Rodrigue Garcia

Apical left ventricular myocardial dysfunction is an early feature of cardiac involvement in myotonic dystrophy type 1.

Left ventricular (LV) dysfunction is a major prognostic determinant in myotonic dystrophy type 1 (DM1). Therefore, markers of early‐stage LV impairment may be useful. The aim of this study was to evaluate 2D echocardiographic LV strain in a cohort of DM1 patients with preserved left ventricular ejection fraction (LVEF) and to compare the results with matched controls.

Twenty-four-hour ambulatory ECG monitoring relevancy in myotonic dystrophy type 1 follow-up: Prognostic value and heart rate variability evolution

Patient prognosis in type 1 myotonic dystrophy (DM1) is very poor. Annual 24‐hour holter ECG monitoring is recommended but its relevance is debated. Main objective was to determine whether holter ECG parameters could predict global death in DM1 patients and secondarily to assess whether they could predict cardiovascular events and sudden cardiac death, to compare DM1 patients and healthy controls, and to assess their evolution in DM1 over a 5‐year period.

Right ventricular longitudinal strain: a tool for diagnosis and prognosis in light-chain amyloidosis

Abstract Objectives: Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis. Methods: We retrospectively and consecutively compared clinical, biological and echocardiographic data of 315 patients with IWT: 105 biopsy-proven AL amyloidosis patients, 105 patients with HCM and 105 patients with HTN. The prognostic value of these parameters was analysed in the AL amyloidosis group. Results: Free-wall right ventricular longitudinal strain (FWRVLS) worse than −21.2% discriminates AL amyloidosis [area under the curve (AUC) = 0.744)] from patients with IWT due to other aetiologies. In AL amyloidosis, FWRVLS is the strongest echocardiographic prognostic marker with AUC =0.722 and −16.5% as the optimal cut-off value, beyond which overall mortality increases significantly. It is also the only independent echocardiographic predictor of overall mortality (HR =1.113; 95%CI 1.029–1.204; p = .007), even when adjusted to the Mayo stage and global left ventricular longitudinal strain. Conclusions: FWRVLS should be considered in the diagnostic and prognostic workup in light-chain amyloidosis.

Cardiac pacing for complete atrioventricular block complicating heart lymphoma: A challenging issue

Primary cardiac lymphoma is a rare tumor that may be difficult to diagnose. Atrioventricular block (AV) is one of the possible clinical presentations. The histological diagnosis must be given right away since the systemic therapy can influence the prognosis. However, treatment of conduction disorders associated with primary cardiac lymphoma has not yet been clearly defined. A 60-year-old patient presenting a transthoracic echocardiography (TTE) for dyspnea. The exam showed a cardiac tamponade, a thickened biventricular wall with a right atrial mass and two masses in the left atrium (Video 1). Emergency pericardial drainage removed 500 cm of hemorrhagic liquid. The day after, 12-lead electrocardiogram (ECG) showed a complete atrioventricular block with a narrow QRS complex and a heart rate of 55 beats per minute. The patient had no episode of syncope. Histopathological examination of the pericardial fluid confirmed a large B-cell non-Hodgkins lymphoma. Full-body CT scan revealed no abnormalities in the other organs, consistent with primary cardiac lymphoma. Treatment was initiated and consisted in R-CHOP (rituximab, cytoxan, adriamycin, vincristine, prednisolone) chemotherapy. After two cycles of treatment (21 days), TTE showed significant tumor size reduction and no pericardial effusion. Despite a good response to the chemotherapy, the AV block persisted (figure 1). Therefore, a Magnetic Resonance Imaging (MRI)-compatible permanent pacemaker (KORA 250 DR, Sorin Group) was implanted and programmed in Safe R mode (AAI-DDD).

Power of resting echocardiographic measurements to classify pulmonary hypertension patients according to European society of cardiology exercise testing risk stratification cut-offs

BACKGROUND Right ventricular function is the major determinant of morbidity and mortality in pulmonary arterial hypertension (PAH). The ESC risk assessment strategy for PAH is based on clinical status, exercise testing, NTproBNP, imaging and haemodynamics but does not include right ventricular function. Our aims were to test the power of resting echocardiographic measurements to classify PAH patients according to ESC exercise testing risk stratification cut-offs and to determine if the classification power of echocardiographic parameters varied in chronic thrombo-embolic pulmonary hypertension (CTEPH). METHODS We prospectively and consecutively recruited 46 PAH patients and 42 CTEPH patients referred for cardio-pulmonary exercise testing and comprehensive transthoracic echocardiography. Exercise testing parameters analyzed were peak oxygen consumption, percentage of predicted maximal oxygen consumption and the slope of ventilation against carbon dioxide production. Receiver operator characteristic curves were used to determine the optimal diagnostic cut-off values of echocardiographic parameters for classifying the patients in intermediate or high risk category according to exercise testing. RESULTS Measurements of right ventricular systolic function were the best for classifying in PAH (area under the curve 0.815 to 0.935). Measurements of right ventricular pressure overload (0.810 to 0.909) were optimal for classifying according to exercise testing in CTEPH. Measurements of left ventricular function were of no use in either group. CONCLUSIONS Measurements of right ventricular systolic function can classify according to exercise testing risk stratification cut-offs in PAH. However, this is not the case in CTEPH where pressure overload, rather than right ventricular function seems to be linked to exercise performance.

Prevalence and outcome of patients referred for chest pain with high-sensitivity troponin elevation and no diagnosis at discharge

Specialized chest pain units appear to increase the proportion of patients with acute chest pain who are properly evaluated, but some of them remain doubtful.

An unusual pacemaker migration

http://dx.doi.org/10.1016/j.ihj.2016.06.003 0019-4832/ 2016 Published by Elsevier B.V. on behalf of Cardiological Society of creativecommons.org/licenses/by-nc-nd/4.0/). Two days after implantation, the patient developed a generatorpocket hematoma due to anticoagulation for paroxysmal atrial fibrillation. The patient underwent initial surgery to drain the pocket hematoma. Subsequent to the development of a generator pocket infection with cutaneous fistulization, the patient was referred to our center. Transesophageal echocardiography did not show evidence of endocarditis. Blood cultures and local samples were also negative. The device was found neither at the clinical examination nor with a programmer on the incision site. Chest Xray confirmed its location in the inferior portion of the left breast (Fig. 1). Device removal was performed with a delto-pectoral and an inferior mammary approach. In light of other research, this is the first case of pacemaker migration on the breast. The most common site of migration is rather the anterior thoracic region. Migration is more frequent in elderly patients who have a loss of collagen and therefore a loss of tissue elasticity. Moreover, pocket hematoma, pocket infection, and excess of fat tissue resulting in non-formation of device capsule are associated with increased risk of migration, which can be prevented by securing the device to the deep pectoral muscles with non-absorbable sutures.