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Biomedical subjects

R D Rowe

Publications and source records attributed to R D Rowe.

At least 127 records · Page 7Linked to original sources
PubMed1978

Permanent cardiac pacing in infants and children.

This clinical review details our 15 year experience with permanent cardiac pacemakers in 81 infants and children. Pacing was found inappropriate in one infant. The other 80 patients were paced because of congenital heart block [24], post-operative block [50], or sick sinus syndrome [6]. Maintenance of long-term pacing requires all too frequent re-operation for battery depletion [37%], lead related problems [32%] or wound dehiscence [31%]. Problems related to pacemaker size and the presence of a high myocardial threshold are particularly important in the pediatric patient. In spite of these problems, children requiring cardiac pacemakers can be extremely well, their prognosis depending almost entirely on the presence of underlying heart disease.

Adolescent↗
PubMed1978

Nonstructural heart disease in the newborn. Observations during one year in a perinatal service.

One-third of 327 newborn infants referred to the perinatal service of the Hospital for Sick Children during 1975 with suspected cardiopulmonary disorders proved to have nonstructural heart disease. Most of these were term infants with transient tachypnoea or cyanosis who recovered. A history of fetal distress or difficult delivery was commonly associated. The haemodynamic disorder for most was a delay in the normal progress of the transitional circulation. Evidence of myocardial ischaemia was present in 40%, and about half of these developed congestive heart failure. Aids to diagnosis of the ischaemic complication included echocardiography and myocardial perfusion scanning. For a small proportion specific metabolic disturbances, myocarditis, or dysrhythmia seemed the primary cause but even for these there were reasonable grounds to suspect a prenatal origin. Current general supportive measures were of value in treatment.

Arrhythmias, Cardiac↗
PubMed1978

The prostaglandin challenge. Test to unmask obstructed total anomalous pulmonary venous connections in asplenia syndrome.

The patient with complex congenital heart disease, severe pulmonary outflow obstruction, and visceral heterotaxia, may have 'silent' obstruction of the pulmonary venous return. Severe reduction of pulmonary blood flow secondary to pulmonary stenosis or atresia in such patients may prevent the usual radiographic appearance of pulmonary oedema. If such obstructed anomalous pulmonary venous connections are not diagnosed before operation, construction of a systemic to pulmonary artery anastomosis will unmask the obstruction, usually resulting in pulmonary oedema and death. We have recently 'challenged' a neonate with dextrocardia, vesceral heterotaxia, presumed asplenia, and complex congenital heart disease including pulmonary atresia, with an infusion of prostaglandin E1 to increase pulmonary blood flow via his ductus arteriosus. This resulted in severe pulmonary oedema which partially resolved after the infusion was discontinued. This was interpreted as consistent with obstructed total anomalous pulmonary venous return. This was confirmed at necropsy. Thus, the infusion of prostaglandin E1 before operation in the patient with asplenia or similar cardiac disease may be of aid in unmasking 'silent' obstructions of the pulmonary veins, and is of obvious value in the preoperative assessment of such patients.

Heart Defects, Congenital↗
PubMed1978

The reliability and practicality of single crystal echocardiography in the evaluation of single ventricle. Angiographic and pathological correlates.

A prospective clinical and echocardiographic diagnosis of single ventricle was made in 42 patients. Each was evaluated for the number of atrioventricular (A/V) valves, presence of an outflow chamber (OC), A/V valve-semilunar continuity, and orientation of the great arteries. Angiographic correlations were subsequently obtained in 40 and autopsies in 12. The overall diagnosis of single ventricle was substantiated in 39. Two other patients diagnosed as single ventricle by angiography were thought to have large ventricular septal defects on echocardiography. Tricuspid valve was interpreted as septum in one. The angiographic diagnosis of single ventricle was incorrect in another, correctly diagnosed by echocardiography and confirmed at pathology. The differential diagnosis also included A-V canal, L-transposition of the great arteries, double outlet right ventricle, and tricuspid atresia. This last condition has to be differentiated on clinical evidence. The echocardiograms were of particular value in determining the number of A/V valves. Two great arteries were demonstrated in 74% of patients and their relationship was correctly determined in 79% of these. Both imaging techniques agreed closely as to A/V valve-semilunar continuity and presence of an OC, but both showed some inaccuracies compared to pathological specimens. The echocardiogram helps both in planning catheterization and in evaluating the overall diagnosis.

Adolescent↗
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

Down syndrome with congenital heart malformation.

Two hundred fifty-one patients with Down syndrome and congenital heart disease was based on clinical (41%), catheterization (38%), surgical (11%), or autopsy data (10%). The most common lesions were endocardial cushion defect (43%), ventricular septal defect (32%), secundum atrial septal defect (10%), tetralogy of Fallot (6%), and isolated patent ductus arteriosus (4%). Thirty percent had multiple cardiac defects. The most common associated lesions were patent ductus arteriosus (16%) and pulmonic stenosis (9%). Twenty-five percent of the patients uncerwent cardiac surgery. Motality in the 68 patients undergoing surgery was 26% for open heart procedures and 11% for closed heart surgery. In 32% of nonsurgically treated patients with large left-to-right shunts, irreversible pulmonary vascular disease developed. Improved medical and surgical care have decreased morbidity and mortality in these patients in recent years.

Abnormalities, Multiple↗
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

Bronchopulmonary circulation in d-transposition of the great arteries: possible role in genesis of accelerated pulmonary vascular disease.

Hemodynamic and angiograhic data from 209 patients with d-transposition of the great arteries were reviewed to estimate the incidence of prominent bronchopulmonary circulation and to explore its role in the genesis of accelerated pulmonary vascular disease in these patients. The degree of bronchopulmonary circulation was assessed visually by considering the extent of the pulmonary arterial opacification and the circulation to the left atrium. An initial survey study revealed a marked degree of collateral circulation in 20 of 138 patients with d-transposition having cardiac catheterization before age 2 years at the Hospital for Sick Children, Toronto, between 1967 and 1972. Detailed analysis of 71 additional patients with d-transposition aged 1 week to 72 months (mean 17 months) studied at Children's Memorial Hospital, Chicago, between 1967 and 1974 showed collateral circulation of marked degree in 23 and of mild degree in 14. The bronchopulmonary collateral vessels were more freqently demonstrated in the patients with intact ventricular septum than in those with ventricular septal defect or left ventricular outflow tract stenosis. In a prospective study in 12 of 15 patients during cardiac catheterization the functional patency of the bronchopulmonary collateral circulation was demonstrated by obstructing pulmonary blood flow in the right or left pulmonary artery, or both, with an inflated balloon and obtaining from the pulmonary arterial segment distal to the occlusion blood with an oxygen saturation similar to that of the aorta. A hypothesis is presented concerning the role of systemic hypoxemia and local pulmonary hypoxemia induced by way of the bronchopulmonary collateral vessels and the bronchial arterial vasovasorum in promoting pulmonary vasoconstriction. It is suggested that increased pulmonary blood flow and pressure due to the physiologic features of ventricular septal defect, patent ductus arteriosus or transposition of the great vessels, in the face of this regionally increased hypoxemia results in accelerated pulmonary vascular disease.

Aortography↗
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

Evaluation of children with ventricular arrhythmias.

Ventricular arrhythmias are rare in childhood but may be associated with syncope and sudden death. This report describes 8 children with ventricular arrhythmias, 6 of whom suffered syncopal episodes. Ventricular tachycardia was documented in 5. One boy died suddenly. Complete cardiac investigation was carried out with exercise testing, Holter monitoring, echocardiography, cardiac catheterization, angiography, and electrophysiological studies. The spectrum of abnormalities related to the arrhythmias included prolapsing mitral valve, prolonged QT syndrome, sick sinus syndrome, congenital heart disease, cardiomyopathy, and idiopathic ventricular tachycardia. Exercise testing and Holter monitoring were particularly useful in documenting the arrhythmias and monitoring response to therapy.

Adolescent↗
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↗