Therapy For Pulmonary Hypertension Research Articles

Vasopressin as adjunctive therapy in pulmonary hypertension associated with refractory systemic hypotension in term newborns.

The use of vasopressin as an adjunctive therapy in pulmonary hypertension associated with refractory systemic hypotension has increased. The objective of our study is to describe its effects on term infants. Retrospective observational study. Setting in a referral level IV neonatal intensive care unit from a middle-income region. The patients are term neonates admitted to our NICU who required vasopressin due to severe Pulmonary Hypertension and refractory hypotension during a 49-month period (December 2019 and December 2023). We identified 68 term infants, all in mechanical ventilation, receiving inhaled nitric oxide (iNO), and a phased protocol management for hypotension. Vasopressin was a started at a mean of 2 days with a mean duration of 80 h. Regarding hemodynamic outcome: diastolic, systolic, and median systemic pressure significantly increased during the first 4 h of treatment, as well as arterial pH and urine output. Accordingly, lactate and Vasoactive Inotropic Score (VIS) score decreased after 4 and 8 h, respectively, after vasopressin was started. Regarding oxygenation markers: oxygen requirements and mean airway pressure decreased significantly (and therefore the oxygenation index decreased in concordance) after 4 h of vasopressin. Echocardiographic indices of pulmonary hypertension progressively improved after vasopressin infusion with a significant decrease of tricuspid ingurgitation velocities and the rate of right-to- left ductal shunt through the ductus arteriosus. In the same way, left and right ventricular output increased after the initiation of vasopressin. This study showed that the use of vasopressin in neonates with persistent pulmonary hypertension was associated with a rapid and significant improvement in oxygenation and hemodynamic markers of perfusion, including blood pressure. Its effects begin early during the first hours of treatment.

Usefulness of risk assessment tools in predicting hemodynamic outcome after balloon pulmonary angioplasty: a comparative analysis

ABSTRACT Objectives Several parameters of widely used risk assessment tools for pulmonary arterial hypertension (PAH) have been linked to hemodynamic outcomes of balloon pulmonary angioplasty (BPA). Therefore, we aimed to determine whether these risk assessment tools could be used to predict hemodynamic outcomes following BPA. Methods In this retrospective study, we included 139 patients with chronic thromboembolic pulmonary hypertension who had undergone BPA at Center for Pulmonary Vascular Diseases, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College (Beijing, China). We compared the accuracies of seven well-validated risk assessment tools for predicting hemodynamic outcomes following BPA. A favorable hemodynamic outcome was defined as a mean pulmonary arterial pressure < 30 mmHg at follow-up. Results The baseline risk profiles varied significantly among the risk assessment tools. The US Registry to Evaluate Early and Long-Term PAH Disease Management risk scales and the French risk assessment tools rated most patients as high-risk, while the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) series and laboratory examination-based risk scales categorized most patients as having intermediate-risk profile. COMPERA 2.0 (4-strata) exhibited the highest predictive power among all risk stratifications. Noninvasive risk stratification (COMPERA 2.0 [3-strata]) showed a comparable predictive ability to that of invasive risk stratification (COMPERA 1.0) (area under the curve 0.649 vs. 0.648). Moreover, incorporating diffusing capacity of the lungs for carbon monoxide and tricuspid regurgitation velocity into COMPERA 2.0 (4-strata) further enhanced its predictive power (net reclassification index 0.153, 95% confidence interval 0.009–0.298, p = 0.038). Additionally, this refined COMPERA version had a high calibration accuracy (slope 0.96). Conclusion Although the risk strata distribution varied among different risk assessment tools, the proportion of patients achieving favorable hemodynamics decreased with the escalation of risk stratification in most models. The well-validated risk assessment tools for PAH could also predict hemodynamic outcomes following BPA, and the refined COMPERA 2.0 model exhibited the highest predictive ability among these. Applying risk assessment tools before BPA can facilitate early identification of patients in need of closer monitoring and more intensive interventions, contributing to a better prognosis after BPA.

Effects of medical therapy and age on cardiac output changes following balloon pulmonary angioplasty: Implications for combination therapy in chronic thromboembolic pulmonary hypertension

BACKGROUNDSome patients with chronic thromboembolic pulmonary hypertension (CTEPH) exhibit exercise intolerance due to reduced cardiac output (CO) even after successful balloon pulmonary angioplasty (BPA). Medical therapy is a potential option for such cases; however, it is unclear which patients necessitate it even after BPA. METHODSThis study included 286 patients with CTEPH who underwent BPA and right heart catheterization one year after the final BPA and classified them into No-medication and Withdrawal groups. The No-medication group comprised patients without pulmonary hypertension (PH) medications before and after BPA, while the Withdrawal group included patients who received PH medications before BPA and discontinued them after BPA. We assessed differences in the changes in CO after BPA from baseline (ΔCO) between the two groups. Additionally, we evaluated the ΔCO among different age categories within each group: younger (< 60 years), middle-aged (60 to 70 years), and older-adults (≥ 70 years). RESULTSAfter adjusting baseline covariates, overall ΔCO did not differ significantly. However, ΔCO was significantly increased in No-medication group but decreased in Withdrawal group (0.32 and −0.33, difference in ΔCO: −0.65, 95% confidence intervals: −0.90 to −0.40). A significantly positive effect on ΔCO was observed only in younger individuals, with a significant interaction between age and ΔCO in both groups. CONCLUSIONSIncreasing CO with BPA alone may be challenging with age in patients with CTEPH. Given that discontinuation of PH medication after BPA decreased CO more than the effect of BPA, medical therapy might be necessary even after successful BPA.

Unmet need in pulmonary hypertension-associated interstitial lung disease (PH-ILD): a clinician survey of real-world management of PH-ILD in Europe.

With no approved therapies for pulmonary hypertension (PH) associated with interstitial lung disease (PH-ILD) in Europe, we surveyed clinician perceptions on PH-ILD management and unmet need to understand current real-world practices. An online clinician survey on PH-ILD management was conducted in France, Germany, Italy, Spain and the UK. 55 clinicians (78% pulmonologists), each managing a median 20 PH-ILD patients (interquartile range (IQR) 10-50), participated. Upon PH suspicion, clinicians referred a median 50% (IQR 20-73%) of patients for echocardiography alone and 35% (IQR 20-78%) for echocardiography, followed by right heart catheterisation. Upon diagnosis, a median 20% (IQR 9-30%), 40% (IQR 20-50%) and 35% (IQR 20-55%) of patients fell under the pulmonary arterial pressure ranges of 21-24 mmHg, 25-34 mmHg and >35 mmHg, respectively. 50% of patients received off-label treatment for their PH and, of those, off-label phosphodiesterase-5 inhibitor (PDE-5i), endothelin receptor antagonist (ERA) and prostacyclin analogues were prescribed first-line by 78%, 9% and 7% of clinicians, respectively. Upon PDE-5i non-response, 35% of clinicians proceed with an ERA, 35% with no further therapy. 55% of clinicians used dual-therapy. Yearly median inpatient admissions and emergency visits were 2.0 (IQR 1.3-2.9) and 1.5 (IQR 1.0-2.0), respectively (n=31 responses). Most clinicians (69%) highlighted lack of efficacy or evidence for current therapies as a key gap in PH-ILD management. This study gives insight into real-world European PH-ILD diagnosis and management. With significant use of off-label treatment, there is a large unmet need due to lack of approved therapies. Despite updated guidelines, more evidence is needed to standardise PH-ILD management.

Invasive haemodynamics predict outcomes in paediatric pulmonary artery hypertension.

Invasive haemodynamics are often performed for initiating and guiding pulmonary artery hypertension therapy. Little is known about the predictive value of invasive haemodynamic indices for long-term outcomes in children with pulmonary artery hypertension. We aimed to evaluate invasive haemodynamic data to help predict outcomes in paediatric pulmonary artery hypertension. Patients with pulmonary artery hypertension who underwent cardiac catheterisation (2006-2019) at a single centre were included. Invasive haemodynamic data from the first cardiac catheterisation and clinical outcomes were reviewed. The combined adverse outcome was defined as pericardial effusion (due to right ventricle failure), creation of a shunt for pulmonary artery hypertension (atrial septal defect or reverse Pott's shunt), lung transplant, or death. Among 46 patients with a median [interquartile range (IQR)] age of 13.2 [4.1-44.7] months, 76% had CHD. Median mean pulmonary artery pressure was 37 [28-52] mmHg and indexed pulmonary vascular resistance was 6.2 [3.6-10] Woods units × m2. Median pulmonary artery pulsatility index was 4.0 [3.0-4.7] and right ventricular stroke work index was 915 [715-1734] mmHg mL/m2. After a median follow-up of 2.4 years, nine patients had a combined adverse outcome (two had a pericardial effusion, one underwent atrial level shunt, one underwent reverse Pott's shunt, and six died). Patients with an adverse outcome had higher systolic and mean pulmonary artery pressures, higher diastolic and transpulmonary pressure gradients, higher indexed pulmonary vascular resistance, higher pulmonary artery elastance, and higher right ventricular stroke work index (p < 0.05 each). Invasive haemodynamics (especially mean pulmonary artery pressure and diastolic pressure gradient) obtained at first cardiac catheterisation in children with pulmonary artery hypertension predicts outcomes.

(956) - Relationship Between Use of Preoperative Pulmonary Hypertension Therapy and Need for Postoperative Therapy in Patients Undergoing Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension

(956) - Relationship Between Use of Preoperative Pulmonary Hypertension Therapy and Need for Postoperative Therapy in Patients Undergoing Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension

Use of pulmonary arterial hypertension therapies in patient swith sarcoidosis-associated pulmonary hypertension.

Use of pulmonary arterial hypertension therapies in patient swith sarcoidosis-associated pulmonary hypertension.

Congenital alveolar dysplasia and alveolar capillary dysplasia with misalignment of the pulmonary veins: clinical and anatomical comparisons

Congenital alveolar dysplasia (СAD) and alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) are rare genetically determined interstitial lung diseases in infants that manifest in the first hours to days after birth, have fatal outcome and are usually diagnosed at autopsy.The aim of this work was to consider the clinical manifestations and pathomorphologic picture of CAD and ACD/MPV based on clinical observations of newborns who were on a ventilator, received therapy for high pulmonary hypertension and extracorporeal membrane oxygenation (ECMO). The clinical characteristics and medical history of the patients are described. The results of lifetime imaging methods, autopsy examinations with light microscopy and the use of routine staining methods of micro-preparations as well as immunohistochemical methods are considered. Morphologic changes in the lungs characteristic of CAD and ACD/MPV are described in detail in the analysis of histologic examination data.Conclusion. The pathoanatomical examination data are of crucial importance for the diagnosis of CAD and ACD/MPV, which must be suspected in newborns with respiratory insufficiency and severe pulmonary hypertension when ventilation, inhalation of nitric oxide and ECMO are ineffective.

Bosentan in the treatment of persistent pulmonary hypertension in newborns: a systematic review and meta-analysis.

Persistent pulmonary hypertension of the newborn is a life-threatening condition that affects about 1-2 per 1,000 live births worldwide. Bosentan is an oral dual endothelin receptor antagonist that may have a beneficial effect on persistent pulmonary hypertension of the newborn by reducing pulmonary vascular resistance and improving oxygenation. However, its role in persistent pulmonary hypertension of the newborn remains unclear. To systematically evaluate the efficacy and safety of bosentan as an adjuvant therapy for persistent pulmonary hypertension of the newborn in newborns. We searched six English and two Chinese databases from their inception to 1 January 2023 following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We included randomised controlled trials and retrospective studies that compared bosentan with placebo or other drugs for persistent pulmonary hypertension of the newborn in newborns. We performed a meta-analysis using random-effects models and assessed the risk of bias and heterogeneity in the included studies. We included 10 studies with a total of 550 participants. Bosentan significantly reduced the treatment failure rate (relative risk = 0.25, P < 0.001), pulmonary artery pressure (mean difference = -11.79, P < 0.001), and length of hospital stay (mean difference = -1.04, P = 0.003), and increased the partial pressure of oxygen (mean difference = 10.02, P < 0.001) and blood oxygen saturation (SpO2) (mean difference = 8.24, P < 0.001) compared with a placebo or other drugs. The occurrence of adverse reactions was not significantly different between bosentan and a placebo or other drugs. Bosentan is effective in the treatment of persistent pulmonary hypertension of the newborn but adverse reactions such as abnormal liver function should be observed when using it.

Low-frequency ultrasound for pulmonary hypertension therapy

BackgroundCurrently, there are no reliable clinical tools that allow non-invasive therapeutic support for patients with pulmonary arterial hypertension. This study aims to propose a low-frequency ultrasound device for pulmonary hypertension therapy and to demonstrate its potential.MethodsA novel low-frequency ultrasound transducer has been developed. Due to its structural properties, it is excited by higher vibrational modes, which generate a signal capable of deeply penetrating biological tissues. A methodology for the artificial induction of pulmonary hypertension in sheep and for the assessment of lung physiological parameters such as blood oxygen concentration, pulse rate, and pulmonary blood pressure has been proposed.ResultsThe results showed that exposure of the lungs to low-frequency ultrasound changed physiological parameters such as blood oxygen concentration, pulse rate and blood pressure. These parameters are most closely related to indicators of pulmonary hypertension (PH). The ultrasound exposure increased blood oxygen concentration over a 7-min period, while pulse rate and pulmonary blood pressure decreased over the same period. In anaesthetised sheep exposed to low-frequency ultrasound, a 10% increase in SpO2, a 10% decrease in pulse rate and an approximate 13% decrease in blood pressure were observed within 7 min.ConclusionsThe research findings demonstrate the therapeutic efficiency of low-frequency ultrasound on hypertensive lungs, while also revealing insights into the physiological aspects of gas exchange within the pulmonary system.

Therapies of chronic thromboembolic pulmonary hypertension: A review

The article addresses therapies for chronic thromboembolic pulmonary hypertension (CTEPH). The main challenges of patient management at various stages of diagnosis and treatment and the prevalence and pathogenesis of CTEPH are described. Immediate and long-term outcomes after treatment are evaluated. The study aims to present current therapies for CTEPH.

Idiopathic Pulmonary Hypertension and in Situ Thromboembolism: A Difficult Case in Clinical Practice

Pulmonary hypertension is a complex syndrome for differential diagnosis, which is the outcome of various pathological conditions. With the exclusion of the two most common causes of pulmonary hypertension, such as pathology of the left heart chambers and pulmonary embolism, further search for etiology often becomes problematic. Despite the emergence of a number of international and domestic recommendations, as well as certain successes in drug therapy, the long-term prognosis in patients with pulmonary arterial hypertension remains unfavorable. In the description of this clinical case in a 39-year-old woman suffering from idiopathic pulmonary arterial hypertension, the main complaint of the patient was very non-specific. The woman could not bear the child, all her attempts, lasting more than 19 years, remained unsuccessful. Even in absolutely healthy women, pregnancy is associated with the highest risks and is a powerful “test” of the body, not to mention patients suffering from rare diseases. The patient has a “subacute” course and a fairly rapid progression of the disease with a significant deterioration in the quality of life, which led to the impossibility of carrying a pregnancy. There were also signs that aggravated the prognosis, such as increased dyspnea, loss of consciousness, a significant decrease in working capacity and a high degree of pulmonary hypertension (according to echocardiography, systolic pressure in the pulmonary artery &gt; 128 mm Hg). Due to the ineffectiveness of standard therapy with a selective inhibitor of cycloguanosine monophosphate — specific phosphodiesterase type 5 — sildenafil; the option of specific therapy for pulmonary hypertension was considered, which made it possible to change the situation and bring the patient into a stable state and draw conclusions about the pregnancy.

Percutaneous Closure of Hemodynamically Significant Postoperative Residual Ventricular Septal Defects.

The experience with percutaneous closure of postoperative residual ventricular septal defects (VSDs) is expanding with improved device technology and techniques. To report our experience with percutaneous closure of residual VSDs after cardiac surgeries. Retrospective clinical data review of patients who had percutaneous closure of postoperative residual VSDs at our institution between 2010 and 2022. Patients' demographics, procedural, and follow-up data were looked at. Twelve patients (50% males) with a median age of 9.2years (range 0.9-22) were identified. Baseline surgeries were 8 tetralogy of Fallot corrections, 2 pulmonary bandings for large muscular VSD (mVSD) including 1 coarctation repair, 1 atrioventricular septal defect repair, 1 sub-aortic membrane resection-induced iatrogenic VSD, 1 isolated VSD closure, and 1 additional mVSD. Median duration between baseline surgery and percutaneous VSD closure was 2.2years (range 0.2-8.3). Residual VSD shunting was secondary to surgical patch leakage in 8/12 patients. The median angiographic defect diameter was 6.8mm (range 4.8-14). The defect was balloon-calibrated in 3/12 patients. Defects were tackled retrogradely in 3/9 patients. Amplatzer Membranous VSD occluder (n = 1), Lifetech Multifunctional (n = 5), Membranous (n = 1) and muscular VSD occluders (n = 2) and Occlutech Membranous (n = 1) and Muscular (n = 2) VSD occluders were used. The procedure was successful in 10/12 patients. Two devices embolized to the pulmonary artery and were snare-retrieved. Both patients were referred for surgery. The median follow-up was 1.3years (range 0.1-12). Six-month ultrasound showed one trivial residual shunt and one mild right ventricular outflow obstruction. One patient is receiving targeted therapy for pulmonary hypertension at 2years of follow-up. Transcatheter closure of postoperative residual VSDs is a feasible yet challenging intervention. Procedural complications can be encountered.

Targeting hypoxia-inducible factors: therapeutic opportunities and challenges.

Hypoxia-inducible factors (HIFs) are highly conserved transcription factors that are crucial for adaptation of metazoans to limited oxygen availability. Recently, HIF activation and inhibition have emerged as therapeutic targets in various human diseases. Pharmacologically desirable effects of HIF activation include erythropoiesis stimulation, cellular metabolism optimization during hypoxia and adaptive responses during ischaemia and inflammation. By contrast, HIF inhibition has been explored as a therapy for various cancers, retinal neovascularization and pulmonary hypertension. This Review discusses the biochemical mechanisms that control HIF stabilization and the molecular strategies that can be exploited pharmacologically to activate or inhibit HIFs. In addition, we examine medical conditions that benefit from targeting HIFs, the potential side effects of HIF activation or inhibition and future challenges in this field.

Predictors of therapeutic response to specific therapy in distal chronic thromboembolic pulmonary hypertension

Aim. To assess therapeutic response to specific therapy in patients with distal CTEPH who are ineligible for surgical management as pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty (BPA).Materials and methods. The study included inoperable patients with CTEPH (n=53) who are ineligible for surgical management as pulmonary endarterectomy or balloon pulmonary angioplasty. The effectiveness of specific therapy were assessed after dividing the patients into improvement or nonimprovement groups based on the following indicators: functional class I-II (WHO), mean pulmonary artery pressure according to right heart catheterization ≤40 mm Hg, pulmonary vascular resistance ≤400 dyn×s×cm-5, cardiac index ≥2,5 L/min/m2 , decrease in N-terminal brain natriuretic peptide &gt;30%, and increase in 6-minute test distance &gt;30 m at 12th-month follow-up compared to baseline parameters.Results. There were no differences in groups by gender, age, and received specific therapy. Multiple logistic regression was performed, resulting in a model for predicting clinical improvement with sensitivity (57,1%) and specificity (87,1%), p=0,002. It was revealed, that the absence of the loss of pulmonary vascularity by X-ray at the time of verification of the diagnosis in patients with CTEPH by 5,91 times increases the chance of achieving clinical improvement after 12 months of the specific therapy, while the absence of signs of heart failure by 5,79 times, and degree of pulmonary valve insufficiency 0-1 according to echocardiography by 9,2 times, respectively.Conclusion. Early initiation of specific therapy in inoperable CTEPH patients due to distal lesions with the absence of severe vascular remodeling and heart failure symptoms leads to the better therapeutic response after 12 months of treatment.

Perioperative and Long-Term Outcomes in Infants Undergoing a Tracheostomy from a Neonatal Intensive Care Unit.

The purpose of this study was to identify risk factors for perioperative complications and long-term morbidity in infants from the neonatal intensive care unit (NICU) presenting for a tracheostomy. This single-center retrospective cohort study included infants in the NICU presenting for a tracheostomy from August 2011 to December 2019. Primary outcomes were categorized as either a perioperative complication or long-term morbidity. A severe perioperative complication was defined as having either (1) an intraoperative cardiopulmonary arrest, (2) an intraoperative death, (3) a postoperative cardiopulmonary arrest within 30 days of the procedure, or (4) a postoperative death within 30 days of the procedure. Long-term morbidities included (1) the need for gastrostomy tube placement within the tracheostomy hospitalization and (2) the need for diuretic therapy, pulmonary hypertensive therapy, oxygen, or mechanical ventilation at 12 and 24 months following the tracheostomy. One-hundred eighty-three children underwent a tracheostomy. The mean age at tracheostomy was 16.9 weeks while the mean post-conceptual age at tracheostomy was 49.7 weeks. The incidence of severe perioperative complications was 4.4% (n = 8) with the number of pulmonary hypertension medication classes preoperatively (OR: 3.64, 95% CI: (1.44-8.94), p = 0.005) as a significant risk factor. Approximately 81% of children additionally had a gastrostomy tube placed at the time of the tracheostomy, and 62% were ventilator-dependent 2 years following their tracheostomy. Our study provides critical perioperative complications and long-term morbidity data to neonatologists, pediatricians, surgeons, anesthesiologists, and families in the expected course of infants from the NICU presenting for a tracheostomy. 3 Laryngoscope, 134:1945-1954, 2024.

Stem cell therapy in pulmonary hypertension: current practice and future opportunities.

Pulmonary hypertension (PH) is a progressive disease characterised by elevated pulmonary arterial pressure and right-sided heart failure. While conventional drug therapies, including prostacyclin analogues, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors, have been shown to improve the haemodynamic abnormalities of patients with PH, the 5-year mortality rate remains high. Thus, novel therapies are urgently required to prolong the survival of patients with PH. Stem cell therapies, including mesenchymal stem cells, endothelial progenitor cells and induced pluripotent stem cells, have shown therapeutic potential for the treatment of PH and clinical trials on stem cell therapies for PH are ongoing. This review aims to present the latest preclinical achievements of stem cell therapies, focusing on the therapeutic effects of clinical trials and discussing the challenges and future perspectives of large-scale applications.

Real-world safety and effectiveness of inhaled nitric oxide therapy for pulmonary hypertension during the perioperative period of cardiac surgery: a post-marketing study of 2817 patients in Japan

ObjectiveTo evaluate the real-world safety and effectiveness of inhaled nitric oxide (INOflo® for Inhalation 800 ppm) for perioperative pulmonary hypertension associated with cardiac surgery in Japan.MethodsThis was a prospective, non-interventional, all-case, post-marketing study of pediatric and adult patients who received perioperative INOflo with cardiac surgery from November 2015–December 2020. Safety and effectiveness were monitored from INOflo initiation to 48 h after treatment completion or withdrawal. Safety outcomes included adverse drug reactions, blood methemoglobin concentrations, and inspired nitrogen dioxide concentrations over time. Effectiveness outcomes included changes in central venous pressure among pediatrics, mean pulmonary arterial pressure among adults, and the partial pressure of arterial oxygen/fraction of inspired oxygen ratio (PaO2/FiO2) in both populations.ResultsThe safety analysis population included 2,817 Japanese patients registered from 253 clinical sites (pediatrics, n = 1375; adults, n = 1442). INOflo was generally well tolerated; 15 and 20 adverse drug reactions were reported in 14 pediatrics (1.0%) and 18 adults (1.2%), respectively. No clinically significant elevations in blood methemoglobin and inspired nitrogen dioxide concentrations were observed. INOflo treatment was associated with significant reductions in both central venous pressure among pediatrics and mean pulmonary arterial pressure among adults, and significant improvements in PaO2/FiO2 among pediatrics and adults with PaO2/FiO2 ≤ 200 at baseline.ConclusionsPerioperative INOflo treatment was a safe and effective strategy to improve hemodynamics and oxygenation in patients with pulmonary hypertension during cardiac surgery. These data support the use of INOflo for this indication in Japanese clinical practice.

Comparison of different treatment strategies in patients with chronic thromboembolic pulmonary hypertension: a single centre real-world experience

BackgroundPulmonary endarterectomy (PEA) has been the most effective therapy for chronic thromboembolic pulmonary hypertension (CTEPH). However, there is a substantial proportion of patients deemed not operable in whom other treatment strategies are available: medical therapy and balloon pulmonary angioplasty (BPA). We aimed to compare different CTEPH treatment strategies effect in a real-world setting. MethodsAll patients with CTEPH referred to our centre were included. We compare the short-term clinical, functional, exercise and haemodynamic effect of medical therapy (irrespective of subsequent treatment strategies), PEA and BPA (irrespective of previous/subsequent treatment strategies); we also describe the long-term outcome of the different patient groups. ResultsWe included 467 patients (39% were treated only with medical therapy, 43% underwent PEA, 13% underwent BPA and 5% were not treated with any therapy). Patients treated only with medical therapy were the oldest; compared to patients undergoing PEA, they had a lower exercise capacity, a higher risk profile and gained a lower haemodynamic, functional and survival benefit from the treatment. Patients undergoing BPA had a lower haemodynamic improvement but a comparable functional, exercise and risk improvement and a similar survival compared to patients undergoing PEA; their survival is anyway better than patients undergoing only medical treatment. Untreated historical control patients had the worst survival. ConclusionsWe confirm the superiority of PEA compared to any alternative treatment in CTEPH patients and we observe that BPA, in patients deemed not operable or with persistent/recurrent PH after PEA, leads to a better outcome than medical therapy alone.

Prenatal MRI assessment of mediastinal shift angle as a feasible and effective risk stratification tool in isolated right-sided congenital diaphragmatic hernia.

To develop a mediastinal shift angle (MSA) measurement method applicable to right-sided congenital diaphragmatic hernia (RCDH) in fetal MRI and to validate the predictive value of MSA in RCDH. Twenty-seven fetuses with isolated RCDH and 53 controls were included in our study. MSA was measured on MRI axial image at the level of four-chamber view of the fetal heart. The angle between the sagittal midline landmark line and the left boundary landmark line touching tangentially the lateral wall of the left ventricle was used to quantify MSA for RCDH. Appropriate statistical analyses were performed to determine whether MSA can be regarded as a valid predictive tool for postnatal outcomes. Furthermore, predictive performance of MSA was compared with that of lung area to head circumference ratio (LHR), observed/expected LHR (O/E LHR), total fetal lung volume (TFLV), and observed/expected TFLV (O/E TFLV). MSA was significantly higher in the RCDH group than in the control group. MSA, LHR, O/E LHR, TFLV, and O/E TFLV were all correlated with postnatal survival, pulmonary hypertension (PH), and extracorporeal membrane oxygenation (ECMO) therapy (p < 0.05). Value of the AUC demonstrated good predictive performance of MSA for postnatal survival (0.901, 95%CI: (0.781-1.000)), PH (0.828, 95%CI: (0.661-0.994)), and ECMO therapy (0.813, 95%CI: (0.645-0.980)), which was similar to O/E TFLV but slightly better than TFLV, O/E LHR, and LHR. We developed a measurement method of MSA for RCDH for the first time and demonstrated that MSA could be used to predict postnatal survival, PH, and ECMO therapy in RCDH. Newly developed MRI assessment method of fetal MSA in RCDH offers a simple and effective risk stratification tool for patients with RCDH. • We developed a measurement method of mediastinal shift angle for right-sided congenital diaphragmatic hernia for the first time and demonstrated its feasibility and reproducibility. • Mediastinal shift angle can predict more prognostic information other than survival in right-sided congenital diaphragmatic hernia with good performance. • Mediastinal shift angle can be used as a simple and effective risk stratification tool in right-sided congenital diaphragmatic hernia to improve planning of postnatal management.