Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure r...Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure refractory to standard therapies. Adult patients with congenital heart disease are an increasingly recognized and growing population and include various groups, such as undiagnosed cases in childhood and palliated and/or corrected cases, which require subsequent care because of residual lesions, cardiac arrest/insufficiency, and arrhythmias, among other conditions. In addition, these patients are prone to developing pathologies that are typical of adulthood with a generally increased risk of morbidity and mortality because of their low reserves and organic damage associated with the underlying heart disease, which makes them candidates for ECMO. These patients represent an additional challenge in this therapy because malformations and the presence of a shunt can generally affect the usual cannulation methods and hemodynamic and oximetry monitoring. Thus, the configuration decision must be made on a case-by-case basis. Here, we present a cannulation method, venopulmonary artery ECMO, which provides hemodynamic and respiratory support, and may be ideal for patients with shunts and/or right ventricular dysfunction. To our knowledge, this is the first report of this configuration in patients with congenital heart diseases.展开更多
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...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.展开更多
AIM To find parameters from transthorathic echocardiography(TTE) including speckle-tracking(ST) analysis of the right ventricle(RV) to identify precapillary pulmonary hypertension(PH).METHODS Forty-four patients with ...AIM To find parameters from transthorathic echocardiography(TTE) including speckle-tracking(ST) analysis of the right ventricle(RV) to identify precapillary pulmonary hypertension(PH).METHODS Forty-four patients with suspected PH undergoing right heart catheterization(RHC) were consecutively included(mean age 63.1 ± 14 years, 61% male gender). All patients underwent standardized TTE including ST analysis of the RV. Based on the subsequent TTE-derived measurements, the presence of PH was assessed: Left ventricular ejection fraction(LVEF) was calculated by Simpsons rule from 4Ch. Systolic pulmonary artery pressure(s PAP) was assessed with continuous wave Doppler of systolic tricuspid regurgitant velocity and regarded raised with values ≥ 30 mmH g as a surrogate parameter for RA pressure. A concomitantly elevated PCWP was considered a means to discriminate between the precapillary and postcapillary form of PH. PCWP was considered elevated when the E/e' ratio was > 12 as a surrogate for LV diastolic pressure. E/e' ratio was measured by gauging systolic and diastolic velocities of the lateral and septal mitral valve annulus using TDI mode. The results were then averaged with conventional measurement of mitral valve inflow. Furthermore, functional testing with six minutes walking distance(6MWD), ECG-RV stress signs, NT pro-BNP and other laboratory values were assessed.RESULTS PH was confirmed in 34 patients(precapillary PH, n = 15, postcapillary PH, n = 19). TTE showed significant differences in E/e' ratio(precapillary PH: 12.3 ± 4.4, postcapillary PH: 17.3 ± 10.3, no PH: 12.1 ± 4.5, P = 0.02), LV volumes(ESV: 25.0 ± 15.0 mL, 49.9 ± 29.5 m L, 32.2 ± 13.6 m L, P = 0.027; EDV: 73.6 ± 24.0 mL, 110.6 ± 31.8 mL, 87.8 ± 33.0 mL, P = 0.021) and systolic pulmonary arterial pressure(sP AP: 61.2 ± 22.3 mm Hg, 53.6 ± 20.1 mm Hg, 31.2 ± 24.6 mm Hg, P = 0.001). STRV analysis showed significant differences for apical RV longitudinal strain(RVAS: -7.5% ± 5.6%, -13.3% ± 4.3%, -14.3% ± 6.3%, P = 0.03). NT pro-BNP was higher in patients with postcapillary PH(4677.0 ± 7764.1 pg/m L, precapillary PH: 1980.3 ± 3432.1 pg/mL, no PH: 367.5 ± 420.4 pg/mL, P = 0.03). Patients with precapillary PH presented significantly more often with ECG RV-stress signs(P = 0.001). Receiver operating characteristics curve analyses displayed the most significant area under the curve(AUC) for RVAS(cut-off < -6.5%, AUC 0.91, P < 0.001), sPAP(cut-off > 33 mmH g, AUC 0.86, P < 0.001) and ECG RV stress signs(AUC 0.83, P < 0.001). The combination of these parameters had a sensitivity of 82.8% and a specificity of 17.2% to detect precapillary PH. CONCLUSION The combination of non-invasive measurements allows feasible assessment of PH and seems beneficial for the differentiation between the pre- and postcapillary form of this disease.展开更多
Right ventricular failure(RVF)remains the primary cause of death in patients with pulmonary arterial hypertension.We review the pathophysiology of RVF,including the remodeling and ventriculoarterial uncoupling that oc...Right ventricular failure(RVF)remains the primary cause of death in patients with pulmonary arterial hypertension.We review the pathophysiology of RVF,including the remodeling and ventriculoarterial uncoupling that occurs when the failing right side of the heart is unable to compensate for a rising afterload.Secondly,the noninvasive imaging techniques used in the assessment of RVF are explored,including echocardiography,cardiac magnetic resonance imaging,computed tomography,and positron emission tomography.Third,we describe how these imaging techniques and a patient’s clinical characteristics may be used to determine prognosis.Lastly,we explore the medical and surgical/interventional treatment options for RVF.Despite these treatment options,morbidity and mortality remain high in this patient population.The discovery of new prognostic indicators,use of hybrid imaging for early detection of RVF,and strategies to prevent the development of RVF will be important if outcomes in this patient population are to improve.展开更多
Objective: The aim of the study was to determine usefulness of isovolumic acceleration to assess right ventricular function in patients with pulmonary hypertension. Background: Right ventricular dysfunction is...Objective: The aim of the study was to determine usefulness of isovolumic acceleration to assess right ventricular function in patients with pulmonary hypertension. Background: Right ventricular dysfunction is a powerful predictor of adverse outcomes in patients with pulmonary hypertension. Although isovolumic acceleration is a sensitive and relatively load-independent parameter for ventricular contractile function, it has not been widely used. So, the aim of the study was to determine the usefulness of isovolumic acceleration in assessing right ventricular function in patients with pulmonary hypertension. Methods: A single center, prospective observational case-control study has been carried out for the period of one year at Osmania general hospital (Hyderabad). A total of 32 consecutive patients with pulmonary hypertension and 32 age matched healthy controls were recruited in the study as per the inclusion and exclusion criteria of the study. Receiver operator characteristic curves were used to study sensitivity, specificity, positive predictive value, negative predictive values as well as pearson’s correlation coefficients. Results: The results of the study showed that isovolumic acceleration has good significant correlation with right ventricular fractional area change (r = 0.554, p = 0.001) and tissue Doppler myocardial performance index (r = -0.599, p = 0.001) whereas a lesser but significant correlation was observed with tricuspid annular plane systolic excursion (r = 0.327, p = 0.034). Isovolumic acceleration showed higher specificity (81%) and positive predictive value (88%) in diagnosing right ventricular dysfunction in pulmonary hypertension compared to tricuspid annular systolic excursion velocity and tissue Doppler myocardial performance index parameters. Therefore isovolumic acceleration may be a better variable for diagnosing right ventricular dysfunction. Conclusions: Isovolumic acceleration is a sensitive and specific marker of right ventricular function in pulmonary hypertension.展开更多
Objective:To evaluate the effectiveness of discharge planning on maternal caring knowledge,maternal caring behavior,maternal discharge readiness and the rehospitalization of children with ventricular septal defect and...Objective:To evaluate the effectiveness of discharge planning on maternal caring knowledge,maternal caring behavior,maternal discharge readiness and the rehospitalization of children with ventricular septal defect and pulmonary arterial hypertension(VSD-PAH).Background:Children with congenital heart disease(CHD)with pulmonary arterial hypertension(PAH)have more complications after surgery than those without PAH.Discharge planning is an effective strategy to help children leave the hospital safely,and receive appropriate care after discharge.Methods:A quasi-experimental design was used.Sixty children and their mothers were recruited and divided into two groups:the control group received conventional care,the intervention group received both conventional care and additional discharge planning care.Results:(1)After admission,maternal caring knowledge between the two groups was similar.(2)At discharge,maternal discharge readiness,maternal caring knowledge and maternal caring behavior in the intervention group was significantly higher compared to the control group(t=3.35,p=0.001;F=84.74,p<0.001;F=23.82,p<0.001).This difference persisted after discharge,and was evident at one month and three months after discharge.(3)However,no significant difference in the readmission rate of children after discharge was evident between the two groups.Conclusions:Discharge planning improves the maternal discharge readiness,maternal caring knowledge and maternal caring behaviors.However,this planning did not reduce the readmission rate of children with CHD-PAH.展开更多
Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary emb...Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. The prognostic value of the RV/LV ratio in patients with idiopathic pulmonary arterial hypertension(IPAH) is still unknown. Methods We retrospectively enrolled 95 consecutive patients with newly diagnosed IPAH and 16 of them were reevaluated by echocardiography at 3-12 months following targeted therapy.Follow-up data were obtained by telephone interviews and review of the patients’ records.Results The RV/LV ratio was in parallel with the severity of World Health Orgnization(WHO) functional class and mean right atrial pressure.The RV/LV ratio was positively correlated with total pulmonary resistance(P P P 2 saturation(P P = 0.001),weight and absence of targeted therapy were independent predictors of death.No significant changes in the RV/LV ratio before and after targeted therapy were observed. A baseline RV/LV ratio≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis. Conclusions The RV/LV ratio helps to assess the severity of IPAH and serves as an independent predictor of prognosis in patients with IPAH.展开更多
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 ar...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.展开更多
Background: Right ventricle failure(RVF) is associated with serious cardiac and pulmonary diseases that contribute significantly to the morbidity and mortality of patients. Currently, the mechanisms of RVF are not ful...Background: Right ventricle failure(RVF) is associated with serious cardiac and pulmonary diseases that contribute significantly to the morbidity and mortality of patients. Currently, the mechanisms of RVF are not fully understood and it is partly due to the lack of large animal models in adult RVF. In this study, we aim to establish a model of RVF in adult ovine and examine the structure and function relations in the RV.Methods: RV pressure overload was induced in adult male sheep by revised pulmonary artery constriction(PAC). Briefly, an adjustable hydraulic occluder was placed around the main pulmonary artery trunk. Then, repeated saline injection was performed at weeks 0, 1, and 4, where the amount of saline was determined in an animal-specific manner. Healthy, age-matched male sheep were used as additional controls. Echocardiography was performed bi-weekly and on week 11 post-PAC, hemodynamic and biological measurements were obtained.Results: This PAC methodology resulted in a marked increase in RV systolic pressure and decreases in stroke volume and tricuspid annular plane systolic excursion, indicating signs of RVF. Significant increases in RV chamber size, wall thickness, and Fulton's index were observed. Cardiomyocyte hypertrophy and collagen accumulation(particularly type III collagen) were evident, and these structural changes were correlated with RV dysfunction.Conclusion: In summary, the animal-specific, repeated PAC provided a robust approach to induce adult RVF, and this ovine model will offer a useful tool to study the progression and treatment of adult RVF that is translatable to human diseases.展开更多
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 neurohormon...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.展开更多
Background The goal of this study was to explore the significance of left ventricular(LV)ejection/filling parameters in assessing severity and prognosis of pulmonary arterial hypertension(PAH).Methods Newly diagnosed ...Background The goal of this study was to explore the significance of left ventricular(LV)ejection/filling parameters in assessing severity and prognosis of pulmonary arterial hypertension(PAH).Methods Newly diagnosed PAH patients were recruited between July 2011 and December 2013 prospectively.Cardiopulmonary hemodynamics derived from right heart catheterization,and LV ejection/filling parameters derived from CMR were collected.Follow-up data were collected periodically using a specifically-designed network database until April 30,2016.展开更多
Background: Pulmonary hypertension is defined as a mean arterial pressure in the pulmonary artery exceeding 20 mm Hg at rest, measured by means of right heart catheterization. Patients with pulmonary hypertension unde...Background: Pulmonary hypertension is defined as a mean arterial pressure in the pulmonary artery exceeding 20 mm Hg at rest, measured by means of right heart catheterization. Patients with pulmonary hypertension undergoing surgical procedures such as hemiarthroplasty present a high risk of fatal complications. Nonetheless, there are no widely accepted protocols explaining their perioperative care in great detail. Case presentation: We present a case with an 89-years-old patient, with comorbidities such as GOLD 4D chronic obstructive pulmonary disease (COPD) and, as a consequence of this, severe pulmonary hypertension with signs of right ventricular dysfunction, thus requiring of pulmonary vasodilator, that has suffered a subcapital hip fracture requiring urgent surgery. Surgery is carried out successfully, under regional lumbar-sacral plexus block and sedation assisted by non-invasive ventilation. Conclusion: Multidisciplinary specialized treatment, preoperative optimization, as well as the careful selection of both the surgical and anesthetic techniques to be used, are among the strategies that improve the perioperative outcome in patients with pulmonary hypertension with right ventricle systolic dysfunction. Regional lumbar-sacral plexus block plus sedation is a technique that maintains hemodynamic stability;however, these patients require advance measures and postoperative monitoring under intensive care.展开更多
Right ventricular function is independently associated with poor clinical outcomes in patients with various cardiovascular diseases including pulmonary hypertension(PH).The complex geometry and mode of contractility m...Right ventricular function is independently associated with poor clinical outcomes in patients with various cardiovascular diseases including pulmonary hypertension(PH).The complex geometry and mode of contractility means the right ventricle(RV)inlet and outlet are not in line;hence,2-dimensional echocardiography fails to accurately quantify RV volumes and function in such patients.Three-dimensional echocardiography(3DE)allows for reliable and reproducible quantification of RV volumes and function by overcoming these limitations of conventional echocardiography.This review focuses on the 3DE assessment of RV function in patients with PH and discusses the following points:(1)acquiring an RV data set for 3DE imaging,including details of all available 3DE systems and software utilized in daily practice;(2)the reliability and feasibility of RV remodeling measured with 3DE with different modalities in patients with PH;and(3)the prognostic value of 3DE-derived RV function in such patients.展开更多
Background:The potential impact of pre-existing coronary artery stenosis(CAS)on acute pulmonary embolism(PE)episodes remains underexplored.This study aimed to investigate the association between pre-existing CAS and t...Background:The potential impact of pre-existing coronary artery stenosis(CAS)on acute pulmonary embolism(PE)episodes remains underexplored.This study aimed to investigate the association between pre-existing CAS and the elevation of high-sensitivity cardiac troponin I(hs-cTnI)levels in patients with PE.Methods:In this multicenter,prospective case-control study,88 cases and 163 controls matched for age,sex,and study center were enrolled.Cases were patients with PE with elevated hs-cTnI.Controls were patients with PE with normal hs-cTnI.Coronary artery assessment utilized coronary computed tomographic angiography or invasive coronary angiography.CAS was defined as≥50%stenosis of the lumen diameter in any coronary vessel>2.0 mm in diameter.Conditional logistic regression was used to evaluate the association between CAS and hs-cTnI elevation.Results:The percentage of CAS was higher in the case group compared to the control group(44.3%[39/88]vs.30.1%[49/163];P=0.024).In multivariable conditional logistic regression model 1,CAS(adjusted odds ratio[OR],2.680;95%confidence interval[CI],1.243-5.779),heart rate>75 beats/min(OR,2.306;95%CI,1.056-5.036)and N-terminal pro-B type natriuretic peptide(NT-proBNP)>420 pg/mL(OR,12.169;95%CI,4.792-30.900)were independently associated with elevated hs-cTnI.In model 2,right CAS(OR,3.615;95%CI,1.467-8.909)and NT-proBNP>420 pg/mL(OR,13.890;95%CI,5.288-36.484)were independently associated with elevated hs-cTnI.Conclusions:CAS was independently associated with myocardial injury in patients with PE.Vigilance towards CAS is warranted in patients with PE with elevated cardiac troponin levels.展开更多
Objective:This study aims to investigate the impact of tricuspid regurgitation(TR)severity on the accuracy of echocardiographic estimation of systolic pulmonary arterial pressure(sPAP)in patients with pulmonary arteri...Objective:This study aims to investigate the impact of tricuspid regurgitation(TR)severity on the accuracy of echocardiographic estimation of systolic pulmonary arterial pressure(sPAP)in patients with pulmonary arterial hypertension(PAH).Methods:Patients who were diagnosed with PAH and had a right heart catheterization(RHC)and echocardiography examination were selected retrospectively from May 2018 to December 2021.sPAP measured by RHC is used as the gold standard.A difference in sPAP of less than 10 mmHg between echocardiographic estimation by peak TR velocity and RHC measurement was defined as accurate,with a difference≥10 mmHg considered inaccurate.The factors affecting the accuracy of echocardiographic sPAP estimation were analyzed by univariate and multivariate analysis.Results:A total of 138 patients aged(45.57±15.97)years with PAH were enrolled.sPAP measured by echocardiography and RHC were(80.83±23.46)and(81.62±30.05)mmHg,respectively.The values of the 2 methods were highly correlated(r=0.809,P<0.01)and Bland-Altman plots showed good consistency.The accuracy rate of sPAP estimation by echocardiography was 42.03%(58/138).In the 57.97%(80/138)of patients where echocardiography was inaccurate,sPAP was overestimated in 28.26%(39/138)and underestimated in 29.71%(41/138).Univariate analysis showed that there was a statistically significant difference between the accurate and inaccurate groups in World Health Organization-Function Class,N-terminal pro-B-type natriuretic peptide,severity of TR,tricuspid annular plane systolic excursion(TAPSE),sPAP-RHC,mean pulmonary artery pressure,pulmonary vascular resistance(P<0.05).Multivariate logistic regression analyses identified the TR severity(odds ratio=2.292,95%confidence interval:1.126–4.667,P=0.022)and TAPSE(odds ratio=0.733,95%confidence interval:0.621–0.865,P<0.001)as independent predictors for the accuracy of echocardiographic sPAP estimation.Conclusion:Higher TR severity and lower TAPSE values reduce the accuracy of sPAP estimated by echocardiography.Therefore,TR severity and right heart function should be considered when echocardiography is used to estimate sPAP by the TR velocity.展开更多
There has been significant progress in our understanding of the pathobiology, epidemiology and prognosis of pulmonary vascular disease and, over the past few years, there has been an explosion of clinical therapeutic ...There has been significant progress in our understanding of the pathobiology, epidemiology and prognosis of pulmonary vascular disease and, over the past few years, there has been an explosion of clinical therapeutic trials for pulmonary arterial hypertension (PAH). The increasing number of different conditions now associated with PAH and the appearance of new diagnostic techniques have led to a need for a systematic diagnostic approaches and a new disease classification, which has resulted in notable improvements in the quality and efficacy of clinical care. We appreciate traditional resting right heart catheterization techniques (which still remain the gold standard for diagnosing PAH and managing patients on therapy) and look forward to novel invasive techniques (e.g. intravascular ultrasound) that add greatly to our understanding of right ventricle and pulmonary circulation, and for the interpretation of data from clinical trials as well.展开更多
Congenital heart disease(CHD)-associated pulmonary arterial hypertension(PAH)includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation.CHD-associated PAH has an estim...Congenital heart disease(CHD)-associated pulmonary arterial hypertension(PAH)includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation.CHD-associated PAH has an estimated prevalence of 5– 10% in adult patients,with an increasing number of patients surviving to adulthood because of advances in the surgical management and the development of pulmonary arterial hypertension(PAH)-targeted pharmacotherapy.Although limited data exist,targeted PAH pharmacotherapy has proven to be benefi cial in patients with CHD-associated PAH,with observed improvement in functional class,increase in exercise capacity,and improvement in quality of life and cardiopulmonary hemodynamics.Additionally,there has been increasing interest in the“treat-to-close”strategy.PAH-targeted pharmacotherapy may be used to optimize cardiopulmonary hemodynamics so as to improve patients’operability in repairing the cardiac defect.Although there have been signifi cant advances in the management of this disease state in the past 2 decades,mortality remains high,and ongoing clinical trials are needed to better understand the treat-to-close strategy.展开更多
BACKGROUND Since 1923,only a few hundred cases of pulmonary arterial sarcoma(PAS)have been reported.It is easy for PAS to be misdiagnosed as pulmonary thromboembolism,which makes treatment difficult.The median surviva...BACKGROUND Since 1923,only a few hundred cases of pulmonary arterial sarcoma(PAS)have been reported.It is easy for PAS to be misdiagnosed as pulmonary thromboembolism,which makes treatment difficult.The median survival time without surgical treatment for PAS is only 1.5-3 mo.Echocardiography is widely used in screening for pulmonary artery space-occupying lesions in patients with chest pain,dyspnea,and cough;furthermore,it is typically considered the first imaging examination for patients with PAS.CASE SUMMARY In May 2017,a 39-year-old male patient experienced chest pain with no particular obvious cause.At that time,the cause was thought to be pulmonary embolism.In July 2017,positron emission tomography–computed tomography revealed spaceoccupying lesions in the right lung and multiple metastases in both lungs.The lesions of the right lung were biopsied,and pathology revealed undifferentiated sarcoma.Chemotherapy had been performed since July 2017 in another hospital.In December 2019,the patient was admitted to our hospital for the sake of CyberKnife treatment.Echocardiography suggested:(1)A right ventricular outflow tract(RVOT)solid mass of the main pulmonary artery;and(2)mild pulmonary valve regurgitation.Ultrasonography showed the absence of a thrombus in the deep veins of either lower limb.CONCLUSION PAS is a single,central space-occupying lesion involving the RVOT and pulmonary valve.Echocardiography of PAS has its own characteristics.展开更多
The field of percutaneous valvular interventions is one of the most exciting and rapidly developing within interventional cardiology.Percutaneous procedures focusing on aortic and mitral valve replacement or intervent...The field of percutaneous valvular interventions is one of the most exciting and rapidly developing within interventional cardiology.Percutaneous procedures focusing on aortic and mitral valve replacement or interventional treatment as well as techniques of percutaneous pulmonary valve implantation have already reached worldwide clinical acceptance and routine interventional procedure status.Although techniquesof percutaneous pulmonary valve implantation have been described just a decade ago,two stent-mounted complementary devices were successfully introduced and more than 3000 of these procedures have been performed worldwide.In contrast,percutaneous treatment of tricuspid valve dysfunction is still evolving on a much earlier level and has so far not reached routine interventional procedure status.Taking into account that an "interdisciplinary challenging",heterogeneous population of patients previously treated by corrective,semi-corrective or palliative surgical procedures is growing inexorably,there is a rapidly increasing need of treatment options besides redo-surgery.Therefore,the review intends to reflect on clinical expansion of percutaneous pulmonary and tricuspid valve procedures,to update on current devices,to discuss indications and patient selection criteria,to report on clinical results and finally to consider future directions.展开更多
Pulmonary diseases associated with diurnal hypoxemia are known to be associated with pulmonary hypertension in some patients. In this study we examined the effects of daily hypoxia (10% oxygen;8h/day for 14 days) on t...Pulmonary diseases associated with diurnal hypoxemia are known to be associated with pulmonary hypertension in some patients. In this study we examined the effects of daily hypoxia (10% oxygen;8h/day for 14 days) on two strains of rats to simulate sleep related hypoxia in pulmonary diseases expecting to find differences in vascular responses, the develop-ment of right ventricular hypertrophy and pulmonary hypertension according to genetic background. In response to daily hypoxia, Sprague Dawley rats developed right ventricular hypertrophy while Brown Norway rats did not. Both strains developed pulmonary hypertension (elevated right ventricular pressure) although the increase was significantly greater in the Sprague Dawley strain. Pulmonary artery (first branch) vasoconstrictive responses to potassium chloride were increased equally in both strains and the subsequent vasodilation with acetylcholine were reduced equally with daily hypoxia in both strains. Taken together, these findings suggest that the genetic makeup of the rats contributed significantly to the development of right ventricular hypertrophy and the degree of pulmonary hypertension. Moreover, this response is not secondary to differences in the intralobar pulmonary vascular reactivity. Genetic background could explain why certain patients do worse with hypoxia inducing pulmonary vascular diseases.展开更多
文摘Mechanical circulatory and/or respiratory assistance with extracorporeal membrane oxygenation (ECMO) has become a standard of care for patients with circulatory (venoarterial) and/or respiratory (venovenous) failure refractory to standard therapies. Adult patients with congenital heart disease are an increasingly recognized and growing population and include various groups, such as undiagnosed cases in childhood and palliated and/or corrected cases, which require subsequent care because of residual lesions, cardiac arrest/insufficiency, and arrhythmias, among other conditions. In addition, these patients are prone to developing pathologies that are typical of adulthood with a generally increased risk of morbidity and mortality because of their low reserves and organic damage associated with the underlying heart disease, which makes them candidates for ECMO. These patients represent an additional challenge in this therapy because malformations and the presence of a shunt can generally affect the usual cannulation methods and hemodynamic and oximetry monitoring. Thus, the configuration decision must be made on a case-by-case basis. Here, we present a cannulation method, venopulmonary artery ECMO, which provides hemodynamic and respiratory support, and may be ideal for patients with shunts and/or right ventricular dysfunction. To our knowledge, this is the first report of this configuration in patients with congenital heart diseases.
文摘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.
基金Supported by An unrestricted grant of Actelion Pharmaceuticals Deutschland GmbH
文摘AIM To find parameters from transthorathic echocardiography(TTE) including speckle-tracking(ST) analysis of the right ventricle(RV) to identify precapillary pulmonary hypertension(PH).METHODS Forty-four patients with suspected PH undergoing right heart catheterization(RHC) were consecutively included(mean age 63.1 ± 14 years, 61% male gender). All patients underwent standardized TTE including ST analysis of the RV. Based on the subsequent TTE-derived measurements, the presence of PH was assessed: Left ventricular ejection fraction(LVEF) was calculated by Simpsons rule from 4Ch. Systolic pulmonary artery pressure(s PAP) was assessed with continuous wave Doppler of systolic tricuspid regurgitant velocity and regarded raised with values ≥ 30 mmH g as a surrogate parameter for RA pressure. A concomitantly elevated PCWP was considered a means to discriminate between the precapillary and postcapillary form of PH. PCWP was considered elevated when the E/e' ratio was > 12 as a surrogate for LV diastolic pressure. E/e' ratio was measured by gauging systolic and diastolic velocities of the lateral and septal mitral valve annulus using TDI mode. The results were then averaged with conventional measurement of mitral valve inflow. Furthermore, functional testing with six minutes walking distance(6MWD), ECG-RV stress signs, NT pro-BNP and other laboratory values were assessed.RESULTS PH was confirmed in 34 patients(precapillary PH, n = 15, postcapillary PH, n = 19). TTE showed significant differences in E/e' ratio(precapillary PH: 12.3 ± 4.4, postcapillary PH: 17.3 ± 10.3, no PH: 12.1 ± 4.5, P = 0.02), LV volumes(ESV: 25.0 ± 15.0 mL, 49.9 ± 29.5 m L, 32.2 ± 13.6 m L, P = 0.027; EDV: 73.6 ± 24.0 mL, 110.6 ± 31.8 mL, 87.8 ± 33.0 mL, P = 0.021) and systolic pulmonary arterial pressure(sP AP: 61.2 ± 22.3 mm Hg, 53.6 ± 20.1 mm Hg, 31.2 ± 24.6 mm Hg, P = 0.001). STRV analysis showed significant differences for apical RV longitudinal strain(RVAS: -7.5% ± 5.6%, -13.3% ± 4.3%, -14.3% ± 6.3%, P = 0.03). NT pro-BNP was higher in patients with postcapillary PH(4677.0 ± 7764.1 pg/m L, precapillary PH: 1980.3 ± 3432.1 pg/mL, no PH: 367.5 ± 420.4 pg/mL, P = 0.03). Patients with precapillary PH presented significantly more often with ECG RV-stress signs(P = 0.001). Receiver operating characteristics curve analyses displayed the most significant area under the curve(AUC) for RVAS(cut-off < -6.5%, AUC 0.91, P < 0.001), sPAP(cut-off > 33 mmH g, AUC 0.86, P < 0.001) and ECG RV stress signs(AUC 0.83, P < 0.001). The combination of these parameters had a sensitivity of 82.8% and a specificity of 17.2% to detect precapillary PH. CONCLUSION The combination of non-invasive measurements allows feasible assessment of PH and seems beneficial for the differentiation between the pre- and postcapillary form of this disease.
文摘Right ventricular failure(RVF)remains the primary cause of death in patients with pulmonary arterial hypertension.We review the pathophysiology of RVF,including the remodeling and ventriculoarterial uncoupling that occurs when the failing right side of the heart is unable to compensate for a rising afterload.Secondly,the noninvasive imaging techniques used in the assessment of RVF are explored,including echocardiography,cardiac magnetic resonance imaging,computed tomography,and positron emission tomography.Third,we describe how these imaging techniques and a patient’s clinical characteristics may be used to determine prognosis.Lastly,we explore the medical and surgical/interventional treatment options for RVF.Despite these treatment options,morbidity and mortality remain high in this patient population.The discovery of new prognostic indicators,use of hybrid imaging for early detection of RVF,and strategies to prevent the development of RVF will be important if outcomes in this patient population are to improve.
文摘Objective: The aim of the study was to determine usefulness of isovolumic acceleration to assess right ventricular function in patients with pulmonary hypertension. Background: Right ventricular dysfunction is a powerful predictor of adverse outcomes in patients with pulmonary hypertension. Although isovolumic acceleration is a sensitive and relatively load-independent parameter for ventricular contractile function, it has not been widely used. So, the aim of the study was to determine the usefulness of isovolumic acceleration in assessing right ventricular function in patients with pulmonary hypertension. Methods: A single center, prospective observational case-control study has been carried out for the period of one year at Osmania general hospital (Hyderabad). A total of 32 consecutive patients with pulmonary hypertension and 32 age matched healthy controls were recruited in the study as per the inclusion and exclusion criteria of the study. Receiver operator characteristic curves were used to study sensitivity, specificity, positive predictive value, negative predictive values as well as pearson’s correlation coefficients. Results: The results of the study showed that isovolumic acceleration has good significant correlation with right ventricular fractional area change (r = 0.554, p = 0.001) and tissue Doppler myocardial performance index (r = -0.599, p = 0.001) whereas a lesser but significant correlation was observed with tricuspid annular plane systolic excursion (r = 0.327, p = 0.034). Isovolumic acceleration showed higher specificity (81%) and positive predictive value (88%) in diagnosing right ventricular dysfunction in pulmonary hypertension compared to tricuspid annular systolic excursion velocity and tissue Doppler myocardial performance index parameters. Therefore isovolumic acceleration may be a better variable for diagnosing right ventricular dysfunction. Conclusions: Isovolumic acceleration is a sensitive and specific marker of right ventricular function in pulmonary hypertension.
文摘Objective:To evaluate the effectiveness of discharge planning on maternal caring knowledge,maternal caring behavior,maternal discharge readiness and the rehospitalization of children with ventricular septal defect and pulmonary arterial hypertension(VSD-PAH).Background:Children with congenital heart disease(CHD)with pulmonary arterial hypertension(PAH)have more complications after surgery than those without PAH.Discharge planning is an effective strategy to help children leave the hospital safely,and receive appropriate care after discharge.Methods:A quasi-experimental design was used.Sixty children and their mothers were recruited and divided into two groups:the control group received conventional care,the intervention group received both conventional care and additional discharge planning care.Results:(1)After admission,maternal caring knowledge between the two groups was similar.(2)At discharge,maternal discharge readiness,maternal caring knowledge and maternal caring behavior in the intervention group was significantly higher compared to the control group(t=3.35,p=0.001;F=84.74,p<0.001;F=23.82,p<0.001).This difference persisted after discharge,and was evident at one month and three months after discharge.(3)However,no significant difference in the readmission rate of children after discharge was evident between the two groups.Conclusions:Discharge planning improves the maternal discharge readiness,maternal caring knowledge and maternal caring behaviors.However,this planning did not reduce the readmission rate of children with CHD-PAH.
文摘Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. The prognostic value of the RV/LV ratio in patients with idiopathic pulmonary arterial hypertension(IPAH) is still unknown. Methods We retrospectively enrolled 95 consecutive patients with newly diagnosed IPAH and 16 of them were reevaluated by echocardiography at 3-12 months following targeted therapy.Follow-up data were obtained by telephone interviews and review of the patients’ records.Results The RV/LV ratio was in parallel with the severity of World Health Orgnization(WHO) functional class and mean right atrial pressure.The RV/LV ratio was positively correlated with total pulmonary resistance(P P P 2 saturation(P P = 0.001),weight and absence of targeted therapy were independent predictors of death.No significant changes in the RV/LV ratio before and after targeted therapy were observed. A baseline RV/LV ratio≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis. Conclusions The RV/LV ratio helps to assess the severity of IPAH and serves as an independent predictor of prognosis in patients with IPAH.
文摘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.
文摘Background: Right ventricle failure(RVF) is associated with serious cardiac and pulmonary diseases that contribute significantly to the morbidity and mortality of patients. Currently, the mechanisms of RVF are not fully understood and it is partly due to the lack of large animal models in adult RVF. In this study, we aim to establish a model of RVF in adult ovine and examine the structure and function relations in the RV.Methods: RV pressure overload was induced in adult male sheep by revised pulmonary artery constriction(PAC). Briefly, an adjustable hydraulic occluder was placed around the main pulmonary artery trunk. Then, repeated saline injection was performed at weeks 0, 1, and 4, where the amount of saline was determined in an animal-specific manner. Healthy, age-matched male sheep were used as additional controls. Echocardiography was performed bi-weekly and on week 11 post-PAC, hemodynamic and biological measurements were obtained.Results: This PAC methodology resulted in a marked increase in RV systolic pressure and decreases in stroke volume and tricuspid annular plane systolic excursion, indicating signs of RVF. Significant increases in RV chamber size, wall thickness, and Fulton's index were observed. Cardiomyocyte hypertrophy and collagen accumulation(particularly type III collagen) were evident, and these structural changes were correlated with RV dysfunction.Conclusion: In summary, the animal-specific, repeated PAC provided a robust approach to induce adult RVF, and this ovine model will offer a useful tool to study the progression and treatment of adult RVF that is translatable to human diseases.
文摘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.
文摘Background The goal of this study was to explore the significance of left ventricular(LV)ejection/filling parameters in assessing severity and prognosis of pulmonary arterial hypertension(PAH).Methods Newly diagnosed PAH patients were recruited between July 2011 and December 2013 prospectively.Cardiopulmonary hemodynamics derived from right heart catheterization,and LV ejection/filling parameters derived from CMR were collected.Follow-up data were collected periodically using a specifically-designed network database until April 30,2016.
文摘Background: Pulmonary hypertension is defined as a mean arterial pressure in the pulmonary artery exceeding 20 mm Hg at rest, measured by means of right heart catheterization. Patients with pulmonary hypertension undergoing surgical procedures such as hemiarthroplasty present a high risk of fatal complications. Nonetheless, there are no widely accepted protocols explaining their perioperative care in great detail. Case presentation: We present a case with an 89-years-old patient, with comorbidities such as GOLD 4D chronic obstructive pulmonary disease (COPD) and, as a consequence of this, severe pulmonary hypertension with signs of right ventricular dysfunction, thus requiring of pulmonary vasodilator, that has suffered a subcapital hip fracture requiring urgent surgery. Surgery is carried out successfully, under regional lumbar-sacral plexus block and sedation assisted by non-invasive ventilation. Conclusion: Multidisciplinary specialized treatment, preoperative optimization, as well as the careful selection of both the surgical and anesthetic techniques to be used, are among the strategies that improve the perioperative outcome in patients with pulmonary hypertension with right ventricle systolic dysfunction. Regional lumbar-sacral plexus block plus sedation is a technique that maintains hemodynamic stability;however, these patients require advance measures and postoperative monitoring under intensive care.
基金supported by the National Natural Science Foundation of China(82270057)the Clinical Research Award of the First Affiliated Hospital of Xi'an Jiaotong University,China(XJTU1AF-CRF-20i9-010)Fundamental Project Plan in Shaanxi Province,China(2020JM-364).
文摘Right ventricular function is independently associated with poor clinical outcomes in patients with various cardiovascular diseases including pulmonary hypertension(PH).The complex geometry and mode of contractility means the right ventricle(RV)inlet and outlet are not in line;hence,2-dimensional echocardiography fails to accurately quantify RV volumes and function in such patients.Three-dimensional echocardiography(3DE)allows for reliable and reproducible quantification of RV volumes and function by overcoming these limitations of conventional echocardiography.This review focuses on the 3DE assessment of RV function in patients with PH and discusses the following points:(1)acquiring an RV data set for 3DE imaging,including details of all available 3DE systems and software utilized in daily practice;(2)the reliability and feasibility of RV remodeling measured with 3DE with different modalities in patients with PH;and(3)the prognostic value of 3DE-derived RV function in such patients.
基金supported by grants from the National Key Research and Development Program of China(No.2022YFC2703902)the National High Level Hospital Clinical Research Funding(No.2022-PUMCH-B-099)the Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences(Nos.2020-I2M-C&T-B-003,and 2021-I2M-1-018).
文摘Background:The potential impact of pre-existing coronary artery stenosis(CAS)on acute pulmonary embolism(PE)episodes remains underexplored.This study aimed to investigate the association between pre-existing CAS and the elevation of high-sensitivity cardiac troponin I(hs-cTnI)levels in patients with PE.Methods:In this multicenter,prospective case-control study,88 cases and 163 controls matched for age,sex,and study center were enrolled.Cases were patients with PE with elevated hs-cTnI.Controls were patients with PE with normal hs-cTnI.Coronary artery assessment utilized coronary computed tomographic angiography or invasive coronary angiography.CAS was defined as≥50%stenosis of the lumen diameter in any coronary vessel>2.0 mm in diameter.Conditional logistic regression was used to evaluate the association between CAS and hs-cTnI elevation.Results:The percentage of CAS was higher in the case group compared to the control group(44.3%[39/88]vs.30.1%[49/163];P=0.024).In multivariable conditional logistic regression model 1,CAS(adjusted odds ratio[OR],2.680;95%confidence interval[CI],1.243-5.779),heart rate>75 beats/min(OR,2.306;95%CI,1.056-5.036)and N-terminal pro-B type natriuretic peptide(NT-proBNP)>420 pg/mL(OR,12.169;95%CI,4.792-30.900)were independently associated with elevated hs-cTnI.In model 2,right CAS(OR,3.615;95%CI,1.467-8.909)and NT-proBNP>420 pg/mL(OR,13.890;95%CI,5.288-36.484)were independently associated with elevated hs-cTnI.Conclusions:CAS was independently associated with myocardial injury in patients with PE.Vigilance towards CAS is warranted in patients with PE with elevated cardiac troponin levels.
文摘Objective:This study aims to investigate the impact of tricuspid regurgitation(TR)severity on the accuracy of echocardiographic estimation of systolic pulmonary arterial pressure(sPAP)in patients with pulmonary arterial hypertension(PAH).Methods:Patients who were diagnosed with PAH and had a right heart catheterization(RHC)and echocardiography examination were selected retrospectively from May 2018 to December 2021.sPAP measured by RHC is used as the gold standard.A difference in sPAP of less than 10 mmHg between echocardiographic estimation by peak TR velocity and RHC measurement was defined as accurate,with a difference≥10 mmHg considered inaccurate.The factors affecting the accuracy of echocardiographic sPAP estimation were analyzed by univariate and multivariate analysis.Results:A total of 138 patients aged(45.57±15.97)years with PAH were enrolled.sPAP measured by echocardiography and RHC were(80.83±23.46)and(81.62±30.05)mmHg,respectively.The values of the 2 methods were highly correlated(r=0.809,P<0.01)and Bland-Altman plots showed good consistency.The accuracy rate of sPAP estimation by echocardiography was 42.03%(58/138).In the 57.97%(80/138)of patients where echocardiography was inaccurate,sPAP was overestimated in 28.26%(39/138)and underestimated in 29.71%(41/138).Univariate analysis showed that there was a statistically significant difference between the accurate and inaccurate groups in World Health Organization-Function Class,N-terminal pro-B-type natriuretic peptide,severity of TR,tricuspid annular plane systolic excursion(TAPSE),sPAP-RHC,mean pulmonary artery pressure,pulmonary vascular resistance(P<0.05).Multivariate logistic regression analyses identified the TR severity(odds ratio=2.292,95%confidence interval:1.126–4.667,P=0.022)and TAPSE(odds ratio=0.733,95%confidence interval:0.621–0.865,P<0.001)as independent predictors for the accuracy of echocardiographic sPAP estimation.Conclusion:Higher TR severity and lower TAPSE values reduce the accuracy of sPAP estimated by echocardiography.Therefore,TR severity and right heart function should be considered when echocardiography is used to estimate sPAP by the TR velocity.
基金Supported by Programa de Desarrollo de las Ciencias Básicas,Ministry of Education and Culture
文摘There has been significant progress in our understanding of the pathobiology, epidemiology and prognosis of pulmonary vascular disease and, over the past few years, there has been an explosion of clinical therapeutic trials for pulmonary arterial hypertension (PAH). The increasing number of different conditions now associated with PAH and the appearance of new diagnostic techniques have led to a need for a systematic diagnostic approaches and a new disease classification, which has resulted in notable improvements in the quality and efficacy of clinical care. We appreciate traditional resting right heart catheterization techniques (which still remain the gold standard for diagnosing PAH and managing patients on therapy) and look forward to novel invasive techniques (e.g. intravascular ultrasound) that add greatly to our understanding of right ventricle and pulmonary circulation, and for the interpretation of data from clinical trials as well.
文摘Congenital heart disease(CHD)-associated pulmonary arterial hypertension(PAH)includes a heterogeneous patient population that can be characterized by the underlying cardiac malformation.CHD-associated PAH has an estimated prevalence of 5– 10% in adult patients,with an increasing number of patients surviving to adulthood because of advances in the surgical management and the development of pulmonary arterial hypertension(PAH)-targeted pharmacotherapy.Although limited data exist,targeted PAH pharmacotherapy has proven to be benefi cial in patients with CHD-associated PAH,with observed improvement in functional class,increase in exercise capacity,and improvement in quality of life and cardiopulmonary hemodynamics.Additionally,there has been increasing interest in the“treat-to-close”strategy.PAH-targeted pharmacotherapy may be used to optimize cardiopulmonary hemodynamics so as to improve patients’operability in repairing the cardiac defect.Although there have been signifi cant advances in the management of this disease state in the past 2 decades,mortality remains high,and ongoing clinical trials are needed to better understand the treat-to-close strategy.
文摘BACKGROUND Since 1923,only a few hundred cases of pulmonary arterial sarcoma(PAS)have been reported.It is easy for PAS to be misdiagnosed as pulmonary thromboembolism,which makes treatment difficult.The median survival time without surgical treatment for PAS is only 1.5-3 mo.Echocardiography is widely used in screening for pulmonary artery space-occupying lesions in patients with chest pain,dyspnea,and cough;furthermore,it is typically considered the first imaging examination for patients with PAS.CASE SUMMARY In May 2017,a 39-year-old male patient experienced chest pain with no particular obvious cause.At that time,the cause was thought to be pulmonary embolism.In July 2017,positron emission tomography–computed tomography revealed spaceoccupying lesions in the right lung and multiple metastases in both lungs.The lesions of the right lung were biopsied,and pathology revealed undifferentiated sarcoma.Chemotherapy had been performed since July 2017 in another hospital.In December 2019,the patient was admitted to our hospital for the sake of CyberKnife treatment.Echocardiography suggested:(1)A right ventricular outflow tract(RVOT)solid mass of the main pulmonary artery;and(2)mild pulmonary valve regurgitation.Ultrasonography showed the absence of a thrombus in the deep veins of either lower limb.CONCLUSION PAS is a single,central space-occupying lesion involving the RVOT and pulmonary valve.Echocardiography of PAS has its own characteristics.
文摘The field of percutaneous valvular interventions is one of the most exciting and rapidly developing within interventional cardiology.Percutaneous procedures focusing on aortic and mitral valve replacement or interventional treatment as well as techniques of percutaneous pulmonary valve implantation have already reached worldwide clinical acceptance and routine interventional procedure status.Although techniquesof percutaneous pulmonary valve implantation have been described just a decade ago,two stent-mounted complementary devices were successfully introduced and more than 3000 of these procedures have been performed worldwide.In contrast,percutaneous treatment of tricuspid valve dysfunction is still evolving on a much earlier level and has so far not reached routine interventional procedure status.Taking into account that an "interdisciplinary challenging",heterogeneous population of patients previously treated by corrective,semi-corrective or palliative surgical procedures is growing inexorably,there is a rapidly increasing need of treatment options besides redo-surgery.Therefore,the review intends to reflect on clinical expansion of percutaneous pulmonary and tricuspid valve procedures,to update on current devices,to discuss indications and patient selection criteria,to report on clinical results and finally to consider future directions.
文摘Pulmonary diseases associated with diurnal hypoxemia are known to be associated with pulmonary hypertension in some patients. In this study we examined the effects of daily hypoxia (10% oxygen;8h/day for 14 days) on two strains of rats to simulate sleep related hypoxia in pulmonary diseases expecting to find differences in vascular responses, the develop-ment of right ventricular hypertrophy and pulmonary hypertension according to genetic background. In response to daily hypoxia, Sprague Dawley rats developed right ventricular hypertrophy while Brown Norway rats did not. Both strains developed pulmonary hypertension (elevated right ventricular pressure) although the increase was significantly greater in the Sprague Dawley strain. Pulmonary artery (first branch) vasoconstrictive responses to potassium chloride were increased equally in both strains and the subsequent vasodilation with acetylcholine were reduced equally with daily hypoxia in both strains. Taken together, these findings suggest that the genetic makeup of the rats contributed significantly to the development of right ventricular hypertrophy and the degree of pulmonary hypertension. Moreover, this response is not secondary to differences in the intralobar pulmonary vascular reactivity. Genetic background could explain why certain patients do worse with hypoxia inducing pulmonary vascular diseases.