False lumen intervention to promote remodelling and thrombosis-The FLIRT concept in aortic dissection.

OBJECTIVE: Thoracic endovascular aortic repair (TEVAR) has changed the management of aortic dissection by induced remodelling. Beyond reconstructing the true lumen, we describe the concept of False Lumen Intervention to promote Remodelling and Thrombosis (FLIRT) in both type A and B aortic dissection. METHODS: Between 2011 and 2017, 10 patients with aortic dissection (5 type A; 5 type B) underwent FLIRT using a combination of patent foramen ovale (PFO) or atrial septal defect (ASD) occluders, coils and glue. Patients were followed by computed tomography (CT) angiogram prior to, and 6 months following, discharge to evaluate false lumen (FL) thrombosis and aortic remodelling. Outcomes analyzed comprised successful device delivery, completeness of FL thrombosis and aortic remodelling, procedure related complications and mortality. RESULTS: FLIRT induced aortic remodelling in all cases of proximal dissection, with aortic shrinkage from 63.8 ± 7.5 pre-FLIRT, to 50.2 ± 6.6 mm (P = 0.057) and an increase in true lumen area from 5.8 ± 3.6 to 11.4 ± 2.5 cm2 (P = 0.006). In distal dissection (after previous TEVAR with residual FL flow), FLIRT successfully induced FL thrombosis in 4 of 5 cases at first attempt (1 case required additional coiling of the gutter between left subclavian artery and stent-graft for complete thrombosis). While maximal aortic diameter remained unchanged (55.6 ± 9.1 pre-FLIRT and 54.4 ± 13.7 mm at follow-up), true lumen area increased from 7.8 ± 2.3 pre-procedure, to 10.6 ± 1.5 cm2 at follow-up (P = 0.016), consistent with remodelling. CONCLUSION: Interventional FL management, using the FLIRT concept, is feasible in selected cases of aortic dissection, promotes FL thrombosis and induces successful remodelling.

Incidence, Outcomes, and Predictors of Ventricular Thrombus after Reperfused ST-Segment-Elevation Myocardial Infarction by Using Sequential Cardiac MR Imaging.

Purpose To characterize the incidence, outcomes, and predictors of left ventricular (LV) thrombus by using sequential cardiac magnetic resonance (MR) imaging after ST-segment-elevation myocardial infarction (STEMI). Materials and Methods Written informed consent was obtained from all patients, and the study protocol was approved by the committee on human research. In a cohort of 772 patients with STEMI, 392 (mean age, 58 years; range, 24-89 years) were retrospectively selected who were studied with cardiac MR imaging at 1 week and 6 months. Cardiac MR imaging guided the initiation and withdrawal of anticoagulants. Patients with LV thrombus at 6 months were restudied at 1 year. For predicting the occurrence of LV thrombus, a multiple regression model was applied. Results LV thrombus was detected in 27 of 392 patients (7%): 18 (5%) at 1 week and nine (2%) at 6 months. LV thrombus resolved in 22 of 25 patients (88%) restudied within the first year. During a mean follow-up of 181 weeks ± 168, patients with LV thrombus displayed a very low rate of stroke (0%), peripheral embolism (0%), and severe hemorrhage (n = 1, 3.7%). LV ejection fraction (LVEF) less than 50% (P < .001) and anterior infarction (P = .008) independently helped predict LV thrombus. The incidence of LV thrombus was as follows: (a) nonanterior infarction, LVEF 50% or greater (one of 135, 1%); (b) nonanterior infarction, LVEF less than 50% (one of 50, 2%); (c) anterior infarction, LVEF 50% or greater (two of 92, 2%); and (d) anterior infarction, LVEF less than 50% (23 of 115, 20%) (P < .001 for the trend). Conclusion Cardiac MR imaging contributes information for the diagnosis and therapy of LV thrombus after STEMI. Patients with simultaneous anterior infarction and LVEF less than 50% are at highest risk. © RSNA, 2017 Online supplemental material is available for this article.

Case report of asymptomatic very late presentation of ALCAPA syndrome: review of the literature since pathophysiology until treatment.

BACKGROUND: Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is an unusual congenital heart defect which affects approximately 1 in 300 000 live births and accounts for 0.5% of all congenital heart disease. Without surgical intervention, most patients die in infancy (nearly 90%). CASE SUMMARY: We present a rare case of an asymptomatic 67-year-old female. Transthoracic echocardiography demonstrated a dilated right coronary artery (RCA) and multiple collaterals. ALCAPA was confirmed by multidetector computed tomography. The left main artery was seen originating from the pulmonary artery and well-developed collaterals were visualized between the RCA and LCA. No areas of myocardial infarction were identified on cardiac magnetic resonance. Stress studies showed no inducible ischaemia. DISCUSSION: Our clinical case of an ALCAPA patient who survived and remained asymptomatic to their late 60's, highlights the importance of well-collateralized and pressurized coronary system to maintain adequate myocardial perfusion. Physicians should be aware of this congenital anomaly as appropriate early diagnosis is crucial to prevent irreversible myocardial damage, acute ischaemia, and arrhythmias, and can improve patient outcomes. Surgical treatment is suggested irrespective of symptomatology or the presence of inducible myocardial ischaemia.

Different Phenotypes of Anderson-Fabry Disease Identified with Cardiac Magnetic Resonance Imaging in a Family with the Same Late-Onset Mutation.

BACKGROUND Cardiac magnetic resonance imaging (CMR) is the only noninvasive test capable of differentiating between hypertrophic cardiomyopathy (HCM) and late-onset Anderson-Fabry disease (AFD). The purpose of this report is to show how CMR led to diagnosis of AFD in 3 family members, 1 of whom previously was misdiagnosed with HCM, and how late-onset AFD can present with different cardiac phenotypes, even in a family with the same pathogenic mutation. CASE REPORT A 60-year-old man was referred because of evidence of left ventricular hypertrophy (LVH) on an electrocardiogram (ECG) that was performed to screen for cardiomyopathy. One of his siblings previously had been diagnosed with HCM and atrial fibrillation. The patient's ECG and echocardiographic findings were suspicious for HCM. CMR showed severe symmetrical LVH but tissue characterization sequences were highly suggestive of AFD cardiomyopathy. Enzymatic and genetic testing confirmed the diagnosis of late-onset AFD (presence of the GLA p.F113.L mutation). The brother of the index patient then was re-evaluated and also diagnosed with late-onset AFD. He was found to have the same pathogenic mutation but with a presentation of asymmetrical septal LVH. The daughter of the index patient was positive for the same mutation but did not have LVH. CONCLUSIONS The fact that patients with late-onset AFD can present with different LVH and fibrosis patterns, even in the presence of the same pathogenic mutation, underscores the importance of including AFD in the differential diagnosis of HCM. CMR is fundamental for differentiating between those 2 entities and defining the pathological phase of AFD. A correct diagnosis can have a substantial impact on patient management, and more so on thier families.

Predictors of atrial tachyarrhythmias in adults with congenital heart disease.

BACKGROUND: Atrial tachyarrhythmias (ATs) represent the major late complications of congenital heart diseases (CHDs) following surgery. Little is known about the association between echocardiographic parameters and AT. AIMS: This study aimed to investigate a potential correlation among clinical, echocardiographic, and electrocardiographic parameters and AT as well as to analyze outcomes in adults with CHD and AT. METHODS: A retrospective case­control study was performed in adults with CHD. We included 71 patients with AT and 71 control individuals matched by sex, age, and the type of CHD without AT, all from the same institute. Medical records, electrocardiograms, and echocardiograms were reviewed. Adverse cardiovascular events were recorded and defined as cardiovascular mortality, admission for heart failure, or stroke. The univariate and multivariate logistic regression analysis of possible risk factors and the Kaplan-Meier analysis of adverse cardiovascular events were performed. RESULTS: Subpulmonary ventricular systolic pressure≥40 mm Hg (hazard ratio [HR], 6.8; 95% CI, 2.4-18; P <0.001), right atrial dilatation≥21 cm2 (HR, 3.1; 95% CI, 1.2-7.6; P = 0.01), and significant tricuspid regurgitation (HR, 4; 95% CI, 1.3-10; P = 0.02) were identified as the main risk factors for AT. Patients with AT had worse outcomes, more frequently developed adverse cardiovascular events (86% vs 14%; P <0.01), and exhibited a 58% event­free survival rate compared with 98% of the patients without AT after 8 years of follow­up (log rank, 6.6; P = 0.01). CONCLUSIONS: Among patients with CHD, the main risk factors for AT include right atrial dilatation, high subpulmonary ventricular systolic pressure, and significant tricuspid regurgitation. The presence of AT may increase the risk of adverse cardiac events.

Unusual distal right pulmonary artery origin from right ductus arteriosus with uncommon left-sided aortic arch / Inusual origen de la parte distal de la arteria pulmonar derecha en un ductus arterioso derecho en arco aórtico izquierdo no común

Abstract A bilateral persistent ductus arteriosus with right proximal pulmonary artery agenesis is a very unusual anatomic variant. These malformations are assumed to occur during the transformation of the sixth aortic arch with the pulmonary trunk in the development of the aorta and its branches. A 2-months preterm baby was referred by severe bronchopulmonary dysplasia. Transthoracic echocardiography showed a large pulmonary trunk and left pulmonary artery. No right proximal pulmonary artery was found. Cardiac computed tomography study showed bilateral ductus arteriosus. The right proximal pulmonary artery was originated from an arterial structure coming off the right subclavian artery, a right ductus arteriosus. The aortic arch was left-sided, with a peculiar variant at the exit of the supraaortic vessels. Right subclavian artery emerged as the first supraaortic vessel, followed by a common trunk, giving rise to both carotids and the left subclavian artery as the last vessel. Right lung was hypoplastic with interstitial disease. The calibre of the right proximal pulmonary artery was inadequate to perform a corrective surgery. LDA closure was performed, keeping a prostaglandin infusion to maintain right ductus arteriosus patency until right proximal pulmonary artery size will be big enough to perform a successful surgical reconstruction. Unfortunately, right ductus arteriosus became narrow at its origin and she developed severe pulmonary hypertension and the patient died.

Resumen El ductus arterioso bilateral con agenesia proximal de la arteria pulmonar derecha es una variante anatómica rara producida por alteraciones de la transformación del sexto arco aórtico en las arterias pulmonares, la aorta y sus vasos, durante el desarrollo embrionario. Se presenta el caso de un bebé prematuro de dos meses, que fue derivado a nuestro hospital por displasia broncopulmonar severa. El ecocardiograma transtorácico no identificó el origen de la arteria pulmonar derecha. El estudio por tomografía computarizada cardiaca mostró ductus arterioso bilateral e identificó el origen distal de la arteria pulmonar derecha en una rama de la arteria subclavia derecha, identificada como ductus arterioso derecho. El pulmón derecho era hipoplástico y presentaba alteraciones intersticiales. A su vez, el patrón de los vasos supraaórticos era anómalo. El primer vaso correspondió a la arteria subclavia derecha, seguido por tronco común de ambas carótidas y por último la arteria subclavia izquierda. El calibre de la arteria pulmonar derecha fue inadecuado para realizar una cirugía correctiva, por lo cual se decidió cerrar el ductus arterioso izquierdo y mantener infusión de prostaglandina hasta que la arteria pulmonar derecha tuviera un calibre adecuado para la cirugía. Infortunadamente, el ductus arterioso derecho se cerró en su origen, desarrollándose hipertensión pulmonar severa y la paciente falleció.

[Influence of age in short and long term prognostic of ischemic cardiogenic shock]. / Influencia de la edad en el pronóstico a corto y largo plazo del choque cardiogénico de origen isquémico.

OBJECTIVE: To compare the prognosis during hospitalization and maximum follow-up of 4 years in patients with myocardial infarction complicated with cardiogenic shock. METHOD: Prospective observational study practiced in a coronary Care Unit managed by cardiologists. We included patients with myocardial infarction complicated with cardiogenic shock who received early coronary revascularization. Patients were divided into two groups: older than 75 years (group A) and lower (group B), and we compared the evolution during hospitalization and maximum follow-up of 4 years. Primary end point was mortality rate in the maximum follow-up of 4years. Secondary end point was mortality rate during hospitalization. RESULTS: Ninety-seven patients were included, 45% Group A. Patients of Group B were mostly men (81% vs. 57%; P=.014), diabetics (48% vs. 21%; P=0.006), and smokers (39.6% vs. 5%). Mortality rate during hospitalization was higher in Group A (54.5%) vs. 30.2% in Group B (P=.022). Mortality rate during follow-up (primary variable) was 73% in Group A vs. 38% in Group B (P=.007). CONCLUSIONS: Myocardial infarction complicated with cardiogenic shock in elderly patients is an entity with high mortality during hospitalization and continues to worsen during long term follow-up.

Influencia de la edad en el pronóstico a corto y largo plazo del choque cardiogénico de origen isquémico / Influence of age in short and long term prognostic of ischemic cardiogenic shock

Objetivo: Comparar la evolución durante el ingreso y a largo plazo de pacientes con choque cardiogénico de origen isquémico. Método: Estudio observacional prospectivo unicéntrico llevado a cabo en la unidad coronaria de un hospital terciario manejada por cardiólogos. Se incluyen pacientes con choque cardiogénico de origen isquémico que recibieron revascularización coronaria precoz. Dividimos a los pacientes en 2 grupos: mayores de 75 anos (grupo A) y menores (grupo B), y comparamos la evolución durante el ingreso y en un seguimiento máximo de 4 años. El objetivo principal fue estimar la mortalidad a los 4 años. El objetivo secundario se definió como la mortalidad intrahospitalaria. Resultados: Incluimos a 97 pacientes, 44 del grupo A (45%). Los pacientes del grupo B eran con más frecuencia varones (81 vs. 57%, p = 0.014), diabéticos (49 vs. 21%, p: 0.006) y fumadores (39.6 vs. 4.5%, p < 0.05). La mortalidad hospitalaria fue superior en el grupo A (54.5 vs. 30.2%, p = 0.022). El objetivo principal ocurrió en 32 pacientes del grupo A frente a 20 del grupo B (73 vs. 38%, p = 0.007). Conclusión: El choque cardiogénico de origen isquémico en paciente mayores de 75 anos presenta una alta mortalidad durante la estancia hospitalaria y en el seguimiento a largo plazo.

Objective: To compare the prognosis during hospitalization and maximum follow-up of 4 years in patients with myocardial infarction complicated with cardiogenic shock. Method: Prospective observational study practiced in a coronary Care Unit managed by cardiologists. We included patients with myocardial infarction complicated with cardiogenic shock who received early coronary revascularization. Patients were divided into two groups: older than 75 years (group A) and lower (group B), and we compared the evolution during hospitalization and maximum follow-up of 4 years. Primary end point was mortality rate in the maximum follow-up of 4years. Secondary end point was mortality rate during hospitalization. Results: Ninety-seven patients were included, 45% Group A. Patients of Group B were mostly men (81% vs. 57%; P =.014), diabetics (48% vs. 21%; P = 0.006), and smokers (39.6% vs. 5%). Mortality rate during hospitalization was higher in Group A (54.5%) vs. 30.2% in Group B(P = .022). Mortality rate during follow-up (primary variable) was 73% in Group A vs. 38% in Group B (P =.007). Conclusions: Myocardial infarction complicated with cardiogenic shock in elderly patients is an entity with high mortality during hospitalization and continues to worsen during long term follow-up.