Clinically silent preoperative brain injuries do not worsen with surgery in neonates with congenital heart disease.

OBJECTIVE: Preoperative brain injury, particularly stroke and white matter injury, is common in neonates with congenital heart disease. The objective of this study was to determine the risk of hemorrhage or extension of preoperative brain injury with cardiac surgery. METHODS: This dual-center prospective cohort study recruited 92 term neonates, 62 with transposition of the great arteries and 30 with single ventricle physiology, from 2 tertiary referral centers. Neonates underwent brain magnetic resonance imaging scans before and after cardiac surgery. RESULTS: Brain injury was identified in 40 (43%) neonates on the preoperative magnetic resonance imaging scan (median 5 days after birth): stroke in 23, white matter injury in 21, and intraventricular hemorrhage in 7. None of the brain lesions presented clinically with overt signs or seizures. Preoperative brain injury was associated with balloon atrial septostomy (P = .003) and lowest arterial oxygen saturation (P = .007); in a multivariable model, only the effect of balloon atrial septostomy remained significant when adjusting for lowest arterial oxygen saturation. On postoperative magnetic resonance imaging in 78 neonates (median 21 days after birth), none of the preoperative lesions showed evidence of extension or hemorrhagic transformation (0/40 [95% confidence interval: 0%-7%]). The presence of preoperative brain injury was not a significant risk factor for acquiring new injury on postoperative magnetic resonance imaging (P = .8). CONCLUSIONS: Clinically silent brain injuries identified preoperatively in neonates with congenital heart disease, including stroke, have a low risk of progression with surgery and cardiopulmonary bypass and should therefore not delay clinically indicated cardiac surgery. In this multicenter cohort, balloon atrial septostomy remains an important risk factor for preoperative brain injury, particularly stroke.

Extracardiac conduit versus lateral tunnel cavopulmonary connections at a single institution: impact on outcomes.

OBJECTIVE: To compare outcomes of extracardiac conduit and lateral tunnel Fontan connections in a single institution over a concurrent time period. METHODS: Between January 1994 and September 1998, 60 extracardiac conduit and 47 lateral tunnel total cavopulmonary connections were performed. Age, sex, and weight did not differ between the 2 groups. Compared with the lateral tunnel group (LT group), patients undergoing the extracardiac conduit procedure (EC group) had a trend to a higher incidence of morphologically right ventricle (EC group 48% vs LT group 32%; P <.09), a higher incidence of isomerism/heterotaxy syndrome (EC 22% vs LT 0%; P <.001), worse atrioventricular valve regurgitation (EC 11% moderate-plus vs LT 0%; P <.06), and lower McGoon indices (EC 1.8 +/- 0.5 vs LT 2.1 +/- 0.5; P <.03). Preoperative arrhythmias, transpulmonary gradients, room air oxygen saturations, ejection fractions, ventricular end-diastolic pressure, and pulmonary artery distortion did not differ between groups. Cardiopulmonary bypass times and fenestration usage were similar in both groups. RESULTS: Overall operative mortality was 5.6% and did not differ between groups. The LT group had a significantly higher incidence of postoperative sinoatrial node dysfunction (45% vs EC group 15%; P <.007), supraventricular tachycardia (33% vs EC group 8%; P <.0009), and need for temporary postoperative pacing (32% vs 12%; P <.01). Median duration of intensive care unit stay (EC 2 days, range 1-10 days, vs LT 2.8 days, range 1-103 days; P <.07) and ventilatory support (EC 1 day, range 0.25-10 days, vs LT 1 day, range 0.25-99 days; P <.03) were all longer in the LT group. Median chest tube drainage (EC 8 days, LT 9 days) was similar in both groups. Follow-up averaged 2.5 +/- 1.4 years in the EC group and 2.8 +/- 1.9 years in the LT group. There were 2 late deaths. Overall survival is 94% at 1 month, 92% at 1 year, and 92% at 5 years. Late ejection fraction or atrioventricular valve function did not differ between groups. Intermediate follow-up Holter analysis showed a higher incidence of atrial arrhythmias in the LT group (23% vs 7%; P <.02). Multivariable analysis showed that (1) prolonged cardiopulmonary bypass time was the only independent predictor for perioperative mortality, prolonged ventilation and intensive care unit length of stay, and increased time to final removal of chest tube drains and (2) lateral tunnel Fontan connection is an independent predictor of early postoperative and intermediate atrial arrhythmias. CONCLUSIONS: Although patients in the EC group were at higher preoperative risk, their outcomes were comparable with those of the LT group. Use of the extracardiac conduit technique for the modified Fontan operation reduces the risk of early and midterm atrial arrhythmia.

Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes.

BACKGROUND: The historic outcome of the Fontan procedure in children with single ventricle and heterotaxy syndrome has been poor, and in the current era it has been incompletely described. METHODS: From January 1993 through April 2000, 30 patients (age range, 5.3 +/- 3.6 years) had total cavopulmonary connection for single ventricle and isomerism and heterotaxy syndrome. Right atrial isomerism and heterotaxy (n = 19) or left atrial isomerism and heterotaxy (n = 11) were associated with a morphologic right ventricle (n = 26), a common atrioventricular valve (n = 25), pulmonary atresia (n = 9) or stenosis (n = 17), anomalous pulmonary venous drainage (partial, n = 7; total, n = 11; obstructed, n = 4), and interrupted inferior vena cava (n = 13). A previous systemic to pulmonary artery shunt had been performed in 21 patients, and 3 patients had previous pulmonary artery banding. The Fontan procedure was staged in all but 3 patients at a median age of 12 months (range, 3 to 114 months). Before the Fontan procedure, atrial arrhythmia was present in 7 patients (23%). RESULTS: An extracardiac conduit was constructed in 22 patients (median diameter, 20 mm; range, 16 to 27 mm), and a lateral tunnel was constructed in 4 patients. Associated procedures included pulmonary artery augmentation (n = 17), repair of anomalous pulmonary venous drainage (n = 7), and repair of a regurgitant atrioventricular valve (n = 2). The mean cardiopulmonary bypass time was 134 +/- 57 minutes. Cardioplegic cardiac arrest was used in 12 patients for a mean duration of 62 +/- 28 minutes. There were four hospital deaths (13%), three that occurred in children having concomitant repair of anomalous pulmonary venous drainage (previously undetected in one). Thirteen children (41%) developed early postoperative atrial arrhythmias, 11 of the 13 children required temporary pacing. There was one late death. Follow-up (3.4 +/- 2.7 years) was available on 24 of 25 survivors. All but 2 patients were in sinus rhythm. CONCLUSIONS: Early and midterm outcomes after the Fontan procedure in this patient group improved and may be further ameliorated by detecting and repairing associated obstruction to pulmonary venous flow and performing atrioventricular valvuloplasty before the Fontan procedure.

Are outcomes of surgical versus transcatheter balloon valvotomy equivalent in neonatal critical aortic stenosis?

BACKGROUND: For neonates with critical aortic valve stenosis who are selected for biventricular repair, valvotomy can be achieved surgically (SAV) or by transcatheter balloon dilation (BAV). METHODS AND RESULTS: Data regarding 110 neonates with critical aortic valve stenosis were evaluated in a study by the Congenital Heart Surgeons Society from 1994 to 1999. Reduced left ventricular function was present in 46% of neonates. The initial procedure was SAV in 28 patients and BAV in 82 patients. Mean percent reduction in systolic gradient was significantly greater with BAV (65+/-17%) than SAV (41+/-32%; P<0.001). Higher residual median gradients were present in the SAV versus BAV group (36 mm Hg [range, 10 to 85 mm Hg] versus 20 mm Hg [0 to 85 mm Hg], P<0.001). Important aortic regurgitation was more often present after BAV (18%) than SAV (3%; P=0.07). Time-related survival after valvotomy was 82% at 1 month and 72% at 5 years, with no significant difference for SAV versus BAV, even after adjustment for differences in patient and disease characteristics. Independent risk factors for mortality were mechanical ventilation before valvotomy, smaller aortic valve annulus (z score), smaller aortic diameter at the sinotubular junction (z score), and a smaller subaortic region. A second procedure was performed in 46 survivors. Estimates for freedom from reintervention were 91% at 1 month and 48% at 5 years after the initial valvotomy and did not differ significantly between groups. CONCLUSIONS: SAV and BAV for neonatal critical aortic stenosis have similar outcomes. There is a greater likelihood of important aortic regurgitation with BAV and of residual stenosis with SAV.

Outcomes of intraoperative device closure of muscular ventricular septal defects.

BACKGROUND: The surgical management of muscular ventricular septal defects (mVSD) in the small infant is a challenge particularly when multiple and associated with complex cardiac lesions. Devices for percutaneous implantation have the advantage of ease of placement and for the double umbrella designs a wide area of coverage. We reviewed our experience and clinical outcomes of intraoperative mVSD device closure for such defects in small infants. METHODS: Since October 1989, intraoperative VSD device closure was a component of the surgical strategy in 14 consecutive patient implants (median age, 5.5 months; range, 3 to 11 kg), whose defects were thought difficult to approach using conventional techniques. Nine patients had associated complex cardiac lesions, 10 multiple mVSDs, and 4 patients had a previous pulmonary artery banding. RESULTS: There were 2 early deaths, 1 in a severely ill child who preoperatively had pulmonary hypertension and left ventricular failure and another in a patient with a hypoplastic left heart. Mean pulmonary to systemic flow ratio before device insertion was 3.5:1. Complete closure was achieved in 5 patients and clinically insignificant residual shunts persisted in 7. In 2 infants with significant residual lesions concomitant pulmonary artery banding was required. Postoperative mean pulmonary to systemic flow ratio was 1.7:1. In follow-up of the 12 surviving infants (mean, 41 months), 8 had complete closure and 3 persistent residual shunts. One patient with no residual shunting required heart transplantation for progressive ventricular failure 9 years after operation. All devices were well positioned on postoperative echocardiograms. There was 1 late death due to aspiration in a patient with a tiny residual shunt. CONCLUSIONS: Infants requiring operative intervention with mVSDs are difficult to manage and have an increased mortality and morbidity. Intraoperative VSD device placement for closure of mVSDs is feasible, can avoid ventriculotomy, division of intracardiac muscle bands, and is ideally suited for the neonate or infant.

Collaborative interventional and surgical strategies in the management of congenital heart lesions.

In the last 15 years the development of catheter-directed percutaneous therapies have improved the delivery of care to children with congenital heart lesions. Paralleling these advances, enhanced surgical techniques are now applied to complex cardiac lesions previously thought to be inoperable. This chapter outlines several treatment strategies which utilize surgical and catheter-based algorithms to address congenital heart disorders.

Total cavopulmonary connections in children with a previous Norwood procedure.

BACKGROUND: Outcomes of the Fontan operation in children initially palliated with the modified Norwood procedure are incompletely defined. METHODS: From August 1993 to January 2000, 45 patients (mean age 2.6 +/- 1.1 years, weight 12.7 +/- 2.8 kg) who were palliated with staged Norwood procedures (hypoplastic left heart syndrome, n = 32; nonhypoplastic left heart syndrome, n = 13) underwent a modified Fontan operation. Preoperative features included moderate/severe atrioventricular valve regurgitation (n = 5, 11%), reduced ventricular function on echocardiography in 11 patients, McGoon index 1.56 +/- 0.38, and pulmonary artery distortion in 18 patients (40%). RESULTS: A lateral tunnel (n = 16) or an extracardiac conduit (n = 29) connection with fenestration in 38 patients (84%) was used. Concomitant procedures included pulmonary artery reconstruction (n = 24, 53%), atrioventricular valve repair (n = 4, 9%) or replacement (n = 1). Before Fontan, 12 patients (27%) had an intervention to address neoaortic obstruction, and 7 patients required balloon dilation/stenting of the left (n = 5) or right pulmonary artery (n = 5). Intraoperatively, left (n = 5) or right pulmonary artery (n = 1) stenting was performed in 5 patients (11%). On follow-up, 8 patients required additional interventional procedures to address left pulmonary artery narrowing (n = 5), or venous (n = 5) or arteriopulmonary collaterals (n = 1). Perioperative mortality was 4.4% (n = 2). There were 2 late deaths at a mean follow-up of 39 +/- 20 months. CONCLUSIONS: In relatively high-risk patients, midterm results of the Fontan operation for children initially palliated with the Norwood procedure were good. Combined interventional-surgical treatment algorithms can lead to improved outcomes.

Ruptured sinus of valsalva aneurysm: early recurrence and fate of the aortic valve.

BACKGROUND: We reviewed our experience with congenital ruptured sinus of Valsalva aneurysms (RSVA) to determine patterns of early recurrence and the fate of the aortic valve (AV). METHODS: Over a 28-year period, RSVA was identified in 34 patients, (mean age 31.6 years). Primary closure of the RSVA was performed in 10 patients, and a patch employed in 24. Aortic insufficiency was present in 24 patients. AV replacement (AVR) was performed in 5 patients; AV repair in 6. RESULTS: Follow-up of 9.2 +/- 8.3 years (6 months to 24 years) was complete in all but 2 patients. Five early fistula recurrences (in 4 patients) correlated with primary rather than patch closure (p < 0.03). Kaplan-Meier survival at 10 years is 90 +/- 7%. Freedom from reoperative AVR at 10 years is 83 +/- 9%. Late AVR was performed in 6 patients for progressive aortic insufficiency due to bicuspid valve (n = 3), cusp disease of affected sinus (n = 2), or aortic root dilatation (n = 2). CONCLUSIONS: Patch closure of the RSVA should be routinely employed. A bicuspid valve may be associated with the late need for AVR.

Aortic valve repair for adult congenital heart disease: A 22-year experience.

BACKGROUND: Aortic valve-preserving procedures have resulted in excellent outcomes in selected patients, particularly those with normal aortic valve leaflets and dilated aortic roots. However, several congenital heart lesions are associated with abnormal aortic valve leaflets. The long-term results of aortic valve repair for these lesions are not well defined. METHODS AND RESULTS: We reviewed the clinical records of 54 adult (age >18 years) patients who underwent repair of congenital abnormalities of the aortic valve between 1976 and September 1999. Follow-up data were available on 52 (96%) patients (mean 50+/-67 months, range 1 to 266). Patients underwent repair at a mean age of 34+/-14 years with associated diagnoses of subaortic stenosis (n=10), ventricular septal defect with prolapsing aortic valve (n=17), bicuspid aortic valve (n=23), sinus of Valsalva aneurysm (n=10), and bacterial endocarditis (n=2). There was 1 operative death (1.9%) and 3 late deaths. Survival at 5 and 10 years was 98+/-2% and 74+/-12%, respectively. Freedom from reoperation was 74+/-9% and 51+/-15% at 5 and 10 years, respectively. The presence of a ventricular septal defect predicted failure of valve repair (59% versus 22%, P:=0.01). A bicuspid aortic valve, subaortic stenosis, or the requirement for mitral valve surgery did not affect outcomes. CONCLUSIONS: Aortic valve repair in adult patients with congenital heart disease can be performed with minimal morbidity and mortality rates. The medium-term results of repair are acceptable, regardless of valvular or associated pathology. However, only 31 patients (57%) demonstrated long-term competence of the aortic valve, suggesting that most adult patients with congenital aortic valve disease will eventually require aortic valve replacement.

Common brachiocephalic trunk: strategies for revascularization.

BACKGROUND: A common brachiocephalic trunk, in which both common carotid arteries and the right subclavian artery arise from a single trunk off the arch, is a normal variant of aortic arch branching that occurs in approximately 10% of the population. Because three of the four primary sources of cerebral blood flow arise from a single aortic branch, stenosis or occlusion of a common trunk can cause severe ischemic consequences. Common trunk revascularization has been described, but there have been no reports focusing on the management options for occlusive disease of this vascular anatomy. METHODS: A retrospective review of our experience with innominate artery revascularization identified 6 patients who underwent revascularization of a common brachiocephalic trunk between 1977 and 1997. All patients were symptomatic, with either total occlusion (n = 3) or critical stenosis (n = 3) caused by atherosclerosis (n = 5) or Takayasu's arteritis (n = 1). Revascularization was achieved by a prosthetic bypass graft from the ascending aorta to the innominate or left common carotid arteries or both (n = 5); or transarterial endarterectomy (n = 1). Concomitant endarterectomy of branch vessels was performed in 3 patients. RESULTS: There was one perioperative death from myocardial infarction, and one perioperative stroke, with death occurring 1 month after hospital discharge. One patient developed cerebral hyperperfusion syndrome 1 week after endarterectomy that resolved without sequelae with antihypertensive medications. During a follow-up period ranging from 1 to 20 years, there was one late death from congestive heart failure 5 years after operation. All surviving patients are alive and free from symptomatic recurrence. CONCLUSIONS: Revascularization for occlusive disease of a common brachiocephalic trunk can be achieved with effective and durable relief of symptoms using either a prosthetic bypass graft or endarterectomy. However, neurologic complications in 2 patients, which were fatal in 1, attest to the potential cerebral ischemic threat posed by occlusive disease of a common brachiocephalic trunk.

Surgical management of subclavian-vein effort thrombosis as a result of thoracic outlet compression.

BACKGROUND: There is considerable variability and controversy in the current management of subclavian-vein effort thrombosis. The purpose of this study was to determine the long-term effectiveness and the functional outcome of our preferred treatment strategy of early thrombolysis/recanalization and prompt extensive supraclavicular decompression. PATIENTS AND METHODS: Thirty-three patients who ranged in age from 15 to 60 years underwent operative decompression of 34 primary subclavian-vein thromboses, one of which was bilateral. There were 21 patients with acute thrombosis 7 of whom had had prior unsuccessful balloon venoplasty, 1 with stent placement and 8 patients with chronic/recurrent thrombosis 5 of whom had had 9 unsuccessful prior operations for attempted decompression. Four patients had high-grade symptomatic stenosis and positional occlusion. A supraclavicular approach was used in 32 cases and, in 23 cases, was complemented by an infraclavicular (n = 21) or transaxillary (n = 2) incision. Complete subclavian-vein decompression was achieved by first-rib resection (n = 31), scalenectomy (n = 33), and circumferential venolysis (n = 34). RESULTS: Follow-up was obtained in 30 patients at a mean of 31 months. Twenty of the 21 patients with acute thrombosis had a complete resolution of symptoms with a return to full activity; the other patient was lost to follow-up. Four of the 8 patients with chronic thrombosis reported a mild relief of symptoms but still had limitations of activities of daily living. All of the patients with high-grade symptomatic stenosis with positional occlusion had a complete relief of symptoms and a return to full activity. CONCLUSION: The optimal management of acute effort thrombosis of the subclavian vein includes anticoagulation therapy, thrombolysis/recanalization, confirmatory positional venography, and early supraclavicular decompression of the subclavian vein. In the patients with chronic subclavian-vein thrombosis and positional venographic evidence of compression of first-rib bypass graft collaterals, the initial anticoagulation therapy should be followed by the surgical decompression of the collaterals. The supraclavicular approach alone or with an infraclavicular incision provides optimal exposure for scalenectomy, total first-rib resection, and circumferential venolysis.

Innominate artery reconstruction: over 3 decades of experience.

SUMMARY BACKGROUND DATA: Symptomatic atherosclerotic occlusive disease of the innominate artery is a threatening disease pattern that offers a major challenge in achieving definitive surgical repair. To assess the evolution of treatment strategies and their outcomes for this disease, the authors undertook a review of the cumulative experience for more than 3 decades at one institution. METHODS: Between 1960 and 1997, 94 patients (mean age, 62 years) underwent direct innominate artery revascularization for occlusive atherosclerotic disease to relieve neurologic (n = 85) and/or right upper extremity (n = 26) symptoms or asymptomatic critical stenosis (n = 3). The pattern of atherosclerotic involvement revealed by angiography included critical stenosis (n = 77), complete occlusion (n = 10), and moderate stenosis with plaque ulceration (n = 7). A common brachiocephalic trunk was present in five patients. Transsternal (n = 68) or transcervical (n = 4) innominate endarterectomy was performed in 72 patients and bypass grafting in 22. Forty-one patients underwent concomitant endarterectomy or bypass of innominate branches or adjacent arch vessels, and 3 had coronary bypass grafting. RESULTS: There were three perioperative deaths (3%), all due to cardiac causes. Postoperative morbidity included four strokes (three resolved), two myocardial infarctions, two transient ischemic attacks, and one sternal dehiscence. Follow-up ranged from 8 months to 20 years. Postoperative actuarial survival rate was 96% at 1 year, 85% at 5 years, and 67% at 10 years. Freedom from recurrence requiring reoperation was 100% at 1 year, 99% at 5 years, and 97% at 10 years. CONCLUSIONS: Innominate artery reconstruction is safe and durable when either endarterectomy or prosthetic bypass is used. The anatomic variation and disease distribution permit endarterectomy for most patients. The technique of innominate endarterectomy can be extended safely to outflow and adjacent vessels.

In situ autogenous reconstruction of the thoracoabdominal aorta and branches for treatment of an infected thoracoabdominal aortobifemoral bypass graft.

Graft infection is an uncommon but potentially lethal complication of prosthetic aortic repair. We describe a novel technique for upper abdominal aortic and visceral revascularization after percutaneous drainage and antibiotics failed to cure a thoracofemoral prosthetic graft infection. One week after axillofemoral and femorofemoral bypass grafting, the infected thoracoabdominal graft was removed and a bifurcated iliac artery autograft was used to replace the upper abdominal aorta and revascularize the abdominal viscera. The infected graft was removed from the thorax and retroperitoneum, the infection resolved, and the patient remained well until his death of lung cancer 9 years later.

Percutaneous stenting for symptomatic stenosis of aberrant right subclavian artery.

Aberrant origin of the right subclavian artery is the most common abnormality of the aortic arch vessels and occurs in approximately 0.5% to 1% of the population. Symptoms can result from compression of the esophagus by the aberrant vessel, aneurysm formation, or atherosclerotic occlusion. Occlusive symptoms are typically relieved by surgical revascularization (i.e., transposition or carotid-subclavian bypass) through a cervical approach. An alternative approach to the management of stenosis of normal subclavian arteries is percutaneous angioplasty and stenting, an approach not previously used for occlusive disease of an aberrant right subclavian artery. We describe a case of focal stenosis of an aberrant right subclavian artery causing dizziness and arm claudication in a patient who underwent successful percutaneous angioplasty and stenting.

DTEF-1, a novel member of the transcription enhancer factor-1 (TEF-1) multigene family.

M-CAT motifs mediate muscle-specific transcriptional activity via interaction with binding factors that are antigenically and biochemically related to vertebrate transcription enhancer factor-1 (TEF-1), a member of the TEA/ATTS domain family of transcription factors. M-CAT binding activities present in cardiac and skeletal muscle tissues cannot be fully accounted for by existing cloned isoforms of TEF-1. TEF-1-related cDNAs isolated from heart libraries indicate that at least three classes of TEF-1-related cDNAs are expressed in these and other tissues. One class are homologues of the human TEF-1 originally cloned from HeLa cells (Xiao, J. H., Davidson, I., Matthes, H., Garnier, J. M., and Chambon, P. (1991) Cell 65, 551-568). A second class represents homologues of the avian TEF-1-related gene previously isolated (Stewart, A. F., Larkin, S. B., Farrance, I. K., Mar, J. H., Hall, D. E., and Ordahl, C. P. (1994) J. Biol. Chem. 269, 3147-3150). The third class consists of a novel, divergent TEF-1 cDNA, named DTEF-1, and its preliminary characterization is described here. Two isoforms of DTEF-1 (DTEF-1A and DTEF-1B) were isolated as 1.9-kilobase pair clones with putative open reading frames of 433 and 432 amino acids whose differences are attributable to alternative splicing at the C terminus of the TEA DNA binding domain. Cardiac muscle contains high levels of DTEF-1 transcripts, but unexpectedly low levels are detected in skeletal muscle. DTEF-1 transcripts are present at intermediate levels in gizzard and lung, and at low levels in kidney. DTEF-1A is a sequence-specific M-CAT-binding factor. The distinct spatial pattern of expression, and unusual amino acid sequence in its DNA binding domain, may indicate a particular role for DTEF-1 in cell-specific gene regulation. Recent work also suggests that at least one more TEF-1-related gene exists in vertebrates. We propose a naming system for the four TEF-1 gene family members identified to date that preserves existing nomenclature and provides a means for extending that nomenclature as additional family members may be identified.