[Hypoplasia of trachea with tracheal bronchus as a complication of surgical correction of tetralogy of Fallot]. / Hypoplazie trachey s tracheálním bronchem jako prícina komplikací po korekci Fallotovy tetralogie.

A 10 months old infant underwent repair of tetralogy of Fallot with hypoplastic annulus of the pulmonary valve, diminutive pulmonary arteries, right aortic arch and left superior vena cava. The right ventricular outflow tract was reconstructed using a monocusp patch. The postoperative course was unfavourably influenced by respiratory complications due to tracheal bronchus and hypoplasia of trachea, which were not diagnosed preoperatively. Extreme emphysema of the right upper and middle lobes compromised haemodynamics. Repeated reoperations were required. The upper and the middle lobes of the right lung had to be resected, the hypoplastic trachea reconstructed with a pericardial patch and pulmonary homograft inserted. One year later, homograft had to be replaced and tricuspid annuloplasty performed for pulmonary and tricuspid regurgitation and right ventricular dilatation as a consequence of increased pulmonary artery pressure. Three years after the original surgery the patient remains in good clinical condition.

[The Budd-Chiari syndrome in a patient with primary thrombocythemia treated with interferon alfa and transjugular portosystemic shunt]. / Budduv-Chiariho syndrom u pacientky s primární trombocytémií lécené interferonem alfa resený transjugulární portosystémovou spojkou.

The case report of a young female patient with personal history of primary thrombocythaemia, treated with interferon alpha, admitted to our medical department for severe abdominal pain, hepatomegaly, ascites and alteration of hepatic function is presented. Magnetic resonance imaging showed the picture typical for Budd-Chiari syndrome caused by external obstruction of the intrahepatal portion of inferior vena cava. The cause of the syndrome remains uncertain, possibility of the haematogenic infiltration of the liver or venal thrombosis within primary or secondary (interferon-induced) antiphospholipid syndrome is discussed. Liver biopsy could elucidate the exact cause, but it was not performed for technical problems.

Long term results of percutaneous balloon valvoplasty of congenital aortic stenosis: independent predictors of outcome.

OBJECTIVE: To evaluate long term results and independent predictors of outcome of aortic valvoplasty. DESIGN: Retrospective follow up study. Independent predictors of outcome identified by multiple logistic regression. SETTING: Tertiary referral centre. PATIENTS: 269 consecutive patients treated at the median age of 8 months (0-23 years): 80 (30%) under 4 weeks, 59 (22%) between 4 weeks and 1 year, and 130 (48%) over 1 year. The follow up period was up to 14.8 years (median 5.3, in survivors 6.4 years). INTERVENTIONS: Percutaneous balloon valvoplasty with mean (SD) balloon to annulus ratio 0.97 (0.08). MAIN OUTCOME MEASURES: Restenosis > or = 70 mm Hg, grade 3 aortic insufficiency, cusps disruption, surgery, death, and valvoplasty failure (significant restenosis or insufficiency or surgery or death). RESULTS: The mortality rate was 10.4% (n = 28), the restenosis rate was 16.7% (n = 45), significant insufficiency developed in 22.3% (n = 60), surgery was needed in 20.1% (n = 54), and "valvoplasty failure" occurred in 41.6% (n = 112) patients. Mean (SEM) survival probability 14.4 years after the procedure was 0.89 (0.02) and mean (SEM) probability of surgery-free survival was 0.50 (0.08). The independent predictors were as follows. For restenosis: small aortic annulus; for cusp disruption: large aortic annulus; for insufficiency: bicuspid aortic valve; for need for surgery: bicuspid aortic valve; for death: small aortic annulus, low left ventricular shortening fraction, and low sequential number of the valvoplasty; and for valvoplasty failure: small aortic annulus, bicuspid aortic valve, and high grade of mitral insufficiency. CONCLUSION: Independent predictors of unfavourable outcome are small aortic annulus, bicuspid aortic valve, poor function of left ventricle or mitral valve, and limited operator experience.

Role of late potentials in identifying patients at risk for ventricular tachycardia after surgical correction of congenital heart disease.

This study evaluates the role of late potentials in identifying patients with sustained ventricular tachycardia (VT) after surgery for congenital heart defects using right ventriculotomy. Nine patients with early (1 to 12 months) or late VT (spontaneous in 7 [induced during electrophysiologic study in 6 of the 7] and induced in 2) and 104 patients without VT were studied. All patients had complete right bundle branch block. Late potentials were quantified by 3 time-domain parameters of the signal-averaged electrocardiogram: total filtered QRS duration, root-mean-square voltage in the last 40 ms, and low-amplitude signal duration of the terminal filtered QRS complex. Root-mean-square voltage filtered at 80 to 250 Hz was lower in patients with than without VT (9 +/- 6 vs 16 +/- 8 microV, p < 0.01). By multivariate analysis, more complex surgery (i.e., insertion of extracardiac conduits, Kawashima operation, etc.), lower root-mean-square voltage, and higher Lown grade (2 to 5) of ventricular arrhythmias on routine postoperative Holter recordings were the only independent predictors of VT (p < 0.001, < 0.05, and < 0.05, respectively). A combination of more complex surgery, root-mean-square voltage < 14 microV, and Lown grade 2 to 5 ventricular arrhythmias was highly predictive of VT (positive predictive value 75%). Conversely, patients without complex surgery and with root-mean-square voltage of > 13 microV were not likely to develop VT (negative predictive value 100%). Late potentials are an independent predictor of VT and may improve the identification of patients at low and high risk of VT after surgery for congenital heart defects using right ventriculotomy.

[Magnetic resonance imaging of the postoperative status in children with congenital heart defects]. / Zobrazení pooperacních stavu detí s vrozenou srdecní vadou magnetickou rezonancí.

The justification of examinations by magnetic resonance and the results of visualization of cardiac and vascular structures in relation to the performed surgical operation were evaluated retrospectively in 24 children after surgery of congenital heart disease. Thirteen patients had an operation of coarctation or a double aortic arch and four had an operation of an anomaly of the branch of the pulmonary artery-its loop or atresia. Seven patients had operations, mostly complicated inborn heart disease, of the common arterial trunk and transposition of the large arteries with a conduit, tricuspidal atresia after Fontan's or Glenn's modified operation and ligature of an aortal aneurysm. At the time of examination the patients were 4 to 18 years old. Spin echo sequences obtained by basic reconstructions of T1 and possibly T2 weighted images on a 1.5 T Magnetom apparatus Siemens proved an excellent visualization in the area of the large arteries and the capacity of detailed visualization of cardiac structures, incl. changes of the vascular wall in relation to the performed operation. Magnetic resonance examinations provided accurate information on central anastomoses and conduits as well as on changes of the vascular lumen. The investigation confirmed that magnetic resonance is suitable for visualization of postoperative changes and findings of morphological structures in particular at sites of cardiovascular anastomoses where magnetic resonance supplements the visualization capacity of echocardiography and substitutes invasive examinations where only an angiographic examination is needed. In eight instances the examination was supplemented by invasive assessment of haemodynamic conditions.

[Bidirectional cavopulmonary anastomosis in palliative surgical treatment of complex congenital heart defects]. / Bidirekcní kavopulmonální anastomóza pri paliativní chirurgické lécbe komplexních vrozených srdecních vad.

Bidirectional cavopulmonary anastomosis was used during the past six years for palliative treatment of 34 children with complex cardiac disease with a low pulmonary blood flow and a functional common ventricle. The age at the time of operation was 5 months to 16 years (median 55 months). Two children (5.9%) died soon after operation and one patient died in conjunction with reoperation seven months later. After operation a significant increase of saturation and decrease of the haematocrit occurred in all patients. The condition of 31 patients during an out-patient check-up 2-64 months after operation was satisfactory and postoperative examinations revealed a satisfactory function of the anastomosis.

[Regional differences in the prevalence of congenital heart defects]. / Krajové rozdíly výskytu vrozených srdecních vad.

Prevalence of congenital heart diseases was studied prospectively in all 664,218 infants live born in Bohemia from 1977 to 1984. All children who died were autopsied and those suspected of having a heart disease were examined at the specialized center. In total, 664/100,000 infants were born with a heart defect, most of them in Prague (957,9/100,000) and in Central Bohemia (739.4) and in Northern Bohemia (619.3). The lowest prevalence was found in West Bohemia (515.1/100000 live births). The relative frequency of congenital heart defect categories differed significantly among regions. Evidence of regional differences in prevalence of congenital heart diseases and their forms suggests that environmental factors can play an important role in the etiology of congenital heart defects.

[Cardiac arrhythmia following total correction of tetralogy of Fallot]. / Herzrhythmusstörungen nach Totalkorrektur der Fallotschen Tetralogie.

41 patients after surgical correction of the tetralogy of Fall of aged 2-24 years (mean 11.2 y.) were followed for 12-65 months (mean 39.5 m.) for known or suspected dysrhythmias. 24-hour ambulatory ECG and exercise testing demonstrated nonsignificant ventricular ectopy (grade 0-1 according to a modified Lown classification) in 28 patients (group I) and significant (grade 2-5) ventricular dysrhythmias in the remaining 13 patients (group II). There were 2 cases of sudden late death in group II, whereas all patients in group I are alive. The victims of sudden death had no, other serious dysrhythmias and received no antiarrhythmic treatment. Ejection fraction of the functional part of the right ventricle estimated by radionuclide angiocardiography was not different in both groups whereas, global right ventricular ejection fraction differed nearly significantly (group I: 39.6 +/- 8.3%, group II: 31.9 +/- 10.4%, p = 0.052). Thus, a diminished global right ventricular ejection fraction caused by an extensive resection and large patch in the right ventricular outflow tract may be connected with the occurrence of higher grades of ventricular ectopy. These may lead to sudden late death.

Lorcainide treatment of Wolff-Parkinson-White syndrome in children and adolescents.

Lorcainide was used in 17 children and adolescents aged 14 days to 18 years (mean 6.8 years) with the preexcitation syndrome (W-P-W type). Lorcainide was able to control attacks of supraventricular tachycardia in eight of 11 patients with the W-P-W syndrome and tachyarrhythmias. Long-term maintenance therapy prevented new attacks of tachyarrhythmia for an average period of nine (5-15) months in all seven patients who tolerated lorcainide administration. Normalization of the W-P-W pattern was reached in nine of 11 children with the W-P-W syndrome who had tachyarrhythmias and in three of six asymptomatic children with the ECG pattern of W-P-W. Single effective doses ranged from 12.5 mg orally in the neonates to 100 mg in the adolescents. The effect of lorcainide on the ECG usually appeared 2 h after the oral administration of the drug. Dizziness in three with insomnia and vomiting in one patient complicated the treatment. No drug-associated abnormalities in blood cell counts and biochemical values were identified.

Results of radical surgical correction of ventricular septal defect.

This study evaluates results of radical correction of simple and complex ventricular septal defect in 85 children. 35 children were under 3 years of age. Two babies operated at the age of 6 and 8 months in a critical condition died after surgery. Total operation mortality was 2.3%. In 51 patients examined one year and more after surgery, the long-term results are in 90% of the cases excellent and in 10% favourable. In none of the patients did the authors find an unfavourable result of the operation. The authors regard radical surgical correction of ventricular septal defect as a method of choice regardless of the patient's age and weight.