Potential Contribution of Pulmonary Thromboembolic Disease in Pulmonary Hypertension in Sickle Cell Disease.
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ABSTRACT:
SUBMITTER: Nouraie M
PROVIDER: S-EPMC7328185 | biostudies-literature | 2020 Jul
REPOSITORIES: biostudies-literature
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Potential Contribution of Pulmonary Thromboembolic Disease in Pulmonary Hypertension in Sickle Cell Disease.
Nouraie Mehdi M Zhang Xu X Srisuwananukorn Andrew A Machado Roberto F RF Gordeuk Victor R VR Gladwin Mark T MT Saraf Santosh S
Annals of the American Thoracic Society 20200701 7
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Project description: Not available
| S-EPMC4715583 | biostudies-literature
Project description:Balloon pulmonary angioplasty (BPA) is beneficial for patients with chronic thromboembolic pulmonary disease (CTEPD) with pulmonary hypertension (PH). However, the clinical benefit of BPA for the patients with CTEPD without PH remains unknown. In this study, we aimed to evaluate the efficacy, safety, and long-term outcomes of BPA in patients with CTEPD without PH. We retrospectively analyzed the data from 84 CTEPD patients with mean pulmonary artery pressure (mPAP) < 25 mmHg and 39 CTEPD patients with mPAP ≤ 20 mmHg (without PH). Among the 39 patients with CTEPD without PH, 14 underwent BPA (BPA-treated group), and the remaining 25 received no treatment (untreated group). In the patients with CTEPD without PH, BPA led to improvements in symptoms, pulmonary vascular resistance (3.6 ± 1.6 to 2.6 ± 1.1 Wood units, p < 0.001), peak oxygen consumption (16.1 ± 4.0 to 18.8 ± 4.3 mL/kg/min, p = 0.033), minute ventilation versus carbon dioxide production slope (41.4 ± 12.2 to 35.1 ± 6.7, p = 0.026), and mPAP/cardiac output slope (7.0 ± 2.6 to 4.4 ± 2.0 mmHg/L/min, p = 0.004) and facilitated the discontinuation of home oxygenation therapy, with no serious complications. Kaplan-Meier analysis showed no significant difference in all-cause mortality between the untreated and BPA-treated groups. BPA may be a safe treatment option for the patients with CTEPD without PH that can alleviate symptoms, improve exercise capacity, and facilitate weaning from home oxygen therapy. Further prospective randomized trials are needed to confirm these findings.
| S-EPMC11219285 | biostudies-literature
Project description:Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hypertension patients have precapillary pulmonary hypertension with potential etiologies of (1) a nitric oxide deficiency state and vasculopathy consequent to intravascular hemolysis, (2) chronic pulmonary thromboembolism, or (3) upregulated hypoxic responses secondary to anemia, low O2 saturation, and microvascular obstruction. The remainder have postcapillary pulmonary hypertension secondary to left ventricular dysfunction. Although the pulmonary artery pressure in SCD patients with pulmonary hypertension is only moderately elevated, they have a markedly higher risk of death than patients without pulmonary hypertension. Guidelines for diagnosis and management of SCD-related pulmonary hypertension were published recently by the American Thoracic Society. Management of adults with sickle-related pulmonary hypertension is based on anticoagulation for those with thromboembolism; oxygen therapy for those with low oxygen saturation; treatment of left ventricular failure in those with postcapillary pulmonary hypertension; and hydroxyurea or transfusions to raise the hemoglobin concentration, reduce hemolysis, and prevent vaso-occlusive events that cause additional increases in pulmonary pressure. Randomized trials have not identified drugs to lower pulmonary pressure in SCD patients with precapillary pulmonary hypertension. Patients with hemodynamics of pulmonary arterial hypertension should be referred to specialized centers and considered for treatments known to be effective in other forms of pulmonary arterial hypertension. There have been reports that some of these treatments improve SCD-related pulmonary hypertension.
| S-EPMC4760088 | biostudies-literature
Project description:Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct subtype of pulmonary hypertension (PH). One disease hypothesis is that CTEPH results from the non-resolution of venous thromboembolism. CTEPH is characterised by the presence of obstructive fibrotic thromboembolic material in the major pulmonary vessels, with concomitant microvascular arteriopathy, resulting in progressive PH. The clinical presentation of CTEPH is similar to pulmonary arterial hypertension with nonspecific symptoms, but it is distinguished from pulmonary arterial hypertension by the presence of mismatched segmental defects on the ventilation/perfusion scan. The exact prevalence and incidence of CTEPH are unknown, but are thought to have been underestimated in the past. CTEPH is unique among the subgroups of PH in that it is potentially curable with pulmonary endarterectomy, a surgical intervention intended to remove the occlusive material from the pulmonary vasculature. However, in some patients the obstructions are technically inaccessible or the risk/benefit ratios are unfavourable, making the condition inoperable. It is thought that the involvement of the smaller, more distal vessels is a target for medical treatment. Untreated, CTEPH may result in right heart failure and death. The pathophysiological mechanisms which cause CTEPH are complex and have not yet been fully elucidated.
| S-EPMC9487810 | biostudies-literature
Project description:Pulmonary hypertension (PH) is associated with adverse outcomes in adults with sickle-cell disease (SCD), but its importance in children is less clear. The aim of this study was to define the incidence and causes of PH in pediatric patients with SCD. Children with SCD (n = 310) and matched controls (n = 54) were prospectively enrolled under basal conditions. Participants underwent echocardiography, pulse oximetry, 6-minute walk tests, and hematologic testing. Echocardiographic measures were compared between patients with SCD and control subjects before and after adjusting for hemoglobin. Correlations of echocardiographic and clinical parameters were determined. Tricuspid regurgitation velocity (TRV) was elevated compared to controls (2.28 vs 2.10 m/s, p <0.0001). Increased TRV was associated with left ventricular diastolic diameter, hemoglobin, and estimated left atrial pressure. TRV remained elevated when controlling for left ventricular diameter and left atrial pressure. Echocardiographically derived pulmonary resistance was not significantly different between patients with SCD and controls, although it was elevated in the SCD subgroup with elevated TRV. When controlling for hemoglobin, TRV was no longer statistically different, but pulmonary insufficiency velocity, septal wall thickness, and estimated pulmonary resistance were statistically higher. TRV, pulmonary insufficiency end-diastolic velocity, and markers of increased cardiac output were correlated with indicators of adverse functional status, including history of acute chest syndrome, stroke, transfusions, and 6-minute walk distance. In conclusion, children with SCD had mildly increased TRV that was correlated with increased cardiac output and left ventricular filling pressures. Hemoglobin-adjusted analysis also suggested a contribution of primary vascular changes.
| S-EPMC3197734 | biostudies-literature
Project description:Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention.
| S-EPMC6351341 | biostudies-literature
Project description:Chronic pulmonary thromboembolic disease is an important cause of severe pulmonary hypertension, and as such is associated with significant morbidity and mortality. The prognosis of this condition reflects the degree of associated right ventricular dysfunction, with predictable mortality related to the severity of the underlying pulmonary hypertension. Left untreated, the prognosis is poor. Pulmonary endarterectomy is the treatment of choice to relieve pulmonary artery obstruction in patients with chronic thromboembolic pulmonary hypertension and has been remarkably successful. Advances in surgical techniques along with the introduction of pulmonary hypertension-specific medication provide therapeutic options for the majority of patients afflicted with the disease. However, a substantial number of patients are not candidates for pulmonary endarterectomy due to either distal pulmonary vascular obstruction or significant comorbidities. Therefore, careful selection of surgical candidates in expert centres is paramount. The current review focuses on the diagnostic approach to chronic thromboembolic pulmonary hypertension and the available surgical and medical therapeutic options.
| S-EPMC4391185 | biostudies-literature
Project description:We sequenced RNA from pulmonary arteries from CTEPH, IPAH or healthy, failed donors
2019-02-18 | GSE84538 | GEO
Project description:Pulmonary hypertension (PH) is a leading cause of morbidity and early mortality in adults with sickle cell disease (SCD). However, the prevalence, hemodynamic profile and prognosis of SCD-PH remain controversial and need frequent updates. Pulmonary hypertension determined by right heart catheterization (RHC) occurs in 6% to 10% of adults with SCD. Hemodynamically, SCD-PH may be pre-capillary or post-capillary in nature. The exact etiology is unknown and often multifactorial; hence a thorough diagnostic evaluation following established PH guidelines is essential to determine disease prevalence, etiology and outcomes. Data on the efficacy and safety of pulmonary arterial hypertension (PAH) therapy are limited in SCD; clinical trials in these patients are urgently needed. This review provides an overview of RHC-determined hemodynamic characteristics, current management modality and outcomes; we also highlight recent advances and unmet research needs in SCD-PH.
| S-EPMC5072484 | biostudies-other
Project description:Rationale: Chronic thromboembolic pulmonary disease (CTEPD) is defined by chronic organized thrombi in the pulmonary circulation without or with pulmonary hypertension. The current definition of chronic thromboembolic pulmonary hypertension (CTEPH) has adopted lower mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) thresholds. Objectives: Our aim was to identify its impact on the characterization of patients with CTEPD. Methods: All consecutive patients with CTEPD referred for cardiopulmonary exercise testing (CPET) in a pulmonary hypertension center were divided into four groups on the basis of pulmonary hemodynamics: group A, mPAP ≤ 20 mm Hg; group B, mPAP > 20 mm Hg with PVR > 2 and ≤3 Wood units (WU); group C, mPAP > 20 mm Hg with PVR > 3 WU; and group D, mPAP > 20 mm Hg with PVR < 2 WU ("unclassified"). We compared CPET, computed tomography pulmonary angiography, and cardiac magnetic resonance imaging data across the groups. Results: There was mild aerobic capacity impairment, mild/moderate ventilatory inefficiency, and no significant cardiac limitation on CPET in all groups. However, patients in groups A and D had better ventilatory efficiency and less oxygen desaturation on exercise because of lower dead-space ventilation. There was no difference in chronic pulmonary embolus burden and distribution or resting right ventricular function among the groups. Seventeen patients were reclassified as having "CTEPH" on the basis of the current definition. No functional deterioration was noted within a median period of 13 months on repeat CPET. Conclusions: CTEPD patients with similar clot burden and right ventricular function without or with mild/moderate pulmonary hypertension displayed a similar pattern of cardiopulmonary limitation, except for ventilatory efficiency. The current definition of CTEPH may lead to the reclassification of CTEPH in a considerable number of patients.
| S-EPMC7617186 | biostudies-literature