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Biomedical subjects

J M Pearl

Publications and source records attributed to J M Pearl.

At least 19 recordsLinked to original sources

Primum atrial septal defect.

UNLABELLED: The purpose of this report is to review our surgical experience with primum atrial septal defect. Since 1982, infants with primum atrial septal defect have undergone complete repair consisting of closure of the cleft of the left atrioventricular valve and atrial septal defect with a pericardial patch. Ages at operation ranged from early neonatal period until 5 years. In most patients, echocardiography was diagnostic and cardiac catheterization was performed in children with associated defects. Severe congestive heart failure and left atrioventricular valve regurgitation necessitated earlier correction. Infants with coarctation of the aorta and primum atrial septal defect underwent a two-stage procedure involving coarctation resection followed by complete repair. The early mortality rate is less than 1% and has a reoperation rate of less than 3%. The overall long-term survival of patients with primum atrial septal defect matches that of the general population. CONCLUSION: The diagnosis of primum atrial septal defect can easily be made by echocardiography with cardiac catheterization reserved for patients with associated left-sided obstruction. For patients in stable condition, the total repair can be performed before 2 to 3 years of age with minimum mortality. In infants with severe congestive heart failure, earlier correction should be contemplated, although it carries a higher morbidity. The associated coarctation of aorta is infrequent, but requires resection before intracardiac repair. The long-term results with this lesion repair are excellent.

Aortic Coarctation

Intermediate and complete forms of atrioventricular canal.

The surgical management of patients with complete atrioventricular canal has evolved over the past 20 years from a staged approach with preliminary pulmonary artery banding and eventual definitive repair, to an era of complete repair in early infancy. Although once considered high risk, the early mortality is now low and is primarily related to pulmonary hypertension secondary to a prolonged delay in diagnosis and referral. While controversy still exists over the techniques of repair, evaluation of outcome of atrioventricular canal should now be focused on the long-term function of the mitral valve, and the overall long-term survival. A detailed description of the single-patch technique and management of the atrioventricular valve, as well as an indepth discussion of outcome is presented.

Atrioventricular Node

Fumarate-enriched blood cardioplegia results in complete functional recovery of immature myocardium.

Amino acid enrichment of cardioplegic solutions has been shown to improve both the metabolic and functional recovery of ischemic myocardium. However, because of the marked systemic vasodilatation involved, use of amino acid enrichment is limited to the periods of induction and reperfusion. Fumarate is a Krebs' cycle intermediate whose conversion to succinate is responsible for the generation of adenosone triphosphate and the oxidation of the reduced form of nicotinamide-adenine nucleotide which is the pathway by which aspartate exerts its effect. Fumarate may also function as a free-radical scavenger and is involved in calcium transport. To determine if fumarate-enriched blood cardioplegia would improve the functional recovery of the neonatal heart, 14 neonatal piglet hearts were isolated and placed on a blood-perfused working heart circuit. After the baseline functional and metabolic assessment was done, cold ischemic arrest was initiated with either standard blood cardioplegic solution (group I; N = 7) or fumarate-enriched (13 mmol/L) blood cardioplegic solution (group II; N = 7). Cardioplegic solution was given at a pressure of 40 mm Hg every 20 minutes for 2 hours, and topical hypothermia was used. Sixty minutes after warm whole blood reperfusion, the functional recovery at left atrial pressures of 3, 6, 9, and 12 mm Hg was 70%, 66%, 66%, and 65%, respectively, in group I, versus 102%, 106%, 105%, and 109%, respectively, in group II (p < 0.05). The tissue creatinine phosphate levels after reperfusion were significantly higher in group II hearts (15.0 +/- 1.2 mumol/g dry heart tissue) than in group I hearts (9.2 +/- 1.9 mumol/g dry heart tissue), although the adenosine triphosphate levels were not significantly different.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenosine Triphosphate

Spontaneous closure of fenestrations in an interatrial Gore-Tex patch: application to the Fontan procedure.

The concept of the partial Fontan procedure, first described with the adjustable atrial septal defect (ASD) and more recently with the fenestrated technique, has become an accepted approach for the management of high-risk patients undergoing the Fontan procedure. Experience with both techniques has shown that a patent ASD placed in a prosthetic interatrial baffle may close spontaneously over a period of weeks to months. The mechanism and timing of spontaneous closure, as well as the effect of antiplatelet therapy on this process, are poorly understood. To better define this process, the interatrial septum of 15 mongrel dogs was excised and replaced with a fenestrated Gore-Tex (W.L. Gore, Flagstaff, AZ) patch. Postoperative echocardiography confirmed the patency of the ASD and left-to-right shunting. Animals were sacrificed 4 to 6 weeks postoperatively, or sooner if infection or other postoperative complications developed. Eight animals underwent no antiplatelet or anticoagulation therapy postoperatively, and 7 received antiplatelet therapy with aspirin. Patches were removed at the end of the study period and analyzed. By 6 weeks, all 2.7-mm and 4-mm holes had closed spontaneously in all animals that had not received antiplatelet therapy. The earliest closure occurred at 1 week. With antiplatelet therapy, hole closure was found to be delayed but not prevented, and was complete by 6 weeks in all but 1 animal. Histologic examination of the explanted patches revealed that closure was accomplished primarily through the ingrowth of fibrous tissue, accompanied by an inflammatory cell infiltrate.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals

Truncal valve repair: initial experience with infants and children.

The truncal valve regurgitation that frequently arises in patients with truncus arteriosus accounts for increased operative and late mortality. Five patients underwent truncal valve repair at UCLA Medical Center between August 1990 and September 1991. This group consisted of 2 infants who underwent complete repair and 3 who underwent valve repair together with right ventricle-pulmonary artery conduit replacement. The techniques used for repair were individualized according to the specific valve morphology, and consisted of the suturing of partially developed commissures, suspension of the cusps, resection of redundant portions of the cusps, annuloplasty at the commissures, and resection of excrescences on the surface of valve leaflets. In 1 infant who had a severely dysplastic truncal valve, stenosis and regurgitation recurred and progressed, and he died 4 months after truncal valve replacement. The remaining 4 patients, who were followed for from 8 to 21 months after repair, are in New York Heart Association class I, and have minimal or no aortic regurgitation. Except in patients with severely deformed and dysplastic valves, truncal valve repair can be an attractive and successful alternative to valve replacement.

Child

Loss of endothelium-dependent vasodilatation and nitric oxide release after myocardial protection with University of Wisconsin solution.

University of Wisconsin solution has proved to be a superior form of cardioplegia for cardiac transplantation, demonstrating better functional recovery than that provided by extracellular crystalloid solutions. Furthermore, experimental data have suggested a role for University of Wisconsin solution in protection of the neonatal heart during operations for congenital heart defects. However, significant concerns have been raised regarding potential endothelial injury from the high potassium concentration contained in University of Wisconsin solution that could affect its safety and thus its clinical application. Fourteen neonatal (aged 1 to 3 days) piglet hearts were harvested and supported on an isolated, blood-perfused circuit. Endothelium-dependent vasodilatation was measured by bradykinin (10(-6) mol/L) infusion and nitric oxide release was determined. Endothelium-independent vasodilatation was then induced by sodium nitroprusside (10(-6) mol/L) infusion. A 2-hour period of cold cardioplegic arrest was instituted with multidose University of Wisconsin solution (group 1, n = 7) or blood cardioplegia (group 2, n = 7). After reperfusion and stabilization, another stimulation with bradykinin and nitroprusside was carried out and nitric oxide was again measured. After 2 hours of arrest with University of Wisconsin solution, there was a near-complete loss of vasodilatation in response to bradykinin infusion; coronary blood flow reached 245% of baseline before arrest versus only 117% of baseline after arrest (p = 0.0011). This correlated with an inability of the endothelium to release nitric oxide (96 +/- 30 nmol/min before arrest versus -32 +/- 9 nmol/min after arrest, p = 0.0039. In group 2, the vasodilatory response to bradykinin was preserved after arrest and reperfusion; 265% of baseline before arrest versus 222% of baseline after arrest. These results demonstrate a loss of endothelium-dependent vasodilatation after multidose University of Wisconsin cardioplegia caused by the inability of the endothelium to release nitric oxide. In contrast, blood cardioplegia does not result in impaired endothelial function.

Adenosine

Perfluorochemical reperfusion yields improved myocardial recovery after global ischemia.

Reperfusion injury remains a limiting factor in extending ischemic storage time for human heart transplantation. In this study, initial myocardial reperfusion with an oxygenated perfluorochemical (Fluosol) was investigated as a means of limiting such injury. Neonatal piglet hearts were arrested with crystalloid cardioplegia, excised, and stored for 12 hours in saline solution at 0 degrees C. Initial reperfusion (10 minutes) was either with whole blood (n = 6), unmodified perfluorochemical (n = 8), or aspartate/glutamate-enriched perfluorochemical cardioplegia (n = 6), and was followed by an additional 40 minutes of whole blood perfusion. Functional evaluation was then completed, and left ventricular biopsy specimens were taken. A control group (n = 7) was evaluated without an intervening period of ischemia. At a left ventricular end-diastolic pressure of 9 mm Hg, hearts stored in whole blood cardioplegia developed a left-ventricular stroke work index of 3.8 +/- 2.3 x 10(3) erg/g (mean +/- standard error of the mean). Under the same conditions, perfluorochemical-reperfused hearts achieved a stroke work index of 14.6 +/- 1.3 x 10(3) erg/g, significantly greater than that of the whole blood group (p < 0.001). Stroke work index for hearts reperfused with aspartate/glutamate-enriched perfluorochemical cardioplegia was 19.8 +/- 1.6 x 10(3) erg/g, significantly increased over that of the nonenriched perfluorochemical group (p < 0.01) and not different from values obtained in controls (19.2 +/- 0.8 x 10(3) erg/g).(ABSTRACT TRUNCATED AT 250 WORDS)

Adenosine Triphosphate

Girdling effect of nonstimulated cardiomyoplasty on left ventricular function.

The precise hemodynamic effects of latissimus dorsi cardiomyoplasty have not been well characterized. We prospectively studied 11 mongrel dogs using a rapid ventricular pacing model of congestive heart failure. Six dogs received a nonstimulated left latissimus dorsi cardiomyoplasty wrap, and 5 control dogs were paced only. Two-dimensional transthoracic echocardiography was performed on all dogs at baseline and then weekly for 4 weeks. Measurements obtained included left ventricular diameters, lengths, volumes, and ejection fractions. Progressive left ventricular enlargement, increase in volumes, and worsening ejection fractions developed in both groups. However, less left ventricular dilatation and higher ejection fractions were seen in dogs that received a cardiomyoplasty wrap. A nonstimulated cardiomyoplasty wrap significantly attenuated the degree of left ventricular enlargement, increase in left ventricular volumes, and decrease in ejection fraction in a rapid pacing model of congestive heart failure. Apart from its effect on systolic augmentation with a stimulated muscle wrap, cardiomyoplasty may have an important "girdling" effect on the left ventricle that prevents dilatation and deterioration of left ventricular function.

Animals

Damus-Stansel-Kaye procedure: current indications and results.

Between October 1983 and August 1991, 29 consecutive Damus-Stansel-Kaye procedures were performed. Indications for operation included restrictive bulboventricular foramen or subaortic stenosis associated with complex univentricular congenital heart disease (25) and Taussig-Bing heart, subaortic stenosis, or both associated with complex biventricular congenital heart disease (4). Twelve patients underwent concurrent Fontan procedures. Average age at operation was 39.8 months (range, 1 to 132 months). Average outflow tract gradient was 28 mm Hg (range, dynamic to 80 mm Hg). Of the 29 patients, 23 were male and 6 were female. There were three early deaths (10%), two in patients who had a concurrent Fontan procedure. Although there was a trend toward lower age and higher outflow tract gradients in nonsurvivors, these and other factors were not statistically significant predictors of death. Actuarial freedom from cardiac-related death was 88% at 5 years (n = 7). In a mean follow-up of 3.5 years (range, 0.1 to 7.7 years), 3 patients have required reoperation (10%), 2 for aortic valve insufficiency (5 days and 2.75 years) and 1 for a gradient across the anastomosis (5.75 years). Actuarial freedom from reoperation related to a failed Damus-Stansel-Kaye procedure was 90% at 4 years and 75% at 6 years (n = 7).

Actuarial Analysis

Gross and microvascular distribution of retrograde cardioplegia in explanted human hearts.

In this report, explanted hearts from transplant recipients with the diagnosis of idiopathic cardiomyopathy underwent a blood cardioplegia arrest and extended subatrial resection to preserve their coronary sinus venous system. The coronary sinus and left and right coronary arteries were then cannulated and warm blood cardioplegia retrograde infused at a pressure of 30 to 40 mm Hg. Effluent from the coronary arteries and thebesian veins was then collected. Hearts were subsequently fixed with retrograde glutaraldehyde perfusion and perfused retrograde with NTB-2 (an inert intracapillary marker). Histologic sections were examined from 12 separate sites. There was no significant difference in the percentage of capillaries perfused by retrograde-delivered cardioplegia between corresponding regions of the left and right ventricles. However, effluent analysis indicated that 67.2% +/- 6.4% of retrograde-delivered blood cardioplegia was shunted through thebesian veins, thereby bypassing the microvasculature, whereas 29.3% +/- 6.3% and 3.5% +/- 3.1% traversed the myocardium supplied by the left and right coronary arteries, respectively. The results indicate that all regions of both ventricles are perfused by retrograde blood cardioplegia. However, they also suggest that nutrient flow to the microvasculature of the right ventricle is minimal during retrograde cardioplegia.

Blood

Aortic valve replacement using a continuous suture technique.

The continuous suture technique has been proposed as an alternate method for aortic valve replacement (AVR). Advantages include a decreased ischemic and bypass time. Despite reports of a low incidence of perivalvular leak, wide use of the continuous suture technique has not been adopted. This report reviews our experience with the continuous suture technique. From January 1984 through November 1991, 181 consecutive patients underwent AVR using the continuous suture technique. The mean age was 61 years (range 6 to 88 years). Diagnoses included pure aortic stenosis (AS) in 41%, aortic insufficiency (AI) in 31%, and a combination of AS and AI in 28%. Fifty-six patients underwent isolated AVR and 125 underwent AVR combined with other procedures. The overall early mortality was 5.5%. Early mortality for isolated AVR was 0% (0/56) and was 8.0% (10/125) for those undergoing concomitant procedures. Late mortality was 4.7% in a mean follow-up of 30 months (range 1 to 86 months). The incidence of perivalvular leak was 2.3% (4/171 operative survivors). Perivalvular leak was mild in two, and moderate in two; none required reoperation. Perivalvular leak developed only in patients whose suture line was not reinforced with glutaraldehyde treated pericardium. The continuous suture technique is a quick and effective method for AVR and results in a low incidence of perivalvular leak.

Adolescent

Normocalcemic blood or crystalloid cardioplegia provides better neonatal myocardial protection than does low-calcium cardioplegia.

Although standard blood cardioplegia provides good myocardial protection for cardiac operations in adults, protection of the cyanotic, immature myocardium remains suboptimal. Calcium, which has been implicated in reperfusion injury and in the development of "stone heart" in mature myocardium, is routinely lowered in standard cardioplegic solutions. Immature, neonatal myocardium has lower intracellular calcium stores and is more reliant on extracellular calcium for contraction. To determine if normocalcemic cardioplegia would result in improved cardiac function in the neonatal heart, we conducted a series of experiments using an isolated, blood-perfused working heart model. Thirty-two neonatal piglet hearts (24 to 48 hours) were excised without intervening ischemia and were placed directly on a blood-perfused circuit. Baseline stroke work index was assessed. Hearts were then arrested with cold cardioplegic solution delivered at 45 mm Hg for 2 minutes: group I, low-calcium blood cardioplegic solution (Ca = 0.6 mmol/L); group II, normal-calcium blood cardioplegic solution (Ca = 1.1 mmol/L); group III, University of Wisconsin solution; and group IV, University of Wisconsin solution with added calcium (Ca = 1.0 mmol/L). Cardioplegic solution was administered every 20 minutes for 2 hours and topical hypothermia was used. Hearts were then reperfused with warm whole blood. Functional recovery, expressed as a percentage of control stroke work index, was determined minutes after reperfusion. Hearts preserved with normocalcemic cardioplegic solution (groups II and IV) had complete functional recovery at 60 minutes, whereas hearts preserved with low-calcium cardioplegic solution (groups I and III) achieved functional recoveries of only 80% and 65%, respectively, at a left atrial pressure of 9 mm Hg. Electron micrographs taken 1 hour after reperfusion showed minimal edema and only mild myofibrillar changes. They were identical in both the low-calcium and normocalcemic groups. Complete functional recovery is possible in immature myocardium when calcium is added to either blood or an intracellular crystalloid cardioplegic solution. The addition of calcium does not result in ultrastructural damage and does result in good functional recovery.

Adenosine Triphosphate

Fluosol cardioplegia results in complete functional recovery: a comparison with blood cardioplegia.

Blood cardioplegia is considered by many to be the preferred solution for myocardial protection. Proposed benefits include the ability to deliver oxygen and the ability to maintain metabolic substrate stores. However, the decreased capacity of blood to release oxygen at hypothermic conditions as well as the presence of deleterious leukocytes, platelets, and complement may limit complete functional recovery. Fluosol is an asanguineous solution with the ability to bind and release oxygen linearly at low temperatures. Neonatal piglet hearts (24 to 48 hours old) were excised and supported on an isolated, blood-perfused working heart model. After baseline stroke-work index was determined, hearts were arrested with either normocalcemic blood cardioplegia (group 1, n = 8) or normocalcemic Fluosol cardioplegia (group 2, n = 8). Cold cardioplegia was administered at 45 mm Hg every 20 minutes for 2 hours. Hearts were then reperfused with whole blood. Functional recovery, expressed as percent of control stroke-work index, was determined 60 minutes after reperfusion at left atrial pressures of 3, 6, 9, and 12 mm Hg. Functional recovery at 60 minutes was similar between group 1 (95%, 93%, 93%, 88%) and group 2 (100%, 94%, 94%, 95%) at left atrial pressures of 3, 6, 9, and 12 mm Hg, respectively. Mean lactate consumption 5 minutes after reperfusion was significantly greater (p = 0.0001) in group 1 (31.8 +/- 6.3 micrograms.min-1 x g-1) than in group 2 (-0.59 +/- 0.1 microgram.min-1 x g-1), indicating superior metabolic recovery in the blood cardioplegia hearts. Edema formation, as determined both by water content (group 1, 81.10%; group 2, 81.63%) and by electron microscopy, was not significantly different between groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenosine Triphosphate

Damus-Stansel-Kaye procedure: technical modifications.

The Damus-Stansel-Kaye procedure has been applied for the relief of outflow tract obstruction caused by a restrictive bulboventricular foramen or subaortic stenosis in patients with complex univentricular heart disease. The procedure may also be part of a biventricular repair of a Taussig-Bing transposition of the great arteries. This report details technical modifications of the procedure to ensure unobstructed blood flow from the pulmonary artery to the aorta and to maintain the integrity of the pulmonary and aortic valves.

Anastomosis, Surgical

Leukocyte-depleted reperfusion of transplanted human hearts prevents ultrastructural evidence of reperfusion injury.

The present study examines whether leukocyte depletion can prevent postreperfusion ultrastructural injury in transplanted human hearts. Thirty-two patients undergoing orthotopic cardiac transplantation were randomized to receive either enriched, warm, whole blood (Group I; n = 16) or enriched, warm, leukocyte-depleted blood (Group II; n = 16) reperfusion. Donor hearts were arrested with 1 liter of 4 degrees C crystalloid cardioplegia and topically cooled. RV endomyocardial biopsies taken at end-ischemia and following reperfusion were assessed in a blinded fashion and graded according to injury (1 = minimal to 4 = severe). The mean ischemic time (Group I = 142 min, Group II = 153 min) was similar in the two groups. End-ischemic biopsies showed mild-moderate interstitial edema and mild capillary endothelial swelling in both groups with similar injury scores (Group 1 = 1.3 +/- 0.09 (means +/- SEM), Group 2 = 1.25 +/- 0.08). Postreperfusion biopsies in Group I showed nuclear chromatin clumping, moderate mitochondrial swelling, marked capillary endothelial swelling, and marked interstitial edema with a grade of 2.6 +/- 0.14 (P less than 0.001, paired t test). In contrast, postreperfusion biopsies in Group II showed minimal changes with a grade of 1.33 +/- 0.09, P less than 0.0001 in comparison to Group I Leukocyte-depleted reperfusion of human transplanted hearts prevents ultrastructural injury. This may allow safe extension of the ischemic period and result in improved graft function.

Biopsy