Prenatally diagnosed pseudoaneurysm of mitral-aortic intervalvular fibrous area.

The mitral-aortic intervalvular fibrosa (MAIVF) is an avascular, fibrous structure that provides continuity between the anterior leaflet of the mitral valve and the aortic valve. Pseudoaneurysm of the MAIVF is rare and has been most commonly described in adults and, more rarely, in children following cardiac surgery or endocarditis. Few reports have been published on cases with congenital pseudoaneurysm of the MAIVF. Here, we describe five cases of congenital pseudoaneurysm of the MAIVF identified prenatally and an additional six cases diagnosed postnatally. This is an unusual finding of varying clinical significance, which can be isolated or associated with complex congenital heart disease but, importantly, can be identified and monitored in the fetus. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Pharmacological therapy for patients with Kawasaki disease.

Kawasaki disease is a systemic vasculitis of unknown aetiology that has been reported worldwide since its initial description in Japanese children. The most significant sequelae of acute Kawasaki disease are related to the inflammation of small to medium sized arteries and, in particular, the development of coronary artery aneurysms. Because the aetiology is unknown, pharmacological therapy is nonspecific and directed towards modulation of the inflammatory response and inhibition of platelet activation with the aim of preventing coronary artery aneurysms. In the US, the recommended treatment for Kawasaki disease in the acute phase is a single, high dose of intravenous gammaglobulin (2 g/kg) and high dose aspirin (80 to 100 mg/kg/day). Use of this regimen has resulted in a significant decrease in the incidence of coronary artery abnormalities. Although the American Heart Association currently recommends high dose aspirin, moderate doses are used in Japan and the optimal dose of aspirin is not known. There has been renewed interest in the use of corticosteroids in the treatment of acute Kawasaki disease: however, their precise role remains unclear. Newer antiplatelet agents have also shown some promise in the treatment of patients with coronary artery aneurysms. Long term pharmacological therapy consists primarily of anticoagulation in patients with persistent coronary artery abnormalities. In this review, current recommendations for pharmacological therapy in Kawasaki disease are reviewed and some of the controversies in management of this disease, including management of patients who do not respond to initial therapy and the role of corticosteroids in the acute setting, are outlined.

Lifting of the degeneracy in semibullvalenes by remote and direct substituents: a quantitative study using variable-temperature carbon-13 NMR spectroscopy.

A series of six 1,5-(ethylmethyl)semibullvalenes (1a <==> 1a', 2 <==> 2', 3 <==>3') and two 4(2)-substituted semibullvalenes (4 <==> 4'), each undergoing Cope equilibria between nondegenerate valence tautomers, was investigated by carbon-13 NMR spectroscopy at a range of temperatures in several different solvents. Gompper's treatment of substituent perturbation was extended, specifically accounting for the effects of the substituents on chemical shifts, to allow the determination of the thermodynamic parameters for these skewed equilibria. These new treatments were used to determine the population difference (f - f ') between the valence tautomers and the perturbation thermodynamic quantities DeltaH(P), DeltaS(P), and DeltaG(P). The slow-exchange limit was reached for the parent 1,5-(ethylmethyl)semibullvalenes 3a <==> 3a' from which it was established that the preferred valence tautomer is 3a with the ethyl group on the cyclopropane ring. Despite considerable effort, the slow-exchange limit could not be reached in any of our other remotely substituted semibullvalenes. Provided that the ethyl group always prefers the cyclopropyl position as in 3a, the 1-ethyl-5-methylsemibullvalenes 1a, 2, and 3 are more stable by DeltaH(P) = 0.7-1.7 kJ mol(-1) than their valence tautomers 1a', 2', and 3'. In the directly substituted semibullvalenes (4 left harpoon ovet right harpoon 4'), the preferred valence tautomers 4a and 4b have the bromine atom or the nitrile group on the vinyl position (C(4)) rather than on the cyclopropane ring (C(2)) and are more stable than 4a' and 4b' by DeltaH(P) = 4.8 and 7.0 kJ mol(-1), respectively.

An unusual cause of severe cyanosis in infancy.

An infant presented with cyanosis due to a diaphragmatic Morgagni hernia compromising right ventricular diastolic filling and resulting in right-to-left atrial-level shunting as demonstrated by contrast echocardiography. There was complete resolution of cyanosis after repair of the hernia.

Quantitative assessment of ventricular function in children with single ventricles using the Doppler myocardial performance index.

Assessment of ventricular function in patients with functionally single ventricles and unusual ventricular geometry is difficult. The Doppler myocardial performance index (MPI) may provide a method of assessing function in these patients. The purposes of this study were to compare the MPI in normal children and in children with a functionally single ventricle, and to compare the MPI in patients with single ventricles before and after bidirectional cavopulmonary anastamosis (BCPA). Echocardiograms of all patients who underwent BCPA between January 1994 and December 1998 were reviewed before and after surgical palliation. Age-matched normal subjects (n = 30) served as controls. The MPI was calculated from Doppler tracings of ventricular inflow and outflow. Of the 60 patients with single ventricles who underwent BCPA, 41 had adequate preoperative Doppler studies to allow calculation of the MPI, and 21 of 41 had adequate studies before and after BCPA. The MPI was higher in patients with single ventricles than in normal controls (0.67 +/- 0.14 vs 0.32 +/- 0.10, p < 0.001). There was no significant difference in the MPI in patients with single ventricles before and after surgery (0.70 +/- 0.16 vs 0.66 +/- 0.15). However, patients who had a BCPA at < 1 year of age had a significant decrease in the MPI after surgery (0.71 +/- 0.10 vs 0.61 +/- 0.11, p = 0.01). Compared with controls, the MPI was significantly higher in patients with single ventricles suggesting an altered hemodynamic state consistent with decreased ventricular function. The MPI decreased in patients who underwent BCPA at < 1 year of age, suggesting an improvement in ventricular function. The MPI provides an objective method of assessing and following ventricular function in patients with single ventricles that is independent of ventricular geometry.

Usefulness of the preoperative tricuspid/mitral valve ratio for predicting outcome in pulmonary atresia with intact ventricular septum.

Although tricuspid valve z-scores have been used to predict outcome in pulmonary atresia with intact ventricular septum, they are statistically generated from local populations, and widespread generalization may not be appropriate. To determine if there are echocardiographic predictors of outcome that can be universally used, the records of all infants with this diagnosis since 1988 were reviewed for age, weight, type of surgery, and outcome. Preoperative and follow-up echocardiograms were reviewed for valve diameter and z-scores, and valve ratios were calculated. Thirty-six patients were divided into 2 groups: group 1 included 23 infants who had a successful biventricular repair; group 2 included the remaining 13 infants who did not have a successful repair. Preoperatively, both groups had similar ages, pulmonary, aortic, and mitral z-scores, and pulmonary/aortic ratios, but the patients in group 2 had significantly lower weight (3.5 +/- 0.6 vs 2.9 +/- 0.5 kg), tricuspid z-scores (-0.7 +/- 1.5 vs -2.3 +/- 1.2), and tricuspid/mitral ratios (0.8 +/- 0.2 vs 0.5 +/- 0.1). At similar follow-up, both groups of patients had similar weight, aortic and mitral z-scores, and pulmonary/aortic ratios, but group 2 infants had significantly lower pulmonary and tricuspid z-scores and tricuspid/mitral ratios. Compared with the preoperative echocardiograms, group 1 had significant increases only in pulmonary z-scores, and pulmonary/aortic and tricuspid/mitral ratios. Group 2 had no significant change in any echocardiographic variable. The tricuspid/mitral ratio was >0.5 in all group 1 infants, and in 6 of 13 group 2 infants (2 sepsis deaths, 4 palliations). Compared with a tricuspid valve z-score >-3, a tricuspid/mitral ratio >0.5 was a better predictor of biventricular repair. Thus, infants who have a successful biventricular repair have significantly greater preoperative weight, tricuspid valve z-scores, and tricuspid/mitral valve ratios. A tricuspid/mitral ratio >0.5 was the best predictor of a biventricular repair.

What is the yield of screening echocardiography in pediatric syncope?

OBJECTIVE: To determine the yield of screening echocardiography in the evaluation of pediatric syncope. DESIGN: All patients diagnosed with syncope from January 1993 to January 1999 were identified and their records were reviewed for age, weight, sex, year of presentation, personal and family history, physical examination, and cardiac diagnostic testing. Cardiac defects were identified by reviewing echocardiograms and reports. RESULTS: The 480 patients (268 females) ranged in age from 1.5 to 18.0 years old and ranged in weight from 10.3 to 113.6 kg. Final diagnoses included noncardiac causes in 458, long QT syndrome in 14, arrhythmias in 6, and cardiomyopathy in 2. An abnormal history, physical examination, or electrocardiogram identified 21 of the 22 patients with a cardiac cause of syncope. Of the 322 (67%) echocardiograms performed, abnormalities were detected in 37. These abnormalities included 26 minor valve anomalies, 7 hemodynamically insignificant shunt lesions, 2 mildly decreased left ventricular shortening fractions, and 2 cardiomyopathies. Only the 2 cardiomyopathies were considered to be potential causes of syncope, and in both cases, the electrocardiogram was markedly abnormal. A similar percentage of echocardiograms were ordered during the first and last 3 years of the study (61% vs 71%). CONCLUSION: History, physical examination, and electrocardiography provide a screening protocol that allows the identification of a cardiac cause of syncope in the overwhelming majority of pediatric patients. In the absence of a positive screen result, the echocardiogram does not contribute to the evaluation of syncope in children. We speculate that primary care providers and pediatric cardiologists continue to use echocardiography because of the paucity of data regarding its value in pediatric syncope. However, this study shows little benefit of screening echocardiography and should discourage its routine use.

End-systolic stress-velocity and pressure-dimension relationships by transthoracic echocardiography in mice.

The purposes of this study were to assess load-independent, end-systolic relationships in mice and compare these relationships to ejection phase indexes in assessing contractility. In 13 mice, ejection phase indexes (shortening fraction and velocity of fiber shortening) and end-systolic relationships [pressure-dimension relationship (ESPDR) and stress-velocity relationship (ESSVR)] were determined using M-mode echocardiography and simultaneous left ventricular pressure. Load was altered with phenylephrine and nitroprusside. Contractility was increased with dobutamine and decreased by induction of hypothyroidism. Ejection phase indexes increased with dobutamine infusion but were not significantly decreased with hypothyroidism. However, end-systolic relationships changed significantly with both dobutamine (gamma-intercepts: ESPDR from 22 to 48 mmHg, ESSVR from 3.7 to 6.6 circ/s, P < 0.05) and hypothyroidism (gamma-intercepts: ESPDR from 22 to 11 mmHg, ESSVR from 3.7 to 3.2 circ/s, P < 0.05). We conclude that end-systolic indexes can be accurately measured in the intact mouse by echocardiography with simultaneous left ventricular pressure recording and appear to be more sensitive to inotropic state than ejection phase indexes.

The structures of different diastereomers of bicyclo[2.2.1]hept-5-en-2-yl phenyl sulfoxide.

Three diastereomers of bicyclo[2.2.1]hept-5-en-2-yl phenyl sulfoxide were prepared by Diels-Alder [4 + 2] cycloadditions between phenyl vinyl sulfoxide and cyclopentadiene. The isomers were separated by column chromatography on silica gel and repeated recrystallizations gave the pure racemates of three of the four possible diastereomers. It proved to be impossible to assign the stereochemistry of the products from low-resolution NMR spectra. The X-ray diffraction studies of the three diastereomers showed the relative configuration at the two chiral centers and these stereochemical assignments were, subsequently, correlated with the two-dimensional NMR spectroscopic results. Compound (I), exo-(2R*,8S*)-bicyclo[2.2.1]hept-5-en-2-yl phenyl sulfoxide [or the exo-(2S*,8R*)-isomer], C13H14OS, Mr = 218.31, orthorhombic, P2(1)2(1)2(1), a = 10.517 (2), b = 10.914 (2), c = 9.642 (3) A, V = 1106.7 A3, Z = 4, Dx = 1.31 g cm-3, Cu K alpha, lambda = 1.54178 A, mu = 22.14 cm-1, F(000) = 464, T = 138 (2) K, R = 0.042 for 1151 data. Compound (II), exo-(2RS,8RS)-bicyclo[2.2.1]hept-5-en-2-yl phenyl sulfoxide, C13H14OS, Mr = 218.31, triclinic, P1, a = 8.775 (4), b = 16.353 (8), c = 7.804 (3) A, alpha = 90.67 (3), beta = 101.08 (4), gamma = 85.64 (5) degrees, V = 1095.8 A3, Z = 4, Dx = 1.32 g cm-3, Mo K alpha, lambda = 0.71069 A, mu = 2.18 cm-1, F(000) = 464, T = 138 (2) K, R = 0.062 for 3264 data. The two molecules in the asymmetric unit are crystallographically independent, but their conformations are similar.(ABSTRACT TRUNCATED AT 250 WORDS)

Serum magnesium and copper levels in myocardial infarction.

The serum levels and urinary excretion of magnesium and copper were studied in 66 patients with either acute myocardial ischemia or myocardial infarction. Serum for magnesium and copper determinations was obtained daily for three days. The initial serum magnesium levels were normal in patients with ischemia but were low in some patients with myocardial infarction. Patients developing ventricular arrhythmias with myocardial infarction showed the lowest levels of serum magnesium. Copper in the serum appeared elevated in patients developing acute congestive heart failure but the elevation was not statistically significant. These data indicate that a decrease in serum magnesium as evaluated may be associated with ventricular arrhythmias in patients with myocardial infarction; thus continued observations of magnesium levels in the myocardial infarction patient is warranted.

The metabolism of zinc and selenium in cirrhotic patients during six weeks of zinc ingestion.

Zinc metabolism in cirrhotic patients was studied, indicating some improvement that did not alter the abnormalities of zinc metabolism significantly and showed no evidence of correlation of abnormalities with the clinical improvement or lack thereof in the patients studied.

Tissue trace element and enzyme content in pigs fed a low manganese diet. I. Arelationship between manganese and selenium.

Pigs received a diet containing 0.59 p.p.m. Mn. Control pigs received the samequantity of the same diet which was supplemented with 22 p.p.m. of Mn. At the end of6 weeks, pigs fed the low Mn diet had a diminution in tissue Mn levels as well as a diminution in tissue Se content. Tissue levels of Zn, Cu, Ca, and Mg were notaffected by the dietary regimen. Indicative of the fact that experimental animals were probably Mn-deficient was a statistically significant decrease in hepatic arginaseactivity that could be enhanced by the addition of Mn. These studies represent the first experimental evidence that there may be a specific interaction between tissue Se and Mn. Tissue Se levels were decreased to a statistically significant degree in all tissues studied except the kidney in the Mn-deficient animals.

Serum and tissue enzyme activity and trace-element content in response to zinc deficiency in the pig.

Zinc deficiency is associated with poor growth and development. It has been postulated that a deficiency of the various zinc metalloenzymes results from the lack of dietary zinc. From present study, an examination of some aspects of this postulate, we conclude the following about zinc deficiency in pigs: (1) Of the tissues studied, it results im diminished zinc content only in liver, kidney, and pancreas. (2) It is associated with tissue abnormalities of various trace elements other than zinc. (3) Histochemical enzyme assays are satisfactory for qualitative but not quantitative determination of zinc metalloenzymes. (4) Deficiency of various tissue zinc metalloenzymes is difficult to demonstrate, even in severe zinc deficiency. (5) It is associated with decreased activity of hepatic leucine aminopeptidase and ornithine transcarbamylase, and decreased serum cholesterol concentrations. Our findings are consistent with the postulate that alterations in a number of trace elements may occur in the entity called zinc deficiency.