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.

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).

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).

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.

Combined aortic root and right ventricular outflow tract replacement with mechanical conduits in adult patients after repeated surgery for congenital heart disease.

Objective: To manage aortic root aneurysms and right ventricular outflow tract (RVOT) graft dysfunction in adult patients after repeated surgery for congenital heart disease, we performed combined prosthetic aortic root and RVOT replacement.

Methods: The procedure was performed in 5 patients (2 truncus arteriosus, 2 variants of tetralogy of Fallot, and 1 congenital aortic stenosis), aged 23, 24, 27, 29, and 34 years, who presented with progressive dilation of the aortic root and aortic regurgitation as well as RVOT graft dysfunction. All patients had undergone a median of 3 previous operations and this procedure was their third (in 1), fourth (in 3), or fifth (in 1) operation.

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.

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.

Neonatal Kawashima intraventricular repair for Taussig-Bing anomaly with oblique relationship of the great arteries.

An arterial switch operation is considered the procedure of choice for the repair of Taussig-Bing anomaly, because it can be performed in most patients. However, after taking problems such as neoaortic insufficiency or myocardial perfusion disturbances after the arterial switch operation into account, intraventricular repair might be reconsidered as an option in selected patients. We present a case of Taussig-Bing anomaly in a patient with an oblique relationship of the great arteries, which was successfully managed by Kawashima intraventricular repair in the neonatal period, and discuss the feasibility of this option with a review of the literature.

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.

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.

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.

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.

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.

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.

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.

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.

Aortic valve-sparing reimplantation for dilatation of the ascending aorta and aortic regurgitation late after repair of congenital heart disease.

Objective: Aortic regurgitation resulting from progressive dilatation of the aortic root late after surgical correction of congenital heart disease is now widely recognized. We reviewed our experience with aortic valve-sparing operations to access the effectiveness of this approach.

Methods: Three patients, aged 28, 31, and 32 years, had progressive dilatation of the ascending aorta and aortic regurgitation 25, 27, and 28 years, respectively, after surgical repair of complex congenital heart disease.

Coexistent cardiac rhabdomyoma with mitral valve anomaly in patients with tuberous sclerosis: a case report.

Cardiac rhabdomyoma is frequently associated with tuberous sclerosis. However, very few cardiac malformations have been described with tuberous sclerosis. We report a rare case of coexistent cardiac tumor with mitral valve anomaly in a patient with tuberous sclerosis, who developed mitral regurgitation and required a surgical procedure with histological confirmation of cardiac rhabdomyoma.

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.

Clinical outcome of patients 20 years after Fontan operation--effect of fenestration on late morbidity.

Objective: The Fontan operation has been proposed as definitive palliation for an increasing variety of hearts with complex univentricular anatomy, but late morbidity after Fontan operation is still a matter of concern. This retrospective study evaluates the late outcome in patients with Fontan circulation.

Methods: We included 121 consecutive patients that underwent Fontan operation between 1984 and 2004.