María Rijlaarsdam

The Adult Patient with Ebstein Anomaly: Outcome in 72 Unoperated Patients

Knowledge of the long-term outcome in unoperated adult patients with Ebstein anomaly is limited, and the therapeutic approach is still controversial. We studied unoperated adult patients with Ebstein anomaly to define the patterns of presentation, anatomic characteristics, outcome, and predictive factors for survival. Seventy-two unoperated survivors of Ebstein anomaly aged over 25 years attended from 1972 to 1997 were reviewed and followed-up from 1.6 to 22.0 years. Patients were classified in 3 groups of severity according to the echocardiographic appearance of the septal leaflet attachment of tricuspid valve. The mean age at diagnosis was 23.9 +/- 10.4 years, and the most common clinical presentation was an arrhythmic event (51.4%). There were 30 (42%) deaths, including 6 from arrhythmia, 12 related to heart failure, 7 sudden, 2 unrelated, and 3 unascertained. According to Cox regression analysis, predictors of cardiac-related death included age at diagnosis (hazard ratio 0.89 for each year of age, 95% confidence intervals CI[ 0.84-0.94), male sex (3.93, 95% CI, 1.50-10.29), degree of echocardiographic severity (3.34, 95% CI, 1.78-6.24), and cardiothoracic ratio > or = 0.65 (3.57, 95% CI, 1.15-11.03). During follow-up, morbidity was mainly related to arrhythmia and refractory late hemodynamic deterioration. The magnitude of tricuspid regurgitation, cyanosis, and the New York Heart Association (NYHA) functional class at time zero were significant risk factors according to the univariate analysis, but not after multivariable confrontation. The results of this study suggest that pattern of presentation, clinical course, and prognosis of unoperated adult patients with Ebstein anomaly are influenced by several factors. Although the initial symptoms are usually mild and commonly related to supraventricular arrhythmias, these are not associated with the long-term outcome. The severity of the morbid anatomy was the main determinant of survival only in extreme cases, but not in those with mild or moderate deformations, which are more common in adults. Other independent risk factors such as cardiothoracic ratio, sex, age at diagnosis, and the echocardiographic evaluation may help to determine the therapeutic approach. Adult patients with Ebstein anomaly should not be considered as a simple low-risk group.

Right Atrial and Ventricular Infarction

In order to compare the results of transesophageal echocardiography (TEE) in diagnosis of right atrial (RA) and right ventricular (RV) infarction with those of transthoracic echocardiography (TTE), 11 patients admitted to the coronary care unit with the diagnosis of posteroinferior left ventricular (LV) acute myocardial infarction (MI) and electrocardiographic suspicion of extension to RV were studied. In two of the 11 patients, RA infarction was identified on the basis of akinesis of the RA free wall, dilatation of the atrial cavity, spontaneous echo contrast, mural thrombosis, and poor atrial contribution to RV filling. In all 11 patients, RV infarction was determined by akinesis of one or more segmental regions, dilatation of the cavity in four patients, and tricuspid regurgitation in seven. Only six cases of RV infarction were diagnosed with TTE. The findings indicate that TEE provides additional information to TTE for determining RA and RV infarction during the early stages of MI.

Utility of transesophageal echocardiography in the examination of adult patients with patent ductus arteriosus

Thirteen patients with clinical suspicion of patent ductus arteriosus were evaluated by transthoracic and transesophageal echocardiographic studies. Findings were corroborated during corrective surgery in 8 patients and by cardiac catheterization in 5. Transthoracic echocardiography confirmed the diagnosis in 7 patients; in 2 of the patients endarteritis of the pulmonary artery was demonstrated, and in one infective vegetations in aortic and mitral valves. With transesophageal echocardiography, patent ductus arteriosus was established in all 8 patients and endarteritis of the pulmonary artery was shown in 3, including 1 not discovered by transthoracic technique. In 1 of these patients, vegetations were also found on the pulmonic valve. Both techniques demonstrated significant pulmonary hypertension in 5 cases; contrast studies showed the venoarterial shunt between the pulmonary artery and the aorta with particular clarity in transesophageal images. On the basis of these findings it may be concluded that transesophageal echocardiography complements the information provided by transthoracic recordings in adult patients with patent ductus arteriosus, especially when it is associated with pulmonary hypertension or pulmonary endarteritis.

Transesophageal Echocardiography and Right Atrial Infarction

Transesophageal echocardiographic findings in right atrial infarction are described. In three patients with myocardial infarction of one or both ventricles, the association of right atrial myocardial infarction was suspected because of anatomic (two-dimensional) and hemodynamic (Doppler) alterations obtained from transesophageal images. Transesophageal interrogation may prove widely applicable in the evaluation of patients with suspected right atrial infarction.

Transesophageal echocardiography in adults with congenital cardiopathies

Eighty-seven adult patients with congenital cardiopathy were studied by using transesophageal echocardiography (TEE) over a period of 30 months. Transthoracic echocardiography was practiced on all patients, after which TEE with monoplanar and biplanar probes (74 and 15 patients, respectively) was used to confirm principal diagnoses and determine specific information. TEE findings were compared with those of cardiac catheterization in all 87 cases and with those of surgery in 15 cases. Dextrocardia was found in 11 cases and mesocardia in 2. Situs inversus was demonstrated in 6 and levoisomerism in 4. Intracardiac and extracardiac shunts were diagnosed in 20 cases, Ebsteins anomaly in 27, corrected transposition of great vessels in 15, univentricular atrioventricular connection in 7, cor triatriatum in 1, parachute mitral valve in 1, crisscross heart in 1, and double-outlet left atrium in 1. TEE was of particular value in evaluating total anomalous pulmonary venous connection, common atrioventricular canal, Ebsteins anomaly, corrected transposition of great vessels, and univentricular atrioventricular connection and in diagnosis of the rarer congenital cardiopathies.

Clinical-Echocardiographic Correlation of Myocardial Infarction with Extension to Right Chambers.

In order to determine the transesophageal echocardiographic characteristics in patients with acute myocardial infarction of right ventricle and establish the relationship between these findings, the clinical condition, and their prognostic value, 38 patients consecutively admitted to the Instituto Nacional de Cardiología with a diagnosis of acute left ventricular myocardial infarction with extension to right ventricle and/or atrium were retrospectively studied. Of the left ventricular infarctions, 37 were posteroinferior and one anterior. Significant elevations of CPK and DHL were found in 35. In 30 patients (78%) electrocardiographic evidence of extension of infarction to the right ventricle was found, and in 3, evidence of right atrial infarction. Twenty‐one patients presented clinical data compatible with right ventricular infarction. In 19, cardiac rhythm and atrioventricular conduction disturbances were documented. Coronary angiograms practiced on 34 patients demonstrated single‐vessel (right coronary) disease in 12, affection of two vessels in 14, and lesions in three or more in 6. Coronary arteries presented no significant lesions in two cases. With TEE, alterations of right ventricular segmental mobility were demonstrated in all patients, and in 6, alterations of right atrial mobility as well. As respects the ventricular wall movement index, 68.5% had total scores (RV + LV) of 7lt;5. The other 31.5% had scores ≤ 5. In 26%, the right ventricular wall movement index was ≤ 4. The RVDD/LVDD ratio was 1 or less in 30 patients (78%) and > 1 in only 8 (22%). The conclusions from these findings are that: (1) TEE is an excellent diagnostic means of identifying right ventricular and/or atrial infarction; and (2) a relationship exists between the magnitude of right ventricular damage and a wall movement index of 5 or more or an RV/LV diastolic diameter ratio > 1:postinfarction hemodynamic deterioration is significantly greater and the incidence of intrahospitalary complications higher.

Multiplane Transesophageal Echocardiography with Dobutamine in Patients with Biventricular Inferior Myocardial Infarction

The purpose of this study was to evaluate the alterations of ventricular wall movement in patients with acute posteroinferior myocardial infarction with extension to right cavities with multiplane transesophageal echocardiography (TEE), as well as the utility of dobutamine with this technique to analyze myocardial viability. Nine men with a mean age of 51 years fulfilled the inclusion criteria. Myocardial TEE was performed in all the men 72 hours after the acute event with long‐ and short‐axis transgastric images of both ventricles under basal conditions and with dobutamine infusions of 5 and 10 μg/kg per minute. Results were compared with myocardial perfusion findings obtained with Tc‐99m Sestamibi SPECT. Left ventricular myocardial viability was demonstrated in 28 of 45 altered segments with dobutamine stress myocardial TEE and Tc‐99m Sestamibi SPECT. Right ventricular myocardial viability was identified in 27 of 30 altered segments with dobutamine stress myocardial TEE in transgastric short and long axes, and with Tc‐99m Sestamibi SPECT in 23 of 25 segments only in short‐axis images. Multiplane TEE provided excellent image resolution and better definition of endocardial and epicardial borders, which facilitated detailed evaluation of ventricular segmental wall movement. Infusion of low doses of dobutamine made it possible to identify viable tissue in both ventricles, and results were comparable to those of nuclear medicine.

Transesophageal Echocardiographic Study of Right Atrial Myocardial Infarction and Myocardial Viability

In order to determine the effects of dobutamine on right atrial wall movement, two groups were studied using transesophageal echocardiography. Group A included six patients without ischemic heart disease. Group B included six patients with infarction of the inferior wall of both ventricles and abnormal wall movement of the right atrium. In group A, an increase in the amplitude of right atrial movement was observed with dobutamine at doses of 5 and 10 μ/kg per minute. In group B, infusion of dobutamine did not modify wall akinesis in three patients with right atrial infarction; in the remaining three, alterations of segmental atrial movement were evident, and their responses to dobutamine were related to the patency of right atrial coronary branches. The following conclusions were reached: (1) dobutamine has a positive inotropic effect on atrial myocardium; (2) right atrial ischemia appears in the echocardiogram as altered segmental or global wall movement; (3) dobutamine can be used in the evaluation of atrial myocardial viability.

Echocardiographic diagnosis of a mycotic aneurysm of the main pulmonary artery and patent ductus arteriosus

heart block with no evidence of structural heart disease is not necessarily benign and the baby should be observed carefully for the first week or so. High-grade ventricular ectopy could result because of bradycardia and hence such a low heart rate itself is an indication for pacing these newborns. In emergency situations the umblical vein can be successfully utilized for inserting a temporary pacing lead.

The assessment of juxtaposed atrial appendages by transoesophageal echocardiography.

As part of a prospective study into the diagnostic role of transoesophageal echocardiography in children with complex congenital heart disease, the atrial morphology was assessed in 62 children. Using the direct visualization of atrial appendage morphology, 58 were shown to have usual atrial arrangement, two were documented to have isomerism of the right and two isomerism of the left appendages. Of those with usual arrangement, four children were demonstrated to have left juxtaposition of the atrial appendages. Only two of these patients were identified during praecordial echocardiographic re-evaluation, and three on angiocardiographic examination. Surgical confirmation was obtained in three, and juxtaposition was excluded in the remaining cases. The transoesophageal cross-sectional imaging features of left juxtaposition of the atrial appendages are unique and readily diagnostic of this entity. They include, first, a lateral deviation of the acid-portion of the atrial septum and, second, a frontal orientation of the antero-superior portion forming the floor and the posterior wall of the junction of the right-sided atrial appendage with the venous component of the atrial cavity. The knowledge of these morphologic characteristics is important, as, otherwise, this malformation may be misinterpreted as representing an atrial septal defect. The results suggest that transoesophageal echocardiography will be the most sensitive preoperative diagnostic technique in detecting or excluding juxtaposed atrial appendages.