Michał Florczyk

Detectable Serum Cardiac Troponin T as a Marker of Poor Prognosis Among Patients With Chronic Precapillary Pulmonary Hypertension

Background—Right ventricular failure is a leading cause of death in patients with chronic pulmonary hypertension (PH). We checked whether detection of cardiac troponin T (cTnT), a specific marker of myocyte injury, could be useful in prognostic stratification of those patients. Methods and Results—Initial evaluation of 56 clinically stable patients (age 41±15 years) with pulmonary arterial (51 patients) or inoperable chronic thromboembolic (5 patients) PH (mean pulmonary arterial pressure 60±18 mm Hg) included cTnT test, allowing detection of its serum levels ≥0.01 ng/mL [cTnT(+)]. cTnT was detectable in 8 of 56 (14%) patients (mean±SD, 0.034±0.022; range, 0.010 to 0.077 ng/mL). Despite similar pulmonary hemodynamics, they had higher heart rate (92±15 versus 76±14 bpm, P =0.004), lower mixed venous oxygen saturation (50±10% versus 57±9%, P =0.04), and higher serum N-terminal pro-B–type natriuretic peptide (4528±3170 versus 2054±2168 pg/mL, P =0.03) and walked less during the 6-minute walk test (298±132 versus 396±101 m, P =0.02). Cumulative survival estimated by Kaplan-Meier curves was significantly worse at 24 months in cTnT(+) compared with cTnT(−) (29% versus 81%, respectively, log-rank test P =0.001). Multivariate analysis revealed cTnT status (hazard ratio, 4.89; 95% CI, 1.18 to 20.29; P =0.03), 6-minute walk test (hazard ratio, 0.93 for each 10 m; P =0.01), and pulmonary vascular resistance (hazard ratio, 1.13; P =0.01) as independent markers of mortality. All 3 cTnT(+) patients who survived the follow-up period converted to cTnT(−) during treatment. Conclusions—Detectable cTnT is a so-far ignored independent marker of increased mortality risk in patients with chronic precapillary PH, supporting the role of progressive myocyte injury in the vicious circle leading to hemodynamic destabilization.

Pulmonary Artery Dilatation Correlates With the Risk of Unexpected Death in Chronic Arterial or Thromboembolic Pulmonary Hypertension

BACKGROUND Right ventricular failure does not explain all cases of death in patients with chronic pulmonary hypertension. Searching for alternative explanations, we evaluated the prognostic significance of main pulmonary artery (PA) dilatation in patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH). METHODS A retrospective outcome analysis was made of 264 patients (aged 46 ± 17 years; women, 69%; PAH, 82%) who underwent both CT scan measurement of the PA and right-sided heart catheterization (mean PA pressure, 57.6 ± 16.5 mm Hg) at initial evaluation. RESULTS The diameter of the PA ranged from 28 to 120 mm (mean, 39 ± 8.6 mm; median, 38 mm) and was largest in patients with unrepaired congenital defects (42.6 ± 7.6 mm). Pulmonary pulse pressure (P = .04), lower age (P = .03), and duration of symptoms (P < .001) were independently but weakly related to PA diameter. During follow-up (median, 38 months), 99 patients (37%) died. Of these 99 deaths, 73 (74%) were due to heart failure or comorbidities, and 26 (26%) were unexpected deaths (UE-Ds). PA diameter (hazard ratio [HR], 1.06 per 1 mm; 95% CI, 1.03-1.08), heart rate (HR, 1.30 per 10 beats/min; 95% CI, 1.01-1.66), and systolic pulmonary arterial pressure (HR, 1.02 per 1 mm Hg; 95% CI, 1.01-1.04) were the only independent predictors of UE-D and differed from the usual predictors found in the study group for all-cause mortality. PA diameter ≥ 48 mm had 95% specificity and 39% sensitivity and carried 7.5 times higher risk of UE-D (95% CI, 3.4-16.5; P < .0001) during follow-up. CONCLUSIONS PA dilatation emerges as an independent risk factor for death unexplained by right ventricular failure or comorbidities in patients with PAH and CTEPH. The possible mechanisms include, but are not limited to, PA compression of the left main coronary artery, PA rupture, or dissection with cardiac tamponade.

In-hospital major bleeding predicts mortality in patients with pulmonary embolism: An analysis of ZATPOL Registry data

BACKGROUND There are no data on the association between in-hospital bleeding and mortality in patients with pulmonary embolism (PE). OBJECTIVES To assess whether in-hospital major bleeding predicts in-hospital and 90-day mortality in patients with PE confirmed objectively using validated diagnostic criteria. METHODS ZATPOL is a prospective national registry of consecutive patients with suspected PE admitted to 86 cardiology departments across Poland from January 2007 to September 2008. We retrospectively studied the influence of in-hospital bleeding on outcomes. RESULTS Of 2015 patients enrolled, 1216 were locally diagnosed with PE. Validated diagnostic criteria according to the European Society of Cardiology guidelines were met in 1112 patients. In the latter group, major bleeding occurred in 3.6%, and 0.5% had fatal bleeding. Thrombolytic therapy was administered to 11% of patients. Vascular access site bleeding was the most common (40%). Except for hypotension or shock and cancer, major bleeding was the strongest independent predictor of both in-hospital (OR 3.47; P=0.003) and 90-day mortality (OR 2.75; P=0.009). Other factors independently associated with in-hospital mortality were: shock or hypotension (OR 7.45; P<0.001), cancer (OR 1.9; P=0.044), and presence of ≥1 concomitant disease (OR 2.59; P<0.001). Other predictors of 90-day mortality were: shock or hypotension (OR 5.23; P<0.001), cancer (OR 3.57; P<0.001), presence of ≥1 concomitant disease (OR 2.01; P=0.001) and age>71 years (OR 1.5; P=0.063). CONCLUSION In-hospital major bleeding is a newly described strong independent predictor of both in-hospital and 90-day mortality in patients with objectively confirmed PE.

Recurrent Hemoptysis: An Emerging Life-Threatening Complication in Idiopathic Pulmonary Arterial Hypertension

Hemoptysis is a rarely reported complication of idiopathic pulmonary arterial hypertension (IPAH). We present the case of a 27-year-old woman with IPAH, who suffered from recurrent hemoptysis and who was treated with sitaxsentan and treprostinil and remained stable in World Health Organization functional class II. During several episodes of active hemoptysis, the patient underwent bronchial artery embolization (BAE), always with good immediate results. She developed severe respiratory insufficiency and died of electromechanical dissociation 2 days after another episode, despite effective bleeding control. Recurrent hemoptysis in patients with IPAH emerges as a potential indication for urgent placement on the lung transplant list, independent from the classic prognostic factors of functional class and indices of right-sided ventricular function. Repeated BAE should not be considered as a definitive treatment in patients with pulmonary arterial hypertension with recurrent bleeding, although it may help in bridging patients to lung transplant.

Improvement in Quality of Life and Hemodynamics in Chronic Thromboembolic Pulmonary Hypertension Treated With Balloon Pulmonary Angioplasty

BACKGROUND The effect of balloon pulmonary angioplasty (BPA) on improvement in functional and hemodynamic parameters in chronic thromboembolic pulmonary hypertension (CTEPH) is known, but the quality of life (QoL) of patients treated with BPA has never been studied before.Methods and Results:Twenty-five patients with inoperable or persistent CTEPH were enrolled in the study and filled out the 36-item Short Form (SF-36v2) questionnaire twice: prior to commencement of BPA treatment and after ≥3 BPA sessions. In addition WHO functional class, distance on the 6-min walk test (6MWT) and hemodynamic parameters such as right atrial pressure (RAP), mean pulmonary artery pressure (mPAP), cardiac index (CI) and pulmonary vascular resistance (PVR) were assessed. QoL improved significantly in all domains, except for physical pain. Improvement in RAP (10.5±3.4 vs. 6.2±2.2 mmHg; P<0.05), mPAP (51.7±10.6 vs. 35.0±9.1 mmHg; P<0.05), CI (2.2±0.5 vs. 2.5±0.4 L/min·m2; P=0.04), PVR (10.4±3.9 vs. 5.5±2.2 Wood units; P<0.05), functional class (96% vs. 20% in WHO class III and IV, P<0.05) and improvement in 6MWT distance (323±135 vs. 410±109 m; P<0.05) was observed. The only significant correlation was between the mental component summary score of QoL after completion of treatment and percentage improvement in the 6MWT (-0.404, P<0.05). CONCLUSIONS Alongside improvement in functional and hemodynamic parameters, BPA also provides significant improvement in QoL.

Acute and chronic dissection of pulmonary artery: new challenges in pulmonary arterial hypertension?

Right ventricular failure is a leading cause of mortality in patients with pulmonary arterial hypertension (PAH). However, up to 25% of such patients die unexpectedly, without warning signs of hemodynamical decompensation. We previously documented that pulmonary artery (PA) dilatation significantly increases the risk of those deaths. Some of them may be due to dissection of PA resulting in cardiac tamponade. However, direct confirmation of this mechanism is difficult as most of such deaths occur outside hospitals. We present 4 patients with severe PAH and PA dilatation in whom PA dissection has been confirmed. Three patients had IPAH, one had PAH associated with congenital heart disease. All patients had mean pulmonary artery pressure (PAP) > 50 mmHg at diagnosis and dissection occurred late in the course of apparently well controlled disease (6 to 14 years). Several clinical elements were common to our patients - high systolic PAP, long lasting PH, progressive dilatation of PA to more than 50 mm with chest pain prior to dissection. However, clinical course followed three different patterns: sudden death due to cardiac tamponade, hemopericarditis caused by blood leaking from dissected aneurysm with imminent but not immediate cardiac tamponade, or chronic asymptomatic PA dissection. Indeed, two of our patients are alive and on lung transplantation waiting list for more than 2 years now. Further research is needed to suggest optimal management strategies for patients with stable PAH but significantly dilated proximal pulmonary arteries or confirmed PA dissection depending on the clinical presentation and expected outcome.

Sequential treatment with sildenafil and riociguat in patients with persistent or inoperable chronic thromboembolic pulmonary hypertension improves functional class and pulmonary hemodynamics

BACKGROUND This study evaluated the incremental effect of riociguat on pulmonary hemodynamics in patients with inoperative or persistent chronic thromboembolic pulmonary hypertension (CTEPH) treated previously with sildenafil. METHODS The retrospective study included 28 patients diagnosed with CTEPH who were ineligible for surgical treatment due to distal thrombi location or who suffered from persistent CTEPH after pulmonary endarterectomy and who were treated with sildenafil at a dose of 25 mg TID for a minimum of 3 months. Sildenafil was subsequently discontinued, and riociguat therapy was started with gradually increasing doses. Right heart catheterization was performed and WHO functional class (FC) was assessed in each patient at three time points: before starting sildenafil therapy (baseline), before the transition to riociguat, and after 3 to 6 months of therapy with riociguat. RESULTS Compared to baseline, the use of sildenafil and riociguat significantly decreased pulmonary vascular resistance (PVR) (10.47 ± 3.56 vs. 7.81 ± 3.58 Wood units, p < 0.001) and mean pulmonary arterial pressure (PAP) (54.1 ± 11.6 vs. 46.1 ± 13.2 mm Hg; p < 0.001) while increasing cardiac output (CO) (4.31 ± 0.88 vs. 4.85 ± 0.87 L/min; p = 0.007). Switching from sildenafil to riociguat reduced PVR by 14% (p = 0.005) and the mean PAP by 6% (p = 0.03) while increasing CO by 11% (p = 0,002). The number of patients with WHO FC III and IV symptoms decreased from 71,4% to 57,1% (p = 0,02) after the change from sildenafil to riociguat. CONCLUSIONS Replacing sildenafil with riociguat in patients with inoperable or persistent CTEPH may improve pulmonary hemodynamics and FC.

Severe aortic stenosis in a patient with breast cancer

We present a case of 68-year-old female with severe symptomatic aortic stenosis and locally advanced breast cancer disqualified from mastectomy due to heart failure and from aortic valve replacement due to malignant neoplasm. The patient received neoadjuvant chemotherapy without anthracyclines. The aortic valve replacement was performed and then mastectomy and lymphadenectomy were made without hemodynamic complications. Adjuvant hormonotherapy was started. During 42 months of follow-up the patient remained free of recurrent cancer disease as well as no progression of heart failure was observed. Key wordS: aortic stenosis, breast cancer, anthracyclines, aortic valve replacement, echocardiography Received: 25.01.2016. Accepted: 5.04.2016.

TCT-804 Improvement in the balloon pulmonary angioplasty strategy results in reduction of periprocedural complications and mortality rate

The purpose of present study was to determine safety and hemodynamic impact of refined balloon pulmonary angioplasty (BPA) procedure. Study population comprised of patients with established diagnosis of CTEPH, who were disqualified from pulmonary endarterectomy due to distal localization of