Surgical treatment of adult atrial septal defect: early and long-term results.

OBJECTIVES: The purpose of this study is to determine the early and late results of the surgical repair of atrial septal defect in adults. BACKGROUND: Progressively limiting, untreated atrial septal defect can lead to the early death of middle-aged adults. Recently it has been suggested that the closure of atrial septal defects might be accomplished with interventional cardiac techniques. Although the long-term results of the transcatheter closure are as yet unknown, the outcome of surgical therapy has been shown to be beneficial for almost 40 years. METHODS: Between 1971 and 1991, 166 consecutive patients underwent surgical repair of a secundum or sinus venosus atrial septal defect, or both, at the Brigham and Women's Hospital, Boston. There were 120 women and 46 men in this group; the mean age was 44 years and 58 (35%) of the patients were > or = 50 years old. The average pulmonary to systemic flow ratio was 3.0, and 57 patients had a peak systolic pulmonary artery pressure > 30 mm Hg. RESULTS: There were two operative deaths (early mortality rate 1.2%), and 13% of the patients had a perioperative complication. One hundred fifty-three of the 164 survivors were followed up for a mean of 90 months (range 2 to 247). There were eight late deaths (late mortality rate 4.9%) and a late morbidity rate of 12.4% (in most cases due to arrhythmias). The 5- and 10-year survival rates are 98% and 94%, respectively, and the probability of event-free survival (with no morbidity or mortality) at 5 years is 97% and at 10 years is 92%. CONCLUSIONS: The results indicate that the surgical correction of atrial septal defect in adults is safe and efficacious as confirmed by 20 years of follow-up.

Video-assisted thoracoscopic vascular ring division in infants and children.

OBJECTIVES: This study evaluated our early experience with video-assisted thoracoscopic vascular ring division and compared this approach with division by means of a conventional open thoracotomy. BACKGROUND: Video-assisted thoracoscopic techniques reduce surgical trauma and have been applied to several adult thoracic procedures; however, pediatric applications have been limited. We developed instruments and techniques for video-assisted thoracoscopic vascular ring division in the pediatric population. METHODS: We compared patient characteristics, operative results and postoperative hospital courses of all patients undergoing vascular ring division by a video-assisted approach with a historical control group of all patients undergoing division by an open thoracotomy between January 1991 and December 1992. RESULTS: Eight patients (median age 5 months, range 40 days to 5.5 years; median weight 6.2 kg, range 1.8 to 17.1) underwent video-assisted thoracoscopic vascular ring division. Four had a double aortic arch with an atretic left arch and a left ligamentum, and four had a right aortic arch with aberrant left subclavian artery and a left ligamentum. All eight had successful ring division with symptomatic relief and no mortality. A limited thoracotomy was performed in three patients to divide patent vascular structures, and the hospital period was prolonged in one because of chylothorax. These eight patients were compared with a historical cohort of eight pediatric patients having vascular ring division performed by a conventional thoracotomy. The two groups did not differ in age, weight, intensive care unit or postoperative hospital stay, duration of intubation or thoracostomy tube or hospital charges. Total operating room time was longer for the group undergoing video-assisted operation. CONCLUSIONS: Early results for video-assisted thoracoscopic vascular ring division are comparable to those of the conventional surgical approach. With further refinement in technique and instrumentation, video-assisted surgical intervention may become a viable alternative to open thoracotomy for management of the symptomatic vascular ring.

An institutional experience with second- and third-stage palliative procedures for hypoplastic left heart syndrome: the impact of the bidirectional cavopulmonary shunt.

OBJECTIVES: The aim of this study was to perform a retrospective analysis of an institutional experience with a consecutive series of patients with post-stage I palliation for hypoplastic left heart syndrome (HLHS). BACKGROUND: In a recent review of 212 consecutive patients who underwent stage I operations for HLHS at our institution between 1983 and 1993, we identified risk factors related to stage I mortality. We sought to examine the outcome for these patients at subsequent palliative procedures. METHODS: All patients who underwent stage I reconstruction between January 1983 and June 1993 and also underwent subsequent palliation at our institution were included. Seventy patients underwent palliative procedures and two underwent heart transplantation. Patient-specific factors and features of the stage II operation were analyzed for impact on stage II mortality and actuarial survival. RESULTS: The only independent risk factor for stage II mortality was the performance of a nonfenestrated Fontan operation (p < 0.001). There were nine in-hospital deaths (69%) in the 13 patients undergoing the nonfenestrated Fontan procedure at stage II. Fifty patients underwent intermediate superior vena cava to pulmonary artery anastomosis at stage II, with 4 (8%) early deaths. Pulmonary artery augmentation was performed in 19 patients (38%) at stage II, without increased operative risk. Hypoplastic left heart syndrome anatomic subtype did not influence stage II mortality. The modified fenestrated Fontan procedure has been performed as a third stage in 32 patients whose median age was 28.7 months, with one early death at a median follow-up of 24.5 months. CONCLUSIONS: A second-stage bidirectional cavopulmonary anastomosis for HLHS reduces second-stage mortality and improves intermediate survival. The modified fenestrated Fontan operation may then be performed as a final palliative stage with low operative risk.

Long-term follow-up after surgical repair of ostium primum atrial septal defects in adults.

OBJECTIVES: We sought to determine the long-term survival rates and defect-related morbidity of adult patients who undergo surgical repair of an ostium primum atrial septal defect. BACKGROUND: The natural history of patients undergoing such surgical repair in adulthood remains unclear. METHODS: We followed up 33 patients who underwent surgical correction of an ostium primum atrial septal defect at our institution at a mean age of 42 years (range 20 to 73); 12 of these patients were > 50 years old at the time of operation. Four patients had moderate preoperative exercise incapacity (New York Heart Association functional class > II) and six had atrial fibrillation. Nine and four patients, respectively, had a preoperative mean pulmonary artery pressure > 25 mm Hg or pulmonary vascular resistance > 4 Wood U. Autologous pericardium was used to patch the defect in 30 patients (91%). Mitral valvuloplasty, consisting of cleft repair (n = 10), and mitral valve replacement (n = 2) were performed selectively. RESULTS: There were no operative deaths. At a mean follow-up interval of 5.3 years (range 1 to 18.2), all 28 surviving patients are free of exercise limitation (functional class 1). Late postoperative deaths occurred in five patients (15%) and were related to myocardial infarction, stroke, hepatic failure, renal failure or sepsis. Reoperation within the 1st postoperative year was required in two patients (6%) because of a residual ostium primum defect in one and severe mitral regurgitation in the other. The presence of advanced age at operation, symptoms, atrial arrhythmias, mitral regurgitation or moderately increased pulmonary vascular resistance did not predict late postoperative mortality, complications or functional capacity. CONCLUSIONS: An ostium primum defect can be repaired in adult patients with the expectation of excellent long-term results, independent of age at operation and preoperative mitral valve function and despite the presence of atrial fibrillation or moderately elevated pulmonary vascular resistance.

Characterisation of transient outward current in young human atrial myocytes.

OBJECTIVE: The aim was to characterise the development of the transient outward current (Ito) in atrial myocytes of infants and children. METHODS: Whole cell voltage clamp was used to study outward currents in enzymatically isolated atrial myocytes from infants and children ranging in age from 3 days to 13.2 years. RESULTS: A transient inactivating current characteristic of Ito was observed in 71 myocytes from 22 patients aged 3 days to 13.2 years, including a 10 day old infant born prematurely at 33 weeks gestation. There was no discernible developmental trend in Ito current density [10.74(SEM 0.65) pA.pF-1 at +40 mV, n = 71 cells from 22 patients] or voltage dependence of inactivation, newborn values being similar to those in older children, and in adults reported elsewhere. A developmental reduction in total outward current density was attributable entirely to diminution of the non-inactivating steady state current component. The Ito time course of inactivation showed an apparent maturational evolution, with the youngest infants having slightly but significantly slower inactivation kinetics. The kinetics of Ito recovery from inactivation were well described by a single exponential model with no appreciable developmental trend in time course. CONCLUSIONS: Ito is expressed in human atrial myocytes from early infancy and does not show significant developmental changes in current density. The relative contribution of Ito to myocyte repolarisation might increase with age as a result of diminution in the non-inactivating current component. There is an apparent slight maturational acceleration in the time course of Ito inactivation but not in recovery from inactivation, perhaps excluding the latter as a mechanism for the previously reported functional unavailability of Ito in young human atrial muscle.

Video-assisted thoracoscopic surgery for patent ductus arteriosus in low birth weight neonates and infants.

BACKGROUND: Video-assisted thoracoscopic surgery (VATS) has been assuming an expanded role in the management of cardiothoracic disease. As instrumentation and experience increase, VATS is being applied to treat smaller patients. We report our experience with 34 low birth weight infants undergoing VATS interruption of patent ductus arteriosus (PDA). METHODS: VATS allows PDA interruption without the muscle cutting or rib spreading of a standard thoracotomy. Four small, 3-mm incisions are made along the line of a potential thoracotomy incision. Ports placed through these incisions admit endoscopic instruments, a camera, and a vascular clip applier. RESULTS: Median age at surgery was 15.5 days (range: 1-44 days). Median weight at surgery was 930 g (range: 575-2500 g). Twenty patients weighed <1 kg, and 13 weighed <750 g. All patients had congestive heart failure and had either failed indomethacin therapy or had contraindications to indomethacin. Median surgical time was 60 minutes (range: 31-171 minutes). Echocardiography documented elimination of ductal flow in all patients. Operative mortality was zero. Four patients (4/34 = 12%) required conversion to open thoracotomy: 1 because of difficult exposure, 1 because of pulmonary dysfunction and anasarca, 1 because of a large 1-cm duct, and 1 because of coagulopathy and poor pulmonary compliance. Two patients died before discharge: 1 patient (surgical weight: 605 g) died on postoperative day 2 because of intracranial hemorrhage, and 1 patient (surgical weight: 1725 g) died on postoperative day 88 because of multiple system organ failure. Follow-up has demonstrated no PDA murmur in any patient, but echocardiography revealed trace ductal flow in 2 patients. CONCLUSIONS: VATS offers a minimally traumatic, safe, and effective technique for PDA interruption in low birth weight neonates and infants.

Coronary artery abnormalities detected at cardiac catheterization following the arterial switch operation for transposition of the great arteries.

Because the arterial switch operation has become the routine surgical approach for transposition of the great arteries, there is increasing awareness of adverse sequelae in some survivors. For the arterial switch to be considered the procedure of choice for transposition of the great arteries, long-term patency and normal function of the translocated coronary arteries must be achieved. We reviewed the cineangiograms and hemodynamic data in 366 patients who underwent postoperative catheterization after arterial switch operation. Of these, 13 patients (3%) had previously unsuspected coronary abnormalities diagnosed angiographically. No patient had noninvasive evidence of resting systolic dysfunction. Findings included left main coronary artery stenosis (n = 3) or occlusion (n = 2), anterior descending coronary artery stenosis (n = 1) or occlusion (n = 2), right coronary artery stenosis (n = 1) or occlusion (n = 1), and small coronary artery fistulas (n = 3). One patient died suddenly 3.3 years after surgery, 1 patient is lost to follow-up, and the remaining 10 patients are alive and asymptomatic up to 11 years after surgery.

Video-assisted thoracoscopic surgery for congenital heart disease.

Video-assisted endoscopic techniques have reduced operative trauma in adult thoracic and general surgery, but applications in children with congenital heart disease have been limited. We report the development of video-assisted thoracic surgery procedures for neonates and infants with cardiovascular disease. Endoscopic instruments and techniques for pediatric cardiovascular procedures were designed and tested in the animal laboratory. Forty-eight operations were subsequently performed in 46 pediatric patients ranging in age from 2 hours to 14 years (median 9 months), weighing from 575 grams to 54 kg (median 8.5 kg). Clinical applications included seven different surgical procedures: patent ductus arteriosus interruption in infants (n = 26) and premature neonates (n = 5), vascular ring division (n = 8), pericardial drainage and resection (n = 3), arterial and venous collateral interruption (n = 2), thoracic duct ligation (n = 2), epicardial pacemaker lead insertion (n = 1), and diagnostic thoracoscopy (n = 1). There was no operative mortality. Technical success, defined as a video-assisted procedure completed without incising chest wall muscle or spreading the ribs, was achieved in 39 of 48 procedures (82%), with thoracotomy required to complete nine procedures. Most patients (22/25, 88%) undergoing elective ductus ligation were extubated in the operating room and discharged from the hospital within 48 hours of the operation. Eight of the last 10 patients having ductus ligation were discharged on the first postoperative day. Residual ductal flow was assessed by (1) transesophageal echocardiography in the operating room (incidence: 0/25, 0%, 70% CL 0% to 7.3%); (2) discharge auscultation (incidence: 1/30, 3%, 70% CL 0.5% to 10.8%); and (3) follow-up Doppler echocardiography (incidence: 3/25, 12%, 70% CL 5.4% to 22.6%). Video-assisted thoracoscopic techniques can be safely applied to pediatric patients with patent ductus arteriosus and vascular rings and may become an effective addition to the staged management of more complex forms of congenital heart disease.

Perioperative effects of alpha-stat versus pH-stat strategies for deep hypothermic cardiopulmonary bypass in infants.

OBJECTIVES: In a randomized, single-center trial, we compared perioperative outcomes in infants undergoing cardiac operations after use of the alpha-stat versus pH-stat strategy during deep hypothermic cardiopulmonary bypass. METHODS: Admission criteria included reparative cardiac surgery, age less than 9 months, birth weight 2.25 kg or more, and absence of associated congenital or acquired extracardiac disorders. RESULTS: Among the 182 infants in the study, diagnoses included D-transposition of the great arteries (n = 92), tetralogy of Fallot (n = 50), tetralogy of Fallot with pulmonary atresia (n = 6), ventricular septal defect (n = 20), truncus arteriosus (n = 8), complete atrioventricular canal (n = 4), and total anomalous pulmonary venous return (n = 2). Ninety patients were assigned to alpha-stat and 92 to pH-stat strategy. Early death occurred in four infants (2%), all in the alpha-stat group (p = 0.058). Postoperative electroencephalographic seizures occurred in five of 57 patients (9%) assigned to alpha-stat and one of 59 patients (2%) assigned to pH-stat strategy (p = 0.11). Clinical seizures occurred in four infants in the alpha-stat group (4%) and two infants in the pH-stat group (2%) (p = 0.44). First electroencephalographic activity returned sooner among infants randomized to pH-stat strategy (p = 0.03). Within the homogeneous D-transposition subgroup, those assigned to pH-stat tended to have a higher cardiac index despite a lower requirement for inotropic agents; less frequent postoperative acidosis (p = 0.02) and hypotension (p = 0.05); and shorter duration of mechanical ventilation (p = 0.01) and intensive care unit stay (p = 0.01). CONCLUSIONS: Use of the pH-stat strategy in infants undergoing deep hypothermic cardiopulmonary bypass was associated with lower postoperative morbidity, shorter recovery time to first electroencephalographic activity, and, in patients with D-transposition, shorter duration of intubation and intensive care unit stay. These data challenge the notion that alpha-stat management is a superior strategy for organ protection during reparative operations in infants using deep hypothermic cardiopulmonary bypass.

Primary repair of aortopulmonary septal defect, interrupted aortic arch, and anomalous origin of the right pulmonary artery.

Aortopulmonary septal defect, interruption of the aortic arch, and anomalous origin of the right pulmonary artery from the aorta comprised a challenging constellation of defects in a 13-day-old neonate, who underwent complete repair without homograft tissue or synthetic graft. After the aorta was separated from the pulmonary artery, the descending aorta was anastomosed to the septal defect in the ascending aorta. The right pulmonary artery was anastomosed to the septal defect in the main pulmonary artery, anterior to the aorta. The postoperative course was uncomplicated, and the potential for growth of both great vessels was optimized.

Video-assisted cardioscopy in congenital heart operations.

Recent advances in fiberoptics and endoscopic imaging technology have extended the applications of video assistance in surgical procedures. Video-assisted thoracoscopic surgical techniques have been employed to improve anatomic visualization within the pleural space and to reduce chest wall trauma. Open heart operations for congenital heart disease in neonates and infants also require clear visualization of small structures within confined spaces. By adapting pediatric thoracoscopic instrumentation, we have developed a technique for video-assisted cardioscopy. This technique was used in 4 patients with complex congenital heart disease to expose remote intracardiac structures and facilitate surgical repair. The patients ranged in weight from 4.6 to 17 kilograms, and visualization of the intended structures was achieved in each case within 12 minutes. There were no complications associated with the videoscope. Further experience with video-assisted cardioscopy might broaden its role as an adjunct to the surgical repair of complex congenital heart defects.

Anomalous origin of innominate artery from right pulmonary artery in DiGeorge Syndrome.

We experienced a case of anomalous origin of innominate artery from right pulmonary artery (isolated innominate artery). This patient was a 2-month-old baby girl weighing 3.2 kg with DiGeorge syndrome, who was diagnosed with perimembranous ventricular septal defect, atrial septal defect, and patent ductus arteriosus. This type of anomaly is exceedingly rare.

Minimally invasive diaphragm plication in an infant.

We report the use of video-assisted thoracic surgery to plicate the diaphragm after phrenic nerve injury associated with an operation for congenital heart disease. Right diaphragm paresis developed in a cyanotic newborn girl with pulmonary atresia and intact ventricular septum after a right modified Blalock-Taussig shunt. Diaphragm plication was performed endoscopically and the patient recovered. Refinement of technique and instrumentation may allow wider application of video-assisted thoracoscopic plication of the diaphragm in neonatal and pediatric patients.

Congenital Heart Surgery Nomenclature and Database Project: atrial septal defect.

The extant nomenclature for atrial septal defect (ASD) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect.

The extant nomenclature for ventricular septal defect (VSD) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Four basic VSD types are described: Subarterial, Perimembranous, Inlet, and Muscular. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analysis. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Congenital Heart Surgery Nomenclature and Database Project: atrioventricular canal defect.

The extant nomenclature for atrioventricular (AV) canal/atrioventricular septal defect is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The three general categories are: partial AV canal (ostium primum defect), transitional (intermediate) AV canal, and complete AV canal. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Endovascular stent graft treatment of a pulmonary artery pseudoaneurysm.

A 5-year-old girl with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and multiple pulmonary artery reconstructive procedures presented with an enlarging pulmonary artery pseudoaneurysm. A previous attempt to occlude the aneurysm was unsuccessful and the aneurysm continued to enlarge. We describe the percutaneous placement of an endovascular stent graft to occlude the aneurysm. This novel use of a covered graft effectively treated a potentially lethal problem without reoperative thoracotomy or sternotomy.

Extracardiac Fontan operation without cardiopulmonary bypass.

Choreoathetosis developed after bilateral bidirectional cavopulmonary anastomosis in a 17-month-old boy with univentricular heart. To avoid exacerbating this neurologic problem, the Fontan operation was later completed without cardiopulmonary bypass. The left cavopulmonary anastomosis maintained pulmonary blood flow. A tube graft was anastomosed to the junction of the right cavopulmonary anastomosis. A femoral vein-to-right atrial shunt was established, the inferior vena cava divided, the cardiac end oversewn, and the noncardiac end anastomosed to the tube graft.

Congenital Heart Surgery Nomenclature and Database Project: aortopulmonary window.

The extant nomenclature for aortopulmonary window (AP window) and pulmonary artery origin from ascending aorta (hemitruncus) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Minimally invasive resection of congenital subaortic stenosis.

Fifteen consecutive patients with membranous subaortic stenosis underwent resection by a minimal-access approach through a partial upper sternotomy using a cardioscope. There were no operative deaths and no postoperative complications. Twelve patients (80%) were extubated in the operating room. The mean hospital stay was 3.1 days, and 3 patients (20%) needed blood products. Our experience demonstrates that this modified approach is a safe and effective surgical option for resection of subaortic stenosis.