[Medical care of adults with congenital heart diseases : Present and future]. / Medizinische Versorgung von Erwachsenen mit angeborenen Herzfehlern : Gegenwart und Zukunft.

Congenital heart diseases (CHD) are the most common types of congenital organ defects. Thanks to medical progress in congenital cardiology and heart surgery, most children with CHD reach adulthood. Despite primarily successful treatment residual and subsequent conditions as well as (non)cardiac comorbidities can influence the chronic course of the disease and lead to a higher morbidity and mortality. Adults with congenital heart disease (ACHD) in Germany are not tied to the healthcare structure despite the great need for aftercare. According to the results of the medical care of ACHD (MC-ACHD) study, ACHD centers and specialists in Germany are insufficiently perceived despite increased complication rates and the great need for specialist guidance. General practitioners and patients are not adequately informed about existing ACHD facilities. A better awareness of the ACHD problem should be created at the level of primary medical supply in order to optimize care and to reduce morbidity and mortality. Improved future-oriented patient care includes lifelong regular follow-up and the possibility of interdisciplinary, integrated medical care of CHD.

Evaluation of contraceptive methods in women with congenital heart disease in Germany, Hungary and Japan.

AIMS: For women with congenital heart defects (CHD), pregnancy may pose a health risk. Sexually active women with CHD without the desire for own children or for whom pregnancy would imply considerable health risks require adequate counselling regarding appropriate contraception. This study gathers data on the contraceptive behaviour of women with CHD from three different cultural regions. METHODS AND RESULTS: 634 women with CHD from Germany, Hungary and Japan were surveyed regarding contraception and contraceptive methods (CM) used. The patients were divided into groups according to different criteria such as pregnancy associated cardiovascular risk or "safety" of the contraceptive methods used. 59% of the study participants had already gained experience with CM. The average age at the first time of use was 18.4 years; the German patients were significantly younger at the first time of using a CM than those from Hungary and Japan. Overall the condom was the method used the most (38%), followed by oral contraceptives (30%) and coitus interruptus (11%). The range of CM used in Japan was much smaller than that in Germany or Hungary. Unsafe contraceptives were currently, or had previously been used, by 29% of the surveyed patients (Germany: 25%, Hungary: 37%, Japan: 32%). CONCLUSION: Most women with CHD use CM. There are differences between the participating countries. Adequate contraceptive counselling of women with CHD requires considering the individual characteristics of each patient, including potential contraindications. For choosing an appropriate CM, both the methods' "safety", as well as the maternal cardiovascular risk, are important.

Women with congenital heart disease: long-term outcomes after pregnancy.

BACKGROUND: Pregnancy in women with congenital cardiac disease is more frequent due to an increased lifespan and improved health situations. However, the long-term outcomes in these women are not known. METHODS: We analysed 267 consecutive pregnant women with congenital heart defects who were seen at the German Heart Centre Berlin. This retrospective study included analysis of long-term follow-up data after pregnancy and standard maternal cardiac, obstetric and neonatal outcomes. The long-term data (n = 103) were acquired with a self-assessment questionnaire from each patient. The main primary outcomes of the study included functional class, health, work capability and physical activity. RESULTS: The median age of the patients at delivery was 27 years (range 17-43 years). The median follow-up of all patients was 11 years (range 1-49 years). Twenty-four percent exhibited complex cardiac defects. Primary long-term outcomes included good health in 61 % of the patients. Approximately 68 % worked, and 76 % engaged in physical activity. Thirty-three percent of the women who answered the questionnaire demonstrated a decrease in functional class during pregnancy, but more than two-thirds of these patients subsequently improved. Secondary short-term outcomes included a 4 % miscarriage rate and a 4 % induced abortion rate. The maternal cardiac data revealed that 30 % of the patients lost at least one functional class during pregnancy. Onset arrhythmias were observed in 12 % of the patients. The most prevalent neonatal complication was premature birth, which was present in 12 % of the neonates. CONCLUSION: Two-thirds of the patients tolerated pregnancy without cardiovascular complications. Most patients displayed good long-term health, work capability and physical activity outcomes. Further prospective controlled studies are necessary to confirm these results and safely advise pregnant women.

Comparison of cardiac output measurement using the CardioQP oesophageal Doppler with cardiac output measurement using thermodilution technique in children during heart catheterisation.

The minimally invasive CardioQP oesophageal Doppler probe estimates cardiac output by measuring blood flow velocity in the descending aorta. Individual variables to enter are patient's age, weight and height. We measured cardiac output simultaneously with CardioQP and pulmonary artery catheter thermodilution techniques during heart catheterisation in 40 paediatric patients with congenital heart defects. Median [range] age was 8.2 years [0.5-16.7 years], cardiac output values measured by thermodilution and CardioQP were 3.6 l.min(-1) [1.2-7.1 l.min(-1)] and 3.0 l.min(-1) [0.7-6.7 l.min(-1)], respectively. These values showed only moderate correlation (r = 0.809; p < 0.0001). Bias and precision were 0.66 l.min(-1) and 1.79 l.min(-1) (95% limits of agreement: -1.13 to +2.45 l.min(-1)). Based on our preliminary experience, cardiac output values measured by CardioQP in children do not reliably represent cardiac output values compared with the thermodilution technique. We suggest measurement of individual aortic diameter to improve performance of the CardioQP.

Self-fabricated fenestrated Amplatzer occluders for transcatheter closure of atrial septal defect in patients with left ventricular restriction: midterm results.

To avoid left ventricular failure after transcatheter closure of atrial septal defects in elderly patients with restrictive left ventricular physiology, partial occlusion by fenestrated devices may be an option. If complete defect closure is not possible in these patients, significant reduction of left to right shunting usually results in clinical benefit. We report two patients in whom deterioration of left ventricular function could be avoided by implantation of self-fabricated fenestrated Amplatzer Septal Occluders (ASO) in patients with ongoing restrictive left ventricular physiology. We describe technical preparation of the standard occluder, the specific implantation technique, and the initial and the intermediate term results up to 24 months.

Initial experience with bosentan (Tracleer) as treatment for pulmonary arterial hypertension (PAH) due to congenital heart disease in infants and young children.

INTRODUCTION: Bosentan, a dual endothelin-receptor antagonist, has been shown to be an effective treatment option in patients with the idiopathic form of pulmonary arterial hypertension (PAH). We used bosentan as compassionate treatment in infants and young children with congenital heart disease (CHD) who had a) PAH preoperatively representing a contraindication to corrective surgery or b) persisting PAH after corrective surgery causing right heart failure and reduced exercise tolerance. METHODS: Seven children with PAH due to CHD (median age 3.8 years; range 1.5 to 6.4 years) received 3 mg/kg/d bosentan (Tracleer) orally. Clinical, echocardiographic and hemodynamic parameters were measured and laboratory tests performed before treatment and during steady state while on treatment. Routine liver function parameters were monitored monthly. RESULTS: Mean bosentan treatment time was 8.6+/-5 months. During bosentan therapy there were no significant adverse events. The clinical status remained stable or improved in all patients: NYHA class decreased from 2.6+/-0.6 to 1.7+/-0.6 (p<0.05). This was associated with a mean reduction of the right ventricular systolic pressure (RVSP) from 96+/-11 mmHg to 71+/-26 mmHg (p<0.05). CONCLUSIONS: Treatment with bosentan in infants and young children with PAH due to congenital heart disease was tolerated without significant side effects and resulted in stabilization of clinical status. A significant reduction in right ventricular systolic pressure (RVSP) could be demonstrated. These results suggest that the dose regimen used is appropriate and safe for the treatment of infants and children with PAH, resulting in a reduction of pathologically increased pulmonary vascular resistance.

The CP stent--short, long, covered--for the treatment of aortic coarctation, stenosis of pulmonary arteries and caval veins, and Fontan anastomosis in children and adults: an evaluation of 60 stents in 53 patients.

OBJECTIVE: To evaluate the feasibility and usefulness of the Cheatham platinum (CP) stent in a broad spectrum of lesions. METHODS: Retrospective analysis of 60 implanted CP stents (11-80 mm lengths, 12 covered) between September 2001 and March 2004. PATIENTS: 53 patients aged 2.5-68 years (median 17 years). Body weight ranged from 12-95 kg (median 52 kg). Thirty six patients had aortic (re)coarctation; seven of them had functionally interrupted aortic arches. Thirteen patients had pulmonary artery stenosis and four had stenosis of caval veins or conduits in a total cavopulmonary connection (TCPC). RESULTS: Arterial pressure gradients dropped from 33 mm Hg (range 20-80 mm Hg) to 5 mm Hg (range 0-10 mm Hg) and pressure gradients in TCPC or caval veins dropped from 4 mm Hg (range 4-20 mm Hg) to 0 mm Hg (range 0-3 mm Hg). All stents were placed in the target lesion without complications. Three stent fractures without clinical instability were noted. CONCLUSIONS: The CP stent is suitable for the treatment of vessel stenosis in congenital heart diseases from childhood to adulthood. Whether these good results will be stable in the long term needs to be investigated.

Left ventricular conditioning in the elderly patient to prevent congestive heart failure after transcatheter closure of atrial septal defect.

Transcatheter closure of atrial septal defects (ASDs) is a safe and effective treatment. Over the past years, an increasing number of elderly patients (age > 60 years) have been admitted for transcatheter closure to prevent ongoing congestive heart failure from volume overload. However, recent data point to the risk of serious acute left ventricular dysfunction leading to pulmonary edema immediately after surgical or transcatheter ASD closure in some patients. In this study, we used a technique described before to recognize in advance patients at risk of left heart failure after ASD closure. Those patients at risk were then treated with preventive conditioning medication for 48-72 hr before definitive transcatheter ASD closure was performed. Fifty-nine patients aged over 60 years (range, 60-81.8 years; median, 68 years) were admitted to our institution for transcatheter closure of an atrial septal defect. All patients received evaluation of atrial pressures before and during temporary balloon occlusion of the ASD. Patients with left ventricular restriction due to increased mean atrial pressures (> 10 mm Hg) during ASD occlusion received anticongestive conditioning medication with i.v. dopamine, milrinone, and furosemide for 48-72 hr before definitive ASD closure with an Amplatzer septal occluder was performed. In 44 patients without any signs of left ventricular restriction, ASD closure was performed within the first session. Fifteen (25%) out of 59 patients showed left ventricular restriction. In the majority of patients with LV restriction, the mean left atrial pressures with occluded ASD were significantly decreased after 48-72 hr of conditioning medication. Definitive ASD closure was then performed in a second session. Only two patients received a fenestrated 32 mm Amplatzer occluder due to persistent increased atrial pressures > 10 mm Hg even after conditioning medication. There were no significant differences in shunt, device size, or defect size between the two groups. Balloon occlusion of atrial septal defects identifies patients with left ventricular restrictive physiology before ASD closure. Intravenous anticongestive conditioning medication seems to be highly effective in preventing congestive heart failure after interventional closure of an ASD in the elderly patient with a restrictive left ventricle.

[Transcatheter closure of congenital ventricular septal defects]. / Interventioneller Verschluss angeborener Ventrikelseptumdefekte. Breitere Indikationsstellung dank neuer Implantate.

We report on the transcatheter closure of ventricular septal defects (VSD) in 26 patients with Amplatzer Occluders and Nit- Occlud Coil Systems. Twenty-one patients had a perimembranous and 5 patients a muscular VSD. Patients' age range was 5 months to 59 years (median 8 years) and their body weight 4.5 kg to 167 kg (median 28 kg). Defect diameters were 3-11 mm (median 5 mm). Sixteen patients had left ventricular volume overload and 7 patients pulmonary hypertension (median 50% of systemic pressure). Seven patients suffered from trivial or mild aortic regurgitation. Twenty-eight devices (4-12 mm; median 8 mm) were implanted (16 Amplatzer, 12 Nit-Occlud) through sheaths of 4F to 9F (median 7F). Fluoroscopy times were 8.3- 56.5 min (median 26.2 min). One coil was surgically explanted directly after intervention. One patient needed pulmonary banding due to additional VSDs. After a follow-up of 7 months (1-12 months), 2 patients had a small and 9 a minimal residual shunt. Thirteen defects were completely closed. Transcatheter closure of VSDs with new devices seems to be a promising therapy for suitable defects in different hemodynamic conditions in patients of every age.

[Ebstein's anomaly: long-term results after modified reconstruction of tricuspid valve without ventricle plication]. / Ebstein-Anomalie: Langzeitverlauf nach Trikuspidalklappenrekonstruktion ohne Ventrikelplikatur.

Ebstein's anomaly is a rare congenital heart defect in which the hinges of the septal and/or posterior leaflets are displaced downward to the right ventricle. The anterior leaflet is usually not displaced but is enlarged and sail-like and valve closure is likewise displaced downwards. Since 1988 we have operated on 22 patients with Ebstein's anomaly using a modified repair technique of the tricuspid valve. This technique restructures the valve mechanism at the level of the true tricuspid annulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. We evaluated our long-term results with regard to functional capacity (New York Heart Association functional class), tricuspid valve function, rhythm disturbances and re-operation rate. We quantified the right ventricular function by measuring flow velocity integral of the pulmonary artery (VTIPA). All patients survived the operation. There were two hospital deaths (9%) and the late mortality was 4.5%. The mean followup period was 9 years (range, 1.5 to 13 years) for 19 patients. So far no re-operation has been necessary. Preoperatively, the majority of all patients were in NYHA classes III and IV (79%). After the first postoperative follow-up examination (2.9 months), 17 patients were in NYHA class II. Long-term follow-up examinations showed an additional improvement of 11 patients to NYHA class I. Echocardiographic studies demonstrated a significant improvement of tricuspid valve function. No tricuspid valve stenosis was observed. Significant improvement of VTI(PA) was observed. Analysis of the postoperative deaths demonstrated that all patients were in NYHA class III or IV and had a cardiothoracic ratio of 0.65 or more. A severe reduction in functional capacity seems to be an additional risk factor for mortality beside a cardiothoracic ratio greater than 0.65. We conclude that reconstruction of the tricuspid valve without ventricle plication not only achieves good functional results immediately after the operation but that follow-up examinations demonstrate stable or improved functional capacity in the long term. We postulate that incorporation of the atrialized chamber into the right ventricle may contribute to right ventricular contraction and thereby account for the improved functional capacity of the patients.

[Stent implantation as therapy of first choice in adults with coarctation]. / Stentimplantation als Therapie der ersten Wahl bei Erwachsenen mit Aortenisthmusstenose?

Stent implantation for coarctation of the aorta is an alternative to surgery or balloon dilation. We report our results in 12 patients with a median age of 22 years (10 to 28 years) and a body weight of 60 kg (32 to 97 kg). Nine patients had native stenosis and three had recoarctation after surgery. Invasively measured systolic pressure gradients ranged from 20 to 100 mmHg. Nine patients suffered from brachiocephalic hypertension. Eleven implantations were successful with a median dilatation of 17 mm (15-25 mm). Residual gradients were 0-5 mmHg in seven patients, 5-10 mmHg in three and 15 mmHg in one patient with postoperative recoarctation. Twenty-one months (2-37 months) after intervention, no hemodynamically relevant intimal proliferations, no restenosis, and no aneurysms were present. Thus, stent implantation is a very promising therapy for coarctation of the aorta in adults and is on its way to becoming the therapy of first choice.

[Acute left heart failure after interventional occlusion of an atrial septal defect]. / Akute Linksherzinsuffizienz nach interventionellem Verschluss eines Vorhofseptumdefekts.

Congestive left ventricular failure after surgical closure of an atrial septal defect (ASD) has been repeatedly reported, particularly in the elderly. We present a case of left ventricular failure after a successful transcatheter closure of an ASD, which to our knowledge has not been described before. In a 78-year-old woman (50 kg, 160 cm) with well-preserved left ventricular function (ejection fraction 65%) and without coronary artery disease or arterial hypertension, an ASD (Qp/Qs 1.6:1) was closed with an Amplatzer Septal Occluder without a residual shunt. Two hours after the procedure, she developed pulmonary edema due to left ventricular failure (increase of end-diastolic diameter from 42 mm to 54 mm, ejection fraction 20%), had to be mechanically ventilated for 24 hours and needed catecholamines for 4 days. High doses of diuretics were supplied until the ejection fraction normalized (32%). The patient could not be discharged until two weeks after intervention. A reduced preload for decades may predispose acute left ventricular failure, particularly in the elderly with compromised ventricular compliance.

Early biochemical indicators of hypoxic-ischemic encephalopathy after birth asphyxia.

Hypoxic-ischemic encephalopathy (HIE) after perinatal asphyxia is a condition in which serum concentrations of brain-specific biochemical markers may be elevated. Neuroprotective interventions in asphyxiated newborns require early indicators of brain damage to initiate therapy. We examined brain-specific creatine kinase (CK-BB), protein S-100, and neuron-specific enolase in cord blood and 2, 6, 12, and 24 h after birth in 29 asphyxiated and 20 control infants. At 2 h after birth, median (quartiles) serum CK-BB concentration was 10.0 U/L (6.0-13.0 U/L) in control infants, 16.0 U/L (13.0-23.5 U/L) in infants with no or mild HIE, and 46.5 U/L (21.4-83.0 U/L) in infants with moderate or severe HIE. Serum protein S-100 was 1.6 microg/L (1.4-2.5 microg/L) in control infants, 2.9 microg/L (1.8-4.7 microg/L) in asphyxiated infants with no or mild HIE, and 17.0 microg/L (3.2-34.1 microg/L) in infants with moderate or severe HIE 2 h after birth. No significant difference was detectable in serum neuron-specific enolase between infants with no or mild and moderate or severe HIE 2 and 6 h after birth. A combination of serum protein S-100 (cutoff value, 8.5 microg/L) and CK-BB (cutoff value, 18.8 U/L) 2 h after birth had the highest predictive value (83%) and specificity (95%) of predicting moderate and severe HIE. Cord blood pH (cutoff value, <6.9) and cord blood base deficit (cutoff value, >17 mM) increase the predictive values of protein S-100 and CK-BB. We conclude that elevated serum concentrations of protein S-100 and CK-BB reliably indicate moderate and severe HIE as early as 2 h after birth.

Masked left ventricular restriction in elderly patients with atrial septal defects: a contraindication for closure?

The impact of an atrial septal defect in the elderly with reduced diastolic elasticity of the left ventricle is unclear. We studied the hemodynamic changes during balloon occlusion of atrial septal defects in patients over 60 years of age. In 18 patients (61-78 years old; median, 70), the left atrial pressure and the mitral valve inflow was measured during complete balloon occlusion of the defect and after deflation of the balloon. In seven patients, the left atrial pressure and the E/A ratio of the mitral valve inflow increased markedly (P = 0.02). Mean atrial pressures reached values of 27 mm Hg and the v-wave peak values of 55 mm Hg. Two patients received a transcatheter device closure and developed congestive heart failure. In the elderly, an atrial septal defect can have a decompressive impact on the left ventricle. Therefore, caution appears to be warranted if atrial septal closure is planned.

Late-diastolic forward flow in the aorta induced by left atrial contraction.

Late-diastolic forward flow is a well-described phenomenon detectable by Doppler echocardiography in the pulmonary trunk. It is supported by a restrictive right ventricular diastolic function and by a low end-diastolic pulmonary artery pressure. A similar phenomenon for the left ventricle and the aorta has not been described. We report a case of a preterm infant with aortic stenosis and endocardial fibroelastosis, who underwent balloon valvuloplasty. Restrictive left ventricular diastolic filling led to high left atrial pressure (27 mm Hg) and a very pathologic ratio of early-to-late peak velocities (2.6) for an infant of 29 weeks' gestation. In combination with a low diastolic aortic pressure (24 mm Hg) caused by moderate aortic regurgitation after intervention, a late-diastolic forward flow was detectable in the aorta during left atrial contraction with pulsed Doppler echocardiography.

[Pre- and postoperative evaluation of ventricular function, muscle mass and valve morphology by magnetic resonance tomography in Ebstein's anomaly]. / Prä- und postoperative Beurteilung der ventrikulären Funktion, Muskelmasse und Klappenmorphologie bei der Ebstein-Anomalie mit der Magnetresonanztomographie.

PURPOSE: To evaluate the value of MRT with spin echo (SE) and CINE gradient echo (GE) sequences for the pre- and postoperative assessment of patients with Ebstein's anomaly. METHODS: Twelve patients within the ages of four to 49 years (mean 22 +/- 12 years) were examined pre- (n = 5) or postoperatively (n = 7) after tricuspid valve reconstruction with a 1.5 T scanner. For the anatomical assessment, an ECG-gated transverse SE-sequence, for the assessment of valve morphology and function as well as for volumetry a CINE GE-sequence with retrospective gating was used. With the use of the multislice-multiphase technique, after summing up the manually outlined epi- and endocardial areas, endsystolic (ESV) and enddiastolic volumes (EDV), ejection fraction (EF), stroke volume (SV), and muscle mass (MM) were calculated for both ventricles. RESULTS: The differentiation of the displaced parts of the tricuspid valve (TV) was insufficient with static SE, but was possible in all patients with CINE-MRT. Like in Doppler echocardiography, a qualitative assessment of tricuspid insufficiency was possible in CINE-MRT, the mean incompetence grade preoperative was 1.8 (+/- 0.8), postoperative 0.7 (+/- 0.5). The mean RV-EF in the preoperative group was 41.8% (+/- 6.4), in the postoperative group 47.9% (+/- 10.6), the mean LV-EF preoperative 47.4% (+/- 8.5%), postoperative 63.0% (+/- 9.4). CONCLUSION: CINE-MRT should rather be used than SE for the assessment of valve morphology. EF, muscle mass and tricuspid incompetence can also be calculated pre- and postoperative with CINE-MRT.

3-D ultrasound quantification of neonatal cerebral ventricles in different head positions.

We determined the influence of head position on lateral ventricular cerebral volume in low-birth-weight infants by three-dimensional (3-D) ultrasound (US). Thirty-nine neonates were examined prospectively in a controlled and blinded study. We used a freehand 3-D US system to acquire data sets after head positioning for 3 h on left and right side in random order. The borders of the lateral ventricles were marked in stored cross-sections. Volumes were calculated as mean of duplicate measurements. Median volume of lateral cerebral ventricles was 1.03 (quartiles 0.78-1.36) mL. Median left ventricular volume was slightly larger than right one (p = 0.13). Down-side lateral ventricles showed smaller volumes than up-side positioned ventricles (p < 0.01). Freehand 3-D US allows quantification of small volumes as neonatal lateral cerebral ventricles. Head position influences the lateral cerebral ventricle volume in low-birth-weight infants.

Continuous monitoring of cardiac output in neonates using an intra-aortic Doppler probe.

Continuous monitoring of cardiac output in neonates would be of considerable benefit but, as yet, there is no practical method to achieve this aim. We have now evaluated the feasibility of using an intra-aortic Doppler probe. We introduced a pulsed Doppler probe of 0.46 mm diameter via the umbilical artery in two term and four preterm neonates. Indications in all patients for umbilical arterial catheter is always an unstable cardiopulmonary state. Body weights were between 770 and 3340 g. Velocities of blood flow in the thoracic aorta were continuously recorded to estimate cardiac output on-line for 12 h. No complications were encountered. It proved possible to derive high-quality Doppler curves. The received Doppler signal was stable but it proved sensitive to pathophysiologic changes in flow. Mean velocity of flow in the descending aorta was 16.4 cm/s (range 13.3-19.0 cm/s). We quantified flow by multiplying the mean velocity of the flow by the cross-sectional area of the descending aorta. Calculated mean flow was 135 ml/kg/min (range 111-179 ml/kg/min). These values are consistent with those measured by transcutaneous Doppler, and it should not be raised by left-to-right ductal shunts. This pilot study proved the feasibility of continuous monitoring of cardiac output. The technique should prove of great value in those infants with unstable circulatory conditions, and can be used even in infants with extremely low birth weights.