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<p><b>OBJECTIVE</b>To investigate the clinical characteristics of pulmonary artery sarcoma (PAS) and to improve doctors' awareness and the early diagnosis of this disease.</p><p><b>METHODS</b>The clinical data of 9 cases confirmed by operation and pathology of PAS from November 2001 to November 2012 in Beijing Anzhen Hospital were reviewed. The clinical characteristics, laboratory tests, imaging manifestation, pathology as well as follow-up were studied. Survival was determined by the Kaplan-Merier method.</p><p><b>RESULTS</b>(1)Main clinical manifestations were chest distress (8/9), palpitation (2/9), syncope (2/9), cough (2/9), weight loss (2/9) and chest pain (1/9). (2)Lactate dehydrogenase (LDH) , high-sensitivity C-reactive protein (hs-CRP), erythrocyte sedimentation rate (ESR) were all elevated to different extents, but D-Dimer remained in the normal range. (3) Seven cases received CTPA examination and evidenced filling defect in the main pulmonary artery trunk (6/7), left pulmonary artery (6/7), right pulmonary artery (7/7). 7 cases received TTE examination and showed enlarged right ventricle (6/7) : mean right ventricular end-diastolic diameter was (38.54 ± 16.30) mm; enlarged right atrium (7/7): mean right atrium diameter was (55.11 ± 5.45) mm; and tricuspid insufficiency (7/7) : estimated mean pulmonary artery pressure was (81.14 ± 21.17) mmHg (1 mmHg = 0.133 kPa) , and pulmonary stenosis (5/7) . Four cases received deep venous ultrasound examination and deep venous thrombosis (DVT) was found in 1 patient. Four cases received Ganz catheter examination and the preoperative and postoperative mean pulmonary artery pressure was (52.00 ± 5.23) mmHg and (23.00 ± 5.60) mmHg, respectively. (4) All 9 patients received surgery and intimal sarcoma was diagnosed in all of them. Leiomyosarcoma was diagnosed in 3 cases, leiomyo-angiosarcoma was diagnosed in 1 case, undifferentiated sarcomas was diagnosed in 1 case. (5)The 1-, 3- and 5-month survival was 71.4%, 53.6% and 35.7%, respectively, median survival time was 5 months post surgery.</p><p><b>CONCLUSION</b>PAS is difficult to differentiate with PTE. PAS should be suspected in patient with right atrium, right ventricular enlargement and early appeared right heart failure, and normal D-Dimer level. Outcome for PAS patients is poor despite surgery in this cohort.</p>
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Adult , Aged , Female , Humans , Male , Middle Aged , Follow-Up Studies , Hemangiosarcoma , Diagnosis , Pathology , Pulmonary Artery , Pathology , Retrospective StudiesABSTRACT
Objective To investigate the diagnosis and surgical treatment for the intracardiac leiomyomatosis (ICL).Methods Retrospectively reviews the data of 18 cases of ICL from February 1995 to June 2011 in Anzhen Hospital.Preoperative diagnosis of right atrial myxoma in 2 cases,right atrium/ventricular thrombus in 2 cases,pulmonary embolism in 2 cases,the inferior vena cava and right atrial tumor in 3 cases,the inferior vena cava lesions in 9 cases.18 cases of ICL were diagnosed by echocardiography,magnetic resonance imaging ( MRI ),computer tomography (CT) and angiography.ICL were recected under anesthesia,cardiopulmonaray bypass surgery,he incisions were respectively through the chest,thoraco-abdominal or abdominal incision,cardiopulmonary bypass method were respectively at room temperature,mid-low temperature with cardiac arrest,lowflow,deep hypothermic circulatory arrest.Results There was no operative death.Right atrium,inferior vena cava,bilateral renal vein,bilater alIliac vein vascular were not found leiomyoma by vascular ultrasound.Follow-up time was 5 ～ 106 (49 ± 42 )months,1 patient died of the leiomyoma recurrence after 5 months after the primary operation,the remaining 17 patients were followed up asymptomatically.10 were in NYHA functional class Ⅰ,and 7 in class Ⅱ.The 5yrs survival rate calculated by the Kaplan-Meier survival curve was (93 ±5 )％.Conclusion The ICL preoperative diagnosis is difficult,surgical treatment of ICL can get a good mid-long term survival rate and living quality.
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Objective To compare the results of Danielson procedure with and without prosthetic valve ring in treating Ebstein anomaly and to define the effect of prosthetic valve ring on the procedure.Methods From January 2006 to December 2009,31 cases of Ebstein anomaly over 10 years old were classified as type A or type B according the Carpentier＇s classification scheme.Patients were treated by Danielson procedure or Danielson procedure plus prosthetic valve ring at Anzhen hospital.They were retrospectively classified as Danielson procedure group (group A,n =19) and Danielson procedure plus prosthetic valve ring group (group B,n =12 ).Results There was 1 early in-hospital death due to lung infection and hypoxemia in group A,and no early death in group B ( Fisher exact test,P =0.51 ).The mean follow-up time was ( 23.0 ± 18.5 ) months (5 -41 months).The cumulative follow-up time was 59.42 patient-years.There was one late death in group A due to the redo tricuspid valve plastic procedure because of severe tricuspid regurgitation,and no late death in group B.With echocardiography inspection,11 patients had mild and 7 had moderate to severe tricuspid regurgitation in group A,and only 2 mild tricuspid regurgitation in group B.The tricuspid valve competence after surgery in group B was better than in group A ( Fisher exact test,P=0.024).The 6-minute walk distance test (6MWD) in group B was significantly better than in group A(415 ±41 )m vs ( 382 ± 46 ) m( t test,P =0.047 ).The New York heart functional class in group B was statistically better than in Group A ( P =0.024).Conclusion Although there was no significant difference in the early and late mortality rate between the two groups after surgery,Danielson procedure plus prosthetic valve ring was better than pure Danielson procedure in prevention of late tricuspid regurgitation recurrence,heart function and 6MWD test during follow-up.
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Objective: To study the Ross procedure in surgery for aortic valve disease. Methods: From October, 1994 to May, 2002, 7 patients underwent Ross operation for aortic valve diseases. Pulmonary homografts were used for reconstruction of right ventricular outflow tract (RVOT). Among them, male 4, female 3, age from 11 to 44 years old [average (28?10) years]. All had severe aortic valve regurgitation, 2 associated with moderate aortic valve stenosis, 1 with bicuspid aortic valve, 1 with atrium septal defect, and 1 with ventricular septal defect. Pulmonary valve function was normal in all patients. Results: There was no operative death. One patient died 3 years later of SBE. Echocardiography indicated the normal function of aortic and pulmonary valve and excellent heart function. Conclusion: Ross operation provides is difficult but yields excellent clinical results.
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Objective To improve the understanding of primary pulmonary artery sarcoma(PAS)and its early diagnosis and treatment.Methods The clinical data of 3 PAS patients in Beijing Anzhen Hospital and related literature,the clinical features,diagnosis and therapy of the disease were retrospectively studied.Results Pulmonary artery sarcoma had similar clinical presentations to pulmonary thromboembolism(PTE).Although CT pulmonary angiography and Color Doppler echocardiography etc were helpful to the diagnosis of the entity,the confirmed diagnosis needed the operation.The surgery was main therapy for the disease.Conclusion Pulmonary artery sarcoma is easily misdiagnosed as PTE.The clinicians should pay more attention to the disease so as to early diagnose and treat it.