Balloon-expandable stents for recoarctation of the aorta in small children. Two centre experience.

BACKGROUND: In young patients with native aortic coarctation (CoA), the management of choice is surgery. However, in re-coarctation (re-CoA) surgery is associated with increased morbidity and even mortality. Some children with native CoA present relative contraindications for surgery. METHODS: From 2006 to 2017, thirty-four patients (male n = 20; 59%) from two centres with re-CoA (31) and native CoA (3) were managed by stent implantation with premounted balloon expandable stents. Inclusion criteria were age < 3 years and >1 month, weight < 16 kg. Median age was 6,5 months (min. 1; max. 34 months), median weight 6,2 kg (min. 3,7; max. 16 kg). Thirteen patients (38%) had Re-CoA and hypoplastic left heart syndrome (HLHS). In three patients (9%) the native CoA was stented due to contraindications for surgical treatment. RESULTS: All procedures were successful. The median peak invasive systolic pressure gradient declined from 31 mm Hg (max. 118; min. 4) to 0 mm Hg (max. 32; min.-7) (p < 0.001). The median minimal diameter of the narrowed segment of aorta increased from 3 mm (max. 6,9; min. 1,0) to 7 mm (max. 11,5; min. 3,5) (p < 0.001). There were no serious complications. The median follow-up time was 12,5 months (max. 88; min. 0 month). During this time ten patients (29%) required re-dilatation and two of them re-stenting. CONCLUSION: Percutaneous stent implantation for Re-CoA and in selected patients for native CoA can be performed successfully in very young patients with a good immediate hemodynamical result. However, repeated stent angioplasties and further on interventional 'opening' of the stent is necessary to augment the aorta to adult size.

Early pulmonary arterial hypertension immediately after closure of a ventricular or complete atrioventricular septal defect beyond 6months of age.

BACKGROUND: Pulmonary arterial hypertension (PAH) is virtually absent after closure of ventricular septal defect (VSD) in the first six months of life. However the prevalence of PAH in patients, who underwent VSD closure later, is not clear. The aim of this study was to analyse the prevalence of PAH after a successful VSD closure after the age of 6months and whether there are risk factors for developing PAH. METHODS: Echocardiographic and right heart catheter data of patients with VSD or complete atrioventricular septal defect, who underwent VSD closure after the age of 6months in our institution between 01/2005 and 06/2014, were retrospectively analyzed. PAH was defined as mean pulmonary arterial pressure (mPAP) of ≥25mmHg or tricuspid regurgitation jet velocity of ≥3.5m/s. RESULTS: In 228 patients (median age at shunt closure 4.0years, range 0.5-69) and 174 complete follow-up data (median follow-up 3.7years, range 0.5-39.4), 9 patients needed pulmonary vasodilator therapy after shunt closure, 4 of them temporarily for up to 79months. Three patients are still on vasodilator treatment 1, 2.6 and 6years after surgery, other two were lost to follow-up. Another 6 patients with preoperatively borderline hemodynamics due to elevated mPAP and pulmonary vascular resistance, recovered well without signs of postoperative PAH. CONCLUSION: With the current practice for safe late VSD closure, PAH is very rare at least in the first years of follow-up. In most patients with perioperative PAH, this condition appears to be transient and shows good response on pulmonary vasodilator treatment.

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.

Predictors for biventricular repair in pulmonary atresia with intact ventricular septum.

BACKGROUND: Pulmonary atresia with intact ventricular septum (PA-IVS) is a complex congenital heart defect with a large variety of right heart-sided morphologies. METHODS: We undertook a retrospective review of 86 patients with PA-IVS with a special emphasis on the angiographic findings. The aim of the study was to determine predictors for biventricular repair. Initial surgical procedures depended on the right ventricular morphology, the tricuspid valve size and coronary anomalies. RESULTS: Fifty-five patients (64%) underwent decompression of the right ventricle (RV) as an initial procedure; 16 of them required an additional systemic-to-pulmonary artery shunt. Twenty-six patients (30%) had only a systemic-to-pulmonary artery shunt as their initial procedure. Five patients underwent interventional procedures performed by pediatric cardiologists. Biventricular repair was possible in 56 patients (65%). Univentricular palliation was achieved in 16 patients. Fourteen patients had only palliation with a systemic-to-pulmonary artery shunt. Mean tricuspid valve size was significantly bigger in patients with biventricular repair (z-score -3.6 +/- 2.6) than in patients who did not undergo biventricular repair (-5.2 +/- 1.7, P = 0.003). Predictors for biventricular repair were right ventricular decompression with or without systemic-to-pulmonary artery shunt ( P < 0.001), tripartite right ventricle ( P < 0.001) and the absence of coronary fistulae ( P < 0.001). Long-term survival was 80% +/- 13% at 25 years for patients undergoing biventricular repair. CONCLUSIONS: Decompression of the RV as an initial surgical procedure improves the possibility of achieving biventricular repair with good long-term results. However, morphological factors such as right ventricular size and the absence of coronary fistulae are significant predictors for biventricular repair.

Cardiac index monitoring by pulse contour analysis and thermodilution after pediatric cardiac surgery.

OBJECTIVES: To validate a new device (PiCCO system; Pulsion Medical Systems, Munich, Germany), we compared cardiac index derived from transpulmonary thermodilution and from pulse contour analysis in pediatric patients after surgery for congenital heart disease. We performed a prospective clinical study in a pediatric cardiac intensive care unit of a university hospital. METHODS: Twenty-four patients who had had cardiac surgery for congenital heart disease (median age 4.2 years, range 1.4-15.2 years) were investigated in the first 24 hours after admission to the intensive care unit. A 3F thermodilution catheter was inserted in the femoral artery. Intracardiac shunts were excluded by echocardiography intraoperatively or postoperatively. Cardiac index derived from pulse contour analysis was documented in each patient 1, 4, 8, 12, 16, 20, and 24 hours after admission to the intensive care unit. Subsequently, a set of three measurements of thermodilution cardiac indices derived by injections into a central venous line was performed and calculated by the PiCCO system. RESULTS: The mean bias between cardiac indices derived by thermodilution and those derived by pulse contour analysis over all data points was 0.05 (SD 0.4) L x min x m(-2) (95% confidence interval 0.01-0.10). A strong correlation between thermodilution and contour analysis cardiac indices was calculated (Pearson correlation coefficient r = 0.93; coefficient of determination r2 = 0.86). CONCLUSIONS: Pulse contour analysis is a suitable method to monitor cardiac index over a wide range of indices after surgery for congenital heart disease in pediatric patients. Pulse contour analysis allows online monitoring of cardiac index. The PiCCO device can be recalibrated with the integrated transpulmonary thermodilution within a short time frame.

Tissue Doppler echocardiography before and after the surgical reconstruction of an insufficient mitral valve in a patient with Fontan circulation.

We report a patient with tricuspid atresia after total cavopulmonary connection who developed significant mitral valve regurgitation. Doppler myocardial echocardiography tracings showed "pseudonormalization" of diastolic velocities. After successful surgical reconstruction of the mitral valve, the diastolic myocardial velocities changed to the typical Fontan physiology with predominant late diastolic ventricular filling.

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.

Myocardial perfusion and coronary flow reserve assessed by positron emission tomography in patients after Fontan-like operations.

Ventricular dysfunction in patients after Fontan-like operations (FLOs) is a serious complication that might contribute to poor long-term results. Ischemic heart disease will have debilitating consequences on a Fontan heart. Ten patients (15.8 +/- 5.01 years) after FLO had transesophageal echocardiography and cardiac catheterization 9.3 +/- 4.2 years after surgery. Myocardial perfusion was assessed by NH3-positron emission tomography (rest/adenosine) and compared with that of 10 healthy adults (26.1 +/- 6.3 years). Ventricular function was normal in 4 and reduced in 6 patients; end systolic and end diastolic meridional wall stress was significantly elevated in the FLO group. Coronary angiography revealed no stenosis of the coronaries. Compared to normals, myocardial blood flow (MBF) at rest was higher in the FLO group (0.99 +/- 0.25 vs 0.77 +/- 0.17 ml/g/min, p <0.05), whereas MBF after vasodilatation (2.12 +/- 0.78 vs 3.10 +/- 0.85 ml/g/min, p <0.05) and coronary flow reserve (CFR) was reduced (2.5 +/- 0.88 vs 4.1 +/- 1.01, p <0.05), especially in those with impaired ventricular function. Coronary vascular resistance after vasodilatation was elevated in the FLO group (38.2 +/- 17.4 vs 24.5 +/- 8.3 mmHg/ml/g/min, p <0.05). Altered MBF, increased meridional wall stress, and impaired CFR are common findings in FLO. Attenuated CFR and reduced ventricular function are significantly correlated and may be risk factors for the long-term outcome.

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.

Single centre experience on primary correction of common arterial trunk: overall survival and freedom from reoperation after more than 15 years.

OBJECTIVE: To identify predictors of early and late outcomes of common arterial trunk (CAT) after primary surgical correction, such as clinical state prior to surgery, age and weight at presentation, implications of truncal valve abnormalities and associated anomalies of this complex congenital malformation. METHODS: A consecutive series of 106 patients, operated between 1976 and 1998, were reviewed retrospectively. Using the 'Van Praagh' classification, 59 patients presented as A1, 33 as A2, six as A3, and eight as A4. The mean age at operation was 8.6 months (range, 14 days-8.9 years; median, 4.4 months), and the mean weight was 5.2 kg (range, 2.5-30.8 kg; median, 4.4 kg). At the time of operation, 32 patients had congestive heart failure, five were on ventilator support for less than 1 week, and 21 for more than 1 week. RESULTS: Between 1976 and 1989, the early mortality was 21%, and between 1990 and 1998, it dropped to 13%. After 1, 10 and 15 years, the overall survival was 66, 61 and 59%. The 1, 5, 10 and 15 year freedom from reoperation was 82, 60, 22 and 10%, respectively. Clinical condition prior to intervention, truncal valvar dysfunction, and coronary anomalies were significantly associated with poor surgical outcome, whereas weight and age at presentation in our series were not. CONCLUSIONS: In the current era of paediatric cardiac surgery, primary surgical repair of CAT can be carried out with reasonable early and late mortality. However, our data suggest that a high incidence of reoperation, mainly due to the outgrowth and failure of the conduit, has to be expected. The patient's clinical state after diagnosis is decisive for the timing of intervention.

Intracardiac thrombus formation after the Fontan operation.

OBJECTIVES: Intracardiac thrombus formation is suspected to be a specific sequela after the Fontan operation and is difficult to determine by means of routine transthoracic echocardiography. The aim of our study was to evaluate the occurrence of intracardiac thrombi in the different types of Fontan modifications and to identify predisposing risk factors. METHODS: We evaluated 52 patients who had undergone a Fontan-type operation and were free of symptoms regarding thrombosis as determined by transesophageal echocardiography. RESULTS: In 17 (33%) patients thrombus formation could be found without clinical evidence of thromboembolic complications. Neither underlying morphologic disease nor age at operation, type of Fontan operation, sex, follow-up interval, arrhythmias, or laboratory or hemodynamic findings could be identified as predisposing risk factors. CONCLUSION: In patients having had a Fontan operation with inadequate or without anticoagulation medication, we would recommend routine transesophageal echocardiography to exclude eventual thrombi. Because of the high incidence of thrombi, we suggest oral anticoagulation therapy in all patients.

Repair of interrupted aortic arch: results after more than 20 years.

BACKGROUND: This study focused on the influence of concomitant anomalies, the individual surgical approach, and the probability for reinterventions. METHODS: Between 1975 and 1999, 94 patients with interrupted aortic arch were evaluated for short- and long-term results after surgical treatment. RESULTS: Interrupted aortic arch was associated mainly with a ventricular septal defect (85%) and left ventricular outflow tract obstruction (LVOTO, 13%). Mean follow-up was 6.7 years (median 6.9 years, 628.4 patient years). A single-stage operation was performed in 76 cases. Early mortality for two-stage procedures was 37% and late mortality was 26%, compared with single-stage procedures, with an early mortality of 12% and a late mortality of 20%, respectively. Early mortality in patients with additional LVOTO was 42% and late mortality was 50%. Freedom from reoperation at 5 years was 62%, and at 10 years was 49%. Reinterventions were performed mainly for residual arch stenosis, also with bronchus or tracheal compression, or LVOTO. CONCLUSIONS: Arch continuity and repair of associated anomalies can be achieved with an acceptable overall risk in this often complex entity. Associated anomalies play an important role in the outcome. Single-stage repair with primary anastomosis of the arch should be the surgical goal. The long-term probability for reoperation is high.

[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.