Late cardiotoxicity after treatment for a malignant bone tumor

Cardiac function was assessed in long-term survivors of malignant bone tumors who were treated according to Rosen's T₅ or T₁₀ protocol, both including doxorubicin. Thirty-one patients, ages 10–45 years (median age 17.8 years) were evaluated 2.3–14.1 years (median 8.9 years) following completion of treatment. Cumulative doses of doxorubicin were 225–550 mg/m² (median dose 360). The evaluation consisted of a history, physical examination, electrocardiogram (ECG), signal averaged ECG, 24-hour ambulatory ECG, echocardiography and radionuclide angiography. Eighteen of 31 (58%) patients showed cardiac toxicity, defined as having one or more of the following abnormalities: late potentials, complex ventricular arrhythmias, left ventricular dilatation, decreased shortening fraction, or decreased ejection fraction. The incidence of cardiac abnormalities increased with length of follow-up (P ≤ .05). No correlation could be demonstrated between cumulative dose of doxorubicin and cardiac status, except for heart rate variability. When adjusted to body surface area, the left ventricular posterior wall thickness (LVPW index) was decreased in all patients. The incidence of doxorubicin-induced cardiotoxicity is high and increases with follow-up, irrespective of cumulative dose. Life-long cardiac follow-up in these patients is warranted. The results of our study suggest that heart rate variability and LVPW index could be sensitive indicators for cardiotoxicity. © 1996 Wiley-Liss, Inc.     

M-mode echocardiographic findings in children with idiopathic restrictive cardiomyopathy

Summary The M-mode echocardiographic findings in five pediatric patients, ages 4–15 years, with primary idiopathic restrictive cardiomyopathy, diagnosed by cardiac catheterization, and of 12 normal children (control group) are presented. The M-mode echocardiographic findings in patients with restrictive cardiomyopathy were (1) normal left and right ventricular end-diastolic dimension, (2) normal left ventricular posterior wall and interventricular septal thickness (three patients) or mild concentric hypertrophy (two patients), (3) normal opening and closing velocity of the mitral valve, (4) consistently enlarged left atrium (more than 40 mm) in all, and (5) right ventricular systolic time intervals compatible with pulmonary artery hypertension. The left ventricular ejection phase parameters (systolic time intervals, shortening fraction, and mean velocity of circumferential fiber shortening) were normal. Left ventricular relaxation phase parameters (diastolic function) were abnormal. The isovolumic relaxation time index was prolonged, 68±40 ms (±SD), in the study group as compared with 11±6 ms (±SD) in the control group (P<0.001). Percent relaxation of left ventricular posterior wall endocardium at 50% of diastole was decreased, 58±4% (±SD), in the study group as compared with 85±6% (±SD) in the control group (P<0.005). We conclude that M-mode echocardiography provides arelatively useful and specific noninvasive method for the diagnosis of primary restrictive cardiomyopathy in pediatric patients. This work was supported in part by NHLBI grant HL07436.     

Echocardiographic profile of infants of diabetic mothers

Echocardiographic M-mode examinations were performed in 30 infants of diabetic mothers (IDM). Twelve of them had severe symptoms with hyperbilirubinemia, respiratory distress syndrome, septicemia and hypoglycemia in the neonatal period. The gestational age ranged from the 34th week to the 40th week, the birth weight ranging between 2280 g and 5820 g (mean 3455 g). Fifteen patients were restudied at different ages. In group A (age 1-14 days) out of 23, in group B (age 14 days-6 months) 5 from 13 IDM and in group C (older than 6 months, (n=10) no IDM had an increased ratio of intraventricular septal thickness (IVS) to left ventricular posterior wall thickness (LVPW). One patient showed a systolic anterior motion of the mitral valve (SAM) without having other symptoms for a left ventricular outflow tract obstruction. All measured dimensions normalized within 6 months. It is concluded that a form of prematurity may be responsible for the transient, asymmetrical, septal hypertrophy.     

Late echocardiography assessment of systolic and diastolic function of the left ventricle in pediatric cancer survivors after anthracycline therapy

BACKGROUND: The usefulness of daunorubicin (DAUNO) and doxorubicin, members of the anthracycline class of anticancer drugs, is limited by their cardiotoxicity. The purpose of our echocardiographic study was to assess the left ventricular (LV) function in long-term pediatric cancer survivors who had received DAUNO and dox as part of their therapy. PATIENTS AND METHODS: Seventy patients and 70 age, sex, and body surface area matched healthy controls were evaluated. Among the patients, the mean cumulative anthracycline dose was 321.6 (range, 150 to 868 mg/m); the mean interval from cancer diagnosis to evaluation was 13.9 years (range, 7 to 30 y). RESULTS: Fifteen of the 70 (21.4%) patients had a reduced ejection fraction (EF). Compared with the healthy controls, the mean EF and E/A were decreased to low normal levels in the patients, whereas deceleration time and isovolumetric relaxation time were prolonged. The E/A and deceleration time were significantly different for the females but not the males. The Tissue Doppler Index was normal but the Myocardial Performance Index was prolonged and correlated with EF (r=-0.499, P<0.001). There was a correlation between EF with cumulative anthracycline dose (r=-0.306; P=0.010) and time off therapy (r=-0.281; P=0.019). Diastolic indices suggested a tendency toward abnormal LV relaxation. Myocardial Performance Index seems to be a good index for monitoring LV status, because it was prolonged as EF decreased. CONCLUSIONS: This study suggests that long-term survivors who received doxorubicin and DAUNO may be found to have subclinical features of myocardial dysfunction when evaluated years after the completion of therapy.     

Interventricular septal motion and left ventricular function in patients with atrial septal defect

Summary In order to assess whether the paradoxical motion of the interventricular septum seen in patients with atrial septal defect (ASD) is due to a true abnormality in septal contraction, eight patients with ASD (age, 1.6–17 years) and eight age-matched control patients were studied using qualitative and quantitative two-dimensional (2D) and M-mode echocardiography. 2D-echocardiographic images recorded from the parasternal short-axis projection at the level of the papillary muscles and 2D-directed M-mode tracings at this level were obtained. Comprehensive wall motion analysis of the left ventricular (LV) endocardial and epicardial borders was performed using both fixed reference and center of mass (floating reference) models.Our results indicate that interventricular septal wall motion and function are normal in patients with ASD. The apparent paradoxical motion is due to excessive anterior motion of the entire left ventricle, and is present only when a fixed reference system is used to assess myocardial motion, but is not present when a center of mass (floating reference system) is employed. Left ventricular function assessed by % area and perimeter change, mean radial shortening fraction, and mean radial wall thickening (2D) as well as LV shortening fraction and septal and posterior wall thickening (M-mode) was not significantly different between the two groups. Standard M-mode tracings can therefore be used to assess LV function despite this apparent abnormal septal motion.     

Left ventricular function in beta thalassaemia major.

The left ventricular dimension and posterior wall dynamics were studied by computer assisted analysis of M mode echocardiography in 25 normal children (group 1) and 32 transfusion dependent children with beta thalassaemia major who had no evidence of heart failure (group 2). Twenty seven of those in group 2 remained well but five died of cardiac decompensation within 12 months. Compared with group 1, the left ventricular fractional shortening and ejection fraction were normal in those in group 2 who survived but diminished in those who died. Evaluation of left ventricular dimension and posterior wall dynamics during systole (peak shortening rate, peak velocity of circumferential fibre shortening, and peak posterior wall thickening rate) showed similar findings in that only the group who died had abnormal values. The left ventricular dimension and posterior wall diastolic dynamics (peak relaxation rate, normalised peak relaxation, peak wall thinning, and normalised peak wall thinning rate), however, showed progressively slower rates in all the children in group 2. The findings suggest that left ventricular diastolic dysfunction occurs early in myocardial impairment in patients with beta thalassaemia major. When there are abnormalities in both diastole and systole, the myocardial impairment is advanced and the prognosis is poor.     

Cardiac effects of a single course of antenatal betamethasone in preterm infants

AIM: To show the effects of a single course of antenatal betamethasone on cardiac measurements and systolic functions in premature newborn infants. METHODS: Seventy six newborn infants with a gestational age of 25-33 weeks were included in the study. They were first classified according to their gestational age: 25-29 weeks (n = 28) and 30-33 weeks (n = 48). They were then reclassified as betamethasone positive (mother received one course of betamethasone) or betamethasone negative (mother did not receive any antenatal glucocorticoid treatment). Cross sectional M mode echocardiographic scans were performed during the first three postnatal days and at the end of the first and third weeks. Left interventricular septum (IVS), left ventricular posterior wall (LVPW), left ventricular end diastolic (LVED), and left ventricular end systolic (LVES) dimensions, aortic root (AO), and left atrial diameters (LAs) were measured. The IVS to LVPW ratio was calculated to identify asymmetrical septal hypertrophy. RESULTS: In neither group was any statistically significant difference noted in IVS, LVED, LVES, LVPW, LA, and AO measurements during the three cardiac ultrasonography scans. Systolic function, as assessed by fractional shortening, was not significantly different in infants who received betamethasone antenatally, in either age group. There was no difference in the IVS/LVPW ratios between those who received antenatal steroid and those who did not for the 25-29 week and 30-33 week groups during these three consecutive scans. CONCLUSION: One course of antenatal betamethasone did not affect the cardiac wall thicknesses and systolic function in premature infants.     

Echocardiographic evaluation of mitral valve in patients with pure rheumatic mitral regurgitation

The aims of this study were to evaluate the structure of the mitral valve (MV) and subvalvar apparatus in patients with rheumatic mitral regurgitation (MR) by echocardiography and to compare the differences in morphologic abnormalities between subgroups of patients with and without mitral valve prolapse (MVP). Two-dimensional and color Doppler echocardiographic examinations were performed in 20 consecutive patients with isolated rheumatic MR and in 15 healthy subjects as controls. Annular diameter, left ventricular end-diastolic dimension, anterior leaflet length, and both leaflet thicknesses were greater in MR than those of controls. Anterior leaflet and chordal lengths were greater in severe MR than in mild or moderate MR. Sixty percent of rheumatic MR patients had nodules on the body or tip of the anterior mitral leaflet and MR was more severe in these patients. Nine of 20 patients (45%) had MVP. MR was more severe in the patients with MVP than those without prolapse. Rheumatic etiology should be suspected in patients with MR when irregular focal thickening of MV, relatively immobile posterior leaflet, eccentric regurgitant jet, and anterior MVP are found in echocardiographic study.     

Early predictors of myocardial disease in children and adolescents with type 1 diabetes mellitus

Background: The spectrum of diabetic heart disease involves a progression from normal heart to preclinical left ventricular diastolic and systolic dysfunction followed by overt echocardiographic evidence of left ventricular (LV) dysfunction and finally symptomatic heart failure. Objective: To compare the value of tissue Doppler imaging (TDI) over the conventional echocardiography in the assessment of early myocardial dysfunction in type 1 diabetics in correlation with serum N‐terminal pro‐brain natriuretic peptide (NT‐pro‐BNP), state of metabolic control, and diabetes duration. Methods: Sixty subjects were included; 40 type 1 diabetics (aged 12–18 years). Twenty matched subjects served as controls. They were subjected to clinical examination with assessment of cardiovascular reflexes for autonomic neuropathy. Laboratory investigations included mean random blood sugar (MRBS), hemoglobin A1c (HbA1c), urinary microalbumin, and serum determination of NT‐pro‐BNP. Echocardiography for chamber dimensions, systolic and diastolic function, Tie index, and longitudinal myocardial global biventricular function by pulsed TDI of 6 LV walls and right ventricle (RV) free wall. Results: All diabetics and controls had normal LV dimensions, LV mass index and systolic functions except for higher left ventricular posterior wall (LVPW) in diabetics (P < 0.05). LV and RV diastolic dysfunction diagnosed in 25% of diabetics by conventional Doppler with higher peak A (P < 0.05, P < 0.05) and lower E/A (P < 0.05, P < 0.05) compared to controls. Diabetics had larger Tie index (P < 0.05). TDI showed delayed myocardial relaxation in 52.5% of diabetics with lower LV and RV peak Em (P < 0.05, P < 0.01) and Em/Am (P < 0.01, P < 0.001) compared to controls. NT‐pro‐BNP was elevated in diabetics (P < 0.01) with best cut‐off value = 62.5 Fmol/mL, sensitivity (82%), and specificity (95%) for detection of isolated diastolic dysfunction in diabetics. It was correlated negatively with LV Em (P < 0.05), Em/Am (P < 0.01) and positively with Am (P < 0.01), impaired diastolic velocities were associated with higher HbA1c. Conclusion: Asymptomatic diabetics had evidence of subtle right and LV dysfunction with delayed myocardial relaxation which was related to metabolic control. Tissue Doppler (TD) has an additional value in evaluating ventricular filling. NT‐pro‐BNP is considered a sensitive, specific, and predictive marker for diastolic dysfunction.     

Magnetic resonance imaging of lesions of the posterior fossa and upper cervical cord in childhood

Magnetic resonance imaging (MRI) promises to be an effective, noninvasive means of visualizing intracranial pathology. It should be especially useful in the evaluation of posterior fossa and cervical spinal cord disease of childhood; computed tomographic (CT) evaluation is frequently suboptimal in this region. MRI results are reported for 46 consecutively seen children with posterior fossa and/or cervical spinal cord disease (28 had brain malignancies; seven had congenital anomalies; three had cerebrovascular accidents). MRI was performed primarily by the partial saturation on a .12 Tesla resistive proton unit. All patients underwent concurrent CT evaluation. MRI demonstrated abnormalities in 96% of scans in patients with structural CNS disease (48 of 50). CNS malignancies were visualized in 100% (28 of 28) of children studied. MRI was especially useful in demonstrating the full extent of infiltrating gliomas and the anatomic location of other mass lesions. MRI frequently demonstrated disease to be more extensive than seen on CT. MRI was more sensitive than CT in documenting response to treatment and disease relapse in patients with infiltrating tumors. Cystic regions within tumors were poorly seen on MRI. Congenital anomalies were demonstrated in all patients evaluated and were better delineated using MRI than CT. MRI is sensitive in the evaluation of posterior fossa and cervical spinal cord disease of childhood and it has obvious advantages over CT; however, its specificity in such evaluations has yet to be proven.     

Echocardiographic Evaluation of Systolic and Diastolic Function in Postoperative Coarctation Patients

Postoperative coarctation (CoA) patients are often found to have signs of persistent myocardial dysfunction. M-mode echocardiography was performed to study left ventricular (LV) size, mass, and systolic function and Doppler ultrasonography to study LV filling and flow velocity in the LV outflow tract and aorta in 28 ``healthy' postoperative CoA patients (5–21 years) and 28 age- and sex-matched controls. The early (E) and late (A) diastolic transmitral velocities were significantly higher in the patient group than in the controls (p < 0.05). Other diastolic parameters (isovolumic relaxation time, E/A ratio, and deceleration time of the early diastolic velocity) were similar in the two groups (p= NS). The left atrial diameter, LV wall average, end-diastolic diameter of the LV and LV mass were higher in the patients than controls (p < 0.05). The patients also had a higher stroke volume, cardiac output, and cardiac index than the controls (p < 0.01). The fractional shortening was similar in the two groups (p= NS). Blood flow velocities in both the LV outflow tract and aorta were higher in the patients than the controls (p < 0.0002). We found LV hypertrophy with signs of a hyperdynamic circulation (increased cardiac index and stroke volume) in our patients. An increase in A is associated with LV hypertrophy and seems to be a sensitive marker of diastolic abnormality. The rise in E is paradoxical but has been seen in other studies. The increased blood flow velocity in the LV outflow tract suggests obstruction to LV outflow and increased flow in the descending aorta is due to residual coarctation.     

Left ventricular diastolic function during the first month of life

In order to assess possible changes in myocardial relaxation occurring during the neonatal period, M-mode echocardiograms were recorded serially in 9 normal term infants and in another group of 10 one-month-old infants. The tracings were studied with an M-mode calculator. Although individual variations were greater in the data collected during the first 24 h, no significant difference was found in the indices of diastolic function of the left ventricle during the first 4 days of age. The following changes were observed between data recorded at 4 days and 1 month, respectively: normalized peak rate of left ventricle filling, 4.03 vs. 4.71 cm/s; diastolic peak velocity of early posterior motion of aortic root, 1.89 vs. 5.15 cm/s; peak velocity of left ventricle posterior wall motion in diastole, 3.31 vs. 3.50 cm/s; mitral valve EF slope, 59.05 vs. 84.92 mm/s; left ventricle isometric relaxation time, 43.88 vs. 28.50 ms. In conclusion: (1) greater individual variations are observed in indices of left ventricle diastolic function during the first day of life, and (2) significant increase in left ventricle compliance occurs during the first month of life. These changes should play a critical role in the clinical course of newborn with cardiopulmonary disease.     

Normal serial variability for M-mode and digitized echocardiography in a pediatric population

Summary The normal serial variability of M-mode and digitized echocardiography was assessed in 18 healthy children aged 3–16 years. M-mode echocardiograms were done one month apart in each child and two sets of three beats were analyzed twice for each month's tracing. Ejection fraction and shortening fraction were obtained in the standard manner and the tracing digitized and analyzed for peak and normalized left ventricular velocities, and for posterior wall and septal wall velocities in systole and diastole. The component variabilities were determined by a univariate four-factor hierarchical variance components analysis and the sum of these variability limits established. Standard M-mode echocardiographic measurements of shortening fraction and ejection fraction (15% and 10% of mean) were the least variable. Peak and normalized velocities were more variable with left ventricular velocities (18%–29% of mean), posterior wall velocities (26%–45% of mean) and septal velocities (38%–68% of mean) showing increasing variabilities. Thus there is less variability in M-mode echocardiography than digitized echocardiography. The normal limits of variability are established and should be used for follow-up studies.     

Hypothalamic-pituitary vascularization in pituitary stalk transection syndrome: is the pituitary stalk really transected?

We examined 14 patients, aged 10–25 years, with idiopathic hypopituitarism. All presented an ectopic posterior pituitary at the median eminence with a hypoplastic anterior pituitary on magnetic resonance imaging (MRI). Eight patients had isolated growth hormone deficit (IGHD) and six had multiple hormone deficits (MPHD). Unenhanced MRI showed the pituitary stalk, which was extremely thin, in only three patients, while T1-weighted images obtained after intravenous injection of gadopentetate dimeglumine (Gd-DTPA) showed a thin pituitary stalk in seven patients (six with IGHD and one with MPHD), demonstrating a preserved vascular component of the stalk. MRI with Gd-DTPA was more sensitive than unenhanced MRI in detecting the pituitary stalk in patients with hypopituitarism with an ectopic posterior pituitary: the stalk was demonstrated in 50 % of the cases (seven patients), versus 21.4 % (three patients) by unenhanced MRI. The dynamic study of the hypothalamo-hypophyseal axis performed with turbo-FLASH sequences after bolus injection of Gd-DTPA showed the residual anterior pituitary to have arterial enhancement times, which suggests that an arterial system compensates for the absent or diminished blood supply from the portal system, independent of stalk detection. Received: 23 January 1996 Accepted: 31 January 1996     

Evaluation of left ventricular function in long-term survivors of childhood Hodgkin disease

BACKGROUND: Data on the presence of myocardial abnormalities in long-term Hodgkin disease survivors are contradictory. The purpose of this study was to determine if myocardial performance index (MPI) was capable of discovering cardiac abnormalities. PROCEDURE: Echocardiographic evaluation was performed in 31 survivors of Hodgkin disease (mean age 17.0 years), who received doxorubicin as part of chemotherapeutic treatment (median dose 164.8 +/- 42.5 mg/m(2)). Control group comprised 22 healthy subjects (mean age 16.7 years). RESULTS: Peak A velocity was increased (P = 0.004) and peak E/A velocity ratio was lower (P = 0.002) in patients compared to controls. Mean isovolumetric contraction time was longer in patients than in controls (P = 0.0001). Ejection time was significantly shorter in patients than in the controls (P = 0.001). Consequently, the MPI was significantly greater in the patients than in the controls (P = 0.0001). Abnormal MPI was found in 25/31 patients (83%). CONCLUSIONS: The Doppler-derived index of combined systolic and diastolic myocardial performance demonstrates the presence of subtle cardiac abnormalities in the majority of Hodgkin disease survivors.     

Cardiovascular Manifestations in Fabry's Disease — Age-Related Changes in Hemizygotes and Heterozygotes —

Fabry's disease is an X-linked recessive disorder of glycosphingolipid metabolism. Accumulation of neutral sphingolipid in vascular endothelium is known to cause various clinical manifestations, including cardiac problems. Female heterozygotes, however, may be asymptomatic or mildly affected. Cardiovascular manifestations in Fabry's disease were studied noninvasively in 10 male hemizygotes and 10 female heterozygotes from seven families. Mitral valve prolapse was found in both hemizygotes and heterozygotes. Measured echocardiographic variables were normalized by dividing them by normal values calculated from basal surface area (BSA) and age. Normalized thickness of the interventricular septum [IVS(n)] and left ventricular posterior wall thickness [LVPW(n)] increased significantly with age both in hemi- and heterozygotes. [IVS(n): Y=0.94+0.014X (age in years), r=0.68, LVPW(n): Y= 1.03+0.015X (age in years), r=0.64]. The incidence of ST-T changes in the ECG was also observed in hemi- and heterozygotes. A short PR interval was recorded in one hemizygote and one heterozygote from the same family. Thus, heterozygotes appear not to be free from cardiac manifestations, as previously believed. It is possible that the cardiac changes in heterozygotes occur regardless of the amount of sphingolipid accumulation.     

Long-term study of anthracycline-induced cardiomyopathy in children and adolescents

In order to detect changes in cardiac function, electrocardiographic, echocardiographic and radiologic studies were performed in 71 patients (age 1-26 yrs) who had been treated at the University Children's Hospital Berlin because of malignant diseases according to protocols of the DAL/GPO and had received anthracyclines (doxorubicin or daunorubicin) at cumulative doses up to 480 mg/m2 (median 280 mg/m2). Clinically overt cardiomyopathy was observed in one girl and required transient administration of digoxin. In 2 patients echocardiographic changes led to a reduction of the scheduled anthracycline doses. No patient died from congestive heart failure. During treatment, an increase in the cardiothoracic index was observed in 14 children, and in 16 the ECG showed pathological findings. A decrease in contractility to 25% or less as detected by ultrasound was seen in 15 patients. More than 50% of patients developed echocardiographical signs indicating an impairment of myocardial function. However, persistent changes were only found in 1 patient after discontinuation of treatment. Long-term echocardiographic follow-up studies for 4-48 months (median 14 months) after completion of anthracycline therapy were performed in 18 patients and the findings compared to matched-pair controls. The observed M-mode parameters were completely normal, and the ejection fractions calculated from the two-dimensional ultrasound investigations were in the normal range but slightly diminished compared to controls. Cardiac ultrasound is a non-invasive, reproducible and tolerable procedure for early detection of an anthracycline cardiomyopathy. In our patients, time of occurrence and the extent of pathological changes were not associated with the cumulative applied anthracycline doses.(ABSTRACT TRUNCATED AT 250 WORDS)     

The late consequences of anthracycline treatment on left ventricular function after treatment for childhood cancer

The purpose of this study was to determine the incidence of changes in left ventricular function in patients in long-term remission after treatment with anthracyclines for a childhood malignancy. The authors examined 155 patients in disease remission who underwent treatment protocols utilising anthracyclines in childhood. The group comprised 90 males and 65 females aged 15±4.9 years (range 5–29 years, median 15 years). The age at the time of diagnosis and start of treatment was 8.6±4.9 years (range 1–18 years, median 8 years). The time of follow-up was 7.3±4 years (range 1–21 years, median 6.3 years). The patients were given a cumulative dose of doxorubicin or daunorubicin of 250±131 mg/m² (range 50–1200 mg/m², median 240 mg/m²). The values of ejection fraction below 55% and fractional shortening below 30% assessed by means of echocardiography were considered as pathological. The control group consisted of 41 volunteers. Pathological values of fractional shortening were found in 12 patients (8%). Only one patient (0.64%) showed the development of heart failure due to cardiomyopathy. The group of the patients after chemotherapy revealed significantly worse values of left ventricular endsystolic wall stress, mean velocity of circumferential fibre shortening, Tei index, and isovolumic relaxation period in comparison with the control group. We found a correlation between the given cumulative dose of anthracyclines and indicators of systolic function of the left ventricle, but not a relation to the time indicators (age at diagnosis, time of follow-up). Conclusion:in the mean period of 6 years after chemotherapy, subclinical cardiotoxicity was found in 11 patients (7%) and cardiomyopathy with heart failure in one patient. Further indicators of subclinical damage are elevation of afterload (end-systolic stress), impaired relaxation and increased value of the Doppler index of global left ventricular function. Further monitoring and evaluation of the relevant subclinical abnormalities over a longer period of time are needed.Abbreviations CD cumulative dose - DT deceleration time - E/A index of the diastolic filling of the left ventricle - EF ejection fraction - ESS end-systolic stress - FS fractional shortening - HR heart rate - IRT isovolumic relaxation time - LV left ventricle - LVPWDd end-diastolic diameter of the left ventricular posterior wall - LVPWex excursion of the left ventricular posterior wall - LVPWP percentage of the systolic thickening of the left ventricular posterior wall - MPI myocardial performance index - mVcf _c mean velocity of circumferential fibre shortening     

Noninvasive cardiac evaluation of young patient with cystic fibrosis

Cardiac involvement was evaluated by echocardiography in 26 young cystic fibrosis patients. The mean age was 48.4 months (range 3 months to 15 years). The findings were compared with 26 age- and sex-matched children without a history of cardiopulmonary complaints. All patients had normal values of left ventricular ejection fraction and fractional shortening. Interventricular septal and posterior left ventricular wall thicknesses were similar to control group but right ventricular free wall thickness was found greater than in the control group. Abnormal septal motion was documented in six patients. Right ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in two patients and there was a negative correlation with clinical Shwachman scores (r: -0.55). Left ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in five patients. For both mitral and tricuspid valves, the mean ratios of peak velocity during passive filling (E) phase of diastole to peak velocity during atrial contraction (A) phase were found significantly lower than in the control group (p < 0.05). Early diastolic peak velocity was similar to that in the control group but late atrial peak velocity was higher in the patient group (p < 0.05). Isovolumic relaxation time was found the same as in the control group. We conclude that cardiac changes in diastolic and systolic functions begin at very young ages in cystic fibrosis patients.     

The Marfan syndrome —analysis of growth and cardiovascular manifestation

Forty-eight children and adolescents (mean age 10.5 years, range 1.25-18 years) with clinical evidence of Marfan syndrome were studied. Height and weight percentiles were established. Cardiac dimensions and morphology were studied by M-mode and 2D-echocardiography. At diagnosis left atrial and left ventricular end-diastolic diameter and left ventricular posterior wall thickness were within normal limits except in a few adolescent patients. Interventricular septum was thickened in about 20% and aortic diameter increased in 56% of the patients. An additional 13% of patients developed aortic dilation during the study period. At diagnosis regression analysis revealed a significant (P less than 0.05) correlation of the aortic diameter, septal thickness and the posterior left ventricular wall thickness and body surface area. Follow up studies of 19 patients allowed documentation of the development of aortic root dilation.