Late complex biventricular repair after bidirectional cavopulmonary shunt.

A child diagnosed with transposition of great arteries, multiple (Swiss cheese) ventricular septal defects, and a small right ventricle underwent pulmonary artery banding and patent ductus arteriosus ligation at the age of six months. At the age of three years bidirectional cavopulmonary shunt was performed as a first stage for univentricular repair. However, the patient was lost follow-up for four years, following which further evaluation showed that the right ventricle was reasonably adequate to support pulmonary circulation if the ventricular septal defects (VSDs) were closed using percutaneous techniques. Four VSDs were then closed using Amplatzer devices (AGA Medical Corporation, Plymouth, MN, USA). At the age of eight years she underwent complex biventricular repair in the form of arterial switch, closure of atrial septal defect, take down of Glenn shunt, and reanastomosis of the distal end of the superior vena cava to the distal superior vena cava stump on the right atrium. One year later the patient is alive and well. In conclusion; biventricular repair may be considered before completion of Fontan whenever cardiac anatomy allows.

Play safe: band the late presenting complete atrioventricular canal.

Primary repair of complete atrioventricular canal in patients who present beyond one year of age carries a high mortality. Between January 1995 and February 2000, 16 patients aged 8 to 24 months (mean, 14.5 months) received pulmonary artery banding at presentation and underwent total correction at 24 to 96 months old (mean, 41.9 months). There was one hospital death (mortality, 6.25%). During a mean follow-up of 10.2 months (range, 6 to 28 months), there was no late death, 13 of the 15 survivors (87%) were in New York Heart Association functional class I, and 2 (13%) were in class III. In patients with complete atrioventricular canal who present late with severe reactive pulmonary hypertension, banding followed by complete repair reduces the risk associated with primary repair.

Two-stage arterial switch operation: is late ever too late?

Results of the two-stage arterial switch operation in 49 patients with transposition of the great arteries, performed between January 1995 and September 2000, were reviewed retrospectively. Twenty-one patients had a ventricular septal defect. Anatomical correction was carried out 21.89 +/- 9.86 months after pulmonary artery banding, with or without a modified Blalock-Taussig shunt. Hospital mortality was 8% (4 patients). During follow-up of 30.12 +/- 14.38 months, there was 1 late death and 1 patient required reoperation for pseudoaneurysm of the ascending aorta. Actuarial survival and freedom from reoperation at 5 years were 90% and 97%, respectively. Late anatomic correction (> 6 months) after the preliminary procedure can be performed with an acceptable mortality and morbidity, but undue delay may lead to left ventricular dysfunction, arrhythmias, and new aortic valve regurgitation or subaortic stenosis.

Minimally invasive transventricular implantation of pulmonary xenograft.

Minimally invasive valve replacement is now a clinical reality. A number of ingenious techniques have been described for percutaneous aortic and pulmonary valve replacement as well as transventricular aortic valve replacement. We describe a technique for transventricular pulmonary valve replacement utilizing off-the-shelf bovine tissue valve, vascular stents, and simplified delivery system. After median sternotomy with limited exposure of the right ventricle, Contegra 200 pulmonary valve (Medtronic, Inc., Minneapolis, MN, USA) is transfixed inside a CP stent (NuMed, Inc., Hopkinton, NY, USA) using multiple 5/0 prolene sutures. The valve/stent composite is crimped on a Cristal balloon catheter (Balt, Montmorency, France). The valve/stent and the balloon are then introduced via a small purse-string placed at the RVOT. The stent/valve composite is then expanded in the pulmonary valve position.

Severity scoring system for ventricular septal defect.

INTRODUCTION: Residual ventricular septal defect (RVSD) occurs in one-third of patients undergoing patch closure of congenital VSD. Indications for re-intervention are often based on either patient's symptoms or echocardiographic or hemodynamic studies. We report a novel scoring system for RVSD that takes into account all of the above criteria. METHODS: RVSD size, Qp:Qs ratio, RV to LV pressure ratio, and heart failure symptoms are scored as follows: (A) RVSD size is subdivided into three categories: <3 mm, 3 to <4 mm, and >/=4 mm; (B) Qp:Qs ratio is also subdivided into three categories: <1.5, 1.5 to <2, and >/=2.0; (C) The right and left ventricular pressure ratio is subdivided into the following: <0.5, 0.5 to <0.75, and >/=0.75; (D) Heart failure symptoms are subdivided into three categories: NYHA class II/III, NYHA class IV, and pulmonary edema requiring assisted ventilation. Each of these categories is given a score value of one (less severe), two (intermediate severity), and three (severe). Intra-operative severity score of RVSD is calculated by adding the total score of A, B, and C. For post-operative RVSD severity, the score values of A, B, C, and D are added. According to the total score, the clinical significance of an intra-operative RVSD is then defined as mild, moderate, or severe for a score of /=6, respectively. Similarly, post-operative RVSD is then labeled as mild, moderate, or severe for a score of /=8, respectively. CONCLUSION: From our experience and review of literature, severe RVSD (and moderate VSD post Tetrology of Fallot (TOF) repair) require immediate closure of RVSD. Other patients with mild or moderate RVSD need close follow-up with a repeat of transesophageal echocardiography (TEE) before discharge, and six months after surgery. This scoring system, however, needs further prospective evaluation to assess its potential role in decision-making in the management of RVSD.

Cor triatriatum: operative results in 20 patients.

Cor triatriatum is an uncommon cardiac anomaly occurring in 0.1% of children with congenital heart disease. We reviewed our results from 20 patients presenting with cor triatriatum in the last 20 years (from March 1983 to August 2002). Mean age at repair was 20 months (1-132 months). During a mean follow-up period of 31 months (2-156 months) there were no deaths. All patients were in sinus rhythm except one patient who needed pacemaker insertion one week after repair. A total of 14 patients were asymptomatic and 6 were in New York Heart Association Class I. Cor triatriatum is amenable to surgical repair with excellent results when diagnosed early, and when not associated with other severe cardiac anomalies.