The needle and the damage done: pericardial effusion with tamponade after needle ingestion in an infant.

OBJECTIVE: Paediatric foreign bodies may present with vague and nonspecific symptoms. It is important to have a high index of suspicion when managing such cases. METHOD: We report the case of a nine-month-old infant who presented with a wheeze, cough and fever following ingestion of a needle. RESULTS: This patient developed pericardial tamponade as a consequence of the needle ingestion, and required a thoracotomy for retrieval. We discuss the pathophysiology involved and the surgery required. CONCLUSION: Pericardial tamponade is a rare but potentially fatal manifestation of an ingested foreign body.

Biventricular repair in patients with heterotaxy syndrome.

OBJECTIVE: Complex intracardiac and extracardiac anatomy is often confronted during biventricular repair in patients with heterotaxy syndrome. We examined factors affecting surgical outcomes in these patients. METHODS: Between January 1990 and July 2007, 371 patients received a diagnosis of heterotaxy syndrome; 91 (91/371, 24.5%) underwent biventricular repair. Left atrial isomerism was present in 73% (66/91) and right atrial isomerism in 10% (9/91), with indeterminate atrial anatomy in 17% (16/91). Median age at biventricular repair was 6.8 months (5 days to 22.3 years). Systemic venous anomalies were present in 75 patients, pulmonary venous anomalies in 26, and endocardial cushion defects in 36. Transposition complexes were present in 15 patients with atrioventricular discordance in 10; 8 underwent double switch, 2 received a physiologic repair, 2 underwent arterial switch, and 3 underwent the Rastelli operation. Other conotruncal anomalies included double-outlet right ventricle in 10 patients, tetralogy of Fallot in 3, and hemitruncus in 2. Separation of systemic from pulmonary venous return included intra-atrial baffling in 48 patients and extracardiac grafting in 2. Combined lesions were common, occurring in 99% (90/91). Statistical analysis with Kaplan-Meier and Cox proportional hazards models were performed. RESULTS: Average follow-up was 44.9 +/- 57.5 months (3 days to 189.3 months). Kaplan-Meier estimated survival was 93.4% at 10 years; unbalanced complete atrioventricular canal was the only risk factor for mortality (P = .006). Subsequent procedures were common with a 10-year freedom from reoperation or reintervention of 38% +/- 7.5%. Arrhythmias occurred in 36 (39.6%) patients; bradyarrhythmia in 27 (29.7%) and tachyarrhythmia in 15 (16.5%). Freedom from any arrhythmia was 53.9% +/- 6.7% at 10 years. CONCLUSIONS: Excellent survival for patients with heterotaxy undergoing biventricular repair can be expected, even for multiple, complex lesions. Reintervention is common, and arrhythmia is a long-term concern. This experience shows that patients with heterotaxy syndrome and complex cardiac anatomy can be considered for biventricular repair. Patients with unbalanced complete atrioventricular canal are a high-risk group for which selection criteria are particularly important.

Aortopulmonary size discrepancy is not a contraindication to the pediatric Ross operation.

BACKGROUND: Modification of the aortic annulus or the ascending aorta, or both, may be required in pediatric patients undergoing the Ross operation. The fate of these autografts remains uncertain. METHODS: Retrospective review of 15 patients undergoing Ross operation without aortic annular modification (group 1), 11 patients requiring annular reduction (group 2, n = 11), and 8 patients requiring annular enlargement (group 3, n = 8). Autograft function and dimensions were evaluated by echocardiography. RESULTS: Autograft insufficiency was less than or equal to mild in 33 patients and moderate in 1 patient. The annulus body surface area ratio increased in group 1 from 19.7 +/- 5 to 20.3 +/- 5 mm/M2 (p = 0.8). The average annular reduction in group 2 was 5 +/- 1.5 mm, and 10 of 11 patients required reduction of the ascending aorta (mean 11 +/- 5 mm). The annulus body surface area ratio increased from 18.6 +/- 7 to 20.5 +/- 9 mm/M2 (p = 0.2). The mean augmentation in annulus diameter in group 3 was 6 +/- 4 mm; the annulus body surface area ratio decreased from 23.7 +/- 14 to 20.3 +/- 8 mm/M2 (p = 0.5). CONCLUSIONS: We continue to offer the Ross operation to pediatric patients even when aortic annular or ascending aortic size discrepancies mandate surgical modifications.

Regional low-flow perfusion provides somatic circulatory support during neonatal aortic arch surgery.

BACKGROUND: Regional low-flow perfusion has been shown to provide cerebral circulatory support during neonatal aortic arch operations. However, its ability to provide somatic circulatory support remains unknown. METHODS: Fifteen neonates undergoing arch reconstruction with regional perfusion were studied. Three techniques were used to assess somatic perfusion: abdominal aortic blood pressure, quadriceps blood flow (near-infrared spectroscopy), and gastric tonometry. RESULTS: Twelve patients required operation for hypoplastic left heart syndrome, and 3 required arch reconstruction with a biventricular repair. There was one death (7%). Abdominal aortic blood pressure was higher (12+/-3 mm Hg versus 0+/-0 mm Hg), and quadriceps blood volumes (5+/-24 versus -17+/-26) and oxygen saturations (57+/-25 versus 33+/-12) were greater during regional perfusion than during deep hypothermic circulatory arrest (p < 0.05). During rewarming, the arterial-gastric mucosal carbon dioxide tension difference was lower after circulatory arrest than after regional perfusion (-3.3+/-0.3 mm Hg versus 7.8+/-7.6 mm Hg, p < 0.05). CONCLUSIONS: Regional low-flow perfusion provides somatic circulatory support during neonatal arch surgical procedures. Support of the subdiaphragmatic viscera should improve the ability of neonates to survive the postoperative period.

The effect of cytokine gene polymorphisms on pediatric heart allograft outcome.

BACKGROUND: Cytokines play a major role in the inflammatory and immune responses that mediate allograft outcome. Several studies have shown that the production of cytokines varies among individuals and these variations are determined by genetic polymorphisms, most commonly within the regulatory region of the cytokine gene. The aim of this study was to assess the effect of these allelic variations on acute rejection after pediatric heart transplantation. METHODS: We performed cytokine genotyping using polymerase chain reaction-sequence specific primers in 93 pediatric heart transplant recipients and 29 heart donors for the following functional polymorphisms: tumor necrosis factor-alpha (TNF-alpha) (-308), interleukin (IL)-10 (-1082, -819, and -592), TGF-beta1 (codon 10 and 25), IL-6 (-174), and interferon-gamma (INF-gamma) (+874). The distribution of polymorphisms in this population did not differ from published controls. The patients were classified as either non-rejecters (0 or 1 episode) or rejecters (> 1 episode) based on the number of biopsy proven rejection episodes in the first year after transplantation. RESULTS: Forty-two of the 69 TNF-alpha patients (61%) in the low producer group were non-rejecters, while 9 of the 24 (37.5%) with high TNF-alpha were non-rejecters (p = 0.047). In contrast, IL-10 genotype showed the opposite finding. Forty-two of the 66 patients (64%) in the high and intermediate IL-10 group were non-rejecters, while 9 of the 26 (35%) in the low IL-10 group were non-rejecters (p = 0.011). The combination of low TNF-alpha with a high or intermediate IL-10 genotype was associated with the lowest risk of rejection (34/49 or 69% non-rejecters). Neither the distribution of the IL-6, INF-gamma, and TGF-beta1 genotype in recipients nor the donor genotype showed any association with acute rejection. CONCLUSION: Genetic polymorphisms that have been associated with low TNF-alpha and high IL-10 production are associated with a lower number of acute rejection episodes after pediatric heart transplantation.

Cardiopulmonary transplantation for congenital heart disease in the adult.

BACKGROUND: Patients surviving into adulthood with congenital heart disease (CHD) often succumb to progressive cardiopulmonary dysfunction. For these patients transplantation is often considered. METHODS: We performed a retrospective review of 69 adults (age >18 years) with CHD transplanted between 1984 and 1999. RESULTS: We evaluated 31 heart-lung (HLTxp), 30 lung (LTxp), and 8 heart (HTxp) transplants performed in 22 men and 47 women with CHD. Mean age was 37 +/- 10 years with a mean follow-up of 3.1 +/- 3.5 years. A concomitant cardiovascular procedure was performed in 1 HLTxp, 23 LTxp, and 2 HTxp. Early mortality (>30 days) was 26% (8/31) for HLTxp, mostly due to bleeding. Early LTxp mortality was 23% (7/30), largely due to graft failure. One and 3-year survival was similar in adults transplanted for CHD and adults transplanted for other disease. Early mortality among HTxp recipients was 50% (4/8) from rejection or technical complications. Survival for patients undergoing HLTxp versus LTxp with cardiac repair was similar. When examined by era, the survival of patients transplanted for CHD between 1992 and 1999 was greater than that of patients transplanted between 1984 and 1991. CONCLUSIONS: Adults undergoing HLTxp and LTxp for CHD can expect survival comparable to that of non-CHD adults. In the presence of a reparable cardiac lesion, LTxp with cardiovascular repair for CHD is an attractive option, optimizing organ allocation. Specific technical concerns are discussed. Survival of adults undergoing cardiopulmonary transplantation for CHD has improved over time.

Hypothermic cardiopulmonary bypass alters oxygen/glucose uptake in the pediatric brain.

OBJECTIVES: Neurologic morbidity related to cardiac surgery has been recognized as a major morbidity. A variety of causes related to cardiopulmonary bypass, including microemboli, nonpulsatile flow, hemodilution, and inflammatory mediation, have been proposed. Because oxygen and glucose are the predominant metabolic substrates for the brain, we sought to examine the uptake of these substrates by the pediatric brain during hypothermic cardiopulmonary bypass. METHODS: Eleven children (median age 5 months, range 1 day-17 years) undergoing a variety of cardiac surgical procedures with the use of hypothermic cardiopulmonary bypass were studied. Cerebral arteriovenous differences for oxygen, glucose, and lactate were obtained before, during, and after bypass. On the basis of the predictable stoichiometric relationship for the oxidation of glucose, the relationship of substrate uptake was expressed as the oxygen/glucose index.Oxygen/glucose index (%) = (arteriovenous oxygen difference [micromol/mL]/arteriovenous glucose difference [micromol/mL] x 6) x 100 RESULTS: All children survived with no obvious neurologic sequelae. During cooling on cardiopulmonary bypass, the oxygen/glucose indexes fell significantly from prebypass values (53% +/- 19% at 28 degrees C and 54% +/- 25% at 24 degrees C vs 117% +/- 70%; P <.05, analysis of variance). This decline resulted from decreased oxygen uptake with stable glucose uptake (P <.05). Although oxygen and glucose uptake both increased with rewarming, the net effect was only a slight increase in oxygen/glucose index (62% +/- 16%). Postbypass oxygen/glucose index exceeded prebypass values (149% +/- 83%). CONCLUSIONS: Hypothermic cardiopulmonary bypass alters the relationship between oxygen and glucose uptake in the pediatric brain. The relationship of these findings to bypass-related neurologic morbidity remains to be explored.

Management of hepatic venous obstruction after split-liver transplantation.

Stenosis of the hepatic vein anastomosis is an unusual but critical complication after liver transplantation. In pediatric liver transplantation, the scarcity of size-matched donors has required the use of segmental liver allografts, either as reduced-size or split-liver grafts. This report illustrates the primary use of a hepatic vein stent to manage hepatic venous outflow obstruction in a pediatric split-liver recipient, and reviews experience in the management of hepatic venous outflow obstruction after liver transplant using stent methods.

Endomyocardial biopsy in pediatric heart transplant recipients: a useful exercise? (Analysis of 1,169 biopsies).

The objective of this study was to define the diagnostic yield for endomyocardial biopsy (EMB) procedures performed for various indications in a large pediatric heart transplant population. Endomyocardial biopsy procedure has been employed as the 'gold standard' for rejection surveillance. Previous studies have questioned the value of surveillance EMB beyond the early post-transplant period. We retrospectively reviewed data on 82 pediatric heart transplant recipients with serial EMB. A total of 1,169 EMB were performed during a follow-up period of 2-149 months (median 41 months). EMB were classified by age at transplantation, time from transplant, immunosuppressive regimen used [tacrolimus vs. cyclosporin A (CsA)] and indication, i.e. surveillance, follow-up after rejection or lowering of immunosuppression, non-specific clinical symptoms and graft dysfunction. During the first year after heart transplantation, surveillance EMB demonstrated significant rejection [International Society for Heart and Lung Transplantation (ISHLT) grade > or = 3A] in 18% of biopsies with the yield being 14-43% for all other indications. Surveillance EMB 1-5 yr post-transplantation were found to have a lower diagnostic yield in infants (4%, vs. 13% in children) and in patients with favorable first-year rejection history (9% vs. 17% in 'frequent rejectors'). Tacrolimus-based immunosuppression was associated with significantly less rejection, but only in the first year post-transplantation (14% in tacrolimus vs. 24% in CsA surveillance EMB, p = 0.035). Surveillance EMB remains an important diagnostic tool for rejection surveillance during the first 5 years after pediatric heart transplantation. Endomyocardial biopsy is particularly warranted after reduction of immunosuppression and for monitoring for ongoing rejection after treatment of acute rejection episodes.

Management of retroperitoneal arterial injury after heart catheterization in children.

With the expansion of interventional cardiology into the pediatric population, vascular complications related to cardiac catheterization can be expected to occur. Cardiac surgeons must be prepared to treat these life-threatening injuries. We present a case and detail the technique of the surgical management of retroperitoneal arterial injury after interventional cardiac catheterization in a 6-month-old boy.

Cardiac transplantation in survivors of lymphoma: a multi-institutional survey.

BACKGROUND: Cardiac transplantation has been successfully performed in patients with a history of presumably cured Hodgkin's disease (HD) and non-Hodgkin's lymphoma (NHL). Though the risk of recurrence is a major concern, the long-term influence of prior cancer and cancer therapy on posttransplant outcome has not been previously investigated. METHODS: Questionnaires were sent to 130 cardiac transplant centers in the United States registered with the United Network for Organ Sharing. Data collected included patient demographics; type, stage, and timing of HD/NHL; treatment for HD/NHL; posttransplant immunosuppressive regimen, rejection history, and outcomes; and Epstein-Barr virus status. RESULTS: Thirty-four cardiac transplant recipients with a previous history of HD (n=16) or NHL (n=18) were identified. HD patients averaged 41+/-15 years of age, with a mean disease-free interval of 15+/-9 years at the time of transplantation. NHL patients averaged 42+/-17 years of age with a mean disease-free interval of 10+/-9 years at the time of transplantation. The mean follow-up for the entire group was 50 months (range, 2 days to 136 months), and mean follow-up for the survivors was 67 months (range, 23-136 months). The 1-, 3-, 5-, 7-, and 10-year actuarial survival estimates for the entire group are 77%, 64%, 64%, 64%, and 50%, respectively. Actuarial survival was lower in HD patients (P=0.04) and in patients who had previously undergone splenectomy (P=0.008). Cox regression analysis identified only prior splenectomy (P=0.02) as an independent risk factor for mortality after cardiac transplantation with an adjusted relative risk of 6.2 (1.7-21.9, 95% confidence intervals). CONCLUSIONS: Although the numbers are small, these data strongly suggest that there is an increased mortality risk for cardiac transplant recipients with prior HD who have undergone splenectomy.

Regional low-flow perfusion provides cerebral circulatory support during neonatal aortic arch reconstruction.

OBJECTIVE: Because of concerns regarding the effects of deep hypothermia and circulatory arrest on the neonatal brain, we have developed a technique of regional low-flow perfusion that provides cerebral circulatory support during neonatal aortic arch reconstruction. METHODS: We studied the effects of regional low-flow perfusion on cerebral oxygen saturation and blood volume as measured by near-infrared spectroscopy in 6 neonates who underwent aortic arch reconstruction and compared these effects with 6 children who underwent cardiac repair with deep hypothermia and circulatory arrest. RESULTS: All the children survived with no observed neurologic sequelae. Near-infrared spectroscopy documented significant decreases in both cerebral blood volume and oxygen saturations in children who underwent repair with deep hypothermia and circulatory arrest as compared with children with regional low-flow perfusion. Reacquisition of baseline cerebral blood volume and cerebral oxygen saturations were accomplished with a regional low-flow perfusion rate of 20 mL x kg(-1) x min(-1). CONCLUSIONS: Regional low-flow perfusion is a safe and simple bypass management technique that provides cerebral circulatory support during neonatal aortic arch reconstruction. The reduction of deep hypothermia and circulatory arrest time required may reduce the risk of cognitive and psychomotor deficits.

Complete surgical repair of aortic atresia with a normal left ventricle.

Aortic atresia with a normal left ventricle and normal mitral valve is an uncommon congenital lesion. We present two such cases and describe two different approaches of achieving biventricular surgical repair.

Cardiac operations in solid-organ transplant recipients.

BACKGROUND: The success of solid organ transplantation has resulted in an increasing pool of patients that subsequently require cardiac surgical procedures, yet the perioperative management of these patients is not well documented. We report a single institutional experience with the management techniques used and the outcomes of the cardiac surgical procedures performed in solid organ transplant recipients with functioning allografts. METHODS: Sixty-four patients underwent 66 cardiac procedures broken down as follows: coronary artery bypass grafting, 30; single or combined valve replacement-repair, 24; combined coronary artery bypass grafting and valve repair, 3; aortic repair, 4; pericardiectomy, 3; transmyocardial laser revascularization, 1; and native cardiectomy, 1. Patients consisted of 40 kidney, 16 liver, 5 heart, 2 lung, and 1 liver and kidney transplant recipients. The mean interval from the time of transplantation to the cardiac operation was 53 months (range, 1 day to 220 months). Forty-six male and 18 female patients in New York Heart Association functional class III or IV had a mean age of 53 years (range, 19 to 77 years); 50% (32/64) were diabetic, and 97% (62/64) were hypertensive. Immunosuppressive therapy, cardiopulmonary bypass, and medical management were similar in all patients. RESULTS: There were two (3%) perioperative deaths, one of which was caused by an arrhythmia-induced cardiac arrest, and there were seven (11%) late deaths from non-cardiac-related causes. Major complications included 12 infections (19%), ten mediastinal reexplorations for the control of bleeding (16%), and nine others (15%). Sixteen of the 64 (25%) transplant recipients had chronic renal failure (serum creatinine levels, > 3 mg/dL), including 13 of 40 (33%) kidney transplant patients. Acute renal failure developed postoperatively in 7 (54%) of these 13 patients; the grafts failed permanently in 3 (23%). Three patients (5%), 2 kidney transplant recipients and 1 lung transplant recipient, experienced transient acute rejection. Fifty of the 55 surviving patients are alive and well (New York Heart Association functional class I or II) without recurrent cardiac disease at a mean follow-up period of 22 months. CONCLUSIONS: Although the short-term morbidity was significant, the low mortality and low incidence of permanent graft dysfunction indicate that solid organ transplant recipients can safely and effectively undergo subsequent cardiac surgical procedures.

Massive sinus of Valsalva aneurysm presenting with coronary insufficiency.

A 50-year-old man with a massive acquired sinus of Valsalva aneurysm presenting with coronary insufficiency is presented. Annuloaortic ectasia and severe aortic insufficiency mandated composite aortic valve and root replacement, with reimplantation of the coronary arteries. Clinical characteristics, treatment principles, and surgical outcomes are described.

Lung transplantation for respiratory failure resulting from systemic disease.

BACKGROUND: Lung transplantation for pulmonary failure resulting from systemic disease is controversial. We reviewed our transplant experience in patients with sarcoidosis, scleroderma, lymphangioleiomyomatosis, and graft-versus-host disease. METHODS: This retrospective review examined the outcome of 23 patients who underwent pulmonary transplantation for these systemic diseases. Group 1 included 15 patients with pulmonary hypertension who underwent transplantation (9 for sarcoidosis, 6 for scleroderma), and group 2 included 8 patients with normal pulmonary artery pressures who underwent transplantation (5 for lymphangioleiomyomatosis, 3 for graft-versus-host disease). The incidences of infection and rejection, pulmonary function, and survival were measured and compared with those of patients who underwent transplantation for isolated pulmonary disease. RESULTS: Although there were no differences in the rate of infection between patients who underwent transplantation for systemic versus isolated disease, patients with pulmonary hypertension who underwent transplantation for systemic disease had significantly lower rates of rejection. Four patients with sarcoidosis and 2 with lymphangioleiomyomatosis demonstrated recurrence in the allograft. Survival was similar between patients who underwent transplantation for systemic versus isolated disease. CONCLUSIONS: Patients with respiratory failure resulting from these systemic diseases can undergo transplantation with outcomes comparable to those obtained in patients who undergo transplantation for isolated pulmonary disease.

Acute and chronic morbidity differences between muscle-sparing and standard lateral thoracotomies.

INTRODUCTION: Opinions differ regarding differences between totally muscle-sparing thoracotomy and standard lateral thoracotomy approaches to pulmonary resection with respect to operative time, postoperative pain and morbidity, and occurrence of chronic postthoracotomy pain syndromes and subjective shoulder dysfunction. METHODS: Three hundred thirty-five consecutive patients undergoing muscle-sparing thoracotomy (n = 148) or lateral thoracotomy (n = 187) to accomplish lobectomy for stage I lung cancer during a 40-month period were evaluated. Local rib resection was not employed, and two chest tubes were routinely used after operation in both thoracotomy groups. Epidural analgesia use was similar after operation in the two groups (muscle-sparing thoracotomy 38%, lateral thoracotomy 38%). The postoperative hospital courses and patient functional statuses at 1 year were examined. RESULTS: Demographic analyses demonstrated no differences between groups in age, sex, or association of significant comorbid medical illness. Although the operative time required for muscle-sparing thoracotomy was shorter, there were no differences between thoracotomy approaches in any of the other primary acute postoperative variables analyzed (chest tube duration, length of hospital stay, postoperative narcotic requirements, and postoperative mortality). The frequencies of chronic pain and shoulder dysfunction assessed 1 year after operation were also similar between thoracotomy groups. CONCLUSIONS: The relative efficacies and rates of occurrence of acute or chronic morbidity are equivalent after muscle-sparing thoracotomy and standard lateral thoracotomy. Although muscle-sparing thoracotomy may possibly be performed more expediently, it appears that the singular advantage of muscle-sparing thoracotomy over standard lateral thoracotomy involves the preservation of chest wall musculature in case rotational muscle flaps should be needed later.

Unsuspected lung cancer found in work-up for lung reduction operation.

BACKGROUND: Lung reduction surgery is gaining acceptance in the treatment of patients suffering from severe diffuse emphysema. At the University of Pittsburgh 210 patients have been evaluated and 128 patients have undergone lung reduction operations. METHODS: Ten patients, representing 7.8% of the operated group, have had asymptomatic cancerous or neoplastic lesions diagnosed on preoperative evaluation or pathologic analysis of resected tissue. RESULTS: Six primary lung cancers (three squamous, three adenocarcinoma) and four other neoplastic lesions (squamous dysplasia, chemodectoma, and two carcinoid tumorlets) have been identified. All patients were heavy smokers, and all had markedly impaired pulmonary function. Patients whose lesions were identified on preoperative testing underwent thoracoscopic wedge excision of the tumor alone. CONCLUSIONS: Our experience suggests that patients with impaired pulmonary function (chronic obstructive pulmonary disease) presenting for lung reduction operations are at a high risk of harboring an unsuspected neoplastic lesion. Complete preoperative evaluation of radiographic studies and preoperative bronchoscopic examination are mandatory.

Diagnosis of postpneumonectomy bronchopleural fistula using ventilation scintigraphy.

Occult bronchopleural fistulas are frequently present when empyemas develop in a postpneumonectomy space. Recognition of the bronchial stump disruption can be difficult, which may lead to delays or errors in the management of these difficult problems. We report the use of xenon ventilation nuclear scintigraphy as an effective noninvasive means of confirming the diagnosis of occult postpneumonectomy bronchopleural fistulas.