Pulmonary Veno-occlusive Disease: A Case Report and Review of Literature

Abstract

An 18-year-old male was referred from a local hospital with history of progressive shortness of breath for six months. He also complained of a syncopal attack on exertion lasting for few seconds. The patient had no significant past medical history except mild intermittent bronchial asthma controlled on inhaled albuterol as required. He denied use of any medication and there was no family history of respiratory or cardiac illness. On examination, he was found to have regular heart rate of 90 beats per minute, respiratory rate of 22 breaths per minute, blood pressure of 117/74 mmHg and an oxygen saturation of 91% on room air. Cardiopulmonary examination revealed raised jugular venous pressure, positive left parasternal heave, loud pulmonary component of 2 nd heart sound, tricuspid regurgitation murmur, minimal basal crackled on chest auscultation and mild pedal edema. Abdominal examination showed soft, non-tender, lax abdomen with no organ enlargement and neurological examination was normal.Laboratory including complete blood count, liver and kidney functions were within normal range. Pro-B type natriuretic peptide was elevated at 654 pg/mL (normal <100 pg/mL). Screening for autoantibodies was negative.Chest X-ray (Figure 1) revealed enlarged pulmonary arteries and increased bronchovascular markings. ECG showed sinus tachycardia, right axis deviation and prominent P wave.A 2-dimensional transthoracic cardiac echocardiogram showed a severely dilated right ventricle, moderate tricuspid regurgitation with estimated systolic pulmonary artery pressure of 50 mmHg. Left ventricle was normal with preserved systolic function. Right heart catheterization result revealed mean pulmonary artery pressure >60 mmHg, Right atrial pressure 6 mmHg, pulmonary capillary wedge pressure 8 mmHg, pulmonary vascular resistance 13.78 Wood units and cardiac index 1.98 L/m/min.A computed tomographic angiogram (CTA) of chest (Figure 2) did not show any evidence of pulmonary embolism but there were dilated main pulmonary arteries, enlarged right atrium and ventricle, septal thickening with nodularity, centrilobular ground glass densities

Targeted therapy in pulmonary veno-occlusive disease: time for a rethink?

“…A total of 30 patients with PVOD receiving PAH-targeted drugs during their hospitalizations were reported, and 16 out of them (53.3%) were male. Sixteen patients received monotherapy [2, 11–17, 19, 22], the commonest targeted drug used for these patients was epoprostenol (75%), and 12 patients were treated with sequential dual combination therapy [2, 18, 21, 24–26], one with initial dual combination therapy [2], one with sequential triple combination therapy, bosentan 125 mg bid stabilized his condition for 7 months, sildenafil 40 mg tid as an add-on therapy enabled remarkable clinical stabilization with a 6MWD of 560 m, then a low dose of epoprostenol (1–2 ng/kg/min) was added for declined exercise capacity but was stopped due to deterioration of oxygen saturation, sequential iloprost 2.5 μg q4h maintained his status till double lung transplantation [23]. Sildenafil was demonstrated to be effective and safe [17], and it is one of the most frequently used drugs in combined therapy for PVOD patients [18, 21, 23–26], those receiving sildenafil as monotherapy [17] or combined therapy [18, 21, 23, 25, 26] almost survived more than 1 year.…”