YEAR IN CARDIOLOGY SERIES
The Year in Congenital Heart Disease
Thomas P. Graham, Jr, MD, FACC*
Vanderbilt Children’s Hospital, Nashville, Tennessee
Manuscript received March 9, 2006;
revised manuscript received April 6, 2006,
accepted April 11, 2006.
* Reprint requests and correspondence: Dr. Thomas P. Graham, Jr., Division of Pediatric Cardiology, Vanderbilt Children’s Hospital, 2200 Children’s Way, Suite 5230, Nashville, Tennessee 37232-9119. (Email: tom.graham{at}vanderbilt.edu).
It has been another active year in congenital heart disease, with new developments in imaging, surgery, and transcatheter therapy. I have grouped papers by lesions and by management strategies which encourage treatment options from the neonate to older adults.
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Septal defects
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Amin et al. (1) report on 28 cases of erosion of the Amplatzer septal occluder (AGA Medical Corp., Golden Valley, Minnesota) after closure of atrial septal defects (ASDs). Deficient atrial aortic rim was seen in 89%, with the defect described as a high ASD suggesting a deficient superior rim. The incidence of erosion in the U.S. was 0.1%. Once again, this proves that there is no free lunch. This device is safe and effective in most patients, should be deployed by experienced interventionalists, and used sparingly, if at all, when there is a deficient superior rim and/or impingement on the aortic root. Long-term follow-up is needed to determine the true incidence of erosion, which can occur weeks to months after implantation.
Fu et al. (2) report on transcatheter closure of membranous ventricular septal defects (VSDs) using the Amplatzer membranous VSD occluder (Fig. 2 in Fu et al. [2]). The 35 patients had a median age of 7.7 years (range 1.2 to 54 years). Median VSD size was 7 mm (range 4 to 15 mm). The device was placed successfully in 91% of patients, and complete closure rates were 63% at one month and 96% at six months. There were three patients with serious adverse events, including complete heart block, perihepatic bleeding, and ruptured tricuspid chordae. One wonders why a defect of 4 mm with a QP-QS of 1.0 was closed in this series. Long-term follow-up is important, because late heart block and aortic regurgitation can occur. Patient selection will be extremely important to be certain that only defects that would require surgery are sent to the catheterization lab and that the device is at least as safe as surgery.
Atiq et al. (3) report 17 children with hypoplastic right ventricles (RVs) who underwent device occlusion of an ASD. Nine children had pulmonary atresia with intact septum, six had Ebstein’s anomaly, and two isolated RV hypoplasia. Mean age at closure was 6.6 years, oxygen saturation improved from 91% to 98%, and mean right atrial pressure increased from 9 to 11 mm Hg. Exercise tolerance improved with no evidence of RV decompensation. It appears that many patients with small RVs and mild cyanosis will tolerate this procedure.
Shahriari et al. (4) report follow-up after repair of sinus venosus ASDs with partial anomalous pulmonary venous connection. There were 54 patients who had operation at a mean age of 13 years (range 1.5 to 58 years). Thirteen of these patients had high insertion of anomalous pulmonary veins and underwent the Warden technique, which consists of caval division and atriocaval anastomosis. All remained in sinus rhythm, and only one patient developed symptomatic pulmonary vein obstruction. The caval division technique appears to be quite useful for avoiding caval obstruction as well as in avoiding the sinus node artery. With the advent of earlier repair of ASDs, the question frequently arises of when to operate on an asymptomatic young child with a large sinus venosus ASD with high drainage of pulmonary veins. It would be nice to have more data to determine the true incidence of caval stenosis, pulmonary venous stenosis, and/or atrial arrhythmias in a younger patient group.
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Tetralogy of Fallot
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Therrien et al. (5) report cardiac magnetic resonance (CMR) findings in 17 adults with repaired tetralogy of Fallot (ToF) who underwent pulmonary valve replacement (PVR). The right ventricular end-diastolic volume (EDV) returned to normal levels in patients with preoperative RVEDV  170 ml/m2 and whose right ventricular end-systolic volume (RVESV) was 85 ml/m2. The RV ejection fraction (EF) was unchanged in 32% before and 34% after surgery. This is the first report of a clear quantification of how much RVEDV increase these patients tolerate before return to a normal or near normal volume after PVR.
Dave et al. (6) report on early PVR for chronic pulmonary regurgitation (PR) in ToF patients. Cardiac magnetic resonance was used to measure RVEDV before and 6 months after surgery in 21 of 39 enrolled patients who completed the studies. Considering RVEDV 100 ml/m2 as the desired outcome postoperatively, seven patients with RVEDV at 170 ± 21 ml/m2 achieved normalization, with a mean of 93 ± 9 ml/m2 within six months after surgery. The remaining 14 patients, with a preoperative value of 204 ± 36 ml/m2, had persistent RV dilation with RVEDV 125 ± 28 ml/m2. In addition, the postoperative left ventricle (LV) EF was higher, 60% versus 54%, in those whose RV volumes normalized after surgery. These authors feel that an RVEDV of 150 ml/m2 is a practical cut-off value to proceed with PVR.
van Straten et al. (7) report on 16 ToF patients who had PVR and CMR before and 8 and 22 months after PVR. Eight months after PVR, RVEDV decreased from 164 to 113 ml/m2 and RV EF was unchanged from 44% to 47%. Twenty-two months after PVR, RVEDV was 107 ml/m2 and the RVEF improved significantly to 50%. Diastolic function, as measured by early filling of the RV, did not show improvement at 8 months but was improved at 22 months. These authors did not report their data in terms of whether or not a certain preoperative RVEDV, such as, 150 ml/m2, was associated with unsatisfactory improvement in systolic and diastolic function.
Kurotobi et al. (8) assessed RV volume characteristics using CMR in postoperative patients with RV outflow tract obstruction. The RVEF was plotted as a function of an RV stress index calculated as RV peak pressure/RV mass-volume ratio. This ratio theoretically provides an index of RV afterload. In patients without symptoms and in the control group, a significant inverse correlation was observed between RVEF and RV stress index. All patients whose RVEF ("normalized" for stress index) decreased to <95% confidence limits had symptoms of RV failure. The RV stress index decreased after reoperation, but RVEF remained at <95% limits if it was below normal before surgery. The RVEF as a function of stress index may be useful in assessing RV function and establishing a time for surgical intervention in RV outflow tract obstruction.
Khambadkone et al. (9) report percutaneous PVR in 59 consecutive patients with a variant of ToF or transposed great arteries (TGAs) with VSD and pulmonary stenosis (PS). The RV systolic pressure decreased from 64 to 50 mm Hg, and PR decreased after PVR. Cardiac magnetic resonance showed significant reduction in RVEDV, from 94 to 82 ml/m2. Sixteen patients showed improvement of peak oxygen intake, from 26 to 29 ml/kg/min. This is the first large number of patients reported consecutively. The technique can be used only in patients who are somewhat older; the youngest patient was nine years old and weighed 25 kg. It requires a large introducer sheath, and RV outflow diameter must be 22 mm. It is usually deployed in patients with a conduit that may be as much stenotic as regurgitant. There were adverse events, including stenosis, migration of stent, stent fracture, excessive bleeding, arrhythmia, and endocarditis. This device should be available on a research protocol in the U.S. later this year.
Tan et al. (10) report intrinsic abnormalities of the aortic root in 17 ToF patients. There were histologic changes ranging from medionecrosis, fibrosis, cystic medial necrosis, and elastic fragmentation with lamella disruption. These changes were present as early as a few days after birth. I had felt that aortic root dilation in TOF was primarily due to the increased flow through the aorta in cyanotic patients. It appears that there are genetic causes as well as hemodynamic ones.
d’Udekem et al. (11) report on 82 consecutive patients with pulmonary atresia and VSD with major aortopulmonary collateral arteries (MAPCAs). There were 119 shunts, 130 MAPCA transplantations, and 76 MAPCA ligations. Hospital mortality for preliminary procedures was 4% and concurrent follow-up rate 80%. Sixty-five percent had complete repair. Hospital mortality was 8%, and nine late deaths occurred, all cardiac related. The overall survival of all patients to the age of 30 years was 58%, and survival 12 years after completer repair was 51%. Central shunts promoted growth of central pulmonary arteries in all cases; serial measurements of 29 MAPCAs showed no significant signs of growth. Long-term survival into adulthood with pulmonary atresia, VSD, and MAPCAs has been achieved with a multistage approach. However, late survival depends on the growth of the native circulation. The few unifocalized MAPCAs that did not thrombose failed to grow. This is a somewhat discouraging report in terms of unifocalization. In a discussion for this paper, Dr. Hanley recommends surgery as early as possible and hopefully by three to four months, with extensive unifocalization needed in many patients. Whether or not this strategy will be useful in all hands is unclear, but certainly the earlier one can get flow into pulmonary arteries whether central or peripheral the more likely one can achieve a reasonable pulmonary artery tree for repair later.
Stewart et al. (12) report on 102 consecutive patients who underwent ToF repair with attempt at a valve-sparing strategy which was applied in 80% of patients. They used a strategy of transatrial transpulmonary repair with aggressive pulmonary valve commissurotomy, extensive subvalvar resection, pantaloon pericardial supravalvar pulmonary patching, and, when required, subvalvar infundibular augmentation with a GoreTex patch (W. L. Gore and Associates, Medical Products Division, Flagstaff, Arizona). They are willing to accept an RV/LV pressure of 0.70 early after repair. Only 2 of 73 patients with RV/LV <0.70 required reoperation, whereas 3 of 9 with RV/LV pressure >0.70 required reoperation. Fifteen of 25 patients with a prior shunt had valve-sparing procedures. Postoperative PR, moderate to severe, was present in 15% of patients in the valve-sparing group and in 70% of patients in the transannular patch group. The late postoperative peak echo gradient across the RV outflow tract was 29 mm Hg in the valve-sparing group and 34 mm Hg in the transannular patch group.
There is a renewed attempt to preserve some type of valve in all ToF patients to avoid the effects of severe PR and the need for PVR. Leaving an RV/LV pressure ratio of 0.7 seems high, and one would think these patients with the higher RV pressure could be prone to RV dysfunction and arrhythmias. Further follow-up data will be useful in this regard.
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Transposition of the great arteries
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Babu-Narayan et al. (13) report on late gadolinium enhancement (LGE) during CMR imaging of the systemic RV in 30 consecutive adults with atrial switch for TGA and no pacemakers. Late gadolinium enhancement was seen in 22 patients (61%). These patients were older, with increased RVESV, decreased RVEF, increased QRS duration, and increased QRS dispersion compared with those without LGE. Documented arrhythmia and/or syncope also were significantly higher in the LGE group. Here is another indication of myocardial abnormalities in many adults with systemic RVs. Whether or not the LGE is due to inadequate myocardial blood supply for the severe hypertrophy that some patients get, myocardial ischemia induced at the time of surgical repair or before repair, or some other cause is unclear. Late gadolinium enhancement could serve as another marker to evaluate patients when dysfunction becomes difficult to manage medically, and it might hasten the decision to proceed to transplantation.
McQuillen et al. (14) performed preoperative MRI studies in 29 consecutive term neonates with TGA. Twelve patients (41%), had focal or multifocal brain injury. None had birth asphyxia; all patients with injury had balloon atrial septostomy (BAS) with no other significant correlation with this injury. This remarkable and unexpected result should cause us to re-examine preoperative workup and particularly BAS. Is this really the mechanism for this cerebral injury, or are some of these lesions present before BAS? Further study with pre-BAS MRI studies done on a number of patients would be useful. As a side issue, when preoperative stroke is identified, the question has always been how long to wait before putting patients on cardiopulmonary bypass. These authors state that they delayed surgery 7 to 14 days in 4 of 12 patients with multiple lesions or a single large lesion. In no case did preoperative lesions enlarge or undergo hemorrhagic transformation.
Morell et al. (15) report on 12 patients with TGA, VSD, and PS who underwent aortic translocation and biventricular outflow tract reconstruction. Three patients, in addition, had AV discordance and required a Senning procedure. There was one hospital death, and at median follow-up of 33 months all others were alive. Four reoperations occurred in three patients, including two for conduit obstruction. The LV outflow obstruction was relieved by dividing the outlet septum and excising the pulmonary valve, performing a Lecompte maneuver, and reconstructing the LV outflow tract with the aortic autograft and the VSD patch with re-establishment of RV-pulmonary artery continuity using a pulmonary homograft. This appears to be a viable option in patients who are unsuitable for a Rastelli repair and another option to achieve an arterial switch in patients who were once deemed impossible to have this operation.
Kampmann et al. (16) report on late results for percutaneous transluminal coronary angioplasty (PTCA) for coronary stenosis after arterial switch. Seven patients were evaluated 3 to 15 months after initial PTCA and again after 3 to 5 years. There were no early or late deaths, and the degree of stenosis ranged from 74% to 97% immediately before to 5% to 10% immediately after and 0% to 3% at three to five years after PTCA. All children showed normal development of the treated coronary artery. Hopefully, these patients will be rare and similar results can be duplicated.
Taylor et al. (17) report on MR coronary angiography and late myocardial enhancement in 16 asymptomatic children who underwent arterial switch and were studied at a mean age of 11 years. In 23 of 32 coronary arteries, diagnostic-quality images were acquired. This increased to 100% in subjects older than 11 years. No coronary ostial stenoses were seen, and the proximal course of the coronaries was visualized. Two subendocardial viability defects were detected that corresponded to known compromised arteries that supplied that territory. Global function was preserved with no regional wall abnormalities. This is the good news for this group of patients, and the article includes exquisite studies of anatomy and wall motion.
Pedra et al. (18) used intracoronary ultrasound to assess coronary arteries after arterial switch in 22 asymptomatic children at a median age of 9.5 years. Thirty-three arteries (89%) displayed variable degrees of proximal eccentric proliferation, with maximal intimal thickening of 0.26 mm at the most thickened site. According to the Stanford classification, all children had coronary artery involvement, with 50% having moderate to severe lesions. No risk factors for such abnormalities were encountered, including age, origin of the arteries, hemodynamics, and follow-up duration. These observations suggest the development of early atherosclerotic changes in the reimplanted coronary artery. This lends further credence to the need for careful follow-up for these patients with hemodynamic and functional evaluation of myocardial function, perfusion, and wall motion.
Hui et al. (19) used dobutamine stress echocardiography to determine LV function and wall motion in 31 patients with arterial switch performed at a mean of 15 days and studied at an age of 9 years. All had normal coronary angiographic findings. Fractional shortening and LVEF were significantly lower in patients than in controls. Impaired LV contractility was detected in 19 patients at rest, with older age at operation, longer bypass and circulatory arrest times, and an unusual coronary artery pattern associated with impaired contractility. Dobutamine stress echocardiography unmasked wall motion abnormalities in 74%, and exercise myocardial perfusion scans which were performed in 22 patients showed reversible perfusion defects in 17 patients. These defects corresponded to segments of hypokinesia as detected by echocardiography. This appears to be the bad news. A significant proportion of asymptomatic patients had impaired baseline contractility, reversible myocardial perfusion defects, and mild wall motion abnormalities with dobutamine stress.
Koh et al. (20) report on intermediate results of the double-switch operation for congenitally corrected TGA. Forty-five patients underwent this operation at 6 months to 21 years. An atrial plus arterial switch was performed in 7 patients and a Rastelli type ventriculoarterial switch procedure in 38 patients. Early mortality was 8.9% since 1993. Six patients died late. Actuarial survival at 5 and 10 years was 84% and 78%, respectively. Freedom from arrhythmia was 89% at 5 years and 78% at 10 years. The systemic ventricular EF was 0.57 at 1 year, 0.56 at 5 years, and 0.54 at 10 years. Intermediate follow-up data show reasonably good results for this difficult procedure. There is no agreement in most circles as to when to use the double-switch procedure and which patients are optimal and less optimal candidates for it. The use of the operation at a relatively early age, particularly if any degree of LV retraining is needed, seems to be one caveat. Many younger surgeons have little experience with the Senning operation and will need help in learning this technique.
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Fontan surgery
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Brown et al. (21) report on 21 children, median age two years, with heterotaxy syndrome and interrupted inferior vena cava with azygous continuation, who underwent the Kawashima operation. Follow-up was complete in all but 1 survivor at the median age of 4.5 years, and pulmonary arteriovenous malformations were diagnosed at a median of 5 years after the operation in 11 of 20 (58%) patients. Overall survival was 90% at 10 years. Multivariate analysis demonstrated presence of bilateral superior vena cava and interval longer than two years between Kawashima operation and completion of Fontan operation as predictors of pulmonary arteriovenous malformations. This interesting phenomenon is most likely due to dilation of known channels, recruitment of new channels, or an angiogenic process and develops in most patients when there is no hepatic blood flow going directly to the lungs. In most cases these will regress after completion of the Fontan operation. Earlier completion of the Fontan operation is indicated in these patients to prevent this problem which sometimes does not regress as rapidly or as completely if the interval between development of malformations and completion of Fontan is delayed.
McElhinney et al. (22) report on incorporation of the hepatic veins into the cavopulmonary circulation with heterotaxy and pulmonary AV malformations in 16 patients after a Kawashima procedure. In 11 of 15 patients, oxygen saturations rose to 90% in a year and remained at 93% or more at 2.8 to 10 years. Four patients had persistent hypoxemia and residual AV valve malformations at follow-up of 1.5 to 8 years after surgery. This again, supports the early incorporation of hepatic venous effluent to the pulmonary circulation to prevent or treat this problem.
Morales et al. (23) report on salvaging the failing Fontan with lateral tunnel versus extracardiac conduit connections in 35 patients. In addition, 24 of 35 patients underwent a modified Maze procedure, and 29 of 35 patients had pacemaker implants. There were no hospital deaths or arrhythmias at discharge. With intermediate follow-up at 29 months, survival was 94%, and 97% were in New York Heart Association (NYHA) functional class I or II, with 91% freedom from late arrhythmias requiring medication and no patient requiring transplant. Both the lateral tunnel and extracardiac connection revisions can provide symptomatic benefit for a failing Fontan connection and have equivalent early and intermediate results.
Mavroudis et al. (24) report on AV valve procedures in 80 patients with repeat Fontan operations and the influence of valve pathology, ventricular function, and arrhythmias on outcome. Arrhythmia surgery was required in 78 of 80 patients, venous pathway revision in 78 of 80 patients, and AV valve repair/replacement in 15 of 80. Mean ages were 20 years at reoperation and 7 years at earlier Fontan operation. Ventricular dysfunction, valvular dysfunction, and atrial arrhythmias increased during the 12 years before the repeat Fontan operation. The AV valve procedures were performed on 8 functional mitral valves and 7 functional tricuspid valves. Multivariate analysis for death, heart transplant, or renal dialysis showed severe ventricular dysfunction, age >25 years, right or ambiguous functional ventricle, and ischemic time >100 min to be significant. Mitral valve repairs were inconsistent owing to probable technical misjudgements, but most tricuspid valves could not be repaired and had to be replaced. Operative and late mortalities was 1.2% and 5%, respectively. Emergent and late transplants were 1.2% and 3.7%, respectively. One of the difficult management issues is when to abandon the Fontan and go directly to transplantation and when to try to salvage it with this type of operation. It does appear that many of these patients can be salvaged, at least temporarily, with the Fontan re-do when valve pathology and arrhythmias can be treated simultaneously.
Chowdhury et al. (25) report on 11/2 ventricular repairs in 84 patients, ages 4 to 504 months. There were 69 survivors with operative and late mortalities of 11% and 8%, respectively. Perioperative and postoperative atrial arrhythmias were observed in 14% and 16% of patients. Mean late superior caval pressure was 14 mm Hg and right atrial pressure 7 mm Hg at the median follow-up of 87 months, and 90% were in NYHA functional class I or II. Cardiac magnetic resonance imaging demonstrated significant growth of tricuspid valve and RV cavity in 45% of patients. These repairs can be performed with acceptable risks, and the operation maintains a low pressure in the inferior vena caval tributaries. Patients with a tripartite RV demonstrated a tendency toward enlargement of the ventricular chambers commensurate with somatic growth. These authors demonstrate good results in patients with moderate RV hypoplasia, with a tricuspid valve Z score of –1.5 to –4.8 (45% to 70% of normal), and the RV cavity one- to two-thirds of normal. This procedure is not useful with elevated pulmonary vascular resistance or when used to treat acute postoperative RV dysfunction.
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Hypoplastic left heart syndrome
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Tabbutt et al. (26) retrospectively compared the outcomes of all neonates who underwent a stage I reconstruction with the use of an RV-PA conduit versus modified Blalock Taussig (BT) shunt interspersed over 2 years. There were 95 patients with a BT shunt and 54 patients with an RV-PA modification. There was a preference for RV-PA conduit with aortic atresia: 67% RV-PA versus 30% with a BT shunt. There was no difference in surgical mortality, time to extubation, or length of hospital stay. There was an increased incidence of shunt reinterventions with RV-PA conduits, and these patients returned earlier for stage II reconstruction, at 5.6 versus 6.5 months. There was no difference in overall mortality with a median duration of follow-up of 18 months. These data, not strictly randomized, give a portrayal of the two procedures as being more or less equal in terms of short- and mid-term outcomes. The RV-PA conduit, theoretically, has the advantage of not causing a critical reduction in systemic output such as can occur with a large BT shunt. This problem can be minimized with sustained afterload reduction and smaller shunts, as many centers have demonstrated. Whether or not the higher aortic diastolic pressure with the RV-PA conduit and potential impact on coronary blood flow will result in less interim morbidity and mortality is unclear. In addition, the question of long-term damage to the systemic RV with the ventriculotomy is a potential negative risk factor for the RV-PA conduit.
Jeffries et al. (27) report on gastrointestinal morbidity after Norwood palliation for hypoplastic left heart syndrome. Gastrointestinal complications occurred in 48 of 117 patients, including 18% with necrotizing enterocolitis (NEC), 18% who required feeding tubes, and 8% who required prolonged hospital stay for nutritional support. Multivariate analysis revealed weight <2.5 kg and development of NEC were each independently related to death. Gastrointestinal complications are common in these infants, and these authors speculate that damage to the left vagus nerve during dissection may be a contributing factor. They and others recommend gastrostomy instead of nasogastric feedings to avoid the potential aspiration problems that could occur at home with dislodgement of the tube and/or reflux.
Bacha et al. (28) report on 14 high-risk neonates who underwent a hybrid stage I procedure with pulmonary arteries banded and a ductal stent delivered via pulmonary artery puncture and positioned under fluoroscopic guidance. In addition, ASD stenting was required in several patients. Hospital survival was noted in 11 of 14 (79%) patients, but only 6 of 14 patients achieved stage II operation without mortality. In this high-risk group, this represents a reasonably good achievement for this beginning strategy. Currently, they do not recommend this technique for patients with questionable preductal coarctation, as occasionally seen with aortic atresia.
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Valvular interventions
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Boston et al. (29) report on a 30-year experience with tricuspid valve repair in 52 children with Ebstein’s anomaly. Mean age was 7 years with a range of 5 months to 12 years. Other procedures included ASD closure in 46 patients, division of accessory pathway in 4 patients, VSD closure in 3 patients, and others in 7 patients. Early mortality was 5.8%. Morbidity included transient atrial and ventricular arrhythmias and early reoperation. There was no need for permanent pacemaker. Mean follow-up was 12 years. Survival was 90% at 15 years, and freedom from reoperations at 10 years was 77% and at 15 years 61%. Moderate tricuspid regurgitation on dismissal echocardiogram was the only risk factor for reoperation. There was no tricuspid stenosis at late follow-up, and 89% were in NYHA functional class I or II. These 52 patients who had valve repair were from a total group of 186 patients, age 12 years or younger, who came for operation. These authors use this technique in valves that have at least 50% of the anterior leaflet delaminated and a leading edge not adherent to the endocardium. In addition, contrary to early experience, plication of the atrialized portion is not performed unless it is severely thinned out. It appears that extensive placation may be associated with ventricular arrhythmias postoperatively.
Brown et al. (30) report on experience with mitral valve replacement (MVR) using a pulmonary autograft in 8 patients, 12 to 46 years of age, with congenital or acquired mitral valve disease. There were no deaths; 3 of 4 patients required mechanical MVR 6 to 14 months later. This technique deserves consideration in younger patients in sinus rhythm, particularly in patients who want to avoid anticoagulation and in whom a large effective orifice is needed.
McElhinney et al. (31) report on management of severe congenital mitral stenosis (MS) in 108 infants and children who underwent balloon mitral valvuloplasty or surgical intervention at a mean age of 18 months. Anatomic subtypes were typical congenital MS in 78 patients, supravalvular MS in 46 patients, parachute mitral valve in 28 patients, double orifice mitral valve in 11 patients, and multiple types in >50 patients. Overall, survival was 92% at one month, 84% at one year, and 77% in five years. Initial MVR at a younger age was associated with worse survival; survival in 1 year free from failure or biventricular repair or mitral valve reintervention was 55% for patients who underwent balloon mitral valvuloplasty and 69% for patients who underwent supravalvular mitral ring resection initially. In contrast to earlier admonitions from the interventionalists, balloon mitral valvuloplasty now may be useful in some children with severe congenital MS who require intervention. This is associated with significant mitral regurgitation but may provide palliation so that a larger valve may be implanted at a later date. This catheter intervention should be performed only by those with considerable experience and with surgical back-up available, because significant worsening of mitral regurgitation is possible.
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Coarctation and left ventricular outflow obstruction
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Cowley et al. (32) report on long-term outcomes in 21 children randomized to surgery or balloon angioplasty (BA) for native coarctation. After an average time of 11 years there was a higher incidence of aneurysm in 35% versus 0% and a greater arm/leg difference in blood pressure with exercise among BA subjects. Only 50% of BA patients remained free of both aneurysm formation and repeat intervention versus 88% of surgical subjects. This incidence of aneurysm formation in native coarctation is significantly higher than a number of earlier reports. In addition, the arm/leg blood pressure differential may have been the result of femoral artery injuries sustained at the time of intervention. Lower-profile catheters now will decrease this complication. Nevertheless, in native coarctation, there does appear to be a higher risk of aneurysm formation with BA.
Head et al. (33) report on postnatal diagnosis of coarctation in infants whose fetal study suggested this diagnosis. There were 144 fetuses with suspected coarctation who were followed postnatally. Forty-three had coarctation and 4 had interruption of the aortic arch. Three infants presented late, at 7 to 13 weeks of age, 6 to 12 weeks after closure of their arterial duct. Postnatal follow-up in infants suspected of coarctation will need to go well beyond the time of ductal closure for accurate diagnosis.
Tworetzky et al. (34) report on CMR imaging of LV endocardial fibroelastosis after fetal intervention for aortic stenosis. These infants had fetal balloon valvuloplasty followed by extensive resection of endocardial fibroelastosis postnatally. This technically challenging surgical procedure may lead to a two-ventricle circulation in patients in whom this rare diagnosis is made.
Luciani et al. (35) report 90 patients, age 29 ± 10 years (range 6 to 49 years), who underwent a Ross procedure with follow-up for an average of 5 years. Ten-year survival was 98%. Ten-year freedom from autograft dilation was 43% and from aortic regurgitation 75%. The Ross procedure provides excellent hemodynamics, but issues with aortic root dilation and aortic regurgitation cloud the picture. Operations to replace the pulmonary homograft were rare. Modifications of the technique of replacement of the valve and part of the root with buttressing may help the dilation.
Tierney et al. (36) report on cryopreserved homografts in pediatric patients with 26 in the Ross group and 70 in a non-Ross group. Homograft failure rates were 12% for Ross and 51% for non-Ross patients. Freedom from intervention was 93% for Ross and 66% for non-Ross patients.
Karamlou et al. (37) report on 198 aortic valve replacements (AVRs) in 160 patients <18 years of age with operation between 1974 and 2004. This study involved both mechanical and tissue valves. The two risk factors for decreased time to repeat AVR was early age at operation and use of bioprosthetic or homograft valve. Pulmonary autograft use was associated with decreased mortality, slower gradient progression, and smaller left ventricular dimension for follow-up.
Brown et al. (38) report on follow-up of apical aortic conduits in children with complex left ventricular outflow obstruction. There were 21 patients, ages 2 weeks to 19 years, who underwent insertion of apical aortic conduit between 1979 and 1993. All were symptomatic, and 79% had two previous aortic valvotomies and/or left ventricular outflow tract operations. Hospital mortality was 11%, and overall 25-year survival was 57%. Reduction of LV-aortic peak gradient after surgery was from 82 to 15 mm Hg. Fourteen surviving patients had undergone subsequent procedures from 5 months to 16 years after surgery owing to recurrent aortic stenosis, and 1 had transplantation. Late survivors had normal LV function. There are still a few patients around who have had this operation which has provided good relief of obstruction in many patients with complex LV outflow obstruction. In the last 15 years, this procedure has been replaced by the Ross-Konno procedure. Current indication for this procedure might be a child with serious reservations about doing a repeat sternotomy, such as with prior mediastinitis or sternal wound infection. Preoperative assessment of the lower thoracic aorta by computerized tomography is useful to ensure a safe location for a distal conduit.
Baker et al. (39) report on ascending aortic extension for enlargement of the aortopulmonary space in seven patients with a mean age of 2.9 years who had developed severe pulmonary artery (PA) stenosis after complex neonatal aortic reconstruction. All patients underwent aortic extension with an interposition graft and patch angioplasty of the PA to open the aortopulmonary space and relieve PA narrowing. There was one early death and one late cardiac death from aspiration pneumonia. Early follow-up demonstrated relief of PA stenosis and unobstructed aortic flow. This procedure may be necessary in patients with severe PA stenosis complicating their Fontan approach in most cases.
Lambert et al. (40) report on surgical management of airway compression of vascular origin in 37 consecutive patients with a median age of 4 months using computerized tomography. Seventeen patients had persisting respiratory symptoms or prolonged mechanical ventilation after cardiac surgery. High-quality diagnostic images were obtained in all cases without complications. These studies can be valuable in complex patients in whom both PA stenosis is the issue, as in the preceding report (39) or when airway compression complicates ventilation management.
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Surgery for complex conditions
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Odim et al. (41) report on follow-up of 106 patients with pulmonary atresia with intact ventricular septum between 1982 and 2001. Over this period, children were assigned to mild, moderate, or severe RV hypoplasia, and this grouping, along with severity of coronary anomalies, included 45% with RV coronary fistulae and 16% with RV-dependant coronary circulation and allowed triaging of children to eventual single versus biventricular repair. Actuarial 10-year survival was 86%, with mortality predicted by severe hypoplasia and non-Caucasian race. Right ventricle-dependant coronary circulation was present in 16 patients, and 6 (38%) died before definitive repair, versus 4% who died before definitive repair when these connections were absent. Severe hypoplasia resulted in death before definitive repair in 18%, and mild to moderate hypoplasia resulted in death in only 2% before definitive repair. The classification of RV size is extremely important in this condition. These authors termed mild hypoplasia as RV size two-thirds or more of normal and a tricuspid valve Z score of 0 to –2, moderate hypoplasia as RV one- to two-thirds of normal and a Z score of –2 to –4, and a severely hypoplastic RV as less than one-third of normal and a Z score below –4. The RV-dependent coronary circulation was deemed present when a major portion of LV myocardial perfusion was exclusively from the RV cavity. This occurred with proximal left coronary stenosis with RV-LV coronary fistulae, extensive RV-coronary fistulae, or myocardial perfusion scans indicative of reversible defects. Patients with borderline RV had the ASD partially closed with an adjustable snare put in to attempt to keep right atrial pressure below 15 mm Hg and preserve LV preload and cardiac output in the midst of acceptable oxygen saturations in the mid 80s.
Konstantinov et al. (42) report on truncus arteriosus associated with interrupted aortic arch in 50 neonates in a multi-institutional study. Truncal valve stenosis was present in 12% and regurgitation in 22%, and there were 34 deaths with a single early hazard phase. Overall survival was 44% at 6 months and 31% at 10 years. One patient had primary cardiac transplantation, and 4 died without intervention. Results have improved somewhat over time. At five years after aortic arch repair, only 28% were alive without conduit reoperation. This remains a very difficult lesion to treat effectively. In the commentary, Dr. Lacour-Gayet indicates that the correct technique for treating this complex malformation includes one-stage repair in the first days of life, including mobilization of the descending aorta, direct aortic anastomosis, transection of the common trunk, harvesting of a large pulmonary artery branches cuff, patch enlargement of the ascending aorta, and a valved conduit reconstruction of the RV outflow tract. He feels that an ascending aorta patch enlargement is essential to prevent ascending aortic stenosis due to the difference between the truncal root size and the small ascending aorta. Hopefully, results will continue to improve with this technique which may not have been used in the majority of the patients in this series.
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Transplantation/pulmonary vein stenosis
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Choong et al. (43) report on 35 patients who had repair of congenital heart disease and lung transplantation and 16 who had heart-lung transplantation between 1990 and 2003. Freedom from bronchiolitis obliterans at one, three, and five years was 73%, 55%, and 55% for the repair of congenital heart disease and lung transplantation and 78%, 52%, and 39% for the heart-lung transplantation group. Survival at one, three, and five years was 63%, 51%, and 51% for repair of congenital heart disease and lung transplantation and 67%, 67%, and 60% for the heart-lung transplantation group. These patients are usually at the end of the line and have been waiting a considerable period of time before they get their lung transplantation Unfortunately, bronchiolitis obliterans remains a major issue and when this occurs, morbidity and mortality are severe.
They do advocate attempted sutureless repair of pulmonary vein stenosis when present before transplant.
Devaney et al. (44) report on 36 patients who underwent repair of pulmonary vein stenosis between 1989 and 2003. Fourteen underwent scar excision and primary repair, four underwent intraoperative stent placement, and eight had pericardial marsupialization. Twenty-two with acquired stenosis after total anomalous pulmonary venous connection repair underwent anastomotic revision and/or vein repair and 11 sutureless pericardial marsupilization. Follow-up ranged from 1 month to 14 years, with a median of 30 months. Among the 14 with congenital pulmonary vein stenosis, 8 died. Among the six survivors, five had not developed restenosis, including four after marsupialization. Among 11 of 22 with acquired pulmonary vein stenosis undergoing an anastomotic repair, there were 5 deaths, and 1 of the 6 survivors developed restenosis. Of the remaining 11 undergoing marsupialization, there was 1 late death with restenosis and 10 survivors with no restenosis. With this new type of marsupialization technique for pulmonary vein stenosis, this article gives further hope for patients.
Price et al. (45) report on a symptom complex associated with transplant coronary disease and sudden death. These authors studied 66 heart transplant patients and attempted to correlate abdominal, chest, and arm pain with coronary disease as defined with coronary angiography by interval narrowing of a vessel or by at least 50% obstruction to one or more coronary arteries by histologic examination of explanted or autopsied hearts. There was a high correlation between any pain described and coronary disease as defined with a positive predictive value of 82%. The relative risk of transplant coronary artery disease being present in patients with a history of pain was four times that of patients without pain. Sudden death or resuscitated sudden death occurred in 68% of 22 patients with pain versus 9% of 44 patients without pain. These data suggest that the reinnervation that has been described in adults with transplantation can occur in pediatric patients. In addition, there is a suggestion that any type of pain in these patients should be evaluated for ischemia, including possibly coronary artery angiography with intravascular ultrasound as well as searching for induced myocardial ischemia with stress-echocardiography and/or perfusion studies.
Dubin et al. (46) report on cardiac resynchronization therapy (CRT) in 73 patients with congenital heart disease, 16 with cardiomyopathy, and 14 with complete AV block. The CRT resulted in systemic ventricular EF increasing by 13%. Of 18 patients who underwent CRT while listed for heart transplantation, 3 improved sufficiently to allow removal from the waiting list, 5 underwent transplant, 2 died, and 8 others were awaiting transplantation. Cardiac resynchronization therapy is not without difficulty in obtaining good leads, particularly in patients with multiple previous cardiac procedures, but can be useful in selected patients with poor function and a wide QRS.
Hinton et al. (47) report on genetic causes and developmental insights into congenital heart disease. This review of genetic causes of congenital heart disease is quite useful in terms of the amazing number of genetic defects that have been found to be associated with various congenital heart defects (as shown in Fig. 3 of Hinton et al. [47]).
Rhodes et al. (48) report on cardiac rehabilitation and the results of exercise testing in 19 patients with congenital heart disease. Improvements were found in 15 of 16 patients with peak oxygen intake increasing from 26 to 31 ml/kg/min.
I had always hoped that cardiac rehabilitation could be performed with the appropriate physician instructions and perseverance by the patient. Unfortunately, it appears that a more structured rehabilitation program is necessary to achieve significant results and to keep at it in patients with serious congenital heart disease.
Biagini et al. (49) report on dilated-hypokinetic evolution of hypertrophic cardiomyopathy (HCM) in pediatric and adult patients. Dilated-hypokinetic evolution is rare but not exceptional in HCM. Young age at diagnosis, family history of HCM, and greater wall thickness are incremental risk factors for this evolution which carries an ominous prognosis.
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References
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Top
Septal defects
Tetralogy of Fallot