Jan Škovránek

Prevalence, treatment, and outcome of heart disease in live-born children: A prospective analysis of 91,823 live-born children

SummaryAll 91,823 children born in 1980 in Bohemia (population 6.314 million; area 52,478 square kilometers) were examined at least four times during infancy and at the age of three and four years. All children who died were autopsied and those with heart disease were selected. A total of 779 children (8.223/1000 live births) were suspected by provincial pediatric cardiologists of having a heart disease. All of these were examined at the age of four years at our Center of Pediatric Cardiology. At this age heart disease was proved in 613 alive or decreased children (6.676/1000 live births), congenital cardiac malformations in 589 (6.415/1000 live births), and cardiomyopathies in 24. The most frequent congenital heart defects (CHD) were ventricular septal defect (VSD) (31.41%), atrial septal defect (ASD) (11.37%), aortic stenosis (AS) (7.64%), pulmonary stenosis (PS) (7.13%), coarctation of the aorta (CoA) (5.77%), and transposition of the great arteries (TGA) (5.43%), followed by persistent ductus arteriosus (PDA) (4.75%), atrioventricular septal defect (AVSD) and hypoplastic left heart syndrome (HLHS) (4.07% each), tetralogy of Fallot (TF) (3.56%), and pulmonary atresia (PA) (2.38%). A prevalence of less than 0.1/1000 live births was found for the remaining cardiovascular defects.One hundred fifty-nine (25.9%) patients were admitted to our highly specialized center, 116 (19.7%) catheterized and 85 (13.9%) treated surgically, during the first four years of life. A total of 440 (71.8%) patients survived the fourth year of life. The percentage of deaths was 25.6% among those with congenital heart diseases and 71% with cardiomyopathies. The overall mortality rate was 27% in surgically and 26% in medically treated patients.

Prenatal ultrasound screening of congenital heart disease in an unselected national population: a 21-year experience

Objective To determine the prevalence and spectrum of congenital heart disease (CHD) and the impact of a national prenatal ultrasound screening programme on outcome in a well-characterised population. Design and setting A comprehensive registry was created of all paediatric and fetal patients with CHD over a 21-year period (1986–2006) in the Czech Republic. The centralised healthcare system enabled confirmation of prenatal and postnatal findings clinically and by post mortem. Patients and results In the entire cohort of 9475 fetuses referred for detailed cardiac evaluation, 1604 (16.9%) had CHD, of which 501 (31.2%) had additional extracardiac anomalies. In the pregnancies which continued, 59 (8.6%) of 685 fetuses died in utero, and 626 (91.4%) babies were born alive. Prenatal detection rate was highest in double outlet right ventricle (77.3%) and hypoplastic left heart (50.6%). Detection rate increased significantly (p<0.001) for 12/17 lesions comparing 1986–1999 and 2000–2006. In recent years, detection of hypoplastic left heart reached 95.8% while transposition of the great arteries was diagnosed antenatally in only 25.6%. Conclusion The nationwide prenatal ultrasound screening programme enabled detection of major cardiac abnormalities in 1/3 of patients born with any CHD and 80% of those with critical forms. Nevertheless, owing to the severity of lesions and associated extracardiac anomalies, the overall mortality of antenatally diagnosed CHD remains high. These findings are important for the understanding natural history of CHD for the establishing of screening programmes in Europe.

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 ⩾ 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.

Long term ventricular performance after intra-atrial correction of transposition: left ventricular filling is the major limitation

Objective To establish the incidence of systolic and diastolic dysfunction of the right and left ventricle in a large cohort of patients after Mustard or Senning operations and to assess changes in the incidence on long term follow up. Design Postoperative case-control study using radionuclide ventriculography. Ejection fractions, peak filling rates, rapid filling periods and fractions, slow filling periods and fractions, and atrial contraction periods and fractions were studied. Setting Tertiary care centre, ambulatory and hospital inpatient care. Patients A convenience sample of 153 patients studied at median age of 6.9 years (median 4.4 years after surgery). In 99 cases another study was available at a median age of 15.3 years (median 13 years after surgery and 8.8 years after the first study). Results Respective incidences of dysfunction in the first and the second study were as follows: ejection fraction–right ventricle 7.8% and 8.1%, left ventricle 7.2% and 10.1%; peak filling rate–right ventricle 0% and 4.2%, left ventricle 14.3% and 29.5% (p < 0.05); rapid filling period– right ventricle 18.3% and 11.6%, left ventricle 30.2% and 30.5%; slow filling period—right ventricle 4.8% and 3.2%; left ventricle 11.9% and 23.2%;atrial contraction period–right ventricle 0.8% and 4.2%, left ventricle 15.1% and 26.3%; rapid filling fraction–right ventricle both 0%, left ventricle 82.5% and 79.0%; slow filling fraction–right ventricle 0.8% and 4.2%, left ventricle 37.3% and 30.5%; atrial contraction fraction–right ventricle both 0%, left ventricle 79.4% and 71.6%. Conclusions The incidence of systolic ventricular dysfunction is 8% (right ventricle) and 10% (left ventricle) 13 years after surgery, without a significant increase over the eight year follow up. Diastolic filling is abnormal in up to 80% of patients and left ventricular peak filling rate deteriorates with time.

Differences in muscle blood flow in upper and lower extremities of patients after correction of coarctation of the aorta.

SUMMARY Using the method of 1333Xe clearance we investigated blood flow and calculated vascular resistances simultaneously in the muscles of the upper and lower extremities in 58 patients following successful surgical correction of aortic coarctation carried out at age 11.5 (±2.9) years. The interval from operation to investigation was 11.5 (±4.5) years. Resting and maximal ischemic exercise blood flows in the upper extremity were decreased and the duration of maximal blood flow was shortened. Values recorded from the lower ex- tremities did not differ from normal controls. The difference between upper and lower extremities was statistically significant. Vascular resistance during maximal blood flow was higher in the upper extremities than in the lower. Differences between upper and lower extremities did not change after vasodilation elicited by amyl nitrite. The degree of differences was not dependent upon the age at operation, the age of the patients at investigation, or on the time interval between operation and investigation.

Seven-Year Experience of Noninvasive Preoperative Diagnostics in Children with Congenital Heart Defects: Comprehensive Analysis of 2,788 Consecutive Patients

The spectrum of patients operated on without preoperative catheterization and angiography, the accuracy of echocardiographic diagnosis and its impact on the results of surgical treatment were prospectively assessed in 2,788 children consecutively operated for congenital heart defects (CHD) between 1986 and 1992. The overall percentage of surgery based solely on noninvasive preoperative examination increased from 63% in 1986 to 81% in 1990 and decreased to 72% in 1992. There were no differences in the preoperative diagnostic approach between groups of newborn, infants and children. A high percentage of patients with patent ductus arteriosus (96.5%), atrial septal defect (94%), incomplete atrioventricular septal defect (88.6%), ventricular septal defect (86.3%), coarctation of the aorta (80.2%) and total anomalous pulmonary venous connection (79.3%) was referred for surgery without prior invasive examination, while a lower percentage was found in univentricular heart (48.4%), pulmonary atresia (34.6%) and double outlet right ventricle (27.7%). More patients with pulmonary and tricuspid atresia were catheterized before complete repair compared to those who underwent palliative surgery (p < 0.01 and p < 0.0001, respectively). The echocardiographic diagnosis was correct in 96% of patients. Two patients of those with incomplete preoperative diagnosis died early postoperatively, both with missed apical ventricular septal defect. One with tetralogy of Fallot died after reoperation, the other with persistent truncus arteriosus due to sepsis. When the echocardiographic findings are in full agreement with the clinical status, physical examination, ECG and chest X-ray, we recommend cardiac surgery without prior catheterization in many patients with CHD.

Intermediate to late results of surgical relief of vascular tracheobronchial compression

Since 1979, a total of 69 infants and children aged 0.1 to 11.9 (median 0.7) years required surgical intervention for: double aortic arch (26), anomalous origin of innominate artery (26), right aortic arch with left ligamentum arteriosum (9), pulmonary artery sling (5), retroesophageal right subclavian artery (3). Before operation, the morbidity was high: 84% of children were symptomatic in the first trimester of life with 24 patients requiring mechanical ventilation. Seven children had an accompanying heart defect. Left thoracotomy was the preferred approach. There were 2 early (asphyxic brain damage, postoperative pneumonia) and 2 late (tracheomalacia, complex heart defect) deaths. Five patients (2 originally operated elsewhere) needed reoperation for persistent symptoms. All 65 survivors are well 1 month to 11.9 (mean 3.9 +/- 3.62) years after surgery. Tracheobronchoscopy, magnetic resonance imaging, and lung function testing were helpful for postoperative evaluation. Minor tracheal compression was revealed in 4 patients despite their good clinical condition.

Results of primary and two-stage repair of interrupted aortic arch

OBJECTIVE Early results of primary and two-stage repair of interrupted aortic arch have improved. Experience with different surgical approaches should be analysed and compared. METHODS Forty neonates and infants with interrupted aortic arch underwent primary repair (19 patients) or palliative operation (21 patients). Twenty (50%) patients were followed-up for 5.1+/-4.3 years. All patients were regularly examined with the aim of determining clinical development, presence of residual lesions or complications and need for re-intervention. Aortic arch and the left ventricular outflow tract growth were assessed by echocardiographic examination. Data from hospital and outpatient department records were analysed. RESULTS The early mortality was 61.9% after palliative operations and 36.8% after the primary repair. Presence of complications (P < 0.001), earlier year of surgery (P < 0.01), bad clinical condition and acidosis (P < 0.05) represented statistically significant risk factors for death in the whole series. In seven (87.5%) out of eight early survivors, after the initial palliative operation, closure of ventricular septal defect and debanding were done, and in three (37.5%) patients, re-operation for aortic arch obstruction was also required. Out of 12 patients, after the primary repair, one required early re-operation for persistent left ventricular outflow tract obstruction and two needed late re-intervention for left bronchus obstruction. In three (25%) patients, after the primary repair, left ventricular outflow tract obstruction with a maximal systolic pressure gradient higher than 30 mmHg developed. At present, all 20 early survivors are alive. Five patients, after palliative operation, are in NYHA class 1, but in three patients, who are in class III or IV, the outcome is influenced by severe complications. All patients after the primary repair are in class I or II. CONCLUSIONS Our experience confirmed better results after the primary repair of interrupted aortic arch, which was associated with lower mortality, prevalence of severe complications and need for re-intervention. Higher prevalence of subaortic stenosis after primary repair could be explained by patient selection early in our experience. We recommend the primary repair of interrupted aortic arch and associated heart lesions in neonates, however, in unfavourable conditions an individualised surgical approach with initial palliative surgery should be considered.

Coagulation profile and liver function in 102 patients after total cavopulmonary connection at mid term follow up

Objective: To examine coagulation factors and liver function test abnormalities in patients after total cavopulmonary connection (TCPC). Design: Cross sectional study comprising clinical and echocardiographic evaluation, and biochemical and coagulation profile screening. Setting: Tertiary referral centre. Methods: 102 patients aged 4–24 years (median 10 years) at one to eight years (median five years) after TCPC were examined. All patients were maintained on a low dose of aspirin. 96% of patients were in a good clinical condition (New York Heart Association class I or II). No intracardiac thrombi were detected on echocardiography and ventricular function was good in 91% of patients. Results: Total bilirubin was increased in 27% and γ glutamyltransferase in 54% of patients. Serum total protein, albumin, and prealbumin were normal in almost in all patients. Compared with the control group, patients after TCPC had significantly lower fibrinogen, factor V, factor VII, and protein C concentrations, prolonged international normalised ratio, and increased antithrombin III concentration. Factor V concentration was abnormally decreased in 35%, factor VII in 16%, and protein C in 28% of patients. Antithrombin III was increased in 23% of patients. Factor VII, factor V, protein C, and antithrombin III correlated significantly with serum prealbumin. There was also a significant correlation between procoagulant factor VII and both anticoagulant protein C and antithrombin III. Conclusions: Almost half of patients after TCPC had laboratory signs of mild cholestasis. Decreased liver synthesis of procoagulant and anticoagulant factors was observed but overall coagulation homeostasis appeared to be in balance in this selected group of patients with a good clinical outcome.

Long-term (up to 20 years) results of percutaneous balloon angioplasty of recurrent aortic coarctation without use of stents

AIMS To assess the efficacy, safety, and long-term results of the balloon angioplasty of recoarctation. METHODS AND RESULTS The angioplasty was performed in 99 consecutive patients aged 36 days to 32.6 years (median 268 days). Recoarctation to descending aorta diameter ratio increased from 0.44 (0.35/0.50) to 0.66 (0.57/0.77), P < 0.001. Systolic gradient was reduced from 34.0 (26.0/44.75) to 15.0 (8.25/27.0) mmHg, P < 0.001. In seven patients (7.1%) the procedure was ineffective. One patient (1%) with heart failure died within 24 h after a successful angioplasty and in another (1%) an intimal abruption necessitated surgical revision. The follow-up ranged up to 20.7 years (median 8.1 years). Actuarial probability of survival 20.7 years after the procedure was 0.91, and of reintervention-free survival was 0.44. Older age at the angioplasty was associated with a higher incidence of reinterventions (hazard ratio 1.057; 95% confidence interval 1.012-1.103; P = 0.010). The type of surgery and the recoarctation anatomy did not influence the outcome. In 69 patients aneurysm formation was studied by high-sensitive methods with only one positive finding per 462 patient-years. CONCLUSION Angioplasty is safe and effective regardless of the type of surgery used and the recoarctation anatomy. Older age at the angioplasty is associated with a higher incidence of reinterventions.