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At least 415 records · Page 23Linked to original sources
PubMed1992

[The diagnosis of congenital heart defects today. Part 1: Ventricular septal defect, pulmonary stenosis, patent ductus arteriosus, atrial septal defect].

Most congenital heart diseases are detected in childhood, but some, such as atrial septal defect, can remain undiagnosed until adulthood. In the first part of this review, taking the auscultation findings and the clinical picture as a basis, the diagnosis of four common congenital heart diseases is discussed: ventricular septal defect, pulmonary stenosis, patient ductus arteriosus, and atrial septal defect. In addition, the latest developments in the field of therapy are briefly considered.

Ductus Arteriosus, Patent↗
PubMed2002

Diverticulum of the right ventricle associated with pulmonary stenosis, rhabdomyoma and Wolff-Parkinson-White syndrome.

Congenital cardiac diverticulum is a rare anomaly that may present as an isolated lesion or in association with other malformations. Diverticulum of the left ventricle is more common than that of the right ventricle. We report a case of cardiac diverticulum over the right ventricular outflow tract and associated pulmonary stenosis, right atrial rhabdomyoma, and Wolff-Parkinson-White syndrome in a 9-month-old boy. The delta wave disappeared after removal of the atrial rhabdomyoma.

Diverticulum↗
PubMed2006

Effects of volume and/or pressure overload secondary to congenital heart disease (tetralogy of fallot or pulmonary stenosis) on right ventricular function using cardiovascular magnetic resonance and B-type natriuretic peptide levels.

The aim of this study was to analyze the effect of pressure and/or volume overload on right ventricular (RV) function and brain natriuretic peptide (BNP) levels in patients with surgically corrected congenital heart disease. Forty-two consecutive patients aged 17 to 57 years (median 30) with congenital heart disease (32 with tetralogy of Fallot and 10 with pulmonary stenosis) were examined. The RV systolic pressure was estimated using Doppler echocardiography. Cardiovascular magnetic resonance imaging was used to obtain the RV volumes, ejection fraction (EF) and corrected EF (cEF). Plasma BNP levels were determined by immunoradiometric assay. Patients were categorized as having volume overload when pulmonary regurgitation was > or =10% and pressure overload when the RV systolic pressure was >40 mm Hg. Patients with RV volume overload had a lower RVEF compared with patients with pressure overload (p = 0.02) and lower left ventricular EF (p <0.001). BNP was higher in patients with volume overload than in patients with pressure overload (p = 0.002). BNP correlated with pulmonary regurgitation, RVEF, RV cEF, and left ventricular EF. In linear regression analysis, RV cEF was an independent predictor for BNP, after adjustment for age. Without the parameter of RV cEF in the regression model, pulmonary regurgitation and RVEF were independently associated with BNP level, after adjustment for age. In conclusion, patients with RV volume overload had higher BNP levels and lower RV function than patients with RV pressure overload. BNP levels were independently associated with the degree of RV volume overload and RV function.

Adolescent↗
PubMed1988

Changes in myocardial repolarization in patients undergoing balloon valvuloplasty for congenital pulmonary stenosis: evidence for contraction-excitation feedback in humans.

Alterations in ventricular loading conditions lead to changes in action potential duration and arrhythmias via contraction-excitation feedback; a decrease in load leads to prolongation of repolarization. To determine whether changes in right ventricular load alter ventricular repolarization in man, the corrected QT interval, a measure of overall ventricular repolarization, was measured in 32 patients before and after valvuloplasty for pulmonary stenosis. Right ventricular systolic pressure decreased (82.5 +/- 30.7 to 40.5 +/- 9.5 mm Hg, p less than .001) and the QTc increased concurrently (409.1 +/- 24.3 to 440.7 +/- 28.0 msec, p less than .001) after successful valvuloplasty. The increase in QTc was most marked for those patients with a greater than 30 mm Hg decrease in right ventricular pressure (40.0 +/- 23.3 vs 16.3 +/- 21.3 msec, p = .006). In a subset of seven patients in whom monophasic action potentials were recorded, monophasic action potential duration, a measure of local repolarization, was prolonged (230.0 +/- 24.3 vs 216.9 +/- 21.9, p less than .001) after successful valvuloplasty, confirming that the QTc prolongation reflected changes in local ventricular repolarization. In addition, during nine acute right ventricular outflow tract occlusions in a subset of six patients, monophasic action potential duration shortened (206.6 +/- 17.6 vs 221.7 +/- 20.9 msec, p less than .01) and early afterdepolarizations developed consistent with contraction-excitation feedback. These data suggest that, in humans, changes in mechanical load are associated with changes in ventricular repolarization consistent with contraction-excitation feedback.

Action Potentials↗
PubMed1995

[A case report of the radical correction of a truncus arteriosus and peripheral pulmonary stenosis in association with Holt-Oram syndrome].

A one year and 6 months old female infant was admitted to our department for a radical correction of a truncus arteriosus (Collett-Edwards type II). She also had a peripheral pulmonary stenosis and Holt-Oram syndrome. Under a cardiopulmonary bypass with deep hypothermia, PFO was closed directly. First of all, the pulmonary arteries were dissected from the aorta, the defect of the aorta was closed directly and VSD was closed with Dacron fabric patch. The reconstruction of the pulmonary arteries were performed with valved conduit which consisted of Dacron graft and 14 mm Björk-Shily valve. The post-operative course was uneventful. Cardiac catheterization study, done 77 days after surgery, revealed 44 mmHg of pressure gradient between RV outflow tract and right PA. The patient was doing well and two years have passed since the surgery. She should be observed carefully at the out-patient clinic due to the mechanical valve and the presence of pressure gradient at RV outflow tract.

Blood Vessel Prosthesis↗
PubMed1978

Successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance associated with dextrocardia and pulmonary stenosis.

The association of complete atrioventricular canal with other complex congenital cardiac anomalies has represented a significant challenge for the cardiac surgion. The combination of double-outlet right ventricle with complete atrioventricular canal has been particularly difficult to correct, with no surgical successes having been reported until recently. This is a report of the first successful repair of double-outlet right ventricle, complete atrioventricular canal, and atrioventricular discordance (ventricular incersion) associated with common atrium, bilateral superior venae cavae, dextrocardia, and pulmonary stenosis. The specialized conduction tissue was identified by intracardiac electrophysiological mapping, and normal sinus rhythm was preserved. Postoperative cardiac catheterization showed excellent hemodynamics. One year postoperatively, the patient was attending school, playing swimming without difficulty, and taking no cardiac medications. This good result lends encouragement for considering total repair for similar patients with the combination of double-outlet right ventricle, complete atrioventricular canal, and other associated congenital cardiac anomalies.

Angiocardiography↗
PubMed1990

[A rare adult case of double-outlet right ventricle without pulmonary stenosis: an autopsy case].

Double outlet right ventricle (DORV) is a rare cardiac malformation especially in adulthood. We report a woman with DORV who survived to the age of 33 years. She had not been seriously limited, before she visited our hospital for cardiac evaluation at the age of 25 years. She was cyanotic, and had clubbing of fingers and toes and facial edema. Holosystolic murmur and diastolic regurgitant murmur were audible along the left sternal border. Chest X-ray showed cardiomegaly and enlarged pulmonary trunks. Electrocardiography showed right axis deviation and biventricular hypertrophy. Laboratory examination revealed polycythemia (Hb: 22.4 g/dl), increased levels of hepatic enzymes due to congestive liver and marked hypoxemia (Pao2: 40 mmHg), Diagnosis of DORV was made with cardioangiography. Surgical repair was not indicated. Thereafter, she experienced recurrent heart failure which progressed gradually. She died probably of ventricular arrhythmia at the age of 33 years. At autopsy, the heart showed dilatation of the right atrium and ventricle, and hypertrophy of the bilateral ventricles. Both the pulmonary artery without stenosis and the aorta originated completely from the right ventricle, which were almost normally positioned with bilateral conus. Large ventricule septal defect was found at the subaortic portion. Patent ductus arteriosus and preductal aortic coarctation co-existed. To our knowledge, few cases with DORV who survived over 30 years have been reported, and they all manifested pulmonary stenosis. The present case is a very rare DORV that survived over 30 years without the complication of pulmonary stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2003

Technical aspects of the Rastelli and atrial switch procedure for congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis or atresia: results of therapy.

In conventional surgery for the associated lesions of congenitally corrected transposition of the great arteries, the right ventricle remains in the systemic circulation. In this situation, the right ventricle and tricuspid valve fail in an unpredictable manner. The double switch procedure was introduced to restore the morphologic left ventricle to the systemic circulation and considerable success has been seen over the last 10 years with this approach. The Rastelli and atrial switch procedure can be applied to patients with congenitally corrected transposition of the great arteries and pulmonary stenosis or atresia and a suitably placed ventricular septal defect in the outlet septum of the ventricle beneath the aortic valve. Thus, the left ventricle can be restored to the systemic circulation. The Rastelli-atrial switch is a complex operative procedure, but the operative risk and long-term results are good without evidence in the mid-term of ventricular failure as has been associated with the conventional repair. A disadvantage is that these patients require valved conduit changes over the years.

Abnormalities, Multiple↗
PubMed1986

Pulmonary stenosis with ventricular septal defect, common aorticopulmonary trunk, and dextroposition of the aorta: morphologic and qualitative physiologic effects in caffeine-treated chick embryos.

Effects of caffeine administration to Hamburger-Hamilton stage 19 chick embryos (3 days of incubation) were investigated. A morphologic study of the effect of caffeine on cardiogenesis showed that caffeine produced total cardiac malformations in the chick in a dose-related fashion. A maximum frequency of 70.6% was observed with 4.7 mg caffeine. Major malformations included common aorticopulmonary trunk and dextroposition of the aorta accompanied by ventricular septal defect with/without pulmonary stenosis. Qualitative analysis of cinegraphs following exposure of embryos to a single teratogenic dose of caffeine (3.5 mg/egg) produced marked alterations in cardiac function when compared with chick Ringer's controls. Within 3 minutes after exposure to caffeine, dilation of the common ventricle and weak ventricular contractility were observed and persisted for 1 hour. Dose-response data and microcinematographic observations suggest that caffeine induced cardiac anomalies by a direct toxic effect on the embryo rather than by altering cardiac cell function. Our data also suggest that pathophysiologic changes in cardiac function may play an important role in the pathogenesis of caffeine-induced cardiac anomalies in the chick embryo.

Abnormalities, Drug-Induced↗
PubMed2002

Lung perfusion scintigraphy in the diagnosis of peripheral pulmonary stenosis in patients after repair of Fallot tetralogy.

BACKGROUND: The frequency of peripheral pulmonary artery stenosis in patients after surgical repair of tetralogy of Fallot (TOF) ranges from 20 to 40%. This can be either primary or secondary to the surgical intervention. The influence of resulting lung perfusion alterations on the life quality of patients is difficult to predict. The aim of this study was to compare the utility of the diagnostic procedures in this group of patients, with particular focus on lung perfusion scintigraphy. MATERIAL AND METHODS: This study comprised 104 patients who underwent repair of TOF at ages from 5 months to 25 years. The patients have been followed up for from 4.2 to 25 years. On the basis of chest x-ray peripheral pulmonary artery stenosis was suspected in 11 patients, in 12 on the basis of echocardiography examination. RESULTS: Lung perfusion scintigraphy has been performed on 87 patients. The disturbances in lung perfusion (mostly in the left lung) were show by means of lung perfusion scintigraphy in 43 (49%) of patients. In 27 of them heart catheterisation has been performed. Angiography revealed stenosis of the lung artery branch in 15/43 (34.9%) patients with abnormal perfusion lung scan and in 4/44 (9%) in patients with normal perfusion lung scan. Intervention procedures were carried out on 10 patients. CONCLUSIONS: Lung perfusion scintigraphy may prove a valuable, non-invasive screening tool in the assessment of patients after TOF repair, although both false-negative and false-positive results may happen. Therefore, it should play an auxiliary role together with other diagnostic modalities.

Journal Article↗