Refined Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Hypertension: Initial Results of U.S. Regional Program.

Objectives: We sought to evaluate the efficacy and safety of refined balloon pulmonary angioplasty (BPA) in the treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Background: BPA is rapidly evolving therapeutic option for patients with nonsurgical CTEPH. There are few US studies that have reported on the outcomes of this novel therapeutic option. Methods: This is a retrospective study of CTEPH patients that underwent BPA at Temple University Hospital. The primary efficacy endpoint was the change in pulmonary vascular resistance (PVR) after BPA as compared to baseline and the primary safety endpoint was the rate of hemoptysis within 24 hours. Secondary endpoints included death, WHO functional class, and 6-minute walk distance (6MWD). We used logistic regression to evaluate factors associated with a hemodynamic and functional response. Results: A total of 211 BPA sessions were performed on 77 patients (average 2.7 ± 1.7 sessions/patient). After BPA the mean PVR improved by 26% (P<0.001) while the mean 6MWD improved by 71.7 meters (P <0.001) and WHO functional class improved by one functional class (P <0.001). Ten sessions (4.7%) were complicated by hemoptysis. The independent factors associated with a improved functional and hemodynamic response included the pre-procedural use of riociguat, reduce baseline PA compliance and > 3 BPA sessions per patient. Conclusion: This single center study from the US showed that BPA with refined techniques in patients with CTEPH was safe and was associated with significant improvements in pulmonary hemodynamics and functional capacity.

Identifying Patients With a Higher Potential for Recovery Post Left Ventricular Assist Device: A Single-Center Experience.

Background: Few patients with a left ventricular assist device (LVAD) achieve functional myocardial recovery to the point of LVAD explantation. The aim of this study was to highlight some of the hemodynamic and echocardiographic parameters we observed in patients who recovered. Methods: We conducted a retrospective analysis of 7 patients who received the HeartMate II LVAD (Abbott) at Temple Heart and Vascular Institute and subsequently underwent successful explantation following myocardial recovery. We compared baseline characteristics, echocardiographic data, and hemodynamic data. Results: Baseline characteristics of the cohort were as follows: age 51.6 ± 12.0 years, 57.1% male, 42.9% with nonischemic cardiomyopathy, and mean duration of LVAD support of 10.6 months. Comparison of echocardiographic and hemodynamic data (preimplant vs preexplant) revealed the following: left ventricular ejection fraction (%) was 12.8 ± 6.9 vs 52.8 ± 8.1 (P=0.0001), right atrial pressure (mmHg) was 12.3 ± 3.4 vs 5.0 ± 4.0 (P=0.022), mean pulmonary artery pressure (mmHg) was 36.0 ± 7.8 vs 15.4 ± 7.1 (P=0.01), cardiac output (L/min) was 3.6 ± 1.3 vs 5.5 ± 1.8 (P=0.004), and cardiac index (L/min/m2) was 1.8 ± 0.5 vs 2.7 ± 0.7 (P=0.008). Mean LVAD-free survival was 49.1 months. Results were consistent in both ischemic and nonischemic LVAD explants. Conclusion: A potential for LVAD explantation exists in patients with both ischemic and nonischemic cardiomyopathy. Myocardial recovery may be more likely among young patients with nonischemic cardiomyopathy and patients with recently diagnosed ischemic cardiomyopathy. Future prospective studies are needed.

Differences in right ventricular morphology, not function, indicate the nature of increased afterload in pulmonary hypertensive subjects with normal left ventricular function.

BACKGROUND: The aim of study was to assess whether a specific morphology of the right ventricle (RV) by 2D echo predicts the hemodynamic nature of pulmonary hypertension (PH). METHODS: We reviewed clinical, 2D echo, and hemodynamic data of 100 patients with PH: divided into three groups: PH from pulmonary vascular disease (PHPVD ; n = 34) with pulmonary vascular resistance (PVR) > 3 mm Hg/L/min (Wood unit [WU]) and pulmonary artery wedge pressure (PAWP) ≤ 15 mm Hg, pulmonary venous hypertension (PVH; n = 33) with PVR < 3 WU and PAWP > 15 mm Hg and PHMIXED (n = 33) with PVR > 3 WU and PAWP > 15 mm Hg. We analyzed several two-dimensional parameters of right heart morphology and function, including the degree of tapering of the RV diameter from base (just above tricuspid annulus) to apex (level of moderator band) in the apical four-chamber view. P = <.05. RESULTS: Baseline characteristics were similar in all three groups: age 62 ± 14.4 years, 69% females, 57% Caucasians. Hemodynamics and 2D echo data of PHPVD vs PVH vs PHMIXED were as follows: PVR 13 ± 6 vs 2 ± 1 vs 7 ± 2 WU, mean pulmonary artery pressure 53 ± 14 vs 34 ± 8 vs 49 ± 8 mm Hg and cardiac index 2.0 ± 0.5 vs 2.8 ± 0.7 vs 2.2 ± 0.7 L/m2 , RV base/apex ratio during systole (sRVb/a ) 1.3 ± 0.2 vs 2.6 ± 0.5 vs 1.5 ± 0.3. Thus, sRVb/a was twofold higher in the PVH vs PHPVD cohort. On ROC analysis, the AUC for sRVb/a for predicting PVR > 3 WU was 0.873, with optimal cutoff of 1.5. CONCLUSION: Systolic RV base/apex ratio is a simple 2D index of RV shape that powerfully predicts a PVR > 3 WU and provides powerful discriminating ability between PVH and PHPVD .

Frequency and predictors of non-compliance to dietary recommendations among hypertensive patients.

Compliance to dietary recommendations among hypertensive people is a major health care issue. Non-compliance can nullify the effects of even the most scientific and optimum treatment plan. The main aim of this study was to determine the frequency and predictors of non-adherence in our region. We also investigated the possible factors based on patient opinions that could increase compliance. A sample of 400 adult patients, diagnosed with hypertension for at least 3 years, who visited Civil Hospital during the time period April-June 2013 were included in this cross sectional study. Patient data and opinions were collected by two trained interviewers using a pre-coded questionnaire. Compliance was assessed based on patients self report. More than three quarters (n = 310, 77.5%) of the hypertensive patients were non-compliant. More than one social gathering in a week, peer-influence, no friends to follow the recommended diet plan and lack of believe regarding diet as an effective measure to control blood pressure were found to be the significant predictors of non-compliance (P values <0.0001). Counseling was thought to be the most effective way by the patients to increase adherence. Non-compliance to dietary recommendations is a major problem prevalent especially in Pakistan. There is an urgent need for doctors and nurses to counsel their patients effectively to prevent future morbidities and mortalities because of non-compliance.

Thalassemia and premarital screening: potential for implementation of a screening program among young people in Pakistan.

Pakistan has a high prevalence of ß-thalassemia (ß-thal) but lacks a screening program for its prevention. This questionnaire-based cross-sectional study was conducted in six randomly chosen non medical universities to assess the students' knowledge of ß-thal and premarital screening, and their attitude towards such a program. Comparison was made between the respondents' attitude towards premarital screening before and after providing them some information regarding the disease. Only 54.5% (207) of 380 students had heard of ß-thal, with a mean knowledge score of 13.0 ± 4.4 out of 27 questions. Most respondents were aware of the concept of premarital screening. Out of 207 students, 60.4% wanted to know if they were carriers, 69.1% wanted to know their spouse's carrier status and 59.4% wanted premarital screening to be made mandatory in Pakistan. These figures increased to 72.5, 78.3 and 67.6%, respectively after provision of written information (p values: 0.03, 0.02, and 0.01, respectively). The positive attitude towards premarital screening with low background knowledge of the disease highlights the need of a mass awareness campaign and subsequent implementation of a premarital screening program.

Strong BCL2 expression in Burkitt lymphoma is not uncommon in adults.

BACKGROUND: The distinction between Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL) is not always easy, so much so that the WHO 2008 Blue Book has incorporated a provisional category of "B-cell lymphoma, unclassifiable with features intermediate between DLBCL and BL." One of the immunohistochemical (IHC) markers used at times to differentiate between the two is IHC expression of BCL2, which was initially believed to be consistently negative in BL. Later it was recognized that weak BCL2 expression is still compatible with the diagnosis of BL. To verify or otherwise this current view this study was undertaken. MATERIALS AND METHODS: We retrieved 39 confirmed cases of BL, in both children and adults. All these cases had typical morphology, IHC profile, and Mib-1 index that are typical of BL. All these cases were then stained with a monoclonal antibody against BCL2 oncoprotein, using 2-step Envision system. RESULTS: Out of 39 cases, 31 cases (79.4%) were completely negative for BCL2, whereas 5 (12.8%) were weak focal positive. However, another 4 (10.2%) cases did show strong diffuse cytoplasmic staining for BCL2. Fluorescent in-Situ hybridization (FISH) for t(14:18) was optimally done on 6 out of 9 cases. All these 4 cases were from adults with 3 out of 4 arising in the parotid region. Two out of 4 cases also showed t(8:14) on FISH. CONCLUSIONS: It was concluded that contrary to the common belief, strong BCL2 IHC expression is possible in typical BL in adults and cannot be absolutely relied upon to distinguish between BL and DLBCL.