Right superior vena cava draining into the left atrium in a patient with sinus venosus defect.

Right superior vena cava draining into the left atrium is an extremely rare anomaly of systemic venous return. It can be isolated or it can be associated with other congenital heart defects, thus clinical presentation may vary. A case of a 9-year-old asymptomatic patient with sinus venous defect and bilateral superior vena cava with the right-sided draining into the left atrium is described.

Large single centre experience with the Cera™ and CeraFlex™ occluders for closure of interatrial communications: usefulness of the flexible rotation feature.

We present our experience with the Cera (CO) and the CeraFlex occluder (CFO) in transcatheter closure of interatrial communications (IAC). Between 2013 and 2016, 201 patients (75 males, 16 with patent foramen ovale), aged 27 ± 19 (5-75) years, underwent percutaneous closure of IAC using CO or CFO in our institution. After transoesophageal imaging, the procedure was aborted in 7 young paediatric (6-13 years old) patients (3 multiple holes, 3 too small septum, 1 leak with 38 mm occlusion balloon). The occluder was removed prior to release in 11 patients (5.7%), while occlusion was successful in 183 patients (94.3%) with 44 CO, 136 CFO, and 3 Cera multifenestrated occluders. There were no deaths, embolizations, or major complications. Small residual shunts were demonstrated in 8 patients immediately after implantation, 4 (8.5%) with CO and 4 (2.9%) with CFO, all disappearing after 3 months. Over 1.8 ± 1.7 year follow-up, all patients improved with 2 asymptomatic, transient pericardial effusions and 5 adults with transient supraventricular arrhythmias, treated medically for 6 months. IAC closure with CO and CFO proved safe with favourable success rates and few, nonserious complications. The CFO flexible rotation feature helped in conforming to various septal anatomies, minimising manoeuvres and possibly post-occlusion leaks.

Ross procedure in a child with Aspergillus endocarditis and bicuspid aortic valve.

The case is presented of a previously healthy infant with a known asymptomatic bicuspid aortic valve who developed fungal endocarditis. The patient underwent aortic root replacement with a pulmonary autograft (Ross procedure). Cultured operative material revealed Aspergillus infection. The patient had an excellent recovery and remained well one year later.

Thirty-three years old modified senning operation.

Numerous technical modifications and various complications of the Senning procedure have been described in the literature. We describe the excellent clinical status and anatomic result of a 33-year-old patient who underwent a modified Senning operation using the left atrial appendage for reconstruction more than 30 years prior to presentation.

Diversity of congenital cardiac defects and skeletal deformities associated with the Holt-Oram syndrome.

INTRODUCTION: The Holt-Oram syndrome is a rare congenital disorder involving the skeletal and cardiovascular systems. It is characterized by upper limb deformities and cardiac malformations, atrial septal defects in particular. PRESENTATION OF CASE: Four consecutive patients 1-15 years old with the Holt-Oram syndrome presented over a 10 year span for surgical treatment of their cardiac maladies. The spectrum of the heart defects and skeletal deformities encountered in these patients are described and discussed. DISCUSSION: The Holt-Oram syndrome is an autosomal dominant condition; however absence of the morphological features of the trait in close family members is not rare. Although patients are known to predominately present with atrial septal defects, other cardiovascular anomalies, including rhythm abnormalities, are not uncommon. Skeletal disorders vary as well. CONCLUSION: Cardiovascular disorders, skeletal malformations and familial expression of the Holt-Oram syndrome, vary widely.

Ablation of atrial tachycardias with radiofrequency current after surgical repair of complex congenital heart defects.

INTRODUCTION: Atrial tachycardias are a common problem following the surgical repair of complex congenital heart defects (CHD) and have a poor response to medication. The aim of this study was to describe the results of the treatment of such tachycardias with radiofrequency ablation in our hospital. METHODS: A retrospective study was performed of the medical records and electronically stored data from electrophysiological studies (EPS) and ablation procedures in patients with atrial tachycardias following the surgical repair of CHD. Established electrophysiological techniques were used in all patients, while newer three-dimensional imaging methods were also employed in some cases. Ablation was carried out using a radiofrequency generator and catheters with a 4-10 mm terminal electrode. RESULTS: Twenty-two patients aged 11-45 years (30.6 +/- 10.8), including 9 women, underwent an EPS and ablation of atrial tachycardias after the surgical repair of CHD. All had recurrent tachycardias refractory to antiarrhythmic medication. The anatomical diagnoses were tetralogy of Fallot (8), transposition of the great arteries (6), single ventricle (3), and other (5). Most patients had typical atrial flutter and/or incisional atrial reentrant tachycardia. The initial success rate of ablation for all arrhythmias was 88% in patients with tetralogy of Fallot, 83% for transposition of the great arteries, 66% for single ventricle, and 80% for the other diagnoses. Three-dimensional imaging was of significant assistance in mapping and ablation and in reducing the duration of fluoroscopy. Two vascular complications (femoral arteriovenous fistulae) were observed. The tachycardia recurrence rate was 54%. After repeated procedures, 59% of patients were free of arrhythmias and antiarrhythmic medications. No patient with a single ventricle remained free of arrhythmias during follow up, while in the other groups the arrhythmia/drug-free rate was 66-75%. Two patients with unsuccessful ablation died, one from heart failure and the other because of the tachycardia. CONCLUSIONS: Atrial tachycardias in patients with surgically treated CHD are associated with increased morbidity and mortality. Radiofrequency ablation has satisfactory results. In spite of the high relapse rate, a significant number of patients can become free of arrhythmias after repeat procedures.

Doppler tissue imaging evaluation of right ventricular function at rest and during dobutamine infusion in patients after repair of tetralogy of Fallot.

BACKGROUND: Reliable, non-invasive evaluation of right ventricular function, especially in congenital heart disease, is challenging. OBJECTIVES: The aim of this study was to evaluate Doppler tissue imaging (DTI) parameters of lateral tricuspid annular motion, mean rate of pressure rise during RV contraction (mean dP/dt) and indexed right ventricular (RV) stroke volume (RVSVi) as RV function indices in repaired tetralogy of Fallot (TOF). METHODS: DTI evaluation was performed in 25 repaired-TOF patients, aged 11+/-6, at rest and during dobutamine infusion and 20 controls at rest. RESULTS: TOF patients had lower (P<0.05) peak systolic velocity (Sa) (11.4+/-4 vs. 13.7+/-3.1 cm/s), early diastolic velocity (Ea) (11+/-3.1 vs. 16.3+/-3.5 cm/s) and Sa/time from onset of QRS to Sa (Sa/Q-Sa) (68.8+/-26.4 vs. 92.3+/-29.4 cm/s2) versus controls. Dobutamine increased (P<0.01) Sa (11.4+/-2.8-17.7+/-4.7 cm/s), Ea (11+/-3.1-15.6+/-3.9 cm/s), late diastolic velocity (Aa) (8.4+/-2-14.8+/-5 cm/s), Sa/Q-Sa (68.8+/-26.4-17 6.8+/-84.5 cm/s2), mean dP/dt (180+/-74-537+/-37 2 mmHg/s), and RVSVi (7.8+/-3.9-11.9+/-5.6 L/min/m2). RVSVi increase correlated (P<0.01) with that in Sa (r=0.6), Ea (r=0.5), Sa/Q-Sa (r=0.71), and mean dP/dt (r=0.57) while mean dP/dt increase correlated strongly with Sa/Q-Sa increase (r=0.88). CONCLUSION: DTI evaluation of tricuspid annular motion during dobutamine infusion in repaired TOF correlates with dP/dt and RV stroke volume and may help in assessing RV function and reserve.

Nonfluoroscopic catheter navigation for radiofrequency catheter ablation of supraventricular tachycardia in children.

BACKGROUND: Radiofrequency catheter ablation (RCA) of supraventricular tachycardia (SVT) in children is highly successful but requires exposure to radiation. Nonfluoroscopic mapping systems may significantly reduce fluoroscopy time. METHODS: Forty consecutive pediatric patients who underwent RCA for accessory pathways (AP) or AV nodal reentrant tachycardia (AVNRT) with use of a nonfluoroscopic navigation system (Ensite NavX) (group A) were compared retrospectively to 40 consecutive patients with similar diagnoses who underwent RCA with fluoroscopic guidance only (group B). RESULTS: Group A (mean age 12.1+/-2.9 years, mean weight 47+/-13.9 kg) consisted of 11 patients (27.7%) with AVNRT and 29 (72.5%) with AP. Group B (mean age 10.9+/-3.1 years, mean weight 47.1+/-17.1 kg) consisted of 7 patients (17.5%) with AVNRT and 33 (82.5%) with AP. There were no significant differences in AP location, patients with congenital heart disease, and number of radiofrequency lesions. Fluoroscopy time was significantly shorter in group A than in group B (10.4+/-6.1, range 3.1-28.8 minutes, vs 24.9+/-16.0, range 4.4-82.0 minutes, P<0.0001). Procedure duration was also significantly shorter in group A than in group B (170+/-68.5, range 90-420 minutes, vs 218+/-69.3, range 90-360 minutes, P<0.0001). Initial success was 95% in group A and 100% in group B. Tachycardia recurrences occurred in two patients in group A (5%) and six patients in group B (15%). Final success, including repeat ablations for recurrences or failures, was 100% in both groups. CONCLUSIONS: The use of a nonfluoroscopic system for catheter navigation significantly reduced fluoroscopy exposure and total procedure duration of RCA of common SVT substrates in children.

Normative angiographic data relating to the dimensions of the aorta and pulmonary trunk in children and adolescents.

BACKGROUND: Definition of normative data of the great arteries from neonatal to adult ages may aid in assessment of the growth of cardiovascular structures, thus guiding the timing and type of intervention in patients with congenital cardiac disease. METHODS: We calculated the cross-sectional areas of the arterial roots at the basal attachment of the valvar leaflets, the sinuses, and standardized distal sites using cineangiograms of 59 normal children and adolescents with mean age of 5.4 plus or minus 4.7 years and a range from 0.1 to 16 years, the children having a mean weight of 21.2 plus or minus 15.7 kilograms, with a range from 2.2 to 68 kilograms, and mean height of 108 plus or minus 35 centimetres, with a range from 43 to 184 centimetres. Values at each site were calculated averaging end-diastolic and end-systolic measurements, and indexed to body surface area. Results are expressed as the mean plus or minus the standard deviation. RESULTS: The diameter of the aortic root at the basal attachment of the leaflets was 249 plus or minus 26, the midpoint of the sinuses 379 plus or minus 59, the sinutubular junction 290 plus or minus 58, the isthmus 158 plus or minus 36, the postisthmic region 152 plus or minus 33, and the descending aorta at the level of diaphragm 130 plus or minus 18 millimetres squared per metre squared. The pulmonary root measured at the basal attachment of the leaflets was 253 plus or minus 28, the midpoint of the sinuses 352 plus or minus 58, the sinutubular junction 293 plus or minus 58, the right pulmonary artery 176 plus or minus 25, the left pulmonary artery 153 plus or minus 20, and sum of right and left pulmonary arteries 330 plus or minus 37 millimetres squared per metre squared. All indexes were consistent over a wide range for body surface areas. CONCLUSIONS: Definition of normative data of the great vessels may aid in the evaluation of congenital or acquired abnormalities, serving as guidelines for intervention during medical or surgical management and follow-up.

Diverse experience with the CardioSEAL and STARFlex septal occluders.

Apart from closure of atrial septal defects, there is little information concerning the use of the CardioSEAL family of occluders in congenitally malformed hearts. We review here our initial experience using the CardioSEAL and STARFlex occluders in 12 patients aged 17.3 +/- 11.2 years, with a range from 4 to 34 years. Of the patients, 5 had fenestrated extracardiac Fontan procedures, 5 had persistent patency of the arterial duct, 1 had a leak across a Mustard baffle, and the final patient had a huge pulmonary arteriovenous malformation. We implanted successfully 9 CardioSEAL, and 3 STARFlex occluders, with sizes from 17 to 40 mm. In one patient, the occluder embolized to the right pulmonary artery, from where it was retrieved through the catheter. In two patients, there was a trivial residual leak immediately after implantation, but no patient had a residual leak after 6 months of follow-up. We noted improved ventricular dimensions, without any fractures of the arms of the occluders, perforations, or disturbances of flow after 2.4 +/- 0.9 years of follow-up. We have demonstrated, therefore, the versatility of the CardioSEAL and STARFlex occluders, which have been used safely and effectively to close a variety of intra and extracardiac communications other than atrial septal defects.