German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease: Report 2020-Comprehensive Data from 6 Years of Experience.

BACKGROUND: Based on a quality assurance initiative of the German Society for Thoracic and Cardiovascular Surgery (DGTHG) and the German Society for Pediatric Cardiology and Congenital Heart Defects (DGPK), a voluntary registry was founded for assessment of treatment and outcomes of patients with congenital heart disease in Germany. This evaluation by the German Registry for cardiac operations and interventions in patients with congenital heart disease reports the data and the outcome over a 6-year period in patients undergoing invasive treatment. METHODS: This real-world database collects clinical characteristics, in-hospital complications, and medium-term outcome of patients who underwent cardiac surgical and interventional procedures within the prospective, all-comers registry. Patients were followed-up for up to 90 days. RESULTS: In the period from 2013 to 2018, a total of 35,730 patients, 39,875 cases, respectively 46,700 procedures were included at up to 31 German institutions. The cases could be subcategorized according to the treatment intention into 21,027 (52.7%) isolated operations, 17,259 (43.3%) isolated interventions, and 1,589 (4.0%) with multiple procedures. Of these, 4,708 (11.8%) were performed in neonates, 10,047 (25.2%) in infants, 19,351 (48.5%) in children of 1 to 18 years, and 5,769 (14.5%) in adults. Also, 15,845 (33.9%) cases could be allocated to so-called index procedures which underwent a more detailed evaluation to enable meaningful comparability. The mean unadjusted in-hospital mortality of all cases in our registry ranged from 0.3% in patients with isolated interventions and 2.0% in patients with surgical procedures up to 9.1% in patients undergoing multiple procedures. CONCLUSION: This annually updated registry of both scientific societies represents voluntary public reporting by accumulating actual information for surgical and interventional procedures in patients with congenital heart disease (CHD) in Germany. It describes advancements in cardiac medicine and is a basis for internal and external quality assurance for all participating institutions. In addition, the registry demonstrates that in Germany, both interventional and surgical procedures for treatment of CHD are offered with high medical quality.

Repair of critical aortic coarctation in neonatal age.

BACKGROUND: The data of 111 (male: 64; female: 47) in the period of 1967 until 12/93 consecutive operated neonatals (<1 month) were studied retrospectively (mean weight 3270 g, mean age at operation 14 days). METHODS: Preductal anatomy was present in 96 patients. The coarctation was isolated in 30 patients (group I), 34 patients had additional large ventricular septal defects (group II) and 47 had complex heart disease (group III). The preoperative heart catheterization revealed a gradient of <20 mmHg in 35%, >20 mmHg in 51.4% and >50 mmHg in 12.9%. The indication for repair was conservatively untreatable heart insufficiency. In the vast majority (n=97) of patients resection and end-to-end anastomosis were performed, in 31 cases using an absorbable suture, in 18 of these using a continuous suture line. In 4 patients a subclavian flap angioplasty (SFA) was done, in 4 a patch enlargement, 4 times a repair was described as not possible and in 2 patients there was no gradient after division of the ductus. RESULTS: Early lethality was 3.3% (n=1) in group I, 24.2% (n=8) died in group II and 39.1% (n=18) in group III; after introducing Prostaglandin E1 0% in group I, 15% in II and 25% in III. Relevant recoarctation (Gradient >20 mmHg) developed in 9 (among them 4 with hypoplastic arch, 2 after SFA) of the 77 long-term survivors; 6 of these were reoperated on, 5 without residual gradient, 1 with a gradient of 25 mmHg without clinical symptoms (after 4 years). In the last 3 patients a balloon dilation was carried out without residual gradient. Mean follow-up time was 6 (0-24) years. No patient needs antihypertensive treatment. The cumulative survival rate is 96.7% (+6.6%) for group I, 77.4% (+15.0%) for II and 51.9% (+16.6%) for III. CONCLUSIONS: Resection and end-to-end anastomosis using a continuous absorbable suture is the method of choice at theoretical considerations and in our experiences. The number of recoarctations in neonatal age is relatively high; reinterventions (operation respectively dilation) can be done safely and successfully.

Long-term results after repair of total anomalous pulmonary venous connection.

BACKGROUND: Operative strategies and early results concerning repair of Total Anomalous Pulmonary Venous Connection (TAPVC) are relatively well known. Less well defined data are available to evaluate the long-term outcome. We would therefore like to contribute our long-term data in this presentation. PATIENTS AND METHODS: Between 1958 and 1992 52 consecutive patients aged two days to 42 years (15 neonates, 16 infants, 9 children and 12 adults) with TAPVC were operated on. The data were collected retrospectively from the records. In 24 patients, a current follow-up study was performed, including clinical evaluation, echocardiography, and a twenty-four-hour ambulatory ECG. RESULTS: Early mortality was 34.6% (n = 18). The postoperative follow-up period ranged from 4 months to 28 years (mean 10.7 years). There were 4 late deaths, yielding an overall long-term mortality of 7.7% (4/52). Causes of death were severe hypoplasia of central pulmonary veins in 1, ventricular fibrillation (2) and non-cardiac in one case. 80% of the operative survivors were available for assessment. Preoperatively, 11 of these patients were in NYHA functional class II, six in class III and seven in class IV. After treatment, 22 patients were in class I and two in class II. Ventricular function was evaluated by echocardiography and invasive catheterization. Only two of 24 patients (8%) showed an abnormal IVS-motion and enlargement of the right ventricle. Cardiac catheterization revealed a mean PA pressure of 26 mmHg, the peak systolic pressure in the RV was 34 mmHg. All 24 long-term survivors underwent assessment of cardiac rhythm by 24 h electrocardiogramm (ECG) monitoring. Significant arrhythmias were recorded in 11 of 24 cases (46%), including sinus node dysfunction in 3 patients. Multiform ventricular ectopic beats were evaluated in 9 cases. According to the Lown classification, 7 patients were class I while 2 cases were considered to be class IV. CONCLUSIONS: A normal hemodynamic state can be achieved in most cases. Significant arrhythmias may exist in asymptomatic patients late after surgical correction of TAPVC, and therefore, long-term follow-up of these patients, including 24 h ECG monitoring, is recommended, even if they are asymptomatic.

Balloon valvoplasty in infants with tetralogy of Fallot: effects on oxygen saturation and growth of the pulmonary arteries.

Balloon valvoplasty was undertaken in 27 patients with tetralogy of Fallot for first-stage palliation. Indications were arterial saturation of oxygen < 80%, hypoxic spells and duct-dependant pulmonary perfusion. The dilation was performed following diagnostic heart catheterization. Saturations improved from 75% +/- 8.5 before valvoplasty to 85% +/- 8.4 after the procedure, and worsened little to 83% +/- 9.6 at follow-up after 3.4 months. The pulmonary valvar orifice was hypoplastic in most patients (Z = -3.3 +/- 1.2), and did not change after the procedure. The cross-sectional area of the pulmonary arteries was diminished initially, reflected by a Nakata index of 186 +/- 95 mm2/m2. After valvoplasty, we found widening of the vessels (Nakata index 225 +/- 100 mm2/m2). At follow-up no further growth of the arteries was observed (Nakata index 209 +/- 109 mm2/m2). The procedure was complicated by hypoxic spells in three patients which were controlled by intravenous propranolol, and deep venous thrombosis in four patients. Our data demonstrate that balloon valvoplasty is feasible for initial palliation in patients with tetralogy of Fallot. It does not, however, produce growth of the pulmonary arteries or of the pulmonary valve.

Determinants of left ventricular diastolic function during myocardial ischemia: influence of myocardial structure and pericardial constraint.

OBJECTIVE: To assess the influence of myocardial structure and pericardial constraint during exercise ischemia on regional left ventricular passive elastic properties. METHODS: Left ventricular regional function was assessed at rest and during exercise using biplane angiography and high-fidelity pressure measurements. Twenty patients with either normal (n = 7) or stenotic coronary arteries (n = 13) were studied before and after successful bypass surgery. At the time of surgery, left ventricular transmural biopsies were taken from a normally perfused and a hypoperfused left ventricular region. RESULTS: Regional stiffness increased in the ischemic zone during exercise, but remained unchanged after revascularization. Regional fibrosis was significantly enhanced in the ischemic region compared with that in the normally perfused zone. No correlation was found between structural data and regional passive elastic properties, but there was a significant correlation between right atrial pressure and the asymptote of the diastolic pressure--volume relationship. CONCLUSIONS: Acute regional diastolic dysfunction can be observed during exercise in patients with coronary artery disease. Structural changes seem to have a minor role in the occurrence of diastolic dysfunction in the absence of myocardial infarction. The observed upward shift of the pressure-volume relationship during ischemia can be attributed to pericardial constraint that is manifested by an increase in right arterial pressure.

Effect of intermittent warm blood cardioplegia on functional recovery after prolonged cardiac arrest.

BACKGROUND: There is some evidence that continuous warm blood cardioplegia offers good myocardial protection; however, the effects of interrupting cardioplegia remain controversial. To study this, we compared the effects of continuous and intermittent antegrade warm (37 degrees C) blood cardioplegia on functional recovery after prolonged cardiac arrest (180 minutes). METHODS: Twenty-four juvenile pigs were randomly assigned into four groups. Group 1 received continuous cardioplegia, group 2 underwent several periods of 15 minutes of cardioplegia interrupted by 5 minutes of normothermic ischemia, and group 3 underwent several periods of 10 minutes of cardioplegia interrupted by episodes of 10 minutes. The hearts of group 4 received no cardioplegia. Left ventricular systolic function was assessed from fractional left ventricular shortening and percentage left ventricular wall thickening, and left ventricular diastolic function was determined from the time constant of relaxation and the constant of myocardial stiffness. RESULTS: Systolic and diastolic functions were slightly depressed 1 and 2 hours after cross-clamp removal in all four groups, without significant differences among the groups. CONCLUSIONS: These data suggest that antegrade warm blood cardioplegia can be interrupted for up to 10 minutes without obvious negative effects on left ventricular function in the normal myocardium, provided that the intermittent doses of cardioplegia are sufficient to restore the metabolic demands of the arrested myocardium.

Complete AV-block in two children with immunohistochemical proven myocarditis.

UNLABELLED: We report two children with acquired third degree AV-block caused by acute myocarditis. The diagnosis was proven by endomyocardial biopsy. Severe lymphocytic myocardial infiltration was shown using immunohistological methods. One of the children was treated with prednisone During therapy conduction disturbance nearly disappeared and infiltration was markedly reduced in a subsequent biopsy. In the other patient the parents refused immunosuppressive treatment and a permanent pacemaker was necessary for persistent bradycardia. CONCLUSION: Immunohistological staining of an endomyocardial biopsy can be used to establish the diagnosis of myocarditis in patients with atypical clinical manifestation, such as complete AV-block, and can support the decision for therapy. In one patient improvement was documented by the disappearance of inflammatory activity in a repeated biopsy.

Clinical experience with propafenone for cardiac arrhythmias in the young.

Seventy-two children were treated with propafenone between 1980 and 1990. The mean age was 34 months (range 0-192). Arrhythmias included atrioventricular re-entry tachycardia in 32 patients (44%), atrial flutter in 16 (22%), atrial or junctional ectopic tachycardia in 10 (14%), atrial re-entry tachycardias in three (4%) and ventricular arrhythmias in 11 patients (16%). The efficacy of oral treatment was good in patients with atrio-ventricular re-entry tachycardia (80%), atrial flutter (71%) and atrial ectopic tachycardia (83%); it was poor in ventricular arrhythmias (40%). The mean oral dose was 13.5 mg.kg-1. day-1. Dosage and serum levels of propafenone did not differ whether the patients were treated successfully or not. No correlation between dosage and serum level was observed. Intravenous propafenone administration was only partially successful in suppressing supraventricular tachycardias (6 of 11 patients). The presence of a congenital heart defect and the time of onset of the arrhythmias had a significant influence on the efficacy of propafenone. Better results were observed in patients with normal hearts and in whom onset of arrhythmia was pre-natal (success 80%) as well as in patients with arrhythmias seen early after surgery for congenital heart defects (success 87%). Success (65%) was also observed in patients without congenital heart defects and postnatal onset of supraventricular arrhythmias. Patients with ventricular or supraventricular arrhythmias late after corrective surgery showed the poorest response (31%).

Coronary artery size in mitral regurgitation and its regression after mitral valve surgery.

The relationship between coronary artery size and left ventricular (LV) muscle mass was studied in 10 control subjects and in 10 patients with chronic mitral regurgitation before and 28 +/- 15 months after mitral valve surgery. Left and right coronary artery size was determined by quantitative coronary arteriography. Left coronary artery size was significantly increased before surgery (26 mm2) and decreased after operation (23 mm2), but was still larger than in control subjects (14 mm2). The right coronary artery was also enlarged preoperatively (13 mm2; controls = 9 mm2), but was normalized after surgery (11 mm2). A linear correlation was found between LV muscle mass and left (r = 0.88, p < 0.001) and right coronary artery size (r = 0.84, p < 0.001) as well as between right coronary artery size and mean pulmonary artery pressure (r = 0.56, p < 0.01). Thus in chronic mitral regurgitation the enlargement of the left and right coronary artery is proportional to the degree of LV hypertrophy. The increase in right coronary artery size is probably the result of right ventricular pressure overload. Postoperatively there is only partial regression of left coronary artery size but normalization of right coronary artery size.

Non-invasive evaluation of left ventricular systolic function late after coarctation repair: influence of early vs late surgery.

The study was designed to assess non-invasively the long-term effect of coarctation repair on systemic blood pressure, left ventricular (LV) muscle mass (LMM) and LV systolic function. Blood pressure and pressure gradients across the coarctation site were measured at rest and during exercise. LV systolic function and LMM were assessed by echocardiography. Twenty-eight patients late after successful coarctation repair were divided according to their age at surgery into two groups: group 1: < 1 year (10 days-12 months, mean 0.2 years) and group 2: > 1 year (1-19 years, mean 9.7 years). A group of age- and sex-matched patients with normal LV function served as controls. LMM was increased late postoperatively in both groups irrespective of the age at surgery and was correlated significantly with the elevated systolic blood pressure and the residual pressure gradient at exercise. End-systolic wall stress was normal at rest and the stress/velocity relationship revealed normal contractility in all patients. Despite successful operation of aortic coarctation, residual LV hypertrophy persists 2 to 19 years after surgery irrespective of the age at surgery. LV systolic function is normal. Hypertrophy can be explained by the residual arm-leg pressure gradient during exercise which persists even after successful repair.

[Coronary artery size in chronic mitral valve insufficiency before and following mitral valve surgery]. / Koronararterienkaliber bei chronischer Mitralinsuffizienz vor und nach Mitralklappenoperation.

The increase of coronary artery size in myocardial hypertrophy represents an adaptive mechanism to keep coronary blood flow normal. The relationship between coronary cross-sectional area and left ventricular muscle mass was determined angiographically in 10 patients with severe mitral regurgitation before and 28 +/- 15 months after successful mitral valve surgery. 10 subjects with atypical chest pain without coronary artery disease served as controls (C). Left ventricular muscle mass was increased preoperatively in mitral regurgitation (257 g vs C = 129 g; p < 0.001) and decreased postoperatively (205 g; p < 0.01 vs preop. and vs C). The cross-sectional area of the left coronary (= left anterior descending+left circumflex) artery was augmented preoperatively (26.5 vs C = 14.0 mm2; p < 0.001) and decreased postoperatively (22.9 mm2; p < 0.05 vs preop. and vs C). The cross-sectional area of the left coronary artery per 100 g LV muscle mass was not different in the three groups. The cross-sectional area of the right coronary artery was also increased before surgery (12.7 vs C = 8.8 mm2; p < 0.05) and decreased postoperatively (11.3 mm2; p < 0.05 vs preop. ns vs C). Our data show that in mitral regurgitation the size of the left coronary artery increases proportionally to the increase in left ventricular muscle mass. Also, the right coronary artery shows slight enlargement which is probably due to the pressure overload of the right ventricle. After surgery there is regression but not normalization of the size of the coronary arteries.