YEAR IN CARDIOLOGY SERIES
The Year in Congenital Heart Disease
Thomas P. Graham, Jr, MD, FACC*
Department of Pediatric Cardiology, Vanderbilt University Medical Center, Children’s Hospital, Nashville, Tennessee
Manuscript received February 28, 2005;
revised manuscript received March 22, 2005,
accepted April 5, 2005.
* Reprint requests and correspondence: Dr. Thomas P. Graham, Jr., Vanderbilt Children’s Hospital, Division of Pediatric Cardiology, D-2220 Medical Center North, Nashville, Tennessee 37232 (Email: tom.graham{at}vanderbilt.edu).
This year has been one in which there have been important developments related to the entire spectrum of the fetus to the older adult with congenital heart disease (CHD). I have tried to group these papers in age-related categories. I believe this is an indication of how the management of patients with CHD now encompasses this ever-enlarging patient population from the beginnings of life until middle to late adulthood.
 |
Fetal cardiology
|
|---|
Tworetzky et al. (1) reported results of management of 24 fetuses predicted to progress to hypoplastic left heart syndrome (HLHS) if left untreated. Twenty underwent attempted dilation of aortic stenosis (AS) with technical success in 14. Fetal echocardiograms after intervention demonstrated growth arrest of left heart structure in unsuccessful cases, and in those who declined the procedure. Significant left heart growth was seen in successful cases which resulted in a two-ventricle circulation at birth in three neonates. Performing this procedure is a real tour-de-force requiring the collaboration of a dedicated team. Although complications and mortality were high, this study demonstrates the feasibility of this intervention for selected fetuses at risk for developing HLHS.
Marshall et al. (2), with the same group of investigators, report atrial septal defect (ASD) creation or enlargement in fetuses with HLHS and intact or highly restrictive atrial septum. These infants are known to have severe early postnatal distress. Ultrasound-guided fetal atrial septoplasty consisting of septal puncture and ASD dilation was found to be feasible in six of seven patients. There was one fetal death after the procedure; the remaining fetuses were live born at term. Although technically feasible, the real dilemma is whether these fetuses would be benefit by going to term with intervention immediately after birth or in utero. We would need detailed data on pulmonary vascular assistance and/or pulmonary venous images to better understand whether the early intervention reduces risk.
Rychik et al. (3) used echocardiography to evaluate heart function before, during, and early after fetal surgery for noncardiac congenital anomalies in 83 fetuses at a mean gestational age of 23 weeks. Heart rate increased acutely and combined cardiac output diminished at the time of fetal incision for all groups. Ventricular dysfunction and valvular dysfunction were identified in all groups as well as acute constriction of the ductus arteriosus. These acute changes indicate the importance of echocardiographic monitoring in the management of patients with severe congenital anomalies who can benefit from fetal surgery.
Jaeggi et al. (4) report on a standardized treatment approach including administration of maternal dexamethasone and beta stimulation for complete atrioventricular block (CAVB) and fetal heart rates <55 beats/min. There were 37 consecutive cases of fetal CAVB. The age at diagnosis was 26 weeks, and in 92% maternal autoantibodies were present. This therapy remarkably improved the survival rate and decreased the incidence of immune-mediated conditions such as myocarditis, hepatitis, and cardiomyopathy.
|
Neonatal and infant cardiology
|
|---|
Vlahos et al. (5) report a 13-year experience treating HLHS infants with intact or very restrictive atrial septum. There were 33 newborns that underwent urgent/semiurgent catheterization to create or enlarge an ASD before surgical palliation. Preoperative and early postoperative mortality was 48% with 7 of 33 dying before surgery compared with 0 of 66 control HLHS patients without restrictive ASD. Freedom from death at six months was 42% in patients with restrictive ASD and 79% in control HLHS patients. This study lends support to fetal intervention in these patients (2). However, there is still room for comparison of current day management of immediate postnatal septostomy with fetal intervention.
Sano et al. (6) report a multi-institutional study on first-stage palliation of HLHS using a right ventricle-to-pulmonary artery (RV-PA) shunt. There were 61 (84%) hospital survivors, including 5 of 6 patients weighing <2 kg, and 8 late deaths. Risk factors for hospital mortality included preoperative treatment without ventilatory support and surgeon’s experience for the first 10 cases. These authors report improved survival with this procedure but clearly show the effects of a learning curve for this modification of HLHS first-stage palliation.There is still controversy about whether RV-PA versus standard first-stage palliation using an aorta-to-pulmonary artery (AO-PA) shunt is the better operation for these infants.
Tanoue et al. (7) report on measures of contractility and afterload as well as right ventricular efficiency in 21 HLHS patients who underwent both the bidirectional cavopulmonary connection (BCPC) and a total cavopulmonary connection (TCPC) after initial Norwood palliation. Eleven patients initially had the AO-PA shunt, and 10 had the RV-PA modification. Data was obtained at cardiac catheterization before and after the BCPC and approximately one year after TCPC. Mid-term ventricular performance of the RV-PA conduit group was comparable with the AO-PA shunt group in terms of ventricular efficiency. However, after the BCPC, contractility in patients with an RV-PA connection was inferior to that in patients with an AO-PA shunt. Although the difference in contractility was small, this study raises concern about the effect of right ventriculotomy on ventricular function. Further studies of ventricular performance and contractility in patients after palliation for HLHS will be important with attempts to match patients with similar risk factors in both groups including cross-clamp time, valvular insufficiency, and afterload excess.
Fenton et al. (8) studied 35 infants who underwent placement of AO-PA shunts for pulmonary atresia with intact ventricular septum. The right ventricles were severely hypoplastic in 22 of 35 infants, and hospital death occurred in 2 patients, 1 with severe hypoplasia. There are a total of 5 deaths after discharge and before second-stage operation in the remaining 33 patients. All had severe right ventricular hypoplasia. There were two deaths after second-stage operation. This interim mortality rate is similar to that in HLHS, and must be considered when different management options are considered. Shunt thrombosis and myocardial infarction accounted for four of five infant deaths.
Sittiwangkul et al. (9) studied 225 consecutive patients with tricuspid atresia between 1971 and 1999. There was no significant change in Fontan operative mortality over this period. Total survival was 90% at age 1 month, 70% at 10 years, and 60% at 20 years. There were 7 early deaths, 11 late deaths, and 3 patients progressing to heart transplantation.
This report is discouraging in terms of the continued slow mortality with time in patients who we usually consider as good candidates for the Fontan operation. These authors noted a significant incidence of shunt thrombosis even in patients on aspirin. They suggest an aggressive interstage home surveillance program including monitoring weight gain, oxygen saturation, and early symptomology. In addition, early caval shunting is suggested as another means to prevent this interim mortality.
Alwi et al. (10) studied patients with duct-dependant pulmonary circulation who underwent catheterization with the intent of patent ductus arteriosus (PDA) stenting as first palliation. Successful PDA stenting was performed in 51 patients (91%) and failed in 5 patients. Morbidity included stent dislodgement with migration to the left femoral artery in one patient and transient intravascular hemolysis in another patient. There was no procedure-related mortality. At follow-up from three months to two years, eight patients developed significant stent stenosis requiring re-intervention. Seven patients developed worsening of pre-existing branch pulmonary artery stenosis. This experience with PDA stenting indicates the potential usefulness of this technique in patients who do not have pulmonary artery stenosis at or near the PDA. This technique requires a great deal of skill and experience, as well as careful monitoring after intervention because the ductus may narrow with time. It can be an attractive alternative for avoiding early operation and then proceeding to an early cavopulmonary shunt for selected patients (Figs. 1 and 2).
View larger version (121K):
[in this window]
[in a new window]
|
Figure 1 Transcatheter approaches of stent implantation. (a and b) Pre- and post-stent implantation in tetralogy of Fallot-pulmonary atresia. A 6-F Guiding Judkin’s right catheter was passed through ventricular septal defect and anterogradely into ascending aorta with guide wire securely anchored in distal pulmonary artery; (c) normal ductus morphology in tricuspid atresia; (d) post-stent implantation by retrograde transarterial approach. Reprinted with permission from Alwi et al. (10).
|
|
Batra et al. (11) studied the site of systemic pulmonary artery shunt insertion and growth of pulmonary arteries. A total of 101 patients with modified Blalock-Taussig shunts were evaluated. With shunts to the right pulmonary artery and no antegrade pulmonary flow, the left pulmonary artery was significantly smaller than when the distal connection was to the main pulmonary artery. Absence of antegrade pulmonary flow resulted in significantly smaller right and left pulmonary sizes in general. These studies suggest that, in the absence of antegrade pulmonary flow, a modified Blalock-Taussig shunt to the main pulmonary artery may promote more uniform growth in branch pulmonary arteries and be particularly important in patients with univentricular palliation with subsequent cavopulmonary shunts and Fontan procedures.
Dibardino et al. (12) report results in 125 consecutive neonatal and infant arterial switch operations for transposition (TGA) performed between 1995 and 2003. A total of 63% had intact ventricular septum, 30% had ventricular septal defect (VSD), and 7% had Taussig-Bing anomaly. The 30-day mortality was 1.6% with two late deaths, and overall actuarial survival rate was 96.3% at 7 years. The incidence of complex coronary ostial origin and branching, including single coronary in 6.4% and intramural coronary artery in 6.4%, was not associated with increased operative risk. All patients were without symptoms at the latest follow-up. There were no late coronary events, and 5.8% have required cardiovascular reoperation at an average of 15 months. These patients are mostly free from clinically apparent complications. Pulmonary artery stenosis, aortic root dilation, aortic insufficiency, and asymptomatic coronary occlusion do occur, and surveillance for these potential problems is needed.
McMahon et al. (13) report on risk factors for neo-aortic root enlargement and aortic regurgitation after arterial switch operation. Prior pulmonary artery banding, the presence of VSD, and Taussig-Bing banding are risk factors for severe aortic root enlargement. Surgical intervention for aortic regurgitation was rare, only 2% in this cohort of 119 patients.
Mohammadi et al. (14) report on left-sided lesions in 109 of 120 survivors after anatomic repair of TGA, VSD, and coarctation. Two-stage repair was performed in 42 patients and single-stage repair was performed in 67 patients. Early and late survivals were better for those patients who had neonatal single-stage repair with pulmonary homograft aortic augmentation. Despite neoaortic root enlargement, most patients had trivial or mild aortic regurgitation. Recoarctation required angioplasty in nine patients and repeat surgery in nine patients. There were two late deaths from coronary artery stenosis and myocardial infarction. Subaortic stenosis developed in seven patients with four deaths. This was mainly due to VSD malalignment and, therefore, disease-related. It is an important postoperative complication to be aware of.
Burch et al. (15) report on persistent pulmonary hypertension late after neonatal aortic valvotomy. This rare complication of treatment for neonatal AS appears to be related to severe left ventricular diastolic dysfunction with preserved systolic function. This restriction to left ventricular filling fortunately is rare but very difficult to treat medically. Whether or not left ventricular endocardial resection would be useful for these patients or whether they will require heart or heart/lung transplantation is unclear. Further follow-up on these rare patients with attempts to determine optimal strategy is needed.
Vandekerckhove et al. (16) report on the closure of a PDA with indomethacin in two infants with excessive pulmonary blood flow and associated CHD. Both were full-term, one born at 39 weeks and one at 41 weeks gestation. Indomethacin was given on day 14 in one infant after an AO-PA shunt for tricuspid and pulmonary atresia. Closure of PDA was confirmed 24 h later. The second infant had right heart hypoplasia and had an AO-PA shunt performed on day 3. Because of excessive pulmonary flow, indomethacin was given and the PDA closed.
Whether or not these case reports indicate that a PDA can respond to indomethacin in full-term infants is unclear. It does appear that there was a response in these two cases, although one would have to have more evidence to be absolutely certain that spontaneous PDA constriction did not occur. Nevertheless, this represents a potential new paradigm, and further use of this modality may be indicated in selected term infants to see if surgery can be avoided in patients with excessive flow from a PDA.
|
Childhood cardiology
|
|---|
Basso et al. (17) reported an echocardiographic study of 817 apparently healthy primary school students. Bicuspid aortic valve (BAV) was found in 0.5% of cases with a higher prevalence in males (0.75% vs. 0.24%) and was significantly associated with aortic root enlargement compared with children who had tricuspid aortic valves.
Fernandes et al. (18) report on the morphology of BAV in a review of 1,135 patients <18 years of age who were identified by echocardiography between 1986 and 1999. Median age was three years, and 67% of the patients were males. Right and left coronary leaflet fusion was the most common type occurring in 70%; AS that was moderate or greater was observed most often in patients with right coronary/noncoronary fusion. Similarly, right coronary/noncoronary fusion was more often associated with moderate or greater aortic regurgitation. The majority of patients with coarctation had fusion of right /left coronary leaflets. Prevalence of BAV in simple coarctation was 55%, complex coarctation was 18%, HLHS or interrupted aortic arch was 11%, and a similar prevalence to that in the general population (0.6% to 0.8%) in other lesions studied.
Gurvitz et al. (19) report on aortic root dimensions and valve dynamics in 76 children with BAV and compared these with values derived from 41 normal children. The children with BAV had significantly larger aortic roots regardless of the presence of AS or aortic regurgitation.
Cripe et al. (20) report on 50 probands with BAV. A third-generation family history and echocardiograms of first-degree relatives were obtained; BAV was identified in 74 individuals with a prevalence of 24%. A total of 97 individuals (31%) had BAV and/or other cardiovascular malformations including coarctation, VSD, ASD, abnormal mitral valve, aortic root dilation, or HLHS. The high heritability of BAV suggests that, in this study population, BAV determination is almost entirely genetic. Heritability suggests that valve formation can be primary to defective valvulogenesis or secondary to other elements of cardiogenesis.
Cecconi et al. (21) report on dimensions of the entire aorta at different anatomic levels using two-dimensional echocardiography in 162 consecutive patients with isolated BAV without significant aortic valve dysfunction. The aortic dilation involved aortic root and ascending aorta but was not present in the descending and abdominal aorta. A significant increase in the dimensions of the aortic arch was found in patients >40 years of age.
Cattaneo et al. (22) report on surgery for aortic root aneurysm in 50 children. There was no operative or hospital mortality. Twenty-six children had aortic root replacement with a composite graft, 10 patients had replacement with homograft aortic root, and 14 patients had a David II valve-sparing procedure. Long-term results were excellent in the 26 children receiving a composite graft. There were three late deaths (11, 16, and 17 years, postoperatively). The question still remains as to when to intervene in children with large aortic roots. Most of these operations were done in patients with Marfan’s syndrome. An aortic diameter of 5 cm with a family history of rupture/dissection or 5.5 cm with no other indications were used as criteria for surgery. In children <12 years of age, these authors recommend surgery when the aneurysm satisfies the above criteria or if the diameter has increased 1 cm or more in 12 months. In addition, the emergency of aortic regurgitation is an indication for valve-sparing procedures.
McMahon et al. (23) report on risk factors for aortic valve dysfunction and aortic regurgitation in 220 children with discrete subvalvar AS. Excluding patients with previous surgical or balloon valvuloplasty, a higher maximal Doppler gradient was an independent risk factor for moderate/severe aortic regurgitation. Independent predictors of low-risk patients included none or trivial aortic regurgitation, peak gradient  30 mm Hg, thin and mobile aortic valve leaflets, and an associated VSD.
Puchalski et al. (24) report on the follow-up of hypoplasia of left heart structures in 55 patients with isolated coarctation. All patients with isolated coarctation and mitral/aortic valve, Z-score <–2 who underwent neonatal repair were reviewed. All patients were alive and well, and none had evidence of mitral stenosis. Both mitral and aortic annulus increased significantly; nine patients developed left ventricular outflow tract obstruction by echocardiographic criteria. Despite initial annular hypoplasia, the need for intervention for mitral stenosis or subaortic stenosis is uncommon in patients with isolated coarctation.
Ou et al. (25) report on late systemic hypertension and aortic geometry after successful repair of coarctation. There was an association between a gothic type of postoperative aortic architecture in which there is acute angulation between the ascending and descending aorta with the horizontal aorta being short or absent. This type of architecture was found in 45% of patients and was associated with a higher prevalence of hypertension. It is unclear as to whether or not this type of architecture alone represents an independent risk factor for systemic hypertension and later complications. Another factor in successfully repaired coarctation that can lead to hypertension includes abnormal wall dynamics at the site of repair, which may not propagate the arterial pulse normally and can result in pressure gradients with exercise when there is no anatomical constriction.
Nielsen et al. (26) report on cardiac magnetic resonance imaging (CMR) in 31 subjects for assessment of native or recurrent coarctation in terms of severity as assessed at catheterization. The smallest aortic cross-section area adjusted for body surface area for CMR and heart rate corrected mean flow deceleration to descending aorta measured by phase-velocity cine CMR showed a strong correlation with predicted catheter gradient of >20 mm Hg. In a subsequent validation study, the prediction model correctly classified 9 of 10 patients with no false negatives. These functional parameters from CMR can provide much of the data needed to determine whether or not a patient needs intervention without the need for diagnostic catheterization.
Sheikh et al. (27) report on successful conversion of failing Fontan atriopulmonary connections to TCPC with and without arrhythmia surgery in 15 patients. There was no mortality, and average hospitalization was 18 days. At follow-up, atrial arrhythmias have recurred in 3 to 4 patients with intraatrial TCPC without ablation and in 1 of 11 with extracardiac TCPC with ablation. All patients are asymptomatic or have minimal symptoms with exercise ability improved by 69% on a Bruce protocol.
Nurnberg et al. (28) report on new onset arrhythmias after the extracardiac TCPC compared with the intraatrial TCPC for Fontan operation. Seventy-four consecutive patients received either an intra-atrial Fontan operation (29 patients) or extracardiac Fontan operation (45 patients). Median follow-up was 4.4 years; there were five early deaths and one late death (a total mortality of 8%). Sinus rhythm persisted in 86% of extracardiac patients and 50% of intraatrial patients. The extracardiac Fontan operation decreased the incidence of post-op or new onset arrhythmias during early and mid-term follow-up compared with intraatrial Fontan operation, although follow-up was shorter for the extracardiac Fontan operation (4.4 vs. 7.9 years).
Gupta et al. (29) report on risk factors for persistent pleural effusions after the Fontan operation in 100 consecutive patients with a median age 3.1 years. Factors for pleural effusions lasting more than two weeks were lower preoperative O2 saturation and the presence of postoperative infections. Risk factors for effusions draining at more than 20 ml/kg/day were preoperative O2 saturations, smaller conduit size, and longer duration of cardiopulmonary bypass. Although this problem appears to have decreased with staging to a Fontan procedure with a prior bidirectional Glenn as well as fenestration in any patient with high-risk factors for morbidity or mortality, effusions continue to plague some Fontan patients.
Wu and Huang (30) report on a new procedure for Ebstein’s anomaly in 34 consecutive patients operated at a mean of 17 years. Tricuspid regurgitation (TR) was moderate in 12 patients and severe in 22 patients. The repair technique includes detaching the displaced posterior leaflet with chordae tendineae and corresponding papillary muscle and then reattaching to the native posterior annulus and reimplantation of the papillary muscle. All patients survived or recovered uneventfully. Echocardiography showed TR disappeared in 29 patients, and was mild in 5 patients. This procedure needs further follow-up data, and one wonders if it can be produced by other centers. It is unclear what the anatomical and hemodynamic factors are, which makes this an attractive operation for certain Ebstein patients.
Chen et al. (31) report early and mid-term results for 25 patients who underwent repair of Ebstein’s anomaly at an average age of 14 years and average follow-up of 4 years. The technique consisted of vertical applications of the atrialized ventricle and leaflet reimplantation after clockwise rotation. Three required re-operation. Eighteen children (95%) and five adults (83%) demonstrated significant improvement in exercise capacity late postoperatively. Two children died suddenly 11 months and 4 years after repair. It is not absolutely clear when is the best time to intervene in patients with moderate Ebstein’s anomaly with moderate right ventricular dilation. Right ventricular dysfunction certainly can occur with long-standing volume overload, and one wants to avoid getting to this point before operation.
Hraska et al. (32) report on 123 patients with congenitally corrected TGA (CCTGA) and 2 patients with functional ventricles who underwent either a traditional two-ventricle repair or Fontan procedure. Five-, 10-, and 15-year survivals were 75%, 68%, and 61%, respectively. Patients requiring tricuspid valve replacements anytime during follow-up had a significantly worse outcome, whereas the best outcome was seen in patients undergoing the Fontan procedure. Right ventricle end-diastolic pressure >17 before the operation, complete heart block after the operation, subvalvular pulmonary stenosis, Ebstein malformation of the tricuspid valve, and preoperative systemic right ventricle dysfunction were identified as risk factors for death at any time. The long-term outcome of patients with corrected TGA after classical approaches is frequently unsatisfactory. Alternative approaches such as the double-switch, Senning-Rastelli, or Fontan procedure may achieve better long-term results, especially in the high-risk groups.
Knauth et al. (33) report transcatheter device closure of congenital postoperative VSDs in 170 patients using successive generations of the STARFlex-type device (NMT Medical, Inc., Boston, Massachusetts). Between one and seven devices were placed per patient (median, one) with multiple devices placed in 40%. There was a significant decrease in left-to-right shunt after the device implantation and significant improvement in VSD size/severity, and device position proved stable. Of 332 adverse events, 39 were related to the device, and 261 were related to catheterization. At a median follow-up of 24 months, 14 patients had died, and 18 had devices explanted. Congenital postoperative VSD closure using these devices resulted in stable improvement in clinical status and decreased interventricular shunting. Although periprocedural events occurred frequently, late events caused by the device were rare.
Holzer et al. (34) report on a U.S. registry of device closure of congenital muscular VSDs using the Amplatzer VSD occluder (AGA Medical Corp., Golden Valley, Minnesota). Data were prospectively collected from 83 procedures involving 75 patients who underwent an attempt of percutaneous and/or periventricular (surgical) device closure of hemodynamically significant muscular VSDs. The patients’ median age was 1.4 years. The median VSD size was 7 mm, and 34 of 78 patients had multiple VSDs. The device was implanted successfully in 86.7%, and multiple devices were implanted in 20.5%. Procedure-related major complications occurred in 10.7%; device immobilization occurred in two patients, and procedure-related death occurred in two patients. A 24-h postprocedural complete closure rate was 47%, increasing to 70% in 6 months and 92% in 12 months. Six patients underwent successful closure using the periventricular surgical approach, with complete closure at day 1 in three patients and trivial/small residual shunts in the remainder. This device appears to be effective in most patients with congenital muscular VSDs or postoperative VSDs (Fig. 3). This procedure is not for the "faint of heart," and requires the work of an experienced and talented group to perform this intervention.
View larger version (110K):
[in this window]
[in a new window]
|
Figure 3 Cine fluoroscopic images in a seven-month-old female baby with single muscular ventricular septal defect (VSD), demonstrating the steps of percutaneous device closure. (A) Left ventricular (LV) angiogram in the four-chamber view, demonstrating a 6-mm mid-muscular VSD (arrow). (B) Cine fluoroscopic image in the straight frontal projection, demonstrating the guide wire (arrow) across the VSD into the right ventricular (RV) and down the inferior vena cava. The wire was snared from there and exteriorized into the right internal jugular vein. (C) Delivery sheath with an 8-mm Amplatzer device inside (arrow), passing from the right internal jugular vein through the VSD and terminating into the LV. (D) The LV disk is being deployed and pulled against the septum (arrow). (E) Left ventricular angiogram confirms good LV disk position. (F) Cine angiogram after deployment of the connecting waist and RV disk (arrow). (G) Cine fluoroscopic image after the device was released. (H) Final LV angiogram confirming good device position and no residual shunting through the device. Reprinted with permission from Holzer et al. (34).
|
|
Zahn et al. (35) report on interventional catheterization performed early postoperatively to address residual abnormalities in 62 patients with a mean age of four months who underwent 66 catheterizations on median postoperative day 9. Nine patients required extracorporeal cardiopulmonary support. Success rates by procedure were: angioplasty, 100%; stent implantation, 87%; vascular/septal occlusion, 100%; and palliative pulmonary valvotomy, 75%. Complications included stent migration (one patient), cerebral vascular injury (one patient), and left pulmonary artery stenosis (one patient). Transcatheter interventions can be successfully performed in the early postoperative period in selected patients. Difficulty in ballooning or stenting cross suture lines remains a potential hazard of this work. Results can be extremely gratifying in selected patients (Fig. 4).
View larger version (101K):
[in this window]
[in a new window]
|
Figure 4 (A) Right ventricular angiogram performed via a sheath (black asterisk) placed directly into the right ventricular outflow tract (RVOT) via an open sternum in a four-month-old 6 h after RVOT reconstruction and pulmonary arterioplasty. Note the severe proximal left pulmonary artery stenosis (black arrowhead) at the anastomosis with the RVOT patch. (B) After stent placement, there has been complete relief of the stenosis without evidence for vascular disruption. Reprinted with permission from Zahn et al. (35).
|
|
Sideris et al. (36) report total percutaneous correction of tetralogy of Fallot (TOF) variant with dominant pulmonary valve stenosis. Two patients, ages four and seven years, with O2 saturations of 72% and 88% had severe right ventricular outflow obstruction with dominant valvular/pulmonary stenosis, and large malalignment VSDs. Balloon valvuloplasty was first performed, followed by balloon test occlusion of the VSD and double balloon patch occlusion. After 48 h the supporting balloons were extracted releasing the patches. Both patients became acyanotic with O2 saturations of 96% with residual infundibular pulmonary stenosis (gradients 40 to 30 mm Hg). Both patients were doing well at the 6- and 12-month follow-up. This report represents an unusual variant of tetralogy. The VSD patch used requires 48-h balloon support until it is embedded into the septal wall. It is unclear that this type of patch will become the device of choice for percutaneous VSD closure, but this report shows feasibility in these two patients.
Novick et al. (37) report on surgical closure of a VSD in children with elevated pulmonary vascular resistance using a double-patch flap valve procedure. Ninety-one children with a median age of four years underwent VSD closure in a number of institutions worldwide by six different surgeons. Patients had elevated pulmonary vascular resistance at an average of 10.5 Wood units. The overall mortality rate was 7 of 91 or 7.7% with 7 late deaths: 2 in patients with simple VSDs and 5 in patients with complex defects. Although patients with this type of increased pulmonary vascular resistance are rare currently in the U.S., there are many patients worldwide who present with significantly elevated pulmonary vascular resistance. The use of this double-patch technique appears to provide some benefit in terms of "tiding them over" early postoperatively.
Chen et al. (38) report on trends and outcomes in transplantation for complex CHD. There were 106 patients who underwent transplantation and were followed for a median of 56 months. Thirty-seven (35%) of the patients died. Male gender and later year of transplantation were protective, and neonatal age and pulmonary artery reconstruction were detrimental in multivariable modeling of overall mortality. Transplantation to a physiologic or anatomic single lung did not impact survival. These patients had comparable survival estimates when compared with those in the entire cohort without complex CHD.
Wald et al. (39) report on 22 children with isolated ventricular noncompaction, and attempted to determine risk factors for outcome. Echocardiographic features at presentation including increased noncompacted to compacted segment ratio and enlarged left ventricular dimension were found to be associated with poor outcomes including heart transplantation, transplant listing, or death. This diagnosis is being made with increasing frequency, sometimes in asymptomatic or minimally symptomatic patients. Further data are needed on patients with minimal symptomology to determine what is the best management strategy for this complex condition.
Pappone et al. (40) report a randomized study in which prophylactic radiofrequency ablation of accessory pathways was compared with no ablation in asymptomatic children age 5 to 12 years with Wolff-Parkinson-White syndrome who were deemed high-risk by electrophysiological testing. The primary end point was the occurrence of arrhythmic events. Twenty patients underwent prophylactic ablation, 27 had no treatment; characteristics of the two groups were similar. There were three ablation-related complications, one of which lead to hospitalization during follow-up. One child in the ablation group (5%) and 12 in the control group (44%) had arrhythmic events. Two children in the control group had ventricular fibrillation, and one died suddenly. In asymptomatic high-risk children with Wolff-Parkinson-White syndrome, prophylactic catheter ablation performed by an experienced operator may reduce the risk of life-threatening arrhythmias. This represents a major deviation from current practice in most centers.
Strieper et al. (41) report on cardiac resynchronization therapy (CRT) for ventricular dysfunction in seven patients with complex CHD. The prepacing QRS duration ranged from 120 to 280 ms and post-CRT from 80 to 180 ms. Ejection fraction improved from a mean 16% to 36%, and left ventricular dimension decreased from 5.9 to 4.8 cm. This study demonstrates that there appears to be a role for CRT in young patients with dilated cardiac myopathy, congestive heart failure, and congenital heart defects.
Janousek et al. (42) report on CRT in eight patients with a systemic right ventricle and right bundle branch block with QRS averaging 161 ms; CRT resulted in QRS decreasing by 28%, filling time improving by 11%, right ventricle maximum dP/dt increasing by 46%, aortic velocity time interval increasing by 7%, and right ventricle ejection fraction increasing by 10%. Right ventricle fractional area change increased from of 18% to 29.5% at last follow-up without a change to the end-diastolic area. Cardiac resynchronization therapy appears to be useful as a promising adjunct to the treatment and prevention of systemic right ventricular failure in patients with a systemic right ventricle.
Bacha et al. (43) report on cardiac CRT in 26 single-ventricle patients with a mean age of 28 months. Three-dimensional data were analyzed to obtain regional volume-time curves for 16 three-dimensional segments of the systemic ventricle. Multisite pacing performed on median postoperative day 2 resulted in QRS duration decreasing from 94 to 72 ms, systolic blood pressure increasing from 86 to 94 mm Hg, cardiac index increasing from 3.2 to 3.7, and the index of asynchrony improving in 8 of 10 patients; CRT may improve ventricular performance after single-ventricle palliation.
|
Adult CHD
|
|---|
Veldtman et al. (44) report on outcomes of pregnancy in 43 women with TOF who had 112 pregnancies. Eight women had unrepaired TOF. At first assessment, 6 patients had pulmonary hypertension, 3 had moderate or severe right ventricular systolic dysfunction, and 13 had severe right ventricular dilation due to pulmonary regurgitation. Sixteen patients had 30 miscarriages and 1 term stillbirth. Mean overall birth weight was 3.2 kg. Unrepaired TOF and morphologic pulmonary artery abnormality were independently predictive of infant birth weight. Six patients had cardiovascular complications: supraventricular tachycardia (SVT) in two, heart failure in two, pulmonary embolism in one, and progressive right ventricular dilation in one. Five infants (6%) had congenital anomalies. Patients with TOF have an increased risk of fetal loss, and offspring are more likely to have congenital anomalies than the general population. Adverse maternal events, although rare, may be associated with left ventricular dysfunction, severe pulmonary hypertension, and severe pulmonic regurgitation with right ventricular dysfunction. Most patients with TOF who have a reasonably good repair with normal or mildly decreased right ventricular function, and mild-to-moderate pulmonary regurgitation and/or pulmonary stenosis, tolerate pregnancy well. This report indicates the difficulties that can occur in patients with more significant abnormalities.
van Straten et al. (45) report on CMR at 7 months, and 19 months after pulmonary valve replacement in 25 consecutive patients with TOF. There was a marked decrease in right ventricular volume from 167 ml/m2 to 114 ml/m2 at 7 months and to 112 ml/m2 at 19 months follow-up. Ejection fraction remained the same and was 43% preoperative and 47% at 19-month follow-up (p = 0.1). Results are encouraging for early valve replacement in patients with right ventricular dilation and severe pulmonary regurgitation; CMR appears to be the most useful way for assessment of right ventricular size and function as well as pulmonary artery anatomy both before and after valve replacement.
Guedes et al. (46) report on the impact of pregnancy on the systemic right ventricle of 16 women who completed 28 pregnancies after an atrial repair for TGA. Twenty-one of the women were asymptomatic, and two had mild symptoms before pregnancy. Functional class deteriorated in six, with no return to the prepregnancy level after delivery in two. Right ventricular dilation progressed in five women, with no recovery in all patients at the last follow-up. Right ventricular systolic dysfunction was absent in 16, mild-to-moderate in 4, or severe in 1 before pregnancy and progressed in 4 women with no recovery in three cases. The TR was absent in eight, mild in nine, and moderate in three before pregnancy and deteriorated in eight women with no recovery in three patients. Pregnancy after atrial repair of TGA is clinically well tolerated in most of these patients who have no symptoms before pregnancy, but carries a risk of deteriorating right ventricular function in patients with mild-to-moderate right ventricular dilation and dysfunction before pregnancy.
Roos-Hesselink et al. (47) report a decline in ventricular function and clinical condition after atrial repair for TGA in 91 consecutive patients operated on between 1973 and 1980. While all patients had good right ventricular function 14 years after repair, 61% of patients showed moderate-to-severe dysfunction after 25 years when studied by echocardiography. In addition, the QRS complex widened, and exercise capacity decreased.
Kammeraad et al. (48) report on 47 patients after atrial repair of TGA who experienced a sudden death event with 34 being sudden death and 13 being near-miss sudden death. Each patient was matched with two controls with the same operation without a sudden death event. Presence of symptoms of arrhythmia, or heart failure at most recent follow-up, or history of documented atrial flutter or atrial fibrillation was found to increase the risk of sudden death. Electrocardiogram (ECG), chest X-ray, and Holter ECG findings were not predictive of sudden death. Neither medication nor pacing was found to be protective; most sudden death events occurred during exercise. Recorded ventricular tachycardia/ventricular fibrillation were the recorded rhythm during sudden death in 21 of 47 patients. These authors suggest electrophysiologic study for inducible ventricular tachycardia/ventricular fibrillation in patients presenting with symptoms or with documented atrial flutter/atrial fibrillation. If ventricular tachycardia/ventricular fibrillation is induced using moderately aggressive pacing protocols, implantable cardioverter-defibrillator should be considered.
van den Bosch et al. (49) report on the long-term outcomes and quality of life in 36 adult patients after the Fontan operation. Of the initial 36 patients, 10 died at a mean of age 10 years after the Fontan operation, and 1 patient underwent cardiac transplantation. Reoperations were performed in 21 patients. Sustained SVT was observed in 20 patients with an increase incidence of arrhythmias with longer follow-up. Thromboembolic events were detected in nine patients, five of who had adequate anticoagulant levels at the time of the event. A total of 195 hospital admissions were recorded, and quality of life assessment showed physical functioning, mental health, and general health perception to be significantly lower for Fontan patients than for the normal Dutch population.
Varma et al. (50) report on silent emboli in adults after the Fontan operation. Thirty consecutive adult Fontan patients were evaluated using ventilation-perfusion scanning and blood testing for thrombophilia tendency. If the ventilation-perfusion (VQ) scan showed an intermediate or high probability for pulmonary emboli, a computerized tomographic pulmonary angiogram was performed. Thirty patients were included in the study, and five (17%) had an intermediate or high probability for pulmonary emboli in VQ scans, all of which were confirmed by computed tomography pulmonary angiography. No patient had a thrombophilia tendency. Pulmonary emboli were not present in any patient taking warfarin. Late age at time of Fontan operation and type of Fontan anatomy were associated with increased risk of silent pulmonary emboli (Fig. 5).
View larger version (122K):
[in this window]
[in a new window]
|
Figure 5 Left anterior oblique projections of the ventilation (A) and perfusion (B) images illustrate a perfusion defect in the left upper lobe (thin arrow, B), in Patient 20. This is indicative of a high probability for pulmonary embolus. Coronal (C) and sagittal oblique (D) maximum intensity projections from the computerized tomography pulmonary angiogram are shown. In the coronal projection (C), gradation of vessel contrast from the lower lobe to the upper lobe vessels is seen, and relative paucity of contrast within the upper lobe pulmonary arteries on the left compared to the right is shown near the asterisk. Endoluminal filling defect, in keeping with thrombus, is shown within a segmental upper lobe pulmonary artery (dashed arrow, C and D). Incidental nonocclusive thrombus is also seen in this patient in a lower lobe pulmonary artery (thick arrow, C). Reprinted with permission from Varma et al. (50).
|
|
Oliver et al. (51) report on 235 adults with coarctation who were retrospectively reviewed. Treatment by surgery had been performed in 181 patients or by balloon angioplasty or stenting in 28 patients. There were no previous interventions in 26 patients with mild coarctation. Aortic wall complications were present in 37 patients (16%) with no difference in the three groups with respect to total complications. Only aging and BAV were significantly related to these complications (Fig. 6). It is increasingly apparent that coarctation, particularly when associated with BAV, is a generalized arteriopathy. These patients need long-term follow-up, and CMR is an excellent way of screening for abnormalities along their entire aorta.
View larger version (163K):
[in this window]
[in a new window]
|
Figure 6 (A) Transesophageal echocardiography showing aortic bicuspid valve and right sinus of Valsalva aneurysm ruptured into the right atrium late after balloon dilation of coarctation of the aorta. (B) Magnetic resonance image (MRI) of an ascending aorta aneurysm in a patient with unrepaired mild coarctation of the aorta. (C) This MRI shows a descending aorta giant false aneurysm in another patient with unrepaired mild coarctation. (D) This MRI shows an abnormal bulge of aorta (aneurysm) and a false aneurysm (pseudoaneurysm) late after patch aortoplasty; aortobronchial fistula, causing massive hemoptysis, was also demonstrated (arrow). An = true aneurysm; PsA = false aneurysm. Reprinted with permission from Oliver et al. (51).
|
|
Beauchesne et al. (52) report on the prevalence of clinical manifestations of 22 q11.2 microdeletion in adults with selected conotruncal anomalies. There were 103 consecutive adults who were prospectively screened for this microdeletion, and clinicians were asked to predict microdeletion status on the basis of clinical features. Six patients (5.8%) had microdeletions and, in two of these, clinicians incorrectly predicted absence of the deletion. In three, typical dysmorphic features were absent (Fig. 7). Screening for microdeletion should be considered in adults with high-risk cardiac lesions because of its importance in reproductive counseling and surveillance for associated extracardiac manifestations.
View larger version (75K):
[in this window]
[in a new window]
|
Figure 7 Facial views of two of the patients with 22q11.2 microdeletion illustrating the spectrum of facial dysmorphism. (A) Except for mild upper eyelid changes associated with normal aging, this 47-year-old man does not have typical dysmorphic changes of 22q11.2 microdeletion. (B) This 27-year-old woman has facial features of 22q11.2 microdeletion including a long face, paranasal bossing, and hooded appearance of the upper eyelids with narrow palpebral fissures. Reprinted with permission from Beauchesne et al. (52).
|
|
Salehian et al. (53) report on improvements in cardiac form and function after transcatheter closure of secundum ASDs in 25 patients with an average age of 45 years. There was a statistically significant improvement in right ventricular myocardial performance index and left atrial volume index after closure of ASD. In addition, there was an increase in left ventricular size and no change in ejection fraction after closure.
Silversides et al. (54) report on symptomatic atrial arrhythmias in 132 consecutive patients who underwent transcatheter ASD closure. Those without a history of arrhythmia had a low incidence of atrial tachycardia during follow-up (6%) and intermediate follow-up (1% per year), while all patients with persistent atrial tachycardia before closure remained in atrial flutter/atrial fibrillation. Of patients in sinus rhythm but with a previous history of atrial tachycardia, two-thirds remained arrhythmia-free at follow-up, with overall incidences of paroxysmal or persistent atrial tachycardia of 17% and 11% a year, respectively. Device closure of an ASD before the onset of atrial arrhythmias may protect against the subsequent development of arrhythmia, particularly in patients <55 years of age.
Azarbal et al. (55) report on 86 adult patients who underwent transcatheter closure of an interatrial communication. Migraine headaches were present in 42% of patients before the procedure, and, three months after the procedure, the migraine headaches disappeared completely in 75% of patients with migraine and aura and in 31% with migraine without aura. Of the remaining patients, 40% had significant improvement of migraine headaches.
Reisman et al. (56) report on 160 consecutive patients with paradoxical cerebral embolism who underwent transcatheter patent foramen ovale closure for prevention of recurrent cryptogenic stroke or transient ischemic attacks. A one-year retrospective analysis of migraine symptoms was performed. Preclosure, active migraine was present in 35% of patients, and 68% experienced migranious aura; 50 patients were available for analysis at one year. Complete resolution of migraine symptoms occurred in 56% of patients, and 14% of patients reported significant reduction of migraine frequency. Patients reported an 80% reduction in the mean number of migraine episodes per month after patent foramen ovale closure. These two studies suggest that intraatrial communications may play a role in the etiology of migraine headaches either through paradoxical embolism or from blood-borne factors bypassing the pulmonary circulation. These studies suggest a strong rationale for further investigation of the pathophysiology and treatment of migraine. Because both migraine and patent foramen ovale have a high frequency in the general population, further study of appropriately matched patients with migraine with and without patent foramen ovale closure will be needed to determine whether or not this is a causative relationship.
|
Summary
|
|---|
This review emphasizes aggressive management strategies in fetuses, infants, and children with CHD. There is an as ongoing need for long-term follow-up, especially in adults with CHD, many of whom are not receiving continuity of care. An expanded work force will need to be trained to take care of this rapidly expanding population.
View larger version (93K):
[in this window]
[in a new window]
|
Figure 2 Stent stenosis. (a) In-stent stenosis: layer of neointimal proliferation between white arrows; (b) stenosis of unstented segment of a long vertical ductus arising from left subclavian artery; (c) an additional stent was implanted over the stenosed and unstented segment of the ductus. Reprinted with permission from Alwi et al. (10).
|
|
|
References
|
|---|
1. Tworetzky W, Wilkins-Haug L, Jennings RW, et al. Balloon dilation of severe aortic stenosis in the fetus. Potential for prevention of hypoplastic left heart syndrome candidate selection, technique, and results of successful intervention Circulation 2004;110:2125-2131.[Abstract/Free Full Text]
2. Marshall AC, van der Velse ME, Tworetzky W, et al. Creation of an atrial septal defect in utero for fetuses with hypoplastic left heart syndrome and intact or highly restrictive atrial septum Circulation 2004;110:253-258.[Abstract/Free Full Text]
3. Rychik J, Tian Z, Cohen MS, et al. Acute cardiovascular effects of fetal surgery in the human Circulation 2004;110:1549-1556.[Abstract/Free Full Text]
4. Jaeggi ET, Fouron J-C, Silverman ED, et al. Transplacental fetal treatment improves the outcome of prenatally diagnosed complete atrioventricular block without structural heart disease Circulation 2004;110:1542-1548.[Abstract/Free Full Text]
5. Vlahos AP, Lock JE, McElhinney DB, et al. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum Circulation 2004;109:2326-2330.[Abstract/Free Full Text]
6. Sano S, Ishino K, Kado H, et al. Outcome of right ventricle-to-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndromea multi-institutional study. Ann Thorac Surg 2004;78:1951-1958.[Abstract/Free Full Text]
7. Tanoue Y, Kado H, Shiokawa Y, et al. Midterm ventricular performance after Norwood procedure with right ventricular-pulmonary artery conduit Ann Thorac Surg 2004;78:1965-1971.[Abstract/Free Full Text]
8. Fenton KN, Pigula FA, Gandhi SK, et al. Interim mortality in pulmonary atresia with intact ventricular septum Ann Thorac Surg 2004;78:1994-1998.[Abstract/Free Full Text]
9. Sittiwankul R, Azakie A, Van Arsdell GS, et al. Outcomes of tricuspid atresia in the Fontan era Ann Thorac Surg 2004;77:889-894.[Abstract/Free Full Text]
10. Alwi M, Choo KK, Latiff HA, et al. Initial results and medium-term follow-up of stent implantation of patent ductus arteriosus in duct-dependent pulmonary circulation J Am Coll Cardiol 2004;44:438-445.[Abstract/Free Full Text]
11. Batra AS, Starnes VA, Wells WJ, et al. Does the site of insertion of a systemic-pulmonary shunt influence growth of the pulmonary arteries? Ann Thorac Surg 2005;79:636-640.[Abstract/Free Full Text]
12. Dibardino DJ, Allison AE, Vaughn WK, et al. Current expectations for newborns undergoing the arterial switch operation Ann Surg 2004;239:588-598.[CrossRef][Web of Science][Medline]
13. McMahon CJ, Ravekes WJ, O’Brian Smith E, et al. Risk factors for neo-aortic root enlargement and aortic regurgitation following arterial switch operation Pediatr Cardiol 2004;25:329-335.[Web of Science][Medline]
14. Mohammadi S, Serraf A, Belli E, et al. Left-sided lesions after anatomic repair of transposition of the great arteries, ventricular septal defect, and coarctationsurgical factors. J Thorac Cardiovasc Surg 2004;128:44-52.[Abstract/Free Full Text]
15. Burch M, Kaufman L, Archer N, et al. Persistent pulmonary hypertension late after neonatal aortic valvotomya consequence of an expanded surgical cohort. Heart 2004;90:918-920.[Abstract/Free Full Text]
16. Vandekerckhove K, Macrae D, Slavik Z. Nonsurgical treatment of patent arterial duct in term neonates with congenital heart disease: the role of intravenous indomethacin. Available at: http://www.springerlink.com/app/home/contribution.asp?wasp=66a7505c1dd74b3c800e12e2b62f8999&referrer=parent&backto=issue,14,59;journal,1,56;browsepublicationsresults,1882,2439;. Accessed April 20, 2005..
17. Basso C, Boschello M, Perrone C, et al. An echocardiographic survey of primary school children for bicuspid aortic valve Am J Cardiol 2004;93:661-663.[CrossRef][Web of Science][Medline]
18. Fernandes SM, Sanders SP, Khairy P, et al. Morphology of bicuspid aortic valve in children and adolescents J Am Coll Cardiol 2004;44:1648-1651.[Abstract/Free Full Text]
19. Gurvitz M, Chang R-K, Drant S, et al. Frequency of aortic root dilation in children with a bicuspid aortic valve Am J Cardiol 2004;94:1337-1340.[CrossRef][Web of Science][Medline]
20. Cripe L, Andelfinger G, Martin LJ, et al. Bicuspid aortic valve is heritable J Am Coll Cardiol 2004;44:138-143.[Abstract/Free Full Text]
21. Cecconi M, Manfrin M, Moraca A, et al. Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction Am J Cardiol 2005;95:292-294.[CrossRef][Web of Science][Medline]
22. Cattaneo SM, Bethea BT, Alejo DE, et al. Surgery for aortic root aneurysm in childrena 21-year experience in 50 patients. Ann Thorac Surg 2004;77:168-176.[Abstract/Free Full Text]
23. McMahon CJ, Gauvreau K, Edwards JC, et al. Risk factors for aortic valve dysfunction in children with discrete subvalvar aortic stenosis Am J Cardiol 2004;94:459-464.[CrossRef][Web of Science][Medline]
24. Puchalski MD, Williams RV, Hawkins JA, et al. Follow-up of aortic coarctation repair in neonates J Am Coll Cardiol 2004;44:188-191.[Abstract/Free Full Text]
25. Ou P, Bonnet D, Auriacombe L, et al. Late systemic hypertension and aortic arch geometry after successful repair of coarctation of the aorta Eur Heart J 2004;25:1853-1859.[Abstract/Free Full Text]
26. Ma Nielsen JC, Powell AJ, Gauvreau K, et al. Magnetic resonance imaging predictors of coarctation severity Circulation 2005;111:622-628.[Abstract/Free Full Text]
27. Shiekh AM, Tang ATM, Roman K, et al. The failing Fontan circulationsuccessful conversion of atriopulmonary connections. J Thorac Cardiovasc Surg 2004;128:60-66.[Abstract/Free Full Text]
28. Nurnberg JH, Ovroutski S, Alexi-Meskishvili V, et al. New onset arrhythmias after the extracardiac conduit Fontan operation compared with the intraatrial lateral tunnel procedureearly and midterm results. Ann Thorac Surg 2004;78:1979-1988.[Abstract/Free Full Text]
29. Gupta A, Daggett C, Behera S, et al. Risk factors for persistent pleural effusions after the extracardiac Fontan procedure J Thorac Cardiovasc Surg 2004;127:1664-1669.[Abstract/Free Full Text]
30. Wu Q, Huang Z. A new procedure for Ebstein’s anomaly Ann Thorac Surg 2004;77:470-476.[Abstract/Free Full Text]
31. Chen JM, Mosca RS, Altmann K, et al. Early and midterm results for repair of Ebstein J Thorac Cardiovasc Surg 2004;127:990-999.[Abstract/Free Full Text]
32. Hraska V, Duncan BW, Mayer Jr. JE, et al. Long-term outcome of surgically treated patients with corrected transposition of the great arteries J Thorac Cardiovasc Surg 2005;129:182-191.[Abstract/Free Full Text]
33. Knauth AL, Lock JE, Perry SB, et al. Transcatheter device closure of congenital and postoperative residual ventricular septal defects Circulation 2004;110:501-507.[Abstract/Free Full Text]
34. Holzer R, Balzer D, Cao Q-L, et al. Device closure of muscular ventricular septal defects using the Amplatzer muscular ventricular septal defect occluder J Am Coll Cardiol 2004;43:1257-1263.[Abstract/Free Full Text]
35. Zahn EM, Dobrolet NC, Nykanen DG, et al. Interventional catheterization performed in the early postoperative period after congenital heart surgery in children J Am Coll Cardiol 2004;43:1264-1269.[Abstract/Free Full Text]
36. Sideris EB, Macuil B, Justiniano S, et al. Total percutaneous correction of a tetralogy of Fallot variant with dominant pulmonary valve stenosis Heart 2005;91:345-347.[Abstract/Free Full Text]
37. Novick WM, Sandoval N, Lazorhysynets VV, et al. Flap valve double patch closure of ventricular septal defects in children with increased pulmonary vascular resistance Ann Thorac Surg 2005;79:21-28.[Abstract/Free Full Text]
38. Chen JM, Davies RR, Mital SR, et al. Trends and outcomes in transplantation for complex congenital heart disease1984–2004. Ann Thorac Surg 2004;78:1352-1361.[Abstract/Free Full Text]
39. Wald R, Veldtman G, Golding F, et al. Determinants of outcome in isolated ventricular noncompaction in childhood Am J Cardiol 2004;94:1581-1584.[CrossRef][Web of Science][Medline]
40. Pappone C, Manguso F, Santinelli R, et al. Radiofrequency ablation in children with asymptomatic Wolff-Parkinson-White syndrome N Engl J Med 2004;351:1197-1205.[Abstract/Free Full Text]
41. Strieper M, Karpawich P, Frias P, et al. Initial experience with cardiac resynchronization therapy for ventricular dysfunction in young patients with surgically operated congenital heart disease Am J Cardiol 2004;94:1352-1354.[CrossRef][Web of Science][Medline]
42. Janousek J, Tomek V, Chaloupecky V, et al. Cardiac resynchronization therapya novel adjunct to the treatment and prevention of systemic right ventricle failure. J Am Coll Cardiol 2004;44:1927-1931.[Abstract/Free Full Text]
43. Bacha EA, Zimmerman FJ, Mor-Avi V, et al. Ventricular resynchronization by multisite pacing improves myocardial performance in the postoperative single-ventricle patient Ann Thorac Surg 2004;78:1678-1683.[Abstract/Free Full Text]
44. Veldtman GR, Connolly HM, Grogan M, et al. Outcomes of pregnancy in women with tetralogy of Fallot J Am Coll Cardiol 2004;44:174-180.[Abstract/Free Full Text]
45. van Straten A, Vliegen HW, Hazekamp MG, et al. Right ventricular function after pulmonary valve replacement in patients with tetralogy of Fallot Radiology 2004;233:824-829.[Abstract/Free Full Text]
46. Guedes A, Mercier L-A, Leduc L, et al. Impact of pregnancy on the systemic right ventricle after a Mustard operation for transposition of the great arteries J Am Coll Cardiol 2004;44:433-437.[Abstract/Free Full Text]
47. Roos-Hesselink JW, Meijboom FJ, Spitaels SEC, et al. Decline in ventricular function and clinical condition after mustard repair for transposition of the great arteries (a prospective study of 22 to 29 years) Eur Heart J 2004;25:1264-1270.[Abstract/Free Full Text]
48. Kammeraad JAE, van Deurzen CHM, Sreeram N, et al. Death after Mustard or Senning repair for transposition of the great arteries J Am Coll Cardiol 2004;44:1095-1102.[Abstract/Free Full Text]
49. van den Bosch AE, Roos-Hesselink Jolien W, van Domburg R, et al. Long-term outcome and quality of life in adult patients after the Fontan operation Am J Cardiol 2004;93:1141-1145.[CrossRef][Web of Science][Medline]
50. Varma C, Warr MR, Hendler AL, et al. Prevalence of "silent" pulmonary emboli in adults after the Fontan operation J Am Coll Cardiol 2003;41:2252-2258.[Abstract/Free Full Text]
51. Oliver JM, Gallego P, Gonzalez A, et al. Risk factors for aortic complications in adults with coarctation of the aorta J Am Coll Cardiol 2004;44:1641-1647.[Abstract/Free Full Text]
52. Beauchesne LM, Warnes CA, Connolly HM, et al. Prevalence and clinical manifestations of 22q11.2 microdeletion in adults with selected conotruncal anomalies J Am Coll Cardiol 2005;45:595-598.[Abstract/Free Full Text]
53. Salehian O, Horlick E, Schwerzmann M, et al. Improvements in cardiac form and function after transcatheter closure of secundum atrial septal defects J Am Coll Cardiol 2005;45:499-504.[Abstract/Free Full Text]
54. Silversides CK, Siu SC, McLaughlin PR, et al. Symptomatic atrial arrhythmias and transcatheter closure of atrial septal defects in adult patients Heart 2004;90:1194-1198.[Abstract/Free Full Text]
55. Azarbal B, Tobis J, Suh W, et al. Association of interatrial shunts and migraine headaches J Am Coll Cardiol 2005;45:489-492.[Abstract/Free Full Text]
56. Reisman M, Christofferson RD, Jesurum J, et al. Migraine headache relief after transcatheter closure of patent foramen ovale J Am Coll Cardiol 2005;45:493-495.[Abstract/Free Full Text]
|
Top
Fetal cardiology
Neonatal and infant cardiology