Novel arterioplasty of severe left pulmonary artery stenosis using native main pulmonary artery.

There are multiple approaches described for the repair of stenosed branch pulmonary arteries. Regardless of the technique used, restenosis is common. We describe a case of severe left pulmonary artery stenosis repaired with a novel technique using the native main pulmonary artery which was transected and turned down to create a direct anastomosis with the left pulmonary artery. This tension-free tissue to tissue anastomosis resulted in hemodynamically gratifying results with the potential for growth.

One stage combined repair of intracardiac defects with vascular ring employing sternotomy approach.

We present an unusual case of atrial septal defect and ventricular septal defect with a vascular ring formed by a right-sided aortic arch with an aberrant left subclavian artery that gave rise to a patent ductus arteriosus connecting to the main pulmonary artery. We performed a single-stage repair of the intracardiac defects and division of vascular ring with a sternotomy instead of the traditionally practiced dual approach. This included division and reimplantation of the aberrant left subclavian artery to the left carotid artery after transection. This approach has not been described so far.

Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication.

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention.

INTRODUCTION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. OBJECTIVES: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence-based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.

Management of infants with large, unrepaired ventricular septal defects and respiratory infection requiring mechanical ventilation.

OBJECTIVES: We sought to describe the hospital management and early outcome of critically ill infants presenting with large ventricular septal defects and pneumonia requiring mechanical ventilation at a referral center in a developing country. Infants with large ventricular septal defects who have pneumonia might present with respiratory failure requiring mechanical ventilation. In the developing world this presentation is relatively common, but few data exist describing patient management strategies. METHODS: Hospital data of consecutive infants admitted with large ventricular septal defects and pneumonia requiring mechanical ventilation were reviewed and analyzed. RESULTS: We identified 18 infants (mean age, 3.6 +/- 3.0 months). On admission, all the infants were significantly malnourished, and echocardiography showed bidirectional shunting (predominantly right-to-left shunting) in 6 infants. Thirteen (72%) patients improved with intensive medical management that included mechanical ventilation for 1 to 16 days (median, 6.5 days); unequivocal left-to-right shunting was subsequently documented by means of echocardiography in all 13 patients. Twelve patients underwent surgical repair, and 11 (91.6%) were discharged after median mechanical ventilation of 100 hours (range, 42-240 hours) and intensive care unit stay of 8 days (range, 4-15 days). Five of 6 unoperated patients died, 4 of them within a few hours of admission. One child with multiple ventricular septal defects was discharged and subsequently underwent pulmonary artery banding. CONCLUSION: Corrective cardiac surgery for selected critically ill infants with large ventricular septal defects, severe malnutrition, and pneumonia requiring mechanical ventilation is feasible and should be considered a viable management strategy.

New technique of right heart bypass in congenital heart surgery with autologous lung as oxygenator.

BACKGROUND: Modifications have been made in cardiopulmonary circuit to reduce the inflammatory deleterious effects and cost. We present our experience of one such right heart bypass (RHB) circuit utilizing autologus lung as oxygenator. METHODS: From September 2001 to December 2002, 15 patients underwent congenital heart surgery with this technique. Bypass circuit consisted of a reservoir and a roller pump along with a cardiotomy sucker. The left pulmonary artery and main pulmonary artery were used for arterial return, and venous drainage was achieved with innominate vein cannulation. Inferior vena cava cannulation was performed when needed. Thirteen patients underwent bidirectional Glenn shunt surgery (12 to 24 months, 6 to 10 kg). One patient (26 years old) underwent central shunt with enlargement of confluence and left pulmonary artery. Another patient (18 months old) underwent 1.5 ventricle repair. RESULTS: There were no hospital deaths. Mean flow achieved on RHB was 0.57 +/- 0.3 L/min/m(2), central venous pressure was 3.3 +/- 1.8 mm Hg (0 to 7 mm Hg), and mean arterial pressure could be maintained satisfactorily in all patients (54 +/- 14 mm Hg). Mean RHB time was 54 +/- 14 min. Mean central venous pressure was 10.1 +/- 2.4 mm Hg after procedure and saturation was similar to that on (RHB 88% +/- 8%). The mean amount of drainage was 9.1 +/- 4.2 mL/kg per 24 hours. Avoiding an oxygenator and reducing the number of tubings achieved a combined cost savings of 40% for all procedures. CONCLUSIONS: Right heart bypass is a simple, safer, and less expensive alternative to conventional cardiopulmonary bypass. This technique allows effective decompression of superior vena cava, adequate oxygenation, and predicts saturation after Glenn shunt. It can also be applied for central shunts and pulmonary artery reconstructions with cost containment.

Limited right posterior thoracotomy approach to atrial septal defect.

Experience of atrial septal defect closure via a limited posterior thoracotomy is described. From July 1999 to May 2001, 75 prepubertal girls with a median age of 7 years (range, 3 to 13 years) and a median weight of 18 kg (range, 10 to 46 kg) underwent atrial septal defect closure through a limited right posterior thoracotomy. All but 2 patients had an uneventful postoperative recovery. The median duration of ventilation was 13.3 hours (range, 4 to 24 hours). Median hospital stay was 6 days (range, 6 to 8 days). All patients were followed up for 7 to 32 months (mean, 15 months). The wounds healed well without any restriction of limb movement. The limited posterior thoracotomy gave excellent cosmetic results and can be used as a safe alternative approach for atrial septal defect closure in prepubertal females.

Tricuspid valve detachment for transatrial closure of ventricular septal defects.

Tricuspid leaflet detachment improves visualization and accuracy of closure of ventricular septal defects via the transatrial route. Between July 1998 and March 2001, surgical correction was performed in 296 cases of isolated ventricular septal defect, 215 cases of tetralogy of Fallot, and 16 cases of double-outlet right ventricle. Of these, 132 patients (79 with isolated ventricular septal defect, 49 with tetralogy of Fallot, and 4 with double-outlet right ventricle) underwent transatrial repair with temporary detachment of tricuspid leaflets for ventricular septal defect closure. The septal leaflet was detached in most cases, with anterior or posterior leaflets being detached when indicated. Median duration of intensive care was 3.6 days, and median hospital stay was 7 days. There was no incidence of tricuspid regurgitation attributable to leaflet detachment, as confirmed by postoperative echocardiography. Reoperation was not required for a residual defect or tricuspid regurgitation. The benefits of temporary leaflet detachment for transatrial repair of various difficult defects far outweigh the risk of postoperative tricuspid regurgitation.

Determinants of early outcome after neonatal cardiac surgery in a developing country.

OBJECTIVE: Significant technologic advances have improved outcomes in neonatal cardiac surgery over the past 3 decades. However, outcomes might be different in developing countries with resource limitations. We sought to identify the determinants of early outcome after neonatal cardiac surgery in a tertiary referral center in South India. METHODS: Hospital records of 330 consecutive neonates who underwent surgical intervention between January 1999 and April 2006 were reviewed, and perioperative variables were recorded. Main outcome measures were 30-day mortality, postoperative bloodstream infection, and hospital stay of longer than 10 days. Multivariate logistic regression analysis was performed. RESULTS: Overall mortality was 8.8%. Mortality significantly decreased from 21.4% before 2002 to 4.3% after 2002 (3.2% for corrective operations, P < .0001). The prevalence of postoperative bloodstream infection remained the same, whereas surgical site infection and hospital stay significantly increased after 2002. Predictors of outcomes on multivariate analysis were as follows: (1) mortality--operation before 2002 (odds ratio, 5.5), age less than 7 days (odds ratio, 3.8), preoperative antibiotic use (odds ratio, 5.6), and postoperative exchange transfusion (odds ratio, 14.9); (2) postoperative bloodstream infection (21.2%)--use of cardiopulmonary bypass (odds ratio, 2.0), reintubation (odds ratio, 7.7), and surgical site infection (odds ratio, 4.1); and (3) hospital stay of longer than 10 days (61.2%)--use of cardiopulmonary bypass (odds ratio, 2.8), delayed sternal closure (odds ratio, 3.6), reintubation (odds ratio, 12.1), surgical site infection (odds ratio, 13.8), and postoperative antibiotic use (odds ratio, 4.4). CONCLUSIONS: With increasing experience, neonatal cardiac surgery can be performed with excellent outcomes in developing countries with resource limitations. Infectious complications contribute significantly to morbidity and mortality, and improvements in infection-control practices should be emphasized to improve outcomes further.

Somatic growth after ventricular septal defect in malnourished infants.

OBJECTIVE: To assess somatic growth after ventricular septal defect (VSD) repair in severely malnourished infants. STUDY DESIGN: Ninety consecutive infants (age at surgery: 7.2 +/- 3.2 months) were followed after VSD closure at a referral center in southern India to evaluate somatic growth. RESULTS: At surgery, 44% and 30% of patients had weight and height Z score <-3, respectively. On follow-up (age 5.4 +/- 0.8 years), despite a significant improvement from baseline (Z score -1.8 +/- 1.2 vs -2.8 +/- 1.3, P <.001), weight was significantly lower compared with healthy Indian children, particularly for boys. Height Z score improved significantly only in girls (-0.8 +/- 1.3 vs -1.8+/-2.1, P = .01). Weight, height, and combined weight and height Z scores (failure to thrive) of <-2 were observed in 42%, 27%, and 18 % of patients, respectively. On multivariate analysis, weight Z score <-2 on follow-up was predicted by weight Z score at surgery and male sex, height Z score <-2 by maternal height and male sex, and failure to thrive by maternal height and caloric intake. CONCLUSIONS: There is suboptimal recovery of somatic growth after repair of VSD in severely malnourished infants. Preoperative malnutrition affected only weight on follow-up, whereas height recovery and failure to thrive were influenced by constitutional factors.

Cardiac herniation following closure of atrial septal defect through limited posterior thoracotomy.

OBJECTIVES: We report an unusual complication following closure of atrial septal defect through right limited posterior thoracotomy. METHODS: An eight-year-old girl underwent closure of atrial septal defect through right limited posterior thoracotomy. She developed cardiac herniation in the early post-operative period following a tension pneumothorax on the left side, while recovering in the intensive care unit. RESULTS: Cardiac herniation was promptly recognized, albeit subtle hemodynamic changes, and was reduced through re-operation. CONCLUSION: Liberal use of pericardium for closure of intra-cardiac defects results in a gap in the pericardial sac. Posterior thoracotomy approach for closure of atrial septal defect has a higher propensity for cardiac herniation owing to the small size and postero-lateral location of the pericardial defect. Recognition and early treatment of cardiac herniation is important since it can mimic cardiac tamponade. Cardiac herniation can be avoided either by enlarging the pericardial defect or by closing it with a prosthetic patch.

Novel repair for obstructed total anomalous pulmonary venous connection to coronary sinus.

A 4-month-old infant underwent surgery for total anomalous pulmonary venous connection to the coronary sinus with obstructions both at the entry of the common chamber into the coronary sinus and at the patent foramen ovale. Using a novel technique, the right atrium was opened with a transverse incision. The coronary sinus was de-roofed. The coronary sinus-common chamber junction was split open into the common chamber. The incision extended up to the drainage of the right-sided pulmonary veins into the venous chamber. The common chamber was then anastomosed to left atrium thereby establishing an unobstructed pathway. The atrial septal defect was closed with a pericardial patch.

Outcome of ventricular septal defect repair in a developing country.

OBJECTIVES: To examine the impact of nutrition and lung infection on outcome early after ventricular septal defect (VSD) repair in infants in a developing country. STUDY DESIGN: One hundred consecutive infants (age, 7.4 +/- 3.3 months) with large VSD(s) who underwent surgical repair at one institution in South India from July 1998 to June 2000 were analyzed. Primary outcome variables were postoperative death, duration of mechanical ventilation, intensive care unit (ICU) stay, and hospital stay. Preoperative variables analyzed included age, weight and length Z scores, and lung infection. RESULTS: Preoperative nutrition was poor (weight Z score, -2.8 +/- 1.3), and 25 patients had pneumonia. Six patients died after repair. No preoperative variable was associated with death. Mechanical ventilation, ICU stay, and hospital stay were longer for younger patients (r (s) for ventilation, -0.23, P =.02; for ICU stay, -0.33, P <.001; for hospital stay, -0.27, P =.007) and for those with preoperative pneumonia (median ventilation duration, 46 vs 24 hours, P <.001; median ICU stay 7 vs 4 days, P <.001; median hospital stay 10 vs 7 days, P =.001). Preoperative weight and length Z scores were not associated with any outcome variable. CONCLUSIONS: Poor nutritional status, preoperative pneumonia, and age do not increase mortality rates after VSD repair. Repair of large VSDs should not be delayed because of these preoperative characteristics.