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Biomedical subjects

R M Freedom

Publications and source records attributed to R M Freedom.

At least 307 records · Page 17Linked to original sources
PubMed1978

Endocardial cushion defect and significant hypoplasia of the left ventricle: a distinct clinical and pathological entity.

We have identified 12 patients with endocardial cushion defect and marked under-development of the left ventricle. Most of these patients had significant obstructive anomalies of the aortic arch. Pathologically, the left ventricle was very small, but without endocardial fibroelastosis, significant subaortic narrowing was evident, resulting from maladherent atrioventricular valve tissue to the left ventricular outflow tract, and the left ventricular posterior leaflet component of the atrioventricular valve was both grossly deficient and dysplastic. In all 12 a large ostium primum atrial communication was present, but the ventricular contribution to the defect was small. The clinical presentation of severe, intractable congestive heart failure in these neonatal patients would suggest the diagnosis of typical hypoplastic left heart syndrome. Important departures in some of these patients included a leftward, superior and counterclockwise frontal vector on the electrocardiogram and a widely split second sound. Selective biplane angiocardiography will reveal the underdeveloped left ventricle, with an elongated, fixed, 'gooseneck' deformity of the left ventricular outflow tract, severe 'mitral' regurgitation, and a small ascending aorta. Selective right ventriculography will opacify the very much larger right ventricle and pulmonary artery. Finally, these cases broaden the concept of double inlet right ventricle and exaggerated displacement of the atrioventricular canal towards the bulbus cordis.

Angiocardiography↗
PubMed1978

The tricuspid valve in pulmonary atresia and intact ventricular septum: a morphological study of 60 cases.

A morphologic study of the tricuspid valve was performed in 70 patients with pulmonary atresia and intact ventricular septum who had died and subsequently undergone autopsy. The right ventricular cavity was enlarged in 8 patients, normal in 4 patients, mildly underdeveloped in 18 patients, and markedly underdeveloped in 24 patients. In the patients with dilated and enlarged right ventricle, the tricuspid valve exhibited features of dysplasia or Ebstein's malformation (downward displacement), or both, with a dilated anulus. The majority of patients had restriction of the tricuspid valve apparatus and an underdeveloped anulus, usually of a severe degree. The right ventricle appeared to have normal dimensions in four patients, but the tricuspid valve and tensor apparatus, even in these patients, exhibited departures from the normal. This study suggests that in many of these patients adequate right ventricular growth will not be achieved because of inadequate right ventricular filling. This seems directly related to moderate or severe underdevelopment of the tricuspid valve.

Child, Preschool↗
PubMed1978

The straddling mitral valve: morphological observations and clinical implications.

The morphological features of 23 patients with straddling or overriding mitral valve are presented. Levocardia was present in 20 of 23; visceroatrial situs solitisu in 20 of 23, with 3 patients, 2 with asplenia and 1 with polysplenia, having visceral heterotaxia. A concordant D-ventricular loop was present in the 20 patients with visceroatrial situs solitus. Six of these had double outlet right ventricle; 2 had asplenia syndrome; 1 had D-transposition of the great arteries, ventricular defect and pulmonary atresia; 1 with tricuspid atresia and double outlet-outlet chamber; 1 with polysplenia syndrome; and 12 had endocardial cushion defect with marked underdevelopment of the left ventricle, and normally related great arteries. Left ventricular size was related to the amount of mitral valve (or left-sided component of a common atrioventricular valve) connected to it. In those patients in whom little effective mitral orifice was connected to the left ventricle, the left ventricle was diminutive. Endocardial fibroelastosis of the left ventricle was noted in only a single patient. Six of the 7 patients with double outlet right ventricle (including one with double outlet bulbus) had subpulmonary obstruction, and in one of these, this was related in part to the straddling mitral valve. In 1 patient with double outlet right ventricle, there was a double orifice mitral valve, and it was the accessory mitral orifice that straddled. The diagnosis of overriding mitral valve should be suspected in any patient with significant conotruncal anomalies and underdeveloped left ventricle, especially the patient with double outlet right ventricle, and in the patient with endocardial cushion defect, hypoplasia of the left ventricle, and obstructive anomalies of the aortic arch. In certain patients, selective left atriography, left ventriculography, and single and two dimensional echocardiography may be diagnostic of this condition.

Abnormalities, Multiple↗
PubMed1977

Double outlet right ventricle: clinical spectrum and prognosis.

Patients with double outlet right ventricle can be separated into four distinct groups. In the largest (Group III), patients have a subaortic ventricular septal defect and pulmonary stenosis and clinically resemble patients with tetralogy of Fallot. In the next largest group (Group I), patients have a subpulmonry ventricular septal defect and no pulmonary stenosis and clinically resemble children with D-transposition of the great arteries and a ventricular septal defect. These patients have a high rate of coarctation of the aorta leading to early congestive heart failure, and their overall prognosis is poor. In the next largest group (Group II), patients have a subaortic ventricular septal defect and no pulmonary stenosis. Their presentation is similar to that of children with a large ventricular septal defect and pulmonary hypertension. In the smallest group (Group IV), the ventricular septal defect is uncommitted. Survivors in this group also clinically resemble children with a large ventricular septal defect and pulmonary hypertension. When present, coarctation of the aorta and severe mitral valve abnormalities greatly influence the prognosis in double outlet right ventricle. Although the prevalence of associated cardiac abnormalities is large, asplenia, polysplenia, chromosomal abnormalities and other congenital noncardiac abnormalities occur in only 12.5% of patients with double outlet right ventricle.

Aortic Coarctation↗
PubMed1977

Conal anatomy in aortic atresia, ventricular septal defect, and normally developed left ventricle.

Although aortic valve atresia is usually associated with severe underdevelopment of the mitral apparatus and left ventricle in rare cases of aortic atresia, the left ventricle may be of normal size, or even enlarged. This occurrence seems related to the presence of a significant ventricular septal defect. We have presented the morphologic findings in seven patients with aortic atresia and normally developed left ventricle, (six necropsied patients, and one studied angiocardiographically). Four autopsied patients had conal type ventricular septal defects, characterized in three by conoventricular malalignment. Subaortic atresia in these patients resulted from leftward deviation of the conal septum. One patient with aortic atresia and well-developed left ventricle had a membranous defect, and one patient had a complete A-V canal. The ventricular septal defect in the patients with conoventricular malalignment are very similar to the conal VSD observed in patients with aortic arch interruptions. Although ultimate survival with these uncommon groupings of anomalies necessitates patency of the ductus arteriosus, clinical recognition rests on (1) awareness of its existence, (2) ultrasonography, and (3) selective biventricular and aortic angiography. It is possible that some of these patients might be candidates for ventriculo-aortic reconstitiution.

Aorta↗
PubMed1977

Development of "subaortic stenosis" after pulmonary arterial banding for common ventricle.

Progressive narrowing of the bulboventricular foramen is documented in four patients with single ventricle. The morphologic features in each case corresponded to single left ventricle with infundibular chamber. Two patients had a D-ventricular loop and the other two an L-loop. All four patients had transposition of the great arteries. Restriction of the bulboventricular foramen thus resulted in functional subaortic stenosis because the aorta originated above the infundibular chamber. The development of subaortic obstruction was documented by serial cardiac catheterization studies in two infants, one of whom underwent unsuccessful surgical enlargement of the bulboventricular foramen. In the other two patients the initial cardiac catheterization revealed no pressure gradient between the ventricle and aorta, but examination at necropsy revealed very severe obstruction at the bulboventricular foramen, thus suggesting that the obstruction had been acquired. In each patient, the progressive narrowing of the bulboventricular foramen occurred after pulmonary arterial banding and may have been causally related to this procedure. This functional subaortic obstruction developed in 4 of 31 patients (12.9 percent) with single left ventricle, transposition of the great arteries and pulmonary arterial banding. Clinical recognition of this development is predicated on (1) awareness that narrowing of the bulboventricular foramen in patients with single ventricle and pulmonary arterial banding may be common; (2) presence of symptoms such as angina; and (3) lack of continued clinical improvement in a patient whose pulmonary arterial band has significantly reduced pulmonary blood flow. Management of this subaortic stenosis requires surgical intervention. In the infant, a ventriculotomy in the outlet chamber will usually provide excellent exposure of the bulboventricular foramen, and surgical enlargement can be accomplished. In the older child with severe obstruction, marked muscle hypertrophy may obliterate the ventricular cavity, making ventricular partitioning difficult if not impossible.

Aortic Stenosis, Subvalvular↗
PubMed1977

Ventricular septal defect in interruption of aortic arch.

A necroscopy study of 34 patients with interruption of the aortic arch was carried out to characterize more fully the ventricular septal defect and the anatomic basis of subaortic obstruction. In 21 patients (61.8 percent) the site of interruption was between the left common carotid artery and the left subclavian artery; in 13 (38.2 percent) it was distal to the left subclavian artery; no case of interruption just distal to the innominate artery was found. A ventricular septal defect was present in all but two patients, both with large aortopulmonary fenestrations. All patients had visceroatrial situs solitus and D-ventricular loop. The great arteries were normally related in 33 patients and D-transposition was found in one patient. Twenty-one patients had a ventricular septal defect involving the conal septum. Conoventricular malalignment resulted in a typical subpulmonary ventricular defect. The malignant was characteristically in a leftward direction allowing for potential muscular narrowing of the left ventricular outflow tract. In some patients, the conal ventricular septal defect was characterized by a deficiency of a the conal septum without malalignment. In 4 of 21 patients with a ventricular septal defect involving the conal septum, the defect was immediately adjacent to the pulmonary.value. Typical infracristal membranous ventricular defects five patients), cushion defects (3 patients) and muscular defects (3 patients), were also found. The potential for subaortic narrowing was present in some of these patients as well. No relation between position of ventricular septal defect and type of arch interruption could be discerned. The presence or absence of subaortic obstruction was not predictive of a specific type of interruption. Similarly, although deformity of the aortic valve was not uncommon in this series, it was not associated with any specific type of interruption.

Aorta↗
PubMed1977

Angiocardiography of subaortic obstruction in infancy.

There is considerable heterogeneity among pathologic mechanisms responsible for subaortic obstruction in infancy. Although the relatively discrete forms of subaortic stenosis are uncommon in this age group, conoventricular malalignment, maladherent mitral valve, accessory endocardial cushion tissue, restrictive bulboventricular foramen, and a poorly expanded suboartic conus are responsible for most types of subaortic stenosis in this age group. The angiocardiographic recognition of these types of subaortic obstruction is aided by awareness of the anatomic framework in which they are found. For example: (1) conoventricular malalignment is commonly noted in patients with complete interruption of the aortic arch; (2) restriction at bulboventricular foramen level results in functional subaortic stenosis in those patients whose transposed aorta originates above an infundibular chamber; and (3) a poorly expanded subaortic conus may result in subaortic stenosis in patients whose ventricular loop and conotruncus have twisted in opposite directions (the anatomically corrected malposition). Subaortic obstruction in infancy is not uncommon, occurring in 99 of 1,362 necropsied infants with congenital heart disease. Nearly 70% of the patients with subaortic stenosis had obstructive anomalies of the aortic arch. Subaortic obstruction can be demonstrated angiographically, but high quality angiocardiographic technique is a prerequisite.

Angiocardiography↗
PubMed1977

Isolated ventricular inversion with double inlet left ventricle.

2 patients with viscero-atrial situs solitus, isolated ventricular inversion (IVI) and double inlet right-sided morphologic left ventricle are presented. Isolated ventricular inversion is a rare cardiac anomaly characterized by ventricular inversion, subpulmonary conus, and ventriculo-arterial concordance. Their angiocardiographic and pathologic features are presented, and the morphologic findings of the 9 patients in the literature with isolated ventricular inversion are reviewed. Of the 11 known patients with isolated ventricular inversion, levocardia was present in 10 and dextrocardia in 1; viscero-atrial situs solitus in 9 and inversus in 2; L-ventricular loop in 9 and D-loop in 2. The atrial septum was intact in 4. An intact ventricular septum was noted in only 2 patients while in 3, more than one ventricular septal defects were present, and 2 patients exhibited morphologic single ventricle. A solitary ventricular septal defect was noted in the remainder. Significant tricuspid valve abnormalities, including atresia, stenosis or hypoplasia with supravalvular fibrous ring were found in 7 patients. In 2 of these, both with significant obstruction at the tricuspid valve, both atrioventricular valves emptied into the morphologic left ventricle--thus isolated ventricular inversion with double inlet left ventricle. Pulmonary outflow tract obstruction was evident in only 3 patients. Total anomalous pulmonary venous return occurred twice and right juxtaposition of the atrial appendages once. Thus, while the patient with isolated ventricular iversion may present with clinical and hemodynamic features characteristic of classical transposition physiology the high frequency of significant associated anomalies would complicate this. Finally, the anomaly must be differentiated from the levo-transposition, isolated atrial inversion, and the anatomically corrected malpositions.

Adult↗
PubMed1977

Aneurysm of the membraous septum in ventricular septal defect.

An aneurysm of the membranous septum was found frequently in patients with ventricular septal defect; although appearance at catheterization during infancy was not common, aneurysms were detected in about half of the patients older than 2 years of age. A significant decrease in the left-to-right shunt occurred with the development of an aneurysm, but progression to complete spontaneous closure was not seen in any patient. In three older patients, an aneurysm of the membranous septum "disappeared" with time, even though complete spontaneous closure had not occurred.

Child↗
PubMed1976

Pulmonary arteriography in congenital heart disease.

Pulmonary outflow tract obstruction may occur at subvalve, valve, or pulmonary artery level. Selective ventriculography will usually define the intracardiac obstruction (whether valve, subvalve, or both) and may define pulmonary arterial abnormalities. Increasing experience in the surgical management of patients with tetralogy of Fallot and other conotruncal abnormalities characterized by significant pulmonary outflow tract obstruction suggests that preoperative recognition of pulmonary arterial stenosis is essential to the successful operative management of these patients (1-4). Because of the anatomicospacial relationships of the pulmonary arteries in "simple" (tetralogy of Fallot) and complex conotruncal malformations, selective ventriculography may not invariably provide sufficient detail about the morphologic state of the pulmonary arteries. Similarly, even selective pulmonary arteriography in the standard anterioposterior, lateral, and oblique views may not always allow accurate anatomic assessment of these vessels. Therefore, we wish to document a maneuver that has proved valuable in the assessment of pulmonary arteries during selective pulmonary arteriography. This maneuver was originally suggested to Peter M. Olley by Dr. L. M. Bargeron, Jr., and the surgical valve obtained from this angiographic view was stressed by Dr. John Kirklin in his John Keith lecture at the Canadian Cardiovascular Society (5). Although it is being used at some cardiovascular centers, we feel its application warrants this report.

Child, Preschool↗