Functional total anomalous pulmonary venous drainage by left atrium-to-superior vena cava shunt.

Total anomalous pulmonary venous connection is occasionally associated with a functional single ventricle and is thought to be a risk for the staged Fontan approach. We report a patient with a very rare presentation of mitral atresia and intact atrial septum with physiologically total anomalous pulmonary venous drainage by a connecting vein between the left atrium and the superior vena cava. A surgical atrioseptectomy as a functional repair of total anomalous pulmonary venous connection and pulmonary artery banding was successfully performed as the first palliation for Fontan completion.

Regression of marked myocardial sinusoids associated with hypoplastic left heart syndrome during staged Fontan approach.

The anatomic subtype of mitral stenosis, aortic atresia, and myocardial sinusoids is reported to be associated with increased mortality for staged repair for hypoplastic left heart syndrome. We herein report a very rare case of this subtype of hypoplastic left heart syndrome presenting with marked myocardial sinusoids in the left ventricle, which nearly disappeared after successful Fontan completion. Our successful experience suggests marked myocardial sinusoids per se are not a risk factor for surgical palliation in hypoplastic left heart syndrome.

Cardiac surgery late after heart transplantation: a safe and effective treatment option.

OBJECTIVE: Owing to the improved long-term outcome after cardiac transplantation, cardiac allograft vasculopathy or valvular disease is developing late after heart transplantation in a growing number of patients. In this study, we evaluated our results of nonretransplant cardiac surgery in these patients and compared them with those after retransplantation. METHODS: Since 1983, a total of 867 heart transplantations have been performed at our institution. Among them, 44 patients underwent nonretransplant cardiac surgery, 4 of them repeatedly. The procedures included 19 coronary artery revascularizations, 20 tricuspid valve procedures, 4 other valvular procedures, 4 aortic operations, and 1 right atrial thrombectomy. Long-term results of these patients were compared with those of 20 patients after late cardiac retransplantation. RESULTS: Indications for nonretransplant surgery included cardiac allograft vasculopathy, tricuspid regurgitation, aortic and mitral valve insufficiency, as well as acute aortic dissection type A. Mean interval between heart transplantation and reoperation was 8.4 years. Mean follow-up was 5.8 years. Early mortality was 4.5% (2/44). The early deaths were caused by intracerebral bleeding and acute rejection. Actuarial survivals at 1, 5, and 7 years were 84%, 64%, and 58%, respectively. In comparison, early mortality in the retransplant group was 20% (4/20) and survivals at the same time points were 70%, 70%, and 47%, respectively. CONCLUSIONS: According to these results, we consider nonretransplant surgical options for cardiac allograft vasculopathy and valvular disease a safe and effective therapeutic approach with low early mortality and acceptable long-term results.

Size reduction of donor organs in pediatric lung transplantation.

Lobar transplantation and peripheral segmental resection allow downsizing of larger lungs for use in smaller recipients, particularly with regard to pediatric patients on the high urgency waiting list. We studied the safety and outcome of these techniques in children. All pediatric patients who underwent reduced size LTx between January 2000 and March 2009 were retrospectively reviewed and compared with pediatric patients who underwent full size LTx during the same period. Patient characteristics, intra-operative variables, and post-operative morbidity and mortality were compared. Among 28 primary LTxs, 16 (57%) were performed in reduced size technique. Preoperatively, there was a trend toward a higher rate of mechanical ventilation and a higher capillary pCO(2) in the reduced size group. Surgical procedures tended to be longer in that group. Post-operative complications, survival and functional parameters were comparable between both groups. Our study demonstrates that reduced size LTx in children is a reliable therapeutic option that provides results comparable to full size LTx. This technique might help to reduce waiting list mortality by expanding the donor pool in pediatric LTx.

Acute treatment of ST-segment-elevation myocardial infarction: is there a role for the cardiac surgeon?

BACKGROUND: Several attempts from single institutions to treat acute myocardial infarctions with bypass surgery never reached widespread acceptance in the cardiology and surgical community. Owing to a variety of new surgical techniques, this old dogma has to be reconsidered under the light of patient-adjusted optimal treatment algorithms. METHODS: Between August 2002 and August 2007, 112 patients, mean age of 66 years (range, 41 to 85 years), underwent emergency coronary artery bypass grafting (untreatable or rejected by the referring cardiologists within 48 hours after onset of symptoms). Thirty-seven patients (33%) exhibited cardiogenic shock, and 18 (16%) had prior cardiopulmonary resuscitation. Preoperative support by intraaortic balloon pump was initiated in only 10%, and 65% had left main stem stenosis. RESULTS: All patients showed a significant elevation of cardiac markers (creatine kinase-MB) and ST-segment elevation. The mean number of grafts was 2.4 (range, 1 to 4). The cardiopulmonary bypass time ranged from 48 to 261 minutes. Intraaortic balloon pump for weaning from extracorporeal circulation was used in 42 patients (38%); 3 patients needed extracorporeal membrane oxygenation support. Postoperative complications included rethoracotomy for bleeding in 4% and stroke in 2%. Thirty-day mortality was 20% in the whole group, 30% in the group with cardiogenic shock, and 15% in those without hemodynamic deterioration (p = 0.044). The multivariate analysis revealed the preoperative need for catecholamines as the only risk factor for 30-day mortality (odds ratio, 6.4; 95% confidence interval, 2 to 21; p = 0.002). CONCLUSIONS: Emergency coronary artery bypass grafting in patients with acute myocardial infarction can be performed with acceptable results, especially in those without cardiogenic shock. Therefore, operative revascularization should not be considered only as a rescue therapy.

Current surgical management of ascending aortic aneurysm in children and young adults.

BACKGROUND: The low incidence of aortic aneurysm among children and young adults limits information about etiology, surgical indications, procedures of choice, and operative results. METHODS: From 2003 to 2008, 35 patients aged 7 to 35 years (mean 21) underwent replacement of the ascending aorta for 19 ascending aortic aneurysms and 16 aortic root aneurysms. Underlying diseases included 10 congenital aortic stenoses, 10 bicuspid aortic valves, 8 congenital heart diseases, 6 Marfan syndromes, and 1 aortitis. RESULTS: Indications for surgery were maximal diameter of 200% of normal for isolated aneurysms, and 160% of normal in case of associated aortic valve dysfunction or symptoms. Operative procedures included 27 ascending aortic replacements with or without aortic valve replacement (including 22 conduits) and 8 valve-sparing operations, performed in 6 patients with Marfan syndrome and 2 with congenital heart diseases. There was 1 in-hospital death. Thirty-four patients survived the operation and are in New York Heart Association functional class II or less at a maximum of 5 years of follow-up. All patients are free from reoperation, but 1 patient had a thromboembolic event. Aortic valve function is good in all 8 patients after the valve-sparing operation. CONCLUSIONS: Ascending aortic aneurysm in children and young adults was surgically treated with excellent midterm outcome. A diameter of 200% of normal was the indication for surgery; however, in case of associated lesions, smaller diameters should be considered for surgery. Conduit implantation is the gold standard. Valve-sparing operation can be performed in selected patients with encouraging results.

Surgical repair of anomalous origin of the left coronary artery arising from the left pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. We report an extremely uncommon variety of ALCAPA, in which the left coronary artery arose from the left pulmonary artery in a patient who presented with severe heart failure in early infancy. After direct reimplantation of the left coronary artery into the ascending aorta, the patient's cardiac function recovered successfully.

Aneurysm of the aortic root in the setting of Wiskott-Aldrich syndrome.

Wiskott-Aldrich syndrome is a rare X-linked disease, associated with immunodeficiency, infections, thrombocytopaenia, and eczema. Aortitis and formation of aneurysms have also been described. We describe here our experience with a 7-year-boy with this syndrome. He survived replacement of the aortic root because of an aneurysmal ascending aorta, and subsequent bone marrow transplantation.

First experiences with intraoperative Levosimendan in pediatric cardiac surgery.

Levosimendan is a calcium-sensitizing agent with effective inotropic properties. It has been shown to improve cardiac function, hemodynamic performance, and survival in adults with severe heart failure. However, the effect of Levosimendan in pediatric cardiac surgery has not yet been investigated. Thus, we report on our experience with the intraoperative application of Levosimendan in seven infants (body weight range 2.6-6.3 kg) with severe myocardial dysfunction after complex congenital heart surgery. During the administration of Levosimendan, the heart rate, mean arterial blood pressure, and central venous pressure did not change. The mean arterial lactate level significantly decreased 24 and 48 h after the first infusion compared to baseline. Central venous oxygen saturation increased significantly 24 and 48 h after the onset of Levosimendan infusion. We found intraoperatively administered Levosimendan to be well tolerated in the seven infants with severe myocardial dysfunction after complex congenital heart surgery. Levosimendan is a new rescue drug which has beneficial effects, even in pediatric cardiac surgery.

Improved results after repair of complete atrioventricular septal defect.

BACKGROUND: This study evaluates the historical impact on the outcomes of early primary repair of complete atrioventricular septal defect (AVSD) at our institute. METHODS: Since 1976, a total of 185 children with complete AVSD have been referred to our unit. Prior to 1990, 78 children received conservative therapy, and selected 51 patients underwent surgical repair (group 1). After 1990, all referred children underwent surgical repair (n = 56; group 2). Pre- and postoperative parameters were analyzed and compared among the groups. RESULTS: Age at operation was 15.4 +/- 20.4 versus 9.9 +/- 18.0 months in group 1 and group 2, respectively. Association with Down syndrome (53% vs. 82%; p < 0.01) and with patent ductus arteriosus (16 vs. 34%; p < 0.05) was less frequent in group 1. No difference was seen regarding preoperative pulmonary vascular resistance index (RPI). Actuarial survival at 15 years has improved in group 2 (69.3 +/- 6.7 vs. 90.8 +/- 3.9%; p < 0.05). Freedom from reoperation of the left atrioventricular valve at 15 years was not significantly different (78.8 +/- 6.8 vs. 90.6 +/- 4.7%; p = 0.23). Risk factor analysis identified an RPI >6.0 WU/m(2) as a risk for early death. CONCLUSION: By operating on the patients with complete AVSD earlier and not excluding patients with Down syndrome, recent results had definitely improved over the last decades. Despite this positive result, a high RPI exceeding 6 WU/m(2) still remains a risk factor for early mortality independent of early primary repair.

Cardiac retransplantation: is it justified in times of critical donor organ shortage? Long-term single-center experience.

OBJECTIVE: Survival after heart transplantation has improved significantly over the last decades. There are a growing number of patients that require cardiac retransplantation because of chronic allograft dysfunction. With regard to the critical shortage of cardiac allograft donors the decision to offer repeat heart transplantation must be carefully considered. METHODS: Since 1983 a total of 807 heart transplantations have been performed at our institution. Among them 41 patients received cardiac retransplantation, 18 patients because of acute graft failure and 23 because of chronic graft failure. Data were analyzed for demographics, morbidity and risk factors for mortality. The acute and chronic retransplant group was compared to those patients undergoing primary transplantation. RESULTS: The mean interval between primary transplantation and retransplantation was 1.9 days in the acute and 6.7 years in the chronic retransplant group. Mean follow-up was 6.9 years. Baseline characteristics were similar in the primary and retransplant group. Actuarial survival rates at 1, 3, 5 and 7 years after primary cardiac transplantation compared to retransplantation were 83, 78, 72 and 64% vs 53, 50, 47 and 36%, respectively (p<0.001). Early mortality after acute retransplantation was significantly higher compared to late retransplantation (10/18, 55.6% vs 4/23, 17.4%, p=0.011). Major causes of death were acute and chronic rejection, infection and sepsis. CONCLUSIONS: Cardiac retransplantation is associated with lower survival rates compared to primary transplantation. However, results after retransplantation in chronic graft failure are significantly better compared to acute graft failure. Therefore, we consider cardiac retransplantation in chronic graft failure a justified therapeutic option. In contrast, patients with acute graft failure seem to be inappropriate candidates for cardiac retransplantation.

Surgical repair of aortico-left ventricular tunnel arising from the left aortic sinus.

Aortico-left ventricular tunnel is a rare congenital cardiac defect, which bypasses the aortic valve via the paravalvar connection from the left ventricle to the aorta. In most of the cases, the tunnel arises from the right aortic sinus. We herein report a case of aortico-left ventricular tunnel, of which the aortic orifice was arising from the left aortic sinus, requiring special attention for avoiding left coronary artery injury at the time of surgical repair.

Somatic development long after the Fontan operation: factors influencing catch-up growth.

OBJECTIVE: As mortality and morbidity after the Fontan operation has improved, long-term outcome, including developmental aspects, have become more important. To understand the long-term effects of this operation, we followed somatic development for up to 15 years. METHODS: We evaluated 90 patients who underwent the Fontan operation between 1984 and 2004 (mean follow-up, 11.8 +/- 4.2 years). The modified Fontan operations were atriopulmonary anastomosis (n = 19) and total cavopulmonary connection (n = 71). Mean age at the time of surgical intervention was 5.5 +/- 4.8 years. Weight, height, and body mass index were evaluated preoperatively and postoperatively and given as percentiles on a normal growth curve. RESULTS: Postoperative weight, height, and body mass index reached the 47.2 +/- 35.6, 37.9 +/- 30.4, and 41.6 +/- 31.2 percentiles, which were significantly better than preoperative values (the 21.6 +/- 25.9, 25.9 +/- 25.7, and 20.0 +/- 25.1 percentiles). Although neither early surgical intervention nor anatomic features affected postoperative growth, early Fontan completion demonstrated better somatic development in subgroups of tricuspid atresia. Prior bidirectional Glenn shunting provided better weight gain before the Fontan operation. Prior atrioseptectomy, central shunt, and pulmonary artery reconstruction were associated with impaired somatic development. Reoperation and catheter-based intervention improved somatic development. CONCLUSIONS: Long-term catch-up growth can be observed in patients after the Fontan operation. Early volume-unloading procedures might lead to better somatic growth. Prior atrioseptectomy, central shunt, and pulmonary artery reconstruction are associated with impaired weight and height gain, implying that the severity of the underlying diseases affects postoperative somatic development.

Heart-lung transplantation in a 14-year-old boy with Alström syndrome.

We present a 14-year-old boy who suffered from progressive biventricular cardiac failure and secondary pulmonary artery hypertension associated with the rarely seen Alström syndrome. The boy underwent successful heart-lung transplantation. We conclude from this report that heart-lung transplantation in patients with Alström syndrome is a viable therapeutic option in select cases.

Symptom experience after lung transplantation: impact on quality of life and adherence.

BACKGROUND: Patients' perceptions of immunosuppression- related symptom experience may impact on quality of life (QoL) and medication adherence. METHODS: A total of 308 lung transplant recipients were screened for study inclusion. Two hundred eighty-seven patients (response rate 93%) completed a 91-item questionnaire consisting of subscales focusing on symptom experiences (frequency and distress), and adherence. QoL was assessed by a 40-item standardized instrument. Impact of symptom experiences on QoL and adherence were assessed. Potential determinants of immunosuppression induced symptom experiences were evaluated. RESULTS: The most frequent reported symptoms were tremor (70%) and hirsutism (68.1%), whereas Cushingoid appearance (38.6%) and muscle weakness (31.9%) appeared to be the most distressing symptoms. Women (p < 0.001) and younger patients (<40 yr; p < 0.0001) reported a significantly higher level of symptom experience compared with their counterparts respectively. Symptom experiences negatively influenced QoL in all dimensions (p < 0.006). Those who described experiencing adverse effects reported significantly more "drug holidays" (p < or = 0.004) compared with those reporting minor frequent adverse effects. Patients' self-reported strategies to reduce adverse effects were to postpone medication intake (30%), to drop doses (8%), or to reduce doses (9%). CONCLUSIONS: This study establishes a relationship between patients' perceptions of immunosuppression-related symptom experiences and the impact on QoL and adherence. Immunosuppression is accompanied by significant adverse effects in both symptom frequency and distress. Most frequently experienced symptoms do not necessarily have the greatest impact on perceived distress, and vice versa. High levels of adverse effects tend to negatively influence patients' QoL and adherence. Future research is required to understand the relationship of these complex variables.

Heart-lung and lung transplantation in grown-up congenital heart disease: long-term single centre experience.

OBJECTIVE: Because of considerable progress in paediatric cardiac surgery life expectancy of patients with congenital heart disease (CHD) has improved significantly over the years. There are a growing number of adults with CHD presenting with progressive decline of cardiopulmonary function and Eisenmenger's syndrome. We analysed our experience with heart-lung and lung transplantation in this patient group. METHODS: Since 1988, a total of 46 heart-lung transplantations and 5 double lung transplantations have been performed in adults with CHD at our institution. Underlying diagnoses were: ventricular septal defect, atrial septal defect, persistent ductus arteriosus and others. Pulmonary hypertension was present in all patients. Twelve patients had undergone previous cardiac procedures. All patients were included in this retrospective analysis. Mean follow up was 5.1+/-4.7 years. Patient survival was estimated with the Kaplan-Meier method and analysed using the log-rank test. RESULTS: Thirty-day mortality was 11.8% (n=6). Survival was 80% at 1 year, 69% at 5 years and 53% at 10 years. Major causes of death were infection and sepsis, chronic rejection, initial graft failure and acute rejection. Compared to the overall mortality after lung and heart-lung transplantation for other indications at our institution there was no significant difference (1 year, 5 years, 10 years: 76%; 60%; 45%), but a tendency towards a better long-time survival of the CHD patients. CONCLUSIONS: Lung and heart-lung transplantation can be performed with an acceptable risk and a favourable long-term outcome in patients with grown-up CHD. Careful patient selection and planning of the surgical strategy is essential in this high-risk patient population.

Valve-sparing operation for aortic root aneurysm late after Mustard procedure.

The aortic valve reimplantation technique leads to excellent clinical outcome in patients with aortic valve incompetence and aneurysms of the ascending aorta. This technique is now applied for aneurysms of ascending aorta, aortic dissection type A, and even dilatation of pulmonary autograft after the Ross operation. We report a case of aortic root dilatation late after a Mustard operation for transposition of the great arteries that was successfully managed by valve-sparing aortic root reimplantation.

Evaluation of 188 consecutive homografts implanted in pulmonary position after 20 years.

OBJECTIVE: Homografts are considered the gold standard for right ventricular outflow tract reconstruction. Their long-term durability is limited, and alternatives became available. We evaluate their long-term hemodynamic performance to permit comparisons with alternative devices. METHODS: Between 1985 and 2004, 188 homografts were implanted in pulmonary position at our institution. Mean patient age was 24.8 years (range 2 days-75 years); 56 were female and 132 male. Total follow-up time was 1073 years. Fifty-eight percent were Ross procedures (mean age 31.5 years) and 42% were different procedures (mean age 15.6 years); main diagnoses were tetralogy of Fallot (48%), truncus arteriosus (14%), transposition of the great arteries (11%). Twenty-six percent were redo implantations. We evaluated freedom from death, explantation, insufficiency, relevant gradient, degeneration, and the interval between diagnosis of degeneration and therapeutic procedure (therapeutic gap). Results were stratified by indication, age, history, homograft size, and origin. RESULTS: Ten-year-freedom-from explantation was 82% in homografts >19 mm and 45% in smaller ones. Ten-year freedom from degeneration was 68% after Ross procedure and 25% after other operations; it was 83% in patients older than 10 years at implantation and 51% in younger ones. 'Non-Ross-procedure' and 'implantation age below 10 years' were the only independent risk factors for degeneration. The observed trend towards therapeutical gap reduction was not statistically significant. CONCLUSIONS: Homograft implantation in the pulmonary position can be performed with good long-term freedom from explantation. However, freedom from degeneration is a matter of concern. Therefore, alternative valved conduits are required especially for pediatric patients.

Safety of the use of Tissucol Duo S in cardiovascular surgery: retrospective analysis of 2149 patients after coronary artery bypass grafting.

OBJECTIVE: Fibrin sealant is widely used in almost all fields of surgery and has proved to be an effective therapeutic tool in cardiothoracic surgery. Nevertheless, there have been concerns about early bypass graft occlusion associated with the use of fibrin glue. This analysis has been performed to assess the risks and benefits of Tissucol Duo S in coronary artery bypass grafting (CABG) surgery. METHODS: Two thousand one hundred forty-nine patients were included in this retrospective study, 879 (40.9%) were intra-operatively treated with Tissucol Duo S fibrin glue, 1270 (59.1%) did not receive fibrin glue (control group). Patient characteristics were documented according to the EuroScore. Intra- and postoperative data were collected. Primary endpoint of this study was the 30-day all-cause mortality rate in the Tissucol Duo S treated group compared to the control group. RESULTS: Mean age was 66.6+/-9.4 years, 76.3% of the patients were male. There was an increased 30-day-mortality rate in the Tissucol Duo S group compared to the control group (8.5 vs 3.5%, p<0.001). In order to determine if and to what extent the apparent fibrin effect might be due to confounding effects from covariates, an adjustment for potential confounding was done. However, multivariable adjustment did not reduce the risk of fibrin glue below an odds ratio of 2.2. CONCLUSION: Although the apparent increase in mortality risk associated with the use of fibrin glue could not be eliminated statistically, we consider Tissucol Duo S fibrin glue a safe and effective therapeutic tool in CABG surgery when it is applied correctly. Due to the retrospective character of this study some detailed information about the indication for the use of fibrin glue and its application is missing which may be important cofactors for mortality. For further clarification a prospective randomized study may be useful.