Hypertension Referral Center Research Articles

Risk assessment and real-world outcomes in chronic thromboembolic pulmonary hypertension: insights from a UK pulmonary hypertension referral service

ObjectivesThis study was conducted to evaluate the ability of risk assessment to predict healthcare resource utilisation (HCRU), costs, treatments, health-related quality of life (HRQoL) and survival in patients diagnosed with...

Validation of Artificial Intelligence Cardiac MRI Measurements: Relationship to Heart Catheterization and Mortality Prediction.

Background Cardiac MRI measurements have diagnostic and prognostic value in the evaluation of cardiopulmonary disease. Artificial intelligence approaches to automate cardiac MRI segmentation are emerging but require clinical testing. Purpose To develop and evaluate a deep learning tool for quantitative evaluation of cardiac MRI functional studies and assess its use for prognosis in patients suspected of having pulmonary hypertension. Materials and Methods A retrospective multicenter and multivendor data set was used to develop a deep learning-based cardiac MRI contouring model using a cohort of patients suspected of having cardiopulmonary disease from multiple pathologic causes. Correlation with same-day right heart catheterization (RHC) and scan-rescan repeatability was assessed in prospectively recruited participants. Prognostic impact was assessed using Cox proportional hazard regression analysis of 3487 patients from the ASPIRE (Assessing the Severity of Pulmonary Hypertension In a Pulmonary Hypertension Referral Centre) registry, including a subset of 920 patients with pulmonary arterial hypertension. The generalizability of the automatic assessment was evaluated in 40 multivendor studies from 32 centers. Results The training data set included 539 patients (mean age, 54 years ± 20 [SD]; 315 women). Automatic cardiac MRI measurements were better correlated with RHC parameters than were manual measurements, including left ventricular stroke volume (r = 0.72 vs 0.68; P = .03). Interstudy repeatability of cardiac MRI measurements was high for all automatic measurements (intraclass correlation coefficient range, 0.79-0.99) and similarly repeatable to manual measurements (all paired t test P > .05). Automated right ventricle and left ventricle cardiac MRI measurements were associated with mortality in patients suspected of having pulmonary hypertension. Conclusion An automatic cardiac MRI measurement approach was developed and tested in a large cohort of patients, including a broad spectrum of right ventricular and left ventricular conditions, with internal and external testing. Fully automatic cardiac MRI assessment correlated strongly with invasive hemodynamics, had prognostic value, were highly repeatable, and showed excellent generalizability. Clinical trial registration no. NCT03841344 Published under a CC BY 4.0 license. Online supplemental material is available for this article. See also the editorial by Ambale-Venkatesh and Lima in this issue. An earlier incorrect version appeared online. This article was corrected on June 27, 2022.

Impact of Pulmonary Arterial Hypertension on Employment, Work Productivity, and Quality of Life - Results of a Cross-Sectional Multi-Center Study

IntroductionData on burden of pulmonary arterial hypertension (PAH) are mostly limited to physical and clinical endpoints as well as quality of life. Research on employment, work productivity, and educational background is scarce. The aim of this study was to assess the impact of PAH on employment status and work productivity in Germany.Materials and MethodsIn a multicenter cross-sectional survey, patients with PAH were surveyed in two large pulmonary hypertension referral centers in Germany. The survey contained questions on education, employment, work productivity and impairment (WPAI, also at the time of diagnosis), quality of life, and socioeconomic status. Additional data was assessed using clinical research database for 6-min walk distance (6MWD), WHO functional class, and N-terminal fragment of pro-brain natriuretic peptide. All patients provided written informed consent, and the institutional review board approved this study.ResultsIn total, 212 patients were surveyed (72% female; median, 57 years) approximately 6 years after initial PAH diagnosis. A total of 76% had an idiopathic PAH followed by hereditary and associated PAH (10% each). Employment at the time of diagnosis was 48% (34% full-time, 14% part-time), with productivity measured by a WPAI score of 6 points and decreased to 29% (21% full-time, 8% part-time) at the time of the survey (with a WPAI score of 2 points, p < 0.001). Logistic regression showed education and 6MWD as predictors for employment. Patients in moderate or high educational category had a 3.6- or 5.6-fold chance, respectively, of being employed (p = 0.025 and p = 0.019), and per 50-m increase of 6MWD, the odds of being employed were 1.2, p = 0.042.ConclusionPatients with PAH had a reduced employment rate, which was influenced by education and 6MWD. There was a considerable loss of employment over the course of the disease. Employment should be one possible treatment goal in patients with PAH to provide social participation to this patient group.

400 Refractory pulmonary hypertension in a young woman

Abstract Aims Clinical case—Twenty-four years old Moroccan woman. Family history: parents and three siblings in good health. Methods and results Past medical history—In 2016, when she was 19 years old, she developed worsening exercise-induced dyspnoea. A right heart catheterization (RHC) was performed with evidence of increased median pulmonary artery pressure (mPAP 60 mmHg, wedge pressure 8 mmHg), cardiac index 2.27 l/min/m2. She was diagnosed with idiopathic pulmonary hypertension and combination therapy with sildenafil and macitentan was started with partial improvement. In August 2019, she became pregnant and vasodilatory therapy was suspended. The pregnancy was complicated by premature labor with foetal death. Specific therapy with sildenafil and macitentan was then restarted. Due to further clinical and haemodynamic impairment, triple combination therapy with selexipag was initiated. However, symptomatic deterioration progressed, and she was referred to a Pulmonary Hypertension Referral Center where HRTC of the chest showed centrilobular ground-glass opacities and interlobular septal thickening. Based on the imaging that was highly suspicious pulmonary veno-occlusive disease (PVOD), and the severe haemodynamic impairment (with pulmonary vascular resistance at RHC &amp;gt; 20 WU), assessment for lung transplantation was started. Recent medical history—she was transferred to our Center with Transplant Unit for lung transplant assessment. Pulmonary function testing demonstrated a restrictive disorder with severe reduction of DLCO (14%). At 6-min walking test, she could walk 100 m with desaturation up to 90% on O2 therapy. Evaluated by our multidisciplinary team, indications for lung transplantation were confirmed and she entered the transplant waiting list. At the end of February 2021: further clinical and haemodynamic deterioration with respiratory distress, reduction in urinary output and signs and symptoms of right-side heart failure; she was hospitalized in intensive care unit and required extra-corporeal membrane oxygenation (ECMO) circulatory support. Selexipag was suspended. On echocardiography: severe right ventricle hypertrophy and dilatation, tricuspid regurgitation velocity &amp;gt;4 m/s. Few days later, she underwent bilateral lung transplantation; anatomo-pathological evaluation of explanted organs confirmed PVOD. However, during post-operative monitoring, she suffered from two episodes of cardiac arrest on VT/VF which required multiple DC-shocks. Since no triggering causes were identified, an ICD was implanted for secondary prevention. Conclusions PVOD is a rare disease with clinical presentation and haemodynamic profile often similar to pulmonary arterial hypertension (and, therefore, the diagnosis is challenging) but the clinical course is more severe. The diagnosis requires clinical history and physical examination, together with multimodality imaging and functional testing. Bronchoalveolar lavage might be necessary. Patients do not usually respond satisfactorily to vasodilatory therapy but rather they are at high risk of drug-induced pulmonary oedema. Some case reports and case series have reported a slight benefit and/or clinical stabilization with vasodilatory therapy in selected patients; therefore, specific therapy can be used but cautiously and in experienced referral centres. Definitive therapy is lung transplantation (or heart-lung transplantation) and a multidisciplinary team is necessary for the appropriate management of these complicated patients. Ventricular arrhythmias are rare in patients with Group 1 pulmonary hypertension. In our specific clinical case, we suspect that ventricular arrhythmias might be related to the severely hypertrophic, and potentially fibrotic, right ventricle.

Abstract 11614: Investigating Outcomes in Patients with Pulmonary Hypertension Diagnosed with Covid-19 in a Tertiary Pulmonary Hypertension Referral Center

Introduction: Preexisting endothelial dysfunction and effects of selective pulmonary vasodilator therapy are postulated to prevent vascular remodeling and decrease morbidity and mortality in pulmonary arterial hypertension (PAH) patients with COVID-19. However, research on outcomes in PAH patients with COVID-19 is limited. We studied morbidity and mortality from COVID-19 in a tertiary PAH referral center. Methods: Of 596 clinic patients on at least one PAH specific medication, 27 patients were diagnosed with COVID-19 between March 2020 and January 2021. Retrospectively evaluated outcomes included mortality, hospitalization, intensive care unit admission (ICU), mechanical ventilation (MV), extracorporeal membrane oxygenation (ECMO), lung transplant, and hospital readmission within 30 days. Results: Of 27 PAH patients with COVID-19 (mean age=60 years; 74% female; 96% non-Hispanic; 74% White), 15 (56%) were hospitalized, 7 (26%) died, and 1 (4%) underwent ECMO followed by lung transplant. Hospitalized patients, compared to non-hospitalized patients, had higher mean age (64 vs 56 years), number of patients on IV prostacyclin (20% vs 0%), mean REVEAL 2.0 score (8.4 vs 6.5), and moderate-severe RV dysfunction (6, 40% vs 3, 25%). There was no difference in NYHA FC and mean pulmonary arterial pressure (45 mm Hg vs 43.7 mm Hg) between hospitalized and non-hospitalized patients. Complications during hospitalization included worsened hypoxemia (13, 87%), cardiac ischemia (4, 15%), new or worsening arrhythmias (3, 11%), and acute kidney injury (AKI) (6, 22%). Four patients (27%) required ICU admission and MV. Of these, 2 (50%) had new or worsening arrhythmias and all 4 (100%) had AKI. Of 15 patients hospitalized, 5 (19%) were readmitted to the hospital within 30 days. The 7 patients who died during hospitalization, compared to 8 who survived, had increased rates of ICU admission and MV (43% vs 12.5%) and higher REVEAL 2.0 scores (10.1 vs 6.9). Conclusion: COVID-19 in hospitalized PAH patients was associated with high rates of multi-organ complications, mortality, ICU admission, and hospital re-admission. Advanced age, IV prostacyclin therapy, higher REVEAL score, moderate to severe RV dysfunction, and AKI were associated with adverse outcomes.

Impact of routine screening on detection, severity and outcome of pulmonary arterial hypertension in systemic sclerosis

Abstract Introduction It has been reported that up to 20% of systemic sclerosis (SSc) patients can be asymptomatic at the time of pulmonary arterial hypertension (PAH) diagnosis. The significant prevalence rate, lack of symptoms and high morbidity and mortality from SSc-PAH as well as the potential benefit from early intervention with the more widely available therapeutic options provide a strong rationale for active screening programs. The DETECT algorithm was developed in 2013 from a large prospective and multicentre study in SSc patients with higher risk of PAH. The objective of this study was to examine the impact of a screening program on the early detection of SSc-PAH. We looked at serial patients diagnosed with SSc associated PAH (SSC-PAH) in a large national pulmonary hypertension referral centre. Patients and methods All newly diagnosed adult patients with SSc-PAH prospectively enrolled in a large national pulmonary hypertension referral centre. The current study included newly diagnosed patients between January 2006 and January 2018. Results Three-hundred and five patients were diagnosed with SSc-PAH in our centre between 2006 and 2018. Of these, 164 patients were diagnosed before 2013 (January 2006 - December 2012) and 141 after 2013 (January 2013 - January 2018). Demographics were similar at presentation between the two groups. The non-invasive (WHO-FC, 6-MWD and NT-proBNP) and haemodynamic measurements (RAP, CI and SvO2) were used to calculate the ESC guidelines risk score. It was noted that higher proportion of patients in the post-2013 were in the higher risk categories than the pre 213 group (84.4% vs. 78%) but this was not found to be statistically significant (p value 0.356). There was no statistically significant difference in survival between the two groups (Post 2013 group, 1-, 3- year and 5-year survival was 87.9%, 60.4% and 52.1%, respectively and pre-2013 group 1-, 3- year and 5-year survival 89.6%, 65.2% and 49%, respectively) with a log rank p value of 0.869. Applying Cox regression analysis of proportional hazard and adjusting for ESC risk score at baseline, predicted survival was not found to be statistically different between the two groups. The ESC risk category at baseline was a highly significant predictor of survival as expected. Conclusion There remains a strong rationale for active screening for PAH in SSc patients which has a poor prognosis despite advances in therapeutic strategies. However, the current screening programme does not seem to have resulted in significantly earlier detection in this cohort. It would be important to analyse other patient populations in order to understand the impact of screening programmes. The current screening programs limitations may explain why we have not been able to detect more patients in the lower risk categories. Further development of these programs in order to overcome their shortfalls is direly needed. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): National Health ServiceRoyal Free NHS Trust Survival analysis

Прогноз и его предикторы при легочной гипертензии, ассоциированной с заболеваниями соединительной ткани (данные первого украинского реестра)

Целью исследования было: провести анализ структуры пациентов, которые обследовались в специализированном центре; оценить украинскую реальность относительно выживаемости больных легочной артериальной гипертензией (ЛАГ) и хронической тромбоэмболической легочной гипертензией (ХТЭЛГ), которые проходили лечение в референтном центре (ранее у нас не было украинских данных); определить предикторы смерти. Материалы и методы. Проанализированы данные 359 больных с легочной гипертензией (ЛГ): с идиопатической ЛАГ — 72 (20,1 %), с ЛАГ, ассоциированной с заболеваниями соединительной ткани, — 20 (5,6 %), с ЛАГ, ассоциированной с ВИЧ-инфекцией, — 8 (2,2 %), с ЛАГ, ассоциированной с портальной гипертензией, — 4 (1,1 %), с ЛАГ, ассоциированной с врожденными пороками сердца, — 171 (47,6 %), с ЛГ вследствие заболеваний левых отделов сердца — 8 (2,2 %), ЛГ вследствие заболеваний легких — 6 (1,7 %), с ХТЭЛГ — 62 (17,3 %), ЛГ смешанной этиологии — 8 (2,2 %). Для оценки выживания включен 281 пациент (52 (18,5 %) с ХТЭЛГ и 229 (81,5 %) с ЛАГ), которые прошли лечение в центре легочной гипертензии ГУ «ННЦ «Институт кардиологии имени академика Н.Д. Стражеско» НАМН Украины». Диагноз легочной гипертензии устанавливался на основании данных катетеризации правых отделов сердца. Выживание определяли методом построения кривых Kaplan — Meier. Срок наблюдения составлял 51 месяц. Предикторы определяли с помощью бинарной логистической регрессии и Сох-регрессионного анализа. Результаты. Выживание общей когорты пациентов составляло 93,3; 86,8 и 81,5 % на этапах один, два и три года соответственно. Лучшее выживание было у пациентов с ЛАГ, ассоциированной с врожденными пороками сердца (92,7 %), по сравнению с идиопатической ЛАГ (67,5 %) (long rank P = 0,002), ЛАГ, ассоциированной с заболеваниями соединительной ткани (49,7 %) (long rank P = 0,001), и ХТЭЛГ (83,2 %) (long rank P = 0,04). По данным унивариантного Cox-анализа, предикторами смерти были: IV функциональный клас по ВОЗ (OR = 4,94; 95% ДИ 2,12–11,48), наличие асцита (OR = 4,52; 95% ДИ 2,21–9,24), ЛАГ, ассоциированная с заболеваниями соединительной ткани (OR = 3,07; 95% ДИ 1,07–8,87), ЛАГ, ассоциированная с врожденным пороком сердца (OR = 0,28; 95% ДИ 0,11–0,68), ЧСС на фоне лечения &gt; 105 уд/мин (OR = 7,85; 95% ДИ 1,83–33,69), офисное систолическое АД &lt; 100 мм рт.ст. (OR = 2,78; 95% ДИ 1,26–6,1), дистанция 6-минутного теста на фоне лечения &lt; 340 м (OR = 3,47; 95% ДИ 1,01–12,35), уровень NT-proBNP &gt; 300 пг/мл (OR = 4,98; 95% ДИ 1,49–16,6), площадь правого предсердия &gt; 22 см2 (OR = 14,2; 95% ДИ 1,92–104,89), площадь правого желудочка в диастолу (OR = 1,08; 95% ДИ 1,03–1,14), площадь правого желудочка в систолу (OR = 1,08; 95% ДИ 1,02–1,11), среднее давление в правом предсердии на 1 мм рт.ст. повышения (OR = 1,02; 95% ДИ 1,02–1,19). При многовариантном Cox-регрессионном анализе независимыми предикторами смерти были наличие асцита, офисное систолическое АД &lt; 100 мм рт.ст. и уровень NT-proBNP &gt; 300 пг/мл. ЛАГ, ассоциированная с врожденными пороками сердца, уменьшала вероятность смерти. Выводы. Это первый результат исследования, которое было проведено в единственном украинском центре по диагностике и лечению легочной гипертензии. Показано, что структура больных с ЛАГ в Украине существенно отличается от таковой в центрах других стран, но показатели выживания сопоставимы. Наихудшее выживание отмечалось при ЛАГ, ассоциированной с заболеваниями соединительной ткани, в сравнении со всеми другими ее формами: выживание составляло лишь 49,7 % в течение 3 лет. Установлены достаточно простые показатели, которые можно оценить в рутинной практике и которые являются независимыми предикторами смерти.

Short-Term Impact of Iron Deficiency in Different Subsets of Patients with Precapillary Pulmonary Hypertension from an Eastern European Pulmonary Hypertension Referral Center.

BackgroundOver the last few decades, interest in the role of iron status in pulmonary hypertension (PH) has grown considerably due to its potential impact on symptoms, exercise capacity (as assessed by the 6-minute walk distance [6MWD]), prognosis, and mortality. The aim of the present study was to identify iron deficiency (ID) prevalence in specific precapillary PH subgroups of Romanian patients and its short-term impact on 6MWD.Patients and MethodsComplete datasets from 25 precapillary PH adults were examined and included in the analysis. Data were collected at baseline and after continuous follow-up of an average of 13.5 months. Enrolled patients were assigned to group 1 (pulmonary arterial hypertension) or subgroup 4.1 (chronic thromboembolic pulmonary hypertension), and individualized targeted therapy was prescribed. General characteristics, World Health Organization functional class, 6MWD, pulse oximetry, laboratory parameters, and echocardiographic and hemodynamic parameters were recorded. Ferritin values and transferrin saturation were used to assess ID.ResultsAt baseline, 16 out of 25 patients were iron deficient. The univariate linear regression analysis did not show a statistically significant impact of ID on 6MWD (p=0.428). In multivariate regression analysis, possible predictors of 6MWD, including ID, were not statistically significant at baseline or after an average of 13.5 months follow-up (p=0.438, 0.361, respectively) and ID indicates a negative impact on 6MWD independent of applied corrections.ConclusionThe results of this study demonstrate that 1.4.1 subgroup PAH patients have an increased prevalence of ID compared with other etiologies. ID has a negative impact on the functional status (assessed by 6MWD), in specific groups and subgroups of patients with precapillary PH, albeit not independently nor significant to other known predictors such as age, gender, oxygen saturation, and hemoglobin value. These data can be integrated with global research and are consistent with phenotypes of patients diagnosed with PH of different etiologies.

Outpatient specialist clinics for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension in the Nordic countries.

Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are rare conditions that require complex interventions by multidisciplinary teams. The European Society of Cardiology (ESC)/the European Respiratory Society (ERS) 2015 guidelines included recommendations for pulmonary hypertension (PH) referral centers including minimum number of patients, staff, facilities, and network. The aim of the present study was to investigate how the PH-specialist centers in the Nordic countries are presently organized. A descriptive, questionnaire was sent to all PH-specialist centers in the Nordic countries in 2018. Sixteen of 20 PH-specialist centers completed the questionnaire. Seven centers (43%) followed less than 50 patients and three centers (19%) followed 125 patients or more. All had a physician or nurse attending or available at the clinic and eight had support staff such as physiotherapists, counsellors, dieticians, or psychologists directly connected to the center. Twelve centers were available by telephone five days or more per week. Nine centers offered a nurse-led outpatient clinic and of those, six had nurses delegated to make protocol-led changes in pulmonary arterial hypertension-specific treatment. Half of the centers had cooperation with a patient organization. All centers except one used international guidelines to guide care and treatment. More than half of the Nordic PH-specialist centers adhered to the ESC/ERS 2015 guidelines recommendations for volumes and staff in 2018, but there is potential for improvement. However, when formulating recommendations of patient volumes in guidelines, the situation for the geographical large but sparsely populated Nordic countries needs to be considered.

Prognostic value of six-minute walk distance at a South American pulmonary hypertension referral center.

Six-minute walk distance (6MWD) assessment is recommended for pulmonary arterial hypertension multidimensional risk stratification. However, current 6MWD cut-off values were mainly derived from North American and European pulmonary arterial hypertension registries. Therefore, it is unknown if such cut-off values broadly apply to other geographical populations. In this study, we aimed to identify 6MWD cut-off values for Brazilian pulmonary arterial hypertension patients and to contrast our findings to current international Pulmonary Hypertension guidelines recommendations. One-hundred four consecutive pulmonary arterial hypertension patients were allocated in groups according to their 6MWD, considering 50 m as a clinically relevant 6MWD difference. Next, patients were categorized into different 6MWD ranges based on similar survival rates in each group: < 250 m, 250–400 m, and >400 m. The study outcome was all-cause mortality and transplantation according to the 6MWD range. Survival was truncated at five years. Median follow-up period was 4.35 years (0.48–5.00). Survival rates at 1, 2, 3, and 5 years were 96%, 89%, 81%, and 73%, respectively. Cox analyses adjusted for age, sex, and pulmonary arterial hypertension etiology showed that 6MWD < 250 m and >400 m were associated with higher and lower risk of all-cause mortality and transplantation. According to Harrell's c-statistic, the prognostic discrimination of the 6MWD cut-off value identified by the current study was 0.70 while international Pulmonary Hypertension guidelines 6MWD cut-offs value was 0.61. In conclusion, our findings suggest that 6MWD geographical variations should be considered when assessing risk stratification in pulmonary arterial hypertension.

Percepção de pacientes com diabetes sobre o autocuidado

Objetivo: Analisar a percepção de pacientes com diabetes mellitus sobre o autocuidado. Métodos: Estudo qualitativo, realizado em um centro de referência em diabetes e hipertensão em Fortaleza, Ceará, Brasil, no período de fevereiro a junho de 2016. A amostra foi composta por 12 pacientes com diagnóstico de diabetes mellitus do tipo 2, que frequentavam os encontros do Programa de Responsabilidade Social. Para coleta de dados, realizou-se grupo focal, seguido da análise temática, emergindo quatro categorias: aquisição de hábitos de vida saudáveis; alimentação e atividade física aliadas ao controle do diabetes; uso contínuo de medicamentos na prevenção de agravos; dia a dia nos cuidados com a prevenção do pé diabético. Resultados: Pode-se compreender, por meio dos relatos, que há um desenvolvimento da conscientização embasada na prática de educação e saúde apresentada pelos participantes vinculados ao programa. Os pacientes expressavam para o grupo a importância da sua conscientização em relação aos cuidados essenciais para o controle da diabetes, destacando boas práticas de saúde, tais como: uma alimentação balanceada, a prática regular de exercícios físicos, o não abandono do tratamento medicamentoso e a higienização e o cuidado com os pés. Conclusão: Constatou-se que o Programa de Responsabilidade Social proporcionou mudanças nos hábitos de vida, considerando a prática de educação em saúde fundamental na percepção dos pacientes diabéticos.

Abstract P228: Text-Facilitated Home Blood Pressure Monitoring: A Qualitative Analysis of Health Behavior Change

Uncontrolled hypertension constitutes a major challenge for healthcare systems. Home blood pressure monitoring (HBPM) is widely recommended and may lower BP when combined with other supports. However, scalable and systematic HBPM interventions are lacking and the behavioral mechanism(s) through which BP is lowered remain poorly understood. Our team designed the MyBP program with video-based education and a fully automated, bi-directional texting to facilitate longitudinal HBPM. Exit interviews conducted after six-weeks of MyBP revealed that most participants made at least one healthy behavior change. The current study examines why participants made healthy behavior changes, and what specific components of the MyBP program facilitated those changes. Adults with hypertension were recruited from either an urban emergency department, a primary care office, or a hypertension referral center. The 40 enrolled participants were widely representative: age range 34-70, 23 women, 24 minority, 14 completed only high school, BP range 110-250/70-130 mm Hg, and prescribed BP medications range 0-5. A thematic analysis of transcribed exit interview audio-recordings identified three themes contributing to patients’ decision to initiate a behavior change: 1) improved hypertension literacy from viewing educational videos; 2) increased day-to-day salience of one’s BP as a result of consistent HBPM; and 3) use of BP readings as feedback on participants’ health behaviors, with high readings often triggering intrinsic motivations to make behavior changes. These themes and associated sub-themes were found to have analogous constructs in the Health Belief Model and Social Cognitive Theory. The presentation of educational materials at baseline, followed by regular BP self-monitoring, increased confidence and motivation to initiate changes in health behaviors. The receipt of bi-weekly reports then acted as feedback fueling participants’ motivation to maintain or add healthy behaviors. Facilitation of HBPM with automated texting, in conjunction with educational videos and regular feedback, appears to stimulate improvements in hypertension self-management via mechanisms consistent with recognized models of behavior change.

Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal

ObjectivesThis study aims to assess the long-term survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients followed in a Portuguese pulmonary hypertension (PH) referral center. MethodsWe studied PAH and CTEPH patients diagnosed between January 2005 and December 2016. Cumulative survival was estimated using the Kaplan-Meier method. Survival trends were compared over two periods (2005-2010 vs. 2011-2016). ResultsOf the 142 studied PH patients (age 54±18 years; 31% male), 47 had CTEPH and 95 had group 1 PH. Most patients with CTEPH and idiopathic/heritable PAH (I/HPAH) were in NYHA III-IV at diagnosis (64% and 57%, respectively). At the time of death, 31% of patients with connective tissue disease (CTD)-associated PAH (CTD-PAH) and all I/HPAH patients were on double or triple combination therapy. No patient underwent lung transplantation. Pulmonary endarterectomy or angioplasty were performed in 36% of CTEPH patients. Age at diagnosis tended to increase over time in CTD-PAH (53±15 vs. 63±15 years; p=0.13) and I/HPAH (39±15 vs. 51±19 years; p=0.10). The five-year survival estimates for I/HPAH, CTD-PAH and CTEPH patients were 80%, 52%, and 81%, respectively. Over time, CTD-PAH and CTEPH showed better five-year survival (33 vs. 67% and 77 vs. 84%), but I/HPAH did not (84 vs. 75%). ConclusionsOur data indicate a trend toward improved survival over time of CTD-PAH and CTEPH patients treated at a Portuguese referral PH center. Earlier diagnosis, increasing use of parenteral prostanoids, and surgical treatment may further improve PH prognosis.

Fluid Challenge During Right Heart Catheterization: An Experience From a Tertiary Pulmonary Hypertension Referral Centre

Differentiating pre-capillary or pulmonary arterial hypertension (PAH) from pulmonary hypertension (PH) related to left-sided heart disease (PH-LHD) can be challenging. Pulmonary arterial wedge pressure (PAWP) measured at right heart catheterization is required for classifying PH. However, a normal PAWP is a poor predictor for absence of left heart disease. Therefore, there has been an interest in using fluid challenge to unmask PH-LHD. The purpose of our study is to evaluate the impact of fluid challenge in the classification and management of a real-world population.

Diagnostic and prognostic significance of cardiovascular magnetic resonance native myocardial T1 mapping in patients with pulmonary hypertension

BackgroundNative T1 may be a sensitive, contrast-free, non-invasive cardiovascular magnetic resonance (CMR) marker of myocardial tissue changes in patients with pulmonary artery hypertension. However, the diagnostic and prognostic value of native T1 mapping in this patient group has not been fully explored. The aim of this work was to determine whether elevation of native T1 in myocardial tissue in pulmonary hypertension: (a) varies according to pulmonary hypertension subtype; (b) has prognostic value and (c) is associated with ventricular function and interaction.MethodsData were retrospectively collected from a total of 490 consecutive patients during their clinical 1.5 T CMR assessment at a pulmonary hypertension referral centre in 2015. Three hundred sixty-nine patients had pulmonary hypertension [58 ± 15 years; 66% female], an additional 39 had pulmonary hypertension due to left heart disease [68 ± 13 years; 60% female], 82 patients did not have pulmonary hypertension [55 ± 18; 68% female]. Twenty five healthy subjects were also recruited [58 ±4 years); 51% female]. T1 mapping was performed with a MOdified Look-Locker Inversion Recovery (MOLLI) sequence. T1 prognostic value in patients with pulmonary arterial hypertension was assessed using multivariate Cox proportional hazards regression analysis.ResultsPatients with pulmonary artery hypertension had elevated T1 in the right ventricular (RV) insertion point (pulmonary hypertension patients: T1 = 1060 ± 90 ms; No pulmonary hypertension patients: T1 = 1020 ± 80 ms p < 0.001; healthy subjects T1 = 940 ± 50 ms p < 0.001) with no significant difference between the major pulmonary hypertension subtypes. The RV insertion point was the most successful T1 region for discriminating patients with pulmonary hypertension from healthy subjects (area under the curve = 0.863) however it could not accurately discriminate between patients with and without pulmonary hypertension (area under the curve = 0.654). T1 metrics did not contribute to prediction of overall mortality (septal: p = 0.552; RV insertion point: p = 0.688; left ventricular free wall: p = 0.258). Systolic interventricular septal angle was a significant predictor of T1 in patients with pulmonary hypertension (p < 0.001).ConclusionsElevated myocardial native T1 was found to a similar extent in pulmonary hypertension patient subgroups and is independently associated with increased interventricular septal angle. Native T1 mapping may not be of additive value in the diagnostic or prognostic evaluation of patients with pulmonary artery hypertension.

Heritable pulmonary hypertension: from bench to bedside.

Mutations in the BMPR2 gene, and more rarely in ACVRL1, endoglin, caveolin-1, KCNK3 and TBX4 genes predispose to heritable pulmonary arterial hypertension, an autosomal dominant disease with incomplete penetrance. Bi-allelic mutations in the EIF2AK4 gene predispose to heritable pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis, an autosomal recessive disease with an unknown penetrance.In France, the national pulmonary hypertension referral centre offers genetic counselling and testing to adults and children. Predictive testing is also proposed to adult relatives at risk of carrying a predisposing mutation. In that context, we offer all asymptomatic BMPR2 mutation carriers a programme to detect pulmonary arterial hypertension at an early phase, as recommended by the 2015 European Society Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines. Finally, pre-implantation genetic diagnosis has been conducted on five embryos from two couples in which the fathers were carriers of a pathogenic BMPR2 mutation.

Pulmonary arterial hypertension associated with congenital heart disease: Comparison of clinical and anatomic–pathophysiologic classification

Between 5% and 10% of patients with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH-CHD). Patients can be classified using either a clinical or anatomic-pathophysiologic system. No study has previously utilized both systems in a large cohort of patients. Two hundred forty consecutive PAH-CHD patients diagnosed at a pulmonary hypertension referral center during 1995 to 2014 were identified from our unit database. Baseline characteristics, treatment and survival data were retrieved and survival analyses was performed. Both systems identified clear differences in baseline characteristics between subgroups. The anatomic-pathophysiologic system identified patients with post-tricuspid defects as having superior survival from point of referral to those with pre-tricuspid or complex defects (p < 0.05). Survival from point of referral was, however, not significantly different when patients were grouped using the clinical classification, although survival in all 4 groups was superior to that of 175 patients with idiopathic pulmonary arterial hypertension. Older age and higher creatinine, lower transfer factor of the lung for DLCO percent predicted and FEV1 percent predicted were independent adverse prognostic factors. Both PAH-CHD classification systems identified groups of patients distinct in terms of baseline characteristics. In our cohort, however, only the anatomic-pathophysiologic classification identified significantly different survival from point of referral. The presence of adverse prognostic markers may be useful in identifying patients requiring more aggressive pulmonary vascular therapy.

0386: Prevalence, characteristics and prognosis of moderate to severe tricuspid regurgitation in patients with precapillary pulmonary hypertension

Our study aim to characterize clinical, biological, echocardio-graphic and hemodynamic parameters related with severity of TR and if TR is associated with higher risk of acute right ventricular heart failure (ARVHF) or death in PPH patiens. We reviewed data from 116 stable patients referred to the French pulmonary hypertension referral center. All patients had right heart catheterization with mean pulmonary pressure (mPAP) >25mmHg, with pulmonary wedge pressure <15mmHg and pulmonary vascular resistance (PVR) >3 WU. On echo-cardiography, severity of TR was determined from 2-dimensional and Doppler color flow image in the apical 4-chamber view. Patients were divided in two groups according to the severity of TR (TR <grade 3 and TR ≥grade 3). 37 (32%) patients have TR grade ≥3. Compared with patients TR <grade 3, no difference were observed in gender, NYHA class, and 6-minute walk distance. On RCH, both groups had similar mPAP, cardiac index, right atrial mean pressure and pulmonary vascular resistance. On echocardiography, Patients with TR grade ≥3 have higher right atrial volume (57 (45-70) vs 36 (32-40)mL/m 2 , p<0.001), higher end-diastolic right ventricular area (16 (14-17) vs 14 (13-15)cm 2 /m 2 , p=0.04), higher end-systolic right ventricular area (12 (10-14) vs 10 (9-11)cm 2 /m 2 , p=0.01), and higher BNP level (248 (141-355) vs 102 (59-144)pg/mL, p=0.003). There were no difference in tricuspid annular plane systolic excursion nor in tricuspid S’ velocity. During a median follow up of 17 months, death or ARVHF was observed in 14% of patients with TR grade ≥3 and 7% of patients with TR grade <3 with no statistical difference. PPH patients with TR grade ≥3 have higher right atrial volume, end-diastolic and end-systolic right ventricular area and BNP level compared with patients with TR <grade 3. There was more numerous major events in follow up in this group, without statistical difference.

The referral centers for the diagnosis and treatment of hypertension in adolescents

Primary hypertension in adolescence was felt to be quite rare. However, the worldwide childhood obesity epidemic has had a profound impact on the frequency of high blood pressure (BP) with the result that primary hypertension should now be viewed as one of the most common health conditions in the young (estimated prevalence 1–5%). Therefore, current guidelines recommend that all children and adolescents seen in a medical setting should have their BP measured. The availability of BP tables with normal BP percentiles for age, sex and height has improved BP values classification. Studies conducted at referral clinics for evaluation of hypertension have indicated that as many as 30 to 40% of adolescents may actually have in a clinical setting white-coat hypertension. This may lead to a misdiagnosis of “true” hypertension in a considerable number of cases. The usefulness of out-of-office BP evaluation using ambulatory or home monitoring is well established. These measurements allow the detection of the white-coat and masked hypertension, the opposite of white-coat hypertension, and are more closely associated with organ damage and cardiovascular risk than office measurements. A thorough familial and personal history is of primary importance as well as the physical examination that should be focused on the search for signs suggestive for an underlying cause and/or for the severity of hypertension. Following investigations must be tailored to the child's age, anamnesis and clinical examination and to the severity of BP elevation, in order to investigate not only the possible cause of hypertension, but also associated diseases and target organs damage. Therapeutic approach should firstly include non-pharmacological measures, and the use of medications when indicated. A key role in the management of the adolescents with hypertension may be attributed to the hypertension referral centers (table ​(table11). Table 1 What is required at the referral centers in the management of hypertension in adolescence

LGE Patterns in Pulmonary Hypertension Do Not Impact Overall Mortality

ObjectivesThe goal of this study was to determine the prognostic value of late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) features in patients with pulmonary hypertension. BackgroundThe prognostic significance of LGE in the clinical assessment of patients with pulmonary hypertension remains uncertain. MethodsConsecutive patients with suspected pulmonary hypertension seen at a specialist pulmonary hypertension referral center who underwent right heart catheterization and CMR with LGE imaging within 48 h were identified. Short-axis late-enhancement imaging was performed using a 3-dimensional gradient spoiled echocardiography sequence on a 1.5-T scanner. Three groups were identified: 1) no late enhancement of the myocardium; 2) late enhancement at the right ventricular insertion points (LGE-IP); and 3) late enhancement involving the right ventricular insertion points and the interventricular septum (LGE-S). ResultsOf 194 patients, 162 had pulmonary hypertension. LGE was identified in 135 of 162 (83%) patients with pulmonary hypertension, and 47 (29%) of patients demonstrated LGE-S. Patients with LGE-S had significantly higher right ventricular end-diastolic volume index (p = 0.013) and lower mixed venous oxygen saturation (p = 0.045) than patients with LGE-IP alone. The presence of LGE-S (p = 0.022), but not LGE-IP alone, right ventricular end-systolic volume (p = 0.045), right ventricular ejection fraction (p = 0.034), mixed venous oxygen saturation (p = 0.021), mean right atrial pressure (0.027), and male sex (p = 0.002) predicted mortality. At multivariate analysis, male sex was the only significant predictor of mortality independent of covariate predictors (p = 0.027). ConclusionsThe presence of LGE at the right ventricular insertion points is suggestive of the presence of pulmonary hypertension. LGE may also be more extensive, involving the septum; however, after multivariable analysis including other factors associated with pulmonary hypertension, septal LGE was not associated with an increase in overall mortality.