Chronic Thromboembolic Pulmonary Hypertension

Kim, Nick H.; Delcroix, Marion; Jenkins, David P.; Channick, Richard N.; Dartevelle, Philippe; Jansa, Pavel; Lang, Irene; Madani, Michael M.; Ogino, Hitoshi; Pengo, Vittorio; Mayer, Eckhard

doi:10.1016/j.jacc.2013.10.024

Cited by 503 publications

(409 citation statements)

References 63 publications

Supporting

Mentioning

357

Contrasting

Unclassified

Order By: Relevance

“…7,32 According to the current CTEPH registry, PEA can be performed in most CTEPH patients (60%) with 4.7% in-hospital mortality. The present multicenter registry also shows a 3-year survival of 89% in operated patients, which has to be compared with 81% 8 and 76% 9 3-year survival rates reported 15 and 8 years ago in 2 large single-center cohorts.…”

Section: Operated Patientsmentioning

confidence: 99%

“…31 Under optimal conditions, including experienced centers and selected patients, PEA can be performed with low perioperative mortality, with improvements in hemodynamics, symptoms, and survival. 7,32 According to the current CTEPH registry, PEA can be performed in most CTEPH patients (60%) with 4.7% in-hospital mortality. The present multicenter registry also shows a 3-year survival of 89% in operated patients, which has to be compared with 81% 8 and 76% 9 3-year survival rates reported 15 and 8 years ago in 2 large single-center cohorts.…”

Section: Operated Patientsmentioning

confidence: 99%

See 1 more Smart Citation

Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension

et al. 2016

Self Cite

View full text Add to dashboard Cite

C hronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism with uncertain prevalence, ranging from 0.4% to 9.1%. 1 Diagnosis is strongly associated with a history of acute venous thromboembolism (VTE). 2,3 It is caused by nonresolving fibrothrombotic obstructions of large pulmonary arteries, combined Background-Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension. Methods and Results-A total of 679 patients newly diagnosed with chronic thromboembolic pulmonary hypertension were prospectively included over a 24-month period. Estimated survival at 1, 2, and 3 years was 93% (95% confidence interval [CI], 90-95), 91% (95% CI, 87-93), and 89% (95% CI, 86-92) in operated patients (n=404), and only 88% (95% CI, 83-91), 79% (95% CI, 74-83), and 70% (95% CI, 64-76) in not-operated patients (n=275). In both operated and not-operated patients, pulmonary arterial hypertension-targeted therapy did not affect survival estimates significantly. Mortality was associated with New York Heart Association functional class IV (hazard ratio [HR], 4.16; 95% CI, 1. P=0.0065 and HR, 4.76; 95% CI, P=0.0021), increased right atrial pressure (HR, 1.34; 95% CI, 0.95-1.90; P=0.0992 and HR, 1.50; 95% CI, 1.20-1.88; P=0.0004), and a history of cancer (HR, 3.02; 95% CI, 1.36-6.69; P=0.0065 and HR, 2.15; 95% CI, 1.18-3.94; P=0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with pulmonary arterial hypertension-targeted drugs, postoperative pulmonary hypertension, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease in not-operated patients. Conclusions-The long-term prognosis of operated patients currently is excellent and better than the outcome of notoperated patients. with small-vessel arteriopathy in some patients. Both proximal and small-vessel obstruction increase pulmonary vascular resistance (PVR), leading to progressive pulmonary hypertension, right heart failure, and ultimately death. CTEPH can be cured by pulmonary endarterectomy (PEA) 4 and clinically improved by medical therapy. 5,6 Therefore, current guidelines recommend PEA as first-choice therapy, whereas the use of pulmonary arterial hypertension (PAH)-targeted drugs is restricted to inoperable patients or patients with persistent/recurrent pulmonary hypertension after PEA.7 Balloon pulmonary angioplasty, a new and promising treatment method for ...

show abstract

Section: Operated Patientsmentioning

confidence: 99%

Section: Operated Patientsmentioning

confidence: 99%

Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension

et al. 2016

Self Cite

View full text Add to dashboard Cite

show abstract

“…These CT findings are not specific, as they can be seen in pulmonary infarctions as well as in infections and neoplasms [1]. The patient denied consent to contrast medium injection and underwent perfusion lung scanning showing non-segmental perfusion defects [2], due to impressions on the lung of the enlarged central cardiovascular structures, not compatible with chronic thromboembolic pulmonary hypertension (Fig. 3).…”

Section: Differential Diagnosismentioning

confidence: 99%

Pulmonary hypertension nosography: are all patients classifiable?

et al. 2017

View full text Add to dashboard Cite

A 73-year-old never smoking woman was admitted for exertional dyspnoea to the Respiratory Medicine Section of our hospital in June 2014. The patient's past clinical history revealed systemic arterial hypertension, persistent atrial fibrillation, non-insulin dependent diabetes, hypothyroidism, and hypercholesterolemia. She was taking in treatment with acenocoumarol and metformin. The patient had progressive exercise dyspnoea (NYHA class II) and ankle swelling since 2011. She had been diagnosed with interstitial lung disease with pulmonary hypertension (PAPs of 60 mmHg by transthoracic echocardiography) and hypoxemic respiratory insufficiency. She was referred to our hospital following further worsening of dyspnoea and reduction in exercise tolerance despite long-term oxygen therapy (1 L/min). At admission, she had dyspnoea on minor activities (modified Medical Research CouncilmMRC = 3, NYHA class III), cyanosis, increased respiratory rate (22 breaths/min), and ankle swelling. She had systemic pressure at lower limits of normality (105/ 65 mmHg) and a BMI of 28 (158 cm, 71 kg). At physical examination, fine crackles were audible at both lung bases. She had low (54 mmHg) arterial oxygen tension in oxygen therapy with 1 L/min with CO 2 tension of 50 mmHg, in a framework of compensated respiratory acidosis (HCO 3 -33 mmHg). No alterations of serum electrolytes were found. We got sufficient correction of hypoxia (PaO 2 63 mmHg) by increasing flow oxygen support at 3 L/min with a modest increase of hypercapnia (PaCO 2 54 mmHg). The chest radiograph showed increased cardiac size, bulging of the second arch on the left mediastinal border compatible with enlargement of the pulmonary artery common trunk (Fig. 1). No signs of pulmonary congestion were observed. The electrocardiogram showed atrial fibrillation with average ventricular rate of 77 beats per minute, intraventricular conduction delay, and right axial deviation. Transthoracic echocardiography showed severe tricuspidal regurgitation with TAPSE (tricuspid annular plane systolic excursion) of 15 mm, PAPs of 70 mmHg, right and left atrial enlargement, interventricular septum paradoxical movement, inferior vena cava dilatation with no change on inspiration, mild pericardial effusion, and normal size and wall thickness of the left ventricle, without alteration on the segmental kinesis. Cardiac ejection fraction was preserved (61%). No diastolic relaxation abnormalities were detected. E/A ratio [early transmitral flow (E) and the late flow with atrial contraction (A)] was 0.8 and the ratio E/e 0 [early filling velocity on transmitral Doppler (E) with the early relaxation velocity on tissue Doppler (e 0 )] was 7. Only mild mitral regurgitation was described. Calcification of the cusps of the aortic valve with normal opening was described. Aortic valve peak gradient was 22 mmHg.We decided to treat the patient with furosemide 20 mg iv TID, oxygen therapy (3 L/min) with a partial clinical

show abstract

“…It is important that the diagnosis of CTEPH is made, as pulmonary endarterectomy is associated with increased survival and a favorable functional outcome in CTEPH 1. The 2013 World Symposium on Pulmonary Hypertension recommended single photon emission computed tomography (SPECT) ventilation/perfusion (V/Q) scintigraphy as the preferred screening test for CTEPH,8 but this entails injection of 100 MBq of 99mTc‐labeled macroaggregated human albumin, resulting in exposure to ionizing radiation with an effective dose of 0.017 mSv/MBq 9…”

mentioning

confidence: 99%

Lung perfusion: MRI vs. SPECT for screening in suspected chronic thromboembolic pulmonary hypertension

Johns

Swift

Rajaram

et al. 2017

Magnetic Resonance Imaging

View full text Add to dashboard Cite

PurposeTo assess the diagnostic accuracy of magnetic resonance imaging (MRI) perfusion against perfusion single photon emission tomography (SPECT) screening for chronic thromboembolic pulmonary hypertension (CTEPH). Ventilation/perfusion (V/Q) scintigraphy is recommended to screen for suspected CTEPH. It has previously been shown that 3D dynamic contrast‐enhanced (DCE) lung perfusion MRI has a similar sensitivity for diagnosing CTEPH in comparison to planar perfusion scintigraphy; however, planar scintigraphy has now been largely replaced by SPECT, due to higher spatial resolution and sensitivity.Materials and MethodsConsecutive patients with suspected CTEPH or unexplained pulmonary hypertension attending a referral center, who underwent lung DCE perfusion MRI at 1.5T, perfusion SPECT, and computed tomography pulmonary angiography (CTPA) within 14 days of right heart catheterization, from April 2013 to April 2014, were included. DCE‐MR, SPECT, and CTPA were independently analyzed by two blinded radiologists. Disagreements were corrected by consensus. The gold standard reference for the diagnosis of chronic thromboemboli was based on a review of multimodality imaging and clinical findings.ResultsIn all, 74 patients with suspected CTEPH underwent all three modalities. Forty‐six were diagnosed with CTEPH (36) or chronic thromboembolic disease (CTED) (10). 3D DCE perfusion MRI correctly identified all patients (sensitivity of 100%), compared with a 97% sensitivity for SPECT.ConclusionDCE lung perfusion MRI has increased sensitivity when compared with perfusion scintigraphy in screening for CTEPH. As MRI does not use ionizing radiation, it should be considered as a first‐line imaging modality in suspected CTEPH. Level of Evidence: 3 Technical Efficacy: Stage 3J. Magn. Reson. Imaging 2017;46:1693–1697.

show abstract

Chronic Thromboembolic Pulmonary Hypertension

Cited by 503 publications

References 63 publications

Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension

Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension

Pulmonary hypertension nosography: are all patients classifiable?

Lung perfusion: MRI vs. SPECT for screening in suspected chronic thromboembolic pulmonary hypertension

Contact Info

Product

Resources

About