Long-Term Results of Congenital Aortic Stenosis Treatment in the Era of Percutaneous Balloon Valvuloplasty: Up to 33 Years Follow-Up.

Background The goal of this study was to evaluate long-term results of percutaneous balloon valvuloplasty (BVPL) used exclusively for initial management of congenital aortic stenosis in children. Methods and Results A total of 409 consecutive pediatric patients (134 newborns, 275 older patients) who underwent BVPL as initial treatment of aortic stenosis in a single nationwide pediatric center were subjected to a retrospective follow-up study. The resulting follow-up time reached a median of 18.5 (interquartile range, 12.2-25.1) years. Successful BVPL was defined by residual Doppler gradient <70/40 (systolic/mean) mm Hg. The primary end point was death; secondary end points included any valve reintervention, balloon revalvuloplasty, any aortic valve surgery, and aortic valve replacement, respectively. BVPL effectively reduced the peak and mean gradient both immediately and at the latest follow-up (P<0.001). There was significant procedure-related progression of aortic insufficiency (P<0.001). Higher aortic annulus z score was predictive for severe aortic regurgitation (P<0.05) and lower z score for insufficient gradient reduction (P<0.05). The actuarial probability of survival/survival free from any valve reintervention was 89.9%/59.9%, 85.9%/35.2%, and 82.0%/26.7% at 10, 20, and 30 years after first BVPL, respectively. Left ventricular dysfunction or arterial duct dependency as the indication for BVPL was predictive of both worse survival and survival free from any reintervention (P<0.001). Lower aortic annulus z score and lower balloon-to-annulus ratio were predictive of a need for revalvuloplasty (P<0.001). Conclusions Percutaneous BVPL provides good initial palliation. In patients with hypoplastic annuli and left ventricular or mitral valve comorbidity, the results are less favorable.

Influence of fenestration on long-term Fontan survival.

INTRODUCTION: Fenestration in the total cavopulmonary connection system may improve the outcome of patients with significant risk factors for Fontan haemodynamics. Our study aims to analyse the difference in long-term survival between non-fenestrated and fenestrated patients. METHODS: All consecutive patients (n = 351) who underwent total cavopulmonary connection between 1992 and 2016 were identified. Six early deaths were excluded resulting in a group of 345 patients. Median (interquartile range,) length of follow-up was 14.4 (7.1-19.7) years. Freedom from the composite endpoint of death, total cavopulmonary connection take-down or indication for a heart transplant was analysed. RESULTS: Fenestration was absent in 237 patients (68.7%, Group 1), was created and closed later in 79 patients (22.9%, Group 2), and remained open in 29 patients (8.4%, Group 3). Mean survival probability until composite endpoint was 97.1 and 92.9% at 10 and 20 years, respectively. Patients with patent fenestration had worse survival (p < 0.001) as compared to both the non-fenestrated and fenestration closure groups. Despite a similar outcome, exercise capacity was lower in Group 2 than 1 (p = 0.013). In 58 patients with interventional fenestration closure, Nakata index was lower at the time of closure than pre-operatively, and both the pressure in the circuit and oxygen saturation in the aorta increased significantly (p < 0.001). CONCLUSIONS: Patients with persisting risk factors preventing fenestration closure are at higher risk of reaching the composite endpoint. Patients after fenestration closure have the worse functional outcome; their survival is, however, not different from the non-fenestrated group.

Unexpected ischemia during transcatheter patent arterial duct closure.

A child undergoing routine transcatheter patent arterial duct closure developed severe transient ischemic changes in the electrocardiogram (Pardee waves) while the aortic retention skirt of the Amplatzer™ Duct Occluder was pulled against the duct orifice. The occluder was then released, and the delivery system was pulled back to inferior caval vein which led to electrocardiogram normalisation. Aortic root angiography showed a single coronary artery originating from the right sinus of Valsalva with the left coronary stem wedged behind the posterior aspect of the right ventricular outflow tract. We believe that the left coronary artery was compressed while applying tension on the occluder delivery system.

Improved results after the primary repair of interrupted aortic arch: impact of a new management protocol with isolated cerebral perfusion.

OBJECTIVE: The aim of this retrospective study was to analyse results and risk factors of death after the repair of the interrupted aortic arch, and to compare results obtained with the use of deep hypothermic circulatory arrest versus isolated cerebral perfusion. METHODS: The primary repair of the interrupted aortic arch and associated heart lesions was performed in 50 consecutive patients. The median age was 5 days and the mean weight was 3.1+/-0.6 kg. The interrupted aortic arch was of type A in 12 (24%) patients, type B in 37 (74%) and type C in one (2%) patient. Ventricular septal defect was present in 48 (96%) patients, subaortic stenosis in 15 (30%), truncus arteriosus in 14 (28%), transposition of the great arteries in two (4%), aortopulmonary window in two (4%) and double-outlet right ventricle in one (2%). The surgery consisted of reconstruction of the aortic arch by direct anastomosis and repair of associated heart lesions. In 25 (50%) patients, aortic arch reconstruction was performed using hypothermic circulatory arrest (group I) and in 25 by isolated cerebral perfusion (group II). The duration of cardiopulmonary bypass, aortic cross-clamping and circulatory arrest or isolated cerebral perfusion was 191+/-46 min, 90+/-24 min and 40+/-14 min, respectively, in group I; and 194+/-39 min, 74+/-20 min and 31+/-6 min, respectively, in group II. RESULTS: There were 10 (20%) deaths in this series, eight (32%) in group I and two (8%) in group II. Out of 12 patients operated before 1995, seven (58%) patients died; and out of 38 patients operated between 1995 and 2009, three (8%) patients died (p=0.008). By Cox multifactorial analysis, the earlier date of operation represented the only risk factor of death (p=0.037). Twelve (71%) survivors in group I and five (22%) survivors in group II required re-intervention, most often for subaortic stenosis, aortic arch obstruction or conduit obstruction. All patients remain in the New York Heart Association (NYHA) class I or II at median 12.6 years in group I, and 1.7 years in group II, respectively, after surgery. CONCLUSIONS: Interrupted aortic arch can be repaired in neonates with a mortality of 5-10%. The results depend on experience. Isolated cerebral perfusion was joined with decreased mortality but it did not influence the occurrence of neurological complications.

Long-term results after correction of persistent truncus arteriosus in 83 patients.

OBJECTIVE: This study aims to analyse long-term results after correction of truncus arteriosus in all patients operated in one institution over 28 years. METHODS: Between 1981 and 2009, 83 patients, median age 54 days, underwent repair of truncus arteriosus. Interrupted aortic arch was present in 14 (17%), severe truncal valve insufficiency in 10 (12%) and non-confluent pulmonary arteries in five (6%) patients. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in 80, and other methods in three patients. At the same time, correction of interrupted aortic arch was done in 14 and truncal valve repair in eight patients. Survivors were repeatedly examined echocardiographically for assessment of residual heart lesions. RESULTS: The early mortality was 19 (23%). Out of 35 patients operated between 1981 and 1996, 17 (46%) died, and out of 48 patients operated between 1997 and 2009, two (4%) died. Operation before 1997 (p=0.001) and aortic cross-clamping time >90min (p=0.009) were found to be risk factors of death. Eight (10%) patients died late, a median of 68 days after surgery. Fifty-seven (69%) patients were followed for 10.9 + or - 6.7 years. Three (4%) patients were lost. Twenty-five (30%) patients are alive with their original conduit 7.5 + or - 5.2 years after correction. Twenty-eight patients required 41 re-operations for conduit dysfunction with first replacement at mean 5.8 + or - 4.1 (range 0.1-14.1 years) years after correction. Nine (11%) patients required 12 truncal valve replacements. Eleven (13%) patients required balloon dilatation or stent for conduit obstruction, pulmonary branch stenosis, aortic arch obstruction or stenosis of vena cava. Recent clinical examination was undertaken in 53 (64%) patients and 49 (59% or 77% of early survivors) are in good/very good condition. CONCLUSIONS: Truncus arteriosus remains a challenging heart disease. With growing experience, the early mortality decreased to 4%, but numerous re-interventions for conduit obstruction, pulmonary branch stenosis and truncal valve insufficiency are required. Surgery before 1997 and prolonged cross-clamping were risk factors of death. Pulmonary homografts had the best re-intervention-free survival. Statistically, however, the difference between conduits was not significant. Dysplastic valve and truncal valve insufficiency represent risk factors presenting the need for truncal valve replacement.

Haemodynamic changes due to delayed sternal closure in newborns after surgery for congenital cardiac malformations.

BACKGROUND: Delayed sternal closure is used to prevent low cardiac output syndrome in selected newborns shortly after cardiac surgery for congenital cardiac defects. Sternal closure itself often causes haemodynamic and ventilatory instability that cannot be entirely assessed by standard monitoring means. Therefore, we used transpulmonary thermodilution technique for an exact evaluation of the haemodynamic changes. PATIENTS AND METHODS: Between April, 2006, and December, 2008, 23 neonates aged from 1 to 30 days, with a median of 7 days, and weighing from 1.9 to 4.2 kilograms, with a median of 3.25 kilograms, were studied after biventricular corrections. Residual intracardiac shunts were excluded by echocardiography. Haemodynamic and ventilatory parameters, along with those obtained by the transpulmonary thermodilution technique, were recorded before and immediately after the sternal closure, and then at 0.5, 1, 2, 6, 12, 24, and 48 hours. RESULTS: Chest closure caused significant decrease in systolic arterial pressure from 80.04 +/- 11.48 to 69.48 +/- 9.63 mmHg (p < 0.001), cardiac index from [median (25th/75th centile)] 2.640 (2.355/2.950) to 2.070 (1.860/2.420) l/min/m2 (p < 0.001), stroke volume index from 18.50 (16.00/20.00) to 14.00 (11.00/17.00) ml/m2 (p < 0.001), and dynamic lung compliance from 2.45 (2.31/3.00) to 2.30 (2.14/2.77) ml/cmH2O (p = 0.007). Stroke volume variation increased from 14.00 (9.25/16.75) to 18.00 (15.00/21.00) % (p < 0.001). The oxygenation index transitorily increased from 2.50 (2.14/3.15) to 3.36 (2.63/4.29) (p < 0.001). Serum lactate decreased from 1.40 (1.12/2.27) to 1.0 (0.8/1.3)mmol/l, p < 0.001 in coincidence with a haemodynamic stabilisation at a later time after chest closure. Cardiopulmonary instability caused by the sternal closure necessitated therapeutic intervention in 18 of 23 patients (78.3%). CONCLUSION: Delayed sternal closure causes a significant transitory decrease in stroke volume, cardiac output and arterial blood pressure. Also lung compliance and blood oxygenation are temporarily significantly compromised.

Normal limits for heart rate as established using 24-hour ambulatory electrocardiography in children and adolescents.

BACKGROUND: To the best of our knowledge, normal limits of heart rate with respect to gender, and as established using 24-hour ambulatory Holter electrocardiography, have yet to be published for the entire age range of children and adolescents. OBJECTIVES: To establish the normal limits for heart rate in newborns, infants, children, and adolescents of both genders. PATIENTS AND METHODS: We obtained 24-hour Holter recordings from 616 healthy subjects aged from birth to 20 years with structurally normal hearts. The subjects were not receiving medication, and had not been submitted to prior cardiac intervention. Off-line analysis was performed with Mars 8000 scanners, analysing 5 consecutive RR intervals by the software available for automatic calculation of heart rate. All subjects were in sinus rhythm. Best-fit non-linear regressions were applied to correlate age and gender with minimum and mean heart rate, as well as with maximal RR-interval, and to calculate the 5th, 25th, 75th and 95th percentiles. RESULTS: We observed significant gender-dependent differences in heart rate for persons aged 10 years and older, with the males exhibiting lower minimal and mean heart rates, and higher RR-intervals, than the females. Correlation of heart rate with age and gender could be established with sufficient accuracy using non-linear regression (p less than 0.0001): Minimum heart rate (male: R(2)=0.778, female: R(2) = 0.664) and mean heart rate (male: R(2) = 0.820, female: R(2) = 0.736) decreased with age, while the maximal RR-interval prolonged (male: R(2) = 0.562, female: R(2) = 0.486). Age and gender-related graphs of centiles were constructed. CONCLUSIONS: Heart rate, as documented using Holter recodings, can be correlated with age and gender, permitting establishments of normal gender-specific limits for children and adolescents.

Long-term (up to 20 years) results of percutaneous balloon angioplasty of recurrent aortic coarctation without use of stents.

AIMS: To assess the efficacy, safety, and long-term results of the balloon angioplasty of recoarctation. METHODS AND RESULTS: The angioplasty was performed in 99 consecutive patients aged 36 days to 32.6 years (median 268 days). Recoarctation to descending aorta diameter ratio increased from 0.44 (0.35/0.50) to 0.66 (0.57/0.77), P < 0.001. Systolic gradient was reduced from 34.0 (26.0/44.75) to 15.0 (8.25/27.0) mmHg, P < 0.001. In seven patients (7.1%) the procedure was ineffective. One patient (1%) with heart failure died within 24 h after a successful angioplasty and in another (1%) an intimal abruption necessitated surgical revision. The follow-up ranged up to 20.7 years (median 8.1 years). Actuarial probability of survival 20.7 years after the procedure was 0.91, and of reintervention-free survival was 0.44. Older age at the angioplasty was associated with a higher incidence of reinterventions (hazard ratio 1.057; 95% confidence interval 1.012-1.103; P = 0.010). The type of surgery and the recoarctation anatomy did not influence the outcome. In 69 patients aneurysm formation was studied by high-sensitive methods with only one positive finding per 462 patient-years. CONCLUSION: Angioplasty is safe and effective regardless of the type of surgery used and the recoarctation anatomy. Older age at the angioplasty is associated with a higher incidence of reinterventions.

Cardiac resynchronization therapy: a novel adjunct to the treatment and prevention of systemic right ventricular failure.

OBJECTIVES: This study aimed to evaluate the technical feasibility and hemodynamic benefit of cardiac resynchronization therapy (CRT) in patients with systemic right ventricle (RV). BACKGROUND: Patients with a systemic RV are at high risk of developing heart failure. Cardiac resynchronization therapy may improve RV function in those with electromechanical dyssynchrony. METHODS: Eight patients (age 6.9 to 29.2 years) with a systemic RV and right bundle-branch block (n = 2) or pacing from the left ventricle (LV) (n = 6) with a QRS interval of 161 +/- 21 ms underwent CRT (associated with cardiac surgery aimed at decrease in tricuspid regurgitation in 3 of 8 patients) and were followed-up for a median of 17.4 months. RESULTS: Change from baseline rhythm to CRT was accompanied by a decrease in QRS interval (-28.0%, p = 0.002) and interventricular mechanical delay (-16.7%, p = 0.047) and immediate improvement in the RV filling time (+10.9%, p = 0.002), Tei index (-7.7%, p = 0.008), estimated RV maximum +dP/dt(+45.9%, p = 0.007), aortic velocity-time integral (+7.0%, p = 0.028), and RV ejection fraction by radionuclide ventriculography (+9.6%, p = 0.04). The RV fractional area of change increased from a median of 18.1% before resynchronization to 29.5% at last follow-up (p = 0.008) without a significant change in the end-diastolic area (+4.0%, p = NS). CONCLUSIONS: The CRT yielded improvement in systemic RV function in patients with spontaneous or LV pacing-induced electromechanical dyssynchrony and seems to be a promising adjunct to the treatment and prevention of systemic RV failure.