Risk Stratification and Prognosis of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Background:Current guidelines for managing pulmonary arterial hypertension(PAH)recommend a risk strati-fication approach.However,the applicability and accuracy of these strategies for PAH associated with congenital heart disease(PAH-CHD)require further validation.This study aims to validate the reliability and predictive accuracy of a simplified stratification strategy for PAH-CHD patients over a three-year follow-up.Additionally,new prognostic variables are identified and novel risk stratification methods are developed for assessing and managing PAH-CHD patients.Methods:This retrospective study included 126 PAH-CHD patients.Clinical and biochemical variables across risk groups were assessed using Kruskal-Wallis and Fisher’s exact tests.Indepen-dent risk factors were identified using ordered logistic regression,while Kaplan-Meier and Cox proportional hazards regression analyses evaluated their impact on all-cause mortality.A new stratification model for the PAH-CHD population was constructed based on these analyses.Results:Significant survival differences across stratified risk groups were observed(p<0.001),validating the effectiveness of the simplified risk stratification method in PAH-CHD patients.Prothrombin activity was a strong independent predictor of adverse outcomes of PAH-CHD patients(Hazard ratio 0.95,p<0.001,C-index 0.70).A model combining N-terminal pro-brain natriuretic peptide,prothrombin activity,albumin,and right atrial area achieved an area under the curve of 0.89 and a C-index of 0.85.Conclusions:The simplified risk stratification method is applicable to PAH-CHD patients.Prothrombin activity is a strong independent predictor of adverse outcomes.A comprehensive risk stratification approach,incorporating both established and novel biomarkers,enhances accessibility and offers predictive efficacy during follow-up for PAH-CHD patients,comparable to established models.

Treatment of Coronary Heart Disease from the Perspective of Liver Controlling Dispersion

The liver is in charge of distributing and regulating the movement of qi throughout the whole body,coordinating the transportation and transformation of the internal organs in the middle part of the body,promoting the biochemical circulation of qi,blood,and body fluids,and regulating emotions.Liver dysfunction can disrupt the transportation and transformation of qi,blood,and body fluids,causing phlegm turbidity,blood stasis,and other unwanted symptoms.Poor regulation of emotion further aggravates the accumulation of pathological substances,resulting in the obstruction of heart vessels,and ultimately coronary heart disease(CHD).Through regulating lipid metabolism,inflammatory reaction,vasoactive substances,platelet function,neuroendocrine,and other factors,liver controlling dispersing qi plays a comprehensive role in the prognosis of atherosclerosis,the primary cause of CHD.Therefore,it is recommended to treat CHD from the perspective of liver-controlling dispersion.

Salvia Polyphenolates Combined with Doxophylline in the Treatment of Patients with Chronic Pulmonary Heart Disease in the Compensated Stage

Objective:To statistically determine the effect of salvia polyphenolates combined with doxophylline treatment in patients with chronic pulmonary heart disease in the compensated stage.Methods:From January 2023 to January 2024,76 patients with chronic pulmonary heart disease in the compensated stage were selected as research subjects.The patients were divided into a research group and a reference group using a randomized numerical table method.The research group was treated with salvia polyphenolates combined with doxophylline,while the reference group received conventional therapy.The treatment effects of the two groups were compared.Results:The patients in the research group,treated with salvia polyphenolates combined with doxophylline,showed maximal ventilation of 73.26±4.83 L/min,left ventricular ejection fraction of 56.14±1.98%,and total effective treatment rate of 94.74%.These results were better than those of the reference group.The differences between the data of the research group and the reference group were statistically significant(P<0.05).Conclusion:For patients with chronic pulmonary heart disease in the compensated stage,treatment with salvia polyphenolates combined with doxophylline significantly improves maximum ventilation and left ventricular ejection fraction,and also results in a higher total effective treatment rate.

Hemodynamic monitoring in heart failure and pulmonary hypertension: From analog tracings to the digital age

Hemodynamic monitoring has long formed the cornerstone of heart failure(HF) and pulmonary hypertension diagnosis and management. We review the long history of invasive hemodynamic monitors initially using pulmonary artery(PA) pressure catheters in the hospital setting, to evaluating the utility of a number of implantable devices that can allow for ambulatory determination of intracardiac pressures. Although the use of indwelling PA catheters has fallen out of favor in a number of settings, implantable devices have afforded clinicians an opportunity for objective determination of a patient's volume status and pulmonary pressures. Some devices, such as the CardioM EMS and thoracic impedance monitors present as part of implantable cardiac defibrillators, are supported by a body of evidence which show the potential to reduce HF related morbidity and have received regulatory approval, whereas other devices have failed to show benefit and, in some cases, harm. Clearly these devices can convey a considerable amount of information and clinicians should start to familiarize themselves with their use and expect further development and refinement in the future.

Treatment with neurohormonal inhibitors and prognostic outcome in pulmonary arterial hypertension with risk factors for left heart disease

BACKGROUND Despite major advances in pharmacologic treatment,patients with pulmonary arterial hypertension(PAH)still have a considerably reduced life expectancy.In this context,chronic hyperactivity of the neurohormonal axis has been shown to be detrimental in PAH,thus providing novel insights on the role of neurohormonal blockade as a potential therapeutic target.AIM To evaluate the application and prognostic effect of neurohormonal inhibitors(NEUi)in a single-center sample of patients with idiopathic PAH and risk factors for left heart disease.METHODS We analyzed data retrospectively collected from our register of right heart catheterizations performed consecutively from January 1,2005 to October 31,2018.Patients on beta-blocker,angiotensin-converting enzyme inhibitor,angiotensin receptor blocker or mineralocorticoid receptor antagonist at the time of right heart catheterization were classified as NEUi users and compared to NEUi nonrecipients.RESULTS Complete data were available for 57 PAH subjects:27 of those(47.4%)were taking at least one NEUi at the time of right heart catheterization and were compared with the remaining 36 NEUi non-recipients.NEUi users were older and had a higher cardiovascular risk profile compared to non-recipients.Additionally,NEUi non-users had a higher probability of dying during the course of follow-up than NEUi recipients(56.7%vs 25.9%,log-rank P=0.020).CONCLUSION The above data highlighted a subgroup of patients with PAH and comorbidities for left heart disease in which NEUi use has shown to be associated with improved survival.Future prospective studies are needed to identify the most appropriate therapeutic strategies in this subset population.

Treatment of patients with bosentan in postoperation of congenital heart disease with pulmonary arterial hypertension:a double-blind,randomized controlled trial

Objective Endothelin is a key role in the pathogenic of pulmonary arterial hypertension. High concentrations of endothelin 1 have been recorded in plasma and lungs of patients with pulmonary artery hypertension associated with congenital heart disease,and the concentrations of endothelin-1 was correlated with severity degree

Development of pulmonary hypertension remains a major hurdle to corrective surgery in Down syndrome

Down syndrome is the most common chromosomal abnormality encountered in clinical practice with 50%of them having associated congenital heart disease(CHD).Shunt lesions account for around 75%of all CHDs in Down syndrome.Down syndrome patients,especially with large shunts are particularly predisposed to early development of severe pulmonary hypertension(PH)compared with shunt lesions in general population.This necessitates timely surgical correction which remains the only viable option to prevent long term morbidity and mortality.However,despite clear recommendations,there is wide gap between actual practice and fear of underlying PH which often leads to surgical refusals in Down syndrome even when the shunt is reversible.Another peculiarity is that Down syndrome patients can develop PH even after successful correction of shunt.It is not uncommon to come across Down syndrome patients with uncorrected shunts in adulthood with irreversible PH at which stage intracardiac repair is contraindicated and the only option available is a combined heartlung transplant.However,despite the guidelines laid by authorities,the rates of cardiac transplant in adult Down syndrome remain dismal largely attributable to the high prevalence of intellectual disability in them.The index case presents a real-world scenario highlighting the impact of severe PH on treatment strategies and discrimination driven by the fear of worse outcomes in these patients.

Managing end-stage carcinoid heart disease:A case report and literature review

BACKGROUND Gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs)are rare tumors,often diagnosed in an advanced stage when curative treatment is impossible and grueling symptoms related to vasoactive substance release by tumor cells affect patients’quality of life.Cardiovascular complications of GEP-NENs,primarily tricuspid and pulmonary valve disease,and right-sided heart failure,are the leading cause of death,even compared to metastatic disease.CASE SUMMARY We present a case of a 35-year-old patient with progressive dyspnea,back pain,polyneuropathic leg pain,and nocturnal diarrhea lasting for a decade before the diagnosis of neuroendocrine carcinoma of unknown primary with extensive liver metastases.During the initial presentation,serum biomarkers were not evaluated,and the patient received five cycles of doxorubicin,which he did not tolerate well,so he refused further therapy and was lost to follow-up.After 10 years,he presented to the emergency room with signs and symptoms of right-sided heart failure.Panneuroendocrine markers,serum chromogranin A,and urinary 5-hydroxyindoleacetic acid were extremely elevated(900 ng/mL and 2178µmol/L),and transabdominal ultrasound confirmed hepatic metastases.Computed tomo-graphy(CT)showed liver metastases up to 6 cm in diameter and metastases in mesenteric lymph nodes and pelvis.Furthermore,an Octreoscan showed lesions in the heart,thoracic spine,duodenum,and ascendent colon.A standard trans-thoracic echocardiogram confirmed findings of carcinoid heart disease.The patient was not a candidate for valve replacement.He started octreotide acetate treatment,and the dose escalated to 80 mg IM monthly.Although biochemical CONCLUSION Carcinoid heart disease occurs with carcinoid syndrome related to advanced neuroendocrine tumors,usually with liver metastases,which manifests as right-sided heart valve dysfunction leading to right-sided heart failure.Carcinoid heart disease and tumor burden are major prognostic factors of poor survival.Therefore,they must be actively sought by available biochemical markers and imaging techniques.Moreover,imaging techniques aiding tumor detection and staging,somatostatin receptor positron emission tomography/CT,and CT or magnetic resonance imaging,should be performed at the time of diagnosis and in 3-to 6-mo intervals to determine tumor growth rate and assess the possibility of locoregional therapy and/or palliative surgery.Valve replacement at the onset of symptoms or right ventricular dysfunction may be considered,while any delay can worsen right-sided ventricular failure.

Organizing pneumonia secondary to pulmonary tuberculosis:A case report

BACKGROUND Organizing pneumonia secondary to pulmonary tuberculosis is rare.Moreover,the temporal boundary between pulmonary tuberculosis and secondary organizing pneumonia has not been defined.We report a case of secondary organizing pneumonia associated with pulmonary tuberculosis occurring after nine months of antituberculosis treatment.CASE SUMMARY A 54 years old man,previously diagnosed with pulmonary tuberculosis and tuberculous pleurisy,underwent nine months of antituberculosis treatment.Follow-up lung computed tomography revealed multiple new subpleural groundglass opacities in both lungs,and a lung biopsy confirmed organizing pneumonia.Treatment continued with anti-tuberculosis agents and hormone therapy,and subsequent dynamic pulmonary computed tomography exams demonstrated improvement in lesion absorption.No disease recurrence was observed after corticosteroid therapy discontinuation.CONCLUSION When treating patients with active pulmonary tuberculosis,if an increase in lesions is observed during anti-tuberculosis treatment,it is necessary to consider the possibility of tuberculosis-related secondary organizing pneumonia,timely lung biopsy is essential for early intervention.

The Application Effect of Predictive Nursing on Cardiopulmonary Rehabilitation of Patients Undergoing Heart Valve Surgery with Extracorporeal Circulation

Objective:To evaluate the application effect of predictive nursing on patients undergoing heart valve surgery with extracorporeal circulation(ECC).Methods:92 ECC patients admitted to the hospital between July 2021 and July 2023 were selected and grouped by random number table method;the observation group practiced predictive nursing,while the reference group practiced conventional nursing.The cardiopulmonary rehabilitation and other indexes were compared between the groups.Results:The postoperative rehabilitation time of the observation group was shorter than that of the reference group,the treatment compliance was higher than that of the reference group,the cardiopulmonary function indexes were all better than that of the reference group,and the complication rate was lower than that of the reference group(P<0.05).Conclusion:The implementation of predictive nursing for ECC patients can promote postoperative rehabilitation,improve patients’treatment compliance,and enhance the cardiopulmonary rehabilitation effect,and nursing safety is high.

Twin pregnancy with sudden heart failure and pulmonary hypertension after atrial septal defect repair: A case report

BACKGROUND Pulmonary arterial hypertension(PAH)in pregnancy is one of the major obstetric complications and is considered a contraindication to pregnancy as it is classified as a class IV risk in the revised risk classification of pregnancy by the World Health Organisation.Pregnancy,with its adaptive and expectant mechanical and hormonal changes,negatively affects the cardiopulmonary circulation in pregnant women.Do patients with repaired simple congenital heart disease(CHD)develop other pulmonary and cardiac complications during pregnancy?Can pregnant women with sudden pulmonary hypertension be treated and managed in time?In this paper,we present a case of a 39-year-old woman who underwent cesarean section at 33 wk'gestation and developed PAH secondary to repaired simple CHD.Our research began by a PubMed search for"pulmonary hypertension"and"pregnancy"and"CHD"case reports.Three cases were selected to review PAH in pregnancy after correction of CHD defects.These studies were reviewed,coupled with our own clinical experience.CASE SUMMARY Herein,a case involving a woman who underwent atrial septal defect repair at the age of 34,became pregnant five years later,and had a sudden onset of PAH and right heart failure secondary to symptoms of acute peripheral edema in the third trimester of her pregnancy.As a result,the patient underwent a cesarean section and gave birth to healthy twins.Within three days after cesarean delivery,her cardiac function deteriorated as the pulmonary artery pressure increased.Effec-tive postpartum management,including diuresis,significant oxygen uptake,vasodilators,capacity and anticoagulants management,led to improvements in cardiac function and oxygenation.The patient was discharged from hospital with a stable recovery and transferred to local hospitals for further PAH treatment.CONCLUSION This case served as a reminder to obstetricians of the importance of pregnancy after repair of CHD.It is crucial for patients with CHD to receive early correction.It suggests doctors should not ignore edema of twin pregnancy.Also,it provides a reference for the further standardization of antenatal,in-trapartum and postpartum management for patients with CHD worldwide.

Epidemiology and Management of Heart Failure in the Medicine Department of Tombouctou Hospital

Introduction: Heart failure is a complex syndrome and very frequently observed and secondary to the outcome of many advanced heart diseases. It is made up of a set of symptoms that reflect the tissue and visceral consequences of heart failure. The objective was to determine the hospital prevalence and to describe the management of heart failure in the medical department of Tombouctou hospital in Mali. Methods: This is a descriptive cross-sectional study with retrospective data collection, carried out over 24 months and involving the analysis of 266 records of patients hospitalized for heart failure. Results: The frequency of heart failure was 17.6% with a female predominance. The average age was 48 years with extremes of 16 and 90 years. Cardiovascular risk factors were dominated by high blood pressure with 38%. Global heart failure was the mode of discovery in 72.2% of cases. The etiologies were dominated by hypertensive dilated cardiomyopathy in 36.8% and peripartum cardiomyopathy in 32% of cases. The majority of our patients benefited from diuretic treatment, i.e. 95.9% frequency;with a favorable evolution in 96.2% of cases. We recorded 10 deaths, i.e. a lethality of 3.7%. We report some difficulties encountered during our security study because Tombouctou is a war zone and the lack of a technical platform for biology such as (BNP or NT-proBNP). Conclusion: Heart failure is a real public health problem. Its prevalence is increasing due to the aging of the population and especially the poor management of arterial hypertension in our context.

Pancytopenia and Pulmonary Tuberculosis: A Case Report

Background: Hematopoietic system is seriously affected by tuberculosis. It exerts a dazzling variety of hematological effects involving both cell lines and plasma components [1]. Anemia and leukopenia are not unusual with tuberculosis (TB), but pancytopenia is rare [2]. Findings: In this report, we described a case of a 42 years man presenting bleeding and pancytopenia;bacteriological pulmonary TB was established by genotypic rapid test and treatment following the WHO guidelines on drug-sensitive TB treatment. Patient recovered entirely with the WHO recommended regimen associated with general and local treatment of the bleeding. Conclusion: This case report emphasizes the importance of always suspecting tuberculosis in a tuberculosis-endemic area, even when the clinical manifestations are atypical, like pancytopenia and also of properly investigating the differential diagnosis. Even though prognosis seems to be less good, actual treatment regimen is still effective.

Assessment of Intracardiac and Extracardiac Deformities in Patients with Various Types of Pulmonary Atresia by Dual-Source Computed Tomography

Background:Pulmonary atresia(PA)is a group of heterogeneous complex congenital heart disease.Only one study modality might not get a correct diagnosis.This study aims to investigate the diagnostic power of dualsource computed tomography(DSCT)for all intracardiac and extracardiac deformities in patients with PA compared with transthoracic echocardiography(TTE).Materials and Methods:This retrospective study enrolled 79 patients and divided them into three groups according to their main diagnosis.All associated malformations and clinical information,including treatments,were recorded and compared among the three groups.The diagnostic power of DSCT and TTE on all associated malformations were compared.The surgical index(McGoon ratio,pulmonary arterials index(PAI),and total neopulmonary arterial index)and radiation dose were calculated on the basis of DSCT.Results:Of the patients,32,30,and 17 were divided into the groups of PA with ventricular septal defect(VSD),PA with VSD and major aortopulmonary collateral arteries,and PA with other major malformations,respectively.Consequently,182,162,and 13 intracardiac,extracardiac,and other major malformations were diagnosed,respectively.Moreover,DSCT showed a better diagnostic performance in extracardiac deformities(154 vs.117,p<0.001),whereas TTE could diagnose intracardiac deformities better(159 vs.139,p=0.001).The McGoon ratio,PAI,and treatment methods were significantly different among the three groups(p=0.014,p=0.008,and p=0.018,respectively).Conclusion:More than one imaging modality should be used to make a correct diagnosis when clinically suspecting PA.DSCT is superior to TTE in diagnosing extracardiac deformities and could be used to roughly calculate surgical indices to optimize treatment strategy.

Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction:A Rare Case Report

Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in TOF.We present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified MAPCAs.She underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO.Echocardiographicfindings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the diagnosis.It was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory outcome.Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature.Furthermore,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.

General awareness of symptoms of myocardial infarction and the need for urgent treatment:A cross-sectional,street survey in Chennai,Tamil Nadu

Objective:To investigate the golden-time-awareness of and the necessary actions in response to acute myocardial infarction among the general public.Methods:This study was conducted with the use of a descriptive research design and convenience sampling.A sample of 800 subjects,using self-structured knowledge questionnaires,was selected for data collection and analysis.The study was conducted at Dr.M.G.R.Educational and Research Institute,India,from February to November 2022.Awareness of myocardial infarction symptoms and intervention timeline(the golden time)was investigated.A multivariate logistic regression analysis was performed to identify the demographic factors affecting the recognition of the golden time of acute myocardial infarction.Results:A total of 800 subjects were included.Among the subjects,367(45.8%)were male,433(54.2%)were female,and 443(55.3%)participants failed to recognize the golden time.Our multivariate logistic regression analysis showed that people with a bachelor’s degree(OR=0.41,95%CI:0.23-0.74,P=0.03)and a high school level diploma(OR=0.55,95%CI:0.31-0.97,P=0.03)are more likely to know about myocardial infarction(golden time)than illiterate people.Additionally,people who are employed(OR=0.59,95%CI:0.41-0.85,P=0.05)are more likely to know about myocardial infarction(golden time)than those who are not.Conclusions:The majority of people in the awareness group realize that taking myocardial infarction patients to the hospital would be the best course of action.The unawareness of the ideal treatment window for myocardial infarction may cause a delay in seeking medical attention,which can lead to an increase in mortality and morbidity.

Pulmonary arterial hypertension confirmed by right heart catheterization following COVID-19 pneumonia: A case report and review of literature

BACKGROUND Pulmonary arterial hypertension(PAH)is a disease of the arterioles resulting in an increased resistance in pulmonary circulation with associated high pressures in the pulmonary arteries,causing irreversible remodeling of the pulmonary arterial walls.Coronavirus disease 2019(COVID-19)has been associated with development of new onset PAH in the literature leading to symptoms of dyspnea,cough and fatigue that persist in spite of resolution of acute COVID-19 infection.However,the majority of these cases of COVID related PAH were diagnosed using echocardiographic data or via right heart catheterization in mechanically ventilated patients.CASE SUMMARY Our case is the first reported case of COVID related PAH diagnosed by right heart catheterization in a non-mechanically ventilated patient.Right heart catheterization has been the gold standard for diagnosis of pulmonary hypertension.Our patient had right heart catheterization four months after her initial COVID-19 infection due to persistent dyspnea.CONCLUSION This revealed new onset PAH that developed following her infection with COVID-19,an emerging sequela of the infection.

Evaluating the Effectiveness of Emergency Ventilator Treatment for Severe Acute Left Ventricular Heart Failure

Objective:To evaluate the efficacy of emergency ventilator therapy in severe acute left heart failure.Methods:A total of 75 patients with severe acute left ventricular heart failure who were admitted to the hospital from July 2020 to July 2023 were randomly divided into two groups.Group A received additional emergency ventilator treatment,and group B received conventional treatment.The efficacy was compared.Results:The curative effect of patients with severe acute left heart failure in group A was higher than that in group B(P<0.05);all blood gas indicators in group A were better than those in group B(P<0.05);all vital signs indicators in group A were better than those in group B(P<0.05);group A was more satisfied with the treatment of severe acute left ventricular heart failure than group B(P<0.05).Conclusion:Patients with severe acute left heart failure who receive emergency ventilator treatment can stabilize vital signs,improve blood oxygen supply,and enhance curative effect.

Diagnosis and treatment of acute pulmonary inflammation in critically ill patients: The role of inflammatory biomarkers

Pneumonia and acute respiratory distress syndrome are common and important causes of respiratory failure in the intensive care unit with a significant impact on morbidity, mortality and health care utilization despite early antimicrobial therapy and lung protective mechanical ventilation. Both clinical entities are characterized by acute pulmonary inflammation in response to direct or indirect lung injury. Adjunct anti-inflammatory treatment with corticosteroids is increasingly used, although the evidence for benefit is limited. The treatment decisions are based on radiographic, clinical and physiological variables without regards to inflammatory state. Current evidence suggests a role of biomarkers for the assessment of severity, and distinguishing sub-phenotypes (hyperinflammatory versus hypo-inflammatory) with important prognostic and therapeutic implications. Although many inflammatory biomarkers have been studied the most common and of interest are C-reactive protein, procalcitonin, and pro-inflammatory cytokines including interleukin 6. While extensively studied as prognostic tools (prognostic enrichment), limited data are available for the role of biomarkers in determining appropriate initiation, timing and dosing of adjunct anti-inflammatory treatment (predictive enrichment)

Diagnosis and surgical treatment of intraveneous leiomyomatosis extending into the heart:two cases report and review of the literature

Objective： To investigate the clinical characteristics, diagnosis and surgical treatment of intravenous leiomyomatosis （IVL）, and outline the differences between Chinese and overseas cases. Methods： Clinical data of two IVL cases, treated in our hospital, were analyzed retrospectively and the related literature was also reviewed. The data of preoperative diagnostic rate, surgical procedures, and postoperative recurrence between patients in China and other countries were compared. Results： The first stage operation was performed successfully in 2 patients. However, they refused subsequent therapies, including a second stage operation to excise the remnants Of the tumor, uterus, bilateral oviducts and ovaries, and anti-estrogen therapy. Both suffered from IVL recurrence, one at 6-month and the other at 9-month, and died at 16-month and 12-month respectively after the first stage surgery. Worldwide reports of 110 IVL cases were reviewed, which included 28 cases in China and 82 cases in other countries. In the majority of the Chinese patients, tumors only extended into the fight atrium rather than the right ventricle （RA 22 cases vs RV 4 cases）. However, among the overseas patients, the rate of extension into the right atrium was similar to that of extension into the right ventricle （RA 41 cases vs RV 38 cases）. The rate of hysterectomies was not significantly different between Chinese and overseas patients （ 67.86% vs 55.9%, P=0.278）. The rate of correct preoperative diagnosis in the Chinese patients was significantly lower than that in the overseas patients （32.14% vs 80%, P=0.000002.）, as well asthe rate of complete excision of the tumor （22.7@0 vs 75.5%, P=0.000001）. The proportion of patients who undergoing a single-stage or a two-stage operation was similar in Chinese and overseas patients. The recurrence rate was significantly higher in the Chinese patients than in the overseas patients （36.8% vs 9.1%, P=0.0055）, and the patients with tumor recurrence were partial tumor excision patients. Conclusion： The possibility of IVL should be considered if echocardiography in female patients demonstrates a tumor in the right heart and a mass in the inferior vena cava （IVC）. Further imaging should be performed to confirm the diagnosis. The correct diagnosis and accurate preoperative delineation of tumor extension are essential for an optimal surgical outcome. The key point in IVL treatment is the complete excision of tumors （single-stage or two-stage surgical procedure）.