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Background: Cardiac involvement in Fabry disease (FD) usually manifests as a concentric left ventricular hypertrophy with rare cases developing left ventricular outflow tract obstruction (LVOTO), symptoms varying from fatigue and exercise associated dyspnoea to angina or arrhythmias. Case summary: We present the case of a 54-year-old man with cardiovascular risk factors and aggravated exertional dyspnoea in the past year, in whom the echocardiography showed hypertrophic obstructive cardiomyopathy (HOCM). Cardiac magnetic resonance was used as differential diagnosis tool between sarcomeric HOCM and other phenocopies, suggesting a cardiac involvement of FD. Final diagnosis was formulated based on genetic testing and enzymatic activity of α-galactosidase. Left ventricular outflow tract obstruction was addressed by alcohol septal ablation successfully both in short term as well as at 1-year follow-up. Discussion: The present case illustrates the complex clinical pathway of a patient with HOCM due to FD, where multimodality imaging was instrumental from differential diagnosis to therapeutic choices, which addressed both the pathogenic background and the organ involvement. Although at the moment the number of patients with of FD cardiomyopathy undergoing LVOTO reduction therapies is scarce, current recommendations should be extended to also include these patients.
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Pulmonary hypertension (PH) is a progressive and invalidating condition despite available therapy. Addressing complications such as left main coronary artery compression (LMCo) due to the dilated pulmonary artery (PA) may improve symptoms and survival. Nevertheless, clear recommendations are lacking. The aim of this study is to analyze the prevalence, characteristics, predictive factors and impact of LMCo in a heterogenous precapillary PH population in a single referral center. Two hundred sixty-five adults with various etiologies of precapillary PH at catheterization were reviewed. Coronary angiography (CA) was performed for LMCo suspicion. Revascularization was performed in selected cases. Outcomes were assessed at a mean follow-up of 3.9 years. LMCo was suspected in 125 patients and confirmed in 39 (31.2%), of whom 21 (16.8%) had 50%-90% stenoses. Nine revascularizations were performed, with clinical improvement. The only periprocedural complication was a stent migration. LMCo was associated with PH etiology (p 0.003), occuring more frequently in congenital heart disease-associated PH (61.5% of all LMCo cases, 66.6% of LMCo ≥ 50%). Predictors of LMCo ≥50% were PA ≥ 37.5 mm (Sn 81%, Sp 74%) and PA-to-aorta ≥1.24 (Sn 81%, Sp 69%), with increased discrimination when considering RV end-diastolic area. LMCo ≥ 50% without revascularization presented clinical deterioration and worse survival (p 0.019). This analysis of a heterogeneous pre-capillary PH population provides LMCo prevalence estimation, predictive factors (PA size, PA-to-aorta, RV end-diastolic area and PH etiology) and long-term impact. While LMCo impact on survival is inconclusive, untreated LMCo ≥ 50% has worse prognosis. LMCo revascularization may be performed safely and with good outcomes.
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AIMS: There is little evidence guiding the choice between a one-stent and a two-stent approach in unprotected distal left main coronary artery disease (UDLMCAD) presenting as acute coronary syndrome (ACS). We aim to compare these two techniques in an unselected ACS group. METHODS AND RESULTS: We conducted a single center retrospective observational study, that included all patients with UDLMCAD and ACS undergoing PCI between 2014 and 2018. Group A underwent PCI with a one-stent technique (n = 41, 58.6%), Group B with a two-stent technique (n = 29, 41.4%). A total of 70 patients were included, with a median age of 63 years, including n = 12 (17.1%) with cardiogenic shock. There were no differences between Group A and B in terms of patient characteristics, including SYNTAX score (median 23). The 30-day mortality was 15.7% overall, and was lower in Group B (3.5% vs. 24.4%, p = 0.02). Mortality rate at 4 years was significantly lower in Group B (21.4% vs. 44%), also when adjusted in a multivariable regression model (HR 0.26, p = 0.01). CONCLUSIONS: In our study, patients with UDLMCAD and ACS undergoing PCI using a two-stent technique had lower early and midterm mortality compared to one-stent approach, even after adjusting for patient-related or angiographic factors.
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OBJECTIVE: Pulmonary arterial hypertension (PAH) can lead to left main coronary artery compression (LMCo), but data on the impact, screening and treatment are limited. A meta-analysis of LMCo cases could fill the knowledge gaps in this topic. METHODS: Electronic databases were searched for all LMCo/PAH studies, abstracts and case reports including pulmonary artery (PA) size. Restricted maximum likelihood meta-analysis was used to evaluate LMCo-associated factors. Specificity, sensitivity and accuracy of PA size thresholds for diagnosis of LMCo were calculated. Treatment options and outcomes were summarized. RESULTS: A total of five case-control cohorts and 64 case reports/series (196 LMCo and 438 controls) were included. LMCo cases had higher PA diameter (Hedge's g 1.46 [1.09; 1.82]), PA/aorta ratio (Hedge's g 1.1 [0.64; 1.55]) and probability of CHD (log odds-ratio 1.22 [0.54; 1.9]) compared to non-LMCo, but not PA pressure or vascular resistance. A 40 mm cut-off for the PA diameter had balanced sensitivity (80.5%), specificity (79%) and accuracy (79.7%) for LMCo diagnosis, while a value of 44 mm had higher accuracy (81.7%), higher specificity (91.5%) but lower sensitivity (71.9%). Pooled mortality after non-conservative treatment (n = 150, predominantly stenting) was 2.7% at up to 22 months of mean follow-up, with 83% survivors having no angina at follow-up. CONCLUSION: PA diameter, PA/aorta ratio and CHD are associated with LMCo, while hemodynamic parameters are not. Data from this study support that a PA diameter cut-off between 40 and 44 mm can offer optimal accuracy for LMCo screening. Preferred treatment was coronary stenting, associated with low mid-term mortality and symptom relief. Diagnosis and management of left main coronary artery compression (LMCo) in patients with pulmonary arterial hypertension (PAH).
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Hipertensión Pulmonar , Hipertensión Arterial Pulmonar , Angina de Pecho/etiología , Vasos Coronarios/diagnóstico por imagen , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/terapia , Arteria Pulmonar/diagnóstico por imagenRESUMEN
The Covid-19 pandemic has severely affected and changed medical activity worldwide. Public hospitals have been suffocated by the large number of Covid-19 patients, sometimes making it difficult for patients with other conditions to be admitted to hospital. This was also true for patients with cardiovascular disease. In the present article we describe the main variations in the activity of interventional cardiology laboratories in Romania between the pre-pandemic year 2019 and the pandemic year 2020 in private and public hospitals.
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Background. Percutaneous coronary intervention (PCI) of unprotected left main coronary artery disease (ULMCAD) have become a feasible and efficient alternative to coronary artery bypass surgery, especially in patients with acute coronary syndrome (ACS). There are limited data regarding early and late outcomes after ULMCAD PCI in patients with ACS and stable angina.The aim of this study was to compare early and four-year clinical outcomes in patients with ULMCAD PCI presenting as ACS or stable angina in a high-volume PCI center.Methods. We conducted a single center retrospective observational study, which included 146 patients with ULMCAD undergoing PCI between 2014 and 2018. Patients were divided in two groups: Group A included patients with stable angina (n = 70, 47.9%) and Group B patients with ACS (n = 76, 52.1%).Results. 30-day mortality was 8.22% overall, lower in Group A (1.43% vs 14.47%, p = 0.02). Mortality and major adverse cardiac events (MACE) rates at 4 years were significantly lower in Group A (9.64% vs 33.25%, p = 0.001, and 24.06% vs 40.11%, p = 0.012, respectively). Target lesion revascularization (TLR) at 4 year did not differ between groups (15% in Group A vs 12.76% in Group B, p = 0.5).Conclusions. In our study patients with ULMCAD and ACS undergoing PCI had higher early and long-term mortality and MACE rates compared to patients with stable angina, with similar TLR rate at 4-year follow-up.
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Síndrome Coronario Agudo/etiología , Angina Estable/etiología , Enfermedad de la Arteria Coronaria/complicaciones , Enfermedad de la Arteria Coronaria/cirugía , Intervención Coronaria Percutánea , Síndrome Coronario Agudo/diagnóstico por imagen , Adulto , Anciano , Anciano de 80 o más Años , Angina Estable/diagnóstico por imagen , Causas de Muerte , Angiografía Coronaria , Enfermedad de la Arteria Coronaria/diagnóstico por imagen , Femenino , Mortalidad Hospitalaria , Humanos , Masculino , Persona de Mediana Edad , Intervención Coronaria Percutánea/efectos adversos , Complicaciones Posoperatorias/mortalidad , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Coronavirus disease has overwhelmed the healthcare systems around the globe at an unprecedented level. The same was observed in the Romanian medical system. Interventional cardiology units have experienced a dramatic reduction in elective and urgent interventional procedures. The present report highlights the total number of coronary interventions, peripheral interventions, and interventions for structural heart diseases that were performed in 2020 in Romania in comparison to 2019.