Anatomically corrected malposition of great arteries.

Anatomically corrected malposition of great arteries (ACMGA) is a rare form of congenital heart disease in which the great arteries arise above the anatomically correct ventricles but have abnormal spatial relationship. We report the case of a 26-year-old female with ACMGA and tunnel type of subaortic obstruction. The abnormal relationship and segmental arrangement necessitates systematic approach in evaluation for proper diagnosis and surgical repair. This unusual case is reported for its rarity and to highlight the need for awareness to differentiate it from other more common conditions.

Myocardial membrane injury in pediatric cardiac surgery: An animal model.

OBJECTIVE: Reduced myocardial performance invariably follows pediatric cardiac surgery and is manifested by a low cardiac output state in its severest form. The role of myocardial membrane proteins in this setting is unknown. Dystrophin and dysferlin are involved in membrane integrity, whereas aquaporins selectively transport water. These proteins were examined in a model of pediatric cardiac surgery, together with a trial of poloxamer 188, which may reduce membrane injury. METHODS: Eight lambs were randomized to saline with or without poloxamer 188. Lambs underwent 2 hours of cardiopulmonary bypass and aortic crossclamping. After a further 9 hours of monitoring, the hearts were assessed for water content, capillary leak, and protein expression. RESULTS: Dystrophin expression was unaffected by ischemia/reperfusion, but dysferlin expression was reduced. Aquaporin 1 protein increased after ischemia/reperfusion. Poloxamer 188 administration was associated with supranormal levels of dystrophin, preservation of dysferlin expression, and normalization of aquaporin 1 expression. Poloxamer 188 was associated with less capillary leak, maintained colloid osmotic pressure, and less hemodilution. Poloxamer 188 was associated with an improved hemodynamic profile (higher blood pressure, higher venous saturation, and lower lactate), although the heart rate tended to be higher. CONCLUSIONS: Changes in protein expression within the myocardial membrane were found in a clinically relevant model of pediatric cardiac surgery. Indicators of reduced performance, such as lower blood pressure and lower oxygen delivery, were lessened in association with the administration of the membrane protecting poloxamer 188. Poloxamer 188 was also associated with potentially beneficial changes in membrane protein expression, reduced capillary leakage, and less hemodilution.

Surgical experience of aortopulmonary window repair in infants.

The aim of this study was to retrospectively analyze our results of both simple and complex aortopulmonary window (APW) repair in infants. From September 1994 to May 2003, 21 infants which included 15 with simple APW (weight 3.9+/-0.8 kg and age 5.1+/-3.7 months) and six with complex APW (weight 4.03+/-1.1 kg and age 5+/-3.8 months) underwent APW repair at the All India Institute of Medical Sciences, New Delhi, India. The approach for APW repair was ligation without CPB in four patients, division and suturing using CPB in one patient, trans-aortic with Goretex patch closure in 11 patients, trans-pulmonary in one patient and trans-window in four patients. The hospital mortality was 13% and 33% for simple and complex APW, respectively. On mean follow-up of 39 months there was no re-operation or late death. An early repair of APW is mandatory to achieve a good surgical result. Trans-aortic repair of APW is the procedure of choice for all APWs, except in the case of large defects where anterior sandwich patch technique (trans-window repair) may be done. In our view, simple ligation without CPB should be avoided due to the possibility of residual APW and distortion of pulmonary artery.

Extrapleural intrathoracic implantation of permanent pacemaker in the pediatric age group.

Permanent pacemaker implantation in pediatric patients poses challenges in finding a suitable pocket for generator implantation. We present our experience with 6 patients in whom the pacemaker was placed in an extrapleural intrathoracic location. We find that an extrapleural intrathoracic pocket is a useful site for generator placement in the neonatal and pediatric age group. It is safe, reproducible, and both the lead and generator can be placed through a single incision. We briefly discuss the advantages and disadvantages of other techniques that require a single incision for both lead and generator placement.

Primary repair of d-transposition with complete atrioventricular canal defect.

A 4-month-old boy underwent successful single-stage anatomic repair of d-transposition of the great arteries with complete balanced atrioventricular canal defect, using the arterial switch procedure and two-patch repair of the atrioventricular canal defect. He had associated persistent left superior vena cava draining to the coronary sinus, and a patent ductus arteriosus.

Interlocking sternotomy: initial experience.

An interlocking sternotomy using a lazy-S-shaped incision was performed in 91 patients undergoing cardiac surgical procedures (group A). The results were compared with those of 77 patients (group B) who underwent a standard sternotomy by the same surgical team. In group A, the incidence of sternal instability was 2.27% (2/88) versus 8.21% (6/73). No dehiscence or mediastinitis was noted in group A, whereas 6.85% (5/73) in group B had this complication. Analysis of diabetics revealed no sternal complication in group A compared to 50% (3/6) in group B. The interlocking sternotomy significantly reduced the incidence of sternal instability and helped to prevent sternal dehiscence and mediastinitis. Use of this safe, simple, and reproducible technique is strongly recommended, especially in diabetics.

Transaortic double valve replacement with total chordal preservation.

Very few cases of transaortic double valve replacement have been reported in the literature. A 26-year-old man presented to us with severe aortic regurgitation, mitral valve thickening, and mild mitral regurgitation 6 years after he had undergone a Ross procedure and open mitral commissurotomy. At his 2nd operation, he underwent transaortic double valve replacement with total chordal preservation of the mitral apparatus. Due to recurrent rheumatic activity, this patient had experienced a recurrence of valvulopathy Because we have observed this in other young patients with rheumatic heart disease, we no longer perform the Ross procedure in such patients, especially if there is associated mitral valve disease. In selected patients with dilated aortic annulus, the transaortic approach provides excellent access for safe mitral valve replacement with total chordal preservation. The surgical technique and a brief review of the literature are presented.