Cardiac status in bone tumor survivors up to nearly 19 years after treatment with doxorubicin: a longitudinal study.

BACKGROUND: Longitudinal assessment of cardiac toxicity in anthracycline-treated long-term bone tumor survivors. PROCEDURES: Cardiac status was assessed in 29 patients 14.1 (range 7-18.7) years after treatment with doxorubicin (DOXO) 360 mg/m(2) (median 225-550). The median age of the patients at the time of the study was 32.5 years (range 19.7-52). The evaluation consisted of an electrocardiogram (ECG), 24-hr ambulatory ECG with analysis of heart rate variability (HRV) and echocardiography. The results were compared to those of a study of the same patients that was performed 5 years earlier 8.9 years (range 2.3-14.1) after treatment. [Postma et al.: Med Pediatr Oncol 26:230-237, 1996] RESULTS: We found no progression of ECG abnormalities, arrhythmias, or echocardiographic abnormalities. Females were at risk for reduced contractility (P = 0.006). HRV was significantly reduced compared to age- and sex-matched controls and compared to the previous results. CONCLUSIONS: Anthracycline-related late echocardiographic abnormalities and arrhythmias detected 8.9 years after treatment, showed no further deterioration with ongoing follow-up. However, there was a significant reduction of HRV. This suggests that HRV might be a sensitive test for detection of anthracycline-induced cardiac toxicity.

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, age 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 dilation, decreased shortening fraction, or decreased ejection fraction. The incidence of cardiac abnormalities increased with length of follow-up (P< or = .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.

The value of 2-D Doppler echocardiography in the evaluation of asymptomatic patients with Mustard operation for transposition of the great arteries.

2-D Doppler echocardiography was used to assess the occurrence of haemodynamic abnormalities in 45 asymptomatic patients, aged 4 to 16 years (median 7.4) after a Mustard operation for transposition of the great arteries. The findings were compared with those derived from cardiac catheterization. Thirty-five cardiac lesions were correctly diagnosed by 2-D Doppler echocardiography in 23 patients, but on six occasions, minor abnormalities were missed. 2-D Doppler echocardiography demonstrated systemic venous pathway obstruction of more than 3 mmHg at cardiac catheterization in nine patients, and in five of the six patients with pulmonary venous channel obstruction. A left ventricular outflow tract obstruction (pressure difference greater than 15 mmHg) was diagnosed correctly by Doppler echocardiography in seven patients. Baffle leakage was found in two patients with a left to right shunt of 25% or more of pulmonary blood flow, but was missed in five out of nine patients with small shunts. Tricuspid regurgitation was well defined in eight patients. The absence of symptoms and a routine examination after a Mustard operation do not rule out haemodynamic abnormalities. However, these, with the possible exception of minor baffle leakage, can be detected by 2-D Doppler echocardiography.

Left atrioventricular valve after surgical repair in atrioventricular septal defect with separate valve orifices ("ostium primum atrial septal defect"): an echo-Doppler study.

Left atrioventricular (AV) valve dysfunction is the most frequent major postoperative hemodynamic complication in patients with AV septal defect. The anatomy and function of the left AV valve were investigated in 64 patients with separate valve orifices (ostium primum atrial septal defect) who had survived corrective surgery. M-mode and cross-sectional echocardiograms of the left AV valve were obtained. Doppler flow tracings were obtained at the left AV valve orifice to determine if regurgitation was present. The findings were related to the position of the commissures between the leaflets, the size of the 3 leaflets and the position of the papillary muscles. Left AV valve regurgitation was present in 29 of 51 patients (57%). These patients had a significantly different left AV valve leaflet configuration, characterized by a large mural leaflet and a small inferior bridging leaflet. The size of the superior bridging leaflet is not a determinant factor. Thus, the configuration of the left AV valve in AV septal defect is related to the postoperative functional result. Awareness of the echocardiographic anatomy may influence the surgical approach to this defect.

Anatomic and functional "obstruction" of the outflow tract in atrioventricular septal defects with separate valve orifices ("ostium primum atrial septal defect"): an echocardiographic study.

Left ventricular (LV) outflow tract (OT) obstruction can be treacherous in any form of atrioventricular (AV) septal defect. The properties of the LVOT were investigated echocardiographically in 64 patients with separate valve orifices ("ostium primum atrial septal defect") who had survived corrective surgery. M-mode and cross-sectional echocardiographic (echo) images were made of the LVOT. The degree of malalignment of the aorta with the ventricular septum, the left atrium-aortic ratio, the fractional LV shortening and the diameter of the LVOT were recorded. Fixed anatomical obstruction was found in 3 patients, consisting of muscular bands or abnormal attachment of tension apparatus. Malalignment of the aorta with the ventricular septum was found in 62% of the patients. The diameter of the LVOT was smaller than that of the aortic root in 71% of the cases. The mean diameter of the LVOT was 92 +/- 27% (range 35 to 143%) of the aortic root diameter. Because its walls are mainly muscular, the LVOT constricts during systole. The mean end-systolic diameter of the LVOT was 77 +/- 22% (range 23 to 129%) of the aortic root diameter. Sequential measurements showed that the LVOT constricted gradually, but the velocity of constriction in patients with the most severe narrowing showed a distinct maximum in the first fifth of systole. In conclusion, a series of elements contribute to a potentially perilous arrangement of the LVOT in patients with AV septal defect. This intrinsically narrow tunnel was constricted during systole by its muscular walls.(ABSTRACT TRUNCATED AT 250 WORDS)