Coronary interventions in congenital heart diseases: from preterm to young adult patients.

BACKGROUND: The small vessel size of infants and children makes interventional treatment of impaired coronary perfusion, such as stenoses, complete occlusions, and fistulae, demanding. Materials and techniques appropriate for this young age group have to demonstrate their ability to effectively treat these lesions. METHODS AND RESULTS: Between 2004 and 2011, 14 patients with an age of 9 days to 25 years (median 4.6 years) and a bodyweight of 1.7-65 kg (median 14 kg) underwent coronary intervention. In 3 cases, emergency revascularization of the left coronary artery (CA) was performed successfully, followed by stent implantation in 1 patient. Embolization of coronary arterial fistulae with coils and vascular plugs was effective in 10 patients. An antegrade, retrograde or combined approach to achieve the most distal device placement preserved all side branches. One infant with pulmonary atresia and an intact ventricular septum was prepared for biventricular repair by step-by-step closure of the right ventricular to the CA connections. No procedure-related deaths occurred. CONCLUSION: Congenital and post-procedural coronary obstructive lesions can be considered for effective treatment with balloon dilation at any age as a salvage procedure. In coronaries impaired by external compression, stent implantation can restore perfusion, but long-term results are missing. Interventional closure of coronary fistulae has shown improvement of coronary arterial perfusion. The latter techniques can be used to close right ventricular to CA connections in patients with pulmonary atresia to prepare for biventricular repair, but bail-out strategies should be planned in all coronary interventions.

Balloon valvuloplasty in the treatment of congenital aortic valve stenosis--a retrospective multicenter survey of more than 1000 patients.

BACKGROUND: The value of balloon valvuloplasty of the aortic valve in childhood is still under debate. OBJECTIVE: To evaluate the results of the procedure in a retrospective multicenter survey of a large cohort over a long time interval. METHODS: Retrospective analysis of 1004 patients with balloon valvuloplasty of the aortic valve performed between 9/1985 and 10/2006 at 20 centers in Germany, Austria and Switzerland. Amongst others, the following parameters were evaluated before and after the procedure as well as at the end of follow-up or before surgery: clinical status, left ventricular function, transaortic pressure gradient, degree of aortic regurgitation, freedom from re-intervention or surgery. PATIENTS: Patients from 1 day to 18 years of age with aortic valve stenosis were divided into four groups: 334 newborns (1-28 days); 249 infants (29-365 days); 211 children (1-10 years), and 210 adolescents (10-18 years). RESULTS: Median follow-up was 32 months (0 days to 17.5 years). After dilatation the pressure gradient decreased from 65 (± 24)mm Hg to 26 (± 16)mm Hg and remained stable during follow-up. The newborns were the most affected patients. Approximately 60% of them had clinical symptoms and impaired left ventricular function before intervention. Complication rate was 15% in newborns, 11% in infants and 6% in older children. Independently of age, 50% of all patients were free from surgery 10 years after intervention. CONCLUSIONS: In this retrospective multicenter study, balloon valvuloplasty of the aortic valve has effectively postponed the need for surgery in infants, children and adolescents up to 18 years of age.

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.

Integrated home monitoring predicts lead failure in a pacemaker dependent 4-year-old girl.

A 4-year-old girl with post-surgical complete atrioventricular block received an epicardial dual chamber pacemaker system. During further growth intermittent exit block occurred, first misinterpreted as neurological seizures. The epicardial lead was replaced using a transvenous approach, and a pacemaker with an integrated home monitoring facility was implanted. After her discharge, a rise in the pacing threshold automatically initiated an event message. On the basis of this information, the patient was called in and imminent dislodgement of the ventricular lead was diagnosed by x-ray. The lead was repositioned and was found stable over 1-year follow-up.

[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.

Transseptal approach in children after patch occlusion of atrial septal defect: first experience with the cutting balloon.

Two children required a transseptal approach to the left heart for endovascular stent redilation late after pericardial patch closure of atrial septal defects performed at the time of their initial surgical intervention. Following perforation of thickened interatrial patches in both patients, cutting balloons were used to create adequate interatrial communications. Cathet. Cardiovasc. Intervent. 48:378-381, 1999.

[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.

[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.

Various reasons for repeat dilatation of stented pulmonary arteries in paediatric patients.

OBJECTIVE: To evaluate the reasons for repeat intervention in patients treated with balloon expandable stents for pulmonary artery stenoses, with particular analysis of the time intervals between interventions. DESIGN: A retrospective observational study. SETTING: A single paediatric cardiology centre. PATIENTS: 38 patients, mean age 6.9 years, range 6 days to 34 years (one adult patient); mean weight 19.7 kg, range 2.5-75 kg. INTERVENTION: Implantation of balloon expandable stents in 46 cases of main pulmonary artery stenosis, right pulmonary artery stenosis, left pulmonary artery stenosis, or right ventricle to pulmonary artery conduits. MAIN OUTCOME MEASURES: Adaptation of stent diameter to patient growth; development of pulmonary arteries; pressure gradient in the right ventricle. RESULTS: 56 stents were implanted in 46 lesions. During a mean follow up time of 2.2 years, 40 repeat dilatations were performed on 28 of 42 reinvestigated stents. A second repeat dilatation was performed on eight stents, and a third on four stents. The mean time period between implantation and repeat dilatations was 15.5 months. CONCLUSIONS: Repeat dilatation of stented pulmonary arteries was done mainly to adapt the stent diameter to the patient's growth. However, repeat dilatations were also performed to relieve stent obstruction caused by intimal proliferation (17.5%), or to overcome progressive external compression (25%). While most repeat interventions in adult patients are for intimal hyperplasia, in paediatric patients they are needed for a variety of reasons.

Stent implantation of the arterial duct in newborns with duct-dependent circulation.

AIMS: Little is known about the medium term results after stenting of the arterial duct in neonates and infants with duct-dependent cyanotic congenital heart disease. We report the results of stent implantation of the arterial duct in 21 neonates and infants. The defects for which the arterial duct was stented included pulmonary atresia with intact ventricular septum, critical pulmonary stenosis, and more complex defects with associated reduced pulmonary blood flow. METHODS AND RESULTS: Palmaz stents were used and successfully implanted in all the 21 patients. There were no major complications during the stent implantation procedure although two hospital deaths occurred 2 and 14 days after stent implantation. Cardiac catheterization was repeated electively 3 to 6 months after stent implantation. Stent stenosis due to intimal proliferation was noted in 11/13 patients who underwent recatheterization. Stenosis of the inner stent lumen ranged from 25% to 100%, mean 74%. Re-dilatation of the stent was required in five patients who were awaiting corrective surgery. In babies with pulmonary atresia or critical pulmonary stenosis, who also underwent additional balloon dilatation of the pulmonary valve, spontaneous closure of the stented arterial duct was well tolerated and when it occurred, the right ventricular size had increased and the circulation was no longer duct-dependent. In patients who required subsequent surgical corrective treatment, stenting of the duct allowed the definite corrective operation to be performed as the first surgical procedure. During the follow-up period, ranging between 2 months and 2 years, mean 8.7 months increased growth of the pulmonary arteries was seen in all the patients. No distortion of the branch pulmonary arteries was seen. CONCLUSION: In patients with cyanotic congenital heart disease stenting of the arterial duct is an effective alternative to surgical aorto-pulmonary shunts.