Elisabeth Schermer

The pediatric mechanical circulatory support program in Innsbruck, Austria, and the impact of such programs on lack of donor hearts in Europe.

Strategy and results of the Innsbruck Mechanical Circulatory Support Program are presented, and the impact of such programs on pediatric heart transplantation (HTX) in Europe is discussed. Venoarterial extracorporeal membrane oxygenation (vaECMO) and ventricular assist devices (VADs) were used in 21 pediatric patients (median age 3.3 years, 2 days to 17 years) for acute heart failure (AHF) following a bridge or bridge-to-bridge strategy. Twelve patients were treated with vaECMO: eight were weaned after 2–10 days, two died, and two were switched to a VAD. Of the last, one was weaned 47 days later and the other underwent HTX 168 days later. In nine patients, VAD was implanted without preceding vaECMO. One such patient died (cerebral hemorrhage) after 236 days; of the remaining eight patients three were weaned and five underwent HTX. Waiting time for HTX (high-urgency status) varied from 4 to 372 days. Fifteen patients were discharged (follow up: 2–74 months); 14 are doing very well (New York Heart Association (NYHA) Functional Classification Class I, neurologically normal), whereas one suffers from severe neurologic damage, presumably from resuscitation before vaECMO. Data from Eurotransplant on pediatric HTX in 2004, 2005, and 2006 (33, 49, and 34 transplanted hearts, respectively; recipients <16 years of age) are discussed. Mechanical circulatory support (MCS) substantially improves survival with AHF in pediatric patients. Medium-term support (up to 400 days in our patients) is possible and outcome of survivors is excellent. Wide spread use of MCS might slightly aggravate the lack of donor organs, which could result in longer support times.

Former Very Preterm Infants Show an Unfavorable Cardiovascular Risk Profile at a Preschool Age

Cardiovascular disease is the leading cause of death worldwide. Evidence points towards an unfavorable cardiovascular risk profile of former preterm infants in adolescence and adulthood. The aim of this study was to determine whether cardiovascular risk predictors are detectable in former very preterm infants at a preschool age. Five- to seven-year-old children born at <32 weeks’ gestational age were included in the study. Same-aged children born at term served as controls. Basic data of study participants were collected by means of follow-up databases and standardized questionnaires. At study visit, anthropometric data, blood pressure readings and aortic intima-media thickness were assessed. Blood samples were obtained after an overnight fast. In comparison to children born at term, former preterm infants had higher systolic and diastolic blood pressure readings (odds ratio [95% confidence interval] per 1-SD higher blood pressure level 3.2 [2.0–5.0], p<0.001 and 1.6 [1.1–1.2], p = 0.008), fasting glucose levels (OR [95% CI] 5.2 [2.7–10.1], p<0.001), homeostasis model assessment index (OR [95% CI] 1.6 [1.0–2.6], p = 0.036), and cholesterol levels (OR [95% CI] 2.1 [1.3–3.4], p = 0.002). Systolic prehypertension (23.7% vs. 2.2%; OR [95% CI] 13.8 [3.1–60.9], p = 0.001), elevated glucose levels (28.6% vs. 5.9%; OR [95% CI] 6.4 [1.4–28.8], p = 0.016), and hypercholesterolemia (77.4% vs. 52.9%; OR [95% CI] 3.0 [1.3–7.1], p = 0.010) were significantly more prevalent in the preterm group. As former very preterm infants display an unfavorable cardiovascular risk profile already at a preschool age, implementation of routine cardiovascular follow-up programs might be warranted.

How Mechanical Circulatory Support Helps Not to Need It—New Strategies in Pediatric Heart Failure

During the past 3 years, seven potential candidates for mechanical circulatory support (MCS) were treated at our center. Ultimately, only one of them needed MCS (extracorporeal membrane oxygenation [ECMO] for 16 days), although 5 years earlier, all would have been considered for MCS at our center. Seven consecutive patients were seen in this period: four toddlers (three suffering from fulminant myocarditis and one with dilated cardiomyopathy associated with spongy myocardium) and three adolescents (two with postmyocarditis cardiomyopathy and one with hypertrophic cardiomyopathy and severe restrictive dysfunction after an ischemic event with cardiopulmonary resuscitation [stunned heart]). All patients presented in acute cardiocirculatory decompensation. All were admitted to the intensive care unit (ICU); all but one were sedated and intubated. A combination of levosimendan, milrinone, and nesiritide was administered to all patients. Use of catecholamines was kept short (<48 h in six individuals). MCS (ECMO, Berlin Heart Excor Pediatric, and Heartware) was always available. MCS initiation was indicated in only one patient, who was developing progressive multiorgan failure (MOF). The three toddlers with myocarditis recovered with complete normalization of myocardial function within 6 months. The fourth toddler is still at the ICU while waiting for transplantation. The three adolescents were listed with high urgency for heart transplantation, and all received a graft within 3 weeks. The adolescent with the stunned heart developed progressive MOF and was successfully supported with ECMO until transplantation. All six patients with completed course were discharged home in New York Heart Association Heart Failure Functional Classification System I condition without neurological deficits. Combined use of levosimendan, milrinone, and nesiritide, avoidance of catecholamines as much as possible, and MCS as backup are the new strategies at our center. This cardioprotective approach gives excellent outcome at lower risk and better cost-effectiveness in our pediatric patients with acute heart failure. Pediatric trials are recommended to evaluate combined use of newer cardioprotective drugs.

Acute Lung Failure During Mechanical Circulatory Support

The use of venoarterial extracorporal membrane oxygenation and ventricular assist-devices in children with end stage heart failure is well established. The use of a bridge-to-bridge strategy leads to excellent survival rates in pediatric patients. We present an adolescent, who acquired acute respiratory failure, due to possible transfusion related lung injury, and who was successfully treated with venovenous extracorporal membrane oxygenation while on ventricular assist-device support.

Heart Transplantation in a 14-Year-Old Boy in the Presence of Severe Out-of-Proportion Pulmonary Hypertension due to Restrictive Left Heart Disease: A Case Report

A 14-year-old boy after balloon valvuloplasty of severe aortic valve stenosis in the neonatal period was referred for heart-lung transplantation because of high grade pulmonary hypertension and left heart dysfunction due to endocardial fibroelastosis with severe mitral insufficiency. After heart catheterization, hemodynamic parameters were invasively monitored: a course of levosimendan and initiation of diuretics led to a decrease of pulmonary capillary wedge pressure (from maximum 35 to 24 mmHg). Instead of an expected decrease, mean pulmonary artery pressures (mPAP) increased up to 80 mmHg with increasing transpulmonary pressure gradient (TPG) up to 55 mmHg. Oral bosentan and intravenous epoprostenol then led to a ~50% decrease of mPAP (TPG between 16 and 22 mmHg). The boy was listed solely for heart transplantation which was successfully accomplished 1 month later.

Prolonged But Successful Weaning from Left Ventricular Assist Device After Cardiac Decompensation due to Late-Recognized Coarctation of the Aorta in a Toddler

A 2-year-old boy was presented with late-recognized coarctation of the aorta and pulmonary hypertension due to left ventricular failure. The coarctation was corrected at the day of admission with a good postoperative result. However, weaning from the respirator failed despite multiple drug support due to left ventricular failure. Consequently, a left ventricular assist device (LVAD) was implanted 22 days later. The further course was complicated by systemic hypertension and ongoing pulmonary hypertension requiring extensive antihypertensive therapy. The first attempt to wean from LVAD failed and the left ventricle was left completely unloaded for additional 4 weeks. The second weaning attempt, using a very smooth weaning protocol, led to a recovered left ventricle and facilitated the removal of the assist device after a total of 120 days. The patient was discharged with normal cardiac function, but he still requires antihypertensive therapy. We believe that the slow reduction of the LVAD support was the key measure that leads to the successful weaning of the patient, thereby avoiding heart transplantation.

PS-100 Blood Pressure And Aortic Distensibility As Early Markers Of An Increased Cardiovascular Risk In Preschool Children Born Preterm – Preliminary Data

Background and aims Numerous studies have shown that young adults born preterm have an increased risk of cardiovascular diseases. The aim of this study was to investigate which markers of an increased cardiovascular risk are already present at preschool age in children born preterm. Methods In preschool children born preterm (gestational age less than 32 weeks) and healthy control subjects born at term, blood pressure was determined oscillometrically. Elastic properties of the ascending and descending aorta were calculated using computerised wall contour analysis out of transthoracic M-mode echocardiographic tracings. Results 119 children were examined at 5 to 7 years of age (45 born preterm and 74 children born at term). Preschool children born preterm had significantly higher systolic blood pressure in comparison to preschool children born at term (p < 0.001). Descending aorta distensibility was significantly decreased in preschool children born preterm in comparison to preschool children born at term (p < 0.05). Diastolic blood pressure, distensibility of the ascending aorta, and stiffness index of ascending and descending aorta did not differ significantly between the two groups. Conclusions Children born preterm already have higher systolic blood pressure and decreased distensibility of the descending aorta in comparison to children born at term at preschool age. Preliminary data of this study underline the importance of a continuous and systematical follow-up of children born preterm. Further studies are needed to investigate the clinical relevance of these findings and to find possible preventive measures to lower cardiovascular risk of this population.