Long-term Treatment With Sildenafil Citrate in Pulmonary Arterial Hypertension

Rubin, Lewis J.; Badesch, David B.; Fleming, Thomas R.; Galiè, Nazzareno; Simonneau, Gérald; Ghofrani, Hossein Ardeschir; Oakes, Michael; Layton, Gary; Serdarevic-Pehar, Marjana; McLaughlin, Vallerie V.; Barst, Robyn J.

doi:10.1378/chest.10-0969

Cited by 242 publications

(152 citation statements)

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“…The SUPER-2 study reported that the majority of patients who entered the SUPER-1 trial improved or maintained their FC and 6MWD. 12 Similar to the results of SUPER-2 study, the present study demonstrated that sildenafil monotreatment resulted in maintenance or improvement of NYHA FC in the majority of all enrolled patients (91.3%). Therefore, we consider that these results demonstrate the superiority of sildenafil as a first-line drug for PAH.…”

Section: Discussionsupporting

confidence: 89%

Impact of First-Line Sildenafil Monotreatment for Pulmonary Arterial Hypertension

Yanagisawa

Kataoka

Tanaka

et al. 2012

Circ J

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Section: Discussionsupporting

confidence: 89%

Impact of First-Line Sildenafil Monotreatment for Pulmonary Arterial Hypertension

Yanagisawa

Kataoka

Tanaka

et al. 2012

Circ J

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“…PAH patients treated with Sildenafil 20, 40, or 80 mg t.i.d. have reported good results on 6-min walk distance, improving and maintaining functional class (38), haemodynamics parameters (37), and renal function (increased glomerular filtration rate, decreased serum creatinine) (38).…”

Section: Discussionmentioning

confidence: 99%

Reynolds Syndrome: a rare rheumatologic disease that internists should have in mind. Case report

2017

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Introduction: Reynolds syndrome (RS) is an autoimmune disorder characterized by overlapping primary biliary cirrhosis (PBC) and limited cutaneous systemic sclerosis (lcSSc). Some published cases do not report pulmonary arterial hypertension (PAH), and diagnoses are usually based on clinical, immunological and histological findings, mainly focused on dermatologic features, on those associated with Sjögren's syndrome, or on an interesting presentation of malignant thymoma; only one case of reported PAH was found, but it was an image report.

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“…7 In the last decade we witnessed the introduction of specific therapies which showed to improve exercise capacity, NYHA functional class, hemodynamics and progression of disease in several clinical trials. [2][3][4][5][6][8][9][10][11] Despite the number of PAH patients on treatment is increasing worldwide, there are no data published on patients with a very long survival and a good hemodynamic response. In this paper we report three iPAH cases with a very long survival (>10years), impressive hemodynamic response (almost normalization of PVR) ad reverse remodelling of the RV.…”

Section: Discussionmentioning

confidence: 99%

Untitled

2015

MOJCR

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Abbreviations: CI, cardiac index; iNO, inhaled nitric oxide; iPAH, idiopathic pulmonary arterial hypertension; NYHA, new york heart association; PAH, pulmonary arterial hypertension; PAP, pulmonary artery pressure; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; RHC, right heart catheterization; RV, right ventricular; TAPSE, tricuspidal anular plane systolic excursion; WHO, world health organization; WP, wedge pressure; WU, wood units; 6MWD, 6-minute walking distance IntroductionPulmonary arterial hypertension (PAH) is a progressive disease characterized by an increase in pulmonary vascular resistance (PVR) and pulmonary arterial pressure (PAP) leading to right ventricular failure and ultimately death.1 The past decade has witnessed major advances in PAH management, due to the introduction of specific therapies. Furthermore, oral drugs have been available in the last decade consenting a more wide treatment of the disease among clinicians' community. ET (endothelin)-receptor antagonists and PDE-5 (phosphodiesterase-5) inhibitors showed to improve exercise capacity, NYHA functional class, hemodynamics and progression of disease in several clinical trials.2-6 However, many patients have clinical worsening during oral treatment. Thus, addition of prostanoids represents an important chance to reach clinical improvement for these patients. During a 16-year period of activity in our referral center we treated almost 500 severe PH patients, and near 100 patients were exposed to parenteral prostanoids. In this paper we describe the clinical course of 3 long-term survivor patients (>10years), as they could be considered to have had an exceptional good response to treatment. Case reportsPatient 1 is a 65-year-old woman with idiopathic pulmonary arterial hypertension (iPAH) diagnosed in 1997 after a rapidly progressive reduction of exercise capacity in the last year. At first evaluation at our center, the patient was in NYHA/WHO functional class III, with a reduced effort capacity (six-minute walk distance -6MWD 370meters). ECG showed right ventricular (RV) strain, echocardiography showed marked RV dilatation, with a still preserved systolic function (tricuspidal anular plane systolic excursion -TAPSE 22mm). Right heart catheterization (RHC) revealed a moderate precapillary pulmonary hypertension (mean PAP 42mmHg, wedge pressure -WP 9mm Hg, cardiac index -CI 2.6l/min/m 2 , pulmonary vascular resistance -PVR 7.8WU-Wood Units), with no acute vasoreactivity to inhaled nitric oxide (iNO, 20ppm) (Figure 1). At that time, continuous iv infusion of epoprostenol was the only treatment available, so it was started and up-titrated to the maximum tolerated dosage. During the following months we observed a progressive improvement in clinical status and effort tolerance. After 2years (epoprostenol dose 32ng/kg/ min) she was in class II with a good effort tolerance (6MWD 490m), and a second invasive evaluation documented an improvement of hemodynamics (mPAP 26mmHg, CI 2.9l/min/m 2 , PVR 4.7WU). In the following 15years, s...

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Long-term Treatment With Sildenafil Citrate in Pulmonary Arterial Hypertension

Cited by 242 publications

References 22 publications

Impact of First-Line Sildenafil Monotreatment for Pulmonary Arterial Hypertension

Impact of First-Line Sildenafil Monotreatment for Pulmonary Arterial Hypertension

Reynolds Syndrome: a rare rheumatologic disease that internists should have in mind. Case report

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