Decellularized aortic homografts for aortic valve and aorta ascendens replacement.

Objectives: The choice of valve prosthesis for aortic valve replacement (AVR) in young patients is challenging. Decellularized pulmonary homografts (DPHs) have shown excellent results in pulmonary position. Here, we report our early clinical results using decellularized aortic valve homografts (DAHs) for AVR in children and mainly young adults.

Use of latissimus dorsi pedicled myocutaneous flap for reconstruction in the chest area of an 8-month-old female infant with ectopia cordis.

Ectopia cordis (EC) is characterized by a complete or partial malposition of the heart outside the thorax. Despite the interdisciplinary treatment, the repair of EC is still very difficult and offers new surgical challenges because of its complexity and various combinations with other anomalies. We report the successful outcome after using a pedicled latissimus dorsi flap in reconstructive surgery in the setting of chronic wound dehiscence in an 8-month-old female infant born with a thoracic EC and omphalocele.

Surgical Treatment of Thoracic Aortic Aneurysms in Patients with Congenital Heart Disease.

Background: This study we evaluate our results for surgical treatment of thoracic aortic aneurysms in patients with congenital heart disease.

Patients And Methods: Fifty patients aged between 12 and 71 years were treated for 51 thoracic aortic aneurysms. Forty-four of the patients developed aneurysms in the ascending aorta and seven developed aneurysms in the descending aorta.

Semilunar valve replacement with decellularized homograft after Damus-Kaye-Stansel anastomosis and fontan procedure.

We describe a patient in whom severe neoaortic (anatomic pulmonary) valve regurgitation developed late after Damus-Kaye-Stansel anastomosis and Fontan operation. The valve was replaced with a fresh decellularized homograft, which we developed and applied in the normal pulmonary and aortic position in more than 100 patients. During follow-up of more than 2 years, the valve function is excellent, and no infectious or thromboembolic complications were seen.

A modified technique of Konno aortoventriculoplasty for redo aortic valve replacement procedures.

This article puts forward a modified technique of Konno aortoventriculoplasty for repeat procedures. After incision of the ventricular septum, this approach involves aortic valve replacement using a mechanical valved conduit, reimplantation of the coronary arteries, and graft replacement of the ascending aorta. This modification allows the removal of the diseased ascending aortic wall caused by a previous patch enlargement or poststenotic dilation.

Surgical repair of partial anomalous pulmonary venous connection shunting from left atrium to innominate vein.

Partial anomalous pulmonary venous connection (PAPVC) causes a left-to-right shunt from the anomalous pulmonary vein (PV) to a systemic vein. We report an uncommon adult case of PAPVC, in which the left upper PV drained into both the innominate vein and the left atrium (LA), demonstrating retrograde shunting from the LA to the innominate vein. The anomaly was surgically repaired.

Aortic root replacement in a patient with bicuspid pulmonary valve late after arterial switch operation.

Mild to moderate neoaortic root dilatation late after arterial switch operation (ASO) is a well-documented morbidity, but rarely needs surgical replacement. Here, we report on a 22-year-old man, who developed marked dilatation of neoaortic root and needed an aortic root replacement with mechanical conduit. His aortic valve was bicuspid.

Removal of temporary pacemaker after cardiac surgery in infants: A harmless procedure?

External pacemakers (PM) via temporary epicardial leads are routinely applied to infants and children during heart surgery, which usually, after an uneventful post surgical course, can be removed without complications. We report about two infants with complex congenital heart defects after cardiac surgery (arterial switch and Mustard operation for Transposition of the great arteries). Intraoperative these patients received temporary epicardial PM wires.

Risk factors for distal Contegra stenosis: results of a prospective European multicentre study.

Objectives The EUCon study was designed to identify risk factors for distal anastomotic stenosis after bovine jugular vein (Contegra) implantation in children. Methods Between March 2006 and August 2008, 104 devices were implanted in nine European centers. Preoperative, intraoperative, and follow-up data (at discharge, 6, 12, 24 months) including standardized echocardiography were prospectively registered, source data verified and collected in a central database.

Use of fresh decellularized allografts for pulmonary valve replacement may reduce the reoperation rate in children and young adults: early report.

Background: Degeneration of xenografts or homografts is a major cause for reoperation in young patients after pulmonary valve replacement. We present the early results of fresh decellularized pulmonary homografts (DPH) implantation compared with glutaraldehyde-fixed bovine jugular vein (BJV) and cryopreserved homografts (CH).

Methods And Results: Thirty-eight patients with DPH in pulmonary position were consecutively evaluated during the follow-up (up to 5 years) including medical examination, echocardiography, and MRI.

Intrapericardial migration of dislodged sternal struts as late complication of open pectus excavatum repairs.

Unlabelled: We present a case of sternal steel strut dislodgement and migration in a patient undergoing Ravitch repair for pectus excavatum (PE) 37 years ago. Broken struts perforated the right ventricle and right ventricular outflow tract (RVOT) and additionally migrated into the left upper lobar bronchus.Dislodged sternal struts represent rare complications after surgical repair of patients suffering from pectus excavatum.

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.

Long-term morbidity and quality of life after surgical repair of transposition of the great arteries: atrial versus arterial switch operation.

Since a growing number of patients after surgical repair of transposition of the great arteries (TGA) survive until adulthood the focus of attention has shifted to the management of associated long-term morbidity and quality of life (QoL). Therefore, we reviewed all patients that underwent surgical repair of TGA at our institution and compared long-term results after atrial and arterial switch operation. Between 1973 and 2000, a total of 302 patients underwent either atrial switch operation (n=222) or arterial switch operation (n=80).

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.

Pulmonary valve replacement in chronic pulmonary regurgitation in adults with congenital heart disease: impact of preoperative QRS-duration and NT-proBNP levels on postoperative right ventricular function.

Background: Chronic severe pulmonary regurgitation (PR) causes progressive right ventricular (RV) dysfunction and heart failure. Parameters defining the optimal time point for surgery of chronic PR are lacking. The present study prospectively evaluated the impact of preoperative clinical parameters, cardiorespiratory function, QRS duration and NT-proBNP levels on post operative RV function and volumes assessed by cardiac magnetic resonance imaging (CMR) in patients with chronic severe PR undergoing pulmonary valve replacement.

Physical stress testing of bovine jugular veins using magnetic resonance imaging, echocardiography and electrical velocimetry.

Bovine jugular veins (BJVs) (Contegra) are valve-bearing pulmonary artery substitutes. Their valves have higher profiles than human pulmonary valves; this might result in less optimal performance. Therefore, we investigated the impact of stress and undersizing on conduit performance with ergometry, echocardiography and magnetic resonance imaging (MRI).

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).

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.

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.