Diagnosis, Genetics, and Management of 24 Patients With Cardiac Paragangliomas: Experience From a Single Center.

Context: Paragangliomas located within the pericardium represent a rare yet challenging clinical situation. Objective: The current analysis aimed to describe the clinical characteristics of cardiac paragangliomas, with emphasis on the diagnostic approach, genetic background, and multidisciplinary management. Methods: Twenty-four patients diagnosed with cardiac paraganglioma (PGL) in Peking Union Medical College Hospital, Beijing, China, between 2003 and 2021 were identified. Clinical data was collected from medical record. Genetic screening and succinate dehydrogenase subunit B immunohistochemistry were performed in 22 patients. Results: The median age at diagnosis was 38 years (range 11-51 years), 8 patients (33%) were females, and 4 (17%) had familial history. Hypertension and/or symptoms related to catecholamine secretion were present in 22 (92%) patients. Excess levels of catecholamines and/or metanephrines were detected in 22 (96%) of the 23 patients who have completed biochemical testing. Cardiac PGLs were localized with 131I-metaiodobenzylguanidine scintigraphy in 11/22 (50%), and 99mTc-hydrazinonicotinyl-tyr3-octreotide scintigraphy in 24/24 (100%) patients. Genetic testing identified germline SDHx mutations in 13/22 (59%) patients, while immunohistochemistry revealed succinate dehydrogenase (SDH) deficiency in tumors from 17/22 (77%) patients. All patients were managed by a multidisciplinary team through medical preparation, surgery, and follow-up. Twenty-three patients received surgical treatment and perioperative death occurred in 2 cases. Overall, 21 patients were alive at follow-up (median 7.0 years, range 0.6-18 years). Local recurrence or metastasis developed in 3 patients, all of whom had SDH-deficient tumors. Conclusion: Cardiac PGLs can be diagnosed based on clinical manifestations, biochemical tests, and appropriate imaging studies. Genetic screening, multidisciplinary approach, and long-term follow-up are crucial in the management of this disease.

Outcomes of surgery for functional cardiac paragangliomas: A single-center experience of 17 patients.

OBJECTIVE: Cardiac paragangliomas are rare neuroendocrine tumors. Early surgical treatment improves clinical symptoms and prolongs survival. We review our experience in 17 patients who underwent surgical resection for functional cardiac paraganglioma. METHODS: Seventeen patients underwent surgery for functional cardiac paraganglioma from 2004 to 2017 were identified. Clinical data and long-term outcomes were extracted and analyzed. RESULTS: All 17 patients with cardiac paraganglioma (11 males) with a median age of 35 years (range, 11 to 51 years) were hormonally functional and underwent operations. A 24-hour urine catecholamine assay documented elevation of norepinephrine, epinephrine, and dopamine. Tumors were determined with octreotide scintiscan in all 17 patients and metaiodoben-zylguanidine scintigraphy in 10 of 15 patients. Tumor sites were right atrioventricular groove in 4 patients, between the main arteries in 10 patients, and interatrial groove in 4 patients. All patients underwent complete resection. Concomitant surgeries were: structural reconstruction in 16 patients (94%) and coronary artery bypass graft in 8 patients (47%). One patient had a history of incomplete resection of an intra-atrial tumor. One patient died postoperatively. Operative mortality is 5.8%. During follow-up (mean, 6.5 years; range, 1.5 to 14.2 years), 16 patients are alive in functional class I or II. Two patients developed tumor recurrence and 14 remained symptom-free with normal urinary catecholamines. CONCLUSIONS: With a multidisciplinary treatment, early diagnosis, complete resection for the tumor along with complex reconstruction is achievable for most patients, and it offers promising long-term survival.

Surgical Treatment Strategies of Intravenous Leiomyomatosis withRight Cardiac Cavities Extension.

Objective To investigate the diagnosis and surgical treatment strategies of intravenous leiomyomatosis(IVL)extending through inferior vena cava into the right cardiac cavities. Methods Thirty patients of IVL extending through inferior vena cava into the right cardiac cavities were treated in Peking Union Medical College Hospital from November 2002 to January 2015.The following variables were studied: age,cardiopulmonary bypass time,deep hypothermic circulatory arrest time,origins of IVL,blood loss,duration of post-operative hospital stay,hospitalization expenses,edema of lower extremity,blood transfusion,postoperative complication,residual IVL,and re-grow or recurrence. Results Thirteen of 30 patients reported double lower limb edema. The cardiopulmonary bypass was applied in 27 cases,and the average duration of cardiopulmonary bypass was(106.9±53.7)min. Then,21 patients were treated with the deep hypothermic circulatory arrest,and the mean time was(28.2±11.6) min. The tumors originated from the genital veins in 9 cases,the iliac vein in 13 cases,and both veins in 8 cases. The average intra-operative blood loss volume was (2060.5±2012.3)ml,and 21 patients received blood transfusion. The average hospitalization time was(18.9±8.3)days and the average hospitalization expenses was (80 840.4±28 264.2)RMB yuan. While 14 patients had postoperative complications,there was no serious postoperative complication or death.All patients have shown a favorable outcome.Conclusions Tumor embolus extending through inferior vena cava into the right cardiac cavities should be suspected in patients with multiple hysteromyoma. Successful therapy for IVL with right cardiac cavities extension is dependent on reasonable surgical treatment strategies. Surgical removal of the ovaries is vital to avoid IVL re-grow or recurrence.

Surgical treatment of pulmonary artery aneurysm: an institutional experience and literature review.

OBJECTIVES: The occurrence of pulmonary artery aneurysm (PAA) is extremely rare in the clinical setting. Careful treatment should be considered because of the possibility of fatal complications including rupture, dissection, pulmonary embolism and heart failure. Our goal is to contribute a better understanding of this disease and its treatment. METHODS: Information on patients diagnosed with PAA was retrieved from the institute's database. The detailed data including aetiology, clinical presentation, diagnostic methods, operation methods and long-term outcomes of the surgical cases were outlined and analysed. RESULTS: In total, 21 patients were diagnosed with PAA in Peking Union Medical College Hospital from 1980 to 2015, among whom 5 patients received surgical treatment, including 2 patients with giant PAAs. The complete remission rate of surgical cases was 80%, and the average postoperative hospital stay was 8.5 ± 1.29 days. One postoperative death occurred due to distributive shock. CONCLUSIONS: PAAs must be seriously classified by aetiology to be treated appropriately. Patients with giant-size PAAs, and those with pulmonary hypertension, anatomical anomalies, and rapid growth and compression of neighbouring critical structures, are proper candidates for surgery. Surgical options include aneurysm repair and replacement with allogeneic/synthetic grafts, depending on the situation. Additionally, the correction of associated abnormalities should be performed simultaneously during surgery. Surgical outcomes are effective, and long-term prognoses are satisfactory.

[Endoscopic Saphenous Vein Harvesting versus Open Vein Harvesting Techniques].

OBJECTIVE: To investigate whether endoscopic saphenous vein harvesting (EVH )reduces leg wound morbidity and improves rehabilitation of lower extremity as compared to open vein harvesting (OVH) in patients with coronary artery disease. METHODS: The clinical data of 333 patients who underwent isolated coronary artery bypass graft surgery (CABG) between January 2007 and December 2011 were retrospectively analyzed.These patients were divided into EVH group (n=210) and OVH group (n=123). Parameters including age,sex,with/without diabetes mellitus,leg wound complications rate,postoperative hospital stay,hospitalization expense,and vein graft patency were analyzed. RESULTS: The age,sex,with/without diabetes mellitus were not significantly different between two groups (P>0.05). However,the incidences of leg wound complications, poor wound healing, wound infections, wound pain,wound numbness, and edema of lower extremity were significantly lower in the EVH group than the OVH group (P<0.05). The ecchymoma and deep vein thrombosis incidences were not significantly different between these two groups (P>0.05). The postoperative hospital stay showed no significant difference (P>0.05), while the hospitalization expense was significantly higher in the EVH group than in the OVH group (P<0.05). There was no statistical difference of vein graft patency (P>0.05). CONCLUSION: The EVH system is a safe and effective alternative to OVH because it can markedly reduce postoperative leg wound morbidity and the EVH vein graft has good patency.

Angiosarcoma of the pericardium: a case report.

BACKGROUND: Primary malignant tumors of the pericardium are rare, and most primary malignant pericardium tumors are mesotheliomas. Primary pericardial angiosarcoma is extremely rare, and it is associated with a poor prognosis. CASE PRESENTATION: We report of a 47-year-old woman who complained of activity-related chest tightness and shortness of breath. Computed tomography, magnetic resonance imaging, and transesophageal echocardiography revealed an enlarged pericardium with hematic and solid components. An exploratory pericardiotomy was performed, and the results of the histological examination were suggestive of spindle cell hemangioendothelioma. She survived for 9 months after surgery without chemotherapy and radiotherapy, and she had a relatively good quality of life. CONCLUSION: Primary pericardial angiosarcoma is difficult to diagnose, and it has a poor prognosis. Pericardiotomy, radiation therapy, and chemotherapy were associated with a prolongation of survival.

[Cannulated screw fixation through posteromedial approach screw for the treatment of tibial avulsion fracture of the tibial attachment of the posterior cruciate ligament].

OBJECTIVE: To explore therapeutic effects of cannulated screw fixation through posteromedial approach for the treatment of tibial avulsion fracture of the tibial attachment of the posterior cruciate ligament. METHODS: From February 2007 to March 2012, 36 patients with tibial avulsion fracture of the tibial attachment of the posterior cruciate ligament were reviewed. Among the patients, 28 patients were male and 8 patients were female, ranging in age from 16 to 57 years old,averaged 35 years old. All fractures were fresh closed fracture. The posterior drawer test and heavy sign were positive in all patients. AP X-ray of knee joints of all patients showed avulsion fracture flaps at the posterior tibia platform. All the patients were treated with cannulated screw fixation through posteromedial approach, and function exercise performed after operation. The Lysholm criterion was used to evaluate clinical effects. RESULTS: All the patients had successful operation, and were followed up. The duration of follow-up ranged from 6 to 36 months, with an average of 12 months. The fractures healed at 3 months after operation. According to Lysholm knee joint evaluation criteria, 30 patients got an excellent result, 4 good and 2 poor,the average score was 92.2 +/- 3.8. CONCLUSION: Cannulated screw fixation through posteromedial approach for the treatment of tibial avulsion fracture of the tibial attachment of the posterior cruciate ligament is effective to recover joint function to large extent, which is a safe, simple and effective surgical method.

In vivo and in vitro imaging tracing of dual-labeled bone mesenchymal stem cells transplanted into myocardium of F344 rats.

OBJECTIVE: To investigate the feasibility of in vitro and in vivo magnetic resonance imaging (MRI) and fluorescence imaging tracking of transplanted bone mesenchymal stem cells (BMSCs) dual-labeled with ultrasmall superparamagnetic iron oxide (USPIO) and red fluorescence protein (RFP). METHODS: BMSCs were incubated with culture medium containing USPIO for 24 hours. The Prussian-blue staining, transmission electron microscopy and trypan-blue staining were used to study the efficacy and safety of labeling. F344 rat model of acute myocardial infarction was established by ligating the left anterior descending coronary artery. The dual-labeled BMSCs were injected into the margin of the infraction myocardium. Then MRI and fluorescence imaging were performed to trace the cells both in vitro and in vivo. Postmortal study was carried out to observe the distribution of transplanted cells in myocardium. RESULTS: The percentage of dual-labeled BMSCs reached 99% after co-incubating with USPIO for 24 hours. USPIO particles were mainly located in lysosomes. As demonstrated by trypan-blue staining, there was no significant deference in viability between labeled and unlabeled groups (P>0.05). All dual-labeled transplanted BMSCs showed a significant decreasing signal on MRI, and the signal intensity changes had no significant difference over 4 weeks (P=0.66). In vitro cell tracing with fluorescence imaging of isolated heart from F344 rats was successful,while in vivo cell tracing with fluorescence imaging failed. Prussian blue staining showed that USPIO distributed near the infarcted myocardium, corresponding with the fluorescence imaging. CONCLUSION: MRI can be used to trace the dual-labeled BMSCs transplanted into F344 rat hearts in vivo, while fluorescence imaging and pathological fluorescence imaging can trace the transplanted cells in vitro.

Surgical removal of a giant vegetation on permanent endocavitary pacemaker wire and lead / 中国医学科学杂志(英文版)

Pacemaker lead infections are rare. There are only about 0.4%-1.1% of the patients who have been implanted permanent pacemakers suffering from serious infections which lead to endocarditis. Generally, removal of the infected pacemaker wire and lead, long-term anti-infection therapy, and implantation of a new pacemaker to another anatomic site are accepted approaches for these patients.

[Surgical treatment of chronic pulmonary thromboembolism].

OBJECTIVE: To evaluate the perioperative management and safety of pulmonary thromboendarterectomy for chronic pulmonary thromboembolism. METHODS: From March 1999 to March 2004, 12 patients with chronic pulmonary thromboembolism received thromboendarterectomy. The operation was performed under cardio-pulmonary bypass with low flow or circulative arrest and deep hypothermia. The clinical data of the 12 cases were reviewed retrospectively. RESULTS: Pulmonary pressure immediately decreased 20 to 40 mm Hg (1 mm Hg = 0.133 kPa) after operation in 6 cases. Various degrees of postoperative pulmonary edema happened in 8 cases. One patient died at the 19th day after operation due to severe lung infection and pulmonary re-embolism. Eleven patients were followed-up for 2 months to 5 years, the clinical symptom and routine daily activity were improved after surgery. CONCLUSION: Pulmonary thromboendarterectomy is an effective treatment for chronic pulmonary thromboembolism. It is very important to management of postoperative reperfusion injury and pulmonary edema. A key point to enhance the safety of surgical treatment for chronic pulmonary thromboembolism is preoperative correct evaluation and indication selection.