Coil occlusion versus conventional surgical closure of patent ductus arteriosus.

This retrospective cohort study evaluated the clinical outcome and cost-effectiveness of 2 treatment strategies for children with an isolated restrictive patent ductus arteriosus. Results indicate a superior cost-effectiveness of transcatheter coil occlusion compared with conventional surgery for these patients.

Decreased incidence of postoperative pericardial effusions after cardiac surgery for congenital heart disease.

There has been a striking decrease in the incidence of postoperative pericardial effusion to 13.6%. A higher incidence of postoperative pericardial effusion was found in the winter months.

Aortic root dilation after the Ross procedure.

This study evaluated changes in neoaortic root geometry in patients who underwent the Ross procedure. Serial postoperative echocardiographic measurements of the neoaortic root indexed to the square root of body surface area (centimeters divided by meters) were obtained from 30 patients (age range 3.1 to 31.4 years) and compared with paired preoperative and immediate postoperative values. Normal aortic root diameter Z scores were derived from root dimensions obtained from 217 healthy controls. Compared with preoperative values, an immediate stretch of the neoaortic versus pulmonary root (annulus and sinuses of valsalva) was observed at a mean follow-up period of 1 week. Additional aortic annular dilation from baseline prehospital discharge values was observed at 2 to 12 months (baseline vs follow-up annulus Z score: 1.4 vs 2.6, p <0.01, n = 16) and at 16 to 33 months follow-up (0.8 vs 2.0, p <0.05, n = 12). In a similar fashion, there was additional enlargement of the aortic sinus from its stretched state at hospital discharge at 2 to 12 months (baseline vs follow-up sinus Z score: 2.0 vs 3.3, p <0.01, n = 17) and at 16 to 33 months (1.7 vs 3.0, p <0.01, n = 13). There were no differences in root size between 2 to 12 and 16 to 33 months after surgery. There was a decrease in left ventricular size with no alteration in blood pressure or degree of aortic valve regurgitation. Thus, aortic root dilation occurs up to the first year after the Ross procedure but does not appear to progress beyond this time.

Delayed sternal closure after neonatal cardiac operations.

We retrospectively compared the use of primary elective open sternum coupled with delayed sternal closure with the use of primary sternal closure in neonates after cardiac operations. Primary elective open sternum/delayed sternal closure was selectively used in patients who demonstrated hemodynamic or respiratory deterioration, or both, during an intraoperative trial of sternal closure; otherwise primary sternal closure was used. Primary elective open sternum was used in 55 (61.8%) and primary sternal closure in 34 (38.2%) of the 89 patients studied. Eleven (20%) patients having primary elective open sternum died compared with 5 (14.7%) patients having primary sternal closure (p = 0.6). Six (10.9%) of the patients with primary elective open sternum died before delayed sternal closure; the remaining 49 patients comprise the primary elective open sternum/delayed sternal closure group. The durations of mechanical ventilation (9.7 +/- 0.9 days [mean plus or minus standard error of the mean], median 7.7 versus 9.9 +/- 3.4 days, median 4.9; p = 0.0005) and hospital stay (21.1 +/- 1.4 days, median 17.7 versus 19.6 +/- 4.1 days, median 12.9; p = 0.004) were shorter in the primary sternal closure group. The overall morbidity and duration of inotropic support were not significantly different between the two groups, although seven (20.6%) of the patients with primary sternal closure did have to undergo delayed sternal reopening for refractory postoperative low cardiac output. There was one superficial wound infection in the primary elective open sternum/delayed sternal closure group. Primary elective open sternum/delayed sternal closure is an effective treatment for postoperative neonatal mediastinal compression for the following reasons: (1) the morbidity is low; (2) the mortality of the critically ill group of neonates in whom primary elective open sternum/delayed sternal closure was used was similar to that of the less critically ill primary sternal closure group; and (3) 20.6% of the primary sternal closure group eventually had to undergo delayed sternal reopening to treat refractory postoperative low cardiac output.

Endothelial cell-lined skeletal muscle ventricles in circulation.

Skeletal muscle ventricles were constructed from the latissimus dorsi in six dogs by wrapping the muscle around a polypropylene mandrel. Jugular vein endothelial cells were harvested enzymatically and grown in tissue culture. After 3 weeks of vascular delay and 4 weeks of electrical conditioning, five skeletal muscle ventricles were seeded with 5 to 8 x 10(6) autologous endothelial cells by percutaneous injection of a cellular suspension into the lumen of the skeletal muscle ventricle; one skeletal muscle ventricle was injected with culture medium alone as an unseeded control. The autologous endothelial cells were all prelabeled with a lipid-bound cellular marker, PKH-26. After an additional 4 weeks of electrical conditioning, the mandrels were removed and the skeletal muscle ventricles were connected to the descending thoracic aorta and activated to contract during cardiac diastole at a 1:2 ratio with the heart. After 3 hours of continuous pumping, mean diastolic pressure was increased by 35% (58 +/- 7 versus 78 +/- 6 mm Hg, p < 0.05). At this time, the skeletal muscle ventricles were excised for histologic examination. Sections stained with hematoxylin and eosin revealed a continuous cellular layer lining the skeletal muscle ventricle; no cells were present on the lumen of the control skeletal muscle ventricle. All seeded skeletal muscle ventricles exhibited fluorescence as a result of the PKH-26 cellular marker. Immunofluorescent staining with antibodies to von Willebrand factor and ultrastructural analysis with an electron microscope confirmed the endothelial character of these cells lining the lumen of the skeletal muscle ventricles. The ability to create endothelial cell-lined muscular pumping chambers holds important implications for the resolution of thrombotic events in cardiac assist devices as well as toward the clinical application of skeletal muscle ventricles.

Presentation, clinical features and outcome in different patterns of atherosclerotic renovascular disease.

Atherosclerotic renovascular disease (ARD) is an increasingly important cause of renal failure. However, important features of the clinical presentation are not fully described, and the outcome after intervention by angioplasty remains controversial. Ninety-four patients with ARD diagnosed at angiography were reviewed. Twenty-four patients were diabetic. Thirty-nine patients had unilateral renal artery stenosis or occlusion (group A), 28 had bilateral stenosis (group B), and 27 had unilateral occlusion plus contralateral occlusion or stenosis (group C). Two years after presentation, actuarial patient survival was 96%, 74.3% and 47.1% in groups A, B and C, respectively (p < 0.001 for all differences); actuarial renal survival in surviving patients was 97.3%, 82.4% and 44.7%, respectively (p < 0.001 for all differences). Percutaneous transluminal balloon angioplasty (PCTA) was performed in 74 patients. Renal function improved in only a minority of cases, but was stable in 73% of nondiabetic patients 12 months after PCTA. Angioplasty was less effective in diabetic subjects, with only 53.3% having stable renal function at 12 months follow-up. Renal and patient survival were strongly related to the initial angiographic findings. In non-diabetic subjects, PCTA resulted in stabilization of renal function for at least one year in nearly three-quarters of cases, which suggests a benefit from intervention in this disease whose natural history is otherwise of progression.

Cardiac pacemaker dysfunction in children after thoracic drainage catheter manipulation.

Two children underwent placement of permanent, epicardial-lead, dual-chamber, unipolar pacemaker systems for complete heart block. Postoperatively, both patients demonstrated subcutaneous emphysema-in the area of their pulse generators-temporally related to thoracic catheter manipulation. Acutely, each situation was managed with manual compression of the pulse generator, ascertaining appropriate pacemaker sensing and pacing. Maintenance of compression with pressure dressings, vigilant observation/monitoring, and education of the care givers resulted in satisfactory pacemaker function without invasive intervention.

Congenital Heart Surgery Nomenclature and Database Project: double outlet left ventricle.

Double outlet left ventricle (DOLV) is a type of ventriculoarterial connection in which both great arteries arise entirely or predominantly from the left ventricle. Although it was initially believed that bilateral absence of conus is a prerequisite for such diagnosis, all possible conal configurations have been described in this malformation. The morphology of DOLV is encompassed by a careful description of the ventricular septal defect (VSD) with its relationship to the semilunar valves, the presence or absence of pulmonary outflow tract obstruction (POTO) and aortic outflow tract obstruction (AOTO), and the presence or absence of associated cardiac lesions. The preferred surgical treatment involves the connection of the right ventricle to the pulmonary circulation by an intraventricular tunnel repair connecting the VSD to the pulmonary semilunar valve. This ideal surgical therapy is not always possible, because of the presence of confounding anatomical barriers. Several alternative surgical procedures have been devised to accommodate these more complex situations. A framework for the development of the DOLV module of a pediatric cardiac surgical database is proposed.

Risk factors for failure of aortic valvuloplasty in aortic insufficiency with ventricular septal defect.

BACKGROUND: Aortic insufficiency (AI) associated with ventricular septal defect (VSD) is often repaired using a modification of Trusler's aortic valvuloplasty technique. The purpose of this study was to identify the risk factors for failure of aortic valve (AV) repair in patients who underwent repair of associated VSD. METHODS: A univariate analysis was performed in this retrospective study to identify the possible risk factors for failure of the repair resulting in the need for AV replacement (AVR). RESULTS: The study included 24 patients, 15 (62%) boys and 9 (38%) girls, with a mean age of 9.1 +/- 1.2 (SEM) years. The VSD was perimembranous in 15 (62%) and subarterial in 9 (38%). The prolapsed aortic cusp was the right in 13 (54%), the noncoronary in 6 (25%), and both in 5 (21%). Plication was performed at one end of the free edge of the prolapsed cusp(s) in 12 (50%) and at more than one end in 12 (50%) of the patients. The VSD was closed by use of a patch in 21 (88%) and by direct suture closure in 3 (12%). At the mean follow up of 7.3 +/- 1.3 years, the degree of AI was none in 6 (25%), trivial in 5 (21%), mild in 9 (38%), moderate in 1 (4%), and severe in 3 (12%). The 15-year actuarial freedom from reoperation was 81% +/- 19% (95% confidence limit). By univariate analysis, the possible risk factors for AV repair failure were the degree of AI at hospital discharge (p = 0.004), direct closure of the VSD (p = 0.061), smaller size of the VSD (p = 0.081), and plication of more than one end of the prolapsed cusp(s) (p = 0.095). CONCLUSIONS: Trusler's AV repair is an effective and durable technique for the surgical treatment of patients with VSD-AI syndrome. The adequacy of the initial repair is the most important determinant of the long-term results.

Bidirectional cavopulmonary anastomosis with intracardiac repair.

BACKGROUND: Patients with hypoplasia of the right ventricle and tricuspid valve have historically undergone a total cavopulmonary connection or a two-ventricle repair with atrial fenestration. METHODS: We reviewed our experience with 9 patients with hypoplasia of the right ventricle and tricuspid valve who underwent a bidirectional cavopulmonary anastomosis with intracardiac repair. Patient diagnoses included pulmonary atresia with intact ventricular septum (n = 3); hypoplastic right ventricle and tricuspid valve with atrial septal defect, ventricular septal defect, and right ventricular outflow tract obstruction (n = 3); unbalanced atrioventricular canal defect (n = 1); inlet ventricular septal defect with coarctation (n = 1); and tricuspid stenosis with atrial septal defect (n = 1). RESULTS: The median age at operation was 36 months. There was hypoplasia of the right ventricle and tricuspid valve in all patients. The tricuspid valve measured 56.5% of normal (range, 43.6% to 70.4%) by echocardiography, and the median ratio of the tricuspid valve to the mitral valve was 0.67 (range, 0.54 to 0.82). At operation, the median tricuspid valve annulus diameter was 65.6% of published autopsy normals (range, 57.8% to 78.5%) with a median Z value of -3.8 (range, -6.6 to -2.1). All patients survived operation. At a median follow-up of 16 months, 5 patients are asymptomatic, and 2 have occasional early-morning periorbital edema. Two patients are on a regimen of diuretics, 1 of whom is also taking an unloading agent. The patient with unbalanced atrioventricular canal died suddenly at home 6 months postoperatively. CONCLUSIONS: Bidirectional cavopulmonary anastomosis with intracardiac repair may avoid the long-term complications associated with the Fontan modifications and eliminates the need of atrial fenestration in most instances. This operation should be considered for select patients with hypoplasia of the right ventricle and tricuspid valve.

Coronary sinus ostial atresia with persistent left superior vena cava.

BACKGROUND: Atresia of the coronary sinus orifice with a persistent left superior vena cava is an intrinsically benign cardiac anomaly with important surgical implications. METHODS: THe medical records of 5 patients with atresia of the coronary sinus orifice with a persistent left superior vena cava were reviewed retrospectively, and a computer search of the world literature describing this cardiac malformation was undertaken. RESULTS: The 5 patients ranged in age from 9 months to 5 years. In 2, the diagnosis was made preoperatively by angiocardiography, and in 3, the abnormality was found incidentally at the time of cardiotomy for repair of associated congenital heart disease. Four of the 5 patients underwent repair of associated cardiac lesions. During operation, care was taken to avoid disruption of left superior vena cava flow to prevent coronary venous obstruction. All patients survived and are doing well at follow-up. CONCLUSIONS: Atresia of the coronary sinus orifice with persistent left superior vena cava is, in itself, a benign anomaly without physiologic consequence. However, the recognition of this lesion during repair of associated cardiac lesions is of vital importance to the cardiac surgeon. Interruption of this sole route of coronary venous drainage can potentially lead to myocardial ischemia and necrosis.

Arch growth after staged repair of interrupted aortic arch using carotid artery interposition.

BACKGROUND: Between 1980 and 1990, our practice was to perform carotid artery interposition as part of a staged repair of interrupted aortic arch with various associated cardiac defects. METHODS: This procedure was used in 16 patients with IAA type B. The median age at operation was 4.5 days and the median weight, 3.2 kg. Ten of the patients had an associated ventricular septal defect. Six more had complex anatomy. There were two death at carotid interposition, two interim deaths, and two deaths after intracardiac repair. Preoperative echocardiographic and angiographic studies were compared with postoperative studies in 11 survivors of arch repair to assess sequential growth of the interposed carotid artery. Measurements of the carotid artery were normalized to the descending aorta. RESULTS: Preoperatively, the left carotid artery had a median diameter of 3.7 mm and was 42.9% of the descending aortic diameter. Postoperative studies performed at a median age of 5.7 months disclosed that the interposed carotid artery had grown to a median diameter of 7.0 mm and was 69.6% of the diameter of the descending aorta (normal > or = 81%). On follow-up at a median time of 4 years, 6 of 9 patients have no gradient by blood pressure measurements or echocardiographic Doppler studies, and 3 have modest gradients. No patient has required revision of the arch repair. CONCLUSIONS: Survival is good after carotid artery interposition for interrupted aortic arch and growth of the carotid artery approaches that of a normal arch. Carotid artery interposition is a viable alternative for repair of this lesion should primary definitive repair not be feasible.

Repair of an unusual type of total anomalous pulmonary venous connection.

A 9-day-old, 4.5-kg female infant was diagnosed with total anomalous pulmonary venous connections by echocardiography. The unusual nature of the pulmonary veins and their connections led to cardiac catheterization and angiography. Angiography demonstrated the left-sided veins connected, via a retropulmonary artery vertical vein, to the left inominate vein whereas the right-sided pulmonary veins connected to the right superior vena cava. We repaired this lesion uneventfully by creating a neo-common pulmonary vein and anastomosing this confluence to the left atrium.

Influence of a delay on latissimus dorsi muscle flap blood flow.

The regional and total blood flow of canine latissimus dorsi muscle flaps (LDMFs) were examined to study the effect of a delay procedure, ligation of the perforators, before complete flap elevation. The regional blood flow of the middle and distal regions of the nondelayed LDMFs was poor and significantly lower than the proximal region at rest. The regional blood flow during the exercise test was improved and significantly higher in the middle (62% increase) and distal regions (187% increase) of the delayed LDMFs as compared with the nondelayed LDMFs. The mean total blood flow of the delayed LDMFs was 14 mL.min-1.100(-1) g at rest, increased to 30 mL.min-1.100(-1) g during exercise tests with intermittent burst electrical stimulation, and was maximal at 40 mL.min-1.100(-1) g immediately after the exercise. The phasic arterial blood flow of the delayed LDMFs was inhibited during contraction at 9 mL.min-1.100(-1) g, whereas it was 44 mL.min-1.100(-1) g during relaxation. In contrast, the phasic venous blood flow was accentuated during contraction to 32 mL.min-1.100(-1) g compared with 16 mL.min-1.100(-1) g during relaxation.

Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle.

Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both great vessels arise entirely or predominantly from the right ventricle. Although the presence of aortic-mitral discontinuity and bilateral coni are important descriptors, they should not serve as absolute prerequisites for the diagnosis of DORV. The morphology of DORV is encompassed by a careful description of the ventricular septal defect (VSD) with its relationship to the semilunar valves, the great artery relationships to each other, the coronary artery anatomy, the presence or absence of pulmonary outflow tract obstruction (POTO) and aortic outflow tract obstruction (AOTO), the tricuspid-pulmonary annular distance, and the presence or absence of associated cardiac lesions. The preferred surgical treatment involves the connection of the left ventricle to the systemic circulation by an intraventricular tunnel repair connecting the VSD to the systemic semilunar valve. This ideal surgical therapy is not always possible due to the presence of confounding anatomical barriers. A multitude of alternative surgical procedures has been devised to accommodate these more complex situations. A framework for the development of the DORV module for a pediatric cardiac surgical database is proposed.

Pediatric cardiac surgical ECMO: multivariate analysis of risk factors for hospital death.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) has emerged as an effective technique for the mechanical support of many pediatric postcardiotomy patients with medically refractory cardiac failure. METHODS: We retrospectively reviewed the records of 73 pediatric patients with congenital heart disease who were placed on ECMO support between August 1984 and February 1994. The patients were divided into groups defined by the timing of ECMO cannulation relative to the time of operation. Group 1 patients (n = 7, 9.6%) were placed on ECMO preoperatively. Group 2 patients (n = 66, 90.4%) were a heterogeneous population placed on ECMO at any interval after cardiac repair. Subgroup 2A consisted of patients (n = 17, 25.8%) who could not be weaned from cardiopulmonary bypass and were converted directly to ECMO support immediately after repair. Subgroup 2B patients (n = 49, 74.2%) were cannulated postoperatively after an initial period of clinical stability. RESULTS: Hospital survival for all study patients (42/73) and for group 2 patients (38/66) was 58%. Only 4 group 2A patients (23.5%) survived their hospitalization compared with 34 group 2B patients (69.4%) (p = 0.001). Multivariate analysis identified elevated right atrial pressure after ECMO decannulation (p = 0.049) and, possibly, membership in group 2A (p = 0.061) as independent risk factors for hospital death. CONCLUSIONS: Extracorporeal membrane oxygenation is most effective in salvaging pediatric cardiac surgical patients who demonstrate medically refractory hemodynamic deterioration at some interval after being successfully weaned from cardiopulmonary bypass. The right atrial pressure after extracorporeal membrane oxygenation decannulation is an independent predictor of hospital death.

The Ross procedure in children and young adults with congenital aortic valve disease.

METHODS: Thirty-three children and young adults with congenital aortic valve disease underwent pulmonary autograft replacement of the aortic valve between October 1993 and March 1997. There wer six females and 27 males; at operation, median age was 16 years (range: 3 to 41 years) and median body weight 60 kg (range: 14 to 121 kg). Fifteen patients (46%) had undergone one or more previous cardiac surgical procedures. A bicuspid aortic valve was present in 31 patients (94%); moderate to severe aortic stenosis and regurgitation was present in 10 (30%) and 26 (79%), respectively. RESULTS: All patients underwent the Ross procedure while in NYHA class I (64%) or class II (36%). A preoperative shortening fraction of 41 +/- 1.4% suggested well-preserved systolic function, but the mean left ventricular end-diastolic pressure of 16.6 +/- 1.3 mmHg was consistent with preoperative left ventricular pressure and volume overload. The aortic root was replaced using an interrupted suture technique in two patients and with three separate running sutures in 31. The right ventricular outflow tract was reconstructed in all classes with a cryopreserved pulmonary homograft valved conduit (median diameter 23 mm; range 19 to 30 mm). Intraoperative complications included transient atrioventricular dissociation (one), permanent atrioventricular dissociation (one), and left coronary artery distortion relieved by shortening the distal ascending aorta (one). Postoperatively, postpericardiotomy syndrome developed in six patients (18%), supraventricular tachycardia in three (9%), and ventricular tachycardia in one (3%). At three days after surgery, one patient developed ischemic left ventricular dysfunction requiring repositioning of the distorted left coronary artery higher on the neo-aortic root. Hospital survival rate was 100%. During a median follow-up of 17 months (range: 1 to 41 months) one patient suffered a non-cardiac death due to blunt trauma. there has been a significant postoperative improvement in NYHA class among surviving patients (class I, 94%; class II, 6%; p = 0.004 versus preoperative). Postoperative aortic regurgitation was absent or trivial in 17 (60%) and mild in the remaining 11 (40%) patients for whom follow-up echocardiographic data are available. One patient required reoperation 16.5 months after the Ross procedure to replace a rapidly degenerating pulmonary homograft, and one with moderately severe homograft stenosis and five with mild homograft stenosis are being monitored. Postoperatively, a gradual early expansion in the diameter of the neo-aortic root and reduction in echocardiographic indices of left ventricular hypertrophy and dilatation occurred. CONCLUSIONS: Pulmonary autograft replacement of the aortic valve in young patients with congenital aortic valve disease has produced excellent short-term anatomic/physiologic results and symptomatic relief with no mortality. Indices of left ventricular dilatation and hypertrophy regress after repair when the Ross operation precedes important deterioration in preoperative ventricular function. Important technical considerations include: (i) the native distal ascending aorta should be sufficiently shortened before performing the distal aortic anastomosis; and (ii) the left coronary anastomosis should be positioned relatively high on the neo-aortic root with a slight amount of tension. Both of these maneuvers reduce the likelihood of coronary artery distortion. Rapid degeneration of the pulmonary homograft and the propensity towards progressive dilatation of the neo-aorta are important postoperative considerations. Until more is known about the etiology and natural history of these two potential complications, postoperative anti-inflammatory and/or immunosuppressive therapy and strict control of hypertension should be strongly considered.

Skeletal muscle ventricles seeded with autogenous endothelium.

Skeletal muscle ventricles (SMVs) are muscular pumping chambers constructed from skeletal muscle. Previously, SMVs were connected to the systemic circulation with vascular conduits and used to assist the heart. In this study, SMVs were constructed from the latissimus dorsi muscle in eight dogs. The SMVs were seeded with autologous endothelial cells, but not connected to the circulation. Endothelial cells were harvested enzymatically from autogenous external jugular vein and grown in tissue culture. After 9 weeks, 6 electrically conditioned SMVs were seeded with endothelial cells by injecting 4-5 ml of culture medium containing 5-8 x 10(6) autogenous endothelial cells into each SMV lumen adjacent to the mandrel. Conditioning was stopped at the time of endothelial seeding. One week after seeding, electrical conditioning was resumed. Two weeks after seeding, the animals were killed and the SMVs excised. Histologic examination confirmed the presence of a confluent monolayer of cells covering 80-100% of the luminal surface in each seeded SMV. The endothelial nature of the cells lining the SMV lumen was established by fluorescent microscopy. Endothelial cells were pre labeled with the cellular marker PKH before seeding; the SMVs were also incubated with the endothelial marker dil-acetylated LDL. Endothelial cells also were identified by staining with fluorescently labeled antibodies to von Willebrand factor. Based upon these data, electrically conditioned SMVs can be seeded successfully with a near-complete, autologous endothelial monolayer. Additionally, this endothelial monolayer can be maintained on the luminal surface of a contracting SMV. In-circulation studies will determine whether endothelial cell seeding of SMVs can decrease or eliminate the incidence of thromboembolism.

Anomalous origin of the right coronary artery from the pulmonary artery and mitral regurgitation in a newborn.

Anomalous origin of the right coronary artery (RCA) from the pulmonary artery (PA) is a rare congenital anomaly, and only 28 cases have been reported in the pediatric age group. We describe the case of an infant who had progressive mitral regurgitation and papillary muscle dysfunction in association with anomalous origin of the RCA from the PA. The diagnosis was made by color flow Doppler, confirmed by angiography, and the case was successfully corrected by reimplantation of the anomalous RCA to the aorta. This is only the second case of anomalous origin of the RCA from the PA diagnosed in infancy without an associated congenital anomaly of the heart and great vessels.