The heart should not be divided in right and left, whether in health nor in disease. However, the morphological and functional differences between the right and left ventricle should be known and the impact of the ventricle's position considered. Further, the parameters beyond heart rate, contractility, pre- and afterload guaranteeing a sufficient systemic cardiac output have to be integrated in therapeutic measures; preferentially the influence of interventricular mechanics.
View Article and Find Full Text PDFThe hypoplasia of left-sided heart structures shows great variability and complexity. What the many variants have in common is that their heart structures are neither fully developed before nor after birth. Fetuses and newborns require an individual therapy depending on anatomy and function of the heart.
View Article and Find Full Text PDFBackground: Newborns with hypoplastic left heart (HLH) are usually palliated with the Norwood procedure or a hybrid stage I procedure. Hybrid is our preferred approach. Given the critical relationship between stage I, interstage, and comprehensive stage II or advanced biventricular repair, we hypothesized that appropriate drug treatment is a significant therapeutic cornerstone, especially for the management of the high-risk interstage.
View Article and Find Full Text PDFBackground: Dilated cardiomyopathy (DCM) is a leading cause of death in children with heart failure. The outcome of pediatric heart failure treatment is inconsistent, and large cohort studies are lacking. Progress may be achieved through personalized therapy that takes age- and disease-related pathophysiology, pathology, and molecular fingerprints into account.
View Article and Find Full Text PDFObjectives: The monitoring of cerebral tissue oxygen saturation by near-infrared spectroscopy (SO) is used widely in pediatric cardiac anesthesia. However, little information is available on the effects of changes in perioperative SO on brain morphology and neurologic outcome. The primary hypothesis tested in this study was that intraoperative SO during the comprehensive stage II procedure correlated with brain volumes assessed by magnetic resonance imaging and neurodevelopmental scores.
View Article and Find Full Text PDFBackground: Neonatal management of patients with hypoplastic left heart syndrome and complex remains a challenging task, whereby the "hybrid" palliation is often reserved for high-risk patients as a "rescue" procedure.
Aim: This study documents the anesthetic challenges and potential complications associated with the Giessen hybrid stage I approach.
Methods: The Giessen hybrid stage I approach is focused on surgical bilateral pulmonary artery banding.
. The absolute number of patients presenting with failure of the hemi-Fontan or Fontan circulation for cardiac transplantation (cTx) will continuously rise. We aimed to analyze the intraoperative differences in patients undergoing cTx for a failing hemi-Fontan or Fontan circulation (group A) with those undergoing cTx for cardiomyopathy and congenital heart disease (group B).
View Article and Find Full Text PDFComprehensive stage II is the advanced surgical part of the staged treatment of a newborn with hypoplastic left heart syndrome (HLHS) palliated initially by a Giessen-Hybrid approach. We report an almost ideal course following comprehensive stage II operation with focus on postoperative intensive care strategy. Following a short introduction of the postnatally performed Giessen-Hybrid approach, in which the surgical part is focused on bilateral pulmonary banding and duct stenting as well as manipulation of the atrial septum is postponed to transcatheter approach, it should be emphasized, that the quality of inter-stage I is eminently important for the success of the following comprehensive stage II.
View Article and Find Full Text PDFDilated cardiomyopathy (DCM) is a leading cause of cardiac death in children. Current therapeutic strategies are focused on improving symptoms of congestive heart failure (CHF); the potentials of cardiac regeneration especially in infants and young children are neglected in particular when DCM is classified as "end-stage". Heart transplantation (HTx) serves as the only life-saving option, despite is palliative character with limited survival time.
View Article and Find Full Text PDFBackground: Percutaneous pulmonary valve implantation (PPVI) has been established as a safe and effective alternative to surgery treating patients with a failing pulmonary valve conduit. Nevertheless, the majority of patients in need of a valve have a native, non-obstructive right ventricular outflow tract (RVOT). The current approved stent-valves have a balloon-expandable design.
View Article and Find Full Text PDFObjective: Central pulmonary banding has been proposed as a novel alternative for the treatment of left ventricular dilated cardiomyopathy in children. We sought to investigate the effects of central pulmonary banding in an experimental model of doxorubicin-induced left ventricular dilated cardiomyopathy.
Methods: Four-month-old sheep (n = 28) were treated with intermittent intracoronary injections of doxorubicin (0.
Background: Long-term cardiac remodeling after heart transplantation (HT) in children has been insufficiently characterized. The aim of our study was to evaluate ventricular size in HT patients using cardiovascular magnetic resonance (CMR) imaging, to find underlying factors related to potentially abnormal cardiac dimensions and to study its impact on functional class and ventricular function.
Methods: Seventy-five pediatric HT recipients (age 14.
Background: Left atrial congestion results from backward failure in dilated cardiomyopathy. We aimed to evaluate feasibility and efficacy of percutaneous atrioseptostomy to create a restrictive atrial septum defect in management of dilated cardiomyopathy.Methods and resultsFrom June 2009 to December 2016, 27 interventions comprised left atria decompressions in 22 dilated cardiomyopathy patients; 9 females; age: 24 days to 36.
View Article and Find Full Text PDFObjectives: Mechanical circulatory support for pediatric heart failure patients with the Berlin Heart EXCOR ventricular assist system is the only approved and established bridging strategy for recovery or heart transplantation. In recent years, the burden of thromboembolic events has led to modifications of the recommended antithrombotic therapy. Therefore, we aimed to assess modifications of antithrombotic practice among the European EXCOR Pediatric Investigator Group members.
View Article and Find Full Text PDFBackground: Left atrial decompression is considered in patients with symptomatic heart failure with preserved ejection fraction (HFpEF). We aimed to evaluate the feasibility and efficacy of transcatheter generation of a restrictive atrial septum communication to manage HFpEF from infancy to adulthood with cardiomyopathy and congenital heart defect.
Methods And Results: From June 2009 to December 2016, 24 patients (50% with an age less than 16 years) with HFpEF were palliated; NYHA-/Ross class IV (n = 10); median systemic ventricular ejection fraction 64 (range 35-78) %.
Percutaneous pulmonary valve implantation (PPVI) is nowadays an accepted treatment option to repair post-surgical conduit dysfunction of the right ventricular outflow tract (RVOT). In addition, many patients need a pulmonary valve to reconstruct a hemodynamically incompetent native or conduit free outflow tract. Based on our experience with percutaneous stent-valve placement in a cohort of 125 patients, we report here transvenous reconstruction of a conduit-free, patch repaired outflow tract by utilizing balloon-expandable stent-valves in 23 patients with a median age of 22 years (5-60 years).
View Article and Find Full Text PDFObjective: Norwood and hybrid procedure are two options available for initial palliation of patients with hypoplastic left heart syndrome (HLHS). Our study aimed to assess potential differences in right ventricular (RV) function and pulmonary artery dimensions using cardiac magnetic resonance (CMR) in survivors with HLHS.
Methods: 42 Norwood (mean age 2.
Through a series of complex surgical and interventional procedures including downsizing of an unrestricted atrial septal defect, aortic arch reconstruction, pulmonary artery banding, reversed Potts shunt, and Melody valve in a mitral position, biventricular conversion was successfully achieved in a patient with a distinct borderline left ventricle. By use of these interventional steps, flow-mediated and load-mediated growth of a small left ventricle was observed, highlighting the plasticity of hearts in growing children. Surgical and catheter interventional teamwork may lead to fascinating results, provoking us to quote Aristotle's wisdom: "The whole is more than the sum of its parts.
View Article and Find Full Text PDFDe novo heterozygous mutations changing R179 to histidine, leucine, or cysteine in the ACTA2 gene are associated with Multisystemic Smooth Muscle Dysfunction Syndrome (MSMDS). Characteristic hallmarks of this condition, caused only by these specific ACTA2 mutations, are congenital mydriasis (mid-dilated, non-reactive pupils), a large persistent ductus arteriosus (PDA), aortic aneurysms evolving during childhood, and cerebrovascular anomalies. We describe two patients, a 3-day-old newborn and a 26-year-old woman, with this unique mutation in association with a huge PDA and an aorto-pulmonary window.
View Article and Find Full Text PDFThe final therapy of 'end-stage heart failure' is orthotopic heart, lung or heart-lung transplantation. However, these options are not available for many patients worldwide. Therefore, novel therapeutical strategies are needed.
View Article and Find Full Text PDF