Left ventricular noncompaction cardiomyopathy: Recent advances.

From its initial description to the present day, left ventricular noncompaction cardiomyopathy has been the subject of numerous studies and publications. In question as a real cardiomyopathy, left ventricular noncompaction can appear in isolation or in association with other cardiac malformations, genetic syndromes, and neuromuscular disorders. As a genetically heterogeneous disorder, it can be sporadic or familial, with an autosomal dominant pattern with variable penetrance most frequently observed. Different diagnostic criteria have been described through the years, first by using echocardiogram and later on by cardiac magnetic resonance. The lack of universally accepted diagnostic criteria has led to the condition being over-diagnosed in the general population. Differential diagnosis between real cardiomyopathy, epiphenomenon (phenocopy in the setting of loading conditions or even other cardiomyopathies), and physiological hypertrabeculation, like in the athlete's heart must be considered. Clinically it can present as heart failure, ventricular arrhythmias, and even sudden death, but it can also be asymptomatic during familial screening. The main prognosis factors are left ventricular dilatation, dysfunction, and fibrosis. There is no specific treatment. Familial screening is recommended and special recommendations in the case of athletes must be taken into account. In the present article, we review the myth and reality concerning main and more recent aspects of left ventricular noncompaction.

First-line Double Stentriever Thrombectomy for M1/TICA Occlusions : Initial Experiences.

BACKGROUND: Mechanical thrombectomy is the standard of care for acute ischemic stroke due to large-vessel occlusion; however, mechanical thrombectomy fails to achieve adequate recanalization in nearly one third of these cases. Rescue therapy using two stentrievers simultaneously yields good results in clots refractory to single stentriever treatment. We aimed to determine the safety and efficacy of first-line double stentriever thrombectomy for acute occlusion of the M1 segment of the middle cerebral artery and/or terminal internal carotid artery (TICA). METHODS: This single-center study prospectively enrolled consecutive patients with a single M1/TICA occlusion to undergo double stentriever thrombectomy between May and October 2020. Outcomes included successful recanalization (modified thrombolysis in cerebral infarction, TICI 2b/3), first-pass effect, procedure times, number of device passes, symptomatic intracerebral hemorrhage, National Institutes of Health Stroke Scale Score (NIHSS) at discharge, 90-day functional independence (modified Rankin scale 0-2), and 90-day mortality. RESULTS: We analyzed 39 patients median age 79 years (range 42-96 years); 23 (58.9%) female; 19 (48.7%) with TICA occlusions; 5 (12.8%) with mRS 3-5 at admission; mean NIHSS at admission, 17 ± 4.39). Mean time from symptom onset to final angiogram was 238.0 ± 94.6 min; mean intervention duration was 36.0 ± 24.2 min. The mean number of device passes was 1.5 ± 1.07. All patients had final TICI 2b/3, and 27 (69%) had TICI 2c/3 after the first pass. We observed 3 (7.9%) cases of intracerebral symptomatic hemorrhages. At 90 days, 16 (41%) patients were functionally independent and 9 (23%) had died. The percentage of patients with good clinical outcome at 90 days was 55.5% in the first-pass subgroup. CONCLUSION: Our findings suggest that first-line double stentriever thrombectomy is safe and effective for M1/TICA occlusions.

Transcatheter Aortic Valve Implantation and Subclinical and Clinical Leaflet Thrombosis: Multimodality Imaging for Diagnosis and Risk Stratification.

In recent years, the phenomenon of subclinical leaflet thrombosis (SLT) in patients who have undergone transcatheter aortic valve implantation has become increasingly relevant. Hypo-attenuating leaflet thickening and hypo-attenuation affecting motion diagnosed by CT are the hallmarks of SLT, and their incidence varies depending on the intensity of screening. Whether these phenomena are a surrogate for leaflet thrombosis reducing valve durability and increasing the risk of stroke is still a matter of debate. Uncertainty remains over the optimal antithrombotic therapy after TAVI and the best treatment strategy is still not confirmed. Ongoing and future trials will provide more evidence about the best strategy for the prevention and treatment of SLT.

Unusual high 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) tracer deposition on a heart scintigraphy in a patient with AL amyloidosis: A case report.

We present a case of a 40-year-old Spanish man with cardiac amyloidosis in which a Tc-99m-3,3-diphosphono-1,2-propanodicarboxylic acid (Tc-99m-DPD) scintigraphy was strongly suggestive of cardiac amyloidosis by transthyretin (ATTR) but endomyocardial biopsy (EB) analyzed by immunohistochemistry demonstrated a light chain amyloidosis (AL). Even though the Tc-99m-DPD has proven in different published papers that has high sensibility and specificity for differentiating AL and ATTR cardiac amyloidosis, we present an unusual case of an AL cardiac amyloidosis with a Perugini grade 3 on the scintigraphy. Diagnostic approach of cardiac amyloidosis following consensus documents is discussed to avoid diagnostic mistakes based on imaging techniques.

Bicuspid aortic valve: what should I know? Updated review of its clinical and pathophysiological aspects. / Válvula aórtica bicúspide: ¿qué debo conocer? Revisión actualizada de sus aspectos clínicos y fisiopatológicos.

La válvula aórtica bicúspide es la cardiopatía congénita más frecuente en la población general. Lejos de ser solo una malformación valvular inocua, supone una enfermedad compleja y heterogénea. A menudo es identificada como un hallazgo incidental en personas sanas, cursando de manera asintomática. Sin embargo, en un alto porcentaje de pacientes conduce a lo largo de su vida a complicaciones valvulares (estenosis, insuficiencia, endocarditis) o aórticas (dilatación o disección). Con frecuencia estas manifestaciones suceden a una edad temprana y causan una elevada morbimortalidad. A pesar de que en los últimos años se ha producido una intensa investigación en este campo, la fisiopatogenia de la enfermedad no es del todo conocida y muchas preguntas siguen abiertas. En este artículo se revisan de forma actualizada los aspectos clínicos y fisiopatológicos más novedosos y relevantes sobre esta cardiopatía congénita.The most common congenital heart disease in the general population is the bicuspid aortic valve. Far from being just a harmless valve malformation, it is a complex and heterogeneous disease. It is often identified as an incidental finding in healthy people. However, in a high percentage of patients it leads throughout their life towards valvular (stenosis, insufficiency, endocarditis) or aortic (dilatation or dissection) complications. Frequently, manifestations occur at an early age, being responsible for high morbidity and mortality. Even though in recent years intense research has been carried out in this field, the pathophysiogenesis of the disease is not fully known and many questions remain open. In this article, we review the most innovative and relevant clinical and pathophysiological aspects of this congenital heart disease.

Válvula aórtica bicúspide: ¿qué debo conocer? Revisión actualizada de sus aspectos clínicos y fisiopatológicos / Bicuspid aortic valve: what should I know? Updated review of its clinical and pathophysiological aspects

Resumen La válvula aórtica bicúspide es la cardiopatía congénita más frecuente en la población general. Lejos de ser solo una malformación valvular inocua, supone una enfermedad compleja y heterogénea. A menudo es identificada como un hallazgo incidental en personas sanas, cursando de manera asintomática. Sin embargo, en un alto porcentaje de pacientes conduce a lo largo de su vida a complicaciones valvulares (estenosis, insuficiencia, endocarditis) o aórticas (dilatación o disección). Con frecuencia estas manifestaciones suceden a una edad temprana y causan una elevada morbimortalidad. A pesar de que en los últimos años se ha producido una intensa investigación en este campo, la fisiopatogenia de la enfermedad no es del todo conocida y muchas preguntas siguen abiertas. En este artículo se revisan de forma actualizada los aspectos clínicos y fisiopatológicos más novedosos y relevantes sobre esta cardiopatía congénita.

Abstract The most common congenital heart disease in the general population is the bicuspid aortic valve. Far from being just a harmless valve malformation, it is a complex and heterogeneous disease. It is often identified as an incidental finding in healthy people. However, in a high percentage of patients it leads throughout their life towards valvular (stenosis, insufficiency, endocarditis) or aortic (dilatation or dissection) complications. Frequently, manifestations occur at an early age, being responsible for high morbidity and mortality. Even though in recent years intense research has been carried out in this field, the pathophysiogenesis of the disease is not fully known and many questions remain open. In this article, we review the most innovative and relevant clinical and pathophysiological aspects of this congenital heart disease.

Characterization of Left Ventricular Non-Compaction Cardiomyopathy.

Left ventricle non-compaction cardiomyopathy (LVNC) has gained great interest in recent years, being one of the most controversial cardiomyopathies. There are several open debates, not only about its genetic heterogeneity, or about the possibility to be an acquired cardiomyopathy, but also about its possible overdiagnosis based on imaging techniques. In order to better understand this entity, we identified 38 LVNC patients diagnosed by cardiac MRI (CMRI) or anatomopathological study that could underwent NGS-sequencing and clinical study. Anatomopathological exam was performed in eight available LVNC hearts. The genetic yield was 34.2%. Patients with negative genetic testing had better left ventricular ejection fraction (LVEF) or it showed a tendency to improve in follow-up, and a possible trigger factor for LVNC was identified in 1/3 of them. Nonetheless, cerebrovascular accidents occurred in similar proportions in both groups. We conclude that in LVNC there seem to be different ways to achieve the same final phenotype. Genetic testing has a good genetic yield and provides valuable information. LVNC without an underlying genetic cause may have a better prognosis in terms of LVEF evolution. However, anticoagulation to prevent cerebrovascular accident (CVA) should be carefully evaluated in all patients. Larger series with pathologic examination are needed to help better understand this entity.

Drenaje venoso anómalo y estenosis de vena pulmonar / Anomalous pulmonary venous drainage and pulmonary vein stenosis

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