Evaluation of the prognostic value of papillary muscle-free strain in patients with hypertrophic cardiomyopathy.

BACKGROUND: The prognosis of hypertrophic cardiomyopathy (HCM) varies from mild disease with a normal life expectancy to heart failure and sudden cardiac death (SCD). The identification of patients who are at high risk for SCD remains challenging. AIMS: In this study, we evaluated the prognostic value of papillary muscle-free strain in HCM patients. METHODS AND RESULTS: Seventy-nine patients with a diagnosis of HCM were included in this study. Patients were divided into low/intermediate-risk (n = 57) and high-risk (n = 22) groups. Two-dimensional (2-D) echocardiography and strain imaging were performed for each patient. The mean age of the study population was 53.85 ± 15.88 years; 47 (59.5%) of them were male. During a mean follow-up duration of 74.45 ± 17.03 months, 12 patients died. A comparison of the low-intermediate and high-SCD risk groups revealed that patients in the high-SCD risk group had greater maximal wall thickness, interventricular septum thickness, posterior wall thickness, and left ventricular mass index (LVMI) and lower (less negative) global longitudinal, anterolateral papillary muscle (ALPM) and posteromedial papillary muscle (PMPM) free strain. Additionally, a history of syncope and ICD implantation were found to be more common in patients with high SCD risk scores. The SCD risk score was positively correlated with the global longitudinal strain, ALPM-free strain, and PMPM-free strain (r = .528, r = .658, and r = .600, respectively; p < .001 for all). Our results showed that the LVMI, presence of syncope, global longitudinal strain, and ALPM-free strain were predictors of death. CONCLUSIONS: Decreased papillary muscle-free strain values might have prognostic value in patients with HCM.

Persistent right heart dilatation after percutaneous mitral balloon valvuloplasty: A weird coexistence of iatrogenic and congenital shunts.

We present an interesting case of concomitant congenital anomalies with an iatrogenic defect. The female patient underwent a percutaneous mitral balloon valvuloplasty due to rheumatic mitral stenosis. Unfortunately, an iatrogenic atrial septal defect (ASD) transpired during the procedure. Upon post-procedure examination, partial anomalous pulmonary venous (PAPVR) return was observed. The patient was symptomatic; on imaging, dilatation of the right heart chambers were detected. In addition, another crucial point was that the patient was planning a pregnancy, thus robotic surgery for iatrogenic ASD and PAPVR return was recommended. After a successful operation, the patient was asymptomatic and the size of right heart chambers were normalized.

Giant pseudoaneurysm masquerading as large pericardial effusion in a patient with old myocardial infarction.

Pericardial effusions are common in patients with anterior myocardial infarction and resolve slowly after it. The diagnosis is generally established via echocardiography. However, a persistent, large pericardial effusion after the myocardial infarction may mask a more hazardous complication: pseudoaneurysm. Herein, we report a young gentleman presenting with dyspnea who had myocardial infarction and was misdiagnosed with large pericardial effusion.

Crochetage sign may predict late atrial arrhythmias in patients with secundum atrial septal defect undergoing transcatheter closure.

BACKGROUND: Atrial arrhythmias are well-known complications of atrial septal defect (ASD), and associated with substantial morbidity. After ASD closure, right atrial and ventricular enlargement regresses, however, the risk of atrial arrhythmia development continues. In this study, we aimed to investigate the relationship between the Crochetage sign, which is a possible reflection of heterogeneous ventricular depolarization due to long-term hemodynamic overload, and the development of late atrial arrhythmia after ASD closure. METHODS: This retrospective study included a total of 314 patients (mean age: 39.5 (30-50) years; male: 115) who underwent percutaneous device closure for secundum ASD. The study population was divided into two groups according to the presence or absence of the Crochetage sign. The Crochetage sign was defined as an M-shaped or bifid pattern notch on the R wave in one or more inferior limb leads. Cox-regression analysis was performed to determine independent predictors of late atrial arrhythmia development. RESULT: Fifty-seven patients (18.1%) presented with late atrial arrhythmia. Of these 57 patients, 30 developed new-onset atrial fibrillation/atrial flutter (AF/AFL), and 27 patients with pre-procedure paroxysmal AF/AFL had a recurrence of AF/AFL during follow-up. History of paroxysmal AF/AFL before the procedure (HR: 4.78; 95% CI 2,52-9.05; p < 0.001), the presence of Crochetage sign (HR: 3.90; 95% CI 2.05-7.76; p < 0.001), and older age at the time of ASD closure (HR: 1.03; 95% CI 1.01-1.06; p = 0.002) were found as independent predictors for late atrial arrhythmia. CONCLUSION: The presence of Crochetage sign may be used to predict the risk of late atrial arrhythmia development after transcatheter ASD closure.

Evaluation of subvalvular pannus formation in mechanical aortic valve by transesophageal echocardiography.

Subvalvular pannus formation is a rare but clinically important complication with prosthetic valves. Transthoracic echocardiography still plays a role in the initial assessment of mechanical aortic valve dysfunction. But evaluation with transesophageal echocardiography is more useful. Subvalvular area may be a blind spot of echocardiography due to acoustic shadowing. The case presented herein underlines the significant role of transgastric plane and three-dimensional TEE in the assessment of subvalvular pannus formation in mechanical aortic valves.

Mitral-aortic intervalvular fibrosa pseudoaneurysm causing compression of superior vena cava and right atrium.

The mitral-aortic intervalvular fibrosa (MAIVF) is a fibrous, avascular region between the anterior leaflet of the mitral valve and noncoronary cusp of the aortic valve. This makes MAIVF vulnerable to injury and infection; thus pseudoaneurysm may develop. The pseudoaneurysm can cause compression to coronary arteries which causes angina or pulmonary artery resulting in pulmonary hypertension. We presented the pseudoaneurysm of MAIVF causing compression of superior vena cava and right atrium which was visualized by two- and three-dimensional transesophageal echocardiography and cardiac computed tomography.

Multimodality imaging of an aortic graft infection.

Aortic prosthetic vascular graft infections (PVGI) are rare complications of the aortic surgery, with an incidence of 0.5%-6%. Although rare, they carry mortality rates as high as 25%-88%. Improvement in diagnostic imaging modalities in the last two decades, together with early diagnosis, aortic PVGI associated mortality was reduced. These imaging methods include transesophageal echocardiography (TEE), computed tomography angiography (CTA), and 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT). In this case report, we focus mainly on three imaging modalities as TEE, CTA, and 18F-FDG PET/CT and showed their role in the assessment of aortic PVGI.

Acute myocardial infarction complicated with ventricular septal rupture and intracranial hemorrhage.

Ventricular septal rupture (VSR) is a rare but fatal complication of acute myocardial infarction (AMI) with an associated mortality that ranges from 41% to 80%. The treatment consists of supplemental oxygenation, afterload reduction, intraaortic balloon pump, and surgical repair. In selected patients, extracorporeal membrane oxygenation (ECMO) and/or percutaneous closure of the defect can be considered if anatomically appropriate. Echocardiography evaluates the morphology and location of the defect, anatomical concerns for percutaneous closure, and accompanying pathologies. We present a 48-year-old man with inferior myocardial infarction and basal VSR who was not a candidate for percutaneous closure. Surgery was planned, but he died from extensive subarachnoid and intracranial hemorrhage.

Infarction of the septomarginal band and tricuspid papillary muscle rupture related to alcohol septal ablation for hypertrophic cardiomyopathy.

We presented a 77-year-old man with hypertrophic obstructive cardiomyopathy applied with flail tricuspid leaflet and severe tricuspid regurgitation leading to right heart failure 2 months after the failed septal ablation. The ruptured anterior tricuspid papillary muscle resulted from infarction of the base of anterior papillary muscle of the right ventricle (RV) confirmed by magnetic resonance imaging. As the septomarginal band is frequently lit up by intracoronary contrast that particular attention should be paid to the RV papillary muscles. And, if the papillary muscles or the RV free wall is brightened, then the use of that septal artery should be avoided.

Red Cell Distribution Width with CHADS2 and CHA2DS2-VASc score is associated with Post-operative Atrial Fibrillation after Coronary Artery Bypass Grafting.

BACKGROUND: The use of the CHA2DS2-VASc scoring system and red cell distribution width (RDW) as post-op Atrial Fibrillation (POAF) predictors may be promising for the identification of patients that are at a higher risk of POAF. METHODS: A total of 358 patients (57 patients with POAF, and 301 patients with non-POAF ) with sinus rhythm undergoing a coronary artery bypass graft (CABG) operation were included in the study retrospectively. Preoperative RDW levels and electrocardiograms with sinus rhythm were recorded. Patients with at least one 12-lead electrocardiogram with atrial fibrillation in the postoperative period, with or without medical or electrical cardioversion, were considered to have postoperative atrial fibrillation. A CHADS2 and CHA2DS2-VASc score was calculated for all of the patients. RESULTS: RDW levels were significantly higher in POAF group. RDW levels were significantly correlated with CHADS2 ( r = 0.15, P = .007) and CHA2DS2-VASc (r = 0.19 P = .0001) scores. CHA2DS2-VASc scores were significantly higher in patients with POAF, whereas CHADS2 scores did not differ between groups. In multivariate analysis, left atrial diameter (LAD) (OR:2.44 [95% CI 1.16 - 5.1], P = .018), age (OR:1.04 [95% CI 1.01 - 1.08], P = .01), and RDW (OR:1.16 [95% CI 1.0 - 1.36], P = .05) were found to be predictive for POAF. The area under the receiver-operating characteristic curve of RDW was 0.65 (0.57 - 0.72, P = .0001) with 68.4% sensitivity and 51.2 % specificity to predict POAF. CONCLUSION: Our study showed that age, LAD, and the reduced probability of RDW are predictors of POAF, and that RDW is strongly associated with the thromboembolic risk as determined by CHADS2 and CHA2DS2-VASc scores.

Is right atrioventricular valve always tricuspid?

A 71-year-old male with a medical history of hypertension was admitted to emergency department with hypertensive pulmonary edema. The patient appeared anxious and diaphoretic, and physical examination revealed tachypnea, crepitant rales in the both lungs and a systolic ejection murmur at the right sternal border, radiating to the both carotid arteries. The electrocardiography showed sinus tachycardia with indications of left ventricle hypertrophy Before the aortic valve surgery, transesophageal echocardiography (TEE) was performed for detailed evaluation of the aortic valve. Two-dimensional TEE showed mild tricuspid valve regurgitation and only two of three leaflets of the tricuspid valve at a time.

Evaluation of the congenital supravalvular aortic stenosis by different imaging modalities.

A 36-year-old female was admitted to hospital exhibiting chest pain, dyspnea, and a heart murmur on the right upper sternal border, radiating to both carotid arteries. The blood pressure of the patient's right arm exceeded the pressure in the left by 25 mm Hg (Coanda effect). In spite of laboratory results that did not fall outside the expected range, the left ventricle was revealed to be hypertrophic following electrocardiography. Transthoracic echocardiography revealed a severe supravalvular aortic stenosis (SVAS) with a peak Doppler velocity of 6.04 cm/s and an estimated mean pressure gradient of 89 mm Hg, with moderate aortic and mitral regurgitation. Contrast-enhanced computed tomography (CCT) indicated a partial hourglass-shaped narrowing of the ascending aorta. Lesions associated with supravalvular stenosis of the pulmonary artery, patent ductus arteriosus, and aortic coarctation were ruled out by the CCT. Congenital SVAS is a rare heart condition, and three anatomically distinct forms have been described. The most common type is the "hourglass," which produces a marked thickening and disorganization of the aortic tissue, producing a constricting annular ridge at the superior margin of the sinuses of Valsalva.

A rare complication of mechanical aortic valve replacement: Separation in the region of the mitral-aortic intervalvular fibrosa.

A 28-year-old man was admitted to our emergency service with a shortness of breath and palpitation. On admission, his blood pressure was high and he was in hypertensive pulmonary edema. His physical examination showed rales in both lungs and pansystolic murmur at mitral focus. His medical history included aortic valve replacement (AVR) because of native aortic valve infective endocarditis. Transthoracic echocardiography (TTE) showed normal functional aortic valve. Color flow imaging demonstrated severe mitral regurgitation with posterior eccentric jet. To examine in detail, transesophageal echocardiography (TEE) and three-dimensional (3D) echocardiography were performed. TEE disclosed a separation in the subaortic curtain leading to severe mitral regurgitation from the left ventricle to the left atrium. In addition to severe mitral regurgitation with posterior eccentric jet, 26-mm-long pouch was seen in mitral-aortic intervalvular fibrosa (MAIVF) at 120° TEE view. This pouch was separated from the mitral anterior leaflet junction releasing the mitral anterior leaflet and causing prolapse and chorda rupture in the A2 scallop of the mitral anterior leaflet. The MAIVF connects the anterior mitral leaflet to the posterior portion of the aortic annulus. The separation of the MAIVF represents a complication of the aortic valve replacement.

Spontaneous embolization of an atrial septal defect occluder device into the left ventricular outflow tract in a patient with pulmonary stenosis.

A 24-year-old man was admitted to our outpatient clinic for his routine checkup of consecutively percutaneously treated atrial septal defect (ASD) and pulmonary valvular stenosis 45 days ago. A 24 mm ASD occluder device was implanted under transthoracic echocardiographic guidance and 80 mm Hg peak-to-peak pulmonary valvular gradient decreased to 20 mm Hg gradient after pulmonary valve dilatation with 23 mm NUMED II transluminal valvuloplasty catheter balloon. Atrial septal defect (ASD) closure is now routinely performed using a percutaneous approach under echocardiographic guidance especially transthoracic echocardiography (TEE). Centrally located, ostium secundum type and less than 3.5 cm in size are considered ideal for device closure. Although there is considerable variation in size and location of the defects, TEE guidance is quite important for this proportion of ASDs. The selection of patients for percutaneous transcatheter closure of a secundum ASD requires accurate information regarding the anatomy of the defect such as its maximal diameter and the amount of circumferential tissue rims.

Value of latent outflow obstruction to predict clinical course of patients with hypertrophic cardiomyopathy.

Objective The frequency, significance and prognostic value of left ventricle obstruction (LVO) induced with provocation (latent LVO) is controversial for hypertrophic cardiomyopathy (HC) patients. This study was designed to assess the value of latent LVO in predicting the clinical course in 101 patients with HC. Methods and results Patients were followed for a mean of 82 ± 48 months (range 2 to 148 months) for clinical end points defined as a composite of cardiovascular death resuscitated cardiac arrest, appropriate defibrillator shock or hospitalization due to worsening of heart failure symptoms. Presence of LVO (hazard ratio 3.63; 95% confidence interval, 1.85 to 7.12; P = 0.0001) and log NT-proBNP levels (hazard ratio, 1.40; 95% confidence interval, 1.14 to 1.72; P = 0.001) were the independent variables associated with an increased risk of experiencing clinical end points. HC patients with latent LVO have a trend toward decreased survival when compared with HC patients without LVO (log rank P = 0.027), but better survival than patients with resting LVO (log rank P = 0.007). HC patients with NT-proBNP levels <1,000 pg/ml had also better survival. LVO and NT-proBNP levels are the major determinants of clinical end points in patients with HC. Conclusions Evaluation of patients without resting LVO to demonstrate latent obstruction is of critical importance in respect of outcome and selection of patients for septal reduction therapies, so routine provocative testing with physiological exercise and measurement of NT-proBNP is recommended in this patient population for risk stratification.

Left atrial remodeling in patients undergoing percutaneous mitral valve repair with the MitraClip system: an advanced echocardiography study.

BACKGROUND: The goal of this study was to determine changes in left atrial (LA) function with two-dimensional speckle tracking echocardiography (2DSTE) and real-time full-volume three-dimensional echocardiography (RT3DE) after percutaneous mitral valve repair with the MitraClip system. Furthermore, we investigated whether baseline and/or changes in LA function after MitraClip repair had any impact on prognosis. METHODS: A total of 25 consecutive patients (age: 57±12 years, 76% male) with moderate-to-severe or severe mitral regurgitation (MR) were included. Patients underwent 2DSTE and RT3DE before the clip implantation and after the 12-month follow-up. Prognostic data were also recorded via the use of telephone calls and follow-up visits for 12 months after the procedure. RESULTS: Compared with the baseline, the LA reservoir strain (LA-Res) (7.66±4.3% vs 11.15±7.5%, P<.001) and LA contraction strain (LA-Pump) (4.64±4.3% vs 7.63±5.8%, P=.001) improved significantly after MitraClip repair; significant improvements were also seen in three-dimensional (3D) minimum LA volume index (LAV min) and maximum LA volume index (LAV max). On the other hand, conventional LA indices did not change. In total, eleven major adverse cardiac events (MACE) were observed at the 1-year follow-up. In univariate analyses, the preprocedural echocardiographic parameters that were associated with the MACE within 1 year after MitraClip repair were 3D-LAV min and LA-Res. Furthermore, these indices significantly correlated with improved functional parameters and MR reduction. CONCLUSION: In conclusion, a successful MitraClip procedure can reverse the process of LA remodeling within 12 months, and this can be detected by 2DSTE and RT3DE. Also, patients with preprocedural lower LA-Res and higher 3D-LAV min had the worst prognoses at the 1-year follow-up.

An unexpected complication of the MitraClip device: one of the arms gets stuck inside the guide catheter in the left atrium.

Percutaneous edge-to-edge mitral valve repair using the MitraClip (Abbot Vascular, USA) system is a promising technique for mitral regurgitation treatment in select high-risk surgical patients. Although the safety and efficacy of the technique have been demonstrated, a few complications of the MitraClip device have been reported. In this report, we present a rare complication that recently occurred during the performance of a MitraClip procedure in a patient with severe functional mitral regurgitation. One MitraClip arm got stuck inside the guide catheter in the left atrium and a decision was made to discontinue percutaneous intervention because the problem could not be resolved.

Thrombolytic therapy for the treatment of prosthetic heart valve thrombosis in pregnancy with low-dose, slow infusion of tissue-type plasminogen activator.

BACKGROUND: Prosthetic valve thrombosis during pregnancy is life-threatening for mother and fetus, and the treatment of this complication is unclear. Cardiac surgery in pregnancy is associated with very high maternal and fetal mortality and morbidity. Thrombolytic therapy has rarely been used in these patients. The aim of this study is to evaluate the safety and efficacy of low-dose (25 mg), slow infusion (6 hours) of tissue-type plasminogen activator for the treatment of prosthetic valve thrombosis in pregnant women. METHODS AND RESULTS: Between 2004 and 2012, tissue-type plasminogen activator was administered to 24 consecutive women in 25 pregnancies with 28 prosthetic valve thrombosis episodes (obstructive, n=15; nonobstructive, n=13). Mean age of the patients was 29±6 years. Thrombolytic therapy sessions were performed under transesophageal echocardiography guidance. The mean dose of tissue-type plasminogen activator used was 48.7±29.5 mg (range, 25-100 mg). All episodes resulted in complete thrombus lysis after thrombolytic therapy. One patient had placental hemorrhage with preterm live birth at the 30th week, and 1 patient had minor bleeding. CONCLUSIONS: Low-dose, slow infusion of tissue-type plasminogen activator with repeated doses as needed is an effective therapy with an excellent thrombolytic success rate for the treatment of prosthetic valve thrombosis in pregnant women. This protocol also seems to be safer than cardiac surgery or any alternative medical strategies published to date. Thrombolytic therapy should be considered first-line therapy in pregnant patients with prosthetic valve thrombosis.

PE with ST-segment elevation in leads V1-3 and AVR treated successfully by catheter directed high-dose bolus thrombolytic therapy during CPR.

A 55-year-old man presented with the emergency department after having a short syncopal episode and angina during the exertion for 1 month. His initial electrocardiogram showed minimal ST-segment changes on precordial leads. While waiting for the laboratory tests, abruptly, the patient went into cardiopulmonary arrest. After a short resuscitation, a new electrocardiogram revealed ST-segment elevations in leads V1-3 and AVR, mimicking an anteroseptal myocardial infarction. Although, the angiography showed severe coronary artery disease, coronary flow was normal and main branches of pulmonary artery were almost fully occluded by large pulmonary emboli. Recombinant tissue plasminogen activator bolus (25 mg) was given 2 times at 5-minute intervals immediately into pulmonary artery by pig-tail catheter under the cardiopulmonary resuscitation. The patient had an excellent response to high-dose bolus thrombolytic therapy. We conclude that in the case of massive pulmonary embolism with small chance of resuscitation, the catheter-directed high-dose bolus injection of recombinant tissue plasminogen activator could enrich the therapeutical possibilities.