Review articleChronic thromboembolic pulmonary hypertension
Section snippets
Natural history
The pathophysiologic events leading to CTEPH are not entirely understood. Contributing to the gaps in the understanding of this process has been the typical presentation of patients late in the course of the disease, often without a history of a venous thromboembolic event or events. Consequently, a retrospective piecing together of historical and clinical clues, along with a knowledge of the natural history of acute thromboembolic events, has by necessity become the foundation of the
Clinical presentation and patient evaluation
At initial presentation, CTEPH patients most commonly complain of exertional dyspnea and an unexpected decline in exercise capabilities. Although individual tolerances vary, the physiologic basis for these complaints relates to limitations in cardiac performance caused by an elevated pulmonary vascular resistance and increased minute ventilatory needs from an elevated alveolar dead space. Patients can experience a nonproductive cough, especially with exertion. Hemoptysis occurs rarely and is
Surgical selection and preoperative management
Patients with suspected chronic thromboembolic pulmonary hypertension undergo evaluation with the goals of establishing the need for surgical intervention, determining the surgical accessibility of the chronic thromboemboli, and estimating the risk of surgery and anticipated hemodynamic outcome for the individual patient. Most patients who ultimately go on to surgery exhibit a pulmonary vascular resistance greater than 300 dynes/second/cm−5. At centers reporting their experience with pulmonary
Pulmonary thromboendarterectomy
Because the details of pulmonary thromboendarterectomy are reviewed by Thistlethwaite and Jamieson in an accompanying article in this issue and in other publications [20], [83], [84], only selected features of the operation are highlighted here. Surgical success requires a true endarterectomy to remove the organized thrombi, not an embolectomy. The chronic thromboembolic material is fibrotic and is incorporated into the native vascular wall. An endarterectomy involves identification of the
Outcome following pulmonary thromboendarterectomy
Meticulous management of patients following pulmonary thromboendarterectomy and an understanding of the physiologic changes that occur postoperatively are essential to achieving successful outcomes [87]. In addition to complications seen in other forms of cardiac surgery involving cardiopulmonary bypass, such as arrhythmias, coagulation disorders, wound infections, delirium, and nosocomial pneumonia, postendarterectomy patients often experience two unique problems that adversely effect
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Cited by (54)
Imaging Pulmonary Arterial Thromboembolism. Challenges and Opportunities.
2015, Magnetic Resonance Imaging Clinics of North AmericaCitation Excerpt :Similarly, Pengo and colleagues49 found a cumulative incidence of symptomatic CTEPH of 3.8% after 2 years in patients with an acute episode of PE. Instead of thrombolysis, the thromboembolic material follows an aberrant path of organization and recanalization, leading to characteristic abnormalities such as intraluminal webs and bands, pouchlike endings of arteries, irregularities of the arterial wall, and stenotic lesions (Figs. 5 and 6).50–52 This aberrant path of obstruction and reopening occurs in repeated cycles over many years.
Chronic Thromboembolic Pulmonary Hypertension
2012, Heart Failure ClinicsCitation Excerpt :Recent literature has suggested that obliteration of central PAs caused by organized thrombi may not be the sole underlying mechanism leading to the progressive PH and right heart failure. While thromboemboli may be the inciting event, the role of pulmonary microvascular arteriopathy is gaining recognition as an important contributor to disease progression.4–6 Until relatively recently, CTEPH was a diagnosis made primarily at autopsy, but advances made in diagnostic modalities and surgical pulmonary endarterectomy techniques have made this disease treatable and even potentially curable.
Survival after pulmonary thromboendarterectomy: Effect of residual pulmonary hypertension
2011, Journal of Thoracic and Cardiovascular SurgeryAngiographic predictors of hemodynamic improvement after pulmonary endarterectomy
2010, Annals of Thoracic SurgeryCitation Excerpt :As shown in Table 1, our patients with cardiac comorbidity were less likely to die in hospital by univariate analysis, which may be due to the limited number of cases. The only unequivocal risk factor for PEA seems to be severe parenchymal lung disease with obstructive or restrictive dysfunction [1, 2, 22, 23]. In the current study, patients with restrictive lung disease were associated with a higher risk of mortality in univariate analysis.
Discussion
2010, Annals of Thoracic SurgeryCitation Excerpt :As shown in Table 1, our patients with cardiac comorbidity were less likely to die in hospital by univariate analysis, which may be due to the limited number of cases. The only unequivocal risk factor for PEA seems to be severe parenchymal lung disease with obstructive or restrictive dysfunction [1, 2, 22, 23]. In the current study, patients with restrictive lung disease were associated with a higher risk of mortality in univariate analysis.