Objective Mitral annular disjunction (MAD) is an abnormality linked to mitral valve prolapse (MVP), possibly associated with malignant ventricular arrhythmias. We assessed the agreement among different imaging techniques for MAD identification and measurement. Methods 131 patients with MVP and significant mitral regurgitation undergoing transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) were retrospectively enrolled. Transoesophageal echocardiography (TOE) was available in 106 patients. MAD was evaluated in standard long-axis views (four-chamber, two-chamber, three-chamber) by each technique. Results Considering any-length MAD, MAD prevalence was 17.3%, 25.5%, 42.0% by TTE, TOE and CMR, respectively (p<0.05). The agreement on MAD identification was moderate between TTE and CMR (κ=0.54, 95% CI 0.49 to 0.59) and good between TOE and CMR (κ=0.79, 95% CI 0.74 to 0.84). Assuming CMR as reference and according to different cut-off values for MAD (≥2 mm, ≥4 mm, ≥6 mm), specificity (95% CI) of TTE and TOE was 99.6 (99.0 to 100.0)% and 98.7 (97.4 to 100.0)%; 99.3 (98.4 to 100.0)% and 97.6 (95.8 to 99.4)%; 97.8 (96.2 to 99.3)% and 93.2 (90.3 to 96.1)%, respectively; sensitivity (95% CI) was 43.1 (37.8 to 48.4)% and 74.5 (69.4 to 79.5)%; 54.0 (48.7 to 59.3)% and 88.9 (85.2 to 92.5)%; 88.0 (84.5 to 91.5)% and 100.0 (100.0 to 100.0)%, respectively. MAD length was 8.0 (7.0-10.0), 7.0 (5.0-8.0], 5.0 (4.0-7.0) mm, respectively by TTE, TOE and CMR. Agreement on MAD measurement was moderate between TTE and CMR (ρ=0.73) and strong between TOE and CMR (ρ=0.86). Conclusions An integrated imaging approach could be necessary for a comprehensive assessment of patients with MVP and symptoms suggestive for arrhythmias. If echocardiography is fundamental for the anatomic and haemodynamic characterisation of the MV disease, CMR may better identify small length MAD as well as myocardial fibrosis.

Background—: In patients with heart failure (HF), during maximal cardiopulmonary exercise test, anaerobic threshold (AT) is not always identified. We evaluated whether this finding has a prognostic meaning. Methods and Results—: We recruited and prospectively followed up, in 14 dedicated HF units, 3058 patients with systolic (left ventricular ejection fraction <40%) HF in stable clinical conditions, New York Heart Association class I to III, who underwent clinical, laboratory, echocardiographic, and cardiopulmonary exercise test investigations at study enrollment. We excluded 921 patients who did not perform a maximal exercise, based on lack of achievement of anaerobic metabolism (peak respiratory quotient ≤1.05). Primary study end point was a composite of cardiovascular death and urgent cardiac transplant, and secondary end point was all-cause death. Median follow-up was 3.01 (1.39–4.98) years. AT was identified in 1935 out of 2137 patients (90.54%). At multivariable logistic analysis, failure in detecting AT resulted significantly in reduced peak oxygen uptake and higher metabolic exercise and cardiac and kidney index score value, a powerful prognostic composite HF index ( P <0.001). At multivariable analysis, the following variables were significantly associated with primary study end point: peak oxygen uptake (% pred; P <0.001; hazard ratio [HR]=0.977; confidence interval [CI]=0.97–0.98), ventilatory efficiency slope ( P =0.01; HR=1.02; CI=1.01–1.03), hemoglobin ( P <0.05; HR=0.931; CI=0.87–1.00), left ventricular ejection fraction ( P <0.001; HR=0.948; CI=0.94–0.96), renal function (modification of diet in renal disease; P <0.001; HR=0.990; CI=0.98–0.99), sodium ( P <0.05; HR=0.967; CI=0.94–0.99), and AT nonidentification ( P <0.05; HR=1.41; CI=1.06–1.89). Nonidentification of AT remained associated to prognosis also when compared with metabolic exercise and cardiac and kidney index score ( P <0.01; HR=1.459; CI=1.09–1.10). Similar results were obtained for the secondary study end point. Conclusions—: The inability to identify AT most often occurs in patients with severe HF, and it has an independent prognostic role in HF.

Aims An independent role for the exercise-induced heart rate (HR) response—and specifically the chronotropic incompetence (CI)—in the prognosis of heart failure (HF) is still debated. The multicentre study reported here sought to investigate the prognostic values of HR and CI variables on cardiovascular mortality in a large cohort of systolic HF patients. Methods and results A total of 1045 HF patients were recruited and prospectively followed in three Italian HF centres. The study endpoint was cardiovascular mortality. Besides a full clinical examination, each patient underwent a maximal cardiopulmonary exercise test at study enrolment. The age-predicted peak HR (%pHR) and the peak HR reserve (%pHRR) according to different cut-off values (60–80% of the maximum predicted) were adopted to identify the presence of CI. The median follow-up was 876 days (interquartile range 386–1590 days). Cardiovascular death occurred in 145 cases (13.8%). Besides LVEF, peak oxygen uptake, ventilation vs. carbon dioxide production slope, and beta-blocker therapy, the multivariate analysis showed that both %pHR and %pHRR were able to predict prognosis when considered as continuous variables. Conversely, the presence of CI was associated with the study endpoint only when the 70% (%pHR <70%, hazard ratio 1.80, confidence interval 1.24–2.61, P = 0.002; %pHRR <70%, hazard ratio 1.77, confidence interval 1.09–2.86, P = 0.020) or the 65% cut-off values (%pHR <65%, hazard ratio 2.04, confidence interval 1.34–3.10, P = 0.001; %pHRR <65%, hazard ratio 1.54, confidence interval 1.03–2.32, P = 0.038) were adopted. Conclusions Our findings demonstrated an additive role of CI in stratifying cardiovascular mortality. Both the 65% and the 70% cut-off values, regardless of the method (%pHR and %pHRR), allow identification of HF patients with the worst prognosis, thus supporting such definitions of CI in HF.

Recently, the number of patients with congenital heart diseases reaching adulthood has been progressively increasing in developed countries, and new issues are emerging: the evaluation of their capacity to cope with physical activity and whether this knowledge can be used to optimize medical management. A symptom-limited cardiopulmonary exercise test has proven to be an essential tool, because it can objectively evaluate the functional cardiovascular capacity of these patients, identify the pathological mechanisms of the defect (circulatory failure, shunts, and/or pulmonary hypertension), and help prescribe an individualized rehabilitation program when needed. The common findings on cardiopulmonary exercise testing in patients with congenital heart diseases are a reduced peak V.o2, an early anaerobic threshold, a blunted heart rate response, a reduced increase of Vt, and an increased V.e/V.co2. All these measures suggest common pathophysiological abnormalities: (1) a compromised exercise capacity from anomalies affecting the heart, vessels, lungs, or muscles; (2) chronotropic incompetence secondary to cardiac autonomic dysfunction or β-blockers and antiarrhythmic therapy; and (3) ventilatory inefficiency caused by left-heart failure with pulmonary congestion, pulmonary hypertension, pulmonary obstructive vascular disease, or cachexia. Most of these variables also have prognostic significance. For these patients, cardiopulmonary exercise testing allows evaluation and decisions affecting lifestyle and therapeutic interventions.