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Biomedical subjects

L W Perry

Publications and source records attributed to L W Perry.

At least 37 records · Page 2Linked to original sources
PubMed1984

Repair of anomalous origin of the left coronary artery in the infant and small child.

Anomalous origin of the left coronary artery from the pulmonary artery is associated with myocardial infarction, left ventricular dysfunction, mitral valve dysfunction and, occasionally, intracardiac congenital abnormalities. A technique that utilizes a flap of the anterior wall of the pulmonary artery to serve as a neocoronary artery to direct aortic flow from a created aortopulmonary window to the pulmonary artery orifice of the anomalous left coronary artery was used in five patients aged 2.5 months to 4.75 years. Two patients were less than 4 months of age at operation. There was one death 2 days after operation and one late death. The two youngest patients required mitral valve replacement. Two of the three surviving patients are well at follow-up at 7 to 44 months. One patient has been lost to follow-up study. One patient had postoperative catheterization which showed an intact repair. The pulmonary artery neocoronary procedure is applicable to infants and small patients with anomalous origin of the left coronary artery from the pulmonary artery.

Child, Preschool↗
PubMed1984

Hematologic disorders and congenital cardiovascular malformations: converging lines of research.

In a population-based study on congenital cardiovascular malformations (CCVM), the occurrence of heritable coagulopathies among case parents and not among controls raised the possibility of an etiologic association of CCVM with blood disorders. The literature was searched for evidence that such an association could be biologically plausible. Reported embryologic and clinical data provided confirmatory findings. The heart and blood arise from common angiogenic cells; endothelial cells, the first components of the primitive heart, synthesize coagulation factors; resultant osmotic alterations of embryonic fluids could alter early cardiac morphogenesis. Bleeding diatheses are common in cyanotic and acyanotic patients with CCVM and hemostatic disorders have been reported in some families. CCVM and blood disorders are joint components of several malformation syndromes. The hypothesis of an etiologic relationship between HBD and CCVM needs to be tested in multiple research areas. Future experimental studies should be based on current theories of cardiac morphogenesis to include investigations of embryonic blood in genetic blood disorders. Clinical studies should clarify hematologic alterations in CCVM probands and their families.

Anemia↗
PubMed1983

Left ventricular false tendons in children: prevalence as detected by 2-dimensional echocardiography and clinical significance.

During a 28-month period, consecutive 2-dimensional echocardiograms were reviewed to determine the prevalence of left ventricular (LV) false tendons, their associated anomalies and clinical significance. LV false tendons were found in 31 (0.8%) of 3,847 consecutive 2-dimensional echocardiograms. Of 31 LV false tendons, 30 passed longitudinally from papillary muscle to septum and 1 went from free wall to free wall. The 31 patients were aged 1 day to 15 years. Associated heart disease, most often ventricular septal defect, bicuspid aortic valve and coarctation of the aorta, was present in 48%, of whom 73% were girls. Of those without heart disease, 69% were boys. In patients with heart disease, precordial murmurs were due to the underlying cardiac anomaly. Of those without heart disease, 15 of 16 (94%) had a precordial murmur, usually of the Still's type over the lower left sternal border. Four of 31 (13%), 1 with and 3 without heart disease, had unifocal premature ventricular contractions that were rate-dependent in the 2 patients undergoing stress testing. LV false tendons appear to occur in 0.8% of pediatric patients and usually are accompanied by a Still's type innocent murmur if unassociated with heart disease. Some LV false tendons are associated with rate-dependent premature ventricular contractions.

Adolescent↗
PubMed1983

Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet.

Fraternal twins who had fasting hypoglycemia, hypoketonemia, muscle weakness, and hepatic dysfunction are reported. The hepatic dysfunction occurred only during periods of caloric deprivation. The surviving patient developed a cardiomyopathy. In this sibling, muscle weakness and cardiomyopathy were markedly improved by a diet high in medium chain triglycerides. There was a marked deficiency of muscle total carnitine and a mild deficiency of hepatic total carnitine. Unlike patients with systemic carnitine deficiency, serum and muscle long-chain acylcarnitine were elevated and renal reabsorption of carnitine was normal. It was postulated that the defect in long-chain fatty acid oxidation in this disorder is caused by an abnormality in the mitochondrial acylcarnitine transport. Detailed studies of the cause of the hypoglycemia revealed that insulin, growth hormone, cortisol, and glucagon secretion were appropriate and that it is unlikely that there was a major deficiency of a glycolytic or gluconeogenic enzyme. Glucose production and alanine conversion to glucose were in the low normal range when compared to normal children in the postabsorptive state. The hypoglycemia in our patients was probably due to a modest increase in glucose consumption, secondary to the decreased oxidation of fatty acids and ketones, alternate fuels which spare glucose utilization, plus a modest decrease in hepatic glucose production secondary to decreased available hepatic energy substrates.

Cardiomyopathies↗
PubMed1982

Early failures of Ionescu-Shiley bioprosthesis after mitral valve replacement in children.

Two infants, 101/2 and 11 1/2 months of age, underwent mitral valve replacement with Ionescu-Shiley bovine bioprosthesis for congenital cardiac defects. Both patients had early valve failure, 19 months and 4 months after implantation, due to the growth of excessive collagen on the ventricular surface of the bioprosthesis with adherence of the collagen primarily to the sewing rings and struts of the valves. The cause of this excessive collagen reaction is unknown. Further study is needed to document the true incidence of this problem.

Bioprosthesis↗
PubMed1982

Echocardiographically assisted balloon atrial septostomy.

Balloon atrial septostomy is an accepted method for palliation of certain types of congenital heart disease. However, malposition of the balloon may lead to cardiac perforation, avulsion of an atrioventricular valve, or laceration of the systemic or pulmonary veins. Inasmuch as single-phase fluoroscopy may not identify balloon position correctly and as biplane fluoroscopy adds significant radiation exposure, two-dimensional echocardiography has been used to assist in balloon atrial septostomy in ten infants. The catheter is advanced from the inferior vena cava to the right atrium across the foramen ovale to the left atrium with the echo transducer in the subxiphoid position. The balloon is inflated and its position within the left atrium is confirmed by echo. The catheter is withdrawn according to the technique of Rashkind. Withdrawal is halted when the balloon traverses the atrial septum. Adequate septostomy is indicated on echo by a defect at least 5 mm in diameter and by flapping of the inferior rim of the atrial septum. There were no complications using this technique and a clinically adequate septostomy was achieved in each patient. Two-dimensional echocardiography-assisted balloon atrial septostomy minimizes risk of complications and decreases exposure to ionizing radiation.

Cardiac Catheterization↗
PubMed1981

Two-dimensional echocardiographic evaluation of mitral bioprosthetic function in infants and children.

Two-dimensional echocardiography (2DE) was performed on 13 bioprosthetic mitral valves in 11 pediatric patients, 1 week to 46 months following implantation. Cardiac catheterization confirmed the echocardiographic findings of seven valves. The 11 normally functional bioprosthetic mitral valves by 2DE had leaflet thickness not exceeding 2.0 mm, freely moving leaflets, and normal sewing ring motion. The left atrium (LA) was enlarged only in the presence of mitral valve obstruction, atrial fibrillation, or left ventricular (LV) dysfunction. One bovine valve was stenotic due to fibrous tissue beneath normal valve leaflets; 2DE demonstrated limited leaflet motion, tissue beneath a thin valve leaflet, increase rocking motion of the sewing ring, and LA enlargement. In another patient, a bovine valve produced subaortic LV obstruction due to growth of fibrous tissue outside the stent; 2DE showed that the valve leaflets were thin and moved normally without excessive rocking of the valve ring, and identified the abnormal fibrous tissue in the LV outflow tract.

Adolescent↗
PubMed1981

Specificity of systolic anterior motion of anterior mitral leaflet for hypertrophic cardiomyopathy. Prevalence in large population of patients with other cardiac diseases.

The value of systolic anterior motion of the anterior mitral leaflet as a diagnostic marker for hypertrophic cardiomyopathy has been questioned because of its reported occurrence in other heart diseases. To determine the true specificity of systolic anterior motion for hypertrophic cardiomyopathy, 721 echocardiograms from patients with a wide variety of cardiac diseases were reviewed for its presence or absence under basal conditions. Systolic anterior motion of the anterior mitral leaflet was present in only 22 (3.0%) of the 721 patients, giving a specificity of 97 per cent. It was most common in patients with d-transposition of the great vessels (11 of 51, or 21%). With patients having transposition of the great vessels excluded from the analysis, the prevalence of systolic anterior motion of the anterior mitral leaflet was only 1.6 per cent (specificity 98%). Of note, eight of the 11 patients with systolic anterior motion of the anterior mitral leaflet and diseases other than transposition of the great vessels had disproportionate thickening of the ventricular septum, making it exceedingly rare in a patient population with normal septal-free wall thickness ratios (prevalence 0.4%; specificity 99%). Hence, while systolic anterior motion is not pathognomonic of hypertrophic cardiomyopathy, it was an uncommon finding in a large population of patients with a variety of cardiac diseases; when present in such patients systolic anterior motion of the anterior mitral leaflet is usually associated with disproportionate septal thickening.

Adolescent↗
PubMed1981

Two-dimensional echocardiography for catheter location and placement in infants and children.

This paper presents our experience with two-dimensional echocardiography in the detection of 61 intravascular catheters or wires in a series of 39 infants and children. It was possible to identify intravascular wires as small as 0.5 mm in diameter and intravascular catheters as small as 0.9 mm in diameter. In no instance, was an intravascular catheter not detected. The two-dimensional echocardiogram is demonstrated to be a tool to assist the physician in placement of intravascular catheters. In the catheterization laboratory, the two-dimensional echocardiogram is useful in determining catheter location, particularly in the presence of complex congenital defects. Two-dimensional echocardiographic guidance of catheters minimizes exposure to ionizing radiation.

Cardiac Catheterization↗
PubMed1981

Mitral valve replacement in infants and children.

Thirteen patients, ranging in age from 10 months to 19 years (mean 7.8 years) and in weight from 6.6 to 60 kg (average 29.5 kg) underwent 14 operations for mitral valve replacement with a heterograft prosthesis between January 1, 1976 and July 1, 1979 for a variety of congenital or acquired lesions. Preoperative indications included severe refractory congestive heart failure in each patient with growth retardation, which was especially prominent in the younger patients. Operative mortality was 14% (2/14) with both deaths occurring within 48 hours of operation in patients less than 6 years of age. All surgical survivors had clinical improvement as manifested by relief of symptoms, decrease in heart size, and significant growth. Routine postoperative catheterization in five patients revealed good initial postoperative results in those studied, with one patient having a second study 20 months after operation. He was found to have had degeneration of his bovine prosthesis and had subsequent successful reoperation with a porcine prosthesis. Further long-term serial catheterizations are needed to further document the history of heterograft prosthesis in children, but they are preferred to mechanical valves became of the lack of need for long-term anticoagulants and the absence of thromboembolism complications. This series suggests that mitral valve replacement, when indicated by refractory congestive heart failure and growth retardation, can be successfully performed even in infants and small children. Surgery should not be postponed to allow for subsequent patient growth if the natural history of the disease is of progression.

Adolescent↗
PubMed1980

Experience with repair of complete atrioventricular canal.

Twenty-nine patients at out institution have undergone repair of complete atrioventricular canal since 1969. There were 3 operative and 5 late deaths, 4 of which were of infectious etiology. Age at operation ranged from 2 months to 12 years (mean, 50 months). Weight ranged from 3.6 kg to 30 kg (mean, 12 kg). Before repair, catheterization studies revealed pulmonary hypertension in all patients with unobstructed pulmonary arteries. Pulmonary to systemic flow ratio ranged from 1.5 to 10.6 (mean, 3.5). Pulmonary vascular resistance (PVR) ranged from 0.7 to 21.7 (mean, 5.3) Wood units/m2. At repair, 14 patients had Rastelli type A anatomy, 14 had type C, and 1 patient had a variant with crossing chordae and double-outlet right ventricle (DORV). Postoperative catheterization has been done in 16 patients 2 months to 8 years (mean, 30 months) after repair. One patient had residual ventricular shunting and later underwent successful repair. A 2-year-old patient had severe mitral regurgitation and died following mitral valve replacement. One patient required a permanent pacemaker. The 21 surviving patients have been followed from 7 months to 10 years 7 months and have excellent hemodynamic status. Long-term studies are needed to assess the ultimate effect on patients with high PVR.

Cardiac Catheterization↗
PubMed1978

Cerebrovascular abnormalities in postoperative coarctation of aorta. Four cases demonstrating left subclavian steal on aortography.

Fifteen children, aged 0.3 to 10.5 years (mean 3.8 years) after repair of coarctation of the aorta, underwent cerebral arteriography as part of postoperative catheterization. Four manifested central nervous system symptoms postoperative catheterization. Four manifested central nervous system symptoms postoperatively: Two had persistent headaches, one had exercise-induced hemianopsia and one had major motor seizures. All four had greater blood pressure in the right arm than in the left and evidence of subclavian steal, with retrograde filling of the left vertebral and left subclavian arteries on selective right vertebral arteriography. Ligation of the left vertebral artery in three patients and left subclavian graft arterioplasty in one resulted in disappearance of symptoms. None of the 11 asymptomatic patients manifested cerebrovascular anomalies, and no patient in the series had berry aneurysm. This study suggests that patients with central nervous system symptoms and a disparity of blood pressure in the arms after surgery for coarctation of the aorta should be evaluated carefully to exclude subclavian steal as the cause of the symptoms.

Aortic Coarctation↗