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.

Arterial tortuosity syndrome: clinical and molecular findings in 12 newly identified families.

Arterial tortuosity syndrome (ATS) is a rare autosomal recessive connective tissue disease, characterized by widespread arterial involvement with elongation, tortuosity, and aneurysms of the large and middle-sized arteries. Recently, SLC2A10 mutations were identified in this condition. This gene encodes the glucose transporter GLUT10 and was previously suggested as a candidate gene for diabetes mellitus type 2. A total of 12 newly identified ATS families with 16 affected individuals were clinically and molecularly characterized. In addition, extensive cardiovascular imaging and glucose tolerance tests were performed in both patients and heterozygous carriers. All 16 patients harbor biallelic SLC2A10 mutations of which nine are novel (six missense, three truncating mutations, including a large deletion). Haplotype analysis suggests founder effects for all five recurrent mutations. Remarkably, patients were significantly older than those previously reported in the literature (P=0.04). Only one affected relative died, most likely of an unrelated cause. Although the natural history of ATS in this series was less severe than previously reported, it does indicate a risk for ischemic events. Two patients initially presented with stroke, respectively at age 8 months and 23 years. Tortuosity of the aorta or large arteries was invariably present. Two adult probands (aged 23 and 35 years) had aortic root dilation, seven patients had localized arterial stenoses, and five had long stenotic stretches of the aorta. Heterozygous carriers did not show any vascular anomalies. Glucose metabolism was normal in six patients and eight heterozygous individuals of five families. As such, overt diabetes is not related to SLC2A10 mutations associated with ATS.

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.

Volumetric analysis of the right ventricle in children with congenital heart defects: comparison of biplane angiography and transthoracic 3-dimensional echocardiography.

BACKGROUND: Three-dimensional echocardiography is a non-invasive imaging technique. The fact that it permits volumetric analyses independently of geometrical assumptions makes it a putatively useful method for the precise measurement of the volumes of the irregularly shaped right ventricles in children. The aim of this study was to assess the feasibility of this method and its agreement with angiocardiography based estimates of right ventricular volume in children with congenital heart disease. METHODS: We studied 102 children with congenital heart disease. The angiocardiographic right ventricular volumetry was performed using a biplanar technique using Simpson's rule and corrected with Lange's correction factors. The echo data sets were registered trans-thoracically with a rotating transmitter. Volumes were calculated after manual planimetry by adding the volumes of the individual slices. RESULTS: Calculation of right ventricular volume echocardiographically was possible only in 34% of patients, mostly infants and toddlers. In comparison to angiocardiography, the measured volumes were 1.1 +/- 6.9 ml (19.5 +/- 34.1%) or 6.3 +/- 9.4 ml (42.5 +/- 33.6%) smaller during systole or diastole, respectively. The limits of agreement were -12.5 and 13.6 ml, or 12.45 and 25.15 ml during systole or diastole, respectively. When plotted to a logarithmical scale, the correlation coefficients r2 were 0.70 for systolic and 0.79 for diastolic measurements. CONCLUSION: Transthoracic 3-dimensional echocardiography with a rotating transmitter is feasible for volumetry only in small children. The volumes measured were significantly smaller than the ones calculated from the angiocardiographic images. The correlation between the two methods is moderate.

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.

Volumetric analysis of the right and left ventricle in a porcine heart model: comparison of three-dimensional echocardiography, magnetic resonance imaging and angiocardiography.

UNLABELLED: Three-dimensional echocardiography and magnetic resonance imaging allow the volumetric analysis of ventricular volumes independent of geometric assumptions. The aim of the study was to compare these methods and the common angiocardiography in a cardiac model of known volume. METHODS/MATERIALS: Right and left ventricular (RV, LV-) volumes were measured in a specific animal model directly ('true volume') and with different imaging techniques. Three-dimensional echocardiography (3D-Echo) and magnetic resonance imaging (MRI), both of which permit a volume estimation without necessitating geometric assumptions, and angiocardiographic volumetry which is based on the Simpson rule were used in this study. RESULTS: The best results were achieved with MRI (RV: r(2)=0.99, mean difference: -1. 9+/-3.3%; LV: difference r(2)=0.99,: 2.9+/-5.0%). Likewise, 3D-Echo showed a very good correlation with the true volumes (RV: r(2)=0.93, difference: 9.3+/-6.3%; LV r(2)=0.96, difference: 4.8+/-9.9%). The greatest deviations were observed during angiocardiographic volumetry (LV: r(2)=0.98; difference: 14.4+/-9.2%), particularly when measuring the right ventricle (RV: r(2)=0.82, difference: 57. 9+/-40.1%). Consequently, the direct comparison between 3D-Echo and the other methods yielded the best correspondence with MRI (RV: Bias: 3.7 ml, limits of agreement: 7.7 ml; LV: Bias: 3.7 ml, limits of agreement: 4.9 ml). In contrast, the differences between 3D-Echo and angiocardiography were marked (RV: Bias: 25.5 ml, limits of agreement: 11.1 ml; LV: Bias: 8.7 ml, limits of agreement: 13.2 ml). CONCLUSION: In a porcine cardiac model, 3D-Echo permits a relatively precise measurement of ventricular volumes with a slight under-estimation. MRI yielded the most precise volumetry, and the correlation between 3D-Echo and MRI was quite good. Particularly for the right ventricle, the angiocardiographic measurement was attached with the greatest error and thus appears ill-suited for the volumetry of geometrically more complex ventricles.

Volume measurement of the left ventricle in children with congenital heart defects: 3-dimensional echocardiography versus angiocardiography.

BACKGROUND: Volume measurement of the left ventricle is currently done by 2-dimensional echocardiography or angiocardiography. However, for the calculation of volumes by these methods, geometrical assumptions must be made. For a precise ventricular volumetry, independent of mathematical assumptions, imaging techniques as the 3-dimensional echocardiography (3D-echo) are required, which permit the imaging of the real ventricular shape. The aim of the study was therefore to detect, whether 3D-echocardiography is suitable for left ventricular volumetry in children with congenital heart disease and whether the 3D-echocadiographically measured volumes correlate sufficiently well with angiocardiographically measured left ventricular volumes. METHODS: 102 children with congenital heart disease were investigated. For angiocardiographic left ventricular volume measurement Simpson's rule was used. Results were corrected with Lange's correction factors. The 3D-echo data sets were registered with a rotating transthoracic transducer. Ventricular volumes were calculated after manual planimetry by summation of the volumes of the single slices. RESULTS: The left ventricular volume could be calculated by 3D-echo in 83% of patients of all ages. In comparison to angiocardiography, the measured volumes were 0.6 +/- 3. 3 ml (0.9 +/- 25.8%) or 7.1 +/- 28.4 ml (7.4 +/- 12.1%) smaller during systole or diastole, respectively. The correlation coefficients r(2) were 0.89 for systolic and 0.93 for diastolic measurements after logarithmic transformation. Pressure or volume overload did not influence significantly the difference between the two methods. CONCLUSION: Transthoracic 3D-echocardiography with a rotating transmitter is feasible for volumetry of the left ventricle in most children. The volumes measured by 3D-echo were significantly smaller than those calculated from the angiocardiography by Simpson's rule. The measurements were not influenced by the kind of load of the ventricle. The correlation between the two methods is good.

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.

Anomalous origin of all coronary arteries from the pulmonary trunk.

The origin of both coronary arteries from the pulmonary artery is a rare cardiac malformation. We report a baby who presented with an echocardiographically diagnosed perimembranous ventricular septal defect and normal left ventricular (LV) function. Later on the boy developed failure to thrive and increasing tachypnea. At the age of 5 weeks the ECG showed that LV strain and echocardiographic LV function had worsened (FS 18%). Echocardiography and heart catheterization showed that all coronary arteries originated from the pulmonary trunk. Intraoperative inspection revealed a single ostium for the right and left coronary artery in the nonfacing sinus of the pulmonary trunk. A tube was constructed connecting the coronary artery to the ascending aorta. Coronary perfusion was sufficient and the sinus rhythm was restored. However, in the early postoperative period there was a sudden deterioration of cardiac output followed by cardiac arrest. Reanimation was not successful.

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.

Tortuosity of coronary arteries in chronic pressure and volume overload.

The role of coronary tortuosity in the pathophysiology of chronic pressure and volume overload is still unclear. A new method for measuring coronary tortuosity in patients with chronic pressure and volume overload was evaluated in 62 patients. Sixteen controls, 14 patients with arterial hypertension, and 32 patients with aortic regurgitation were included in the present analysis. The left anterior descending (LAD) and circumflex (LCX) coronary arteries were traced, and tortuosity was determined in the 30 degrees right (RAO) and 60 degrees left (LAO) anterior oblique projection. Tortuosity index (TI, %) was defined as the percent ratio of calculated shortest distance divided by total length of the coronary artery. TI was 104.1 +/- 3.2% at end-diastole in controls, 105.7 +/- 3.8% in hypertensives (P < 0.05 vs. controls), and 102.9 +/- 2.5% in patients with aortic regurgitation (P < 0.05 vs. controls, P < 0.001 vs. hypertensives). Respective values at end-systole were 107.8 +/- 4.7% in controls, 109.8 +/- 7.1% in hypertensives (ns vs. controls), and 104.3 +/- 3.3% in patients with aortic regurgitation (P < 0.001 vs. controls and vs. hypertensives). No differences were found in tortuosity between RAO and LAO projection or between LAD and LCX artery. There was a significant correlation between TI and left ventricular (LV) muscle mass, LV volume, and age. Females tended to have more tortuous vessels than males. Coronary tortuosity is more pronounced in patients with chronic pressure than with volume overload. Determinants of coronary tortuosity are gender, age, LV volume, and muscle mass. Thus, coronary tortuosity seems to play an important role as a physiologic determinant for the flow and the mechanics of the vessel wall.

Left ventricular function in chronic mitral regurgitation: preoperative and postoperative comparison.

OBJECTIVES: The present study was designed to evaluate the effects of surgical procedure on left ventricular systolic and diastolic function in patients with mitral regurgitation. BACKGROUND: Left ventricular systolic function has been shown to decline after operation in patients with chronic mitral regurgitation. METHODS: Using simultaneous cineangiography and left ventricular micromanometry, we evaluated left ventricular systolic and diastolic function in 14 patients with chronic mitral regurgitation both preoperatively and at an average of 22 months after operation. Eight patients underwent mitral valve reconstruction, and six had a valve replacement with interruption of the chordae tendineae. We compared these patients with 10 control subjects. RESULTS: Preoperatively, patients with mitral regurgitation demonstrated normal global and regional left ventricular systolic function. Peak rate of diastolic filling was increased (p < 0.01), and passive chamber stiffness was decreased, compared with that in control subjects (p < 0.01), and there was normal myocardial stiffness. Postoperatively, systolic and diastolic function returned to normal in patients undergoing mitral valve reconstruction. In contrast, global systolic function was depressed in patients after valve replacement (p < 0.05), with regional dysfunction in the area of papillary muscle attachment (p < 0.01). Diastolic function was depressed in this group, with a prolonged time constant of pressure decay (p < 0.01) and a depressed rate of early diastolic filling and strain rate (p < 0.05). Passive elastic stiffness was within the normal range in all postoperative patients. CONCLUSIONS: The type of operation performed to correct chronic mitral regurgitation has an important effect on postoperative left ventricular function. Systolic and diastolic function are preserved after mitral valve reconstruction. Mitral valve replacement with chordal interruption is associated with global and regional systolic dysfunction and early diastolic filling and relaxation abnormalities.

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.

Left ventricular diastolic dysfunction late after coarctation repair in childhood: influence of left ventricular hypertrophy.

OBJECTIVES: Left ventricular systolic and diastolic function were evaluated late after successful operation for aortic coarctation in childhood. BACKGROUND: Persistent arterial hypertension and left ventricular hypertrophy after coarctation repair might impair left ventricular function. METHODS: Biplane angiography and simultaneous high fidelity pressure measurements were performed in 12 patients 3 to 12 years postoperatively (residual pressure gradient 4 mm Hg). Eight patients were normotensive and four had borderline hypertension. Data at rest and after nitroprusside infusion (1.7 micrograms/kg per min) were evaluated and compared with data from 12 control subjects. RESULTS: Systolic left ventricular function (ejection fraction-end-systolic wall stress relation) was normal in all patients. However, left ventricular muscle mass (113 vs. 86 g/m2), right atrial pressure (5.2 vs. 1.9 mm Hg) and left ventricular end-diastolic pressure (16 vs. 11 mm Hg) were significantly higher in patients than in control subjects. There was a linear relation between muscle mass and left ventricular end-diastolic (r = 0.66, p < 0.001) or right atrial (r = 0.60, p < 0.01) pressure. Left ventricular relaxation and myocardial stiffness were normal. However, there was an upward shift of the diastolic pressure-volume curve when compared with control values, but this shift was reversed by the administration of nitroprusside. CONCLUSIONS: Systolic function is normal late after coarctation repair. However, diastolic function can be abnormal with an upward shift of the diastolic pressure-volume curve that is reversed by nitroprusside administration and is probably due to residual left ventricular hypertrophy.