Cardiac output measurement in children: comparison of the Ultrasound Cardiac Output Monitor with thermodilution cardiac output measurement

Objective: To compare the assessment of cardiac output (CO) in children using the noninvasive Ultrasound Cardiac Output Monitor (USCOM) with the invasive pulmonary artery catheter (PAC) thermodilution cardiac output measurement. Design and setting: Prospective observational study in atertiary center for pediatric cardiology of auniversity children's hospital. Patients: Twenty-four pediatric patients with congenital heart disease without shunt undergoing cardiac catheterization under general anesthesia. Measurements and results: CO was measured by USCOM using asuprasternal CO Doppler probe in children undergoing cardiac catheterization. USCOM data were compared to CO simultaneously measured by PAC thermodilution technique. Measurements were repeated three times within 5 min in each patient. Amean percentage error not exceeding 30% was defined as indicating clinical useful reliability of the USCOM. CO values measured by PAC ranged from 1.3 to 5.3 l/min (median 3.6 l/min). Bias and precision were −0.13 and 1.34 l/min, respectively. The mean percentage error of CO measurement by the USCOM compared to PAC thermodilution technique was 36.4% for USCOM. Conclusions: Our preliminary data demonstrate that cardiac output measurement in children using the USCOM does not reliably represent absolute CO values as compared to PAC thermodilution. Further studies must evaluate the impact of incorporating effective aortic valve diameters on CO measurement using the USCO

Oxygen Availability in Respiratory Muscles During Exercise in Children Following Fontan Operation

Introduction: As survival of previously considered as lethal congenital heart disease forms is the case in our days, issues regarding quality of life including sport and daily activities emerge. In patients with Fontan circulation, there is no pump to propel blood into the pulmonary arteries since the systemic veins are directly connected to the pulmonary arteries. The complex hemodynamics of Fontan circulation include atrial function, peripheral muscle pump, integrity of the atrioventricular valve, absence of restrictive, or obstructive pulmonary lung function. Therefore, thoracic mechanics are of particular importance within the complex hemodynamics of Fontan circulation.Methods: To understand the physiology of respiratory muscles, the aim of this study was to examine the matching of auxiliary respiratory muscle oxygen delivery and utilization during incremental exercise in young male Fontan patients (n = 22, age = 12.04 ± 2.51) and healthy Controls (n = 10, age = 14.90 ± 2.23). All subjects underwent a cardiopulmonary exercise test (CPET) to exhaustion whereas respiratory muscle oxygenation was measured non-invasively using a near-infrared spectrometer (NIRS).Results: CPET revealed significantly lower peak power output, oxygen uptake and breath activity in Fontan patients. The onset of respiratory muscle deoxygenation was significantly earlier. The matching of local muscle perfusion to oxygen demand was significantly worse in Fontans between 50 and 90% V.O2peak.Findings: The results indicate that (a) there is high strain on respiratory muscles during incremental cycling exercise and (b) auxiliary respiratory muscles are worse perfused in patients who underwent a Fontan procedure compared to healthy Controls. This might be indicative of a more general skeletal muscle strain and worse perfusion in Fontan patients rather than a localized-limited to thoracic muscles phenomenon

Bleeding and thrombotic risk in pregnant women with Fontan physiology

Background/objectives Pregnancy may potentiate the inherent hypercoagulability of the Fontan circulation, thereby amplifying adverse events. This study sought to evaluate thrombosis and bleeding risk in pregnant women with a Fontan.  Methods We performed a retrospective observational cohort study across 13 international centres and recorded data on thrombotic and bleeding events, antithrombotic therapies and pre-pregnancy thrombotic risk factors.  Results We analysed 84 women with Fontan physiology undergoing 108 pregnancies, average gestation 33 +/- 5 weeks. The most common antithrombotic therapy in pregnancy was aspirin (ASA, 47 pregnancies (43.5%)). Heparin (unfractionated (UFH) or low molecular weight (LMWH)) was prescribed in 32 pregnancies (30%) and vitamin K antagonist (VKA) in 10 pregnancies (9%). Three pregnancies were complicated by thrombotic events (2.8%). Thirty-eight pregnancies (35%) were complicated by bleeding, of which 5 (13%) were severe. Most bleeds were obstetric, occurring antepartum (45%) and postpartum (42%). The use of therapeutic heparin (OR 15.6, 95% CI 1.88 to 129, p=0.006), VKA (OR 11.7, 95% CI 1.06 to 130, p=0.032) or any combination of anticoagulation medication (OR 13.0, 95% CI 1.13 to 150, p=0.032) were significantly associated with bleeding events, while ASA (OR 5.41, 95% CI 0.73 to 40.4, p=0.067) and prophylactic heparin were not (OR 4.68, 95% CI 0.488 to 44.9, p=0.096). Conclusions Current antithrombotic strategies appear effective at attenuating thrombotic risk in pregnant women with a Fontan. However, this comes with high (>30%) bleeding risk, of which 13% are life threatening. Achieving haemostatic balance is challenging in pregnant women with a Fontan, necessitating individualised risk-adjusted counselling and therapeutic approaches that are monitored during the course of pregnancy

Neuromonitoring with Near-Infrared-Spectroscopy and Biochemical Markers in Children

Perinatale Asphyxie kann zu schwerwiegenden entwicklungsneurologischen Beeinträchtigungen führen. Ebenso kann es bei Neugeborenen, Säuglingen und Kindern mit angeborenen Herzfehlern, welche sich komplexen Prozeduren wie Herzkatheteruntersuchungen oder Herzoperationen unterziehen müssen, schwerwiegende neurologische Komplikationen geben. Neuromonitoring zur Erkennung von zerebralen Schäden rückt daher zunehmend in den Mittelpunkt des Interesses bei der Behandlung dieser neonatalen bzw. pädiatrischen Patientengruppen. Die hier vorgestellten Arbeiten haben sich in einem Schwerpunkt systematisch mit Vergleichsstudien und klinischer Anwendung der Nahinfrarot-Spektroskopie (NIRS) beschäftigt. Der zweite Schwerpunkt liegt in der Analyse biochemischer Parameter (Protein S-100, CK-BB und NSE) in Bezug auf ihren Stellenwert als früher Marker zur Erkennung einer hypoxämisch- ischämischen Enzephalopathie nach Asphyxie und ihre Vorhersagekraft für entwicklungsneurologische Folgeschäden. Darüber hinaus wurde das Vorkommen von Protein S-100 in verschiedenen extrazerebralen Flüssigkeiten vor und nach Herzoperationen untersucht, um ein besseres Verständnis für die Beurteilung von Protein S-100 Konzentrationen nach Herzoperationen zu bekommen. Methodik Alle Untersuchungen wurden im Rahmen der klinischen Routine entweder auf der kinderkardiologischen bzw. neonatologischen Intensivstation, im Herzkatheterlabor, im Operationssaal oder im klinischen Labor durchgeführt. 1\. Nah-Infrarot-Spektroskopiegeräte liefern sogenannte zerebrale Oxygenierungsindices: das Gerät NIRO 200 bzw. 300 der Firma Hamamatsu Photonics (Hamamtsu Photonics, Japan) liefert den zerebralen Gewebeoxygenierungs-Index, den tissue oxygenation index (=TOI-Wert), das Gerät INVOS 5100 (Somanetics Corporation, Troy, U.S.A.) einen vergleichbaren zerebralen „regionalen Gewebesättigungswert“ (= rSO2 Wert). Um den zerebralen Oxygenierungsindex (TOI, NIRO 300 der Firma Hamamatsu Photonics) interpretieren zu können, wurden Vergleiche zwischen dem TOI und den simultan oximetrisch gemessenen Sauerstoffsättigungen im Bulbus der Vena jugularis sowie im rechten Vorhof vorgenommen. Der zerebrale Gewebesättigungswert (rSO2, INVOS 5100 von Somanetics Corp.) wurde mit der Sättigung im Bulbus jugularis und in der oberen Hohlvene verglichen. Parallel dazu wurde ein Gerätevergleich durchgeführt, indem die oximetrisch gemessenen Sättigungen im Bulbus jugularis und in der oberen Hohlvene zeitgleich sowohl mit dem TOI als auch mit der rSO2 verglichen wurden. 2\. Bei Patienten nach Herzoperationen mit Herzfehler- bedingter pulmonaler Hypertension wurde in der unmittelbar postoperativen Phase mit Hilfe von NIRS die zerebrale Oxygenierung und ihre Beeinflussung durch ein pulmonal- antihypertensives Medikament (Sildenafil) untersucht. Darüber hinaus wurde die Wertigkeit der NIRS in der Beurteilung klinisch extrem kritischer Situationen (Reanimation) analysiert. 3\. Die biochemischen Parameter Protein S-100, CK-BB und NSE im Serum wurden unmittelbar nach einem geburtsasphyktischen Ereignis in definierten Zeitabständen bestimmt, um ihre prognostische Wertigkeit hinsichtlich der Entwicklung einer hypoxisch- ischämischen Enzephalopathie (HIE) zu analysieren. Nach 20 Monaten wurden die Patienten entwicklungsneurologisch nachuntersucht und die Ergebnisse mit den Serumkonzentrationen der biochemischen Marker korreliert. 4\. Zur Klärung möglicher Störeinflüsse bei der Verwendung des Protein S-100 als zerebraler Marker nach Herzoperationen im Kindesalter wurde das Protein prä- und postoperativ in verschiedenen extrazerebralen Körperflüssigkeiten (native Perikardflüssigkeit, postoperative Perikard-, Pleura- und Peritonealflüssigkeit) untersucht. Resultate 1\. Vergleich zerebraler Oxygenierungsindices Die Arbeiten konnten zeigen, dass die zerebralen Oxygenierungsindices geräteunabhängig sowohl eine Korrelation zu den Sättigungswerten im Bulbus jugularis als auch zu der globalen Oxygenierungssituation (gemessen in der oberen Hohlvene und im rechten Vorhof) aufweisen. Im Gerätevergleich korrelierten die Ergebnisse des INVOS 5100 jedoch besser. 2\. Klinische Anwendungen des Neuromonitorings „NIRS“ Durch den Einsatz von NIRS scheint sich ein direkter Einfluss von Sildenafil auf den neuronalen Zellmetabolismus nachweisen zu lassen, in dem mittels NIRS nach Sildenafilgabe ein Anstieg des oxygenierten Hämoglobins messbar ist. Während kardiopulmonaler Reanimation dokumentiert NIRS zuverlässig hämodynamische Veränderungen in Echtzeit. 3\. Untersuchungen biochemischer Marker zur Erkennung einer hypoxisch-ischämischen Enzephalopathie und deren entwicklungsneurologische Folgen nach Asphyxie Die Kombination einer frühen Serumuntersuchung von Protein S-100 und CK-BB bereits zwei Stunden nach Asphyxie hat einen hohen prädiktiven Wert bezüglich der Vorhersage der Entwicklung einer hypoxisch-ischämischen Enzephalopathie. Andererseits zeigte sich in derselben Patientengruppe kaum ein Zusammenhang zu den biochemischen Parametern im Hinblick auf entwicklungsneurologische Defizite. 4\. Untersuchung von Protein S-100 in verschiedenen extrazerebralen Flüssigkeiten vor und nach herzchirurgischen Eingriffen bei Kindern Protein S-100 lässt sich bereits in nativer Perikardflüssigkeit nachweisen. Nach Operation an der Herz- Lungen-Maschine ist es in Perikard-, Pleura- und Peritonealflüssigkeit vorhanden. Schlussfolgerungen Die in dieser Habilitationsschrift ausgeführten Arbeiten haben weiterführende Antworten auf die Anwendbarkeit des nicht- invasiven zerebralen Überwachungssystems „NIRS“ geliefert, und eine neue Bewertung der biochemischen Marker Protein S-100, CK-BB und NSE im Hinblick auf die Entwicklung einer hypoxisch-ischämischen Enzephalopathie und der Abschätzung neurologischer Folgen ermöglicht.Perinatal asphyxia may cause severe neurodevelopmental disabilities. On the other hand newborns, infants and children with congenital heart defects who undergo complex catheter intereventions or heart operations may suffer from neurological complications. Neuromonitoring for the detection of cerebral injuries is therefore of increasing interest in neonatal and paediatric patient groups. The studies on which this work is based are systematic comparison studies and clinical practice studies concerning the method near- infrared spectroscopy. A further focus is the investigation of biochemical parameters (protein S-100, CK-BB and NSE) in terms of their value in predicting hypoxic-ischemic encephalopathy after birth asphyxia at an early time and in the later follow-up as parameters for neurodevelopmental outcome. Further, the appearance of protein S-100 in extra-cerebral fluids before and after heart operations was analyzed to obtain a better understanding of the protein S-100 concentrations after cardiac surgery. Methology All examinations were performed during routine clinical examinations at the paediatric intensive care unit, the neonatal intensive care unit, the catheterization laboratory, operating theatre or clinical laboratory. 1\. Near-infrared spectroscopy provides tissue oxygenation indices: the NIRO 200 or NIRO 300 device (Hamamtsu Photonics, Japan) provides the so-called “cerebral tissue oxygenation index” (TOI), while the INVOS 5100 device (Somanetics Corporation, Troy, MI, USA) provides a comparable “regional cerebral oxygenation index” (= rSO2). To clarify the cerebral tissue oxygenation index TOI (NIRO 300) we performed comparisons with simultaneous measurement of cerebral TOI, venous jugular bulb saturation and oxygen saturation in the right atrium. The cerebral regional oxygenation saturation rSO2 (INVOS 5100) was compared to the saturation in the jugular bulb and that measured in the superior caval vein. Furthermore, a comparison between the two near- infrared spectrophotometers was performed by comparing the saturations from the venous jugular bulb and the superior caval vein to both the simultaneously measured cerebral TOI and cerebral rSO2 values. 2\. The influence of intravenous sildenafil on cerebral oxygenation as measured by near-infrared spectroscopy after cardiac surgery and on cerebral oyxgenation as measured by near-infrared spectroscopy during circulatory arrest and cardiopulmonary resuscitation was also studied. 3\. Biochemical markers (protein S-100, CK-BB and NSE) were studied in serum after birth asphyxia in defined time intervals to investigate their predictive value concerning the development of hypoxic-ischemic encephalopathy. After 20 months the patients' neurological development was examined and the predictive value of these brain-specific proteins in serum for neurodevelopmental outcome after birth asphyxia was studied. 4\. The appearance of protein S-100 in extra- cerebral fluids (native pericardial fluid, postoperative pericardial, pleural and peritoneal fluid) before and after heart operations in children was analyzed to gain better understanding of the protein S-100 concentrations after cardiac surgery. Results 1\. Comparison of different cerebral oxygenation indices The studies demonstrated, independently of the near- infrared spectrophotometer used, a correlation of the cerebral oxygenation index to the saturation in the venous jugular bulb as well as to the global oxygen saturation measured in the right atrium or in the superior caval vein. Comparison of the two near-infrared devices demonstrated better correlations in the INVOS 5100 device. 2\. Clinical applications of near-infrared spectroscopy Near-infrared spectroscopy measures the direct influence of sildenafil on the neuronal cell metabolism as it is able to detect increases in oxygenated haemoglobin. During circulatory arrest and cardiopulmonary resuscitation near-infrared spectroscopy detects reliable real-time hemodynamic changes. 3\. Biochemical serum markers for detection of hypoxic- ischemic encephalopathy and neurodevelopmental outcome A combination of serum protein S-100 and CK-BB after birth had the greatest value and highest specificity for the prediction of moderate and severe hypoxic-ischemic encephalopathy. On the other hand, neurodevelopmental examinations demonstrated that these parameters seem to be of limited value in predicting severe brain damage after birth asphyxia. 4\. Study of protein S-100 in different extra-cerebral fluids before and after cardiac surgery in children Protein S-100 is detectable in native pericardial fluid in children. After cardiac surgery it is detectable in pericardial, pleural and peritoneal fluid. Conclusions The studies presented here provide answers to questions concerning the reliability and clinical use of the neuromonitoring tool near-infrared spectroscopy. Ratings of the biochemical markers protein S-100, CK-BB and NSE are given in regard to the prediction of hypoxic-ischemic encephalopathy and neurodevelopmental sequelae

Intraoperative renal near-infrared spectroscopy indicates developing acute kidney injury in infants undergoing cardiac surgery with cardiopulmonary bypass: a case-control study

Introduction: Acute kidney injury (AKI) is a frequent complication after cardiac surgery with cardiopulmonary bypass in infants. Renal near-infrared spectroscopy (NIRS) is used to evaluate regional oximetry in a non-invasive continuous real-time fashion, and reflects tissue perfusion. The aim of this study was to evaluate the relationship between renal oximetry and development of AKI in the operative and post-operative setting in infants undergoing cardiopulmonary bypass surgery. Methods: In this prospective study, we enrolled 59 infants undergoing cardiopulmonary bypass surgery for congenital heart disease for univentricular (n = 26) or biventricular (n = 33) repair. Renal NIRS was continuously measured intraoperatively and for at least 24 hours postoperatively and analysed for the intraoperative and first 12 hours, first 24 hours and first 48 hours postoperatively. The renal oximetry values were correlated with the paediatric risk, injury, failure, loss, end (pRIFLE) classification for AKI, renal biomarkers and the postoperative course. Results: Twenty-eight (48%) infants developed AKI based on pRIFLE classification. Already during intraoperative renal oximetry and further in the first 12 hours, 24 hours and 48 hours postoperatively, significantly lower renal oximetry values in AKI patients compared with patients with normal renal function were recorded (P < 0.05). Of the 28 patients who developed AKI, 3 (11%) needed renal replacement therapy and 2 (7%) died. In the non-AKI group, no deaths occurred. Infants with decreased renal oximetry values developed significantly higher lactate levels 24 hours after surgery. Cystatin C was a late parameter of AKI, and neutrophil gelatinase-associated lipocalin values were not correlated with AKI occurrence. Conclusion: Our results suggest that prolonged low renal oximetry values during cardiac surgery correlate with the development of AKI and may be superior to conventional biochemical markers. Renal NIRS might be a promising non-invasive tool of multimodal monitoring of kidney function and developing AKI in infants undergoing cardiac surgery with cardiopulmonary bypass

Mode of Delivery and Pregnancy Outcome in Women with Congenital Heart Disease.

Advances in cardiac surgery and congenital cardiology have led to an increasing number of women with congenital heart disease (CHD) reaching childbearing age. In general, cardiologists recommend vaginal delivery for women with CHD to avoid complications from Caesarean section as many women with CHD tolerate vaginal delivery well.This is a single-center study comparing mode of delivery, pregnancy outcome, indications for Caesarean section and induction of labor between women with and without CHD. A historical cohort study was conducted including 116 patients with CHD. An individual threefold matching with 348 women without CHD was carried out. Caesarean section was performed in 46.6% of pregnancies with CHD (33.6% without CHD, P = 0.012). Primary Caesarean section increases with severity of CHD (P = 0.036), 33.3% of women with CHD had primary planned Caesarean section due to cardiac reasons. Induction of labor was performed in 45.7% of attempted vaginal deliveries in women with CHD (27.9% without CHD, P = 0.001). Lower mean birth weight (P = 0.004) and Small for Gestational Age (SGA) (P < 0.001) were more common in women with CHD. One CHD patient suffered from postpartum hemorrhage.Concerns about maternal deterioration resulting in higher rates of induction of labor seem unjustified in most cases. Along with a possible reduction of Caesarean section on maternal request, a reduction of planned vaginal delivery may be expedient in reducing the rate of Caesarean section in women with CHD

Complete Atrioventricular Septal Defects after the Age of 40 Years

Background: There is an increasing number of adults with complete atrioventricular septal defects (cAVSD). However, data regarding older adults are lacking. The aim of this study is to analyze the outcome of adults with cAVSD over the age of 40 years. Methods: Patients with cAVSD who were ≥40 years of age at any point between 2005 until 2018 were included retrospectively. Data were retrieved from hospital records. The primary endpoint was a combination of death from any cause and unplanned hospitalizations due to cardiac reasons. Results: 43 patients (60.5% female, mean age 43.7 ± 6.0 years, genetic syndrome 58.1%) were included. At begin of follow-up, the majority of patients (n = 41, 95.3%) was in New York Heart Association (NYHA) class I or II. Out of the whole cohort 26 (60.5%) had undergone cardiac surgery. At baseline, at least one extracardiac comorbidity was present in 40 patients (93.0%). Median follow-up was 1.7 years (IQR 0.3–4.6). On univariate Cox analysis, NYHA class at begin of follow-up (hazard ratio: 1.96, CI 95%: 1.04–3.72, p &lt; 0.05) was the only predictor for the primary endpoint. Conclusions: Significant morbidity and mortality is present in cAVSD patients over the age of 40 years. NYHA class is predictive for a worse outcome

Mode of delivery following induction of labor according to severity of CHD.

<p>Mode of delivery following induction of labor according to severity of CHD.</p

Mode of delivery according to severity of CHD.

<p>Mode of delivery according to severity of CHD.</p

Mode of delivery according to prior Caesarean section in women without CHD.

<p>Mode of delivery according to prior Caesarean section in women without CHD.</p