Site of coronary sinus drainage does not significantly affect coronary flow reserve in patients long term after Fontan operation.

This study was designed to investigate the impact of postoperative coronary sinus drainage pressure on coronary flow reserve (CFR) assessed by Doppler guidewire in patients long term after Fontan operation. Twenty-nine patients (median age, 17.4 years female, 11) at a median of 10.6 years after Fontan operation were examined with intracoronary Doppler guidewire during cardiac catheterization. Fourteen patients had coronary sinus (CS) drainage to the systemic venous atrium and 15 patients had CS drainage to the pulmonary venous atrium after Fontan operation. Median CS drainage pressure was significantly higher in systemic venous CS drainage compared to pulmonary venous CS drainage (11 vs 5 mmHg, p < 0.0001). Median CFR values for the right and left coronary artery did not differ significantly with respect to CS drainage. There was a positive correlation between coronary flow reserve and pulmonary arteriolar resistance (p < 0.05) in multivariate regression analysis. The site of coronary drainage into the systemic atrium or the pulmonary venous atrium did not significantly affect CFR. Our data do not support a surgical strategy of elective redirection of coronary sinus blood to a low-pressure compartment but support an early staged approach. The positive correlation between CFR and pulmonary resistance demands further evaluation.

Stenting of a stenosed sano shunt in a neonate with hypoplastic left heart syndrome.

We report on successful stenting of a proximally stenosed Sano shunt in a newborn with hypoplastic left heart syndrome after a stage I Norwood operation.

Coil embolization therapy in congenital coronary arterial fistulas.

Three pediatric patients (8.5 years, 3 years, and 1 month) presented with congenital coronary arterial fistulas. In all cases the fistulas entered into the right side of the heart (main pulmonary artery, n = 1; right ventricle, n = 2). In the first patient, the fistula and an open ductus arteriosus were closed during the same intervention. The second patient presented with a single left coronary ostium and residual shunt from the coronary artery system to the right ventricle after surgery. The third child had pulmonary atresia with intact ventricular septum and a fistula from the left coronary artery to the right ventricle. The fistulas in all patients were managed with coil occlusion. Fistula occlusion was documented with angiocardiography.

Balloon dilation for aortic recoarctation: morphology at the site of dilation and long-term efficacy.

OBJECTIVES: We undertook this study to assess the immediate and long-term outcome of balloon angioplasty performed for recurrent or residual coarctation of the aorta, and to assess the changes in the vessel wall caused by this procedure. METHODS: Clinical, echocardiographic, angiographic and hemodynamic data from 71 patients who underwent balloon angioplasty for recoarctation between January 1987 and January 1998 were analysed retrospectively. RESULTS: Angioplasty was performed after a median of 82.6 months (range 1.4 mo-20.9 y, mean 88.5 mo) following surgery for coarctation. Mean systolic pressure gradients were reduced from 27 +/- 15 mmHg to 11 +/- 11 mmHg after angioplasty (p < 0.0001). The mean diameter at the site of recoarctation increased from 5.5 +/- 2.5 to 7.5 +/- 2.7 mm (p < 0.0001). Outpouchings of contrast agents, indicating the disruption of the inner layers of the vessel wall, were defined as extravasations. They were observed in one-quarter of the angiograms performed immediately after the intervention. Immediate success of angioplasty was achieved in 71%, and persisted in 69% of patients during long-term follow up. The main determinant for immediate success was the age at the time of the procedure (p < 0.05), while the main determinant for long-term success was the increase achieved in diameter. Extravasations did not progress to aneurysms, neither acutely nor during echocardiographic follow-up studies. For further follow-up, more sensitive imaging techniques will be necessary to delineate the morphology of the site of extravasation observed immediately after angioplasty.

Acute inferior cardiac inflow obstruction resulting from inadvertent surgical closure of a prominent Eustachian valve mistaken for an atrial septal defect.

A 5-month-old boy with a VACTERL syndrome underwent cardiac surgery for correction of a common arterial trunk and closure of an atrial septal defect. A prominent Eustachian valve was mistaken for the atrial septum and surgically closed. Thirty months later, after gradual shrinking of the foramen ovale with associated reduction of the right-to-left shunt, the boy presented with acute symptoms of a lower inflow obstruction, characterized by hepatomegaly and engorged abdominal vein pattern (Medusa's head). The boy was reoperated successfully after the condition had been recognized.

[Interventional catheter occlusion of the atrial septal defect of the secundum type--experiences with "CardioSeal" and "Angel Wings"]. / Katheterinterventioneller Verschluss des Vorhofseptumdefektes vom Sekundumtyp--Erfahrungen mit "CardioSeal" und "Angel Wings".

Between July 1996 and July 1998, 34 patients (female n = 16, male n = 18) were scheduled for catheter--interventional occlusion (ASDO) of an atrium septum defect of secundum type (ASD II). Median age was 7.9 years (2.5-54.6), weight 23.1 kg (9-97). 9 defects were closed with "Angel Wings" (AW) and 25 ASD occlusions were attempted with "CardioSeal" (CS). 30 patients had simple ASD II, 3 persisting foramen ovale with suspected paradoxal cerebral embolization and 1 significant residual shunt at atrial level after corrective heart surgery for interrupted aortic arch type B. ASDO was successful in 31/34 cases (91%). One CS was removed surgically due to significant left to right shunt after release and two CS devices were withdrawn back into the sheath. Median ASD diameter was 11.9 mm (6-16), balloon occlusive diameter was 15 mm (6-20) and median left to right shunt was 40% (6-64%). On follow up minimal left to right shunt was seen in 5/31 patients (16%). In selected patients catheter--interventional ASDO may be a good alternative to surgical ASD closure.

Percutaneous closure of secundum atrial septal defect with a new self centering device ("angel wings").

OBJECTIVE: To investigate the safety, efficacy, and clinical application of a new self centering device ("angel wings") for closure of secundum atrial septal defects (ASD II) and persistent foramen ovale in all age groups. DESIGN: Multicentre, prospective, nonrandomised study. INCLUSION CRITERIA: defects with an occlusive diameter of < or = 20 mm and a surrounding rim of > 4 mm; body weight > 10 kg; and an indication for surgical closure of secundum atrial septal defect. Additionally, there were compassionate indications for closure in patients with persistent foramen ovale. INTERVENTIONS: Defects were closed by a transcatheter device consisting of two square frames made of superelastic nitinol wire. The frames are covered by elastic polyester fabric, which is sewn together at a central circle. All procedures, except for three interventions that were carried out under sedation, were performed under general anaesthesia using transoesophageal echocardiography and fluoroscopy to monitor intervention. RESULTS: Closure was attempted in 75 (71%) of 105 patients. An ASD II was present in 35 children and 15 adults. A persistent foramen ovale was present in 25 adults with suspected paradoxical embolism. Transcatheter closure was unsuccessful in three children and crossover to surgery was required. Residual shunts were found in 20 patients (27%) immediately after the procedure. A transient atrioventricular third degree block occurred in three patients (4%) and the right atrial disk was not fully deployed in three. A minor shunt (< 3 mm) was present in only three (4%) of 72 patients during follow up of 1-17 months. Blood clots on the right atrial disks in two patients (one required lysis) were seen during follow up transoesophageal echocardiography. Serious complications demanding surgical removal of the device occurred in three patients. One patient had haemopericardial tamponade because of an aortic lesion. Left atrial thrombus formation due to an unfolded right atrial disk was found in a second patient and dislodgement of the left atrial disk resulted in a large residual shunt in a third. CONCLUSIONS: Percutaneous closure of a central ASD with a diameter < or = 20 mm in paediatric and adult patients is feasible and effective with this new device. It is a promising alternative to surgical closure. Modifications of the design, however, seem to be mandatory as 4% of patients developed serious complications.

Complete thrombotic obliteration of the ascending aorta and the aortic arch as a cause of acute heart failure in a newborn.

Thrombosis of the great vessels, and especially of the aorta, is rare in neonates. We report a case with thrombosis of the ascending aorta, aortic arch, brachiocephalic trunk and subclavian artery. Clinically, severe heart failure occurred on the 1st day of life and the diagnosis was confirmed by echocardiography and cardiac catheterization, including angiocardiography. Left ventricular function was found to be extremely depressed. An infusion with prostaglandin E1 was initiated in order to improve the systemic circulation by dilating the arterial duct. The infant died of neurological complications prior to surgery.

Plasma atrial natriuretic peptide levels in children with cardiac diseases: correlation with cGMP levels and haemodynamic parameters.

In children with various forms of cardiac diseases (aged 2 months to 16 years) significantly higher plasma atrial natriuretic peptide (ANP; range 36-680, median 247 pg/ml) and cyclic 3'5'-guanosine monophosphate (cGMP; range 0.2-46, median 8.2 pmol/ml) levels were found than in control children (p less than 0.0001). In control children (aged 4 months to 17 years) plasma ANP and cGMP levels were measured in the range of 2.4-98 pg/ml and of 0.2-2.8 pmol/ml, respectively. There was a linear correlation between the two parameters in children with cardiac diseases (r = 0.62, p less than 0.01). Children with elevated mean right atrial pressure (i.e., greater than 6 mm Hg) showed significantly higher plasma ANP levels than children with normal atrial pressure (p less than 0.01). However, there was only a weak linear correlation between mean right atrial pressure and plasma ANP levels (r = 0.48, p less than 0.01). Plasma ANP levels from right atrium, pulmonary artery, left atrium and left ventricle were significantly higher than those from vena cava (p less than 0.05). Analysis of ANP-like immunoreactive material by high performance liquid chromatography suggested that alpha-ANP is the major form of circulating ANP in blood of children with cardiac diseases.

[Clinical uses of digital subtraction angiography for imaging anatomy immediately following heart operations in childhood]. / Klinische Anwendungen der digitalen Subtraktionsangiographie zur Darstellung der Anatomie unmittelbar nach Herzoperationen im Kindesalter.

Within the last three years, digital subtraction angiography (DSA) was performed 60 times in 58 children with congenital heart disease (coarctation of the aorta, tetralogy of Fallot or transposition of the great vessels) in the immediate post-operative period to delineate residual defects. The DSA was carried out 3.7 +/- 1 days postoperatively with a total of 3.8 +/- 2.5 ml (0.47 +/- 0.2 ml/kg) Conray 70 or Solutrast 300 injected by hand into a catheter which generally had been positioned preoperatively at the transition between superior vena cava and right atrium. The outflow of the contrast medium was recorded on video tape and, additionally, single images of important anatomical details were obtained. In six patients with resected aortic coarctation, the patency of the entire aortic arch was documented with DSA; these patients had undergone surgery at a mean age of 3.3 +/- 4.8 years. Seven patients, with a mean age of 2.5 +/- 4.7 years, had tetralogy of Fallot, three additionally with pulmonary valve atresia; in all, postoperatively, the entire right ventricular outflow tract and the large pulmonary vessels could be demonstrated. In 45 patients with transposition of the great vessels, mean age 4.5 +/- 3.1 months, an atrial inversion with the Senning procedure was carried out; postoperatively, in all patients, the confluence of the superior and inferior vena cavae into the systemic venous portion of the atrium could be delineated. The pulmonary veins were visualized in one-third of the patients. In eight patients, DSA demonstrated obstruction at the opening of the superior vena cava into the atrium together with the collateral circulation via the azygos vein and the inferior vena cava.(ABSTRACT TRUNCATED AT 250 WORDS)

[Functional studies in rest and during exertion in children and adolescents with ventricular septal defects and pulmonary hypertension]. / Funktionelle Untersuchungen in Ruhe und während Belastung bei Kindern und Jugendlichen mit Ventrikelseptumdefekt und pulmonaler Hypertonie

7 children from 7-15 years of age, who had a large VSD with pulmonary arterial hypertension, were investigated on a bicycle ergometer during stepwise increasing load. In 5 cases a pulmonary artery banding had been performed earlier in spite of distinctly increased pulmonary vascular resistance. In two cases with Eisenmenger-syndrome no operative measures had been attempted. The measurements, which were carried out at rest and during two submaximal loads, comprised beside determination of oxygen uptake and heart frequency also the arteriovenous oxygen difference as well as mean arterial pressure in the pulmonary and the systemic circuit so that flow and resistance could be calculated for both circuits. During exercise a linear increase of flow in the pulmonary circuit was achieved in all cases, accompanied by a distinct rise of the mean pulmonary arterial pressure. The pulmonary vascular resistance stayed, however, generally constant. Systemic vascular resistance, on the contrary, decreased to about one third of the resting values so that the ratio of pulmonary arterial to systemic vascular resistance increased considerably. The result was a progressive increase of the right to left shunt during exercise, and in 3 cases with predominant left to right shunt at rest in spite of the band, a reversed shunt resulted, partially due to the effectiveness of the band, but substantially brought about by the undiminished high pulmonary vascular resistance. The results show that ergometric investigations in children with pulmonary arterial hypertension may contribute to clarify the indication for surgery, but they also confirm that generally a final operation is not possible in children whose banding-operation has been performed at the age of more than 2 years.