Objective To investigate the diagnosis and treatment of pulmonary arterial hypertension ( PAH) due to rare causes. Methods The clinical presentation, laboratory testing, diagnosis and treatment of 4 patients with PAH associated with rare causes in Beijing Anzhen Hospital from January 2001 to March 2008 were analysed retrospectively. Results Primary biliary cirrhosis, hyperthyroidism, antiphospholipid syndrome and pulmonary artery sarcoma may cause PAH, which were improved after corresponding diagnosis and management. Conclusion PAH can result from rare causes. The enhancement of its recognition will help earlier diagnosis and treatment and improve the prognosis.
【Abstract】 Objective To improve the knowledge of idiopathic pulmonary arterial hypertension ( IPAH) to elevate the levels of early diagnosis and treatment. Methods The clinical data of 39 IPAH patients admitted in Beijing Anzhen Hospital from October 1997 to June 2010 were reviewed. Results Of the 39 IPAH patients, 14 cases were male and 25 cases were female, with an average age of ( 29. 7 ±16. 4)years old. Main clinical manifestations were exertional dyspnea/breathlessness ( 90. 9% ) , chest tightness( 72. 7%) , chest pain ( 30. 7% ) , cough ( 41. 0% ) , fatigue ( 48. 7% ) , syncope ( 35. 9% ) , cyanosis( 28. 2% ) , edema of lower extremity ( 43. 6%) , etc. As revealed through echocardiography, 39 cases had a mean systolic pulmonary arterial pressure ( SPAP) of ( 88. 8 ±24. 2) mmHg, with right ventricle enlargementin 37 cases and normal in 2 cases. Pulmonary angiography showed central pulmonary arterial dilatation with pruning of the peripheral blood vessels in 36 cases and normal in 3 cases. Right heart catheterization and acute vasodilator testing was performed with iloprost in 15 patients, systolic pulmonary arterial pressure was( 77. 6 ±27. 8 ) mm Hg, and positive rate was 20. 0% . 24 cases were misdiagnosed at admission, and misdiagnosis rate was 61. 5% . The average time of misdiagnosis was ( 26. 0 ±24. 5) months. 20 cases were treated with general medical therapy and 1 case was managed with lung transplantation before April 2008.Then 13 cases were given pulmonary arterial hypertension-targeted therapies, including sidenafil, iloprost or bosentan. Two patients died in hospital with a mortality rate of 5. 1% . Conclusions IPAH is uncommon and often occurs in young and middle-aged women. The symptoms are nonspecific and easily misdiagnosed.Echocardiography and pulmonary angiography are helpful in diagnosis. Right heart catheterization and acute vasodilator testing should be carried out if available. The patients should be early treated with pulmonary arterial hypertension-targeted therapies. Lung transplantation may be an option for end-stage cases.
Objective To improve the knowledge of pulmonary artery sarcoma ( PAS) and early diagnosis.Methods The clinical data of 8 patients with PAS confirmed by biopsy from April 2001 to April 2012 in Beijing Anzhen Hospital were retrospectively analyzed. Results There were 5 males and 3 females, with mean age of 46. 75 ±11. 47 years [ range: 32-67 years] . The main clinical manifestations were chest tightness, shortness of breath, intermittent syncope, heart palpitations at exertion, etc. Laboratory examinations showed the patients with PAS have no obvious hypoxemia and most of them have normal D-dimer level. Echocardiography revealed pulmonary hypertension, right ventricular enlargement, and echo of massive lumps in main pulmonary truck. Lower limb veins were normal in color doppler ultrasonography. Chest X-ray revealed prominent pulmonary artery segment, full segment of the right pulmonary artery, an increased hilum and pleural effusion. CT pulmonary angiography showed expansion of pulmonary artery, large filling defect in main pulmonary truck and left or right pulmonary artery, combined with pericardial effusion, pleural effusion. Lung ventilation/perfusion imaging did not match, showing radioactive sparse and defects in multiple lung segments and subsegments, involved 3 to 13 lung segments. Pulmonary angiography showed filling defects in the main pulmonary artery, left or right pulmonary artery. 8 patients were confirmed pathologically after operation. Pathological results showed leiomyosarcoma differentiation in 3 cases, undifferentiated sarcoma in2 cases, and undefined pathological type in 3 cases. All 8 patients were misdiagnosed as pulmonary embolism before surgery. The average days of misdiagnosis were 85. 6 ±21. 5 days. 7 cases were given simple surgical resection, one case underwent surgical resection combined with radiotherapy and chemotherapy. 7 cases were relieved and discharged, and one case died. Conclusion PAS is a rare disease clinically and is easily misdiagnosed as pulmonary embolism. Clinicians should enhance the recognition in order to diagnose early and treat comprehensively.