nitric oxide. Five patients receiving long-term therapy (2 to 6 months) with pulse inhaled nitric oxide had improvements in the 6MWD, and three patients had improvements in their NYHA functional class.…”

Section: Discussionmentioning

confidence: 99%

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Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Barnett

Bonura

Nathan

et al. 2009

Chest

145

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Background: Pulmonary hypertension (PH) is a common complication of sarcoidosis that is associated with increased mortality. The pathogenesis of PH in sarcoidosis is uncertain, and the role of pulmonary arterial hypertension (PAH)-specific therapies remains to be determined. Methods: We conducted a retrospective study of patients with sarcoidosis and PH at two referral centers. New York Heart Association (NYHA) functional class, exercise capacity, hemodynamic data, pulmonary function tests, and survival were collected and analyzed. Results: Twenty-two sarcoidosis patients treated with PAH-specific therapies were identified. After a median of 11 months of follow-up, NYHA class was improved in nine subjects. Mean 6-min walk distance (n ‫؍‬ 18) increased by 59 m (p ‫؍‬ 0.032). Patients with a higher FVC experienced a greater increment in exercise capacity. Among 12 patients with follow-up hemodynamic data, mean pulmonary artery pressure was reduced from 48.5 ؎ 4.3 to 39.4 ؎ 2.8 mm Hg (p ‫؍‬ 0.008). The 1-and 3-year transplant-free survival rates were 90% and 74%, respectively. Conclusions: PAH-specific therapy may improve functional class, exercise capacity, and hemodynamics in PH associated with sarcoidosis. Prospective, controlled trials of PAH therapies for sarcoidosis are warranted to verify this apparent benefit. Mortality among the study population was high, highlighting the need for urgent evaluation at a lung transplant center.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Barnett

Bonura

Nathan

et al. 2009

Chest

145

View full text Add to dashboard Cite

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“…Preston et al [21] 2001 Pulmonary hypertension, even if not severe in most cases, increases mortality in sarcoidosis [5,6]. Our patient survived 6 years from diagnosis of SAPH.…”

mentioning

confidence: 56%

Pulmonary Function Tests Leading to the Diagnosis of Vascular Malformations in School-Aged Children

Kusak

Cichocka‐Jarosz

Jedynak-Wąsowicz

et al. 2017

Advances in Respiratory Medicine

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Development of sarcoidosis-associated pulmonary hypertension (SAPH) significantly worsens prognosis in sarcoidosis patients. Unfortunately, there is no treatment of proven benefit for this condition. Medications used for treatment of pulmonary arterial hypertension are of great interest in this respect. Here, we report a case of a patient with severe SAPH treated with sildenafil. A significant, but only temporary improvement in functional status was observed, and the patient died of gradually progressing heart and respiratory failure while awaiting for lung transplantation.

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“…PH can be due to impaired forward flow because of poor left ventricular function and can result from PS in patients with hypoxic vasoconstriction leading to cor pulmonale (1). PH can be caused by encroachment of the pulmonary vasculature due to intimal and medial infiltration by noncaseating granuloma and extrinsic compression of pulmonary arteries by enlarged mediastinal lymph nodes (39). PH is diagnosed based on an estimation of right ventricular systolic pressure (RVSP) using Doppler echocardiography and a modified Bernoulli the management of CS (2).…”

Section: Diagnosismentioning

confidence: 99%