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Biomedical subjects

A J Razzouk

Publications and source records attributed to A J Razzouk.

At least 55 records · Page 3Linked to original sources

Bless the babies: one hundred fifteen late survivors of heart transplantation during the first year of life. The Loma Linda University Pediatric Heart Transplant Group.

There is a rapid growth of interest in heart transplantation therapy during early infancy. From 10% to 25% of the infants who are listed for transplantation annually have died while awaiting a donor heart. There has been no significant trend in this variable. Since November 1985, 140 consecutive orthotopic transplantation procedures were performed in 139 infants who were from 3 hours to 12 months of age. Indications for transplantation included hypoplastic left heart syndrome (63%), other complex structural anomalies (29%), myopathy (6.5%), and tumors (1.5%). Most recipients had ductus-dependent circulation and received continuous infusion of prostaglandin E1. Heart donors were usually victims of trauma, sudden infant death, or birth asphyxia. A donor-recipient weight ratio of 4.0 or less was found to be acceptable. The amount of time the graft underwent cold ischemia, ranged from 64 to 576 minutes. The procurement process was facilitated by a single dose of cold crystalloid cardioplegic solution and cold immersion transport. Profound hypothermic circulatory arrest was used for graft implantation. One hundred twenty-four (89%) recipients survived transplantation and were discharged from the hospital. There were 9 late deaths, which resulted in an 83% overall survival. The 5-year actuarial survival is 80%. The survival among newborn recipients (n = 60) at 5 years is 84%. Chronic immunomodulation was cyclosporine-based and steroid-free. Surveillance was noninvasive and relied heavily on echocardiography, electrocardiography, and clinical intuition. There was one documented late lethal infection, tumor was not encountered, and coronary occlusive disease was known to exist in only one long-term survivor. We concluded that transplantation results in excellent life quality and is a highly effective and durable therapy when applied during early infancy.

Actuarial Analysis↗

Neonatal cardiac transplantation. Intermediate-term results and incidence of rejection. Loma Linda University Pediatric Heart Transplant Group.

Early age at cardiac transplantation may favor successful engrafting with minimal chronic immunosuppression. Fifty-two newborns underwent orthotopic heart transplantation; 47 (90%) survived the operation, and 44 (85%) were late survivors. Actuarial survival was 92% at 1 month, 86% at 1 year, and 84% at 5 years. Forty-four infants who survived 12 weeks and the corresponding 100 rejection episodes were analyzed. Mean follow-up was 2.2 years. The mean number of rejections per year of follow-up was 1.2. No episodes of rejection were identified in six patients. Seven patients had a late rejection episode more than 1 year after transplantation, and only one had a late rejection episode after 2 years. Neonatal cardiac transplantation is effective and durable therapy for uncorrectable heart disease. Intermediate-term results are excellent. Severe rejection is uncommon, and few episodes occur after 1 year of follow-up.

Azathioprine↗

Surgically created Wolff-Parkinson-White syndrome after Fontan operation.

The Wolff-Parkinson-White syndrome is caused by a congenital accessory connection between the atrium and ventricle. We describe a case of symptomatic Wolff-Parkinson-White syndrome that arose after a Björk modification of the Fontan operation. Invasive electrophysiologic and intraoperative mapping indicated that the surgically created atrioventricular connection was functioning as an accessory pathway. Surgical dissection and cryoablation abolished the symptoms and the preexcitation.

Adolescent↗

Primary repair of traumatic aortic rupture: a preferred approach.

Eighty patients with traumatic rupture of the thoracic aorta were treated. Seven patients died during the initial resuscitation. Forty-three patients underwent surgical repair using the clamp-and-sew technique; 14 patients had a heparin-bonded shunt placed, and 16 patients were repaired using cardiopulmonary bypass. An interposition Dacron graft was used in only 19 patients (26%). The last 32 consecutive patients underwent primary repair of the ruptured aorta. Overall mortality was 19.2% (14 of 80); 9 of 14 patients (64%) had laparotomies along with the aortic repair, and 13 of 14 patients (92%) had three or more associated major injuries. Paraplegia occurred in four cases (5.6%). Traumatic aortic rupture remains a difficult surgical problem. Primary repair, without graft interposition, is the preferred technique and can be accomplished even when the two aortic ends have retracted several centimeters.

Adolescent↗

The recognition, identification of morphologic substrate, and treatment of subaortic stenosis after a Fontan operation. An analysis of twelve patients.

Twelve children were identified with subaortic stenosis after Fontan's operation. All had absent resting and isoproterenol-provoked pressure gradient before the Fontan procedure. Six had a univentricular heart of left ventricular morphology, three had a single ventricle of right ventricular morphology, one had tricuspid atresia with transposition of the great arteries, one had pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle, and one had corrected transposition with hypoplastic systemic ventricle. The median interval between the Fontan operation and the recognition of subaortic stenosis was 2.5 years. Ten patients underwent surgical treatment after a prior Fontan operation: Five had myectomy and enlargement of ventricular septal defect with two operative deaths; two had placement of a valved conduit from the ventricular apex to the descending aorta, and both died postoperatively; two with single ventricle had subaortic myectomy, and one had enlargement of ventricular septal defect and pulmonary aortic connection. Complete heart block developed in only one patient. Postoperative testing with Doppler echocardiography with color flow imaging demonstrated good relief of subaortic stenosis. All six children who survived the operation are well 4 months to 4 years later. Subaortic stenosis is a progressive lesion that may develop after a Fontan operation. Its surgical treatment continues to carry a significant mortality. Myectomy and enlargement of ventricular septal defect achieve direct relief of the obstruction with minimal risk of heart block.

Adolescent↗

Failure of cryopreserved homograft valved conduits in the pulmonary circulation.

BACKGROUND: This study evaluates our experience with the cryopreserved homograft valved conduit used for reconstruction of the pulmonary circulation in patients with congenital heart disease. METHODS AND RESULTS: Between July 1, 1985, and December 31, 1990, 219 patients had cryopreserved homograft extracardiac valved conduits placed in the pulmonary circuit. Average age at operation was 7.2 years. Of these, 132 patients had a pulmonary homograft, and 87 had an aortic homograft. Twenty-four patients (11%) died in hospital. Hospital survivors (n = 195) have been followed an average of 29.8 months (SD, +/- 18.4 months). Fourteen patients died during follow-up, almost all related to the complexity of their original cardiac malformation. Thirty-two patients (15%) have required reoperation for conduit-related problems. Actuarial freedom from conduit reoperation is 55 +/- 12% at 5 years. The most common indication for reoperation was calcific stenosis (n = 27). Other indications for reoperation were pseudoaneurysm (n = 2), conduit infection (n = 2), and pulmonary insufficiency (n = 1). Reoperation rate for patients with aortic homografts (16 of 87) compared with that for pulmonary homografts (16 of 132) was not significantly different by the actuarial method. CONCLUSIONS: Long-term function of cryopreserved homograft valved conduits in the pulmonary circulation is disappointing.

Actuarial Analysis↗

Surgical connections from ventricle to pulmonary artery. Comparison of four types of valved implants.

BACKGROUND: Four types of valved conduits used to correct venous ventricle to pulmonary artery (V-PA) discontinuity were compared. METHODS AND RESULTS: Four hundred fifty-seven patients with congenital heart defects requiring a V-PA connection during the past 25 years were reviewed. Age at implant varied from 1 day to 64 years (mean, 9.1 years). Four types of valved prostheses were used: 1) homograft conduit (HC, n = 178), 2) valved Dacron conduit (VDC, n = 126), 3) polystan conduit (PC, n = 47), and 4) orthotopic pulmonary valve implant (PVI, n = 106). There were 83 early deaths (18.2%) and 34 late deaths (8.5%). Follow-up ranged from 1 month to 22 years (mean, 3.5 years). One hundred eight conduit replacements were performed in 93 patients (21%). The overall patient survival was 73 +/- 2.3%, 67 +/- 3.2%, and 56 +/- 6.8% at 5, 10, and 15 years, respectively. Factors predictive of patient survival were diagnosis (p < 0.001) and valve size (p < 0.001). Age at operation (p < 0.001) and type of valve (p < 0.001) were the only risk factors for valve survival. At 5 years, survival of PVI (89 +/- 5%) and VDC (89 +/- 4%) was significantly better than survival of HC (46 +/- 13%) or PC (57 +/- 9%). CONCLUSIONS: Patients who survived the initial construction of a V-PA conduit had a reasonable long-term survival. A PVI was the most durable prosthesis. A Dacron porcine-valved conduit had significantly better durability than either a cryopreserved homograft or a PC.

Actuarial Analysis↗

The Mustard procedure for correction of simple transposition of the great arteries before 1 month of age.

Since April 1976, 34 infants (25 male and 9 female) less than 1 month of age underwent a Mustard intraatrial baffle procedure for repair of simple transposition of the great arteries. Thirty patients were less than 2 weeks old and 19 patients less than 1 week (mean 7.8 +/- 6 days). The weights ranged from 2.6 to 4.4 kg (mean 3.4 +/- 0.4 kg). Rashkind balloon atrial septostomy was performed in the first hours or days of life in 29 patients. The average interval from balloon atrial septostomy to baffle repair was 3.9 days (range 2 hours to 14 days). Mechanical ventilation was required in eight patients preoperatively and prostaglandin E1 was infused in 17 patients to maintain ductal patency. In all patients, the Mustard procedure was performed with the use of deep hypothermic circulatory arrest, averaging 53 minutes (range 37 to 82 minutes). The duration of postoperative intubation and ventilatory support averaged 1.7 +/- 1.0 days (range 1 to 5 days). Inotropic drugs were used in 24 patients during a period of 1.4 +/- 1.3 days (range 1 to 6 days) postoperatively. There were no hospital deaths. Follow-up evaluation has extended from 1 month to 14 years (mean 3 +/- 3 years). One infant died 2 months postoperatively as a result of milk aspiration; no cardiac defects were found at the autopsy. A second infant died at 1 year with right ventricular and tricuspid valve dysfunction. Baffle complications occurred in 6 of the 32 survivors, including superior vena caval stenosis in 4, inferior vena caval stenosis in 1, and pulmonary venous obstruction in 3. Reoperations for baffle obstructions were performed in three patients (8.8%) and balloon angioplasties in two. One patient required permanent pacemaker implantation. Results with the Mustard procedure before 1 month of age show that it can be performed with negligible mortality and a low incidence of late complications at an age comparable to when arterial switching would be performed. Until long-term studies demonstrate superiority of arterial operations, the low operative mortality favors continued evaluation of the neonatal Mustard repair as a valid alternative to the arterial switch.

Cardiac Surgical Procedures↗

The effects of donor-recipient size disparity in infant and pediatric heart transplantation.

To determine the effect of heart donor and recipient size mismatches in infant and pediatric heart transplantation, we studied all 69 patients (age 1 day to 11 years) having 71 orthotopic heart transplants from 1985 to 1989. Patients were divided into three groups based on donor to recipient weight ratios. Group I comprised 13 heart transplants with a donor to recipient weight ratio less than 0.95 (mean 0.81, range 0.48 to 0.94); group II comprised 29 heart transplants with a weight ratio between 0.95 and 1.60 (mean 1.28); and group III had 27 heart transplants with weight ratios greater than 1.60 (mean 2.2, range 1.61 to 3.09). All chests were closed primarily. The cardiothoracic ratio by chest radiography was significantly larger in group III (p = 0.0002); 75% of group III patients had periods of lobar or complete lung collapse by chest radiography compared with 28% of group II and 19% of group I patients (p < 0.05). Despite this, there was no difference in the number of days of ventilator support for any group (p = 0.92). There was no difference in graft ischemic time or inotropic drug use among groups, nor were differences found in the cardiac systolic function parameters of left ventricular preejection time (p = 0.975), left ventricular ejection time (p = 0.975), left ventricular fiber shortening (p = 0.97), and left ventricular fractional shortening (p = 0.596). Thus despite a high incidence of transient lobar or complete lung collapse in high donor to recipient weight ratio transplants, large donor heart size produces very little clinical impairment in recipient lung function. Size mismatches do not influence cardiac systolic function. Overall, large size mismatches appear to be very well tolerated in infant and pediatric heart transplantation.

Body Weight↗

Optimal delivery of cardioplegic solution for "redo" operations.

Increasing experience suggests that retrograde cardioplegia offers several benefits during cardiac reoperations. However, the need for dissection to allow caval snares for open coronary sinus intubation or to palpate the atrioventricular groove for transatrial coronary sinus intubation may disturb diseased vein grafts or require more dissection than necessary. Although antegrade-retrograde techniques can be used, antegrade cardioplegia risks atheromatous embolization from old vein grafts. To optimize delivery of cardioplegic solution, we designed and used "no touch" transatrial intubation of the coronary sinus for retrograde delivery of cardioplegic solution in 63 consecutive patients aged 20 to 87 years (mean 68 years) undergoing 36 redo coronary bypass operations, 7 combined redo coronary bypass/valve replacements, 6 redo aortic valve repairs/replacements, 6 redo mitral valve repairs/replacements, 4 redo double valve repairs/replacements, 2 redo triple valve repairs/replacements, and 2 redo composite aortic valve and arch replacements. "No touch" coronary sinus cannulation was achieved by minimally dissecting the aorta and high right atrium enough for two purse-string sutures. No attempt was made to dissect the junction of the inferior vena cava and atrioventricular groove if old vein grafts were present. The distal pressure line of the Gundry DLP RCSP retrograde cardioplegia cannula (DPL, Inc., Grand Rapids, Mich.) was connected to a transducer, flushed, and then introduced into the right atrium. The pressure tracing thus obtained was observed while the catheter was advanced, using its curved stylet, "blindly" without touching the heart, through the right atrium into the coronary sinus until a coronary sinus waveform was obtained (similar to floating a thermodilution catheter). The catheter's distal balloon was then inflated to occlude the coronary sinus momentarily. A rise in sinus pressure confirmed placement. If pressure did not rise, the cannula was usually in the right ventricle and was repositioned. All coronary sinuses were successfully intubated blindly. Bypass was then instituted, the aorta crossclamped, and the proximal aorta vented. Old vein grafts were cut at the aorta before retrograde cardioplegia was begun; atheromatous material was routinely flushed retrogradely from vein grafts. Only after arrest were hearts dissected as needed. Antegrade cardioplegia was not used. There were two (3%) deaths, both from hospital-acquired pneumonia, no perioperative myocardial infarctions, and no episodes of heart block. Inotropic agents were used in six of 63 patients (10%). We conclude that "no touch" transatrial retrograde cardioplegia offers optimal, simplified myocardial protection for cardiac reoperations, permits arrest of the heart before cardiac manipulations, and expands the use of retrograde cardioplegia by obviating cardiac dissection.

Aged↗

Bullet fragment venous embolus to the heart: case report.

This report describes a case of bullet fragment embolus to the heart following a small-caliber gunshot wound to the mouth. Skull and C-spine films appeared to account for the projectile; however, chest X-ray followed by fluoroscopy and two-dimensional echocardiography demonstrated a venous missile embolus in the right heart. The bullet was palpated, trapped in the right ventricle, and easily extruded.

Adult↗

Surgery for thoracoabdominal aortic aneurysms.

From July 1985 to July 1989, Loma Linda University Medical Center evaluated 46 thoracoabdominal aortic aneurysms (TAAAs). Forty patients were taken to surgery--18 (45%) were operated on an emergency basis for reasons including rupture (12 patients, 30%), dissection (5 patients, 12.5%), and severe pain (1 patient). The overall mortality for all operated patients was five (12.5%-17% for emergency surgery versus 9% for elective surgery). Nonfatal complications occurred in 40 per cent of patients (16). The overall incidence of paraplegia was 10 per cent (4/40), emergency patients 17 per cent (3/18) versus elective patients 4.5 per cent (1/22). Careful preoperative evaluation, standardization of operative technique, and good postoperative management have improved the outlook for these patients who otherwise would progress to eventual rupture and death. Because mortality and morbidity are substantially reduced in elective patients, we recommend that all patients with TAAAs be evaluated for surgery as soon as diagnosis is made.

Adult↗

Visceral ischemia secondary to gastric dilatation: a rare complication of Nissen fundoplication.

Antireflux operations are now commonly performed for severe gastroesophageal reflux with life-threatening presentations like recurrent aspiration pneumonia and apnea. We report a child who presented 2 years after Nissen fundoplication with jejunal volvulus resulting in massive gastric dilatation, gastric rupture, pneumoretroperitoneum, pneumomediastinum, and severely compromised circulation to the lower extremities. Because of the intense intraabdominal pressure from the gastric dilatation, there was severe ischemia of the pancreas, duodenum, small bowel, colon, and gallbladder. We suggest that gastrointestinal symptoms in a child who has had Nissen fundoplication should be promptly evaluated to avoid delay in recognition of acute gastric dilatation and to prevent a fatal outcome.

Child, Preschool↗

Emergency implantation of a left ventricular assist device in adolescents with biventricular failure.

Adolescents with congestive cardiomyopathy who present with intractable arrhythmia or progressive ventricular failure have a very poor prognosis and often die awaiting cardiac transplantation (CTx). We present our recent experience with a pneumatically powered left ventricular assist device (LVAD) implanted emergently to salvage adolescents with severe biventricular failure. Four patients, aged 15-17 years, body surface areas of 1.5-1.7 m2, with dilated cardiomyopathy (LV diastolic dimension, 7.1-8.3 cm); two presented with cardiovascular collapse, one with refractory ventricular tachycardia, and one with cardiac arrest. Hemodynamic and biochemical data before and 1 week after LVAD placement are expressed as mean and range values. None of the patients required right ventricular assist, and all patients achieved functional recovery while on LVAD support (8-71 days). Currently, all four patients are alive (11-22 months) after successful CTx. We conclude that emergency implantation of an LVAD in adolescents with biventricular heart failure can be life saving. As has been shown in the adult population, such a ventricular assist system restores normal circulatory hemodynamics, reverses multi-organ dysfunction, and provides a "safe" bridge to transplantation.

Adolescent↗

Changes in left ventricular mass with rejection after heart transplantation in infants.

Thirty-three infants who underwent successful heart transplantation before 6 months of age were studied to evaluate subacute changes in left ventricular mass (LVM) and its correlation to a history of rejection episodes. LVM and left ventricular wall mass (LVWM) and their percentage of predicted normal values were analyzed by means of M-mode echocardiography. LVM (as a percentage of predicted normal for body surface area) at 1 week, 1 month, and 3 months after transplantation was 103.2% +/- 24.5%, 137.3% +/- 36.0%, and 138.6% +/- 32.0%, respectively. Values for the wall mass were 82.1% +/- 23.0%, 111.3% +/- 35.7%, and 104.6% +/- 30.4%. After 1 and 3 months, both LVM and LVWM were significantly (p less than 0.01) increased from the values in the first week. The patients were subdivided on the basis of a history of rejection. There were six patients without a rejection episode within 1 month (group 1), 17 patients with one rejection episode (group 2), and 10 patients with two or more episodes (group 3). LVM at 1 month was 104.5% +/- 27.7% for group 1, 142.5% +/- 27.7% for group 2 (p less than 0.05), and 148.9% +/- 31.3% for group 3 (p less than 0.05). LVWM at 1 month was 83.4% +/- 24.6%, 114.8% +/- 35.3%, and 122.2% +/- 36.2% (groups 1 through 3, respectively). Thus an increase in posttransplant LVM may signify a rejection episode. Heart transplantation in infancy increases LVM and LVWM (septum and posterior wall); the degree of thickening of septum correlates well with rejection episodes.

Cardiomegaly↗

Orthotopic transplantation for total anomalous pulmonary venous connection associated with complex congenital heart disease.

BACKGROUND: When total anomalous pulmonary venous connection is associated with other complex cardiac malformations, early and late postsurgical morbidity and mortality are excessive. METHODS: In an attempt to modify this outcome, twelve children (4 days to 6.8 years of age) with total anomalous pulmonary venous connection and various congenital cardiac defects were treated with orthotopic heart transplantation. Associated cardiac diagnoses included the following: hypoplastic left heart syndrome (n = 2), unbalanced atrioventricular canal with pulmonary atresia (n = 2), and single ventricle with severe pulmonary stenosis (n = 3) or atresia (n = 5). Two patients had situs inversus, and two had dextrocardia with situs ambiguous. Eight patients had asplenia and one had polysplenia. Palliative pretransplantation procedures in five patients included the following: systemic to pulmonary artery shunt (n = 5), atrioventricular valve annuloplasty (n = 1) and classical Glenn shunt (n = 1). The donor left atrium was anastomosed directly to a common pulmonary venous pool in nine patients; whereas three children required complex reconstruction to baffle the pulmonary venous flow to the donor left atrium. RESULTS: There was one operative death related to an oversized heart and vena caval thrombosis. Follow-up ranged from 16 months to 4.5 years (average 3 years). In two patients (18%) pulmonary venous obstruction developed 3 and 4 months after transplantation. Reoperation to relieve the obstruction was successful in one patient. The second patient underwent three such reoperations and died of sepsis 10 months after orthotopic heart transplantation. CONCLUSION: Orthotopic transplantation is a viable option for children with complex total anomalous pulmonary venous connection that precludes a biventricular repair. Transplantation may improve the dismal prognosis of those children, but it does not eliminate the potential for late pulmonary venous obstruction.

Child↗