Pulmonary stenosis development and reduction of pulmonary arterial hypertension in atrioventricular septal defect: a case report

A 24-year-old patient was admitted for dyspnoea and syncope. He had a previous history of complete atrio-ventricular septal defect and trisomy 21. At the age of 6 months, in 1984, cardiac catheterization revealed a quasi-systemic pulmonary arterial hypertension with a bidirectional shunt corresponding to an Eisenmenger syndrome. Corrective cardiac surgery was not performed at this time because surgical risk was considered too high. Until the age of 20 years old, he showed few symptoms while under medical treatment. But since 2006, his functional status became worse with an increased dyspnoea, syncopes, and severe cyanosis. In these conditions, haemodynamic parameters have been re-evaluated in 2006 and 2008

Neural respiratory drive in chronic thromboembolic pulmonary hypertension: Effect of balloon pulmonary angioplasty

Excessive ventilation (V̇E) during exercise, ascribed to heightened neural ventilatory drive and/or to increased "wasted" ventilation, is a feature of chronic thromboembolic pulmonary hypertension (CTEPH). In selected CTEPH patients, balloon pulmonary angioplasty (BPA) allows near-normalization of resting haemodynamic parameters but does not allow excess exercise hyperventilation to normalize. Neural ventilatory drive can be estimated by studying how arterial PCO2 (PaCO2), end-tidal PCO2 (PETCO2), V̇E and CO2 output (V̇CO2) change across the exercise-to-recovery transition during a cardiopulmonary exercise test. Increased "wasted" ventilation can be quantified by the physiological dead space fraction of tidal volume (VD/VT) calculated with the Enghoff simplification of the Bohr equation. These measurements were made before and after BPA in 22 CTEPH patients without significant cardiac and/or pulmonary comorbidities. Our observations suggest that before BPA, excessive hyperventilation was secondary to both heightened neural ventilatory drive and increased "wasted" ventilation; after BPA, measurements made across the exercise-to-recovery transition suggest that heightened neural ventilatory drive was no longer present

Improved ventilatory efficiency to evidence haemodynamic improvement after balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension

In contrast to the 6-minute walk test, the cardiopulmonary exercise test provides simple,non-invasive parameters that correlate with haemodynamic improvements in patients with chronicthromboembolic pulmonary hypertension undergoing balloon angioplasty

Inappropriate dispatcher decision for emergency medical service users with acute myocardial infarction.

International audienceOBJECTIVES: Current guidelines recommend utilization of prehospital emergency medical services (EMSs) by patients with ST-elevation myocardial infarction (STEMI). The aims of this study were to estimate the percentage of inappropriate initial dispatcher decisions and determine their impact on delays in reperfusion therapy for EMS users with STEMI. METHODS: As part of a prospective regional registry of patients with STEMI, we analyzed the original data for 245 patients who called a university hospital-affiliated EMS call center in France. The primary study outcome was time to reperfusion therapy calculated from the documented date and time of the first patient call. RESULTS: The initial EMS dispatcher's decision was appropriate (ie, dispatching a mobile intensive care unit staffed by an emergency or critical care physician) for 171 (70%) patients and inappropriate for 74 (30%) patients. Inappropriate decisions included referring the patient to a family physician (n = 59), providing medical advice (n = 9), and dispatching an ambulance (n = 6). Inappropriate initial decisions resulted in increased median time to reperfusion for 140 patients receiving fibrinolysis (95 vs 53 minutes; P < .001) and 91 patients undergoing primary percutaneous coronary intervention (170 vs 107 minutes; P < .001). In-hospital mortality was not different between the 2 study groups (6.8% vs 9.9%; P = .42). CONCLUSION: The initial dispatcher's decision is inappropriate for 30% of EMS users with STEMI and results in substantial delays in time to reperfusion therapy. Accuracy of telephone triage should be improved for patients who activate EMSs in response to symptoms suggestive of acute coronary syndrome

Editor's Choice - Recent therapeutic trials on fluid removal and vasodilation in acute heart failure

Recent therapeutic trials regarding the management of acute heart failure (AHF) failed to demonstrate the efficacy of newer therapeutic modalities and agents. Low- versus high-dose and continuous administration of furosemide were shown not to matter. Ultrafiltration was not found to be more efficacious than sophisticated diuretic therapy including dose-adjusted intravenous furosemide and metolazone. Dopamine and nesiritide were not shown to be superior to current therapy. Tezosentan and tovalptan had no effect on mortality. The development of rolofylline was terminated due to adverse effect (seizures). Lastly, preliminary experience with serelaxin indicates a mortality improvement at six months that remains to be confirmed. The disappointing findings of these recent trials may reflect the lack of efficacy of newer therapeutic modalities and agents. Alternatively the disappointing findings of these recent trials may be in part due to methodological issues. The AHF syndrome is complex with many clinical phenotypes. Failure to match clinical phenotypes and therapeutic modalities is likely to be partly responsible for the disappointing findings of recent AHF trials

Place de l’angioplastie percutanée dans le traitement de l’hypertension pulmonaire postembolique

Chronic thromboembolic pulmonary hypertension (CTEPH) is uncommon. Its diagnosis should not be delayed as its prognosis is poor if not treated. In most cases, an acute pulmonary embolism is found in the medical history of the patient. Once suspected, a specific work-up should be performed in a pulmonary hypertension (PH) center. The ventilation/perfusion scan has a central role in this workup but the emergence of non invasive imaging technologies provides morphological and functional information which take part in the therapeutic decision making, such as operability. Surgical endarterectomy remains the only curative treatment. In some specific patients, percutaneous transluminal pulmonary angioplasty (AAP) is performed. This developing technique is a safe and efficient treatment on a clinical and hemodynamic standpoint. The main complication after AAP is lung reperfusion edema

Transition from intravenous epoprostenol to selexipag in pulmonary arterial hypertension: a word of caution

Even for patients who are very stable while receiving i.v. epoprostenol over a long period of time, anytransition to selexipag warrants careful long-term clinical and haemodynamic follow-up becauseworsening is likely to occur

Exercise hyperventilation and pulmonary gas exchange in chronic thromboembolic pulmonary hypertension: Effects of balloon pulmonary angioplasty

BACKGROUND: Excessive ventilation (V̇E) and abnormal gas exchange during exercise are features ofchronic thromboembolic pulmonary hypertension (CTEPH). In selected CTEPH patients, balloon pulmonaryangioplasty (BPA) improves symptoms and exercise capacity. How BPA affects exercisehyperventilation and gas exchange is poorly understood.METHODS: In this longitudinal observational study, symptom-limited cardiopulmonary exercise testsand carbon monoxide lung diffusion (DLCO) were performed before and after BPA (interval, mean(SD): 3.1 (2.4) months) in 36 CTEPH patients without significant cardiac and/or pulmonarycomorbidities.RESULTS: Peak work rate improved by 20% after BPA whilst V ̇ E at peak did not change despiteimproved ventilatory efficiency (lower V ̇ E with respect to CO2 output [V̇CO2]). At the highestidentical work rate pre- and post-BPA (75 (30) watts), V ̇E and alveolar-arterial oxygen gradient(P(Ai-a)O2) decreased by 17% and 19% after BPA, respectively. The physiological dead spacefraction of tidal volume (VD/VT), calculated from measurements of arterial and mixed expired CO2, decreased by 20%. In the meantime, DLCO did not change. The best correlates of P(Ai-a)O2 measured at peak exercise were physiological VD/VT before BPA and DLCO after BPA.CONCLUSIONS: Ventilatory efficiency, physiological VD/VT, and pulmonary gas exchange improvedafter BPA. The fact that DLCO did not change suggests that the pulmonary capillary blood volume andprobably the true alveolar dead space were unaffected by BPA. The correlation between DLCO measuredbefore BPA and P(Ai-a)O2 measured after BPA suggests that DLCO may provide an easilyaccessible marker to predict the response to BPA in terms of pulmonary gas exchange

Hospital costs of Balloon Pulmonary Angioplasty (BPA) procedure and management for CTEPH patients: An observational study based on the French national hospital discharge database (PMSI)

International audienceIntroduction Since 2014, Balloon Pulmonary Angioplasty (BPA) has become an emerging and complementary strategy for chronic thromboembolic hypertension (CTEPH) patients who are not suitable for pulmonary endarterectomy (PEA) or who have recurrent symptoms after the PEA procedure. Objective To assess the hospital cost of BPA sessions and management in CTEPH patients. Methods An observational retrospective cohort study of CTEPH-adults hospitalized for a BPA between January 1st, 2014 and June 30th, 2016 was conducted in the 2 centres performing BPA in France (Paris Sud and Grenoble) using the French national hospital discharge database (PMSI-MCO). Patients were followed until 6 months or death, whichever occurred first. Follow-up stays were classified as stays with BPA sessions, for BPA management or for CTEPH management based on a pre-defined algorithm and a medical review using type of diagnosis (ICD-10), delay from last BPA procedure stay and length of stay. Hospital costs (including medical transports) were estimated from National Health Insurance perspective using published official French tariffs from 2014 to 2016 and expressed in 2017 Euros. Results A total of 191 patients were analysed; mainly male (53%), with a mean age of 64,3 years. The first BPA session was performed 1.1 years in median (IQR 0.3–2.92) after the first PH hospitalisation. A mean of 3 stays with BPA sessions per patient were reported with a mean length of stay of 8 days for the first stay and 6 days for successive stays. The total hospital cost attributable to BPA was € 4,057,825 corresponding to €8,764±3,435 per stay and €21,245±12,843 per patient. Results were sensitive to age classes, density of commune of residence and some comorbidities. Conclusions The study generated robust real-world data to assess the hospital cost of BPA sessions and management in CTEPH patients within its first years of implementation in France