Transfemoral Perimembranous Ventricular Septal Defect Device Closure in Infants Weighing ≤ 10 kg.

Transcatheter closure of Perimembranous VSDs (PMVSD) remains challenging particularly in infants. The aim of this study is to evaluate the efficacy and safety of transfemoral PMVSD device closure in infants weighing ≤ 10 kg in a single centre. Retrospective review of departmental databases and medical charts to define patient cohort and collect demographic, procedural and follow-up data. Between July 2014 and March 2021, 16 patients underwent attempted transfemoral PMVSD device closure (12 retrograde) at a median age of 11 months (interquartile range [IQR] 9-15.5) and a median weight of 8.3 kg (IQR 7.2-9.5). All patients were either symptomatic, had progressive left heart dilation or had VSD associated valve regurgitation. Median defect size on pre-procedural transoesophageal echocardiography was 6.8 mm (IQR 6-8.5). Median device waist size was 6 mm (IQR 4.5-8). Successful device placement was achieved in 14 patients (88%). One patient developed moderate aortic and tricuspid valve regurgitation upon retrograde and antegrade device deployment, respectively, and subsequently underwent surgical closure. The second patient developed progressive aortic regurgitation (AR) 2 days post procedure, and also underwent surgical removal with no residual AR. There was no cases of device embolization and no femoral arterial compromise. On median follow-up of 40.5 months (IQR 25-64), none of the patients developed complete heart block. Three patients (18.75%) had small residual shunts at latest follow-up which have not required any further intervention. Device closure of PMVSD's in children weighing ≤ 10 kg is feasible and safe with good procedural success rates. Use of both the antegrade and retrograde approaches may be necessary depending on anatomical variances.

Short- and medium-term outcomes for patent ductus arteriosus stenting in neonates ≤2.5 kg with duct-dependent pulmonary circulation.

BACKGROUND: Morbidity with surgical systemic-to-pulmonary artery shunting (SPS) in infants ≤2.5 kg has remained high. Patent ductus arteriosus (PDA) stenting may be a valid alternative. The objective of this study is to evaluate outcomes following PDA stenting in patients ≤2.5 kg from four large tertiary centers. METHODS: Retrospective review of all neonates ≤2.5 kg with duct-dependent pulmonary circulation who underwent PDA stenting. Procedural details, pulmonary arterial growth, reinterventions, surgery type, and outcomes were assessed. RESULTS: PDA stents were implanted in 37 of 38 patients attempted (18 female) at a median procedural weight of 2.2 kg (interquartile range [IQR], 2-2.4 kg). Seven patients (18%) had a genetic abnormality and 16 (42%) had associated comorbidities. The median intensive care unit stay was 4 days (IQR, 2-6.75 days), and the median hospital stay was 20 days (IQR, 16-57.25). One patient required a rescue shunt procedure, with three others requiring early SPS (<30 days postprocedure). Twenty patients (54%) required reintervention with either balloon angioplasty, restenting, or both. At 6-month follow-up, right pulmonary artery growth (median z-score -1.16 to 0.01, p = 0.05) was greater than the left pulmonary artery (median z-score -0.9 to -0.64, p = 0.35). Serious adverse effects (SAEs) were seen in 18% (N = 7) of our cohort. One patient developed an SAE during planned reintervention There were no intraprocedural deaths, with one early procedure-related mortality, and three interstage mortalities not directly related to PDA stenting. CONCLUSIONS: PDA stenting in infants ≤2.5 kg is feasible and effective, promoting pulmonary artery growth. Reintervention rates are relatively high, though many are planned to allow for optimal growth before a definitive operation.

Transcarotid Approach to Ventricular Septal Defect Closure in Small Infants.

Perimembranous ventricular septal defect closure in small infants has traditionally been a surgically treated defect, although alternative hybrid strategies are emerging. We aim to describe a novel approach to retrograde device closure of clinically relevant perimembranous ventricular septal defects in small infants via carotid cutdown. A retrospective review of all patients managed with attempted carotid cutdown for device closure of a perimembranous ventricular septal defect was recorded at a single tertiary cardiac centre. We summarized data on successful device deployment, conversion to open repair, complications, and length of stay. Eighteen infants with median (IQR) age of 7 months (5-9 months) and weight of 7.1 kg (6.5-7.8 kg) with clinically relevant PMVSD underwent attempted retrograde closure via carotid cutdown. Median (IQR) defect size was 8 mm (7-9 mm). Successful device deployment without significant aortic or tricuspid valve interference occurred in 15 (83%) patients. Three patients were converted to open repair, one following damage to the tricuspid valve apparatus. Median (IQR) hospital stay was 1 day (1-3 days). There were no complications related to carotid cutdown. Retrograde device closure of hemodynamically significant PMVSD is feasible and effective in small infants. Decision to convert to surgical repair should be made early if suboptimal device placement occurs. Carotid evaluation should be performed to rule out any access-related complications.

Procedural technique for hybrid pulmonary valve replacement in infants and small children.

OBJECTIVES: Hybrid approach to pulmonary valve replacement (PVR) in the paediatric population has been reported, although data in infants and small children are limited. Several strategies are now possible. The aim of this study is to review our hybrid PVR strategy in a complex patient cohort, outlining a variety of approaches employed in our centre. METHODS: We performed a retrospective review of infants and small children who underwent hybrid PVR between May 2017 and April 2019 in a single tertiary cardiology centre. Medical records were reviewed to ascertain demographic, clinical and outcome data. RESULTS: Ten patients with a median (interquartile range) age of 1.5 years (1.1-1.9) and weight of 8.8 kg (8-10.6) were managed with hybrid pulmonary valve insertion. Eight patients had perventricular approach (4 sternotomy and 4 subxiphoid) and 2 patients had surgically sutured valve. Six patients underwent cardiopulmonary bypass for associated lesions. Three had insertion of the valve into conduits and 7 were deployed into native right ventricular outflow tracts. The pulmonary valve was successfully inserted in all 10 patients with no mortality. Postprocedural complications included paravalvar leak in 2 patients, suspected endocarditis in 1 patient who developed early valve regurgitation and wound infection in 1 patient. CONCLUSIONS: Several approaches to hybrid PVR may be employed in small children with a high success rate. Follow-up studies are required to evaluate longer term durability of these approaches compared to standard surgical replacement.

Percutaneous transcatheter occlusion of left atrioventricular valve in a patient with single ventricle and severe atrioventricular valve regurgitation.

Many patients with single ventricle physiology suffer from atrioventricular valve (AVV) regurgitation which may worsen their cardiac function and cause symptoms. It has been postulated that elimination of the nondominant hypoplastic AVV regurgitation, might improve the clinical status in patients post-Fontan surgery. We describe a case of hypoplastic left heart variant, post Fontan surgery who had severe left AVV regurgitation and underwent percutaneous transcatheter occlusion of the hypoplastic left AVV, using a VSD occluder device. At 3 months post procedure, the patient is improved. Transcatheter closure of a regurgitant hypoplastic AVV in a patient with single ventricle helps to improve the patient's cardiac function and clinical status.

Short to medium term outcomes of right ventricular outflow tract stenting as initial palliation for symptomatic infants with complete atrioventricular septal defect with associated tetralogy of Fallot.

OBJECTIVES: To assess the impact of right ventricular outflow tract (RVOT) stenting as the primary palliation in infants with complete atrioventricular septal defect with associated tetralogy of Fallot (cAVSD/TOF). BACKGROUND: Historically, palliation of symptomatic patients with cAVSD/TOF has been achieved through surgical systemic to pulmonary artery shunting. More recently RVOT stenting has evolved as an acceptable alternative in patients with tetralogy of Fallot. METHODS: Retrospective review of all patients with cAVSD/TOF who underwent RVOT stenting as palliation over a 13-year period from two large tertiary referral centers. RESULTS: Twenty-six patients underwent RVOT stenting at a median age of 57 days (interquartile range [IQR] 25.5-106.5). Median weight for stent deployment was 3.7 kg (IQR 2.91-5.5 kg). RVOT stenting improved oxygen saturations from a median of 72% (IQR 70-76%) to 90% (IQR 84-92%), p < .001. There was a significant increase in the median Z-score for both branch pulmonary arteries at median follow-up of 255 days (IQR 60-455). Eight patients required RVOT stent balloon dilatations and 8 patients required re-stenting for progressive desaturation. The median duration between reinterventions was 122 days (IQR 53-294 days). Four patients died during the follow-up period. No deaths resulted from the initial intervention. To date, definitive surgical intervention was achieved in 19 patients (biventricular repair n = 15) at a median age of 369 days (IQR 223-546 days). CONCLUSION: RVOT stenting in cAVSD/TOF is a safe and effective palliative procedure in symptomatic infants, promoting pulmonary artery growth and improving oxygen saturations.

High-risk Fontan completion patients achieve low perioperative risk and benefit from cavopulmonary connection 7 years out†.

OBJECTIVES: Our unit has pursued Fontan completion in all patients except those with immobility or combined poor ventricular function and high pulmonary artery pressures. We assessed retrospectively whether conventional high-risk criteria would predict patients with a poorer outcome. METHODS: One hundred and thirty-three consecutive children who underwent extracardiac Fontan completion (2004-2012) had their outcomes recorded (mean follow-up of 7 years). Three groups were analysed: those with 1 of 6 historical risk factors (outside 6 commandments), those with 1 of reduced systemic ventricular function or pulmonary artery pressure >15 mmHg (outside 2 commandments) versus those with no contraindications. The Fischer's exact test examined frequency differences, with the χ2 test to look for outcome associations. RESULTS: There were no differences in postoperative complication rates between the outside 6 commandments (n = 105) or outside 2 commandments (n = 49) versus the low-risk no-contraindication group (n = 28): arrhythmias [18% (P = 0.3) or 18% (P = 0.3) vs 25%], infection [22% (P = 0.6) or 33% (P = 0.2) vs 21%], cerebrovascular accident [6% (P = 0.5) or 10% (P = 0.3) vs 4%], length of stay [20 days (P = 0.4) or 23 days (P = 0.2) vs 21 days] and duration of chest drainage (P = 0.5). There was 1 predischarge mortality in each group. Long term, the majority of patients in each group had suitable haemodynamics for fenestration closure [95% (P = 0.7) or 95% (P = 0.7) vs 92%]. Long term, there was no difference in the rate of arrhythmias [11% (P = 0.5) or 12.5% (P = 0.3) vs 7%], protein-losing enteropathy [1% (P = 0.1) or 2% (P = 0.3) vs 7%] or moderate or more ventricular dysfunction on echocardiography [2% (P = 0.7) or 4% (P = 0.7) vs 4%]. Notably, there was a higher rate of catheter reinterventions in the high-risk groups [22% (P < 0.05) or 24% (P < 0.05) vs 7%]. CONCLUSIONS: The medium-term benefits of Fontan completion can be achieved for high-risk patients, suggesting that historical selection criteria should be re-examined.

Percutaneous axillary artery approach for ductal stenting in critical right ventricular outflow tract lesions in the neonatal period.

OBJECTIVES: We aimed to assess the experience using a percutaneous axillary artery approach for insertion of arterial ductal stents in patients with critical right ventricular outflow tract lesions at two tertiary pediatric cardiology centers. BACKGROUND: Patent ductus arteriosus stenting is an accepted palliative alternative to BT shunts for neonates with critical right heart lesions. Access to tortuous ductus' may be challenging via the femoral artery, whereas the carotid artery presents a low risk of stroke. Recently, the axillary artery has been utilized for access in these patients. METHODS: We performed a retrospective review of neonates who underwent stent placement or angioplasty using percutaneous axillary artery approach at two tertiary care centers from October 2016 to November 2018. Medical records were reviewed to ascertain demographic, clinical, and outcome data. RESULTS: Axillary artery access was performed in 20 patients (16 primary ductal stents and 4 re-interventions) at a median (IQR) procedural weight of 3.4 (3-3.9) kg. Median (IQR) procedural time was 110 (75-150) min. The median (IQR) ICU stay and intubation times were 14 (0-94) hr and 5 (0-40) hr, respectively. There were three access-related vascular complications which were managed conservatively with no long-term effects. Two patients subsequently died due to non-procedure related causes. CONCLUSIONS: Ductal stenting via a percutaneous axillary artery approach is a viable option in neonates with critical right ventricular outflow tract lesions. This approach provides an additional access site for PDA stenting which may be utilized in patients with vertical duct morphology.

Extrinsic bronchial compression due to patent ductus arteriosus closure device.

Interventional cardiology provides a valuable nonoperative approach for the modern management of patent ductus arteriosus (PDA) in patients with non-complex congenital heart disease. We describe a patient with a right-sided aortic arch who developed severe bronchomalacia after PDA device closure that necessitated extensive surgical repair. Consequently, we advise that in infants with a right-sided aortic arch and PDA inserting into the right pulmonary artery, device closure is challenging due to the potential risk of bronchial compression and subsequent development of bronchomalacia. Consideration should be given to surgical closure or use of a softer duct occlusion device.

Do high-risk infants have a poorer outcome from primary repair of coarctation? Analysis of 192 infants over 20 years.

BACKGROUND: Balloon angioplasty for infant coarctation is associated with a high recurrence rate, making operative repair the gold standard for low-risk infants. Debate exists as to whether high-risk infants might be better served with primary angioplasty. We compared the outcome in high-risk versus low-risk infants over 20 years, in a center that always used surgical repair as the primary intervention. METHODS: Of 192 infants from 1986 to 2005, 56 were considered "high-risk," defined as requiring prostaglandin infusion together with either epinephrine infusion for 24 hours preoperatively, or ventilation and milrinone infusion for 24 hours preoperatively. All high-risk patients had a period of ventricular dysfunction prior to surgery, ranging from mild to severe. Outcomes were compared using Bonferroni comparison of means or the Fischer exact test as appropriate. RESULTS: Although the high-risk patients were smaller (3.3 ± 0.1 vs 4.2 ± 0.2 kg, p < 0.01), younger (18 ± 4 vs 57 ± 7 days, p < 0.01), and more often required a concomitant pulmonary artery band (25% vs 15%, p = 0.05), their cross-clamp times were the same as the low-risk patients (18.9 ± 0.9 vs 18.0 ± 0.4 minutes, p = 0.27) and there was no difference in postoperative morbidity (7% vs 3%, p = 0.11). However, there was a trend toward higher perioperative mortality (7% vs 2%, p = 0.07). When compared with the published studies of primary angioplasty in comparable high-risk infants, the mortality rate in our surgically treated high-risk group is much lower. Additionally, only 11% of our high-risk group required reintervention, with two-thirds treated successfully with a single angioplasty at 3.8 ± 2.2 years later, far lower than recurrence rates with primary angioplasty. CONCLUSIONS: We propose that primary surgical repair of coarctation in infants who are high risk should be the primary treatment, with angioplasty reserved for recurrent coarctation.

Percutaneous closure of postoperative ventricular septal defects with the Amplatzer device.

The objective of this study was to look at the procedure, the results, and the follow-up of patients who underwent percutaneous closure of a residual ventricular septal defect (VSD) following a surgical closure using the Amplatzer VSD device. Four patients had an original diagnosis of tetralogy of Fallot, two patients had a patch leak following a surgical repair of a VSD, and three patients had a VSD not repaired at the time of surgery. All patients fulfilled the currently accepted surgical criterion for reoperation (Qp/Qs>1.5). The mean Qp/Qs was 1.8+/-0.3 (1.5-2.3). Four patients underwent VSD closure using an Amplatzer perimembranous VSD device and in five patients an Amplatzer muscular VSD device was implanted. We performed percutaneous closure in nine patients. The size of the residual shunt ranged from 6 to 14 mm and the size of device used ranged from 8 to 16 mm. The arteriovenous loop needed to be recreated in two patients because of failure to advance the delivery sheath. There was complete closure of the defect in six cases, and a small residual shunt remained in three cases. Percutaneous closure of postoperative VSDs appears to be an effective way to resolve a hemodynamically significant residual shunt. There were no difficulties encountered with implantation of the devices. These promising short-term results need reinforcement with additional long-term data.

Mitral valve reconstruction in a pediatric population: late clinical results and predictors of long-term outcome.

OBJECTIVE: Pediatric mitral valve anomalies present complex management challenges to the surgeon, who may have to choose between valve replacement or repair. We review our 18 years of experience to establish the long-term outcomes of pediatric mitral repair. METHODS: Forty-five children (22 boys) with mitral valve anomalies were studied. Mitral reconstruction was performed in all cases at the first instance. The median age at operation was 2.16 years with 18 (40%) younger than 1 year. Patients were divided into two groups: group 1, isolated (mitral anomaly with or without atrial septal defect or patent ductus arteriosus), contained 30 patients (66.6%), and group 2, complex (mitral anomaly with concurrent intracardiac disease), contained 15 patients (33.3%). RESULTS: In-hospital (30-day) mortality in group 1 was 3.3% (1/30); overall in-hospital mortality was 11.1%. Group 2 had a significantly higher in-hospital death rate of 26.6% (4/15; P < .05). There was 1 late death, that of a child who required reoperation. The median follow-up was 5.08 years (range 1-211 months). The 15-year survival in group 1 was 93%, versus 73% in group 2. Seven patients required 9 revision surgical procedures. Two mitral valve replacements were required at reoperation. The 15-year freedom from reoperation was 81.7%. There were no thromboembolic events. The event-free rate at 15 years was 73.5%. CONCLUSION: This series compares favorably with others, with 74% to 85% survival and 66% to 85.7% freedom from reoperation reported with valve replacement. Patients with significant associated congenital cardiac abnormalities are at a higher risk of early death after mitral reconstructive surgery.

Is extended arch aortoplasty the operation of choice for infant aortic coarctation? Results of 15 years' experience in 181 patients.

BACKGROUND: Recurrent coarctation is an ever-present complication of surgical treatment of coarctation of aorta (CoA) among infants. No single operation appears to have a clear superiority. METHODS: From January 1, 1986, to June 30, 2002, a consecutive series of 181 patients less than 1 year of age (range 1 to 300 days, median 13.5 days) were referred for CoA repair. Neonates accounted for 135 patients, and hypoplastic arch (less than 1 mm/kg plus 1) was present in 107 infants. Coarctation of aorta was simple (group 1) in 71 patients; complicated by ventricular septal defect (group 2) in 62; and complicated by complex congenital heart disease (group 3) in 48. All patients were assessed by right arm/left leg Dynamap pressures and routine follow-up was performed by the cardiologists. Follow-up was complete in all patients (range 6 months to 16 years, median 7.5 years). RESULTS: The overall hospital/30-day mortality was 0.5% (group 1 = 0, group 2 = 0, group 3 = 1 [2.0%]). Complications other than recoarctation occurred in 5 patients (2.7%). Late mortality occurred in 15 (11 at intracardiac repair). Recoarctation, ie, a gradient of more than 20 mm Hg, occurred in 4 patients (2.2%). All 4 patients were noted to have a gradient of more than 10 mm Hg (right arm/left leg) postoperatively and as such had residual coarctation. All 4 were successfully treated by balloon aortoplasty. CONCLUSIONS: Extended arch aortoplasty in association with ductal and coarctation excision provides excellent coarctation repair with a low incidence of recoarctation. Recoarctation occurred only in proximal aortic arch hypoplasia or low birth weight. Balloon aortoplasty easily and effectively relieved the recoarctation in all cases.