Coronary Intravascular Lithotripsy Effectiveness and Safety in a Real-World Cohort.

Background: Coronary artery calcification is a predictor of adverse outcomes after percutaneous coronary intervention (PCI). Intravascular lithotripsy (IVL) is a promising tool for the treatment of calcified lesions. The aim of this study was to assess the effectiveness and safety of IVL. Methods: A single-center observational study of PCI procedure, with assessment of the outcomes of patients undergoing PCI using IVL, was performed. Angiographic procedural success was used as the primary effectiveness endpoint. The primary safety endpoint was defined as a composite of cardiac death, myocardial infarction and target vessel revascularization within 30 days. Results: A total of 111 patients were included. Indications for PCI spanned the spectrum of chronic (53.2%) and acute coronary syndromes (43%). Lesion preparation before IVL was performed with non-compliant (42%), cutting or OPN (14.4%) balloons and with atherectomy techniques in 11% of procedures. Intravascular imaging was used in 21.6% of procedures. The primary effectiveness endpoint was achieved in 100% and the primary safety endpoint in 3.6% of procedures. Peri-procedural complications were minimal and successfully resolved. Conclusions: IVL was an effective and safe technique for the treatment of calcified coronary lesions. These findings contribute to the growing body of evidence supporting the use of IVL in the management of these challenging scenarios.

Development of deep learning segmentation models for coronary X-ray angiography: Quality assessment by a new global segmentation score and comparison with human performance.

INTRODUCTION AND OBJECTIVES: Although automatic artificial intelligence (AI) coronary angiography (CAG) segmentation is arguably the first step toward future clinical application, it is underexplored. We aimed to (1) develop AI models for CAG segmentation and (2) assess the results using similarity scores and a set of criteria defined by expert physicians. METHODS: Patients undergoing CAG were randomly selected in a retrospective study at a single center. Per incidence, an ideal frame was segmented, forming a baseline human dataset (BH), used for training a baseline AI model (BAI). Enhanced human segmentation (EH) was created by combining the best of both. An enhanced AI model (EAI) was trained using the EH. Results were assessed by experts using 11 weighted criteria, combined into a Global Segmentation Score (GSS: 0-100 points). Generalized Dice Score (GDS) and Dice Similarity Coefficient (DSC) were also used for AI models assessment. RESULTS: 1664 processed images were generated. GSS for BH, EH, BAI and EAI were 96.9+/-5.7; 98.9+/-3.1; 86.1+/-10.1 and 90+/-7.6, respectively (95% confidence interval, p<0.001 for both paired and global differences). The GDS for the BAI and EAI was 0.9234±0.0361 and 0.9348±0.0284, respectively. The DSC for the coronary tree was 0.8904±0.0464 and 0.9134±0.0410 for the BAI and EAI, respectively. The EAI outperformed the BAI in all coronary segmentation tasks, but performed less well in some catheter segmentation tasks. CONCLUSIONS: We successfully developed AI models capable of CAG segmentation, with good performance as assessed by all scores.

Aortic annulus rupture with fistula to the right ventricle following aortic valve replacement with a sutureless valve: A surgical complication with a percutaneous solution.

With the development of interventional procedures, iatrogenic aorto-right ventricular fistulae are increasingly reported. They may follow surgical aortic valve replacement or percutaneous aortic valve implantation, leading to high morbidity. Traditionally, treatment of fistulae was based on surgical repair, but with advances in endovascular technologies, more emphasis is now placed on percutaneous closure. We report the case of a 78-year-old patient with severe symptomatic aortic stenosis who underwent surgical aortic valve replacement with a Perceval sutureless valve. One month later, he presented with symptoms and signs of heart failure. Transthoracic and transesophageal echocardiography confirmed the presence of a aorto-right ventricular fistula. The fistula was successfully closed percutaneously with an Amplatzer Vascular Plug II, in an intracardiac echocardiography-guided procedure. Aorto-right ventricular fistula is a rare finding after surgical aortic valve replacement and to our knowledge it has never been associated with sutureless aortic valve replacement. A percutaneous procedure with an appropriately selected device may be encouraged because of the high morbidity and mortality of redo open-heart surgery. To minimize the risk of a second general anesthesia, the use of intracardiac echocardiography to guide the percutaneous procedure is feasible and safe.

The CTo-aBCDE score: A new predictor of success in chronic total occlusions.

INTRODUCTION: Patient selection for percutaneous coronary intervention (PCI) in chronic total occlusions (CTOs) is crucial to procedural success. Our aim was to identify independent predictors of success in CTO PCI in order to create an accurate score. METHODS: In a single-center observational registry of CTO PCI, demographic and clinical data and anatomical characteristics of coronary lesions were recorded. Linear and logistic regression analysis were used to identify predictors of success. A score to predict success was created and its accuracy was measured by receiver operating curve analysis. RESULTS: A total of 377 interventions were performed (334 patients, age 68±11 years, 75% male). The success rate was 65% per patient and 60% per procedure. Predictors of success in univariate analysis were absence of active smoking (OR 2.02, 95% CI 1.243-3.29; p=0.005), presence of tapered stump (OR 5.2, 95% CI 2.7-10.2; p<0.001), absence of tortuosity (OR 6.44; 95% CI 3.02-13.75; p<0.001), absence of bifurcation (OR 1.95; 95% CI 1.08-3.51; p=0.026), absence of calcification (OR 3.1; 95% CI 3.10-5.41; p<0.001), LAD as target vessel (OR 1.9, 95% CI 1.0-3.5; p=0.048), and CTO length <20 mm (OR 3.00, 95% CI 1.69-5.3; p<0.001). Only anatomical factors were independent predictors of success, and an anatomical score (0-11 points) with high accuracy (area under the curve 0.831) was subsequently created. A score <3 was associated with low probability of success (15%), 3-8 with intermediate probability (55%), and >8 with high probability (95%). CONCLUSION: In our sample only anatomical characteristics were predictors of success. The creation of a score to predict success, with good accuracy, may enable selection of cases that can be treated by any operator, those in which a dedicated operator will be desirable, and those with an extremely low probability of success, which should be considered individually for conservative management, surgical revascularization or PCI by a team experienced in CTO.

The sensitivity of DPD scintigraphy to detect transthyretin cardiac amyloidosis in V30M mutation depends on the phenotypic expression of the disease.

Background: There is a growing need for a non-invasive test to detect cardiac involvement in patients with transthyretin-related hereditary amyloidosis (ATTR) caused by V30M mutation. 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scintigraphy is a promising method, but its accuracy in this particular mutation remains unknown.Methods: A cohort of 179 patients: 92 with early-onset disease (EoD, symptoms <50-years-old), 33 with late-onset disease (LoD) and 54 asymptomatic carriers were prospectively evaluated and underwent DPD scintigraphy, which was compared with the results of echocardiogram, ambulatory blood pressure monitoring, 24 h-Holter, myocardial 123I-metaiodobenzylguanidine imaging and NT-proBNP.Results: Amyloid cardiomyopathy, defined as septal thickness ≥13 mm, was present in 32 patients (17.9%) and was more frequent in those with LoD (OR: 3.68, p = .003). Cardiac DPD uptake was present in 22 individuals (12.3%) and correlated with parameters indicative of cardiac amyloidosis. DPD imaging was strongly influenced by the age of disease onset: among patients with myocardial thickening, cardiac DPD retention was present in 11/15 (73.3%) with LoD, in contrast to only 4/17 (26.7%) with EoD (p = .005). Two patients with myocardial thickening and normal DPD scintigraphy underwent endomyocardial biopsy that confirmed ATTR amyloidosis.Conclusion: DPD scintigraphy presents suboptimal sensitivity to detect cardiac involvement in ATTRV30M, particularly in symptomatic patients with EoD.

Comparative analysis of fractional flow reserve and instantaneous wave-free ratio: Results of a five-year registry. / Análise comparativa do fractional flow reserve (FFR) e do instantaneous wave­free ratio (iFR): resultados de um registo de 5 anos.

INTRODUCTION AND OBJECTIVE: Assessment of coronary lesions by the instantaneous wave free ratio (iFR) has generated significant debate. We aimed to assess the diagnostic performance of iFR and its impact on the decision to use fractional flow reserve (FFR) and on procedural characteristics. METHODS: In this single-center registry of patients undergoing functional assessment of coronary lesions, FFR was used as a reference for assessing the diagnostic performance of iFR. An iFR value <0.86 was considered positive and a value >0.93 was considered negative. RESULTS: Functional testing was undertaken of 402 lesions, of which 154 were assessed with both techniques, 222 with FFR only, and 26 with iFR only. Using a cut-off of ≤0.80 for iFR, the area under the curve was 0.73 (95% CI 0.65-0.81), with an optimal value of ≤0.91. FFR was undertaken in 93 out of 94 lesions with an inconclusive iFR and was performed in 69.1% of the remaining iFR-tested lesions. Concordance between iFR and FFR was 87% (chi-square=22.43; p<0.001). Notwithstanding, there were four out of 13 cases (30.7%) of positive iFR with negative FFR and three out of 42 (7.1%) cases of negative iFR and positive FFR. This difference was significant (p=0.026). iFR had no impact on procedure time, fluoroscopy time or radiation dose. CONCLUSION: iFR had a reasonable diagnostic performance. Operators often chose to perform FFR despite conclusive iFR results. iFR and FFR were highly concordant, but a non-negligible proportion of lesions classified as ischemic by iFR were classified as non-ischemic by FFR. iFR had no impact on procedural characteristics.

Cardiotoxicity in Hematological Diseases: Are the Tyrosine Kinase Inhibitors Imatinib and Nilotinib Safe?

Chemotherapy-induced cardiotoxicity is a growing concern. The cardiotoxic impact of new drugs such as tyrosine kinase inhibitors is unknown, especially the ones used for chronic myeloid leukemia. We aim to evaluate nilotinib- and imatinib-induced cardiotoxicity. Single-center prospective study of consecutive patients with chronic myeloid leukemia treated with tyrosine kinase inhibitors was conducted during 2015. Patients underwent an initial clinical, laboratorial and echocardiographic evaluation, repeated after 1 year. Eleven patients were included [60.0 (11) years, 63.6% of males; seven patients treated with imatinib and four with nilotinib]. After 1 year of follow-up, all patients remained in functional NYHA class I, with a similar Minnesota quality of life score. Also there was no difference in the biomarkers evaluated (cystatin-C and NT-proBNP). Likewise, no modification in systolic or diastolic function evaluated by echocardiography was observed. All patients presented normal values of longitudinal, circumferential and radial strain in the baseline study, without changes during follow-up. In addition, there were no differences between the two tyrosine kinase inhibitors used, considering all the aforementioned variables. No clinical, laboratory or echocardiographic evidence of nilotinib- and imatinib-induced cardiotoxicity was observed. However, these results should be confirmed in multicenter studies given the low incidence of chronic myeloid leukemia.

Port-A-Cath Catheter Embolization to Distal Pulmonary Artery Branches: Two Tailored Percutaneous Retrieval Approaches.

Several types of intravascular devices and catheters are frequently used for long-term drug therapy, especially for oncological patients. As a result, complications are becoming increasingly common, namely catheter embolization. Retrieving these devices is important, as embolized fragments may lead to serious consequences, such as arrhythmias, myocardial injury, thrombosis, infection, and even perforation and death. We describe 2 cases of long-term drug catheter (Port-A-Cath) fracture, incidentally documented in a routine chest radiograph. In both cases, percutaneous extraction was attempted, yet the procedure was complicated by embolization of smaller fragments into the arterial pulmonary vasculature. We describe unusual approaches in successfully retrieving the remaining fragments. The ideal approaches for removal of foreign bodies from the cardiovascular system differ from case to case, but percutaneous extraction should be preferred in most of the cases. Less common techniques may be helpful in challenging cases.

Complex phenotype linked to a mutation in exon 11 of the lamin A/C gene: Hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes.

The lamin A/C (LMNA) gene encodes lamins A and C, which have an important role in nuclear cohesion and chromatin organization. Mutations in this gene usually lead to the so-called laminopathies, the primary cardiac manifestations of which are dilated cardiomyopathy and intracardiac conduction defects. Some mutations, associated with lipodystrophy but not cardiomyopathy, have been linked to metabolic abnormalities such as diabetes and severe dyslipidemia. Herein we describe a new phenotype associated with a mutation in exon 11 of the LMNA gene: hypertrophic cardiomyopathy, atrioventricular block, severe dyslipidemia and diabetes. A 64-year-old woman with hypertrophic cardiomyopathy and a point mutation in exon 11 of the LMNA gene (c.1718C>T, Ser573Leu) presented with severe symptomatic ventricular hypertrophy and left ventricular outflow tract obstruction. She underwent septal alcohol ablation, followed by Morrow myectomy. The patient was also diagnosed with severe dyslipidemia, diabetes and obesity, and fulfilled diagnostic criteria for metabolic syndrome. No other characteristics of LMNA mutation-related phenotypes were identified. The development of type III atrioventricular block with no apparent cause, and mildly depressed systolic function, prompted referral for cardiac resynchronization therapy. In conclusion, the association between LMNA mutations and different phenotypes is complex and not fully understood, and can present with a broad spectrum of severity.

Combined MitraClip implantation and left atrial appendage occlusion using the Watchman device: A case series from a referral center.

INTRODUCTION: Patients referred for percutaneous transcatheter mitral valve repair using the MitraClip® system frequently have atrial fibrillation, which imposes additional challenges due to the need for oral anticoagulation. Left atrial appendage occlusion is currently regarded as a non-inferior alternative to anticoagulation in patients with non-valvular atrial fibrillation and both high thromboembolic and bleeding risk. Considering that both MitraClip implantation and left atrial appendage occlusion are percutaneous techniques that require transseptal puncture, it is technically attractive to consider their concomitant use. OBJECTIVES: We aim to evaluate the feasibility of a combined approach with MitraClip implantation and left atrial appendage occlusion in a single procedure. METHODS: We report the first case series regarding this issue, discussing the specific advantages, pitfalls and technical aspects of combining these two procedures. RESULTS: Five patients underwent left atrial appendage occlusion with the Watchman® device followed by MitraClip implantation in the same procedure. All patients experienced significant reduction in mitral valve regurgitation of at least two grades, optimal occluder position, no associated complications and significant clinical improvement assessed by NYHA functional class (reduction of at least one functional class, with four patients in class I at one-month follow-up). CONCLUSION: In selected patients rejected for surgical mitral valve repair, with atrial fibrillation and increased risk of bleeding and embolic events, a combined approach with MitraClip implantation and left atrial appendage occlusion in a single procedure is feasible, safe and effective.

Progression of myocardial sympathetic denervation assessed by 123I-MIBG imaging in familial amyloid polyneuropathy and the effect of liver transplantation. / Progressão da desnervação simpática cardíaca avaliada por cintigrafia com MIBG­I123 na polineuropatia amiloidótica familiar e o impacto da transplantação hepática.

INTRODUCTION: Familial amyloid polyneuropathy (FAP) is a rare disease caused by systemic deposition of amyloidogenic variants of the transthyretin (TTR) protein. The TTR-V30M mutation is caused by the substitution of valine by methionine at position 30 and mainly affects the peripheral and autonomic nervous systems. Cardiovascular manifestations are common and are due to autonomic denervation and to amyloid deposition in the heart. Cardiac sympathetic denervation detected by iodine-123 labeled metaiodobenzylguanidine (MIBG) is an important prognostic marker in TTR-V30M FAP. Liver transplantation, widely used to halt neurological involvement, appears to have a varying effect on the progression of amyloid cardiomyopathy. Its effect on the progression of cardiac denervation remains unknown. METHODS: In this observational study, patients with the TTR-V30M mutation underwent annual cardiac assessment and serial MIBG imaging with quantification of the late heart-to-mediastinum (H/M) ratio. RESULTS: We studied 232 patients (median age 40 years, 54.7% female, 37.9% asymptomatic at the time of inclusion) who were followed for a median of 4.5 years and underwent a total of 558 MIBG scans. During follow-up, 47 patients (20.3%) died. MIBG scintigraphy at inclusion was a strong predictor of prognosis, with the risk of death increasing by 27.8% for each one-tenth reduction in the late H/M ratio. The late H/M ratio decreased with age (0.082/year, p<0.001), but progression of cardiac denervation was so slow that annual repetition of MIBG imaging did not increase its prognostic accuracy. During follow-up, 70 symptomatic patients underwent liver transplantation. The late H/M ratio decreased by 0.19/year until transplantation but no statistically significant differences were detected after the procedure. CONCLUSIONS: Cardiac denervation is common during the progression of TTR-V30M FAP and quantification of the late H/M ratio on MIBG scintigraphy is valuable for prognostic stratification of these patients. Liver transplantation stabilizes cardiac denervation, without recovery or further deterioration in cardiac MIBG uptake after the procedure.

Balloon aortic valvuloplasty in the transcatheter aortic valve implantation era: A single-center registry.

INTRODUCTION: Percutaneous balloon aortic valvuloplasty (BAV) has been limited by the risk of complications and restenosis. However, growing use of transcatheter aortic valve implantation (TAVI) has revived interest in this technique. We analyzed the current indications for BAV and outcomes in a single center. METHODS: Acute results and long-term outcomes were analyzed in a retrospective single-center registry of patients undergoing BAV between January 2013 and January 2016. RESULTS: Twenty-three patients underwent BAV, 56.5% male, mean age 78±7 years. Indications were severe aortic stenosis and decompensated heart failure (n=5), urgent non-cardiac surgery (n=8), or bridge to definitive treatment (n=10). Peak invasive gradient decreased from a median of 54.0±19.0 mmHg to 28.5±13.8 mmHg (p=0.002). Complications included one ischemic stroke, one lower limb ischemia and one femoral pseudoaneurysm requiring surgery. During a mean follow-up of 11±10 months, eight patients underwent TAVI and two underwent surgical aortic valve replacement. Thirteen patients died, nine of non-cardiovascular causes. On Kaplan-Meier analysis mortality was significantly lower among patients undergoing definitive treatment (20.0% vs. 84.6% at two-year follow-up; p=0.005). CONCLUSION: BAV should be considered for selected patients with temporary contraindications to definitive therapy or as palliative therapy.

Hypertriglyceridemia: Is there a role for prophylactic apheresis? A case report.

Severe hypertriglyceridemia has been consistently associated with an increased risk of cardiovascular disease and other complications, namely acute pancreatitis. We report a case of a 64 year-old woman with hypertrophic cardiomyopathy and metabolic syndrome with triglyceride level of 3260 mg/dL. Plasma exchange was performed with simultaneous medical treatment to achieve a rapid and effective lowering of triglycerides in order to prevent clinical complications. After three plasmapheresis sessions a marked reduction in triglyceride and total cholesterol levels was observed. Several cases have shown the importance of plasmapheresis in the treatment of acute pancreatitis. We intend to demonstrate the applicability of this technique as primary prophylaxis in the presence of extremely high serum triglyceridemia levels. Resumo A hipertrigliceridemia grave tem sido associada de forma consistente ao aumento do risco cardiovascular e a outras complicações, nomeadamente, pancreatite aguda. Descrevemos um caso de uma mulher de 64 anos, com miocardiopatia hipertrófica e síndrome metabólica com valor sérico de triglicerídeos de 3260 mg/dL. Foi efectuada plasmaferese e optimizado o tratamento médico para alcançar uma redução rápida e efectiva dos níveis dos triglicerídeos, prevenindo complicações clínicas. Após três sessões de plasmaferese, verificou-se uma redução marcada dos triglicerídeos e do colesterol total. Existem alguns casos descritos na literatura demonstrado a importância da plasmaferese no tratamento da pancreatite aguda em contexto de hipertrigliceridemia grave. Os autores pretendem com este caso demonstrar a aplicabilidade desta técnica em contexto de prevenção primária em doentes com níveis de triglicerídeos extremamente aumentados.

Hypertriglyceridemia: Is there a role for prophylactic apheresis? A case report / Hipertrigliceridemia: há um papel para a aferese profilática? Relato de um caso

Abstract Severe hypertriglyceridemia has been consistently associated with an increased risk of cardiovascular disease and other complications, namely acute pancreatitis. We report a case of a 64 year-old woman with hypertrophic cardiomyopathy and metabolic syndrome with triglyceride level of 3260 mg/dL. Plasma exchange was performed with simultaneous medical treatment to achieve a rapid and effective lowering of triglycerides in order to prevent clinical complications. After three plasmapheresis sessions a marked reduction in triglyceride and total cholesterol levels was observed. Several cases have shown the importance of plasmapheresis in the treatment of acute pancreatitis. We intend to demonstrate the applicability of this technique as primary prophylaxis in the presence of extremely high serum triglyceridemia levels.

Resumo A hipertrigliceridemia grave tem sido associada de forma consistente ao aumento do risco cardiovascular e a outras complicações, nomeadamente, pancreatite aguda. Descrevemos um caso de uma mulher de 64 anos, com miocardiopatia hipertrófica e síndrome metabólica com valor sérico de triglicerídeos de 3260 mg/dL. Foi efectuada plasmaferese e optimizado o tratamento médico para alcançar uma redução rápida e efectiva dos níveis dos triglicerídeos, prevenindo complicações clínicas. Após três sessões de plasmaferese, verificou-se uma redução marcada dos triglicerídeos e do colesterol total. Existem alguns casos descritos na literatura demonstrado a importância da plasmaferese no tratamento da pancreatite aguda em contexto de hipertrigliceridemia grave. Os autores pretendem com este caso demonstrar a aplicabilidade desta técnica em contexto de prevenção primária em doentes com níveis de triglicerídeos extremamente aumentados.

Chronic medical comorbidities in patients with acute coronary syndrome.

BACKGROUND: Patients with acute coronary syndrome (ACS) frequently present chronic noncardiovascular medical comorbidities that can influence treatment and prognosis. Compliance with therapeutic guidelines in ACS is crucial to event reduction and the presence of these comorbidities may be a determining factor in guideline adherence. OBJECTIVE: To assess the prevalence of chronic noncardiovascular medical comorbidities in patients with ACS and their impact on guideline adherence. METHODS: We studied consecutive patients admitted to the coronary care unit of our institution with a diagnosis of ACS. We identified patients with noncardiovascular comorbidities, divided into five groups: chronic renal failure, pulmonary disease, gastrointestinal disease, blood disease or cancer). We assessed complete adherence to pharmacological therapy plus reperfusion (mechanical or pharmacological) in ST-segment elevation myocardial infarction, and use of coronary angiography in non-ST segment elevation myocardial infarction. We compared guideline adherence according to the presence or absence of comorbidities and their impact on in-hospital mortality. RESULTS: The study sample consisted of 146 patients, mean age 64 +/- 13 years and 71% male. In 53% of the patients at least one comorbidity was identified: chronic renal failure in 23%, pulmonary disease in 14%, gastrointestinal disease in 20%, blood disease in 7% and cancer in 9%. Patients with comorbidities were older, and more frequently had a history of ACS, heart failure and peripheral arterial disease. Complete adherence to guidelines was worse in the group with comorbidities (56% vs. 74%; p = 0.025). The presence of noncardiovascular comorbidities was associated with higher in-hospital mortality (9% vs. 0%, p = 0.011). CONCLUSION: Noncardiovascular medical comorbidities are frequently found in patients with ACS. Adherence to therapeutic guidelines for ACS is suboptimal, particularly in patients with chronic noncardiovascular comorbidities. Moreover, the presence of such comorbidities influences short-term prognosis in ACS patients.