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At least 883 records · Page 49Linked to original sources
PubMed1986

Persistent truncus arteriosus: pathologic anatomy in 54 cases.

Fifty-four specimens of heart with persistent truncus arteriosus (PTA) were reviewed anatomically. According to the Collett-Edwards classification there were 28 examples of type I and 26 type II. The sex distribution was equal. The number of the truncal cusps ranged from one to four (42% tricuspid, 30% bicuspid, 24% quadricuspid, and 4% unicommissural). A unicommissural truncal valve has not been previously reported. In 72% of cases, the truncal valve leaflets were thickened or dysplastic. Two valves were stenotic. The truncus arteriosus originated from both ventricles equally in 42% of the cases, predominantly from the right ventricle in 42%, and predominantly from the left ventricle in 16% of the cases. In unoperated cases of PTA originating predominantly from the right ventricle, it appeared to us that usual operative correction might result in left ventricular outflow obstruction. Variations in coronary arterial origins and patterns were present in nearly half of the cases. A single coronary artery was observed in ten cases (18.5%). Stenosis of the ostium of one coronary artery was seen in each of four cases (7%). High posterior origin of the left coronary artery was observed in ten cases (18.5%). Among the associated cardiovascular anomalies, the most common were right aortic arch (36%) and interruption of the aortic arch (11%). Three cases with the latter condition exhibited crossed pulmonary arteries. Isolated cases with tricuspid atresia, vascular sling (left pulmonary artery arising from right pulmonary artery), and persistent common atrioventricular canal were encountered.

Adolescent↗
PubMedDate not supplied

Radiologic diagnosis of different types of pulmonary stenoses.

Right ventricular outflow obstructive lesions are among the most prevalent congenital cardiac anomalies with a frequency ranging from 6--10% when considered as isolated anomalies. As part of a more complicated developmental complex, their frequency is undoubtedly higher. The diagnosis can be suspected by the traditional clinical and hemodynamic findings, but a firm diagnosis can only be made by angiography. An exact diagnosis is most important since most of these lesions can be surgically corrected.

Cardiomegaly↗
PubMed2000

Surgical cryoablation for frequent premature ventricular contraction originating in the left sinus of Valsalva.

Cryoablation in the left ventricular outflow tract and aortic valve replacement were conducted in a 70-year-old man with frequent premature ventricular contraction and nonsustained ventricular tachycardia complicated by Sellers grade 4 aortic regurgitation. The earliest endocardial activation site during premature ventricular contraction was confirmed by a preoperative electrophysiological study of the left sinus of Valsalva. Cryoablation preceded valvulectomy followed by aortic valve replacement. Clinical symptoms of premature ventricular contraction disappeared immediately after surgery.

Aortic Valve↗
PubMed2003

Catheter Ablation of Ventricular Fibrillation in Structurally Normal Hearts Targeting the RVOT and Purkinje Ectopy.

Catheter ablation for ventricular fibrillation in structurally normal hearts is in its infancy. Recently, catheter ablation of idiopathic ventricular fibrillation as well as ventricular fibrillation associated with the long QT and Brugada syndromes has been described. This review article is a summary of our current understanding of the technique and results of catheter ablation of ventricular fibrillation in structurally normal hearts.

Adolescent↗
PubMedDate not supplied

Elastin mutation screening in a group of patients affected by vascular abnormalities.

Supravalvular aortic stenosis is an uncommon but well-characterized congenital form of left ventricular outflow obstruction. The lesion involves the ascending aorta and often occurs in association with pulmonary arterial stenoses or stenoses of other arteries, especially at major branch points. It can occur sporadically, as an autosomal dominant condition, or as one component of Williams-Beuren syndrome. In fact, the clinical and structural characteristics of supravalvular aortic stenosis are identical in both syndromic and nonsyndromic cases. The severity of supravalvular aortic stenosis varies; but if it is left untreated, it may result in heart failure, myocardial infarction, and sudden death. Supravalvular aortic stenosis in Williams-Beuren patients occurs as a consequence of a complete deletion of one copy of the elastin gene on chromosome 7q11.23. However, the underlying genetic cause of isolated supravalvular aortic stenosis has been identified as translations, gross intragenic deletions, and point mutations that disrupt the elastin gene. We report the results obtained in a mutation screening of the elastin gene in 28 patients with supravalvular aortic stenosis and other vascular abnormalities. The aim of the screening was to characterize the molecular cause of this lesion. We have detected 11 changes, including nine polymorphisms and two novel putative missense mutations.

Adolescent↗
PubMedDate not supplied

An unusual aneurysm of the main pulmonary artery presenting as acute coronary syndrome.

A 70-year old man presented with retrosternal chest pain. His electrocardiogram showed nonspecific T wave changes. Cardiac-specific troponin I (cTnI) was elevated. His condition was managed as acute coronary syndrome, following which he had two minor episodes of hemoptysis. A CT pulmonary angiogram showed no evidence of pulmonary embolism, but a large mass lesion was seen in the mediastinum. Echocardiography and cardiac MRI demonstrated a large solid mass, arising from the right ventricular outflow tract and causing compression of the main pulmonary artery (MPA). The differential diagnosis included pericardial and myocardial tumors and clotted aneurysm of the MPA. At surgery, a clotted aneurysmal sac was identified originating from the MPA and the defect was healed. Aneurysms of the MPA are rare. They most commonly present with dyspnea and chest pain. Compression of surrounding structures produces protean manifestations. A high index of suspicion coupled with imaging modalities establishes the diagnosis. Blunt trauma to the chest, at the time of an accident 4 years previously, may explain this aneurysm. The patient's presentation with chest pain was probably due to compression and/or stretching of surrounding structures. Coronary artery compression simulating acute coronary syndrome has been documented in the literature. The rise in cTnI may have been due to right ventricular strain, as a result of right ventricular outflow obstruction by the aneurysm. This has not been reported previously in the literature. The saccular morphology and narrow neck of the aneurysm predisposed to stagnation leading to clotting of the lumen and healing of the tear, which caused the diagnostic difficulty.

Aged↗
PubMed2004

Unusual presentation of a pericardial cyst.

Pericardial cysts are rare abnormalities and usually do not cause any symptoms. Rarely, pericardial cysts cause symptoms and may lead to complications. There have been multiple case reports of rare complications of pericardial cysts including right ventricular outflow obstruction, pulmonary stenosis, pericardial tamponade, and partial erosion into the superior vena cava. We report a case of delayed presentation of a pericardial cyst following trauma causing a syndrome resembling congestive heart failure. To our knowledge there has been only one report of this complication.

Accidental Falls↗
PubMed2000

[Congenital tubular supravalvular aortic stenosis with massive coronary artery dilatation in a 35-year-old man].

Supravalvular aortic stenosis is a rare cause of left ventricular outflow obstruction in adults. It occurs as an isolated defect sporadically or on a hereditary basis with an autosomal dominant trait without further phenotypical anomalies, or as part of the Williams syndrome with mental retardation and multiple other anomalies. This lesion was proved to result from a defect of the elastin coding gene. Supravalvular aortic stenosis is frequently associated with cardiovascular defects, particularly of the peripheral pulmonary arteries, thoracic aorta, carotid, subclavian, and coronary arteries and the aortic valve. The coronary arteries are subject to an increased perfusion pressure leading to dilatation, tortuosity and accelerated arteriosclerosis. We give details of a 35-year-old patient in whom a previously asymptomatic supravalvular aortic stenosis is associated with an excessive dilatation of the right coronary artery and the left anterior descending coronary artery as well as an ostium stenosis of the left common carotid artery. The patient did not present any phenotypical anomalies of the Williams syndrome.

Adult↗
PubMed2004

Metastatic melanoma of the left ventricle: cardiac imaging in the diagnosis and surgical approach.

A mass lesion occupying the left ventricle was noted on a screening CT scan in a 42-year-old man with a history of malignant melanoma. Subsequent echocardiography and cardiac MR imaging provided further hemodynamic and anatomic characterization of the lesion. These studies were also essential in guiding the proper surgical approach to allow extensive resection of the large mass without disrupting cardiac structures and function. The unique clinical aspects of this case are the unusual location for a lone cardiac metastasis of melanoma and the asymptomatic presentation despite the large size of the tumor and its apparent obstruction of ventricular outflow. The clinical and imaging features of this patient's threatening cardiac lesion are presented.

Adult↗
PubMed2006

Diffuse supravalvular aortic stenosis: comprehensive imaging with ECG-gated CT angiography.

Congenital supravalvular aortic stenosis (SVAS) is a rare cause of congenital left ventricular outflow obstruction and pressure overload, with a diffuse form that often extends into the aortic arch, and can be associated with stenoses in other vascular territories. Knowledge of the extent of disease involvement can be complementary to medical management or critically important prior to surgical intervention. We report a case of diffuse SVAS and demonstrate the utility of ECG-gated multidetector computed tomography (CT) as a comprehensive diagnostic tool in evaluating such patients.

Adult↗
PubMed2000

Sarcoidosis and the Heart.

The treatment of sarcoid cardiomyopathy can be considered in part the treatment of the systemic disorder, and in part cardiac involvement, the manifestations of which may differ greatly. Therapy for the systemic disease is corticosteroid. Therapy for cardiac involvement includes prednisone, but because treatment must ameliorate or abolish many differing manifestations, therapy differs among patients. Asymptomatic patients (the majority) who are free from serious manifestations of the disease do not require pharmacologic or interventional treatment. Patients with dilated cardiomyopathy require treatment for congestive heart failure. High-grade atrioventricular conduction delay usually necessitates a permanent electronic pacemaker. Life-threatening arrhythmia usually requires implantation of an automatic implantable cardiac defibrillator (AICD). Antiarrhythmic drugs may also be needed. Cardiac tamponade should be treated by drainage of pericardial fluid. Pericardiectomy is usually the appropriate treatment for patients who develop significant constrictive pericarditis. Calcium channel blockers may be helpful for severe diastolic dysfunction that occurs in those with restrictive cardiomyopathy. Therapy should be given to those few patients who manifest hypertrophic cardiomyopathy to relieve left ventricular outflow obstruction. Cardiac transplantation for intractable heart failure or arrhythmia may be needed.

Journal Article↗
PubMed1986

Temporal relationship between early systolic closure of the aortic valve and aortic flow: experimental study with and without systolic anterior motion of the mitral valve.

To investigate the causative factors of typical early systolic closure (ESC) of the aortic valve (AoV), the temporal relationships of ESC with changes in blood pressures and aortic flow (AoF) were examined in dogs with experimentally induced ESC with and without systolic anterior motion (SAM) of the mitral valve. SAM was induced by dobutamine administration. Typical ESC was observed, but no SAM or left ventricular outflow obstruction, in five of the eight dogs treated with isoproterenol. Regardless of the presence or absence of SAM, the onset of ESC occurred before and the end of ESC immediately after the peak of AoF. A similar time relationship was noted between partial closure of the AoV, and pressure and flow changes before administration of isoproterenol, as well as after its termination. These results exclude a rapid decrease in flow velocity as the cause of ESC in our experimental preparation. The nearly uniform temporal relationship between ESC and changes in AoF observed under various conditions is considered to have important implications in the cause of ESC.

Animals↗
PubMed1989

Magnetic resonance imaging of congenital heart disease: sensitivity and specificity using receiver operating characteristic curve analysis.

MRI has shown promise for the evaluation of various congenital heart lesions. The current study was designed to assess the sensitivity and specificity of the technique for the evaluation of all major anatomic elements of the heart affected in simple and complex congenital heart disease. MR images of 51 patients (31 males and 20 females, ages 3 to 69 years) with a total of 110 congenital heart lesions were reviewed by a panel of two cardiac radiologists and one pediatric cardiologist, who assessed the cardiac pathoanatomy without knowledge of clinical details and assigned a confidence level to each diagnosis. The true diagnosis was established independently by the findings of angiocardiography and catheterization as well as by surgery, where applicable. ROC curves were generated from the responses, and the sensitivity at a specificity level of 90% was determined. MRI was shown to have a high sensitivity in evaluating great vessel relationships (100%), thoracic aorta abnormalities (94%), ASDs (91%) and VSDs (100%), visceroatrial situs (100%), and loop (100%). Three of five anomalies of the pulmonary and systemic veins were diagnosed by MRI. Right ventricular outflow obstructions (95%) were detected with a much higher sensitivity than lesions of the other valves (aortic valve 52%, mitral valve 62%, tricuspid valve 76%). Spin-echo MRI is a sensitive and specific method for the noninvasive assessment of congenital heart disease, with limitations in the evaluation of some valvular anomalies.

Adolescent↗
PubMed1975

Combined hyertrophic subaortic stenosis and calcific aortic valvular stenosis.

A well documented case of combined hypertrophic subaortic stenosis and calcific aortic stenosis is reported. Detection of multilevel involvement in cases of left ventricular outflow obstruction requires a high index of suspicion and precise hemodynamic and angiographic documentation. Careful analysis of the total data base is necessary for proper management of the patient. The pathogenesis of this combined lesion is unclear: Asymmetrical septal hypertrophy may occur as a consequence of the valvular stenosis, or it may be that abnormal leaflet motion in patients with hypertrophic obstruction produces leaflet thickening, calcification, deformity and stenosis.

Aged↗
PubMed1981

Correction of tetrad of Fallot with reduced incidence of right bundle branch block.

In 20 patients who underwent a modified surgical repair of tetrad of Fallot complete right bundle branch block developed in only 8 (40 percent). Standard and intraoperative conduction studies indicated that in these patients the right bundle branch block was due to injury of the right bundle branch near the ventricular septal defect (proximal right bundle branch block). The modified operative technique is aimed at minimizing the injury to the right ventricle and it includes a significantly shorter than usual ventriculotomy incision and avoidance of the septal (moderator) band during infundibulectomy. Intra- and postoperative hemodynamic studies of these patients revealed that relief of the right ventricular outflow obstruction was optimal.

Adolescent↗
PubMed1984

Cardiovascular features of homozygous familial hypercholesterolemia: analysis of 16 patients.

Familial hypercholesterolemia (FH) is characterized by an autosomal codominant inheritance, an abnormality in low-density lipoprotein (LDL) receptor function, elevated plasma cholesterol levels and premature atherosclerosis. Sixteen patients with homozygous FH were studied to correlate the extent of their atherosclerotic disease with their lipid levels and receptor function. The age range at initial presentation was 3 to 38 years (mean 12), and at the last examination, 6 to 43 years (mean 20). The mean pretreatment total plasma cholesterol concentration for all patients was 729 +/- 58 mg/dl (+/- standard error of the mean), and the mean LDL cholesterol level was 672 +/- 58 mg/dl (normal 60 to 176). High-density lipoprotein cholesterol was 28 +/- 3 mg/dl (normal 30 to 74). In the 7 patients with FH who had symptoms of myocardial ischemia (Group I), the mean pretreatment LDL cholesterol value (817 +/- 62 mg/dl) was higher than that of the 9 asymptomatic patients (Group II) (560 +/- 74 mg/dl). In Group I, 5 of 7 patients had left or right coronary ostial narrowing and 3 had significant left ventricular outflow obstruction. Most coronary arterial narrowing occurred in the right coronary and left anterior descending arteries and the least amount in the left circumflex coronary artery. A femoral bruit was the physical finding that correlated best with the Group I population; brother:sister pairs revealed a milder clinical course for the female. Seven of the 16 patients have survived into their third decade without symptoms. Comparison of these persons with those in whom angina developed reveals a marked heterogeneity in their clinical course, which appears to be associated with receptor negative/defective status.

Adolescent↗
PubMed1986

Continuous wave Doppler in the evaluation of simple and complex congenital heart disease in infants and children.

Continuous wave Doppler assessment of systolic pressure gradients was performed using a digital maximal frequency estimator in 118 consecutive infants and children with suspected ventricular outflow obstruction who were undergoing cardiac catheterisation. There was satisfactory correlation with measured systolic pressure gradients in most patients with isolated pulmonary valve stenosis, aortic stenosis or aortic coarctation. Important under-estimation of gradients, however, occurred frequently in patients with more complex lesions. In many of these, the stenotic jet was posteriorly located or obstruction to flow occurred at more than one level. Continuous wave Doppler assessment of outflow tract gradients should be interpreted with caution in complex congenital heart lesions. A low predicted gradient should not be relied upon in isolation for clinical decision making. On the other hand, demonstration of a large gradient by continuous wave Doppler ultrasound provides additional information which may obviate the need for invasive investigation.

Adolescent↗