An unexpected cause of pulmonary hypertension in a young woman: a case report

Background Pulmonary hypertension (PH) is defined as a progressive disease that leads to right heart failure and death if untreated. This case report presents a young woman with reversible precapillary PH in the setting of a gastric cancer. Case summary A 37-year-old woman presented with exertional dyspnoea and syncope. The transthoracic echocardiographic findings were consistent with a cor pulmonale. Right heart catheterization (RHC) proved a precapillary PH. Specific PH therapy with macitentan and tadalafil was initiated. Shortly thereafter, a gastric carcinoma was diagnosed, and oncologic treatment with neoadjuvant chemotherapy and subsequent gastrectomy was promptly initiated. Retrospectively, we considered a pulmonary tumour thrombotic microangiopathy the most probable cause of PH. Follow-up after successful oncologic treatment and cumulative 10 months of specific PH medication showed an excellent clinical response with complete remission of PH confirmed by RHC at rest. Discussion Tumour-related PH is very rare and might be largely underdiagnosed as the clinical course often results in a rapid deterioration and fatal outcome before diagnostics are completed. Post mortem studies have documented tumoural emboli in pulmonary microcirculation in ∼26% of patients with a solid tumour, markedly associated with adenocarcinoma. Prompt initiation of cancer treatment on tumoural PH is essential. To our knowledge, this report documents the first full recovery of tumoural PH at rest after successful cancer treatment and temporary specific PH medication. We therefore conclude that a multidisciplinary approach with an initially combined oncologic and PH therapy may be most beneficial with the potential of complete remission of PH

Being (past and present) President of the ERS: interview about the role, perspectives on career development, and vision for the Society

This article presents the views of the past and current Presidents of the ERS regarding their role, perspectives on career development and vision for the Society, along with important messages to inspire ECMs to build their own successful career. https://bit.ly/3kAvxIM

Risk Factor Profiles Achieved with Medical Therapy in Prevalent Patients with Pulmonary Arterial and Distal Chronic Thromboembolic Pulmonary Hypertension

BACKGROUND The latest pulmonary hypertension (PH) guidelines define therapeutic goals in terms of symptoms, exercise capacity, and haemodynamics for patients with pulmonary arterial hypertension (PAH) and recommend advanced combined medical therapy. For inoperable or post-surgical residual distal chronic thromboembolic PH (CTEPH) medical treatment is similarly advised. OBJECTIVES We analysed whether risk factor goals are achieved and combination therapy is used in prevalent patients with PAH or distal CTEPH. METHODS PAH or distal CTEPH patients who were seen at the University Hospital Zurich during the last year were analysed in terms of demography, clinical data, medication, and therapeutic goals. Achievement of therapeutic goals was defined as New York Heart Association (NYHA) class ≤II, N-terminal pro-brain natriuretic peptide (NTpro-BNP) 440 m. RESULTS A total of 108 PAH patients (age 59 ± 18 years, 62% female, 64% idiopathic, 36% associated) and 38 distal CTEPH patients (age 69 ± 14 years, 55% female) were included. They had been diagnosed on average 66 ± 48 months (±SD) previously. The percentage of PAH/CTEPH patients with NYHA ≤II was 52/53, respectfully, the percentage of those with NTproBNP 440 m 63/50. Overall, 33/31% fulfilled 3 and 29/35% fulfilled 2 of these goals. Regarding therapy, 43% of PAH patients were on double and 10% on triple combination therapy, whereas 16% of distal CTEPH patients were on double and 3% on triple combination therapy. CONCLUSIONS In this real-life cohort of prevalent patients with PAH or distal CTEPH, targeted drug therapy resulted in an achievement of ≥2/3 predefined therapeutic goals in 2/3 of patients. Patients with PAH were more likely to receive combination therapy compared to CTEPH patients

Pulmonale Endarteriektomie und Behandlung der chronisch thromboembolischen pulmonalen Hypertonie

Zusammenfassung. Es handelt sich bei der chronisch thromboembolischen pulmonalen Hypertonie um ein relativ seltenes Krankheitsbild, welches meist als Folge von akuten Lungenembolien auftritt. Typisch ist die persistierende Atemnot nach Lungenembolien trotz adäquater Antikoagulation. In der Rechtsherzkatheter-Untersuchung findet sich eine präkapilläre pulmonale Hypertonie mit einem erhöhten pulmonalvaskulären Widerstand und konsekutiv zunehmender Rechtsherzinsuffizienz, einhergehend mit entsprechenden klinischen Befunden und reduzierter Prognose. Pathologisch findet sich organisiertes thrombotisches Restmaterial, welches von ungeschulten Augen leicht übersehen wird. Therapie der Wahl stellt die pulmonale Endarteriektomie dar, welche eine deutliche Besserung der Symptomatik, Hämodynamik und Prognose bewirkt. Fehldiagnosen oder verzögerte Zentrumsüberweisung führen häufig zu verspäteter Operation, was mit schlechteren Resultaten assoziiert ist. Sollte eine Operation nicht möglich sein, gibt es die Option einer Ballonangioplastie oder medikamentöser Therapien. Pulmonary Endarterectomy and Treatment for Chronic Thromboembolic Pulmonary Hypertension Abstract. Chronic thromboembolic pulmonary hypertension is a relatively rare disease which mostly evolves as a complication of acute pulmonary embolism resulting from the fibrotic organization of residual thrombotic material despite adequate anticoagulation leading to precapillary pulmonary hypertension and persistence of its symptoms. The elevated pulmonary vascular resistance leads to right ventricular heart failure, its symptoms and reduced prognosis. The therapy of choice is the pulmonary endarterectomy, which leads to a reduction of symptoms, optimization of the hemodynamics and improved prognosis. Misdiagnosis and delayed referral often lead to disease progression along with poor surgical outcome. In case of more distal, surgically non-accessible disease, treatment consists of balloon pulmonary angioplasty and pulmonary vasodilator drugs. Endartériectomie pulmonaire et traitement pour hypertension pulmonaire thromboembolique chronique Résumé. L’hypertension pulmonaire thromboembolique est une maladie rare qui souvent présente une séquelle des embolies pulmonaires imminentes. L’organisation des caillots résiduels malgré d’une anticoagulation adéquate induit une hypertension pulmonaire précapillaire, une résistance pulmonaire élevée et une persistance des symptômes. Cela résulte dans une dysfonction ventriculaire droite progressive et un pronostic défavorable. Le traitement de choix est l’endartériectomie pulmonaire chirurgicale, ce qui entraîne une amélioration des symptômes, de l’hémodynamique et du pronostic. Cette amélioration est souvent retardée en raison de diagnostics erronés ou de l’absence d’orientation vers un centre, ce qui est associé à de moins bons résultats. En cas des patients inopérables, l’angioplastie pulmonaire par ballonnet et la thérapie médicamenteuse sont des alternatives effectives

Prediction of maximal oxygen uptake from 6-min walk test in pulmonary hypertension

Maximal oxygen uptake (V'O2 max), assessed by cardiopulmonary exercise testing (CPET), is an important parameter for risk assessment in patients with pulmonary hypertension (PH). However, CPET may not be available for all PH patients. Thus, we aimed to test previously published predictive models of V'O2 max from the 6-min walk distance (6MWD) for their accuracy and to create a new model. We tested four models (two by Ross et al. (2010), one by Miyamoto et al. (2000) and one by Zapico et al. (2019)). To derive a new model, data were split into a training and testing dataset (70:30) and step-wise linear regression was performed. To compare the different models, the standard error of the estimate (SEE) was calculated and the models graphically compared by Bland-Altman plots. Sensitivity and specificity for correct prediction into low-risk classification (V'O2 max >15 mL/min/kg) was calculated for all models. A total of 276 observations were included in the analysis (194/82 training/testing dataset); 6MWD and V'O2 max were significantly correlated (r=0.65, p<0.001). Linear regression showed significant correlation of 6MWD, weight and heart rate response (HRR) with V'O2 max and the best fitting prediction equation was: V'O2 max = 1.83 + 0.031 × 6MWD (m) - 0.023 × weight (kg) - 0.015 × HRR (bpm). SEEs for the different models were 3.03, 3.22, 4.36 and 3.08 mL/min/kg for the Ross et al., Miyamoto et al., Zapico et al. models and the new model, respectively. Predicted mean V'O2 max was 16.5 mL/min/kg (versus observed 16.1 mL/min/kg). 6MWD and V'O2 max reveal good correlation in all models. However, the accuracy of all models is inadequate for clinical use. Thus, CPET and 6MWD both remain valuable risk assessment tools in the management of PH

Partial Pressure of Arterial Oxygen in Healthy Adults at High Altitudes: A Systematic Review and Meta-Analysis

Importance: With increasing altitude, the partial pressure of inspired oxygen decreases and, consequently, the Pao$_{2}$ decreases. Even though this phenomenon is well known, the extent of the reduction as a function of altitude remains unknown. Objective: To calculate an effect size estimate for the decrease in Pao$_{2}$ with each kilometer of vertical gain among healthy unacclimatized adults and to identify factors associated with Pao$_{2}$ at high altitude (HA). Data Sources: A systematic search of PubMed and Embase was performed from database inception to April 11, 2023. Search terms included arterial blood gases and altitude. Study Selection: A total of 53 peer-reviewed prospective studies in healthy adults providing results of arterial blood gas analysis at low altitude (&amp;lt;1500 m) and within the first 3 days at the target altitude (≥1500 m) were analyzed. Data Extraction and Synthesis: Primary and secondary outcomes as well as study characteristics were extracted from the included studies, and individual participant data (IPD) were requested. Estimates were pooled using a random-effects DerSimonian-Laird model for the meta-analysis. Main Outcomes and Measures: Mean effect size estimates and 95% CIs for reduction in Pao$_{2}$ at HA and factors associated with Pao$_{2}$ at HA in healthy adults. Results: All of the 53 studies involving 777 adults (mean [SD] age, 36.2 [10.5] years; 510 men [65.6%]) reporting 115 group ascents to altitudes between 1524 m and 8730 m were included in the aggregated data analysis; 13 of those studies involving 305 individuals (mean [SD] age, 39.8 [13.6] years; 185 men [60.7%]) reporting 29 ascents were included in the IPD analysis. The estimated effect size of Pao$_{2}$ was −1.60 kPa (95% CI, −1.73 to −1.47 kPa) for each 1000 m of altitude gain (τ$^{2}$ = 0.14; I$^{2}$ = 86%). The Pao$_{2}$ estimation model based on IPD data revealed that target altitude (−1.53 kPa per 1000 m; 95% CI, −1.63 to −1.42 kPa per 1000 m), age (−0.01 kPa per year; 95% CI, −0.02 to −0.003 kPa per year), and time spent at an altitude of 1500 m or higher (0.16 kPa per day; 95% CI, 0.11-0.21 kPa per day) were significantly associated with Pao$_{2}$. Conclusions and Relevance: In this systematic review and meta-analysis, the mean decrease in Pao$_{2}$ was 1.60 kPa per 1000 m of vertical ascent. This effect size estimate may improve the understanding of physiological mechanisms, assist in the clinical interpretation of acute altitude illness in healthy individuals, and serve as a reference for physicians counseling patients with cardiorespiratory disease who are traveling to HA regions