A Different Kind of Christmas Tree: Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery (ARCAPA)

Anomalous right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital coronary anomaly that has an incidence of 0.002%. We report a case of a previously healthy female who presented to our hospital with pneumonia and was incidentally discovered to have ARCAPA. This was initially diagnosed on echocardiography by the unusual echocardiographic finding of multiple color flow Doppler signals around the right ventricular free wall and apex which were subsequently confirmed by angiography to be due to extensive collateral circulation between the left and right coronary arteries. This represents an unusual echocardiographic manifestation of this very rare condition.     

Hypoplastic left heart syndrome: is echocardiography accurate enough to guide surgical palliation?

Two-dimensional echocardiography can diagnose hypoplastic left heart syndrome. However, with the advent of the possibility of palliative open heart surgery, complete anatomic diagnosis is necessary. The anatomic findings of 15 neonates with hypoplastic left heart syndrome (age 1 to 10 days, mean 4.1) who had two-dimensional Doppler echocardiographic studies were compared with the results obtained by angiography (6 cases), surgery (11 cases) and autopsy (8 cases). Complete two-dimensional echocardiographic examination of the aortic arch, pulmonary and systemic venous return, atrial septum, ductus arteriosus and proximal coronary arteries was possible in all 15 neonates and correctly diagnosed hypoplastic left heart syndrome in each. Anatomic two-dimensional echocardiographic assessment was accurate in 13 (86%) of the 15 neonates and there were no false positive results. Undiagnosed associated abnormalities were hypoplasia of a left pulmonary artery in one patient and left superior vena cava in another. Accurate quantitation of the size of the tricuspid valve anulus, ascending aorta, pulmonary anulus and right and left pulmonary arteries was possible. Doppler examination was performed in seven patients and confirmed retrograde aortic arch flow and right to left systolic shunting in the patent ductus arteriosus. In selected neonates, surgical palliation can be attempted without angiography.     

Hypoplastic left heart syndrome with right aortic arch, bilateral arterial ducts and origin of the left subclavian artery from the left pulmonary artery.

The rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. Cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation.     

Coronary artery abnormalities and right ventricular histology in hypoplastic left heart syndrome.

OBJECTIVES. To determine whether right ventricular preservation is compromised in any anatomic subgroup of hypoplastic left heart syndrome, we assessed the coronary artery anatomy and myocardial histologic features of 151 postmortem specimens with hypoplastic left heart syndrome. BACKGROUND. Although previous studies have shown that coronary artery abnormalities are more prominent in the subgroup of patients who have a patent mitral valve and obstructed aortic valve, it has not been established that these abnormalities compromise right ventricular perfusion. METHODS. Eighty-nine specimens with a patent mitral valve and aortic atresia, 52 with mitral and aortic atresia and 10 with patent mitral and aortic valves were examined. Histologic sections of the right ventricle, left ventricle and coronary arteries were obtained from 64 study hearts and 5 control hearts. RESULTS. Gross coronary anomalies included coronary-cameral communications (n = 29), single left coronary artery (n = 2), single right coronary artery (n = 1) and tortuosity (n = 19). Coronary-cameral communications and tortuosity were significantly associated with the subgroup that had mitral hypoplasia and aortic atresia. Coronary artery wall thickness relative to lumen diameter was not different among the subgroups. No areas of coronary artery stenosis or interruption were discovered. Although endocardial fibroelastosis of the left ventricle was strongly associated with the mitral hypoplasia and aortic atresia group, the right ventricular histologic findings were similar in all subgroups and were not differentially affected by older age. CONCLUSIONS. Although the incidence of coronary abnormalities is greater in patients with mitral hypoplasia and aortic atresia, in this study there was no apparent difference in perfusion of the right ventricle among the anatomic subgroups of hearts with hypoplastic left heart syndrome.     

Single right coronary artery continuing as left circumflex artery and hypoplastic left anterior descending artery: a rare coronary anomaly

The occurrence of a single coronary artery (SCA) is rare in the absence of other associated anomalies of the heart and is often detected incidentally during coronary angiography. This anomaly is usually benign and various types of SCA have been described. We report a rare type of SCA originating from the right sinus of Valsalva, with the left circumflex artery (LCX) continuing from right coronary artery (RCA) and hypoplastic left anterior descending artery (LAD), which was incidentally found in a 63-year-old female presenting as unstable angina.     

Hypoplastic left coronary artery. In association with occlusive intimal thickening of a coronary artery with ectopic ostium and with atresia of the left coronary ostium

Two cases of hypoplastic coronary artery (HCA) are presented. Case 1, a 13 year old girl, died suddenly during a long distance race. She had HLCA with marked intimal thickening and an ectopic left coronary ostium above the commisure between the non-coronary and left coronary cusp at post mortem examination. The right coronary artery (RCA) was enlarged and also supplied parts of the area normally supplied by the left coronary artery (LCA). Pathological findings revealed a normal RCA and an extremely hypoplastic LCA with occlusive proliferation of the intima and a myocardial infarction of the left ventricle. Case 2, a 6 year old girl, had a history of effort angina. Selective coronary angiography was performed which failed to demonstrate the orifice of the LCA by aortography. However, the hypoplastic LCA was visualized by RCA angiography as a consequence of anomalous collaterals from the atrioventricular branch of the RCA. We postulate that HCA results from various conditions, including stenosis of the coronary artery orifice, an aberrant course between the pulmonary artery and aorta and ectopic positioning of the coronary artery ostium. In addition, HCA may also be associated with occlusive coronary artery abnormalities.     

Cross sectional echocardiographic identification of hypoplastic left heart syndrome and differentiation from other causes of right ventricular overload

To identify the echocardiographic features that can be used to distinguish between hypoplastic left heart syndrome and other causes of right ventricular overload in the sick neonate cross sectional echocardiographic studies of 10 neonates with hypoplastic left heart syndrome were analysed and compared with those in 15 neonates with other causes of right ventricular overload and 15 normal controls. Left ventricular and right ventricular cavity dimensions and the shape and size of the mitral valve annulus and aortic root were recorded and presented both as absolute values (mm) and corrected for body surface area (mm/m2). Logistic regression was used to produce a classification rule to estimate the probability of a neonate having hypoplastic left heart syndrome. The diameter of the mitral valve annulus was the single most discriminative variable. There was, however, considerable overlap of all the calculated features between neonates with hypoplastic left heart syndrome and those with other causes of right ventricular overload. The diagnosis of hypoplastic left heart syndrome should not be based on any one single echocardiographic feature but the general appearance of abnormal left heart valves, small cavity dimensions, and the size of the mitral valve annulus.     

Cross sectional echocardiographic identification of hypoplastic left heart syndrome and differentiation from other causes of right ventricular overload.

To identify the echocardiographic features that can be used to distinguish between hypoplastic left heart syndrome and other causes of right ventricular overload in the sick neonate cross sectional echocardiographic studies of 10 neonates with hypoplastic left heart syndrome were analysed and compared with those in 15 neonates with other causes of right ventricular overload and 15 normal controls. Left ventricular and right ventricular cavity dimensions and the shape and size of the mitral valve annulus and aortic root were recorded and presented both as absolute values (mm) and corrected for body surface area (mm/m2). Logistic regression was used to produce a classification rule to estimate the probability of a neonate having hypoplastic left heart syndrome. The diameter of the mitral valve annulus was the single most discriminative variable. There was, however, considerable overlap of all the calculated features between neonates with hypoplastic left heart syndrome and those with other causes of right ventricular overload. The diagnosis of hypoplastic left heart syndrome should not be based on any one single echocardiographic feature but the general appearance of abnormal left heart valves, small cavity dimensions, and the size of the mitral valve annulus.     

Anomalies of left coronary artery origin affecting surgical repair of hypoplastic left heart syndrome and Shone complex

There has traditionally been less concern regarding coronary anomalies with left-sided congenital heart lesions such as hypoplastic left heart syndrome (HLHS) or Shone complex than with other lesions. However, coronary anomalies in this setting can profoundly affect surgical intervention, particularly when surgical repair involves the ascending aorta. We describe four patients with congenital left-sided heart lesions in which left coronary artery (LCA) anomalies substantially affected intervention and outcome. In the first two cases, the coronary anomalies were not identified prospectively and resulted in surgical injury directly to the coronary or to its surrounding region. In the latter two cases, successful identification of the coronary anomaly preoperatively allowed for modification of surgical technique and/or intervention. We conclude that detailed coronary artery assessment should be part of the routine echocardiographic evaluation of congenital left-sided heart lesions that require surgery.     

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.     

Echocardiographic evaluation of left ventricular function during coronary artery angioplasty

Balloon occlusion of a stenotic coronary artery during percutaneous coronary artery angioplasty provides a unique opportunity to study the effect of acute myocardial ischemia on left ventricular (LV) function. Simultaneous M-mode and 2-dimensional (2-D) echocardiograms and a 6-lead electrocardiogram were recorded during 20 episodes of coronary artery occlusion and release in 12 patients. No patient had previous myocardial infarction and all had normal LV function by angiography. All patients had isolated single coronary artery disease, with left anterior descending stenosis in 8 and right coronary stenosis in 4. In 18 of 20 episodes (90%), M-mode echocardiography during balloon occlusion revealed a significant (p less than 0.001) decrease in LV systolic, diastolic and percent systolic wall thickness; systolic excursion; systolic and diastolic endocardial velocities; and fractional shortening. These changes were observed in the area of the ventricular septum in patients with left anterior descending occlusion and posteroinferior wall in those with right coronary artery occlusion. Two-dimensional echocardiography revealed varying degrees of hypokinesia, akinesia and dyskinesia during balloon occlusion in 18 instances. The echocardiographic changes were observed within 15 to 20 seconds of balloon occlusion and resolved 10 to 20 seconds after balloon deflation. All patients who had echocardiographic changes during balloon occlusion also had concomitant electrocardiographic (ECG) ST-segment elevation, whereas 2 patients with normal LV function had no ECG changes. Both of these patients had profuse collateral blood supply to the stenotic coronary artery. The echocardiographic and ECG abnormalities increased proportionately to the length of balloon occlusion. This study confirms previous animal and recent human studies of transient LV dysfunction during coronary occlusion.(ABSTRACT TRUNCATED AT 250 WORDS)     

Age at death in the hypoplastic left heart syndrome: multivariate analysis and importance of the coronary arteries

Survival analysis of 63 fatal cases of the hypoplastic left heart syndrome was undertaken to determine whether morphologic measurements related to coronary blood flow would predict the length of postnatal survival. Cross-sectional areas of the ascending aorta and proximal left and right coronary arteries, left and right ventricular mass, coronary artery dominance pattern, and the presence of discrete coarctation were determined from autopsy specimens. These values were used to construct survival functions using Cox regression. Survival was significantly prolonged in cases with larger cross-sectional areas of the left (p less than 0.005) and right (p less than 0.05) coronary arteries, right ventricular mass (p less than 0.005), and combined ventricular mass (p less than 0.005). A multivariate model was developed that included combined left and right coronary artery cross-sectional areas, right ventricular mass, and the ratio of combined coronary artery area-to-combined ventricular mass. This model also significantly (p less than 0.001) predicted instantaneous risk of death, and again larger covariate values were associated with longer survival. These results suggest that myocardial perfusion is an important determinant of survival in these infants.     

Subcostal 2-dimensional echocardiographic imaging of peripheral left coronary artery aneurysms in Kawasaki disease

Our previous study provided a new 2-dimensional echocardiographic technique to detect peripheral right coronary artery aneurysms in Kawasaki disease, with use of the subcostal approach. An additional study was performed to detect peripheral left coronary artery aneurysms. Because the left anterior descending artery runs along the anterior interventricular sulcus and the left circumflex artery along the mitral valve ring, these regions were searched for coronary aneurysms by use of the subcostal imaging approach. Among 143 patients with Kawasaki disease, 44 left coronary aneurysms were visualized in 22 patients. Three aneurysms at the origin of the obtuse marginal artery and 1 in the further peripheral site of the left circumflex artery were observed in 3 patients. Two aneurysms at the origin of the second diagonal branch of the peripheral left anterior descending artery were detected. These echocardiographic studies were done prospectively, and their features coincided well in size, shape, and anatomic location with confirmatory angiographic appearances.     

左冠状动脉异常起源于肺动脉的超声心动图诊断价值

目的:探讨左冠状动脉异常起源于肺动脉(ALCAPA)的超声心动图诊断价值。方法:以CTA和手术证实的16例ALCAPA患儿为研究对象,回顾分析其超声心动图表现特征。结果:16例中,超声首诊11例,误诊5例,5例均误诊为心内膜弹力纤维增生症(EFE),复诊检出3例,余2例最后经CTA检出,超声首诊正确率69%。其主要超声表现为:左冠状动脉主干异常开口于肺动脉;右冠状动脉扩张;左心室扩大;心内膜、二尖瓣腱索及乳头肌及回声增强。彩色多普勒显示左冠状动脉内血流为逆向灌注,肺动脉内舒张期见异常血流进入;室间隔可见异常交通循环血流信号。结论:ALCAPA具有特征性超声心动图表现,超声是早期诊断的重要手段。     

Left ventricular dysfunction in acute myocardial infarction due to isolated left circumflex coronary artery stenosis

The characteristics of regional and global left ventricular dysfunction due to isolated left circumflex (LC) artery stenosis were determined from the contrast ventriculograms of 52 patients studied during acute myocardial infarction. In patients with a left dominant coronary circulation (35%), the severity, circumferential extent and location of hypokinesia resembled those of right coronary artery stenosis. However, in patients with a right dominant or balanced circulation (65%), the location of LC artery-related hypokinesia varied over the entire left ventricular contour, overlapping with the territories of the left anterior descending and right coronary arteries. The method for measuring the severity of hypokinesia was adjusted to take into account the wide territory of the LC artery. This enhanced the sensitivity of the method, particularly in the right anterior oblique view, as indicated by the greater severity of hypokinesia measured (-2.5 +/- 0.9 vs -1.8 +/- 1.1 standard deviations by our previously published method, p less than 0.001). However, the overlap of the artery territories may make it difficult to selectively measure the dysfunction due to stenosis of an artery of interest in patients with multiple infarctions. The circumferential extent of hypokinesia due to LC artery thrombosis was greater in the left than the right anterior oblique view. When averaged over both views, the size of the dysfunctional segment approached that due to left anterior descending coronary artery thrombosis, exceeding the size previously reported. These results suggest that current eligibility criteria for thrombolytic therapy select patients with extensive LC artery beds.     

Double left anterior descending coronary artery arising from the left and right coronary arteries: a rare congenital coronary artery anomaly

Double left anterior descending coronary artery arising from the left and right coronary arteries is a very rare congenital coronary artery anomaly. In this report, we describe a patient with double left anterior descending coronary artery originating from the left and right coronary arteries. To the best of our knowledge, dual connection of the left anterior descending coronary artery to the left and right coronary arteries has been described in only five patients.     

Intraoperative echocardiographic assessment of the severe isolated ostial stenosis of left main coronary artery before and after surgical patch angioplasty.

Isolated critical ostial stenosis of left main coronary artery is currently treated by conventional bypass surgery. Surgical patch angioplasty in an alternative surgical approach. Transesophageal echocardiography enables visualization of proximal branches of left and right coronary artery. The report describes intraoperative echocardiographic assessment of surgical left main coronary artery angioplasty.     

Congenital atresia of left coronary ostium.

A two-year-old girl, who presented with congestive heart failure and an extensive anterolateral infarction, was thought to have anomalous origin of the left coronary artery from the pulmonary artery. She improved rapidly and remained symptom free until 13 years of age, when she died suddenly. At necropsy the right coronary artery was found to be normal but in the left aortic sinus a dimple was the only remnant of the left coronary ostium. The proximal segment of the left long term follow-up of a patient with this rare anomaly.     

Selective aortic root angiography in the hypoplastic left heart syndrome.

Selective aortic root angiography was performed in 18 patients with the clinical diagnosis of hypoplastic left heart syndrome in order to allow more precise correlation with echocardiography. Aortic valve atresia and severe hypoplasia of the left heart chambers subsequently were substaniated at necropsy in each instance. Wide variation in the size and shape of the aortic root and ascending aorta was demonstrated angiographically in these patients. In some patients, despite aortic valve atresia, the dimensions of the aortic root and sinuses of Valsalva at angiography were almost normal. In addition, this technique allowed the demonstration of primitive vascular communications between the coronary artery and left ventricular cavity in 1 patient (coronary-cameral fistula). The realization that the aortic root may approach nearly normal size in some patients with the hypoplastic left heart syndrome is essential to the echocardiographic diagnosis of this disease and to the differential diagnosis from other cardiac and noncardiac causes of severe neonatal congestive heart failure.     

Left-right shunt caused by anomalous implantation of the left coronary artery in the trunk of pulmonary artery. Detection by contrast echocardiography

The authors describe a case of an anomalous left coronary artery arising from the main pulmonary artery in an 11 year old child suffering from chest pain on effort and with a continuous murmur in the second left intercostal space. Two dimensional echocardiography (2D E) showed dilatations of the first segment of the right coronary artery and the anomalous origin of the left main coronary artery. A peripheral injection of microbubbles showed a left-to-right shunt between the left coronary artery and the pulmonary artery. Semi-quantitative evaluation of LV regional wall motion showed abnormal contraction of the anterolateral walls. Haemodynamic, angiographic data and the operative findings confirmed the diagnosis. The abnormal coronary ostium was closed and a bypass graft from the aorta to the left anterior descending artery was performed. Clinical and echocardiographic follow-up 7 months after surgery was completely normal.