Ina Michel-Behnke

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.

Bioabsorbable metal stents for percutaneous treatment of critical recoarctation of the aorta in a newborn

In neonates and infants with critical aortic coarctation, balloon angioplasty is considered for rescue therapy of heart failure. The use of conventional stents is limited because of further vessel growth, the need of redilation, and later surgical removal. However, a novel bioabsorbable magnesium stent (AMS) might overcome such restrictions of vessel stenting in newborns. Presented is the first use of an AMS for acute treatment of a newborn with severely impaired heart function due to a long segment recoarctation after a complex surgical repair. We hypothesize that bioabsorbable stents will dramatically change the treatment of coarctation and recoarctation in newborns.

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.

Clinical Outcomes of Palliative Surgery Including a Systemic-to-Pulmonary Artery Shunt in Infants With Cyanotic Congenital Heart Disease Does Aspirin Make a Difference?

Background— Aspirin (ASA) often is used to prevent thrombosis in infants with congenital heart disease after placement of a systemic-to–pulmonary artery shunt, but its effect on outcomes is unknown. Methods and Results— The present multicenter study prospectively collected data on 1-year postoperative rates of death, shunt thrombosis, or hospitalization age <4 months for bidirectional Glenn/hemi-Fontan surgery in 1004 infants. The use and dose of ASA were recorded. Kaplan-Meier event rates were calculated for each event and the composite outcome, and a Cox regression model was constructed for time to event. Model terms were ASA use and type of surgery, with adjustment for age at surgery. Diagnoses were hypoplastic left heart syndrome (n=346), tricuspid atresia (n=103), tetralogy of Fallot (n=127), pulmonary atresia (n=177), heterotaxy syndrome (n=38), and other (n=213). There were 344 shunts placed without cardiopulmonary bypass (closed shunt), 287 shunts with bypass (open shunt), 323 Norwood procedures, and 50 Sano procedures. Overall, 80% of patients received ASA. One-year postoperative events rates were high: 38% for the composite end point, 26% for death, and 12% for shunt thrombosis. After the exclusion of patients with early mortality, patients receiving ASA had a lower risk of shunt thrombosis (hazard ratio, 0.13; P=0.008) and death (closed shunt: hazard ratio, 0.41, P=0.057; open shunt: hazard ratio, 0.10, P<0.001; Norwood: hazard ratio, 0.34, P<0.001; Sano: hazard ratio, 0.68, P=NS) compared with those not receiving ASA. Conclusions— The morbidity and mortality for infants after surgical placement of a systemic-to–pulmonary artery shunt are high. ASA appears to lower the risk of death and shunt thrombosis in the present observational study.

Stenting of the ductus arteriosus and banding of the pulmonary arteries: basis for various surgical strategies in newborns with multiple left heart obstructive lesions

Objective: To present an institutional experience with stent placement in the arterial duct combined with bilateral banding of the pulmonary artery branches as a basis for various surgical strategies in newborns with hypoplastic left heart obstructive lesions. Design: Observational study. Setting: Paediatric heart centre in a university hospital. Patients: 20 newborns with various forms of left heart obstructive lesions and duct dependent systemic blood flow. Interventions: Patients underwent percutaneous ductal stenting and surgical bilateral pulmonary artery banding. Atrial septotomy by balloon dilatation was performed as required, in one premature baby by the transhepatic approach. Main outcome measures: Survival; numbers of and reasons for palliative and corrective cardiac surgery. Results: One patient died immediately after percutaneous ductal stenting. One patient died in connection with the surgical approach of bilateral pulmonary banding. Stent and ductal patency were achieved for up to 331 days. Two patients underwent heart transplantation and two patients died on the waiting list. Ten patients had a palliative one stage procedure with reconstruction of the aortic arch and bidirectional cavopulmonary connection at the age of 3.5–6 months. There was one death. One patient is still awaiting this approach. Two patients received biventricular repair. In one, biventricular repair will soon be provided. Conclusions: Stenting the arterial duct combined with bilateral pulmonary artery banding in newborns with hypoplastic left heart or multiple left heart obstructive lesions allows a broad variation of surgical strategies depending on morphological findings, postnatal clinical conditions, and potential ventricular growth.

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.

Thrombosis in the critically ill neonate: incidence, diagnosis, and management

Among children, newborn infants are most vulnerable to development of thrombosis and serious thromboembolic complications. Amongst newborns, those neonates who are critically ill, both term and preterm, are at greatest risk for developing symptomatic thromboembolic disease. The most important risk factors are inflammation, DIC, impaired liver function, fluctuations in cardiac output, and congenital heart disease, as well as exogenous risk factors such as central venous or arterial catheters. In most clinically symptomatic infants, diagnosis is made by ultrasound, venography, or CT or MRI angiograms. However, clinically asymptomatic vessel thrombosis is sometimes picked up by screening investigations or during routine imaging for other indications. Acute management of thrombosis and thromboembolism comprises a variety of approaches, including simple observation, treatment with unfractionated or low molecular weight heparin, as well as more aggressive interventions such as thrombolytic therapy or catheter-directed revascularization. Long-term follow-up is dependent on the underlying diagnosis. In the majority of infants, stabilization of the patients’ general condition and hemodynamics, which allows removal of indwelling catheters, renders long-term anticoagulation superfluous. Nevertheless, in certain types of congenital heart disease or inherited thrombophilia, long-term prophylaxis may be warranted. This review article focuses on pathophysiology, diagnosis, and acute and long-term management of thrombosis in critically ill term and preterm neonates.

rFVIIa in the treatment of persistent hemorrhage in pediatric patients on ECMO following surgery for congenital heart disease.

Background:  Patients who require extracorporeal membrane oxygenation (ECMO) postsurgery for congenital heart disease (CHD) frequently experience severe bleeding episodes. Whereas recombinant‐activated factor VII (rFVIIa) has proven efficacy in counteracting intractable hemorrhage in various scenarios, its use in patients on ECMO is limited by the increased risk for thrombotic events.

Device closure of ventricular septal defects by hybrid procedures: A multicenter retrospective study†

Objective: This study reports on the feasibility, efficacy, and outcome of hybrid procedures to close ventricular septal defects (VSD), reflecting the experience of 11 centers in Germany, Austria, and Switzerland. Background: Beating heart closure of VSD has attracted interest in small infants, complex anomalies and postinfarction scenarios where patients are at high risk during surgery. Perventricular or intraoperative device placement allows access to the lesions where percutaneous delivery is limited. Methods: Between December 2001 and April 2009, placement of Amplatzer septal occluders was attempted in 26 patients. The defects were located in the perimembranous (n = 5) and muscular septum (n = 21). In 20 patients, a perventricular approach was used, and, in six, the occluders were placed under direct visualization being part of a complex heart surgery. Results: In 23 of 26 procedures, device placement was successful (88.5%). The mean defect size was 7.8 mm (range, 3.5–20). The occluder types were perimembranous VSD occluder (n = 4), muscular VSD occluder (n = 20), postinfarct VSD occluder (n = 1), and ASD occluder (n = 1) with a ratio device/defect of 0.9–2.4 (median 1.15). Device removal was necessary in three due to arrhythmia, malpositioning, and additional defects. Pericardial effusion occurred once. In the remaining 22 patients, there were no procedure or device‐related complications. During mean follow up of 1.4 years (range, 1 day–3.9 years), a residual shunt that was more than trivial was observed in one patient out of 21 successful procedures. Conclusions: Perventricular and intraoperative device closure of VSD is as effective as a surgical patch and averts the increased morbidity of conventional surgical repair in a subgroup of high‐risk patients.

Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK

The European Paediatric Pulmonary Vascular Disease (PVD) Network is a registered, non-profit organisation that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of paediatric pulmonary hypertensive vascular disease, including specific forms such as pulmonary arterial hypertension (PAH)-congenital heart disease, pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, persistent PH of the newborn, and related cardiac dysfunction. Methods The writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990–2015) and held five face-to-face meetings with votings. Clinical trials, guidelines, and reviews limited to paediatric data were searched using the terms ‘pulmonary hypertensioń’ and 5–10 other keywords, as outlined in the other nine articles of this special issue. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on paediatric data only, or on adult studies that included >10% children. Results A total of 9 original consensus articles with graded recommendations (COR/LOE) were developed, and are summarised here. The topics included diagnosis/monitoring, genetics/biomarker, cardiac catheterisation, echocardiography, cardiac magnetic resonance/chest CT, associated forms of PH, intensive care unit/ventricular assist device/lung transplantation, and treatment of paediatric PAH. Conclusions The multipaper expert consensus statement of the European Paediatric PVD Network provides a specific, comprehensive, detailed but practical framework for the optimal clinical care of children with PH.