Christian Jux

Fifteen-year Single Center Experience with the “Giessen Hybrid” Approach for Hypoplastic Left Heart and Variants: Current Strategies and Outcomes

Presented is a retrospective outcome study of a 15-year single institutional experience with a contemporary cohort of patients with hypoplastic left heart syndrome and complex that underwent a “Giessen Hybrid” stage I as initial palliation. Hybrid approach consisting of surgical bilateral pulmonary artery banding and percutaneous duct stenting with or without atrial septum manipulation was developed from a rescue approach to a first-line procedure. Comprehensive Aristotle score defined pre-operative condition. Fifteen-year follow-up mortality is reported as occurring within the staged univentricular palliation or before and after biventricular repair. Hybrid stage I was performed in 154 patients; 107 should be treated by single ventricle palliation, 33 by biventricular repair (BVR), 7 receivedxa0heart transplantation, and 7 were treated by comfort care, respectively. Overall 34 children died. The Aristotle score (mean value 18.2xa0±xa03) classified for univentricular circulations in newborns did not have statistical impact on the outcome. Two patients died during stage I (1.2xa0%), and the interstage I mortality was 6.7xa0%, and stage II mortality 9xa0%, respectively. Stage III was up to now performed in 57 patients without mortality. At 1xa0year, the overall unadjusted survival of HLHS and variants was 84xa0% and following BVR 89xa0%, respectively. The Fifteen-year survival rate for HLHS and variants was 77xa0%, with no significant impact of birth weight of less than 2.5xa0kg. In conclusion, Hybrid stage I fulfilled the criteria of life-saving approach. In our institution, Hybrid procedure replaced Norwood-staged palliation with a considerable mid- and long-term survival rate. Considering interstage mortality close surveillance is mandatory.

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.

Stent Implantation of the Arterial Duct in Newborns with a Truly Duct‐Dependent Pulmonary Circulation: A Single‐Center Experience with Emphasis on Aspects of the Interventional Technique

INTRODUCTIONnDuctal stenting for pulmonary blood supply in newborns with cyanotic congenital heart disease (CHD) might be a low risk and safe alternative to the surgical aorto-to-pulmonary artery (AP) shunt in dual-source lung perfusion. Ductal stenting in truly duct-dependent pulmonary circulation has not been evaluated.nnnMETHODSnProspective interventional and clinical follow-up trial. Ductal stenting based on variable access sites, a 2-wire technique when crossing a tortuous ductus, and use of premounted coronary stents. Primary outcome measures were procedural success and complication rates presented as early and mid-term results.nnnRESULTSnFrom 2003-2009, 58 duct-dependent newborns underwent ductal stenting; 27 of them were truly duct dependent, 20 had pulmonary atresia (PA)/ventricular septum defect or complex CHD, 4 had PA/intact ventricular septum, 2 had PA with Ebstein anomaly, and 1 had PA with tricuspid atresia. Ductal stenting was performed without procedure-related mortality; 3 of 27 required an acute surgical AP-shunt (stent migration in 1, acute duct obstruction in 2). During mid-term follow-up, 4 of 24 needed an AP-shunt and two others stent redilation. Three patients died prior to follow-up surgery (1 unexpectedly at home and 2 due to syndromatic disease). Fifteen patients received staged univentricular palliation, 8 had a biventricular repair, and 1 is awaiting follow-up operation.nnnCONCLUSIONnDuctal stenting is a feasible, safe, and effective palliation in newborns with truly duct-dependent pulmonary circulation irrespective of duct morphology. Vasucular access from various locations is important for technical success rate. Ductal stenting is a minimally invasive procedure to achieve adequate pulmonary artery growth for subsequent palliative or corrective surgery.

Pulmonary artery banding in infants and young children with left ventricular dilated cardiomyopathy: a novel therapeutic strategy before heart transplantation.

BACKGROUNDnDilated cardiomyopathy (DCM) in childhood has a considerable morbidity and mortality and high incidence of heart transplantation. Pulmonary artery banding (PAB) has been proposed in patients with corrected transposition of the great arteries to retrain the sub-pulmonic left ventricle (LV) and to improve a failing sub-aortic right ventricle. We evaluated the short-term and medium-term effects of PAB in young patients with LVDCM.nnnMETHODSnA retrospective single-center observational study was performed to evaluate the possible benefits of a dilatable surgical PAB in infants and young children with LVDCM.nnnRESULTSnReported are 12 patients (10 infants, 2 toddlers) with LVDCM referred for heart transplant who received a surgical PAB. There were no hospital deaths. Clinical functional status improved in all patients. The pressure gradient across the PAB increased within 20 days from 28 ± 7 to 43 ± 15 mm Hg. The LV ejection fraction increased from 14.5% ± 5% pre-PAB to 27% ± 13% at hospital discharge and to 47% ± 10% at 3 to 6 months. The LV end-diastolic diameter (z-score) decreased (p > 0.001) from 46 ± 6.1 (+7.0 ± 1.3) to 35 ± 15 mm (+3.0 ± 1.3) after 3 to 6 months and to 34 ± 15 mm (+1.3 ± 1.14) after a median age of 2 years (maximum 6.6 years), respectively. Plasma B-type natriuretic peptide levels decreased from 3431 ± 2610 to 288 ± 321 pg/ml at discharge and to 102 ± 96 pg/ml 22 months later. Eight children were subsequently de-banded by transcatheter technique and 6 of them are currently at Ross Heart Failure Classification for Children class I. Two patients, both with non-compaction DCM, deteriorated at 5 and 6 months after PAB debanding and finally died.nnnCONCLUSIONnIn young children with LVDCM and still-preserved right ventricular function, PAB led to an improvement of LV and mitral valve function by ventricular interaction.

Intracoronary bone marrow cell application for terminal heart failure in children.

INTRODUCTIONnIn spite of tremendous progress in the medical and surgical treatment of children with congenital heart disease and dilated cardiomyopathy achieved during the past few decades, for some children a heart transplant remains the only option. Clinically relevant benefits of intracoronary injection of autologous stem cells on cardiac function and remodelling have been demonstrated in adult patients with acute myocardial infarction. Experience with autologous stem cell therapy in children with severe congenital or acquired pump failure is limited to a small number of case reports.nnnMETHOD AND RESULTSnBetween 2006 and 2010, nine severely ill children were treated with intracoronary infusion of autologous bone marrow-derived mononuclear cells as part of a compassionate therapy in our centre. No procedure-related unexpected adverse events occurred. There was one patient on extracorporeal membrane oxygenation who died of haemorrhage unrelated to the procedure; three patients proceeded to heart transplantation once a donor heart became available. The other five patients showed an improvement with respect to New York Heart Association classification (greater than or equal to 1), brain natriuretic peptide serum levels, and ejection fraction.nnnCONCLUSIONnSimilar to adults, intracoronary injection of autologous bone marrow cell is technically feasible and safe for children. On the basis of our data, we propose to perform a pilot study for children with congestive heart failure, to formally assess the efficacy of intracoronary autologous bone marrow cell therapy.

Creation of a functional Potts shunt by stenting the persistent arterial duct in newborns and infants with suprasystemic pulmonary hypertension of various etiologies

BACKGROUNDnThe surgical creation of a Potts shunt has been reported in children with suprasystemic idiopathic pulmonary artery (PA) hypertension (IPAH) refractory to any medical therapy. This surgical approach allows acute decompression of the right ventricle (RV) and thereby avoids ventricular failure. We present 4 newborns and infants with different causes of pulmonary hypertension (PH) in whom interventional stenting of the patent ductus arteriosus (PDA) was used to create a functional Potts shunt.nnnMETHODSnSuprasystemic PH was diagnosed by cardiac catheterization in 2 newborns with complex left heart obstructive lesions, a patient with persistent PH of the newborn (PPHN), and an infant with IPAH and was accompanied by RV dilation and imminent RV failure.nnnRESULTSnStenting of the PDA was performed uneventfully during cardiac catheterization in all patients and led to stabilization of clinical symptoms. The 2 patients with complex cardiac lesions subsequently underwent successful biventricular repair. In the PPHN patient, the stented PDA was closed interventionally when PA pressures dropped and a significant left-to-right shunt occurred. PA pressures in the patient with IPAH remained high while the stented PDA still served as RV decompression in avoidance of lung transplantation.nnnCONCLUSIONSnNewborns and infants with suprasystemic PH of varying etiologies may benefit from the creation of a functional Potts shunt by stenting the PDA. This strategy should be considered in patients with suprasystemic IPAH or PPHN as a temporary or permanent therapy when a PDA can be identified at cardiac catheterization. It may also be beneficial in patients with PH due to left heart obstructions by serving as a bridge to further corrective surgery.

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.

Assessment of pulmonary endothelial function during invasive testing in children and adolescents with idiopathic pulmonary arterial hypertension.

OBJECTIVESnThe purpose of our study was to assess pulmonary endothelial function by vasodilator response to acetylcholine (Ach) administered in segmental pulmonary arteries in children with idiopathic pulmonary arterial hypertension (IPAH). We hypothesized that there was a relationship among pulmonary endothelial response to Ach, severity of the disease, and clinical outcome.nnnBACKGROUNDnIPAH may be associated with pulmonary endothelial dysfunction; however, data regarding the impact of endothelial dysfunction on severity and prognosis of this disease are limited.nnnMETHODSnForty-three children and adolescents (mean age: 10.4 ± 5.5 years) with IPAH were included in the study. Changes in pulmonary blood flow in response to Ach were determined using intravascular Doppler flow measurements. Pulmonary flow reserve (PFR) was calculated as the ratio of pulmonary blood flow velocity in response to Ach relative to baseline values.nnnRESULTSnMean PFR of all patients was 1.58 ± 0.67. Mean follow-up after catheterization was 55.7 ± 41.9 months. Freedom from serious cardiovascular events (lung transplantation or death) was 83% after 2 years, 76% after 3 years, and 57% after 5 years. PFR was related significantly to World Health Organization functional class. Receiver-operating characteristic curves revealed a PFR of 1.4 as the best cutoff value. Kaplan-Meier analysis demonstrated that a PFR of <1.4 was highly predictive for cardiovascular events (log-rank [Mantel Cox] chi-square: 12.49, p < 0.0001).nnnCONCLUSIONSnOur study demonstrates a strong relationship between pulmonary endothelial response to Ach and prognosis of children with IPAH. As an adjunct to the usual testing protocol, this method provides additional information for therapeutic guidance.

Two melodies in concert: Transcatheter double‐valve replacement

Percutaneous pulmonary valve implantation has been established as a valuable treatment option for elder children and adolescents with conduit failure in the right ventricular outflow tract. Transcatheter valve implantation in the tricuspid position is restricted to single case reports. A 26‐year‐old male initially diagnosed with tetralogy of Fallot and hypoplastic pulmonary arteries hitherto underwent a total of five open chest procedures including tricuspid valve replacement with a bioprosthesis and a pulmonary homograft exchange. He now presented with severe right heart failure due to a degenerated pulmonary homograft and calcified, severely stenotic tricuspid bioprosthesis with markedly dilated and reduced right ventricular function. We report on the first successful percutaneous transcatheter double‐valve replacement using two Melody valves in the pulmonary and tricuspid position, respectively.

Large-diameter graft-stent (Advanta V12) implantation in various locations: early results

OBJECTIVESnTranscatheter stent placement carries the risk of cardiovascular aneurysm or rupture. Covered stent implantation reduces these risks. The recently marketed Advanta V12 large-diameter-covered stent is pre-mounted and requires 9 (8)-11 Fr delivery systems. The aim was to report on the early results of the treatment of various cardiovascular obstructions by the implantation of a new polytetrafluoroethylene-covered stent (V12).nnnMETHODSnGraft stents on balloons with a diameter (12, 14, 16 millimetres) sufficient to anchor the stent in various obstructions (congenital aortic coarctation, n = 5; obstruction after ascending aorta repair, n = 2; pulmonary arteries, n = 5; inferior caval vein, n = 1; atretic superior caval vein, n = 1; pulmonary vein obstruction, n = 1; and right ventricular outflow tract, n = 1) were implanted using the smallest available delivery system. Secondary dilation with larger-diameter balloons was performed when the residual pressure was gradient, the stent-vessel wall relationship or stent re-coiling due to different reasons needed a re-intervention by pure ballooning or second stent placement.nnnRESULTSnAll 16 patients aged 5-46 years underwent V12 implantation. The variability of the treated lesions and the need for additional interventions were responsible for large ranges in fluoroscopy time between 7.3 to 48.2 minutes (median 17.3). Considering the additional procedures, the V12 stent achieved the desired result in all cases. There were no major complications. At short-term median follow-up of 2 months, all patients are alive and well with no evidence of stent failing.nnnCONCLUSIONnThese initial results show that the covered Advanta V12 large-diameter stent is safe and effective in the immediate treatment of various cardiovascular obstructions. Long-term follow-up is required.