Left ventricular noncompaction associated with a sinoatrial node artery originating from the posterolateral branch of the right coronary artery

Noncompaction of the ventricular myocardium (NVM) is a rare unclassified cardiomyopathy which is characterized by multiple prominent trabeculations and deep intertrabecular recesses. This cardiomyopathy can be isolated or in combination with other congenital cardiac disorders, including coronary artery abnormalities. A 56-year-old female patient presented to the cardiology department with complaints of exertional dyspnoea and chest pain. Transthoracic echocardiography revealed left ventricular dilatation with diffuse hypokinesis. Multiple prominent trabeculations with deep inter-trabecular recesses were observed at the left ventricular apex. Also, coronary angiography demonstrated a sinoatrial node artery originating from the posterolateral branch of the right coronary artery.     

成人左室心肌致密化不全的超声心动图诊断

目的探讨成人左室心肌致密化不全的超声心动图特点。方法对45例成人左室心肌致密化不全患者进行超声心动图检查,多切面重点观察左室心肌与心内膜。结果超声心动图检查表明,左室腔内可见异常粗大呈蜂窝状的肌小梁和交错深陷的隐窝,其中34例有心力衰竭症状,3例进行心脏移植,11例患者受累心肌局限在左室心尖部,无任何症状。彩色多普勒可探及隐窝内有血流与心腔相通。结论超声心动图可快速、无创地诊断心肌致密化不全。     

Isolated noncompaction of the left ventricular myocardium in an elderly patient.

Noncompaction of the ventricular myocardium (NVM) is a rare disorder of endomyocardial morphogenesis characterized by numerous, prominent trabeculations and deep intertrabecular recesses. It is commonly associated with congenital heart disease, but the isolated form (INVM) is not associated with other structural heart diseases. Clinical reports of INVM have been limited to a few case reports and small series of pediatric patients. INVM is considered to be a form of congenital abnormal endomyocardial morphogenesis caused by abnormal cessation of the embryonic development of the ventricular myocardium; most reported cases have been pediatric patients, and autopsy cases of elderly patients have been quite rare. In the present case, an elderly female had INVM associated with severely disturbed left ventricular (LV) function and an enlarged left ventricle similar to dilated cardiomyopathy. The echocardiogram showed prominent trabeculations and deep intertrabecular recesses of the LV walls, especially in the posterior and apical areas. LV contrast echocardiography revealed markedly protruberant trabeculations, which were also observed with computed tomography. Five years later, the patient died of refractory heart failure and ventricular fibrillation. The autopsy revealed numerous excessively prominent trabeculations in the LV myocardium, with deep intertrabecular recesses containing thrombi.     

Myocardial Noncompaction Presenting With Myocardial Bridge: A Case Report

Myocardial noncompaction, namly isolated noncompaction of the left ventricular myocardium (NVM), is a rare congenital disease. It can be either seen in the absence of other cardiac anomalies, or associated with other congenital cardiac defects, mostly stenotic lesions of the left ventricular outflow tract. A myocardial bridge (MB) is thought being associated with coronary heart disease, such as coronary spasm, arrhythmia, and so on. The significance of MB in association with other congenital cardiac conditions is unknown.We report a novel case who was presented NVM and MB. A 34-year-old man complained of chest prickling-like pain and dizzy for 1 year. His blood pressure was 110/70 mm Hg. Echocardiograph revealed increased trabeculations below the level of papillary muscle of left ventricle (LV); deep intertrabecular recesses in the endocardial wall of LV particularly in apex free wall; and LV ejection fraction of 57%. A coronary computerized tomography scan showed that part, 38.9 cm, of left descending artery tunnel was surrounding by cardiac muscles rather than resting on top of the myocardium.The therapeutics interventions included lifestyle cares, agents of anti-ischemia and improvement myocardial cell metabolism. The patient was followed up for 2.6 years, and his general condition was stable.This case indicates that NVM can be developed with MB, and the complete diagnosis of NVM and MB should be made by different image studies.     

Imaging characteristics of anomalous left coronary artery from the pulmonary artery

SUMMARY: The authors report the imaging characteristics of the anomalous origin of the left coronary artery from the pulmonary artery in a 6 1/2-month-old male infant using electrocardiography, echocardiography, angiography, and electron-beam computed tomography. The patient presented with tachycardia, prolonged and interrupted feeding, failure to thrive, and myocardial infarction, and after diagnosis of the anomalous origin of the left coronary artery, survived the reimplantation of the left coronary artery directly to the ascending aorta. When reviewed 7 months after surgery, echocardiography showed improved left ventricular function and surface electrocardiography showed regression of pathologic Q waves in leads I, V 4, V 5, and V 6.     

Successful transcatheter coil embolization of coronary artery to left ventricular fistula associated with absent pulmonary valve with tricuspid atresia in early infancy.

Transcatheter coil embolization for coronary artery to left ventricular fistula was successfully performed in a neonate. At 30 weeks' gestation, fetal echocardiography showed a hypoplastic right ventricle with intact ventricular septum, absent pulmonary valve, tricuspid atresia, and marked distension of the right coronary artery. After birth, the neonate had congestive heart failure and the electrocardiogram showed myocardial ischemic changes in the left ventricular area. Aortography showed a dilated right coronary artery arising from the ascending aorta and draining into the left ventricle. Transcatheter coil embolization was carried out on the 9th day after birth. Since the procedure, no myocardial ischemic changes have been detected. Transcatheter coil embolization is a useful therapy for coronary artery fistula associated with myocardial ischemia.     

Non-invasive diagnosis of a right coronary artery-right ventricle fistula by Doppler echocardiography]

A six-year-old girl with an atypical continuous precordial murmur, was suspected of having a coronary arteriovenous fistula. A markedly dilated right coronary artery was revealed by two-dimensional echocardiography. The pulsed Doppler examination showed a diastolic retrograde flow in the ascending aorta, with a normal flow in the left ventricular outflow tract. With the sample volume in the right ventricle a continuous turbulent flow was observed. Color flow mapping showed a turbulent, systolic-diastolic flow in the right ventricle. The fistula was confirmed by cardiac catheterization.     

A case of anomalous origin of circumflex artery from right sinus of valsalva recognized by transesophageal echocardiography]

A 56-year-old man visited our hospital for further examination of resting electrocardiographic abnormality. Positive exercise stress resulted in mild chest discomfort and 1.5 mm ST depression in II.III.aVF leads. Myocardial imaging perfusion with thallium-201 was normal and an anomalous origin of the left circumflex coronary artery from the right aortic sinus was observed by coronary angiography. MRI showed that the vessel running behind the aorta was connected to the right Valsalva's sinus. Furthermore, transesophageal echocardiography revealed that the vessel connected to the lateral wall of the left ventricle was running from the right Valsalva's sinus between the aorta and left atrium. The above results indicated that this vessel was the left circumflex artery. Although myocardial infarction or sudden death in patients with coronary anomaly has been discussed, the mechanism is still unknown. The present case is a rare one in which the anatomical relation between the anomalous coronary and the great vessels was directly detectable by transesophageal echocardiography. Transesophageal echocardiography is useful for the assessment of this type of coronary anomaly.     

Two dimensional echocardiographic assessment of communications between ascending aorta and pulmonary trunk or individual pulmonary arteries.

The value of two dimensional echocardiography in identifying communications between the ascending aorta and pulmonary trunk or individual pulmonary arteries was assessed in 24 children, all of whom had either angiocardiographic and surgical or angiocardiographic confirmation alone. Fourteen cases had truncus arteriosus, four aortopulmonary window, four anomalous origin of the left pulmonary artery from the ascending aorta, and two anomalous origin of the right pulmonary artery from the ascending aorta. It was possible to identify reliably each individual abnormality with a combination of suprasternal, precordial, and subcostal cuts. Problems only arose in differentiating truncus arteriosus from pulmonary atresia and ventricular septal defect when the main pulmonary artery and infundibular region of the right ventricle were extremely hypoplastic.     

Unusual congenital coronary anomaly and myocardial ischaemia

Angiography was used to diagnose a rare congenital coronary anomaly with myocardial ischaemia in a woman with typical angina. All three coronary arteries arose from a solitary coronary ostium in the right aortic sinus; the left anterior descending coronary artery followed a septal course, the circumflex coronary artery ran behind the ascending aorta, and the right coronary artery followed a normal course. No significant coronary lumen narrowing was found. Transoesophageal echocardiography confirmed the anomalous origin and course of the aberrant coronary arteries. An exercise test reproduced angina, and ECG changes and myocardial perfusion study showed an anterior reversible defect. In contrast to previous reports, myocardial ischaemia was associated with the septal (intramuscular) course of the left anterior descending coronary artery; there was no other significant coronary artery disease.


Keywords: congenital heart defects; myocardial ischaemia; angiography; echocardiography     

心肌致密化不全11例的临床与彩色多普勒超声心动图

目的 探讨彩色多普勒超声心动图诊断心肌致密化不全的临床价值.方法 心肌致密化不全11例,年龄11个月～75岁,用GE VIVID7,SEQUOIA 256彩色超声仪,探头频率2.5～4.5MHz,分析其超声特点及临床表现.结果 11例患者左心室不同程度扩大,心肌收缩功能减退,心尖部心肌由两层构成,外层致密心肌边薄,内层心肌呈小梁网状海绵样结构,有丰富的隐窝,海绵样心肌厚度与致密心肌厚度比值为2.5～5.4,彩色多普勒显示隐窝内低速血流与左心室腔相通.结论 左心室心肌致密化不全有明显的超声特征,彩色多普勒超声心动图是惟一的诊断手段.     

Abnormal origin of the right pulmonary artery from the ascending aorta. Echocardiographic diagnosis apropos of 2 cases

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation. Formerly identified at angiography and during surgery, it can now be diagnosed by two-dimensional echocardiography, as was done in the two cases reported here. The first case was a neonate in heart failure in whom echocardiography revealed the anomalous origin of the right pulmonary artery; this diagnosis was confirmed at post-mortem examination. The second case was a 4-month old infant in heart failure; echocardiography displayed the malformation which was confirmed at angiography and surgery. In both cases echocardiography showed discontinuity between the pulmonary trunk and the right pulmonary artery and continuity between the pulmonary trunk and the left pulmonary artery. There also was continuity between the right pulmonary artery and the ascending aorta. Doppler ultrasound showed a systolic and a diastolic flow in the right pulmonary artery and only a systolic flow in the left pulmonary artery. A colour-coded doppler examination in one case confirmed the anomalous origin of the right pulmonary artery and the absence of any other abnormality. Two-dimensional echocardiography combined with colour-coded doppler ultrasound therefore is the method of choice to diagnose anomalous origin of the right pulmonary artery from the ascending aorta.     

Contrast echocardiography for the assessment of myocardial viability

PURPOSE OF REVIEW: The availability of an accurate, non-invasive method for distinguishing viable from irreversibly damaged myocardium, after acute myocardial infarction or in chronic coronary artery disease, is important in clinical decision making. Such a tool would enable physicians to identify patients most likely to benefit from revascularization strategies in patients with coronary artery disease and left ventricular dysfunction. Myocardial contrast echocardiography is a new technique that utilizes acoustically active gas-filled microspheres (microbubbles), which remain exclusively in the intravascular space and allow the simultaneous assessment of global and regional myocardial structure, function, and perfusion. An increasing body of data supports its role in assessing myocardial viability and predicting the recovery of function. RECENT FINDINGS: Myocardial contrast echocardiography accurately differentiates 'stunning' from necrosis, delineates transmural extent of infarction, predicts recovery of regional and global left ventricular systolic function in the recuperative phase, identifies patients at high risk of left ventricular remodelling, and provides incremental viability data when performed in conjunction with low-dose dobutamine echocardiography. SUMMARY: Technological advances have positioned myocardial contrast echocardiography as a safe, practical bedside technique for the evaluation of myocardial viability. It has comparable accuracy with other non-invasive imaging techniques, such as dobutamine stress echocardiography, radionuclide scintigraphy and cardiac magnetic resonance imaging.     

Anomalous origin of the left coronary artery from the pulmonary trunk in a 45-year-old woman

We report a rare case of anomalous origin of the left coronary artery from the pulmonary trunk in a 45-year-old woman. The approach and technique used for selective catheterization of an anomalous left coronary artery arising from the pulmonary trunk are described. Six years after diagnosis, echocardiography showed left ventricular disfunction, and surgical treatment was indicated again. The origin of the left coronary artery from the pulmonary trunk was closed, and the postoperative period was uneventful, with recovery of left ventricular function and disappearance of ischemic features on stress myocardial perfusion imaging with 99m Tc-sestamibi, performed 4 weeks after surgery.     

Anomalous origin of the left coronary artery from the pulmonary trunk. Potential for false negative diagnosis with cross sectional echocardiography

Cross sectional echocardiography can identify anomalous origin of the left coronary artery from the pulmonary trunk. It has been suggested that identification of the left coronary artery arising from the aorta using this technique excludes the diagnosis. In three such infants the anomalous origin of the left coronary artery was identified in each by cross sectional echocardiography. In all three cases, however, an echo free linear structure apparently arising from the aorta, resembling a normal left coronary artery, was imaged. Anatomical sections in one patient, simulating cross sectional echocardiographic cuts, showed that this structure was almost certainly the transverse sinus of the pericardium. False positive cross sectional echocardiographic diagnosis of this condition is also possible because of the failure to image a normally arising left coronary artery. Thus identification of the anomalous origin of the left coronary artery from the pulmonary trunk appears to be the only reliable echocardiographic finding in this condition, and contrast cineaortography remains necessary in patients in whom the diagnosis is suspected clinically or electrocardiographically.     

Aorto-left ventricular tunnel arising from the left sinus of Valsalva

Aortic-left ventricular tunnel (ALVT) is a rare congenital anomaly in which an abnormal communication connects the ascending aorta with the left ventricle, bypassing the aortic valve. Usually the ALVT takes its origin from the right aortic sinus. We report a patient with an ALVT arising from the left sinus of Valsalva who underwent surgery at five months of age. The diagnosis was first established by two-dimensional echocardiography.     

Anomalous origin of the left coronary artery from the pulmonary trunk. Potential for false negative diagnosis with cross sectional echocardiography.

Cross sectional echocardiography can identify anomalous origin of the left coronary artery from the pulmonary trunk. It has been suggested that identification of the left coronary artery arising from the aorta using this technique excludes the diagnosis. In three such infants the anomalous origin of the left coronary artery was identified in each by cross sectional echocardiography. In all three cases, however, an echo free linear structure apparently arising from the aorta, resembling a normal left coronary artery, was imaged. Anatomical sections in one patient, simulating cross sectional echocardiographic cuts, showed that this structure was almost certainly the transverse sinus of the pericardium. False positive cross sectional echocardiographic diagnosis of this condition is also possible because of the failure to image a normally arising left coronary artery. Thus identification of the anomalous origin of the left coronary artery from the pulmonary trunk appears to be the only reliable echocardiographic finding in this condition, and contrast cineaortography remains necessary in patients in whom the diagnosis is suspected clinically or electrocardiographically.     

Antegrade flow in the aorta ascendens despite aortic atresia: 2 case reports with retrograde coronary perfusion through coronary fistulas and sinusoids

In aortic atresia, coronary perfusion normally occurs through retrograde blood flow in the ascending aorta. We report on two patients with antegrade flow in the ascending aorta despite aortic atresia. In one patient with hypoplastic left heart syndrome (aortic atresia, severe mitral stenosis), an intact interatrial septum/premature closure of the foramen ovale was found. While no other way of left atrial or ventricular decompression was found, echocardiography, angiography and the post-mortem examination showed left ventricular to coronary sinusoids as the sole pathway for systemic oxygenation. In a second patient with complex congenital heart disease, including aortic atresia, antegrade flow in the ascending aorta was through a left coronary fistula with shunt flow originating from the pulmonary trunk. This report describes systemic perfusion depending on retrograde coronary flow due to coronary-cameral (sinusoids) and coronary arterio-venous fistulas leading to the phenomenon of antegrade blood flow in the ascending aorta despite aortic atresia.     

Multiple cavities in myocardium of left ventricle after irradiation therapy for breast cancer: a case report]

A 68-year-old woman was admitted to our hospital with congestive heart failure. She had been diagnosed with hypertrophic cardiomyopathy 12 years ago in another hospital. She had received irradiation therapy for left breast cancer 33 years ago after resection of her left breast. Echocardiography revealed left ventricular hypertrophy and wall motion hypokinesis, and multiple cavities in the myocardium of the left ventricle, interventricular septum, and anterior wall. Some cavities were observed to connect to the left ventricular cavity and Doppler echocardiography showed slow velocity flows in them different from that of the coronary artery. The pathologic diagnosis was severe sclerosis of the left coronary artery, especially the left descending artery and its branch, which was the area with irradiation. Histopathology revealed sclerotic changes of the coronary artery causing acute and chronic myocardial infarction, and incomplete regeneration and hypertrophy of cardiac cells. There was no sign of hypertrophic cardiomyopathy. Myocardial degeneration and deciduation were present next to the cavities connected to left ventricle-like fistulas.     

A rare case of anomalous origin of the left anterior descending artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, with an incidence of <1 in 300 000 live births. We describe a rare case of a 23‐month‐old infant admitted for cough and fever. Echocardiography showed a branch of the left coronary artery originating from the pulmonary artery. The coronary computed tomographic angiography revealed anomalous origin of the left anterior descending (LAD) artery from the pulmonary artery with the left circumflex artery and right coronary artery arising normally from the aorta. The infant successfully underwent surgical reimplantation of the LAD to the ascending aorta. Our case emphasizes that echocardiography and computed tomographic angiography are valuable imaging modalities for making an accurate diagnosis and determining the precise surgical plan.