Differences in cardiac energetics between patients with familial and nonfamilial hypertrophic cardiomyopathy.
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Adenosine Triphosphate↗
Biomedical subjects
J Apitz
Publications and source records attributed to J Apitz.
31P NMR spectroscopy detects metabolic abnormalities in asymptomatic patients with hypertrophic cardiomyopathy.
BACKGROUND: Hypertrophic cardiomyopathy (HCM) often causes sudden, unexpected death in adolescents and young adults. Alterations in myocardial metabolism are considered to be causes for contractile dysfunction. We examined the question of whether metabolic abnormalities antedate the manifestation of symptoms in patients with HCM. METHODS AND RESULTS: Proton-decoupled 31P NMR spectroscopy of the anterior left ventricular wall of the heart of 14 young, asymptomatic patients with HCM was performed with a 1.5-T whole-body imager. Spectra of the phosphate metabolites were compared with those of normal control subjects. The patients exhibited a significantly reduced (P<0.02) ratio of phosphocreatine (PCr) to ATP of 1.98+/-0.37 (mean+/-SD), compared with 2.46+/-0.53 obtained in 11 normal control subjects. In addition, the group of patients with severe hypertrophy of the interventricular septum (n=8) showed a significantly increased (P<0.05) Pi-to-PCr ratio, with a Pi x 100/PCr of 20.0+/-8.3 versus 9.7+/-7.2 in control subjects. Both abnormalities are similar to those found in ischemic myocardium. This view is also supported by a significantly increased (P<0.01) phosphomonoester (PME)-to-PCr ratio, with a PME x 100/PCr of 20.7+/-11.2 compared with 8.4+/-6.7 in control subjects, indicating altered glucose metabolism. CONCLUSIONS: 31P NMR spectroscopy detects alterations of myocardial metabolism in asymptomatic patients with HCM. These alterations may contribute to the understanding of the pathophysiology and natural history of the disease.
Adolescent↗
Detection of phosphomonoester signals in proton-decoupled 31P NMR spectra of the myocardium of patients with myocardial hypertrophy.
Proton-decoupled 31P NMR spectroscopy at 1.5 T of the anterior left ventricular myocardium was used to monitor myocardial phosphate metabolism in asymptomatic patients with hypertrophic cardiomyopathy (HCM, n = 14) and aortic stenosis (AS, n = 12). In addition to the well-known phosphorus signals a phosphomonoester (PME) signal was detected at about 6.9 ppm in 7 HCM and 2 AS patients. This signal was not observed in the spectra of normal controls (n = 11). We suggest that in spectra of patients with myocardial hypertrophy the presence of a PME signal reflects alterations in myocardial glucose metabolism.
2,3-Diphosphoglycerate↗
Effects of cardiopulmonary bypass and inhaled nitric oxide on platelets in children with congenital heart defects.
UNLABELLED: Nitric oxide (NO) reduces platelet aggregation in vitro. However, repeated measurements of platelet aggregation in infants and small children are impossible due to the large blood samples required. Instead, the expression of different platelet receptors mediating platelet adhesion (CD 36 and CD 42b), activation (CD 42b and CD 61) and aggregation (CD 41a) was measured repeatedly by flow cytometry. First, the expression of platelet receptors was quantified in platelet suspensions of 20 healthy volunteers after incubation with different concentrations of NO (0, 25, 100 and 640 ppm) and compared to changes in platelet aggregation and intrathrombocytic cGMP levels. It was then studied in 21 infants and children before, during and up to 3 days after cardiopulmonary bypass surgery. Seven of these patients required NO inhalation postoperatively. The in vitro experiments showed a reduced expression of the CD 41a, CD 42b and CD 61 receptors with increasing doses of NO, predominantly affecting the CD 41a receptor (-11% at 100 ppm and -20% at 640 ppm). This significant effect is in keeping with the observed NO-induced inhibition of platelet aggregation (-44% at 100 ppm) and the rise in platelet cGMP levels (+69% at 100 ppm). In patients without inhaled NO, the expression of CD 41a was slightly attenuated during cardiopulmonary bypass surgery (-15%) but increased significantly afterwards (2 h: +31%, 1st day: +129%, 2nd day: +120%, 3rd day: +111%). Comparable results were obtained regarding the other adhesion molecules CD 36, CD 42b and CD 61. In patients with inhaled NO the same pattern was observed and analysis of variance did not reveal any significant difference between both groups of patients. CONCLUSIONS: NO (> or = 100 ppm) decreases the expression of different platelet adhesion molecules and platelet aggregation, presumably via an increase in intracellular cGMP. However, due to the low dose range used in the clinical setting (1-40 ppm) this is clinically not relevant. Immediately after cardiopulmonary bypass surgery the expression of these adhesion molecules is reduced, but recovers on the 1st postoperative day.
Administration, Inhalation↗
High-resolution cardiac imaging using an interleaved 3D double slab technique.
A three-dimensional (3D) gradient-echo sequence with interleaved double-slab excitation was developed and optimized for the requirements in pediatric cardiac imaging. For this purpose high contrast between blood and myocardium signal should be obtained without the use of contrast agents. An acceptable measuring time for a large region examined with high spatial resolution should be achieved as well, especially with regard to the small structures of the heart and vessels of infants. The presented approach works with gradient moment nulling and a short echo time of 5.5 ms resulting in generally high signal intensity and only minor signal losses due to turbulent flow. The sequence allows simultaneous ECG-gated recording of two separately excited slabs with small thickness (10 mm) and with a distance of several centimeters between them. Thus, common effects of presaturation in 3D imaging can be avoided, although a relatively short measuring time is achievable. In order to get a 3D data set with good signal homogeneity of blood and of the other structures across a large volume of interest several double-slab measurements with suitable positions must be performed. The latter aspect is especially important for postprocessing techniques as multiple planar reconstruction and maximum intensity projection. Examples of applications of the new technique and appropriately postprocessed images are presented allowing demonstration even of subtle cardiac malformations.
Artifacts↗
Proton-decoupled myocardial 31P NMR spectroscopy reveals decreased PCr/Pi in patients with severe hypertrophic cardiomyopathy.
Disturbed myocardial energy metabolism may occur in patients with primary hypertrophic cardiomyopathy (HCM). A noninvasive way to gain insight into cardiac energy metabolism is provided by in vivo 31P nuclear magnetic resonance (NMR) spectroscopy. 31P NMR spectroscopy with proton decoupling was performed in 13 patients aged 13-36 years with HCM on a 1.5 T Magnetom with a double resonant surface coil. A 2D chemical shift imaging (CSI) sequence in combination with slice selective excitation was used to acquire spectra of the anteroseptal region of the left ventricle (volume element: 38 mL). The chemical shifts of the phosphorus metabolites, intracellular pHi, and coupling constants J(alphabeta) and J(gammabeta) were calculated. Peak areas of 2,3-diphosphoglycerate (DPG), Pi, and adenosine triphosphate (ATP) were determined and corrected for blood contamination, saturation, and differences in nuclear Overhauser enhancements (NOE). The maximum thickness of the interventricular septum (IVSmax) was determined from tomographic long-axis images and expressed as number of standard deviations above the mean of the normal population (Z score). The patients were then divided into 2 groups: 6 patients with moderate HCM (HCMm, Z score < or = 5) and 7 patients with severe HCM (HCMs, Z score > 5). No differences between both groups and a control group of healthy volunteers (n = 16) were found with respect to phosphocreatine (PCr)/gamma-ATP ratio, pHi, or the coupling constants. Only the PCr/Pi ratio differed significantly from the control group (HCM(all), alpha < 0.05, HCMs, alpha < 0.02, 2-sided U test). The decrease of the PCr/Pi ratio in patients with HCM is probably caused by ischemically decreased oxygen supply in the severely hypertrophied myocardium.
Adenosine Triphosphate↗
Technical considerations for inhaled nitric oxide therapy: time response to nitric oxide dosing changes and formation of nitrogen dioxide.
UNLABELLED: The aim of the present study was to analyse the time response to nitric oxide (NO) dosing changes as well as the formation of nitrogen dioxide (NO2) with different ventilation systems, respirator settings and application sites during NO inhalation. The inspired NO and NO2 concentrations were continuously measured using chemiluminiscence within a dummy ventilatory system equipped with two different respirator systems (Siemens Servo 900c and Bear BP 2001). NO was either introduced into the afferent limb of the ventilatory circuit close to the endotracheal tube (site A) or into the so-called low pressure port of the Servo 900c respirator, far away from the endotracheal tube (site B). In addition, the decay of the inspired NO concentration after cessation of the NO gas flow was studied. This decay was considerably prolonged when NO was introduced at site B (time constants: tau = 7.19 min versus tau = 0.29 min). Within the concentration range studied (0-25 ppm NO) a linear correlation between the NO and NO2 concentration was found. At site A and an inspired oxygen concentration of > 0.95 NO2 formation amounts to 1.14% +/- 0.11% of the NO concentration. Using this value one can calculate the NO2 formation for a given NO dose. For example, when 40 ppm NO are applied, a concentration of 0.45 ppm NO2 can be expected, which is well below the relevant toxic concentrations. However, when NO was introduced at site B, NO2 formation was significantly increased to 1.61% +/- 0.16%. Passage of the ventilated gas through soda lime led only to a slight and insignificant reduction in NO2 concentration. The continuous flow respirator BP 2001 showed a significantly lower NO2 concentration when compared to the non-continuous flow respirator Servo 900c (0.64 +/- 0.11% vs.1.14 +/- 0.11%). CONCLUSION: The application of NO close to the endotracheal tube is associated with a much faster response of the actual inspired NO concentration to dosing changes and shows the lowest NO2 formation. In order to avoid toxic NO2 concentrations, an upper limit of 40 ppm NO is recommended for continuous NO inhalation.
Analysis of Variance↗
Pulmonary sling: morphological findings. Pre- and postoperative course.
Pulmonary sling (PS) is a congenital condition in which the left pulmonary artery (LPA) arises from the right pulmonary artery (RPA), forming a sling around the trachea causing tracheal compression. The incidence is not so rare as initially thought. Symptoms of severe airway obstruction often begin in the newborn or young infant. Echo-colour-Doppler may reveal the PS but emphysema can mask the typical findings. Deviation of fluid-filled lungs may be detected prenatally. Chest radiographs show unusual air distribution, deviation of heart and mediastinum and altered tracheobronchial angles. Bronchography and bronchoscopy demonstrate the high incidence of associated tracheal anomalies such as cartilagenous rings and long tracheal stenosis. Anterior oesophageal indentation is not always seen in the oesophogram. Magnetic resonance imaging (MRI) and computed tomography (CT) reveal the PS, but cautious interpretation is necessary because of different levels of the anomalous LPA. PS and associated cardiovascular malformations can be clearly detected by angiography. Associated extrathoracic anomalies are common. Early diagnosis and therapy of PS is mandatory and consists of reimplantation of the LPA into the pulmonary trunk and division of the ligamentum arteriosum. The postoperative course may be cumbersome necessitating bronchological interventions. Tracheal resection may be necessary but restenosis is frequent. A one-stage repair has been proposed in such cases and was successfully done in a few reported cases. Relief of respiratory obstruction is often complete when there are no associated tracheobronchial anomalies. Late postoperative course is favourable but respiratory obstructive attacks may occur with decreasing incidence over time and tracheal growth.
Abnormalities, Multiple↗
[Abnormal origin of the right coronary artery from the pulmonary artery. A case report].
An asymptomatic 4-year-old girl with anomalous origin of the right coronary artery from the pulmonary trunk is reported. Echocardiography showed overall dilated coronary arteries with anomalous course of the right coronary artery anterior to the aortic root. During heart catheterization the catheter could be moved from the proximal pulmonary trunk into the aberrant right coronary artery. Because of the coronary-pulmonary steal phenomenon corrective surgery with aortic reimplantation of the right coronary artery is recommended even in asymptomatic patients.
Aortography↗
[Improvement of perioperative hemodynamics and gas exchange by inhalation of nitric oxide in children with congenital cardiopulmonary defects].
OBJECTIVES: In critically ill infants and children before or after surgery for congenital cardiopulmonary disease it was evaluated whether continuous NO inhalation can reduce pulmonary artery pressure (PAP) and improve arterial oxygen saturation (SaO2). METHODS: All patients (n = 24; age 1 day-6.5 years) were intubated and artificially ventilated. They had either secondary pulmonary hypertension (n = 16), acute respiratory distress syndrome (n = 3), or reduced SaO2 (n = 5) due to pulmonary hypoperfusion. NO was introduced into the afferent limb of the ventilator circuit close to the endotracheal tube, while continuously measuring the inspired NO and O2 concentrations. The initially applied concentration of NO was 20 +/- 2.0 ppm. RESULTS: The hemodynamic condition and/or oxygen saturation was significantly improved by NO in 23 patients (95%). Mean PAP declined significantly from 45 +/- 7 to 28 +/- 3.7 mm Hg, while mean systemic arterial pressure remained constant (56 +/- 2.1 vs. 58 +/- 2.5 mm Hg). This was related to a selective reduction in pulmonary vascular resistance by 48 +/- 8.5%. SaO2 increased significantly (p < 0.05) from 83 +/- 2.5% to 93 +/- 1.5% due to a decreased intrapulmonary right-to-left shunt. NO therapy was applied with a median of 6 days (range 1.5-36 days). During NO inhalation methemoglobin concentration was significantly increased (0.77 +/- 0.05% vs. 1.46 +/- 0.15%), but neither was oxygen transport capacity affected, nor was any evidence for accumulation observed. Using a model ventilatory circuit, a nitric dioxide (NO2) formation of 1.14 +/- 0.11% of the applied NO concentration was measured, i.e. approximately 0.5 ppm NO2 at 40 ppm NO. This amount of NO2 in the inspired gas is well below toxicologically relevant concentrations. CONCLUSIONS: Low-dose NO inhalation selectively reduces PAP and improves SaO2 in children with congenital cardiopulmonary disease during perioperative intensive care. It is expected that the overall hemodynamic improvement is related to a reduced afterload of the subpulmonary ventricle without changes in coronary perfusion pressure, as is often observed with other vasodilators applied intravenously. We recommend an upper dose limit of 40 ppm NO for continuous NO inhalation to avoid possible toxicologically relevant NO2 concentrations.
Administration, Inhalation↗
[Legal problems within the framework of intensive care medicine].
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Critical Care↗
Noninvasive blood flow measurement and quantification of shunt volume by cine magnetic resonance in congenital heart disease. Preliminary results.
Based on the phase difference method as described by Nayler et al. we developed a gradient-echo sequence, which refocuses flow related phase shifts even for infants with their higher peak velocity, higher acceleration and faster heart rates. A repetition time (TR) of 15 ms provides a high temporal resolution for dynamic studies. Modification of the flow-rephasing gradient-echo sequence in slice select direction leads to a defined phase shift and the resultant phase difference images allow blood flow measurements in the great arteries and the calculation of blood volume per heart cycle (flow volume) to assess left and right ventricular stroke volume. This can also be achieved by calculation of the ventricular volume from contiguous slices of the whole heart, but, this in excessive measuring times. Both methods were applied in 6 examinations of children with congenital heart diseases (1 pulmonary sling, 1 coarctation of the aorta, 1 ventricular septal defect, 3 atrial septal defects). The age of the patients ranged from 3 months to 13.4 years (mean age 4.9 years). The regression analyses of both methods show a high correlation for systemic flow (y = -0.98 + 1.08 x, r = 0.99, SEE = 2.59 ml) and for pulmonary flow (y = -1.40 + 0.96 x, r = 0.99, SEE = 4.70 ml). The comparison of flow calculated Qp:Qs ratio and chamber size calculated Qp:Qs ratio with data obtained by heart catheterization show also a regression line close to the line of identity (y = -0.01 + 1.04 x, r = 0.98, SEE = 0.15 and y = 0.28 + 0.96 x, r = 0.81, SEE = 0.47, respectively).
Adolescent↗
[Anomalous origin of the left coronary artery from the pulmonary artery. Variability of clinical aspects, echocardiography and angiography findings].
OBJECTIVES: Analysis of symptoms, diagnostical difficulties and follow-up in infants and children with anomalous origin of the left coronary artery from the pulmonary artery. METHODS: Retrospective study; 12 children between 3 weeks and 2 years old; time period: 1980-1991. RESULTS: Three infants were detected on routine examination because of a new cardiac murmur, the others presented with signs of cardiac failure. Cross sectional echocardiography and color Doppler flow mapping allowed to verify the suspected diagnosis. However, in one infant a false-negative cross sectional echocardiographic result was obtained. In this case nuclear magnetic resonance imaging was able to delineate the exact anatomy. Mean preoperative left ventricular ejection fraction: 33 +/- 4%; percentage of infants below the age of 6 months: 92%; surgery related mortality: 66%; mean follow-up of the remaining 4 patients being in good clinical condition: 2.9 +/- 1 years. CONCLUSIONS: 1. An anomalous origin of the left coronary artery should be included into the differential diagnosis when a new cardiac murmur is detected. 2. Possibility of false-negative echocardiographic results is emphasized. 3. With early symptoms and highly reduced left ventricular function, the mortality is still high.
Angiocardiography↗
Early and late results of the modified Waterston shunt with PTFE grafts for palliation of complex congenital cyanotic heart disease in neonates.
During a 12 year period from 1978 to 1989, 35 infants under 4 weeks of age underwent palliative surgery for complex congenital cyanotic heart disease with a short (1-1.5 cm) PTFE graft between the ascending aorta and the right pulmonary artery (modified Waterston shunt). Twenty-three infants had pulmonary atresia and 14 had severe pulmonary stenosis. Underlying cardiac lesions were tetralogy of Fallot (n = 11), single ventricle (n = 7), transposition complexes (n = 6), and intact ventricular septum and hypoplastic right heart syndrome (n = 13). There were 4 early deaths (10.7%) in the entire series, 2 of which were shunt related. Three of the 4 occurred during our initial experience with this shunt in 1978 and 1979. They led to the modified Waterston shunt being abandoned for 3 years in favor of other shunt procedures. Since 1983 one early death occurred in 28 infants (3.5% mortality) with no death in the latest 26 patients. All patients were followed up between 6 and 108 months. There were 4 late deaths, one of which was shunt related. We observed a significant difference in the shunt patency rate between 4 and 5 mm grafts: palliation was adequate after 2 years in 52% of the patients when a 4 mm graft was used and in 89% of the 5 mm graft group (p less than 0.005). Reshunting was necessary in 7 infants between 5 and 60 months after primary surgery. Recatheterization was performed in 17 infants for suspected shunt failure (n = 6) or diagnostic reasons (n = 11).(ABSTRACT TRUNCATED AT 250 WORDS)
Actuarial Analysis↗
[Intensive care of children after heart surgery. Risk groups and possible complications].
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Cardiac Surgical Procedures↗
[Congenital coronary artery anomalies].
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Coronary Vessel Anomalies↗
[Mucocutaneous lymph node syndrome (Kawasaki syndrome) from the cardiological viewpoint].
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Angiography↗
Morphological diagnosis of congenital and acquired heart disease by magnetic resonance imaging.
Since 1987, the authors have examined 60 patients (21 girls, 39 boys) with a variety of congenital and acquired heart diseases by means of ECG-gated magnetic resonance imaging (MRI) using a multislice spin-echo technique. The patients' ages ranged from 10 days to 20 years (mean age 3.7 years), distributed as follows: 9 patients (15%) less than or equal to 4 weeks; 26 (43.4%) greater than 4 weeks less than or equal to 1 year; 9 (15%) greater than 1 year less than or equal to 6 years; 14 (23.3%) greater than 6 years less than or equal to 15 years; and 2 (3.3%) greater than 15 years less than or equal to 20 years. In 4 cases the quality of the images on the first study were of no diagnostic value and so a second investigation took place. Thus, 60 studies could be analysed, and the findings were compared with the previous diagnoses made on the basis of echocardiography (n = 60) and angiocardiography (n = 47). The 66 anomalies of the vessels included 6 that had been misdiagnosed (2, small patent ductus arteriosus (PDA); 1, pulmonary sling; 2, palliative shunt; 1, aortopulmonary collaterals). Amendment of the previous diagnosis was achieved in 8 cases. In 1 case an aorticopulmonary window was first detected by MRI and in another, a recoarctation of the aorta. The extent of an aortic aneurysm could be defined and a dissection of the aortic wall excluded. In 5 cases MRI gave more information on the pulmonary vascular status.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗