Pulmonary hypertension (PH) is relatively common in connective tissue diseases. However, few studies have focused on the pulmonary hypertension (PH) associated with polymyositis (PM). Our aim is to investigate the prevalence of PH and determine the associated factors for PH in patients with PM. Multicenter study of 61 patients with PM underwent evaluation including general information, physical examination, laboratory indictors, thoracic high-resolution CT (HRCT) imaging, and transthoracic echocardiography (TTE). TTE was performed to estimate the pulmonary arterial pressure. PH was defined as resting systolic pulmonary artery pressure (sPAP) ≥40 mmHg. PH was identified in ten patients (16.39 %) who had few cardiopulmonary symptoms. PM patients with PH had higher prevalence of interstitial lung disease (ILD) and pericardial effusion (PE) compared with patients without PH (18 vs. 11.5 %, p = 0.005; 11.5 vs. 9.8 %, p = 0.004; respectively). After controlling for age, gender, and potential factors, ILD and PE were independently associated with PH in patients with PM in multivariate analysis (OR = 8.193, 95 % CI 1.241–54.084, p = 0.029; OR = 8.265, 95 % CI 1.298–52.084, p = 0.025; respectively). Depending on TTE, the possible prevalence of PH was 16.39 % in patients with PM. Both ILD and PE may contribute to the development of PH in PM.
In patients with mitral stenosis (MS), pulmonary hypertension (PH) is a significant contributor to the associated morbidity. We aimed to study factors associated with the presence of significant PH (sPH) and whether incorporating body surface area (BSA) in the mitral valve area (MVA) would improve the predictive value of the latter.The medical records of 558 patients with severe MS undergoing percutaneous balloon mitral commissurotomy were evaluated over a period of 8 years. Factors associated with the presence of significant PH (sPH) defined as mPAP ≥ 40 mm Hg were examined.A total of 558 patients (423 women) were enrolled. Overall, 153 (27%) patients had sPH. Patients with sPH were similar to the rest of the subjects in terms of demographics, body habitus, blood group, and incidence of atrial fibrillation. Among echocardiographic findings, absolute MVA, indexed MVA, and mean transmitral valve gradient were associated with the presence of sPH. Transmitral valve gradient during right heart catheterization had the highest area under the curve for an association with sPH.Age, gender, heart rhythm, and blood group were not associated with the presence of sPH in severe MS. The predictive value of the indexed MVA for the presence of sPH was not higher than that of absolute MVA.Bei Patienten mit Mitralstenose (MS) trägt eine pulmonale Hypertonie (PH) in signifikanter Weise zur Morbidität bei. Ziel der Arbeit war es, Faktoren, die mit dem Vorliegen einer signifikanten PH (sPH) einhergehen, zu untersuchen und ob die Berücksichtigung der Körperoberfläche (KOF) bei der Mitralöffnungsfläche (MVA) den prädiktiven Wert der Letzteren verbessern würde.Über einen Zeitraum von 8 Jahren wurden die Krankenakten von 558 Patienten mit schwerer MS, bei denen eine perkutane Ballon-Mitralkommissurotomie erfolgte, ausgewertet. Dabei wurden Faktoren untersucht, die mit dem Vorliegen einer sPH, definiert als mPAP ≥ 40 mmHg, einhergingen.Es wurden 558 Patienten (davon 423 Frauen) in die Studie eingeschlossen. Insgesamt 153 (27%) Patienten wiesen eine sPH auf. Patienten mit sPH waren in Bezug auf demographische Merkmale, Körperbeschaffenheit, Blutgruppe und Inzidenz des Vorhofflimmerns ähnlich wie die übrigen Teilnehmer. Bei den echokardiographischen Parametern gingen die absolute MVA, die [an die KOF] gekoppelte MVA und der mittlere transmitrale Klappengradient mit dem Vorliegen einer sPH einher. Der transmitrale Klappengradient während der Rechtsherzkatheterisierung wies die größte Fläche unter der Kurve für eine Assoziation mit der sPH auf.Alter, Geschlecht, Herzrhythmus und Blutgruppe standen nicht mit dem Vorliegen einer sPH bei schwerer MS in Zusammenhang. Der prädiktive Wert der gekoppelten MVA für das Vorliegen einer sPH war nicht größer als der der absoluten MVA.
The aim of this study was to assess the prevalence of congenital heart defects (CHDs) and persistent pulmonary hypertension of the neonate (PPHN) in children with Down syndrome (DS) and to assess its impact on neonatal factors. It was a prospective study of a birth cohort of children with DS born between 2003 and 2006 registered by the Dutch Paediatric Surveillance Unit (DPSU). A CHD occurred in 43% of 482 children with trisomy 21. Atrioventricular septal defect was found in 54%, ventricular septal defect in 33.3% and patent ductus arteriosus in 5.8%. The incidence of PPHN in DS was 5.2%, which is significantly higher than the general population (p < 0.001). The reported mortality in newborns with DS was overall 3.3% and was still significant higher in children with a CHD versus no CHD (5.8% versus 1.5%) (p = 0.008). The presence of CHD in children with DS had no influence on their birth weight, mean gestational age and Apgar score. In neonates with DS, we found not only a 43% prevalence of CHD, but also a high incidence of PPHN at 5.2%. Early recognition of the cardiac condition of neonates with DS seems justified.
The incidence of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Previous studies from the United Kingdom and Spain have reported incidence rates of 1.75 and 0.9 per million, respectively. These figures, however, may underestimate the true incidence of CTEPH.We prospectively enrolled patients newly diagnosed with CTEPH within 2016 in Germany. Data were obtained from the three German referral centers and from the German branch of COMPERA, a European pulmonary hypertension registry. The CTEPH incidence was calculated based on German population data, and patient characteristics and treatment patterns were described.A total of 392 patients were newly diagnosed with CTEPH within 2016 in Germany, yielding an incidence of 5.7 new cases per million adults. The (mean ± standard deviation) age was 63.5 ± 15.0 years; males and females were equally affected; 76.3% of the patients had a history of venous thromboembolism. A total of 197 (50.3%) patients underwent pulmonary endarterectomy. Almost all non-operated patients received targeted drug therapy, and 49 patients (25.1% of the non-operated patients) were treated with balloon pulmonary angioplasty.The incidence of CTEPH in Germany 2016 was 5.7 per million adults and thus higher than previously reported from other countries. Half of the patients were operated while the remaining patients received medical or interventional therapies. http://www.clinicaltrials.gov NCT02660463 and NCT01347216.
Pulmonary hypertension (PH) is a pathophysiological disorder defined by an increase in pulmonary arterial pressure which can occur in multiple clinical conditions. Irrespective of etiology, PH entails a negative impact on exercise capacity and quality of life, and is associated with high mortality particularly in pulmonary arterial hypertension. Noninvasive imaging techniques play an important role in suggesting the presence of PH, providing noninvasive pulmonary pressure measurements, classifying the group of PH, identifying a possibly underlying disease, providing prognostic information and assessing response to treatment. While echocardiography, computed tomography (CT) and ventilation/perfusion scans are an integral part of routine work-up of patients with suspected PH according to current guidelines and across centers, innovative new techniques and applications in the field of PH such as 3D echocardiography, dual-energy CT, 4D flow magnetic resonance imaging (MRI), T1 and extracellular volume fraction mapping, non-contrast-enhanced MRI sequences for perfusion and ventilation assessment, and molecular-targeted positron emission tomography are emerging. This review discusses advanced and emerging imaging techniques in diagnosis, prognostic evaluation and follow-up of patients with PH.
Imaging studies have shown that pulmonary hypertension (PH) is associated with inhomogenous right ventricular (RV) regional contraction, or dyssynchrony, and that this is of prognostic relevance. This study aimed at the identification and functional significance of RV dyssynchrony in borderline PH defined by a mean pulmonary artery pressure between (mPAP) 20 and 25 mmHg. RV dyssynchrony was measured by 2-dimensional speckle tracking echocardiography in 17 patients with pulmonary arterial hypertension (PAH), 13 patients with borderline PH and 14 controls. Dyssynchrony was defined as the R-R interval-corrected standard deviation of the times to peak-systolic strain for the basal and medium segments of the RV. All the PH patients underwent a right heart catheterization. RV dyssynchrony amounted to 69 ± 34 ms in PAH, 47 ± 23 ms in borderline PH and 8 ± 6 ms in controls, all different from each other (p < 0.05). RV dyssynchrony in borderline PH was the only parameter of RV systolic dysfunction in 11 of 13 (85%) of the patients. RV dyssynchrony was accompanied by postsystolic shortening and correlated to RV fractional area change, not to mPAP or pulmonary vascular resistance. RV dyssynchrony occurs in borderline PH and may reflect early RV-arterial uncoupling.
We sought to determine the safety of regadenoson stress testing in patients with PH.PH is increasingly recognized at more advanced ages. As many as one-third of patients with PH have coronary artery disease. Because of their physical limitations, patients with PH are unable to adequately exercise. Regadenoson can potentially have an adverse impact due to their tenuous hemodynamics. Current guidelines suggest performing a coronary angiography in patients with PH who have angina or multiple coronary risk factors.We identified 67 consecutive patients with confirmed PH by catheterization (mean PA > 25 mmHg not due to left heart disease) who underwent MPI with regadenoson stress. Medical records were reviewed to determine hemodynamic and ECG response to regadenoson.No serious events occurred. Common side effects related to regadenoson were observed, dyspnea being the most common (70.6%). No syncope occurred. Heart rate increased from 74.6 ± 14 to 96.3 ± 18.3 bpm, systolic blood pressure increased from 129.8 ± 20.9 to 131.8 ± 31 mmHg, and diastolic blood pressure decreased from 77.1 ± 11.4 to 72.9 ± 15.3 mmHg. There was no ventricular tachycardia, ventricular fibrillation, or second- or third-degree atrioventricular block.Regadenoson stress MPI appears to be well tolerated and safe in patients with PH.
The purpose of this study was to evaluate the prevalence and outcomes of pulmonary hypertension in chronic hypersensitivity pneumonitis and to examine the relationship between pulmonary function tests and pulmonary hypertension.We conducted a retrospective review of 120 patients with hypersensitivity pneumonitis seen at two centers for pulmonary diseases over a 5-year interval and identified patients with chronic hypersensitivity pneumonitis for whom both pulmonary function tests and Doppler echocardiography data were available.Chronic hypersensitivity pneumonitis was identified in 83 patients and Doppler echocardiography data were available for 73 of them. Pulmonary hypertension (sPAP ≥ 50 mmHg) was detected in 14 patients (19%), and was associated with a greater risk of death (median survival = 23 months vs. 98 months; P = 0.003). Patients with pulmonary hypertension were older and had a significantly decreased PaO2. There was a weak correlation between pulmonary function parameters and the underlying sPAP, with significance for FVC, FEV1, and PaO2 and inversely with PaCO2.Using Doppler echocardiography for evaluation, pulmonary hypertension seems to be common in patients with chronic hypersensitivity pneumonitis, significantly impacts survival, and correlates with FVC, FEV1, and PaO2 and inversely with PaCO2.
Independent of the underlying cause, pulmonary hypertension (PH) remains a devastating condition that is characterized by limited survival. Cumulating evidence indicates that in addition to a dysbalance of mediators regulating vascular tone and growth factors promoting vascular remodeling, failure to resolve inflammation and altered immune processes play a pivotal role in the development and progression of PH. Here, we highlight the role of key inflammatory pathways in the pathobiology of vascular remodeling and PH, and discuss potential therapeutic interventions that may halt disease progression or even reverse pulmonary vascular remodeling. Perivascular inflammation is present in all forms of PH, and inflammatory pathways involve numerous mediators and cell types including macrophages, neutrophils, T cells, dendritic cells, and mast cells. Dysfunctional bone morphogenic protein receptor 2 (BMPR2) signaling and dysregulated immunity enable the accumulation of macrophages and other inflammatory cells in obliterative vascular lesions. Regulatory T cells (Tregs) were shown to be of particular relevance in the control of inflammatory responses. Key cytokines/chemokines include interleukin-6, functioning via classic or trans-signaling, macrophage migratory inhibitory factor (MIF), but also other mediators such as neutrophil-derived myeloperoxidase. The expanding knowledge on this topic has resulted in multiple opportunities for sophisticated therapeutic interventions.Unabhängig von der zugrunde liegenden Ursache bleibt die pulmonale Hypertonie (PH) eine schwerwiegende Erkrankung mit reduzierter Lebenserwartung. Aktuelle Studien zeigen, dass neben einem Ungleichgewicht bei vasoaktiven Mediatoren und Wachstumsfaktoren insbesondere dysregulierte inflammatorische Prozesse sowie eine veränderte Immunität eine entscheidende Rolle für die Entstehung und Progression einer PH spielen. Die Autoren fassen die Rolle bedeutsamer inflammatorischer Signalwege in der Pathobiologie des vaskulären Remodelings und der PH zusammen und präsentieren potenzielle therapeutische Interventionen, welche pulmonal vaskulärem Remodeling entgegenwirken könnten. Perivaskuläre Inflammation findet sich bei allen Formen der PH, und inflammatorische Signalwege umfassen zahlreiche Mediatoren und Zelltypen (Makrophagen, Neutrophile, T‑Zellen, dendritische Zellen und Mastzellen). Dysfunktionelle „bone morphogenic protein receptor 2“ (BMPR2) Signale und fehlregulierte Immunität ermöglichen die Akkumulation von Makrophagen und anderen inflammatorischen Zellen in obliterativen vaskulären Läsionen. Regulatorische T‑Zellen (Tregs) sind für die Kontrolle inflammatorischer Prozesse von besonderer Bedeutung. Zu den wichtigsten Zytokinen/Chemokinen gehören Interleukin-6, welches durch klassisches oder „trans-signaling“ fungiert, der „macrophage migratory inhibitory factor“ (MIF), sowie andere Mediatoren wie aus Neutrophilen freigesetzte Myeloperoxidase. Das zunehmende Verständnis dieser Zusammenhänge bietet multiple Möglichkeiten für neuartige therapeutische Interventionen.