Andreas Gamillscheg

Right ventricular function in infants, children and adolescents: reference values of the tricuspid annular plane systolic excursion (TAPSE) in 640 healthy patients and calculation of z score values.

BACKGROUND Tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measurement to assess right ventricular systolic function in adults. The aim of this study was to determine growth-related changes in TAPSE to establish references values. METHODS A prospective study was conducted in a group of 640 healthy pediatric patients (age range, 1 day to 18 years; body surface area range, 0.12-2.25 m(2)). The effects of age and body surface area on TAPSE were determined. RESULTS TAPSE ranged from a mean of 0.91 cm (z score +/- 3, 0.56-1.26 cm) in neonates to 2.47 cm (z score +/- 3, 1.84-3.10 cm) in 18-year-olds. TAPSE values showed positive correlations with age and body surface area. There was no significant difference in TAPSE values between female or male children. CONCLUSION In this study, z scores of TAPSE values were calculated and percentile charts were established to serve as reference data for ready application in patients with congenital heart disease in the future.

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.

Systolic Right Ventricular Function in Preterm and Term Neonates: Reference Values of the Tricuspid Annular Plane Systolic Excursion (TAPSE) in 258 Patients and Calculation of Z-Score Values

Background: The tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measurement to assess right ventricular systolic function in adults and children. Objective: We determined growth- and birth weight-related changes of TAPSE to establish reference values in preterm and term neonates. Methods: A prospective study was conducted in a group of 258 preterm and term neonates (age: 25+0 to 40+6 weeks of gestation, birth weight: 530–4,200 g). Results: The TAPSE ranged from a mean of 0.44 cm (Z-score ±2: 0.30–0.59 cm) in preterm neonates in the 26th week of gestation to 1.03 cm (Z-score ±2: 0.85–1.21 cm) in term neonates in the 41st week of gestation. The TAPSE values increased in a linear way from the 26th to 41st week of gestation. TAPSE, week of gestation and weight are strongly correlated: Pearson’s correlation coefficient was 0.93 for week of gestation – TAPSE (p < 0.001), 0.93 for week of gestation – birth weight (p < 0.001), and 0.89 for birth weight – TAPSE (p < 0.001). There was no statistically significant difference of normal TAPSE values between female and male patients (p = 0.987). Conclusion: Z-scores of TAPSE values were calculated and percentile charts were established to serve as reference data for ready application in preterm and term neonates with structurally normal hearts and with congenital heart disease in the future.

Reference values of tricuspid annular peak systolic velocity in healthy pediatric patients, calculation of z score, and comparison to tricuspid annular plane systolic excursion.

The tricuspid annular peak systolic velocity (TAPSV) is an echocardiographic measurement assessing right ventricular systolic function in children and adults. We determined the growth-related changes of the TAPSV to establish the references values for the entire pediatric age group. A prospective study was conducted of a group of 860 healthy pediatric patients (age 1 day to 18 years; body surface area [BSA] 0.14 to 2.30 m(2)). We determined the effects of age, gender, and BSA on the TAPSV values. Stepwise linear multiple regression analysis was used to estimate the TAPSV from the age, BSA, and gender. A correlation of normal TAPSV with normal tricuspid annular plane systolic excursion values was performed. The TAPSV ranged from a mean of 7.2 cm/s (z score ± 2: 4.8 to 9.5 cm/s) in the newborn to 14.3 cm/s (z score ± 2: 10.6 to 18.6 cm/s) in the 18-year-old adolescent. The TAPSV values showed a positive correlation with age and BSA, with a nonlinear course. No significant difference was found in the TAPSV values according to gender. A significant correlation was found between the TAPSV and tricuspid annular plane systolic excursion values in our pediatric population. In conclusion, the z scores of the TAPSV values were calculated, and percentile charts were established to serve as reference data for patients with congenital heart disease.

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.

Systolic Right Ventricular Function in Children and Young Adults with Pulmonary Artery Hypertension Secondary to Congenital Heart Disease and Tetralogy of Fallot: Tricuspid Annular Plane Systolic Excursion (TAPSE) and Magnetic Resonance Imaging Data

OBJECTIVE   The tricuspid annular plane systolic excursion (TAPSE), as echocardiographic index to assess right ventricular (RV) systolic function, has not been investigated thoroughly in children and young adults with tetralogy of Fallot (TOF) and pulmonary artery hypertension secondary to congenital heart disease (PAH-CHD). PATIENTS   TAPSE values of 49 patients with PAH-CHD and 156 patients with TOF were compared with age-matched normal subjects. TAPSE values were also compared with RV ejection fraction (RVEF) and RV indexed end-diastolic volume (RVEDVi) determined by magnetic resonance imaging in PAH-CHD and TOF patients. RESULTS   Patients with a PAH-CHD showed a positive correlation between TAPSE with RVEF (r= 0.81; P < 0.001) and a negative correlation between TAPSE with RVEDVi (r=-0.67; P < 0.001). Similarly, in our TOF patients, a positive correlation between TAPSE with RVEF (r= 0.65; P < 0.001) and a negative correlation between TAPSE with RVEDVi (r=-0.42; P < 0.001) was seen. CONCLUSIONS   Significant pressure overload in PAH-CHD patients and volume overload in TOF patients lead to a decreased systolic RV function, determined by TAPSE and magnetic resonance imaging and to increased RVEDVi values, determined by MRI, with time.

Systolic right ventricular function in pediatric and adolescent patients with tetralogy of Fallot: echocardiography versus magnetic resonance imaging.

OBJECTIVE The tricuspid annular plane systolic excursion (TAPSE) as an echocardiographic index to assess right ventricular (RV) systolic function has not been investigated thoroughly in pediatric patients and adolescents with tetralogy of Fallot (TOF) after surgical repair. METHODS TAPSE was determined in 131 patients with TOF and 252 age-matched normal subjects. TAPSE values were compared with RV ejection fraction (EF) and indexed RV end-diastolic volume (EDVi) determined by magnetic resonance imaging in a cross-sectional study design. TAPSE values were also correlated to QRS duration (QRSd) determined by electrocardiogram. RESULTS The TAPSE values showed a positive correlation with age in normal subjects. The TAPSE was not decreased in infants and young children with TOF compared with normal subjects. A significant reduction of TAPSE values with increasing time after surgical repair was observed. After a mean of 7 years after surgical repair, the TAPSE values become significantly reduced compared with age-matched controls, being below the lower bound of -2 standard deviations. A positive correlation between TAPSE with RVEF and a negative correlation between TAPSE with RVEDVi were observed. A significant positive correlation was found between QRSd and RVEDVi, and a significant negative correlation was found between QRSd and RVEF. CONCLUSION Although TAPSE was initially preserved, impaired TAPSE was observed with increasing time after surgical repair in pediatric patients with TOF.

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.