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.

[Pulmonary hypertension in grown-ups with congenital heart disease: Recommendations of the Cologne Consensus Conference 2016]. / Pulmonale Hypertonie bei Erwachsenen mit angeborenen Herzfehlern: Empfehlungen der Kölner Konsensus-Konferenz 2016.

The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for different forms of PH, and specifically address PH associated with congenital heart disease (CHD). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH in grown-ups with congenital heart disease (GUCH). This article summarizes the results and recommendations of this working group.

Successful treatment of a newborn with acute myocardial infarction on the first day of life.

Cardiogenic shock occurring after acute neonatal myocardial infarction (MI) due to coronary artery thrombosis is very rarely encountered. Acute neonatal MI typically presents suddenly with usually a fatal outcome. Treatment options in patients with this condition are limited. There are previous case reports in the literature advocating the use of extracorporeal membrane oxygenation for hemodynamic support. In this report, we present a newborn with severe MI secondary to thrombus formation within the left anterior descending coronary artery. There also proved to be a Factor V Leiden heterozygotic mutation. The patient initially presented with cardiogenic shock. After resuscitation and thrombolytic therapy were administered, coronary artery patency was restored resulting in myocardial revitalization and recovery of left-ventricular function within 4 weeks.

Primary resection of Kommerell's diverticulum in a 37-year-old patient.

A previously non-operated 37-year-old patient presented with esophageal dysphagia for solid food and stridorous breathing. Computed tomography and magnetic resonance showed a 33-mm wide Kommerell's diverticulum. The diverticulum and the obliterated ductus arteriosus arising from it towards the pulmonary artery were resected. The left subclavian artery, which originated from the diverticulum, was reimplanted into the descending aorta. In addition to dissecting any tissue which is making vascular ring-like structures around the trachea and the esophagus, it is important to resect the diverticulum itself and reimplant the left subclavian artery arising from it, in this way leaving the aortic arch free from any additional dislocating forces.

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.

Progressive intrapulmonary shunting in a child after bidirectional Glenn operation only resolved after total cavopulmonary completion.

We report on a girl with progressive intrapulmonary shunting after bidirectional Glenn (BDG) operation and resolution of these microscopic fistulas after completion to total cavopulmonary connection (TCPC).

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.

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.

Right heart failure due to an unroofed coronary sinus in an adult.

An unroofed coronary sinus is a rare congenital malformation with a strong association to a persistent left vena cava superior. In most cases it occurs together with complex cardiac anomalies such as left isomerism. We report on a 72-year-old man with clinical signs of right heart failure caused by an isolated unroofed coronary sinus: The diagnosis was made by means of transesophageal echocardiography with contrast injection.

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.

Impaired left ventricular function after arterial switch operation: exclusion of significant coronary artery stenosis with an intravascular Doppler guidewire.

Patients after arterial switch operation for transposition of the great arteries are at risk for coronary artery stenosis or obstruction due to intraoperative manipulation. This case describes an infant with impaired left ventricular function 8 months after arterial switch operation. A hemodynamically significant left coronary artery stenosis was excluded by determination of coronary flow reserve using an intravascular Doppler guidewire.