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.

Replacement Therapy for Hemophilia Patients Undergoing Cardiac Surgery: Report of Three Cases.

Hemophilia is an X-linked recessive inherited bleeding disorder. Despite the improved treatment in recent years with the advent of replacement therapies, the progression of atherosclerosis is not slowed down after the reduction of clotting factors in hemophilia. As life expectancy increases, more hemophilia patients will suffer from age-related cardiovascular diseases. Since cardiac surgery needs heparinization and cardiopulmonary bypass (CPB), it is extremely challenging to balance hemostasis and coagulation in patients with hemophilia. Here we report three cases of hemophilia patients who underwent cardiac surgery successfully.

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 of active native mitral infective endocarditis: A meta-analysis of current evidence.

BACKGROUND: The native mitral lesion of active infective endocarditis implies a poor prognosis and is associated with adverse short- or long-term results without surgical treatment. Both mitral valvuloplasty (MVP) and mitral valve replacement (MVR) have been performed in the treatment of active native mitral infective endocarditis (ANMIE). However, the outcomes of the two approaches remain unclear. The aim of this study was to systematically review the two procedures with mortality and survival as the primary endpoints. METHODS: A systematic review of the literature was conducted to identify all relevant studies with comparative data on MVP versus MVR for the treatment of ANMIE. Information on baseline characteristics of patients, operation method, quality of literature, follow-up, and so forth was abstracted using standardized protocols. Pooled odds ratio (OR) or hazard ratio (HR) was calculated and possible publication bias was tested. RESULTS: Nine comparative observational studies with a total of 633 patients (MVP = 265, MVR = 368) were identified for qualitative assessment, data extraction, and analysis. The summary OR for operative mortality, comparing repair with replacement, was 0.37 (95% CI 0.0.18-0.80; p = 0.0005). Summary 1- and 5-year HRs for event-free survival were 0.43 (95% CI 0.20-0.92; p = 0.03) and 0.44 (95% CI 0.25-0.77, p = 0.004), respectively (repair vs. replacement). Summary 1- and 5-year survival HRs were 0.51 (95% CI 0.24-1.08; p = 0.08) and 0.55 (95% CI 0.32-0.96; p = 0.004), respectively (repair vs. replacement). No heterogeneity was revealed between studies, and possible publication bias was insignificant. CONCLUSIONS: This meta-analysis suggests that MVP may be associated with superior postoperative survival outcomes compared with MVR. MVP is desirable, if possible, as a durable alternative to replacement. However, we must consider the influence of different patient characteristics and surgeons' preferences on the choice of surgical approach, and additional powered clinical trials will be required to confirm these findings.

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.

[Infective endocarditis in patients with hypertrophic obstructive cardiomyopathy: five cases report].

OBJECTIVE: To analyze the clinical characteristics of infective endocarditis in patients with hypertrophic obstructive cardiomyopathy. METHODS: Clinical characteristics from 5 patients with infective endocarditis and hypertrophic obstructive cardiomyopathy hospitalized from January 2000 to December 2010 in our hospital were analyzed. RESULTS: Four patients were diagnosed with left ventricular outflow tract obstructive cardiomyopathy with outflow pressure gradient from 36 to 140 mm Hg (1 mm Hg = 0.133 kPa) and left atrial size 44 - 68 mm. Another patient was diagnosed as ventricular hypertrophic cardiomyopathy with significant right-ventricular outflow tract hypertrophy (30 mm), high pressure gradient (164 mm Hg) and enlarged right atrial (56 mm × 53 mm), there was a 17 mm × 8 mm vegetation on right-ventricular outflow tract in this patient. Blood cultures were positive for streptococcus viridans in all five patients, and enterococcus faecium was revealed in one aortic valve vegetation culture. Transthoracic echocardiogram was performed 2 - 4 times for each patient, the vegetations of two patients was detected only by transesophageal echocardiography. The mitral valve vegetation was detected in two patients, the aortic and mitral valve vegetations were detected in one patients, mitral and tricuspid vegetations in one patient and right ventricular outflow tract vegetation in one patient. The four hemodynamically stable patients were successfully treated with antibiotic therapy, one patient received urgent surgery (replacement of the aortic and mitral valve as well as septal myectomy). All patients recovered and follow-up (1 - 6 years) was available in 4 patients and no complication was observed. CONCLUSION: The risk of infective endocarditis complicating hypertrophic obstructive cardiomyopathy is the highest in patients with both outflow obstruction and marked valve insufficiency, these patients should receive prophylactic antibiotic therapy during procedures that predispose to infective endocarditis.

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 primary malignant tumors of the trachea and main bronchus].

OBJECTIVE: To explore the clinical experience of surgical treatment of primary malignant tumors of the trachea and main bronchus. METHODS: The clinicopathological data of 18 patients with primary malignant tumors of the trachea and main bronchus surgically treated from February 1994 to August 2007 were reviewed retrospectively. The surgical management included sleeve tracheal resection in 8 cases, lower trachea and carina resection with carina reconstruction in 4 cases, local enucleation of the tumor in 4 cases, left or right carino-pneumonectomy and carina reconstruction in 2 cases, and resection of the tracheal or bronchial tumor and reconstruction of the airway under cardiopulmonary bypass in 6 cases. RESULTS: Among the 18 cases, there were 7 adenoid cystic carcinomas, 9 squamous cell carcinomas, 1 lymphoepithelial-like carcinoma and 1 follicular non-Hodgkin lymphoma. All the cases recovered well except one who died of endotracheal bleeding and asphyxia at the 10(th) postoperative day. CONCLUSION: Surgical resection is the most effective treatment for primary malignant tumors of the trachea and main bronchus. The selection of operation modes should be individualized according to patients' condition. Both complete resection and safety should be taken into consideration simultaneously.

[Perioperative management for primary tracheal malignant tumors resected under cardiopulmonary bypass].

OBJECTIVE: To retrospectively review the perioperative management for primary tracheal malignant tumors resected under cardiopulmonary bypass. METHODS: The data of 6 patients with primary tracheal malignant tumors who underwent surgery under cardiopulmonary bypass from December 1999 to August 2003 were reviewed. Cardiopulmonary bypass was established through right femoral vessels in 2 patients for emergency operation, through right atrium and ascending aorta in 4 patients. Sleeve tracheal resections in 3 patients, carinal resections and carina reconstructions in 2, and local enucleation in 1 were performed. Respiratory airway was kept patent by coughing and expectorating sputum. RESULTS: All patients' dyspnea were relieved remarkably. The postoperative mechanic ventilation assistance lasted from 10 hours to 7 days. There was no perioperative mortality. CONCLUSION: Resection of primary tracheal malignant tumors with severe tracheal obstruction under cardiopulmonary bypass is practicable. Keeping respiratory airway patent perioperatively is very important and helpful to postoperative recovery.

Thromboendarterectomy for chronic pulmonary thromboembolism.

OBJECTIVE: To evaluate the improving reliability and safety of thromboendarterectomy and perioperative management for chronic pulmonary thromboembolism. METHODS: The clinical data of 12 cases with chronic pulmonary thromboembolism, who underwent thromboendarterectomy assisted by low flow or circulation arrest with deep hypothermia, were reviewed retrospectively. RESULTS: Pulmonary artery pressure decreased 20 to 40 mmHg immediately after surgical procedures in 9 cases. The postoperative pulmonary edema at various degrees happened in 12 cases, among them, 1 died of severe lung infection and pulmonary re-embolism at 19 days postoperation. Computed tomography pulmonary angiography and angiography of 11 cases indicated that the original obstruction of pulmonary artery disappeared. During the follow-up period of 2 months to 5 years, the clinical symptoms and activity was improved. CONCLUSION: Thromboendarterectomy is an effective treatment for chronic pulmonary thromboembolism. The outcome of the surgical procedure needs to be further investigated and followed up regularly according to an evaluative system, because it might be influenced by multiple factors.

[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.