Suspicion Of Pulmonary Hypertension Research Articles

Hemodynamical Approach to Pulmonary Hypertension

Pulmonary hypertension (PH) is a pathophysiological disorder characterized by a vascular obstruction leading to increased right ventricular overload. It can be caused by multiple conditions that we classify into 5 clinical groups. PH is defined hemodynamically and the role and indications of cardiac catheterization are increasingly important. The subject includes a panoply of pathologies with great complexity at the same time physio-pathological, histo-etiopathogenic, genetic... which are gathered under one chapter: the pulmonary vascular pathology. Right heart catheterization (RHC) is crucial and allows to measure and calculate variables to determine patients hemodynamics that are necessary for positive, etiological and severity diagnosis. It can also have therapeutic purpose: vasoreactivity test (APVT), pre-gesture assessment. Materiel and Methods: retrospective cross-sectional study of 143 patients with suspicion of pulmonary hypertension whom underwent RHC (left heart) performed between January 2017 and December 2021 in cardiology A department within Ibn Sina university hospital center. Results: In terms of the results of our study, we have a very diverse review in which PH is assessed at different levels for all groups and subgroups using a multitude of hemodynamic parameters. Our population is characterized by a clear predominance of women The average age of all 143 cases is 38 years. In terms of hemodynamic classification, isolated pre-capillary PH is the most common (52%). 64% of our cases had PVRs >2 Wu indicating the prevalence of pulmonary vasculopathy. Group 3 represents the population with the most preserved hemodynamical severity parameters. The main vasoreactivity test performed was the NO test.

Doppler trans-thoracic echocardiography for detection of pulmonary hypertension in adults.

Doppler trans-thoracic echocardiography for detection of pulmonary hypertension in adults.

Echocardiographic Predictors of Hospital Outcomes in Preterm Neonates with Patent Ductus Arteriosus and Clinical Suspicion of Pulmonary Hypertension

Echocardiographic Predictors of Hospital Outcomes in Preterm Neonates with Patent Ductus Arteriosus and Clinical Suspicion of Pulmonary Hypertension

The haemodynamic assessment of patients with pulmonary arterial hypertension.

[No abstract. Showing first paragraph of article]Right heart catheterization (RHC) has a crucial role in the management of patients with pulmonary hypertension (PH). Firstly, it is mandatory in subjects with the suspicion of PH to confirm the diagnosis. Secondly, it is strongly recommended in the follow-up of patients with confirmed pulmonary arterial hypertension (PAH) to evaluate drug efficacy and in case of clinical deterioration.In order to always perform a complete and correct RHC, clinicians carrying out the examination must be guided not only by technical recommendations, but also by the accurate knowledge of the different clinical issues which have to be addressed in these different situations.

The impact of the new World Symposium on Pulmonary Hypertension definition of pulmonary hypertension on the prevalence of pre-capillary pulmonary hypertension.

Since the first World Symposium on Pulmonary Hypertension (WSPH; Geneva, 1973), pulmonary hypertension (PH) has been defined as a mean pulmonary artery pressure (mPAP) ≥25 mm Hg measured at right heart catheterization (RHC) while at rest in the supine position. At the 6th WSPH congress (Nice, 2018), a new proposal was presented defining pre-capillary PH as mPAP >20 mm Hg, with pulmonary arterial wedge pressure (PAWP) <15 mm Hg, and pulmonary vascular resistance (PVR) >3 WU. The aim of this study was to investigate the impact of the new definition of PH on the number of pre-capillary PH patients. The results of RHC performed with various clinical indications between 2017 and 2018 were analyzed. The 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) and the 6th WSPH congress PH definitions were used to identify PH patients. Fifty-eight RHC procedures were performed in our hospital in a 1-year period. Most were performed with a suspicion of PH (n=52). The remainder (n=6) were performed with indications of valvular heart disease or left heart disease. There were 40 females (69%) and 18 males (31%). The mean age was 53.3±16.6 years. The RHC results revealed a mean PAP of 36.4±16.4 mm Hg, PAWP of 12.6±3.9 mm Hg, and PVR of 4.9±4.4 WU. Forty-three of 58 patients (74.1%) were classified as pre-capillary PH according to the ESC/ERS PH guideline, whereas 50 of 58 patients (86.2%) had pre-capillary PH according to the new WSPH definition. The results of this study indicated that the impact of the new definition of PH on the number of pre-capillary PH patients identified was greater than the predicted <10%.

Effect of pulmonary arteriovenous malformations on the mechanical properties of the lungs

BackgroundPulmonary arteriovenous malformations (PAVMs) are present in approximately 15–50% individuals with hereditary hemorrhagic telangiectasia (HHT). They may be isolated but more often are multiple. The goal of this study was to evaluate the influence of PAVMs on lung mechanical properties.MethodsWe reviewed the files of all adult patients (age ≥ 18 years) referred to our Center for evaluation of HHT between 2005 and 2013. The diagnosis of HHT was based on the Curacao criteria and/or the presence of a pathogenic mutation. Exclusion criteria included: chronic cardiac or lung disease (i.e. asthma or COPD), suspicion of pulmonary hypertension on echocardiography, current or past smoking (>10 pack-years), history of thoracic surgery, previous treatment of PAVMs by embolotherapy, lung infection or thromboembolic disease in the past 3 months, pregnancy and obesity (BMI > 30 kg/m2). Chest high resolution CT-scan and pulmonary function tests were performed the same day in all patients as part of our routine work-up.ResultsOne hundred and fifty five patients with HHT were included (age: 44.4 ± 16.7 yrs – mean ± SD -; males: 39%). Eighty eight patients had no PAVM, 45 had 1–3 PAVMS and 22 had at least 4 PAVMs. Thirty eight patients had unilateral PAVMs and 29 bilateral PAVMs. We found no statistical relationship between the number, the size and the laterality of PAVMs and results of lung flows and volumes.ConclusionWe found no evidence that PAVMs have a significant influence on lung mechanical properties as measured using routine pulmonary function tests in adult patients with HHT, even in case of numerous, macroscopic or bilateral malformations.

Right heart catheterization procedures in patients with suspicion of pulmonary hypertension - experiences of a tertiary center.

IntroductionRight heart catheterization (RHC) is an invasive procedure providing direct and accurate measurements of hemodynamics of the cardiovascular system. Acute pulmonary vasoreactivity testing (APVT) following basal RHC in some patients is an established tool evaluating the reversibility of hypertension in the pulmonary vasculature.AimWe sought to assess the most common indications, vascular approaches and complications during RHC in a single high-volume center.Material and methodsA total of 534 RHC procedures in 348 patients (64% male) were performed. The prospective registry was carried out for 28 months. Collected data included indications for RHC, vascular approaches, hemodynamic and clinical data, complications and response of pulmonary vessels in APVT.ResultsIn 401 (75%) procedures pulmonary hypertension (mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg) was confirmed. Left heart failure was the most common indication (55.8%), mainly ischemic (26%) or dilated cardiomyopathy (19.9%). Other indications included a suspicion of arterial (21.7%), or chronic thromboembolic pulmonary hypertension (14.6%). The right internal jugular vein approach was used in 89.1% of procedures. Acute pulmonary vasoreactivity testing was performed in 143 patients, and it was positive in 67 (46.9%) cases. Complications occurred in 21 (3.9%) procedures and included pulmonary edema (0.2%), pneumothorax (0.2%) and puncture of the artery followed by the insertion of a vascular sheath (0.4%), atrial arrhythmia (0.2%), superior vena cava dissection (0.2%), incidental artery puncture (1.1%) and local hematoma (2.2%).ConclusionsThe most frequent indication for RHC was left heart failure, and the most common approach was the right internal jugular vein. RHC is safe procedure with a low rate of major complications.

The long-term response to treatment with calcium channel blockers in a patient with idiopathic pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a disease characterised by a gradual increase in resistance of pulmonary circulation leading to right ventricular failure and death. In only 10 % of cases, there is a response to acute vasoreactivity testing with a significant reduction in mean pulmonary artery pressure (PAP), while in this group of patients, less than one half of cases benefit from long-term treatment with calcium channel blockers (CCB). This paper describes a case report of a young patient with dyspnoea and suspicion of pulmonary hypertension who was referred to a specialised centre. The complex evaluation of her clinical state led to confirmed diagnosis of idiopathic pulmonary arterial hypertension (IPAH). Because there was a positive response to vasoreactivity testing, the treatment for IPAH was initiated with a high dose of CCB. This treatment markedly improved her clinical state as well as echocardiographic and hemodynamic findings.In this study, the authors present a diagnostic algorithm in pulmonary hypertension and emphasise the role of CCB in treatment of PAH in carefully selected patients (Tab. 1, Fig. 5, Ref. 7).

Hemoglobin sickle cell disease complications: a clinical study of 179 cases

Hemoglobin sickle cell disease is one of the most frequent hemoglobinopathies. Surprisingly, few studies have been dedicated to this disease, currently considered to be a mild variant of homozygous sickle cell disease. The aim of this study was to update our knowledge about hemoglobin sickle cell disease. The study involved a single center series of 179 patients. Clinical and biological data were collected with special attention to the assessment of pulmonary arterial hypertension and nephropathy. Hemoglobin sickle cell diagnosis was delayed and performed in adulthood in 29% of cases. Prevalence of hospitalized painful vasoocclusive crisis, acute chest syndrome and priapism was 36%, 20% and 20%, respectively. The most common chronic organ complications were retinopathy and sensorineural otological disorders in 70% and 29% of cases. Indeed, prevalence of complications reported in homozygous sickle cell disease, such as nephropathy, suspicion of pulmonary hypertension, strokes and leg ulcers was rather low (13%, 4% and 1%, respectively). Phlebotomy performed in 36% of this population (baseline hemoglobin 11.5 g/dL) prevented recurrence of acute events in 71% of cases. Our data suggest that hemoglobin sickle cell disease should not be considered as a mild form of sickle cell anemia but as a separate disease with a special emphasis on viscosity-associated otological and ophthalmological disorders, and with a low prevalence of vasculopathy (strokes, pulmonary hypertension, ulcers and nephropathy). Phlebotomy was useful in reducing acute events and a wider use of this procedure should be further investigated.

Pulmonary Hypertension in Patients with Chronic Obstructive Pulmonary Disease

Pulmonary hypertension (PH) is an important complication in the natural history of chronic obstructive pulmonary disease (COPD). Its presence is associated with reduced survival and greater use of healthcare resources. The prevalence of PH is high in patients with advanced COPD, whereas in milder forms it might not be present at rest but may develop during exercise. In COPD, PH is usually of moderate severity and progresses slowly, without altering right ventricular function in the majority of patients. Nevertheless, a small subgroup of patients (1-3%) may present with out-of-proportion PH, that is, with pulmonary arterial pressure largely exceeding the severity of airway impairment. These patients depict a clinical picture similar to more severe forms of PH and have higher mortality rates. PH in COPD is caused by the remodelling of pulmonary arteries, which is characterized by the intimal proliferation of poorly differentiated smooth muscle cells and the deposition of elastic and collagen fibres. The sequence of changes that lead to PH in COPD begins at early disease stages by the impairment of endothelial function, which is associated with impaired release of endothelium-derived vasodilating agents (nitric oxide, prostacyclin) and increased expression of growth factors. Products contained in cigarette smoke play a critical role in the initiation of pulmonary endothelial cell alterations. Recognition of PH can be difficult because symptoms due to PH are not easy to differentiate from the clinical picture of COPD. Suspicion of PH should be high if clinical deterioration is not matched by the decline in pulmonary function, and in the presence of profound hypoxaemia or markedly reduced carbon monoxide diffusing capacity. Patients with suspected PH should be evaluated by Doppler echocardiography and, if confirmed, undergo right-heart catheterization in those circumstances where the result of the procedure can determine clinical management. To date, long-term oxygen therapy is the treatment of choice in COPD patients with PH and hypoxaemia because it slows or reverses its progression. Conventional vasodilators are not recommended because of their potential detrimental effects on gas exchange, produced by the inhibition of hypoxic pulmonary vasoconstriction and their lack of effectiveness after long-term treatment. In the subgroup of patients with out-of-proportion PH, new specific therapy available for pulmonary arterial hypertension (PAH) [prostanoids, endothelin-1 receptor antagonists and phosphodiesterase-5 inhibitors] may be considered in the setting of clinical trials. The use of specific PAH therapy in COPD patients with moderate PH is discouraged because of the potential detrimental effect of some of these drugs on gas exchange and there are no data demonstrating their efficacy.

Use of enzyme replacement therapy for Gaucher disease during pregnancy

OBJECTIVE: To date there has been little published experience with enzyme replacement therapy in pregnant women with symptomatic type I Gaucher disease. STUDY DESIGN: We describe six patients, including three with repeated early pregnancy loss, five of whom successfully carried pregnancies to term; the last pregnancy was terminated because of pulmonary hypertension. RESULTS: All pregnancies were uneventful and five resulted in healthy newborns. CONCLUSION: We concluded that in patients with Gaucher disease of childbearing age,for whom obstetric complications are an important symptom of the disease, pregnancy is not contraindicated (unless there is evidence or suspicion of pulmonary hypertension) and treatment should not be interrupted because the clinical improvement engendered by enzyme replacement therapy is conducive to fewer complications during pregnancy and delivery and post partum.(Am J Obstet Gynecol 1997;177:12)