Comparison of Doppler flow Tei-indexes with pulmonary artery thermodilution measurement of cardiac output in an experimental porcine model.

The objective of our study was to compare Doppler echocardiography imaging with pulmonary artery thermodilution measurement during mechanical ventilation. Total 78 piglets (6 weeks old, average weight 24 kg, under general anesthesia) were divided into 4 groups under different cardiac loading conditions (at rest, with increased left ventricular afterload, with increased right ventricular preload, and with increased afterload of both heart ventricles). At 60 and 120 min the animals were examined by echocardiography and simultaneously pulmonary artery thermodilution was used to measure cardiac output. Tei-indexes data were compared with invasively monitored hemodynamic data and cardiac output values together with calculated vascular resistance indices. A total of 224 parallel measurements were obtained. Correlation was found between values of right Tei-index of myocardial performance and changes in right ventricular preload (p<0.05) and afterload (p<0.01). Significant correlation was also found between left index values and changes of left ventricular preload (p<0.001), afterload (p<0.001), stroke volume (p<0.01), and cardiac output (p<0.01). In conclusion, echocardiographic examination and determination of the global performance selectively for the right and left ventricle can be recommended as a suitable non-invasive supplement to the whole set of methods used for monitoring of circulation and cardiac performance.

Nonsurgical treatment of patent arterial duct in term neonates with congenital heart disease: the role of intravenous indomethacin.

We present two term neonates in which intravenous indomethacin usage was associated with successful closure of a persistently patent arterial duct. Both children had congenital heart disease with high pulmonary blood flow following surgical placement of contralateral systemic-pulmonary artery shunts.

Pulmonary atresia with intact ventricular septum: predictors of early and medium-term outcome in a population-based study.

OBJECTIVES: Pulmonary atresia with intact ventricular septum is a form of congenital heart disease usually associated with right-heart hypoplasia, with considerable morphologic heterogeneity and often poor outlook. Ascertainment of risk factors for poor outcome is an important step if an improvement in outcome is to be achieved. METHODS: The UK and Ireland Collaborative study of Pulmonary Atresia with Intact Ventricular Septum is an ongoing population-based study of all patients born with this disease from 1991 through 1995. All available clinical, morphologic, and investigative variables were directly reviewed, and risk factor analysis was performed for poor outcome. RESULTS: One hundred eighty-three patients presented with pulmonary atresia with intact ventricular septum. Fifteen underwent no procedure, and all died. Of the remainder, 67 underwent a right ventricular outflow tract procedure (catheter or surgical), 18 underwent an outflow tract procedure with shunt, and 81 underwent a systemic-to-pulmonary shunt alone. One- and 5-year survival was 70.8% and 63.8%, respectively. Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P < .001) were independent risk factors for death. The presence of coronary artery fistulae, right ventricular dependence, or the tricuspid valvar z score did not prove to be risk factors for death. After up to 9 years of follow-up, 29% have achieved a biventricular repair, 3% a so-called one-and-a-half ventricular repair, and 10.5% a univentricular repair, with 16.5% still having a mixed circulation (41% died). CONCLUSIONS: This population-based study has shown which features at presentation place an infant in a high-risk group. This is important information for counseling in fetal life and for surgical strategy after birth.

Milrinone and low cardiac output following cardiac surgery in infants: is there a direct myocardial effect?

We assessed the effect of milrinone on myocardial function in pediatric patients with postoperative low cardiac output syndrome by index of myocardial performance in a prospective, open-label, nonrandomized, consecutive study. Fifteen patients with low cardiac output syndrome following cardiac surgical treatment were studied in the tertiary cardiothoracic pediatric intensive care unit between April 2001 and November 2003 (age range, 0.2-16 months; median, 7; weight, 2.7-11.8 kg; median, 5). Echocardiographic, Doppler-derived, time interval-based index of myocardial performance (Tei index) was used to study cardiac function prior to and while on intravenous milrinone treatment for 18-24 hours. Treatment with milrinone led to improvement in biventricular myocardial function [mean right ventricular index from 0.521 (SD-0.213) to 0.385 (SD-0.215), p = 0.003; mean left ventricular index from 0.636 (SD-0.209) to 0.5 (SD-0.171), p = 0.012). No difference was found in the values of heart rate corrected right or left ventricular ejection time prior to and while on treatment with milrinone (right ventricle: mean, 1.23 (SD-0.42) and 1.14 (SD-0.48), p = 0.29; left ventricles: mean, 1.17 (SD-0.51) and 1.13 (SD-0.48), p = 0.66) Our data support the direct myocardial effect of milrinone as part of the mechanism behind its already proven benefit in children with low cardiac output syndrome following cardiac surgery.

Recombinant t-PA in myocardial ischemia after switch operation.

A well-recognized complication of the anatomic correction (arterial switch operation) of transposition of the great arteries is obstruction of the translocated coronary arteries. Myocardial reperfusion has previously been achieved by surgical revascularization or percutaneous balloon angioplasty. We report the case of a 3-month-old infant who suffered myocardial infarction 11 weeks after the arterial switch operation, in whom myocardial reperfusion was established following infusion of recombinant tissue-type plasminogen activator (Alteplase).

[Improved results in children with acute lymphoblastic leukemia treated with the ALL-BFM 90 protocol in the Czech Republic]. / Zlepsení výsledku lécby detí s akutní lymfoblastickou leukémií podle protokolu ALL-BFM 90 v Ceské republice.

BACKGROUND: Prognosis of children with acute lymphoblastic leukaemia (ALL)--the most common cancer in childhood, has improved remarkably over the last 40 years. The authors report the treatment outcome in children with ALL cured according to ALL-BFM 90 Study protocol in the Czech Republic during the first half of nineties. METHODS AND RESULTS: Children aged 0-18 years were included into the study in 10 centers between 1990 to 1996. Patients were classified into standard-risk (SR), medium-risk (MR) and high-risk (HR) group according to initial leukaemic burden, early treatment response, and genotype of leukaemia. Duration of the chemotherapy was two years. Treatment results were evaluated in 352 children. With a median follow-up of 7.3 years, event-free-survival (EFS) was 71.3% and overall survival 76.4%. EFS was 80.3%, 74% and 28.2% in SR, MR and HR group, respectively. Relapse was diagnosed in 17.8% of the patients. CONCLUSIONS: The treatment outcome of children with ALL improved significantly (p = 0.0045) compared to the previous study ALL-BFM 83 (EFS 62%). These results are comparable to those achieved by leading leukaemia study groups in the world.

Octreotide to treat postoperative chylothorax after cardiac operations in children.

Chylothorax after pediatric cardiac operations is associated with significant morbidity and increased hospitalization. An octreotide (a synthetic somatostatin analogue) infusion (1 to 4 microg/kg per hour) with medium-chain triglyceride diet or parenteral nutrition was used in 4 pediatric cardiac surgical patients after chylothorax was diagnosed. Resolution followed within 5 days in all without recurrence, while on a normal diet.

Are cyanosed adults with congenital cardiac malformations depressed?

OBJECTIVE: To assess the incidence of depression, and the ability to interact socially, in adult patients with chronic cyanosis and congenital cardiac malformations. DESIGN: Prospective study of consecutive patients. SETTING: Single institution, tertiary referral centre. PATIENTS: Between 1993 and 2000, we assessed 76 patients with congenital cardiac malformations and persistent cyanosis, having a median age of 36.5 years, with a range from 19 to 64 years, at the time of referral. Female patients accounted for just under half (48.6%) of the sample. Just under two-fifths of the cohort (39.5%) had functionally univentricular cardiac anatomy, while 14.8% had tetralogy of Fallot with pulmonary atresia and aorto-pulmonary collateral arteries, and 17% had the Eisenmenger syndrome. During the period of follow-up, 17 (22.4%) of the patients died. ASSESSMENT: We used clinical interviews and non-invasive assessment, employing Zung's questionnaire which provides a scale for the self-rating of depression. On this scale, a score above 50 points is indicative of depression. RESULTS: Of the survivors, 32 (54%) completed the self-rating questionnaires. Of these, 20 responders (63%) considered that they lead full lives, including sexual activities, while 26 (81%) had never harboured suicidal thoughts. Depression was diagnosed in 11 responders (34%), with a mean score of 66.9, standard deviation of 8.7, and a range from 53 to 89. The remaining 21 patients (66%) were without signs of depression, scoring a mean of 41.5, with standard deviation of 5.5, and a range from 35 to 46. Depression was associated with older age (40.5 years versus 33.5 years, p = 0.01), worse functional state in the classification of the New York Heart Association (2.95 versus 2.48, p = 0.03), and unemployment (p < 0.0001), but independent from the severity of cyanosis, the level of the haematocrit, the saturation of oxygen, or previous surgical treatment. CONCLUSIONS: To our knowledge, this is the first evidence suggesting a relatively high incidence of depression in adults with congenital cardiac malformations and persistent cyanosis. Larger, multi-centric studies will be needed to confirm or refute these findings.

Octreotide therapy: a new horizon in treatment of iatrogenic chyloperitoneum.

Chyloperitoneum is a rare and challenging complication of abdominal surgery. We report a case of iatrogenic chyloperitoneum. Infusion of octreotide, a somatostatin analogue, together with total parenteral nutrition followed by medium chain triglyceride diet resulted in rapid resolution of chyloperitoneum. We believe this to be the first report of successful use of octreotide in iatrogenic chyloperitoneum in a child.

Rapid onset of intrapulmonary arteriovenous shunting after surgical repair of tetralogy of Fallot with pulmonary atresia.

We describe a 2-year-old girl with tetralogy of Fallot and pulmonary atresia, palliated as a neonate with a right modified Blalock Taussig shunt, who developed severe cyanosis following total correction in the absence of corresponding evidence of parenchymal lung disease on the chest X-ray. Selective pulmonary angiography showed new intrapulmonary shunting involving only the right middle and lower lobes only. The cyanosis resolved rapidly subsequent to inhalation of nitric oxide. To our knowledge, this is the first documented case of rapid onset of localised intrapulmonary right-to-left shunting, involving only two lung lobes, following biventricular repair for complex congenital heart disease.

Heterotopic cardiac transplantation and Batista operation.

We report the case of an infant who was first seen with dilated cardiomyopathy at the age of 3 1/2 months and 2 months later, successfully underwent heterotopic cardiac transplantation in combination with partial left ventriculectomy. The benefits of combining both procedures in such a young infant particularly with regard to containment of early graft dysfunction, reduction of respiratory problems, and weight mismatch are discussed.

[Thrombosis in childhood--etiologic role of congenital thrombophilic conditions]. / Trombóza v detském veku--etiologická role kongenitálních trombofilních stavu.

BACKGROUND: Increasing frequency of thrombosis in podiatry brings about high morbidity and mortality. From published sets of clinical cases with thromboembolic complications can be concluded, that contrary to adults, origin of thrombosis in children is more frequently based on congenital thrombophilic states. The main of the work is: 1. To identify prevalence of the congenital thrombophilic states in the set of patients with venous and arterial thrombosis. 2. Formulate recommendations for the laboratory investigation. 3. Evaluate results of the thrombosis treatment in our set of patients. METHODS AND RESULTS: Set of 24 patients of the average age 6.7 years at the time of thrombosis (16 time venous, 8 times arterial) was retrospectively investigated for the presence of the factor V-Leiden mutation, prothrombine 20210A mutation, deficiency of C and S protein, and antithrombin III. Presence of acquired risk factors was also evaluated. Congenital thrombophilic state was identified in 5 patients (31.2%) with venous thrombosis and in 1 patient (12.5%) with arterial thrombosis. Mutation of the factor V-Leiden was found most frequently. It was identified at 3 patients (18.7%) with venous thrombosis and 1 patient (12.5%) with arterial thrombosis. The central venous catheter was the most frequent acquired risk of thrombosis (50%). In 1 patient with venous thrombosis and in 4 patients with arterial thrombosis no acquired or congenital risks of thrombosis were identified. Results of treatment confirmed beneficial effects of heparinisation and subsequent wafarinszation for the period of increased risk of thrombosis. Systemic thrombolysis was done 3 times without complications. CONCLUSION: Congenital thrombophilic states play significant role in the manifestations of thromboses in children. In majority of children with manifesting thrombosis at least one risk factor was identified. Cerebral infarcts in infants remain largely unrevealed.

Intrapulmonary arteriovenous shunting may be a universal phenomenon in patients with the superior cavopulmonary anastomosis: a radionuclide study.

OBJECTIVE: To evaluate the extent of intrapulmonary right to left shunting in children after bidirectional cavopulmonary anastomosis (BCPA). DESIGN: Prospective study of patients who underwent BCPA in a single centre. PATIENTS: 17 patients with complex cyanotic congenital cardiac malformations who underwent BCPA at 1-45 months of age (median 21 months) were evaluated 15-64 months postoperatively (median 32 months). Five children between 1 and 10 years (median 5 years) with normal or surgically corrected intracardiac anatomy and peripheral pulmonary circulation who required V/Q scanning for other reasons were used as controls. INTERVENTIONS: All patients underwent cardiac catheterisation to exclude angiographically demonstrable venovenous collaterals followed by pulmonary perfusion scanning using (99m)technetium ((99m)Tc) labelled albumen microspheres to quantify the intrapulmonary right to left shunt. MAIN OUTCOME MEASURE: Percentage of intrapulmonary right to left shunt. RESULTS: The mean (SD) level of physiological right to left shunting found in the control group was 5.4 (2.3)%. All patients with BCPA showed the presence of a significantly higher level of intrapulmonary shunting (26.8 (16.9)%, p < 0.001). The degree of shunting was significantly increased in the subgroup of 11 patients with BCPA as the only source of pulmonary blood flow (34.9 (15.8)%), when compared to the six remaining patients with an additional source of pulmonary blood supply (12.0 (2.6)%, p < 0.001). There was a negative correlation between age at BCPA and the shunt percentage found in the patients with a competitive source of pulmonary blood flow (r = -0.63, p < 0. 01). CONCLUSIONS: Intrapulmonary right to left shunting develops in all patients following BCPA. This may be caused by a sustained and inappropriate vasodilatation resulting from absence or decreased levels of a substance that inhibits pulmonary vasodilatation. Augmenting BCPA with an additional source of blood flow containing hepatic factor limits the degree of intrapulmonary arteriovenous shunting and may help provide successful longer term palliation.

Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children.

Normalization of the dimensions of cardiac structures to the size of the body, using so-called Z scores, is becoming increasingly common in the management of infants and children with congenital heart disease. Current published nomograms for the ascertainment of Z scores for cardiac structures in childhood are based largely on normal data obtained in formalin-fixed hearts. Since decisions concerning management are frequently based on the findings of cross-sectional echocardiograms, the dimensions of 15 cardiac structures were measured using cross-sectional echocardiography in 125 normal infants and children. Regression equations were derived relating cardiac dimensions to the size of the body. The expression of size with the highest correlation to cardiac dimensions was body surface area. Nomograms were then developed from which the Z score of a cardiac structure could be estimated from a knowledge of the body surface area and the echocardiographically derived measurement.

The real fate of pulmonary arteries after bidirectional superior cavopulmonary anastomosis: is there a need for concern?

Controversy remains about the growth of the pulmonary arteries following a bidirectional superior cavopulmonary anastomosis in children with complex cyanotic congenital cardiac malformations. This is partially due to the morphological heterogeneity of the patients, and partially due to methodological differences in series published so far. It is further complicated by the variable use, in different centres, of additional sources of pulmonary blood flow. We believe that the fate of these arteries preoperatively is significantly influenced by the amount of pulmonary blood flow and the initial size of the arteries. Separate assessment of the pulmonary arterial development postoperatively is recommended for those who, initially, had relatively small as opposed to larger than normal pulmonary arteries. Measurement of the diameters of both pulmonary arteries just prior to their first point of branching, together with the use of Z-score evaluation rather than the Nakata index, is discussed. It remains to be established whether, over time, the bidirectional cavopulmonary anastomosis is effective in developing adequately the pulmonary arteries in preparation for an ultimate total cavopulmonary connection, or even as isolated long-term palliation. A prospective, multi-institutional study involving sequential non-invasive assessment of pulmonary arterial development (using, for example, magnetic resonance imaging) is required to solve the outstanding problems.

Treatment of Hodgkin's disease in children with VAMP (vinblastine, adriamycin, methotrexate, prednisone) and VEPA (vinblastine, etoposide, prednisone, adriamycin).

Treatment of Hodgkin's disease in children should be directed at maximizing cures and minimizing the long-term effects of alkylating agents, anthracyclines, and bleomycin. In this study methotrexate and etoposide were used in the VAMP/VEPA regimens to treat 60 clinically staged pediatric patients with Hodgkin's disease. Twenty-nine patients with stages I-IIA received four courses of VAMP plus low-dose radiotherapy. Thirty-one IIA bulky disease and IIB-IVB patients received four or six courses of VEPA plus low-dose radiotherapy. There were 6 partial remissions after the completion of chemotherapy and all of these patients relapsed, but 4 were successfully salvaged with ABMT. Two patients have died. The 3.1-year overall survival rate is 97% (100% VAMP, 94% VEPA) and the event-free survival rate is 88% (97% VAMP, 77% VEPA). These results suggest that VAMP is a reasonable treatment for low stages of Hodgkin's disease, but more advanced disease is not adequately treated by VEPA and low-dose radiotherapy.

[Cardiology in the United Kingdom]. / Kardiologem ve Spojeném Království.

The authors describe the contemporary postgraduate system of cardiology in Great Britain with regard to the unification of criteria for specialization in the European Union and discuss opportunities of doctors from the Czech Republic to join this postgraduate system.

Absent pulmonary valve syndrome with intact ventricular septum: role of ductus arteriosus revisited.

We report two patients (four and twelve days old respectively) with heart failure due to absent pulmonary valve and patent ductus arteriosus. The ductus arteriosus in both patients had unusual course arising early from the aortic arch and maintaining an acute angle with the aortic arch. The etiology of absent pulmonary valve syndrome is still not clear. We speculate on the role of arterial duct in the development of absent pulmonary valve syndrome.