Double outlet right ventricle, pulmonary stenosis and congenital mitral stenosis.

A 4-year-old girl child was diagnosed with double outlet right ventricle (DORV), severe pulmonary stenosis, and supramitral ring. This case is presented to bring to light this rare association. Through this report, we aim to stress importance of assessing mitral apparatus on echocardiography during evaluation for situations like DORV and Tetralogy of Fallot (TOF). The physiological differences in such situations as opposed to their isolated counterparts and special postoperative outcomes are also discussed.

Intrapulmonary ectopic liver associated with scimitar syndrome.

We report a case of intrapulmonary ectopic liver associated with Scimitar syndrome. A two month old male child who presented with features of congestive cardiac failure underwent extensive cardiac and radiological investigations and was diagnosed with Scimitar syndrome. He was also found to have a mass in the lower lobe of the right lung, the possibilities being either a pulmonary sequestration or a diaphragmatic hernia. As his dyspnea progressively worsened, a right lower lobectomy was performed at the age of two years. Intraoperatively, no connecting pedicle or hernia sac was seen. The resected lung specimen showed a light brown mass enclosed by the lung parenchyma and microscopy showed cords of normal hepatocytes with portal tracts. The hepatocytes were positive for Hep Par1, and bile ducts were CK 7 positive. To the best of our knowledge, this is the first reported case of an intrapulmonary ectopic liver associated with Scimitar syndrome.

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.

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.

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.

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.