How to select the appropriate candidate of pulmonary arterial hypertension： specific therapy in elderly patients with pulmonary hypertension

Recent reports from pulmonary arterial hypertension （PAH） registries suggest that the mean age at diagnosis is increasing in a growing proportion of elderly patients. The combination of several reasons such as aging popula- tion, increase in life expectancy, growing PAH awareness of physicians and patients, and availability of more treatment options could explain the changing picture of PAH. PAH should be considered as an emerging entity in the elderly.

The clinical features and effects with target therapy for post splenectomy pulmonary hypertension

Objective To analyze the clinical features and effects of target therapy of post splenectomy pulmonary hypertension, and improve the diagnosis and treatment of the disease.Methods Clinical data of 18 patients with post splenectomy pulmonary hypertension admitted to our hospital from October 2006 to March 2017 were systematically reviewed.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension:State of the art

Chronic thromboembolic pulmonary hypertension(CTEPH)is a complex chronic disease in which pulmonary artery stenosis or obstruction caused by organized thrombus can lead to increased pulmonary artery pressure and pulmonary vascular resistance,ultimately triggering progressive right heart failure and death.Currently,its exact mechanism is not fully understood.Pulmonary endarterectomy(PEA)has immediate effects with low perioperative mortality and satisfactory prognosis in experienced expert centers for CTEPH patients with proximal lesions.Nevertheless,37%of patients are deemed unsuitable for PEA surgery due to comorbidities and other factors,and nearly half of the operated patients have residual or recurrent pulmonary hypertension.Riociguat is the only approved drug for CTEPH,although its effect is limited.Balloon pulmonary angioplasty(BPA)is a promising alternative treatment for patients with CTEPH.After more than 30 years of development and refinements,emerging evidence has confirmed its role in patients with inoperable CTEPH or residual/recurrent pulmonary hypertension,with acceptable complications and comparable longterm prognosis to PEA.This review summarizes the pathophysiology of CTEPH,BPA history and development,therapeutic principles,indications and contraindications,interventional procedures,imaging modalities,efficacy and prognosis,complications and management,bridging and hybrid therapies,ongoing clinical trials and future prospects.

Clinical management of portopulmonary hypertension

BACKGROUND: Portopulmonary hypertension (PPH) is defined as the development of pulmonary arterial hypertension associated with increased pulmonary vascular resistance complicated by portal hypertension, with or without advanced hepatic disease. In spite of the relatively rare prevalence, the clinical implications of PPH are significant. It has high perioperative morbidity and mortality. This review is an update of current pathogenesis, diagnosis and therapy of PPH. DATA SOURCES: An English-language literature search was conducted using PubMed (1980-2006) on portopulmonary hypertension. RESULTS: Echocardiographically identified patients with elevated pulmonary artery systolic pressure (>50 mmHg) receive right heart catheterization. Epoprostenol (prostacyclin), a potent pulmonary and systemic vasodilator with anti-platelet aggregating activity, and bosentan, an endothelin receptor antagonist, have so far proven beneficial to patients with PPH. CONCLUSIONS: After an accurate diagnosis of PPH treatment should (at a minimum) focus on reduction of mean pulmonary arterial pressure to less than 35 mmHg prior to orthotopic liver transplantation. However, orthotopic liver transplantation currently remains the only therapy to resolve PPH.

Potential impact of specific therapy on pregnant women with pulmonary arterial hypertension without cardiac shunt:a descriptive study in northern China

Background:Pregnancy in women with pulmonary arterial hypertension(PAH)is a fatal condition,despite the effectiveness of PAH-specific therapies.The coverage status and effect of specific therapies in pregnant patients with PAH without cardiac shunts in China remain unclear.To investigate this issue,we conducted a multicenter retrospective study in northern China.Methods:The study included 85 patients who were admitted to 4 clinical centers in Shandong Province between October 2010 and August 2020.Maternal endpoint events included(1)maternal death and/or(2)major adverse cardiac events,both occurring during pregnancy or within 6 weeks postpartum.Results:Although the overall mortality rate was encouraging(11.8%),the number of patients receiving PAH-specific therapies was extremely low(28.2%).Moreover,only 15.3%of patients received adequate duration of PAH-specific therapy(≥4 weeks)before delivery,and this subgroup showed the lowest major adverse cardiac events rate(7.7%)compared with that in the untreated(19.7%)and short-time treated groups(<4 weeks;54.5%).Conclusion:Pregnant patients with PAH without cardiac shunts face significantly increased mortality risks.Short-term PAH-specific therapy does not guarantee favorable maternal outcomes.Prepregnancy screening,early identification,and timely intervention are expected to improve maternal outcomes in pregnant women with PAH.

Condition Metabolic Balance Disturbance in Patients with Cor Pulmonale： Prevalence, Diagnosis, Risk Factors

To study the risk factors for the development of cor pulmonale （CP） in patients with chronic pulmonary diseases in the Republic of Uzbekistan, the disbalance in parameters of stable metabolites oxides of the nitrogen （SMNO）, of blood oxygen saturation （SaO2）, of respiratory function and on lipid peroxidation of cell membrane were measured. The authors evaluated the effect of base therapy and ozone therapy （OT） in patients with CP. Basis therapy and OT resulted in good parameters of SMNo, SaO2 and metabolic activeness of cell, significant positiveness of hypoxemia and parameters of pulmonary ventilation ability and hemodynamics of pulmonary arteria.

Effect of prenatal tetrandrine therapy on pulmonary vascular structural remodeling in the nitrofen-induced CDH rat model

To examine the effects of prenatal tetrandrine (Tet) therapy on pulmonary arterial structural remodeling in nitrofen-induced congenital diaphragmatic hernia (CDH) Methods CDH was induced in fetal rats by maternal administration of 100*!mg nitrofen by gavage on day 9 5 of gestation (term, day 22) Control animals received olive oil (OO) Tet (24*!mg/kg per day) or normal saline (NS) was given by gavage every day from 16 to 20 days of gestation, and fetuses were delivered by caesarean section on day 21 5 Lung sections from 3 fetuses in each group were studied The number of vessels were calculated, the external diameter (ED), medial wall thickness (MT), percent of medial wall thickness, and wall structure were evaluated by image analysis software Results In the pre-acinar arteries, CDH-NS pups had a significantly increased %MT compared with the OO-NS group ( P <0 05), while CDH-Tet animals had a reduced %MT compared with the CDH-NS rats ( P <0 05) Similar results were seen in the intra-acinar level Significant differences were observed between CDH-NS animals and OO-NS controls in the percentage of muscularized intra-acinar blood vessels ( P <0 001) Tet-treated CDH pups had a reduced percentage of muscularized intra-acinar arteries compared with CDH-NS animals Conclusions Medial hypertrophy is present in both the pre-acinar and intra-acinar arteries in the nitrofen-induced CDH rat model Tet treatment inhibits medial hypertrophy and reduces the percentage of muscularized intra-acinar vessels Prenatal Tet therapy may be efficacious in reducing the risk of PH in human newborns with CDH

Home-made fenestrated amplatzer occluder for atrial septal defect and pulmonary arterial hypertension

We report the management of a patient with secundum atrial septal defect （ASD） and severe pulmonary hypertension. A 65-year-old male with recently diagnosed atrial septal defect was referred to our centre for decompensated right heart failure with rest and exercise induced dispnea and severe pulmonary hypertension. Right heart catheterization confirmed a mean pulmonary pressure of about 55 mmHg and a Qp/Qs of 2.7. An occlusion test with a compliant large balloon demonstrated partial fall of pulmonary arterial pressure. The implantation of a home-made fenestrated Amplatzer ASD Occluder （ASO） was planned in order to decrease left-to-right shunt and promote further decrease of pulmonary arterial pressure in the long-term. Thus, by means of mechanical intracardiac echocardiography study with a 9F 9 MHz Ultralce catheter （Boston Scientific Corp.）, we selected a 34 mm ASO for implantation. Four millimeter fenestration was made inflating a 4 mm non-compliant coronary balloon throughout the waist of the ASO, which was successfully implanted under intmcardiac echocardiography. After six months, a decrease of pulmonary arterial pressure to 24 mmHg and full compensated right heart failure was observed on transthoracic echocardiography and clinical examination. This case suggests that Wanscatheter closure with home-made fenestrated ASD in elderly patients with severe pulmonary hypertension is feasible.

Current practice for pulmonary hypertension

Objective To investigate the current practice of pulmonary hypertension including current epidemiology, diagnosis and treatment. Data sources The review was based on data obtained from the published articles and guidelines. Study selection Articles with high level of evidence or current best evidence in each issue were selected to be reviewed. Results Overall prevalence of pulmonary hypertension was 0.3% to 6% with left heart disease occupying the most proportion, followed by pulmonary disease, pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. In diagnosis, a flow diagram of diagnosis of pulmonary hypertension, differential diagnosis of pulmonary hypertension and how to determine the severity of pulmonary hypertension are explained including recent development of magnetic resonance imaging and gene abnormality study on bone morphogenetic protein receptor ll. In treatment, newly- developed pulmonary vasodilators and the way to use them are shown to treat pulmonary hypertension. Conclusion Safer and more effective treatment algorithm and basic researches and clinical trials are warranted to be explored.

Value of Coronary Sinus During Computed Tomography Pulmonary Angiography for Predicting Pulmonary Hypertension in Patients with Acute Pulmonary Embolism

Objective:Computed tomography pulmonary angiography(CTPA)parameters are valuable for predicting pulmonary hypertension(PH)in patients with pulmonary embolism(PE).However,few studies have used the coronary sinus ostium area(CSOA),derived from CTPA,to detect PH.This study aimed to compare the prognostic values of the CSOA,coronary sinus ostium diameter(CSOD),and right ventricular(RV)/left ventricular(LV)ratio for PH.Methods:This study retrospectively analyzed 78 patients(mean age,(51.94±12.33)years;53.8%male)with acute PE confirmed by CTPA at the Sixth and Eighth Medical Centers of the People’s Liberation Army General Hospital between June 2018 and June 2020.Patients were categorized into 2 groups using a pulmonary artery systolic pressure(PASP)cut-off of 30 mmHg.CTPA parameters were compared between these groups.Results:Patients with PASP>30 mmHg(n=22)had a larger CSOA,CSOD,RV diameter,RV/LV ratio,and inferior vena cava diameter compared with patients with PASP≤30 mmHg(n=56).The CSOA had an area under the receiver-operating characteristic curve(AUC,0.84;95%confidence interval(CI),0.74–0.94;P<0.001)similar to that of the RV/LV ratio(AUC,0.85;95%CI,0.73–0.99;P<0.001),while that of the CSOD was smaller(AUC,0.66;95%CI,0.51–0.81;P<0.05).Conclusion:Both CSOA and CSOD demonstrated a good ability to predict PH,while CSOA better predicted PASP>30 mmHg.

Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease

Background:Pulmonary artery hypertension associated withh congenital heart disease(PAH-CHD)occurs predominantly among patients with uncorrected CHD.Treatment of severe pediatric PAH-CHD remains a major intractability.This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction.Methods:The data for 59 children with severe PAH-CHD who underwent surgical correction,with or without postoperative medication,between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively.A regression analysis,receiver-operating characteristic(ROC)curves,and Kaplan-Meier curves were used for survival analysis.Results:Fifty-nine children with severe PAH-CHD underwent heart catheterization and correction,with or without specific anti-PAH drugs postoperatively,were included in this study.The pulmonary pressure,heart function,and ending events were observed and median observation period was 49±20 months.Twenty-eight patients(50%)received at least one additional anti-PAH drug after correction.The survival rate after 2 years was 91.5%(54/59);two patients were in a critical condition,and three were lost to follow-up.Twelve patients(29%)still received over one additional PAH-specific therapy at follow-up,whereas 42(75%)had successfully stopped drug treatment.Two patients(3.5%)died and one underwent a second thoracotomy to remove the ventricular septal defect patch.Acute vasoreactivity test(AVT)criteria had limited efficacy in predicting pediatric PAH-CHD,whereas pulmonary vascular resistance(PVR)<6.65 Wood units(WU)/m^2 or PVR/systemic vascular resistance(SVR)<0.39 during AVT indicated a good prognosis after surgical correction with an AUC of 98.3%(95%confidence interval[CI]:96.0-100%),98.4%(95%CI:96.0-100%)sensitivity of 100%,100%and specificity of 82.1%,92.9%,respectively.Conclusions:Although the criteria for positive AVT currently used are unsuitable for pediatric patients with PAH-CHD,PVR and PVR/SVR during AVT are excellent predictors of outcome in pediatric PAH-CHD.Surgery aided by anti-PAH drugs is an effective strategy and should be recommended for severe pediatric PAH-CHD with PVR<6.65 WU/m2 and PVR/SVR<0.39 after iloprost aerosol inhalation.

Pulmonary arterial hypertension associated with systemic sclerosis: Current diagnostic approach and therapeutic strategies

Pulmonary arterial hypertension(PAH) represents a devastating vascular complication of systemic sclerosis(SSc) and is found in 10%-15% of cases carrying a severe prognosis. PAH has a dramatic impact on the clinical course and overall survival, being the single most common cause of death in patients with this entity. The clinical course and aggressive progression of PAH has led clinicians to perform annual screening for it, since early detection and diagnosis are the cornerstone of a prompt therapeutic intervention. The diagnosis of PAH can be challenging to clinicians, particularly in its early stages, since in the context of SSc, the multiple causes of dyspnea need to be assessed. Doppler echocardiography represents the best initial screening tool, however, right heart catheterization remains the gold standard and definitive diagnostic means. Remarkable advances have been achieved in elucidating the pathogenesis of PAH in the past two decades, leading to the development of disease-specific targeted therapies: prostacyclin analogues, endothelin receptor antagonists and inhibitors of five phosphodiesterase pathways. However, the clinical response to these therapies in SSc-associated PAH has not been as great as the one seen with idiopathic PAH. This review also focuses on the diagnosis and novel therapies that are currently available for PAH, as well as potential future therapeutic developments based on newly acquired knowledge of diverse pathogenic mechanisms.

Comparison of pulmonary vascular tortuosity and fractal dimension in patients with chronic thromboembolic pulmonary disease and chronic thromboembolic pulmonary hypertension

Objective To compare the differences in pulmonary vascular morphological parameters between patients with chronic thromboembolic pulmonary disease(CTEPD)and chronic thromboembolic pulmonary hypertension(CTEPH),and to explore the value of pulmonary artery morphology in the differential diagnosis of CTEPD and CTEPH.

Pediatric hypertension: An update on a burning problem

A large number of adults worldwide suffer from essen-tial hypertension, and because blood pressures(BPs) tend to remain within the same percentiles throughout life, it has been postulated that hypertensive pressures can be tracked from childhood to adulthood. Thus, chil-dren with higher BPs are more likely to become hyper-tensive adults. These "pre-hypertensive" subjects can be identified by measuring arterial BP at a young age, and compared with age, gender and height-specific references. The majority of studies report that 1 to 5% of children and adolescents are hypertensive, defined as a BP > 95th percentile, with higher prevalence rates reported for some isolated geographic areas. However, the actual prevalence of hypertension in children and adolescents remains to be fully elucidated. In addition to these young "pre-hypertensive" subjects, there are also children and adolescents with a normal-high BP(90th-95th percentile). Early intervention may help pre-vent the development of essential hypertension as they age. An initial attempt should be made to lower their BP by non-pharmacologic measures, such as weight re-duction, aerobic physical exercise, and lowered sodium intake. A pharmacological treatment is usually needed should these measures fail to lower BP. The majority of antihypertensive drugs are not formulated for pediatricpatients, and have thus not been investigated in great detail. The purpose of this review is to provide an up-date concerning juvenile hypertension, and highlight recent developments in epidemiology, diagnostic meth-ods, and relevant therapies.

A fast-track diagnostic and therapeutic strategy for acute pulmonary embolism patients at peri-operational period

Acute pulmonary embolism(APE) in operation room is a constant,lethal but treatable disease.A fast determination of APE in an operation room is currently difficult due to a non-specificity on its signs,symptoms,electrocardiograph findings,arterial blood gas abnormalities and chest X-rays.Clinically,an APE can automatically originate from a small and clinically asymptomatic embolus to massive,proximal emboli with shock during a scheduled surgery period.An accurate,brief and prompt diagnostic and therapeutic strategy for APE in a peri-operation period is crucial for anesthesiologists to make a decision on an appropriate clinical intervention to improve patients' outcomes.The purpose of this mini-review article is to illuminate a fast-track diagnostic and therapeutic strategy for APE patients in a perioperative period through a hybrid of the high sensitive and high specific viable diagnostic modality of D-dimer assay in conjunction with necessary emergent pulmonary angiography(EPA),including digital subtraction angiography(DSA),or a spiral computed tomography angiography(sCTA) for APE patients' diagnosis and treatment during operation.The postulated fast-track diagnostic and therapeutic strategy can effectively confirm or exclude highly suspected APE and simultaneously provide fast therapeutic opportunities for APE patients at an operative period.

Genetic analyses in a cohort of 191 Chinese pulmonarv arterial hypertension patients

Objective Pulmonary arterial hypertension(PAH)is mainly characterized by pulmonary artery obstruction,which is diagnosed by a mean pulmonary artery pressure≥25 mm Hg at rest,and excluding other known causes of pulmonary hypertension.To identify genetic mutations and help make a precise diagnosis,we performed genetic testing in 191 probands with invasively confirmed PAH and tried to analyze the genotype-phenotype correlation.

Congenital Hernia of the Diaphragmatic Dome with Neonatal Revelation, Experience from the Neonatology and Neonatal Intensive Care Unit at the University Hospital Center of Oujda

Congenital hernia of the diaphragmatic dome (CHDD) is an embryonic malformation in which all or part of the diaphragmatic dome fails to develop properly. In the majority of cases (80% to 90%), this malformation affects the left posterolateral part of the diaphragm, while in 10% to 15% of cases it affects the right. Bilateral cases are extremely rare, accounting for less than 1% of cases. This malformation is estimated to occur at a frequency of around 1 in 3500 births, with a male predominance. The diaphragmatic defect causes the abdominal organs to rise into the thoracic cavity during critical phases of lung development. These anomalies result in bilateral pulmonary hypoplasia, a reduced number of pulmonary vessels, and pulmonary arterial hypertension (PAH). The combination of these anatomical and functional anomalies, in varying degrees, explains the wide variability of symptoms at birth. Diagnosis is usually made prenatally by ultrasound, which enables severe forms of the disease to be detected and appropriate management initiated. The prognosis remains generally grave, with a neonatal mortality rate of between 30% and 60% depending on the study, and around half of all children will have long-term sequelae.

Hypertension in kidney transplant recipients

Kidney transplantation is considered the treatment of choice for end-stage kidney disease patients.However,the residual cardiovascular risk remains significantly higher in kidney transplant recipients(KTRs)than in the general population.Hypertension is highly prevalent in KTRs and represents a major modifiable risk factor associated with adverse cardiovascular outcomes and reduced patient and graft survival.Proper definition of hypertension and recognition of special phenotypes and abnormal diurnal blood pressure(BP)patterns is crucial for adequate BP control.Misclassification by office BP is commonly encountered in these patients,and a high proportion of masked and uncontrolled hypertension,as well as of white-coat hypertension,has been revealed in these patients with the use of ambulatory BP monitoring.The pathophysiology of hypertension in KTRs is multifactorial,involving traditional risk factors,factors related to chronic kidney disease and factors related to the transplantation procedure.In the absence of evidence from large-scale randomized controlled trials in this population,BP targets for hypertension management in KTR have been extrapolated from chronic kidney disease populations.The most recent Kidney Disease Improving Global Outcomes 2021 guidelines recommend lowering BP to less than 130/80 mmHg using standardized BP office measurements.Dihydropyridine calcium channel blockers and angiotensin-converting enzyme inhibitors/angiotensin-II receptor blockers have been established as the preferred first-line agents,on the basis of emphasis placed on their favorable outcomes on graft survival.The aim of this review is to provide previous and recent evidence on prevalence,accurate diagnosis,pathophysiology and treatment of hypertension in KTRs.

Clinical Analysis of 15 Patients with Adult-Type Pulmonary Blastoma

OBJECTIVE To study the clinical and histopathological characteristics of pulmonary blastoma(PB),and to review the diagnostic criterion and treatment methods.METHODS Clinical and histopathological data from 15 cases of PB in this hospital,from 1990 to 2006,were retrospectively analyzed.RESULTS Surgical procedures were conducted in all 15 patients,including pulmonary lobectomy in 9,lobectomy of the 2 right pulmonary lobes in 2,excision of pulmonary segments in 1,lobectomy and bronchoplasty in 1,lobectomy and angioplasty in 1,and excision of the whole left lung in 1.Of the 15 cases,11 were misdiagnosed as lung cancer,2 were doubted to be a benign tumor before surgery,1 was suspected to be a malignant mesenchymoma,and only 1 was diagnosed as a PB.CONCLUSION Preoperative final diagnosis of PB is very difficult,since the clinical symptoms of the disease are nonspecific.To confirm the diagnosis,comprehensive consideration of the factors,such as the clinical manifestations,morphological characteristics and immunohistochemical tumor markers,should be taken.Surgery is the treatment of choice for PB,and a thorough removal is the key to a cure.PB has a high malignancy and o en recurs and metastasizes,usually with a poor prognosis.

特发性肺纤维化合并肺动脉高压的发病机制和治疗进展

特发性肺纤维化的晚期常合并肺动脉高压,病理表现为肺血管重塑、增殖和炎症,肺动脉高压的出现加重原有肺部疾病症状,导致右心衰竭,死亡风险增加、预后差、社会及家庭负担重。特发性肺纤维化合并肺动脉高压目前病因尚不明确、治疗棘手。本文对特发性肺纤维化合并肺动脉高压的发病机制及治疗进展进行综述,以期为特发性肺纤维化合并肺动脉高压的研究和治疗方案的制定提供新的思路。