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.

Mitral valve chordal rupture masquerades as endocarditis.

Mitral valve chordal rupture is often associated with an inciting event. There are very few reported pediatric cases of spontaneous mitral valve chordal rupture. We describe a 9-year-old boy with a history of mitral valve prolapse who developed spontaneous mitral valve chordal rupture without evidence of endocarditis or trauma.

Anticoagulation and incidence of late cerebrovascular accidents following the Fontan procedure.

Thromboembolic events are a well-reported complication following the Fontan procedure, but no previous studies have compared the incidence of thromboembolic events relative to the prophylactic anticoagulation strategy utilized. We examined the time-adjusted incidence of late thromboembolic events relative to chronic anticoagulation strategy. All patients who have undergone Fontan palliation and are followed at our institution were reviewed. All thromboembolic and major bleeding events were recorded and compared among different subgroups (anticoagulant medication utilized, Fontan variant, and the presence of a residual right-to-left shunt). The incidence of late cerebrovascular accidents (CVAs) per patient-year was calculated for each subgroup. The records of 132 patients were analyzed (median follow-up, 7.6 years; 1066.5 total patient-years). There were no major bleeding complications. One patient receiving no anticoagulation therapy developed a symptomatic thrombus 6 months after Fontan. Three patients suffered late CVAs (range, 3-7 years); 2 were receiving aspirin, and the other received no anticoagulation therapy. All 3 had lateral tunnel Fontan and a residual right-to-left shunt. The overall incidence of late CVA was 2.3%, with an event rate of 0.28% per patient-year. Late CVA was not related to anticoagulation strategy or time from Fontan procedure but was associated with a residual right-to-left shunt and lateral tunnel-type Fontan palliation (p < 0.001). Regardless of anticoagulation strategy utilized, symptomatic CVA is a rare long-term complication following the Fontan procedure.

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.

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.

Posttransplant diabetes mellitus in pediatric thoracic organ recipients receiving tacrolimus-based immunosuppression.

BACKGROUND: Posttransplantation diabetes mellitus (PTDM) is a well-known complication of tacrolimus-based immunosuppression in both adult and pediatric solid organ recipients. The "natural history" of diabetes in the pediatric thoracic transplant population has not yet been described. METHODS: We identified all pediatric thoracic transplant patients receiving tacrolimus-based immunosuppression who developed PTDM. Medical records were reviewed, with a particular focus on the clinical course of PTDM and its relationship to drug weaning. RESULTS: Diabetes developed in 24 of 143 (17%) 30-day survivors of heart (12/96, 13%) and heart-lung/lung (12/ 47, 26%) transplantation. In 17 (71%) patients, the immunosuppressive regimen at the onset of PTDM also included maintenance corticosteroids. Seventeen patients demonstrated glucose intolerance before the onset of diabetes. Nine patients (38%) developed diabetes during pulsed corticosteroid therapy. Median time of onset after transplantation was 9.0 months. All patients required s.c. insulin for glucose control. The median follow-up from transplant was 49.9 months. There was a significant decrease in mean tacrolimus dosage (P<0.01), tacrolimus level (P<0.04), and steroid dosage (P<0.02) from onset of PTDM to most recent follow-up. Despite this significant reduction in immunosuppression, only 3/24 (13%) patients were successfully weaned off insulin. CONCLUSIONS: Diabetes mellitus is a common complication in pediatric thoracic transplant patients receiving tacrolimus-based immunosuppression. Insulin dependence in our population rarely resolved, even after lowering tacrolimus and steroid doses. Discontinuation of steroids did not guarantee resolution of diabetes.

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.

Repair of tetralogy of Fallot in neonates and young infants.

BACKGROUND: The timing of repair of tetralogy of Fallot (TOF) remains controversial. Advantages to early complete repair include removal of right ventricular outflow tract obstruction, alleviation of systemic hypoxia, and avoidance of palliation with an arteriopulmonary shunt. METHODS AND RESULTS: This is a retrospective review of 99 children with TOF pulmonary stenosis (TOF/PS) or TOF pulmonary atresia (TOF/PA) who were <90 days of age undergoing early complete repair. Fifty-nine were prostaglandin E dependent, and 91% of neonates were symptomatic at the time of repair. Univariate and multivariate analyses of patient characteristics, anatomic features, and operative management showed the diagnosis of TOF/PA and smaller body surface area to be the only independent risk factors for death. Early mortality was 3% (3 of 99), and actuarial survival rates were 94% at 1 year and 91.6% at 5 years. Freedom from catheterization was 86% at 1 year and 73% at 5 years. Patients repaired for TOF/PA had a significantly lower freedom from reoperation than did those repaired for TOF/PS. CONCLUSIONS: Early complete TOF repair can be accomplished with a low mortality. Children with TOF/PA repaired had a lower freedom from reoperation that did those with TOF/PS. Longer follow-up, with emphasis on arrhythmias and right ventricular function, is required to define the long-term benefits of early repair.

The effects of cardiopulmonary bypass on remifentanil kinetics in children undergoing atrial septal defect repair.

UNLABELLED: Cardiopulmonary bypass (CPB) can greatly influence the pharmacokinetics of opioids. This study investigated the pharmacokinetic profile of remifentanil in 12 pediatric patients undergoing CPB for repair of an atrial septal defect. All patients received remifentanil (5 microg/kg) over 1 min into a peripheral vein both before the onset of CPB and after the discontinuation of CPB. Arterial blood samples were obtained at defined time periods, and remifentanil concentration was determined using high-performance liquid chromatography ultraviolet detection. The pharmacokinetic profiles both before and after bypass were determined in all 12 patients. There was no change in the volume of distribution at steady state, the volume of the central compartment, or the alpha- and beta-elimination half-life. Although the clearance values increased 20% in the postbypass period (from 38.7 +/- 9.6 to 46.8 +/- 14 mL x kg(-1) x min(-1), there was no meaningful change in the coefficient of variation (from 25% to 30%). IMPLICATIONS: After cardiopulmonary bypass the clearance of remifentanil increases in children. However, the relative lack of change in the coefficient of variation suggests that remifentanil should be a predictable drug in the postcardiopulmonary bypass period.

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.

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.