Pulmonary Hypertension In Adults Research Articles

Pulmonary hypertension in adults with congenital heart disease in light of the 2022-ESC-PAH guidelines - Part II: Supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesions, left ventricular disorders, univentricular hearts), interventions, intensive care, follow-up, future perspectives

The number of adults with congenital heart defects (CHD) is steadily rising and amounts to approximately 360,000 in Germany. CHD is often associated with pulmonary hypertension (PH), which may develop early in untreated CHD. Despite timely treatment of CHD, PH not infrequently persists or recurs in older age and is associated with significant morbidity and mortality.The revised European Society of Cardiology/European Respiratory Society 2022 guidelines for the diagnosis and treatment of PH represent a significant contribution to the optimized care of those affected. However, the topic of "adults with congenital heart disease" is addressed only relatively superficial in these guidelines. Therefore, in the present article, this topic is commented in detail from the perspective of congenital cardiology.

Examining prevalence and predictors of pulmonary hypertension in adults with idiopathic pulmonary fibrosis: a population-based analysis in the United States.

Pulmonary hypertension (PH) often complicates idiopathic pulmonary fibrosis (IPF), a progressive parenchymal lung disease. We investigated predictors of PH in IPF hospitalizations in the United States. We identified IPF hospital- izations with or without PH using the National Inpatient Sample (2018) and relevant ICD-10-CM codes. We com- pared demographics, comorbidities, PH prevalence, and its multivariable predictors adjusted for confounders among patients with IPF. In 2018, 30,335 patients from 30,259,863 hospitalizations had IPF, of which 8,075 (26.6%) had PH. Black (41%), Hispanic (21.3%), and female (28.7%) patients had higher rates of PH compared to white patients (25%). The IPF-PH cohort was hospitalized more often in urban teaching (77.7% vs. 72.2%), Midwest, and West hospitals vs. non-PH cohort. Comorbidities including congestive heart failure (2.08 [1.81-2.39]), valvular disease (2.12 [1.74-2.58]), rheumatoid arthritis/collagen vascular disease (1.32 [1.08-1.61]) predicted higher odds of PH. The PH-IPF cohort was less often routinely discharged (35.4%) and more likely to be transferred to intermediate care facilities (22.6%) and home health care (27.1%) (P < 0.001). The PH-IPF group had higher rates of all-cause mortality (12.3% vs. 9.4%), cardiogenic shock (2.4% vs. 1%), dysrhythmia (37.6% vs. 29%), and cardiac arrest (2.7% vs. 1.5%) vs. non-PH cohort (all P < 0.001). Patients with PH-IPF also had longer hospital stays (9 vs. 8) and a higher median cost ($23,054 vs. $19,627, P < 0.001). Nearly 25% of IPF hospitalizations with PH were linked to higher mortality, extended stays, and costs, emphasizing the need to integrate demographic and comorbidity predictors into risk stratification for improved outcomes in patients with IPF-PH.

Pulmonary hypertension in adults completing tuberculosis treatment

Background. Pulmonary hypertension (PH) after tuberculosis (TB) is typically not included among the chronic lung diseases causing PH (group 3 PH), with few data available to support the inclusion. Objectives. To determine the prevalence of PH in an adult population completing TB treatment. Methods. This single-centre, cross-sectional study only included patients with their first documented episode of TB, and who were in the second half of treatment or had recently completed treatment. PH was assessed using transthoracic echocardiography. Questionnaires were also completed and spirometry and a 6-minute walk test were performed. Results. One hundred patients were enrolled, with a mean age of 37.1 years, of whom 58% were male and 46% HIV positive. The median time since initiation of TB treatment was 22 weeks. The mean (standard deviation) measured right ventricular systolic pressure (RVSP) was 23.6 (6.24) mmHg. One participant had PH (defined as RVSP ≥40 mmHg; 95% confidence interval (CI) 0.0 - 3.0) and a further 3 had possible PH (RVSP ≥35 and &lt;40 mmHg), with a combined PH prevalence of 4% (95% CI 0.2 - 7.8). Airflow obstruction on spirometry was found in 13.3% of 98 patients, while 25.5% had a reduced forced vital capacity. There was no association between RVSP or PH/possible PH and sex, age, HIV status, systemic hypertension, spirometry measurements or 6-minute walking distance. Smoking status was associated with RVSP, but not with the presence of PH/possible PH. Conclusion. There was a significant prevalence of PH in this preliminary study of predominantly young patients completing treatment for a first episode of TB. Larger and more detailed studies are warranted.

Surgical Treatment of Congenital Heart Diseases Associated with Pulmonary Hypertension in Adults

The outcome of treatment of adult patients with congenital heart disease (CHD) and pulmonary hypertension (PH) depends on the adequacy of diagnosis with a thorough assessment of hemodynamics and the correctness of the chosen tactics. Enormous success has been achieved, various classifications and algorithms for managing children and adults with this nosology have been created and are widely used. Thanks to the creation and widespread introduction into clinical practice of various types of drugs, the results of surgical treatment of CHD with PH have improved significantly. A new cluster of patients has emerged who can undergo palliative surgery against the background of irreversible pulmonary hypertension, leading to a significant improvement in the quality and life expectancy. At the same time, to this day, it is far from always possible to evaluate each specific case with full confidence in the possibility of performing surgical treatment. Modern advances in CHD cardiac surgery have significantly expanded the indications for the correction of defects with high PH in adults. Operability (implying normalization of pulmonary artery pressure) and indications for palliative surgery (without regression of PH, but with an improvement in the quality and life expectancy of patients) are still the main debatable issues.

Pulmonary Hypertension in Adults With Aortic Valve Disease: Prevalence and Influence on Outcomes From the National Echo Database of Australia

Pulmonary Hypertension in Adults With Aortic Valve Disease: Prevalence and Influence on Outcomes From the National Echo Database of Australia

Prevalence of pulmonary hypertension in aortic regurgitation and its influence on outcomes

ObjectiveAortic regurgitation (AR) can lead to pulmonary hypertension (PHT). There is a paucity of data on the prognostic importance of PHT in these patients. We therefore aimed to describe the...

5612390 CORRELATION OF DOPPLER DEFINED ELEVATED PULMONARY PRESSURE WITH LABORATORY PARAMETERS IN ADULT PATIENTS WITH SICKLE CELL DISEASE - A RETROSPECTIVE STUDY

Background: Pulmonary hypertension (PH) is a relatively common complication and known cause of death in adult patients with sickle cell disease (SCD). Trans-thoracic Echocardiography is a non-invasive way to screen SCD patients for elevated pulmonary pressure. An estimation of the pulmonary artery systolic pressure can be made via assessment of the Tricuspid jet regurgitation velocity(TRV). TRV greater than or equal to 2.5 m/sec corresponds to an estimated Pulmonary Artery Systolic pressure of >35 mm Hg, which is approximately two standard deviations above normal 1.The prevalance of elevated pulmonary pressure in adults with SCD is 30-40%, while the prevalance of PH in SCD patients utilizing right heart catheterization (RHC) is between 6-10.5% 2. A major risk factor for PH in sickle cell disease is the severity of hemolytic anemia 3. The level of N terminal fragment of brain natriuretic peptide (NT ProBNP) correlates with the degree of pulmonary vascular resistance in SCD patients and may be an indirect reflection of the end organ damage 4. Aims: To analyse the prevalence of Doppler defined pulmonary hypertension in adult patients with Sickle cell Disease visiting our centre. To correlate the severity of hemolysis using laboratory parameters and NT-ProBNP with the severity of PH in these patients Methods: 92 patients with SCD underwent baseline clinical evaluation, laboratory testing, 2D echocardiography and NT-ProBNP analysis in our Hospital from November 2020 - December 2021. TRVmax of ≥2.5m/s, was used as a surrogate marker of Pulmonary Hypertension. The severity of PH was correlated with the degree of hemolysis and NT Pro BNP levels. Results: IBM SPSS Statistics 24.0 was used for Statistical Analysis. Doppler-defined pulmonary hypertension was found among 29 out of 92 patients denoting a prevalence of 31.5%. The prevalence among males was 20.9% and that among females was 40.8%. TRV max had significant positive correlation with NT-ProBNP(r=0.310,p<0.01) and with markers of hemolysis like LDH (r=0.322,p<0.01)and Reticulocyte count(r=0.212,p<0.05). Serum Ferritin(r=0.233,p<0.05),Hemoglobin S(%)(r=0.374,p<0.01) and Left Ventricular ejection fraction (LVEF) (r=0.352,p<0.01) also had significant correlation with TRV Max. Patients with high serum ferritin and HbS percent had higher TRV max while LVEF was low in those with high TRVmax. TRV Max also exhibited significant negative correlation with Hemoglobin (r=- 0.340, p<0.01). Summary: In our study, the prevalence of doppler - defined Pulmonary hypertension in adults with sickle cell disease was 31.5%. These patients had a positive correlation with markers of hemolysis and levels of NT-Pro BNP. Hence, we conclude, transthoracic echocardiography and NT proBNP can be used as a screening tool and surrogate test to Right heart catheterisation in order to diagnose PH in SCD patients in resource constrained settings.Both these tests have a high sensitivity in screening SCD patients with PH and having the advantage of being non-invasive procedure.

TCTAP A-098 When to Release the Device in Transcatheter Closure for Large Patent Ductus Arteriosus With Severe Pulmonary Hypertension in Adults?

TCTAP A-098 When to Release the Device in Transcatheter Closure for Large Patent Ductus Arteriosus With Severe Pulmonary Hypertension in Adults?

Effect of Phosphodiesterase-5 (PDE-5) Inhibitors on Clinical Outcomes in Patients With Pulmonary Hypertension: A Meta-Analysis of Randomized Control Trials.

We intended to summarize the most recent research pertaining to the use of phosphodiesterase-5(PDE5) inhibitors in pulmonary hypertension in light of recent developments in the knowledge of the pathophysiological mechanisms and treatments for pulmonary hypertension, with major contributions in the area in the last decade. The aim of this meta-analysis is to determine the efficacy of PDE5 inhibitors for pulmonary hypertension in adults. We followed the preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines to carry out this meta-analysis. Online database searching to identify eligible trials was performed in MEDLINE, EMBASE, and the Cochrane Library by two authors independently. Outcomes assessed in the current meta-analysis included change in the cardiac index from baseline in liters per minute per square meter (L/min/m2), mean peripheral arterial pressure (PAP) in mm Hg, mortality, hospitalization, and six-minute walking distance (6MWD) in meters (m). Overall, 17 articles met the inclusion criteria and were included in the current meta-analysis. PDE5 inhibitors significantly improve cardiac index (mean difference: 0.18, 95% CI: 0.04, 0.32, p-value: 0.01), mean PAP (mean difference: -5.61, 95% CI: -7.60, -3.62, p-value: 0.01), and 6MWD (mean difference: 26.26, 95% CI: 16.95, 35.57, p-value: 0.001) as compared to the patients in the control group. No significant difference was found in terms of risk of mortality (risk ratio (RR):0.51, 95% CI: 0.17, 1.54) and risk of hospitalization (RR: 0.59, 95% CI: 0.23, 1.55) between the two groups. The current meta-analysis concluded that PDE5 inhibitors improve 6MWD, mean PAP, and cardiac index in patients with pulmonary hypertension. However, no significant difference was reported in terms of mortality and hospitalization between the two groups.

Применение селективного ингибитора фосфодиэстеразы–5 силденафила при беременности с учетом его возможного влияния на плод

Objective of the Review: To evaluate the possibilities and effectiveness of the use of sildenafil in obstetric practice, its effect on both the fetus and the mother's body. Key points. Sildenafil is a potent vasodilator drug used to treat pulmonary hypertension in adults and children. In addition to the vasodilating effect, sildenafil has a proliferative effect on the vascular network, reduces the muscle mass of the vascular wall. The vasodilating effect of sildenafil also appears on the uterus and myometrial vessels, which leads to increased uterine circulation and thickening of the endometrium. Its use during pregnancy is mainly limited to a few specific indications. In particular, high pulmonary hypertension – is sufficient reason to prescribe this drug off–label even at the stage of pregnancy planning. To date, antenatal therapy with sildenafil for conditions such as preeclampsia, preterm labor, oligohydramnios, congenital diaphragmatic hernia, intranatal fetal distress, and intrauterine growth restriction are being actively studied in many countries. More than 130 in vivo and ex vivo animal studies, pharmacological and toxicokinetic studies have demonstrated the safety of sildenafil. It has been proven that there is no teratogenic effect, as well as an increase in the frequency of congenital anomalies in the fetus. Conclusion. The use of sildenafil during pregnancy is of interest to researchers around the world. Based on preliminary data, it can be assumed that serious side effects in pregnant women, negative effects on the fetus, an increase in the frequency of stillbirths, neonatal deaths and congenital anomalies associated with taking sildenafil were not observed. However, additional fundamental studies are needed, including using experimental animal models, in order to finally evaluate the ability of sildenafil to overcome the transplacental barrier, as well as the possibility and effectiveness of its use in various groups of patients. Keywords: sildenafil, transplacental transfer, safety during pregnancy, pharmacology, hemodynamics.

Bosentan Is Associated with a Reduction in Right Ventricular Systolic Pressure N-Terminal Pro-Hormone B-Type Natriuretic Peptide Levels in Young Patients with Pulmonary Hypertension

Pulmonary hypertension is a rare and potentially fatal disease in children if left untreated. Emerging therapies, including Bosentan, a dual endothelin receptor antagonist, have shown significant benefits in the adult pulmonary hypertension population; however, few studies have assessed the efficacy and safety of endothelin receptor antagonists in infants and young children. Our study was a single-center retrospective analysis of patients less than two years of age with a confirmed diagnosis of pulmonary hypertension and initiated on Bosentan therapy between 2017 and 2020. Twelve cases met eligibility criteria. Demographic data, laboratory data, echocardiographic, and cardiac catheterization data were analyzed. With treatment, there was a statistically significant decrease in mean right ventricular systolic pressure estimated by the tricuspid regurgitation jet (79 ± 23 mmHg reduced to 52 ± 25 mmHg; p < 0.001) N-terminal pro-hormone B-type natriuretic peptide levels (21,071 reduced to 2,037; p < 0.001). Additionally, improvement and eventual normalization of right ventricular function and septal geometry was seen within the first four months of therapy. Patients who underwent cardiac catheterization after therapy initiation (n = 4) demonstrated hemodynamic improvements; however, only the decrease in diastolic pulmonary artery pressure was statistically significant (p = 0.018). No significant differences in hemoglobin, platelet count, or liver function tests were observed between groups. In conclusion, these data suggest that Bosentan may be an effective and relatively safe treatment option for children less than two years of age with pulmonary hypertension. Further long-term randomized control studies are necessary to validate the potential clinical benefit of utilizing this drug therapy in young children.

Optical coherence tomography of the pulmonary arteries in children with congenital heart diseases: A systematic review.

Optical coherence tomography (OCT) is a high-resolution intravascular imaging tool and has shown promise for providing real-time quantitative and qualitative descriptions of pulmonary vascular structures in vivo in adult pulmonary hypertension (PH), while not popular in pediatric patients with congenital heart diseases (CHD). The aim of this review is to summarize all the available evidence on the use of OCT for imaging pulmonary vascular remodeling in pediatric patients. We conducted the systematic literature resources (Cochran Library database, Medline via PubMed, EMBASE, and Web of Knowledge) from January 2010 to December 2021 and the search terms were "PH", "child", "children", "pediatric", "OCT", "CHD", "pulmonary vessels", "pulmonary artery wall". Studies in which OCT was used to image the pulmonary vessels in pediatric patients with CHD were considered for inclusion. Five studies met the inclusion criteria. These five papers discussed the study of OCT in the pulmonary vasculature of different types of CHD, including common simple CHD, complex cyanotic CHD, and Williams-Beuren syndrome. In biventricular anatomy, pulmonary vascular remodeling was primarily reflected by pulmonary intima thickening from two-dimensional OCT. In single-ventricle anatomy, due to the state of hypoxia, the morphology of pulmonary vessels was indirectly reflected by the number and shape of nourishing vessels from three-dimensional OCT. OCT may be an adequate imaging procedure for the demonstration of pulmonary vascular structures and provide additional information in pediatric patients.

Doppler trans-thoracic echocardiography for detection of pulmonary hypertension in adults.

Doppler trans-thoracic echocardiography for detection of pulmonary hypertension in adults.

Organization of pharmaceutical provision of patients with pulmonary hypertension on the example of Kyiv

In Ukraine, pharmaceutical provision of patients with pulmonary hypertension (PH) as a rare (orphan) disease is one of the important components of the State policy. Despite the permanent reform of the medical and pharmaceutical sector, a significant number of problems in the formation of a balanced system for providing the necessary medicinal products (MP) to orphan patients have not yet been resolved.&#x0D; The purpose of the study is to study the process of pharmaceutical provision of patients with PH, using the example of Kyiv.&#x0D; Information on MP purchases for patients in Kyiv, included in the Unified Clinical Protocol for Emergency, Primary, Secondary (Specialized) Medical Care Pulmonary Hypertension in Adults for the State and local budgets for 2018–2021, has been chosen as the object of the study. We have used system-review, documentary, and marketing research methods.&#x0D; The result of the analysis of the annual need for 2018–2021 in INN, necessary for the pharmaceutical provision of Kyiv patients with PH, has shown that the annual decrease in funding compared to 2018, namely: in 2019– by 17.54%, in 2020– by 32.52%, in 2021 – by 50.08%, due to a decrease in the cost of INN Sildenafil (Sildenafilum) by an average of 89.21% annually, compared with the cost in 2018.&#x0D; At the same time, the state of providing MP for patients with PH in Kyiv for the period 2018–2021 indicates that the total need for them was met at the expense of the State Budget of Ukraine and the budget of Kyiv for the implementation of the City Target Program «Health of Kievans» in 2018 – by 81.46%, and in 2019 – by 91.67% of the need.&#x0D; The supply of MP ordered for 2020 for the treatment of PH at the expense of the State Budget took place only at the end of 2021. Only Iloprost (Iloprostum) of the six supplies of INN ordered was completed, which accounted for 82.27% of the declared need.&#x0D; In 2021, only Sildenafil (Sildenafilum) was purchased at the expense of the State Budget of Ukraine, which is 8% of the need to provide treatment for patients with PH in the city of Kyiv. The City Target Program «Health of Kievans» for 2021 does not provide for expenses in the «Pulmonary Hypertension» direction.&#x0D; Thus, the results of a study of the organization of pharmaceutical support for Kyiv residents with PH indicate that there are no financial guarantees for the provision of free medical care to patients with rare (orphan) diseases.

Pulmonary Hypertension in Adults with Congenital Heart Disease.

Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) affects 5% to 10% of adults with CHD and is associated with significant morbidity and mortality. PAH-CHD develops as a consequence of intracardiac or extracardiac systemic-to-pulmonary shunts that lead to pulmonary vascular remodeling through a pathologic process that is similar to other causes of PAH. Eisenmenger syndrome is the most severe phenotype of PAH-CHD and is characterized by severe elevation in pulmonary vascular resistance, with shunt reversal causing hypoxemia and central cyanosis. The primary management strategy for most patients with PAH-CHD is medical therapy, although defect closure is considered in select cases.

Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a 5-year-old Girl with No Risk Factors - A Case Report

Chronic thromboembolic pulmonary hypertension (CTEPH) in pediatric population is rare. We present a case of five-year-old female child, diagnosed as CTEPH on warfarin and home oxygen for 6 months. Imaging showed extensive thrombus in distal right pulmonary artery (RPA) than left pulmonary artery (LPA). She clinically deterioration inspite of maximal medical management and was referred for surgical option. Considering lack of literature evidence on surgical outcomes in pediatric CTEPH, decision to perform surgery was made after multi-disciplinary team discussion between adult and paediatric pulmonary hypertension and cardiothoracic surgical teams across two units in UK. We performed pulmonary thromboendarterectomy (PEA) using cardiopulmonary bypass under deep hypothermic circulatory arrest (DHCA). The surgical procedure was uneventful with good clinical recovery. Our report highlights the need for earlier and prompt diagnosis with a need for referral to a specialized surgical center at an earlier stage before intractable vascular resistance ensues. Surgery can be safely performed for CTEPH in pediatric populations.

PH Roundtable: Transitions in Pulmonary Hypertension

PH Roundtable: Transitions in Pulmonary Hypertension

Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry.

Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD. A total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3±16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369±121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of follow-up, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of "Non-Eisenmenger PAH" patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described. Analyzing "real life data" from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the "Non-Eisenmenger PAH" group and to patients with complex CHD, including Fontan patients. www.clinicaltrials.gov, study identifier: Clinicaltrials.gov NCT01347216.

Patient-Specific Computational Analysis of Hemodynamics in Adult Pulmonary Hypertension.

Pulmonary hypertension (PH) is a progressive disease characterized by elevated pressure and vascular resistance in the pulmonary arteries. Nearly 250,000 hospitalizations occur annually in the US with PH as the primary or secondary condition. A definitive diagnosis of PH requires right heart catheterization (RHC) in addition to a chest computed tomography, a walking test, and others. While RHC is the gold standard for diagnosing PH, it is invasive and posseses inherent risks and contraindications. In this work, we characterized the patient-specific pulmonary hemodynamics in silico for diverse PH WHO groups. We grouped patients on the basis of mean pulmonary arterial pressure (mPAP) into three disease severity groups: at-risk ([Formula: see text], denoted with A), mild ([Formula: see text], denoted with M), and severe ([Formula: see text], denoted with S). The pulsatile flow hemodynamics was simulated by evaluating the three-dimensional Navier-Stokes system of equations using a flow solver developed by customizing OpenFOAM libraries (v5.0, The OpenFOAM Foundation). Quasi patient-specific boundary conditions were implemented using a Womersley inlet velocity profile and transient resistance outflow conditions. Hemodynamic indices such as spatially averaged wall shear stress ([Formula: see text]), wall shear stress gradient ([Formula: see text]), time-averaged wall shear stress ([Formula: see text]), oscillatory shear index ([Formula: see text]), and relative residence time ([Formula: see text]), were evaluated along with the clinical metrics pulmonary vascular resistance ([Formula: see text]), stroke volume ([Formula: see text]) and compliance ([Formula: see text]), to assess possible spatiotemporal correlations. We observed statistically significant decreases in [Formula: see text], [Formula: see text], and [Formula: see text], and increases in [Formula: see text] and [Formula: see text] with disease severity. [Formula: see text] was moderately correlated with [Formula: see text] and [Formula: see text] at the mid-notch stage of the cardiac cycle when these indices were computed using the global pulmonary arterial geometry. These results are promising in the context of a long-term goal of identifying computational biomarkers that can serve as surrogates for invasive diagnostic protocols of PH.

Pulmonary hypertension in adults with congenital heart disease. Clinical phenotypes and outcomes in the advanced pulmonary vasodilator era

BackgroundMortality of pulmonary hypertension associated with congenital heart disease (PAH-CHD) in adults remains high. ObjectivesTo identify predictors of death and to assess the impact of treatment on outcome. MethodsRetrospective, multicenter cohort study of 103 adults with PAH-CHD followed-up for 8.6 ± 4.6 years. Patients were grouped according to underlying shunt type into pre-tricuspid, post-tricuspid and complex. Survival rates were analyzed and predictors of death were investigated with Cox regression models. ResultsIn the post-tricuspid and complex groups (38 and 37 patients, respectively), the most common clinical PAH-CHD subgroup was Eisenmenger syndrome (76.3% and 59.5%, respectively) whereas, in the pre-tricuspid group (28 patients), 46.5% of patients had small or corrected defects. Overall, 88 patients received vasodilators; 39% required combination-therapy. Overall survival at 10 years was 65%. Mortality was highest in the pre-tricuspid group, FC-III-IV and amongst patients receiving monotherapy (p < 0.050). On multivariate analysis, predictors of poor outcome were pericardial effusion (HR: 4,520 [1,470–13,890]; p = 0,008), oxygen saturation(HR: 0.940 [0,900 - 0,990]; p = 0,018) and genetic syndromes(HR: 3,280 [1,098–9,780]; p = 0,033). ConclusionsPatients in advanced stages at initiation of treatment were at high risk of death and strong consideration should be given for more aggressive therapy.