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1.
J Magn Reson Imaging ; 2020 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-32096280

RESUMO

BACKGROUND: The translation of phase-resolved functional lung (PREFUL)-MRI to routine practice in monitoring chronic thromboembolic pulmonary hypertension (CTEPH) still requires clinical corresponding imaging biomarkers of pulmonary vascular disease. PURPOSE: To evaluate successful pulmonary endarterectomy (PEA) via PREFUL-MRI with pulmonary pulse wave transit time (pPTT). STUDY TYPE: Retrospective. POPULATION: Thirty CTEPH patients and 12 healthy controls were included. FIELD STRENGTH/SEQUENCE: For PREFUL-MRI a 2D spoiled gradient echo sequence and for DCE-MRI a 3D time-resolved angiography with stochastic trajectories (TWIST) sequence were performed on 1.5T. ASSESSMENT: Eight coronal slices of PREFUL-MRI were obtained on consecutive 13 days before and 14 days after PEA. PREFUL quantitative lung perfusion (PREFULQ ) phases over the whole cardiac cycle were calculated to quantify pPTT, the time the pulmonary pulse wave travels from the central pulmonary arteries to the pulmonary capillaries. Also, perfusion defect percentage based on pPTT (QDPpPTT ), PREFULQ (QDPPREFUL ), and V/Q match were calculated. For DCE-MRI, pulmonary blood flow (PBF) and QDPPBF were computed as reference. For clinical correlation, mean pulmonary arterial pressure (mPAP) and 6-minute walking distance were evaluated preoperatively and after PEA. STATISTICAL TESTS: The Shapiro-Wilk test, paired two-sided Wilcoxon rank sum test, Dice coefficient, and Spearman's correlation coefficient (ρ) were applied. RESULTS: Median pPTT was significantly lower post PEA (139 msec) compared to pre PEA (193 msec), P = 0.0002. Median pPTT correlated significantly with the mPAP post PEA (r = 0.52, P < 0.008). Median pPTT was distributed more homogeneously after PEA: IQR pPTT decreased from 336 to 281 msec (P < 0.004). Median PREFULQ (P < 0.0002), QDPpPTT (P < 0.0478), QDPPREFUL (P < 0.0001) and V/Q match (P < 0.0001) improved significantly after PEA. Percentage change of PREFULQ correlated significantly with percentage change of 6-minute walking distance (ρ = 0.61; P = 0.0031) 5 months post PEA. DATA CONCLUSION: Perioperative perfusion changes in CTEPH can be detected and quantified by PREFUL-MRI. Normalization of pPTT reflects surgical success and improvement of PREFULQ predicts 6-minute walking distance changes. LEVEL OF EVIDENCE: 3 TECHNICAL EFFICACY STAGE: 2.

2.
Dtsch Med Wochenschr ; 144(19): 1367-1372, 2019 09.
Artigo em Alemão | MEDLINE | ID: mdl-31277079

RESUMO

The ESC/ERS guidelines (published at the end of 2015) and other international recommendations defined pulmonary hypertension (PH) by an invasively measured mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg at rest. At the 6th World Symposium on Pulmonary Hypertension in Nice a modification of this hemodynamic definition in the sense of lowering the threshold to > 20 mmHg was proposed. A pulmonary vascular resistance (PVR) ≥ 3 Wood units (WU) is additionally required for the diagnosis of pre-capillary PH. This modification must be critically reviewed with regard to the underlying rationale and possible consequences. Therefore, a detailed explanation is required. In particular, it must be made clear that this change currently has no influence on the evidence-based and approval-compliant prescription of drugs for the targeted therapy of pulmonary arterial hypertension (PAH).


Assuntos
Hemodinâmica/fisiologia , Hipertensão Pulmonar , Anti-Hipertensivos/uso terapêutico , Cardiologia/organização & administração , Europa (Continente) , Humanos , Hipertensão Pulmonar/classificação , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/fisiopatologia , Guias de Prática Clínica como Assunto , Pneumologia/organização & administração , Resistência Vascular
3.
J Heart Lung Transplant ; 38(7): 748-756, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31128966

RESUMO

BACKGROUND: In patients with pulmonary arterial hypertension who have an insufficient response to oral or inhaled therapies, current guidelines recommend the use of parenteral prostacyclin analogues, although the efficacy of this approach is unknown. METHODS: This retrospective multicenter study evaluated patients with pulmonary arterial hypertension who received intravenous treprostinil as an add-on therapy. The risk at baseline and follow-up (6-12 months after the initiation of treprostinil) was classified as low, intermediate, or high according to current recommendations. The outcome was measured as transplant-free survival after the initiation of treprostinil therapy. RESULTS: A total of 126 patients were analyzed, almost all of them pre-treated with combinations of other pulmonary arterial hypertension medications. Before the initiation of intravenous treprostinil, 2 (2%) patients had a low-risk profile; 100 (79%), an intermediate-risk profile; and 24 (19%), a high-risk profile. At follow-up, 24 (19%) patients were classified as low-risk. These patients had a 5-year transplant-free survival rate >90%. In contrast, patients who remained at intermediate or high risk had transplant-free survival rates of 76%, 43%, and 28% at 1, 3, and 5 years, respectively. Failure to reach a low risk at follow-up was an independent predictor of transplant-free survival (hazard ratio, 9.25; 95% confidence interval, 1.20-71.60; p = 0.033 1). CONCLUSIONS: Risk assessment at 6-12 months after the initiation of add-on intravenous treprostinil in patients with an insufficient response to nonparenteral treatments allows the prediction of transplant-free survival over the ensuing years. Achieving a low-risk profile is associated with excellent outcomes, whereas mortality is high in patients who remain at intermediate or high risk.

5.
Eur Respir J ; 53(1)2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30545980

RESUMO

Pulmonary hypertension (PH) frequently complicates the course of patients with various forms of chronic lung disease (CLD). CLD-associated PH (CLD-PH) is invariably associated with reduced functional ability, impaired quality of life, greater oxygen requirements and an increased risk of mortality. The aetiology of CLD-PH is complex and multifactorial, with differences in the pathogenic sequelae between the diverse forms of CLD. Haemodynamic evaluation of PH severity should be contextualised within the extent of the underlying lung disease, which is best gauged through a combination of physiological and imaging assessment. Who, when, if and how to screen for PH will be addressed in this article, as will the current state of knowledge with regard to the role of treatment with pulmonary vasoactive agents. Although such therapy cannot be endorsed given the current state of findings, future studies in this area are strongly encouraged.

6.
Eur Radiol ; 29(4): 1693-1702, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30311032

RESUMO

OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) can be treated with balloon pulmonary angioplasty (BPA) in inoperable patients. Sensitive non-invasive imaging methods are missing to detect treatment response after a single BPA treatment session. Therefore, the aim of this study was to measure treatment response after a single BPA session using cardio-pulmonary MRI. MATERIALS AND METHODS: Overall, 29 patients with CTEPH were examined with cardio-pulmonary MRI before and 62 days after their initial BPA session. Pulmonary blood flow (PBF), first-pass bolus kinetic parameters, and biventricular mass and function were determined. Multiple linear regression analysis was implemented to estimate the relationship of PBF change in the treated lobe with treatment change of full width at half maximum (FWHM), cardiac output (CO), ventricular mass index (VMI), pulmonary transit time (PTT) and PBF change in the non-treated lobes. Paired Wilcoxon rank sum test and Spearman rho correlation were used. RESULTS: After BPA regional PBF increased in the treated lobe (p < 0.0001) as well as in non-treated lobes (p = 0.015). PBF treatment changes in the treated lobe were significantly larger compared with the non-treated lobes (p = 0.0049). Change in NT proBNP, MRI-derived mean pulmonary artery pressure (mPAP), PTT, FWHM, right ventricular (RV) ejection fraction, RV stroke volume, CO, VMI and PBF in the non-treated lobes correlated with PBF change in the treated lobe (p < 0.05). PBF changes in the treated lobe were independently predicted by PTT as well as PBF change in the non-treated lobes. CONCLUSION: Cardio-pulmonary MRI detects and quantifies treatment response after a single BPA treatment session. KEY POINTS: • Two months after BPA regional parenchymal pulmonary perfusion (PBF) increased in the total lung parenchyma (p = 0.005), the treated lobes (p < 0.0001) and non-treated lobes (p = 0.015). • The PBF treatment changes in the treated lobe were significantly larger than in the non-treated lobes (p = 0.0049). • Change in NT proBNP, MRI-derived mean pulmonary artery pressure, pulmonary transit time, full width at half maximum, right ventricular (RV) ejection fraction, RV stroke volume, cardiac output, ventricular mass index and PBF in the non-treated lobes correlated with PBF change in the treated lobe (p < 0.05).


Assuntos
Angioplastia com Balão/métodos , Hipertensão Pulmonar/terapia , Embolia Pulmonar/terapia , Idoso , Débito Cardíaco/fisiologia , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Angiografia por Ressonância Magnética/métodos , Imagem por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Peptídeo Natriurético Encefálico/sangue , Fragmentos de Peptídeos/sangue , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar/fisiologia , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento , Função Ventricular Direita/fisiologia
7.
Eur Radiol ; 29(3): 1276-1284, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30209593

RESUMO

AIM: To assess the frequency and severity of complications of balloon pulmonary angioplasty (BPA) using C-arm computed tomography (CACT) guidance. MATERIAL AND METHODS: 266 consecutive interventions in 67 patients (42 females, mean age 66 ± 13 years) were included. Selective CACT was acquired prior to the intervention for three-dimensional (3D) guidance and to select appropriate balloon size based on the measured vessel diameter. Complications during and after the procedure, the need for further interventions and the impact on patient safety and outcome were assessed and categorised according to the SIR Classification System to Complications by Outcome (Grade A-F). RESULTS: Overall, 237 interventions were conducted without any complications (89.1%). Minor complications not requiring additional treatment occurred during or after 25 procedures (9.4%), including recurring dry cough in four patients during a total of 11 interventions (4.1%) (Grade A), three focal dissections of the targeted pulmonary artery (1.1%), four cases of pulmonary haemorrhage (1.5%), one case of reperfusion oedema (0.4%) and six cases of post-interventional short-term hemoptysis (2.3%) (Grade B). Four cases of major complications requiring additional treatment were observed (1.5%): one case of pulmonary haemorrhage (0.4%) and two cases of post-interventional haemoptysis (0.8%), all resolved after medical therapy without requiring further intervention, and one case of atrial tachycardia induced during catheterisation, subsequently requiring pharmacological cardioversion (0.4%) (Grade C). No fatal or life-threatening peri- or post-interventional complications or mortality were observed (Grade D-F). CONCLUSION: BPA performed under CACT guidance appears to be a safe procedure with a low risk of severe complications. KEY POINTS: • CACT guidance of BPA is safe and successful. • CACT-guided BPA procedures have a low complication profile. • CACT guidance is a valuable tool to navigate BPA.


Assuntos
Angioplastia com Balão/efeitos adversos , Angioplastia com Balão/métodos , Segurança do Paciente/estatística & dados numéricos , Radiografia Intervencionista/métodos , Tomografia Computadorizada por Raios X/métodos , Idoso , Feminino , Humanos , Imageamento Tridimensional/métodos , Pneumopatias/terapia , Masculino , Artéria Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento
8.
Respir Res ; 19(1): 258, 2018 Dec 19.
Artigo em Inglês | MEDLINE | ID: mdl-30567595

RESUMO

BACKGROUND: Riociguat is a soluble guanylate cyclase stimulator approved for pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTPEH). The objective of this study was to evaluate right heart size and function assessed by echocardiography during long term treatment with riociguat. METHODS: Patients who started riociguat treatment (1.0-2.5 mg tid) within the trials phase II, PATENT, PATENTplus, EAS, CHEST and continued treatment for 3-12 months were included in this study. Echocardiography was analysed off-line at baseline, after 3, 6 and 12 months by investigators who were blinded to clinical data. Last and baseline observation carried forward method (LOCF, BOCF) were performed as sensitivity analysis. RESULTS: Seventy-one patients (45% PAH, 55% CTEPH; 53.5% female; 60 ± 13 years, mean pulmonary arterial pressure 46 ± 10 mmHg, mean PVR 700 ± 282dynes·sec·cm-5) were included. After 6 months, RA and RV area, RV thickness tricuspid regurgitation velocity showed a significant reduction. After 12 months, patients receiving riociguat therapy showed a significant reduction in right atrial (- 2.6 ± 4.4 cm2, 95% CI -3.84, - 1.33; p < 0.001, n = 49) and right ventricular (RV) area (- 3.5 ± 5.2 cm2, 95% CI -5.1, - 1.9; p < 0.001; n = 44), RV thickness (- 0.76 ± 2.2 mm, 95% CI -1.55, 0.03; n = 32), and a significant increase in TAPSE (2.95 ± 4.78 mm, 95% CI 1.52, 4.39; n = 45) and RV fractional area change (8.12 ± 8.87 mm, 95% CI 4.61, 11.62; n = 27). Both LOCF and BOCF showed similar results but lower effect sizes. CONCLUSION: Patients under long-term treatment with riociguat show significantly reduced right heart size and improved RV function in PAH and CTEPH. Further controlled prospective studies are needed to confirm these results.


Assuntos
Ventrículos do Coração/efeitos dos fármacos , Hipertensão Pulmonar/tratamento farmacológico , Embolia Pulmonar/tratamento farmacológico , Pirazóis/uso terapêutico , Pirimidinas/uso terapêutico , Função Ventricular Direita/efeitos dos fármacos , Idoso , Doença Crônica , Método Duplo-Cego , Ativadores de Enzimas/farmacologia , Ativadores de Enzimas/uso terapêutico , Feminino , Ventrículos do Coração/diagnóstico por imagem , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/fisiopatologia , Pirazóis/farmacologia , Pirimidinas/farmacologia , Estudos Retrospectivos , Função Ventricular Direita/fisiologia
9.
J Heart Lung Transplant ; 37(10): 1235-1244, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30293617

RESUMO

BACKGROUND: We examined safety and long-term outcomes of intravenous treprostinil administered via the implantable LENUS Pro pump in patients with severe pulmonary hypertension (PH). METHODS: Patients with PH undergoing pump implantation between December 2009 and October 2016 in German referral centers were retrospectively analyzed (end of follow-up: May 2017). The primary objective was to determine long-term safety of the implantable pump. Secondary end points were 3-year survival and prognostic relevance of pre-implantation hemodynamics. RESULTS: We monitored 129 patients (120 with pulmonary arterial hypertension, 1 with PH due to lung diseases, and 8 with inoperable chronic thromboembolic PH) for 260 patient-years (median follow-up, 19 months; interquartile range, 11-34 months). There were 82 complications/peri-procedural events in 60 patients; of these, 57 were serious adverse events (0.60 per 1,000 treatment-days), including 2 periprocedural deaths due to right heart failure. The incidence of complications related to the pump, catheter, infection, and pump pocket per 1,000 treatment-days was 0.074, 0.264, 0.032 (3 local infections; no bloodstream infections), and 0.380, respectively. Three-year overall and transplant-free survival were 66.5% and 55.7%, respectively (39 patients died; 16 underwent lung transplantation). Baseline cardiac index independently predicted transplant-free survival (multivariate hazard ratio, 1.90; 95% confidence interval, 1.11-3.28; p = 0.019; n = 95). CONCLUSIONS: Our data suggest that intravenous treprostinil via the LENUS Pro pump in advanced PH is associated with a very low risk of bloodstream infections, but other serious adverse events may occur. Therefore, this therapy needs standardization and should be offered in specialized PH centers only. Further technical advances of the pump system and prospective studies are needed.


Assuntos
Epoprostenol/análogos & derivados , Hipertensão Pulmonar/tratamento farmacológico , Bombas de Infusão Implantáveis , Adulto , Causas de Morte , Doença Crônica , Epoprostenol/administração & dosagem , Epoprostenol/efeitos adversos , Falha de Equipamento , Feminino , Seguimentos , Humanos , Hipertensão Pulmonar/mortalidade , Bombas de Infusão Implantáveis/efeitos adversos , Transplante de Pulmão , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Taxa de Sobrevida , Resultado do Tratamento
10.
Eur Respir J ; 52(5)2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30337446

RESUMO

Abbreviated versions of the risk stratification strategy of the European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension guidelines have been recently validated in patients with pulmonary arterial hypertension. We aimed to investigate their prognostic value in medically treated chronic thromboembolic pulmonary hypertension (CTEPH) patients from the COMPERA registry, which collects six variables of interest (World Health Organization Functional Class, 6-min walk distance, brain natriuretic peptide, right atrial pressure, cardiac index and mixed venous oxygen saturation).We included patients with at least one follow-up visit, no pulmonary endarterectomy and at least three of the six variables available, and classified the patients into low-, intermediate- and high-risk groups. As a secondary analysis, the number of noninvasive low-risk criteria was counted. The association between risk assessment and survival was evaluated.Data from inclusion and follow-up (median 7 months) visits were available for 561 and 231 patients, respectively. Baseline 1- and 5-year survival estimates were significantly different (p<0.0001) in the baseline low-risk (98.6% and 88.3%, respectively), intermediate-risk (94.9% and 61.8%, respectively) and high-risk (75.5% and 32.9%, respectively) cohorts. Follow-up data were even more discriminative, with 100%, 92% and 69% 1-year survival, respectively. The number of low-risk noninvasive criteria was also associated with survival.These analyses suggest that the ESC/ERS risk assessment may be applicable in patients with medically treated CTEPH.


Assuntos
Hipertensão Pulmonar/mortalidade , Hipertensão Pulmonar/fisiopatologia , Embolia Pulmonar/complicações , Medição de Risco/métodos , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Europa (Continente)/epidemiologia , Feminino , Humanos , Hipertensão Pulmonar/terapia , Masculino , Pessoa de Meia-Idade , Guias de Prática Clínica como Assunto , Prognóstico , Sistema de Registros , Sociedades Médicas , Análise de Sobrevida
12.
Int J Cardiol ; 272S: 69-78, 2018 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-30195840

RESUMO

Chronic thromboembolic pulmonary hypertension (CTEPH) is a subgroup of pulmonary hypertension that differs from all other forms of PH in terms of its pathophysiology, patient characteristics and treatment. For implementation of the European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension in Germany, the Cologne Consensus Conference 2016 was held and last updated in spring of 2018. One of the working groups was dedicated to CTEPH, practical and controversial issues were commented and updated. In every patient with suspected PH, CTEPH or chronic thromboembolic disease (CTED, i.e. symptomatic residual vasculopathy without pulmonary hypertension) should be excluded. Primary treatment is surgical pulmonary endarterectomy (PEA) in a multidisciplinary CTEPH centre. Inoperable patients or patients with persistent or recurrent CTEPH after PEA are candidates for targeted drug therapy. There is increasing experience with balloon pulmonary angioplasty (BPA) for inoperable patients; this option, like PEA, is reserved for specialised centres with expertise in this treatment method.


Assuntos
Conferências de Consenso como Assunto , Hipertensão Pulmonar/terapia , Guias de Prática Clínica como Assunto/normas , Embolia Pulmonar/terapia , Angioplastia com Balão/métodos , Angioplastia com Balão/normas , Doença Crônica , Alemanha/epidemiologia , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/epidemiologia , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/epidemiologia
13.
Int J Cardiol ; 272S: 46-52, 2018 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-30190155

RESUMO

In June 2016, members of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) met for a Consensus Conference in Cologne, Germany. Aim of this Conference was to compile consensus based practice recommendations based on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. This article summarizes the results and updated recommendations 2018 of the working group on decompensated right heart failure (RHF), intensive care and perioperative management in patients with pulmonary hypertension. The RHF section comprises definition and pathophysiology, diagnosis and monitoring, identification of triggering factors and supportive therapy of RHF, volume management as well as PAH targeting therapy, therapy with inotropic, inodilator and vasopressor drugs, extracorporeal support and transplantation. The second part of this article summarizes preoperative management, perioperative monitoring and choice of anesthesia.


Assuntos
Conferências de Consenso como Assunto , Cuidados Críticos/métodos , Insuficiência Cardíaca/terapia , Hipertensão Pulmonar/terapia , Assistência Perioperatória/métodos , Guias de Prática Clínica como Assunto/normas , Gerenciamento Clínico , Alemanha/epidemiologia , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/epidemiologia , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/epidemiologia , Ensaios Clínicos Controlados Aleatórios como Assunto/métodos , Ensaios Clínicos Controlados Aleatórios como Assunto/normas
14.
Int J Cardiol ; 272S: 30-36, 2018 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-30190156

RESUMO

In the summer of 2016, delegates from the German Respiratory Society, the German Society of Cardiology and the German Society of Pediatric Cardiology met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary arterial hypertension (PAH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to general measures (i.e. physical activity/supervised rehabilitation, pregnancy/contraception, elective surgery, infection prevention, psychological support, travel) and supportive therapy (i.e. anticoagulants, diuretics, oxygen, cardiovascular medications, anaemia/iron deficiency, arrhythmias) for PAH. While the European guidelines provide detailed recommendations for the use of targeted PAH therapies as well as supportive care, detailed treatment decisions in routine clinical care may be challenging, and the relevance of supportive care is often not sufficiently considered. In addition, new evidence became available, thus requiring a thorough reevaluation of specific recommendations. The detailed results and recommendations of the working group on general measures and supportive therapy for PAH, which were last updated in the spring of 2018, are summarized in this article.


Assuntos
Conferências de Consenso como Assunto , Hipertensão Pulmonar/psicologia , Hipertensão Pulmonar/terapia , Cuidados Paliativos/normas , Guias de Prática Clínica como Assunto/normas , Alemanha/epidemiologia , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/fisiopatologia , Cuidados Paliativos/métodos
15.
Int J Cardiol ; 272S: 37-45, 2018 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-30190158

RESUMO

In the summer of 2016, delegates from the German Respiratory Society, the German Society of Cardiology and the German Society of Pediatric Cardiology met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary arterial hypertension (PAH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines and included new evidence, where available. The treatment algorithm for PAH was modified based on the observation that there are now many patients diagnosed with IPAH who are at an advanced age and have significant cardiopulmonary comorbidities. For patients newly diagnosed with classic forms of PAH, i.e. younger patients without significant cardiopulmonary comorbidities, the consensus-based recommendation was to use initial combination therapy as the standard approach. The use of monotherapies was no longer considered appropriate in such patients. The choice of treatment strategies should be based on the risk assessment as proposed in the European guidelines. In patients presenting with a low or intermediate risk, oral combination therapy with endothelin receptor antagonists and phosphodiesterase-5 inhibitors or soluble guanylate cyclase stimulators, respectively, should be used. In high-risk patients, triple combination therapy including a subcutaneous or intravenous prostacyclin analogue should be considered. For patients who suffer from PAH and significant cardiopulmonary comorbidities, initial monotherapy is recommended and the use of combination therapies should be considered on an individual basis. The latter recommendations are based on the scarcity of evidence supporting the use of combination therapy and the higher risk of drug-related adverse events in such patients.


Assuntos
Conferências de Consenso como Assunto , Sistemas de Liberação de Medicamentos/métodos , Hipertensão Pulmonar/tratamento farmacológico , Guias de Prática Clínica como Assunto/normas , Terapia Combinada/métodos , Terapia Combinada/tendências , Sistemas de Liberação de Medicamentos/tendências , Antagonistas dos Receptores de Endotelina/administração & dosagem , Antagonistas dos Receptores de Endotelina/metabolismo , Alemanha/epidemiologia , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/metabolismo , Inibidores da Fosfodiesterase 5/administração & dosagem , Inibidores da Fosfodiesterase 5/metabolismo
16.
Clin Nucl Med ; 43(6): e170-e177, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29688945

RESUMO

BACKGROUND: We evaluated the feasibility of perfusion SPECT/CT for providing quantitative data for estimation of perfusion defect extent in chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: Thirty patients with CTEPH underwent Tc-human serum albumin lung perfusion SPECT/CT. Perfusion defects were quantified using 3 different methods: (1) visual, semiquantitative scoring of perfusion defect extent in each lung segment, (2) threshold-based segmentation of perfused lung volumes, and (3) threshold-based segmentation of perfused lung volumes divided by segmented lung volumes at CT (perfusion index). Imaging findings were correlated with right-sided heart catheterization results and N-terminal pro-B-type natriuretic peptide. Receiver operating characteristic analysis was performed to identify SPECT thresholds for mean pulmonary arterial pressure (PAPm) greater than 50 mm Hg. RESULTS: Assessment of lung perfusion provided similar results using all 3 methods. The perfusion defect score correlated with PAPm (rs = 0.60, P = 0.0005) and was associated with serum levels of N-terminal pro-B-type natriuretic peptide (rs = 0.37, P = 0.04). Perfused lung volume (40% threshold, rs = -0.48, P = 0.007) and perfusion index (40% threshold, rs = -0.50, P = 0.005) decreased as PAPm increased. Receiver operating characteristic analysis showed that perfusion defect score (sensitivity, 88%; specificity, 77%; area under the curve [AUC] = 0.89, P = 0.001), perfused lung volume (sensitivity, 88%; specificity, 64%; AUC = 0.80, P = 0.01), and perfusion index (sensitivity, 88%; specificity, 64%; AUC = 0.82, P = 0.009) could identify patients with PAPm of greater than 50 mm Hg. CONCLUSIONS: Quantitative analysis of perfusion defects at SPECT is feasible, provides a measure of disease severity, and correlates with established clinical parameters. Quantitation of perfusion SPECT may refine the diagnostic approach in CTEPH providing a quantitative imaging biomarker, for example, for therapy monitoring.


Assuntos
Hipertensão Pulmonar/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Tomografia Computadorizada com Tomografia Computadorizada de Emissão de Fóton Único , Feminino , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Medidas de Volume Pulmonar , Masculino , Pessoa de Meia-Idade
18.
Clin Res Cardiol ; 107(7): 548-553, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29450722

RESUMO

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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. CLINICAL TRIALS REGISTRATION: http://www.clinicaltrials.gov NCT02660463 and NCT01347216.


Assuntos
Hipertensão Pulmonar/epidemiologia , Embolia Pulmonar/epidemiologia , Tromboembolia Venosa/epidemiologia , Idoso , Angioplastia com Balão , Anti-Hipertensivos/uso terapêutico , Doença Crônica , Endarterectomia , Feminino , Alemanha/epidemiologia , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/terapia , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/fisiopatologia , Embolia Pulmonar/terapia , Sistema de Registros , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/fisiopatologia , Tromboembolia Venosa/terapia
19.
Pulm Circ ; 8(1): 2045893217753122, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29283044

RESUMO

Symptomatic patients with residual pulmonary perfusion defects or vascular lesions but no pulmonary hypertension at rest are diagnosed with chronic thromboembolic disease (CTED). Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but data regarding the safety and efficacy of BPA in patients with CTED are lacking. We report a prospective series of ten consecutive patients with CTED who underwent 35 BPA interventions (median of four per patient) at two German institutions. All patients underwent a comprehensive diagnostic workup at baseline and 24 weeks after their last intervention. BPA was safe, with one pulmonary vascular injury and subsequent self-limiting pulmonary bleeding as the only complication (2.9% of the interventions, 10% of the patients). After the procedures, World Health Organization functional class, 6-min walking distance, pulmonary vascular resistance, and pulmonary arterial compliance improved, and NT-proBNP concentrations declined in 9/10 patients. BPA may be a new treatment option for carefully selected patients with CTED. A larger, prospective, international registry is required to confirm these results.

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