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Atrial forces in three planes of the timed vectorcardiogram were analyzed for magnitude and direction in 100 normal subjects and 304 patients with proved heart disease. In the horizontal plane, a value of the initial half of the P loop or right atrial component of 0.06 mv. anteriorly for an adult (or 0.08 mv. for a child) included all normal subjects for two standard deviations. Values in excess of these figures —that is, anterior displacement of 0.07 mv. (0.09 mv. for a child) or greater—were consistent with right atrial enlargement. In the horizontal plane, a left atrial vector of 0.045 mv. or less included 96 per cent of the normal subjects, both children and adults; hence, posterior displacement of more than 0.05 mv. of the late portion of the P loop was evidence of left atrial enlargement. A leftward displacement of this component of 0.10 mv. or more in adults (0.14 mv. in children) also exceeded normal limits and was taken as evidence of left atrial enlargement. The timed vectorcardiogram employing the criteria presented was over three times as sensitive in detecting atrial abnormality in the abnormal subjects as the electrocardiogram employing conventional criteria. Better visualization of P wave activity in the conventional electrocardiogram was afforded by increasing the gain 5 to 10 times the normal level. Because right and left atrial enlargement is an important factor in the diagnosis of congenital and acquired heart disease, a more graphic representation of these electrical forces is of great practical value.

A case report of inadvertent right atrial perforation and fatal cardiac tamponade during transseptal left heart catheterization is recorded. Hazards of the procedure are reviewed. It is concluded that gross right atrial enlargement constitutes a strong relative contraindication to the use of this technic, and the reasons for this are outlined.

OBJECTIVES The purpose of this study was to compare the clinical and echocardiographic features of adults who developed atrial tachyarrhythmias (ATs) late after a Fontan procedure with those who have remained free of arrhythmias. BACKGROUND Atrial tachyarrhythmias are a frequent complication of the Fontan operation. However, the outcomes in adult patients with AT who have had the Fontan operation have not been well defined. METHODS We reviewed the outcomes of 94 consecutive patients who underwent the Fontan operation between 1977 and 1994 and were followed as adults at the University of Toronto Congenital Cardiac Centre for Adults. Sixty patients had an atriopulmonary connection, 21 patients had an atrioventricular connection, and 13 patients received a lateral tunnel connection. RESULTS Thirty-nine patients (41%) had sustained AT (atrial fibrillation, atrial flutter or supraventricular tachycardia) after their Fontan procedure. Compared with patients who did not develop AT, those who did were more likely to develop heart failure (46% vs. 13%, p = 0.003) and right atrial thrombus (31% vs. 4%, p = 0.006), exhibit left atrial enlargement (mean [±SD] diameter: 44 ± 10 vs. 37 ± 9 mm, p = 0.002), exhibit right atrial enlargement (mean [±SD] volume: 139 ± 149 vs. 76 ± 54 ml, p = 0.040) and have moderate-to-severe systemic valve regurgitation (31% vs. 7%, p = 0.010). The mean survival time was not significantly different between the arrhythmia group and the arrhythmia-free group (21.2 ± 1.3 and 18.0 ± 0.7 years, respectively; p = 0.900). CONCLUSIONS Systemic atrioventricular valvular regurgitation and biatrial enlargement are commonly observed in patients who develop AT after the Fontan procedure. These patients are more likely to develop right atrial thrombus and heart failure.

SummaryWe report two cases of idiopathic atrial dilatation in two adult siblings, a brother and a sister. The first patient was a 36-year-old man who was referred to our institution for evaluation of atrial fibrillation and syncopes. Transthoracic echocardiography revealed an enlarged right atrium accompanied by a severe tricuspid regurgitation associated with annular dilatation. The diagnosis of idiopathic atrial enlargement was made after all other lesions known to produce it have been excluded. The patient did not consent to the proposed cardiothoracic surgical treatment so he remained on conservative therapy. On electrocardiography, atrial standstill was noted, resulting in the implantation of a VVI cardiac pacemaker one year later. After an asymptomatic period, the patient suffered sudden death at the age of 40 years. His 45-year-old sister suffering from the same symptoms was also referred for examination, also to be diagnosed with idiopathic atrial dilatation and severe tricuspid regurgitation. Idiopathic dilatation of the right atrium, although a rare disorder, should not be forgotten as differential diagnosis for enlarged right atrium. Transthoracic echocardiography is the most commonly used technique and in our cases it was sufficient for establishing the diagnosis.

Two cases of a negative P wave in lead I are presented. In both there were signs of a right atrial overloading and the heart was displaced to the left side of the chest due to left pneumonectomy in one case, and to a destroyed lung in the other. The anticlockwise rotation of the heart and the right atrial enlargement are suggested as being contributory factors to the inversion of P wave.

Electrocardiographic and vectorcardiographic changes in the heart were evaluated in 14 children with bronchial asthma during the acute attack and later during remission. Many more abnormalities were noted on the vectorcardiograms as compared to electrocardiograms. The abnormalities noted were in closer relationship to the particular attack rather than to the severity of asthma as revealed by history.Three patients showed evidence of right atrial enlargement during the acute attack, and all of them returned to normal during remission.Ten patients had abnormal orientation of QRS loop on vectorcardiogram during the acute attack, and of these, seven patients continued to have the abnormality during remission. Of the ten patients, five had evidence suggestive of left ventricular enlargement during the acute attack, and all returned to normal during remission. This finding was very intriguing and the possible explanations are discussed.

Signs of congestive heart failure developed in a 1 -year old male Irish Setter during a 3-week period. Pleural effusion and right atrial enlargement were evident on radiographs. The electrocardiogram had low voltage and small negative P-waves in leads II, III and aVF. The dog did not respond to therapy. At necropsy, a neoplasm was found involving parts of the right atrial wall and filling most of the right atrial lumen. The histological diagnosis was fibroma.

The interatrial septum was studied with B-mode echocardiography to determine whether there were specific ultrasonic features characteristic of such congenital heart diseases as atrial septal defect and endocardial cushion defect. In transverse tomograms in the third and fourth intercostal space the interatrial septum is detected almost parallel to the anterior chest wall. The septum continues leftward to the echo of the anterior mitral leaflet. When the interatrial septum extends beyond the left sternal border, it can also be recorded near the left sternal border in the sagittal tomogram. The interatrial septum can also be recorded by conventional echocardiography. The pattern is wavy and synchronous with the heart beat with an amplitude of about 10 mm. It is seen near the chest wall in patients with left atrial enlargement and far from the chest wall in patients with right atrial enlargement. The defect in the interatrial septum was visualized by B-mode and conventional echocardiogram in patients with ostium secundum atrial septal defect. The defects were no longer observed after surgery.

The atrial activation sequences during 15 episodes of sustained atrial flutters were determined in the isolated hearts of four dogs with surgically induced right atrial enlargement (TI/PS dogs). These sequences were compared with the activation sequences of six episodes of nonsustained atrial tachyarrhythmias induced in three control hearts. Total endocardial activation of both atria during normal sinus rhythm and during the arrhythmias was determined first by recording simultaneously from 192 pairs of recording electrodes positioned into egg-shaped electrode arrays, and then by determining the moment of activation from each of the recorded electrograms. Isochronal maps of total activation were constructed by computer. Nonsustained atrial rhythms inducible in control hearts were due to circus movement excitation either in the left atrium (two episodes) or in the right atrium (four episodes). On the other hand, all 15 episodes of sustained atrial flutter induced and mapped in the TI/PS dog hearts were due to reentrant excitation in the enlarged right atrium. The reentrant pattern could be in a clockwise or counterclockwise pattern. In these episodes of stable flutter an area of functional block was an essential component to the reentrant excitation.

• Cardiac abnormalities have been reported in 25% to 73% of adult patients with acquired immunodeficiency syndrome (AIDS). We are reporting the clinical course of a child with congenital AIDS who developed similar cardiac complications. He presented with congestive heart failure three months after the diagnosis of AIDS. He had cardiomegaly demonstrated on chest roentgenogram, which was previously normal. He had left ventricular hypertrophy and T-wave abnormalities on electrocardiography and left ventricular dysfunction and dilatation on echocardiography. His subsequent echocardiogram continued to show poor contractility, although his congestive symptoms were stabilized with digitalis therapy and diuresis. After a year of maintenance therapy with digitalis, he developed right ventricular and right atrial enlargement and tricuspid valve thickening and nodularity, similar to the valvular changes reported in adults. Thus, children with AIDS should be monitored for cardiac complications. ( AJDC 1986;140:1241-1244)

Five patients with tricuspid stenosis accompanying mitral stenosis were studied. The characteristic features include a diastolic rumbling murmur in the tricuspid area, accentuated by inspiration, prominent "a" wave in the jugular pulse, prominent P wave in the electrocardiogram, absence of physical or electrocardiographic signs of right ventricular hypertrophy, and disproportionate right atrial enlargement by fluoroscopy. The diagnosis may be confirmed by the presence of a significant diastolic pressure gradient across the tricuspid valve. Mitral valvuloplasty alone produces no improvement. The concept of "functional tricuspid stenosis" is introduced and a one-stage operative technic for both mitral and triscuspid valvuloplasty described.

A boy with a history of cardiac disease in childhood was admitted to a hospital for evaluation of his cardiac status at the age of 16 years. Roentgenography in the frontal projection showed a "snowman" configuration of the heart, which was enlarged in the transverse diameter and had a contour suggesting right ventricular hypertrophy. The electrocardiogram suggested right atrial enlargement and right ventricular hypertrophy. Cardiac catheterization demonstrated a higher oxygen saturation in the right side of the heart than in the systemic arterial circulation. The findings were diagnostic of an anomalous connection between pulmonary and systemic veins at a supracardiac level with an atrial septal defect. The diagnosis is important because of the retardation of growth and the mortality among infants with this condition, which can now be corrected surgically.

An asymptomatic patient with idiopathic right atrial enlargement is described in whom the interesting feature was associated pericardial effusion; this association has not been reported previously. Both of these conditions had been present for 16 years and both remained nonprogressive.

The pathologic and clinical findings in 13 cases of tricuspid atresia and increased pulmonary blood flow encountered in UCLA Medical Center were reviewed. In seven, the great vessels were normally related (group I), and in six, the great vessels were transposed (group II). In all group II cases, aortic arch anomalies were encountered, a finding not duly emphasized in previous reports. Comparison of the two groups revealed significant clinical and laboratory differences. Group II patients typically presented with severe congestive heart failure and expired within the first 2 months of life. Group I patients usually presented with mild congestive heart failure which responded to therapy. Five of the latter demonstrated progressive change from increased to decreased pulmonary blood flow during their clinical course and eventually required palliative shunt procedures, four prior to the age of 2 years and one at 5 years of age. While the electrocardiogram demonstrated right atrial enlargement and left ventricular hypertrophy in both groups, the mean frontal plane axis was between –15° and –100° in group I and –75° and +75° in group II. QRS vector loops tended to be superiorly oriented in group I and inferiorly oriented in group II. Because the natural history and prognosis in these two groups differ significantly, diagnostic efforts should include determination of the relationship of the great vessels. When transposition of the great vessels is demonstrated, aortography should be performed because of the high incidence of aortic arch anomalies.

after 6 months (5% of cases) the average survival time was 15 months. Treatment had some effect in prolonging the course of the disease. On radiological examination 93% of cases showed cardiomegaly. On electrocardiographic examination 40% of cases showed right atrial enlargement, 42% left atrial enlargement, 83% right ventricular enlargement, and 54% left ventricular enlargement. Certain electrocardiographic changes?namely, enlargement of the P waves, RS-T depression, and T-wave inversion?are of some value in diagnosis.

New criteria for the electrocardiographic recognition of right atrial enlargement, left atrial enlargement, and combined atrial enlargement were studied in 110 patients with congenital and acquired heart disease. Precise physiologic data were available in all cases. The method of analysis was designed for application to conventional scalar electrocardiography and hence can be used without modification for routine clinical purposes.

The case is reported of an infant with congenital stenosis of each of the four individual pulmonary veins near the left atrium. The characteristic features were failure to thrive, increasing cyanosis and dyspnea, and death in congestive cardiac failure at 7 months of age. A precordial systolic murmur and an early systolic ejection click were heard; the sounds over the base of the heart were diminished in intensity. Roentgenograms of the thorax revealed a heart of normal size with fullness of the main pulmonary artery segment and increased pulmonary vascular markings that had the reticulated appearance associated with pulmonary venous engorgement. Electrocardiography showed right axis deviation, right atrial enlargement, and a pattern of right ventricular systolic overload. Cardiac catheterization demonstrated increased right atrial pressure and markedly elevated right ventricular and pulmonary arterial pressures. Angiocardiographic studies revealed a right-to-left shunt at atrial level, enlargement of the right ventricle, and enlarged and tortuous pulmonary arteries. In the late films the left atrium appeared normal in size, but the pulmonary veins were tortuous and there was evidence of constriction of the pulmonary veins at the veno-atrial junctions, associated with delay in the passage of opaque material from the pulmonary veins to the left atrium. It is suggested that the definitive diagnosis most likely is to be made by a selective angiocardiogram performed from the main pulmonary artery. It would appear feasible, at least in theory, to obtain complete surgical correction of this anomaly.

The seventh case of isolated tricuspid valvular stenosis is reported. Despite the rarity of this lesion, a number of distinguishing clinical features warrant emphasis. The patient is generally a woman who has had a known heart murmur since childhood and who complains of fatigue, dyspnea and edema. Helpful physical signs include a prominent jugular venous a wave, left parasternal presystolic rumble with little if any mid-diastolic component, and generally no opening snap. Both the a wave and the rumble are accentuated during inspiration. Signs of pulmonary vascular congestion are absent, and both the electrocardiogram and the chest roentgenogram reveal only right atrial enlargement. Cardiac catheterization demonstrates a tricuspid diastolic mean gradient of 4 to 8 mm Hg and a valve area ranging from 0.8 to 1.5 cm 2 . The clinical course is variable. In some cases tricuspid valvulotomy or valvuloplasty has resulted in considerable improvement.

A case of idiopathic enlargement of the right atrium is presented and 13 other cases are reviewed. The patient whose case is reported died suddenly at home 6 months after onset of atrial flutter. This represents the first case in which postmortem findings are available. The patient demonstrates that the anomaly cannot be considered entirely benign as has been suggested in previous reports. Other complications that may occur particularly in cases of massive atrial enlargement are atrial arrhythmias, syncope, and right ventricular compression. The importance of recognizing this condition as a clinical entity is stressed because surgical excision of a portion of the enlarged atrium appears to be both easy and curative and because these cases may be mistakenly diagnosed as mediastinal tumor or pericardial effusion. The possible relationship of this apparently congenital abnormality to Uhl's anomaly is discussed.

Summary A case of pediatric cardiomyopathy with massive right atrial enlargement, a right atrial thrombus partially occluding the tricuspid valve, left atrial enlargement and right ventricular enlargement is presented, the patient having expired within 1 year of onset of symptoms. The P wave vectors, when compared to findings in the literature, demonstrated axis rotation more typical of left atrial enlargement, in spite of the predominant right atrium at autopsy, and were exceptional in their magnitude and reversal of the frontal loop. These unusual P wave vectors might be explained in part by the posterior displacement of the right atrium by its own marked enlargement and by the superior posterior push from an enlarged right ventricle within the cinfined anterior mediastinum.

The electrocardiographic and vectorcardiographic findings of 29 patients with tricuspid atresia were related to the coexistent cardiac abnormalities and the status of pulmonary blood flow. Certain electrovectorcardiographic features were found to permit distinction between those patients with tricuspid atresia who had diminished pulmonary blood flow and those who had increased flow. Lead V6 of patients with tricuspid atresia, subpulmonary stenosis and diminished pulmonary blood flow generally revealed shallow Q waves and small R waves. The vectorcardiograms of these patients revealed narrow QRS vector loops in each plane and were posteriorly related. Among the patients with tricuspid atresia without subpulmonary stenosis or with coexistent transposition of the great vessels, tall R waves, frequently in association with deep Q waves, were present in lead V6. Likewise, the QRS vector loops in each plane encompassed a large area. Most patients had left axis deviation, as anticipated. A normal axis, when present, was usually related to coexistent transposition of the great vessels. The patients with subpulmonary stenosis had right atrial enlargement in infancy but left atrial enlargement when older; those without pulmonary stenosis frequently had left or combined atrial enlargement even in infancy. The origin of the left axis deviation in these patients is uncertain. Histologic studies of the myocardium failed to reveal abnormalities that might explain the alteration.

T his 83 pound infant was the product of an uneventful pregnancy and delivery. Apgar scores were 1 and 6. Physical examination revealed a cyanotic newborn with a heart rate of 120 per minute, respiratory rate of 50 per minute, and systolic blood pressure of 90 mm Hg in all extremities. There was a grade 2/6 pansystolic murmur at the lower left sternal border, a prominent mid-diastolic murmur, and a third heart sound. The liver was enlarged 3 cm below the right costal margin and was non-pulsatile. Umbilical arterial blood gases revealed : pH of 7.36; P02 55 mm Hg; Pco2 36 mm Hg. The electrocardiogram showed first degree heart block, right atrial enlargement, right axis deviation, and right bundle

In 1978, an ECG task force reported that the detection of right atrial enlargement should be based on the presence of an increase in P wave amplitude in the limb leads, in association with a peaked P wave morphology, and rightward frontal plane P wave axis. 1 This pattern is known as P pulmonale (Fig 1, upper trace). This suggestion has some support in two autopsy studies. Berliner and Master, 2 in 1938, reported that subjects with isolated left atrial hypertrophy had normal P wave amplitude, while those with biatrial hypertrophy had an increase in P wave amplitude, although in four cases of isolated right atrial hypertrophy, no P wave abnormalities were noted. In 1962, Caird and Wilken 3 found right atrial abnormalities in patients with obstructive lung disease. The P pulmonale ECG pattern was noted in seven of the 11 patients with a dilated right atrium, three patients with

In 16 adult patients, we performed continuous intraopcrative two-dimensional transesophageal echocardiography (2DTEE) to help elucidate the mechanism of rnyocardial dysfunction that accompanies liver transplantation. In 4 of the 16 patients “paradoxical” motion of the interventricular septum consistent with right ventricular failure was seen. An additional three of the 16 patients showed right atrial enlargement and right-to-left deviation of the interatrial septum. Two patients showed evidence of paradoxical embolization (one of whom had right ventricular and right atrial enlargement), and a third patient (who had right atrial enlargement) embolized a large right atrial thrombus into the pulmonary circulation. Two-dimensional transesophageal echocardiography demonstrated that isolated right ventricular failure might account for some of the hemodynamic instability seen during liver transplantation. Venous, pulmonary, and paradoxical embolization of air and thrombi documented by transesophageal echocardiography likely contribute to right heart failure.

The electrocardiogram assists in the diagnosis of infarction when tracings are taken within 24 hours of the pulmonary arterial occlusion, when tracings are repeated at least daily, and when the infarcts are large. Electrocardiographic changes are detected in 70% to 80% of these patients, but if tracings are taken late or infrequently or if infarcts are small, changes are detected in only 10% to 20% of the patients. Electrocardiographic changes occur when at least 65% of the total cross-sectional area of the pulmonary arterial system is occluded (organically or by spasm) and hemodynamic abnormalities are present. Certain ECG changes indicate right atrial enlargement, while others indicate rotation of the right ventricle into the field of the left precordial (V 4 and V 5 ) electrode position (clockwise rotation about the long axis as viewed from the apex). The enlargement is brought about by right atrial and right ventricular strain, and pulmonary

IN THE more advanced stages of chronic obstructive pulmonary disease, characteristic electrocardiographic changes of right atrial enlargement and right ventricular hypertrophy frequently develop.1It is extremely rare to have such a pattern disappear and the electrocardiogram return to normal in chronic obstructive pulmonary emphysema, which so often is slowly progressive. The patient reported herein had typical chronic obstructive pulmonary disease with emphysema, cor pulmonale and right-heart failure, and secondary polycythemia when first seen, but complete return of the ECG to normal occurred in a two-year period with vigorous treatment. Report of a Case A 53-year-old white man was examined in June 1964 because of shortness of breath and ankle swelling. He had smoked two packs of cigarettes per day for 37 years. The shortness of breath had developed progressively over a two-year period, and the ankle swelling had begun three months prior to his examination. Physical examination disclosed plethora