O. N. Krogmann

Evaluation of low dose prostaglandin E1 treatment for ductus dependent congenital heart disease

This study reports our experience with low-dose prostaglandin E1 (PGE1) treatment of 91 newborns with ductus dependent congenital heart disease (CHD). PGE1 efficacy, side-effects as well as the cardiovascular and respiratory profile of the patients were analysed. PGE1 doses > 0.02 μg/kg per minute were used for only 5.3% of the total 23 656 h of treatment. The mean systolic blood pressures did not differ from the normal mean for patients with cyanotic CHD, while the diastolic values were lowered. Respiratory support was required only during 13.7% of the total treatment time. Apnoeas occurred in 21 (38%) of the 55 spontaneously breathing infants, who all had a cyanotic CHD. The incidence of apnoeas was lower during treatment with doses < 0.01 μg/kg per minute.

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.

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 Simpsons rule and corrected with Langes 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.

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 r2 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.

Intermediate-term clinical and hemodynamic results of the neonatal arterial switch operation for complete transposition of the great arteries

We prospectively evaluated 49 consecutive hospital survivors of the arterial switch operation for complete transposition and intact ventricular septum by clinical examination, echocardiography, cardiac catheterization, 12-lead and 24-h Holter ECG. The mean length of follow-up was 40 +/- 18 months. Forty-six children are clinically asymptomatic without medication, 2 died due to coronary related left ventricular dysfunction 3 and 12 months after surgery, and 1 required reoperation because of severe bilateral pulmonary branch stenoses. Except for this case, cardiac catheterization (n = 23) revealed a mean gradient of only 17 +/- 8 mmHg between the right ventricle and distal pulmonary arteries. Left ventricular end-diastolic volume was within normal limits except for 2 cases with volumes slightly below normal, the mean ejection fraction was 78 +/- 5%, and end-diastolic and end-systolic ventricular shapes were normal. The mean cardiac index was 4.14 +/- 0.69 l/min/m2. Left ventricular end-systolic wall stress to velocity of fiber shortening relation was normal in all cases examined (n = 15), indicating normal myocardial contractility. Significant neoaortic valve insufficiency was never observed despite considerably enlarged aortic roots. Twenty-four-hour Holter ECG records (n = 46) provided no evidence of serious atrial arrhythmias, especially sinus node dysfunction. These encouraging intermediate-term results make the arterial switch operation the treatment of choice at present, for neonates with simple transposition.

Left ventricular thrombus in a 2-year-old boy with cardiomyopathy: Lysis with recombinant tissue-type plasminogen activator

A 2-year-old boy with cardiomyopathy and clinical signs of cardiac failure presented with an echodense structure in the left ventricle. This structure was seen from different echocardiographic views adjacent to a hypokinetic area of the apex and lateral free wall. It was different in texture and motion from the underlying myocardium and thus met the diagnostic criteria of a left ventricular thrombus. This thrombus protruded into the cavum and was partly mobile. In view of a high embolic risk, thrombolytic therapy with recombinant tissue plasminogen activator was started. The thrombus resolved within 72 h without any embolic or bleeding complications. No recurrence of the thrombus was observed during a 3-month follow up period.

Cardiac rhythm after Mustard repair and after arterial switch operation for complete transposition

We compared the prevalence of arrhythmias among the first consecutive 45 patients with complete transposition (concordant atrioventricular and discordant ventriculo-arterial connexions) after arterial switch operation and the last 47 patients after Mustard repair in infancy. Both groups had 24-hour Holter electrocardiographic studies at similar periods of follow up (24 +/- 14 and 25 +/- 18 months). A second group of patients undergoing the Mustard procedure had been repaired at an older age before 1981. They were studied to determine the frequency of disturbances of rhythm during later postoperative follow-up (85 +/- 24 months). Symptomatic brady-/tachyarrhythmia syndrome never occurred after the arterial switch and only once in the group of patients repaired by the Mustard procedure in infancy, but developed at a late stage (69 +/- 28 months); five times in the group of patients having Mustards repair at an older age. In addition, Holter monitoring did not detect bradyarrhythmias indicating sinus node dysfunction in a single patient after the arterial switch, but did so to a similar extent in both groups having the Mustard procedure (recent: n = 14; older: n = 18). Three cases of the group of older patients undergoing a Mustard operation developed complete atrioventricular block during follow-up. Normal findings were present in 93% of the cases after arterial switch, but in only 51% of the cases with a similar follow-up repaired by the Mustard procedure, and in 29% of the group having the Mustard repair at an older age.(ABSTRACT TRUNCATED AT 250 WORDS)

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.

Complete AV-block in two children with immunohistological proven myocarditis

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.

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.