Juergen Bauer

Stenting of the Arterial Duct and Banding of the Pulmonary Arteries

Background—Outcome of patients with hypoplastic left heart (HLH) is mainly influenced by the successful first-step palliation according to the Norwood procedure. An alternative approach is heart transplantation (HTX). The feasibility of ductal stenting in newborns with duct-dependent systemic blood flow and bilateral pulmonary artery banding has been reported. But it remains to be elucidated whether this approach allows a new strategy for patients with HLH. Methods and Results—In patients with various forms of HLH (n=11) and prostaglandin E-1 administration, ductal stenting was performed with balloon expandable Jo stents or Saxx stents. Bilateral pulmonary artery banding was surgically accomplished 1 to 3 days after the transcatheter procedure. Unrestricted blood flow through the interatrial septum was secured by balloon dilatation atrial septotomy, as required. Interventional procedures were performed with no mortality. Stent and ductal patency were achieved for up to 331 days. Two patients underwent HTX, and 8 patients had a palliative 1-stage procedure with reconstruction of the aortic arch and bidirectional cavopulmonary connection at the age of 3.5 to 6 months. There were 2 deaths. One patient with preoperative right heart failure died after the reconstructive surgery, and 1 patient died 4 months after ductal stenting and bilateral banding awaiting HTX. Conclusions—The present study is the first clinical trial showing that stenting the duct followed by bilateral pulmonary artery banding in newborns with HLH allows the combination of neoaortic reconstruction, which is part of first-stage palliation of HLH, with the establishment of a bidirectional cavopulmonary connection. Additionally, it allows the chance for HTX after extended waiting periods.

Hybrid Transcatheter–Surgical Palliation

The outcome of patients with hypoplastic left heart (HLH) is determined by many factors, particularly by the first-step palliative procedure in newborns undergoing the Norwood procedure, its Sano modification, or, rarely, through challenging biventricular repairs. Duct stenting combined with bilateral pulmonary artery banding (PAB) is a new method employed as an alternative first-step approach in a number of centers worldwide. We describe this interventional–surgical “hybrid approach” as an additional strategy for the treatment of newborns with HLH syndrome and HLH complex. Between 1998 and April, 2006, 58 newborns underwent ductal stenting and bilateral PAB. These patients underwent surgical bilateral PAB initially, followed by percutaneous duct stenting; the only exception to this were patients in whom duct stenting was performed as a rescue procedure. Various balloon-expandable and self-expandable stents with different widths and lengths were used during the 8-year period of this study. Balloon dilatation of the atrial septum was performed when indicated. This included 5 patients in whom the atrial septum was stented. Aortic arch reconstruction (AAR) combined with a bidirectional cavopulmonary connection (BCPC) was performed at a median age of 4.8 months (range, 2.6–7.5), and total cavopulmonary connection (TCPC) was performed at a median age of 3.1 years (range, 2.5–4). Nine patients were listed for heart transplantation (HTX) and transplanted with AAR when a donor heart was available. Depending on growth of left ventricular structures, biventricular repair (BVR) was performed at a median age of 7.1 months (range, 3.5–10). Overall, 8 of 58 patients (13.8%) treated by the transcatheter–surgical hybrid approach died during the study period. The mortality rate for duct stenting was 1.7% (l/58), and it was 1.7% for bilateral PAB as well. Twenty-seven patients received an AAR/BCPC; 2 of them died (7.4%). Additionally, 1 of 2 patients with AAR/BCPC died while on the waiting list for HTX, resulting in a total mortality rate of 11% with an actuarial survival rate of 89%. One patient is still awaiting AAR + BCPC. Three patients died while on the waiting list for HTX despite successful bilateral PAB and duct stenting. The 30-day mortality rate for TCPC (n = 11), HTX (n = 8), and 18 patients with BVR was 0. The actuarial survival rate for patients with BVR is 93%. Postnatal transcatheter–surgical hybrid palliation expands the surgical options for newborns with HLH. Using hybrid palliation, Norwood stage I operation can be avoided in the neonatal period, the waiting period for children scheduled for cardiac transplantation can be extended, and observation for left ventricular growth suitable for biventricular repair as well.

Stent implantation in the ductus arteriosus for pulmonary blood supply in congenital heart disease

Indications for catheter‐based interventions in patent ductus arteriosus (PDA)‐dependent pulmonary blood flow have yet to be defined. The aim of this study was to assess the acute and midterm outcome after stent implantation in the PDA. Between 1996 and 2002, ductal stenting was performed in 21 neonates and infants (14 females/7 males) to maintain pulmonary blood supply in cyanotic congenital heart disease (CHD). Balloon‐expandable stents were implanted in the PDA with a final diameter of 4–5 mm without procedural deaths. Stent patency was achieved for 8–1,130 days (median, 142). Reintervention was necessary in nine patients. Overall survival rate after 6 years was 86%. Two neonates died a few days after the procedure due to right heart failure not related to PDA stenting. Corrective surgery was possible in six patients. An additional aortopulmonary shunt was needed in three patients; Fontan type operations were performed in six. One patient died after bidirectional Glenn shunt, another five reached palliation by additional perforation of the atretic valve/balloon valvuloplasty, and two are awaiting surgery. We conclude that in many patients with cyanotic CHD, especially in those with ductal pulmonary perfusion and additional forward flow from the right ventricle, ductal stenting is an effective transcatheter approach. Morphology of the PDA predicts the risk of restenosis and necessity of reintervention. Growth of the pulmonary vascular bed allows corrective or palliative surgery, and some patients can be cured by the intervention alone. Catheter Cardiovasc Interv 2004;61:242–252.

A regenerative strategy for heart failure in hypoplastic left heart syndrome: intracoronary administration of autologous bone marrow-derived progenitor cells.

Novel surgical strategies have dramatically improved the initial outcome of newborns with hypoplastic left heart syndrome. However, the single systemic right ventricle remains a major challenge, with limited effectiveness of pharmacologic therapy. The present case documents that the intracoronary administration of autologous bone marrow-derived progenitor cells is technically feasible in a critically ill infant with hypoplastic left heart syndrome and severe heart failure after a hybrid comprehensive stage II procedure. Cell therapy might represent an option before heart transplantation in children with single ventricle physiology presenting with severe heart failure.

Potts Shunt and Atrial Septostomy in Pulmonary Hypertension Caused by Left Ventricular Disease

We report a 20-year-old patient with Shones complex and severe diastolic dysfunction of his small left ventricle (LV) in whom severe pulmonary hypertension and biventricular failure developed while he was awaiting combined heart-lung transplantation. We performed a percutaneous balloon atrial septostomy and a modified Potts shunt (13-mm graft from left pulmonary artery to descending aorta), with the aim of decompressing the hypertensive right ventricle (RV) by reducing left ventricular preload and left atrial hypertension. The procedures were uneventful. The patients condition improved rapidly and biventricular function was restored. In contrast to a Potts shunt in other conditions, patients with pulmonary hypertension caused by left ventricular disease may benefit from an additional atrial left-to-right shunt.

Renal function in children with heart transplantation after switching to CNI-free immunosuppression with everolimus

Behnke‐Hall K, Bauer J, Thul J, Akintuerk H, Reitz K, Bauer A, Schranz D. Renal function in children with heart transplantation after switching to CNI‐free immunosuppression with everolimus. 
Pediatr Transplantation 2011: 15: 784–789.

Pediatric heart allocation and transplantation in Eurotransplant

Pediatric heart allocation in Eurotransplant (ET) has evolved over the past decades to better serve patients and improve utilization. Pediatric heart transplants (HT) account for 6% of the annual transplant volume in ET. Death rates on the pediatric heart transplant waiting list have decreased over the years, from 25% in 1997 to 18% in 2011. Within the first year after listing, 32% of all infants (<12 months), 20% of all children aged 1–10 years, and 15% of all children aged 11–15 years died without having received a heart transplant. Survival after transplantation improved over the years, and in almost a decade, the 1‐year survival went from 83% to 89%, and the 3‐year rates increased from 81% to 85%. Improved medical management of heart failure patients and the availability of mechanical support for children have significantly improved the prospects for children on the heart transplant waiting list.

Perioperative management in pediatric heart transplantation from 1988 to 2001: Anesthetic experience in a single center

Abstract:  Pediatric cardiac transplantation is currently an accepted option for end‐stage heart disease and congenital cardiac malformations. This report focuses on the anesthetic perioperative management in 12 yr. From 1988 to 2001 we performed 90 heart transplantations in 88 children, infants and neonates. The pediatric heart transplant program of the childrens heart center at our university hospital started in June 1988 with the transplantation of a 2‐yr‐old boy who was suffering from congenital heart disease. Since then, 88 transplants have been performed. We divided our patients into two groups. Group 1 ranged from 1988 to 1996 and Group 2 from 1997 to 2001. The patient characteristics have not significantly changed over the years in our institution. At the time of transplantation, mean age of the patients was 2.6 ± 4.3 yr from the period of 1988–1996 and 2.5 ± 4.1 yr from 1997 to 2001. Since 1988, 90 transplants (Tx) in 88 patients have been performed. Two patients needed re‐Tx within 2 days after the initial operation because of primary graft failure. Indications for Tx were congenital heart disease (n = 67) and cardiomyopathy (n = 21). In the subgroup of the patients suffering from congenital heart disease there were 46 with the diagnosis of HLHS, followed by endocardial fibroelastosis (n = 7); the remaining 14 patients had other complex cardiac malformations and some underwent corrective palliative cardiac surgery before Tx. Sixty‐three patients were younger than 1 yr of age and only five children were older than 10 yr. Twenty‐three percent of the patients on the waiting list died before Tx was possible. The overall survival rate was 79% at 1 yr and 73% at 5 and 10 yr. Infants with HLHS had a lower probability of survival after 5 yr compared with other diagnosis (69% vs. 84%). Until now 21 patients have died after Tx. The duration of anesthesia, time of CPB and the age at the time of surgery decreased over the years. It is always a challenge for the anesthesiologist to treat these patients with pulmonary hypertension as one of the most critical risks in this group of patients. The preventive therapy with vasodilators as well as the availability of mechanical assist devices before and after heart transplantation reduces the effects of transitional pulmonary hypertension and prevents the development of post‐operative right heart failure.

Value of soluble adhesion molecules and plasma coagulation markers in assessing transplant coronary artery disease in pediatric heart transplant recipients

Abstract:  Background:  With an increasing number of heart transplantations (HTx) performed in children and an extended long‐term survival of these patients, the importance of transplant coronary artery disease (TCAD) rises in this group of transplant recipients. Reliable serum markers for diagnosis or non‐invasive monitoring of this disease in pediatric transplant recipients are still missing. We studied the systemic expression of adhesion molecules as well as plasma coagulation markers and the occurrence of TCAD and/or rejection in pediatric heart transplant recipients.

Magnetic resonance imaging of an aortopulmonary window type three, with aortic atresia and interrupted aortic arch type B

A 3700-GRAM GIRL BORN AT 39 WEEKS AND 4 days of gestation presented with a murmur on day 2 of life. The child appeared phenotypically normal with no dysmorphic features. An echocardiographic examination revealed a situs solitus with D-loop of the great vessels and aortic atresia with a large aortopulmonary window type three, and an interrupted left aortic arch type B. The descending aortic arch was interrupted below the left common carotid artery. A common feature of interrupted aortic arch is that the ascending aorta goes straight up cranially and does not begin to curve at the brachiocephalic vessel giving a ‘‘Y’’ or ‘‘two-pronged fork’’ appearance, with the aorta dividing into the first two brachiocephalic vessels – that is, innominate or right common carotid and left common carotid – Figures 1 and 2. The magnetic resonance imaging demonstrates this sign and the complex anatomy clearly, with a three-dimensional reconstruction of this rare defect, which is important for surgical planning. Shortly after diagnosis, a prostaglandin E1 infusion was commenced, as well as diuretics for preload reduction. On day 12 of life, bilateral pulmonary artery banding with a 4-millimetre polytetrafluoroethylene band was performed for reduction of pulmonary artery blood flow. At operation, the thymus was noted. The operation was uneventful and the electrocardiogram post surgery showed no signs of ischaemia. One day after surgery, the child developed a circulatory arrest and the post-mortem examination revealed fresh myocardial necrosis