Signal-Averaged Electrocardiography in Children with Anthracycline-Induced Cardiomyopathy

The aim of the present study was to determine if signal-averaged ECG of patients with anthracycline-induced left ventricular dysfunction could differentiate between patients with anthracycline-induced cardiotoxicity and those without. Sixteen children with anthracycline-induced cardiomyopathy, aged 6.5 to 15.5 years (anthracycline dose = 198-737 mg/m2), and 31 patients aged 5.0 to 16.7 years, who received anthracyclines without evidence of left ventricular dysfunction (anthracycline dose = 120-517 mg/m2), were studied with signal averaged ECG. The two groups were comparable in age, body surface area, and time since completion of chemotherapy. Signal averaged ECG parameters of the patients were compared with data obtained from 530 healthy children. These parameters were converted to z-scores to account for growth-related changes in signal averaged ECG recordings. Z-scores for filtered QRS duration and low amplitude terminal signal < 40 microV were significantly lower (p = 0.002 and p = 0.015, respectively), and Z-score for root mean square voltage of the last -30 ms of filtered QRS tended to be higher (p = 0.06) in patients with left ventricular dysfunction. Filtered QRS duration lower than -1.5 SD was found in 4 of 16 patients with left ventricular dysfunction and in only 1 of 31 patients without (p < 0.05) yielding a sensitivity of 25% and a specificity of 97% to detect left ventricular dysfunction. Only 1 patient had late potentials; his left ventricular function was normal. Left ventricular mass index tended to be lower in patients with left ventricular dysfunction (p = 0.07), whereas left ventricular diastolic diameter was similar in the two groups. The mechanism that accounted for the difference in signal averaged-ECG between the two groups of patients could be linked with the decrease in left ventricular mass in patients with left ventricular dysfunction. In conclusion, children with left ventricular dysfunction following anthracycline therapy have a SA ECG different from those without left ventricular dysfunction, which is mainly characterized by a lower filtered QRS duration. A prospective study is needed in order to determine if this modification of SA ECG recordings precedes alteration of left ventricular function, and, therefore, if it could help in early detection of cardiac toxicity of anthracyclines.     

Echocardiography studies for detection of cardiotoxic side effects of anthracycline therapy in childhood

An anthracycline-induced cardiomyopathy can already appear at a total cumulative dose of less then 550 mg/m2. Noninvasive cardiological methods have been used in order to detect these fatal side effects early. For cardiological control we applied echo-cardiography with measurements of ventricular size and left ventricular function. 5 out of 39 children who were off anthracycline therapy for 3 months to 8 years showed a decline in left ventricular function, but no clinical symptoms. At present 16 children with anthracycline therapy are controlled by echocardiography. In 2 patients a transient myocardial dysfunction was diagnosed. After stopping anthracycline therapy the left ventricular function improved within 2 to 3 months without specific cardiac treatment.     

Reversible cardiomyopathy due to carnitine deficiency from renal tubular wasting

The clinical course of a 4-month-old male infant with a dilated cardiomyopathy secondary to renal tubular losses of carnitine is outlined. He was admitted to the hospital with severe congestive heart failure. An echocardiogram demonstrated normal anatomy. The left ventricular shortening fraction measured 10%. A comprehensive cardiomyopathy evaluation was initiated.The total plasma carnitine level was only 25 mol/ml, but the urine carnitine measured 434 nm/mg of creatinine. He was begun on orall-carnitine and weaned from mechanical ventilation and inotropic support 10 days later. Two years later he remains asymptomatic with normal left ventricular function.     

Evaluation of left ventricular function in asymptomatic children about to undergo anthracycline-based chemotherapy for acute leukemia: an outcome study

BACKGROUND: Cardiac toxicity is a well-recognized potential complication of anthracycline use. Children treated with anthracyclines undergo several cardiac screening procedures before therapy, but the usefulness of these pretherapy cardiac studies has never been evaluated. The authors examined whether induction chemotherapy in patients with high-risk acute lymphoblastic leukemia (ALL) was altered based on a pretherapy left ventricular shortening fraction (SF). PATIENTS AND METHODS: Medical records of 134 children registered on treatment protocols of the Pediatric Oncology Group for high-risk B-precursor and T-cell ALL between 1987 and 1998 were reviewed. Demographic information consisting of age at diagnosis, sex, and past cardiac history was collected, as were the results of all echocardiographic evaluations for SF and actions taken based on these evaluations. The outcome measured was whether any changes were made in induction therapy based on initial SF. In addition, secondary SF results obtained at the cumulative anthracycline dose range of 90 to 150 mg/m2 were studied to determine whether modifications of future chemotherapy were made after this limited exposure. RESULTS: Three of 128 children (2.3%) without a previous cardiac history had an initial SF on their pretherapy echocardiogram that prompted additional evaluation but no change in therapy. A secondary analysis of SF in 85 children who completed anthracycline doses of 90 to 150 mg/m2 was performed. There were three (3.5%) with abnormal study results who were evaluated further. Again, no changes were made in the anthracycline doses based on these findings. No cardiac dysfunction occurred among these six patients during later follow-up. CONCLUSIONS: In the absence of a previous cardiac history or signs and symptoms or cardiac disease, pretherapy evaluation of left ventricular function may not be indicated in children about to undergo anthracycline-based treatment of acute leukemia. The timing of initiation of cardiac evaluation remains unclear, but these results suggest that even at a cumulative dose of 90 to 150 mg/m2, studies to determine left ventricular function do not yield data sufficient to warrant a change in the clinical management of these patients.     

A longitudinal study of cardiac function in children with cancer over 40 months

A previous study demonstrated impaired systolic function in 29% of patients treated with an thracycline as part of their therapy for malignant disease. A follow-up echocardiographic study was performed to determine whether there had been further deterioration of cardiac function. At least 40 months after the first study, those patients in whom abnormal systolic function had been detected and who had not received further anthracycline were studied by echocardiography using the same protocol as the initial study (group A). A second group of pediatric oncology patients who had not been given anthracycline but who had previously had cardiac assessment was selected as a control group (group N). The age and sex distributions of the two groups were comparable. Group A comprised 29 patients assessed on 2 occasions at mean times of 46 months and 89 months from the last dose of anthracycline. The mean dose of anthracycline received was 233 mg/m² (range 20-400). Nine of 16 patients and 4 of 5 patients who had abnormal ejection fraction (EF) and fractional shortening (FS) at first assessment had normal EF and FS at the second assessment. There were no significant changes in EF, FS, and left ventricular wall stress (LVWS) between the two examinations. In group N, 20 patients were assessed after a mean interval of 43 months. There were no significant changes in EF, FS, or LVWS between the two examinations. At the first but not the second examination there were significant differences in the left ventricular internal diameters, EF, FS, and LVWS between group A and group N. Mildly abnormal cardiac indices detected in children after cessation of treatment with anthracycline did not deteriorate in 3 to 4 years follow-up. A longer cardiac follow-up study is indicated to assess the late outcome.     

Hemodynamic consequences of inotropic support with digoxin or amrinone in lambs with ventricular septal defect

Inotropic support with digoxin is commonly used in patients with left ventricular volume overload due to ventricular septal defect (VSD). However, the hemodynamic consequences of inotropic agents with VSD have not been experimentally explored. We studied two inotropic agents, digoxin and amrinone, in chronically instrumented lambs with left ventricular volume overload due to a surgically created VSD. Intravenous digoxin (40 micrograms/kg) produced serum levels of 3.5 +/- 0.9 ng/ml (mean +/- SD) in seven lambs 60 min after administration, reduced the heart rate by 16% (172 to 149 beats/min, p less than 0.05), increased the stroke volume 16% (29.8 to 34.5 ml/beat, p less than 0.05) but did not significantly alter the systemic flow index (Qs), the pulmonary flow index (Qp), or the volume of left to right shunt (QL-R, 6.74 to 6.77 liter/min/m2). The mean left atrial pressure (LA) was unchanged (17.6 versus 17.1 mm Hg) following digoxin. Chronic digoxin use in four lambs for 4 days (25 +/- 8 micrograms/kg/8 h) produced trough serum levels of 1.2 +/- 0.2 ng/ml. There was no additional hemodynamic effect compared to acute digoxin, the Qp/Qs ratio was unchanged (3.10 versus 3.08) and evidence of left ventricular volume overload (LA - 14.0 versus 13.4) was unchanged. Amrinone lowered the systemic resistance index in a dose dependent fashion. The peak reduction of 20% (25.3 to 20.3 U/m2, p less than 0.01) occurred at 20 min after an intravenous (3 mg/kg) bolus in seven lambs. The Qs increased from 2.58 to 3.10 liter/min/m2 (p less than 0.01). The Qp was unchanged, thus the Qp/Qs ratio was lowered by 16% (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)     

Increases in serum levels of troponin I are associated with cardiac dysfunction and disease severity in pediatric patients with septic shock.

OBJECTIVES: Myocardial cell injury may contribute to cardiac dysfunction in septic shock. Troponin I is a biochemical marker of myocardial cell injury and death. We hypothesized that troponin I is increased in pediatric patients with septic shock and correlates with cardiac dysfunction and disease severity. DESIGN: Prospective, observational study. SETTING: Children's medical center. PATIENTS: Twenty-three patients with septic shock and cardiovascular failure were enrolled. MEASUREMENTS AND MAIN RESULTS: Serum troponin I was measured at admission and serially over 72 hrs. Within 24 hrs of study enrollment, echocardiograms were performed to determine left ventricular ejection fraction, systolic fractional shortening, heart rate corrected mean velocity of circumferential fiber shortening, and end-systolic wall stress. Requirement for inotropic support (stratified as low, moderate, or high), number of organ system failures, and other demographic data (including Pediatric Risk of Mortality III) were collected. Troponin I was increased on admission in 13 of 23 patients (57%) and at 12 hrs in ten of 22 patients (46%). In all cases, troponin I was maximal within 12 hrs of admission. Admission troponin I was inversely correlated to ejection fraction and fractional shortening and directly correlated to wall stress. Patients who had increased admission troponin I had lower heart rate corrected mean velocity of circumferential fiber shortening (preload and heart rate independent measure of left ventricular systolic function) and higher wall stress (measure of afterload) compared with patients with normal troponin I. Admission troponin I correlated with Pediatric Risk of Mortality III and organ system failure but did not correlate with requirement for inotropic support. CONCLUSIONS: Troponin I was increased in >50% of septic children early in their illness. Increased admission troponin I was associated with decreased measures of systolic cardiac function, as measured by echocardiography, and correlated with severity of illness. Early myocardial cell injury may contribute to the development of subsequent organ failure in septic shock, and measuring troponin I on admission may be helpful in assessing severity of sepsis.     

Ultrasound Myocardial Tissue Characterization by Integrated Backscatter in Children Treated with Anthracyclines

The objective of our study was to evaluate integrated backscatter (IBS) measurement, an ultrasound method of myocardial tissue characterization, in children receiving cardiotoxic anthracyclines for malignancy. Myocardial injury is known to diminish the normal cyclic variation of IBS (CVIBS) during the cardiac cycle. We used a cross-sectional, case-controlled study of children receiving anthracyclines and serial, prospective observation in a subgroup of children. The study took place in a university-affiliated, tertiary referral center for pediatric cardiology and oncology. Children undergoing routine echocardiograms before, during, and after anthracycline treatment participated in this study. Children evaluated in the cardiology clinic for innocent murmurs participated as controls. There was no intervention. CVIBS was measured using specialized echocardiographic software which quantitates the intensity of backscattered echoes returning from myocardial cells within a user-defined region of interest. Standard echocardiographic measures of left ventricular function were also made. The results indicated that abnormal CVIBS was prevalent during anthracycline treatment (17%) and at late follow-up (20%). In serial studies, CVIBS decreased in all children after anthracycline treatment. Anthracycline dose and time since last dose did not predict which children would have abnormalities of left ventricular function or of CVIBS. This report provides preliminary evidence that CVIBS may be a useful supplement to the noninvasive, echocardiographic assessment of the heart during anthracycline treatment in children.     

A LONGITUDINAL STUDY OF CARDIAC FUNCTION IN CHILDREN WITH CANCER OVER 40 MONTHS

A previous study demonstrated impaired systolic function in 29% of patients treated with an thracycline as part of their therapy for malignant disease. A follow-up echocardiographic study was performed to determine whether there had been further deterioration of cardiac function. At least 40 months after the first study, those patients in whom abnormal systolic function had been detected and who had not received further anthracycline were studied by echocardiography using the same protocol as the initial study (group A). A second group of pediatric oncology patients who had not been given anthracycline but who had previously had cardiac assessment was selected as a control group (group N). The age and sex distributions of the two groups were comparable. Group A comprised 29 patients assessed on 2 occasions at mean times of 46 months and 89 months from the last dose of anthracycline. The mean dose of anthracycline received was 233 mg/m² (range 20-400). Nine of 16 patients and 4 of 5 patients who had abnormal ejection fraction (EF) and fractional shortening (FS) at first assessment had normal EF and FS at the second assessment. There were no significant changes in EF, FS, and left ventricular wall stress (LVWS) between the two examinations. In group N, 20 patients were assessed after a mean interval of 43 months. There were no significant changes in EF, FS, or LVWS between the two examinations. At the first but not the second examination there were significant differences in the left ventricular internal diameters, EF, FS, and LVWS between group A and group N. Mildly abnormal cardiac indices detected in children after cessation of treatment with anthracycline did not deteriorate in 3 to 4 years follow-up. A longer cardiac follow-up study is indicated to assess the late outcome.     

Outcomes in Patients with Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart Syndrome

We sought to describe the clinical course for patients with hypoplastic left heart syndrome and persistent ventricular dysfunction and identify risk factors for death or transplantation before stage II palliation. 138 children undergoing stage I palliation from 2004 to 2011 were reviewed. Twenty-two (16 %) patients (seven Hybrid, 15 Norwood) with two consecutive echocardiograms reporting at least moderate dysfunction were included and compared to case-matched controls. Eleven of the 22 patients with dysfunction (50 %) underwent stage II, seven (32 %) were transplanted, and four (18 %) died prior to stage II. Of the patients who survived to hospital discharge (n = 17) following stage 1, 14 (82 %) required readmission for heart failure (HF) compared to only two (10 %) for controls (p < 0.001). Among patients with ventricular dysfunction, there was an increased use of ACE inhibitors or beta-blockers (82 vs. 25 %; p = 0.001), inotropes (71 vs. 15 %; p = 0.001), ventilation (58 vs. 10 %; p = 0.001), and ECMO (29 vs. 0 %; p = 0.014) for HF management post-discharge when compared to controls. There was a lower heart transplant-free survival at 7 months in patients with dysfunction compared to controls (50.6 vs. 90.9 %; p = 0.040). ECMO support (p = 0.001) and duration of inotropic support (p = 0.04) were significantly associated with death or transplantation before stage II palliation. Patients with ventricular dysfunction received more HF management and related admissions. Longer inotropic support should prompt discussion regarding alternative treatment strategies given its association with death or transplant.     

A comparison of magnesium sulphate and sildenafil in the treatment of the newborns with persistent pulmonary hypertension: a randomized controlled trial

The aim of this prospective, randomized and controlled study was to compare the clinical efficacy of intravenous magnesium sulfate (MgSO?) and oral sildenafil therapies with persistent pulmonary hypertension of the newborn. A total of 34 infants in the MgSO? group and 31 infants in the sildenafil group completed the study. The time to reach the adequate clinical response [defined as oxygen index (OI) level of <15, a pulmonary artery pressure of < 20 mmHg) was significantly shorter in the sildenafil group (p = 0.002). Duration of mechanical ventilation was longer and the number of the patients requiring inotropic support was higher in the MgSO? group (p = 0.001 and p = 0.002, respectively). Although among two groups the difference in OI > 5 as speculated in our hypothesis could only be found at 36?h of the treatment, sildenafil was more effective than MgSO? in the treatment of persistent pulmonary hypertension of the newborns with regard to time to adequate clinical response, duration of mechanical ventilation and support requirement with inotropic agents.     

Echocardiographic evaluation of patients cured of childhood cancer: a single center study of 117 subjects who received anthracyclines

BACKGROUND: The risk of cardiomyopathy following exposure to anthracycline in asymptomatic long-term survivors of childhood cancer is still hard to predict and precisely quantify. To identify the impact of different cumulative doses, even within a non-high dose range, and the echocardiographic parameters suitable for evaluating cardiac function, we studied diastolic and systolic echocardiographic parameters in a cohort of patients followed in a single center. PROCEDURE: A total of 117 subjects were studied at a median time of 7 years after treatment completion. A complete M-mode, two-dimensional and Doppler echocardiographic study was obtained at rest in all patients according to the standard recommendations of the American Society of Echocardiography. RESULTS: Ninety-nine patients (85%) had completely normal cardiac function, while 18 had abnormal echocardiographic findings: 12 had one abnormal value, 5 had two, and 1 had three abnormal values. All the changes were in left ventricular dimensions, wall thickness or indices of systolic function; no alterations in left ventricular diastolic function parameters were found. None of the echocardiographic parameters correlated significantly with the cumulative dose of anthracyclines administered either at univariate analysis or after adjusting for sex, body surface area or considered risk factors. CONCLUSIONS: Subjects exposed to a median cumulative dose of 214 mg/m(2) had no echographic abnormalities a median of 7 years later. We did not find any correlation between cumulative anthracycline dose and the echocardiographic parameters tested. We now offer echocardiographic follow-up to patients with mildly reduced fractional shortening and/or ejection fraction to rule out late onset dysfunction.     

Late cardiotoxicity after bolus versus infusion anthracycline therapy for childhood cancers

OBJECTIVE: To compare the long-term myocardial function of patients who had been treated with infusion anthracycline therapy (administered continuously over >24 hr, IG) versus bolus therapy (administered over <30 min, BG). METHODS: We selected 25 patients (BG) and 19 patients (IG) who had three or more years of disease free survival. We evaluated the echocardiograms for left ventricular shortening fraction (SF) obtained at baseline, within one year after the end of therapy (early follow-up), and on long-term follow-up. RESULTS: The mean anthracycline dose in the BG was 385 mg/m(2) and in the IG was 345 mg/m(2) (P = 0.07). During therapy, one patient in BG and none in IG developed diminished SF. During early follow-up, five of the 22 patients in BG and one of the 17 patients in IG developed diminished SF (P = 0.2). Of these five patients with diminished SF, three patients in BG and none in IG continued to have abnormally low SF long-term. At mean of 7 years, five of the 25 patients in BG and two of the 19 in IG had diminished SF on (P = 0.7). Late left ventricular dilatation was seen in 8% in BG and 5% in IG (P = 1.0). CONCLUSIONS: At mean of 7 years after end of therapy, diminished cardiac function was seen in 20% of the patient who had received bolus anthracycline compared to 11% of patients who had received it via infusion. This difference did not prove to be statistically significant.     

The role of aortopulmonary collaterals after an arterial switch operation: a word of caution

This report describes the case of a neonate with dextro-transposition of the great arteries and an intact ventricular septum who required postoperative extracorporeal membrane oxygenation support for an unexplained postoperative left ventricular dysfunction after an arterial switch operation. After surgery, a large aortopulmonary collateral suspected of causing overload to the left ventricle was diagnosed. Percutaneous embolization of the aortopulmonary collateral caused prompt improvement in patient’s conditions and rapid weaning from mechanical ventilation support.     

Histiocytosis Syndromes and Related Disorders of Early Infancy: A Pathologist's Perspective

The objective of this study is to assess the efficacy of ICRF-187 as a protective agent against anthracycline cardiotoxicity. Cardiac function was evaluated by echocardiography before and after each cycle of anthracycline chemotherapy associated with ICRF-187 and compared with that of a second group receiving anthracycline chemotherapy without ICRF-187. The patients were a group of 15 consecutive children affected with various types of solid tumors who were treated with either doxorubicin-daunomycin or epirubicin (average doses 340 and 280 mg/m², respectively), and treatment was associated with ICRF-187. A second group of 15 consecutive children affected with different malignancies werre simultaneously treated with either doxorubicin-daunomycin or epirubicin (average doses 309 and 270 mg/m², respectively), but without ICRF-187 association. None of the patients treated with anthracydines and ICRF-187 association showed abnormalities on echocardiography examination. In the second group of patients treated with anthracyclines but without ICRF-187 association, we observed a decrease in the left ventricular ejection fraction to <55% and a decrease in the. left ventricular fractional shortening to <28% in two patients (13.3%). One of these (6.6%) showed a dilatative cardiomyopathy. Both groups of patients were treated with low doses of anthracyclines. Although this study was not randomized, in patients without ICRF-87 cardioprotection, there was a trend for a worse evolution with one case of clinical cardiomyopathy as well as subclinical cardiac abnormalities.     

Prognostic Value of B-Type Natriuretic Peptide in Surgical Palliation of Children with Single-Ventricle Congenital Heart Disease

The objective of this prospective study was to assess the prognostic role of perioperative B-type natriuretic peptide (BNP) levels in infants and children with single-ventricle congenital heart disease undergoing Norwood, bidirectional cavopulmonary anastomosis (BCPA), or Fontan operation. BNP levels were measured at baseline, after cardiopulmonary bypass, 6 to 12 hours after surgery, and then daily until indwelling vascular catheters were removed. Outcome measures included length of mechanical ventilation, inotropic support, and hospital stay. Twenty subjects underwent 23 surgical procedures (13 Norwood, 5 BCPA, and 5 Fontan). BNP levels were significantly higher in patients undergoing a Norwood procedure compared with a BCPA or Fontan procedure (p < 0.01). BNP levels measured 6 to 12 hours after surgery were predictive of duration of hospitalization (p = 0.005) and inotropic support (p = 0.01). An increase in BNP level within 48 hours of extubation was observed in 92% of patients undergoing a Norwood procedure. Early postoperative BNP levels correlate significantly with the ensuing duration of inotropic support and length of hospitalization. An increase in BNP after extubation may be reflective of the degree of underlying cardiopulmonary instability. Further investigation is necessary to define this important relation.     

Cardiac abnormalities in birth asphyxia

Cardiac abnormalities in birth asphyxia were first recognised in the 1970s. These include (i) transient tricuspid regurgitation which is the commonest cause of a systolic murmur in a newborn and tends to disappear without any treatment unless it is associated with transient myocardial ischemia or primary pulmonary hypertension of the newborn (ii) transient mitral regurgitation which is much less common and is often a part of transient myocardial ischemia, at times with reduced left ventricular function and, therefore, requires treatment in the form of inotropic and ventilatory support (iii) transient myocardial ischemia (TMI) of the newborn. This should be suspected in any baby with asphyxia, respiratory distress and poor pulses, especially if a murmur is audible. It is of five types (A to E) according to Rowe’s classification. Type B is the most severe with respiratory distress, congestive heart failure and shock. Echocardiography helps to rule out critical left ventricular obstructive lesions like hypoplastic left heart syndrome or critical aortic stenosis. ECG is very important for diagnosis of TMI, and may show changes ranging from T wave inversion in one lead to a classical segmental infarction pattern with abnormal q waves. CPK-MB may rise and echocardiogram shows impaired left ventricular function, mitral and/or tricuspid regurgitation, and at times, wall motion abnormalities of left ventricle. Ejection fraction is often depressed and is a useful marker of severity and prognosis. Treatment includes fluid restriction, inotropic support, diuretics and ventilatory resistance if required (v) persistent pulmonary hypertension of the newborn (PPHN). Persistent hypoxia sometimes results in persistence of constricted fetal pulmonary vascular bed causing pulmonary arterial hypertension with consequent right to left shunt across patent ductus arteriosus and foramen ovale. This causes respiratory tension and right ventricular failure with systolic murmur of tricuspid, and at times, mitral regurgitation. Treatment consists of oxygen and general care for mild cases, ventilatory support, ECMO and nitric oxide for severe cases. Cardiac abnormalities in asphyxiated neonates are often underdiagnosed and require a high index of suspicion. ECG and Echo help in early recognition and hence better management of these cases.     

肺静脉指数对肺血减少型先天性心脏病肺血管发育的评估

目的 探索能更准确反映肺血管发育及肺血流情况的指标,为外科手术方案的选择提供依据.方法 采用74例肺血减少型先天性心脏病心血管造影序列,测量左右肺动脉及4根肺静脉直径,分别计算Nakata指数,McGoon指数,肺静脉指数(PVI),分别与术后情况进行相关分析.结果 左、右侧肺静脉大小分别与左右肺动脉大小高度相关,左侧肺静脉与左肺动脉远端的相关性为0.73,左侧肺静脉与左肺动脉近端的相关性为0.72,右侧肺静脉与右肺动脉远端的相关性为0.67,右侧肺静脉与右肺动脉近端的相关性为0.71.PVI与术后监护时间,呼吸机维持时间,正性肌力药物用量的相关性分别为-0.51,-0.478和-0.693,均比Nakata指数,McGoon指数明显增高,能更准确的反映整个肺血管的发育情况.右室流出道重建术后无低心排组与低心排组的McGoon指数分别为1.97±0.58与1.36±0.51(t=2.347,P＜0.05),两组Nakata指数分别为(269±124)mm2/m2和(164±106)mm2/m2(t=2.218,P＜0.05),PVI分别为(273±125)mm2/m2和(152±77)mm2/m2(t=2.936,P＜0.01),低心排组肺血管值均明显小于无低心排组.当PVI小于180 mm2/m2时,术后易出现血流动力学不稳定,低心排,甚至死亡.结论 肺动脉、肺静脉发育彼此相关,PVI能更准确反映肺血管发育及肺血流情况的形态学指标,为外科手术方案的选择提供有效依据.     

Ventricular Tachycardia in Acute Fulminant Myocarditis: Medical Management and Follow-up

The combination of ventricular tachycardia (VT) and severe left ventricular dysfunction presents a serious challenge in management of acute fulminant myocarditis (AFM). We report a case of a 17-month-old girl with AFM, presented with hypotension and VT, successfully treated with respiratory and inotropic support, high-dose intravenous immunoglobulin, and amiodarone. The myocardial function improved significantly within 2 weeks of treatment. The clinical course was complicated by significant amiodarone-induced hepatotoxicity, disseminated intravascular coagulation, and deep-vein thrombosis. She was later diagnosed with congenital dysfibrinogenemia and treated with chronic Lovenox therapy.     

Myocardial function in children after fetal chemotherapy exposure. A tissue Doppler and myocardial deformation imaging study

Chemotherapy and particularly anthracycline exposure are associated with acute and chronic cardiotoxicity. Few data exist on the effect of cardiac function after in utero exposure to maternal chemotherapy. Our recently published multicenter prospective study showed no significant changes in systolic function using conventional echocardiographic parameters. The purpose of this study was to further investigate whether early functional changes can be detected using tissue Doppler imaging (TDI) and two-dimensional (2D) speckle tracking echocardiography (STE). Sixty-two children (median/range age 1.7 (1–9.8)?years) exposed to chemotherapy during fetal life were enrolled and compared to 62 age- and gender-matched controls. TDI velocities were measured at the basal interventricular septum (IVS) and right and left ventricular (LV) free walls. LV global longitudinal and circumferential systolic strains were derived using 2D STE. We found small but significant differences between the groups (patients versus controls) in LV fractional shortening [35 (29–46)% versus 39 (28–53)%, p?<?0.001], LV ejection fraction [66 (57–79)% versus 70 (57–83)%, p?<?0.001], LV posterior wall thickness z score [?0.15 (?2.32–1.81) versus ?0.10 (?1.9–2.0), p?<?0.001], and IVS thickness z score [?1.06 (?2.6–1.3) versus ?0.5 (?2.1–1.7), p?<?0.001]. No significant differences in TDI velocities or LV global strains were observed. Within the patient group, the cardiac functional parameters did not correlate to the number of cycles of anthracycline or the cumulative anthracycline dose. Children exposed to fetal chemotherapy have a lower normal fractional shortening and mildly lower left ventricular wall thickness. Tissue Doppler and strain measurements are within normal range and not statistically different from normal controls. The long-term implications of these findings will be further studied in this prospective cohort study.