Diagnosing left ventricular noncompaction by echocardiography and cardiac magnetic resonance imaging and its dependency on neuromuscular disorders

BACKGROUND: Left ventricular hypertrabeculation (LVHT), also termed noncompaction LVHT, is diagnosed by echocardiography or cardiac magnetic resonance imaging (CMRI), and associated with neuromuscular disorders (NMD). The aim of this study was to assess if LVHT can be diagnosed by CMRI applying echocardiographic definitions. METHODS AND RESULTS: The CMRI images of 19 echocardiographically diagnosed LVHT patients were re-evaluated (10 female, 14-67 y of age). Left ventricular hypertrabeculation was diagnosed by CMRI in 9 cases. Patients with CMRI-diagnosed LVHT were more often females (67% versus 40%), experienced heart failure more often (100% versus 50%), had an LV end diastolic diameter > 57 mm (67% versus 40%), had an LV fractional shortening < 25% (89% versus 40%), and had a larger extension of LVHT than patients without CMRI-diagnosed LVHT. The prevalence of NMD (87%) did not differ between both groups. CONCLUSIONS: Echocardiographic definition for CMRI yielded the diagnosis of LVHT in only 47%. When looking for LVHT by CMRI, LV size, function, and extension of LVHT have to be considered. Copyright (c) 2008 Wiley Periodicals, Inc.     

Cardiac allograft vasculopathy

Cardiac allograft vasculopathy (CAV) is an accelerated form of coronary artery disease affecting both intramyocardial and epicardial coronary arteries and is observed in patients during long-term survival after cardiac transplantation. We report a case of CAV complicated with silent transmural myocardial infarction and massive left ventricular thrombus formation associated with silent pericarditis and with ischemic and non-ischemic scar tissue, as detected by cardiac magnetic resonance imaging (CMRI). The authors suggest CMRI as an additional technique along with echocardiography during follow-up of heart transplant recipients. CMRI may contribute to the early identification of areas of myocardial wall abnormalities suggestive of CAV, thus guiding diagnosis and prompt percutaneous treatment.     

Magnetic resonance imaging findings in apical ballooning syndrome or takotsubo cardiomyopathy

Cardiac magnetic resonance imaging (CMRI) plays an important role in the diagnosis and follow-up of apical ballooning syndrome (takotsubo syndrome), a recently described cardiac condition characterised by transient dyskinesia of the left ventricle secondary to an acute emotional event. We present the CMRI findings in a 53-year-old female diagnosed with apical ballooning syndrome and discuss its value in the diagnosis and follow-up of this condition.     

心尖肥厚型心肌病超声诊断特点及随访

目的 探讨心尖肥厚型心肌病超声诊断特点及预后随访.方法 对27例心尖肥厚型心肌病患者心电图、超声心动图进行1～13年(平均5.2年)随访观察.结果 27例患者心电图胸前导联异常T波深置,V_(3-5)导联最为显著,重者出现巨大倒置T波≥10 mm,胸前导联R波振幅V_4>V_5>V_3,超声心动图显示心尖部明显增厚15～37(18.0±3.3)mm,末次随访心尖部厚度(19.7 ±3.7)mm,左室心尖部厚度与左室后壁厚度比值分别为1.7±0.3和1.9±0.9,随访前后比较差异有统计学意义(P<0.05),但左室舒张末期内径及左室射血分数随访前后未见显著差异.主要心血管事件为心房颤动,心功能Ⅲ～Ⅳ级,前壁心肌梗死及心脏猝死.结论 心尖肥厚型心肌病主要依据心电图胸前导联T波深置及超声心动图心尖肥厚特点作出诊断,该病进展缓慢,一般临床预后较好.     

Clinical role, accuracy, and technical aspects of cardiovascular magnetic resonance imaging in infants

Cardiovascular magnetic resonance imaging (CMRI) has been shown to be an important alternative to echocardiography and catheterization for diagnostic imaging in older patients with congenital heart disease. However, little information is available on its clinical role, accuracy, and technical aspects in infants. We retrospectively identified all patients <1 year of age who underwent a CMRI examination at our institution over a 4-year period (from 1999 to 2002) and reviewed their medical records. Ninety-nine CMRI examinations were performed in 91 patients (median weight 4.9 kg; range 1.2 to 16.3) at a median age of 102 days (range 1 to 358). Technical modifications were used to improve signal-to-noise ratio and temporal resolution, and specialized personnel and equipment were employed. All CMRI studies were performed under general anesthesia without any complications, and all outpatients (n = 44) were discharged the same day. The primary referral questions for CMRI were delineation of the thoracic vasculature (n = 54), assessment of possible airway compression (n = 25), evaluation of cardiac tumor (n = 6), and other (n = 14). For the thoracic vasculature group, findings at surgery (n = 37) and catheterization (n = 16) were concordant with the CMRI diagnoses. In the airway compression group, findings at surgery (n = 16) were also in agreement with CMRI findings. Tumor types by CMRI in the 3 patients with preoperative studies were confirmed by histologic results. Thus, CMRI has a limited but important diagnostic role in infants with cardiovascular disease. It is an accurate and safe method to delineate the thoracic vasculature, evaluate possible airway compression, and characterize cardiac tumors. CMRI may obviate cardiac catheterization and bronchoscopy in selected cases.     

Multimodality imaging evaluation of a primary cardiac lymphoma

Primary cardiac lymphoma is a rare form of non-Hodgkin lymphoma that involves the heart with extension to pericardium and great vessels. Prognosis is poor in the absence of a prompt diagnosis and adequate therapy. Differential diagnosis includes malignant neoplasms such as angiosarcoma or metastatic carcinoma and melanoma. Clinical manifestations may be heterogeneous. Multimodality imaging work-up represents the best method for tumor detection and evaluation of its size and extension: echocardiography, computed tomography, magnetic resonance imaging, and nuclear imaging are the best imaging tools. Definitive diagnosis is achieved with cytological and histological evaluation. We report the case of a 76-year-old woman admitted to our emergency department with symptoms of congestive heart failure. Multimodality imaging work-up showed a mediastinal bulky tumor involving heart and pericardium. Pathology revealed a large B-cell primary cardiac lymphoma.     

Tissue Doppler-derived indices predict exercise capacity in patients with apical hypertrophic cardiomyopathy

BACKGROUND: Although impaired left ventricular (LV) diastolic function is a prominent feature of hypertrophic cardiomyopathy (HCM), diastolic function and its relation to exercise capacity in apical HCM (ApHCM) has not been explored previously. This study was sought to determine the relationship between diastolic mitral annular velocities combined with conventional Doppler indexes and exercise capacity in patients with ApHCM. PATIENTS: Twenty-nine patients with ApHCM (24 men; mean age +/- SD, 57 +/- 10 years) underwent supine bicycle exercise with simultaneous respiratory gas analysis and two-dimensional and Doppler echocardiographic study. RESULTS: The mitral inflow velocities (early filling [E], late filling, and deceleration time) were traced and measured. Early diastolic mitral annular velocity (E') was measured at the septal corner of mitral annulus by Doppler tissue imaging (DTI) from the apical four-chamber view. Pro-brain natriuretic peptide (proBNP) was measured at the time of echocardiography using a quantitative electrochemiluminescence immunoassay. E/E' ratio correlated inversely with maximal oxygen uptake (Vo(2)max) [r = - 0.47, p = 0.0106]. There was a significant positive correlation between E' and Vo(2)max (r = 0.41, p = 0.024). However, no correlation was found between conventional two-dimensional, Doppler indices, and proBNP and Vo(2)max). Of all the echocardiographic and clinical parameters assessed, E/E' ratio had the best correlation with exercise capacity (r - 0.47) and was the strongest independent predictor of Vo(2)max by multivariate analysis (p = 0.0106). CONCLUSIONS: DTI-derived indexes (E', E/E' ratio), an estimate of myocardial relaxation and LV filling pressures, correlate with exercise capacity in patients with ApHCM, suggesting that abnormal diastolic function may be a factor limiting exercise capacity.     

Intrinsic and Extrinsic Cardiac Pseudotumors: Echocardiographic Evaluation and Review of the Literature

Echocardiography is the most common imaging modality for the assessment of cardiovascular tumors, followed by more advanced imaging modalities, such as cardiac computed tomography or cardiac magnetic resonance imaging. Non‐neoplastic lesions that may simulate a true neoplasm on imaging are termed “cardiac pseudotumors.” As echocardiography is the initial imaging modality where pseudotumors are identified, it is imperative to have a fundamental understanding of pseudotumors evaluation using echocardiography. There is paucity of the literature describing the different kinds of pseudotumors. This review is an attempt to describe common cardiac pseudotumors and to classify them based on their origin. The tumors arising from cardiac structures, such as epicardium, endocardium, or myocardium, were termed as “intrinsic” while the pseudotumors with no cardiac origin were termed as “extrinsic.” The more common pseudotumors are described in detail with pertinent echocardiographic features and examples.     

Isolated left ventricular abnormal trabeculation in adults is associated with neuromuscular disorders

BACKGROUND: Isolated left ventricular abnormal trabeculation (ILVAT) is defined as > 3 coarse trabeculations of the left ventricular wall, apically to the papillary muscles, in hearts without congenital malformations. HYPOTHESIS: The aims of the study were to assess by echocardiography the prevalence of ILVAT, to confirm the diagnosis by cardiac magnetic resonance imaging (CMRI), to look for cardiac findings in ILVAT, and to determine whether ILVAT is familial and associated with neurological disorders. METHODS: During a 1-year period (July 1995 to July 1996) all patients in whom transthoracic echocardiography visualized ILVAT, were included in the study. The examination and measurements were performed according to established guidelines. RESULTS: During 1 year, ILVAT was found in 6 of 3,397 patients (0.2%). When applied, CMRI confirmed ILVAT. Four patients had heart failure, all had electrocardiographic (ECG) abnormalities. None of the investigated relatives showed ILVAT. One patient had Becker's muscular dystrophy, three had mitochondrial myopathy, one had polyneuropathy, and one had muscle wasting of unknown origin. CONCLUSIONS: Isolated left ventricular abnormal trabeculation is rare, visible on echocardiography and CMRI, associated with ECG abnormalities, sometimes with heart failure, and always with neuromuscular disorders. Thus, when ILVAT is found, the cardiologist should consider a neurology referral.     

Cardiac magnetic resonance imaging and transesophageal echocardiography in patients with transcatheter closure of patent foramen ovale.

OBJECTIVES: We studied the value of cardiac magnetic resonance imaging (CMRI) before and after closure of patent foramen ovale (PFO) in patients with cryptogenic ischemic events. BACKGROUND: Cardiac magnetic resonance imaging is a powerful noninvasive tool for detailed assessment of cardiac anatomy and function. The relevance of CMRI compared with transesophageal echocardiography (TEE) in patients undergoing transcatheter PFO closure has not been evaluated so far. METHODS: Contrast-enhanced CMRI and TEE were performed in 75 patients before and after PFO closure. Twelve months after PFO closure, both imaging techniques were repeated in 61 patients with contrast application. To determine provokable atrial right-to-left shunting in CMRI, we applied a contrast-enhanced perfusion imaging technique. Detection of atrial septal aneurysm (ASA) was achieved by means of a high-resolution cine imaging technique. RESULTS: Before PFO closure, ASA was seen with CMRI in 28 of 75 cases (37.3%), compared with 47 of 75 (62.7%) cases using TEE. There were a total of 211 CMRI studies with a corresponding TEE performed in 75 patients. No shunt was present in 107 of 211 studies with both techniques. Contrast-enhanced right-to-left shunting was detected by CMRI in 48 of 72 (66.6%) cases with moderate or severe shunts seen with TEE, but only in 6 of 32 (18.8%) studies with mild shunts with TEE. Anomalous venous returns were excluded in all patients. In two patients, coronary anomalies were seen. CONCLUSIONS: The present CMRI technique is inferior to TEE in detection of contrast-enhanced right-to-left shunting and identification of ASA.     

Diagnosis of cardiac abnormalities in patients with nonischemic tachyarrhythmias: additional value of MR imaging

Purpose. To assess the contribution of cardiac magnetic resonance (MR) imaging in the evaluation of patients with nonischemic tachyarrhythmias and equivocal diagnosis following echocardiography and cardiac angiography. Methods and results. Twenty-five patients with nonischemic tachyarrhythmias and equivocal diagnosis were studied using MR imaging. Before the MR examination all patients underwent two-dimensional echocardiography and cardiac catheterization. The type of additional information, obtained with MR imaging, ranged from confirming a suspected diagnosis in 5 patients (20%) to the identification of new, important anatomic or functional information, allowing a correct diagnosis in 18 patients (72%). In two patients (8%) the MR diagnosis was incomplete. Conclusion. MR imaging may be an important additional diagnostic tool in the evaluation of cardiac abnormalities in patients with nonischemic tachyarrhythmias.     

Spontaneous left atrial multiseptated intramural dissecting hematoma

Our case was initially admitted with chest discomfort and congestive heart failure with a presumptive diagnosis of non-ST segment elevation myocardial infarction and was later found to have a large left atrial (LA) mass. In addition to a complete echocardiography study, multimodality imaging was utilized to better characterize the LA mass. Despite this, the diagnosis could not be confirmed. It was decided to proceed to cardiac surgery, and surprisingly, this revealed a large LA mass with pockets of fresh blood within the LA. Pathological specimen examination confirmed a diagnosis of thrombus, effectively outruling other atrial neoplasms. Although contrast echocardiography and cardiac magnetic resonance imaging suggested this diagnosis, in the absence of any predisposing factors, the final diagnosis was made at surgery and histopathological examination.     

New real-time interactive cardiac magnetic resonance imaging system complements echocardiography

Objectives. We conducted an initial clinical trial of a newly developed cardiac magnetic resonance imaging (CMRI) system. We evaluated left ventricular (LV) function in 85 patients to compare the clinical utility of the CMRI system with echocardiography, the current noninvasive gold standard.

Background. Conventional CMRI systems require cardiac-gating and respiratory compensation to synthesize a single image from data acquired over multiple cardiac cycles. In contrast, the new CMRI system allows continuous real-time dynamic acquisition and display of any scan plane at 16 images/s without the need for cardiac gating or breath-holding.

Methods. A conventional 1.5T Signa MRI Scanner (GE, Milwaukee, Wisconsin) was modified by the addition of an interactive workstation and a bus adapter. The new CMRI system underwent clinical trial by testing its ability to evaluate global and regional LV function. The first group (A) consisted of 31 patients with acceptable echocardiography image quality. The second group (B) consisted of 31 patients with suboptimal echocardiography image quality. The third group (C) consisted of 29 patients with severe lung disease or congenital cardiac malformation who frequently have suboptimal echo study. Two independent observers scored wall motion and image quality using the standard 16-segment model and rank-order analysis.

Results. CMRI evaluation was complete in less than 15 min. In group A, no significant difference was found between ECHO and CMRI studies (p = NS). In group B, adequate visualization of wall segments was obtained 38% of the time using ECHO and 97% of the time using CMRI (p < 0.0001). When grouped into coronary segments, adequate visualization of at least one segment occurred in 18 of 30 patients (60%) with ECHO and in all 30 patients (100%) with CMRI (p < 0.0001). In group C, adequate visualization of the wall segments was obtained in 58% (CI 0.53–0.62) of the time using echocardiography and 99.7% (CI 0.99–1.0) of the time using CMRI (p < 0.0001).

Conclusions. The new CMRI system provides clinically reliable evaluation of LV function and complements suboptimal echocardiography. In comparison with the conventional CMRI, the new CMRI system significantly reduces scan time, patient discomfort and associated cost.     

Imaging in Cardiac Echinococcosis

Cardiac echinococcosis is a rare disease. Its diagnosis is sometimes difficult and requires medical imaging such as echocardiography. An 18-year-old man with multiple cardiac hydatid cysts diagnosed by echocardiography and nuclear magnetic resonance is presented. The results from these imaging techniques are presented.     

Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing

BACKGROUND: Cardiac sarcoidosis (CS) causes substantial morbidity and sudden death. Early diagnosis and risk stratification are warranted. METHODS: Ambulatory patients with sarcoidosis were interviewed to determine whether they experienced palpitations, syncope, or presyncope, and were evaluated with ECG, Holter monitoring, and echocardiography (transthoracic echocardiogram [TTE]). Those with symptoms or abnormal results were studied with cardiac MRI (CMRI) or positron emission tomography (PET) scanning. The diagnosis of CS was based on abnormalities detected by these imaging studies. Patients with CS were referred for risk stratification by electrophysiology study (EPS). RESULTS: Among the 62 patients evaluated, the prevalence of CS was 39%. Patients with CS had more cardiac symptoms than those without CS (46% vs 5%, respectively; p < 0.001), and were more likely to have abnormal Holter monitoring findings (50% vs 3%, respectively; p < 0.001) and TTE findings (25% vs 5%, respectively; p = 0.02). The degree of pulmonary impairment did not predict CS. Two of the 17 patients who underwent EPS had abnormal test findings and received implantable cardioverter-defibrillators. No patients died, had ventricular arrhythmias that triggered defibrillator therapy, or had heart failure develop during almost 2 years of follow-up. This diagnostic approach was more sensitive than the established criteria for identifying CS. CONCLUSION: CS is common among patients with sarcoidosis. A structured clinical assessment incorporating advanced cardiac imaging with PET scanning or CMRI is more sensitive than the established criteria for the identification of CS. Sarcoidal lesions seen on CMRI or PET scanning do not predict arrhythmias in ambulatory patients with preserved cardiac function, who appear to be at low risk for short-term mortality.     

Unusual left atrial mass: inverted left atrial appendage caused by massive pericardial effusion

Left atrial appendage inversion is a very rare cause of left atrial mass that usually occur after cardiac surgery. Its echocardiographic appearance often misleads clinicians to the diagnosis of thrombus or cardiac neoplasms. Unfortunately, misdiagnosed patients often undergo unnecessary anticoagulation or surgical treatment. We are presenting a case of left atrial appendage inversion caused by massive pericardial effusion in a patient with no previous history of cardiac surgery together with demonstrative images of transesophageal echocardiography and magnetic resonance imaging.     

A GPS Map for Pulmonary Hypertension: A Review of Imaging Modalities

Pulmonary hypertension (PH) is a lethal disease with a prevalence of 10-20 % in the general population. The current gold standard diagnosis approach is right heart catheterization, directly detecting pulmonary artery pressure. Imaging for PH patients plays an important role in the etiological diagnosis and evaluation of right heart (RV) function, which is a key determinant of morbidity and mortality in PH patients. Currently, echocardiography is the first-line imaging modality for screening PH and evaluating RV function. CMRI is the gold standard method to evaluate RV function. MDCT, radionuclide ventriculography and pulmonary angiography are mostly used in the differential diagnosis of the cause of PH. In this review article, we also mention several newly studied imaging modalities, such as three-dimensional echocardiography, intravascular ultrasound (IVUS) and optical coherence tomography (OCT), which showed promise for early diagnosis of PH.     

Imaging of Ventricular Septal Tumor in an Asymptomatic Adolescent using Multiple Modalities

Primary cardiac tumors are rare at all ages, and especially so in childhood, where the most prevalent type of benign cardiac masses are rhabdomyomas followed by fibromas, both of which have a predilection for ventricular septum. We report an unusual case of a tumor involving the ventricular septum in an asymptomatic 17-year-old adolescent who has been known to have this tumor for 10 years. The use of multiple imaging included myocardial contrast two-dimensional echocardiography, real time three-dimensional echocardiography (with and without myocardial contrast), and magnetic resonance imaging supported the diagnosis of cardiac rhabdomyoma.     

Impaired coronary circulation in patients with apical hypertrophic cardiomyopathy: noninvasive analysis by transthoracic Doppler echocardiography

OBJECTIVES: We designed this study to examine the characteristics of coronary circulation in patients with apical hypertrophic cardiomyopathy (ApHCM) using noninvasive transthoracic Doppler echocardiography (TTDE). BACKGROUND: Recent advances in TTDE have allowed noninvasive assessment of coronary circulation by the measurement of coronary flow velocity (CFV) patterns and coronary flow velocity reserve (CFVR). However, there have been no previous studies evaluating coronary circulation in ApHCM. METHODS: We analyzed CFV and CFVR in the left anterior descending coronary artery (LAD), and apical wall thickness in the left ventricle, in 10 ApHCM subjects and 10 control subjects. Mean diastolic velocity (MDV) and time from the beginning of diastole to peak velocity (TPV), and CFVR, defined as a ratio of drug-induced hyperemic to basal MDV, were measured. RESULTS: At baseline, MDV was higher, and TPV was longer, in ApHCM subjects than in control subjects (29 +/- 5.7 versus 19 +/- 6.5 cm/sec; p < 0.01 and 5.2 +/- 1.0 versus 3.5 +/- 0.6 msec; p < 0.005, respectively). CFVR in ApHCM subjects was significantly lower than in control subjects (1.9 +/- 0.4 versus 3.1 +/- 0.8; p < 0.005). CFVR and basal MDV in ApHCM subjects showed significant correlations with apical/posterior wall thickness ratio [CFVR; r =-0.84, p < 0.01 and MDV; r = 0.74, p < 0.05, respectively]. CONCLUSION: Noninvasive coronary flow assessment by TTDE revealed an impaired coronary circulation with reduced CFVR, high MDV at baseline and prolonged TPV. These results suggest that these characteristics of coronary circulation may provide an additional index for the assessment of ApHCM.