Hemodynamic parameters obtained by transthoracic echocardiography and right heart catheterization: a comparative study in patients with pulmonary hypertension.

BACKGROUND: Hemodynamic evaluation is crucial for the management of patients with pulmonary hypertention. Clinicians often prefer a rapid and non-invasive method. This study aimed to examine the feasibility of transthoracic echocardiography for the measurements of hemodynamic parameters in patients with pulmonary hypertension. METHODS: A prospective single-center study was conducted among 42 patients with pulmonary hypertension caused by different diseases. Transthoracic echocardiography and right-heart catheterization were performed within 24 hours. Pulmonary artery systolic, diastolic and mean pressure (PASP, PADP and PAMP), cardiac output (CO), and pulmonary capillary wedge pressure (PCWP) were measured by both methods. A linear correlation and a Bland-Altman analysis were performed to compare the two groups of hemodynamic parameters. RESULTS: A good correlation was found between invasive and non-invasive measurements for PASP (r = 0.96), PADP (r = 0.85), PAMP (r = 0.88), CO (r = 0.82), and PCWP (r = 0.81). Further agreement analysis done by the Bland-Altman method showed that bias and a 95% confidence interval for PASP, PADP, and CO were clinically acceptable while great discrepancies existed for PAMP and PCWP. CONCLUSIONS: The non-invasive measurements by PASP, PADP, and CO in patients with pulmonary hypertension correlate well with the invasive determinations. Transthoracic echocardiography (TTE) was inappropriate for estimating PCWP and PAMP.

[Value of cardiac magnetic resonance imaging for the diagnosis of cardiac amyloidosis].

OBJECTIVE: To observe the clinical features and cardiac magnetic resonance imaging (CMR) characteristics of patients with endomyocardial biopsy (EMB)-proven cardiac amyloidosis (CA). METHODS: EMB proven CA patients underwent CMR examination from September 2006 to December 2010 were included. The findings of clinical manifestation, electrocardiogram, echocardiography and CMR were analyzed. RESULTS: Among the 18 patients with EMB verified CA, 5 patients underwent CMR. All 5 patients had heart failure symptoms and electrocardiogram was abnormal. Echocardiogram showed concentric left ventricular hypertrophy, granular appearance of the myocardium, left atrial enlargement and moderate to severe left ventricular diastolic dysfunction. CMR revealed increased thickness of the left ventricular wall (especially at the inter-ventricular septum), enlarged bilateral auricle, restricted left ventricular filling with normal or mild to moderate reduced systolic function. Pleural and pericardial effusions were observed in 2 patients. Abnormal late gadolinium enhancement (LGE) was detected in all 5 patients. CMR revealed different patterns of LGE. Left ventricular global subendocardial delayed gadolinium enhancement or transmural delayed gadolinium enhancement were found, and patients also showed line-, granular- or patchy-like enhancement. The degree and range of LGE paralleled the disease course and were consistent with electrocardiogram changes. CONCLUSIONS: As a noninvasive diagnostic tool, CMR is valuable in the diagnosis of CA. For patients with clinical suspicion of CA, CMR could be a helpful diagnostic tool, especially in the hospitals where EMB is not available.

[Clinico-pathological evaluation of restrictive cardiomyopathy].

OBJECTIVE: restrictive cardiomyopathy (RCM) is characterized by impairment of ventricular filling during diastole with preserved systolic function. The clinical and histopathological profile on endomyocardial biopsy of 25 consecutive patients with RCM was analyzed in this study. METHODS: twenty-five patients with diagnosis of RCM and underwent endomyocardial biopsy (EMB) were enrolled in the study. The clinical characteristics, electrocardiogram, serum chemistry, right heart catheter and cardiac pathology results were obtained. RESULTS: heart failure symptom was present in all 25 patients and left ventricular size and function were normal or near normal while serum brain natriuretic peptide (577 pg/ml) was moderately elevated. Right atrial and ventricular end-diastolic as well as pulmonary capillary wedge pressures derived from right heart catheter examination were increased. Amyloid deposition were evidenced in 16 and eosinophilic myocarditis in 2 patients upon pathological examination of EMB. In the remaining 7 patients, 3 were diagnosed idiopathic RCM, 2 were diagnosed as amyloidosis by biopsy from non-cardiac tissue and etiology remained unknown in 2 patients. Thus, conclusive diagnosis was made on EMB samples in 84% (21/25) patients of RCM. CONCLUSION: RCM may result from various local and systemic disorders. EMB is helpful for identifying the underlying etiology.

[The clinico-pathological manifestation of cardiac involvement in eosinophilic diseases].

OBJECTIVE: To investigate the clinical and pathological features of eosinophilic diseases with cardiac involvement. METHODS: We analyzed the clinical and cardiac pathological data of 7 patients with eosinophilic diseases with cardiac involvement under endomyocardial biopsy or autopsy. RESULTS: Seven patients (5 male, average age 51 years) were enrolled. Four patients were diagnosed as idiopathic hypereosinophilic syndrome and three were Churg-Strauss syndrome. Peripheral blood eosinophilia count increased significantly in all patients. Cardiac involvement included angina pectoris, myocardial infarction, heart failure, presyncope and sudden death. Electrocardiogram showed cardiac ischemia, bundle branch block and third degree atrioventricular block. Echocardiography suggested ventricular and atrial enlargement, decreased ventricular systolic function, pulmonary hypertension, valvular prolapse and insufficiency and endocarditis. Pathology displayed infiltration of eosinophils, formation of granulomata, necrotizing vasculitis, myocardial necrosis and endomyocardial fibrosis in heart. Coronary artery could be also affected and led to myocardial infarction. CONCLUSIONS: Cardiovascular complications of eosinophilic diseases are a major source of morbidity and mortality in these disorders. The manifestations are multiple and early recognition and treatment with steroid and immunosuppressant can improve prognosis.

Primary prevention of sudden cardiac death by implantable cardioverter-defibrillator therapy in Chinese patients with heart failure: a single-center experience.

BACKGROUND: An implantable cardioverter-defibrillator (ICD) has been suggested for heart failure patients for primary prevention of sudden cardiac death. However, few data have been reported on the application of ICD as primary prevention of sudden cardiac death in China. We evaluated the value of primary prevention ICD therapy in Chinese patients with heart failure. METHODS: Thirty-four patients at an average age of (60.2 +/- 13.7) years seen in Peking Union Medical College Hospital were treated with ICD implantation for primary prevention of sudden cardiac death from November 2005 to July 2009. Single-chamber ICDs were implanted in 16 (47.0%) cases, and dual-chamber or cardiac resynchronization therapy defibrillators in 18 (53.0%) cases. The patients had an average left ventricular ejection fraction of (26.9 +/- 5.5)% (11% to 35%), of which 18 (53.0%) patients had ischemic cardiomyopathy and 16 (47.0%) patients had non-ischemic cardiomyopathy. All patients were followed up at three months after the implantation and every six months thereafter or when prompted by an ICD event. RESULTS: There were five (14.7%) deaths, including two of heart failure and three with a non-cardiac course, during an average follow-up of (15.0 +/- 11.9) months. Forty-one ICD therapy events were recorded, including 19 (46.3%) appropriate ICD therapies in six patients and 22 (53.7%) inappropriate ICD therapies in four patients with single chamber leads. Inappropriate ICD therapies were mainly due to supraventricular tachyarrhythmias, especially atrial fibrillation. Patients with ischemic cardiomyopathy and non-ischemic cardiomyopathy did not differ in the incidence of either appropriate or inappropriate therapy. CONCLUSIONS: ICD for primary prevention of sudden cardiac death in China prevents patients from arrhythmia death. Relatively high incidence of inappropriate therapies highlights the importance of an atrial lead.

Importance of endomyocardial biopsy in unexplained cardiomyopathy in China: a report of 53 consecutive patients.

BACKGROUND: Although endomyocardial biopsy (EMB) plays a crucial role in the final diagnosis in patients with heart failure of unknown etiology, the invasive nature of this technique limits its clinical application in China. The purpose of this study was to evaluate the clinical application of EMB in diagnosing cardiomyopathy with unexplained etiologies in China. METHODS: Fifty-three consecutive patients (38 males, age 14 - 67 years, median 43 years) were included in the study who were initially diagnosed as unexplained cardiomyopathy and under EMB biopsy in Peking Union Medical College Hospital from 2006 to 2009. The patients were clinically divided into four groups: dilated, hypertrophic, restrictive and unclassified cardiomyopathy. Biopsies were performed via right internal jugular vein with the use of the bioptome under fluoroscopic guidance. Three to five endomyocardial samples were taken from each patient for light microscopy examination and one sample for electron microscopy was taken if necessary. For each patient, an initial clinical diagnosis, an EMB diagnosis and a final diagnosis prior to discharge were established. All the data were compared and analyzed for the evaluation of clinical utility of EMB in China. RESULTS: In 26 patients initially diagnosed with restrictive cardiomyopathy (RCM), the etiology of the condition was finally diagnosed using EMB in 15; including 13 amyloidosis and two eosinophilic myocarditis. We employed EMB in 19 patients clinically diagnosed as dilated cardiomyopathy and detected viral myocarditis in one patient, cardiac involvement due to polymyositis in four and doxorubicin-induced cardiomyopathy in one. In five patients with severe left ventricle hypertrophy undergoing EMB, one patient was diagnosed as autophagic vacuolar cardiomyopathy and one as mitochondrial disease. In the remaining three patients with unclassified cardiomyopathy, EMB revealed infiltration of eosinophils as the cause of atrial ventricular block in one patient. Final diagnoses were made in 24 of the total 53 patients (45%) based on the combination of EMB and clinical data. Transient atrial ventricular block in a patient with prior complete left bundle branch block was the only complication occurred during the procedures. CONCLUSION: The clinical application of EMB is safe. The combination of EMB and clinical data produced a better understanding of the mechanisms behind the clinically diagnosed cardiomyopathy in China.

[Comparison of adenosine versus iloprost by acute vasodilator test in patients of pulmonary arterial hypertension complicated with connective tissue diseases].

OBJECTIVE: To compare the acute hemodynamic effects of adenosine versus iloprost in patients of pulmonary arterial hypertension (PAH) complicated with connective tissue diseases. METHODS: During right heart catheterization, 18 patients of PAH complicated with connective tissue diseases sequentially received intravenous infusion of adenosine and inhaled iloprost. After the baseline hemodynamic data were obtained, an adenosine infusion was started and titrated to the maximal tolerated dose. The hemodynamic parameters were allowed to return to baseline. Then inhalation of iloprost was administered. The effects of both medicines on the patient's hemodynamics were monitored. RESULTS: As compared with the baseline values, the systolic pulmonary artery pressure and pulmonary vascular resistance significantly decreased [(71 +/- 30) vs (80 +/- 29) mm Hg and (712 +/- 440) vs (824 +/- 464) dyn x s x cm(-5) respectively, both P < 0.05) while the heart rate increased significantly [(93 +/- 17) vs (83 +/-16) beat/min, P < 0.05] in the adenosine group. Inhaled iloprost could also lower the systolic pulmonary artery pressure [(66 +/- 29) vs (79 +/- 28) mm Hg, P < 0.05], mean pulmonary artery pressure [(43 +/- 19) vs (52 +/- 19) mm Hg, P < 0.05] and pulmonary vascular resistance [(632 +/- 440) vs (816 +/- 448) dyn x s x cm(-5), P < 0.05] without any effect upon heart rate. Inhaled iloprost exerted more potent effect on lowering mean pulmonary artery pressure and pulmonary vascular resistance than adenosine (P < 0.05). The two medicines did not affect cardiac output, pulse oxygen saturation or systemic blood pressure. The side effects were fewer in the iloprost inhalation group than the adenosine group. CONCLUSION: During acute vasodilator testing, inhaled iloprost was more potent than infused adenosine as a pulmonary vasodilator in PAH complicated with connective tissue diseases.