sildenafil-citrate and Hemoptysis

Sildenafil, usually a well-tolerated drug traditionally used for erectile dysfunction (ED), was recently approved for pulmonary arterial hypertension. In the literature, there are few cases of hemoptysis following sildenafil use for ED; however, to our knowledge, we are reporting the first case of hemoptysis following sildenafil use for pulmonary hypertension. We are documenting a case of a 90-year-old male patient who was admitted to the intensive care unit with hemoptysis and respiratory failure two weeks after he was started on sildenafil.

Topics: Aged, 80 and over; Anti-Inflammatory Agents, Non-Steroidal; Aspirin; Drug Therapy, Combination; Hemoptysis; Humans; Hypertension, Pulmonary; Intensive Care Units; Male; Patient Admission; Piperazines; Purines; Respiratory Insufficiency; Sildenafil Citrate; Sulfones; Vasodilator Agents

A young male patient reported for evaluation of progressive easy fatigability, accompanied by a recent history of recurrent haemoptysis. His clinical examination was unremarkable except for evidence of pulmonary arterial hypertension (PAH). Routine investigations (haemogram, coagulogram, serological tests for connective tissue disorders and a sputum Ziehl Neelsen stain for acid-fast bacilli) were normal. Two-dimensional echocardiography suggested PAH (pulmonary artery systolic pressure-67 mm Hg), whereas the 64-slice spiral CT pulmonary angiogram showed a dilated main pulmonary artery along with bilateral arteriovenous malformations. Cardiac catheterisation performed subsequently confirmed the presence of PAH. On the basis of the above findings, a diagnosis of hereditary haemorrhagic telangiectasia (HHT) complicated with PAH was made, and the patient was started on oral sildenafil therapy to which he responded well. This rare complication of HHT, which requires a high degree of suspicion for diagnosis, is discussed.

Topics: Echocardiography; Familial Primary Pulmonary Hypertension; Fatigue; Hemoptysis; Humans; Hypertension, Pulmonary; Male; Piperazines; Purines; Sildenafil Citrate; Sulfones; Telangiectasia, Hereditary Hemorrhagic; Tomography, X-Ray Computed; Vasodilator Agents; Young Adult

Sildenafil (phosphodiesterase type 5 inhibitor) has been shown to be effective in pulmonary arterial hypertension (PAH). We evaluated the efficacy and safety of oral sildenafil in patients of severe PAH with special emphasis on dose response relationship, time of onset of clinical response and its effects on different haemodynamic parameters.. Forty-four patients of severe PAH of either idiopathic pulmonary arterial hypertension [23 (51.7%)] or Eisenmenger syndrome [21 (48.3%)] were studied. All patients underwent six-minute walk test (SMWT) and echocardiography, while some also underwent cardiac catheterization. Sildenafil was started after a test dose and was gradually increased up to a target dose of 300 mg/day. Patients were followed-up 2 weekly for 10 weeks and monthly thereafter for functional class assessment and SMWT. Echocardiography and cardiac catheterization were repeated after at least 1 month of achieving maximal sildenafil dose (target dose or maximally tolerated dose). Drug safety and tolerability were assessed by monitoring patients for adverse effects including fundus examination.. Mean follow-up duration was 18.7+/-8.8 months (range 7-30 months). Mean maximum dose achieved was 276.1+/-62.2 mg/day (range 75-300 mg/day). A significant improvement in NYHA class (2.54+/-0.5 vs. 1.31+/-0.4, p=0.0001) and in SMWT distance (247.4+/-74.7 vs. 366.3+/-93.8 m, p=0.0001) was noted. All patients reported "feeling better" within 2 weeks of starting 12.5 mg thrice a day sildenafil. Marked improvement was noticed at 150 mg/day dose. Some minor additional benefit was noticed with further increase in the dose up to 225 mg/day. No further benefit was noted in improvement of NYHA class and SMWT distance by further increasing the dose of sildenafil. Haemoptysis as well as chest pain, if present, were also improved. On follow-up cardiac catheterization, a significant reduction in mean pulmonary arterial pressure (from 67.0+/-10.2 to 56.9+/-9.5 mm Hg, p=0.001), PVRI (from 19.5+/-7.0 to 11.1+/-6.9 WU m2, p=0.0001) and PVR/SVR ratio (0.6+/-0.3 vs. 0.4+/-0.2, p=0.013) with increase in cardiac index (2.9+/-1.1 l/min vs. 3.7+/-1.1 l/min, p=0.008) was noted. Systemic as well as pulmonary arterial oxygen saturations also improved significantly. Sildenafil was generally well tolerated, except for rhinorrhoea in 2, bodyache in 1 and headache in 1 patient. No visual symptom or change in fundus examination was noted.. Oral sildenafil improves functional capacity, haemodynamic parameters and is safe in patients with severe PAH. Benefits start as early as 2 weeks. The effects are dose related. A target dose of 150 mg/day appears to be optimal. Being very effective, widely available, relatively inexpensive, and very easy to use and very well tolerated without any major side effect, sildenafil may qualify as a first line medication for these patients.

Topics: Administration, Oral; Adolescent; Adult; Cardiac Catheterization; Chest Pain; Child; Dose-Response Relationship, Drug; Echocardiography; Eisenmenger Complex; Exercise Test; Female; Follow-Up Studies; Hemoptysis; Humans; Hypertension, Pulmonary; Male; Middle Aged; Oxygen; Phosphodiesterase Inhibitors; Piperazines; Purines; Sildenafil Citrate; Sulfones

Topics: Aged; Hemoptysis; Humans; Male; Middle Aged; Piperazines; Purines; Sildenafil Citrate; Sulfones