Creation of a restrictive atrial left-to-right shunt: a novel treatment for heart failure.

Elevated left atrial (LA) filling pressures are associated with poor prognosis in patients with heart failure (HF). Recent evidences have shown that, in this setting, the presence of restrictive small atrial defect may protect from an extreme increase in LA pressure and prevent consecutive pulmonary congestion and acute decompensation, without the expense of an unfavourable significant left-to-right shunt. Therefore, decompression of LA by creating a unidirectional but restrictive left-to-right interatrial shunt has been emerging as a new therapeutic strategy in patients suffering from both HF with preserved and reduced ejection fraction. Two dedicated devices (V-Wave and interatrial septal device system-IASD) are currently available to create a restrictive interatrial communication. Several clinical studies have demonstrated the safety of this approach, with encouraging results regarding the improvements in haemodynamic parameters, functional status and quality of life in treated patients. We aim to summarize the present knowledge concerning this novel therapeutic approach for HF, describe the currently available devices and depict their potential future clinical indications.

Transcatheter Implantable Devices to Monitoring of Elevated Left Atrial Pressures in Patients with Chronic Heart Failure.

Elevated left atrial (LA) pressures are associated with poor prognosis in heart failure (HF). Invasive monitoring of LA-pressures and direct mechanical LA-decompression are associated with functional improvement in patients suffering from HF both with reduced and preserved ejection fraction. We aim to review the current available percutaneously implantable sensors for haemodynamic telemonitoring of LA-pressures (direct LAP sensor device-HeartPOD; right ventricular device-Chronicle; pulmonary artery device-CardioMEMs).

[Decompensated right heart failure, intensive care and perioperative management in patients with pulmonary hypertension]. / Dekompensierte Rechtsherzinsuffizienz, Intensiv- und Perioperativ-Management bei Patienten mit pulmonaler Hypertonie: Empfehlungen der Kölner Konsensus Konferenz 2016.

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to the management of decompensated right heart failure, intensive care management and perioperative management in patients with pulmonary hypertension. This article summarizes the results and recommendations of the working group on decompensated right heart failure, intensive care and perioperative management in patients with pulmonary hypertension.

Prenatal assessment of ventriculocoronary connections and ventricular endocardial fibroelastosis in hypoplastic left heart.

OBJECTIVE: The outlook for newborns with hypoplastic left heart (HLH) has substantially improved over the last decade. However, differences in outcome among various anatomical subgroups have been described. We aimed to describe the incidence of ventriculocoronary communications and endocardial fibroelastosis in HLH and the possible implication on hospital survival (30 d). METHODS: We retrospectively reviewed our medical records, still frames and video loops of 72 fetuses with HLH and critical aortic valve stenosis and evolving HLH from 2008 - 2013. The presence of VCAC and EFE were systematically assessed. Outcome parameters were incidence of VCAC and EFE among different anatomical subgroups of HLH and hospital survival (30 d). RESULTS: 72 fetuses were included in this series. The incidence of VCAC was 11.1 % (8 cases) and EFE occurred in 33.3 % (24 cases). 5 fetuses with VCAC occurred in the subgroup of mitral valve stenosis/aortic valve atresia (MS/AA, 62.5 %) and 2 fetuses with VCAC occurred in the group of mitral atresia/aortic valve atresia (MA/AA, 25 %). Further classification was not possible in one case with VCAC (12.5 %). EFE predominantly occurred in the subgroup of MS/AA, MA/AA and in those cases with aortic valve stenosis and evolving HLH. The overall hospital survival on an intention-to-treat basis was 91.2 % (52/57 newborns). Hospital survival was 91 % for the subgroup of cases with MS/AA and for all other anatomical subgroups. CONCLUSION: The presence of VCAC in HLH can be diagnosed by fetal echocardiography predominantly occurring in cases with obstructed outflow and to some extent patent mitral valve. EFE is a frequent coexisting finding. Hospital survival was comparable among different anatomical subgroups and in cases with VCAC. The presence of VCAC in HLH did not limit the results of surgical palliation within the observation period of 30 days.

Reduction in cortisol inactivation is part of the adrenal stress response to cardiac and noncardiac pediatric surgery: a prospective study using gas chromatography-mass spectrometry analysis.

We examined the hypothesis that major cardiac surgery triggers a more intense adrenal stress response than less intensive noncardiac surgery, which then alters cortisol inactivation. Urinary excretion rates of glucocorticoid metabolites were determined before and after surgery using gas chromatography-mass spectrometry in 29 children undergoing scheduled major cardiac surgery and 17 control children undergoing conventional noncardiac surgery in a prospective observational study. Excretion rates of glucocorticoid metabolites were summed and corrected for creatinine excretion to calculate cortisol production rates (mg/mmol creatinine/m(2) body surface area). Precursor/product ratios from individual metabolites were calculated to characterize cortisol inactivation (11ß-hydroxysteroid dehydrogenase). Postoperatively, median cortisol production rates increased in both groups ( MCS: from 2.7 to 9.3; controls: from 2.7 to 5.8; p<0.001) with no significant difference between groups (p=0.12). Ratios of cortisol to cortisone metabolites, indicating the overall activity of 11ß-hydroxysteroid dehydrogenase, increased postoperatively in both groups (p<0.001). In conclusion, surgery resulted in a distinct postoperative increase in cortisol production. In contrast to our hypothesis, children undergoing major cardiac surgery did not show an increased adrenal stress response compared to children undergoing conventional surgery. Furthermore, the reduction in cortisol inactivation appears to be an essential part of the stress response to pediatric surgery in general.

[Pulmonary arterial hypertension in congenital heart defects with shunt: a heterogeneous and complex constellation]. / Pulmonalarterielle Hypertonie (PAH) bei angeborenen Herzfehlern mit Shuntverbindung: eine heterogene und komplexe Konstellation.

It will be explained how pulmonary vascular disease can develop in congenital heart defects with primary left-to-right shunt across the different ages and stages of development, with and without surgical, interventional or medical treatment. Furthermore it will be discussed, in which specific stages of pulmonary vascular disease "advanced therapy" can be used.

The development and validation of a health-related quality of life questionnaire for pre-school children with a chronic heart disease.

PURPOSE: Heart diseases are often associated with residual injuries, persisting functional restrictions, and long-term sequelae for psychosocial development. Currently, there are no disease-specific instruments to assess the health-related quality of life (HrQoL) of pre-school children. The aims of this study were to develop a parent proxy instrument to measure the HrQoL of children aged 3-7 years with a heart disease and to confirm its validity and reliability. METHODS: Items from the Preschool Pediatric Cardiac Quality of Life Inventory (P-PCQLI) were generated through focus groups of caregivers. In a pilot study, comprehensibility and feasibility were tested. Five subdimensions were defined theoretically. Psychometric properties were analysed within a multicentre study with 167 parental caregivers. RESULTS: The final 52-item instrument contains a total score covering five moderately inter-correlated dimensions. The total score of the questionnaire showed a very high internal consistency (Cronbachs' α = 0.95). Test-retest correlation was at r tt = 0.96. External validity was indicated by higher correlations (r = 0.24-0.68) with a generic paediatric quality of life questionnaire (KINDL) compared to the Strengths and Difficulties Questionnaire (r = 0.17 to 0.59). Low P-PCQLI total scores were significantly associated with inpatient as opposed to outpatient treatment (t = 6.04, p < .001), with at least moderate disease severity ((t = 5.05, p < .001) NYHA classification) and with poorer prognosis (t = 5.53, p < .001) as estimated by the physician. CONCLUSIONS: The P-PCQLI is reliable and valid for pre-school children with a heart disease. It could be used as a screening instrument in routine care, and for evaluation of HrQoL outcomes in clinical trials and intervention research.

Fetal cardiac catheterization using a percutaneous transhepatic access technique: preliminary experience in a lamb model.

OBJECTIVES: Human fetal cardiac intervention has hitherto typically involved a percutaneous transventricular approach. In fetal lambs, a transhepatic approach to access the fetal intra-abdominal veins after exteriorization of the uterus by laparotomy has been described. We aimed to develop a percutaneous transhepatic technique for catheterization of the fetal heart at mid-gestation that avoids maternal laparotomy. METHODS: In 10 fetal lambs (90-97 days' gestation), access to the fetal venous system was attempted by percutaneous puncture with a 5-F sheath into the umbilical vein (n = 1) or a 16-gauge IV-catheter into the hepatic vein (n = 9). This was followed by cardiac catheterization using a 1.8-2.6-F tapered coronary catheter. Euthanasia and postmortem examination were performed immediately postprocedure in two cases, or after normal term delivery in the remaining cases that survived the procedure. RESULTS: In one case fetal position precluded procedural attempts, and in another, the fetus, accessed by a 5-F sheath, died from umbilical hemorrhage. In eight cases, access to the fetal hepatic vein was achieved. In seven of these cases, the access catheter was advanced into the inferior vena cava, followed by catheterization of the right atrium (all cases) and four cardiac chambers (three cases). One fetus died during cardiac catheterization owing to right ventricle perforation, and the other seven fetuses were alive at the end of the procedure (87.5% survival). Immediate postmortem after euthanasia in two of the fetuses that survived the procedure detected intraperitoneal bleeding (4 mL and 20 mL), while postnatal postmortem examination following uneventful delivery at term in the remaining five fetuses revealed no vascular or cardiac trauma. CONCLUSIONS: Ultrasound-guided percutaneous transhepatic cardiac catheterization is feasible in mid-gestational fetal sheep. This technique has the potential for translation into human fetal cardiac and circulatory interventions.

The dilemma of subaortic stenosis--a single center experience of 15 years with a review of the literature.

OBJECTIVE: The term "subaortic stenosis" includes a variety of obstructions of the left ventricular outflow tract (LVOT), ranging from a short (discrete) subvalvular membrane to long, tunnel-like narrowing. An association with other congenital lesions is frequent. We reviewed the reported literature and describe our results, analyzing the nomenclature of and risk factors for restenosis after surgical treatment. METHODS: From 1994 to 2009, 81 children (53 males, 28 females; median age: 57 months, range [ R]: 5-204) underwent surgical relief of a subaortic stenosis. Patients were divided, according to pathology, into short segment (group A, n = 42) and complex obstructions (group B, n = 39), with the latter including long segment stenosis and/or associated anomalies such as aortic coarctation, interrupted aortic arch or Shone's complex. RESULTS: Surgery resulted in a significant reduction of the gradient between the left ventricle and the aorta in both groups (Δ P group A: 51 ± 28 mmHg, group B: 46 ± 25 mmHg). There was no operative mortality. One patient died in the early postoperative period due to pericardial tamponade. Median follow-up was 90 months (R = 0.5-187). Twenty-five (31%) patients required reoperation because of recurrent stenosis after a median of 43 months (R = 0.5-128). Seven (16%) patients belonging to group A developed restenosis, and 18 (46%) in group B. Freedom from reoperation for all patients was 60% after 10 years. 10 (40%) of the patients of group B were ultimately treated with a Ross-Konno reconstruction of the LVOT. CONCLUSION: Despite adequate surgical resection, recurrence of subaortic stenosis within several years after initial surgical treatment is frequent, especially in patients with complex lesions. In cases requiring reoperation, the surgical therapy is often extensive, and even includes Ross-Konno reconstruction of the LVOT.

The fate of the pulmonary autograft in the aortic position: experience and results of 98 patients in twelve years.

OBJECTIVE: The ideal prosthesis for aortic valve replacement in infants and adolescents is still controversially discussed. Implantation of mechanical prostheses or homografts is associated with serious risks such as reoperations, thromboembolic events and infections. This has led many surgeons to prefer the Ross operation. METHODS: Between 1996 and 2008 we performed Ross operations in 98 children and infants with a mean age of 11 years (range 1 mo-25 y), including concomitant procedures (n = 33). The operation was performed as a full-root replacement in 97 and as a subcoronary implantation in one patient. The patients were followed for up to twelve years (follow-up complete) to determine clinical and echocardiographic parameters. Mean duration of follow-up was 60 +/- 37 (range 3-150 months). RESULTS: Overall survival was 98 % (n = 96). In 70 patients (71.4 %) no autograft insufficiency (AI) could be observed. AI grade I was present in 22 patients (22.4 %), AI grade II in 3 patients (3 %), and AI grade III in one patient. The autograft in the latter patient was replaced by a mechanical prosthesis. Aortic root dilatation was observed in 10 patients (10.2 %). Z-score (median) in these patients was 4.1 (normal z-score < or = 2.4). One patient with aortic root dilatation was reoperated. A pacemaker was implanted in two patients. No patient is on constant anticoagulants. CONCLUSION: The anatomy and physiology of the LVOT is best restored by the implantation of a pulmonary autograft. This additionally provides a growth potential for infants at low risk for reoperations. Autograft dilatation is a risk that requires constant evaluation.

[Pulmonary hypertension: hemodynamic evaluation: hemodynamic evaluation ­ recommendations of the Cologne Consensus Conference 2010]. / Pulmonale Hypertonie: invasive Diagnostik: Empfehlungen der Kölner Konsensus-Konferenz 2010.

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension (PH) have been adopted for Germany. Invasive hemodynamic data obtained by right heart catheterization are essential to confirm the diagnosis, test vasoreactivity, assess severity and guide therapy in PH patients. The definition of PH is resting on a mean pulmonary artery pressure ≥ 25 mm Hg obtained by right heart catheterization. Furthermore, a pulmonary capillary wedge pressure > 15 mm Hg excludes pre-capillary PH. Vasoreactivity testing is part of the diagnostic work-up in pulmonary arterial hypertension. Recent data on the use of inhaled iloprost update these guidelines and are of special importance due to the frequent diagnostic use of iloprost in Germany. Other aspects of invasive hemodynamic data in certain PH subgroups as well as their measurement and interpretation in children are discussed. Several aspects of right heart catheterization in PH justify a detailed commentary, and in some areas an update already appears necessary. In June 2010, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Paediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups were initiated, one of which was specifically addressing the invasive hemodynamic evaluation of patients with PH. This commentary summarizes the results and recommendations of this working group.

[Treatment of pulmonary arterial hypertension (PAH): recommendations of the Cologne Consensus Conference 2010]. / Therapie der pulmonal arteriellen Hypertonie (PAH): Empfehlungen der Kölner Konsensus-Konferenz 2010.

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension have been adopted for Germany. The guidelines contain detailed recommendations for the treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2010, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to the treatment of PAH. This commentary summarizes the results and recommendations of the working group on treatment of PAH.

Primary noncompaction of the ventricular myocardium from the morphogenetic standpoint.

This review compiles the current knowledge of normal and abnormal myocardial morphogenesis to facilitate an unambiguous diagnosis of primary myocardial noncompaction. During the early stages of development, the formation of trabeculae with the resulting increase in myocardial surface is a adaptation of the rapidly growing heart to improve nourishment by exchange diffusion from the cardiac lumen. Once the coronary vasculature has developed, the switch to cardiac nutrient supply through active circulation from the subepicardial space is paralleled by gradual compaction of the myocardial trabeculae. This results in a decrease of the inner, trabeculated myocardial layer with a parallel increase in thickness of the outer, compact myocardial layer. Similar to the direction of coronary arterial development, this process proceeds from the epicardium toward the endocardium and from the base of the heart to the apex. Based on developmental data, congenital myocardial noncompaction represents a failure of normal embryonic myocardial maturation. The time of arrest of this process will determine the extension of myocardial noncompaction within the ventricle. Whereas disturbances of myocardial microcirculation are frequent in these hearts, direct communications between the myocardial cavity and the coronary arteries (sinusoids) do not belong to this morphogenetic entity.

Systemic sarcoidosis after cardiac transplantation in a 9-year-old child.

Sarcoidosis is a granulomatous disease of unknown etiology and is only rarely seen in infants and children. We present the case of a 9-year-old boy who developed sarcoidosis with multi-organ involvement 9 years after cardiac transplantation for Shone complex. The patient was on immunosuppressive therapy with tacrolimus and mycophenolate mofetil. He presented with severe respiratory distress due to marked mediastinal lymphadenopathy and bilateral pulmonary infiltrates in association with fatigue, low-grade fever, hepatosplenomegaly and generalized lymphadenopathy. Lymph node histology showed non-caseating epitheloid cell granulomas and giant cells. Initialization of therapy with prednisolone resulted in prompt clinical recovery and resolution of all symptoms except for the development of mild pulmonary fibrosis. Tapering of the steroids led to recurrence of mediastinal lymphadenopathy 5 months after the initial disease, which responded to an increase in steroid dose. The clinical course, the medical management, and the possible role of immunosuppression in the etiology of the disease are discussed.