Nagarajan Muthialu

Outcomes of slide tracheoplasty in 101 children: A 17-year single-center experience

OBJECTIVE Our study describes the results from surgical slide tracheoplasty (STP) in children with long segment tracheal stenosis. METHODS Demographic and preoperative conditions, operative details, and outcome measures, including the need for endoscopic airway intervention and mortality, were collected for children undergoing STP between February 1995 and December 2012. RESULTS One hundred one patients (median age, 5.8 months; range, 5 days-15 years) underwent STP. Seventy-two patients (71.3%) had associated cardiovascular anomalies. Preoperative ventilation was necessary in 56 patients (55.4%), whereas extracorporeal membrane oxygenation was required in 10 patients (9.9%). Abnormal bronchial arborization was present in 39 children (38.6%), which included 13 patients (12.8%) with an anomalous right upper lobe bronchus and 17 patients (16.8%) with tracheal trifurcation. Airway stenosis extended into 1 or both bronchi in 24 patients (23.7%) and preoperative malacia was present in 24 patients (23.7%). STP was extended into the bronchus in 47 patients (46.5%). Overall survival was 88.2% (mortality in 12 patients). Post-STP balloon dilation was necessary in 45 patients (44.6%) and stenting was required in 22 patients (21.8%). Multivariate analysis revealed preoperative extracorporeal membrane oxygenation (P < .05), preoperative malacia (P < .001), and bronchial stenosis (P < .05) to be adverse predictors of survival. Preoperative malacia was a significant risk factor for stenting (P < .05). CONCLUSIONS STP is a versatile and reliable technique associated with low morbidity and mortality when compared with previous strategies for children with long segment tracheal stenosis. The presence of preoperative bronchomalacia is a significant risk factor for death and postoperative stenting.

Determinants of Exercise Capacity After Arterial Switch Operation for Transposition of the Great Arteries

Patients who undergo the arterial switch operation for transposition of the great arteries (TGA) are at risk of reduced exercise capacity, with most reports focusing on chronotropic incompetence as the cause. Residual right ventricular outflow tract (RVOT) obstruction is relatively common after the arterial switch operation, but its effect on exercise capacity is unknown. We studied 60 patients (44 males, age 13.3 +/- 3.4 years) who had undergone a neonatal arterial switch operation using the cardiopulmonary exercise test and transthoracic echocardiography. The peak exercise oxygen uptake (VO(2)), and heart rate were recorded and are expressed as the percentage of predicted values. The greatest velocity detected by echocardiography across the pulmonary valve, pulmonary trunk, or pulmonary branches was used in the analysis as an index of RVOT obstruction. The peak VO(2)% was 84 +/- 15%, and the peak heart rate percentage was 97 +/- 8%. Of the 60 patients, 29 had an abnormal peak VO(2)% (< or =84%) and 3 (5%) had an abnormal peak heart rate percentage (< or =85%). The maximal RVOT velocity was 2.3 +/- 0.6 m/s, and it correlated with the peak VO(2)% (r = -0.392, p = 0.004). On multivariate analysis, the presence of residual RVOT obstruction (p = 0.0007) was the only variable associated with a reduced peak VO(2)%. Patients with a RVOT maximal velocity > or =2.5 m/s had a lower peak VO(2)% than those with lower velocities (p <0.0001). No relation was found between age at testing and the peak VO(2)%. In conclusion, a reduced exercise capacity is relatively common in children and young adults who have undergone an arterial switch operation, but it does not decrease with age. The presence of residual RVOT obstruction seems to have an effect on exercise capacity.

Functional outcomes after the Ross (pulmonary autograft) procedure assessed with magnetic resonance imaging and cardiopulmonary exercise testing

Objective To assess autograft, homograft and ventricular function, as well as exercise capacity, in adult patients who have undergone the Ross procedure. Setting Single centre paediatric and adult congenital heart disease unit. Patients 45 subjects (24.6 years, range 16.9–52.2 years) who underwent the Ross procedure between 1994 and 2006 (8.1 years after the Ross operation, range 2.0–14.0 years). Interventions Cardiovascular magnetic resonance imaging, echocardiography and cardiopulmonary exercise testing. Main outcome measures Autograft and homograft stenosis, and regurgitation. Autograft size. Biventricular function, scar volume and exercise capacity. Results Mean autograft regurgitation was 6.8%±8.3% (trivial regurgitation) and diameter was 40.0±7.0 mm. Mean homograft velocity was 2.4±0.6 m/s (mild-moderate stenosis) and regurgitation was 6.1%±8.3% (trivial regurgitation). Biventricular systolic function was normal (LV EF 63.1±6.4% and RV EF 60.1%±7.6%). In 38% of cases there was evidence of LV scar, mostly noted within the inter-ventricular septum. The mean exercise capacity achieved was 87%±22% of predicted. There was no correlation between exercise capacity and ventricular function or scar. Conclusion This study demonstrates minor autograft and homograft dysfunction in the majority of patients after the Ross procedure, associated with good ventricular function and exercise capacity. In addition, minor scar was present in a third of patients with no functional consequences.

Pediatric Heart Transplantation for Congenital and Restrictive Cardiomyopathy

BACKGROUND Recent reports suggest worse outcomes in pediatric orthotopic heart transplantation (OHT) for congenital heart disease (CHD) and restrictive cardiomyopathy (RCM). We examined early outcomes in these diverse groups of patients in comparison with patients with dilatated cardiomyopathy (DCM). METHODS From 2000 to 2011, 209 patients were included: 50 with CHD, 23 with RCM, and 136 with DCM. Early survival was studied, as was the occurrence of acute rejection, donor-specific antibodies (DSAs) and nondonor-specific antibodies (NSDAs), incidence of pulmonary hypertension (PHT), right ventricular failure (RVF), and the need for mechanical circulatory support (MCS). RESULTS The incidence of preoperative PHT was greatest in the RCM group (χ(2)p = 0.0006); the requirement for mechanical support before OHT was greatest in patients with DCM. Thirty-day survival was 92.0%, 97.1%, and 100% for patients with CHD, DCM, and RCM respectively. The incidence of RVF was highest for patients with RCM (43.5%; versus CHD, 26.0%; versus DCM, 14.7%). One-year survival estimates for patients with CHD, DCM, and RCM were 92.0%, 97.8%, and 82.6%, respectively (log-rank p = 0.165). Multivariable analysis revealed 4 significant risk factors for mortality: age, incidence of acute rejection, preoperative PHT, and the presence of NDSAs. The occurrence of DSAs was similar, although there was a significantly higher incidence of NDSAs in the CHD and RCM groups (36.0% and 30.4%, respectively, versus 14.0% in the DCM group; χ(2)p = 0.0024). CONCLUSIONS Equivalent outcomes are achievable in pediatric OHT despite marked heterogeneity in anatomic and physiologic complexity in recipients. Physiologic factors such as PHT are likely to be more important than anatomic complexities in determining survival. The potential relevance of NDSAs warrants further investigation.

Primary repair of sternal cleft in infancy using combined periosteal flap and sliding osteochondroplasty

Sternal clefts are rare congenital malformations of the chest wall. Immediate correction in neonates or in infancy is beneficial in terms of providing skeletal cover with little or no need for any prosthetic material for reconstruction. In the absence of internal midline defects, one should aim to provide complete correction when the chest wall is still compliant, and cardiorespiratory compromise is expected to be least after correction. We report on 2 children who underwent primary sternal-cleft repair using a periosteal advancement flap along with mobilization of lateral sternal bars after sliding osteotomy of corresponding ribs.

Treatment of postpneumonectomy syndrome with tissue expanders: The Great Ormond Street Hospital experience

Postpneumonectomy syndrome (PPS) is a rare syndrome whereby the airway and oesophagus become obstructed due to extreme rotation of the mediastinum after pneumonectomy. Our treatment of this condition involved mediastinal repositioning and placement of a saline filled tissue expander into the pleural space. This has not been reported in the plastic surgical literature and we describe technical difficulties and how they were overcome.

Repair of Persistent Left Superior Vena Cava to Unroofed Coronary Sinus Defect by Retro-Aortic Implantation (Modified Warden Type Procedure).

Repair of unroofed coronary sinus defect in the presence of left superior vena cava is challenging and requires prosthetic graft material to redirect flow to the right atrium. This may potentially cause a supra‐mitral gradient or pulmonary venous obstruction.

Direct implantation of scimitar vein to the left atrium via sternotomy: a reappraisal

OBJECTIVES There is no consensus about optimal surgical technique for the repair of scimitar vein, an anomalous right pulmonary venous connection to the inferior vena cava. Our current experience with a direct anastomosis of the scimitar vein to the left atrium via sternotomy may be shared with other centres, but has not been widely published. METHODS Six consecutive patients (age 6 months to 17 years, mean 5 years) operated on in 2009-12 were retrospectively reviewed. Through median sternotomy and with cardiopulmonary bypass, the mobilized anomalous right pulmonary vein was brought through a large pericardial opening posterior to the right phrenic nerve and anastomosed onto the right side of the posterior left atrium with access via an existing or a surgically created atrial septal defect. RESULTS Five patients had primary venous repair and one had a previous failed repair using an intra-atrial baffle. The median cardiopulmonary bypass and cross-clamp times were 88 and 38 min, respectively. The median ventilator time was 1 day and the median stay at the intensive care unit 3.5 days. There were no deaths within a median follow-up of 28 months (range 8-41 months), nor reoperations or instances of pulmonary venous obstruction. CONCLUSIONS Anatomic repair of the scimitar vein based on reimplantation onto the left atrium via sternotomy is conceptually appealing. The surgery results in a safe and reliable repair in patients with a wide age spectrum. Durability needs on-going assessment in longer-term follow-up.

Unusual combination of unroofed coronary sinus in a setting of atrioventricular septal defect with Tetralogy of Fallot—a case report

Complete atrioventricular septal defect (AVSD) in the presence of tetralogy of Fallot is a rare entity. Presence of a common AV valve along with an inlet ventricular septal defect makes repair difficult. Haemodynamically, the pulmonary system is protected from high pulmonary flow due to right ventricular outflow obstruction (RVOT). With these, additional anatomy of presence of left superior vena cava (LSVC) draining to an unroofed coronary sinus defect makes repair even more complex. Committing this coronary sinus to the left atrium at the time of repair will lead to intractable cyanosis, and repair involves its roofing in addition to ventricular and valvular septation, while taking care of RVOT relief, as part of tetralogy surgery. We report a young boy who presented with tetralogy anatomy, but on evaluation had complete AVSD and an unroofed coronary sinus with LSVC. Case report

Chest wall reconstruction in Marfan syndrome following aortic root replacement.

Chest wall and spine deformities are common in Marfan syndrome, and often coexist with cardiac defects. Surgery is often needed to both the aortic root and the anterior chest wall, and early spinal surgery is not uncommon. We describe a case of severe thoracic cage deformity with aortic root dilatation, which was managed by a staged approach, with a very good final result.