Successful thromboaspiration of bilateral pulmonary thromboembolism in a Fontan circulation patient: a case report.

Background: Fontan surgery aims to palliate univentricular congenital heart diseases in which biventricular repair is not feasible. A large spectrum of early and late complications has been described in literature. However, pulmonary thromboembolism represents a rare complication in these patients, leading to a scarcity of evidence regarding diagnosis and treatment strategies. Case summary: We present a case of a 27-year-old woman born with a complex cyanotic congenital heart disease, namely pulmonary and tricuspid stenosis with subaortic interventricular communication and atrial septal defect, who underwent palliation surgery with Blalock-Taussig shunt, bidirectional Glenn, and extracardiac Fontan. She developed acute respiratory failure and was admitted to the hospital, being diagnosed with bilateral thromboembolism. Since she was haemodynamically stable, initially, a conservative approach was chosen. However, due to no clinical improvement, she subsequently underwent bilateral thromboaspiration with restoration of pulmonary circulation. Discussion: Due to the unique Fontan pathophysiology, the possible physiological and clinical implications of pulmonary thromboembolism in this condition are profound. Thus, care and imaging tests in specialized centres are important as the management of these patients is different from those with biventricular physiology.

Univentricular hearts not undergoing Fontan: the type of palliation matters.

INTRODUCTION AND OBJECTIVES: There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation. METHODS: SVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death. RESULTS: We identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037). CONCLUSIONS: The type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.

Myocardial fibrosis in congenital heart disease.

Myocardial fibrosis resulting from the excessive deposition of collagen fibers through the myocardium is a common histopathologic finding in a wide range of cardiovascular diseases, including congenital anomalies. Interstitial fibrosis has been identified as a major cause of myocardial dysfunction since it distorts the normal architecture of the myocardium and impairs the biological function and properties of the interstitium. This review summarizes current knowledge on the mechanisms and detrimental consequences of myocardial fibrosis in heart failure and arrhythmias, discusses the usefulness of available imaging techniques and circulating biomarkers to assess this entity and reviews the current body of evidence regarding myocardial fibrosis in the different subsets of congenital heart diseases with implications in research and treatment.

Optimal opportunistic screening of atrial fibrillation using pulse palpation in cardiology outpatient clinics: Who and how.

BACKGROUND: Atrial fibrillation (AF) remain a prevalent undiagnosed condition frequently encountered in primary care. OBJECTIVE: We aimed to find the parameters that optimize the diagnostic accuracy of pulse palpation to detect AF. We also aimed to create a simple algorithm for selecting which individuals would benefit from pulse palpation and, if positive, receive an ECG to detect AF. METHODS: Nurses from four Cardiology outpatient clinics palpated 7,844 pulses according to a randomized list of arterial territories and durations of measure and immediately followed by a 12-lead ECG, which we used as the reference standard. We calculated the sensitivity and specificity of the palpation parameters. We also assessed whether diagnostic accuracy depended on the nurse's experience or on a list of clinical factors of the patients. With this information, we estimated the positive predictive values and false omission rates according to very few clinical factors readily available in primary care (age, sex, and diagnosis of heart failure) and used them to create the algorithm. RESULTS: The parameters associated with the highest diagnostic accuracy were palpation of the radial artery and classifying as irregular those palpations in which the nurse was uncertain about pulse regularity or unable to palpate pulse (sensitivity = 79%; specificity = 86%). Specificity decreased with age. Neither the nurse's experience nor any investigated clinical factor influenced diagnostic accuracy. We provide the algorithm to select the ≥40 years old individuals that would benefit from a pulse palpation screening: a) do nothing in <60 years old individuals without heart failure; b) do ECG in ≥70 years old individuals with heart failure; c) do radial pulse palpation in the remaining individuals and do ECG if the pulse is irregular or you are uncertain about its regularity or unable to palpate it. CONCLUSIONS: Opportunistic screening for AF using optimal pulse palpation in candidate individuals according to a simple algorithm may have high effectiveness in detecting AF in primary care.

Substrate characterization of the right ventricle in repaired tetralogy of Fallot using late enhancement cardiac magnetic resonance.

BACKGROUND: Three-dimensional (3D) substrate characterization by high-resolution late gadolinium enhancement cardiac magnetic resonance (LE-CMR) is useful for guiding ventricular tachycardia ablation of the left ventricle in ischemic heart disease. OBJECTIVE: The purpose of this study was to validate the substrate characterization and 3D reconstruction of LE-CMR images of the right ventricle (RV) in patients with repaired tetralogy of Fallot (rTOF) and to identify the algorithm that best fits with electroanatomic mapping (EAM). METHODS: RV LE-CMR images were compared with RV EAM in 10 patients with rTOF. RV LE-CMR images were postprocessed and analyzed to identify fibrotic tissue on 3D color maps. The 3D RV substrate reconstructions were created using an adjustable percentage of the maximum voxel signal intensity (MSI) of the scar region to define the threshold between core, transitional zone (TZ), and healthy tissue. Extensions of the core and TZ areas were compared with the scar (<0.5 mV) and low-voltage (0.5-1.5 mV) areas obtained by RV EAM. Agreement on anatomic isthmi identification was quantified. RESULTS: The best match between core and scar was obtained at 65% MSI cutoff (mean areas 17.4 ± 9.9 cm2 vs 16.9 ± 10.0 cm2, respectively; r = 0.954; P <.001). Agreement on anatomic isthmi identification was best at 60% MSI cutoff, which identified 95% of isthmi and achieved a total fit in 90% of patients. CONCLUSION: This study demonstrates that characterization of the RV substrate by postprocessing LE-CMR images in rTOF patients is feasible and validates the technique against RV EAM, which could help in planning target ablation.

Determinants of long-term outcome of repaired pulmonary valve stenosis.

INTRODUCTION AND OBJECTIVES: Although pulmonary valve stenosis (PVS) is considered a low risk congenital heart disease, there have been reports of complications and the need for reintervention throughout follow-up. The aims of this study were to evaluate the long-term outcome of repaired PVS and to identify predictors of cardiovascular complications and reintervention. METHODS: We studied 158 adult patients with repaired PVS (repair procedures performed from 1957 to 2010) receiving active follow-up in a tertiary referral center. RESULTS: A total of 95 patients (60%) received surgical treatment, and 63 patients (40%) received percutaneous pulmonary balloon valvuloplasty. At the end of follow-up (27 years, IQR, 20-33 years), most patients (n=134, 84.8%) were in New York Heart Association functional class I, but 61 patients (38.6%) required a reintervention, mainly pulmonary valve replacement (17.7%, n=28), and 19 patients (12%) had at least one cardiovascular complication: 13 (8.2%) supraventricular arrhythmias, 6 (3.8%) heart failure, 5 (3.2%) stroke, 1 (0.6%) death, 1 (0.6%) thromboembolism, and 1 (0.6%) ventricular arrhythmia. Multivariate analysis showed that age at PVS repair (HR, 1.08; 95%CI, 1.04-1.12; P <.001) and the presence of cyanosis before PVS repair (HR, 5.23; 95%CI, 1.99-13.78; P=.001) were independent predictors for cardiovascular complications. CONCLUSIONS: Good long-term outcome can be expected after PVS repair, but complications and the need for reintervention may appear. Older age and the presence of cyanosis at PVS repair emerged as predictors of cardiovascular complications and identified a population that may merit stricter control.

Worse 12-month prognosis in women with non-valvular atrial fibrillation undergoing percutaneous coronary intervention.

BACKGROUND: Limited data exists on the impact of sex on outcomes in non-valvular atrial fibrillation (NVAF) patients undergoing percutaneous coronary intervention (PCI). We explored the impact of sex on ischemic and bleeding events in these patients within 1-year. METHODS: A prospective register included 1021 patients with NVAF undergoing PCI and 253 (24.8%) were women. The primary end point was a composite of cardiovascular death, stroke or systemic embolism (SSE). The secondary end-point was major bleeding events defined as a Bleeding Academic Research Consortium (BARC ≥ 3a). RESULTS: Women were older (76.8 ±â€¯7.7 vs 71.8 ±â€¯9.1 years, p < 0.0001), and presented more often CHA2DS2-VASc ≥ 2 (adjusted HR 1.15; 95%CI 1.13-1.18, p < 0.0001) and HAS-BLED ≥ 3 (adjusted HR 1.12; 95%CI 1.10-1.14, p < 0.0001) than men. The use of oral anticoagulant at discharge was similar in both sexes (55.9% vs 56.5%, p = 0.45). The time in therapeutic range (TTR ≥ 65%) was lower in women than in men (35.6 ±â€¯24.6% vs 48.9 ±â€¯27.2%, p = 0.002). The incidence of adverse events was higher in women (39.9% vs 28.9%, p = 0.01). After adjusting for confounder variables, cardiovascular death or SSE rate (16.6% vs 10.4%; adjusted HR 1.58; 95%CI 1.07-2.31; p = 0.01) and major bleeding (11.5% vs 5.0%; adjusted HR 2.17; 95%CI 1.31-3.59; p = 0.003) were higher in women, as was cardiovascular death (adjusted HR 1.71; 95%CI, 1.18-2.46, p = 0.004). TTR was negatively correlated with any bleeding event in women (r = -0.41; p = 0.03). CONCLUSIONS: Female with NVAF undergoing PCI showed a lower TTR than men and TTR was associated with bleeding events. Female sex was an independent risk factor for cardiovascular death and major bleeding.

Use and Outcomes of Triple Antithrombotic Therapy with Non-Vitamin K Antagonists in Patients with Atrial Fibrillation Undergoing Percutaneous Coronary Intervention.

BACKGROUND: Triple antithrombotic therapy (TT) is recommended for patients with nonvalvular atrial fibrillation (AF) undergoing percutaneous coronary intervention (PCI). However, there is a lack of comparative data in a real-world clinical setting between non-vitamin K antagonist oral anticoagulants (NOAC) and vitamin K antagonists (VKA). The aim of this study was to compare the safety and efficacy of TT with NOAC or VKA after PCI in patients with AF at 1-year of follow-up. MATERIALS AND METHODS: This was an observational retrospective study in 2 tertiary care hospitals during 2013-2016. Patients with indication for anticoagulation due to AF from an initial registry of 5,269 patients undergoing PCI were identified. Safety primary endpoint was the occurrence of major bleeding events as defined by Bleeding Academic Consortium (BARC ≥ 3). The primary efficacy endpoint was defined as major adverse cardiovascular events (MACE). RESULTS: A total of 187 consecutive patients on TT were identified: 45.4% of were discharged on NOAC and 54.6% on VKA. Patients who received VKA presented more comorbidities and had a higher bleeding risk than those who received NOACs. Major bleeding events occurred in 17 patients (9%), with a higher rate in the VKA group (3.5% vs. 13% confidence interval, 0.19-0.86, P = 0.02). There were no differences in the rates of major adverse cardiovascular events, stroke or net clinical benefit. CONCLUSIONS: In this real-world study, patients with AF undergoing PCI treated on NOAC-based TT showed lower bleeding rates than those on VKA, with a lower rate of major bleeding events, while efficacy was similar between groups.