Immediate and Midterm Cardiac Remodeling After Surgical Pulmonary Valve Replacement in Adults With Repaired Tetralogy of Fallot: A Prospective Cardiovascular Magnetic Resonance and Clinical Study.

BACKGROUND: Pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot provides symptomatic benefit and right ventricular (RV) volume reduction. However, data on the rate of ventricular structural and functional adaptation are scarce. We aimed to assess immediate and midterm post-PVR changes and predictors of reverse remoeling. METHODS: Fifty-seven patients with repaired tetralogy of Fallot (age ≥16 y; mean age, 35.8±10.1 y; 38 male) undergoing PVR were prospectively recruited for cardiovascular magnetic resonance performed before PVR (pPVR), immediately after PVR (median, 6 d), and midterm after PVR (mPVR; median, 3 y). RESULTS: There were immediate and midterm reductions in indexed RV end-diastolic volumes and RV end-systolic volumes (RVESVi) (indexed RV end-diastolic volume pPVR versus immediately after PVR versus mPVR, 156.1±41.9 versus 104.9±28.4 versus 104.2±34.4 mL/m2; RVESVi pPVR versus immediately after PVR versus mPVR, 74.9±26.2 versus 57.4±22.7 versus 50.5±21.7 mL/m2; P<0.01). Normal postoperative diastolic and systolic RV volumes (the primary end point) achieved in 70% of patients were predicted by a preoperative indexed RV end-diastolic volume ≤158 mL/m2 and RVESVi ≤82 mL/m2. RVESVi showed a progressive decrease from baseline to immediate to midterm follow-up, indicating ongoing intrinsic RV functional improvement after PVR. Left ventricular ejection fraction improved (pPVR versus mPVR, 59.4±7.6% versus 61.9±6.8%; P<0.01), and right atrial reverse remodeling occurred (pPVR versus mPVR, 15.2±3.4 versus 13.8±3.6 cm2/m2; P<0.01). Larger preoperative RV outflow tract scar was associated with a smaller improvement in post-PVR RV/left ventricular ejection fraction. RV ejection fraction and peak oxygen uptake predicted mortality (P=0.03) over a median of 9.5 years of follow-up. CONCLUSIONS: Significant right heart structural reverse remodeling takes place immediately after PVR, followed by a continuing process of further biological remodeling manifested by further reduction in RVESVi. PVR before RVESVi reaches 82 mL/m2 confers optimal chances of normalization of RV function.

The acute effects of an ultramarathon on biventricular function and ventricular arrhythmias in master athletes.

AIMS: Endurance sports practice has significantly increased over the last decades, with a growing proportion of participants older than 40 years. Although the benefits of moderate regular exercise are well known, concerns exist regarding the potential negative effects induced by extreme endurance sport. The aim of this study was to analyse the acute effects of an ultramarathon race on the electrocardiogram (ECG), biventricular function, and ventricular arrhythmias in a population of master athletes. METHODS AND RESULTS: Master athletes participating in an ultramarathon (50 km, 600 m of elevation gain) with no history of heart disease were recruited. A single-lead ECG was recorded continuously from the day before to the end of the race. Echocardiography and 12-lead resting ECG were performed before and at the end of the race. The study sample consisted of 68 healthy non-professional master athletes. Compared with baseline, R-wave amplitude in V1 and QTc duration were higher after the race (P < 0.001). Exercise-induced isolated premature ventricular beats were observed in 7% of athletes; none showed non-sustained ventricular tachycardia before or during the race. Left ventricular ejection fraction, global longitudinal strain (GLS), and twisting did not significantly differ before and after the race. After the race, no significant differences were found in right ventricular inflow and outflow tract dimensions, fractional area change, s', and free wall GLS. CONCLUSION: In master endurance athletes running an ultra-marathon, exercise-induced ventricular dysfunction, or relevant ventricular arrhythmias was not detected. These results did not confirm the hypothesis of a detrimental acute effect of strenuous exercise on the heart.

The Acute Effects of an Ultramarathon on Atrial Function and Supraventricular Arrhythmias in Master Athletes.

BACKGROUND: Endurance sports practice has significantly increased over the last decades, with a growing proportion of master athletes. However, concerns exist regarding the potential proarrhythmic effects induced by ultra-endurance sports. This study aimed to analyse the acute effects of an ultramarathon race on atrial remodelling and supraventricular arrhythmias in a population of master athletes. METHODS: Master athletes participating in an ultramarathon (50 km, 600 m of elevation gain) with no history of heart disease were recruited. A single-lead ECG was recorded continuously from the day before to the end of the race. Echocardiography and 12-lead resting ECG were performed before and immediately at the end of the race. RESULTS: The study sample consisted of 68 healthy non-professional master athletes. Compared with baseline, P wave voltage was higher after the race (p < 0.0001), and more athletes developed ECG criteria for right atrial enlargement (p < 0.0001). Most of the athletes (97%) had ≥1 premature atrial beats (PAB) during the 24-h monitoring, also organised in triplets (17%) and non-sustained supraventricular tachycardias (NSSVTs) (19%). In contrast, exercise-induced PABs, triplets, and NSSVTs were rare. One athlete developed acute atrial fibrillation during the race. After the race, no significant differences were found in biatrial dimensions. Biatrial function, estimated by peak atrial longitudinal and contraction strains, were normal both before and after the race. CONCLUSIONS: In master athletes running an ultramarathon, acute exercise-induced atrial dysfunction was not detected, and exercise-induced supraventricular arrhythmias were uncommon. These results did not confirm the hypothesis of an acute atrial dysfunction induced by ultra-endurance exercise.

Three-Dimensional Late Gadolinium Enhancement Cardiovascular Magnetic Resonance Predicts Inducibility of Ventricular Tachycardia in Adults With Repaired Tetralogy of Fallot.

BACKGROUND: Adults with repaired tetralogy of Fallot die prematurely from ventricular tachycardia (VT) and sudden cardiac death. Inducible VT predicts mortality. Ventricular scar, the key substrate for VT, can be noninvasively defined with late gadolinium enhancement (LGE) cardiovascular magnetic resonance but whether this relates to inducible VT is unknown. METHODS: Sixty-nine consecutive repaired tetralogy of Fallot patients (43 male, mean 40±15 years) clinically scheduled for invasive programmed VT-stimulation were prospectively recruited for prior 3-dimensional LGE cardiovascular magnetic resonance. Ventricular LGE was segmented and merged with reconstructed cardiac chambers and LGE volume measured. RESULTS: VT was induced in 22 (31%) patients. Univariable predictors of inducible VT included increased RV LGE (odds ratio [OR], 1.15; P=0.001 per cm3), increased nonapical vent LV LGE (OR, 1.09; P=0.008 per cm3), older age (OR, 1.6; P=0.01 per decile), QRS duration ≥180 ms (OR, 3.5; P=0.02), history of nonsustained VT (OR, 3.5; P=0.02), and previous clinical sustained VT (OR, 12.8; P=0.003); only prior sustained VT (OR, 8.02; P=0.02) remained independent in bivariable analyses after controlling for RV LGE volume (OR, 1.14; P=0.003). An RV LGE volume of 25 cm3 had 72% sensitivity and 81% specificity for predicting inducible VT (area under the curve, 0.81; P<0.001). At the extreme cutoffs for ruling-out and ruling-in inducible VT, RV LGE >10 cm3 was 100% sensitive and >36 cm3 was 100% specific for predicting inducible VT. CONCLUSIONS: Three-dimensional LGE cardiovascular magnetic resonance-defined scar burden is independently associated with inducible VT and may help refine patient selection for programmed VT-stimulation when applied to an at least intermediate clinical risk cohort.

The concept of double inlet-double outlet right ventricle: a distinct congenital heart disease.

The aim of this study was to estimate the incidence and to analyze the anatomy of double inlet-double outlet right ventricle complex and its associated cardiac anomalies in our autopsy series. Among the 1640 hearts with congenital heart disease of our Anatomical Collection, we reviewed the specimens with double inlet-double outlet right ventricle, according to the sequential-segmental analysis, identifying associated cardiac anomalies and examining lung histology to assess the presence of pulmonary vascular disease. We identified 14 hearts with double inlet-double outlet right ventricle (0.85%). Right atrial isomerism was observed in 10 hearts, situs solitus in 3 and left atrial isomerism in one. Regarding the mode of atrioventricular connection, all hearts but one had a common atrioventricular valve. Systemic or pulmonary venous abnormalities were noted in all patients with atrial isomerism. In nine patients a valvular or subvalvular pulmonary stenosis was present. Among the functionally "univentricular hearts", double inlet- double outlet right ventricle represents a peculiar entity, mostly in association with right atrial isomerism. Multiple cardiac anomalies are associated and may complicate surgical repair.

[Sudden cardiac arrest in Italian sports facilities in 2015: epidemiological implications of the so-called "Balduzzi decree"]. / L'arresto cardiaco negli impianti sportivi nel 2015: possibili implicazioni epidemiologiche dell'applicazione della "Legge Balduzzi".

Under the Italian Law "Legge Balduzzi", which was issued after the sudden cardiac death of professional athletes Pier Mario Morosini and Vigor Bovolenta in 2012, the presence of an automated external defibrillator (AED) and personnel trained to perform cardiopulmonary resuscitation must be available in every Italian sports facility from 2016. In 2015 the national and local press reported 123 cases of sudden cardiac arrests (SCA) occurring in Italian sport facilities, corresponding to an estimated ≈0.2-0.4% of all SCA and to ≈0.6-1.2% of SCA in public places. The majority of SCA victims were males (93%) and >35 years old (88%, median age 50 years). On the basis of the report of the event on the press, the rate of return of spontaneous circulation was 62% when an AED was used before emergency medical system arrival versus 9% when no bystander cardiopulmonary resuscitation or AED use by lay rescuers was mentioned. These data demonstrated that the Law has the potential to increase the survival to SCA in athletes; however, limiting the obligation of the presence of an AED only to sports facilities is not enough to decrease significantly the incidence of SCA in the general population.

Juxtaposition of the atrial appendages: A nidus for thrombus in atriopulmonary Fontan?

Juxtaposition of atrial appendages is a rare cardiac congenital anomaly, usually associated with other cardiac malformations. Until now, it has not been linked to any significant clinical implications. We report cardiovascular magnetic resonance (CMR) findings of two adult patients who underwent atriopulmonary Fontan operation in the setting of left juxtaposition of the atrial appendages. The patients were in sinus rhythm at the time of the CMR study. Both patients had episodes of sustained atrial tachyarrhythmia requiring electrical cardioversion and were anticoagulated with warfarin with target INR 2-3. CMR images showed a thrombus located in the enlarged and juxtaposed right appendage in both patients. Blood flow frequently appears slow or sluggish in the dilated right atrium following atriopulmonary Fontan surgery. In addition, cine CMR suggested that blood flow reaches very low velocities in the massively dilated juxtaposed right atrial appendage cul-de-sac, thus potentially creating a substrate for clot formation. These findings propose that juxtaposed atrial appendages in atriopulmonary Fontan is an additional risk factor for clot formation, specifically in the dilated right atrial appendage on the left side juxtaposed with the left atrial appendage and that prophylactic anticoagulation is highly justified in these patients.

New speckle-tracking algorithm for right ventricular volume analysis from three-dimensional echocardiographic data sets: validation with cardiac magnetic resonance and comparison with the previous analysis tool.

AIMS: (i) To validate a new software for right ventricular (RV) analysis by 3D echocardiography (3DE) against cardiac magnetic resonance (CMR); (ii) to assess the accuracy of different measurement approaches; and (iii) to explore any benefits vs. the previous software. METHODS AND RESULTS: We prospectively studied with 3DE and CMR 47 patients (14-82 years, 28 men) having a wide range of RV end-diastolic volumes (EDV 82-354 mL at CMR) and ejection fractions (EF 34-81%). Multi-beat RV 3DE data sets were independently analysed with the new software using both automated and manual editing options, as well as with the previous software. RV volume reproducibility was tested in 15 random patients. RV volumes and EF measurements by the new software had an excellent accuracy (bias ± SD: -15 ± 24 mL for EDV; 1.4 ± 4.9% for EF) and reproducibility compared with CMR, provided that the RV borders automatically tracked by software were systematically edited by operator. The automated analysis option underestimated the EDV, overestimated the ESV, and largely underestimated the EF (bias ± SD: -17 ± 10%). RV volumes measured with the new software using manual editing showed similar accuracy, but lower inter-observer variability and shorter analysis time (3-5') in comparison with the previous software. CONCLUSION: Novel vendor-independent 3DE software enables an accurate, reproducible and faster quantitation of RV volumes and ejection fraction. Rather than optional, systematic verification of border tracking quality and manual editing are mandatory to ensure accurate 3DE measurements. These findings are relevant for echocardiography laboratories aiming to implement 3DE for RV analysis for both research and clinical purposes.

Successful Bentall procedure in a patient with a Fontan circulation.

A 33-year old male patient with absent left atrioventricular connection and double outlet left ventricle developed severe aortic root dilatation and aortic regurgitation. He had undergone initial banding of the pulmonary trunk and atrial septectomy, eventually followed by the bidirectional Glenn procedure. At the time of the total cavopulmonary connection (at 27 years of age), his ascending aorta was plicated and wrapped. Subsequently, the aortic root below the wrapped ascending aorta showed further dilatation. The Bentall procedure was successfully carried out.

At-admission risk stratification for in-hospital life-threatening ventricular arrhythmias and death in non-ST elevation myocardial infarction patients.

AIMS: Identification of patients with non-ST elevation acute myocardial infarction (NSTEMI) at higher risk of in-hospital life-threatening ventricular arrhythmias (LT-VA) and death is crucial for determining appropriate levels of care/monitoring during hospitalisation. We assessed predictors of in-hospital LT-VA and all-cause mortality in a consecutive series of NSTEMI patients. METHODS AND RESULTS: We prospectively studied 1325 consecutive patients (69.7% males, median age 70 (61-79) years) presenting with NSTEMI and undergoing continuous electrocardiographic monitoring. The primary study end-point was the occurrence of spontaneous (unrelated to coronary interventions) in-hospital LT-VA, including sustained ventricular tachycardia and ventricular fibrillation; the secondary end-point was in-hospital mortality from all causes. Of 1325 patients, 21 (1.5%) experienced LT-VA and 62 (4.7%) died from either arrhythmias (n=1) or other causes (n=61). Seven of the 20 patients who survived LT-VA subsequently died of heart failure. Independent predictors of in-hospital LT-VA were the Global Registry of Acute Coronary Events (GRACE) score >140 (odds ratio (OR)=7.5; 95% confidence interval (CI) 1.7-33.3; p=0.008) and left ventricular ejection fraction (LV-EF)<35% (OR=4.1; 95% CI 1.7-10.3; p=0.002). GRACE score >140 (OR=14.6; 95% CI 3.4-62) and LV-EF <35% (OR=4.4; 95% CI 1.9-10) also predicted in-hospital all-cause death. The cumulative probability of in-hospital LT-VA and death was respectively 9.2% and 23% in the 98 (7.4%) patients with GRACE score >140 and LV-EF<35%, while it was respectively 0.2% and 0% among the 627 (47.3%) with GRACE score ≤140 and LV-EF ≥35%. CONCLUSIONS: Simple risk stratification at admission based on GRACE score and echocardiographic LV-EF allows early identification of NSTEMI patients at higher risk of both in-hospital LT-VA and all-cause mortality.

Apical ballooning with mid-ventricular obstruction: the many faces of Takotsubo cardiomyopathy.

Takotsubo cardiomyopathy (TTC) is a transient left ventricular dysfunction due to akinesia of the left-ventricular (LV) mid-apical segments (apical ballooning), which can cause severe reduction in LV systolic function. The typical clinical picture of TTC include chest pain, electrocardiographic changes consisting of mild ST-segment elevation followed by diffuse deep T-wave inversion, QTc interval prolongation and mild troponin release in the absence of significant coronary stenoses. The syndrome often affects post-menopausal women and is triggered by sympathetic overstimulation, like intense physical or emotional stress, so that it is called the "broken heart syndrome". Although left-ventricular systolic dysfunction usually fully recovers within few days, heart failure can still complicate the early phase. We report a case of stress-induced cardiomyopathy that had full recovery after 4 weeks of follow up. The main electrocardiographic, angiographic and imaging features are discussed.

Heart failure due to 'stress cardiomyopathy': a severe manifestation of the opioid withdrawal syndrome.

Takotsubo cardiomyopathy (TTC) is a transient left ventricular (LV) dysfunction due to akinesia of the LV mid-apical segments ('apical ballooning') in the absence of critical coronary stenoses which can be complicated in the acute phase by heart failure, mitral regurgitation, life-threatening ventricular arrhythmias, or apical LV thrombosis. The syndrome is typically precipitated by intense emotional or physical stress; however, other causes of sympathetic overstimulation including administration of exogenous sympathomimetics or withdrawal of sympathetic antagonists can trigger TTC. We report the case of a patient who unexpectedly developed an 'apical ballooning' with severe reduction in the LV systolic function and heart failure after the withdrawal of methadone. The case supports the concept that increased sympathetic activity secondary to opioids withdrawal can trigger a stress-induced severe LV dysfunction. Physicians should be aware that the abrupt discontinuation of a long-term therapy with opioids may lead to serious cardiac complications. The administration of clonidine may be considered to prevent early clinical manifestations of addictive withdrawal, including TTC.