Transcatheter management of residual shunts after initial transcatheter closure of a patent arterial duct.

OBJECTIVES: To assess the efficacy and safety of transcatheter reocclusion of persistent leaks following previously attempted transcatheter occlusion of persistent arterial duct. DESIGN: Retrospective study. SETTING: Tertiary pediatric cardiology centre. PATIENTS: From February 1987 through October 1996, trans-catheter occlusion of a residual ductal shunt was attempted in 42 consecutive patients at a median age of 5.0 years (range 1.6 years to 16.2 years). INTERVENTIONS: Fourty patients had successful placement of a double umbrella occluder (n=27) or coils (n=13) across residual shunts. Complications included device embolization in two patients and hemolysis in one patient. OUTCOME MEASURES AND RESULTS: Mean z-score for left ventricular end-diastolic dimension (LVEDD) at initial echocardiography was +2.55 +/- 1.89 (P<0.0001 versus normal); z-score for left pulmonary artery (LPA) diameter was +2.00 +/- 1.52 (P<0.0001). Mean LPA to right pulmonary artery (RPA) diameter ratio was 1.05 +/- 0.18. At follow-up echocardiogram, a median of two years (range six months to 7.7 years) after the second procedure, a shunt was persistent in 3% of the patients. Mean LVEDD and LPA diameter z-value, and mean LPA to RPA diameter had dropped significantly to +0.42 +/- 1.31, +0.07 +/- 1.15 and 0.86 +/- 0.14 (P<0.001), respectively. LPA flow acceleration was present in 25% of patients. Three of nine patients, in whom lung perfusion scan was performed, had left lung perfusion below 40%. Small weight and age at catheterization were significant risk factors for LPA flow disturbance. CONCLUSIONS: Repeat transcatheter occlusion is safe and successful in eliminating residual shunt across the arterial duct. Attention should, however, be addressed to the potential for LPA stenosis and growth, and flow should be regularly assessed.

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

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

Aneurysm of the membranous septum in critical pulmonary stenosis: spontaneous rupture after balloon dilatation.

We report a case of critical pulmonary valve stenosis in which congenital aneurysm of the membranous septum ruptured spontaneously after balloon dilatation of the pulmonary valve. It is considered that the chronic mechanical trauma with phasic protrusion and collapse of the aneurysm during the cardiac cycles was responsible for an aneurysm rupture.

Total cavopulmonary connections in children with a previous Norwood procedure.

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

Comparison of two transcatheter closure methods of persistently patent arterial duct.

A randomized trial of arterial duct occlusion with a double umbrella (DU) or wire coil (WC) was undertaken for patients <18 years of age, weighing >10 kg with isolated ducts < or = 3 mm in diameter. Baseline, procedural, and outcome characteristics were compared in an intention-to-treat analysis according to randomization group. From 40 consecutively screened patients, 2 were not enrolled due to a ductal diameter of >3 mm on initial aortography, 38 patients were randomized to either the DU (n = 20) or WC (n = 18) groups. The groups did not differ significantly with respect to age, weight, gender, duct size, type, or branch pulmonary artery diameters. Crossover occurred only in the DU group, where 4 patients (20%) had a ductal diameter of < or = 1 mm and could not be entered for umbrella placement. All remaining DU group patients had ductal diameters of > or = 1.3 mm (p <0.0001). There were no embolizations or secondary implants in the DU group, but in the WC group there was 1 early and 1 late embolization, with 6 patients (33%) with > or = 2 coils. Mean times for the procedure (DU 68+/-19 minutes; WC 65+/-27 minutes; p = 0.70) and fluoroscopy (DU 14+/-4 minutes; WC 11+/-6 minutes; p = 0.22) did not differ significantly. Angiographic duct closure was documented in 4 of 13 patients (31%) of the DU group and 4 of 18 patients (22%) of the WC group (p = 0.69). Combined with an echocardiogram, closure in 11 of 17 patients with DU (65%) and 13 of 18 patients with WC (72%) (p = 0.64) was documented before hospital discharge. One WC group patient received thrombolytic therapy for a femoral artery thrombus. Follow-up at a median of 6.5 months (range 3.2 to 37) showed closure by Doppler echocardiography in 15 of 19 patients with DU (79%) versus 14 of 18 patients with WC (78%) (p = 1.0). Thus, with a tendency toward similar procedural characteristics and outcomes, the higher cost of the DU system compared with coil implants favors the use of coils for closure of the small arterial duct.

Independent factors associated with longevity of prosthetic pulmonary valves and valved conduits.

OBJECTIVE: To evaluate the age dependence of variables predictive of pulmonary valve prosthesis replacement, we conducted the following analysis. METHODS: Retrospective analysis of 945 operations in 726 patients undergoing placement of pulmonary valve prostheses was performed. Age was identified as a strong independent predictor of valve failure. The database was stratified into age-based subsets and predictors of valve replacement were identified within each subset. RESULTS: For the entire cohort, freedom from valve replacement at 5 years was 81%. Younger age was strongly associated with decreased time to valve replacement by multivariable analysis (hazard ratio: 0.71/log-year, P <.001). Other independent factors included diagnosis, type of prosthesis, and time-dependent requirement for pulmonary valve stent placement. Important predictors of valve failure varied among age groups and are as follows: for Age Less Than 3 Months: valve type; for Age 3 Months To Less Than 2 Years: smaller normalized valve prosthesis size; for Age 2 Years To Less Than 13 Years: sex, smaller normalized valve prosthesis size, placement of endovascular stents, and valve type; for Age 13 Years To 65 Years: smaller normalized valve prosthesis size, placement of endovascular stents, and increased number of previous valve placements. CONCLUSION: Age is a dominant risk factor predictive of pulmonary valve prosthesis failure. A significant interaction exists between age and the effects of diagnosis, valve type, and size on prosthetic pulmonary valve longevity.

What is the optimal age for repair of tetralogy of Fallot?

BACKGROUND: Controversy regarding the timing for the repair of tetralogy of Fallot centers around initial palliation versus primary repair for the symptomatic neonate/young infant and the optimal age for repair of the asymptomatic child. We changed our approach from one of initial palliation in the infant to one of primary repair around the age of 6 months, or earlier if clinically indicated. We examined the effects of this change in protocol and age on outcomes. METHODS AND RESULTS: The records of 227 consecutive children who had repair of isolated tetralogy of Fallot from January 1993 to June 1998 were reviewed. The median age of repair by year fell from 17 to 8 months (P:<0.01). The presence of a palliative shunt at the time of repair decreased from 38% to 0% (P:<0.01). Mortality (6 deaths, 2. 6%) improved with time (P:=0.02), with no mortality since the change in protocol (late 1995/early 1996). Multivariate analysis for physiological outcomes of time to lactate clearance, ventilation hours, and length of stay, but not death, demonstrated that an age <3 months was independently associated with prolongation of times (P:<0.03). Each of the deaths occurred with primary repair at an age >12 months. The best survival and physiological outcomes were achieved with primary repair in children aged 3 to 11 months. CONCLUSIONS: On the basis of mortality and physiological outcomes, the optimal age for elective repair of tetralogy of Fallot is 3 to 11 months of age.

Angiographic findings of persistent primitive hepatic venous plexus with underdevelopment of the infrahepatic inferior vena cava in pediatric patients.

OBJECTIVE: We describe the angiographic diagnosis and significance of persistence of the primitive hepatic venous plexus with underdevelopment of the infrahepatic inferior vena cava. CONCLUSION: We recommend that inferior venacavography be performed in routine assessment before surgery of patients with azygos or hemiazygos continuation of the inferior vena cava, in whom redirection of systemic venous blood to the pulmonary artery is contemplated.

Outcomes of left atrial isomerism over a 28-year period at a single institution.

OBJECTIVES: We determined long-term outcomes in a large cohort with left atrial isomerism (LAI). BACKGROUND: Left atrial isomerism is associated with a complex spectrum of cardiac and noncardiac anomalies that may impact on outcomes. METHODS: The records of all patients with LAI, born between 1970 and 1998, and treated at one center were reviewed. Kaplan-Meier survival was estimated, and independent factors associated with time-related death were identified. RESULTS: There were 163 patients (63% women), and extracardiac anomalies were noted in 36%, including biliary atresia in 10%. Cardiac defects included interrupted inferior caval vein in 92%, anomalous pulmonary veins in 56%, atrioventricular septal defect in 49%, pulmonary atresia or stenosis in 28% and aortic coarctation in 16%, with congenital atrioventricular block in 7%. Of 22 patients with a normal heart, 18% died of extracardiac anomalies. Of 71 patients with hearts suitable for biventricular repair, 62 (87%) had surgery, with survival of 80% at one year, 71% at five years, 66% at 10 years and 63% after 15 years. Of 70 patients with unbalanced cardiac defects suitable for single-ventricle palliation, 47 (67%) had surgery, with survival of 73% at one year, 61% at five years, 53% at 10 years and 48% at 15 years (p < 0.001). Independent factors associated with time-related death included congenital atrioventricular block, aortic coarctation, single ventricle, biliary atresia and other gastrointestinal malformations. CONCLUSIONS: Both cardiac and noncardiac anomalies contribute to a high mortality with LAI. Cardiac transplantation may need to be a considered a primary option for selected high-risk patients.

Outcomes for children with cardiomyopathy awaiting transplantation.

OBJECTIVE: To determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies. BACKGROUND: Childhood cardiomyopathies form a heterogeneous group of diseases, and in many, the prognosis is poor, irrespective of the etiology. When profound heart failure develops, cardiac transplantation can be the only viable option for survival. METHODS: We included all children with cardiomyopathy listed for transplantation between 12/89 and 4/98 in this historical cohort study. RESULTS: We listed 31 patients, 15 male and 16 female, 27 with dilated and 4 with restrictive cardiomyopathy, for transplantation. The median age at listing was 5.7 years, with a range from fetal life to 17.8 years. Transplantation was achieved in 23 (74%), with a median interval from listing of 54 days, and a range from zero to 11.4 years. Of the patients, 14 were transplanted within 30 days of listing. Five patients (16%) died before transplantation. Within the Canadian algorithm, one of these was in the third state, and four in the fourth state. One patient was removed from the list after 12 days, having recovered from myocarditis, and two remain waiting transplantation after intervals of 121 and 476 days, respectively. Patients who died were more likely to be female (5/5 vs. 11/26; p=0.04) and to have been in the third or fourth states at listing (5/5 vs. 15/26; p=0.04). The use of mechanical ventricular assistance, in 10 patients, was not a predictor of an adverse outcome. While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzyme, less mitral regurgitation, a higher mean ejection fraction and cardiac index, and lower right ventricular systolic pressure. CONCLUSIONS: Children with cardiomyopathy awaiting transplantation have a mortality of 16% related to their clinical state at the time of listing.

Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? A 5th aortic arch with a systemic-to-pulmonary arterial connection.

Major abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.

Repair of isolated multiple muscular ventricular septal defects: the septal obliteration technique.

BACKGROUND: Isolated multiple ventricular septal defects (mVSDs) remain a surgical challenge. The dilemma of whether to perform a complete repair ultimately rests with the surgeon, who must decide if all significant septal defects can be located. Avoidance of a pulmonary arterial band (as part of a two-stage repair) will negate the need for future pulmonary arterial reconstruction and will reduce the incidence of late right ventricular diastolic dysfunction. METHODS: We performed a retrospective analysis of hospital and echocardiographic data of eight children who underwent a septal obliteration technique (SOT) as part of their correction of mVSDs (with and without coarctation of the aorta). RESULTS: Eight children with a mean age of 10.5 months (range 1.5 to 36 months), and weight of 6.2 kg (range 2.1 to 13.5 kg), respectively, underwent correction of mVSDs. All had a single, large, perimembranous defect, additional VSDs within the muscular trabecular septum (juxtaposed to the moderator band), and apical mVSDs. All VSDs were repaired via the right atrium, with avoidance of either a right or left ventriculotomy. The posterior and apical defects were excluded from the right ventricular cavity with a pericardial patch (SOT). The follow-up period remains limited to a mean of 20.9 months (8 to 39 months). Two children repaired with SOT had previous pulmonary artery bands (neonatal coarctation repair). All children were successfully discharged home with a mean postoperative Qp:Qs of 1.09:1. One pacemaker was required, but this child has since reverted back to normal sinus rythm. CONCLUSIONS: Our initial experience using the SOT in the treatment of apical VSDs as a component of isolated mVSDs has been rewarding. All children are currently alive, in normal sinus rhythm, and have no residual significant left-to-right shunts.

Transcatheter closure of atrial septal defects using the Cardio-Seal implant.

OBJECTIVE: To review the outcomes of transcatheter closure of atrial septal defects using the Cardio-Seal implant. DESIGN: A prospective interventional study. SETTING: Tertiary referral centre. PATIENTS: The first 50 patients (median age 9.7 years) who underwent attempted percutaneous occlusion. INTERVENTIONS: Procedures were done under general anaesthesia and transoesophageal guidance between December 1996 and July 1998. MAIN OUTCOME MEASURES: Success of deployment, complications, and assessment of right ventricular end diastolic diameter, septal wall motion, and occlusion status by echocardiography. RESULTS: The median balloon stretched diameter was 14 mm. Multiple atrial septal defects were present in 11 patients (22%) and a deficient atrial rim (< 4 mm) in 19 (38%). In four patients (8%), a second device was implanted after removal of an initially malpositioned first implant. There were no significant immediate complications. All patients except one were discharged within 24 hours. At the latest follow up (mean 9.9 months) a small shunt was present in 23 patients (46%), although right ventricular end diastolic dimensions (mean (SD)) corrected for age decreased from 137 (29)% to 105 (17)% of normal, and septal motion abnormalities normalised in all but one patient. No predictors for a residual shunt were identified. Supporting arm fractures were detected in seven patients (14%) and protrusion of one arm through the defect in 16 (32%), the latter being more common in those with smaller anterosuperior rims. No untoward effects resulted from arm fractures or protrusion. There were no complications during follow up, although five patients (10%) experienced transient headaches. CONCLUSIONS: The implantation of the Cardio-Seal device corrects the haemodynamic disturbances secondary to the right ventricular volume overload, with good early outcome.

Selected aspects of cardiac tumors in infancy and childhood.

Considerable literature concerning cardiac tumors in infancy and childhood has accumulated summarizing the prevalence, histologic types, clinical presentation and outcome, and changing imaging algorithms [1, 7, 10, 14, 20, 24, 33, 37, 43, 48, 57, 58, 60-62, 67, 69, 70, 90, 105, 106, 110, 124, 139, 140, 142, 143, 149]. In this review, we focus on selected aspects of cardiac tumors in the neonate, infant, and child, with an emphasis on imaging modalities [6, 13, 15, 18, 21-23, 60, 71, 77, 80, 92, 98, 99, 103, 107, 112, 114, 119, 146]. Various types of primary cardiac tumors in childhood are discussed in this article.

A novel repair for patients with atrioventricular septal defect requiring reoperation for left atrioventricular valve regurgitation.

OBJECTIVE: Left atrioventricular valve regurgitation (LAVVR) is the most frequent indication for reoperation following atrioventricular septal defect (AVSD) repair. We estimate from our experience that within 10 years of initial repair, 14% of patients undergoing repair of atrioventricular septal defect (AVSD) require reoperation for this complication. We have developed a novel leaflet augmentation technique for LAVVR which may avoid failure of conventional repair and/or the need for valve replacement. METHOD: The novel technique consists of insertion of a glutaraldehyde-treated autologous pericardial patch to augment the bridging leaflets of the atrioventricular valve. We describe the outcome of eight patients in whom this technique was used and compared them to 68 other patients with AVSD undergoing reoperation for LAVVR by either conventional repair (n=54) or valve replacement (n=14). RESULTS: There were no early deaths or major complications following patch repair. The mean follow-up is 2.3 years (range 1-8.5 years) during which there were no late deaths. Two patients underwent reintervention at 3.5 and 5 years after patch repair for LAVVR and were successfully rerepaired. Mild residual LAVVR was seen at last echocardiography in six patients and mild to moderate in two. These results compare favorably with the 68 patients who underwent conventional surgery. The 3-year freedom from reoperation was 86% for both repair groups. Dysplastic valve tissue appears to be a major risk factor for failure of conventional repair or for valve replacement. Failure of conventional valve repair led to valve replacement in six of seven patients. CONCLUSIONS: For patients with late LAVVR after AVSD repair, pericardial leaflet augmentation is durable and may avoid failure of conventional repair or valve replacement in patients with dysplastic valves.

Influence of perioperative factors on outcomes in children younger than 18 months after repair of tetralogy of Fallot.

BACKGROUND: There has been a trend toward advocating earlier repair of tetralogy of Fallot and avoiding palliative procedures. The impact of this trend on perioperative outcomes has not been adequately documented. METHODS: Data from consecutive patients undergoing repair of tetralogy of Fallot at less than 18 months of age from May 1987 to September 1994 were reviewed. Independent factors associated with duration of stay in the intensive care unit were sought. RESULTS: Repair was performed in 89 infants at a median age of 13 months (range, 15 days to 18 months). A systemic-pulmonary artery shunt was present in 24% of patients. Mean duration of cardiopulmonary bypass was 119+/-37 minutes; 63% of patients received a transannular patch. There were six deaths (7%), all occurring less than 48 hours after repair. The median duration of stay in the intensive care unit was 5 days (range, 1 day to 8 months). Significant independent factors associated with increasing length of intensive care unit stay included younger age at repair, previous shunt, malformation syndrome, increased total dose and number of inotropic agents used, and respiratory complications. Hemodynamic variables serially recorded in the first 48 hours after repair were independently associated with death or prolonged (>7 days) duration of stay. CONCLUSIONS: Although outcomes after repair of tetralogy of Fallot in infants are good, both younger age at repair and previous palliative procedures were associated with longer duration of stay in the intensive care unit.