Influence of bidirectional superior cavopulmonary anastomosis on pulmonary arterial growth.

Right-sided BSCA provides for satisfactory pulmonary arterial growth in infants and children with complex congenital heart defects, and it could enhance the growth of a small right pulmonary artery. The growth of the left pulmonary artery, particularly in younger patients, needs close attention to confirm the safe role of BSCA in long-term palliation.

Endothelial dysfunction caused by University of Wisconsin preservation solution in the rat heart. The importance of temperature.

The superiority of the University of Wisconsin solution over routinely used crystalloid cardioplegic solutions for myocardial preservation has been demonstrated in animal studies. We have investigated the effect of the University of Wisconsin solution at different temperatures on endothelial function by examining its influence on 5-hydroxytryptamine- and nitroglycerin-induced increase in coronary flow in the isolated rat heart. Thirty-eight rat hearts were perfused on a modified Langendorff preparation. In the control experiments, there was no significant difference in the percentage increase in coronary flow induced by 5-hydroxytryptamine and nitroglycerin after 30 minutes of perfusion with Krebs-Henseleit buffer (n = 6). Continuous infusion of the University of Wisconsin solution for 30 minutes at 4 degrees C or at 10 degrees C did not alter the 5-hydroxytryptamine or nitroglycerin response. However, infusion at 15 degrees C reduced the 5-hydroxytryptamine-induced vasodilation, while at 20 degrees C the 5-hydroxytryptamine response was converted to vasoconstriction without a significant change in nitroglycerin effect (15 degrees C, 5-hydroxytryptamine, before: 30.2% +/- 1.5%, after: 6.0% +/- 1.0%, nitroglycerin, before: 28.8% +/- 1.3%, after: 31.2% +/- 1.8%; 20 degrees C, 5-hydroxytryptamine, before: 32.2% +/- 2.5%, after: -23.8% +/- 3.6%, nitroglycerin, before: 30.3% +/- 1.9%, after: 33.5% +/- 1.7%). Coronary vascular resistance in the control experiments rose from 55.0 +/- 2.5 cm H2O/ml/gm/min to 58.4 +/- 2.3 cm H2O/ml/gm/min (p = not significant). The increase after University of Wisconsin solution infusion at 4 degrees C and at 10 degrees C was similarly not significant. Coronary vascular resistance increased significantly following infusion of University of Wisconsin solution at 15 degrees C (p < 0.001) or at 20 degrees C (p < 0.01). We conclude that University of Wisconsin solution produces temperature-dependent endothelial dysfunction in the isolated rat heart.

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.

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.

A rare cause of profound cyanosis after Kawashima modification of bidirectional cavopulmonary anastomosis.

The expected level of systemic arterial saturation may not be present after bidirectional superior cavopulmonary anastomosis in children with complex congenital cardiac anomalies. We present a case of persistent severe cyanosis in a patient with azygos continuation of the inferior vena cava after bidirectional superior cavopulmonary anastomosis (Kawashima procedure) due to an intrahepatic venovenous malformation. Subsequent transcatheter deployment of two Rashkind double umbrella devices into the malformation reduced the shunt and markedly improved systemic arterial saturation.

Aortic valve repair of congenital stenosis with bovine pericardium.

BACKGROUND: Conservative surgical options in the treatment of congenital aortic stenosis are limited. To relieve the obstruction necessitates full incision of the raphe of the larger valve leaflet, but this inevitably causes prolapse. METHODS: We performed aortic valve repair in 6 children, aged 14 months to 17 years, with congenital aortic stenosis, 2 having had aortic valvotomy as infants. The repair consisted of suturing the base of a triangular piece of bovine pericardium, with a simple vertical fold, to the free edges of the incised raphe. The pericardial fold was then sutured vertically to the aortic wall. RESULTS: At follow-up of 2 to 60 months, the mean peak systolic Doppler gradients had decreased from 80 +/- 15 mm Hg to 26 +/- 9 mm Hg. The effective valvular orifice area increased from 33% +/- 6% to 64% +/- 3%, allowing blood flow to increase by a factor of 3.76. Two patients have mild and 2 have mild-to-moderate aortic regurgitation. CONCLUSIONS: The described conservative repair renders the valve tricuspid and trisinusoidal, and the deficient interleaflet triangle is recreated, preventing cusp prolapse. Longer follow-up is required to assess the durability of unstented pericardium in the aortic position, but the early results are encouraging.

Bidirectional superior cavopulmonary anastomosis: how young is too young?

OBJECTIVE: To define the lowest age at which the bidirectional superior cavopulmonary anastomosis can safely be used in infants with complex congenital heart defects. DESIGN: A retrospective analysis of clinical, echocardiographic, haemodynamic, and angiographic data in four consecutive patients undergoing bidirectional superior cavopulmonary anastomosis below the age of 2 months. PATIENTS: Between November 1990 and September 1993, four infants less than 8 weeks of age (3, 4, 6, and 7 weeks) underwent bidirectional superior cavopulmonary anastomosis as a primary palliation for complex heart disease. The indication for early surgical intervention was progression of cyanosis (n = 3) and high pulmonary blood flow causing heart failure (n = 1). In two infants with tricuspid atresia, surgery was performed through a right thoracotomy without the use of cardiopulmonary bypass. In one infant with double inlet left ventricle and discordant ventriculoarterial connection, cavopulmonary anastomosis was combined with an arterial switch procedure. The final infant had double inlet left ventricle with pulmonary atresia; the central pulmonary arteries were virtually discontinuous and each branch was supplied by a separate arterial duct. The central pulmonary arteries were reconstructed using the subaortic innominate vein. Temporary prostacyclin infusion was used in three patients in the immediate postoperative period. RESULTS: Early postoperative extubation (5, 7, and 48 h) was successful in three patients. The youngest child required ligation of the ductus arteriosus four days later because of severe upper body oedema. The postoperative course was complicated by prolonged effusions in two patients. All were alive and well 14-48 months postoperatively, with satisfactory systemic saturations (80-87%) and haemodynamic indices. CONCLUSIONS: This limited experience challenges the widely held belief that the bidirectional superior cavopulmonary anastomosis cannot be used as a primary palliation for complex heart disease in early infancy. A wider experience is required to determine the safety and indications for this approach.

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.

Use of larger sized aortic homograft conduits in right ventricular outflow tract reconstruction.

Between 1973 and 1993 sixty aortic homograft valved conduits in fifty-six patients were used to establish continuity between the right ventricle and the pulmonary artery in congenital heart disease. Age range was one day to 23.5 years (median 3.6 years) which included twenty-six patients less than one-year-old. Conduit size ranged from 11 to 23 mm (median 17.6 mm). there were nine hospital deaths and eight late deaths. The 45 survivors have been followed for a median of 8.6 years (range 6 months to 20 years). All patients have had serial echocardiographic assessments and 35 have had post repair cardiac catheterization. Almost all patients had mild-to-moderate degrees of homograft regurgitation. There were eleven with severe homograft regurgitation and two are being considered for reoperation. The follow up homograft gradient ranged from 0 to 64 mmHg (mean 24 mmHg). Freedom from reoperation for conduit obstruction was 98.2% at five years falling to 91% (C.L. 82%-100%) at 10 years. Of the 23 homografts inserted more than 10 years ago, only one (4.3%) has been replaced because it was causing important obstruction. None have been replaced for regurgitation. Our results indicate that larger sized aortic homografts used in reconstruction of the right ventricular outflow tract give satisfactory results and there is a low incidence of reoperation for replacement at medium term follow up.

Unusual left atrial mass following cardiac surgery in an infant.

An unexpected left atrial mass was found during routine postoperative transthoracic echocardiogram in an infant following surgical closure of ventricular septal defect. Thrombus could not be excluded. At reoperation it was found to be an inverted left atrial appendage. This case demonstrates the echocardiographic appearance of an inverted left atrial appendage.

Gianturco self-expanding metallic stents in treatment of tracheobronchial stenosis after single lung and heart and lung transplantation.

Three patients with recurrent bronchial stenosis following single lung transplant (SLTx), and one patient with tracheal stenosis following heart-lung transplantation (HLTx), not responding to repeated dilatations (3 patients) and prolonged use of silastic stents (patient with tracheal stenosis), have been treated by the endoscopic insertion of Gianturco self-expanding metallic stents under fluoroscopic control. The stent resulted in immediate improvement in respiratory function in all four patients. One patient (SLTx) had early bronchial re-stenosis due to growth of granulation tissue within the stent which was successfully treated by cryotherapy. In one patient (HLTx), a left lower lobe bronchial stenosis developed 14 months after tracheal stenting. The metallic stent appears to be a promising device in the management of recurrent or resistant bronchial stenosis following SLTx or tracheal stenosis after HLTx.

Operative treatment of infective endocarditis in children.

A retrospective review of 11 children, aged 2 months to 15 years, demonstrates the feasibility of surgical treatment for infective endocarditis in childhood. Except for one case of perinatal infection, in all instances the infective endocarditis was a complication of a congenital heart defect. As medical treatment was not successful, surgery was indicated. Debridement of infected tissue and repair of the congenital heart defect was performed. There were no septic complications postoperatively although 8 patients were operated upon during the active stage of infection. One 2-month-old child did not survive excision of an infected tricuspid valve. The follow-up period of 8 years to 5 months (median 39 months) showed a good haemodynamic result (NYHA class I) in the remaining 10 patients. This included 4 patients with prosthetic valves.

[Seasonal differences in the occurrence of congenital heart defects]. / Sezónní rozdíly výskytu vrozených srdecních vad.

Incidence of congenital heart defects was studied prospectively in all 664,218 children born in 1977 to 1984. Those suspected of having a heart disease were examined at the center specialized in pediatric cardiology. All children who died were autopsied and those in whom a heart defect was proved were included in our series. In total, 4409 infants (6.64/1000 live births) were born with a heart defect in Bohemia. Differences from one year to another were not statistically significant. The incidence of infants with a heart defect was highest in October, lowest in December, June and July (p less than 0.05). Seasonal incidence of individual forms of heart defects differed as well. The seasonal variation was not influenced by the total number of live births. High incidence of congenital heart defects in infants born in October and November coincides with the epidemics of influenza in early pregnancy.

[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.

[The incidence of heart defects in children]. / Výskyt srdecních vad u deti.

By examining all of 91,823 children born in 1980 in Bohemia, congenital heart disease was proved in 589 (6.415/1000 liveborns) and cardiomyopathy in 24 (0.261/1000 liveborns). Further 166 (1.808/1000 liveborns) were follow-up with a diagnosis of congenital heart disease by pediatric cardiologists in the field, but at the age of 4 years we didn't prove the defect any more. Prevalence of heart defects: ventricular septal defect (31.4%), atrial septal defect (11.4%), aortic stenosis (7.6%), pulmonary stenosis (7.1%), coarctation of the aorta (5.8%), transposition of the great arteries (5.4%), patent ductus arteriosus (4.8%), atrioventricular canal and hypoplastic left heart (4.1%), tetralogy of Fallot (3.6%). Other defects occurred less frequently than in 2.5% of all congenital heart disease.

[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.

[Differences in the incidence of congenital heart defects in boys and girls]. / Rozdíl výskytu vrozených srdecních vad u chlapcu a dívek.

4,409 children born in Bohemia (1974-1983) with heart defect were distributed almost between 2,296 (52.1%) boys and 2,213 (47.9%) girls: The boy:girl ratio was 1.09:1. Boys prevailed in double outlet right ventricle (2.68:1), hypoplastic left heart (2.25:1), transposition of the great arteries (2.11:1), aortic stenosis (1.95:1), pulmonary atresia (1.55:1), tricuspid atresia (1.45:1), coarctation of the aorta (1.30:1) and the corrected transposition (1.25:1). Girls prevailed significantly in patent ductus arterious (1.66:1), Ebstein's anomaly of the tricuspid valve (1.57:1), truncus arterious (1.22:1), atrioventricular septal defect (1.17:1) and tetralogy of Fallot (1.12:1). The difference in the remaining heart defects was less than 10%.

[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.

[Heart involvement in Lyme borreliosis. Case report]. / Postizení srdce pri lymeské borelióze. Kasuistika.

Lyme disease is a systemic illness with skin, neurologic, joint, and cardiac involvement. Absence of typical skin lesion--erythema chronicum migrans--during the first period of disease may cause the diagnosis difficult. Syncope due to complete atrioventricular block was the first symptom of disease in our patient, but no tick bite anamnestic data were available. Quick changes on EKG tracing during cardiac pacing were present. Echocardiography revealed left ventricular dysfunction. Specific immunological tests confirmed the diagnostic suspicion. Cardiac pacing, digitalis and antibiotic treatment ensued in complete recovery. No other systemic lesion appeared. Lyme carditis may be the only manifestation of the disease. Temporary cardiac pacing may be necessary.

[Treatment of dilated cardiomyopathy in childhood]. / Moznosti lécby dilatacní kardiomyopatie v detském veku.

Authors reviewed available information concerning etiology and pathophysiology of dilated cardiomyopathy. Diagnostic criteria and current poor results of conservative treatment are discussed. Between August 1984 and August 1991 67 children with various types of cardiomyopathy underwent heart and heart and lung transplantation at Harefield Hospital, U.K. Dilated cardiomyopathy was the indication for transplantation in 47 patients (mean age at operation was 6.5 years, range 0.3-4.8 years). Eighty-one percent actuarial survival at 4 years after operation represents reasonable progress in the treatment of this severe acquired disease. Currently, early transplantation is recommended in children with dilated cardiomyopathy and with low shortening fraction of left ventricle (below 0.13), without any improvement after 3 months of conservative treatment, will familial trait of dilated cardiomyopathy and/or with severe myocardial fibrosis.