Anastasios C. Polimenakos

Aortic cusp extension valvuloplasty with or without tricuspidization in children and adolescents: long-term results and freedom from aortic valve replacement.

OBJECTIVE Aortic cusp extension valvuloplasty is increasingly used in the management of children and adolescents with aortic stenosis or regurgitation. The durability of this approach and the freedom from valve replacement are not well defined. A study was undertaken to investigate outcomes. METHODS From July 1987 to November 2008, 142 patients aged less than 19 years underwent aortic cusp extension valvuloplasty in the form of pericardial cusp extension and tricuspidization (when needed). Three patients with truncus arteriosus and severe truncal valve insufficiency were excluded. From the available follow-up data of 139 patients, 50 had bicuspid aortic valves, 40 had congenital aortic valve stenosis, 41 had combined congenital aortic valve stenosis/insufficiency, and 8 had other diagnoses. Median follow-up was 14.4 years (0.1-21.4). Long-term mortality and freedom from aortic valve replacement were studied. RESULTS There were no early, intermediate, or late deaths. Z-values of left ventricular end-diastolic dimension, aortic annulus, aortic sinus diameter, and sinotubular junction diameter before aortic valve replacement were 4.2 +/- 3.11, 2.3 +/- 1.25, 4.4 +/- 1.23, and 1.84 +/- 1.28, respectively. During the follow-up period, 64 patients underwent aortic valve reinterventions. The Ross procedure was performed in 32 of 139 patients (23%) undergoing aortic cusp extension valvuloplasty. Other aortic valve replacements were undertaken after 16 aortic cusp extension valvuloplasties (11.5%). Freedom from a second aortic cusp extension valvuloplasty or aortic valve replacement at 18 years was 82.1% +/- 4.2% and 60.0% +/- 7.2%, respectively. CONCLUSION Aortic cusp extension valvuloplasty is a safe and effective surgical option with excellent survival and good long-term outcomes in children and adolescents. The procedure provides acceptable durability and satisfactory freedom from aortic valve replacement.

Is additional pulsatile pulmonary blood flow beneficial to patients with bidirectional Glenn

OBJECTIVE To compare the results of bidirectional Glenn when performed with or without pulsatile pulmonary blood flow in a cohort of patients with a single ventricle. METHODS Records of 212 patients undergoing staged single ventricle palliation during a 10-year period were retrospectively reviewed. Of those, 103 (33 in pulsatile group A and 70 in nonpulsatile group B) were selected. RESULTS Demographics and pre- and intraoperative variables were comparable for both groups. There was no difference in oxygen saturations immediately after the bidirectional Glenn in the 2 groups. The duration and output of chest tube drainage, incidence of chylothorax, and total length of stay was higher in group A. There was no difference in the number of diuretics or oxygen requirement upon discharge between groups. Pre-Glenn measurements showed a mean McGoon ratio in group A of 1.5 (1.46-1.57) and in group B of 1.59 (1.53-1.7) (P = .11); however, there was a significant difference in the ratio between groups at pre-Fontan measurements: group A, 1.76 (1.73-1.79) and group B, 1.6 (1.53-1.66) (P < .05). At pre-Fontan measurements there was a significant difference in mean pulmonary artery pressure between group A (14 mm [12.8-15.2]) and group B (10 mm [9.7-11]) (P < .05) and a trend toward higher incidence of venovenous collaterals in group A. There was no perioperative or interstage mortality in either group. CONCLUSIONS Pulsatile bidirectional Glenn is associated with better pulmonary artery growth, which might improve long-term outcomes after Fontan. However, it was associated with a higher postoperative complication rate.

Hypoplastic Left Heart Syndrome: Feasibility Study for Patients Undergoing Completion Fontan at or Prior to Two Years of Age

BACKGROUND There is limited data regarding the management of children with HLHS (hypoplastic left heart syndrome) and completion Fontan (CF) at or prior to 2 years of age. A study was undertaken to investigate intermediate outcomes. METHODS From August 1999 to December 2008, 52 HLHS survivors underwent extracardiac-conduit CF (29 prior to [group A] versus 23 after 25 months of age [group B]). Mean weight and median follow-up was 9.9 +/- 1.3 kg and 79 months (2 to 112) for group A versus 12.1 +/- 2.2 kg and 87 months (1 to 97) for group B, respectively. Polytetrafluoroethylene conduits were used. Perioperative outcome variables were studied. RESULTS There was no hospital mortality and 1 late death (group B). In group A, 16 CF had 20-mm conduit (vs 18 mm in 13). Thirteen group B patients received 18-mm conduit (vs 20 mm in 10). No CF was taken down. Eight patients in each group required fenestration (p = not significant [NS]). Cardiopulmonary bypass was 78 +/- 37.3 minutes in group A versus 77 +/- 33.9 minutes in group B (p = NS). Mean pulmonary artery size, McGoon ratio, and transpulmonary gradient in group A versus group B were 7.94 +/- 0.59 mm versus 7.87 +/- 0.58 (p = NS), 1.79 +/- 0.2 versus 1.77 +/- 0.19 (p = NS), and 4.1 +/- 1.1 mm Hg versus 3.5 +/- 0.9 (p = NS), respectively. Aspirin and warfarin were used postoperatively. Mean hospital length of stay, intensive care unit length of stay, and pleural drainage duration between groups A and B were the following: 10.9 +/- 5.8 days versus 12.7 +/- 6.1 (p = NS); 5.6 +/- 2.6 days versus 6.7 +/- 2.9 (p = NS); and 7.6 +/- 3.8 days versus 8.7 +/- 4.1 (p = NS), respectively. Between groups, no difference in ventilatory support time, arrhythmia, sinus-atrioventricular node dysfunction, protein-losing-enteropathy, and thromboembolic events were noted. CONCLUSIONS In HLHS patients, extracardiac conduit CF can be performed with good intermediate results at or prior to 2 years of age. Earlier unloading of a univentricular heart by means of CF in patients with collateral accessory flow between systemic and pulmonary circulation might be advantageous for future ventricular function preservation. In addition, relief from early cyanosis might alleviate deleterious effects from a prolonged cyanotic state. Fenestration is less often required without effect in pleural drainage duration. Age at CF had no effect in preoperative pulmonary artery growth and conduit size selection.

Selective Tricuspidization and Aortic Cusp Extension Valvuloplasty: Outcome Analysis in Infants and Children

BACKGROUND Encouraging early outcomes of emerging aortic cusp extension valvuloplasty techniques have redirected attention to nonreplacement strategies in the management of younger patients with aortic insufficiency or aortic stenosis. Outcome analysis after aortic cusp extension valvuloplasty in infants and children was undertaken. METHODS From July 1987 to December 2008, 78 patients younger than 10 years of age underwent aortic cusp extension valvuloplasty in the form of pericardial cusp extension and selective use of tricuspidization. Sixteen (20.5%) patients were younger than 1 year of age. Twenty-seven had bicuspid aortic valve, 34, congenital aortic valve stenosis, and 17, congenital or acquired aortic insufficiency or aortic stenosis. Forty-two patients had balloon valvuloplasty or surgical valvotomy before aortic cusp extension valvuloplasty. Median follow-up was 12.4 years (range, 0.1 to 21.6 years). Freedom from aortic valve replacement (AVR) and determinants of outcome were analyzed. RESULTS There were no early or late deaths. During the follow-up period, 23 patients (29.5%) had Ross operation and 8 patients (10.2%) had other AVR. The z values of left ventricular end-diastolic dimension, aortic annulus, aortic sinus diameter, sinotubular junction diameter, and left ventricular wall thickness before AVR were 3.8 +/- 2.95, 2.1 +/- 1.15, 4.2 +/- 1.22, 1.78 +/- 1.24, and 2.92 +/- 1.31, respectively. Actuarial freedom from AVR at 1, 5, and 10 years was 97.3 +/- 2.0%, 71.3 +/- 5.8%, and 55.6 +/- 6.9%, respectively. CONCLUSIONS Aortic cusp extension valvuloplasty with tricuspidization allows left ventricular reverse remodeling with satisfactory long-term durability and freedom from AVR. Used selectively, it represents a reliable and effective approach in infants and children with congenital or acquired abnormal aortic valve.

Proximal Sano Anastomosis Aneurysm Due to Degeneration of Bovine Pericardial Patch

We report a 5-year-old girl with hypoplastic left heart syndrome who originally underwent a Norwood procedure with a Sano shunt. Subsequent procedures included a bidirectional Glenn and extracardiac Fontan with a Gore-Tex graft (W.L Gore and Associates, Flagstaff, AZ). The patient was found to have a heart murmur on a follow-up visit 3 years later. Echocardiography and computed tomography imaging showed an aneurysm of the proximal Sano anastomosis. The patient underwent successful surgical repair.

The Challenge of Hammock Mitral Valve During Infancy: Precise Preoperative Advanced Imaging and Three-Dimensional Modeling Augments Customized Operative Valve Reconstruction

Hammock mitral valve (MV), also known as anomalous mitral arcade, is a rare congenital anomaly. We report a case of a 10-month-old child who presented with congestive heart failure and was found to have severe mitral stenosis (MS) secondary to a hammock MV anomaly. Detailed advanced imaging with cardiac MRI and three-dimensional (3D) echocardiography favorably navigated a customized valve-sparing surgical reconstruction of the congenitally abnormal MV. Repair of a hammock MV is technically difficult with a guarded prognosis. Surgical experience is extremely limited and variable outcomes are reported. A rare case of severe MS secondary to hammock MV deformity with successful surgical repair and review of worldwide literature are presented.

Restoring Fetal Circulation as a Means of Bridging Treatment Prior to Surgical Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta with Persistent Pulmonary Hypertension of the Newborn

Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital anomaly. Even more rarely reported is its presence in conjunction with persistent pulmonary hypertension of the newborn (PPHN). We present a case of a full-term infant, initially thought to have PPHN and later found to have anomalous origin of the right pulmonary artery from the ascending aorta. We discuss our management concept which included use of PgE1 infusion to restore fetal circulation prior to surgical treatment in this unique clinical scenario.