Coronary artery perforation successfully treated with a second drug-eluting stent.

A coronary artery perforation is a rare, but potentially lethal, complication of percutaneous coronary intervention. We present a case of massive main vessel coronary perforation of the right coronary artery in a patient with acute ST segment elevation myocardial infarction, which was successfully treated with a second drug-eluting stent. This uncommon therapeutic approach was used to preserve flow to the large side branch. Early recognition, rapid balloon re-inflation at the perforation site and a "ping-pong" guiding technique allowed us to prepare the optimal strategy and to treat the perforation without developing cardiac tamponade.

Surgical repair of a coronary artery aneurysm associated with coronary artery fistula - a case report.

Coronary artery aneurysm is a relatively rare disorder that is usually discovered as a secondary finding in patients undergoing coronary artery angiography. Coronary artery fistulas are relatively more frequent than rare aneurysms and are often associated with other cardiac abnormalities. The etiology of aneurysms is mostly atherosclerotic, and they are less frequently associated with other acquired or congenital diseases, such as Kawasaki disease, connective tissue diseases, septic emboli, arteritis, and iatrogenic disease. We report a 70-year-old woman with a rare combination of a coronary artery aneurysm associated with a coronary artery fistula, which drained into the pulmonary artery. The diagnosis of our patient was made by selective coronary angiography and confirmed by computed tomography angiography. The patient was treated surgically because of the symptomatic course of the disease.

Malignant mesothelioma associated with localized myocardial fibrosis: a case report.

Left ventricular dysfunction is a common reason for patients' referral to cardiology departments for examination. Cardiac involvement is one of the possible yet rare presentations of malignant mesothelioma. We present a case of a patient in whom a routine cardiac examination and imaging revealed malignant mesothelioma. We discuss a possible association between a malignant tumor and myocardial scarring and how the oncologic treatment is influenced by concomitant heart failure. This article aims to raise awareness of the importance of multidisciplinary cooperation and thinking beyond the daily routine of our specialty to ensure the quality care of our patients. It also forced us to think about the possible causes of the association between malignant mesothelioma and myocardial fibrosis.

The utility of the Mayo Score for predicting the yield of genetic testing in patients with hypertrophic cardiomyopathy.

INTRODUCTION: The yield of genetic testing in hypertrophic cardiomyopathy (HCM) is variable. The Mayo HCM Genotype Predictor score (Mayo Score) provides the pre-test probability of a positive HCM genetic test. In the original cohort of Mayo Score patients, only 9 HCM-associated myofilament genes were evaluated. The aim of this study was to validate the Mayo Score in the national HCM cohort and assess the yield of genetic testing using next generation sequencing (NGS) evaluating up to 229 genes. MATERIAL AND METHODS: We included 336 consecutive unrelated HCM patients (41% women, mean age: 53 ±15 years). We performed NGS-based genomic testing with classification of identified variants according to American College of Medical Genetics and Genomics guidelines. NGS findings were compared with the Mayo Score (ranging from -1 to 5) based on clinical and echocardiographic variables. RESULTS: We identified 72 variants classified as pathogenic or likely pathogenic in 70 (21%) HCM patients. One patient with the highest Mayo Score of 5 had a pathogenic mutation (100% yield). Patients with a Mayo Score of 4 had a pathogenic mutation in 71% of cases. Patients with a Mayo Score of 3 or 2 had a pathogenic mutation in 50 and 35% of cases, respectively. The yield of genetic testing in patients with a Mayo Score of -1 to 1 was low (6-21%). CONCLUSIONS: The overall yield of genetic testing using NGS evaluating up to 229 genes was low. The yield of genetic testing was consistently predicted with Mayo Score values.

Outcome of patients ≥ 60 years of age after alcohol septal ablation for hypertrophic obstructive cardiomyopathy.

INTRODUCTION: The outcome of patients ≥ 60 years of age after alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) remains unresolved. We sought to determine the long-term survival and the causes of death in this population. MATERIAL AND METHODS: We enrolled 156 consecutive patients (69 ±6 years, 69% women, follow-up: 4.8 ±3.5 years) who underwent ASA at ≥ 60 years of age. RESULTS: The 30-day mortality rate was 1.3%. At the last check-up, 81% of patients were in New York Heart Association class ≤ 2 and 76% had a left ventricular outflow tract gradient (LVOG) ≤ 30 mm Hg. A total of 39 patients died (51% of cardiovascular causes, 44% of non-cardiovascular causes, 5% of unknown causes) during the 734 patient-years. The annual sudden mortality, the sudden mortality and the all-cause mortality rates were 0.5%, 1.1%, and 4.8%, respectively. The all-cause mortality was higher compared to the age- and sex-matched general population (p = 0.002). CONCLUSIONS: Alcohol septal ablation was safe and effective in the long-term follow-up. We observed a reduced life expectancy compared to the age- and sex-matched general population. Mortality was almost equally due to cardiovascular and non-cardiovascular causes of death.

Occlusion of Epicardial Coronary Arteries by Localized Pericardial Calcification.

Localized pericardial calcification is a relatively rare finding of often unknown etiology. We present the case of a 68-year-old man who was found to have bulky pericardial calcification, resulting in external compression of epicardial coronary arteries. (Level of Difficulty: Beginner.).

Uncomplicated Pregnancy in a Patient Treated With Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy.

Pregnancy in women with hypertrophic cardiomyopathy (HCM) is associated with a slightly increased risk of maternal morbidity and mortality, as well as a higher risk of premature births. One of the risk factors complicating pregnancies in HCM is left ventricular outflow tract obstruction (LVOTO). We report a case of a symptomatic patient with HCM and LVOTO who was treated with alcohol septal ablation and then had an uncomplicated pregnancy.

Myectomy-Like Extended Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy.

Currently, two therapeutic options are available for patients with drug-resistant obstructive hypertrophic cardiomyopathy. While there is a tendency in extended myectomy to perform a larger septal reduction to eliminate left ventricular outflow tract gradient more effectively, there has been a trend in recent years toward using lower dosages of alcohol during alcohol septal ablation (ASA) to reduce the risk of arrhythmic complications. We report a case of a symptomatic patient with severe septal hypertrophy who underwent an extended ASA of three septal branches. This led to a result similar to an extended myectomy with a favorable short-term follow-up.

Obstruction after alcohol septal ablation is associated with cardiovascular mortality events.

BACKGROUND: Left ventricular outflow tract obstruction (≥30 mm Hg at rest; LVOTO) is considered a possible risk of long-term outcomes in patients with hypertrophic cardiomyopathy (HCM). However, the influence of LVOTO on the occurrence of cardiovascular mortality events in patients after alcohol septal ablation (ASA) for obstructive HCM remains unresolved. METHODS: We compared the outcomes of patients treated with ASA with residual LVOTO <30 mm Hg with those with residual LVOTO ≥30 mm Hg at the first postdischarge check-up (1-6 months after the procedure). RESULTS: A total of 270 patients (60±12 years, median follow-up 5.1 years; 95% CI 4.5 to 5.9 years) treated with a single ASA were included; 208 (77%) and 62 (23%) patients had post-ASA LVOTO <30 and ≥30 mm Hg at the first postdischarge clinical check-up, respectively (LVOTO 13±6 vs 50±27 mm Hg; p<0.01). Freedom from cardiovascular mortality events at 1, 5 and 10 years were 99% (95% CI 96% to 100%) vs 94% (95% CI 85% to 98%), 95% (95% CI 89% to 97%) vs 80% (95% CI 66% to 89%) and 82% (95% CI 69% to 89%) vs 72% (95% CI 55% to 84%) (log-rank test, p<0.01), respectively. In multivariable analysis adjusted for age at ASA, sex, baseline LVOTO and baseline septum thickness, the independent predictors of cardiovascular mortality events were early postdischarge LVOTO ≥30 mm Hg (HR 2.95, 95% CI 1.26 to 6.91; p=0.01) and baseline septum thickness (HR 1.07, 95% CI 1.01 to 1.13; p=0.02). CONCLUSIONS: After ASA for obstructive HCM, LVOTO ≥30 mm Hg at the first postdischarge clinical check-up is associated with significantly higher occurrence of subsequent cardiovascular mortality events.

Mutations in NEBL encoding the cardiac Z-disk protein nebulette are associated with various cardiomyopathies.

INTRODUCTION: Transgenic mice overexpressing mutated NEBL, encoding the cardiac-specific Z-disk protein nebulette, develop severe cardiac phenotypes. Since cardiomyopathies are commonly familial and because mutations in a single gene may result in variable phenotypes, we tested the hypothesis that NEBL mutations are associated with cardiomyopathy. MATERIAL AND METHODS: We analyzed 389 patients, including cohorts of patients with dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), and left ventricular non-compaction cardiomyopathy (LVNC). The 28 coding exons of the NEBL gene were sequenced. Further bioinformatic analysis was used to distinguish variants. RESULTS: In total, we identified six very rare heterozygous missense mutations in NEBL in 7 different patients (frequency 1.8%) in highly conserved codons. The mutations were not detectable in 320 Caucasian sex-matched unrelated individuals without cardiomyopathy and 192 Caucasian sex-matched blood donors without heart disease. Known cardiomyopathy genes were excluded in these patients. The mutations p.H171R and p.I652L were found in 2 HCM patients. Further, p.Q581R and p.S747L were detected in 2 DCM patients, while the mutation p.A175T was identified independently in two unrelated patients with DCM. One LVNC patient carried the mutation p.P916L. All HCM and DCM related mutations were located in the nebulin-like repeats, domains responsible for actin binding. Interestingly, the mutation associated with LVNC was located in the C-terminal serine-rich linker region. CONCLUSIONS: Our data suggest that NEBL mutations may cause various cardiomyopathies. We herein describe the first NEBL mutations in HCM and LVNC. Our findings underline the notion that the cardiomyopathies are true allelic diseases.

Long-term clinical outcome after alcohol septal ablation for obstructive hypertrophic cardiomyopathy: results from the Euro-ASA registry.

AIMS: The first cases of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) were published two decades ago. Although the outcomes of single-centre and national ASA registries have been published, the long-term survival and clinical outcome of the procedure are still debated. METHODS AND RESULTS: We report long-term outcomes from the as yet largest multinational ASA registry (the Euro-ASA registry). A total of 1275 (58 ± 14 years, median follow-up 5.7 years) highly symptomatic patients treated with ASA were included. The 30-day post-ASA mortality was 1%. Overall, 171 (13%) patients died during follow-up, corresponding to a post-ASA all-cause mortality rate of 2.42 deaths per 100 patient-years. Survival rates at 1, 5, and 10 years after ASA were 98% (95% CI 96-98%), 89% (95% CI 87-91%), and 77% (95% CI 73-80%), respectively. In multivariable analysis, independent predictors of all-cause mortality were age at ASA (P < 0.01), septum thickness before ASA (P < 0.01), NYHA class before ASA (P = 0.047), and the left ventricular (LV) outflow tract gradient at the last clinical check-up (P = 0.048). Alcohol septal ablation reduced the LV outflow tract gradient from 67 ± 36 to 16 ± 21 mmHg (P < 0.01) and NYHA class from 2.9 ± 0.5 to 1.6 ± 0.7 (P < 0.01). At the last check-up, 89% of patients reported dyspnoea of NYHA class ≤2, which was independently associated with LV outflow tract gradient (P < 0.01). CONCLUSIONS: The Euro-ASA registry demonstrated low peri-procedural and long-term mortality after ASA. This intervention provided durable relief of symptoms and a reduction of LV outflow tract obstruction in selected and highly symptomatic patients with obstructive HCM. As the post-procedural obstruction seems to be associated with both worse functional status and prognosis, optimal therapy should be focused on the elimination of LV outflow tract gradient.

Comparison of sublingual isosorbide dinitrate and Valsalva maneuver for detection of obstruction in hypertrophic cardiomyopathy.

INTRODUCTION: A left ventricular outflow tract (LVOT) obstruction assessment with a provoking test should be a routine part of the evaluation of patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to compare the utility of the Valsalva maneuver (VM) and sublingual spray application of isosorbide dinitrate (ISDN) for detection of an obstruction. MATERIAL AND METHODS: We prospectively evaluated 81 consecutive HCM patients without severe rest LVOT obstruction (defined as peak rest pressure gradient (PG) ≥ 50 mm Hg). We measured PG at rest, during the VM, after sublingual ISDN spray, and during the VM after ISDN. An obstruction was defined as a PG ≥ 30 mm Hg. RESULTS: An obstruction was present in 15 patients (19%) at rest (median and interquartile range of PG 16 (7-26) mm Hg), in 38 patients (47%) during the VM (PG 28 (12-49) mm Hg), in 50 (62%) patients after ISDN (PG 50 (12-79) mm Hg), and in 55 patients (68%) during the VM after ISDN (PG 59 (20-87) mm Hg). The difference in occurrence of obstruction among different provoking tests was statistically significant for all comparisons (p < 0.001, except for the comparison of the ISDN test with the VM during ISDN, p = 0.025). CONCLUSIONS: The ISDN test and the VM are useful screening methods for the detection of an HCM obstruction. Although ISDN appears to be more precise than the VM, the best option is a combination of both methods, which maximizes inducement of LVOT obstruction in patients with HCM.

Risk and Causes of Death in Patients After Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy.

BACKGROUND: Because the final myocardial scar might be theoretically associated with an increased risk of sudden cardiac death, the long-term clinical course of patients who undergo alcohol septal ablation (ASA) is still a matter of debate. In this retrospective multicentre study, we report outcomes after ASA, including survival, analysis of causes of deaths, and association between time and cause of death. METHODS: We enrolled 366 consecutive patients (58 ± 12 years, 54% women) who were treated using ASA and followed-up for 5.1 ± 4.5 years. RESULTS: The in-hospital and 30-day mortality were 0.5% and 0.8%, respectively; the ASA-related morbidity was < 20%. Overall, 52 patients died during 1867 patient-years, which means the all-cause mortality rate was 2.8% per year. The mortality rates of sudden death and sudden death with an appropriate implantable cardioverter-defibrillator (ICD) discharge were 0.4% and 1% per year, respectively. Patients with sudden death or appropriate ICD discharge experienced these mortality events at younger age than patients who died of other hypertrophic obstructive cardiomyopathy-related causes (60.8 years [range, 52-71.5 years] vs 72.4 years [range, 64.2-75.2 years]; P = 0.048). A total of 292 patients (80%) had an outflow gradient ≤ 30 mm Hg, and 327 patients (89%) were in New York Heart Association class ≤ II at the last clinical check-up. CONCLUSIONS: ASA had low procedure-related mortality, with subsequent 1% occurrence of sudden mortality events per year and 2.8% mortality rate per year in the long-term follow-up. Patients with sudden death or ICD discharge experienced the mortality events approximately 1 decade earlier than patients who died from other causes not related to hypertrophic cardiomyopathy.

Variants in miRNA regulating cardiac growth are not a common cause of hypertrophic cardiomyopathy.

OBJECTIVES: A substantial proportion of patients with hypertrophic cardiomyopathy (HCM) do not have causative mutations in the genes for heart sarcomere. The purpose of this study was to evaluate the association between microRNA (miRNA) sequence variants and HCM. METHODS: We performed genetic testing on 56 HCM patients who had previously been found to be negative for mutations in the 4 major genes for sarcomeric proteins. The coding and adjacent regions (120-220 nt) of selected miRNAs were analyzed for the presence of sequence variants. The testing was based on PCR amplification of DNA-encoding miRNAs and subsequent denaturing capillary electrophoresis. RESULTS: A total of 3 different variants were detected in the 11 selected miRNAs. These included polymorphisms rs45489294 in miRNA 208b, rs13136737 in miRNA 367 and rs9989532 in miRNA 1-2. In the patient group, the most frequent polymorphism was in miRNA 208b (10 times) followed by miRNA 367 (7 times). Both polymorphisms were found to occur with similar frequencies in the group of healthy controls. The remaining detected variant was not present in the control group, but was not connected with the HCM phenotype in the children of the probands. CONCLUSION: Sequence variants in miRNAs of patients with HCM are not frequent and the contribution of these variants to the development of this disease was not demonstrated.

Survival of patients ≤ 50 years of age after alcohol septal ablation for hypertrophic obstructive cardiomyopathy.

BACKGROUND: The long-term efficacy and safety of alcohol septal ablation (ASA) has recently been demonstrated. However, there is still debate about the outcome of younger patients who should be treated using myectomy, according to American College of Cardiology Foundation/American Heart Association guidelines. The aim of this study was to evaluate the long-term outcome of patients ≤ 50 years of age after ASA for hypertrophic obstructive cardiomyopathy (HOCM). METHODS: We retrospectively evaluated consecutive, highly symptomatic patients aged ≤ 50 years with HOCM who underwent ASA. RESULTS: Institutional databases of 3 cardiovascular centres identified 290 patients with HOCM who underwent ASA; 75 (26%) of them were aged ≤ 50 years at the time of their first ASA. Median duration of follow-up was 5.1 years (range, 0.1-15.4 years). Four patients (5%) died during the study period (438 patient-years; the annual mortality rate was 0.91%; 95% confidence interval [CI], 0.25-2.34%; the annual mortality rate combined with the first appropriate implantable cardioverter-defibrillator discharge was 1.43%; 95% CI, 0.52-3.10%). Survival free of all-cause mortality at 1, 5, and 10 years was 97% (95% CI, 89-99%), 94% (95% CI, 84-98%), and 94% (95% CI, 84-98%), respectively. CONCLUSIONS: Results of this first study focused on HOCM patients aged ≤ 50 years who underwent ASA suggest a low risk of all-cause death or appropriate implantable cardioverter-defibrillator discharge in the long-term follow-up.

Long-term survival after alcohol septal ablation for hypertrophic obstructive cardiomyopathy: a comparison with general population.

AIMS: We decided to determine the long-term survival of patients after alcohol septal ablation (ASA) for hypertrophic obstructive cardiomyopathy (HOCM) and compare this with the general population. METHODS AND RESULTS: A total of 178 highly symptomatic, consecutive patients (58 ± 12 years, 53% women) were treated by ASA between April 1998 and April 2013 and followed-up for 4.8 years (IQR 2.1-7.5). At baseline, 155 patients (87%) suffered from dyspnoea ≥3 class of NYHA; at the most recent examination, 87 patients (49%) and 23 patients (13%) reported dyspnoea of NYHA class 1 and ≥3, respectively. The left ventricular outflow gradient was significantly reduced (68 ± 42 vs. 20 ± 25 mmHg; P < 0.01). A total of 19 deaths (11%) occurred during 925 patient-years, which means an overall mortality rate of 2.1% per year. Survival free of all-cause mortality at 1, 5, and 10 years was 97% (95% CI, 93-99%), 92% (95% CI, 87-96%), and 82% (95% CI, 70-90%), respectively. This observed mortality was comparable to the expected survival for age- and sex-comparable general population (P = 0.34). According to multivariate analysis, the only independent predictor of all-cause mortality was age at ASA (hazard ratio 1.09, 95% CI 1.04-1.14; P < 0.01). CONCLUSIONS: This study suggests that in patients with HOCM and important symptoms who underwent ASA, long-term survival after the procedure did not differ significantly from that of the general population.

Effect of rosuvastatin therapy on troponin I release following percutaneous coronary intervention in nonemergency patients (from the TIP 3 study).

Several randomized studies have suggested that pretreatment with statins may reduce a periprocedural biomarker release in patients who underwent percutaneous coronary intervention (PCI); however, results remain controversial. The purpose of this study was to investigate the effect of a 1-day rosuvastatin therapy on troponin I release in patients who underwent nonemergency PCI. A total of 445 patients with angina pectoris were randomly assigned to therapy with rosuvastatin (20 mg 12 hours before coronary angiography + 20 mg immediately before PCI; rosuvastatin group, 220 patients) or PCI without statin therapy (control group, 225 patients). In patients taking statins (73%), rosuvastatin was added to their long-term statin therapy. The primary end point was the incidence of TnI microleak defined as TnI elevation >1.5× upper limit of normal, and the secondary end point was the incidence of post-PCI TnI elevation >3× upper limit of normal. The incidence of primary and secondary end point in the rosuvastatin versus control group was 13.6% versus 12% (p = 0.61) and 8.2% versus 7.1% (p = 0.67), respectively. Patients with C-reactive protein ≥2.0 mg/L had a decreased release of post-PCI TnI in the rosuvastatin group (0.032 [0.010 to 0.143] µg/L vs 0.056 [0.018 to 0.241] µg/L; p = 0.04). In conclusion, 1-day rosuvastatin therapy (20 mg twice a day) did not influence post-PCI TnI release in patients with angina. However, these results suggest that, in patients with an advanced inflammatory status, rosuvastatin loading therapy might have a cardioprotective effect.