Results of the phase I food and drug administration clinical trial of duct-occlud device occlusion of patent ductus arteriosus.

The purpose of this study was to evaluate the safety and the efficacy of transcatheter patent ductus arteriosus (PDA) occlusion with the Duct-Occlud device. Six centers participated in this phase I U.S. Food and Drug Administration-approved clinical trial with investigational device exemption. Patients with restrictive PDA (less than 4-mm minimum diameter) were eligible for the study. Sixty-two patients were enrolled. Forty-eight patients had successful implantation of Duct-Occlud devices. Patient follow-up evaluations were conducted at hospital discharge and after 2 and 12 months. At discharge and after 1 year, all patients had clinical PDA closure. Closure assessed by color flow Doppler was 55% at discharge, 88% at 2 months, and 94% at 1 year. There were no complications related to implantation or noted in follow-up evaluations. The Duct-Occlud device is safe and efficacious for the closure of small- to moderate-size patent ductus arteriosus.

Transcatheter closure of surgical shunts in patients with congenital heart disease.

The aim of this study was to review contemporary techniques, devices, and results of transcatheter occlusion of surgical shunts in 2 pediatric cardiac programs. Closure of superfluous surgical shunts may reduce cardiac work and risk of endocarditis. Previous studies have shown that transcatheter closure of shunts is feasible, but have not demonstrated acceptable efficacy or safety. In addition, the performance of new techniques and devices has not been reviewed. Between 1993 and 1998, 18 patients with congenital heart disease underwent transcatheter closure of 19 Blalock-Taussig shunts. Detachable and standard Gianturco coils and Gianturco-Grifka vascular occlusion devices were employed. All 19 shunts had complete closure. Eight shunts had initial placement of detachable coils. Five shunts had stents placed that bridged the pulmonary end of the shunts. These 5 and 4 additional shunts had closure by standard coils. Two shunts were closed with Gianturco-Grifka devices. There were no complications, no embolizations, and no requirement for surgery precipitated by the procedures. This review of contemporary techniques, devices, and results suggests that transcatheter occlusion of surgical shunts is effective and safe.

Modified Judkins catheters for selective coronary angiography in infants and young children.

The purpose of this study was to assess 4 Fr Judkins catheters with modified shorter tips for performing selective coronary angiography in infants and young children. Twenty patients ranging 6 weeks to 3. 8 years of age were enrolled. Retrograde left heart catheterization and selective coronary angiography were performed. Right (JR) and left (JL) catheters with modified 1.5- and 2.5-cm curves (Cordis) were used. Thirty-six of 37 coronary arteries were successfully cannulated and demonstrated. Median procedure time was 95 sec for the right and 50 sec for the left coronary artery. Median fluoroscopy time was 1.1 min for the right and 0.7 min for the left coronary artery. The JL 1.5 appeared best suited for patients less than 75 cm tall. The JR 1.5 was suitable for patients up to 85 cm tall. Taller patients required the 2.5-cm curves. It is concluded that these modified 4 Fr Judkins catheters were effective.

[Adults with congenital heart defects--clinical spectrum and surgical management]. / Erwachsene mit angeborenen Herzfehlern--klinisches Spektrum und operative Behandlung.

Two hundred adults who underwent surgery for congenital heart disease at our institution within a four year period were the basis for this report. Clinical data was obtained, i.e. demographic details, past medical history, physical findings, EKGs, echocardiograms, catheterization and angiography material as well as the New York Heart Association (NYHA) class. Intraoperative findings, perioperative management, complications and morbidity and mortality were assessed. After discharge the patients were followed on an outpatient basis. Again the clinical findings, laboratory results and NYHA class were recorded. Age ranged from 16 to 75 years (mean = 38). There were 114 females and 86 males. 178 patients had primary surgical correction, 18 were reoperated after prior correction and 4 underwent palliation. Eighty-three percent of the patients were symptomatic preoperatively. Seventeen percent were diagnosed per chance, for example by a preemployment physical examination, without a prior history of heart disease. The patients were grouped according to related diagnoses (Table 1). Among the 130 patients with left to right shunts, 112 had an atrial septal defect, 7 a ventricular septal defect, 5 a persistent ductus arteriosus, and 6 partial anomalous pulmonary venous return. Atrial flutter or fibrillation occurred in approximately 10% of all patients with atrial septal defects. It was terminated either by rapid overdrive pacing or DC cardioversion. Postoperatively the patients with pulmonary hypertension were monitored invasively with arterial lines and thermodilution catheters. Therapy consisted of alkalization, hyperventilation and sedation. There was only one postoperative death in this patient group due to marked pulmonary hypertension (1/130 = 0.8%). Nineteen adults had obstruction to right or left ventricular outflow. Surgery included valvotomy, infundibulectomy and valve replacement by homograft or mechanical valve. One patient with multiple previous surgeries expired due to bleeding (3%). Thirteen patients had coarctation. All of them were hypertensive, some on medication. Surgery consisted of aortic patchplasty or interposition of a graft. There was no mortality. Perioperative antihypertensive therapy was necessary in most patients and consisted of nifedipin, nitroprussid or propanolol intravenously. Upon follow up 11 patients became normotensive, 8 of these without the need for medication. Fifteen cyanotic patients underwent 11 corrective and 4 shunt procedures (3 with tricuspid atresia, 10 with Tetralogy of Fallot and 2 with complex cyanotic heart disease. Three died due to low cardiac output or dysrhythmias (20%). The survivors improved their clinical status markedly. Seven adults with Ebstein's disease had valve reconstruction and/or ASD closure. Five had recurrent supraventricular tachycardia, 2 paradoxical emboli with neurological symptoms and 4 out of 7 had decreased exercise tolerance. One patient died postoperatively because of dysrhythmias (14%). Sixteen patients had a variety of defects, i.e. status post Rastelli operation and conduit obstruction, status post Tetralogy of Fallot with pulmonary valvar disease, corrected transposition with left AV valve insufficiency, congenital mitral valve disease and double aortic arch, no deaths. The overall operative mortality was 6/200 = 3%. the late mortality was 4/200 = 2%. The morbidity included 7 reoperations due to bleeding. Five patients needed short-term hemodialysis. One patient developed hemiplegia and two patients had permanent decrease of their left ventricular function. The mean length of follow up was 21 months. The clinical status improved from a NYHA class mean of 2.1 +/- 0.9 to 1.2 +/- 0.45 (p < 0.001). In Germany significant numbers of adults with operated and unoperated congenital heart disease do exist. Detection of these patients can be difficult due to inconspicuous murmurs or stable clinical status.

Repair of coarctation of the aorta in adults: the fate of systolic hypertension.

BACKGROUND: The benefit of coarctation repair in adults has been questioned by suggesting that hypertension may not be relieved by the operation and that surgical intervention may have no impact on the natural history of the disease. METHODS: To delineate the impact of surgical intervention on systolic hypertension, we conducted a retrospective review of 26 adults with a mean age of 32 +/- 10 years who underwent coarctation repair between 1987 and 1993. All patients were hypertensive (mean systolic blood pressure, 174 +/- 21 mm Hg; range, 140 to 220 mm Hg), and 18 patients (69%) were on a regimen of at least one hypertensive medication at the time of surgical admission. All patients underwent catheterization, and the mean peak systolic gradient across the coarctation was 61 +/- 25 mm Hg (range, 25 to 120 mm Hg). Operation included resection and end-to-end anastomosis (3 patients), resection with an interposition tube graft (6 patients), a bypass graft (11 patients), and patch angioplasty (6 patients). There was no hospital mortality or late morbidity. RESULTS: Intermediate follow-up was available at a mean of 2.3 +/- 2 years (range, 1 to 7 years). At last follow-up, the peak systolic gradient between the upper and lower body was trivial (< or = 10 mm Hg) in 23 patients (88%) and mild (11 to 20 mm Hg) in 3 (12%). All patients had significant improvement in systolic blood pressure (p < 0.001) compared to preoperative values, and the majority (23, 88%) were normotensive. More than half of the patients (14, 54%) were still on a regimen of antihypertensive medication at last follow-up, with a trend (p = 0.06) toward older patients requiring medication. CONCLUSIONS: Surgical repair of coarctation in adults is an effective, low-risk procedure, which results in a significant improvement in systolic hypertension and a decreased requirement of antihypertensive medications.

Truncus arteriosus with total anomalous pulmonary venous return and interrupted arch.

An infant with truncus arteriosus, total anomalous pulmonary venous return of the supracardiac type and interrupted aortic arch distal to the left subclavian artery is presented. One-stage repair was performed in the newborn period using a 12-mm aortic homograft. Recovery was complicated by congenital stenosis of the left main bronchus. The cardiovascular status remains stable 2 years after repair.

Anomalous left coronary artery from the pulmonary artery. Significance of intercoronary collaterals.

Seventeen patients with an anomalous left coronary artery from the pulmonary trunk underwent surgical treatment and were evaluated, with a mean follow-up of 9.5 years. Analysis has included clinical symptoms cardiac laboratory tests, angiographic grading of intercoronary collaterals, echocardiographic and angiographic measurement of left ventricular function, surgical methods, and the postoperative outcome. Ten patients (59%) survived, and there were seven early deaths. The only significant variable relating to survival was the extent of preoperative intercoronary connections. In survivors, the postoperative left ventricular ejection fraction increased from 45% +/- 25% to 64% +/- 14% (p < 0.01). Nine of ten survivors are free of symptoms and have normal left ventricular function. Nevertheless, half of all created anastomoses were occluded within 2 years of the operation. We speculate that even if temporarily constructed, a dual coronary supply allows for left ventricular recovery and normalization of function.

[Transvenous catheter occlusion of the patent foramen ovale in paradoxical embolism]. / Transvenöser Katheterverschluss des offenen Foramen ovale wegen paradoxer Embolien.

Paradoxical emboli are assumed to be one cause for cerebral strokes of unknown etiology. These emboli usually cross from the right to the left heart via a patent foramen ovale. We describe the transvenous catheter occlusion as an alternative to surgical closure. There were no complications. The echocardiographic follow-up of 12 and 14 months demonstrated complete closure of the foramen ovale without recurrence of neurologic symptoms.

Left ventricular performance in neonates on extracorporeal membrane oxygenation.

The evaluation of left ventricular systolic performance in infants undergoing extracorporeal membrane oxygenation (ECMO) using traditional ejection-phase indices is hampered by significant alterations in preload and afterload. Therefore, a load-independent index, which relates heart-rate-corrected mean velocity of circumferential fiber shortening (VCFc) to afterload, measured as end-systolic wall stress (ESS), was used to assess left ventricular function in 18 term neonates undergoing ECMO. The mean age at the onset of ECMO was 75.5 h and the duration of therapy was 171 +/- 106 h. Left ventricular performance was highest before the onset of ECMO (VCFc = 1.65 +/- 0.49 circ/s) and decreased toward normal during (1.38 +/- 0.33 circ/s) and following ECMO (1.29 +/- 0.16 circ/s). Initially, nine of 17 (53%) patients had enhanced performance for the degree of afterload but in only 16 of 48 (33%) studies during ECMO and none following ECMO was VCFc elevated beyond the normal range predicted for ESS. These changes in left ventricular performance may be the result of variations in exogenous, as well as endogenous, catecholamines rather than intrinsic alterations in myocardial contractility. It is concluded that the VCFc/ESS relation permits a meaningful assessment of ventricular performance in critically ill neonates undergoing ECMO.

[Combined surgical-interventional procedure in congenital heart defects with postoperative, left ventricular dysfunction]. / Kombiniertes operativ-interventionelles Vorgehen bei angeborenen Herzfehlern mit postoperativer, linksventrikulärer Dysfunktion.

Creation of a communication between the left and right atrium to decompress the left ventricle can be life-saving after corrective surgery in some patients with congenital heart disease and small left ventricle. After adaptation of the left ventricle, surgical closure of this anastomosis becomes mandatory. We report four patients (2.9 to 8.2 kg) where non-surgical, transcatheter-closure was performed in the first year of life using the Rashkind-PDA-Occluder System (USCI). In a newborn with d-transposition of the great arteries (2.9 kg), in a patient with atrial septal defect (5.1 kg), and in a patient with tetralogy of Fallot with atrial septal defect (4.9 kg) atrial septal defects were not completely closed during correction because of left-ventricular dysfunction. Five to 12 days postoperatively transcatheter closure was performed utilizing the 8F-Rashkind-PDA-Occluder System in the first two patients (2.9 kg and 4.9 kg) and by use of the 11F-Rashkind-PDA-Occluder System in the third patient (4.9 kg). In the patient with supracardiac total anomalous pulmonary venous drainage via a vertical vein, the vertical vein was not closed during surgical correction at the age of 2 months. Transcatheter closure was performed 6 months later using the 11F-Rashkind-PDA-Occluder-System. It is concluded that small, defined atrial septal defects can be closed, even in newborns, using the Rashkind-PDA-Occluder System (USCI) and, furthermore, that large vessels, even without luminal narrowing can be occluded completely. Thus, this combined "surgical-interventional approach" is not limited by age and could reduce the risk of surgical correction in patients with congenital heart disease and small left ventricle.

Enoximone in newborns with refractory postoperative low-output states (LOS).

Enoximone was administered to 16 newborns with postoperative, catecholamine-refractory cardiac low-output states in addition to high-dose catecholamine treatment. Haemodynamic changes were assessed at baseline and during treatment. Haemodynamic parameters were improved in 12 newborns ("responders"), 9 of these survived. Three responders died; one from cardiac low-output and 2 from uncorrectable congenital heart disease verified by autopsy. Four newborns did not respond to enoximone therapy ("non-responders") and died. The haemodynamic effects of enoximone were characterized by an increase in cardiac index (+160%, P less than 0.0008), and a fall in right (-26%, P less than 0.0004) and left (-34%, P less than 0.003) atrial pressures. It is concluded that enoximone can be an effective agent in the treatment of cardiac low-output states refractory to high-dose catecholamines in neonates up to 7 months old.

Mechanical left ventricular support as a bridge to cardiac transplantation in childhood.

The mechanical extrathoracic "Berlin Heart" cardiac assist device was used for left ventricular support in an 8-year-old child of 27 kg body weight. Terminal left ventricular failure and life-threatening ventricular tachyarrhythmias in this child were sequelae of coarctation of the aorta, aortic stenosis and resultant severe left ventricular dilatation, hypokinesia and fibrosis. With the assist device, cardiogenic shock could be reversed. The child was extubated and mobilized during an 8-day waiting period for orthotopic heart transplantation. This case illustrates that the use of ventricular assist devices can be successfully extended to children and may be advantageous compared to other techniques of mechanical circulatory support, such as balloon pumping or extracorporeal membrane oxygenation.