Albrecht Beitzke

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.

Tissue plasminogen activator (alteplase) treatment for femoral artery thrombosis after cardiac catheterisation in infants and children.

OBJECTIVE--To determine the efficacy of fibrinolytic therapy with tissue plasminogen activator (alteplase) in infants and children with arterial thrombosis after cardiac catheterisation. DESIGN--Use of alteplase (Actilyse) in a protocol with prospective data collection. Alteplase was administered to infants and children with arterial thrombosis after cardiac catheterisation. A dose of 0.5 mg/kg/h was given continuously via a peripheral vein for the first hour followed by 0.25 mg/kg/h till clot lysis occurred or treatment had to be stopped because of bleeding complications. SETTING--University hospital, intensive care unit. PATIENTS--17 consecutive infants and children with femoral artery thrombosis after cardiac catheterisation between 1 April 1988 and 31 October 1991. MAIN OUTCOME MEASURE--Reopening of the vessel. RESULTS--Complete clot lysis was achieved in 16 of 17 patients within 4-11 hours after the start of treatment. In one patient only partial lysis occurred. After complete lysis rethrombosis developed in one patient 15 hours after the end of treatment. Bleeding complications were seen in nine patients. These were restricted to the arterial puncture site, except for one who showed mild epistaxis. Three patients had to be treated with packed erythrocytes. CONCLUSIONS--Alteplase was an effective treatment of arterial thrombosis after cardiac catheterisation in infants and children. Further studies are needed to determine whether lower doses will reduce the frequently observed bleeding complications.

Inhibitor to factor V after exposure to fibrin sealant during cardiac surgery in a two‐year‐old child

Muntean W, Zenz W, Finding K, Zobel G, Beitzke A. Inhibitor to factor V after exposure to fibrin sealant during cardiac surgery in a two‐year‐old child. Acta Prediatr 1994;83:84–7. Stockholm. ISSN 0803–5253

Open heart surgery in children of Jehovah's witnesses: Extreme hemodilution on cardiopulmonary bypass

SummaryBetween January 1979 and July 1989, 15 children of Jehovahs Witnesses underwent corrective open surgery for congenital heart disease (CHD) on cardiopulmonary bypass (CPB). Ages ranged from 1.5–17 years and body weight from 9.1–63 kg, with five patients weighing less than 15 kg. Eight children were cyanotic, and two of them had had previous thoracic operations.All operations were performed in moderate to deep hypothermia using a modified version of isovolemic hemodilution with bloodless priming technique of extracorporeal circulation. Mean hematocrit levels decreased from 47.3% (36.9–70%) to 34.6% (27.2–49.1%) after hemodilution, and then to 17.9% (10.5–25.6%) during bypass. They increased again to 34.1% (24.4–50%) at the end of the operation and to 33.4% (25.1–40%) on day 12. All intra- and postoperative hematocrit levels were significantly lower (p<0.001). There was one postoperative death, not related to the technique.Our results demonstrate that bloodless cardiac surgery on bypass is feasible in children as shown in this special group of children of Jehovahs Witnesses. Knowing the risks of homologous blood transfusion this technique should be used more extensively in the future.

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

Einleitung: Die Prognose angeborener Herzfehler hat sich in den letzten Jahrzehnten drastisch verbessert. Gleichzeitig hat auch die Zahl von Frauen mit einem angeborenen Herzfehler, die schwanger werden, stark zugenommen. Bislang existieren dennoch kaum aktuelle prospektive Daten über den Schwangerschaftsverlauf bei Frauen mit angeborenen Herzfehlern. Ziel der vorliegenden Studie war es daher, Art und Häufigkeit mütterlicher und kindlicher Probleme zu erfassen, die im Schwangerschaftsverlauf, bei der Entbindung und im Puerperium bei Frauen mit angeborenen Herzfehlern auftreten. Patienten: In die prospektive Multicenter-Studie wurden 106 Frauen mit angeborenen Herzfehlern eingeschlossen, bei denen es zu 122 Schwangerschaften gekommen war. Bei 72 dieser Frauen war vor der Schwangerschaft eine operative Behandlung des Herzfehlers erfolgt. Das Alter aller Patientinnen lag zwischen 17 und 44, im Median bei 26 Jahren. Erfasst wurden alle kardialen, nicht-kardialen Komplikationen während Schwangerschaft, Entbindung und Puerperium. Zudem erfolgte eine Screeninguntersuchung der Neugeborenen auf einen Herzfehler. Ergebnisse: Zu Beginn der Schwangerschaft befanden sich alle Frauen in der Funktionsklasse I oder II. Vorwiegend im 2. oder 3. Trimenon trat bei 25,5% (n=27) eine Verschlechterung der Funktionsklasse auf. Zu den gravierenderen Problemen, die bei 11,3% auftraten, gehörten klinisch relevante Blutungen, arterielle Hypertonie, Herzrhythmusstörungen, Endokarditis, Leberstauung, Aggravierung einer Zyanose sowie Todesfälle. Fünf Frauen hatten eine Abruptio, 9 einen Spontanabort, 9 eine Frühgeburt, und eine einen intrauteriner Fruchttod. Bei 79% (n=85) war eine Spontanentbindung per via naturalis möglich, bei 21,3% (n=23) erfolgte eine Schnittentbindung. 5,4% (n=6) der 111 lebendgeborenen Kinder hatten ebenfalls einen angeborenen Herzfehler. Schlussfolgerung: Während der Schwangerschaft kommt es zu kardiovaskulären Umstellungsreaktionen. Die veränderte Hämodynamik kann – in Abhängigkeit vom zugrundeliegenden Herzfehler – zu gravierenden Problemen während der Schwangerschaft, bei der Entbindung und im Puerperium führen. Die meisten Frauen, die sich vor Eintritt der Schwangerschaft in einer günstigen Funktionsklasse (I oder II) befinden, tolerieren eine Schwangerschaft gut. Wegen der potentiellen Gefahren für Mutter und Kind ist jedoch während der gesamten Schwangerschaft eine regelmäßige, interdisziplinäre Betreuung erforderlich, in die Kardiologen, Kinderkardiologen und Geburtshelfer eingebunden werden müssen, die über spezielle Erfahrung in der Betreuung angeborener Herzfehler verfügen. Das jeweilige Schwangerschaftsrisiko muss für jeden Herzfehler gesondert abgeschätzt werden und erfordert eine individualisierte Betreuung der Patientinnen. 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.

Catheter-directed thrombolysis with recombinant tissue plasminogen activator for acute pulmonary embolism after fontan operation.

Abstract. We report the case of a 3-year-old girl who presented with near-lethal pulmonary thrombembolism 3 weeks after an uneventful Fontan operation. Complete occlusion of the left lower lobe pulmonary artery had occurred together with a cerebral infarction. Recombinant tissue plasminogen activator (rt-Pa) was used for thrombolysis because of its short half-life and its clot-selective properties. To further minimize the systemic effects of rt-PA, local catheter-directed lysis was performed. A prolonged course of low-dose rt-PA therapy achieved complete lysis without side effects.

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 STARFlex® 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 additon 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. Bei 251 Patienten (mittleres Alter 43,2±12,4) wurde im Zeitraum 06/95 bis 06/01 ein offenes Foramen ovale (PFO) interventionell mittels Katheter verschlossen. 141 Patienten hatten einen ischämischen Schlaganfall, 99 eine transient ischämische Attacke (TIA) oder ein prolongiertes reversibles ischämisch-neurologisches Defizit, 5 periphere arterielle Embolien, 4 litten an Dekompressionskrankheit nach Tauchen und 2 hatten eine transiente globale Amnesie; 59 von ihnen hatten multiple Ereignisse trotz Therapie mit Thrombozytenaggregationshemmern oder Antikoagulantien. Fünf verschiedene Okkludersysteme wurden verwendet: 13 Rashkind®-Okkluder, 20 Amplatzer®-septal-Okkluder, 109 Amplatzer® PFO-Okkluder, 73 CardioSEAL®- und 36 STARFlex®-Okkluder. Die Durchleuchtungszeit betrug 8,3±4,5 Minuten. In 3 Fällen embolisierte ein Schirm und musste aus den Leistengefäßen chirurgisch geborgen werden. An weiteren Komplikationen fanden sich 5 venöse inguinale oder retroperitoneale Hämatome mit einer notwendigen Operation sowie je eine frühe und späte Perforation des linken Atriums durch einen Führungsdraht bzw. linksatrialen Okkluderanteil. 14 Patienten hatten dokumentierte späte Arrhythmien. Während 6 Patienten mit Vorhofflimmern medikamentöse oder elektrische Kardioversion benötigten, benötigten die anderen Patienten mit supraventrikulären Tachykardien, Vorhofflattern oder multiplen supra-ventrikulären Extrasystolen keine Therapie. Bei Kontrollen mittels transösophagealer Echokardiographie (TEE) 6 Monate nach dem Eingriff fanden wir vier signifikante Restdefekte. Diese Defekte wurden mit einem zweiten Okkluder verschlossen.    Zudem wurde bei 17 Patienten (mittleres Alter 38±10,5 Jahre) ein Vorhofseptumdefekt vom Sekundumtyp (ASD) mit 12 Amplatzer®-septal-Okkludern und 5 CardioSEAL®-Okkludern verschlossen. Diese Patienten hatten in 8 Fällen einen Schlaganfall und 9-mal eine TIA erlitten; drei von ihnen hatten multiple Ereignisse. Zwei hatten danach einen signifikanten residuellen Defekt und einmal trat spätes Vorhofflattern auf, welches medikamentös behandelt wurde. 202 PFO-Patienten und 12 Patienten mit ASD wurde nach 6–62 Monaten (24,6±14,2 Monate) nachkontrolliert; zwei starben an einem Verkehrsunfall bzw. Herzinfarkt. Vier Patienten hatten nach PFO-Verschluss ein rezidivierendes neurologisches Ereignis. Wir überblicken nunmehr 210 Patienten mit 348,6 symptomfreien Patientenjahren und haben eine 1-Jahres-Rezidivquote von 1,9%.    Der Katheterverschluss des PFO und Vorhofseptumdefektes ist eine einfache effektive und rasche Methode, welche eine hohe Verschlussrate des Defektes erbringt, lebenslange Antikoagulation vermeidet und eine niedrige Rate rekurrierender neurologischer Ereignisse hat.

Perinatal manifestations of idiopathic long QT syndrome

A neonate who had presented with sustained irregular heart rate during labor was found to have QT prolongation and repetitive polymorphic ventricular tachycardia (torsades de pointes) postnatally. Propranolol and propafenone successfully controlled the ventricular arrhythmias. Follow-up electrocardiograms and Holter records show persistent QT prolongation, bizarre T waves, and intermittent episodes of T wave alternans. On propranolol monotherapy the boy is thriving and completely free of ventricular arrhythmias. In the rate case of long QT syndrome in the neonate, early detection and therapy are mandatory to prevent ventricular arrhythmias and sudden death.

Atypical presentation of Kawasaki disease in an infant

SummaryA 7-month-old male infant with clinical symptoms of severe toxic shock syndrome died on day 9 of illness. At autopsy, demonstration of coronary vasculitis together with thrombosis of the left coronary artery revealed the true diagnosis of atypical Kawasaki disease. The marked similarity in many clinical features makes the distinction between these two diseases difficult when atypical clinical presentation of Kawasaki disease is present.

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.