Midterm results of the modified Ross/Konno procedure in neonates and infants.

BACKGROUND: The management of congenital aortic stenosis in neonates and infants continues to be a surgical challenge. We have performed the modified Ross-Konno procedure for patients who have severe aortic insufficiency or significant residual stenosis after balloon aortic dilation. The midterm results of this procedure were evaluated in this subset of patients. METHODS: Between 1994 and 2010, a total of 24 patients younger than 1 year of age underwent the modified Ross-Konno procedure. The diagnoses were aortic stenosis with or without subaortic stenosis (n = 16), Shone's complex (n = 7), and interrupted aortic arch with subaortic stenosis (n = 1). The aortic root was replaced with a pulmonary autograft, and the left ventricular outflow tract (LVOT) was enlarged with a right ventricular infundibular free wall muscular extension harvested with the autograft. RESULTS: Age at operation ranged from 1 to 236 days (median 28 days). The median follow-up period was 81 months (range 1-173 months). There was 1 early death and no late mortality. Overall the 1-, 2-, and 5-year survival rate was 95% ± 4.5%. Freedom from aortic stenosis was 94.7% ± 5.1% at 1, 2, and 5 years. Less than mild aortic insufficiency was 93.3% ± 6.4% at 2 years, and 74.7% ± 12.9% at 5 years. In total, 23 reoperations and reinterventions were performed; 14 were allograft conduit replacements. Two patients required aortic valve plasty. None required valve replacement. The reintervention-free rate was 64.6% ± 10.8% at 2 years and 36.9% ± 11.3% at 5 years. CONCLUSIONS: Pulmonary autografts demonstrated good durability with low mortality and morbidity. This study shows that the modified Ross-Konno procedure can be a practical choice in selective cases for complex LVOT stenosis in neonates and infants.

Selective right ventricular unloading and novel technical concepts in Ebstein's anomaly.

BACKGROUND: Favorable outcomes in Ebstein's anomaly are predicated on tricuspid valve competence and right ventricular function. Successful valve repair should be aggressively pursued to avoid the morbidity of prosthetic tricuspid valve replacement. We report our experience with valve-sparing intracardiac repair, emphasizing novel concepts and techniques of valve repair supplemented by selective bidirectional Glenn (BDG). METHODS: Between June 1993 and December 2008, 57 nonneonatal patients underwent Ebstein's anomaly repairs. The median age at operation was 8.1 years. All were symptomatic in New York Heart Association (NYHA) functional class II (n = 38), III (n = 17), or IV (n = 1). Preoperatively, 26 had mild or moderate cyanosis at rest. We used a number of valve reconstructive techniques that differed substantially from those currently described. BDG was performed in 31 patients (55%) who met specific criteria. RESULTS: No early or late deaths occurred. At the initial repair, 3 patients received a prosthetic valve. Four patients required reoperation for severe tricuspid regurgitation. Repeat repairs were successful in 2 patients. At follow-up (range, 3 months to 6 years), all patients were acyanotic and in NYHA class I. Tricuspid regurgitation was mild or less in 49 (86%) and moderate in 6 (11%). Freedom from a prosthesis was 91% (52 of 57). CONCLUSIONS: Following a protocol using BDG for ventricular unloading in selected patients with Ebstein's anomaly can achieve a durable valve-sparing repair using the techniques described. Excellent functional midterm outcomes can be obtained with a selective one and a half ventricle approach to Ebstein's anomaly.

Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches.

OBJECTIVES: Patients with a coronary artery arising from the wrong sinus are susceptible to ischemia and sudden death. Risk is higher when the artery courses interarterially--between the pulmonary artery and aorta--has an intramural course, or has an abnormal orifice. In single coronary ostium without intramural course, unroofing and coronary reimplantation are inappropriate, and coronary artery bypass grafting is suboptimal. For this variant, we have devised pulmonary artery translocation. METHODS: A retrospective review of 18 patients undergoing repair between January 1999 and March 2005 was performed. Mean age was 8.1 years (range 6 weeks-16 years). All anomalous arteries coursed interarterially. Ten patients had a right coronary artery from the left coronary sinus; 8 had a left coronary artery from the right sinus. Eleven had an intramural course, and 4 had a single coronary ostium without an intramural course. Ten (56%) patients had symptoms: chest pain (9/10), syncope (3/10), or dyspnea (2/10). Repair was implemented by unroofing (n = 11), reimplantation (n = 3), or pulmonary artery translocation (1 lateral, 3 anterior). All patients were followed up clinically and echocardiographically. RESULTS: At a mean of 2.2 years (2 weeks-5 years), there was no mortality. Symptoms improved and function remained normal in all but 1 patient. He had sustained multiple infarcts in the anomalous artery's distribution and required transplantation despite repair. CONCLUSIONS: Repair is indicated in all patients with coronary insufficiency and in asymptomatic patients with high-risk morphologic abnormalities. We recommend unroofing when an intramural component (or slit-like orifice) is present, reimplantation for separate ostia without an intramural course, and pulmonary artery translocation for single ostium without an intramural course. Coronary artery bypass grafting is thus avoided.

The extracardiac conduit Fontan operation using minimal approach extracorporeal circulation: early and midterm outcomes.

OBJECTIVE: Our approach to the extracardiac conduit Fontan operation has evolved over time from full-pump, to partial-pump, to completely off-pump. This study is designed to report our overall experience with the extracardiac conduit Fontan operation and to evaluate the evolution in bypass technique on postoperative outcomes. METHODS: From September 1992 to April 2005, 285 patients, median age 4.5 years (1.4-44 years), median weight 16 kg (9.4-94 kg), underwent a primary extracardiac conduit Fontan procedure. Early and late outcomes were analyzed for the entire cohort and for 2 patient groups depending on whether an oxygenator was used in the bypass circuit (166 patients; 58%) or not (119 patients; 42%). RESULTS: Early failure (including death and takedown) occurred in 7 patients (2.5%). Prevalence of new early postoperative sinus node dysfunction necessitating a permanent pacemaker was 0.4%, and that of new tachyarrhythmias necessitating discharge home on a regimen of antiarrhythmia medications was 2.5%. Ten-year actuarial freedom from Fontan failure, new sinus node dysfunction necessitating a permanent pacemaker, and reoperation for conduit thrombosis or stenosis was 90%, 96%, and 98%, respectively. Fenestration rate was lower (P = .001) in the no-oxygenator group (8%) than in the oxygenator group (25%). Patients in the no-oxygenator group had lower intraoperative Fontan pressure (12.0 +/- 2.3 vs 13.5 +/- 2.4 mm Hg, P < .001), common atrial pressure (4.6 +/- 1.8 vs 5.3 +/- 1.8 mm Hg, P = .003), and transpulmonary gradient (7.5 +/- 2.1 vs 8.3 +/- 2.2 mm Hg, P = .013) than did the oxygenator group. CONCLUSIONS: The extracardiac conduit Fontan operation coupled with minimal use of extracorporeal circulation is associated with favorable intraoperative hemodynamics, low fenestration rate, minimal risk of thrombosis or stenosis, and minimal early and late rhythm disturbance.

Unifocalization of major aortopulmonary collaterals in single-ventricle patients.

BACKGROUND: Unifocalization of major aortopulmonary collateral arteries (MAPCAs) in pulmonary atresia with ventricular septal defect and intracardiac repair has become the standard of care. However, there are no reports addressing unifocalization of MAPCAs in single-ventricle patients. It is unknown whether their pulmonary vascular bed can be reconstructed and low enough pulmonary vascular resistance achieved to allow for superior or total cavopulmonary connections. METHODS: We reviewed data on all patients with functional single ventricles and unifocalization procedures of MAPCAs. From 1997 to 2005, 14 consecutive children with various single-ventricle anatomies were operated on. RESULTS: Patients had a median of three surgical procedures (range, 1 to 5). Two patients had absent, all others diminutive central pulmonary arteries, with an average of 3.5 +/- 1.2 MAPCAs. Seven patients (50%) had bidirectional Glenn procedures, and 3 of these had Fontan procedures. Median postoperative pulmonary artery pressures measured 12.5 mm Hg (Glenn) and 14 mm Hg (Fontan), respectively. Six patients are alive today (46%), with 1 patient lost to follow-up. Three patients died early and 3 late after initial unifocalization to shunts. One other patient survived unifocalization, but was not considered a candidate for a Glenn procedure and died after high-risk two-ventricle repair. Another patient with right-ventricle-dependent coronary circulation died of sepsis late after Glenn. CONCLUSIONS: In selected patients with functional single ventricles and MAPCAs, the pulmonary vascular bed can be reconstructed sufficiently to allow for cavopulmonary connections. Venous flow to the pulmonary vasculature decreases cardiac volume load and is likely to increase life expectancy and quality of life for these patients.

Homograft valved right ventricle to pulmonary artery conduit as a modification of the Norwood procedure.

BACKGROUND: The use of a right ventricle to pulmonary artery (RV-PA) conduit in the Norwood procedure has been proposed to increase postoperative hemodynamic stability. A valve within the conduit should further decrease RV volume load. We report our clinical experience with this modification. METHODS AND RESULTS: From February 2002 through August 2005, we performed 88 consecutive Norwood procedures using RV-PA conduits. We used composite valved conduits made from cryopreserved homograft and polytetrafluoroethylene (PTFE) in 66 cases (54 pulmonary, 12 aortic homografts), other valved conduits in 14, and unvalved PTFE in 8 cases. Hospital survival was 88.6% overall and increased to 93.1% after the initial year. Early interventions were required in 18 patients (16 for cyanosis). Prestage II cardiac catheterization was performed at a mean age of 126 days. Mean Qp/Qs was 1, with mean aortic saturation 71%, mean O2 extraction 24%, and mean right ventricular end-diastolic pressure 9 mm Hg. Patient weight, use of an aortic homograft valve in the conduit, stage I palliation within the first year of our experience, and low O2 extraction and high transpulmonary gradient prestage II were risk factors for overall death. Early interventions were more frequent in aortic valve conduits compared with all other conduits. CONCLUSIONS: The valved RV-PA conduit was associated with low early mortality after the Norwood procedure. The majority of these patients had normal cardiac output and well-maintained RV function. There may be a higher risk for early conduit interventions and death when aortic valve homografts are used in the RV-PA conduit.

Induced fibrillation is equally effective as crystalloid cardioplegia in the protection of fetal myocardial function.

BACKGROUND: Fetal cardiac intervention represents a potential advance in the treatment of congenital cardiac lesions that increase in complexity during development. Prenatal repair of a primary defect might prevent pathologic blood-flow patterns that can result in hypoplasia of a cardiac chamber or great vessel. However, strategies to optimize fetal myocardial protection have not been studied. A biventricular working fetal heart preparation was used to evaluate the cardioprotective properties of induced fibrillation and crystalloid cardioplegia. METHODS: Hearts from 16 fetal lambs at 115 to 125 days' gestation were harvested and perfused with Krebs-Henseleit solution. The descending aorta was ligated distal to the ductal insertion and the branch pulmonary arteries were ligated to simulate the parallel circulation of the fetus. Hearts were arrested with normothermic fibrillation (n = 8) or hypothermic crystalloid cardioplegia (n = 8) before reperfusion with Krebs-Henseleit solution. Baseline and postarrest myocardial function measurements were obtained from analysis of pressure-dimension relationships. RESULTS: Fibrillatory and cardioplegic arrest were equally effective at preserving postarrest systolic function (left ventricle, 70% +/- 5% vs 68% +/- 15%, P =.52; right ventricle, 68% +/- 4.5% vs 65% +/- 4.5%, P =.26) and preventing increased diastolic stiffness (left ventricle, 32% +/- 5.3% vs 38% +/- 11%, P =.24; right ventricle, 25% +/- 3.3% vs 27% +/- 2.1%, P =.46). Myocardial water content was unchanged in hearts arrested with fibrillation and cardioplegia (84% +/- 1.5% vs 83.7% +/- 0.9%, P =.71). CONCLUSIONS: Normothermic fibrillation and hypothermic crystalloid cardioplegia provide equal protection of the fetal myocardium. In the setting of diminished fetal myocardial reserve and because of the limited ability to manipulate the surrounding temperature in the fetus, normothermic fibrillation may be preferable for in utero repairs of selected congenital heart defects.

Fetal myocardial protection is markedly improved by reduced cardioplegic calcium content.

BACKGROUND: Fetal cardiac surgery holds a clear therapeutic benefit in the treatment of lesions that increase in complexity due to pathologic blood flow patterns during development. Fetal and neonatal myocardial physiology differ substantially, particularly in the regulation of myocardial calcium concentration. To examine issues of calcium homeostasis and fetal myocardial protection, a novel isolated biventricular working fetal heart preparation was developed. METHODS: Hearts from 20 fetal lambs, 115 to 125 days gestation, were harvested and perfused with standard Krebs-Henseleit (K-H) solution. The descending aorta was ligated distal to the ductal insertion and the branch pulmonary arteries were ligated to mimic fetal cardiovascular physiology. Hearts were arrested for 30 minutes with normocalcemic (n = 8), hypocalcemic (n = 6), or hypercalcemic (n = 6) cold crystalloid cardioplegia before reperfusion with K-H solution. RESULTS: Compared with normocalcemic cardioplegia, hypocalcemic cardioplegia improved preservation of left ventricular (LV) systolic function (88% +/- 2.2% vs 64% +/- 15% recovery of end-systolic elastance, p = 0.02), diastolic function (12% +/- 21% vs 38% +/- 11% increase in end-diastolic stiffness, p = 0.04), and myocardial contractility (97% +/- 9.6% vs 75.2% +/- 13% recovery of preload recruitable stroke work [PRSW], p = 0.04). In contrast, the fetal myocardium was sensitive to hypercalcemic arrest with poor preservation of LV systolic function (37.5% +/- 8.4% recovery of elastance), diastolic function (86% +/- 21% increased stiffness), and overall contractility (32% +/- 13% recovery of PRSW). Myocardial water content was reduced in hearts arrested with hypocalcemic cardioplegia (79% +/- 1.8% vs 83.7% +/- 0.9%, p = 0.0006). CONCLUSIONS: This study demonstrates the sensitivity of the fetal myocardium to cardioplegic calcium concentration. Hypocalcemic cardioplegia provides superior preservation of systolic, diastolic, and contractile function of the fetal myocardium.

Surgical creation of aortopulmonary window in selected patients with pulmonary atresia with poorly developed aortopulmonary collaterals and hypoplastic pulmonary arteries.

OBJECTIVE: The morphologic characteristics of the pulmonary circulation vary widely in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. Although we favor single-stage unifocalization and complete repair as the procedure of choice, a subgroup of patients who meet specific criteria have been treated with initial surgical creation of an aortopulmonary window. METHODS: Eighteen patients who were considered unsuitable candidates for single-stage repair underwent surgical creation of an aortopulmonary window. Selection criteria included the following: (1) presence of centrally confluent true pulmonary arteries 1.0 to 2.5 mm in diameter, with a well-developed peripheral arborization pattern; (2) multiple small aortopulmonary collateral vessels, most of which communicated with the true pulmonary arterial system; and (3) the presence of marked cyanosis. RESULTS: There were no early deaths, and the 2 late deaths were both unrelated to the procedure. Follow-up angiography (n = 17) demonstrated good growth of true pulmonary arteries in 15 of 17 cases (88%). Mean pulmonary artery diameter increased from 1.76 mm to 3.45 mm. Subsequent operations have been performed in 15 of 18 cases (83%). Intracardiac repair with or without additional unifocalization was achieved in 8 of these 15 (53%). Seven patients (47%) have undergone staged unifocalization as the next procedure; of these, 3 were able to have intracardiac repair. Thus 11 of the 15 patients who have undergone second operations (73%) have had complete intracardiac repair. CONCLUSIONS: The initial surgical creation of an aortopulmonary window in carefully selected patients can increase the size of the true pulmonary arteries, making these patients better candidates for eventual intracardiac repair. The procedure should be avoided in patients with pulmonary overcirculation, a predominance of isolated supply collaterals, or true pulmonary arteries larger than 2.5 mm in diameter, and it is not applicable without a true pulmonary artery central confluence.

Cavopulmonary anastomosis induces pulmonary expression of the angiotensin II receptor family.

BACKGROUND: Cavopulmonary anastomosis is used for palliation of cyanotic cardiac lesions. Postoperative development of pulmonary arteriovenous malformations can be significant in 10% to 25% of patients. To study the basis for formation of arteriovenous malformations, we developed an ovine model that reliably induces their development 8 weeks after cavopulmonary anastomosis. Previously, we found that cavopulmonary anastomosis inhibits the expression of pulmonary angiotensin-converting enzyme and suppresses angiotensin II production. OBJECTIVE: This study examines the role of the angiotensin II receptors, type 1 and type 2, in this setting of pulmonary vascular remodeling. METHODS: Lambs, aged 40 to 50 days, underwent cavopulmonary anastomosis. In age-matched control animals, a sham operation was performed. Messenger RNA and protein expression in lung specimens was measured at successive time points after cavopulmonary anastomosis or sham operations (n = 3 at each time point). RESULTS: Angiotensin type 1 mRNA was maximally upregulated 2-fold at 5 weeks after cavopulmonary anastomosis (P =.006). Expression of angiotensin type 1 protein was increased at least 2-fold at 2, 5, and 15 weeks after cavopulmonary anastomosis (P =.005). Cavopulmonary anastomosis also increased angiotensin type 2 mRNA and protein expression at least 2-fold at 2 and 5 weeks (P =.02) after surgical intervention. At 15 weeks, expression of angiotensin type 2 mRNA and protein was unchanged from that seen in control animals. Immunolocalization in pulmonary tissue sections 2 weeks after cavopulmonary anastomosis revealed markedly enhanced staining of angiotensin II receptor type 1 in vascular smooth muscle and angiotensin II receptor type 2 in the endothelium of pulmonary arteries. CONCLUSIONS: Rapid elevation in the expression of the type 1 and 2 angiotensin II receptors in the affected pulmonary vasculature after cavopulmonary anastomosis suggests their involvement in the pathologic vascular remodeling that occurs after cavopulmonary anastomosis.

Robotic-assisted endoscopic thoracic aortic anastomosis in juvenile lambs.

BACKGROUND: Advances in robotic technology have enabled a wider range of applications for minimally invasive techniques in cardiac surgery, including mitral valve repair and coronary artery bypass grafting. With increased technical sophistication, robotic-assisted techniques can be developed for the endoscopic repair of certain congenital cardiac lesions. OBJECTIVE: The purpose of this study was to assess the feasibility of closed chest thoracic aortic anastomosis in a juvenile ovine model. METHODS: Lambs, aged 45 to 55 days, underwent surgery that was performed using the da Vinci robotic surgical system. Using 3 ports, the surgeon dissected the descending thoracic aorta and mobilized it free from attachments, using single-lung ventilation and CO2 insufflation. Snares were introduced through 2 stab wounds for aortic occlusion proximally and distally. In 4 lambs, the aorta was completely transected and reanastomosed using interrupted nitinol sutures. One lamb underwent longitudinal aortotomy, and patch aortoplasty was performed with the placement of a Gore-Tex patch. Snares were released and the animals were recovered once hemodynamically stable. Animals were sacrificed at 6 to 12 hours after surgery and the descending aorta was harvested. Burst-pressure testing was performed on the anastomoses. RESULTS: All 5 lambs survived the procedure with stabilization of hemodynamic parameters following surgery. The mean aortic clamp time was 47 +/- 17 minutes, and the anastomosis was completed in 26 +/- 5 minutes. The mean burst pressure was 163 +/- 9 mm Hg. CONCLUSIONS: Endoscopic thoracic aortic anastomosis can be performed safely and with adequate exposure in a juvenile large-animal model using computer-assisted surgical techniques. With further refinements, these approaches could be applied to the repair of congenital anomalies of the aorta, including interrupted aortic arch and aortic coarctation.