scholarly journals Pulmonary Hypertension in Thalassemia Patients

2021 ◽  
Author(s):  
Ahmed Shemran Mutlaq Alwataify ◽  
Sabih Salih Alfatlawy ◽  
Yahia Abid Alshahid Altufaily
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Heart Sound ◽  
Specific Treatment ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Third Heart Sound ◽  
Chronic Hemolysis ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) is defined in children as a mean pulmonary arterial pressure (PAP) greater than 25 mmHg at rest or 30 mmHg during physical activity, with increased pulmonary artery capillary wedge pressure and an increased pulmonary vascular resistance greater than 3 Wood units × M2. it is the main cause of morbidity and mortality in the group of thalassemia, if no treatment leads to right ventricular heart failure and death. The development of pulmonary arterial hypertension (PAH) is assumed to be the result of many multifactorial pathogenic mechanisms including chronic hemolysis, iron overload, hypercoagulability, and erythrocyte dysfunction as a result of splenectomy, inflammation and nitric oxide (NO) depletion. PAH symptoms are non-specific, their signs consist of right ventricular lift, an accentuated pulmonary component of the second heart sound, a (gallop rhythm) right ventricular third heart sound, and parasternal heave meaning a hypertrophied right ventricle. The diagnosis of PAH requires a clinical suspicion based on symptoms and physical examination. Echocardiography is frequently used to screen for PAH, monitor progression over time and allow identification of patients for whom diagnostic right heart catheterization (RHC) is warranted and its treatment includes hemoglobinopathy specific treatment and PAH specific therapy.

Diagnosis and clinical investigation of patients presenting with pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2507-2511 ◽  
Author(s):  
Daniela Calderaro ◽  
Luis Felipe Prada ◽  
Rogério Souza
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Investigation ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Reference Centre ◽  
Mean Pulmonary Arterial Pressure ◽  
Invasive Test ◽  
Pulmonary Arterial

The diagnosis of pulmonary hypertension (PH) relies on the haemodynamic criterion of mean pulmonary arterial pressure greater than or equal to 25 mmHg, assessed by right heart catheterization. The scope of this chapter is to discuss the key elements of clinical assessment of PH patients and the decision process to indicate right heart catheterization. Investigation must get through all the possible causes of PH according to their probability and frequency in the population. Echocardiography is the most important non-invasive test as an indicator for further diagnostic evaluation. Patients who are eligible for right heart catheterization should always be referred to PH centres, where technical skills and standardized procedures will enable maximal reliability of haemodynamic measurement. In the reference centre, a multidisciplinary team will discuss clinical and haemodynamic data, to propose the best therapeutic and follow-up schedule.

scholarly journals Right ventricular dimension index by cardiac magnetic resonance for prognostication in connective tissue diseases and pulmonary hypertension

Rheumatology ◽  
2019 ◽  
Author(s):  
Nobuya Abe ◽  
Masaru Kato ◽  
Michihito Kono ◽  
Yuichiro Fujieda ◽  
Hiroshi Ohira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
Dimension Index

Abstract Objectives Pulmonary hypertension (PH) in patients with CTD is a heterogeneous condition affected by left heart disease, chronic lung disease and thromboembolism as well as pulmonary vascular disease. Recent studies using cardiac magnetic resonance (CMR) have shown that right ventricular dysfunction is predictive for mortality in patients with PH, but limited to pulmonary arterial hypertension. This study aimed to analyse prognostic factors in PH-CTD. Methods This retrospective analysis comprised 84 CTD patients, including SSc, who underwent both CMR and right heart catheterization from 2008 to 2018. Demographics, laboratory findings, and haemodynamic and morphological parameters were extracted. The prognostic value of each parameter was evaluated by multivariate analysis using covariables derived from propensity score to control confounding factors. Results Of 84 patients, 65 had right heart catheterization-confirmed PH (54 pulmonary arterial hypertension, 11 non-pulmonary arterial hypertension). Nine out of these PH patients died during a median follow-up period of 25 months. In 65 patients with PH, right ventricular end-diastolic dimension index (RVEDDI) evaluated by CMR was independently associated with mortality (hazard ratio 1.24; 95% CI: 1.08–1.46; P = 0.003). In a receiver operating characteristic analysis, RVEDDI highly predicted mortality, with area under the curve of 0.87. The 0.5–2-year follow-up data revealed that RVEDDI in both survivors and non-survivors did not significantly change over the clinical course, leading to the possibility that an early determination of RVEDDI could predict the prognosis. Conclusion RVEDDI simply evaluated by CMR could serve as a significant predictor of mortality in PH-CTD. A further validation cohort study is needed to confirm its usability.

scholarly journals Recurrent Hemoptysis in Patient with Primary Pulmonary Hypertension – A Case Report and Literature Review

2017 ◽  
Vol 3 (1) ◽  
pp. 45
Author(s):  
Indra Widya Nugraha ◽  
Bambang Irawan ◽  
Lucia Kris Dinarti
Keyword(s):  
Pulmonary Hypertension ◽  
Bronchial Artery ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Emergency Procedure ◽  
Artery Embolization ◽  
Bronchial Arteries ◽  
Mean Pulmonary Arterial Pressure ◽  
Abnormal Distribution ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) is defned as an increase in mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg at rest as assessed by right heart catheterization (RHC). The symptoms of PH are non-specifc and mainly related to progressive right ventricular (RV) dysfunction. In some patients the clinical presentation may be related to mechanical complications of PH andthe abnormal distribution of blood flow in the pulmonary vascular bed, include hemoptysis related to rupture of hypertrophied bronchial arteries. Hemoptysis is a serious complication that is rarely reported in patients with pulmonary arterial hypertension (PAH). Hemoptysis severity ranges from mild to very severe leading tosudden death. Hemoptysis are reported to be a terminal stage ofa complication due to PAH with prevalence is variable, from 1% to 6%. Although the incidence is quite rare, the presence of recurrent hemoptysis in patients with pulmonary hypertension is a sign of poor prognosis. Bronchial artery embolization is suggested as an acute emergency procedure in the case of severe hemoptysis or as elective intervention in cases of frequent mild or moderate episodes. 

scholarly journals Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Verônica Silva Vilela ◽  
Marcio Macri Dias ◽  
Ângelo Antunes Salgado ◽  
Bruno Rangel Antunes da Silva ◽  
Agnaldo José Lopes ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Right Heart Catheterization ◽  
Diagnostic Tools ◽  
Heart Catheterization ◽  
Systematic Screening ◽  
Mean Pulmonary Arterial Pressure ◽  
Common Group ◽  
Pulmonary Arterial

Abstract Background Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population. Methods A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated. Results Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20– < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively. Conclusions Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. Trial registration: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.

scholarly journals The Right Ventricular Diameter can Predict the Presence of Pulmonary Hypertension

2016 ◽  
Vol 30 (2) ◽  
pp. 48-52 ◽  
Author(s):  
Abrar Kaiser ◽  
Fazilatunnessa Malik ◽  
Tuhin Haque ◽  
Iftekhar Alam ◽  
Abdullah Al Masud ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Non Invasive ◽  
Pulmonary Arterial

Background: Pulmonary arterial hypertension (PAH) is a severe disease characterized by a progressive increase of pulmonary pressure and resistance leading to right heart failure. Pulmonary arterial hypertension is commonly diagnosed at a late stage of the disease and is associated with progressive clinical deterioration and premature death. The assessment of pulmonary artery pressure is important in clinical management and prognostic evaluation of patients with cardiovascular and pulmonary disease. Although PH can be detected invasively by right ventricular (RV) catheterization, accurate non-invasive assessment by echocardiography has many advantages. Reliable non-invasive evaluation of pulmonary pressure at present is still a problem as echocardiographic measurement of pulmonary hypertension relies on the presence of tricuspid regurgitation (TR). Objective: The purpose of this study was to determine whether right ventricular end diastolic diameter can predict the presence of pulmonary hypertension. Methods: Eighty consecutive patients with echo detectable tricuspid regurgitation who underwent right heart catheterization for either diagnostic or therapeutic procedure were recruited. They were divided into two groups on the basis of pulmonary artery systolic pressure (PASP). Group I consists of 40 patients with PASP >35 mm Hg and Group II 40 patients having PASP d• 35 mm Hg. Right ventricular end-diastolic diameter (RVD) was measured in the apical 4 chamber view. PASP was measured from right heart catheterization. Results: The RVD has strong correlation with catheter-derived PASP, at a cutoff value of >3 cm, predicted the presence of PAH with 78% sensitivity and 71% specificity. Conclusion: RVD is a good non-invasive predictor for PAH. RVD can predict the presence of PAH even in absence of TR and correlates well with PASP measured by RV catheterization.Bangladesh Heart Journal 2015; 30(2) : 48-52

scholarly journals Experience with exercise right heart catheterization in the diagnosis of pulmonary hypertension: a retrospective study

2014 ◽  
Vol 9 ◽  
Author(s):  
Stephan Keusch ◽  
Anina Bucher ◽  
Séverine Müller-Mottet ◽  
Elisabeth Hasler ◽  
Marco Maggiorini ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Life Experience ◽  
Real Life ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Hemodynamics ◽  
Mean Pulmonary Arterial Pressure ◽  
Exercise Hemodynamics ◽  
Pulmonary Arterial

Background: Data on exercise pulmonary hemodynamics in healthy people and patients with pulmonary hypertension (PH) are rare. We analyzed exercise right heart catheterization (RHC) data in a symptomatic collective referred with suspected PH to characterize the differential response by diagnostic groups, to correlate resting with exercise hemodynamics, and to evaluate safety. Methods: This is a retrospective single-center study reviewing data from patients in whom an exercise RHC was performed between January 2006 and January 2013. Patients with follow-up RHC under PH -therapy were excluded. Results: Data from 101 patients were analyzed, none of them had an adverse event. In 35% we detected a resting PH (27.8% precapillary, 6.9% postcapillary). Exercise PH (mean pulmonary arterial pressure (mPAP) >30 mmHg at exercise) was found in 38.6%, whereas in 25.7% PH was excluded. We found a remarkable number of exercise PH in scleroderma patients, the majority being postcapillary. 83% of patients with mPAP-values between 20 and 24.9 mmHg at rest had exercise PH. Patients with resting PH had worse hemodynamics and were older compared with exercise PH ones. Conclusion: In this real-life experience in symptomatic patients undergoing exercise RHC for suspected PH, we found that exercise RHC is safe. The facts that the vast majority of patients with mPAP-values between 20 and 24.9 mmHg at rest had exercise PH and the older age of patients with resting PH may indicate that exercise PH is a precursor of resting PH. Whether earlier treatment start in patients with exercise PH would stabilize the disease should be addressed in future studies.

Pathology and Pathobiology of Pulmonary Hypertension

2017 ◽  
Vol 38 (05) ◽  
pp. 571-584 ◽  
Author(s):  
Peter Dorfmüller ◽  
Christophe Guignabert
Keyword(s):  
Pulmonary Hypertension ◽  
Vascular Inflammation ◽  
Right Heart Catheterization ◽  
Point Of View ◽  
Heart Catheterization ◽  
Pulmonary Endothelium ◽  
Hemodynamic State ◽  
Pulmonary Arterial ◽  
Genetic And Environmental Factors ◽  
And Migration

Pulmonary hypertension (PH) is a hemodynamic state defined by a mean pulmonary artery pressure ≥ 25 mm Hg during resting right heart catheterization. PH can result from precapillary (arterial) or postcapillary (venous) pathophysiological mechanisms. Interestingly, recent PH pathology has shown that pulmonary arterial or pulmonary venous remodelling are rarely independent phenomena, but frequently occur in combined fashion in lungs from patients suffering from different forms of PH, including pulmonary arterial hypertension (PAH). In PAH, it is now becoming clear that aberrant signals present in vessel wall microenvironment, which is largely orchestrated by dysfunctional pulmonary endothelial cells, are key contributors of the pulmonary vascular remodeling process, fostering proliferation, and survival and migration of resident pulmonary vascular cells such as smooth muscle cells, myofibroblasts, and pericytes. In addition, both genetic and environmental factors are also critical in the development of pulmonary vascular inflammation and chronic impairment of the pulmonary endothelium. This article outlines the current understanding of this disease from the point of view of pathology and pathobiology.

scholarly journals Right Heart Size and Right Ventricular Reserve in Pulmonary Hypertension: Impact on Management and Prognosis

Diagnostics ◽  
2020 ◽  
Vol 10 (12) ◽  
pp. 1110
Author(s):  
Ekkehard Grünig ◽  
Christina A. Eichstaedt ◽  
Rebekka Seeger ◽  
Nicola Benjamin
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Ventricular Function ◽  
Right Ventricular Function ◽  
Right Heart Failure ◽  
Right Heart Catheterization ◽  
Contractile Reserve ◽  
Heart Catheterization ◽  
Right Heart ◽  
Heart Size

Various parameters reflecting right heart size, right ventricular function and capacitance have been shown to be prognostically important in patients with pulmonary hypertension (PH). In the advanced disease, patients suffer from right heart failure, which is a main reason for an impaired prognosis. Right heart size has shown to be associated with right ventricular function and reserve and is correlated with prognosis in patients with PH. Right ventricular reserve, defined as the ability of the ventricle to adjust to exercise or pharmacologic stress, is expressed by various parameters, which may be determined invasively by right heart catheterization or by stress-Doppler-echocardiography as a noninvasive approach. As the term “right ventricular contractile reserve” may be misleading, “right ventricular output reserve” seems desirable as a preferred term of increase in cardiac output during exercise. Both right heart size and right ventricular reserve have been shown to be of prognostic importance and may therefore be useful for risk assessment in patients with pulmonary hypertension. In this article we aim to display different aspects of right heart size and right ventricular reserve and their prognostic role in PH.

scholarly journals Right heart catheterization for pulmonary hypertension during the coronavirus disease 2019 pandemic

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402094878 ◽  
Author(s):  
Kanza N. Qaiser ◽  
James E Lane ◽  
Adriano R. Tonelli
Keyword(s):  
Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Clinical Practices ◽  
Right Heart ◽  
Diagnostic Modality ◽  
Hypertension Therapy ◽  
Pulmonary Arterial ◽  
Pulmonary Arterial Hypertension Therapy

Right heart catheterization is an essential diagnostic modality in the evaluation of pulmonary hypertension. The coronavirus disease 2019 pandemic has resulted in deferral of elective procedures including right heart catheterization. The benefits of proceeding with right heart catheterization, such as further characterization of hemodynamic subtype and severity of pulmonary hypertension, initiation of targeted pulmonary arterial hypertension therapy, as well as further hemodynamic testing, need to be carefully balanced with the risk of potentially exposing both patients and health care personnel to coronavirus disease 2019 infection. This review article aims to provide best clinical practices for safely performing right heart catheterization in pulmonary hypertension patients during the coronavirus disease 2019 pandemic.

scholarly journals Right ventricle performances with echocardiography and 99mTc myocardial perfusion imaging in pulmonary arterial hypertension patients

2018 ◽  
Vol 243 (9) ◽  
pp. 754-761
Author(s):  
Jie Liu ◽  
Lei Fei ◽  
Guang-Qing Huang ◽  
Xiao-Ke Shang ◽  
Mei Liu ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Myocardial Perfusion ◽  
Arterial Pressure ◽  
Right Ventricle ◽  
Arterial Hypertension ◽  
Pulmonary Arterial Pressure ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial

Right heart catheterization is commonly used to measure right ventricle hemodynamic parameters and is the gold standard for pulmonary arterial hypertension diagnosis; however, it is not suitable for patients’ long-term follow-up. Non-invasive echocardiography and nuclear medicine have been applied to measure right ventricle anatomy and function, but the guidelines for the usefulness of clinical parameters remain to be established. The goal of this study is to identify reliable clinical parameters of right ventricle function in pulmonary arterial hypertension patients and analyze the relationship of these clinical parameters with the disease severity of pulmonary arterial hypertension. In this study, 23 normal subjects and 23 pulmonary arterial hypertension patients were recruited from January 2015 to March 2016. Pulmonary arterial hypertension patients were classified into moderate and severe pulmonary arterial hypertension groups according to their mean pulmonary arterial pressure levels. All the subjects were subjected to physical examination, chest X-ray, 12-lead electrocardiogram, right heart catheterization, two-dimensional echocardiography, and technetium 99m (99mTc) myocardial perfusion imaging. Compared to normal subjects, the right heart catheterization indexes including right ventricle systolic pressure, right ventricle end diastolic pressure, pulmonary artery systolic pressure, pulmonary artery diastolic pressure, pulmonary vascular resistance, and right ventricle end systolic pressure increased in pulmonary arterial hypertension patients and were correlated with mean pulmonary arterial pressure levels. Echocardiography parameters, including tricuspid regurgitation peak velocity, tricuspid regurgitation pressure gradient, tricuspid annular plane systolic excursion and fractional area, right ventricle-myocardial performance index, were significantly associated with the mean pulmonary arterial pressure levels in pulmonary arterial hypertension patients. Furthermore, myocardial perfusion imaging was not observed in the normal subjects but in pulmonary arterial hypertension patients, especially severe pulmonary arterial hypertension subgroup, and showed potential diagnostic properties for pulmonary arterial hypertension. In conclusion, mean pulmonary arterial pressure levels are correlated with several right heart catheterization and echocardiography markers in pulmonary arterial hypertension patients; echocardiography and 99mTc myocardial perfusion can be used to evaluate right ventricle performance in pulmonary arterial hypertension patients. Impact statement In this study, we analyzed the clinical parameters for evaluating RV function, including right ventricle catheterization (RHC), echocardiography, and technetium 99m (99mTc) myocardial perfusion imaging (MPI) in normal Asian subjects and PAH patients ( n = 23 for each group). Our results demonstrated that six RHC indexes, four echocardiography indexes and MPI index were significantly altered in PAH patients and correlated with the levels of mean pulmonary arterial pressure. Importantly, we evaluated the diagnostic performance of MPI and found that MPI has a strong diagnostic accuracy in PAH patients. The findings from this study will be of interest to clinical investigators who make diagnosis and therapeutic strategies for PAH patients.

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