Spyros Rammos

Birth weight of children with congenital heart disease

Birth weights of 843 children with congenital heart disease (CHD) were compared to the respective data of a normal West German population. On average, the CHD group had significantly lower birth weights, but the weight deficit was far less pronounced than in previous studies. The decrease in birth weight was distinct only in children with tetralogy of Fallot and atrial septal defect. Compared to normal newborns, patients with CHD were more often small for gestational age (15.0%) or had a low (<2500 g) birth weight (8.6%). The prevalence of prematurity was not increased. Extracardiac malformations were not significantly more common in CHD patients, who were small for gestational age, than in CHD patients with normal birth weight.

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.

Transcatheter closure of coronary arterial fistulas using the new Amplatzer vascular plug.

We report our initial experience in using the Amplatzer vascular plug for closure of coronary arterial fistulas. The self-expanding, cylindrical, device is made from Nitinol wire mesh, and is available from 4 to 16 millimetres in diameter. We have now used the device to close fistulas in 3 patients, aged from 3 to 14 years, who presented with ratios of pulmonary-to-systemic flow from 1.5 to 3. In 2 patients, fistulas arising from the proximal right and left coronary arteries, with maximal diameters of 9 and 10 millimetres, respectively, had their narrowest diameter, of 6 millimetres, proximal to the entrance into the right atrium via a saccular aneurysm. The third fistula, with a maximal diameter of 16 millimetres, and with its origin from the circumflex coronary artery, entered the right atrium with nearly unrestricted flow, its narrowest diameter being 8 millimetres. For interventional closure, we chose plugs twice the diameter of the narrowest segment of the fistula, thus using 2 devices of 12 millimetres and one of 16 millimetres diameter. An arteriovenous loop was established through the fistula by snaring an exchange guide wire. Using a 7 or 8 French guide catheter inserted through the femoral vein, all plugs were placed at the narrowest segment of the fistula, leading to immediate complete closure of 2 fistulas. The third patient, with a fistula of the circumflex coronary artery, who received the largest plug initially had residual flow, but the fistula was found to be completely occluded at 12 months follow-up examination. We have demonstrated, therefore, safe and effective usage of the new vascular plug for transcatheter closure of moderate- to large-sized coronary arterial fistulas. The plug offers an alternative to cardiac surgery, or occlusion using coils.

The abnormal pulmonary venous connexion: a developmental approach

If embryonic communications exist between the splanchnic and cardinal venous systems, then they can explain variations in partially or completely abnormal pulmonary venous return as seen in congenitally malformed hearts. With this in mind, we investigated complete serial sections of 52 human embryos, ranging from 4.4 to 25 mm crown-rump length (Streeters horizons XIII-XXII) and 57 mouse embryos (Mus musculus albus CPB-S) from 7.8 to 19.7 days of gestation. Specifically, we investigated whether communications persisted between the splanchnic, the pulmonary and the cardinal venous systems. In the early stages of development, the pulmonary vascular bed shared the route of drainage of the peripheral splanchnic plexus into the umbilical-vitelline and cardinal venous systems. As the pulmonary plexus developed, it obtained a new direct route of drainage into the left atrium via the pulmonary veins. A persistence of embryonic connexions was encountered in one human and 14 mouse embryos. In all these cases, the stage of development was such that the connexions between the splanchnic and pulmonary plexuses should have disappeared. An abnormality in the development of the central pulmonary venous system was also found in the human embryo. Whether this was primary or secondary to the persistence of the embryonic connexions remained unresolved.

The Amplatzer ® Membranous VSD Occluder and the vulnerability of the atrioventricular conduction system

Transcatheter closure of ventricular septal defects with the Amplatzer Membranous VSD Occluder has yielded promising initial results, but disturbances of conduction, including complete heart block, have been reported. We report our experience with the Amplatzer occluder in 35 patients with a median age 4.5 years, the defects being sized angiographically at 4.4 plus or minus 1.1 millimetres, with a range from 3 to 8 millimetres, and the size of the occluder varying from 4 to 12 millimetres. Over a median follow-up of 2.5 years, the rate of complete closure was 87% and 91%, at 1 and 2 years respectively, while 2 patients required surgical closure of the defect subsequent to the insertion of the device. Persistent regurgitation across the tricuspid valve related to the occluder was observed in 3 patients, and in 6 patients across the aortic valve. Abnormalities of conduction related to the procedure were noted in 7 patients, one-fifth of the cohort. The disturbances were transient in 1 patient, but permanent in 6, in one of the latter progressing after 6 months from left bundle branch block to intermittent Mobitz II second-degree atrioventricular block in association with expansion of the occluder. We conclude that transcatheter closure of perimembranous ventricular septal defects with the Amplatzer occluder is effective with limited complications, but the incidence of immediate and progressive disturbances of conduction related to the proximity of conduction tissues to the rims of the occluder stress the importance of larger and longer studies to assess the safety of this procedure.

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)

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.