Ligang Fang

Danon disease as a cause of concentric left ventricular hypertrophy in patients who underwent endomyocardial biopsy

BACKGROUND Danon disease is an X-linked dominant disorder; concentric left ventricular hypertrophy (LVH) is one of its manifestations. In this study, we investigated the prevalence of Danon disease in patients with concentric LVH who underwent endomyocardial biopsy (EMB). METHODS AND RESULTS A total of 50 patients with concentric LVH underwent EMB from January 2008 to December 2010. Cardiac amyloidosis was diagnosed in 14 patients; genetic analysis of lysosome-associated membrane protein 2 (LAMP2) was done in the remaining 36 patients. Three novel LAMP2 frameshift mutations were found. They were c.808_809 insG in exon 6, c.320_321 insCATC in exon 3, and c.257_258delCC in exon 3, leading to a premature stop codon on cDNA analysis. The prevalence of Danon disease was seen in 6% (3 of 50) of unselected concentric LVH patients who underwent EMB, or 8% (3 of 36) after excluding cardiac amyloidosis through EMB. All the three patients were male teenagers with a mean age of 15 ± 1 years, and had mild mental retardation, two of the three with Wolff-Parkinson-White (WPW) syndrome and markedly increased left ventricular voltage. All the three patients had increased serum hepatic enzymes and creatine kinase (CK) concentrations. There was no death or cardiovascular hospitalization during 20 ± 15 months of follow-up. CONCLUSIONS Danon disease may account for a number of patients with concentric LVH who underwent EMB. Danon disease should be suspected in the male teenager with concentric LVH, especially with elevated serum hepatic enzymes and CK concentrations, and/or WPW syndrome with markedly increased voltage of the left ventricle. Genetic analysis of LAMP2 can help make the diagnosis.

Utility of Combined Indexes of Electrocardiography and Echocardiography in the Diagnosis of Biopsy Proven Primary Cardiac Amyloidosis

Objective: Primary cardiac amyloidosis (CA) is associated with poor prognosis. However, the noninvasive diagnostic tools are limited. The aim of the study is to assess the utility of combined indexes of electrocardiography (ECG) and echocardiography (ECHO) in the diagnosis of primary CA.

Poor prognosis in chronic heart failure patients with reduced ejection fraction in China.

Most Chinese cardiologists are challenged by the high mortality rate of heart failure (HF) in patients with reduced ejection fraction in China. This study was designed as a single-center, retrospective study. All consecutive HF patients with left ventricular ejection fraction (LVEF) ≤ 45% from January 1, 2007, to December 31, 2009, were enrolled. The primary outcome was all-cause mortality. The secondary outcome was all-cause mortality or the first cardiovascular readmission event. A total of 187 patients comprised the study population, classified into two groups: LVEF ≤ 35% (n=83) and LVEF 36% to 45% (n=104). The median follow-up was 18 months (2-41 months). All-cause mortality was 27% among patients with LVEF ≤ 35%, as compared with 14% among those with LVEF 36% to 45% (P=.025). All-cause mortality or first cardiovascular readmission rates were 53% and 32% among patients with LVEF ≤ 35% and 36% to 45% (P=.003), respectively. The predictors of all-cause mortality were advanced age and New York Heart Association functional class, chronic kidney disease, oral β-blockers, and statins at discharge. The prognosis of chronic HF patients with LVEF ≤ 45% was poor in China, especially for patients with LVEF ≤ 35%. Cardiologists should provide further efforts to improve the prognosis of HF in Chinese patients.

Mitral regurgitation after anthracycline-based chemotherapy in an adult patient with breast cancer: A case report

Rationale: Anthracyclines cardiotoxicity characterized by dilated myocardiopathy has been well described in the literature. However, anthracyclines-induced valvular diseases have been seldom reported. Patient concerns: In this study, we present the case of a 62-year-old Chinese female patient with breast cancer developing severe mitral regurgitation after anthracycline exposure. Diagnoses: The patient was diagnosed with mitral regurgitation with preserved left ventricular ejection fraction and normal cardiac chamber dimensions in the sixth month after the last course of anthracycline-containing chemotherapy. However, continued decrease in LVEF with normal left ventricular wall thickness, and serial increases in left atrial and ventricular dimensions were observed in the follow-up echocardiography. Interventions: Treatments with oral itraconazole at a dose of 75 mg/day and local wound care with ciclopirox olamine ointment were administered. Outcomes: The patient responded well to the treatment with perindopril, metoprolol succinate, spirolactone, and furosemide, and symptoms associated with heart failure were dramatically relieved. Lessons: The incipient mitral regurgitation may serve as an early sign of myocardial dysfunction that can facilitate a timely recognition of cardiotoxicity, which is crucial to a timely change of chemotherapy regimen and an appropriate initiation of antiremodeling therapy that could limit anthracycline cardiotoxicity and improve overall outcome.

Takayasu arteritis presented with acute heart failure: case report and review of literature

Acute heart failure due to myocarditis is not common in Takayasu arteritis, let alone in combination with thrombosis affecting both ventricles and pulmonary arteries. The concomitant infection of non‐tuberculosis mycobacterium further complicates the clinical scenario and poses challenges for implementation of tailored treatments. This case report describes a teenage girl with a history of intermittent claudication and Erythema Nodosum who developed acute heart failure. Detailed clinical investigations and imaging techniques confirmed the diagnosis.

Increased Urokinase-Type Plasminogen Activator Receptor Expression on Circulating Monocytes Is Correlated with Clinical Instability and Long-Term Adverse Cardiac Events in Patients with Coronary Artery Disease.

Objectives: This study sought to investigate the clinical correlates and prognostic roles of urokinase-type plasminogen activator receptor (uPAR) on circulating monocytes in patients with coronary artery disease (CAD). Methods: 263 angina patients were included in this study. The percentage of uPAR expressing monocytes (PUEM) and the mean fluorescence intensity (MFI) index of uPAR were measured using flow cytometry. Patient follow-up was on average 604 days. Major adverse cardiac events (MACE) were defined as a composite of cardiac death, reinfarction, acute heart failure and hospitalization for revascularization. Results: The PUEM and MFI index levels were significantly more elevated in acute coronary syndrome patients than in stable ones. uPAR expressions on circulating monocytes at admission were correlated to inflammatory biomarkers and myocardial necrosis. Logistic regression analysis revealed that PUEM ≥15% (OR 21.96, 95% CI 7.31-65.98, p < 0.001) and uPAR MFI index ≥3.00 (OR 3.54, 95% CI 1.18-10.59, p = 0.024) were independent determinants of clinical instability in patients with CAD. When followed up, a high PUEM level at admission was an independent prognostic parameter for long-term MACE (HR 3.99, 95% CI 1.31-12.11, p = 0.015). Conclusions: uPAR expression on circulating monocytes is associated with clinical instability and myocardial necrosis and independently predicts the risk of MACE in patients with CAD.

Left ventricular strain is associated with acute postoperative refractory hypotension in patients with constrictive pericarditis and preserved ejection fraction

Background Pericardiectomy is an effective treatment for constrictive pericarditis (CP). Early postoperative complications such as refractory hypotension and congestive heart failure occur in these patients and are associated with increased morbidity and mortality. We hypothesized that left ventricular (LV) myocardial strain measured by two-dimensional speckle tracking echocardiography (2DSTE) could identify early cardiac dysfunction and relate to acute postoperative adverse events in CP patients. Methods Forty-four CP patients with preserved left ventricular ejection fraction (LVpEF, 64%±8%) and 44 age- and sex-matched controls were enrolled. Conventional 2DSTE was performed before pericardiectomy. Global and segmental peak systolic strain values were measured. The primary endpoint was a composite of postoperative refractory hypotension, congestive heart failure and cardiogenic death. Refractory hypotension was defined as hypotension requiring prolonged usage of intravenous inotropic medication (IVIM) (≥2 days). Results Postoperative refractory hypotension occurred in 26 cases, and no patients experienced congestive heart failure or cardiogenic death. Compared to controls, CP patients had decreased absolute global and segmental circumferential strain (CS), radial strain (RS), and longitudinal strain (LS) except septal LS. Patients with refractory hypotension exhibited lower epicardial CS (P=0.04). Epicardial CS was an independent risk factor correlated with postoperative adverse outcome [P=0.014, OR =1.236 (1.044-1.464)] while LVEF was not. Lower absolute value of epicardial CS was related to higher (P=0.02) and longer usage of intravenous furosemide (P=0.04) to keep negative fluid balance perioperatively. Conclusions LV strain value is markedly reduced in patients with CP and LVpEF. Lower preoperative epicardial CS value is associated with greater risk of early refractory hypotension and more aggressive fluid management.

Is T1-Mapping Truly Superior to Late Gadolinium Enhancement-Imaging in Demonstrating Myocardial Fibrosis in Myopathy- and Non-Myopathy Associated Noncompaction? – Reply –

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Effect of Right Heart Systolic Function on Outcomes in Patients with Constrictive Pericarditis Undergoing Pericardiectomy.

Background:To determine the influence of right ventricular function in patients with constrictive pericarditis (CP) undergoing surgery and to compare the outcomes of patients who received surgery with those managed medically. Methods:Patients with the diagnosis of CP and healthy volunteers were recruited from January 2006 to November 2011. Patients with CP chose to either receive pericardiectomy or medical management. Echocardiographic measurements were performed to evaluate heart function, and survival was recorded. Results:A total of 58 patients with CP (36 received pericardiectomy, 22 managed medically), and 43 healthy volunteers were included. CP patients who received surgery had a higher survival rate than those managed medically (P = 0.003), and higher survival was also seen in the subgroup of CP patients with severely impaired right systolic function. Albumin level, left ventricular end-diastolic dimension, and tricuspid regurgitation velocity were associated with survival in CP patients who received surgery. Conclusions:Preoperative right heart function does not affect surgical outcomes. Patients with severely impaired preoperative right systolic function obtain a greater survival advantage with surgery than with medical treatment.