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.

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.

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.

[Clinico-histologic-morphometric correlations in pulmonary arteries among patients with heart malformations ]. / Klinisch-histologisch-morphometrische Korrelationen an Lungenarterien bei angeborenen Herzfehlern.

To improve the evaluation of grades of pulmonary vascular lesions in congenital heart malformations, we studied the extent to which there is a measurable relationship between pressure conditions in the pulmonary circulation and the area of the media in small and medium-sized pulmonary arteries, and the possibility of a correlation between the subjective evaluation of grades of hypertensive pulmonary vascular lesions according to Heath and Edwards (1958). Material and methods. The lungs in 68 children (mean age, 22.7 months) with congenital malformations of the heart or great vessels resulting in pulmonary hypertension were examined. The lungs were fixed in a 4% formalin solution passed via the trachea under a constant filling pressure of 150 cm H2O. Peripheral as well as central tissue was removed from all lobes of the lungs; the specimens were stained with Elastica-van Gieson. The extent of hypertensive pulmonary vasculopathy was staged, without knowledge of the pressure conditions, according to the grading system developed by Heath and Edwards. The diameter and the area of all muscular arteries with diameters smaller or larger than 100 micron were measured using a semiautomatic measurement device for quantitative morphometric analysis (MOP/AM 01). Cardiac catheter values were available for all cases. The quotient of systolic pressures in the pulmonary artery and the aorta was taken as the measure of hemodynamic conditions in the pulmonary circulation. The Pearson-Bravais correlation coefficient (r) was computed from the respective area quotient and the corresponding pressure values. In addition, the coefficient of determination (r2) and regression functions were determined. Results. A linear correlation (r = 0.70) exists between the pressure quotient (Psyst. pulm. art./ Psyst. aorta) and the vessel area quotient (media area/total area). The correlation is expressed by the following functions: x = 1.89 y - 0.08 y = 0.26 x + 0.24 Using the Heath and Edwards grading, the following frequencies were obtained: Grade 0: 21, Grade I: 7, Grade II: 16, Grade III: 15, Grade IV: 6, and 3 children were undeterminable. No relationship exists between the grades and certain heart malformations. In spite of the fact that hypertensive vasculopathy becomes progressively more severe as the child grows older, we found four cases of Grade IV in children under the age of one. On the whole, a comparison of the measured area quotients and the Heath and Edwards grading showed a good correlation. Although we considered only pressure quotients and no other hemodynamic parameters, conclusions can be drawn about the operability of congenital heart malformations based on these findings. Using measured pressure values, the morphologic state of the pulmonary arteries can be approximately evaluated.

[Aberrant pulmonary vessels from the abdominal aorta in children with congenital heart disease (author's transl)]. / Aberrierende Lungengefässe aus der Aorta abdominalis bei Kindern mit angeborenem Herzfehler

Ten children are described in whom aberrant pulmonary vessels arising from the abdominal aorta were found in the course of cardiac investigations. In five, sequestrated lung, as described Pryce, was demonstrated; in the others, these vessels were found incidentally without any demonstrable abnormality in the lung. Our findings indicate that one must distinguish between aortic pulmonary vessels with and without pulmonary sequestration during childhood. The need for deliberate opacification of the aorta is pointed out. This is also necessary in arriving at the indications for sergery. In the presence of congenital heart defects, these arterial vessels are found particularly in the presence of hypoplastic lungs and with the scimitar syndrome (3 cases).

Repair of symptomatic aortic coarctation in the first three months of life. Early and late results after resection and end-to-end anastomosis and subclavian flap angioplasty.

During a 9 year period between January 1977 and December 1985, 98 consecutive infants under 3 months of age underwent surgical repair of symptomatic aortic coarctation. Resection and end-to-end anastomosis was performed in 73, subclavian flap angioplasty in 14, and other procedures in 11 patients. There were 20 (20.5%) early and 12 (12.5%) late deaths. No early deaths occurred in the isolated coarctation group. Associated complex cardiac malformations and age under 2 weeks at operation influenced significantly early and late outcome but not any particular surgical procedure. The survivors were followed from 6 months to 8 years and 8 months postoperatively. There were 16 (28%) re-coarctations among 56 survivors after end-to-end anastomosis requiring re-operation in 7 (12%) infants and 3 (30%) re-coarctations among 10 survivors after subclavian flap angioplasty requiring re-operation in 1 infant. After end-to-end anastomosis re-coarctation as well as re-operation rate was markedly lower when an interrupted suture line for the entire anastomosis was used as compared to the group with a continuous suture line of the posterior aortic wall (21% vs. 33% re-coarctation rate and 4% vs. 18% re-operation rate respectively). From our results it is concluded that subclavian flap angioplasty for relief of aortic coarctation in early infancy is not superior to resection and end-to-end anastomosis. In the end-to-end anastomosis group an interrupted suture line has a lower re-coarctation as well as re-operation rate as compared to a continuous suture line of the posterior aortic wall.

[Differential diagnosis and prognosis of primary and post-myocarditis cardiomyopathy in childhood]. / Zur Differentialdiagnose und Prognose der primären und postmyokarditischen Kardiomyopathie im Kindesalter.

It is still difficult to differentiate between the various stages of myocarditis and primary dilated cardiomyopathy (DCM). Hence, we analyzed the history, as well as the laboratory and virological data of 22 children with a dilated, poorly functioning left ventricle, aged 2 months to 16.7 years (m = 4.2 y), on whom we had performed endomyocardial biopsies about 4 months after the beginning of the illness. Specimens were investigated by light and electron microscopy and, in addition immunoserological (n = 15) and immunohistological investigations (n = 7) were performed. On the basis of cellular infiltration in the histological examination we diagnosed resolving/resolved myocarditis in 6 patients and DCM in 15 patients. Previous respiratory infection or sudden onset were found in 40-50% of patients in both groups. Antimyolemmal and antisarcolemmal antibodies showed no preference. Follow-up (3.1 +/- 2.8 years) of the patients showed equal mortality (33% vs. 38%) in the post-myocarditis and DCM group. Complete normalization of all findings in 3 patients of the DCM group makes the classification among the post-myocarditis group probable, which would, then give a lower mortality rate (22% vs. 46%) and a chance of restitution in 55% of cases. In any one specific case all the diagnostic methods do not allow undoubted distinction between both entities. The prognosis of myocarditis seems to be better than that of DCM in childhood.

[Secondary diseases of the heart muscle and their differential diagnosis in childhood]. / Sekundäre Herzmuskelerkrankungen und ihre Differentialdiagnose im Kindesalter.

Possible causes of specific cardiac muscle disease, diagnosis, follow-up and the therapeutic management are discussed on the basis of a series of cases. 8 out of 30 patients who showed the clinical picture of dilative cardiomyopathy (DCM) were found to have a specific cardiac muscle disease. 4 patients had DCM following adriamycin therapy. Fibromuscular dysplasia with renal hypertension, thalassaemia major with secondary haemosiderosis, long-overlooked and untreated athyroidism each caused one case of dilative specific cardiac muscle disease. Once DCM was preceded by the Kawasaki syndrome for over 2 years. Amongst 47 patients with hypertrophic cardiomyopathy there were two children who had undergone ACTH treatment, 6 children born of diabetic mothers, 4 cases of Pompe's disease, and one patient with hypothyroidism resulting in reversible hypertrophy of the cardiac muscle. Different neurodegenerative diseases were associated with cardiac muscle disease in 4 cases, partly dictating the clinical course. Extremely rare was the development over 6 years of cardiac hypertrophy following a burns injury.

[Abnormal origin of the right coronary artery from the pulmonary artery. A case report]. / Fehlabgang der rechten Koronararterie aus der Arteria pulmonalis. Eine Fallbeschreibung.

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.

[Transumbilical heart diagnosis and ballon atrioseptostomy]. / Transumbilikale Herzdiagnostik und Ballonatrioseptostomie

The way of catheter insertion through the umbilical vein is presented. We attempted the transumbilical heart catheterization in 63 patients, in 14 we failed to make an exact diagnosis. Furthermore, in 17 out of 26 patients we could perform the balloon atrial septostomy transumbilically. The possible complications are discussed and this access is recommended as an elegant and time-saving method.

Primary heart tumors in infancy and childhood. Report of four cases and review of literature.

Primary heart tumors are rare. The findings in 4 children with heart tumors are reported: a rhabdomyosarcoma, a rhabdomyoma, a fibroma and a myxoma. The symptoms depend upon the location (intrapericardial, intramural or intracavitary). ECG and X-ray provide no essential diagnostic information; echocardiography can give a decisive diagnostic indication in parietal and intracavitary tumors. Surgery provides a specimen for histology, but the tumor cannot always be removed. The prognosis, therefore, depends upon the result of surgery, but malignant tumors, mostly being diagnosed too late, have a very bad prognosis.

Primary rhabdomyosarcoma of the heart.

A primary rhabdomyosarcoma of the heart observed in a 3-week-old infant, provides an opportunity to review this rare clinical entity; 61 reported cases are collated. The more common sites are right ventricle, left atrium, and right atrium; the tumour may protrude into the cavity and cause obstruction. Echocardiography can demonstrate the tumour, which may also been seen as a filling defect on angiography. Primary heart tumours seldom occur, especially in infancy and childhood. According to Van der Hauwaert, the most common cardiac tumour in infancy is the rhabdomyoma. No primary malignant tumour was seen in 15 European centres within a 5-year period.

[Interventional cardiology in children]. / Interventionskardiologie im Kindesalter.

Interventional heart catheterizations are catheterizations for a therapeutic purpose. The widest spread example is the balloon atrioseptostomy (BAS) according to Rashkind, which is performed in patients with transposition of the great arteries preceding interatrial corrective surgery, in patients with right-sided valve atresias and interatrial obstruction and in patients with complex mitral atresia. In 248 BAS, performed within 20 years we observed 26 (= 10.5%) minor and 3 (= 1.2%) lethal complications. When the BAS is ineffective or the atrial septum very muscular, the Park-blade-septostomy may be performed, provided corrective surgery is out of question. intravasal foreign bodies, e.g. catheter fragments can be extracted without harm and risk by the Dotter retriever. This prevents grave complications. Arteriovenous aneurysms, coronary fistulas or aortopulmonary collaterals can be embolized by steel coils, detachable balloons or Ivalon particles. With increasing frequency patent ducts, seldom also atrial or ventricular septal defects are closed by means of the Rashkind occluder. Balloonvalvuloplasty (BVP) of pulmonary or aortic stenosis became generally accepted. In cooperative studies of the German Society of Pediatric Cardiology in 305 BVP's of pulmonary stenosis 4% complications with one late death have been observed. In aortic stenosis of children early mortality was 1.6%, complication rate 20%, in the critical aortis stenosis of infants early mortality was 19%, complication rate 50%. So BVP of pulmonary and non-critical aortic stenosis may be considered as the treatment of choice, while BVP of critical aortic stenosis and other stenosis of valves or vessels has to be further evaluated.

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)

[Cardiomyopathies in childhood]. / Kardiomyopathien im Kindesalter.

Primary cardiomyopathies are heart muscle diseases of unknown cause. In hypertrophic cardiomyopathy (HCM) a hypertrophy of unknown aetiology results either in asymmetric septal hypertrophy (mostly with obstruction) or in concentric hypertrophy of the left ventricle (mostly without obstruction). This condition is rare in childhood, partly inherited and sometimes observed already in newborns. Sudden cardiac death is a main complication in the second decade. In non-obstructive HCM endomyocardial biopsy is the tool to differentiate secondary forms. Dilated cardiomyopathy is recognized by aetiologically unclear dilatation of left or right or both ventricles. In childhood this condition has to be differentiated from coronary anomalies, endocardial fibroelastosis, and myocarditis. Also rare secondary forms have to be considered because of possible therapeutic consequences. From the very few histologically proven reports a reliable prognosis for this disease in childhood cannot be deduced.

Dilated cardiomyopathy in childhood: problems of diagnosis and long-term follow-up.

Clinical profile and course of 13 infants and children (median age 2 years, range 2 months to 17 years) with dilated cardiomyopathy are presented. Endocardial fibroelastosis and myocarditis were excluded by transvascular endomyocardial biopsy. Elevated cardio-thoracic ratio (mean 0.67), depressed fractional shortening (mean 13%) and cardiac index (mean 2.52 l min-1 m-2) were the indicators of congestive heart failure. During a mean follow-up of 41 months (range 6 to 204 months) five patients died, four remained in a stable condition, four improved. The main complications were rhythm disturbances (23%) and thrombus formation (15%). We could not identify any predictors of survival.