sildenafil-citrate and Hemangioma--Capillary

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

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