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Twelve-Year Survival in a Patient With Systemic Sclerosis-Associated Pulmonary Arterial Hypertension on Nifedipine Monotherapy.

التفاصيل البيبلوغرافية
العنوان: Twelve-Year Survival in a Patient With Systemic Sclerosis-Associated Pulmonary Arterial Hypertension on Nifedipine Monotherapy.
المؤلفون: Helgeson SA; Department of Pulmonary and Critical Care Medicine, Mayo Clinic, Jacksonville, FL., Enderby CY; Department of Pharmacy, Mayo Clinic, Jacksonville, FL., Moss JE; Department of Pulmonary and Critical Care Medicine, Mayo Clinic, Jacksonville, FL., Gass JM; Department of Laboratory Genetics and Genomics, Greenwood Genetic Center, SC., Zeiger TK; Department of Pulmonary Medicine, Mayo Clinic, Jacksonville, FL., Burger CD; Department of Pulmonary and Critical Care Medicine, Mayo Clinic, Jacksonville, FL.
المصدر: Mayo Clinic proceedings. Innovations, quality & outcomes [Mayo Clin Proc Innov Qual Outcomes] 2019 Jul 19; Vol. 3 (3), pp. 376-379. Date of Electronic Publication: 2019 Jul 19 (Print Publication: 2019).
نوع المنشور: Case Reports
اللغة: English
بيانات الدورية: Publisher: Elsevier Inc Country of Publication: Netherlands NLM ID: 101728275 Publication Model: eCollection Cited Medium: Internet ISSN: 2542-4548 (Electronic) Linking ISSN: 25424548 NLM ISO Abbreviation: Mayo Clin Proc Innov Qual Outcomes Subsets: PubMed not MEDLINE
أسماء مطبوعة: Original Publication: [Netherlands] : Elsevier Inc., [2017]-
مستخلص: Pulmonary arterial hypertension is a progressive vascular disease with a high mortality rate without proper therapy. Identification of the appropriate treatment for each patient is critical in regard to adverse effects, health care costs, ease of treatment, and the potential for prognostication. Treatment strategies typically begin with acute vasoreactivity testing, which is performed during a right heart catherization. If positive, a calcium channel blocker may work; however, another pulmonary arterial hypertension-specific medication is necessary when testing is negative. Acute vasoreactivity testing is currently recommended to be performed only in certain subgroups of pulmonary arterial hypertension, but not when related to connective tissue disease. In this report, we describe a patient who had systemic sclerosis-related pulmonary arterial hypertension with a positive acute vasoreactivity test result. The patient was placed on calcium channel blocker monotherapy that has been well tolerated for 12 years, resulting in improved symptoms and exercise capacity. The long-term response to calcium channel blocker therapy in systemic sclerosis-associated pulmonary arterial hypertension has not been previously described. In addition, pulmonary artery pressures have been well controlled. The absence of genetic smooth muscle variants prevalent in vasoresponsive idiopathic pulmonary arterial hypertension is also unique.
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فهرسة مساهمة: Keywords: AVT, acute vasoreactivity testing; CCB, calcium channel blocker; CREST, calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia; CTD, connective tissue disease; PAH, pulmonary arterial hypertension; PAOP, pulmonary arterial occlusion pressure; PVR, pulmonary vascular resistance; RHC, right heart catherization; SSc, systemic sclerosis; TTE, transthoracic echocardiogram; mPAP, mean pulmonary arterial pressure
تواريخ الأحداث: Date Created: 20190906 Latest Revision: 20201001
رمز التحديث: 20231215
مُعرف محوري في PubMed: PMC6713838
DOI: 10.1016/j.mayocpiqo.2019.06.002
PMID: 31485576
قاعدة البيانات: MEDLINE
الوصف
تدمد:2542-4548
DOI:10.1016/j.mayocpiqo.2019.06.002