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1.
Isolated ventricular noncompaction 总被引:3,自引:0,他引:3
Isolated ventricular noncompaction of myocardium is a rare congenital disease due to an arrest of myocardial morphogenesis during foetal development. It is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. The persistence of myocardial noncompaction is usually an associated anomaly in patients with congenital left or right ventricular outflow tract obstruction. However, isolated noncompaction of myocardium is not associated with any factors that would explain it apart from the foetal arrest of compaction of the ventricular myocardium. The disease results in systolic and diastolic ventricular dysfunction, systemic embolism and ventricular arrhythmias. We describe a case of isolated noncompaction of the ventricular myocardium in a 20-year-old man who presented initially with ventricular tachycardia. 相似文献
2.
Right ventricular involvement in a patient with isolated noncompaction of the ventricular myocardium
Yuichi Sato Naoya Matsumoto Shinro Matsuo Yoshitaka Sakai Taeko Kunimasa Shinobu Imai Shunichi Yoda Shigemasa Tani Yuji Kasamaki Tadateru Takayama Satoshi Kunimoto Motoichiro Takahashi Satoshi Saito 《Cardiovascular Revascularization Medicine》2007,8(4):275-277
Isolated noncompaction of the ventricular myocardium (INVM) is an unclassified cardiomyopathy and is thought to be due to arrest of myocardial morphogenesis. Although right ventricular involvement is not uncommon, the correct diagnosis is often difficult by echocardiography. In this report, we describe a patient with INVM in whom magnetic resonance imaging was useful to detect right ventricular morphological and functional abnormalities. 相似文献
3.
COMA-CANELLA I.; GAMALLO C.; ONSURBE P. MARTINEZ; LOPEZ-SENDON J. 《European heart journal》1988,9(5):534-540
Isolated right ventricular infarction has been found in casesof right ventricular hypertrophy, but there are no reports onright ventricular infarction secondary to massive pulmonaryembolism. Six autopsied patients with massive pulmonary embolismand pure right ventricular infarction, suspected to be secondaryto the embolism, were selected from a population of 216 autopsies.Pulmonary embolism was the suspected diagnosis in five casesdue to typical clinical, electrocardiographic and haemodynamicdata. Right ventricular infarction was a post-mortem finding,not previously diagnosed. In every case the thickness of theright ventricular myocardium was normal. The necrosis of theright ventricle was transmural in four cases and subendcardialin two and the entire right ventricular wall (anterolateralas well as posterior) was involved. No mural thrombi were presentand in no case did the necrosis involve the left ventricle.In one case the coronary arteries were normal, in the otherfive significant lesions of the right or the left coronary arterieswere observed. These lesions may have been, in part, responsiblefor the necrosis of the right ventricle when the massive pulmonaryembolism was added. We conclude that right ventricular infarctionmay be secondary to pulmonary hypertension in the setting ofmassive pulmonary embolism, even in the absence of right ventricularhypertrophy and with normal or stenotic coronary arteries. 相似文献
4.
Regional right ventricular dysfunction in acute pulmonary embolism and right ventricular infarction. 总被引:2,自引:0,他引:2
Franco Casazza Amedeo Bongarzoni Angela Capozi Ornella Agostoni 《European journal of echocardiography》2005,6(1):11-14
BACKGROUND: A normally contracting right ventricular apex associated to a severe hypokinesia of the mid-free wall ('McConnell sign') has been considered a distinct echocardiographic pattern of acute pulmonary embolism. OBJECTIVE: To evaluate the clinical utility of the 'McConnell sign' in the bedside diagnostic work-up of patients presenting to the Emergency Department with an acute right ventricular dysfunction due to pulmonary embolism or right ventricular infarction. DESIGN: Among 201 patients, consecutively selected from our clinical database and diagnosed as having massive or submassive pulmonary embolism or right ventricular infarction, 161 were suitable for an echocardiographic review of regional right ventricular contraction and were included in the study. There were 107 cases with pulmonary embolism (group 1) and 54 cases with right ventricular infarction (group 2). All echocardiographic studies were randomly examined by two experienced and independent echocardiographers, blinded to the patient diagnosis and without Doppler informations. RESULTS: The McConnell sign was detected in 75 of 107 patients in group 1 (70%) and in 36 of 54 patients in group 2 (67%); the finding was absent in 32 cases in group 1 and in 18 cases in group 2 (P=0.657). The sensitivity, specificity, positive and negative predictive values of the McConnell sign for the diagnosis of pulmonary embolism were respectively 70, 33, 67 and 36%. CONCLUSIONS: In a clinical setting of patients with acute right ventricular dysfunction the McConnell sign cannot be considered a specific marker of pulmonary embolism. 相似文献
5.
Live three-dimensional transthoracic echocardiographic assessment of ventricular noncompaction 总被引:1,自引:0,他引:1
Bodiwala K Miller AP Nanda NC Patel V Vengala S Mehmood F Upendram S Frans E 《Echocardiography (Mount Kisco, N.Y.)》2005,22(7):611-620
We present eight adult patients with noncompaction (four with isolated left ventricular noncompaction and four with combined left and right ventricular noncompaction) in whom live three-dimensional transthoracic echocardiography (3D TTE) demonstrated multiple, prominent myocardial trabeculations, deep intertrabecular recesses communicating with the ventricular cavity, and a typical honeycombing appearance. In the four patients with combined right and left ventricular noncompaction, very extensive trabeculations in the right ventricle were identified, much more than in normal or hypertrophied right ventricles. Five of the eight patients were not definitively identified to have noncompaction on two-dimensional (2D) TTE, but the diagnosis was made with 3D TTE. These cases demonstrate the potential usefulness of 3D TTE as a supplement to 2D TTE in the assessment of noncompaction. 相似文献
6.
目的观察血压正常的急性肺栓塞患者右室超声心动指标与肺血管阻塞严重程度相关性及治疗有效性的预测价值。方法回顾我院6年间收治的56例血压正常的急性肺栓塞患者的临床资料,分析其超声心动图所测得的右室收缩末期及舒张末期面积与肺灌注扫描灌注缺损数和增强螺旋CT所代表的肺血管阻塞之间的相关性。结果与正常对照相比,肺栓塞患者右室舒张末面积和收缩末面积增大,且与肺灌注缺损数呈正相关(r=0.76,P<0.01;r=0.77,P<0.01),而舒张与收缩末面积的变化率则降低,与肺灌注缺损数呈负相关(r=-0.44,P<0.01)。增强CT显示血管阻塞越广泛,收缩末面积越大(P<0.05),舒张与收缩末面积的变化率越小(P<0.05)。治疗有效时,肺灌注缺损数随右室功能的好转而降低。结论血压稳定的急性肺栓塞患者右室功能的异常程度与肺血管的阻塞程度密切相关,右室功能的检查可用于观察肺灌注情况以及治疗有效情况的相关指标。 相似文献
7.
Acute right ventricular dysfunction has been established both as a diagnostic and prognostic indicator in pulmonary embolism. This report illustrates the utility of thallium-201 scintigraphy as an adjunctive noninvasive test in the diagnosis of pulmonary embolism by demonstrating increases in regional right ventricular perfusion and its subsequent resolution with treatment presumably as a result of decreased pressure work. 相似文献
8.
Isolated left ventricular noncompaction is a primary, genetic cardiomyopathy thought to be caused by arrest of normal embryogenesis of endocardium and myocardium, characterised by a pattern of excessively prominent trabecular meshwork and deep intertrabecular recesses with to-and-from flow in continuity with the ventricular flow and the absence of other structural heart diseases. Isolated noncompaction of the ventricular myocardium is considered to predominantly affect the left ventricle. Our earlier observations suggest that both ventricles may be involved. We present another case of isolated noncompaction of both ventricles. 相似文献
9.
Isolated noncompaction of the ventricular myo-cardium (INVM) is a rare congenital disorder characterized by an arrest of myocardial morphogenesis. We experienced a case of a 23-year-old man, referred to our emergency room, whose symptoms included shortness of breath. ECG-gated cardiac magnetic resonance imaging depicted distinctive features of INVM in both the left and right ventricles. We present the clinical characteristics and morphological features of this case in comparison with some recent reports. 相似文献
10.
Sudden cardiac death in isolated right ventricular hypertrabeculation/noncompaction cardiomyopathy
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Soad Bekheit MD PhD FHRS Boutros Karam MD Farshid Daneshvar MD Julie Zaidan MD Rabih Tabet MD Jonathan Spagnola MD James Lafferty MD 《Annals of noninvasive electrocardiology》2018,23(4)
Hypertrabeculation/noncompaction of the myocardium is a rare disorder that involves most commonly the left ventricle of the heart and it has been recognized as a distinct cardiomyopathy by the World Health Organization. However, it is extremely rare for this condition to involve exclusively the right ventricle. We report the cases of three patients who presented with ventricular tachyarrhythmia and sudden cardiac death. They were found to have isolated right ventricular hypertrabeculation/noncompaction on echocardiography. This supports the hypothesis that this condition is highly arrhythmogenic and is associated with high mortality similarly to the left ventricular hypertrabeculation/noncompaction cardiomyopathy. 相似文献
11.
Jayan P Shankarappa RK Ananthakrishna R Nanjappa MC 《Echocardiography (Mount Kisco, N.Y.)》2011,28(7):E137-E139
Isolated left ventricular noncompaction is an inherited cardiomyopathy characterized by multiple prominent trabeculations with deep intertrabecular recesses. The diagnosis is often missed because echocardiography poses inherent problems of poor echo window in assessment of the LV apex, which is most commonly involved in noncompaction. We report a case in which conventional 2D echocardiography failed to demonstrate multiple prominent trabeculations. Contrast echocardiography confirmed the presence of multiple trabeculations with deep intertrabecular recesses. This report emphasizes the importance of contrast echocardiography in the diagnosis of ventricular noncompaction. 相似文献
12.
Noncompaction of the ventricular myocardium (NVM), also known as spongy myocardium, is a rare type of cardiomyopathy that has a serious impact on fetuses, children, and adults. NVM mainly affects the left ventricle, as isolated right ventricular noncompaction (IRVNC) is rare. Pulmonary artery sling (PAS) is a rare condition in which the left pulmonary artery anomalously originates from a normal positioned right pulmonary artery, and only a few studies have reported PAS in fetuses. Fetal IRVNC complicated with PAS has not been reported yet. Here, we report a case of IRVNC complicated with PAS that was diagnosed prenatally at 30 weeks gestation and confirmed by postpartum anatomy and pathology. 相似文献
13.
Harvinder S. Dod Amit Nanda Navin C. Nanda Berthold Klas Satinder Singh Ahmed Y. Salama Raziye E. Akdogan Hanan Fadala Mahmoud G. A. Abdelsalam Mohamed Ahmed Rohil Bedi Sachin Dod Ravindra Bhardwaj 《Echocardiography (Mount Kisco, N.Y.)》2019,36(8):1581-1585
Massive and submassive pulmonary thromboembolism carry significant morbidity and mortality. We present an elderly female who was diagnosed with a submassive pulmonary embolism by computed tomographic angiography and treated with ultrasound‐facilitated thrombolysis (UFT). This case demonstrates the usefulness of right ventricular longitudinal strain measurements by two‐dimensional speckle tracking echocardiography in the evaluation of right ventricular function before and after UFT. Evaluation of right ventricle longitudinal strain by speckle tracking echocardiography may supplement other parameters in the assessment of right ventricular function in these patients. 相似文献
14.
C Cevik N Shah JM Wilson RF Stainback 《Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital》2012,39(4):550-553
The major clinical features of myocardial noncompaction are heart failure, arrhythmias, and thromboembolic events. Prominent myocardial trabeculae and deep recesses characteristic of myocardial noncompaction can cause stagnant blood flow and the formation of left ventricular clots. We describe the case of a 62-year-old woman who presented with symptoms of heart failure secondary to left ventricular noncompaction. Transthoracic and transesophageal echocardiography revealed multiple left ventricular thrombi, which had formed despite the patient's long-term therapy with aspirin.Anticoagulative therapy should be considered for patients with myocardial noncompaction who also have risk factors for thromboembolism, such as atrial fibrillation, a history of systemic embolism, or severe left ventricular systolic dysfunction. However, chronic antiplatelet therapy may not sufficiently prevent clot formation in patients who have myocardial noncompaction and severe left ventricular systolic dysfunction. 相似文献
15.
BACKGROUND: The noncompaction of myocardium is a rare myocardiopathy. The isolated right ventricular involvement is exceptional. We report two cases of isolated noncompaction of the right ventricular myocardium. CASE 1: I., 2 years old, investigated for a congenital cyanosis with severe functional repercussion. Her echocardiography revealed a complex congenital heart disease associated with isolate noncompaction of the right ventricle. CASE 2: A., 5 years old, was referred for history of syncope for the 2 last years. The echocardiography showed a severe pulmonary hypertension and an isolate noncompaction of the right ventricle. CONCLUSION: Due to the rarity of the isolate non-compaction of the right ventricle, it diagnosis remains difficult. The treatment and the outcomes of this disease are still unclear. An early diagnosis may help to reduce its complication. 相似文献
16.
Sato Y Matsumoto N Yoda S Inoue F Kunimoto S Fukamizu S Tani S Takayama T Tokai K Kasamaki Y Saito S Uchiyama T Koyama Y 《Heart and vessels》2006,21(3):192-194
A 66-year-old woman was admitted to our hospital because of left ventricular failure and nonsustained ventricular tachycardia.
Two-dimensional echocardiography demonstrated prominent trabeculations and deep intertrabecular recesses, findings consistent
with noncompaction of the ventricular myocardium. Myocardial perfusion scintigraphy demonstrated a defect in the anterobasal
left ventricular segment. Coronary angiogram was normal, but the left ventriculogram showed an aneurysm in the anterior myocardial
segments. This is the first reported case with isolated noncompaction of the ventricular myocardium associated with left ventricular
aneurysm. 相似文献
17.
Ranganathan A Ganesan G Sangareddi V Pillai AP Ramasamy A 《Echocardiography (Mount Kisco, N.Y.)》2012,29(7):E169-E172
Isolated noncompaction of ventricular myocardium (INVM) is a genetic cardiomyopathy due to abnormal arrest in endomyocardial embryogenesis between fetal 5th and 8th week. Noncompaction of right ventricle alone is rare. Here we present one such case where a young man presented with progressive right heart failure and atrial fibrillation. Subsequent evaluation by echo and cardiac magnetic resonance imaging confirmed our diagnosis. The cardinal manifestations of INVM are heart failure, arrhythmia, and embolic events and our case presented with former two manifestations. Echocardiographic criteria for diagnosing INVM are discussed. 相似文献
18.
Hem Desai Bhupinder Natt Christian Bime Joshua Dill James E. Dalen Joseph S. Alpert 《The American journal of medicine》2017,130(1):93.e29-93.e32
Background
Appropriate management of pulmonary embolism patients with right ventricular dysfunction is uncertain. Recent guidelines have stressed the need for more data on the use of thrombolytic agents in the stable pulmonary embolism patient with right ventricular dysfunction. The objective of this study is to investigate the hypothesis that thrombolytic therapy in hemodynamically stable pulmonary embolism patients with right ventricular dysfunction is not associated with improved mortality.Methods
We did a retrospective analysis using multi-institutional observational data from the Nationwide Inpatient Sample database. International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes were used to identify the patients with pulmonary embolism and right ventricular dysfunction. In-hospital mortality was defined as the primary outcome of interest.Results
Over the 4 years of the study period, 3668 patients with right ventricular dysfunction and pulmonary embolism were found, of which 3253 patients were identified as having hemodynamically stable right-sided heart failure with pulmonary embolism. There was no significant difference in mortality between hemodynamically stable pulmonary embolism patients with right ventricular dysfunction who received thrombolytic agents compared with those who did not. When outcomes were assessed for patients with right ventricular dysfunction and hemodynamic instability, a significant improvement in mortality was noted for patients with right ventricular dysfunction who received thrombolytic agents, which confirmed previous reports that thrombolytic therapy decreases mortality in pulmonary embolism patients who are hemodynamically unstable.Conclusion
Our data support the use of less aggressive treatment for stable pulmonary embolism patients with right ventricular dysfunction. These results argue against the reflexive use of thrombolytic agents in stable pulmonary embolism patients with right ventricular dysfunction. 相似文献19.
Isolated noncompaction left ventricular myocardium and polymorphic ventricular tachycardia 总被引:6,自引:0,他引:6
A 57-year-old woman with syncope was admitted. She had a family history of sudden death: two brothers had died suddenly at the age of 47. Transesophageal echocardiography showed numerous prominent trabeculations and deep intertrabecular recesses in the anterior and lateroapical zones. Isotopic left ventricular ejection fraction was 46%. Cardiac catheterization showed coronary arteries with no angiographic lesions. A prominent trabecular zone and deep intertrabecular recesses were seen in the anterior wall on left ventriculography. Right ventriculography was normal. The diagnosis of isolated noncompaction left ventricular myocardium was established. Continuous 24-h electrocardiographic registry showed episodes of polymorphic ventricular tachycardia. Programmed ventricular stimulation performed at the right ventricular apex with up to three extrastimuli failed to induce ventricular arrhythmias. Treatment with beta blockers was initiated, but short runs of polymorphic ventricular tachycardia persisted. A dual-chamber automatic implantable defibrillator was implanted. We discuss the physiopathology of the arrhythmia. It appears that several factors could be responsible for the malignant arrhythmias in this entity. 相似文献
20.
Preserved systolic function with isolated left ventricular noncompaction in an elderly patient. 总被引:1,自引:0,他引:1
Dursun Aras Omac Tufekcioglu Serkan Topaloglu Kumral Ergun Ozcan Ozeke Ali Yildiz Sule Korkmaz 《European journal of echocardiography》2006,7(1):71-74
Isolated left ventricular noncompaction (IVNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. Clinical presentations of IVNC include systolic and diastolic dysfunction, systemic embolism and ventricular arrhythmias. In recent years there has been an increasing awareness of this anomaly; however, especially in elderly, clinical characterization and natural course of IVNC are still in question. In this case, we report a case of a 78-year-old patient with the diagnosis of IVNC with preserved left ventricular (LV) systolic function. To the best of our knowledge, this is the oldest IVNC case with preserved LV systolic function in the literature. 相似文献