Clinical course of arrhythmogenic right ventricular cardiomyopathy with end-stage heart failure and outcome after heart transplantation.

BACKGROUND: Few data exist on the characteristics and outcomes of patients with arrhythmogenic right ventricular cardiomyopathy and advanced heart failure who undergo heart transplantation. AIM: To explore the pretransplant course and outcomes of patients with arrhythmogenic right ventricular cardiomyopathy after heart transplantation. METHODS: This observational retrospective monocentric study included all consecutive patients with arrhythmogenic right ventricular cardiomyopathy who underwent heart transplantation during a 13-year period (2006-2019) at Pitié-Salpêtrière University Hospital (Paris). RESULTS: A total of 23 patients with arrhythmogenic right ventricular cardiomyopathy underwent heart transplantation between 2006 and 2019. The median time from diagnosis to heart transplantation was 9 years, and the median age at transplantation was 50 years. At diagnosis, half of the patients had left ventricular dysfunction, 59% had extensive T-wave inversion and 43% had a history of sustained ventricular tachycardia. Only five patients were involved in intensive sport activity. Indications for heart transplantation were end-stage biventricular dysfunction in 13 patients, end-stage right ventricular heart failure in seven and electrical storm in three. Only three patients had pulmonary hypertension, and half of the patients had atrial arrhythmias. The survival rate 1 year after heart transplantation was 74% (95% confidence interval 53-88%). Eight patients experienced primary graft dysfunction needing extracorporeal membrane oxygenation. CONCLUSIONS: Patients with arrhythmogenic right ventricular cardiomyopathy who eventually needed heart transplantation mostly exhibited extended disease with biventricular dysfunction at diagnosis. Intensive sport activity did not seem to be a major determinant. Advanced heart failure usually occurred late in the course of the disease. Primary graft dysfunction after heart transplantation was frequent, and should be anticipated. Additional data are needed to identify the optimal timing for heart transplantation and predictors of end-stage heart failure in patients with arrhythmogenic right ventricular cardiomyopathy.

Added Value of Aortic Pulse Wave Velocity Index in a Predictive Diagnosis Decision Tree of Coronary Heart Disease.

BACKGROUND: Coronary heart disease (CHD) is among the main causes of death in the world. Individual study of cardiovascular risk is an important way to predict CHD risk. The aim of this study was to evaluate the added role of the aortic pulse wave velocity (PWV) index in the prediction of CHD risk. METHODS: A cross-sectional study was conducted from December 2012 to September 2017; 530 patients were included: 99 CHD, 338 non-CHD patients, and 93 nonhypertensives, nondiabetics and non-CHD subjects, whose theoretical PWV were calculated. Theoretical PWV was calculated according to age, blood pressure, gender, and heart rate. The results were expressed as an index ((measured PWV - theoretical PWV)/theoretical PWV) for each patient. The differences observed, the differential diagnostic performance, and the quantification of the added value of diagnostic performance of PWV index were tested using logistic regression, comparisons between receiver operating characteristic (ROC) curves, and decision tree nonlinear methodology. RESULTS: PWV index (P = 0.006), carotid plaque (P = 0.005), and dyslipidemia (P = 0.04) were the independent modulators of CHD diagnosis. PWV index appears to be the highest specific classifier (81%) compared to carotid plaque (75%) and dyslipidemia (78%). For the decision tree, sensitivity, specificity, and area under the ROC curve for CHD diagnosis were 62%, 83%, and 0.87, respectively. CONCLUSIONS: PWV index yielded added value to CHD by assessment of combined classifiers with clinical determinants and decision tree construction and significantly increased the specificity of the differential diagnostic performances of the common risk factors of CHD in daily clinical practice.

Atherothrombotic out-of hospital cardiac arrest: watch out for a second train.

A 63-year-old man presented with an out-of-hospital cardiac arrest. The coronary angiogram with intravascular imaging was performed and showed a mid-left anterior descending artery atherosclerotic cap rupture with thrombus burden treated with drug-eluting stent (DES) implantation. During the hospitalisation in the intensive care unit, the patient experienced recurrent ventricular fibrillations and asystole with transient ST-segment elevation in the inferior leads with normal coronary angiography. A methylergonovine provocative test was therefore performed and showed an occlusive right coronary artery (RCA) spasm. Due to recurrent RCA spasm, the patient was treated with DES implantation with favourable results at 3-month follow-up.