Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum

Coronary artery pathology is a major determinant of treatment strategy and outcome in patients with pulmonary atresia and intact ventricular septum (PA/IVS). For this reason, infants with PA/IVS routinely undergo preoperative cardiac catheterization. The goal of this study was to identify echocardiographic predictors of coronary artery pathology in infants with PA/IVS. The initial preoperative echocardiograms of 30 consecutive infants with PA/IVS (median age at diagnosis 1 day) were reviewed for indexes predicting the degree of coronary pathology. The tricuspid valve (TV) annulus diameter Z- score was determined and evidence of abnormal flow in the coronary arteries by Doppler was evaluated. Coronary pathology was defined by angiography and graded as: 0 = no fistulae; 1 = fistulae/no right ventricular (RV)-dependent coronary arteries; 2 = fistulae with 1 RV-dependent coronary; 3 = fistulae with >/=2- vessel RV-dependent coronary arteries. Outcome was classified as: 2 ventricles, "1.5" ventricles, and 1 ventricle. By angiography, 30% of the patients had grade 0 coronary pathology, 30% had grade 1, 20% had grade 2, and 20% had grade 3. There was 1 death in a patient with grade 3 coronary pathology. Among the survivors (median age at follow-up 28. 6 months), biventricular circulation existed in 12 patients (41%), 7 patients (24%) were 1.5, and 10 (34%) were 1 ventricle. All patients with TV Z-score -2.5. The sensitivity, specificity, positive, and negative predictive values of TV Z-score 相似文献   

Coronary artery fistula with left or right ventricular communication and sinus-node dysfunction

Coronary artery fistulae that communicate with the left ventricle are quite rare; those accompanied by sinus-node dysfunction are even more unusual. We report 2 cases of congenital coronary-artery-to-left-ventricle fistula with sinus-node dysfunction. In each of these patients, fistulae arose from both left and right coronary arteries. One patient had, in addition, a right coronary artery fistula that communicated with his right ventricle. Sinusnode dysfunction encountered in these 2 patients was likely caused by chronic general ischemia arising from a steal syndrome associated with the fistulae.     

Multiple coronary artery-left ventricular fistulae: haemodynamic quantification by intracoronary Doppler ultrasound.

Multiple coronary artery-left ventricular fistulae involving all three major coronary arteries are extremely rare. Clinical findings are heterogeneous but include a history of typical or atypical angina pectoris in most cases. Coronary arteriography in a 65 year old woman who presented with chest pain at rest revealed multiple fine fistulae arising from the left anterior descending, left circumflex, and right coronary arteries. Left-to-left shunt was estimated by measurements of coronary artery flow velocity with intravascular Doppler ultrasound.     

Coronary flow characteristics of multiple coronary artery-left ventricular microfistulae in young monozygotic twins.

Coronary artery multiple fistulae of the left ventricular chamber are extremely rare congenital malformations. We report on 13-year-old monozygotic twin brothers who have identical abnormalities of coronary flow reserve as well as the same morphological findings of multiple coronary fistulae of the 3 major coronary arteries to the left ventricular chamber. The left circumflex coronary artery (LCX) had abundant fine communications with the left ventricular chamber and had a higher flow rate than the left anterior-descending coronary artery. The coronary flow reserve obtained from the LCX was lower than the normal value matched for the same age group. The increased shunt flow resulted in enlargement of the left ventricular chamber. This is the first report of coronary flow characteristics in children with multiple coronary fistulae.     

Coronary‐pulmonary arterial fistula in a neonate with pulmonary atresia—ventricular septal defect and single coronary artery

In cases of pulmonary atresia with ventricular septal defect (PA‐VSD), coronary‐pulmonary arterial fistula (CPAF) as the main source of pulmonary blood supply is extremely rare. These fistulae may arise from the left coronary artery, right coronary artery, or a single coronary artery. Fistulae from a single coronary artery are unusual. We are reporting a case of PA‐VSD with single coronary artery and CPAF as the main source of pulmonary supply in addition to two major aortopulmonary collateral arteries (MAPCAS). Successful surgical correction with VSD closure and right ventricle (RV) to the pulmonary artery (PA) conduit was made.     

Multiple coronary artery fistulae treated with a single Amplatzer vascular plug: Check the back door when the front is locked

Congenital coronary artery fistulae are rare anomalies, which can result in myocardial ischemia or infarction, congestive heart failure, fistula rupture, or death. In this report, the authors describe a 56‐year‐old woman with new onset chest pain and palpitations. Exercise myocardial perfusion imaging was significant for a reversible perfusion defect in the anterior left ventricular wall. Left and right heart catheterization demonstrated multiple fistulous communications between the right coronary and left anterior descending coronary arteries with the pulmonary artery. All fistulae drained into the pulmonary artery by a common ampulla. Closure was achieved via a percutaneous approach using a single Amplatzer vascular plug. © 2009 Wiley‐Liss, Inc.     

Decreased right ventricular function after coronary artery bypass grafting

Decreased right ventricular function after coronary artery bypass grafting is a common and well-known (if not well-understood) phenomenon.We prospectively evaluated right ventricular function via echocardiographic tricuspid annular motion, tricuspid annular velocity, and right ventricular strain analysis before and after coronary artery bypass grafting. We also evaluated the effect of right coronary artery disease and revascularization on post-coronary artery bypass grafting, right ventricular function, and interventricular septal motion.We performed baseline echocardiography in 250 candidates for coronary artery bypass grafting, and we repeated echocardiography in 240 of those patients 1 year after coronary artery bypass grafting. We evaluated right ventricular function via tricuspid annular motion, tricuspid annular velocity, and right ventricular strain analysis, all measured at the right ventricular free wall.Right ventricular function as evaluated by tricuspid annular motion showed a significant reduction 1 year after coronary artery bypass grafting (21.7 vs 12.1 mm; P < 0.001) compared with preoperative measurements. Right ventricular tissue velocity (14.0 vs 7.0 cm/s; P < 0.001) and right ventricular strain (20.3% vs 11.6%; P < 0.001) were also significantly reduced after coronary artery bypass grafting. Interventricular septal motion was paradoxical in 97% of the patients 1 year after coronary bypass.Right ventricular function remained depressed for as long as 1 year after coronary artery bypass grafting. These findings were independent of the state of the right coronary artery and the graft. It is likely that the interventricular septum is recruited to maintain right ventricular stroke volume after coronary artery bypass grafting.     

Effect of opening the pericardium on right ventricular hemodynamics during cardiac surgery

The impact of the pericardium on right ventricular performance in the presence of normal filling pressures was evaluated using a rapid response RVEF thermodilution pulmonary artery catheter and TEE. In eight patients with normal right coronary arteries undergoing coronary artery bypass surgery, hemodynamic measurements revealed increased right ventricular end-diastolic and end-systolic volumes with diminished RVEF after opening the pericardium. In eight additional patients with right coronary artery disease, directionally similar changes in right ventricular volume were seen. Ejection fraction, however, was unchanged possibly due to altered right ventricular compliance. Echocardiogram evaluation of right ventricular area changes in patients with compromised right coronary systems corresponded to ejection fraction determinations obtained with thermodilution technique.     

Incidence of exertional right ventricular wall motion abnormalities in patients with coronary artery disease

To evaluate the role of analysis of right ventricular function with exercise in patients with presumed coronary artery disease referred for radionuclide ventriculography, the records of 55 patients referred to our laboratory over a 19-month period were reviewed. All underwent rest and exercise first-pass radionuclide stress testing and cardiac catheterization within a period of four months. Three groups were identified: (1) patients with normal exercise right ventricular function (n = 24); (2) patients with exercise-induced right ventricular regional wall motion abnormalities (n = 15); and, (3) patients with abnormal resting right ventricular function without new exercise abnormalities (n = 16). Patients in each group were similar in age, sex, baseline left ventricular function, medication usage, and indication for study. The incidence of right coronary artery disease was identical in the three groups, as was the incidence of left ventricular functional abnormalities with exercise. Patients with proximal right coronary artery disease were more likely to have reduced left ventricular ejection fraction and more extensive coronary artery disease than those without disease at this site. We conclude that: (1) analysis of rest and exercise right ventricular function does not allow prediction of coronary anatomy in an unselected group of patients; (2) normal right ventricular function with exercise is compatible with extensive coronary artery disease, including proximal right coronary artery disease; and (3) abnormal exercise right ventricular function may be due to exertional left ventricular dysfunction in the absence of proximal right coronary artery disease.     

Transcatheter embolisation of coronary artery fistulae

BACKGROUND: Most children with coronary artery fistulae are asymptomatic, but because of associated late morbidity, early intervention is usually indicated. AIM: To assess the outcome following transcatheter embolisation of coronary artery fistulae. PATIENTS AND METHODS: Six children, with a median age of 9.5 years (range: 1.3-13.7 years), underwent transcatheter embolisation of coronary artery fistulae. Four patients had simple fistulae, which drained from the right coronary artery to the right ventricle (n = 2), the left coronary artery to the right ventricle (n = 1), or the left coronary artery to the coronary sinus (n = 1). Two patients had complex multiple fistulae arising from both coronary arteries, which communicated with either the right ventricle or the pulmonary arterial system. RESULTS: A stable position with a 5-Fr coronary catheter was obtained proximally and a 3-Fr coaxial catheter was advanced through the coronary catheter to a distal position in the coronary artery fistulae. The number of embolisation microcoils used per procedure ranged from one to 12, and the coil diameter ranged from 3 mm. Polyvinyl alcohol foam embolisation particles (1000 microm) were used to embolise small fistulae to the pulmonary arterial tree. Complete occlusion was obtained in four patients, while two children were left with insignificant residual shunts. There were no early or late cardiac complications. CONCLUSION: Transcatheter embolisation of coronary artery fistulae is a safe and effective therapy in patients with suitable anatomy.     

Coronary artery-left ventricular fistulae associated with apical hypertrophic cardiomyopathy.

We describe an unusual case of coronary artery-left ventricular fistulae associated with apical hypertrophic cardiomyopathy in a 63-year-old man who had a 2-year history of angina pectoris without significant coronary atherosclerosis. It is important to recognize this anomaly as it may be the source of angina in patients without angiographic evidence of major atherosclerotic coronary artery disease.     

Enhanced detection of proximal right coronary artery stenosis with the additional analysis of right ventricular thallium-201 uptake in stress scintigrams

The value of right ventricular thallium-201 analysis in detecting proximal right coronary artery stenosis in exercise myocardial scintigraphy was analyzed in 52 patients, 27 with and 25 without proximal right coronary artery stenosis. For the detection of proximal right coronary artery stenosis, the sensitivity and specificity of thallium scintigraphic analysis were 59 and 88% for a right ventricular abnormality, 67 and 68% for a left ventricular inferior wall abnormality, and 93 and 56% for an abnormality of either. When both right and left ventricular thallium images were abnormal, all 9 patients had proximal right coronary artery stenoses, and when both were normal, 26 of 28 patients had a normal proximal right coronary artery. The sensitivity and specificity of blood pool scintigraphic variables during exercise (right ventricular ejection fraction and left ventricular inferior wall motion) were not significantly different for detection of proximal right coronary artery stenosis.Thus, the additional analysis of the right ventricle on thallium-201 stress scintigrams can improve the detection of proximal right coronary artery stenosis. When both right ventricular and left ventricular thallium scintigrams are abnormal (or normal), the ability to predict the presence (or absence) of proximal right coronary artery stenosis is very high.     

Right ventricular infarction: role of the moderator band artery in determining infarct size

We studied 19 patients with proximal right coronary artery occlusions associated with acute myocardial infarcts less than 30 days old. Right ventricular infarct size, determined as a percentage of right ventricular surface area, ranged from 0% to 29%. Correlation of 24 variables measuring infarct size, chamber size and coronary artery disease failed to demonstrate a significant correlation with the extent of right ventricular infarction. However, estimates of the degree of obstruction to potential collateral flow into the right coronary arterial system from the left anterior descending coronary artery, especially through the moderator band artery, showed a significant positive correlation with infarct size (p less than 0.02). Among the five patients with massive (greater than 25%) right ventricular infarction, four had significant (greater than 75%) obstruction of the left anterior descending system, resulting in potentially impaired collateral blood flow; the other patient had normal coronary arteries and embolic occlusion of the proximal right coronary artery with contraction band necrosis. The study suggests that collateral flow to the right ventricular myocardium, especially through the moderator band artery, protects against massive infarction in the presence of proximal right coronary artery occlusion.     

Bilateral coronary fistulae to pulmonic valve in presence of severe three-vessel coronary artery disease

A 62-year-old man was admitted to the coronary care unit due to anginal pain and palpitations--coronary angiography revealed three-vessel coronary artery disease. The unexpected finding was the presence of coronary to pulmonary artery fistulae bilaterally, from both the proximal RCA and the proximal LAD. Right heart catheterization revealed normal right ventricular and pulmonary artery pressure and absence of hemodynamically significant left to right shunt. The patient underwent a triple coronary bypass including the closure of bilateral fistulae, which were draining into the left sinus of the pulmonary valve. One month after the operation he was in good health and had no complaints. Bilateral coronary artery fistulae is a rare anomaly diagnosed in 0.002-0.0013% of adult coronary angiograms. (Int J Cardiovasc Intervent 1999; 2: 249-251).     

Modified Nikaidoh procedure in a patient with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction with unusual coronary anatomy

The Rastelli operation has been the most common procedure for the repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction. A relatively recent approach is the Nikaidoh procedure. Despite the fact that it seems promising, the operation lacks long-term follow-up data. It has been postulated that patients with anomalous coronary arteries are high-risk candidates for the Nikaidoh procedure and its modifications. In this report, we present the case of a patient with transposition of the great arteries with remote restrictive ventricular septal defect and left ventricular outflow tract obstruction with coronary anomaly - with the right coronary artery originating from the left anterior descending coronary artery and crossing the right ventricular outflow tract - who underwent successful modified Nikaidoh operation.     

Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum

Children who die after operation for pulmonary atresia and intact ventricular septum may have myocardial ischemia. The relation between histologic evidence of myocardial ischemic injury and the presence of a right ventricle to coronary artery fistula, coronary artery dysplasia and operation in 17 autopsy specimens was assessed. Age at death ranged from 1 day to 16 years (median, 11 days). Of the 17 hearts, 6 (35%) had right ventricle to coronary artery fistulas, 5 of which had coronary artery dysplasia. In three cases, there was segmental or complete absence of a coronary artery. Ischemia was present in four of these six hearts, two of which had right ventricular outflow reconstruction. Six of the 11 hearts without right ventricle to coronary artery fistulas also had myocardial ischemia. Of these six cases, four had right ventricular outflow reconstruction and two had shunt operations. Death occurred from 1 to 8 days (mean 3) after operation. Hearts with pulmonary atresia and intact ventricular septum may have myocardial ischemia with or without either right ventricle to coronary artery fistulas or coronary artery dysplasia. Myocardial ischemia may occur after right ventricular outflow reconstruction or shunt operations. Thus, myocardial ischemia occurs commonly in patients with pulmonary atresia and intact ventricular septum and is not always related to coronary abnormalities or operation.     

Relation between the site of origin of ventricular premature complexes and the presence and severity of coronary artery disease

The clinical implications of ventricular premature complexes in patients with coronary heart disease have received increasing interest. It has been suggested that ventricular premature complexes of right ventricular origin have more benign implications than those that originate from the left ventricle. To define more precisely the relation between the site of origin of ventricular premature complexes and the presence and severity of coronary heart disease in patients with a chest pain syndrome, 39 patients with ventricular premature complexes of right or left ventricular contour who were undergoing cardiac catheterization and coronary arteriography for evaluation of chest discomfort were studied. Ninteen patients had left and 17 had right ventricular premature complexes and 3 had both. Of the 19 with left ventricular premature complexes, 15 had coronary artery disease (12 with two or three vessel obstruction and 3 with single vessel obstruction). Four had normal cardiac catheterization studies. Twelve patients had asynergy on ventriculography. The 17 patients with right ventricular premature complexes had similar angiographic findings. Eleven of the 17 had coronary artery disease (8 with triple vessel disease and 3 with isolated obstruction of the left anterior descending coronary artery). Six had normal arteries. Eight of the 11 with coronary artery disease and right ventricular premature complexes also had asynergy. All three patients with both left and right ventricular premature complexes had coronary obstructive disease. These findings indicate that in patients with a chest pain syndrome there is no relation between the site of origin of ventricular premature complexes and either the prevalence or severity of coronary artery disease.     

Right ventricular hypertrophy is an important determinant of right ventricular infarction complicating acute inferior left ventricular infarction

To explore the role of right ventricular hypertrophy and chronic obstructive pulmonary disease in the pathogenesis of right ventricular infarction, 27 consecutive patients with a first inferior left ventricular infarction were prospectively studied. Right ventricular infarction was diagnosed using established hemodynamic criteria. Right ventricular hypertrophy was defined as right ventricular free wall thickness greater than or equal to 5 mm. Patients were classified into two groups: Group I patients with right ventricular infarction (n = 15), and Group II patients without right ventricular infarction (n = 12). The ratio of forced expiratory volume over forced vital capacity (FEV1/FVC) and forced expiratory flow between 25 and 75% expired volume (FEF) as a percent of predicted values were significantly reduced in Group I versus Group II (90 +/- 5 versus 105 +/- 6% and 63 +/- 13 versus 103 +/- 15%, respectively; p less than 0.05). This was associated with increased right ventricular wall thickness (Group I 5.5 +/- 0.3 mm versus Group II 3.9 +/- 0.2 mm, p less than 0.001). Multiple logistic regression analysis demonstrated that right ventricular wall thickness was the strongest predictor of right ventricular infarction (p less than 0.0005). No significant difference was found in the site of right coronary occlusion, collateral blood supply or extent of coronary artery disease between the two groups. These findings suggest that right ventricular hypertrophy predisposes patients with acute inferior myocardial infarction to right ventricular infarction independent of the site or extent of coronary artery disease.     

Pressure-flow relations in the pulmonary artery during myocardial ischaemia: implications for right ventricular function in coronary disease

Because exercise induced pulmonary hypertension may disturb optimal coupling between the right ventricle and pulmonary artery in coronary artery disease, high fidelity pulmonary artery and right ventricular pressure and electromagnetic pulmonary artery flow velocity data were recorded at rest and during supine exercise in 10 control subjects free of detectable cardiovascular disease and in 11 patients with coronary artery disease. The pulmonary artery impedance and power spectra were calculated from Fourier analysis of pressure and flow waveforms. Total hydraulic power expended per unit of forward flow was computed as an index of right ventricular-pulmonary artery coupling. In coronary artery disease exercise produced substantial increases in pulmonary artery pressure, pulmonary artery characteristic impedance, and total power per unit flow. These changes did not occur in control subjects. Despite a significant exercise increase in right ventricular end diastolic pressure and peak right ventricular dP/dt, and independent of the presence of right coronary artery involvement, the right ventricular stroke output response during exercise was significantly blunted in the coronary artery disease patients. Pulmonary vascular resistance was unchanged by exercise in either group. Exercise induced ischaemia presents an increased pulsatile hydraulic load to the right ventricle. Increased pulmonary artery input impedance impairs the hydraulic efficiency of right ventricular-pulmonary artery coupling and may contribute to the limitation of right ventricular ejection performance in coronary artery disease.