[How does the time of diagnosis affect the course of disease in children with kawasaki syndrome? A retrospective analysis at one center]. / Welchen Einfluss haben unterschiedliche Diagnosezeiten auf den Krankheitsverlauf von Kindern mit Kawasaki-Syndrom? Eine retrospektive monoinstitutionelle Analyse.

BACKGROUND: Kawasaki syndrome was described for the first time by Tomisaku Kawasaki in 1967. This disease is characterized by panvasculitis of the small blood vessels of the skin, the mucous membranes, the internal organs and the coronary vessels and has an unclear etiology. Inflammatory changes in the coronary vessels or late diagnosis are prognostically unfavorable for the early and late mortality. AIM OF THE STUDY: Since two of our patients with Kawasaki syndrome with a short, severe course died despite receiving state-of-the-art treatment, we retrospectively evaluated the medical records of all the children we have treated since October 1978 with regard to the symptoms at the time of diagnosis, intervals between the onset of the disease, diagnosis, beginning of treatment and the result of treatment. PATIENTS: Kawasaki syndrome was diagnosed in 80 patients in the period from October 1978 to October 2007. The patients were grouped according to the phase of the disease and the number of organs affected at the time of diagnosis (Asai-Score) as well as the treatment carried out. The time of the first presentation for diagnosis by the pediatrician was also considered. METHOD: This is a single-institution retrospective analysis of the medical records, echocardiography and angiography findings of all patients. In view of the change of therapy in that year, patients who had been diagnosed before 1987 were compared with those diagnosed after 1987. RESULTS: Before 1987, the patients were treated solely with high doses of acetylsalicylic acid (50-100 mg/kg/day p.o. over two to four weeks). Out of a total of 36 patients, 13 showed involvement of the coronary arteries that persisted in seven patients despite treatment. After 1987, all patients received intravenous immunoglobulins (4 x 0.5 g/kg/day resp. 1 x 2 g/kg i.v. over 12 hours). In 18 out of 44 patients, the coronary arteries were affected at the time of diagnosis, but this did not persist in any of the patients. One child died in each group. Comparing the two treatment groups also revealed that a physician was consulted for the first time after a very much shorter duration of the disease in the second treatment period (3+/-1.8 vs. 6+/-2.4 days after onset of the illness) and that a pediatrician was consulted much more frequently as the first port of call. This was reflected in a significantly earlier beginning of treatment and a simultaneous significantly lower Asai score. CONCLUSION: The retrospective evaluation of all medical records did not reveal any plausible explanation for the fatal course of the disease in one child in each of the two treatment periods. Besides the combination therapy with intravenous immunoglobulin and oral administration of acetylsalicylic acid, the greater age and the earlier commencement of treatment appeared to be salient factors resulting in complete cure of the disease in the surviving patients in the second period of treatment.

High-dose intravenous immunoglobulin in recurrent postpericardiotomy syndrome.

Postpericardiotomy syndrome is a frequent complication following cardiac surgery. It is characterized by fever, chest pain, pericardial friction rub or pericardial effusion, and laboratory signs of inflammation. Treatment includes the use of nonsteroidal antiinflammatory agents, corticosteroids, and pericardial drainage. We describe a patient with chronic postpericardiotomy syndrome and steroid dependency who was successfully treated by single administration of high-dose intravenous immunoglobulin.

Disastrous course of recurrent infective endocarditis after tooth extraction in a young patient with bicuspid aortic valve.

In a patient with moderate malformations of the left-ventricular valves disregard of antibiotic prophylaxis after tooth extraction resulted in massive mitral- and aortic regurgitation. After surgical reconstruction a relapse produced an aorto left-ventricular fistula with septic embolizations and cardiac decompensation. Valve replacement with a homograft reconstituted the hemodynamic situation. During both episodes the Duke criteria for infective endocarditis supported the diagnosis.

[Spontaneous pneumomediastinum as a cause of chest pain]. / Spontanes Pneumomediastinum als Ursache für akuten Thoraxschmerz.

Two patients, both 16 years old, presented because of chest pain after extended sports activity. The thoracal X-ray showed trapped air in the mediastinum especially around the cervical vessels in one patient. In the thoracal X-ray of the other patient a vertical lucent streak along the left side of the heart, showing the pleura as a fine opaque line, was found. Both developed subcutaneous emphysema after a few hours, which led to the diagnosis spontaneous pneumomediastinum. The chest pain resolved under analgetic medication, and after resorption of the subcutaneous emphysema both patients recovered completely. The main differential diagnosis of the spontaneous pneumomediastinum is besides pericarditis and myocarditis, the spontaneous esophageal perforation (also called Boerhaave syndrome), with the high morbidity, it has to be ruled out consequently.

Pregnancy in congenital cardiac disease: an increasing challenge for cardiologists and obstetricians -- a prospective multicenter study.

OBJECTIVES: Aim of this study was to assess the occurrence of pregnancy-related complications of mother and child during pregnancy, delivery and puerperium in women with CCD prospectively. STUDY DESIGN, POPULATION: This prospective multicenter study included 122 pregnancies in 106 women with CCD (72 with, 34 without previous cardiac surgery). Patient age was 17-44, median 26 years. Cardiac and non-cardiac complications, mode of delivery, abortion, and CCD of the newborn were assessed. RESULTS: Initially all women were in Functional Class I or II. Worsening during pregnancy occurred in 25.5% (n=27), mainly during the second and third trimester. Significant problems due to bleeding, hypertension, rhythm disturbances, endocarditis, liver congestion, increasing cyanosis or death, occurred in 11.3%. Twelve per cent of deliveries were premature. Five women had therapeutic abortion, nine spontaneous abortions, nine preterm births, and one intrauterine death. Seventy-nine per cent (n=85) delivered spontaneously; 21.3% (n=23) had caesarean section. Of the 111 live born children, 5.4% (n=6) had a CCD. CONCLUSIONS: Most women with CCD and a good functional class before pregnancy tolerate pregnancy without major problems. However, pregnancy may induce serious cardiac and obstetric complications. The specific risks require an individualized multidisciplinary patient-management by experienced physicians.

[Interventional occlusion of foramen ovale and atrial septal defects after paradoxical embolism incidents]. / Interventioneller Verschluss von Foramen ovale und Vorhofseptumdefekten nach paradox embolischen Ereignissen.

Two hundred and fifty-one patients with a persistent foramen ovale (PFO), mean age 43.3 +/- 12.4 years, underwent catheter closure between 6/1995 and 6/2001. One hundred and forty-one had an ischemic stroke, 99 a transient ischemic attack (TIA) or prolonged reversible ischemic neurologic deficit, 5 peripheral arterial embolism, 4 suffered from decompression sickness after diving and 2 had transient global amnesia. Fifty-nine of them had multiple events in spite of antiplatelet or anticoagulant therapy. The patients received five different devices: 13 Rashkind Occluders, 20 Amplatzer septal Occluders, 109 Amplatzer PFO-Occluders, 73 CardioSEAL and 36 STAR-Flex devices. Time of fluoroscopy was 8.3 +/- 4.5 min. In three patients a device embolized and had to be removed from the groin vessels. We saw five inguinal or retroperitoneal venous hematomas with the need for operation in one patient. One early and one late perforation of the left atrium caused by a guide wire and a left-atrial disc, respectively, also needed surgery. Fourteen patients had documented late arrhythmias. Six patients with atrial fibrillation needed drugs or cardioversion while the other patients with runs of supraventricular tachycardia, atrial flutter and multiple extrasystoles needed no therapy. On transesophageal echocardiography (TEE) 6 months after implantation we found four significant residual leaks. These patients had the defect closed with a second device. In addition a secundum atrial septal defect (ASD) was closed in 17 patients (mean age 38 +/- 10.5 years) with Amplatzer septal Occluders (12) and CardioSEAL devices (5). These patients had experienced eight strokes and nine TIAs, 3 of them had had multiple events. Two of these patients had a significant residual defect and one had atrial flutter following the procedure. Two hundred and two PFO-patients and 12 ASD patients were followed for 6-62 (24.6 +/- 14.2) months; 2 died due to a traffic accident and a myocardial infarction, respectively. Four patients had another neurologic event following PFO-closure. We now overlook 210 patients with 348.6 symptom-free patient years and have a 1-year recurrence rate of neurologic events of 1.9%. Catheter closure of the PFO and atrial septal defect is a simple, effective and quick method which ensures a high closure rate, avoids life-long anticoagulation and has a low recurrence rate of neurologic events.

[Interventional occlusion of interatrial communication after modified Fontan operation]. / Interventioneller Verschluss von interatrialen Verbindungen nach modifizierter Fontanoperation.

UNLABELLED: After modified Fontan operations various communications between the systemic and pulmonary venous returns may cause persistent or increasing postoperative cyanosis. Interventional closure of these right-to-left shunts may be necessary to eliminate hypoxemia and to reduce the risk of paradoxical embolic complications. PATIENTS AND METHODS: Eighteen patients with a mean age of 5.6 +/- 4.1 (2.5-17.5) years underwent interventional closure of a right-to-left shunt 17.4 +/- 15.8 (3-60) months after a modified Fontan operation. After test balloon occlusion fenestrations were closed in 13 patients using an Amplatzer Septal occluder (n = 7), a Rashkind PDA occluder (n = 3), a CardioSeal umbrella (n = 1) and detachable coils (n = 2). Residual leaks at the suture lines between the interatrial patch and the right atrial wall were closed using detachable coils and a Rashkind PDA occluder in 2 and 1 patients, respectively. In 3 patients intracardiac venous collateral channels were closed by means of detachable coils. RESULTS: The mean aortic oxygen saturation increased from 85 +/- 4.5 (70-89)% to 91.4 +/- 2.8 (83-95)% (p < 0.001) breathing room air and the mean tunnel pressure rose from 10.7 +/- 1.8 (6-14) mmHg to 12.1 +/- 2.4 (6-16) mmHg (p < 0.001). Calculated Qs decreased from 5.15 +/- 2.1 (2.1-11.3) l/min/m2 to 3.6 +/- 1.0 (1.8-5.6) l/min/m2 (p < 0.001). Mixed venous saturation (66.4 +/- 7.4% vs 65 +/- 7%) and mean systemic arterial pressure (73 +/- 8 mmHg vs 73 +/- 9 mmHg) remained unchanged. In one patient an additional leak of the tunnel could not be closed because of an increase to more than 18 mmHg of the mean pressure in the lateral tunnel during balloon test occlusion. In 2 patients residual leaks after umbrella and coil occlusion of a fenestration and an additional venous collateral channel were closed by means of coils after 16 and 21 months, respectively. At a follow-up of 42 +/- 23 (7-99) months, mean oxygen saturation measured by pulse oxymetry was 93 +/- 2 (90-97)%. In 2 patients color-coded Doppler echocardiography revealed a minimal residual right-to-left shunt. In 2 patients contrast echocardiography demonstrated the additional presence of intrapulmonary fistulas. All patients remained free from device migration, thromboembolic events and hemolysis. CONCLUSION: After modified Fontan operations various right-to-left shunts between the systemic and pulmonary venous returns can be successfully closed using umbrella devices or coils to eliminate cyanosis and to reduce the risk of paradoxical embolism.

Catheter closure of the persistent foramen ovale: mid-term results in 162 patients.

Four different types of occluder systems were used to close a persistent foramen ovale (PFO) in 162 patients with paradoxical embolic events. Ninety-eight patients had ischemic stroke, 60 transient ischemic attacks (TIA) or prolonged reversible ischemic neurological deficit (PRIND), and 4 had peripheral arterial embolism. The age of the patients was 40.2 +/- 11.9 years and the ischemic event had happened 7 +/- 10 months before device closure. CardioSeal and Amplatzer occluders were the most commonly used devices (73 and 77 cases, respectively). Implantations were successful in all patients. Serious catheter-related complications included two device embolizations and two venous bleedings. Six patients had documented supraventricular arrhythmias within the first month after implantation, which disappeared spontaneously within some weeks without therapy in three patients; the other three patients with atrial fibrillation needed conversion to sinus rhythm. Residual leaks were found in 5 out of 116 patients who had been followed by transesophageal echocardiography (TEE) and one leak was closed by a second device. During a follow-up period of 19.4 +/- 16.2 months per patient, TIA and PRIND occurred in 3 of 116 patients. Interventional closure of PFO is a simple, effective and quick method that is superior to surgery and avoids the problems of life-long anticoagulation.

Atypical Bland-White-Garland syndrome with stenosis of the origin of the left coronary artery: catheter intervention after mammary artery bypass stenosis and residual fistula to the pulmonary trunk.

A 16-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery, Bland-White-Garland syndrome, underwent a mammary artery bypass grafting to the left coronary artery (LCA) together with closure of the stenosed origin of the left coronary artery. A residual LCA to pulmonary artery fistula and stenosis of the shunt at the implantation site developed that resulted in a dominant perfusion of the LCA from the right coronary artery. Interventional catheterization was performed with stenting of the LCA mammary artery anastomosis and subsequent coil occlusion of the residual fistula. After this intervention the LCA was exclusively perfused by the mammarian bypass with no residual leak to the pulmonary artery.

The influence of different strategies on clinical outcome in patients undergoing total cavopulmonary connection.

BACKGROUND: We report on results of a prospective clinical trial designed to demonstrate the influence of various strategies in "Total Cavopulmonary Connection" (TCPC) for palliative therapy of patients with "single ventricle" physiology. METHODS: From 1989 to 1997, a total of 47 patients (mean age 4.8 +/- 3.6 years) underwent definitive TCPC at our unit. 31 patients (66%) underwent one-stage TCPC, in 16 patients (34%) we performed a two-stage modified Fontan operation; 21 patients had central fenestration (4 mm). Inhalative NO therapy in the immediate postoperative period was adopted in 1993. RESULTS: Overall 5-year survival was 76.4%, after two-stage TCPC 87.5%, and 81.3% in patients undergoing fenestrated procedures. Two of three patients survived perioperative Fontan take-down. We lost 11 patients (nine early and two late deaths): three patients died primarily because of neurologic dysfunction and eight patients because of cardiac failures. Under perioperative NO therapy there was no early death. After a mean follow-up of 35.9 +/- 23.3 months, 76% of all patients were in NYHA I and 21 % in NYHA I-II. 89.7% had sinus rhythm. 42% of our patients suffered from temporary pleuropericardial effusions. CONCLUSIONS: Definitive palliation with TCPC achieves acceptable clinical results. Two-stage repair, fenestration, and postoperative inhalative NO therapy - each have a positive influence on early and long-term survival.

Transcatheter coil occlusion of residual interatrial communications after Fontan procedure.

OBJECTIVE: To assess the use of detachable coils as an alternative method to occlude interatrial communications after Fontan operations. DESIGN: Descriptive clinical study of selected patients after Fontan operation with interatrial communications inappropriate for transcatheter umbrella occlusion. SETTING: Tertiary paediatric cardiac referral centre. PATIENTS: Seven patients after Fontan operation with residual interatrial communications of various types producing a right to left shunt. INTERVENTIONS: Transcatheter placement of detachable coils with a diameter of 3 or 5 mm within the interatrial communication. RESULTS: A total of 14 coils were successfully placed within persistent patent fenestrations of the interatrial baffle, residual leaks at the suture line between the patch material and the right atrial wall, and unusual venous interatrial communications. The mean (SD) aortic oxygen saturation increased from 88 (1.1)% (range 86-89%) to 92 (1.3)% (range, 89-93%; p < 0.001) and the mean (SD) right atrial pressure rose from 9.7 (2) mm Hg (range, 6-11) to 10.6 (2.4) mm Hg (range, 6-13; p < 0.05) after coil implantation. In five patients, complete obliteration of the interatrial shunt was shown by angiography after coil implantation. At a mean (SD) follow up of 10 (4) months (range, 3-15) a residual interatrial shunt was detected by Doppler colour echocardiography in only one patient, and oxygen saturations ranged from 90% to 95% (mean, 92%). There were no late coil embolisations, thromboembolic events, or haemolysis in any patient. CONCLUSIONS: Detachable coils can be used successfully to occlude residual interatrial communications after the Fontan procedure. In selected cases, in whom intended transcatheter umbrella occlusion of residual interatrial leaks is not possible, the use of detachable coils might offer a safe alternative method to eliminate interatrial right to left shunting after the Fontan procedure.

Inhaled nitric oxide in infants and children after open heart surgery.

OBJECTIVE: To assess the effects of inhaled nitric oxide (NO) on oxygenation and pulmonary circulation in infants and children with critical pulmonary perfusion and/or hypoxemia after open heart surgery. STUDY: A prospective case series report. SETTING: A multidisciplinary pediatric intensive care unit in a University hospital PATIENTS: From June 1993 to March 1996 37 pediatric patients after open heart surgery were treated with inhaled NO. Their mean age was 2.9+/-0.6 years, their mean body weight 12.6+/-1.8 kg. METHODS: Inhaled NO was applied using a microprocessor controlled delivery system which continuously measured NO and NO2 by the chemilumniscence method. Monitoring included ECG, continuous pulse oximetry (SaO2), arterial (AP), central venous (CVP) and left atrial (LAP) pressures and in 8 patients a pulmonary artery (PAP) pressure. Inhaled NO was started at an SaO2 <90% with a fraction of inspired oxygen concentration (FiO2) >0.7, at a mean pulmonary artery pressure (MPAP) >50% of the mean arterial pressure (MAP), and in patients after Fontan-procedure at a CVP-LAP pressure gradient >10 mmHg. RESULTS: The mean dose of inhaled NO was 3.7+/-0.3 ppm and the mean duration was 112+/-14.7 hours. For the whole group SaO2 increased from 79.6+/-2.3 to 90.1+/-1.5% (p<0.01) within 20 minutes of NO-inhalation. Inhaled NO significantly decreased the MPAP from 47.8+/-4 to 27.5+/-2.3 mmHg (p<0.01) in 8 patients with postoperative pulmonary hypertension and significantly decreased the transpulmonary pressure (CVP-LAP) from 14.3+/-0.8 to 7.3+/-0.9 mmHg (p<0.01) in 16 patients after Glenn- or Fontan-procedure. CONCLUSIONS: Inhaled NO is very effective to decrease pulmonary artery pressure, to improve oxygenation, and to improve Fontan-circulation in infants and children after open heart surgery.

[Closure of the persistent ductus with detachable coils]. / Verschluss des persistierenden Duktus mit ablösbaren Coils.

34 patients aged 3 months to 20 years underwent transcatheter-occlusion of their patent ductus arteriosus with detachable coils (Cook). Eight patients had a residual ductus after previous implantation of a Rashkind-occluder; 7 patients had various other cardiac malformations in addition. Only 6 patients had a large ductus with a diameter between 3 and 4 mm; all had systolic-diastolic murmurs. All other patients had ductus-diameters below 3 mm; three of them had systolic-diastolic murmurs, 17 had systolic murmurs, and 8 patients had no murmur at all. The ductus was closed in 24 patients using arterial access only, in 6 patients via a venous, and in 4 patients both via venous and arterial catheterization. One coil was used in 23 patients, 2 coils in 9, and 3 coils in 2 patients. There were no complications of the intervention. Within 24 hours 31 patients (93%) had complete closure of the ductus and 32 patients (94%) after 6 months. Coil-embolisation of the persistent ductus is a quick, safe and cheap method to close a ductus and has clear advantages compared to an operation.

[Accessory mitral valve tissue as a rare cause of subaortic stenosis and valvular aortic insufficiency]. / Akzessorisches Mitralklappengewebe als seltene Ursache einer Subaortenstenose und valvulären Aorteninsuffizienz.

An unusual case of subaortic stenosis and aortic regurgitation caused by accessory mitral valve tissue in a 10 year old boy is reported. Two-dimensional and Doppler echocardiography revealed the characteristic feature of a mobile, parachute-like mass in the left ventricular outflow tract pro-lapsing into the aortic valve during systole and, thus, producing a systolic pressure gradient of 70 mm Hg between the left ventricle and aorta and causing mild aortic regurgitation. The accessory valve tissue was completely excised via an aortotomy without injury to the normal mitral and aortic valves. Two dimensional echocardiography provides excellent morphological information about the relationship between the accessory mitral valve tissue and the mitral and aortic valves, respectively. Accurate preoperative evaluation by two-dimensional echocardiography facilitates the successful surgical management of this rare condition.

Dissection of the descending aorta after balloon angioplasty of native coarctation.

A 12-year-old girl underwent successful balloon angioplasty for a waist-like native coarctation. The balloon size/coarctation diameter ratio was 3.3. Postdilatation angiography showed a small aneurysm at the coarctation site. On frequent review the patient remained symptom-free and normotensive. Recatheterization was performed 14 months after balloon aortoplasty, when angiography revealed a massive aortic dissection extending from the origin of the left subclavian artery to both iliac arteries. She underwent partial replacement of the thoracic aorta. Balloon angioplasty of a narrow waist-like native coarctation may lead to extensive wall dissection and should be considered critically.

Inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis.

OBJECTIVE: The aim of this study was to evaluate the effects of inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis. METHODS: Inhaled nitric oxide (mean 4.1 +/- 0.7 ppm, 1.5 to 10 ppm) was administered in 13 patients (mean age 5.6 +/- 1.6 years, 1.5 to 17 years) with critical pulmonary perfusion (central venous pressure > 20 mm Hg or transpulmonary pressure gradient > 10 mm Hg) in the early postoperative period after total cavopulmonary connection (n = 9) or after bidirectional Glenn anastomosis (n = 4). RESULTS: In patients after total cavopulmonary connection inhaled nitric oxide therapy decreased central venous pressure by 15.3% +/- 1.4% (p = 0.0001) and transpulmonary pressure gradient by 42% +/- 8% (p = 0.0008) and increased mean systemic arterial and left atrial pressures by 12% +/- 3.6% (p = 0.011) and 28% +/- 8% (p = 0.007), respectively. Arterial and venous oxygen saturations improved by 8.2% +/- 1% (p = 0.005) and 14% +/- 4.3% (p = 0.03), respectively. In patients after bidirectional Glenn anastomosis inhaled nitric oxide therapy resulted in a decrease of central venous pressure by 22% +/- 1% and of the transpulmonary pressure gradient by 55% +/- 6% and improved arterial and venous oxygen saturations by 37% +/- 29% and 11% +/- 3%, respectively. Mean systemic arterial and left atrial pressures remained nearly unchanged. No toxic side effect was observed in any patient. CONCLUSION: Inhaled nitric oxide may play an important role in the management of transient critical pulmonary perfusion caused by reactive elevated pulmonary vascular resistance in the early postoperative period after Fontan-type operations and bidirectional Glenn anastomosis.