Primary repair versus surgical and transcatheter palliation in infants with tetralogy of Fallot.

OBJECTIVES: Treatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study. METHODS: This a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated. RESULTS: Patients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P<0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09). CONCLUSIONS: We found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure.

Surgery for simple and complex subaortic stenosis in children and young adults: results from a prospective, procedure-based national database.

OBJECTIVE: To identify the outcomes of surgically treated subaortic stenosis in a national population. METHODS: From 2000 to 2013, 1047 patients aged < 40 years underwent 1142 subaortic stenosis procedures. Of the 1047 patients, 484 (46.2%) were considered to have complex stenosis (CS) because at or before the first operation they had mitral valve (MV) disease, aortic valve disease, aortic coarctation or an interrupted aortic arch. RESULTS: The 30-day mortality was 0.7% for simple stenosis (SS), 2.3% for CS (P = .06), and 1.6% overall. Age < 1 year (P < .01), MV procedure (P = .02) and an interrupted aortic arch at the index procedure (P < .01) were risk factors for early death. Konno-type procedure early mortality was 2.4%. The 12-year survival was 97.1%, with a significant difference between SS and CS (hazard ratio [HR], 4.53; P = .02). Having MV disease alone (HR, 4.11; P = .02), MV disease plus aortic coarctation (HR, 6.73; P = .008), and age < 1 year (HR, 6.72; P < .001) were risk factors for late mortality. Freedom from subaortic reintervention overall was 92.3% and 88.5% at 5 and 12 years, respectively, much greater with CS than with SS (HR, 4.91; P < .0001). The independent risk factors for reintervention were younger age at the index procedure (HR, 0.1/y; P = .002), concomitant MV procedure (HR, 2.68; P = .019), ventricular septal defect plus interrupted aortic arch (HR, 3.19; P = .014), and ventricular septal defect plus aortic coarctation (HR, 2.41; P = .023). Undergoing a concomitant aortic valve procedure at the index procedure was protective (HR, 0.29; P = .025). CONCLUSIONS: Patients with SS had excellent outcomes. However, those with CS had worse long-term survival and freedom from reintervention, with morbidity and mortality greatest in young patients with multiple lesions. Additional evaluation in large-scale prospective studies is warranted.

Underrecognition of elevated blood pressure readings in children after early repair of coarctation of the aorta.

Hypertension after repair of coarctation of the aorta (CoA) is the outcome variable most closely associated with adverse long-term events such as stroke and myocardial infarction. This study sought to evaluate the outpatient management of casual blood pressure (BP) measurements in young children after early repair of CoA. A retrospective analysis was performed of clinical findings, echocardiographic data, casual BP recordings, and subsequent BP management of 114 children with CoA repair aged 1-13 years during 338 outpatient visits managed at two congenital cardiac centers. Children with associated significant congenital heart disease or corrective surgery after the age of 6 months were excluded from the study. Blood pressure was documented at 233 clinic visits (69%), and systolic BP (SBP) was above 95th percentile for age and sex in 45 instances (19%). This represented an elevated SBP recording for 31 children (27%), with two or more successive elevated recordings for 11 children (10%). Of 12 subjects receiving antihypertensive medication, three had inadequate BP control. Blood pressure is not documented at approximately 30% of outpatient visits of children with repaired CoA. When elevated BP is documented, in all cases no recorded action was taken. This may have significant implications for cardiovascular outcomes in this cohort of patients.

Surgical approach for aortic coarctation influences arterial compliance and blood pressure control.

BACKGROUND: Increased arterial stiffness is linked to hypertension in adults after surgical repair for coarctation of the aorta. We evaluated the influence of surgical approaches, namely, subclavian flap repair (SFR) and end-to-end anastomosis (EEA), on arterial stiffness, blood pressure, cardiac output, and cardiac baroreceptor function in a cohort of young children after coarctation repair to determine if the surgical approach influenced longer term blood pressure control. METHODS: We measured pulse wave velocity in 21 children with a mean age of 5 years, after early (less than 6 months) coarctation repair (SFR, n = 11; EEA, n = 10), and compared these with 18 matched controls. Blood pressure was recorded on three occasions from the right arm. Cardiac output was recorded using a transthoracic bioimpedence technique. We measured spontaneous baroreceptor reflex sensitivity to evaluate whether increased arterial stiffness was associated with reduced aortic baroreflex sensitivity. RESULTS: Right arm systolic blood pressure (108.3 + or - 3.5 mm Hg SFR versus 97.8 + or - 2.9 mm Hg EEA, p = 0.03) and pulse wave velocity (6.0 + or - 0.2 ms(-1) SFR versus 5.2 + or - 0.2 ms(-1) EEA, p = 0.02) were significantly greater in the SFR compared with EEA group. Blood pressure and pulse wave velocity were also higher in the SFR group compared with controls. These differences were not demonstrated when comparing the EEA group with controls. There was no difference in stroke volume, spontaneous baroreceptor reflex sensitivity, or heart rate or blood pressure variability between the groups. CONCLUSIONS: Young children undergoing SFR have higher blood pressure and stiffer upper limb arteries compared with matched children undergoing EEA. Our data suggest that better longer-term cardiovascular outcome is to be expected with the EEA surgical approach.

Balloon expandable stent implantation for native and recurrent coarctation of the aorta--prospective computed tomography assessment of stent integrity, aneurysm formation and stenosis relief.

BACKGROUND: Stenting for aortic coarctation is known to be effective in the medium term. Aneurysm formation following stent implantation is a recognised complication. However, data regarding aortic wall injury and stent integrity following stent placement are sparse. OBJECTIVES: We report comprehensive clinical, echocardiographic and prospective CT follow-up data following stenting for aortic coarctation from a single centre. Methods Full data analysis of all patients undergoing balloon expandable stent implantation and follow-up procedures in a single tertiary congenital cardiac unit. RESULTS: Between October 2002 and April 2008, we performed 102 coarctation stent procedures on 88 patients. Median age was 20.6 years (range 8.5-65) and median weight 65 kg (range 34-101). 94 stents (26 covered) were implanted. 12 procedures were re-dilatations. Stenting resulted in a reduction of the gradient across the site of coarctation, from a median of 20 mm Hg to 4 mm Hg. There were no procedure-related deaths. Four patients had immediate complications (one requiring emergency surgery). During median follow-up of 34.5 months (range 4.2-72.8), two patients had late complications requiring additional stent procedures. Follow-up CT data are available in 84 patients with MRI in one patient (96.5%). Only one patient developed a procedure-related aortic aneurysm. All stent fractures (n=7) occurred with a single stent design. CONCLUSIONS: Stenting for aortic coarctation and re-coarctation is effective with low immediate complication rates. CT is useful in the longer term for assessment of stent integrity and post-procedural aneurysm formation. Overall incidence of post-procedural aneurysm is rare and stent fractures were not seen with newer generation stents.

Self-expanding and balloon expandable covered stents in the treatment of aortic coarctation with or without aneurysm formation.

OBJECTIVES: We describe our complete experience with covered stent implantation for aortic coarctation including short- to medium-term outcomes. BACKGROUND: Coarctation of the aorta is a heterogeneous disease process with multiple associated complications both with and without treatment. Covered stents have evolved to provide greater support to the aortic wall and a varied approach with choice of stent tailored to the anatomy of the patient is required. METHODS: We retrospectively analyzed consecutive patient data from two congenital heart centers from March 2003 to October 2007. RESULTS: We implanted 38 covered stents in 37 patients (20 female) of which three were self-expanding stents. Median age was 29.6 years (9-65) with median weight of 71.5 kg (35-95). The indications for stent placement were native coarctation (n = 13); recurrent coarctation following surgical treatment (n = 11); aneurysm associated with previous coarctation surgery (n = 7); aorto-bronchial fistula leading to acute hemoptysis (n = 2); stent fracture (n = 1); associated arterial duct (n = 2). One patient had a combination of acquired coarctation and false aneurysm. The median screening time was 10 min (3-22). The median systolic gradient of 26 mm Hg (10-60) was reduced to 4 mm Hg (0-20) postprocedure (P < 0.001). There were no deaths on median follow up of 11.5 months (1-56). One patient developed aortic rupture during the procedure and required emergency surgery. CONCLUSION: Covered stent implantation for treatment of aortic coarctation is safe and highly effective in selected patients. Self-expanding stent grafts may be preferable to balloon expandable stents when there is aneurysm formation in the setting of aortopathy.