Hitendu Dave

Modified Blalock Taussig shunt: a not-so-simple palliative procedure

OBJECTIVES Thirty-two consecutive isolated modified Blalock Taussig (BT) shunts performed in infancy since 2004 were reviewed and analysed to identify the risk factors for shunt intervention and mortality. METHODS Sternotomy was the only approach used. Median age and weight were 10.5 (range 1-74) days and 2.9 (1.9-4.4) kg, respectively. Shunt palliation was performed for biventricular hearts (Tetralogy of Fallot/double outlet right ventricle/transposition of great arteries_ventricular septal defect_pulmonary stenosis/pulmonary atresia_ventricular septal defect/others) in 21, and univentricular hearts in 11, patients. Hypoplastic left heart syndrome patients were excluded. Two procedures required cardiopulmonary bypass. Median shunt size was 3.5 (3-4) mm and median shunt size/kg body weight was 1.2 (0.9-1.7) mm/kg. Reduction in shunt size was necessary in 5 of 32 (16%) patients. RESULTS Three of 32 (9%) patients died after 3 (1-15) days due to cardiorespiratory decompensation. Lower body weight (P = 0.04) and bigger shunt size/kg of body weight (P = 0.004) were significant risk factors for mortality. Acute shunt thrombosis was observed in 3 of 32 (9%), none leading to death. Need for cardiac decongestive therapy was associated with univentricular hearts (P < 0.001), bigger shunt size (P = 0.054) and longer hospital stay (P = 0.005). Twenty-eight patients have undergone a successful shunt takedown at a median age of 5.5 (0.5-11.9) months, without late mortality. CONCLUSIONS Palliation with a modified BT shunt continues to be indicated despite increased thrust on primary corrective surgery. Though seemingly simple, it is associated with significant morbidity and mortality. Effective over-shunting and acute shunt thrombosis are the lingering problems of shunt therapy.

Comparison of surgical and interventional therapy of native and recurrent aortic coarctation regarding different age groups during childhood

OBJECTIVE The aim of the study was to analyze immediate results, rate of complications and re-interventions during medium-term outcome in pediatric patients with native or recurrent aortic coarctation. We focused on an age-related therapeutic approach comparing surgical and trans-catheter treatment. METHODS This is a retrospective, single-centre, clinical observational trial including 91 consecutive patients (age: 1 day-18 years) treated for native coarctation in 67 and recurrent aortic coarctation in 24 patients. Surgical treatment was performed in 56, trans-catheter treatment with balloon dilatation in 17, and by stent implantation in 18 patients. According to the age groups, we treated 48 children in group A (<6 months of age), 16 in group B (6 months-6 years), and 27 in group C (>6 years). A total of 41 patients in group A were operated (85%), patients in group B received either surgical or trans-catheter treatment (50% vs 50%), and 16 patients in group C were treated by stent implantation (62%). RESULTS Immediate results were excellent with a significant release of pressure gradient in all three age groups (64.7% in group A, 69.1% in group B, and 63.3% in group C). Complication rate and re-intervention rate (surgical and interventional) both were [corrected] comparable between the three age groups (complications: group A 8.3%, group B 6.3%, and group C 3.7%, [corrected] re-interventions: group A 16.6%, group B 18.8%, and group C 14.8%). [corrected] Midterm outcome after a median follow-up period of 17.5 months was satisfactory with a re-intervention-free survival after 17.5 months of 83.4%, 81.2%, and 81.5% in group A, group B, and group C, respectively. CONCLUSIONS The current strategy of an age-related therapy for native and recurrent aortic coarctation in our institution is surgery in infants <6 months (group A), either surgery or balloon dilatation in younger patients <6 years (group B), while in older children >6 years of age (group C) the trans-catheter treatment with stent implantation is an excellent alternative to surgery. Balloon dilatations showed limited results with an overall re-intervention rate of 53% and, therefore, should mainly be performed as a rescue procedure or in recurrent aortic coarctation in neonates.

Severe Congenital Heart Defects Are Associated with Global Reduction of Neonatal Brain Volumes

OBJECTIVES To determine neonatal global and regional brain volumes in infants with congenital heart disease (CHD) in comparison with healthy controls and to determine brain growth. STUDY DESIGN Prospective cohort study in infants undergoing open-heart surgery for complex CHD. Global and regional volumetric measurements on preoperative cerebral magnetic resonance imaging were manually segmented in children without overt brain lesions. RESULTS Preoperative brain volumetry of 19 patients demonstrates reduction in total and regional brain volumes, without any specific regional predilection compared with 19 healthy control infants (total brain volume reduction: 21%, regional brain volume reduction 8%-28%, all P < .001). CONCLUSIONS Infants with CHD undergoing bypass surgery have smaller brain volumes prior to surgery without a specific regional predilection. This suggests a fetal origin of reduced brain growth.

Risk Factor Analysis of 170 Single-Institutional Contegra Implantations in Pulmonary Position

BACKGROUND The aim of this study is to evaluate risk factors affecting survival of Contegra grafts used in the pulmonary position. METHODS One hundred seventy Contegra implanted (2001 to 2007) in the pulmonary position for replacement after a prior repair (90), Ross procedure (29), tetralogy of Fallot and variants (22), truncus arteriosus (13), Rastelli procedure (8), and miscellaneous (8) were reviewed. Median age was 107 (0.1 to 894) months. Follow-up was 96% complete with a median duration of 65 (7 to 98) months. RESULTS There were 7 early and 6 late deaths (none Contegra related) with a survival of 92%±2% at 98 months. Thirty-four Contegra were replaced at a median duration of 43 (7 to 82) months. Eight of 28 balloon-dilated Contegra remain palliated at 49 (23 to 73) months. Multivariate analysis showed Contegra less than 16 mm (p<0.001; HR [hazard ratio] 0.07), high pulmonary pressure (p<0.001; HR 4), and prior operation era (p=0.006; HR 0.3) as independent risk factors for Contegra replacement. The freedom from replacement for Contegra less than 16 mm and 16 mm or greater were 48%±8% and 98%±2%; for presence and absence of high pulmonary pressure were 52%±11% and 88%±3% and for era 1 and 2 were 77%±5% and 88%±4% at 60 months, respectively. Twenty-three of 123 surviving Contegra have a mean Doppler gradient greater than 20 mm Hg. Contegra less than 16 mm, smaller age group, obstructive arborization, and era 1 were significantly associated with higher gradients. While 18 (15%) surviving Contegra have moderate or more regurgitation, 88 (72%) have no significant gradient or regurgitation. CONCLUSIONS Contegra grafts integrate well into the body. Larger Contegra show predictable function with a survival approaching homografts over medium term. Better case selection as one ascends the learning curve tends to improve survival. With easy availability and predictable quality, Contegra grafts continue to be a promising complement to homografts.

Hybrid approach for hypoplastic left heart syndrome and its variants: the fate of the pulmonary arteries

OBJECTIVES To analyse the results of hybrid palliation of hypoplastic left heart syndrome (HLHS) patients and its variants with an emphasis on the long-term fate of the pulmonary arteries. METHODS We analysed 28 neonates (2006-11) with HLHS and its variants, who underwent bilateral pulmonary artery banding, patent ductus arteriosus (PDA) stenting and balloon atrial septostomy using a true hybrid approach. Median age and weight were 4 (0-36) days and 3 (1.9-3.7) kg respectively. Diagnoses included 23 HLHS and 5 variants. The fate of all surviving branch pulmonary arteries (PA) after a hybrid approach were compared with their counterparts in 29 Norwood I survivors (2002-11). RESULTS Four of 28 hybrid procedures needed to be converted to a Norwood procedure. Mortality after stage I hybrid palliation was 3/24 (12.5%). All 21 acute survivors underwent a comprehensive stage II at a median age of 4 (2.3-5.7) months, without any mortality (0%). Eleven of 21 comprehensive stage II survivors have undergone extracardiac Fontan; including 1 who underwent a rescue Fontan at 7 months of age and died (1/11: 9%). While 18/21 (86%) needed branch PA intervention in the hybrid group during the median follow-up duration of 39 (10-81) months, 9/29 (31%) needed the same in the Norwood group during a median follow-up duration of 58 (16-128) months (P < 0.001). Eight of 21 (38%) needed stenting [all on the left pulmonary artery (LPA)] in the hybrid group vs 5/29 (17%) in the Norwood group (P = 0.097). Ten of 21 (48%) patients had surgical/catheter intervention on both branch PA in the hybrid group vs 2/29 (7%) in the Norwood group (P = 0.001). Pre-Fontan Nakata index was significantly better in the Norwood group 206 (118-406) compared with the hybrid group 153 (56-230) mm(2)/m(2) (P = 0.01). The comparable lower lobe indices were 149 (103-333) and 137 (45-178) mm(2)/m(2) (P = 0.04), respectively. CONCLUSIONS Hybrid approach can be pursued with a low mortality. However, the high frequency of catheter and/or surgical interventions, and the sluggish growth of the branch PA pre-Fontan need innovative solutions. A comparison of the neurodevelopmental outcome for the hybrid vs the Norwood cohort would define the role of the hybrid strategy in the treatment of HLHS and its variants.

Mid-term results of right axillary incision for the repair of a wide range of congenital cardiac defects

OBJECTIVE We evaluated the mid-term results of the right axillary incision used for the repair of various congenital heart defects. METHODS All the patients who were operated with this incision between March 2001 and December 2007 were reviewed. There were 123 patients (median age 4.7 {0.4-19.4} years and median weight 16.6 {3.8-62} kg) undergoing atrial septal defect (ASD) closure (62), repair of partial anomalous pulmonary venous connection (PAPVC) (22), correction of partial atrioventricular septal defect (AVSD) (19), and restrictive perimembranous ventricular septal defect (VSD) (20). Additional procedures involved tricuspid valve plasty (10), mitral annuloplasty (3), reduction plasty of the aortic sinus (2), resuspension of the aortic valve cusp (2), sub aortic membrane resection (1), or reimplantation of Scimitar vein (1). The surgical technique involved peripheral (groin) and central (SVC+/-aorta) cannulation for institution of cardiopulmonary bypass. Fibrillatory arrest was used for repair of ASDs and cardioplegic arrest for repairs involving the atrioventricular valves as well as VSDs. The median CPB and aortic clamp times were 72 (35-232) and 0 (0-126) min, respectively. RESULTS There was no need for conversion to another approach in any patient. Early morbidity included transient paresis of left upper arm (1), stenting of SVC after repair of a sinus venosus defect (1) and revision for bleeding (1). Follow-up echo showed no residual defect in 116 patients and minor residual defects in 7 patients: tiny ASD (2), tiny VSD (1) and mitral regurgitation (4). One patient developed stenosis in the right external iliac artery used for cannulation, necessitating surgical intervention. All the patients are in excellent condition after a median follow-up of 4.1 (0.4-7.1) years. The incision healed well and the thorax and the breast showed no deformity on follow-up. CONCLUSIONS The right axillary incision provides a quality of repair for various congenital defects similar to that obtained by using standard surgical approaches. Because of its deceitful location, and the camouflaging effect of being hidden by the resting arm, it has superior cosmetic appeal compared to conventional incisions. The incision does not interfere with subsequent development of the thorax or the breast (in case of females).

Cerebral lesions on magnetic resonance imaging correlate with preoperative neurological status in neonates undergoing cardiopulmonary bypass surgery

OBJECTIVES To determine the prevalence, spectrum and course of cerebral lesions in neonates with congenital heart disease (CHD) undergoing full flow cardiopulmonary bypass (CPB) surgery using magnetic resonance imaging (MRI) and to examine the correlation between cerebral lesions and clinical neurological abnormalities. METHODS Prospective cohort study of neonates with d-transposition of the great arteries (n = 22), univentricular heart malformation with hypoplastic aortic arch (n = 6) and aortic arch obstructions (n = 2) undergoing CPB. Neonates underwent cerebral MRI and blinded standardized neurological examination before (median day 6) and after surgery (day 13). The MRI findings were compared with those of 20 healthy controls. RESULTS Preoperative cerebral lesions were present in 7 of 30 patients (23%) with isolated mild or moderate white matter injury (WMI) (n = 4), isolated small cerebral stroke (n = 1) and combined WMI and stroke (n = 2). None of the healthy controls had cerebral lesions on MRI. CHD neonates with preoperative cerebral lesions had more neurological abnormalities (P = 0.01) than neonates without cerebral lesions. Low arterial oxygen saturation (P = 0.03) was a risk factor for preoperative cerebral lesions, while balloon atrioseptostomy (P = 0.19) was not. After surgery, preoperative cerebral lesions persisted in 5 of 7 neonates, and 2 neonates (7%) showed signs of additional WMI in their postoperative MRI. CONCLUSIONS In neonates with severe CHD, WMI was the predominant preoperative finding, while cerebral strokes were less frequent. New postoperative lesions were rare. Preoperative neurological abnormalities correlated with the presence of cerebral lesions on MRI.

Simplified surgical-hybrid Melody® valve implantation for paediatric mitral valve disease

Children suffering from left atrioventricular valve (LAVV) disease not amenable to repair represent a significant challenge. The results of surgical reconstruction are not optimal. Valve replacement as an alternative is associated with poor results. The surgical-hybrid approach with implantation of a stented biological valve (bovine jugular vein graft, Melody valve) seems to represent a new therapeutic option. Here we demonstrate our case, the consideration and the approach to extreme clinical findings in a small child. We describe a simplified surgical-hybrid Melody valve implantation in a LAVV position. The technique of implantation is relatively simple and the immediate postoperative result very good.

Management and outcome of Ebstein's anomaly in children

OBJECTIVES To assess clinical presentation, treatment, and outcome of children with Ebsteins anomaly. BACKGROUND Data on long-term outcome of children with Ebsteins anomaly are scarce. METHODS Retrospective analysis of all children with Ebsteins anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention. RESULTS A total of 42 patients were diagnosed with Ebsteins anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1-16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%. CONCLUSION In children, Ebsteins anomaly is usually diagnosed in the first year of age. Even though children with Ebsteins anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

Acute chemotherapy-induced cardiomyopathy treated with intracorporeal left ventricular assist device in an 8-year-old child.

Evolution of ventricular assist devices (VADs) leading to miniaturization has made intracorporeal implantation in children feasible. Ventricular assist device therapy for anthracycline-induced cardiomyopathy (CMP) in adults has been reported. We report the case of an 8-year-old child (body surface area 0.97 m2) presenting with anthracycline-induced CMP being successfully treated with an intracorporeal left ventricular assist device (LVAD) as a bridge to candidacy/recovery. We present our institutional algorithm, which advises intracorporeal LVAD implantation for long-term ventricular assist, in children with a body surface area >0.6 m2. Advantages are better mobilization and the possibility to discharge home, leading to enhanced quality of life.