scholarly journals Pulmonary hypertension: the importance of correctly diagnosing the cause

2016 ◽  
Vol 25 (142) ◽  
pp. 372-380 ◽  
Author(s):  
Sanjay Mehta ◽  
Jean-Luc Vachiéry

Pulmonary hypertension (PH) is a complex condition that can occur as a result of a wide range of disorders, including left heart disease, lung disease and chronic pulmonary thromboembolism. Contemporary PH patients are older and frequently have a multitude of comorbidities that may contribute to or simply coincide with their PH. Identifying the cause of PH in these complicated patients can be challenging but is essential, given that the aetiology of the disease has a significant impact on the management options available. In this article, we present two cases that highlight the difficulties involved in obtaining a precise diagnosis of the cause of PH within the setting of multiple comorbidities. The importance of performing a comprehensive, multidimensional diagnostic work-up is demonstrated, in addition to the need to specifically consider cardiopulmonary haemodynamic data in the context of the wider clinical picture. The article also illustrates why achieving an accurate diagnosis is necessary for optimal patient management. This may involve treatment of comorbidities as a priority, which can ameliorate the severity of PH, obviating the need to consider PH-targeted medical treatment.

2018 ◽  
Vol 4 (1) ◽  
pp. 13-22
Author(s):  
V. Sachpekidis ◽  
H. Karvounis ◽  
G. Giannakoulas

2015 ◽  
Vol 309 (9) ◽  
pp. L924-L941 ◽  
Author(s):  
Siegfried Breitling ◽  
Krishnan Ravindran ◽  
Neil M. Goldenberg ◽  
Wolfgang M. Kuebler

Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure leading to right-sided heart failure and can arise from a wide range of etiologies. The most common cause of PH, termed Group 2 PH, is left-sided heart failure and is commonly known as pulmonary hypertension with left heart disease (PH-LHD). Importantly, while sharing many clinical features with pulmonary arterial hypertension (PAH), PH-LHD differs significantly at the cellular and physiological levels. These fundamental pathophysiological differences largely account for the poor response to PAH therapies experienced by PH-LHD patients. The relatively high prevalence of this disease, coupled with its unique features compared with PAH, signal the importance of an in-depth understanding of the mechanistic details of PH-LHD. The present review will focus on the current state of knowledge regarding the pathomechanisms of PH-LHD, highlighting work carried out both in human trials and in preclinical animal models. Adaptive processes at the alveolocapillary barrier and in the pulmonary circulation, including alterations in alveolar fluid transport, endothelial junctional integrity, and vasoactive mediator secretion will be discussed in detail, highlighting the aspects that impact the response to, and development of, novel therapeutics.


Author(s):  
Antonino Saccà ◽  
Andrea Salonia

Erectile dysfunction (ED) has progressively emerged as an important sentinel marker of cardiovascular and overall health among men. A timely and accurate diagnosis of ED may thus represent a significant opportunity not only to diagnose the dysfunction per se, but also to comprehensively identify co-morbid and potentially life-threatening conditions. Basic work-up for a man seeking help for ED should start considering that ED may share several modifiable and unmodifiable common risk factors with cardiovascular disorders and other potential life-threatening conditions. Overall, most patients with ED can be adequately managed with a basic diagnostic work-up; this includes a comprehensive medical and sexual history, along with a physical examination and some laboratory tests. Conversely, only some selected patients may also need specific diagnostic tests.


PEDIATRICS ◽  
1973 ◽  
Vol 52 (1) ◽  
pp. 29-37
Author(s):  
Magda Campbell ◽  
Harry Breuer ◽  
Sandra R. Wolman

A 3-year-old male with 47XXY karyotype is presented as an example of how various genetic and environmental (including psychosocial) factors can contribute to delayed and/or disturbed behavior. The young preschool child's behavioral repertoire is so small, undifferentiated, and nonspecific that a variety of causes can evoke the same symptom(s) and behavior, and the danger of misdiagnosis, therefore, is great. Such disturbances may not be entirely or partially caused by a disturbed parent-child relationship. The pediatrician is usually the first to see such a young patient and in a position to initiate appropriate referrals for a complete diagnostic work-up, including cytological, hearing, and speech studies, not only for accurate diagnosis, but also for prognosis, treatment, and parental counseling.


2017 ◽  
Vol 49 (6) ◽  
pp. 1602409 ◽  
Author(s):  
Karen M. Olsson ◽  
Christoph B. Wiedenroth ◽  
Jan-Christopher Kamp ◽  
Andreas Breithecker ◽  
Jan Fuge ◽  
...  

Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH).We report on a prospective series of 56 consecutive patients who underwent 266 BPA interventions (median, five per patient) at two German institutions. All patients underwent a comprehensive diagnostic work-up including right heart catheterisation at baseline and 24 weeks after their last intervention.BPA resulted in improvements in WHO functional class, 6 min walk distance (mean change, +33 m), right ventricular function and haemodynamics, including a decline in mean pulmonary artery pressure by 18% and in pulmonary vascular resistance by 26%. Procedure-related adverse events occurred in 9.4% of the interventions. The most common complications were related to pulmonary vascular injury and consecutive pulmonary bleeding. Most of these events were asymptomatic and self-limiting, but one patient died from pulmonary bleeding, resulting in a mortality rate of 1.8%.BPA resulted in haemodynamic and clinical improvements but was also associated with a considerable number of complications, including one fatal pulmonary bleeding. As the effects of BPA on survival are unknown, randomised controlled outcome trials comparing BPA with approved medical therapies in patients with inoperable CTEPH are required to allow for appropriate risk–benefit assessments.


2000 ◽  
Vol 4 (4) ◽  
pp. 205-207 ◽  
Author(s):  
Diya F. Mutasim ◽  
Brian B. Adams

Background: A thorough work-up is paramount in the accurate diagnosis of oral erosive diseases. Objective: A case of pemphigoid with negative direct immunofluorescence but positive indirect immunofluorescence is presented. Methods and Results: A 77-year-old man presented with erosions of the oral and glans mucosa and with esophageal stenosis. Histologic examination revealed nonspecific mucositis. Indirect immunofluorescence was characteristic of pemphigoid. Conclusion: This case illustrates the importance of performing a complete diagnostic work-up in the evaluation of patients with oral erosive disease.


2020 ◽  
Vol 30 (6) ◽  
Author(s):  
Henok Tadele ◽  
Etsegenet Gedlu

BACKGROUND፡ Unilateral pulmonary artery absence or discontinuity is a rare congenital abnormality. Its reported prevalence is 1 in 150,000 adults.CASE PRESENTATION: A 22-month-toddler girl was referred from a general hospital for cardiac follow-up to our hospital after diagnosis of severe pulmonary hypertension was made. She presented with generalized body swelling, cough, fast breathing and high-grade fever of four days duration. She had repeated severe chest infections. Echocardiography and chest computed tomography revealed right pulmonary artery discontinuity. Acute care was given and chronic care was established.CONCLUSION:Pulmonary artery discontinuity is a rare anomaly. It should be considered in any child presenting with recurrent chest infections/pulmonary hypertension. Appropriate and timely diagnostic work up will avoid misdiagnosis.


2020 ◽  
Vol 19 (4) ◽  
pp. 33-40
Author(s):  
A. V. Sudarkina ◽  
A. P. Dergilev ◽  
N. A. Gorbunov ◽  
V. V. Kozlov ◽  
Yu. A. Fokina ◽  
...  

Introduction. Mediastinal lymphadenopathy can be caused by a wide range of benign and malignant states. Determination of the genesis of lymphadenopathy is crucial for treatment planning and prognosis of the disease.The purpose of the study was to evaluate the diagnostic accuracy of diffusion weighted imaging (DWI) with apparent diffusion coefficient (ADC) measurements in differentiating malignant versus benign mediastinal lymphadenopathy. Material and Methods. 48 consecutive patients with at least one enlarged mediastinal lymph node revealed on CT-scans were examined on 1,5 T MR-machine with conventional images and respiratory-triggered DWI. In all patients one of the biggest solid lymph nodes was selected for ADC measurements and mean ADCs of each node were recorded. ADCs were correlated with the results of complete diagnostic work-up (including histopathological diagnosis in 41 patients) and follow-up CT. Statistics included Student’s t-test, Mann-Whitney U-test and ROC-curve analysis. Results. 27 lymph nodes were classified as malignant (metastases, lymphoma) and 21 lymph nodes were classified as benign (sarcoidosis, reactive hyperplasia, tuberculosis). Mean ADC of malignant lymph nodes (1,02 ± 0,29×10−3 mm2/s) was significantly lower than that of benign lymph nodes (1,57 ± 0,32×10−3 mm2/s), p<0,0001. The cut-off value of ≤1,3×10−3mm2/s for ADC indicated the malignancy with a sensitivity of 81,5 % and a specificity of 85,7%. The area under the ROC-curve was 0,89 (95 % confidence interval: 0,77, 0,96), p<0,0001. Conclusion. DWI is a promising technique in chest pathology. DWI with ADC measurements could be used as a good complementary tool in the diagnostic work-up of patients with mediastinal lymphadenopathy.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Tanyeri ◽  
O Y Akbal ◽  
B Keskin ◽  
A Hakgor ◽  
A Karagoz ◽  
...  

Abstract Background and aim In this study we evaluated the predictive value of currently available European Society of Cardiology 2015 Pulmonary Hypertension (PH) Guidelines two-step echocardiographic (Echo) work-up algorithm for two PH definitions as invasively evaluated pulmonary arterial mean pressures (PAMP) ≥25 mmHg and >20 mmHg. Methods Study group comprised the retrospectively evaluated 1299 patients (pts) (53.1±18.8 years, female 807, 62.1%) who underwent right heart catheterisation (RHC) with different indications between 2006 and 2018. Echo data obtained from same day and pre-RHC evaluation were available in all pts. Chemla method was used for Doppler estimation of PAMP from tricuspid regurgitant jet. For two PH definitions as PAMP ≥25 mmHg (definition-A) and PAMP >20 mmHg (definition-B) predictive value of the ESC Echo algorithm was tested with two logistic regression models based on only PAMP-Echo (Model-1) and PAMP-Echo and Echo findings supportive for PH (Model-2). Results RHC revealed that criteria for PH definitions A and B were met in 891 (68.6%) and 1051 (80.9%) of overall pts. Pre-RHC Echo findings supportive for PH were noted in 529 (40.7%) of pts. Because pulmonary regurgitant velocity data were imprecise, other 6 supportive Echo findings were utilised. The % of the supportive Echo measures were as follows; 1 (11%), 2 (10%),3 (8.1%), 4 (7.2%), 5 (2.5%) and 6 (1.8%). In Model-1, for definitions A and B, odds ratio (OR) of PAMP-Echo were (1.07; 0.99–1.19, p=0.058, LRX2:139, c: 0.72) and (1.05; 0.98–1.12, p=0.102, LRX2:129, c: 0.71), respectively. In Model-2, for definition-A, OR of PAMP-Echo and number of supportive findings were (1.05; 0.98–1.12, p=0.162) and (1.58; 1.42–1.75, p<0.001) (LRX2:217, c: 0.77), respectively. Using the same model for definition-B, OR of PAMP-Echo and number of supportive findings were (1.04; 0.97–1.11, p=0.264) and (1.56; 1.40–1.73, p<0.001),(LRX2:203, c: 0.76), respectively. Receiver operating curve (ROC) analysis yielded that PAMP-Echo >39.8 mmHg had a sensitivity of 80% and a specificity of 54% for definition-A, and a sensitivity of 81% and a specificity of 52% for definition-B, respectively. Regardless of the definition tresholds for PH on RHC, number of supportive Echo findings were responsible for nearly 70% of the overall variance in the PH diagnosis. Conclusions Irrespective of the RHC definition criteria as PAMP ≥25 mmHg or >20 mmHg, number of supportive Echo findings but not PAMP-Echo only approach seems to be reliable in the diagnostic work-up for PH.


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