Sivakumar Sivalingam

Contemporary long-term outcomes of an aggressive approach to mitral valve repair in children: is it effective and durable for both congenital and acquired mitral valve lesions?

OBJECTIVES We analysed the long-term outcomes of mitral valve (MV) repair in children and compared the repairs for both congenital and acquired lesions. METHODS A review of 634 children (≤18 years) who underwent MV repair from 1992 to 2011 was conducted [excluding patients with complete atrioventricular septal defect (AVSD), single ventricle and atrioventricular (AV) discordance]. Associated cardiac anomalies were present in 473 patients (75%). Congenital mitral lesions were found in 270 (43%) patients compared with an acquired aetiology in 364 (57%) [mainly rheumatic: 329 patients (90%)]. Mitral regurgitation (MR) was predominant in 606 (96%) patients, and 544 (86%) of these showed ≥3+ MR. Modified techniques of MV reconstructions were used. RESULTS The early mortality rate was 2% (14 patients). The mean follow-up was 55 months (1-240 months; 85% complete). The late mortality rate was 4% (23 patients) and survival rates at 10 and 15 years were 91 and 86%, respectively. There was no significant difference in 10-year survival between repairing the congenital (98%) and acquired lesions (87%) (P = 0.17). The rate of freedom from reoperation after MV repair for the entire population was 79% at 10 years, with no significant difference between congenital (80%) and acquired lesions (79%) (P = 0.20). Fifty-six patients (9%) required reoperation. Mixed MV lesions, commissural fusions and residual MR (≥2+) were the predictors of valve failure and reoperation. All survivors remain in New York Heart Association class I and none had thromboembolism or pacemaker insertion. CONCLUSIONS MV repair can be successfully applied to both congenital and acquired MV disease in children. Aggressive repair techniques and avoidance of residual MR have improved durability and survival.

Persistent valve of systemic venous sinus: a cause of neonatal cyanosis.

Incomplete involution of valve of systemic venous sinus can present across a spectrum of anatomical lesions ranging from eustachian valve to division of right atrium (cor triatriatum dexter) with overlapping features. We present the case of a neonate presenting with cyanosis, having persistent valve of systemic venous sinus with anatomical details of the redundant tissue in right atrium suggesting an intermediate form between Chiari network and division of right atrium.

Primary Arterial Switch Operation for Late Referral of Transposition of the Great Arteries with Intact Ventricular Septum in the Current Era: Do We Still Need a Rapid Two-Stage Operation?

Objective: We introduced primary arterial switch operation for the patient with transposition of great arteries and intact ventricular septum (TGA-IVS) who had more than 3.5 mm of posterior left ventricle (LV) wall thickness. Methods: Between January 2013 and June 2015, a total of 116 patients underwent arterial switch operation. Of the 116 patients, 26 with TGA-IVS underwent primary arterial switch operation at more than 30 days of age. Results: The age and body weight (mean ± SD) at the operation were 120.4 ± 93.8 days and 4.1 ±1.0 kg, respectively. There was no hospital mortality. The thickness of posterior LV wall (preoperation vs postoperation; mm) was 4.04 ± 0.71 versus 5.90 ± 1.3; P < .0001; interval: 11.8 ± 6.5 days. The left atrial pressure (mm Hg; postoperative day 0 vs 3) was 20.0 ± 3.2 versus 10.0 ± 2.0; P < .0001; and the maximum blood lactate level (mmol/dL) was 4.7 ± 1.4 versus 1.4 ± 0.3; P < .0001, which showed significant improvement in the postoperative course. All cases had delayed sternal closure. The patients who belonged to the thin LV posterior wall group (<4 mm [preoperative echo]: n = 13) had significantly longer ventilation time (days; 10.6 ± 4.8 vs 4.8 ± 1.7, P = .0039), and the intensive care unit stay (days) was 14 ± 9.2 versus 7.5 ± 3.5; P = .025, compared with thick LV wall group (≥4.0 mm: n = 13). Conclusions: The children older than 30 days with TGA-IVS can benefit from primary arterial switch operation with acceptable results under our indication. However, we need further investigation for LV function.

Is it worth repairing rheumatic mitral valve disease in children? Long-term outcomes of an aggressive approach to rheumatic mitral valve repair compared to replacement in young patients†

OBJECTIVES Contemporary experience in mitral valve (MV) repair for children with rheumatic heart disease (RHD) is limited, despite the potential advantages of repair over replacement. We reviewed our long-term outcomes of rheumatic MV repair and compared them with the outcomes of MV replacement in children with RHD. METHODS This study is a review of 419 children (≤18 years) with RHD who underwent primary isolated MV surgery between 1992 and 2015, which comprised MV repair (336 patients; 80.2%) and MV replacement (83 patients; 19.8%). The replacement group included mechanical MV replacements (MMVRs) (n = 69 patients; 16.5%) and bioprosthetic MV replacements (n = 14 patients; 3.3%). The mean age with standard deviation at the time of operation was 12.5 ± 3.5 (2-18) years. Mitral regurgitation (MR) was predominant in 390 (93.1%) patients, and 341 (81.4%) patients showed ≥3+ MR. The modified Carpentier reconstructive techniques were used for MV repair. RESULTS Overall early mortality was 1.7% (7 patients). The mean follow-up was 5.6 years (range 0-22.3 years; 94.7% complete). Survival of patients who underwent repair was 93.9% both at 10 and 20 years, which was superior than that of replacement (P < 0.001). Freedom from reoperation at 10 and 20 years after MV repair was 81.7% and 72.6%, respectively, compared to 83.2% for MV replacement (P = 0.580). Forty patients underwent reoperation after the initial surgery with no operative deaths. Mixed mitral lesion and postoperative residual MR (≥2+) were the predictors for reoperation in the repair group, whereas lower body surface area and usage of bioprosthesis were significant factors for the replacement group. Freedom from thrombotic, embolic and haemorrhagic events at 10 and 20 years for patients with repair was 98.2% compared to 90.1% in patients with replacement and 67.6% for patients with MMVR (P = 0.004). CONCLUSIONS Twenty-three years of follow-up shows that MV repair is superior to MMVR in children with RHD. Hence, the rheumatic MV should be repaired when technically feasible to maximize the survival and reduce the valve-related morbidity with comparable durability to MMVR.

Stenting of right ventricular outflow tract in Tetralogy of Fallot with subarterial ventricular septal defect: A word of caution

We report a case of Tetralogy of Fallot with severe cyanosis who underwent a successful right ventricular outflow tract stenting. Follow-up echocardiography revealed moderate aortic regurgitation due to the impingement of the stent on the aortic valve. The patient underwent successful surgical correction at which time the stent was removed completely with a resolution of the aortic regurgitation.

Strategy for ventricular septal defect closure after prior gastric pull-through

A 9-year-old boy was referred with a perimembranous ventricular septal defect. At birth, he had undergone a right thoracotomy with ligation of a tracheoesophageal fistula, cervical esophagostomy, and feeding gastrostomy. At 2 years of age, he had gastric tube reconstruction with a pull-through retrosternally, anterior to the heart, and an end-to-end esophagogastric anastomosis. Via a right anterolateral thoracotomy through the previous scar, the entire gastric tube was mobilized away from the sternum to facilitate a median sternotomy. With the patient supine, a median sternotomy was performed without difficulty, and the ventricular septal defect was closed under cardiopulmonary bypass.

Aneurysmal Dilatation Without Distal Obstruction: A Rare Complication of Valved Bovine Jugular Vein Conduit

Valved bovine jugular vein (Contegra) conduit is considered a suitable choice for pediatric population with congenital heart defect requiring right ventricle to main pulmonary artery connection. Intermediate follow-up studies have shown the propensity of developing distal conduit stenosis and valve thrombosis. We present a rare case of aneurysmal dilatation of the conduit leading to valve failure requiring conduit explantation.

An Uncommon Mediastinal Cyst With a Rare Neurologic Consequence

Enteric cysts are uncommon posterior mediastinal cysts, usually presenting with respiratory symptoms in infancy. We present a rare case of posterior mediastinal enteric cyst extending from below the diaphragm and perforating into the left atrium, causing a thromboembolic cerebral infarct in a 5-year-old boy.