Mild type of the Ebstein anomaly

Ebstein anomaly is a congenital malformation of the heart that is characterised by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialisation of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. We present a case of a mild type Ebstein anomaly leading to moderate tricuspid valve regurgitation and some degree of right ventricular dysfunction.     

Echocardiographic features of carcinoid heart disease

We reviewed the records of the Mayo Clinic patients with known carcinoid syndrome in whom echocardiographic studies had been done. Nineteen patients had M-mode and 2-dimensional echocardiographic examinations, and 1 patient had an M-mode examination only. Of the 20 patients, 8 had no evidence by echocardiogram of carcinoid heart disease; 2 had changes in the tricuspid valve echogram suggestive of early carcinoid heart disease, and the other 10 patients had the following distinctive echocardiographic findings: (1) the pattern of right ventricular volume overload (enlarged right ventricle with abnormal septal motion); (2) abnormal right-sided valves, including (a) a striking appearance of the tricuspid valve, the leaflets appearing thickened, retracted, and fixed in a semiopen position throughout the cardiac cycle, and (b) thickened, retracted pulmonic valve cusps, when visualized; and (3) the left-sided valves and chambers rarely involved. These echocardiographic features are distinctive of advanced carcinoid heart disease and correlate closely with pathologic findings.     

Echocardiographic assessment of the right ventricle in Ebstein's anomaly: relation to clinical outcome

Two-dimensional echocardiography was performed in 16 patients with Ebstein's anomaly to assess right ventricular anatomy and function in relation to clinical features and prognosis. Measurements of right ventricular anatomy and function were established in 10 normal subjects for comparison. Ten patients were in New York Heart Association functional class I, four in class II and one each in classes III and IV. Right ventricular morphology and the three tricuspid valve leaflets were assessed from right ventricular inflow tract and apical four chamber views. The anterior tricuspid leaflet was abnormal but not displaced in all patients; the septal and posterior leaflets were displaced in 14 (88%) and 11 (69%) patients respectively. The posterior leaflet was best visualized from the right ventricular inflow tract, and in two patients this view was required for the echocardiographic diagnosis of Ebstein's anomaly, based on displacement of the septal tricuspid valve leaflet. An index of right ventricular function, the fractional area contraction, was defined as the difference between the end-diastolic and the end-systolic area, normalized to the end-diastolic area. This index was calculated for both the proximal (atrialized) right ventricle and the total right ventricle. Total right ventricular end-diastolic area and fractional area contraction exceeded 95% confidence limits when compared with values in the normal group. During a median follow-up period of 4 years three patients died. They had severe right heart morphologic or functional abnormalities; two were in functional class III or IV and one was asymptomatic. None of the survivors had severe symptoms.(ABSTRACT TRUNCATED AT 250 WORDS)     

Demonstration of Ebstein's anomaly by simultaneous catheter-tip localization of the tricuspid valve visualization: A new method.

The diagnosis of Ebstein's anomaly has traditionally been made by angiocardiography and confirmed by simultaneous intracardiac electrocardiographic and pressure recordings. These techniques may result in false positive or negative tests. A new method is proposed, whereby the right coronary artery is used an an angiographic marker for the tricuspid annulus and a pressure catheter simultaneously marks the position of the tricuspid valve. In the right anterior oblique position the tip of the pressure catheter should be just under the right coronary artery as the tricuspid leaflets close in systole, indicating the normal relationship of the tricuspid leaflets and annulus. In Ebstein's anomaly the tip of the catheter extends well past the coronary artery in systole, demonstrating the characteristic displacement of the attachments of the tricuspid valve downward toward the right ventricular apex.     

Percutaneous Treatment of a Four-Leaf Clover Valve Using the MitraClip Technology

Percutaneous treatment of tricuspid valve regurgitation using MitraClip can be performed safely achieving improvement in reduction of regurgitation. Tricuspid valve shows different anatomic variations, in particular regarding the number of leaflets, which could represent a challenge for transcatheter valve intervention. We present a case of massive tricuspid regurgitation in a 4-leaf clover valve. We implanted a first MitraClip into the anteroseptal commissure and then a second one between the 2 posterior leaflets, with a successful reduction of residual regurgitation. In conclusion, this approach can be safely performed in a 4-leaflet right ventricular valve.     

Two dimensional echocardiography and the tricuspid valve. Leaflet definition and prolapse.

The tricuspid valve was studied in 143 subjects using two dimensional echocardiography. The groups studied were 40 normal subjects, 31 patients with mitral valve prolapse, 22 with clinically probable tricuspid valve prolapse, 20 with congestive cardiac failure, and 30 with miscellaneous cardiac conditions but no features of right heart disease. Using multiple views it was possible to record all three leaflets in 74.8% of cases and anterior and septal leaflets in 95%. Prolapse of the tricuspid valve was recognised in 13 patients: six (19.5%) of the group with mitral valve prolapse and seven (6%) of the remaining patients. Prolapse of all three leaflets was shown in one patient, anterior and septal prolapse in six patients, anterior and posterior in three patients, septal leaflet prolapse alone in two patients, and anterior alone in one patient. Two dimensional echocardiography allows definition of individual tricuspid leaflets and prolapse of any or all leaflets can be diagnosed. Tricuspid valve prolapse is commonly associated with prolapse of mitral valve leaflets but isolated cases are recognised.     

Two-dimensional echocardiography in the diagnosis of carcinoid heart disease

Tricuspid valve disease accounts for significant morbidity and mortality in the carcinoid syndrome, but M-mode echocardiography is often insensitive in completely defining the tricuspid valve. We performed two-dimensional echocardiography (2DE) in seven patients with proven carcinoid syndrome. There were five males and two females whose ages ranged from 53 to 79 years. The carcinoid syndrome had been present by symptoms for 12 to 84 months and by 5-HIAA levels for 6 to 84 months prior to 2DE. Short, thickened, immobile tricuspid valve leaflets, fixed in a partially open position, were visualized in two patients and confirmed in one patient at surgery. Tricuspid regurgitation was demonstrated angiographically in one and by contrast 2DE in the other. A third patient had clinical evidence of tricuspid stenosis with a doming tricuspid valve on 2DE. The motion of the tricuspid value viewed in real time was clearly distinct in these two situations. Four patients had both normal M-mode and 2DE studies despite the fact that clinical and biochemical evidence of carcinoid disease had been present for equally long periods of time. The tricuspid valve was best visualized in the parasternal right ventricular long-axis and short-axis views. The apical four-chamber view was less helpful. Thus, 2DE demonstrated specific tricuspid valve abnormalities in the carcinoid syndrome with thickening, shortening, and immobility of the leaflets when valvular regurgitation was present and thickening and doming when the valve was stenotic. 2DE should be a useful method in the diagnosis and sequential evaluation of patients with carcinoid heart disease.     

Morphological aspects of Ebstein's anomaly in adults

The essence of the Ebstein's malformation is that the tricuspid valve leaflets do not attach normally to the valve annulus, and the effective orifice is displaced downward into the right ventricular cavity at the junction of the inlet and trabecular components of the right ventricle. Only the septal and posterior leaflets are displaced and divide the right ventricle into two portions. The inlet portion is usually integrated functionally with the right atrium ("atrialized portion"), while the other, including the trabecular and outlet portions, constitutes the functional right ventricle. The proximal atrialized right ventricle often has a wall thinner than the distal functional right ventricle, due to partial congenital absence of myocardium. An atrial septal defect is present in more than one-third of hearts, and the majority of the remainder has a patent foramen ovale resulting in a right-to-left shunt. The downward displacement of the septal tricuspid valve leaflet is associated with discontinuity of the central fibrous body and septal atrioventricular ring, thus creating a potential substrate for accessory atrioventricular connections and ventricular pre-excitation making the patient at risk of sudden death. Angiography has demonstrated that a significant number of patients with Ebstein's anomaly also have morphofunctional abnormalities of the left ventricle, which may be explained by increased fibrosis in the left ventricular wall and ventricular septum as demonstrated by histological studies. Regarding embryology, the leaflets and tensile apparatus of the tricuspid valve are believed to be formed mostly by a process of delamination of the inner layers of the inlet zone of the right ventricle. The downward displacement of the leaflets in Ebstein's anomaly suggests that delamination from the inlet portion failed to occur.     

Entrapment of permanent pacemaker lead as the cause of tricuspid regurgitation.

Transthoracic 2-dimensional (D) echocardiography (echo) is often used to assess tricuspid regurgitation (TR) after implantation of permanent pacemakers. However, its ability to define the precise anatomical relationship between the tricuspid valve and the pacemaker lead is quite limited. This report presents a 58-year-old male with aggravation of TR after pacemaker implantation for heart block. Three-D echo precisely depicted the entrapment of the lead shaft in the fused and fibrotic septal and posterior tricuspid leaflets. The patient underwent tricuspid valve annuloplasty and the symptoms of right heart failure improved soon after the operation.     

Severe tricuspid valve stenosis secondary to pacemaker leads presenting as ascites and liver dysfunction: a complex problem requiring a multidisciplinary therapeutic approach

Tricuspid stenosis secondary to ventricular pacemaker leads is uncommon. We present a unique case of iatrogenic tricuspid stenosis secondary to fusion of the valve leaflets to transvenous implanted pacing leads. This occurred in an adult with childhood repaired Tetralogy of Fallot and high grade surgical heart block following multiple pacemaker procedures. The case was complicated by superior vena cava (SVC) and innominate vein stenosis secondary to implanted pacing leads, severe tricuspid valve (TV) stenosis, perforation of the heart by one of the implanted transvenous ventricular pacing leads, prolapse of the transvenous atrial pacing lead into the right ventricle, and unusual coronary sinus anatomy. We describe a multidisciplinary approach to management.     

Echocardiographic findings in left ventricular to right atrial shunts.

The echocardiographic abnormalities of tricuspid valve motion in 2 patients with left ventricular to right atrial shunts are described. In both patients the abnormal anatomy was defined at surgery, in one patient the shunt being above the tricuspid valve leaflets (supravalvar) and in the other patient through the septal leaflet (intravalvar). Different patterns of tricuspid valve systolic fluttering were seen in these two cases and the possible reasons for this are discussed. After surgical closure of the defects the systolic fluttering of the tricuspid valve was no longer observed. Echocardiography appears to be useful in detecting the presence of left ventricular to right atrial shunts which otherwise may be difficult to diagnose.     

Clinical and echocardiographic comparison of patients with the carcinoid syndrome with and without carcinoid heart disease

To correlate clinical and laboratory variables in carcinoid heart disease, clinical data, echocardiograms, 24-hour urinary 5-hydroxyindoleacetic acid levels and liver function tests were evaluated in 30 patients with the carcinoid syndrome. The dominant cardiac lesion of carcinoid heart disease by echocardiography and Doppler was severe tricuspid regurgitation with right ventricular volume overload. A characteristic finding was thickened, retracted tricuspid valve leaflets that were fixed in a partially open position. Carcinoid heart disease was progressive and often fatal. The 17 patients with echocardiographic evidence of carcinoid heart disease had higher peak levels of urinary 5-hydroxyindoleacetic acid (331 +/- 231 vs 58 +/- 78 mg, p less than 0.001) and more severe hepatic dysfunction than the 13 patients without carcinoid heart disease. Although duration of symptoms of the carcinoid syndrome before echocardiography was similar for patients with and without carcinoid heart disease (5.4 +/- 6.4 vs 6.2 +/- 5.9 years, respectively, p greater than 0.1), survival after echocardiography was shorter for those with carcinoid heart disease (1.9 +/- 1.4 vs 3.8 +/- 2.9 years, p = 0.05). The findings support the concept that long-term exposure of the endocardium to serotonin in the right side of the heart leads to the development of heart lesions; in addition, progressive hepatic dysfunction may allow more serotonin to bypass liver enzymes and reach the right side of the heart.     

Ebstein malformation of the tricuspid valve: Current concepts in management and outcomes

Ebstein anomaly is a myopathy of the right ventricle that results in variable degrees of failure of delamination of the tricuspid valve leaflets from the underlying endocardium, leading to severe tricuspid valve regurgitation and some degree of right ventricular dysfunction. In neonates or infants who remain in congestive heart failure or profoundly cyanotic while receiving appropriate medical therapy, operation is required. Current strategies include biventricular or single-ventricle repair. In children and adults, medical management may be used, but most patients eventually require surgery. Tricuspid valve repair is preferred; we believe the cone repair is the most anatomic repair and is the operation of choice. Tricuspid valve replacement may be necessary in cases in which the valve is not repairable. A bidirectional cavopulmonary shunt is useful in patients with severe right ventricular dilatation and/or dysfunction. Transplantation rarely is necessary.     

Clinical significance of incomplete tricuspid valve closure seen on two-dimensional echocardiography

Incomplete closure of the tricuspid valve without apparent cusp disease was noted on two-dimensional echocardiography in 31 patients. This abnormality was defined as a failure of the tricuspid valve leaflet tips to reach the plane of the tricuspid valve anulus by at least 1 cm in the standard apical four chamber view at the point of maximal systolic closure. This resulted in a final systolic leaflet position deeper within the right ventricular cavity than is normally seen. The finding was present in the following diagnostic subgroups: Group A, pulmonary hypertension (11 patients); Group B, rheumatic heart disease (4 patients); Group C, dilated cardiomyopathy (9 patients) and Group D, previous myocardial infarction (7 patients). Right atrial, right ventricular and tricuspid anulus measurements were made and compared with those from a group of 67 normal subjects. The results were as follows: right atrial endsystolic area = 27.2 +/- 8.6 cm2 (normal = 13.4 +/- 2.0); right ventricular end-systolic area = 25.6 +/- 8.7 cm2 (normal = 10.9 +/- 2.9); right ventricular end-diastolic area = 31.5 +/- 9.1 cm2 (normal = 20.1 +/- 4.9) and tricuspid valve anular end-systolic dimension = 4.0 +/- 0.6 cm (normal = 2.2 +/- 0.3). The differences from the normal data were all statistically significant (p less than 0.001). Incomplete closure of the tricuspid valve, although a nonspecific diagnostic finding, is primarily associated with right-sided chamber enlargement. Tricuspid regurgitation may be present. The mechanism could be related to geometric changes in valve apparatus dynamics secondary to right-sided cardiac enlargement and tricuspid valve anular dilation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Two-dimensional echocardiographic anatomy in crisscross heart

The anatomy of crisscross heart was studied in 14 patients, aged 2 days to 26 years, using 2-dimensional (2-D) echocardiography. The visceroatrial situs was solitus in all 14 patients. Crossing of the atrioventricular (AV) valves could be seen in each case by scanning in a subxiphoid or apical 4-chamber view. The subpulmonary infundibulum was deficient in 13 of the 14 patients, resulting in approximation of the pulmonary and tricuspid valves. Subpulmonary stenosis, seen in 11 patients, resulted from proximity of the infundibular septum, the tricuspid valve and the subpulmonary infundibular free wall. A subaortic infundibulum, present in all cases, was well developed in 13 patients, who had malposition of the great arteries, and was short in 1 patient, with nearly normally related great arteries. In cases that conformed to the inverse loop rule (segmental combination (S,D,L) or (S,L,D)), the pulmonary valve was posterior to the tricuspid valve and wedged between the AV valves. In 3 patients (double outlet right ventricle (S,D,L)) the mitral valve straddled into a large, left-sided subaortic infundibulum. In both patients with arterial malposition who did not follow the inverse loop rule, the pulmonary trunk was anterior to the tricuspid valve and well separated from the mitral valve. In patients with crisscross heart the ventricles appeared to have been rotated about their long axes without concomitant motion of the AV valve anuli, producing actual crossing of the ventricular inflow tracts. The right ventricular sinus was significantly smaller and the infundibulum significantly larger in the patients with crisscross heart than in age-matched control subjects.(ABSTRACT TRUNCATED AT 250 WORDS)     

Overriding atrioventricular valves. An angiographic-anatomical correlate

An analysis of 6 patients with overriding atrioventricular valve was performed in order to correlate the anatomic and angiographic features of this malformation. The atrial arrangement was usual (situs solitus) in each patient. The ventricular chambers were normally related in 4 and the mirror image of normal in 2. The atrioventricular connexion was concordant in parallel fashion in 4, discordant in 1 and double inlet left ventricle in 1 heart. In 2 of 4 hearts with concordant atrioventricular connexion, the right atrioventricular valve (tricuspid) overrode the septum and connected partially to the left ventricle. In 2, the left atrioventricular valve (mitral) overrode the septum and partially connected with the right ventricle. One heart with discordant atrioventricular connexion had its right atrioventricular valve (mitral) overriding the septum. The remaining heart with double inlet left ventricle had an overriding left atrioventricular valve. Each overriding atrioventricular valve had its "septal" leaflet (the one committed to the contralateral ventricle) divided into 2 segments by a large cleft. Ventricular septal defects at different locations were present in all cases. The ventriculoarterial connexion was double outlet right ventricle in 5 patients and was discordant in 1. Malalignment of the atrial and ventricular septa was present to some degree in all cases. This was well depicted on ventriculograms in four chamber view in those with an overriding tricuspid valve. It was not detected in those with an overriding mitral valve. The displaced leaflets of the overriding and/or stradding valve had a vertical motion on cineangiograms similar to those observed in atrioventricular septal defects. Abnormal morphology and motion of the septal leaflet of the overriding atrioventricular valve and malalignment of the atrial and ventricular septal structures were the most important angiographic findings in this series.     

Echocardiographic correlates of atrial contraction in normal and abnormal atrial rhythm

Echocardiographic manifestations of normal atrial contraction on the left ventricular posterior wall (LVPW), ventricular septum (VS), right ventricular anterior wall (RVAW), and the aortic root have not been described previously in detail. In 150 consecutive echocardiograms recorded from patients in normal sinus rhythm, we have been able to find correlates of normal atrial contraction (A waves) on the LVPW in 130, on the VS in 95, on the RVAW in 27, and on the aortic root in 107. These waves occurred on the aortic root 0.04 0.06 sec after the onset of the P wave, and they occurred on the other cardiac structures 0.06 0.10 sec after the onset of the P wave. That these waves were due to atrial contraction was established by their close temporal relationship to the P wave in normal and abnormal atrioventricular (AV) conduction and by their absence whenever the QRS complex was not preceded by a P wave. First-degree AV block was associated with “premature” mitral closure. Premature atrial contractions produced definite A waves on the aortic root, but less distinct waves on the other structures. These echocardiographic correlates of atrial contraction provide another noninvasive means of assessing the atrial contribution to ventricular filling.

Echocardiographic study of ten patients in atrial flutter showed that atrial flutter contractions produced regular undulations on the mitral valve leaflets, tricuspid valve leaflets, left ventricular posterior wall, ventricular septum, aortic root and cusps, and the anterior and posterior left atrial wall.

Echocardiographic observations made in 40 patients in atrial fibrillation frequently showed undulations on the mitral and tricuspid valves, aortic root, pulmonary valve cusp, and left atrial anterior wall. In some instances, the diastolic undulations on the mitral valve leaflets showed only minimal variations in amplitude and frequency even though the simultaneously recorded electrocardiogram showed marked irregularity of amplitude and frequency of atrial undulations.