sildenafil-citrate and Dyspnea

The prognosis of patients with untreated pulmonary arterial hypertension (PAH) has historically been poor. Previous studies have recommended that sildenafil was beneficial, but the dose varies greatly. In this study, we aimed to evaluate the safety and effectiveness of sildenafil [dose: 20 mg/three times a day (TID)] for adult Asian PAH patients.. Electronic databases (MEDLINE, Embase, Web of Science, the Cochrane Library, CBM, CNKI, and Wanfang Data) were searched from their inception to January 2022. We recruited all randomized controlled trials and non-randomized studies of interventions that compared sildenafil (20 mg/TID) versus placebo or symptomatic treatment for adult Asian PAH patients.. A total of 10 studies involving 480 participants were included. Compared to symptomatic treatment, sildenafil-treated patients were more likely to walk 57.68 meters further in six-minute walk distance [mean difference (MD) =57.68 m, 95% confidence interval (CI): 41.55 to 73.81], achieve an improvement in systemic arterial oxygen saturation (MD =2.48%, 95% CI: 1.26 to 3.71), and increase the score of the Borg scale for dyspnea (MD =-0.99 points, 95% CI: -1.45 to -0.53). The total number of patients with World Health Organization class III and IV also exhibited a downtrend. Compared to the placebo, sildenafil was associated with a reduction in the mean pulmonary artery pressure (MD =-4.13 mmHg, 95% CI: -6.52 to -1.74) and the level of brain natriuretic peptide (MD =-86.16 pg/mL, 95% CI: -103.39 to -68.93). The most common adverse events were headache, flushing, dyspepsia, and diarrhea, which were relatively mild.. Sildenafil at a dose of 20 mg/TID is well tolerated in adult Asian PAH patients, and is associated with statistically significant improvements in exercise capacity, cardio-pulmonary function, and haemodynamic indices. The long-term prognosis still needs to be evaluated and confirmed by further trials.

Topics: Adult; Dyspnea; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Quality of Life; Sildenafil Citrate; Treatment Outcome

Patients with fibrotic interstitial lung disease have symptom control and quality of life (QoL) needs. This review aims to evaluate the evidence for the use of interventions in improving dyspnoea, other symptoms and QoL.. Eleven databases, relevant websites and key journals were hand-searched. Studies were assessed and data extracted independently by two researchers using standardised proformas. Meta-analyses were performed where possible with 95% CI.. 34 papers with 19 interventions in 3635 patients were included. Meta-analyses showed no significant effect of interferon γ-1b or sildenafil on 6-minute walking distance (6MWD) or dyspnoea. Pulmonary rehabilitation and pirfenidone had a positive effect on 6MWD (mean difference (95% CI) 27.4 (4.1 to 50.7)) and 24.0 (4.3 to 43.7), respectively), and pulmonary rehabilitation had a mixed effect on dyspnoea. Both pulmonary rehabilitation and sildenafil showed a trend towards significance in improving QoL. There was weak evidence for the improvement of 6MWD using oxygen; dyspnoea using prednisolone, diamorphine, D-pencillamine and colchicine; cough using interferon α and thalidomide; anxiety using diamorphine; fatigue using pulmonary rehabilitation; and QoL using thalidomide and doxycycline. A wide range of outcome scales was used and there were no studies with economic evaluation.. There is strong evidence for the use of pulmonary rehabilitation and pirfenidone to improve 6MWD and moderate evidence for the use of sildenafil and pulmonary rehabilitation to improve QoL. Future recommendations for research would include careful consideration of the dichotomy of radical and palliative treatments when deciding on how symptom and QoL outcome measures are used and data presented.

Topics: Anti-Bacterial Agents; Anti-Inflammatory Agents, Non-Steroidal; Colchicine; Cough; Dyspnea; Exercise Test; Fibrosis; Glucocorticoids; Heroin; Humans; Immunologic Factors; Lung; Lung Diseases, Interstitial; Narcotics; Oxygen Inhalation Therapy; Piperazines; Prednisolone; Purines; Pyridones; Quality of Life; Sildenafil Citrate; Sulfones; Thalidomide; Tubulin Modulators

Topics: Anticoagulants; Diuretics; Drug Therapy, Combination; Dyspnea; Female; Humans; Hypertension, Pulmonary; Middle Aged; Phosphodiesterase 5 Inhibitors; Phosphodiesterase Inhibitors; Piperazines; Practice Guidelines as Topic; Purines; Sildenafil Citrate; Sulfones; Warfarin

Pulmonary arterial hypertension is a group of diseases which forms a small subset of those with elevated pulmonary artery pressure (pulmonary hypertension). The recent development of selective pulmonary vasodilator has lead to a substantial resurgence of interest in what have been previously regarded as rare and incurable diseases. This review aims to describe the spectrum of pulmonary vascular diseases, the evolving understanding as to pathogenesis, the evolving evidence of efficacy for drug therapies, trying to put this into a contemporary Australian context. Several key pathogenic pathways may be involved: prostacycline, Nitric Oxide-cGMP-phosphodiesterase 5 and endothelin- all of which are exploited for therapeutic benefit by newly available drug therapies. A recently modified classification system reasserts the importance of precise diagnosis. The cardinal symptom of exertional dyspnea warrants careful evaluation in an attempt to prevent (frequently occurring) substantial delay in diagnosis. Echocardiogram is the cornerstone of screening for pulmonary arterial hypertension; however, a detailed evaluation including a carefully performed right heart catheterisation with sufficient data to allow calculation of pulmonary vascular resistance is key to accurate diagnosis. These new approaches to therapy are already substantially improving quality of life and prognosis.

Topics: Adolescent; Adult; Aged; Arterioles; Australia; Bosentan; Cardiac Catheterization; Diagnostic Imaging; Disease Progression; Dyspnea; Endothelin A Receptor Antagonists; Epoprostenol; Exercise Test; Female; Forecasting; Heart-Lung Transplantation; Humans; Hypertension, Pulmonary; Iloprost; Lung Transplantation; Male; Middle Aged; Nitric Oxide; Piperazines; Prognosis; Pulmonary Artery; Purines; Randomized Controlled Trials as Topic; Sildenafil Citrate; Sulfonamides; Sulfones; Vascular Resistance; Vasodilator Agents; Ventricular Dysfunction, Right

Nintedanib is an approved treatment for idiopathic pulmonary fibrosis (IPF). A subgroup analysis of a previously published trial suggested that sildenafil may provide benefits regarding oxygenation, gas exchange as measured by the diffusion capacity of the lungs for carbon monoxide (Dl. We randomly assigned, in a 1:1 ratio, patients with IPF and a Dl. A total of 274 patients underwent randomization. There was no significant difference in the adjusted mean change from baseline in the SGRQ total score at week 12 between the nintedanib-plus-sildenafil group and the nintedanib group (-1.28 points and -0.77 points, respectively; P=0.72). A benefit from sildenafil treatment was not observed with regard to dyspnea as measured with the use of the University of California, San Diego, Shortness of Breath Questionnaire. No new safety signals were observed, as compared with previous trials.. In patients with IPF and a Dl

Topics: Adult; Aged; Double-Blind Method; Drug Therapy, Combination; Dyspnea; Enzyme Inhibitors; Female; Humans; Idiopathic Pulmonary Fibrosis; Indoles; Male; Middle Aged; Phosphodiesterase 5 Inhibitors; Protein-Tyrosine Kinases; Pulmonary Gas Exchange; Quality of Life; Sildenafil Citrate; Treatment Outcome

Idiopathic pulmonary fibrosis (IPF) is a form of idiopathic interstitial pneumonia characterized by temporally and spatially heterogeneous fibroblast proliferation and poor prognosis. No therapies have been shown in randomized clinical trials (RCT) to influence survival. Twenty-nine subjects were assigned randomly in a pilot study to a double-blind, placebo-controlled, RCT to test sildenafil in patients with IPF with forced vital capacity 40-90% and diffusing capacity 30-90% of predicted. During the 6-month experimental treatment period, patients underwent 6-min walk tests and estimation of dyspnea using the Borg scale at baseline (0 months), 3 months, and 6 months. Participants had moderate impairment of pulmonary function, and there were no significant differences between placebo (n = 15) and sildenafil (n = 14)-treated groups. Sildenafil did not significantly increase 6-min walk test distance (mean distance +/- SD after 6-month protocol: placebo 355 +/- 82 m, sildenafil 324 +/- 41 m; p = 0.256) nor did it lessen dyspnea after exercise (mean Borg score after 6-month protocol: placebo 3.4 +/- 1.6, sildenafil 4.1 +/- 2.3; p = 0.492). Adverse reactions were few and minor in nature. In this trial, sildenafil did not significantly increase 6-min walk test distance or decrease the Borg dyspnea index in patients with clinically typical IPF. This trial was registered at clinicaltrials.gov as NCT00359736.

Topics: Aged; Aged, 80 and over; Double-Blind Method; Dyspnea; Exercise Test; Exercise Tolerance; Female; Humans; Idiopathic Pulmonary Fibrosis; Male; Middle Aged; Phosphodiesterase Inhibitors; Pilot Projects; Piperazines; Pulmonary Diffusing Capacity; Purines; Sildenafil Citrate; Sulfones; Time Factors; Treatment Outcome; Vasodilator Agents; Vital Capacity

Sildenafil, a phosphodiesterase-5 inhibitor, may preferentially improve blood flow to well-ventilated regions of the lung in patients with advanced idiopathic pulmonary fibrosis, which could result in improvements in gas exchange. We tested the hypothesis that treatment with sildenafil would improve walk distance, dyspnea, and quality of life in patients with advanced idiopathic pulmonary fibrosis, defined as a carbon monoxide diffusion capacity of less than 35% of the predicted value.. We conducted a double-blind, randomized, placebo-controlled trial of sildenafil in two periods. The first period consisted of 12 weeks of a double-blind comparison between sildenafil and a placebo control. The primary outcome was the proportion of patients with an increase in the 6-minute walk distance of 20% or more. Key secondary outcomes included changes in oxygenation, degree of dyspnea, and quality of life. The second period was a 12-week open-label evaluation involving all patients receiving sildenafil.. A total of 180 patients were enrolled in the study. The difference in the primary outcome was not significant, with 9 of 89 patients (10%) in the sildenafil group and 6 of 91 (7%) in the placebo group having an improvement of 20% or more in the 6-minute walk distance (P=0.39). There were small but significant differences in arterial oxygenation, carbon monoxide diffusion capacity, degree of dyspnea, and quality of life favoring the sildenafil group. Serious adverse events were similar in the two study groups.. This study did not show a benefit for sildenafil for the primary outcome. The presence of some positive secondary outcomes creates clinical equipoise for further research. (Funded by the National Heart, Lung, and Blood Institute and others; ClinicalTrials.gov number, NCT00517933.)

Topics: Administration, Oral; Aged; Double-Blind Method; Dyspnea; Exercise Test; Female; Humans; Idiopathic Pulmonary Fibrosis; Male; Middle Aged; Oxygen; Piperazines; Pulmonary Diffusing Capacity; Purines; Quality of Life; Respiratory Function Tests; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilator Agents

It has been demonstrated that sildenafil is effective in patients with pulmonary arterial hypertension (PAH). However, the impact of sildenafil on PAH in adults with congenital heart disease (CHD) has been less investigated.. In this prospective, open-label, uncontrolled and multicenter study, 60 patients with PAH related to CHD received oral sildenafil (75 mg/day) for 12 weeks. The enrolled patients underwent six-minute walk test (SMWT) and cardiac catheterization at the beginning and the end of the 12 weeks. The primary end point was the changes in exercise capacity assessed by SMWT; the secondary end point included assessment of functional class, evaluation of cardiopulmonary hemodynamics, and clinical worsening (defined as death, transplantation, and rehospitalization for PAH). Drug safety and tolerability were also examined.. Oral sidenafil significantly increased SMWT distances (422.94 ± 76.95 m vs. 371.99 ± 78.73 m, P < 0.0001). There was also remarkable improvement in Borg dyspnea score (2.1 ± 1.32 vs. 2.57 ± 1.42, P = 0.0307). Moreover, significant improvements in World Healthy Organization (WHO) functional class and cardiopulmonary hemodynamics were also discovered (mean pulmonary artery pressure, P = 0.0002; cardiac index, P < 0.0001; pulmonary vascular resistance, P < 0.0001). Side effects in this study were mild and consistent with reported studies. None of the enrolled patients experienced significant clinical worsening.. This study confirmed and extended previous studies. It suggested that oral sildenafil was safe and effective for the treatment of adult patients with CHD-related PAH.

Topics: Administration, Oral; Adolescent; Adult; Antihypertensive Agents; Blood Pressure; Cardiac Catheterization; China; Drug Administration Schedule; Dyspnea; Exercise Test; Exercise Tolerance; Familial Primary Pulmonary Hypertension; Female; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Male; Piperazines; Prospective Studies; Purines; Sildenafil Citrate; Sulfones; Time Factors; Treatment Outcome; Vascular Resistance; Vasodilator Agents; Young Adult

Topics: Benzhydryl Compounds; COVID-19; Dyspnea; Fatigue; Glucosides; Humans; Sildenafil Citrate

A 24-year-old woman, a baby-sitter with no known comorbidities, presented to the outpatient department with complaints of modified Medical Research Council grade IV breathlessness for 3 months, chest pain, and dry cough for 2 weeks. There was no known disease history, including respiratory, flu-like illness, or connective tissue disorder. There was no use of chemotherapeutic, oral contraceptive drugs, exposure to toxic substances, or smoking. A review of systems was negative for fever, arthralgia, myalgia, Raynaud phenomenon, skin thickening, rash, or leg swelling. The patient had no family history suggestive of a genetic syndrome.

Topics: Chest Pain; Computed Tomography Angiography; Cough; Diagnosis, Differential; Dyspnea; Echocardiography; Endothelin A Receptor Antagonists; Female; Hemangioma, Capillary; Humans; Hypertension, Pulmonary; Lung Neoplasms; Lung Transplantation; Mutation; Oxygen Inhalation Therapy; Phosphodiesterase 5 Inhibitors; Protein Serine-Threonine Kinases; Pulmonary Veno-Occlusive Disease; Pyrimidines; Respiratory Function Tests; Sildenafil Citrate; Sulfonamides; Young Adult

Topics: Acetamides; Adolescent; Antihypertensive Agents; Asymptomatic Diseases; Bone Morphogenetic Protein Receptors, Type II; Cardiac Catheterization; Disease Progression; Dyspnea; Echocardiography; Exercise Tolerance; Female; Genetic Testing; Heterozygote; Humans; Male; Phenylpropionates; Pulmonary Arterial Hypertension; Pyrazines; Pyridazines; Sildenafil Citrate; Syncope; Vasodilator Agents

Chest pain and shortness of breath are chief complaints frequently evaluated in the emergency department. ACS, pulmonary embolism, and disorders involving the lung parenchyma are some of the disease processes commonly screened for. Occasionally, patients presenting with histories and clinical exams consistent with these common illnesses may end up having more rare pathology. We present the case of a young patient who presented with chest pain and dyspnea with ECG changes and history concerning for pulmonary embolism who was ultimately diagnosed with idiopathic primary pulmonary hypertension. The importance of a prompt diagnosis of this condition along with emergency department management of complications related to the disease is discussed in this report.

Topics: Adult; Chest Pain; Computed Tomography Angiography; Diagnosis, Differential; Dyspnea; Electrocardiography; Emergency Service, Hospital; Familial Primary Pulmonary Hypertension; Furosemide; Humans; Male; Sildenafil Citrate; Spironolactone

Closure of congenital atrial communications in the presence of either severe pulmonary arterial hypertension (PAH) with pulmonary-to-systemic (right-to-left) shunting, or severe left ventricular (LV) non-compliance with left-to-right shunting is often considered prohibitive. Thus, the recognition of durable reversibility of these physiologic conditions is crucial. We describe a hemodynamic conundrum in a patient with five septal communications in whom the coexistence of unmasked bidirectional physiologic shunting, severe PAH, and worsening left-sided overload dissuaded initial closure. We report our strategy for hemodynamic evaluation and successful closure of all defects.

Topics: Balloon Occlusion; Cardiac Catheterization; Dyspnea; Echocardiography, Transesophageal; Heart Septal Defects, Atrial; Hemodynamics; Humans; Hypertension, Pulmonary; Male; Middle Aged; Phosphodiesterase 5 Inhibitors; Septal Occluder Device; Sildenafil Citrate; Ventricular Dysfunction, Left

Dyspnea is a hallmark symptom of idiopathic pulmonary fibrosis (IPF), and dyspnea induced physical activity limitation is a prominent driver of quality of life impairment among IPF patients.. We examined response data for the 21 physical activity items (the first 21 of 24) from the University of California San Diego Shortness of Breath Questionnaire (UCSD) collected at baseline in a recently conducted IPF trial. We used Rasch analysis and hypothesis testing with conventional statistical methodology to achieve three objectives: 1) to examine the items to identify the one characteristic that distinguishes one from another; 2) to asses these items for their ability to measure dyspnea severity in IPF; 3) to use the items to develop a dyspnea ruler.. The sample comprised 178 subjects. The 21 items fit the Rasch model. There was very strong correlation between Rasch item severity and their metabolic equivalents (METS) values (r = -0.86, p < 0.0001). With the sample stratified on scores from the 21 items, there were significant between group differences in FVC%, DLCO% and distance walked during the six-minute walk test. The dyspnea ruler can be used to put dyspnea levels in a more easily understood clinical context.. The first 21 items from the UCSD compose a unidimensional dyspnea-with-activity scale and are both sensibly ordered and distinguished from each other by their METS values. These 21 items can be used confidently to formulate clinically-relevant inferences about IPF patients and should be considered for use as a meaningful endpoint in IPF research.

Topics: Aged; Controlled Clinical Trials as Topic; Dyspnea; Exercise Test; Female; Humans; Idiopathic Pulmonary Fibrosis; Male; Middle Aged; Piperazines; Purines; Quality of Life; Reproducibility of Results; Research Design; Severity of Illness Index; Sildenafil Citrate; Sulfones; Surveys and Questionnaires; Vasodilator Agents; Walking

A 43-year-old man presented with dyspnea on exertion. Right heart catheterization demonstrated pulmonary arterial hypertension (PAH). He was treated with bosentan, sildenafil and intravenous epoprostenol. Despite the administration of such intensive therapy, the patient's condition deteriorated to a World Health Organization functional class (WHO-FC) of IV. He participated in a clinical trial of imatinib for PAH. After three months of treatment with imatinib, the chest X-ray and echocardiography findings improved, and the WHO-FC class was III. One year after, however, the PAH worsened again, and the patient died 2.6 years after the first diagnosis. At autopsy, patchy capillary proliferation was observed in the lungs. The definitive diagnosis was pulmonary capillary hemangiomatosis.

Topics: Adult; Antihypertensive Agents; Antineoplastic Agents; Autopsy; Benzamides; Bosentan; Cardiac Catheterization; Dyspnea; Echocardiography; Epoprostenol; Fatal Outcome; Hemangioma, Capillary; Humans; Hypertension, Pulmonary; Imatinib Mesylate; Lung; Lung Neoplasms; Male; Piperazines; Purines; Pyrimidines; Radiography, Thoracic; Sildenafil Citrate; Sulfonamides; Treatment Failure

In Eisenmenger syndrome (ES), oral phosphodiesterase type-5 inhibitors, which are preferential pulmonary vasodilators, reduce the elevated pulmonary artery pressure and pulmonary vascular resistance index by increasing cyclic guanosine monophosphate (cGMP). However, no information is available as to how pulmonary vasodilatation alleviates the accompanying dyspnoea and improves patient's exercising ability.. As the natural stimulus of juxtapulmonary capillary (J) receptors is an increase in interstitial pressure, the aim was to estimate their threshold level stimulation chemically by intravenous lobeline, before and after 6 weeks of sildenafil therapy in treatment-naive ES patients.. Nine Eisenmenger syndrome patients [mean age=26 (SD=1.6) years] underwent 6MWT and an exercise test before and 6 weeks after oral sildenafil (20mg 3× D). Their respiratory responses to threshold doses of intravenous lobeline were determined at both these stages.. After 6 weeks of sildenafil therapy, the 6MWD [from 453.3 (SD=50.9) m to 516.6 (SD=48.9) m; P=0.001] and the duration of exercise with the modified Bruce protocol from 7 min 53 s (SD=0.04) to 10 min 44 s (SD=0.88) (P=0.001) improved significantly. However, the improvement in oxygen saturation was not noteworthy. The lobeline dose required to produce threshold level of respiratory effects was higher in ES patients [37.5 (SD=3.4) μg/kg] and with sildenafil therapy it fell significantly [20.6 (SD=1.8) μg/kg; P=0.001].. J receptor threshold doses were elevated in ES patients and fell significantly with sildenafil therapy that was associated with improved exercise tolerance, implying thereby a role of J receptors in producing dyspnea in ES patients.

Topics: Adult; Dyspnea; Eisenmenger Complex; Exercise Test; Female; Humans; Male; Piperazines; Purines; Sensory Receptor Cells; Sildenafil Citrate; Sulfones; Vasodilator Agents

We describe the case of a woman who presented to the intensive care unit with acute respiratory failure that required mechanical ventilation. She had severe pulmonary hypertension secondary to interstitial lung disease, and her history included sarcoidosis and tuberculosis. She was dependent on inhaled nitric oxide (INO) to maintain safe arterial oxygen saturation and could not be weaned from mechanical ventilation. Echocardiography revealed a patent foramen ovale with substantial right-to-left shunt, which probably contributed to her hypoxemia. Sildenafil enabled weaning from INO and substantially reduced the flow through the patent foramen ovale. She was successfully extubated and discharged home. To our knowledge, this is the first report of weaning from INO and mechanical ventilation in a patient with both severe secondary pulmonary hypertension and a right-to-left shunt through a patent foramen ovale.

Topics: Aged; Bronchodilator Agents; Comorbidity; Dyspnea; Female; Foramen Ovale, Patent; Humans; Hypertension, Pulmonary; Nitric Oxide; Phosphodiesterase 5 Inhibitors; Piperazines; Purines; Respiratory Insufficiency; Sarcoidosis, Pulmonary; Sildenafil Citrate; Sulfones; Vasodilator Agents; Ventilator Weaning

Chronic phosphodiesterase-5 inhibition improves peak oxygen consumption, ventilatory efficiency (VE/VCO(2) slope) and pulmonary artery pressure (PAP) in heart failure (HF). In 40 male patients, Sildenafil treatment produced a significant (p<0.001) decrease in dyspnea upon exertion (DOE) at maximal exercise. The correlations between the change in systolic PAP and both the change in the VE/VCO(2) slope (r=0.57, p<0.001) and DOE at maximal exercise (r(s)=0.49, p<0.001) were significant. DOE at maximal exercise is significantly reduced and the degree of improvement in PAP is reflected by the degree of improvement in the VE/VCO(2) slope and DOE following Sildenafil therapy.

Topics: Aged; Dyspnea; Exercise Test; Heart Failure; Humans; Hypertension, Pulmonary; Male; Middle Aged; Oxygen Consumption; Physical Exertion; Piperazines; Purines; Quality of Life; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilator Agents; Work of Breathing

The long-term effects of drugs developed for the control of pulmonary arterial hypertension (PAH) are little known, since multicenter studies usually last 12 to 16 weeks.. To evaluate the two-year outcome of PAH patients receiving monotherapy with sildenafil (a phosphodiesterase-5 inhibitor), with regard to their functional capacity.. Twenty four patients (ages between 8 and 54 years) with idiopathic PAH (IPAH, n = 9) or congenital heart disease-associated PAH (CHD-PAH, n = 15) were treated with sildenafil for two years, with daily oral doses ranging from 60 to 225 mg (tid). Physical capacity was assessed by the distance walked in the 6-minute walk test (DW6M) and by the degree of dyspnea at the end of the walk (Borg scale); peripheral oxygen saturation was also recorded (SpO(2)6M, pulse oximetry).. In the 18 patients who completed the two-year follow-up, there was a progressive and sustained increase in DW6M, both in the IPAH group (from 239 +/- 160 m to 471 +/- 66 m, p = 0.0076) and in the CHD-PAH group (from 361 +/- 144 m to 445 +/- 96 m, p = 0.0031), with improvement of dyspnea at the end of the walk (p<0.05 for both groups). No decrease in SpO(2)6M was observed in the groups; in patients with CHD-PAH, in particular, SpO(2)6M went from 77 +/- 20% to 79 +/- 16% (p = 0.5248). Five deaths occurred (three in the IPAH group) and one patient was lost to follow-up during the study period.. In a two-year follow-up, sildenafil proved useful in the control of the functional status of PAH patients, with significant improvement in both groups considered.

Topics: Adolescent; Adult; Child; Dyspnea; Exercise Test; Female; Follow-Up Studies; Humans; Hypertension, Pulmonary; Male; Middle Aged; Oxygen Consumption; Phosphodiesterase 5 Inhibitors; Piperazines; Prospective Studies; Purines; Sildenafil Citrate; Statistics, Nonparametric; Sulfones; Time Factors; Treatment Outcome; Young Adult

Topics: Antihypertensive Agents; Bosentan; Dyspnea; Hemodynamics; Histiocytosis, Langerhans-Cell; Humans; Hypertension, Pulmonary; Male; Middle Aged; Piperazines; Purines; Severity of Illness Index; Sildenafil Citrate; Sulfonamides; Sulfones

Selective right pulmonary arteriography and 3-dimensional computed tomography revealed multiple severe stenoses of the peripheral pulmonary artery associated with poststenotic aneurysms in a 65-year-old woman. She was referred to the hospital for evaluation of dry cough, gradually increasing dyspnea and multiple nodular shadows on a chest radiograph. Echocardiography and cardiac catheterization showed severe pulmonary hypertension, though other structural heart diseases or well-characterized congenital syndromes were ruled out. She was diagnosed as isolated peripheral pulmonary artery branch stenosis. Recent advances in CT technology enable a less-invasive assessment of pulmonary artery, and can be useful in the management of pulmonary arterial hypertension.

Topics: Aged; Aneurysm; Arterial Occlusive Diseases; Cardiac Catheterization; Constriction, Pathologic; Cough; Dyspnea; Epoprostenol; Female; Humans; Hypertension, Pulmonary; Imaging, Three-Dimensional; Oxygen Inhalation Therapy; Piperazines; Pulmonary Artery; Purines; Sildenafil Citrate; Sulfones; Tomography, X-Ray Computed; Ultrasonography; Vasodilator Agents; Warfarin

Topics: Aged; Dyspnea; Erectile Dysfunction; Female; Humans; Idiopathic Pulmonary Fibrosis; Male; Piperazines; Purines; Quality of Life; Sildenafil Citrate; Sulfones; Vasodilator Agents

The authors report a case of severe pulmonary arterial hypertension (PAH) in a 75-year-old woman who had received a diagnosis of neurofibromatosis type 1 (NF1) 23 years before. She presented with progressive dyspnoea and recurrent syncope. Even though the patient initially improved after starting supportive and specific treatment for PAH, she then deteriorated and died from respiratory failure 11 months after the diagnosis of PAH. Prompt recognition of such an unusual association between PAH and NF1 and appropriate therapeutic intervention could ameliorate quality of life and prolong survival in this patient population.

Topics: Aged; Anticoagulants; Combined Modality Therapy; Diuretics; Dyspnea; Fatal Outcome; Female; Follow-Up Studies; Furosemide; Humans; Hypertension, Pulmonary; Neurofibromatosis 1; Oxygen Inhalation Therapy; Piperazines; Purines; Recurrence; Sildenafil Citrate; Spironolactone; Sulfones; Syncope; Vasodilator Agents

There are several possible pathophysiological links between the development of pulmonary hypertension and myelofibrosis with myeloid metaplasia. We report a woman with myelofibrosis and myeloid metaplasia who presented with dyspnea and massive, painful splenomegaly. Right heart catheterization evidenced pulmonary hypertension. Her management consisted of splenic irradiation associated to sildenafil. Dyspnea in patients with myelofibrosis and myeloid metaplasia can be secondary to pulmonary hypertension and conversely the differential diagnosis of pulmonary hypertension should include a myeloproliferative syndrome.

Topics: Anticoagulants; Cardiac Catheterization; Diagnosis, Differential; Diuretics; Drug Therapy, Combination; Dyspnea; Echocardiography, Doppler; Electrocardiography; Female; Fibrinolytic Agents; Furosemide; Heparin, Low-Molecular-Weight; Humans; Hypertension, Pulmonary; Middle Aged; Myeloproliferative Disorders; Palliative Care; Phosphodiesterase Inhibitors; Piperazines; Primary Myelofibrosis; Purines; Sildenafil Citrate; Splenic Diseases; Splenomegaly; Sulfones; Vasodilator Agents

Pulmonary veno-occlusive disease (PVOD) is a disorder which causes progressive pulmonary hypertension, usually presenting with worsening dyspnoea and right heart failure. Pulmonary oedema induced by pulmonary vasodilator therapy to reduce pulmonary arterial pressure has been well described in PVOD, but here we describe a case of PVOD presenting with recurrent episodes of acute non-cardiogenic pulmonary oedema, in the absence of significant pulmonary hypertension. Concern over the risk of precipitating pulmonary oedema led us to use inhaled nitric oxide to predict the safety and efficacy of sildenafil.

Topics: Adult; Bronchodilator Agents; Dyspnea; Humans; Male; Nitric Oxide; Piperazines; Pulmonary Edema; Pulmonary Veno-Occlusive Disease; Purines; Recurrence; Sildenafil Citrate; Sulfones; Tomography, X-Ray Computed; Vasodilator Agents

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.

Topics: Adult; Antihypertensive Agents; Drug Therapy, Combination; Dyspnea; Epoprostenol; Humans; Lung; Male; Phosphodiesterase Inhibitors; Piperazines; Pulmonary Veno-Occlusive Disease; Purines; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilator Agents

Topics: Activities of Daily Living; Aged; Disease Progression; Dyspnea; Humans; Hypertension, Pulmonary; Male; Piperazines; Purines; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilator Agents

Topics: Adult; Anti-Inflammatory Agents; Cyclophosphamide; Drug Therapy, Combination; Dyspnea; Female; Humans; Hypertension, Pulmonary; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Methylprednisolone; Piperazines; Purines; Sildenafil Citrate; Sulfones; Treatment Failure; Vasodilator Agents