The malignant obesity hypoventilation syndrome (MOHS)

We have coined the term ‘malignant obesity hypoventilation syndrome’ (MOHS) to describe a severe multisystem disease due to the systemic effects of obesity. Patients with this syndrome have severe obesity‐related hypoventilation together with systemic hypertension, diabetes and the metabolic syndrome, left ventricular hypertrophy with diastolic dysfunction, pulmonary hypertension and hepatic dysfunction. This syndrome is largely unrecognized as physicians do not make the association between the patients' multiple medical problems and obesity. Because of the delayed diagnosis and progressive morbidities of this condition, all patients with a body mass index of more than 40 kg m^?2 should be screened for MOHS. The management of patients with MOHS includes short‐term measures to improve the patients' medical condition and long‐term measures to achieve enduring weight loss. Bariatric surgery reverses or improves the multiple metabolic and organ dysfunctions associated with MOHS and should be strongly considered in these patients.     

Septal Dyskinesia and Global Left Ventricular Dysfunction in Pediatric Wolff‐Parkinson‐White Syndrome with Septal Accessory Pathway

LV Dysfunction in WPW Syndrome. Introduction: Echocardiographic studies have shown that some patients with Wolff‐Parkinson‐White (WPW) syndrome have myocardial dyskinesia in the segments precociously activated by an accessory pathway (AP). The aim of the present study was to determine the extent to which the AP contributes to global left ventricular (LV) dysfunction. Methods: Electrophysiological and echocardiographic data from 62 children with WPW (age at diagnosis = 5.9 ± 4.2 years) were retrospectively analyzed. Results: The left ventricular ejection fraction (LVEF) of patients with septal APs (53 ± 11%) was significantly lower than that of patients with right (62 ± 5%) or left (61 ± 4%) APs (P = 0.001). Compared to patients with normal septal motion (n = 56), patients with septal dyskinesia (n = 6) had a reduced LVEF (61 ± 4% and 42 ± 5%, respectively) and an increased LV end diastolic dimension (P < 0.001 for both comparisons). Multivariate analysis identified septal dyskinesia as the only significant risk factor for reduced LVEF. All 6 patients with septal dyskinesia had right septal APs, and a preexcited QRS duration that was longer than that of patients with normal septal motion (140 ± 18 ms and 113 ± 32 ms, respectively; P = 0.045). After RFA there were improvements in both intraventricular dyssynchrony (septal‐to‐posterior wall motion delay, from 154 ± 91 ms to 33 ± 17 ms) and interventricular septal thinning (from 3.0 ± 0.5 mm to 5.3 ± 2.6 mm), and a significant increase in LVEF (from 42 ± 5% to 67 ± 8%; P = 0.001). Conclusion: The dyskinetic segment activated by a right septal AP in WPW syndrome may lead to ventricular dilation and dysfunction. RFA produced mechanical resynchronization, reverse remodeling, and improvements in LV function. (J Cardiovasc Electrophysiol, Vol. 21, pp. 290–295, March 2010)     

QT Interval Prolongation with Global T‐Wave Inversion: A Novel ECG Finding in Acute Pulmonary Embolism

Objective: The purpose of this study was to report a novel electrocardiographic (ECG) phenomenon in acute pulmonary embolism characterized by QT interval prolongation with global T‐wave inversion. Methods: Among a total of 140 study patients with a confirmed diagnosis of acute pulmonary embolism, patients who fulfilled the inclusion criteria for QT interval prolongation with global T‐wave inversion were examined. Each of these patients had undergone a detailed clinical evaluation including testing for myocardial injury and echocardiography. Results: QT interval prolongation with global T‐wave inversion was found in five patients (age 51–68 years) with acute pulmonary embolism. Four were women. Acute pulmonary embolism was diagnosed by ventilation‐perfusion scan in three patients and by spiral computed tomography in other two patients. None of the patients had any right or left ventricular regional wall motion abnormalities on echocardiography. All patients had changes characteristic of hemodynamically significant pulmonary embolism, including right ventricular stunning or hypokinesis and dilatation in five patients with paradoxical septal motion in four. Acute coronary syndrome was ruled out in each patient by clinical evaluation, serial ECGs and cardiac markers, and lack of regional wall motion abnormalities on echocardiography. Prolongation of QT intervals (QTc 456–521 ms) with global T‐wave inversion was noted on presentation. The ECG changes gradually resolved in 1 week in all patients with appropriate treatment of acute pulmonary embolism. One patient died. None of the patients developed torsade de pointes. Conclusions: Acute pulmonary embolism may occasionally result in reversible QT interval prolongation with deep T‐wave inversion, and, thus should be considered among the acquired causes of the long QT syndrome.     

Hemodynamic correlates of right ventricular ejection fraction measured with gated radionuclide angiography

Right ventricular function was studied in 60 patients with equilibrium gated radionuclide angiography. The mean (± standard deviation) right ventricular ejection fraction in 20 normal subjects was 53 ± 6 percent, a value in agreement with previous data from both radionuclide and contrast angiographie studies. This value was similar (55 ± 7 percent) in 11 patients with coronary artery disease but normal left ventricular function.Radionuclide measurements of right ventricular ejection fraction were correlated with right heart hemodynamics. There was a significant negative linear correlation between right ventricular ejection fraction and mean pulmonary arterial pressure (r = ?0.82) and between right ventricular ejection fraction and right ventricular end-diastolic pressure (r = ?0.67). Furthermore, patients with elevated right ventricular enddiastolic pressure and mean pulmonary arterial pressure had a more severely depressed ejection fraction than did those with an elevated mean pulmonary arterial pressure alone.Thus, an abnormal value for right ventricular ejection fraction by gated radionuclide angiography in the absence of primary right ventricular volume overload suggests abnormal right heart pressures, whereas a normal value excludes severe pulmonary arterial hypertension or an elevated right ventricular end-diastolic pressure.     

Transcatheter Closure of Right‐to‐Left Interatrial Shunts to Resolve Hypoxemia

Background. Right‐to‐left interatrial shunts through an atrial septal defect or patent foramen ovale may result from congenital and acquired conditions. Right‐to‐left shunts may occur with normal pulmonary artery pressure and resistance as in right ventricular outflow tract obstruction leading to hypertrophy and reduced diastolic function, in right ventricle ischemia, myocarditis or cardiomyopathy and in orthodeoxia–platypnea syndrome. Methods. We have tested the tolerance of trans‐catheter closure of right‐to‐left interatrial shunts in 5 selected patients in whom the morphology and hemodynamics implied that it would be well‐tolerated and improve functional capacity. Right‐to‐left shunt was demonstrated in 5 profoundly cyanotic patients by trans‐thoracic and trans‐esophageal echocardiography with intravenous injection of agitated saline. The patients were catheterized for hemo‐dynamic study and tested for tolerance of transient balloon occlusion of the defects. Results. Diastolic right ventricular dysfunction with elevated end‐diastolic pressure was the primary cause of right‐to‐left shunt. Most shunts occur via atrial septal defects. Patients' ability to tolerate temporary balloon occlusion of the defects predicted a favorable outcome following permanent device occlusion. Cyanosis resolved in all patients following closure of the defects without congestive right heart failure. A marked improvement in functional capacity was observed in 4 patients. One died of preinterventional hypoxic brain damage. Conclusion. Transcatheter closure of right‐to‐left shunts is well‐tolerated and a rewarding approach. It should be applied in selected patients following careful morphologic and hemodynamic evaluation. Tolerance of temporary occlusion of the defect is predictive of a favorable procedural outcome.     

Right ventricular longitudinal deformation parameters and exercise capacity

Objective

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease characterized by increased pulmonary vascular resistance resulting in pulmonary hypertension and right heart failure. The six-minute walk test (6MWT) distance is associated with the prognosis of CTEPH patients. Speckle tracking echocardiography (STE) is a reliable method for determining ventricular function. The aim of this study was to assess and compare the right ventricular (RV) function of CTEPH patients according to their 6MWT distances.

Methods

Forty-nine consecutive CTEPH patients (mean age, 50?±?16 years; 22 male) who were referred to our center for pulmonary thromboendarterectomy (PTE) were included in the study. All patients underwent the 6MWT and right heart catheterization (RHC). Standard echocardiography and STE were performed on all patients before PTE. Patients were divided into two groups based on their 6MWT distance being less or more than 300 m.

Results

Patients with a shorter 6MWT distance had a significantly larger RV, while they had a significantly lower RV fractional area change and higher myocardial performance index suggesting impaired RV function. Both RV basal–lateral strain and strain rate measures were significantly lower in patients with shorter 6MWT distances than those with longer 6MWT distances. Similarly, they had lower RV basal–septal, mid-lateral, and global strain measures. 6MWT distances were correlated with RV basal–lateral and mid-lateral strain measures (r?=?0.349, p?=?0.025 and r?=?0.415, p?=?0.008, respectively).

Conclusion

Our data suggest that RV myocardial deformation parameters are associated with 6MWT distances. Determination of RV dysfunction by STE may be helpful in identifying patients with a poor prognosis.     

Right‐Sided Free Wall Accessory Pathway Refractory to Conventional Catheter Ablation: Lessons From 3‐Dimensional Electroanatomic Mapping

Right Free Wall Accessory Pathway Ablation . Introduction: The aim of this study was to delineate the electroanatomic substrates of right‐sided free wall (RFW) accessory pathways (APs) that were refractory to conventional catheter ablation utilizing 3‐dimensional (3‐D) mapping. Methods and Results: Eleven patients with RFW APs that failed initial conventional catheter ablation(s) by a mean of 1.9 ± 0.5 attempts were enrolled in the study. Electroanatomic mapping of the right atrium was performed during orthodromic reciprocating tachycardia in 3 patients and right ventricular pacing in 8 patients. The earliest atrial activation site, which represented the atrial insertion of the AP, was separated from the tricuspid annulus by an average of 14.3 ± 3.9 mm, and the local activation time was 27.8 ± 17.0 ms earlier than that of the corresponding annular point. One patient exhibited an AP with wide branching on the atrial side. RF ablation with an irrigated catheter successfully interrupted AP conduction in all patients without complications. Conclusions: RFW APs resistant to conventional catheter ablation might be due to unique anatomic AP features such as more epicardial course at the annulus level with atrial insertion distant from the tricuspid annulus. Electroanatomic mapping is helpful to accurately localize the atrial insertion sites of these APs and facilitates catheter ablation. (J Cardiovasc Electrophysiol, Vol. 21, pp. 1317‐1324, December 2010)     

Transcatheter closure of atrial septal defect with Amplatzer® device in children aged less than 10 years old: Immediate and late follow‐up

Aims: To analyze the efficacy and follow‐up results of percutaneous closure of Atrial septal defect (ASD) with the Amplatzer® septal occluder in children aged <10 years old. Methods: Between November 1998 and September 2005, 27 patients diagnosed with ASD were treated percutaneously with an Amplatzer septal occluder. The procedure was carried out in the cathlab, under general anesthesia and with both fluoroscopy and transesophageal echocardiography guidance. Basal physical examinations and echocardiograms were performed prior to the procedure and at 30 days, 6, and 12 months of follow‐up. Survival free of symptom was estimated by Kaplan–Meier. Results: The mean age, weight, height, body mass index, and corporal surface was: 5.35 ± 2.11 years, 23.07 ± 9.43 kg, 110.55 ± 17.6 cm, 16.77 ± 2.42 kg/m², and 1.24 ± 2.44 m². The prevalence of septal aneurysm was 3.7% and all patients presented single secundum ASD. The mean stretched diameter by fluoroscopy and transesophageal echocardiography were 17.18 ± 6.75 mm and 16.77 ± 5.99 mm, and the prostheses sizes were 18.83 ± 6.98 mm, ranging from 10 to 30 mm. The systolic and diastolic pulmonary pressures were 25.26 ± 5.97 mm Hg and 13.38 ± 3.40 mm Hg, respectively. The procedure time was 82.92 ± 29.14 min and the hospital stay was 2.20 ± 0.26 days. Clinical and echocardiography follow‐ups were performed within 11.59 ± 4.42 months and all devices were in the correct position with no residual shunt. Right ventricular diameter decreased from 19.38 ± 5.23mm to 11.38 ± 11.92 (P 0.001). No major complications or deaths occurred; two patients had a hematoma at the vascular access. Conclusion: Secundum atrial septal defect closure can be safely and successfully performed with the Amplatzer septal occluder in children younger than 10 years old. © 2008 Wiley‐Liss, Inc.     

Cross sectional Doppler echocardiography as the initial technique for the diagnosis of acute pulmonary embolism.

OBJECTIVE--To determine the value of cross sectional Doppler echocardiography and derived indices of right ventricular pressure and function in the initial diagnosis of pulmonary embolism. BACKGROUND--Most deaths from acute pulmonary embolism occur because of a delay in diagnosis. Ventilation-perfusion scans are not sufficiently sensitive, whereas angiography is invasive and associated with complications. The use of cross sectional Doppler echocardiography to assess acute changes in right ventricular filling pressure and function, and in pulmonary arterial systolic pressure and its relation to embolism has not been studied in a large population. METHODS--60 consecutive patients with acute symptoms or haemodynamic instability suggestive of pulmonary embolism were studied. Confirmatory investigations included a ventilation-perfusion scan (36 patients), angiography (18 patients), surgery (5 patients), or necropsy (5 patients). RESULTS--There was evidence of right ventricular pressure or volume overload in all. This took the form of increased right ventricular end diastolic diameter and leftward bulging of the interventricular septum in diastole (56 patients); tricuspid valve regurgitation (56 patients) with the peak velocity of the regurgitant jet > 2.6 m/s; and a low collapse index for the inferior vena cava of < 40%, indicating raised mean right atrial pressure (in 49 patients). Intracardiac or pulmonary thrombi were visualised in 10 patients. In 14 patients treatment was undertaken on the basis of the echocardiographic signs alone. Four of them (with visible thrombi) recovered: the other 10 died. Lung emboli were demonstrated in 4 of 5 patients in whom necropsy was performed. CONCLUSIONS--Cross sectional Doppler echocardiography is a sensitive technique for the rapid identification of right ventricular overload in acute pulmonary embolism. It enables further investigations on treatment to be appropriately directed without delay. Resolution of emboli can also be assessed by serial measurement of the described indices.     

Usefulness of measuring right ventricular anterior wall thickness in patients with right ventricular overload using M-mode echocardiography

Right ventricular anterior wall thickness measured by M-mode echocardiography and right ventricular systolic pressure obtained by right heart catheterization were correlated in 62 patients with chronic right ventricular overload including congenital heart disease and primary pulmonary hypertension. The patients were divided into two groups; one, with right ventricular systolic pressures of 39 mmHg or less; the other, 40 mmHg or more. The following results were obtained. 1. The correlation coefficient for right ventricular anterior wall thickness and right ventricular systolic pressure was r = 0.90 (p less than 0.001), and the regression equation was y = 13.2x-1.3. 2. Right ventricular end-diastolic dimension increased significantly in both groups, but no statistically significant differences were detected between the two. Right ventricular anterior wall thickness increased significantly in the group with higher right ventricular pressures (7.1 +/- 0.5 mm vs 3.1 +/- 0.5 mm). 3. When right ventricular anterior wall thickness was more than 4.0 mm, pulmonary hypertension was detected, with a sensitivity of 97.5% and a specificity of 90.9%. In conclusion, measurements of right ventricular anterior wall thickness by M-mode echocardiography via the anterior chest wall proved to be potentially useful in predicting right ventricular systolic pressures in patients with chronic right ventricular overloads.     

Paradoxical ventricular septal motion with right ventricular dilatation as a manifestation of pure pressure overload due to pulmonary veno-occlusive disease

Paradoxical interventricular septal motion with right ventricular dilatation has been considered the hallmark of right ventricular volume overload. We report a 43-year-old woman with severe pulmonary hypertension due to pulmonary veno-occlusive disease who exhibited these echocardiographic abnormalities. Right ventricular volume overload was excluded by physical examination, echocardiography with saline contrast study and by cardiac catheterization, angiography, and shunt study. These echocardiographic findings are thus not pathognomonic of right ventricular volume overload and can be seen with pressure overload as well.     

Growth of a solitary pulmonary nodule after 6 years diagnosed as oncocytic carcinoid tumour with a high 18‐fluorodeoxyglucose (18F‐FDG) uptake in positron emission tomography‐computed tomography (PET‐CT)

Introduction: Pulmonary carcinoid tumour is low‐grade neuroendocrine malignancy that is seen 1%–2% of all lung neoplasms. Oncocytic carcinoid type is a rarely seen variant of pulmonary carcinoids. As carcinoid tumours have hypometabolic activity, they usually have lower 18‐fluorodeoxyglucose (18F‐FDG) uptake than expected for lung carcinoma on positron emission tomography (PET). Case Report: A 45‐year‐old non‐smoking man had a stable solitary pulmonary nodule followed for 6 years; the tumour remained the same size (1.5 × 2.4 cm) during this period. The patient was admitted to the hospital with complaints of repetitive sneezing and rhinorrhoea. He also experienced flushing and bronchospasm. His chest X‐ray revealed a minimal increase in the size of the solitary pulmonary nodule (2.0 × 2.8 cm). In PET‐computed tomography (CT), the parenchymal nodule in the anterior segment of the right lung had a standard uptake value of 38.0 mg/mL, which was interpreted as a malignant nodule. He underwent fibre‐optic bronchoscopy, but cytology showed no evidence of malignancy. Right upper and middle bilobectomy was performed, and a pulmonary carcinoid tumour with an oncocytic subgroup was diagnosed. The diagnosis of carcinoid syndrome was further confirmed by an elevated 24‐h urinary excretion of 5‐hydroxyindoleacetic acid. Conclusion: We present a rare case of an oncocytic carcinoid tumour with an increase in the size of a solitary pulmonary nodule after 6 years' follow‐up. In addition, PET‐CT showed a very high 18F‐FDG uptake in this patient, which is an unexpected finding with a pulmonary carcinoid tumour. Please cite this paper as: Turan O, Ozdogan O, Gurel D, Onen A, Kargi A and Sevinc C. Growth of a solitary pulmonary nodule after 6 years diagnosed as oncocytic carcinoid tumour with a high 18F‐FDG uptake in positron emission tomography‐computed tomography (PET‐CT). Clin Respir J 2013; 7: e1–e5.     

Right Ventricular Remodeling after Transcatheter Closure of Atrial Septal Defect

Background: Right ventricular (RV) volume overload is a well‐known cardiac consequence of atrial septal defect (ASD) shunt, accounting for most of its long‐term complications. Thus cardiac volumetric unloading is a major aim of transcatheter ASD closure. We set to study the right ventricular remodeling after transcatheter ASD closure in patients with secundum ASD. Methods: We enrolled 46 patients who underwent successful transcatheter closure of ASD. We performed routine transthoracic echocardiographic studies, including three‐dimensional echocardiography and right ventricular myocardial performance index (RVMPI), before transcatheter ASD closure, and 3 days, 1 month after transcatheter ASD closure. Results: We found that: (1) the right ventricular end‐diastolic volume (RVEDV) and right ventricular end‐systolic volume (RVESV) (respectively 106.54±25.97 vs 69.78±10.46 mL, P < 0.05; 59.73±17.59 vs 33.84±7.18 mL, P < 0.05) were enlarged in patients with ASD compared with those in control subjects, resulting in a marked decrease of the right ventricular ejection fraction (RVEF) (44.82%±4.51% vs 54.11%±5.89%, P < 0.05) from normal values; (2) the isovolumic relaxation and isovolumic contraction times (respectively [77.61±16.49] ms vs (64.09±11.82) ms, P < 0.05; [28.04±9.57] ms vs [20.45±6.53] ms, P < 0.05) were prolonged and ejection time ([250.02±24.21] ms vs [272.73±20.51] ms, P < 0.05) was shortened in patients with ASD compared with that in control subjects, resulting in a marked increase of the MPI (0.41±0.07 vs 0.31±0.05, P < 0.05) from normal values; and (3) after transcatheter closure, the RVEDV and RVESV decreased and the RVEF increased markedly and RVMPI decreased markedly. Conclusions: Transcatheter closure of ASD results in rapid normalization of RV volume overload and improvement of RV function. (ECHOCARDIOGRAPHY, Volume 26, November 2009)     

Percutaneous pulmonary valve implantation preceded by routine prestenting with a bare metal stent

Objectives: To evaluate the effectiveness and safety of percutaneous pulmonary valve implantation (PPVI) with routine prestenting with a bare metal stent (BMS). Background: PPVI is a relatively new method of treating patients with repaired congenital heart disease (CHD). Results of PPVI performed with routine prestenting have never been reported. Methods: Consecutive patients who underwent PPVI for homograft dysfunction with prestenting with BMS were studied. The schedule of follow‐up assessment comprised clinical evaluation, cardiovascular magnetic resonance, transthoracic echocardiography, and chest X‐ray to screen for device integrity. Results: PPVI was performed with no serious complications in all patients (n = 10, mean age 26.8 ± 4.0 years, 60% males). In nine patients with significant pulmonary stenosis, peak right ventricular outflow tract (RVOT) gradient was reduced from a mean of 80.6 ± 22.7 to 38.8 ± 10.4 mm Hg on the day following implantation (P = 0.001). At 1‐month and 6‐month follow‐ups, mean RVOT gradient was 34.0 ± 9.8 and 32.0 ± 12.2 mm Hg, respectively. In patients with significant pulmonary regurgitation, mean pulmonary regurgitation fraction decreased from 19% ± 6% to 2% ± 1% (P = 0.0008). Relief of RVOT obstruction and restoration of pulmonary valve competence were associated with significant decrease in right ventricular (RV) end‐diastolic and end‐systolic volumes (125.5 ± 48.6 to 109.2 ± 42.9 mL/m²; P = 0.002 and 68.4 ± 41.5 vs. 50.9 ± 40.6 mL/m²; P = 0.001) as well as improvement in RV ejection fraction (48.8% ± 13.1% to 57.6% ± 14.4%; P = 0.003) and New York Heart Association class (P = 0.003). All patients completed 6‐month follow‐up. No stent fractures were observed. Conclusions: PPVI with routine prestenting with BMS is a safe and effective method of treatment in patients with repaired CHD. © 2010 Wiley‐Liss, Inc.     

联合检测二维超声心动图、BNP、6MWT、MMRC对慢性阻塞性肺疾病急性加重期患者右心功能不全的诊断意义

目的探讨联合检测二维超声心动图、B型脑钠肽(BNP)、6 min步行试验(6WMT)、呼吸困难量表(MMRC)对慢性阻塞性肺疾病急性加重期(AECOPD)患者右心功能不全的诊断意义。 方法选取2016年5月至2018年5月我院急诊科收治临床确诊的AECOPD患者作为研究对象,分别收集患者一般资料及进行BNP、6MWT、MMRC、二维超声心动图实验指标测定,统计学分析二维超声心动图指标与BNP、6MWT、MMRC联合检测对AECOPD患者右心功能不全的诊断差异。 结果共收集AECOPD患者249例,以四项试验指标中任一项阳性结果判断入组患者右心功能不全,则阳性94例(37.8%),正常者155例(62.2%)。统计学分析,显示二维超声心动图与BNP、6MWT、MMRC判定AECOPD患者发生右心功能不全之间有统计学差异(P<0.05),二维超声心动图指标与BNP、6MWT、MMRC中任一项联合检测,二维超声指标+BNP组合优于其他组合(同时阳性和同时阴性所占百分比分别为：67.9%,42.6%,54.2%)。二维超声心动图指标联合BNP、6MWT、MMRC中任两项检测,BNP+6MWT+二维超声指标优于其他组合(结果同时阳性和同时阴性所占百分比分别为：44.6%,36.1%,42.2%)。 结论联合检测二维超声心动图、BNP、6WMT、MMRC对AECOPD患者右心功能不全诊断效果优于单一检测指标,其中BNP+超声指标组优于其他组合,可作为COPD患者合并右心功能不全诊断首选组合方案。     

Prognostic role of biomarkers and right ventricular dysfunction in pulmonary embolism

Pulmonary embolism is morbidity and mortality remain high. The short-term prognosis of pulmonary embolism depends on haemodynamic status and underlying disease massive pulmonary embolism, defined as pulmonary embolism that is associated with systemic hypotension which increases up to 50% in hospital mortality. However, the in-hospital mortality of patients with normal blood pressure pulmonary embolism has been reported to vary from 3 to 15%. Right ventricular dysfunction showed by echocardiography or computerized tomography and elevated cardiac enzymes have been used to determine mortality in these patients with pulmonary embolism. In this article; we reviewed the prognostic value of right ventricular dysfunction as evaluated by echocardiography or spiral computerized tomography, and the prognostic value of increased levels of cardiac markers in patients with pulmonary embolism.     

Non-invasive diagnostic and functional evaluation of cardiac involvement in patients with systemic sclerosis

Elevated serum brain natriuretic peptide (BNP) released from myocytes of ventricles upon stretch have been found in patients with isolated right ventricular (RV) pressure overload. However, limited data suggest that serum BNP may be elevated in systemic sclerosis (SSc) patients, especially with RV dysfunction. We assessed serum N-terminal proBNP (NT-proBNP) in SSc and evaluated whether it reflects the severity of RV overload. We prospectively studied 51 consecutive patients (47F, mean age 53.3 +/- 15.2 years) with SSc (mean disease duration 9 +/- 12.4 years). The control group formed 31 healthy subjects (27F, mean age 52.6 +/- 12.1 years). NT-proBNP level, 6-minute walking test (6MWT), and transthoracic echocardiography (TTE) for the assessment of RV overload were performed. Serum NT-proBNP exceeded the reference value of 125 pg/mL in 31 (61%) SSc patients. The mean serum log NT-proBNP concentration in SSc was higher than in controls (2.138 +/- 0.527 vs. 1.634 +/- 0.420 pg/mL, p < 0.001). 13 (25%) SSc patients have tricuspid regurgitation peak gradient (TRPG) exceeding 31 mmHg reflecting pulmonary arterial hypertension (PAH). The SSc presented other echocardiographic signs of RV overload. Mean 6MWT distance was shorter in SSc than in controls (528 +/- 100 vs. 617 +/- 80 m, p < 0.001). NT-proBNP level correlated positively with TRPG, RV diameter, RV Tei index and negatively with 6MWT distance. ROC analysis identified >115 pg/ml as the best NT-proBNP threshold predicting PAH for SSc patients (sensitivity 92%, specificity 44%). Results of our study suggest that NT-proBNP measurement is a useful screening method for PAH in SSc patients. Since elevated plasma NT-proBNP level reflects the degree of right ventricular overload and limitation of exercise capacity, abnormal NT-proBNP levels should imply further evaluation including echocardiography.     

Long‐Term Exposure to Biomass Fuel and Its Relation to Systolic and Diastolic Biventricular Performance in Addition to Obstructive and Restrictive Lung Diseases

Background: Previous studies have demonstrated an increased risk for cardiovascular events and pulmonary disease in patients with biomass fuel exposure (BFE). However, biventricular heart function has yet to be investigated in these patients. Left ventricular (LV) myocardial performance index (LVMPI), which is an index of global ventricular function, incorporates ejection, isovolumic relaxation, and contraction times. In this study, pulmonary function and biventricular heart function were investigated in nonsmoking female patients with BFE. Methods: Our study population consisted of 46 female patients with BFE (group 1) and 31 control subjects (group 2). Pulmonary function tests and transthoracic echocardiographic examination were performed. Right ventricular myocardial performance index (RVMPI) and LVMPI were obtained by tissue Doppler imaging echocardiography (TDI). Results: BFE caused obstructive and restrictive spirometric impairments. RVMPI was higher in group 1 (0.55 ± 0.07) than group 2 (0.46 ± 0.06) (P = 0.042) and LVMPI was higher in group 1 (0.54 ± 0.08) than group 2 (0.47 ± 0.05) (P = 0.032). Also, pulmonary artery systolic pressure was higher in group 1 than group 2 (P = 0.02). Conclusions: BFE causes both obstructive and/or restrictive lung disease and systolic and diastolic biventricular dysfunction. Nonetheless, long‐term studies are needed to understand on BFE‐related ventricular dysfunctions and to document subsequent cardiovascular events. (Echocardiography 2011;28:52‐61)     

Plasma Brain Natriuretic Peptide Levels Increase in Proportion to the Extent of Right Ventricular Dysfunction in Pulmonary Hypertension

Objectives. This study sought to investigate the influence of right ventricular (RV) hemodynamic variables and function on the secretion of brain natriuretic peptide (BNP) in patients with isolated RV overload.Background. Plasma BNP is known to increase in proportion to the degree of left ventricular (LV) overload. However, whether BNP secretion is also regulated in the presence of RV overload remains unknown.Methods. Plasma BNP and atrial natriuretic peptide (ANP) levels in the pulmonary artery were measured in 44 patients with RV overload: 18 with RV volume overload (RVVO) due to atrial septal defect and 26 with RV pressure overload (RVPO) due to primary or thromboembolic pulmonary hypertension. Right heart catheterization was performed in all patients. RV and LV ejection fraction, myocardial mass and volume of the four chambers were determined by using electron beam computed tomography.Results. Although both plasma BNP and ANP levels were significantly elevated in patients with RV overload compared with values in control subjects, plasma BNP and the BNP/ANP ratio were significantly higher in patients with RVPO than with RVVO (BNP 294 ± 72 vs. 48 ± 14 pg/ml; BNP/ANP 1.6 ± 0.2 vs. 0.8 ± 0.2, both p < 0.05). Plasma BNP correlated positively with mean pulmonary artery pressure (r = 0.73), total pulmonary resistance (r = 0.79), mean right atrial pressure (r = 0.79), RV end-diastolic pressure (r = 0.76) and RV myocardial mass (r = 0.71); it correlated negatively with cardiac output (r = −0.33) and RV ejection fraction (r = −0.71). Plasma BNP significantly decreased from 315 ± 120 to 144 ± 54 pg/ml with long-term vasodilator therapy (total pulmonary resistance decreased from 23 ± 4 to 15 ± 3 Wood U).Conclusions. Plasma BNP increases in proportion to the extent of RV dysfunction in pulmonary hypertension.     

Beneficial Effects of Atorvastatin on Lung Structural Remodeling and Function in Ischemic Heart Failure

BackgroundStudies have suggested some benefit of 3-hydroxy-3-methyl-glutaryl-CoA reductase inhibitors in congestive heart failure (CHF), although the mechanisms remain uncertain. We hypothesized that statins could improve pulmonary hypertension and right ventricular function in ischemic CHF by reducing lung remodeling.Methods and ResultsTwo weeks after myocardial infarct, rats received atorvastatin (n = 23) or no treatment (n = 23) for 3 weeks and were compared with a sham group (n = 16). Infarct size was similar by echocardiography and pathologic evaluations. Atorvastatin greatly reduced pulmonary hypertension and right ventricular hypertrophy: right ventricular systolic pressure 42 ± 5 vs. 28 ± 2 mm Hg (P < .01). Atorvastatin did not reduce left ventricular fibrosis and had minimal effects on left ventricular function. Right ventricular myocardial performance index was markedly improved by therapy (P < .01). CHF caused a restrictive lung syndrome with a downward shift of the respiratory pressure-volume loop, increased dry lung weight, and interstitial fibrosis that were greatly improved by atorvastatin. Reduced lung nitric oxide synthase expression was normalized by treatment. Atorvastatin also reduced isolated lung myofibroblasts proliferation after transforming growth factor–β stimulation (-36 ± 6%, P < 0.01).Conclusions3-hydroxy-3-methyl-glutaryl-CoA reductase inhibition reduces lung remodeling and dysfunction associated with heart failure with prevention of right ventricular hypertrophy and pulmonary hypertension.