Joanne P. Starr

Perventricular device closure of muscular ventricular septal defects on the beating heart: technique and results

OBJECTIVE Both surgical management and percutaneous device closure of muscular ventricular septal defects have drawbacks and limitations. This report describes our initial experience with intraoperative device closure of muscular ventricular septal defects without cardiopulmonary bypass in 6 consecutive patients. METHODS A median sternotomy or a subxiphoid minimally invasive incision was performed. Under continuous transesophageal echocardiographic guidance, the right ventricle free wall was punctured, and a wire was introduced across the largest defect. The Amplatzer (AGA Medical Corporation, Golden Valley, Minn) muscular ventricular septal defect occluding device (a self-expandable double-disk device) was used. An introducer sheath was fed over the wire, with the sheath tip positioned in the left ventricle cavity. The device was then advanced inside the sheath and deployed by retracting the sheath. Associated cardiac lesions, if any, can then be repaired during cardiopulmonary bypass. A similar technique can also be applied for periatrial closure of complex atrial septal defects. RESULTS The initial 6 patients are presented. Cardiopulmonary bypass was not needed in any patient for placement of the device and needed in 4 patients for repair of concomitant malformations only (double-outlet right ventricle, aortic arch hypoplasia, pulmonary artery band removal). No complications from using this technique occurred. Discharge echocardiograms showed no significant shunting across the ventricular septum. CONCLUSIONS Perventricular closure of multiple muscular ventricular septal defects is safe and effective. We believe that this could become the treatment of choice for any infant with muscular ventricular septal defects or any child with muscular ventricular septal defect and associated cardiac defects.

Acute hemodynamic benefit of multisite ventricular pacing after congenital heart surgery.

BACKGROUND Cardiac dysfunction after congenital heart surgery is a major cause of morbidity and mortality. Cardiac resynchronization through multisite ventricular pacing (MSVP) improves cardiac index and ventricular function, and lowers systemic vascular resistance (SVR) in adults with heart failure and interventricular conduction delay. METHODS The acute hemodynamic effects of MSVP after congenital heart surgery were assessed. Twenty-nine patients (aged 1 week to 17 years) with prolonged QRS interval had atrial and ventricular unipolar epicardial temporary pacing leads placed at surgery. Group 1 consisted of patients with a single ventricle (n = 14); group 2 included patients with two-ventricle anatomy (tetralogy of Fallot, ventricular septal defect) undergoing ventricular surgery (n = 10); and group 3 included patients with two-ventricle anatomy undergoing other cardiac surgery (n = 5). At a mean postoperative day 1 (range, 0 to 6), blood pressure, systemic and mixed venous oxygen saturations, electrocardiograms, and echocardiograms were obtained before and after 20 minutes of MSVP. RESULTS The QRS duration decreased with MSVP in all patients (mean, 23%, p < 0.005). Systolic blood pressure improved in all patients (mean, 9.7%, p < 0.005). Cardiac index improved in 19 of 21 patients tested, with no change in 2 patients (mean, 15.1%, p = 0.0001). In 2 patients, MSVP facilitated weaning from cardiopulmonary bypass. Echocardiographic mitral or tricuspid valve inflow was not significantly different with MSVP. CONCLUSIONS Multisite ventricular pacing results in improved cardiac index and increased systolic blood pressure, and it can also facilitate weaning from cardiopulmonary bypass. Multisite ventricular pacing may be used as adjunct to standard postoperative treatment of cardiac dysfunction after congenital heart surgery.

Multicenter Experience with Perventricular Device Closure of Muscular Ventricular Septal Defects

Hybrid procedures are becoming increasingly important, especially in the management of congenital heart lesions for which there are no ideal surgical or interventional options. This report describes a multicenter experience with perventricular muscular venticular septal defect (VSD) device closure.Three groups of patients (n = 12) were identified: infants with isolated muscular VSDs (n = 2), neonates with aortic coarctation and muscular VSDs (n = 3) or patients with muscular VSDs and other complex cardiac lesions (n = 2), and patients with muscular VSDs and pulmonary artery bands (n = 5). Via a sternotomy or a subxyphoid approach, the right ventricle (RV) free wall was punctured under transesophageal echocardiography guidance. A guidewire was introduced across the largest defect. A short delivery sheath was positioned in the left ventricle cavity. An Amplatzer muscular VSD occluding device was deployed across the VSD. Cardiopulmonary bypass was needed only for repair of concomitant lesions, such as double-outlet right ventricle, aortic coarctation, or pulmonary artery band removal. No complications were encountered using this technique. Discharge echocardiograms showed either mild or no significant shunting across the ventricular septum. At a median follow-up of 12 months, all patients were asymptomatic and 2 patients had mild residual ventricular level shunts. Perventricular closure of muscular VSDs is safe and effective for a variety of patients with muscular VSDs.

Surgical issues in giant right ventricular fibroma

Cardiac fibroma is a rare and benign tumor usually seen in infants and children. We report a 3-year-old asymptomatic child who presented with a giant right ventricular (RV) fibroma. He underwent complete surgical resection and is doing well at follow-up. Because of the size of the tumor and the potential need for resection of RV free wall, it is essential to have contingency plans to deal with postoperative RV failure.

Autologous repair of supravalvar pulmonic stenosis

Native supravalvar pulmonary stenosis is a rare anomaly, but iatrogenic supravalvar pulmonary stenosis occurs after various repairs for congenital heart disease with relative frequency. Surgical techniques such as patching carry the risk of restenosis. We describe a technique of repair using only autologous tissues that can be applied to both native and iatrogenic supravalvar pulmonary stenosis. There were no complications and no patient developed restenosis at follow-up. Autologous repair of supravalvar pulmonary stenosis is an effective technique.