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Biomedical subjects

R M Freedom

Publications and source records attributed to R M Freedom.

At least 235 records · Page 13Linked to original sources
PubMed1987

Critical aortic stenosis. Survival and management.

The factors associated with survival in 40 neonates (age less than 28 days) with critical aortic stenosis undergoing either open (22 patients) or closed (18 patients) transventricular aortic valvotomy were reviewed. Significant adverse correlates with survival included evidence of poor perfusion preoperatively (low pH, greater than Grade 2/6 soft ejection systolic murmur) and marked congestive heart failure (hepatomegaly, cardiomegaly, elevated left atrial pressure). Congenital mitral stenosis (anulus less than 11 mm), a small aortic anulus (less than 6.5 mm), and failure to achieve an adequate aortic orifice (greater than 6 mm), at operation were identified as factors associated with increased mortality. Initial perioperative survival was better with closed aortic valvotomy. However, there was no significant difference in overall operative survival between closed (9/18, 50%) and open (8/22, 36%) aortic valvotomy (p = 0.26). The incidence of early reoperation (less than 1 year of age) was greater in perioperative survivors undergoing closed valvotomy (7/13, 54%) rather than open valvotomy (1/10, 10%) (p less than 0.05). In conclusion, long-term survival among patients with critical neonatal aortic valve stenosis remains disturbingly low (13/40, 32%) and has not significantly improved over the past 20 years.

Aortic Valve↗
PubMed1986

Radionuclide angiographic evaluation of ventricular function in isolated congenitally corrected transposition of the great arteries.

Eight asymptomatic patients (mean age 19 years, range 7 to 32) with congenitally corrected transposition of the great arteries (CCTGA) underwent equilibrium gated radionuclide angiocardiography at rest and during supine bicycle exercise to assess systemic (morphologic right) and pulmonary (morphologic left) ventricular function. Five patients had normal intracardiac hemodynamic values, 2 had trivial atrioventricular valve regurgitation and 1 patient had trivial pulmonary ventricular outflow tract obstruction. Average exercise duration was 11 +/- 1 minute, with limitation due only to fatigue. At peak exercise, heart rate increased 225% and systolic blood pressure 152% over the rest value. Pulmonary ventricular ejection fraction at rest was 51 +/- 3% (mean +/- standard error of the mean); it did not change significantly at peak stress, 53 +/- 2%. Systemic ventricular ejection fraction was 48 +/- 4% at rest and increased to 64 +/- 4% at peak exercise (p less than 0.01). Count-based volume changes for the pulmonary chamber showed no significant change in end-diastolic or systolic counts at peak exercise (109 +/- 8% and 106 +/- 9% of rest value, respectively). However, end-diastolic counts decreased 13% (87 +/- 3% of rest value) and end-systolic counts 34% (62 +/- 7% of rest value) at peak exercise in the systemic ventricle. These data suggest normal systemic and impaired pulmonary ventricular function in patients with congenitally corrected transposition of the great arteries unaccompanied by significant associated lesions. These findings have important clinical implications in the setting of complex congenital heart disease in patients in whom a morphologic right ventricle functions as the systemic pumping chamber. Despite the pulmonary ventricular dysfunction, symptoms were not apparent at rest or during exercise.

Adolescent↗
PubMed1986

Cerebral complications following balloon angioplasty of coarctation of the aorta.

A 10-month-old male child underwent balloon angioplasty for a recurrent coarctation of the aorta. Postprocedural hemorrhagic cerebral infarction led to the child's death. At necropsy, hypoplasia of the left posterior communicating artery of the circle of Willis was found. The implications of such variations in cerebral blood flow circulation on the angioplasty technique are presented and discussed.

Angioplasty, Balloon↗
PubMed1986

Severe obstruction to systemic blood flow in congenitally corrected transposition (discordant atrioventricular and ventriculo-arterial connexions): an analysis of 14 patients.

The more common associated cardiac anomalies in any cohort of patients with discordant atrioventricular and ventriculo-arterial connexions (congenitally corrected transposition) include ventricular septal defect, left ventricular (subpulmonary) outlet obstruction, and displacement and/or dysplasia of the morphologically tricuspid valve. There is scant mention in the literature of severe obstruction to systemic blood flow at aortic valve level or beyond in these hearts. The present study reviews those pertinent morphological-clinical and follow-up data on 14 patients with congenitally corrected transposition and aortic valve atresia (one patient); aortic arch interruption or aortic arch atresia (two patients) and coarctation of the aorta (11 patients). All but one patient (the patient with aortic valve atresia) had a perimembranous ventricular septal defect. Mild outflow tract obstruction of the morphologically left ventricle was identified in one patient. Nine patients, however, demonstrated a significant structural and functional disturbance (Ebstein-like with dysplasia) of the systemic morphologically tricuspid valve. No patient in this series was identified with isolated aortic coarctation.

Adolescent↗
PubMed1986

Results of total correction in complete atrioventricular septal defects with congenital or surgically induced right ventricular outflow tract obstruction.

The outcome of total repair in 29 children who had complete atrioventricular septal defect (AVSD) and congenital or surgically induced right ventricular outflow tract obstruction (RVOTO) is reviewed. All 11 patients with congenital RVOTO had normal pulmonary artery (PA) pressure before the complete repair. Of the 18 children who had undergone PA banding, seven had PA pressure above 30 mm Hg (mean, 53.5). Two had elevated pulmonary vascular resistance (greater than 3 units). Early mortality was 18.2% for the patients with congenital RVOTO and 44.4% for those who had undergone PA banding (p not significant). After a mean follow-up of 5 years, the results are good in the survivors of both groups. Analysis of multiple-risk factors indicate that, for the total group of patients, death was significantly more common in children less than 5 years of age (p less than 0.01) or less than 15 kg (p less than 0.02) than in older or larger patients.

Adolescent↗
PubMed1986

Pulsed Doppler echocardiography in the preoperative evaluation of total anomalous pulmonary venous connection.

Pulmonary venous flow was evaluated by pulsed Doppler echocardiography in 38 patients with total anomalous pulmonary venous connection. Twenty-nine of these 38 had no associated intracardiac anomaly (Group I), and 9 had complex intracardiac anatomy associated with low pulmonary blood flow (Group II). In Group I the drainage was infracardiac in nine, supracardiac in seven, intracardiac in eight and mixed in five. In both groups, in those with venous obstruction the flow in the individual pulmonary veins and ascending or descending vein was nonphasic, varying only with respiration. Flow in the absence of obstruction was phasic, varying with the cardiac cycle. Distal to a site of obstruction the flow was nonlaminar and of high velocity irrespective of the amount of pulmonary blood flow. The pulsed Doppler technique provides important physiologic information in the patient with total anomalous pulmonary venous connection before surgical intervention.

Child, Preschool↗
PubMed1986

Subaortic stenosis, the univentricular heart, and banding of the pulmonary artery: an analysis of the courses of 43 patients with univentricular heart palliated by pulmonary artery banding.

Subaortic stenosis is well known to complicate the clinical course of patients with single ventricle or univentricular hearts, and we have previously suggested that the development of subaortic stenosis in such patients may be causal to and/or accelerated by previous banding of the main pulmonary trunk. To further define the relationship between banding of the pulmonary artery in patients with univentricular hearts and the development of subaortic stenosis, we examined the morphologic substrate and timing of the development of subaortic stenosis in 43 patients seen at our institution from January 1, 1970, through June 30, 1985. These 43 patients include all patients in this period with an unequivocal univentricular heart whose longitudinal data was available for follow-up. We excluded patients who died within 1 week of surgery, patients lost to follow-up, and patients with evidence of subaortic stenosis before banding. Thirty-one of 43 patients (72.1%) developed subaortic stenosis subsequent to banding of the main pulmonary artery. The mean age at banding of those patients who developed subaortic stenosis was 0.21 years, and subaortic stenosis was recognized at a mean age of 2.52 years. For the specific cohort of patients whose ventricular morphology was a main chamber of left ventricular type supporting the pulmonary artery and a rudimentary right ventricle supporting the transposed aorta (32 patients), 27 developed subaortic stenosis (84.4%). Subaortic stenosis in the classic form of single ventricle usually results from progressive restriction of a wholly muscular interventricular communication. Banding of the pulmonary artery by producing myocardial hypertrophy undoubtedly accelerates the potential for subaortic stenosis in these patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiomyopathy, Hypertrophic↗
PubMed1986

Pulsed Doppler echocardiographic assessment of the pulmonary venous pathway after the Mustard or Senning procedure for transposition of the great arteries.

Pulsed Doppler evaluation of pulmonary venous flow was performed in three groups of patients who had undergone either a Mustard or Senning procedure. Group I consisted of 43 patients in sinus rhythm who had undergone the Mustard procedure 9 months or more before the Doppler examination and 16 who were evaluated immediately after surgery. Group II consisted of 12 patients in sinus rhythm who had undergone the Senning procedure 9 months or more before the Doppler evaluation. Group III consisted of eight patients with mid baffle obstruction of the pulmonary venous atrium and seven with isolated stenosis of the pulmonary vein of which all but one had previously had associated mid baffle obstruction. In those without obstruction, the pulmonary venous flow pattern mirrored the left atrial pressure trace, with peak forward flow occurring during the x and y descent. Obstruction produced a specific high-velocity turbulent pattern, whether at the mid baffle or pulmonary venous level. This technique provides reliable noninvasive information about pulmonary venous flow after the Mustard or Senning procedure.

Child↗
PubMed1986

"Horseshoe" lung: report of five new cases.

A "horseshoe" lung is a rare congenital anomaly in which the posterobasal segments of the right and left lungs are fused behind the pericardial reflection at the cardiac apex. Most patients with such pulmonary fusion share many of those cardiovascular anomalies typical of the "scimitar" or hypogenetic right lung syndrome. The clinical diagnosis of horseshoe lung can be made by pulmonary angiography, which shows a branch of the right pulmonary artery originating from its proximal but inferior aspect and coursing into the left hemithorax. Thus, there are two conditions in which an artery from the right lung supplies part or all of the left lung: errant right pulmonary artery in the horseshoe lung deformity and so-called left pulmonary artery sling.

Angiography↗
PubMed1986

Congenital aortico-right atrial communications. The dilemma of differentiation from coronary-cameral fistula.

Four cases of an unusual form of aortico-right atrial communication are described. All patients were asymptomatic but had an atypical continuous murmur on examination. A distinctive appearance was noted on the angiograms, with a large tortuous tunnel noted superior to the left sinus of Valsalva, passing posterior to the aortic root before terminating near the right atrial-superior vena caval junction. This structure was readily identified by two-dimensional echocardiography. The defect was successfully closed surgically in three of four patients. The presence of normal major coronary arteries and absence of any small myocardial coronary branches from the tunnel argues against the structure being a coronary-cameral fistula and supports the diagnosis of aortico-right atrial tunnel.

Adolescent↗
PubMed1986

Double aortic arch with atretic right dorsal segment.

A patient in whom there was a double aortic arch with atresia of the right dorsal arch segment between the right subclavian artery and right ductus arteriosus is presented. The radiographic and aortographic features are demonstrated and the embryology is discussed.

Aorta, Thoracic↗
PubMed1985

Pulmonary valve morphology after balloon dilatation of pulmonary valve stenosis.

Percutaneous balloon valvuloplasty (BV) for pulmonic valve stenosis (PS) is increasingly becoming a nonsurgical alternative in patient management. However, the mechanism by which BV dilates the obstructive lesion has not been firmly established. We have had the opportunity to examine the effects of BV in the setting of PS and present two illustrative cases documenting the morphology after BV of the stenotic bicuspid and tricuspid pulmonary valve.

Adolescent↗
PubMed1985

The progressive nature of subaortic stenosis in congenital heart disease.

Data derived from serial hemodynamic and angiocardiographic investigations on pediatric patients not subjected to intervening intracardiac operations support the view that subaortic stenosis in congenital heart disease tends to be a progressive disorder. Our data are obtained from two groups of patients. The first comprised 22 patients with discrete subaortic stenosis in relative isolation. The second was made up of 19 patients with the fibrous or fibromuscular forms of discrete subaortic stenosis associated with a perimembranous ventricular septal defect. The results from both groups support our initial contention. The progressive character of subaortic stenosis in these two situations illustrates the dynamic nature of congenital heart disease, and the tendency of a changing form and function.

Angiocardiography↗
PubMed1985

A method of treating serous fluid leak from a polytetrafluoroethylene Blalock-Taussig shunt.

A 3 1/2-year-old girl with complex cyanotic congenital heart disease underwent palliation with a modified Blalock-Taussig shunt, for which a 6 mm polytetrafluoroethylene graft was used. Seroma formation with serous leakage occurred. Two periods of conservative management failed. At reoperation, treatment of the graft with intraluminal "fibrin glue" resulted in immediate resolution of the leak.

Blood Vessel Prosthesis↗