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M Rijlaarsdam

Publications and source records attributed to M Rijlaarsdam.

At least 19 recordsLinked to original sources
PubMed2000

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.

Adult↗
PubMed1999

Tetralogy of Fallot with absent pulmonary valve and total anomalous pulmonary venous connection.

It has been shown that congenital absence of the pulmonary valve rarely occurs by itself but tends to be associated with other heart defects, especially tetralogy of Fallot. Other malformations and other complex cardiac malformations also have been described in patients with absent pulmonary valve. In this report we describe the findings of a patient with this combination of cardiac defects who survived spontaneously to adulthood.

Adult↗
PubMed1999

Echocardiographic, catheterization, and nuclear medicine findings of an aneurysm of the muscular interventricular septum associated with aneurysm of the interatrial septum.

The unusual case of a young woman with an aneurysm of the muscular interventricular septum associated with an aneurysm of the interatrial septum and a muscular interventricular septal defect is presented. The echocardiographic, electrocardiographic, catheterization, and nuclear medicine findings are described.

Adult↗
PubMed1998

Congenitally corrected transposition with atrioventricular septal defect.

We describe 4 cases of congenitally corrected transposition associated with atrioventricular septal defect, diagnosed by echocardiography and angiocardiography. Two had usual atrial arrangement and two had mirror imaged atrial arrangement . All cases were associated with subpulmonary valvar stenosis. All patients presented with cyanosis and were in sinus rhythm. Atrioventricular septal defect with common atrioventricular junction was easily diagnosed on the basis of a common atrioventricular valve permitting interatrial and interventricular communications. All patients had balanced right and left ventricles. The echocardiographic recognition of the ventricles was based on the presence of the moderator band within the morphologically right ventricle, the characteristics of the apical septal trabeculations, and the shape of the ventricles. Angiocardiographic recognition of the ventricles was achieved on the basis of right and left ventriculography. In one case with usual atrial arrangement, we recorded two His bundle potentials, one anteriorly and another posteriorly. Atrial stimulation revealed blocked atrioventricular conduction at the level of the posterior bundle, and normal atrioventricular conduction through the anterior bundle. In both cases with atrial mirror-imagery, only a posterior His bundle potential was found, with normal atrioventricular conduction revealed by atrial stimulation The clinical course with this combination depends on the other lesions present in addition to the common atrioventricular valve. Our electrophysiological studies show that the conduction system in presence of a common atrioventricular valve is as expected for congenitally corrected transposition with two atrioventricular valves.

Adolescent↗
PubMed1995

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.

Adolescent↗
PubMed1994

Transthoracic and transesophageal echocardiographic study of pulmonary autograft valve in aortic position.

Eleven patients who underwent pulmonary valve autograft to aortic position with placement of bovine pericardial prosthesis in pulmonary position were studied with echocardiography. The etiology of aortic valvuloplasty as determined by anatomopathological examination was rheumatic in five, degenerative in four, and congenital in two. Important mitral stenosis coexisted in two patients, and during the same operation as the Ross surgery, a mitral valvuloplasty with Carpentier ring was practiced on one and an open mitral commissurotomy on the other. Transthoracic echocardiography, which helped to confirm the viability of the surgery by determining the diameters of the semilunar valve rings and quantifying the severity of the aortic valve lesions, was performed on all patients before surgery and repeated 3 months later. Transesophageal echocardiograms were practiced on nine patients during the surgical procedure and repeated after 6 months on seven. The latter technique aided in immediate postoperative evaluation, and repetition at 6 months served to explore the ventricular infundibuli and evaluate pulmonary valve performance in aortic position. In conclusion, transthoracic and transesophageal echocardiography provide a practical and valuable means of investigating the pre-, trans-, and postoperative conditions of patients undergoing Ross surgery.

Adult↗
PubMed1993

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, Ebstein's 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, Ebstein's anomaly, corrected transposition of great vessels, and univentricular atrioventricular connection and in diagnosis of the rarer congenital cardiopathies.

Adult↗
PubMed1991

Assessment of anomalous systemic and pulmonary venous connections by transoesophageal echocardiography in infants and children.

OBJECTIVE: To assess the value of transoesophageal echocardiography in the preoperative definition of systemic and pulmonary venous connections. DESIGN: Transoesophageal echocardiographic studies were performed prospectively under general anaesthesia in 76 consecutive unoperated children. Results were compared with those obtained by earlier transthoracic ultrasound studies (n = 76), cardiac catheterisation (n = 62), and subsequent surgical inspection (n = 58). SETTING: Two tertiary referral centres. PATIENTS: 76 unoperated infants and children (age 0.2-14.8 years, mean age 4.1 years) with congenital heart disease. MAIN OUTCOME MEASURE: Identification of anomalous systemic and pulmonary venous connections. RESULTS: Transoesophageal studies showed anomalous venous connections in 14 patients. Two had both anomalous systemic and pulmonary venous connections. Transoesophageal studies showed 12 anomalous systemic venous connections in nine patients. In eight patients these were confirmed at operation or catheterisation: one patient is awaiting operation. Six anomalous systemic venous connections were missed during earlier transthoracic studies. Anomalous pulmonary venous connections (one mixed total, six partial) were shown in seven patients. These were confirmed at operation in six and by cardiac catheterisation in one. Four of these patients were missed during earlier transthoracic ultrasound studies. No patient defined as having normal venous connections by the transoesophageal study was subsequently shown to have anomalous venous connections at operation or angiography. CONCLUSIONS: Transoesophageal echocardiography is a highly sensitive tool for the preoperative definition of systemic and pulmonary venous connections. In this series it was better than transthoracic ultrasound and complemented cardiac catheterisation and angiocardiography.

Adolescent↗
PubMed1990

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.

Adolescent↗
PubMedDate not supplied

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.

Aged↗
PubMedDate not supplied

[Fenestrated Fontan surgery in high risk patients].

A retrospective analysis is presented of all patients who had fenestrated Fontan procedure between january 1990 and may 1996. Surgery was indicated in the presence of anyone of the following risk factors: mean pulmonary pressure higher than 20 mmHg; pulmonary vascular resistance higher than 2 UW; ejection fraction less than 60%; systemic ventricular end diastolic pressure higher than 8 mmHg; Nakata index less than 200 mm2/m2, McGoon index less than 2. The diagnosis were: Absent right atrio-ventricular connection with concordance ventriculo-arterial connection 10 patients; pulmonary atresia with intact septum, 1 patient; Ebstein's malformation, 1 patient and absent left A-V connection with discordance VA connection, 1 patient. The mean of age was 6.7 years (range 2.5-11 years). Overall mortality was 23%. No significant difference in risk factors was found between survivals and no survivals. Nonsurvivors had between two an four risks factors. Postoperative complications were 1 patient with protein losing enteropathy and stroke (1 patient). The mean duration of pleural effusion was 16 days (range 4-45 days). We consider fenestrated Fontan procedure useful for patients with congenital heart disease with a one hypoplastic ventricle and one o more risks factors.

Child↗
PubMedDate not supplied

Ebstein's anomaly. Clinical profile in 174 patients.

The study population consisted of 148 patients who did not undergo surgical treatment and 26 who were operated, most of them diagnosed after the age of 2, with a follow-up from 6 months to 25.3 years. Patients were divided in three groups of clinical deterioration according to their functional class and cardiothoracic index (CTR) long-term follow-up in 148 nonoperated patients showed significant differences for mortality between groups I and III (p < 0.001), and between groups II and III (p < 0.02). Predictors of death included the association among functional class III or IV CTR > or = 65% with either cyanosis or arrhythmias (p < 0.05). The multivariate analysis showed that clinical deterioration (p < 0.0001), CTR (p < 0.0002) and functional class (p < 0.001), were significant for mortality. Kaplan-Meier analysis showed a survival rate of 81% in the overall patients free from surgical treatment. According to Kaplan-Meier analysis, the rate of survival was lower in patients with CTR > or = 65% (63.5%), in patients who had functional class IV (52.5%) and in patients included in group III of clinical deterioration (38.2%). Despite the fact that the association of functional class III or IV plus CTR > or = 65% with either cyanosis or arrhythmias is a good predictor for death, the mortality in patients who had only one of these variables was lower. Patients included in group II of clinical deterioration in stable condition presented long survival with medical treatment. Due to the high mortality rate found in group III, surgical treatment of Ebstein's anomaly must be done before deteriorating into group III. Surgical indication must be done considering the surgical risk of each group according to the experience of the Institution and comparing the rate of surgical mortality with the rate of survival without surgery.

Adolescent↗
PubMedDate not supplied

[Electrocardiography and echocardiography aspects of hypertrophic myocardiopathy in pediatrics].

Hypertrophic cardiomyopathy (HCM) is a heterogeneous disease, characterized by asymmetric hypertrophy of the left and/or right ventricle with disarray of myocardial fibers. In order to know its clinical and electrocardiographic manifestation in the pediatric age group, we made a retrospective study of 24 cases from 1986 to 1995. There were: 15 girls and 9 boys, with a mean age of 6 years (age range: 1 month to 17 years). Clinical manifestations were dyspnea (71%), syncope (42%) and palpitations (42%). Physical examination disclosed an aortic systolic murmur in all patients, a mitral regurgitation in 42% and physical signs of congestive heart failure in 54% of patients. Chest X rays showed cardiac enlargement in 71% and pulmonary capillary hypertension in 42%. The most frequent ECG abnormalities were: a prolonged time in the intrinsecoid deflection onset on leads corresponding to the affected region, more or less deep and clean Q waves on leads aVF, aVL, V5 and V6, as well as supraventricular and ventricular rhythm disturbances in 11 patients (46%) with and without congestive heart failure. Bidimensional echocardiography confirmed antero-septal hypertrophy in all patients. The mortality rate was 17%. HCM is rare disease in the pediatric age group. Mortality increases when congestive heart failure and arrhythmias are present. Treatment must be individualized in all cases.

Adolescent↗