Case Report: Left bundle branch pacing in an amyloid light-chain cardiac amyloidosis patient with atrioventricular block.

Introduction: Amyloid light-chain cardiac amyloidosis is a progressive infiltrative disease characterized by the deposition of amyloid fibrils in the cardiac tissue, which can cause serious atrioventricular block requiring pacemaker implantation. Left bundle branch pacing has emerged as an alternative method for delivering physiological pacing to achieve electrical synchrony of the left ventricle. However, left bundle branch pacing in patients with amyloid light-chain cardiac amyloidosis has not been studied in detail. Therefore, in this study, we present a case of left bundle branch pacing in a patient with amyloid light-chain cardiac amyloidosis. Case summary: A 66-year-old male patient with amyloid light-chain cardiac amyloidosis presented with syncope for 1 month. Holter monitoring revealed intermittent third-degree atrioventricular block. Left bundle branch pacing was performed successfully. During the 1-year follow-up, it was observed that the left bundle branch capture threshold remained stable without any pacemaker-related complications or left ventricle systolic dysfunction, and there was no recurrence of syncope. Conclusion: Left bundle branch pacing appears to be a safe and feasible option for patients with amyloid light-chain cardiac amyloidosis experiencing atrioventricular block.

Case report: Recurrent syncope as initial symptom in a patient with neck lymphoma.

Syncope may have many different causes, requiring careful identification. Recurrent syncope is uncommon as an initial symptom of neck lymphoma. Head and neck tumors involving the carotid artery cause syncope associate with carotid sinus syndrome. We report the case of a 72-year-old man who suffered from recurrent syncope due to compression of the right carotid sinus by diffuse large B-cell lymphoma and was successfully treated with immunochemotherapy. Syncope may be an early or sole sign of a neck or head tumor. We should be aware of the possibility of an underlying malignancy in patients with unexplained syncope after initial evaluation.

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. METHODS: A total of 20 consecutive patients (7 men, mean age 50 ± 12 years) referred for endomyocardial biopsy (EMB) were included. Eleven of these patients (55%) confirmed primary CA, the rest of 9 patients were EMB negative and used for the control. RESULTS: The voltage of S(V1) + R(V6) < 1.2 mV has a sensitivity of 91% and specificity of 89% for the identification of primary CA, yields the positive and negative predictive values of 91% and 89%, respectively. Among ECHO parameters, there were no significant differences between the 2 groups, except for left ventricular ejection fraction (47 ± 12% in primary CA vs 67 ± 11% in the control, P < 0.001). However, the combined indexes of ECG and ECHO parameters, including the ratio of R(I) /LVPW as well as R(V5) /LVPW and R(V6) /LVPW, were significantly lower in the patients with primary CA than the control. The ratio of R(I) /LVPW < 0.4 has the sensitivity of 91% and specificity of 100%, yields the positive and negative predictive values of 100% and 91%, respectively. The ratios of R(V5(6)) /LVPW < 0.7 have the sensitivity of 91% and specificity of 89%, yield the positive and negative predictive values of 91% and 89%, respectively. CONCLUSION: Patients with clinically suspected primary CA, combined indexes of ECGs and ECHOs could be used as the noninvasive diagnostic tools.

[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.

[Electrocardiographic and echocardiographic features of patients with primary cardiac amyloidosis].

OBJECTIVE: To summarize the electrocardiography and echocardiography features of patients with cardiac amyloidosis (CA) diagnosed by endo-myocardial biopsy (EMB). METHODS: A total of 20 consecutive patients [7 men, mean age (50 ± 12) years] referred for EMB because of clinical suspicion of CA from September 2006 to October 2009 were included in the study. Primary CA was diagnosed in 11 out of 20 patients (55%) by EMB and biomarkers examination. The electrocardiography and echocardiography features were analyzed. RESULTS: The voltage of all the limb leads were low in the 11 CA patients [mean values of (0.33 - 0.51) mV], the incidence of low voltage and pseudo-infarction patterns were 45% and 45%, respectively. Concentric hypertrophy and normal left ventricular diameters were evidenced in all CA patients on echocardiography, left atrial enlargement (n = 10, 91%), granular/sparking appearance of the myocardium (n = 9, 82%) and moderate to large pericardial effusion (n = 7, 64%) as well as left ventricular systolic dysfunction (n = 8, 73%) were often presented in CA patients. CONCLUSIONS: The diagnosis of primary CA should be considered in patients with unknown origin of heart failure, concentric hypertrophy and normal left ventricular diameters with granular/sparking appearance of the myocardium or pericardial effusion presented on echocardiography and low voltage of limb leads or pseudo-infarction pattern presented on electrocardiography. EMB and serum (urine) biomarkers examinations should be then performed to confirm or exclude the diagnosis of CA.

[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.